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Keywords:

  • enucleation;
  • evisceration;
  • exenteration;
  • eye amputation;
  • eye trauma;
  • health related quality of life;
  • neoplasm;
  • orbital implants;
  • painful blind eye;
  • perceived stress;
  • phantom eye syndrome;
  • phantom pain;
  • self-rated health;
  • visual hallucinations

Abstract.

  1. Top of page
  2. Abstract.
  3. The Eye Amputated – Consequences of Eye Amputation with Emphasis on Clinical Aspects, Phantom Eye Syndrome and Quality of Life
  4. List of Papers
  5. Acknowledgements
  6. Conflicts of interest
  7. Abbreviations
  8. Introduction
  9. Aims of the Ph.D. Study and its Hypotheses
  10. Materials and Methods
  11. Strengths and Limitations of Methods
  12. Results and Discussion
  13. Conclusions of Thesis and Future Perspectives
  14. Summary in Danish
  15. References
  16. Recommended Books and Articles for EA Patients, Relatives and all Health Professionals with Contact with EA Patients

In this thesis the term eye amputation (EA) covers the removing of an eye by: evisceration, enucleation and exenteration. Amputation of an eye is most frequently the end-stage in a complicated disease, or the primary treatment in trauma and neoplasm. In 2010 the literature is extensive due to knowledge about types of surgery, implants and surgical technique. However, not much is known about the time past surgery.

The purpose of the PhD thesis was:  To indentify the number of EA, the causative diagnosis and the indication for surgical removal of the eye, the chosen surgical technique and to evaluate a possible change in surgical technique in Denmark from 1996 until 2003 (paper I); To describe the phantom eye syndrome and its prevalence of visual hallucinations, phantom pain and phantom sensations (paper II); To characterise the quality of phantom eye pain, including its intensity and frequency among EA patients. We attempted to identify patients with increased risk of developing pain after EA and investigated if preoperative pain is a risk factor for a later development of phantom pain (paper III); In addition we wanted to investigate the health related quality of life, perceived stress, self rated health, job separation due to illness or disability and socio-economic position of the EA in comparison with the general Danish population (paper IV).

The studies were based on:  Records on 431 EA patients, clinical ophthalmological examination and an interview study of 173 EA patients and a questionnaire answered by 120 EA patients.

Conclusions:  The most frequent indications for EA in Denmark were painful blind eye (37%) and neoplasm (34%). During the study period 1996–2003, the annual number of eye amputations was stable, but an increase in bulbar eviscerations was noticed. Orbital implants were used with an increasing tendency until 2003. The Phantom eye syndrome is frequent among EA patients. Visual hallucinations were described by 42% of the patients. The content were mainly elementary visual hallucinations, with white or colored light as a continuous sharp light or as moving dots. The most frequent triggers were darkness, closing of the eyes, fatigue and psychological stress. Fifty-four percent of the patients had visual hallucinations more than once a week. Ten patients were so visually disturbed that it interfered with their daily life.

Approximately 23% of all EA experience phantom pain for several years after the surgery. Phantom pain was reported to be of three different qualities: (i) cutting, penetrating, gnawing or oppressive (= 19); (ii) radiating, zapping or shooting (= 8); (iii) superficial burning or stinging (= 5); or a mixture of these different pain qualities (= 7). The median intensity on a visual analogue scale, ranging from 0 to 100, was 36 [range: 1–89]. One-third of the patients experienced phantom pain every day. Chilliness, windy weather and psychological stress/fatigue were the most commonly reported triggers for pain. Factors associated with phantom pain were: ophthalmic pain before EA, the presence of implant and a patient reported high degree of conjunctival secretion. A common reason for EA is the presence of a painful blind eye. However, one third of these patients continue to have pain after the EA. Phantom sensations were present in 2% of the patients.

The impact of an eye amputation is considerable. EA patients have poorer health related quality of life, poorer self-rated health and more perceived stress than does the general population. The largest differences in health related quality of life between the EA patients and the general population were related to role limitations due to emotional problems and mental health. Patients with the indication painful blind eye are having lower scores in all aspects of health related quality of life and perceived stress than patients with the indication neoplasm and trauma. The percentage of eye amputated which is divorced or separated was twice as high as in the general population. Furthermore, 25% retired or changed to part-time jobs due to eye disease and 39.5% stopped participating in leisure activities due to their EAs.

Acta Ophthalmologica Thesis http://www.actaophthalmologica.com

The Eye Amputated – Consequences of Eye Amputation with Emphasis on Clinical Aspects, Phantom Eye Syndrome and Quality of Life

  1. Top of page
  2. Abstract.
  3. The Eye Amputated – Consequences of Eye Amputation with Emphasis on Clinical Aspects, Phantom Eye Syndrome and Quality of Life
  4. List of Papers
  5. Acknowledgements
  6. Conflicts of interest
  7. Abbreviations
  8. Introduction
  9. Aims of the Ph.D. Study and its Hypotheses
  10. Materials and Methods
  11. Strengths and Limitations of Methods
  12. Results and Discussion
  13. Conclusions of Thesis and Future Perspectives
  14. Summary in Danish
  15. References
  16. Recommended Books and Articles for EA Patients, Relatives and all Health Professionals with Contact with EA Patients

Marie Louise Roed Rasmussen University of Copenhagen Department of Neuroscience and Pharmacology Section of Eye Pathology Frederik den V's vej 11 DK-2100 Copenhagen Denmark

Contents
List of Papersiv
Acknowledgementsv
Abbreviationsvi
Abstract1
Key Words1
Introduction1
Aims of the Ph.D. Study and its Hypotheses5
Epidemiological Study – Paper I5
Interview Study – Paper II5
Interview Study – Paper III5
Questionnaire Study – Paper IV6
Materials and Methods6
Paper I6
Paper II–III6
Clinical examination6
Interview (Paper II and III)7
Analysis of data from interview7
Paper IV7
First and second questionnaire8
Analysis of data from first and second questionnaire8
Third questionnaire8
Analysis of data from third questionnaire9
Strengths and Limitations of Methods9
Clinical Examination10
Interviews10
Questionnaires10
Results and Discussion11
Epidemiological Study (Paper I)11
The causative diagnosis and indications for EA11
Possible change in surgical technique11
Conclusion on the basis of paper I12
Interview (Paper II and III)12
Phantom eye syndrome13
Phantom vision13
Phantom pain14
Phantom sensations16
Identification of risks factors for developing phantom pain16
Treatment of painful blind eye with EA17
Reassurance and treatment of phantom eye syndrome17
Conclusion on the basis of paper II and III18
Self-Administered Questionnaires (Paper IV)18
Emotional and social consequences of EA18
Health related quality of life, perceived stress, self-rated health and job-separation due to illness and disability18
Conclusion on the basis of paper IV21
Conclusions of Thesis and Future Perspectives21
Summary in Danish22
References23
Recommended Books and Articles for EA Patients, Relatives and All Health Professionals with Contact with EA Patients26

List of Papers

  1. Top of page
  2. Abstract.
  3. The Eye Amputated – Consequences of Eye Amputation with Emphasis on Clinical Aspects, Phantom Eye Syndrome and Quality of Life
  4. List of Papers
  5. Acknowledgements
  6. Conflicts of interest
  7. Abbreviations
  8. Introduction
  9. Aims of the Ph.D. Study and its Hypotheses
  10. Materials and Methods
  11. Strengths and Limitations of Methods
  12. Results and Discussion
  13. Conclusions of Thesis and Future Perspectives
  14. Summary in Danish
  15. References
  16. Recommended Books and Articles for EA Patients, Relatives and all Health Professionals with Contact with EA Patients

This thesis is based on the following publications that will be referred to by their Roman numerals:

I. Rasmussen MLR, Prause JU, Johnson M. and Toft PB. Review of 345 eye amputations in the period 1996-2003. Rigshospitalet, Denmark. Acta Ophthalmol. 2010: 88: 218–221.

II. Rasmussen MLR, Prause JU, Johnson M. and Toft PB. The phantom eye syndrome. Types of visual hallucinations and related phenomena. Ophthalmic Plastic and Reconstructive Surgery. 2009: 25(5):390–393.

III. Rasmussen, MLR, Prause, JU, and Toft, PB. Phantom pain after eye amputation. An interview study of 173 eye amputated patients. Accepted for publication in Acta Ophthalmol. 2010.

IV. Rasmussen MLR, Ekholm O, Prause JU and Toft PB. Quality of life of eye amputated patients. Acta Ophthalmol. Submitted 18. June 2010.

Supervisors:

Jan Ulrik Prause, MD, Professor, Dr.Med. Sci., Dr. H.C., University of Copenhagen, Department of Neuroscience and Pharmacology, Section of Eye Pathology.

Peter Bjerre Toft, MD, Dr.Med.Sci. University of Copenhagen, Department of Ophthalmology, Rigshospitalet.

Finn Kamper-Jørgensen, MD, Lic.Med. ph.d. University of Southern Denmark, Institute of Public Health.

Acknowledgements

  1. Top of page
  2. Abstract.
  3. The Eye Amputated – Consequences of Eye Amputation with Emphasis on Clinical Aspects, Phantom Eye Syndrome and Quality of Life
  4. List of Papers
  5. Acknowledgements
  6. Conflicts of interest
  7. Abbreviations
  8. Introduction
  9. Aims of the Ph.D. Study and its Hypotheses
  10. Materials and Methods
  11. Strengths and Limitations of Methods
  12. Results and Discussion
  13. Conclusions of Thesis and Future Perspectives
  14. Summary in Danish
  15. References
  16. Recommended Books and Articles for EA Patients, Relatives and all Health Professionals with Contact with EA Patients

First of all - I would like to thank all the EA patients who took part in this investigation. Without your time and confidence this would never have been successful.

The present thesis is based on a continuous collaboration with Jan Ulrik Prause since 2004 when I was a young medical student. Jan have guided in many aspects of life and science, not only as my supervisor - but as a teacher, mentor, friend and as a father. I have had the privileged to be your last PhD. student in a stage of life where you always had time for discussions and conversations. It has been six intense years and I would like to express my deepest gratitude.

My deepest thanks to Peter Bjerre Toft - you have been an invaluable mentor and my best critics. I am indebted to Martin Johnson, for your guidance into the world of occularists and manufacturing of all kinds of prothesis, as well as for your optimism and help all along the way. Thank to Finn Kamper-Jørgensen and Ola Ekholm at the National Institute of Public Health in Denmark for their introduction into public health, supervision and for brilliant statistical assistance. My warmest thanks to Steffen Heegaard for his friendship, help and education into ocular pathology and scientific practice.

Constructive collaboration and support was provided by friends and collages at the Eye Department at Rigshospitalet and the Eye Pathology Institute. It has been a pleasure working with you.

Special thanks go to the secretaries at Rigshospitalet: Ann-Berit Fonnesgaard, Katja Ryba, Katja Vaag Mortensen, Charlotte Michaelsen, Birte Birkestrøm Due, Anne Sander and Ditte Walther Jensen - without your help the project had never been realized. Niels Rath - thank for support in the beginning of the project, especially when all the patients came to the clinical examination.

Finally I would like to thank my husband Toke for his patience with my ambitious drive into science and his loving support. - I am fortunate to have you in my life. Love to my good friend Sara Malou Strandvad and my sister Anne Sofie Roed Rasmussen for all kinds of support and inspiration into the more sociological areas of science.

I would like to thank James G. Willis, Shriley Weyland and Ian McRobbie, for permission to use of the figure ‘‘Cycle of change’’ in the thesis.

This study was made possible by grants from: The VELUX foundation, the Danish Eye Research Foundation, Aase and Ejnar Danielsens foundation, Fabrikant Einar Willumsens Foundation, Danish Ophthalmological Society, Emmy Lange f. Kamps foundation, Grosser Chr. Andersen and Wife Ingeborg Andersen, f. Schmidts foundation, Alfred Helsted and wife Eli Møllers foundation, Svend Arvid Schrøder and wife Ketty Lydia Larsen Schrøders foundation

Marie Louise Roed Rasmussen, September 2010.

Abbreviations

  1. Top of page
  2. Abstract.
  3. The Eye Amputated – Consequences of Eye Amputation with Emphasis on Clinical Aspects, Phantom Eye Syndrome and Quality of Life
  4. List of Papers
  5. Acknowledgements
  6. Conflicts of interest
  7. Abbreviations
  8. Introduction
  9. Aims of the Ph.D. Study and its Hypotheses
  10. Materials and Methods
  11. Strengths and Limitations of Methods
  12. Results and Discussion
  13. Conclusions of Thesis and Future Perspectives
  14. Summary in Danish
  15. References
  16. Recommended Books and Articles for EA Patients, Relatives and all Health Professionals with Contact with EA Patients

Bodily pain (BP)

Eye Amputation (EA)

General health perceptions (GH)

Health-Related Quality of Life (HRQOL)

Mental health (MH)

Perceived Stress Scale (PSS)

Physical functioning (PF)

Relative Risk (RR)

Role limitations due to emotional problems (RE)

Role limitations due to physical problems (RP)

Short Form-36 (SF-36)

Social functioning (SF)

Visual Analogue Scale (VAS)

Vitality (VT)

Introduction

  1. Top of page
  2. Abstract.
  3. The Eye Amputated – Consequences of Eye Amputation with Emphasis on Clinical Aspects, Phantom Eye Syndrome and Quality of Life
  4. List of Papers
  5. Acknowledgements
  6. Conflicts of interest
  7. Abbreviations
  8. Introduction
  9. Aims of the Ph.D. Study and its Hypotheses
  10. Materials and Methods
  11. Strengths and Limitations of Methods
  12. Results and Discussion
  13. Conclusions of Thesis and Future Perspectives
  14. Summary in Danish
  15. References
  16. Recommended Books and Articles for EA Patients, Relatives and all Health Professionals with Contact with EA Patients

Each year approximately 150 persons loose an eye in Denmark (Hansen et al. 1999). To lose an eye is often the last surgical attempt in a complicated series of disease treatments, the primary necessary treatment of severe ocular trauma or a step in treatment of intraocular neoplasm.

We have termed these patients eye amputated (EA) (Roed Rasmussen et al. 2009; Rasmussen et al. 2010). Immediately the term ‘Eye amputated’ may induce an offensive reaction in ophthalmologists. However, the term is relevant for the patient and is inducing a more faceted reaction towards the subject among doctors. The patients have lost a part of their body, like amputation of an ear, a leg or a hand. The patients have not only been enucleated or eviscerated; they have lost an eye – a part of their vision, a part of their face and for some, a part of their personality.

The mean annual gender- and age-adjusted incidence of EA in a Western country has been found to be between 1.16 and 2.8 per 100 000 inhabitants (Erie et al. 1992; Sigurdsson et al. 1998). Consequently there should be around 3000 people in Denmark who have lost an eye. However the exact number in Denmark is not known.

In relation to the EA it is important to differentiate between the disease that leads to loss of ocular function, i.e. the causative diagnosis and the condition that leads to the final surgery, i.e. the indication for EA. It may be the same disease like severe trauma and malignant neoplasm. However, in other cases they may be different and be separated by large time intervals.

Former studies have grouped the causative diagnosis into the following categories: Neoplasms, infections and inflammations, glaucoma, traumas, retinal detachment, surgical complications, perinatal diseases and other causes (Lim & Cinotti 1976; Sigurdsson et al. 1998; Hansen et al. 1999).

Indication for EA has been listed as; Neoplasm, acute trauma, painful blind eye, infection and inflammations, disfiguring blind eye and other reasons (Lim & Cinotti 1976; Sigurdsson et al. 1998; Hansen et al. 1999; Shoamanesh et al. 2007). However, it is possible to have more than one indication for EA. National differences in incidences of diseases and their treatment (or lack of treatment) is present and give different indications for EA around the world (Bal et al. 2007; Chaudhry et al. 2007).

The most frequent neoplasms leading to EA are mainly choroidal melanomas in adults and retinoblastoma in children (Lim & Cinotti 1976; Hansen et al. 1999; Moshfeghi et al. 2000).

Trauma to the eye is related to the male sex in all ages and emerges in relation work, home, traffic accidents, sports, leisure and fireworks (Sigurdsson et al. 1998; Hansen et al. 1999; Chaudhry et al. 2007). By definition the eye have to be surgically removed within the first 14 days after the initial trauma to be an indication for EA (Sigurdsson et al. 1998).

Painful blind eye can occur after various diseases and is often the end point in a long clinical journey with various treatments and surgical procedures (Sigurdsson et al. 1998; Shah-Desai et al. 2000; Merbs 2006).

Infection and inflammations is a diverse group consisting of corneal ulcers, endophthalmitis and panophthalmitis. Corneal ulcers are mostly due to herpes simplex keratitis but also non herpetic chronic ulcerative keratitis, bullosa keratitis, acanthamoeba keratitis, radiation keratitis and streptococcus keratitis. Agents coursing endopthalmitis and panophthalmitis that lead to EA are mostly gram-positive cocci and fungal infections. However, infectious agents can only be verified in <20% of cases (Sigurdsson et al. 1998; Hansen et al. 1999).

Disfiguring blind eye, as indication for EA, has been used in Denmark when the patients were not able to use a cosmetic shell or had a request of having their eye removed. Phthisis bulbi is a shrunken non functional eye. Phthisis can develop after trauma, glaucoma, infection, inflammation and neoplasm treated with radiation. Phthisis can end up with an EA because of a disfiguring or painful blind eye.

Three types of surgeries are applied when performing EA: evisceration, enucleation and exenteration. Evisceration describes the removal of the intraocular content leaving the sclera with all the muscles intact in the orbit. Enucleation refers to the surgical removal of the entire globe including the sclera. Exenteration of the orbit refers to the surgical removal of the eye and the affected orbital contents with or without the eyelids (Levin & Dutton 1991; Rahman et al. 2005a).

Evisceration is today the most used surgical procedure. Enucleation is used to treat malignant neoplasms, severe ocular trauma with ruptured globe – were the risk of sympathetic ophthalmia is a concern – phthisis bulbi, chronic inflammation of the sclera and scleromalasia (Moshfeghi et al. 2000). Exenteration of the orbit is restricted to patients suffering from malignant neoplasms in the orbit or eyelids where surgery cannot spare the eye and rarely in severe infections (Levin & Dutton 1991; Rahman et al. 2005a).

The incidence of sympathetic ophthalmia after traumas is between 0.28% and 1.9% (Albert & az-Rohena 1989; Bilyk 2000). To avoid the risk of sympathetic ophthalmia the eye is enucleated within the first 2 weeks after severe ocular trauma with ruptured globe. However, the risk of sympathetic ophthalmia after evisceration, as compared to enucleation remains unsolved.

After evisceration and enucleation an implant is often placed into the orbit to fill out the lost volume and to get better movement of the prothesis (Fig. 1). The first orbital implants were introduced by Mules (1885), using hollow glass, gold or silver spheres (Mules 1885; Sami et al. 2007). Since then all possible types of materials and shapes have gained favour for use in orbital implants. The ideal implant should have many characteristics: adequate volume replacement, good motility transmitted to the prostheses, adequate support for the prostheses, low rate of complications, low economical cost, technically easy to implant, biocompatibility and non-degradability (Christmas et al. 1998; Chalasani et al. 2007).

image

Figure 1.  (A) Implant and prothesis in situ. (B) Implant, prothesis, peg and sleeve in situ. (C) Close up figure of prothesis, peg and sleeve.

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Since 1990 state of the art has been the porous implant (Perry 1990). Porous implants resembling the trabecular structure of human bone, allowing fibro-vascularisation (Ferrone & Dutton 1992; Shields et al. 1992; Massry & Holds 1995; Christmas et al. 1998; Custer 2000; Custer et al. 2003; Sami et al. 2007; Shoamanesh et al. 2007). Today the most used porous implant (and most expensive) is consisting of hydroxyapatite. Hydroxyapatite is a calcium phosphate salt, which is also present in the mineralized human bone (Moshfeghi et al. 2000; Sami et al. 2007). Another non porous type of implant with is frequently used is autologous fat-dermis from the hip (Nunery & Hetzler 1985; Meltzer 1992). Both hydroxyapatite and fat-dermis have been proven to be safe in primary as well as secondary procedures (Bosniak 1985; Nunery & Hetzler 1985; Borodic et al. 1989; Massry & Holds 1995; Toft et al. 2010).

Wrapping may be used to cover the porous implant prior to implantation. Wrapping reduces migration and exposure of the implant. Furthermore the wrapping lies as an extra layer between the implant and the thin conjunctiva. If possible, autologous sclera is used as wrapping material to further reduce the amount of complications (Perry 1990; Remulla et al. 1995). However, numerous types of materials have been used (Soll 1974; Morax 1990; Perry 1990; Jordan et al. 1995; Beaver et al. 1996). Today the patient’s own sclera and vicryl mesh is the most used materials for wrapping in Denmark.

For patients with porous implants it is possible to improve motility of the prosthesis, by drilling a sleeve into the implant and then by using a peg – coupling the implant and prothesis together (Fig. 1B, C). This pegging procedure is safer if the patient demonstrates adequate vascularisation of the implant by MRI before pegging (De Potter et al. 1992; Sami et al. 2007; Shoamanesh et al. 2007). More recently, primary placement of the titanium sleeve in porous implants have been suggested to reduce cost and complication rates (Liao et al. 2005a,b).

Artificial eyes (prostheses) serve two purposes an aesthetic and a functional. The artificial eye serves to mimic the natural eye and make the face complete but it also serves as volume substitution.

Artificial eyes are made by two different techniques and materials; glass and acrylic. In Venice the first glass eye were made in 1579, but these eye were very fragile thin shells, and not comfortable at all (Kelley 1970). Not many modifications came before the German occularist Ludwig Müller-Uri in 1850 developed a superior type of glass. The German glass consists of a mixture of kryolit and crystal, and is manufactured by heating up a preshaped hollow glass ball with a precoloured iris, and then by blowing, moulding the glass prosthesis to the shape of the anophthalmic socket (Kelley 1970; Müller Uri 2010; Fig. 2),

image

Figure 2.  Preshaped hollow glass balls in different colors before manufacturing of prothesis and blowing of the glass into right shape.

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During the Second World War the need for artificial eyes raised dramatically and among the Allied soldiers, but since only the Germans occularist had the knowledge of manufacturing and blowing glass it was only the occupied countries and Germany that could be supplied. This created a need for a new type of material to make artificial eyes, and the US government established a research program to develop its own glass. But they never came up with something useful. At the same time methyl-methacrylate was used in the dentures and was well tolerated by human tissue. And in 1944 the first acrylic eye (poly-methyl-methacrylate) was made in the US (Kelley 1970; Lyberg & Grip 1990; Jahrling 1999).

Acrylic eyes are tailored by two ways. The most accurate is the impression method. By this method each prostheses is moulded individually to the patient’s socket and eye colour of the opposite eye. The occularist take an impression of the socket in silicone, and creates a vax impression similar to the shape of the socket. The vax impression can be moulded to optimize the fit. As the fitting proceeds, the model is shaped to form the lid opening to match the normal eye as closely as possible. At the same time the iris is hand painted, duplicating the iris on the opposite site. The placement of the iris is determined in the vax. Once the requirements have been met, the model is cast in a plaster mould and duplicated in white plastic. All the details, including the small blood vessels, are then painted to match the natural eye (Fig. 3). After the final surface painting process, the eye is covered with a layer of clear plastic to seal in the artwork and to give the eye prosthesis a rounded shape. Once finished, it is ready to be inserted into the socket for a final fitting evaluation (Allen 1970; Lyberg & Grip 1990; Jahrling 1999). The other technique applied is by taking prefabricated (a stock eye/ready-made eye) and then trying different shapes into the socket until a satisfactory lid positioning is achieved. This can be done over one visit alone and is therefore preferred in some outlying areas (Kelley 1970; Jahrling 1999).

image

Figure 3.  Painting of acrylic iris, and impression of the socket in silicone (green), vax impression (reed), iris button, and plaster mould.

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In Denmark all the mentioned types of materials and methods for prosthesis are used. There are advantages and disadvantages of both types of artificial eyes. Glass is a very clean material with no allergic reactions. It only takes an hour to make a glass prosthesis and it can be made almost everywhere. Glass prostheses are hollow and they are therefore optimal for patients without an implant – which creates a need for very large prostheses. But glass is fragile, cannot be moulded after it has been made and is very chilly for the patient to wear in cold or windy weather. Furthermore, the glass surface is broken down with time like dental carries, and therefore the glass prothesis has to be replaced every one to second year (Lyberg & Grip 1990).

Acrylic prosthesis has very large mechanically resistance and can therefore last 5–10 years. After it have been made it is possible to mould it several times. Because the prosthesis is moulded after the socket it is possible to make a ‘flush fit’ to the socket and by that transferring maximum motility. Acrylic prosthesis can be drilled to fit the pegging system. With acrylics it normally takes two till three visits to create a prothesis. Acrylics can theoretically course allergic reactions. To maintain the surface smooth and clean the prosthesis has to be polished once a year (Lyberg & Grip 1990).

Cosmetically disfigured blind eyes (non-painful) may be treated by wearing coloured contact lenses or cosmetic shells. Coloured contact lenses (corneal and scleral) are used by patients without any significant complications. However, they may be costly. Cosmetic shells are very thin acrylic prothesis and are worn as a contact lens. The shell may add a little extra volume. Some patients feel discomfort by wearing theses shells, and everything should be fitted perfectly avoid it. It may take months to adapt to wear these shells – but ophthalmologist should encourage their patients to give it a serious try. The patient may start out wearing the shell for 30 min/day and gradually increases the period until the patient are able to wear it the time he or she are away from their home during the day time. Some patients are able to wear the shell 24 hr a day. Various lubricants may be useful in the adapting process and a conjunctival flap covering the cornea may be useful. By these techniques it is possible to avoid EA in many of these patients (Lascola 1970; Le Grand 1970; Foss et al. 1994; Jahrling 1999; Smith 1999).

If a patient is having an orbital exenteration or a severe contraction of the socket the use of a normal artificial eye is impossible. Instead, it is possible to create an exo-prothesis, a facial prothesis including eyelids. It consists of silicone and can be attached to spectacles, by glue to the skin or retained directly to the orbit by bone anchored magnets and magnets attached to the prothesis.

Complications after EA may be grouped according to time of their appearance: surgical, postoperative and late complications. Surgical complications are loss of muscles, destruction of tissue and haemorrhage. Very rare surgical complications are removal of the wrong eye and creation of fistulae between the sinuses and orbit (Moshfeghi et al. 2000). Postoperative complications occur within the first days to months after surgery and includes; infections, haemorrhage and wound dehiscence (Meltzer 1992; Moshfeghi et al. 2000).

Complications related to implants are included in the group of late EA complications. In 2010 most patients have an orbital implant and complications only related to the EA are very difficult to separate from complications related to the implant. However, patients without an implant might suffer greater cosmetic complications over time. Comparing enucleated to eviscerated patients, the eviscerated are associated to a reduced incidences of postoperative complications because evisceration causes less disruption of the normal anatomic relationships within the orbit (Dortzbach & Woog 1985; Nakra et al. 2006).

Late complications normally occur months to years after surgery: implant exposure/extrusion, implant migration, orbital cyst formation, orbital volume deficiencies, lid abnormalities, discharge and phantom eye syndrome (Meltzer 1992; Moshfeghi et al. 2000; Soros et al. 2003; Chalasani et al. 2007; Shoamanesh et al. 2007). Sulcus superior syndrome (lax socket syndrome/anophthalmic socket syndrome/post enucleation syndrome) covers the most frequent complications after EA. It includes enophthalmos, exophthalmos, ptosis, deep superior sulcus, ectropion, entropion, lagophthalmos, inadequate lower fornix and lax/sagging lower fornix (Paris & Spohn 1980; Willis et al. 1990; Migliori 1999; Fig. 4). The patomechanism of the sulcus superior syndrome is almost unknown (Soll 1982; Kaltreider et al. 1987; Kronish et al. 1990a,b). However, parts of the sulcus superior syndrome will arrive over years, no matter whether there is an implant or not (Fig. 5). Age, gravity and the weight of the prothesis will result in a sagging lower lid which creates space for the implant to migrate. This will deepens the superior sulcus and give an impression of ptosis. This often results in an unsatisfactory cosmetic appearance leading to fitting of new, more complex and heavier prothesis – which will increase the tendency to sagging lower lid, may course ectropion or entropion of the lower lid, more migration of soft tissue, enophthalmos and hypoglobus. This is called the cycle of change (Willis et al. 1990).

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Figure 4.  EA patient with sulcus superior syndrome. Note the deep superior sulcus, ptosis, lax lower eye lid and mild hypoglobus.

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Figure 5.  Cycle of change (with permission from Willis).

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Phantom pain is a known sequela to amputations in all parts of the human body. Up to 80% of patients with limb amputation experience phantom pain (Flor 2002), and as many as 70% are troubled even 25 years after amputation (Sherman et al. 1984). Phantom sensations and pain after amputation have been described in various organs: limbs (Sherman et al. 1984; Jensen et al. 1985; Flor 2002), mammae (Krone-Munzebrock 1950; Kroner et al. 1989), rectum (Ovesen et al. 1991), larynx (Mal & Baldwin 2009), teeth (Marbach 1996), parts of the face (Hoffman 1955), bladder (Bors 1951), uterus and even the appendix (Ramachandran & Hirstein 1998).

Phantom eye syndrome is defined as any sensation that a patient reports as originating in the eye despite it being amputated. Phantom eye syndrome includes phantom vision, phantom pain and phantom sensations.

Phantom vision is a visual hallucination (Cohn 1971). A hallucination is a sensory experience, possessing the compelling sense of reality of a true perception, but occurring without external stimulation of the relevant sensory organ (Menon et al. 2003; Wilkinson 2004). Visual hallucinations are in this thesis divided into:

  •  Elementary visual hallucinations: These include all simple visual phenomena lacking meaning and form such as simple geometric shapes, points of light, spots, and line elements (Kolmel 1984; Wilkinson 2004)
  •  Complex visual hallucinations: These include structured objects and scenes, sometimes related to past experience (Menon et al. 2003; Wilkinson 2004)
  •  Other visual hallucinations: These include the sensation of seeing with two eyes, extreme sensitivity to light, difficulties focusing because of irritation from the non-existing eye, and other sensations which cannot be classified as elementary or complex visual hallucinations.

Phantom eye pain is defined as any painful sensations that the patients refers to the amputated eye. In addition, a clinically significant cause for pain in the remaining orbit or surroundings should not be present. The incidence of phantom pain has been reported to range from 25% to 28% (Nicolodi et al. 1997; Gerding et al. 2003; Soros et al. 2003, 2005).

We define ophthalmic pain as any pain from the ophthalmic region, i.e. the orbit with its content, the preseptal orbit including eyelids. Exenterated patients have special complications due to the more severe trauma of the surgery. A discussion of these complications is outside the scope of this thesis.

Aims of the Ph.D. Study and its Hypotheses

  1. Top of page
  2. Abstract.
  3. The Eye Amputated – Consequences of Eye Amputation with Emphasis on Clinical Aspects, Phantom Eye Syndrome and Quality of Life
  4. List of Papers
  5. Acknowledgements
  6. Conflicts of interest
  7. Abbreviations
  8. Introduction
  9. Aims of the Ph.D. Study and its Hypotheses
  10. Materials and Methods
  11. Strengths and Limitations of Methods
  12. Results and Discussion
  13. Conclusions of Thesis and Future Perspectives
  14. Summary in Danish
  15. References
  16. Recommended Books and Articles for EA Patients, Relatives and all Health Professionals with Contact with EA Patients

Epidemiological study – Paper I

During the period 1975–1996 a shift in type of surgery applied for EA was observed (Hansen et al. 1999). This was associated to a change in treatment of diseases like glaucoma.

My hypothesis I is that in the time period 1996–2003 there was no change in number of EA, causative diagnosis and the indication for surgery or surgical technique, except for the use of orbital implants.

The aim of investigation I was to indentify the number of EA, the causative diagnosis and the indication for surgery, the chosen surgical technique and to evaluate a possible change in surgical technique in Denmark from 1996 until 2003. Hydroxyapatite was approved for use in orbital implants in 1989 (Perry 1991), and introduced in Denmark a few years later. An analysis of the consequences of these shifts seems also to be of interest. The results of study I are given in paper I:

  •  Rasmussen MLR, Prause JU, Johnson M and Toft PB. Review of 345 eye amputations in the period 19962003. Rigshospitalet, Denmark. Acta Ophthalmol. 2010:88:218–221.

Interview study – Paper II

My hypothesis II is that the phantom eye syndrome is present, but significantly underreported. Different types of visual hallucinations might be present and can be categorised.

The aim of study II was to describe the phantom eye syndrome and its prevalence of visual hallucinations, phantom pain and phantom sensations. A special emphasise was given to the description of the content, presence of triggers and stoppers and the personal emotions related to the visual hallucination. The results of study II are given in paper II:

  •  Rasmussen MLR, Prause JU, Johnson M and Toft PB. The phantom eye syndrome. Types of visual hallucinations and related phenomena. Ophthalmic Plastic and Reconstructive Surgery. 2009:25:390–393.

Interview study – Paper III

EA has been used to treat patients with a painful blind eye.

It is my hypothesis III that most EA patients did not have phantom pain and that the pain frequency and intensity are low.

The aims of study III were to characterise the quality of phantom eye pain, including its intensity and frequency among EA patients. Possible triggers and relievers of phantom eye pain were also investigated. Did the patients take any medication against their pain and did it have any effect. We attempted to identify patients with increased risk of developing pain after EA and investigated if preoperative pain is a risk factor for a later development of phantom pain. Furthermore, the efficacy of EA in relieving pain associated with a painful blind eye was assessed.

The results of study III is given in paper III:

  •  Rasmussen MLR, Prause JU and Toft PB. Phantom pain after eye amputation. An interview study of 173 eye amputated patients. Accepted for publication in Acta Ophthalmol. 2010.

Questionnaire study – Paper IV

To lose an eye can cause a persistent drop in health related quality of life and an increase in perceived stress. Many of the EA patients are very troubled by the loss of their eye, and it takes very long time to accept to wear the prothesis and the permanent change in their face and appearance – some of the patients newer accept this.

My hypothesis IV is that EA has wide spread and permanent effect on patients job situation and socio-economic position. The aims of study IV were to investigate the health related quality of life perceived stress, self rated health, job separation due to illness or disability and socio-economic position of the EA in comparison with the general Danish population. The results of study IV is given in paper IV:

  •  Rasmussen MLR, Ekholm O, Prause JU and Toft PB. Quality of life of eye amputated patients. Acta Ophthalmol. Submitted 18 June 2010.

Materials and Methods

  1. Top of page
  2. Abstract.
  3. The Eye Amputated – Consequences of Eye Amputation with Emphasis on Clinical Aspects, Phantom Eye Syndrome and Quality of Life
  4. List of Papers
  5. Acknowledgements
  6. Conflicts of interest
  7. Abbreviations
  8. Introduction
  9. Aims of the Ph.D. Study and its Hypotheses
  10. Materials and Methods
  11. Strengths and Limitations of Methods
  12. Results and Discussion
  13. Conclusions of Thesis and Future Perspectives
  14. Summary in Danish
  15. References
  16. Recommended Books and Articles for EA Patients, Relatives and all Health Professionals with Contact with EA Patients

All parts of the thesis and the four papers (I–IV) are based on the same basic patient population with later selection of those who came to the clinical examination and drop-outs of patients.

In Denmark, every hospitalized patient is registered under a specific civil personal number (CPR) and is entered in the hospital database according to causative diagnoses and surgical procedures.

The hospital database at Rigshospitalet, Copenhagen, was screened using surgery codes (ICD 10) for patients who had undergone bulbar evisceration, enucleation or orbital exenteration during the period 1996–2003. Rigshospitalet is a public and national specialist hospital which serves eastern Denmark, with a recruitment area including eastern Denmark (approximately 2–3 million people). Nearly all cases of EA in eastern Denmark were treated at Rigshospitalet in this time period.

Paper I

A total of 431 patient records were identified as matching the inclusion criteria. All their medical records were collected and reviewed for data on: gender; age; causative diagnosis (the disease process leading to the clinical condition responsible for indications for EA); indications for EA (indications for surgery as stated in the patient’s record); type of surgery; time since surgery, whether an implant (hydroxyapatite) was used and if radiotherapy has been applied to the region.

If there was a discrepancy between the operation code and data from the record, the final causative diagnosis was based on the clinical history and the results of examinations, investigations and histopathological diagnosis. If a record was missing, information from the hospital database was used and, when possible, the patient and his or her family were questioned during a clinical examination.

Paper II–III

In September 2005 we started study II–IV. At this point of follow-up, 126 were deceased, seven were under the age of 18 years, eight had left the country, and 23 patients were from Greenland or the Faeroe Islands and thus were excluded. The remaining 267 patients were invited to participate; 59 patients refused, 28 did not respond to correspondence, and seven did not come to their interview appointment; 173 patients agreed to participate and had clinical examinations as well as an individual interview.

Clinical examination

First it were assured that the prothesis were in the right position in the socket. After that a digital picture of the patient with both ‘eyes’ open and looking straight ahead was taken.

Signs of secretion or redness in the eye area were observed (yes or no).

Ptosis was assessed by objective grading into four categories (normal, almost not visible, clearly visible and severe).

For the amputated eye we recorded:

  •  Exophthalmos, were measured as a quantification by the observer grading into three categories (no, slight and moderate).
  •  Enophthalmos were measured in two ways; first as a quantification by the observer by grading into three categories (no, slight and moderate), and secondly by Hertels exophthalmometer (Supplemented by Lüddes ruler if the enophthalmos was severe).
  •  The globeline was evaluated by the observer into three categories (normal, deeper than normal and very deep).
  •  The placements of the amputated eye in the orbital area were assessed by:
    •  Horizontal displacement. Five categories (none, telecantus, telecorae, hypotelorisme, hypertelorisme).
    •  Vertical displacement (none, hyperglobus and hypoglobus).

For both eyes the vertical aperture (interpalperal distance) as well as the horizontal aperture was measured in mm.

The motility of the amputated eye as well as the remaining eye was estimated in the four prime directions of gaze by using a perimeter arc. The patient was asked to look in the four primary directions of gaze and the motility in degrees was estimated by inspecting the prosthesis motility when moving the contra lateral eye. The motility was observed when following a pencil light with the other eye and maximum movements of the prothesis. If a patient was EA on both sides it was only possible to measure maximum motility (Toft et al. 2010).

Visual acuity on the remaining eye was measured using Snellen charts.

The socket was investigated for:

  •  Infections (no signs of infections, slightly atrophic conjunctiva, signs of conjunctivitis, giant papillary conjunctivitis).
  •  Exposure (no exposure, small exposure and extrusion of the implant).
  •  Laxity of the lower fornix by simply pulling down the lower eyelid and observe how quickly it returned to the initial position (normal speed, some latency time, extended latency time and returns only after manipulation of the eye area).
  •  The depth of the fornix superior and inferior were measured with a special curved ruler to follow the natural curvature of the fornix (Fig. 6).
  •  The motility of the socket was measured horizontal and vertical by comparing with the motility of the prothesis (no movement, worse movement, the same degree of movement, better movement).
image

Figure 6.  The special curved ruler and its use.

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Interview (Paper II and III)

The interview was structured and conducted by the author (MLRR). The interview guide was developed on the basis of a review of the existing literature (Sherman et al. 1997; Craig 1999; Jensen & Nikolajsen 1999; Melzack & Katz 1999; Zakrzewska 1999; Soros et al. 2003) and pilot interviews of patients and doctors.

The following questions were asked in relation to phantom eye syndrome:

  •  Do you sometimes feel you are able to see with the eye that has been removed?
    •  If yes, what do you see?
    •  Do you have any other kind of visual sensations, experiences?
    •  How often do you have visual hallucinations?
    •  Are there triggers and stoppers related to your visual hallucinations?
    •  Do you find the phenomenon annoying, disturbing, pleasant, or is it not related to any emotions?
  •  Do you have pain in the eye or in the area around the missing eye?
  •  Did you have pain in the eye or in the area around the eye before you lost it?
  •  Do you have any other sensations located to the eye or eye surroundings?

The following questions were asked in relation to phantom pain:

  •  Did you have pain in the eye before the eye amputation?
  •  Do you have pain in the amputated eye at present or during the last year?
  •  If yes;
    •  The patient was asked to mark on three different visual analogy scale (VAS) the intensity of pain in the morning, at the middle of the day, and in the evening. The VAS was a continuous, horizontal scale (0–10), with the anchor points ‘no pain’ and ‘worst imaginable pain’. Total distance between statements was 10 cm.
    •  How often do you have this pain? Four categories (every day, some times a week, ones a week or less than every week)
    •  What kind of quality does the pain have? Three categories, more than one category were accepted. (i) Cutting, penetrating, gnawing or oppressive pain. (ii) Radiating, zapping or shooting pain. (iii) Superficial burning or stinging pain.
    •  Are there triggers of the pain?
    •  Are there relievers of the pain?
    •  Do you take any medication against the pain?
      •  If yes:
        •  What type?
        •  When was the last time you took medication?
        •  Did it help? Three categories (yes, to some extent, no).
  •  How often do you have secretion around the artificial eye or in the socket? Four categories (no secretion, infrequently, daily, constantly).
  •  If you had pain before the EA – how is the pain you feel at present? Four categories (the pain has gone, less, the same, worse).
Analysis of data from interview

The statistical program spss was used for the statistical analyses. Fisher’s exact tests were used for comparison of frequencies; for ordered groups with more than two categories the chi-square test for trends was used. T-tests for independent samples were used to evaluate any differences in sex, age or time since EA, and t-tests for paired means were applied for analysis of pain intensity; p < 0.05 (two-tailed) was considered statistically significant.

The statistical tests were used to determine if phantom vision and phantom pain after EA are associated with any of the following factors: gender, age, time since EA, type of surgery for EA, causative diagnosis, indication for EA, presence of an orbital implant, pegging, exposure of the orbital implant, radiotherapy to the region, visual acuity of the remaining eye, patient reports of pain before EA, and reports concerning secretion.

Paper IV

Three self-administered questionnaires were part of the investigation. The eligible population for this part of the study was the 173 patients that had an interview and examination in 2005.

All questionnaires were sent by ordinary mail with a cover letter and a prepaid envelope for the answer. Patients, who had not returned the questionnaire after 1 month, received a reminder along with a new copy of the questionnaire and a new prepaid envelope.

Around 4 weeks after the examination the patients received the first questionnaire; one patient died 3 weeks after examination. 158 completed and returned the first questionnaire. Three patients specified that they did not wish to receive any further questionnaires. Therefore only 155 patients received questionnaire number two, which was send out January 2007. In total 134 completed and returned the second questionnaire. In February 2008 a total of eleven patients have died. The third questionnaire was therefore sent out to 159 patients. In total 131 patients completed and returned the third questionnaire (Fig. 7).

image

Figure 7.  Flowchart of included participants in the questionnaire studies.

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First and second questionnaire

The content of the first and second questionnaire had character of a survey, with questions about: every day managing of the prothesis, interest in improved motility of the prothesis, cosmetic outcome, rehabilitation, response from surroundings, fear of blindness and if the eye amputation has changed their life.

The questionnaires were validated by individually instructing teen patients in filling out the questionnaire. Subsequently the patients completed the questionnaire alone. After that the author and the patients went thought all the questions, the interviewer trying to understand the patients understanding of all the questions.

Questions with misinterpretations, multiple or missing answer possibilities were not taken in to analysis.

Not all questions from questionnaire one and two are included in this thesis. However, I have chosen to bring some of the results because they are concurrent with our other findings and because it gives some interesting aspects to the discussion of the life after an EA. Furthermore it might show some areas that could need more attention in the rehabilitation. The questions that I have selected to include are the following:

Change of life after EA and fear of blindness: (1) ‘Have your life changed significantly after you lost your eye?’ (Yes; no) and (2) ‘Have you felt afraid of getting blind?’ (Yes; no)

Adaptation and rehabilitation after EA: (1) ‘How long time does it take physically to get used to wear the artificial eye?’ (1 week; 1 month; 6 months; 1 year; more than 1 year), (2) ‘How long time does it take mentally to get used to wear the artificial eye?’ (1 month; 2 till 6 months; 7 months till 2 years; more than 2 years; have not accepted this yet), (3) ‘How much does it fill in your every day life, that you have to wear an artificial eye?’ (Nothing at all; a little; it slightly comes to my interest; a good deal; a lot, it is something I use a much of energy on), (4) ‘How many times on a single day, do you think of your artificial eye?’ (Newer; one or two times; two till five; five till ten; all the time I am awake) and (5) ‘Do you feel that people are watching you in the street?’ (Yes; no)

Satisfaction with cosmetic outcome: (1) ‘How satisfied are you with the cosmetic result, including the artificial eye?’ (Very satisfied; satisfied; dissatisfied and very dissatisfied) and (2) ‘Do you think the artificial eye looks like you other normal eye?’ (Yes; no; blind)

Need for contact with other EA patients: (1) ‘Have you talked to other people who have lost an eye? (Yes; no) and (2) ‘Would you like to talk to others who have lost an eye?’ (Yes; no)

Analysis of data from first and second questionnaire

Data from first and second questionnaire are reported as percentages, and are not correlated to any external variables. Data should be regarded as a survey. There are a fluctuating number of respondents due to the fact that not all participants have replied all questions.

Third questionnaire

The third questionnaire included; Short Form-36 (SF-36), Perceived Stress Scale (PSS) and questions about self-rated health, job separation due to illness or disability as well as socio-economic position.

The SF-36 is an instrument that measures HRQOL. It contains 36 items measuring eight dimensions of health and well-being: ‘physical functioning’ (PF), ‘role limitations due to physical problems’ (RP), ‘bodily pain’(BP), ‘general health perceptions’(GH), ‘vitality’ (VT), ‘social functioning’ (SF), ‘role limitations due to emotional problems’(RE) and ‘mental health’(MH). Each dimension was scored from zero (worst possible health state) to 100 (best possible health state) (Bjorner et al. 1997). The Danish adapted version has been tested and validated in 1998 (Bjorner et al. 1998a,b,c). The self-administrated SF-36 have been used in various ophthalmological studies (Cruickshanks et al. 1999; Chia et al. 2003, 2004, 2006b; Varma et al. 2006; Elliott et al. 2009; Gall et al. 2009) and have been found useful in older patients as well as in visually impaired (Chia et al. 2006a). From earlier studies we knew that the majority of our patients had a good visual acuity in the remaining eye (Roed Rasmussen et al. 2009), and for that reason we did not choose the magna print version of SF-36.

The PSS is a 10-items measure of the degree to which respondents appraise stressful situations that occurred during the past month (Cohen et al. 1983; Cole 1999; Sewitch et al. 2001). The items are scored on a five-point scale from zero to four, the total score provides a global measurement of the extent to which an individual feels overwhelmed by stressful situations that occurred in the past month. Total scores range from zero to 40; higher scores indicate greater perceived stress (Cohen et al. 1983; Cole 1999).

Questions about self-rated health, job separation due to illness or disability and socio-economic position is taken from the Danish Health Interview Survey 2005 (Ekholm et al. 2009). Self-rated health is measured in two ways. First as a question: ‘In general, would you say your health is:’ (Excellent, very good, good, fair, poor). Secondly the respondents are asked to indicate how they rate their own health state that day, by drawing a line on vertical 14 cm visual analogue scale (VAS) which quantifies from 0 = worst imaginable health to 100 = best imaginable health. Self-rated health on a VAS is a part of EuroQol EQ-5D scales (EQ-VAS). The EQ-5D scale has earlier been used in Danish studies (Kessing et al. 2006; Vogel et al. 2006), and have been translated and validated in many countries (http://www.euroqol.org).

Job separation due to illness or disability was indentified through the following two questions: (1) ‘Have you ever needed to change your working hours to part time, quit a job or change job or work tasks due to disease, disorder, illness or injury?’ (Yes, worked part time; yes, changed job or work tasks; yes, stopped working; yes, first changed job or work tasks and then stopped working; no). (2) ‘If the answer is yes to the previous question – was this because of eye disease?’ (Yes; no).

Marital status was addressed by the question: ‘What is your marital status?’ Socio-economic position was measured with one question about the subject’s own occupation; ‘What is your occupation?’ Two questions were used to address leisure activities. (1) ‘Are there leisure activities that you no longer take part in due to your lost eye?’ (Yes; no) (2) ‘Please write down the types of activities’.

Analysis of data from third questionnaire

Comparison group: Data from the EA patients were compared with data from the nationally representative Danish Health Interview Survey 2005 (Ekholm et al. 2009). The Danish National Institute of Public Health, University of Southern Denmark, has carried out national representative health interview surveys among adult Danes since 1987. The survey in 2005 was based on a region-stratified random sample of 21 832 Danish citizens aged 16 years or older. Data were collected via face-to-face interviews at the respondents’ homes (response rate: 67%). Following the interviews, all respondents were asked to complete a self-administered questionnaire. Information about sociodemographic characteristics and job changes due to illness or disability were collected via face-to-face interviews and the SF-36, the PSS and self-rated health on a VAS were included in the self-administered questionnaires.

Statistical analyses of data from third questionnaire: Since the standard errors for the general population estimates were very small, the estimates were considered to be ‘true’ values representing the adult Danish population. Thus, and since the t-test do not require any assumption of normal distribution in adequately large samples (Lumley et al. 2002), the one-sample t-test was used to compare the study populations’ SF-36 and PSS mean scores with age-standardized mean values for the general Danish population (Danish normative data). Effect sizes (Cohen’s d) were also calculated as an indication of the magnitude of the mean differences between the study population and the general population. The effect sizes were calculated by dividing the difference between the means scores of each group by the pooled standard deviations for those means. Following Cohen’s recommendation, effect sizes of 0.20, 0.50 and 0.80 were considered to be small, moderate and large, respectively (Cohen 1988).

The non-parametric (Kruskal–Wallis) test was applied to compare the SF-36 and the PSS mean scores between different subgroups in the study population. Furthermore, the one-proportion z-test was used to compare prevalences in the study population with age-adjusted prevalences from the general population.

Strengths and Limitations of Methods

  1. Top of page
  2. Abstract.
  3. The Eye Amputated – Consequences of Eye Amputation with Emphasis on Clinical Aspects, Phantom Eye Syndrome and Quality of Life
  4. List of Papers
  5. Acknowledgements
  6. Conflicts of interest
  7. Abbreviations
  8. Introduction
  9. Aims of the Ph.D. Study and its Hypotheses
  10. Materials and Methods
  11. Strengths and Limitations of Methods
  12. Results and Discussion
  13. Conclusions of Thesis and Future Perspectives
  14. Summary in Danish
  15. References
  16. Recommended Books and Articles for EA Patients, Relatives and all Health Professionals with Contact with EA Patients

In this investigation we surged the Rigshospitalet database of surgery codes (ICD10 codes). Rigshospitalet is a specialised hospital and eye departments in eastern Denmark refer most patients with a need for EA to Rigshospitalet. However, some uncomplicated cases may have been EA at other hospitals. This may give a selection bias, due to lack of uncomplicated cases. Very complicated cases referred from other hospitals with the wish of secondary implantation of implants were excluded from the study, due to these patients’ special needs and complications.

Comparing our results with the incidence of EA in Denmark (Hansen et al. 1999) and Island (Sigurdsson et al. 1998) found in earlier studies we estimate that the present research involves one-third of the annual number of EA in Denmark and most of the eyes with neoplasms (Hansen et al. 1999).

Why not all EA patients in Denmark? We chosen to focus only on patients from Rigshospitalet because it was easy assessable, there were lot of patients and only three main surgeons with known skills and practice with EA and different types of implants. Furthermore we wanted to estimate the long time complications with and without the hydroxyapatite implant. In the time period from 1993 to 2003 the use of hydroxyapatite were not well established in all departments. The pathology database on the Eye Pathology Institute could have been used, instead of using the hospital database as primary source of information. This would have included more patients from other hospitals, but would have included patients operated by several surgeons with limited annual output of EA. The sight advising centres in Denmark or the occularists could also have been used as a source of EA patients. However, the validity of these systems is not known.

The validity of the clinical reports can be questioned. Twenty one patients were miscoded in the hospital database. They all had both eyes and at the same time a surgery code for EA. The explanation for this might be that; some surgeries at Rigshospitalet are not routines and not all operations have a code. Furthermore the ICD10 codes have been used primarily to reimburse the clinical department. However, the system is regarded as valid and is used all over Denmark.

Clinical examination

Originally 431 patients were included. But 126 patients were deceased out of the total study population in 2005. In 2010 this number has increased into 137 patients (40% of the total study population). Many had deceased from age but some must have died of their neoplasm. The mean age of patients receiving the diagnosis malignant melanomas is 55–60 years (Egan et al. 1988). Five years survival is 31% and 25 years survival is 49% (Kujala et al. 2003; Damato 2006). In respect in our observation time of 3–62 years – we find it plausible that many have died from their neoplasm in the follow up period.

We choose to exclude all patients from Greenland and Faeroe Islands, because they consist of a highly selected group of patients with a different contact to the health care system and way of living compared to the Danish population. Patients below 18 years of age were excluded from the clinical investigation due to issues concerning permissions from the authorities, and because they also consisted of a little and special group of patients (seven patients).

The clinical examination has minor lacks according to effectively and objectively measuring all aspects of the sulcus superior syndrome. Lagophthalmos could have been measured by asking the patient to close his eyes and the by the ruler measuring the size of the lagophthalmos.

Also measures of entropion and ectropion are missing. However, entropion and ectropion are a result of the sagging lower lid. But it could have been measured objectively.

Degree of ptosis has been defined as a noticeable droop of the upper lid relative to the other side usually more than one mm difference in the midpupil to upper-lid distance (Oestreicher et al. 1997; Lliff & Pacheco 2001). This could have been used as a more exact measurement. The margin reflex distance (corneal light reflex to upper eyelid margin) could also have been used instead of the subjective grading (Lliff & Pacheco 2001; Collin 2006).

Willis have suggested a measurement for hypo- and hyperglobus by assessing the right angled distance from the pupil to the eyebrow (Willis et al. 1990). This would have been valuable instead of the subjective grading. An exact measurement of the depth of the superior sulcus would be very helpful in the clinical evaluation, but do not exist today.

Interviews

Patient reports of visual hallucinations are subjective in nature, and we did not attempt to confirm them clinically. However, patients never spontaneously gave statements of visual sensations. Therefore, figures are minimum values. The personalized interview did not specifically explore if patients had experienced similar visual hallucinations before surgery, and it may be questioned if the eye amputation and the missing eye are the cause of the phenomena. The disease leading to eye amputation, in some cases, could be the cause of the visual hallucination. However, no patient spontaneously reported any experience of similar visual hallucinations occurring before surgery, and in question 1, patients are specifically asked about symptoms related to ‘the eye that has been removed’. Another cause of the visual hallucinations could be the trauma related to the orbital/periocular surgery rather than the eye amputation itself, and we cannot completely exclude the surgical trauma as the cause in some of our patients. The incidence of persistent visual hallucinations after orbital/periocular surgery without eye amputation is unknown, but it is probably low.

It is well known that pain is one of the clinical symptoms that is difficult to evaluate. Its subjective character and the complex feelings that the pain evokes make accurate measurements difficult.

A qualitative interview design was chosen for this study because the selection of pain descriptors can be difficult for patients to address in a questionnaire, and because the interviewer can instruct the patients in the correct use of the VAS. In an interview setting, patients also have the opportunity to ask the meaning of unfamiliar words (Klepac et al. 1981). Our patients came to the hospital as a part of a clinical investigation/examination, and it was therefore not too complicated to get an interview. However, the interview form makes it more difficult to compare with results from studies with self-administered questionnaires (Klepac et al. 1981).

The VAS was chosen to measure pain intensity because of its widespread use in Denmark and many Danish patients are familiar with its use. The VAS is useful for detecting differences during the day, and for comparing three different pain intensities during the day within the patient group (Williamson & Hoggart 2005). Studies of the VAS have shown that it is an accurate, sensitive, cost–effective and easy to use method for measuring a subjective symptom (Ohnhaus & Adler 1975; Banos et al. 1989; Melzack & Katz 1999; Coll et al. 2004; Williamson & Hoggart 2005).

We have no indication that participation in the interview was biased by patients having pain. In the invitation to participate in the study, we did not mention pain; however, having pain may be a stimulus to participation. The interviewer (MLRR) was in 2005 a medical student, not biased by an interest in a good outcome of surgery, and her position may also have inspired patients to report phantom pain more freely.

In our interviews with patients, we did not differentiate between stump pain and phantom pain (Jensen et al. 1983, 1985; Woodhouse 2005). Stump pain is common in the early postoperative period, but in most patients it subsides with healing. Five to 10% of patients with a limb amputation experience persistent stump pain (Nikolajsen & Jensen 2001). In the current study, median follow-up time since EA was 4 years, and most cases of stump pain would probably have disappeared by the time of the interview.

Questionnaires

After the validation of questionnaire it became clear that some of the interviewed EA patients had difficulties to find the right box to the different answer possibilities, because of their impaired stereopsis. Therefore, we interposed an underlining on the text and its answer boxes, to facilitate the completion of the third questionnaire in the total study group.

The time span between the measured visual acuity and administration of the questionnaires was 2 years (from December 2005 to February 2008). In this time span, visual acuity may have worsened. But again, the majority of the patients had good visual acuity in the remaining eye and combined with the median age of 50 years, we do not think this can explain the results.

Information about job separation due to illness or disability among the general population sample was obtained via face-to-face interviews, and this may have influenced the comparisons between the study population and the general population. However, the different modes of data collection cannot explain the vast differences in prevalence of job separation due to illness or disability between the two populations.

Self-rated health on a VAS is a part of EuroQol EQ-5D scales (EQ-VAS). The EQ-5D scale has earlier been used in Danish studies (Kessing et al. 2006; Vogel et al. 2006), and have been translated and validated in many countries (http://www.euroqol.org).

In the EQ-5D the VAS is a 20 cm line together with five questions. The use of VAS alone and on a 14 cm line for estimation of self-rated health has not been validated. By critics the anchor points – best possible health and worst possible health – is said to be undefined and comparisons between respondents may be problematic. End-aversion bias is also problematic (Torrance et al. 2001). However, self-rated health was measured with the use of a 14 cm VAS scale in the Danish Health Interview Survey 2005 (Sorensen et al. 2009). And our results from the self-rated health on VAS corroboratively support the results found in the question reporting excellent and very good health.

Results and Discussion

  1. Top of page
  2. Abstract.
  3. The Eye Amputated – Consequences of Eye Amputation with Emphasis on Clinical Aspects, Phantom Eye Syndrome and Quality of Life
  4. List of Papers
  5. Acknowledgements
  6. Conflicts of interest
  7. Abbreviations
  8. Introduction
  9. Aims of the Ph.D. Study and its Hypotheses
  10. Materials and Methods
  11. Strengths and Limitations of Methods
  12. Results and Discussion
  13. Conclusions of Thesis and Future Perspectives
  14. Summary in Danish
  15. References
  16. Recommended Books and Articles for EA Patients, Relatives and all Health Professionals with Contact with EA Patients

Epidemiological study (paper I)

The causative diagnosis and indications for EA

For the time period from 1996 to 2003 the causative diagnosis and indications for EA in Denmark are seen in Table 1. ‘Neoplasm’ as the indication for EA comprises only the causative diagnosis of neoplasm. Likewise, the indication ‘recent injury’ covers only traumatic lesion as a cause. However, the indication ‘painful blind eye’ refers to a heterogenic group of causative diagnoses.

Table 1.   Causative diagnoses and indications for EA in 345 patients in Denmark during 1996–2003.
Causative diagnosisIndication for amputation of the eyeTotal
Painful blind eyeNeoplasmInfectionDisfiguring blind eyeRecent injuryPrevention of sympathetic ophthalmiaOther reasons
Neoplasm811900000127
Traumatic lesions280315252073
Complications after operations3809201151
Infections and inflammations15020302040
Glaucoma2909000038
Perinatal diseases700300010
Other reasons00000011
Unknown20120005
Total12711942252552345

‘Other reasons’ included one patient in whom the blind eye was used for auto transplantation of the cornea to the seeing eye. Reasons for EA were unknown in another patient, whose medical record was missing and the patient was senile and unable to recount a medical history.

We found a painful blind eye, neoplasm, infection and recent injury to be the most common indications for EA, as in other studies (Erie et al. 1992; Sigurdsson et al. 1998). In the Icelandic study, the majority of patients had trauma (40%) and glaucoma (39%) as the cause for a painful blind eye leading to EA (Sigurdsson et al. 1998). Neoplasm was the most frequent causative diagnosis for EA (37%), and led to enucleation in 93% of cases. Nearly all of these had a large uveal melanoma, indicating that, despite modern eye-saving melanoma therapy, a number of patients are still diagnosed late in their disease, by which time tumours are large and patients prefer enucleation to conservative treatment. In addition, three patients with choroidal melanoma were enucleated because of a painful blind eye. They had all been successfully treated for their tumour with radioactive plaque therapy, but had developed a neovascular, painful glaucoma. In Denmark the treatment of cosmetically disfigured, non-painful and blind eyes (Table 1) is no longer EA but coloured contact lenses and cosmetic shells.

As in other studies, males dominate in the overall distribution of EA (males 53%, females 47%) because a higher number of males (74%) suffer from trauma (Erie et al. 1992; De et al. 1994). Females, in contrast, were in this study more frequently represented in the causative diagnoses ‘glaucoma’ (76%), ‘perinatal diseases’ (70%).

Possible change in surgical technique

The surgical techniques applied were: evisceration (174); enucleation (154), and orbital exenteration (17). Type of operation correlated to causative diagnosis (p < 0.0001). Evisceration was used in the treatment of all groups of causative diagnosis except malignant neoplasms, with ‘traumatic lesion’ (36%) representing the largest group (Fig. 8). Five patients in the evisceration group had been treated for extraocular orbital cancer (causative diagnosis: cancer). However, despite the successful treatment of their cancer, they ended up with a painful blind eye as a result of radiation sequelae.

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Figure 8.  Type of eye amputation surgery in relation to causative diagnosis.

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Enucleation was primarily used for ocular cancer (69%). The total number of surgical interventions remained stable throughout the time period. However, there was a significant increase in the number of eviscerations compared with the number of enucleations performed (p = 0.002). This trend has been found earlier by Hansen et al. (1999), but continues up till 2003.

The number of orbital exenterations remained constant during the study time. Exenteration of the orbit was used because of orbital cancer in 94% of cases, primarily malignant melanomas. One patient underwent orbital exenteration as a result of a life threatening panophthalmia with orbital cellulitis after a cataract operation. Rahman et al. from Manchester are reporting increasing numbers of orbital exenterations due to raise in eyelid basal cell carcinomas with insignificantly surgical removal under a variety of non-ophthalmic specialities (Rahman et al. 2005a). In more sunny parts of the world squamous cell carcinomas are the most frequent reason for exenteration. The mortality of patients with orbital exenterations are high with a 5 year survival of 57% (Rahman et al. 2005b).

The number of causative diagnoses of glaucoma as a reason for EA has decreased significantly in Denmark (Hansen et al. 1999). However, complications to surgery’ is now cited frequently as the cause of a painful blind eye (Fig. 9). This may be because the incidence of surgery for retinal detachment and cataract has increased dramatically in Denmark over the last 10 years. The spectrum of causative diagnoses for enucleation remained stable over the time period.

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Figure 9.  Correlation between number of eviscerations, year of surgery and causative diagnosis. A significant increase in number of eviscerations due to post surgical complications is noted.

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In Denmark the most used implant the last 17 years have been the porous implants (Hydroxyapatite) and the percentage of patients in whom an Hydroxyapatite implant was applied increased from 33% in 1997 to 67% in 2003 (Fig. 10). The increasing use of orbital implants is in line with the current trend to use integrated orbital implants to improve cosmetic outcome after EA (Shields et al. 1994; Ashworth et al. 1997; Sami et al. 2007).

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Figure 10.  The annual number of implants increases significantly during the study period (p < 0.0001 chi square).

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Conclusion on the basis of paper I

My hypothesis I have been proven right since the number of EA in Denmark seemed stable during the time period. The most frequent causative diagnoses were neoplasm and trauma. And the most frequent indications during the time period were painful blind eye and neoplasm. A shift in surgical technique from enucleation towards evisceration was observed. This may reflect that today a high number of ocular diseases, which were previously treated by enucleation, now are treated with eye conserving therapies. This may ultimately lead to an increase in number of painful blind eyes that are later eviscerated. A second consequence of the shift in surgical technique is the increasing use of orbital implants, a procedure that favours evisceration because the sclera is ideal for coverage of a hydroxyapatite implant. The numbers of patients receiving an orbital implant were dramatically increasing during the observed time period.

Interview (paper II and III)

The demographic data for the group of patients who came to the interview and the clinical examination is listed in Table 2. All 173 patients were part of paper I.

Table 2.   Demographic data.
 Females/MalesAge in years – Median (range)Observation period in years – Median (range)Types of surgery
All patients n = 17384/8953 (18–89)5 (2–64)Evisceration: 96 Enucleation: 75 Exenteration: 2
Phantom eye syndrome

The present two studies are the largest cohort of patients with phantom eye syndrome that has been reported. The studies imply that 51% of people who lose an eye may suffer from phantom eye syndrome. Phantom vision was described by 72 patients (42%), 39 had phantom pain (22%) and three patients had phantom sensations (2%).

Phantom vision

Descriptions of visual hallucinations are showed in Table 3. A full description of all three types of visual hallucinations can be found in paper II (Roed Rasmussen et al. 2009).

Table 3.   Features of the visual hallucinations.
 Number of patients
Visual hallucinations – all types72
Elementary visual hallucinations63
 White light (or yellow) – total53
 Constant/steady16
 Flashing/moving37
 Coloured light – total30
 Geometric shapes (kaleidoscopic)13
 Dots, flashes in colours17
 Shadows Black, brown shadows outside the visual field9
Complex visual hallucinations
 Faces, objects, scenes2
Other visual hallucinations – total24
 Impression of seeing with both eyes16
 Impression of being blind for the second time after removal of a blind eye2
 Photophobia4
 Difficulty in focusing2
 Bilaterally amputated patient claiming to be able to differentiate between light and dark1
 Unable to describe character of visual hallucinations3

Thirty-six percent of our patients had elementary visual hallucinations (Fig. 11). This corresponds well to the Soros et al. ( 2003) study. However, only 1% of our cohort had complex visual hallucinations as opposed to 20% in the Soros et al. study. We have no specific explanation for this large difference. Methodologically there is a difference between the methods used, in favor of the questionnaires in the Soros et al. study for receiving more confidential information. Patients are often reluctant to mention visual hallucinations and therefore might be more willing to write than talk about these sensations. The feeling of being able to see with both eyes was experienced by 16 of our patients. Almost all had the feeling when it was dark or when they were tired. One explanation may be that the rod signal dominate in scotopic conditions (Guyton & Hall 2000). The cone-dependent stereopsis is missing in both the one-eyed and two-eyed patients; therefore, they use the same other measures for depth perception.

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Figure 11.  Elementary visual hallucination. The picture is a drawing of a patient with phantom vision, described likes all shapes of white lines exploding and fading away. A single line only persists for 2 seconds, which means that the hallucination is in constant movement. The most frequent description of a visual hallucination is white light as a desk lamp right in the face, every time the patients close his eyes.

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Patients generally had difficulties describing the frequency of their visual hallucinations. Therefore, the answers have been grouped in four categories: (i) every day: 14%; (ii) often (more than once a week): 40%; (iii) rarely (less than once week): 32%; and (iv) discontinued having visual hallucinations: 14%. When the visual hallucinations stopped it usually happened 3–6 months after the amputation of the eye. The most frequent triggers were darkness, closing of the eyes, fatigue, and psychological stress. Visual hallucinations were most often experienced just before going to sleep or when waking in the middle of the night. Seventeen of the patients only experienced the visual hallucinations when they closed their eyes. Four patients mentioned sounds, and two mentioned intense sunlight as a trigger. Other triggers were hard physical work, turning of the head, and taking out the prosthesis. Approximately 50% (38) of the patients had no triggers or stoppers of their visual hallucinations. The triggers and stoppers in the present study are similar to findings in other studies of visual hallucination after amputation of an eye (Soros et al. 2003; Maloof 2004) and in patients with Charles Bonnet syndrome (Manford & Andermann 1998). The association between sounds and visual hallucinations, as present in four of our patients, is known as visual synesthesia (colored hearing) and is a crossmodal association, mostly exclusively in a one-way direction (Ramachandran & Hubbard 2003; Hubbard & Ramachandran 2005; Knoch et al. 2005). In the present study, our patients only experienced visual hallucinations triggered by sounds and not in the reverse direction. There has been a report of acquired visual synesthesias after visual field loss and blindness. The synesthesia appeared in the ‘blind’ region. All reported cases had lesions of the optic nerve or chiasm (Jacobs et al. 1981).

Most of the patients (60%) did not have any emotions related to their visual hallucination, but some found the hallucination psychologically disturbing (27%). A minority of patients (13%) described strong emotions. All patients showed critical insight; they were aware that the phenomena were unreal. Many were embarrassed to discuss their visual experiences. They feared that the interviewer would think they had mental illness. Almost all patients with visual hallucinations kept them as a secret even to their relatives. Ten patients were so visually and emotionally disturbed that it interfered with daily life (Fig. 12).

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Figure 12.  Elementary visual hallucination, the same patient as in Fig. 11 as it is seen with the eyes open. The hallucination is constantly present, and adds a filter to the view of the external world. The patient is very disturbed by the hallucination, and is not able to live a normal life.

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The experience of phantom eye syndrome seems to be independent of the visual acuity of the remaining eye and 90% of the patients had good visual acuity (Table 4).

Table 4.   Visual accuracy in the remaining eye.
Corrected visual acuity≥6/6 to 6/246/36 to 6/60<6/60 to +L−L
All patients (n = 173)1524125
Phantom pain

Thirty-nine (23%) of the 173 interviewed patients reported phantom pain after EA (Table 2). Thirty-one percent of the patients with phantom pain experienced pain every day, 15% had pain several times a week, 18% had pain once a week, and 36% had pain less often than every week. Patients with daily phantom pain reported the most intense pain (Fig. 13.)

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Figure 13.  Frequency of phantom pain and mean pain intensity during the day. Mean pain intensity is the mean of the VAS score for the morning, noon and evening.

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The median pain intensity for an entire day on the VAS scale was 36 [range: 1–89]. Pain intensity increased during the day. In the morning the median pain intensity was 23 [range: 1–89], at noon 34 [range: 1–89] and in the evening 50 [range: 1–98]. The increase in pain intensity from morning and lunchtime till evening was significant (p = 0.009 and p = 0.018, respectively, t-tests for paired means). Six patients could not answer this part of the interview because their phantom pain was highly irregular. For limb amputees with 6 months of follow up, the patient rated median pain intensity was 22 [range: 3–82] on a 0–100 mm VAS (Nikolajsen et al. 1997). For limb amputees with 26 years (mean) of follow-up, the patient rated mean pain intensity was 5.3 on a scale from zero to 10 on a self-administered questionnaire (Sherman et al. 1984, 1997). Patient age, past pain experience, type of surgery, and the length of the follow up were very diverse in our study. This may add uncertainty to the levels of intensity and frequency of pain reported. However, the fact that our patients clearly could state the quality, intensity, frequency and triggers/relievers of their pain, demonstrates that their pain is a severe, still ongoing complication. We did not specifically question the patients about the duration of their single pain attacks. However, the fact that many of the patients were able to differentiate the intensity of their phantom pain attacks during the day indicates that the pain is present for several hours a day.

Nineteen of the 39 patients with pain after EA reported a cutting, penetrating, gnawing or oppressive quality pain. Radiating, ‘zapping’ or shooting pain was experienced by eight patients, and five had a superficial burning or stinging pain. Seven patients reported a mixture of the different pain qualities. However, phantom pain among limb amputees is mainly described as shooting, pricking, boring, crushing and burning. Qualities of pain experienced from the limbs before amputation (Nikolajsen et al. 1997; Mortimer et al. 2002). Therefore, the qualities of pain that had been experienced in the diseased but now amputated organ may explain the differences in the spectrum of phantom pain qualities found between limb amputees and EA patients.

Our patients reported chilliness and windy weather as the most frequent triggers. This corresponds very closely with studies of limb amputees were chilliness and windy weather are the most frequent trigger (Sherman et al. 1984). One hypothesis is that the chilliness is painful for the EA patients because the blood flow is markedly reduced in the orbit due to the lost tissue. The patients are not able to maintain a normal temperature of their eyelid and conjunctiva, and thus feel pain. Some patients reported that cold weather made the conjunctival secretions freeze, which was painful.

There is some evidence that rest, heat, firm pressure, use of prosthesis and distraction can reduce the severity of phantom pain in limb amputees (Jensen et al. 1983, 1985). Several of the patients in the current study described similar approaches to dealing with pain. In Figs 14 and 15 the consecutive diagnoses and indications for EA correlated for pain are showed.

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Figure 14.  Incidence of phantom pain in relation to causative diagnosis. For patients with a diagnosis of perinatal diseases, 55% had phantom pain. Congenital/perinatal diseases include: retinopathy of prematurity (= 4), microophthalmus (= 3), congenital glaucoma (= 2), Norrie’s disease (= 1) and Rieger’s syndrome (= 1).

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Figure 15.  Incidence of phantom pain in relation to indication for EA. The largest group of patients (= 59) had EA to treat a painful blind eye; 21 (36%) of these patients still had pain at follow up.

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The nature of the types of pain can either be phantom pain, dysfunctional socket pain or both types together in combination. We find it most possible that the type of pain the EA patients suffers from is phantom pain; because of the pattern of observed incidence, frequency, intensity, triggers and relievers of the pain. Previous studies of phantom eye pain have found the same incidence as in the current study (Nicolodi et al. 1997; Gerding et al. 2003; Soros et al. 2003). The frequency of pain reported in the current study is different from the study by Gerding et al. (2003), in which 71% of the patients had phantom pain less than once a month (Gerding et al. 2003). The explanation for this difference may be that they found a lower frequency because they used a self-report questionnaire and had lower participation rates. The higher frequency in our study is in addition similar to the frequency of phantom pain in limb amputees, with 27% of these types of patients experiencing pain almost every day (Sherman et al. 1984). The previous studies of phantom eye pain do not describe the quality, intensity, triggers and relievers of phantom pain, and we therefore compared our findings with studies of phantom pain in limb amputees.

One argument suggesting a dysfunctional socket is causing pain is that a number of the mentioned triggers and relievers are related to the prosthesis. The associations between pain and implants, pegging, as well as secretion can all be interpreted as evidence of a dysfunctional socket. Most patients had secretion from their socket (Table 5). However, patients with pain had more secretion than did patients without pain (p = 0.008, Chi-Square).

Table 5.   Secretion from the socket and phantom pain after EA.
Degree of secretion from the socketPhantom pain after EANo phantom pain after EA
No secretion16
Infrequently631
Daily855
Constantly2442
Total39 (χ2, p = 0.008)134

Arguments against dysfunctional socket pain are that patients with a painful blind eye are frequently treated with evisceration and an implant and they are more likely to be offered a peg. Furthermore, these patients are also the most likely to have pain after EA. However, it illustrates that we have a selection bias regarding our patient’s indication for implant. None of the patients with pain had exposure of their hydroxyapatite implant or any other clinical cause for pain found in the clinical examination. In addition, the pain continued years after the EA and we therefore believe that the pain experienced by the majority of the participants in the study is phantom pain (Nicolodi et al. 1997; Gerding et al. 2003; Soros et al. 2003).

Phantom sensations

Phantom sensations (itching around the eye, feeling of nonexistent eyelids, and a feeling of opening and seeing with both eyes) were found in three patients (2%). These three patients had the complete spectrum of symptoms of phantom eye syndrome (visual hallucination, phantom pain, and phantom sensations).

Identification of risks factors for developing phantom pain

In our study of phantom pain there is a high correlation between ophthalmic pain before EA and phantom pain afterwards (p = 0.004, Fisher’s exact test). This has previously also been reported by Soros et al. (Gerding et al. 2003; Soros et al. 2003). However, if we to the 59 patients EA because of pain add the 18 patients that stated they had ophthalmic pain before surgery we find no correlation (p = 0.145, Fisher’s exact test). This indicates that patients do have a recall bias associated with the length of time since surgery. In the present study, 22 (13%) had pain together with visual hallucinations at the time of the interview (p = 0.033).

Patients with preoperative eye pain were more likely to develop visual hallucinations (54%) than patients without preoperative eye pain (46%), but this was not statistically significant (p = 0.39). However, because of the retrospective nature of our study, some patients may have forgotten about their preoperative pain at the interview. A causal role of preoperative pain for phantom eye syndrome has been suggested by earlier studies and cannot be disregarded (Nicolodi et al. 1997; Soros et al. 2003, 2005). ‘Other visual hallucinations’ were significantly correlated to old age (p = 0.007). Neither phantom eye syndrome nor visual hallucinations alone were correlated to any of the other explicatory variables.

Type and degree of limb phantom pain, however, have no direct association with cause of amputation, anatomical level of amputation, sex or age of the patient (Parkes 1973; Jensen et al. 1985), which is similar to the patients in the current study. In this study, the pain is associated with all categories of diagnoses. This may be the result of the often long time span between diagnosis and final indication for EA. It is noteworthy that 55% of the patients with the diagnosis ‘perinatal diseases’ were found to have phantom pain (Fig. 14).

The presence of pain was not associated with the type of surgery used for the EA (Table 2.) Of the 39 patients with pain, 27 (70%) were treated with an evisceration and 12 (30%) with an enucleation in the time period 1959–2003. Among the entire group of EA patients in the same period, 43% were eviscerated, 52% were enucleated and 5% had an orbital exenteration (Rasmussen et al. 2010).

Patients with an implant are more likely to suffer from phantom pain (p = 0.040, Fisher’s exact test). Whether this is because the implant triggers pain or the presence of an implant changes the psychological reaction of the patient is unknown. However, it is noteworthy that none of the patients with phantom pain and implant had exsposure or other complications.

Treatment of painful blind eye with EA

EA by evisceration or enucleation has for centuries been considered an effective treatment for a painful blind eye (Erie et al. 1992; Sigurdsson et al. 1998; Custer & Reistad 2000). Custer and Reistad found pain relief in 93% of patients who received enucleation to relieve ocular pain. Their study was based on an e-mail follow up with 58 patients in 1999 (Custer & Reistad 2000). A retrospective study by Shah-Desai et al. (2000) indicated there was pain relief for all 24 patients treated with enucleation or evisceration.

In the current study, 64% of the 59 patients treated with EA due to a painful blind eye had complete pain relief. The intensity of the pain in the remaining 21 patients was reduced in 16, two patients experienced a pain with similar intensity to the pain prior to EA and two patients experienced an increased intensity of pain. One patient had experienced complete pain relief after EA, but after some years the pain returned with a new quality (radiating).This is indicating that patients with a painful blind eye as the indication for EA have a significant increased risk of developing phantom eye pain (p = 0.004, Fisher’s exact test). However, earlier studies have shown that patients tend to overestimate their pain before amputation, and that recall of pain is influenced by mood, affective state, pain history and type of pain (Erskine et al. 1990; Nikolajsen et al. 1997). Our findings are in contrast to earlier studies that reported pain after evisceration is rare (Glatt et al. 1993; Custer & Reistad 2000; Shah-Desai et al. 2000).

Reassurance and treatment of phantom eye syndrome

Prevention of the phantom eye syndrome is important. Therefore, when eye amputation is considered, the risk for phantom eye syndrome and other socket-related complications should be included as factors in the decision process. Patients who are not disturbed by their visual hallucinations are sufficiently treated by reassurance. Written material for patients describing that visual hallucination is common after eye amputation may be useful. Patients finding the visual hallucination psychologically disturbing and patients having strong emotions related to their visual hallucinations may need treatment. We found no effective pharmacologic treatment of phantom eye syndrome in the literature. But the similarity between these hallucinations and those experienced by patients with migraine and epileptical aura suggests that medications for these disorders might be useful in severe cases. Support for this is that neuroleptics have been used in Charles Bonnet syndrome, however, with varying effect (Menon et al. 2003). In 14% of the patients with visual hallucinations, the hallucination stopped 3–6 months after eye amputation. This finding suggests that visual hallucinations present 6 months after surgery will probably continue.

The value of analgesics, neuroleptics, and psycho pharmaceuticals in relation to phantom pain in the anophthalmic orbit is unknown. Many of the patients in the study used analgesics, although with variable efficacy. However, the similarity between phantom pain in limbs and orbit suggests that the medications used for phantom pain in limbs might also be useful in severe orbital cases (Jensen & Nikolajsen 1999). Conventional analgesics and opioids have proven effective for phantom pain in limbs (Jensen & Nikolajsen 1999; Nikolajsen & Jensen 2001). Severe cases of limb phantom pain have been treated with neuroleptics, anticonvulsants (Carbamazepine), tricyclic antidepressants, β-blockers, intravenous calcitonin, and N-methyl-d-aspartate receptor antagonist (ketamine). No treatment completely eliminates the pain, and persistent long-term phantom pain may be resistant to any treatment (Ramachandran & Hirstein 1998; Jensen & Nikolajsen 1999; Nikolajsen & Jensen 2001). Furthermore, the value of patient education for patients with pain should not be disregarded (Wilder-Smith & Schuler 1992; Mortimer et al. 2002). And doctors could on that basis encourage their patients to talk to other EA patients with phantom eye syndrome.

Conclusion on the basis of paper II and III

On the basis of paper II and III we can conclude that my hypothesis II is supported, since the phantom eye syndrome is frequent (51% of the EA patients). Visual hallucinations were described by 42% of the patients. The content were mainly elementary visual hallucinations, with white or colored light as a continuous sharp light or as moving dots. The most common triggers were darkness, closing of the eyes, fatigue and psychological stress. More than half of the EA patients had the experience of visual hallucinations more than once a week. Five percentages of the patients were so visually disturbed that it interfered with their daily life.

My hypothesis III was not fully supported since phantom pain was described by as many as 23% of the patients. However, the study III gave valuable new information. The phantom pain was reported to be of three different qualities: (i) cutting, penetrating, gnawing or oppressive (49%); (ii) radiating, zapping or shooting (20%); (iii) superficial burning or stinging (13%); or a mixture of these different pain qualities (18%). The median intensity on a visual analogue scale, ranging from 0 to 100, was 36 [range: 1–89]. One-third of the patients experienced phantom pain every day. Chilliness, windy weather and psychological stress/fatigue were the most commonly reported triggers for pain. Half of the patients used analgesics to reduce their pain, but most with a varying efficacy. Risk factors for developing phantom pain were ophthalmic pain before EA and presence of an implant.

Phantom sensations were present in 2% of the patients. The efficacy of EA in reliving pain associated with a painful blind eye can be regarded in two ways. First, it could be argued that EA is efficient in pain relive in approximately 64% of patients. It is a good treatment and focus should be placed on how to help the remaining group of patients with persistent pain. The other way around is to stop treating painful blind eyes with EA due to inefficiency in 36% of patients. However, patients with a painful blind eye who are not EA are undoubtedly also having lower HRQOL than the general population, and at the moment no other evidenced based treatment is available (paper IV).

Self-administered questionnaires (paper IV)

Emotional and social consequences of EA

Recovery after loss of an eye has been investigated in two previous questionnaire studies (Linberg et al. 1988; Coday et al. 2002). In 1988, one study found that among 125 EA patients, 37% had experienced permanent changes in their lives due to the EA, 10% had problems with employment, and 17% suffered from anxiety or poor self image (Linberg et al. 1988). In 2002 a questionnaire investigation of 58 EA patients and seven functionally one-eyed patients found changes in employment (23%), changes in driving status (39%), socially affected lives (40%) and difficulties with sports and hobbies (50%) (Coday et al. 2002). Patients in both prior studies had an average age around 25 years and 60–67% of the EAs were performed due to a recent trauma (Linberg et al. 1988; Coday et al. 2002). In the present study, the average age of EA patients is 51.4 years and the most common indications were painful blind eye and neoplasm.

However, results from our survey are quite similar: 41% stated that the eye amputation had changed their life considerably. More than half of the patients (65%) were feared by turning blind. The majority of the patients (73%) got used to wear the artificial eye during the first 6 months. However, 20% found that it took more than 1 year. One third (35%) reported that they had still not accepted the EA. And 41% of the EA reports that wearing an artificial eye is something they use a good deal or much energy on. Sixteen percent of the EA patients report that they think of their artificial eye all the time they are awake. The feeling of being stared at on the street was reported by 27%.

Satisfaction with the cosmetic outcome incl. the artificial eye: 33% were very satisfied, 53% were satisfied and 14% were dissatisfied or very dissatisfied. Nineteen percent do not think that the artificial eye looks like the other normal eye. The need to talk to similar patients, especially early after the eye amputation, was noticed by 42%. Thirty-four percent had talked to other EA patients already – half of them thought that it was a good thing and half of them believed that it was waste of time, unless the timing were just around the EA.

Health related quality of life, perceived stress, self-rated health and job-separation due to illness and disability

The sociodemographic characteristics of the study population and the general population are presented in Table 6. Approximately twice as many members of the sample of patients are divorced or separated compared to the general population (12.5% versus 6.3%). We defined patients with visual acuity of 6/36 or less as visually impaired (= 11). Data for this group were analysed separately due to this severe handicap. Of the total sample of 120 patients, 63 had evisceration, 55 enucleation and two exenteration of the orbit. Median observation time since EA was 5 years [range: 2–64 years].

Table 6.   Sociodemographic characteristics of the study population and the Danish Health Interview Survey 2005. Percentage.
 Study populationDanish Health Interview Survey 2005
Sex
 Men50.048.4
 Women50.051.6
Age
 <44 years41.743.7
 44–64 years31.735.9
 >65 years26.720.4
Marital status
 Married48.354.4
 Cohabiting15.015.5
 Single (separated, divorced)12.56.3
 Single (widowed)8.36.9
 Single (unmarried)14.216.9
 No information1.70.0
No. of respondents12014 566

Our study indicates that EA patients have poorer HRQOL and self-rated health and more perceived stress than the general population does (Fig. 16 and Table 7). HRQOL among 134 anophthalmic patients in Korea was investigated in 2008, and compared to 48 healthy volunteers (Ahn et al. 2010). We presume that the authors defined anophthalmic to mean patients who have lost their eyes, and not patients who were born anophthalmic/microophthamic. The anophthalmic Korean patients scored significantly lower on all eight domains of the SF-36 compared to the healthy volunteers. However, many differences exist between the Korean and Danish study populations concerning cultural, economic, social and religious factors. Furthermore, the sociodemographic factors are diverse: Koreans in the sample were around 10 years younger than were the Danes, 10% more of the Korean sample participants were married, and more than 50% had lost their eyes due to a trauma. Despite the many differences between the Koreans and Danes, the same differences in SF-36 between the EA and healthy individuals/general population were found in their study as well as the present (Ahn et al. 2010). We do not have access to the raw SF-36 scores from the Korean study, but it appears that the levels on all eight domains are lower for the healthy Korean volunteers than for the general Danish population. Data from the Korean sample also indicated that one-third of the patients exhibited anxiety and depression (Ahn et al. 2010).

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Figure 16.  SF-36 mean scores of persons in the study populations (crude means) and in the Danish general population (age-standardized means).

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Table 7.   SF-36 and PSS mean scores (crude) by indication for eye amputation.
 Painful blind eyeNeoplasmTraumaDisfiguring blind eyeStudy population (all indications)General population (age-standardized)Effect size
  1. *p-value < 0.05.

  2. Effect sizes of 0.20, 0.50 and 0.80 were considered to be small, moderate and large, respectively.

Bodily pain (BP)62.1*78.470.263.170.2*78.1−0.33
General health (GH)61.1*75.969.266.769.0*75.4−0.32
Mental health (MH)69.4*82.876.270.775.9*83.0−0.46
Physical functioning (PF)75.9*89.185.478.582.5*87.6−0.26
Role emotional (RE)64.0*75.879.468.972.7*86.8−0.47
Role physical (RP)64.2*68.370.261.767.2*81.3−0.41
Social functioning (SF)78.0*90.884.085.184.7*91.9−0.40
Vitality (VT)55.4*69.461.956.562.8*69.9−0.34
PSS14.1*11.412.511.612.4*11.00.23
No. of respondents3830211512011 238 
Mean age (years)50.657.842.252.351.448.9 

In Table 8 the SF-36 score and PSS median are stratified on the youngest (≤44 years) and the eldest (≥45). The analysis shows a significant difference in the subscale physical functioning (p = 0.0036).

Table 8.   SF-36 and PSS median scores (range) by age.
 ≤44 years≥45 yearsp-value
Bodily pain (BP)84 (100)74 (100)0.5239
General health (GH)83.5 (100)72 (95)0.0500
Mental health (MH)84 (100)84 (88)0.2745
Physical functioning (PF)100 (100)90 (100)0.0036
Role emotional (RE)100 (100)100 (100)0.2569
Role physical (RP)100 (100)75 (100)0.0720
Social functioning (SF)100 (100)100 (62)0.9000
Vitality (VT)65 (95)65 (95)0.7821
PSS10.5 (39)11 (27)0.8458
No. of respondents5070 
Mean age (years)35.163.0 

Younger EA patients (44 years or younger) had a significantly higher score on the SF-36 PF scale than the older EA patients (45 years or older) indicating a better physical function in the youngest age group. Reduced physical function with increasing age is also seen in the general population (Bjorner et al. 1997).

Differences in HRQOL scores of mono- and bilaterally visually impaired patients have also been demonstrated using the SF-36. Patients with mono-lateral visual impairment had lower scores on three dimensions and bilateral patients had lower scores on five dimensions compared to persons with no visual impairment (Chia et al. 2003, 2004). Our study supports this. The eleven patients, visually impaired in the only remaining eye, had lower SF-36 scores in all eight dimensions (Fig. 16).

The SF-36 scores and PSS mean, stratified on the four main indications for EA, are listed in Table 7. The analysis did not show a statistically significant difference in mean scores (all p > 0.05) between the four main indications for EA. However, the analysis indicated that painful blind eye, as an indication for EA, had the largest impact on health related quality of life and more perceived stress. It is noteworthy that the mean age in this group is 50.6 years.

For cancer patients, the EA was part of a lifesaving treatment. Studies of the relationship between cancer and HRQOL have found that survivors of cancer have almost the same levels of HRQOL as the general population, which is similar to the findings from our study (Eiser et al. 2000; Peuckmann et al. 2007). Such findings could be interpreted as changes in the patients’ internal standards, values and conceptualisations of life over time due to survival of cancer; a ‘response shift’ (Sprangers 2002). Studies of facially disfigured patients have indicated that the course of injury is related to self-consciousness and anxiety, which supports our findings (Tebble et al. 2004, 2006). Trauma patients also tend to have a good HRQOL similar to the cancer patients. Like the cancer patients they lost their eye within the first 14 days after trauma, and in most cases the surgery was inevitable. Type of surgery indicated the same tendency, i.e. eviscerated patients having lower mean values in most dimensions of SF-36, and more perceived stress. Eviscerated patients generally have the indication painful or disfiguring blind eye, and all cancer patients are enucleated (Table 9; Rasmussen et al. 2010). Studies of patients with disfigurements have found that levels of psychosocial distress are not well predicted by the severity of disfigurement (Dropkin 2001; Rumsey et al. 2003).

Table 9.   SF-36 mean score in different diagnostic subgroups.
 Phantom eye syndromeObservation timeType of surgery for EA
YesNop-value2–9 years>10 yearsp-valueEviscerationEnucleationp-value
Bodily pain (BP)63.577.20.0168.774.30.4165.674.50.19
General health (GH)65.772.30.1968.669.90.8065.672.40.38
Mental health (MH)73.578.50.1976.474.60.8171.780.30.01
Physical functioning (PF)81.483.70.9980.488.40.0578.087.20.14
Role emotional (RE)69.076.60.2770.778.10.4469.475.60.37
Role physical (RP)61.972.80.2265.671.80.3768.065.10.57
Social functioning (SF)82.387.20.0986.479.80.1381.288.30.05
Vitality (VT)59.865.90.1363.261.60.5458.267.50.07
PSS12.412.40.6612.013.40.3212.512.10.79
No. of respondents6159 8832 6355 
Mean age (years)50.951.9 53.346.2 49.453.5 

Effect sizes indicated that the largest differences in HRQOL between the EA patients and the general population were related to role limitations due to emotional problems (= −0.47) and mental health (= −0.46) (Table 7). One might speculate that feelings of being different and looking different (altered body images) result in poor self-confidence and self-image. This is also demonstrated by the lower self-rated health among the EA patients, as seen in Table 10.

Table 10.   Comparison of self-rated health and job separation due to illness or disability between the study population and the general Danish population.
 Study population (Crude %)General population (Age-standardized %)p-value
Excellent or very good self-rated health43.352.10.05
Changed job due to illness20.07.4<0.01
Retired due to illness25.89.2<0.01
Part-time due to illness10.84.1<0.01
No. of respondents12014 566 

The presence of phantom eye syndrome did not predict significantly poorer HRQOL or more perceived stress, except for the dimension of bodily pain (Table 9), which is to be expected, since around 44% of patients with phantom eye syndrome indicate phantom pain (Soros et al. 2003, 2005; Roed Rasmussen et al. 2009). In the current sample of patients, the incidence of visual hallucinations was 42% and phantom pain was 23% (Roed Rasmussen et al. 2009). From the interviews, we knew that many of the patients find the visual hallucinations disturbing and most patients keep their visual hallucinations a secret (Roed Rasmussen et al. 2009). We found no differences between patients with short- and long-observation time (Table 9) with the exception of physical functioning.

In all, 43.3% of the EA patients had excellent or very good self-rated health (Table 10). This percentage is lower than for the general population (52.1%). However, the difference was not statistically significant (p = 0.05). Measuring self-rated health by means of the VAS also indicated (p < 0.01) that patients experience poorer health than does the general population (study sample: mean: 70.4, SD: 22.2; general population: age-standardized mean: 78.6, SD: 17.2). The visually impaired group had a mean VAS score of 59.5 (SD: 18.9).

Socioeconomic position of the EA patients was categorised as: employed (= 53), self-employed (= 8), unemployed (= 4), student (= 2), retired pensioner (= 35) and disability pensioner (= 12). Six participants had missing values for socioeconomic position.

Thirty-one participants in the study population were retired due to illness; of those, 18 (58%) said it was because of their eye disease. Thirteen patients only worked part-time; eight (62%) of those due to the eye disease. A job change due to illness was noted by 24 patients, out of which 13 (54%) reported that it was due to the eye disease. In total, 25% of the study population retired or changed to part-time jobs due to their eye diseases (Table 10), which is very similar to earlier studies(Coday et al. 2002).

In the study population, 39.5% stated there were leisure activities they no longer took part in because of their EAs. The types of leisure activities the patients had to discontinue included: ball games (= 20); physical activities like walking, hiking, running and aerobics (= 7); bicycling (= 7); swimming (= 7); reading books and other activities requiring concentration (= 6); car driving (= 4); social activities (= 2); household chores (= 3); and shooting, skiing, travelling and cultural contexts(= 7). This is similar to earlier studies describing difficulties with sports and hobbies in about 50% (Coday et al. 2002).

Conclusion on the basis of paper IV

On the basis of paper IV we can conclude that my hypothesis IV is valid since EA do have a permanent effect on most of the EA patients HRQOL, perceived stress, self rated health and job separation due to illness and disability.

Conclusions of Thesis and Future Perspectives

  1. Top of page
  2. Abstract.
  3. The Eye Amputated – Consequences of Eye Amputation with Emphasis on Clinical Aspects, Phantom Eye Syndrome and Quality of Life
  4. List of Papers
  5. Acknowledgements
  6. Conflicts of interest
  7. Abbreviations
  8. Introduction
  9. Aims of the Ph.D. Study and its Hypotheses
  10. Materials and Methods
  11. Strengths and Limitations of Methods
  12. Results and Discussion
  13. Conclusions of Thesis and Future Perspectives
  14. Summary in Danish
  15. References
  16. Recommended Books and Articles for EA Patients, Relatives and all Health Professionals with Contact with EA Patients

The study population is similar in distribution to other national representative studies of consecutive diagnosis and indications for EA. The most frequent indications for EA in Denmark were painful blind eye (37%) and neoplasm (34%). During the study period from 1996 to 2003, the annual number of eye amputations was stable, but an increase in bulbar eviscerations were noticed. Orbital implants were used with an increasing frequency until 2003.

The Phantom eye syndrome is frequent among EA patients. Visual hallucinations were described by 42% of the patients. The content were mainly elementary visual hallucinations, with white or colored light as a continuous sharp light or as moving dots. Approximately 23% of all EA experience phantom pain for several years after the surgery and 38% of these patients stated that they did not have ophthalmic pain before the surgery. The phantom pain after EA is similar to the phantom pain known from limb amputees. Factors associated with phantom pain were: ophthalmic pain before EA, the presence of implant and a patient reported high degree of conjunctival secretion. A common reason for EA is the presence of a painful blind eye. However, one third of these patients continue to have pain after the EA. Phantom sensations were present in 2% of the patients. We recommend that surgeons inform their patients about the phenomenon. Treating physicians should stress that visual hallucinations after EA are a common phenomena and do not indicate mental illness, furthermore it is essential to discuss the risk of phantom pain.

In the questionnaires, we found that EA patients have poorer health related quality of life, poorer self-rated health and more perceived stress than does the general population. Effect sizes showed that the largest differences in health related quality of life between the EA patients and the general population were related to role limitations due to emotional problems and mental health. Furthermore, 25% retired or changed to part-time jobs due to eye disease and 39.5% stopped participating in leisure activities due to their EAs. Forty-one percent of the EA patients stated that the EA had changed their life considerable.

These results make the issue of rehabilitation relevant. In Denmark there exists no standardized rehabilitation protocol for patients after EA, and there is no formal association for EA patients. The EA is often regarded as the final ophthalmological treatment and often also the last contact with the speciality. The personal loss and consequences is one thing. However, the high numbers of EA who have retired under disability provisions or changed job due to the eye disease are also a loss for the society. To retain these people into labour marked participation is essential in rehabilitation both for to the individual and for society.

In Denmark the National Board of Health has developed a national strategy for chronic disease management. Chronic diseases are characterised by:

  •  The condition is permanent and leads untreated to poorer quality of life and/or early death.
  •  The earlier the disease is discovered, the bigger possibilities there are for a better prognosis and prevention of poor outcome.
  •  The disease has permanent implications.
  •  There is a need for long time treatment or care.
  •  There is a need for rehabilitation.

In these terms the EA patients suffer from a chronic disease. And according to the National Board of Health, chronic diseases should have a disease management program (The national board of health in Denmark 2008).

Chronic disease self-management education programs aim to empower patients through providing information and teaching skills and techniques to improve self-care and doctor–patient interaction, with the ultimate goal of improving quality of life (Dørfler & Hansen 2005; The national board of health in Denmark 2007). In Norway the patient association for EA – Norsk interesseforening for enucléerte (NIFE) – organize self-management education, self-management support, annual meetings with sharing of knowledge, and invited health professionals. Also local area networks are organized (NIFE 2010, http://www.nife.no/; Svensson 2004). Virtually the homepage ‘http://www.losteye.com’ is also running some of the same purpose.

Self-management education programmes by lay leaders for people with chronic conditions have been found to reduce disability, pain, fatigue and self-efficacy (Foster et al. 2007). In rehabilitation of facially disfigured patients with difficulties in social situations, behavioural therapy and social interaction skill training have showed good results; these treatments may also be useful to EA patients (Robinson et al. 1996; Newell 1999; Newell & Marks 2000).

Lack of knowledge among nurses and doctors about how and when to deliver psychosocial support after facial surgery can also be a problem (Konradsen et al. 2009). Discrepancies between patients need for emotional support and the care received was found, stressing that patient perspective on positive support included the need for staff approachability, awareness and time to talk (Furness 2005). In Denmark there are many different groups of health professionals that could extend their co-operation in order to improve the rehabilitation of EA patients and win more insight in the many aspects of being EA. Development of a shared care program with a structured cooperation between; hospital ophthalmologists and surgeons, general ophthalmologists, occularists, sight advising centres and general practitioners would also be valuable.

Danish cancer patients have social inequality in incidence and survival from cancer. Income, job marked inclusion, education, marital status, living area and comorbidity with depression and schizophrenia are all factors that influence the incidence and survival of cancer. For some cancers it may also show inequalities in the use of health care (Dalton et al. 2008). It was found that cancer survivors had a small but significantly increased risk for unemployment following cancer. Especially amongst persons aged 50–60 years at time of diagnosis. Risk factors for unemployment were found to be manual work, medium income and vocational education (Carlsen et al. 2008). Since only 25% of our patients have cancer a direct comparison to our study is not possible.

EA have many risks and complications but for most EA patients the treatment was lifesaving and was the best treatment available. However, for a few patients the benefits of EA seams limited. Especially for patients with disfiguring- or painful- blind eyes. The risk of late cosmetic complications, phantom eye syndrome, decrease in health related quality of life, more perceived stress, divorce and early retirement should make EA the last option.

Summary in Danish

  1. Top of page
  2. Abstract.
  3. The Eye Amputated – Consequences of Eye Amputation with Emphasis on Clinical Aspects, Phantom Eye Syndrome and Quality of Life
  4. List of Papers
  5. Acknowledgements
  6. Conflicts of interest
  7. Abbreviations
  8. Introduction
  9. Aims of the Ph.D. Study and its Hypotheses
  10. Materials and Methods
  11. Strengths and Limitations of Methods
  12. Results and Discussion
  13. Conclusions of Thesis and Future Perspectives
  14. Summary in Danish
  15. References
  16. Recommended Books and Articles for EA Patients, Relatives and all Health Professionals with Contact with EA Patients

I denne afhandling dækker betegnelsen øjenamputation (ØA) over fjernelse af øjenindholdet selv eller øjet med øjenomgivelser ved operationerne: evisceration, enucleation og exenteration. ØA er slutbehandling i et kompliceret sygdomsforløb, eller primærbehandling ved traumer og intraokkulær cancer. I 2010 er mængden af viden om typer af kirurgiske teknikker og implantater stor. Viden om hvorledes patienten har det globalt postoperativt er imidlertid meget begrænset.

Afhandlingen har til formål: At beskrive antallet af ØA, de tilgrundliggende diagnoser og indikationer for ØA, de valgte kirurgiske teknikker samt at påvise en mulig ændring i kirurgisk teknik i Danmark for perioden 1996–2003 (artikel I).

At beskrive øjenfantomsyndromet og dets komponenter: visuelle hallucinationer, fantomsmerte og fantomfølelse (artikel II). At karakterisere kvaliteten og kvantitet af øjenfantomsmerte og at identificere patienter med særlig risiko for udvikling af smerte efter ØA(artikel III).

At undersøge den helbredsrelaterede livskvalitet, oplevet stress, selvvurderet helbred, ophør af arbejdsmarkedstilknytning grundet sygdom eller erhvervsudygtighed samt de socialøkonomiske perspektiver af ØA, sammenlignet med den gennemsnitlige danske befolkning (artikel IV).

Studierne er baseret på: en journalgennemgang af 431 ØA patienter, en klinisk undersøgelse og et interview studie af 173 ØA samt et spørgeskema der blev besvaret af 120 ØA patienter.

Konklusion: Den hyppigste årsag til ØA i Danmark var i perioden 1996–2003: smertende blindt øje (37%) samt kræft (34%). I tidsperioden 1996–2003 var det årlige antal af ØA stabilt, men der var en stigning i brugen af evisceration. Orbitale implantater blev anvendt med stigende frekvens frem til 2003. Øjenfantomsyndromet er hyppigt blandt de ØA. Visuelle hallucinationer blev beskrevet af 42% af patienterne. Indholdet var primært elementære visuelle hallucinationer såsom hvidt eller farvet lys som et kontinuerligt skarpt lys eller som lysende objekter i bevægelse. De hyppigste udløsende faktorer for fantomsyn beskrives som; mørke, lukning af øjne, træthed og stress. 54% af patienterne oplevede visuelle hallucinationer mere end en gang om ugen. Ti patienter var så forstyrrede af deres fantomsyn at det forstyrrede dem i at leve deres liv som de ønskede.

Cirka 23% af alle ØA oplever fantomsmerte op til flere år efter operationen. Fantomsmerte blev beskrevet i tre kvaliteter: (i) skærende, borende, murrende eller trykkende (n = 19); (ii) jagende, udstrålende smerte eller elektriske stød (n = 8); (iii) overfladisk brændende, sviende eller stikkende smerte (n = 5); eller en kombination af to smertekvaliteter (n = 7). Den gennemsnitlige smerteintensitet (median) på en visuel analog skala gående fra 0 til 100, var 36 [range 1–89]. En tredjedel af patienterne oplever fantomsmerte hver eneste dag. De hyppigste udløsende faktorer for fantomsmerte beskrives som; kulde, blæst, stress og træthed. Smerte i øjet før ØA, tilstedeværelse af et implantat og rapportering af større mængder af sekret fra konjunktivalhulen er alle faktorer som er associeret med fantomsmerte. Den hyppigste årsag ØA er et smertende blindt øje, men en tredjedel af patienterne forsætter med at have smerte efter operationen. Fantomfølelse var til stede hos to procent af patienterne.

Indvirkningen af en ØA på patienternes liv synes omfattende. ØA patienter har dårligere helbredsrelateret livskvalitet, ringere selvvurderet helbred og mere oplevet stress end den gennemsnitlige danske befolkning. De største forskelle i helbredsrelateret livskvalitet mellem de ØA og den gennemsnitlige danske befolkning findes i områderne psykiske problemer og psykisk velbefindende. Patienter med indikationen smertende blindt øje har lavere score i alle dimensioner af helbredsrelateret livskvalitet og oplevet stress end patienter med indikationerne kræft og traume. Andelen af skilsmisse og separation er dobbelt så høj bland ØA som i normalbefolkningen. Yderligere er 25% gået på nedsat tid eller pension pga. øjensygdom og 39.5% af patienterne er ophørt med fritidsaktiviteter grundet deres ØA.

References

  1. Top of page
  2. Abstract.
  3. The Eye Amputated – Consequences of Eye Amputation with Emphasis on Clinical Aspects, Phantom Eye Syndrome and Quality of Life
  4. List of Papers
  5. Acknowledgements
  6. Conflicts of interest
  7. Abbreviations
  8. Introduction
  9. Aims of the Ph.D. Study and its Hypotheses
  10. Materials and Methods
  11. Strengths and Limitations of Methods
  12. Results and Discussion
  13. Conclusions of Thesis and Future Perspectives
  14. Summary in Danish
  15. References
  16. Recommended Books and Articles for EA Patients, Relatives and all Health Professionals with Contact with EA Patients
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Recommended Books and Articles for EA Patients, Relatives and all Health Professionals with Contact with EA Patients

  1. Top of page
  2. Abstract.
  3. The Eye Amputated – Consequences of Eye Amputation with Emphasis on Clinical Aspects, Phantom Eye Syndrome and Quality of Life
  4. List of Papers
  5. Acknowledgements
  6. Conflicts of interest
  7. Abbreviations
  8. Introduction
  9. Aims of the Ph.D. Study and its Hypotheses
  10. Materials and Methods
  11. Strengths and Limitations of Methods
  12. Results and Discussion
  13. Conclusions of Thesis and Future Perspectives
  14. Summary in Danish
  15. References
  16. Recommended Books and Articles for EA Patients, Relatives and all Health Professionals with Contact with EA Patients
  • • 
    Lis Mellemgaard. Kun et øje, 1 ed. Copenhagen: Borgens forlag, 1986.
  • • 
    Jay Adkisson. Lost eye: coping with monocular vision after enucleation or eye loss from cancer, accident or disease, 1 ed. Lincoln: iUniverse, 2006.
  • • 
    Karin Åbacken-Bjaring. Att offra ett öga för att vinna liv, 1 ed. Stockholm: Formulera, 1994.
  • • 
    Frank B. Brady. A Singular View: The Art of Seeing With One Eye. Hughes (6th ed) 2004.