The laparoscopic management of intersex patients: the preferred approach

Authors


Francisco T. Dénes, Division of Urology, University of São Paulo School of Medicine Hospital, Caixa Postal 11273–9, CEP 05422–970, São Paulo, SP, Brazil.
e-mail: f.c.denes@br2001.com.br

Abstract

In the world's largest series of patients with intersex treated by laparoscopy, authors from Sao Paulo found that this technique allowed easy identification and removal of gonads. They also found that other organs could be removed and genitoplasty performed.

OBJECTIVE

To present possibly the largest series of the use of laparoscopy for treating intersex patients.

PATIENTS AND METHODS

Fifty intersex patients (34 with male and two with female pseudohermaphroditism, nine with gonadal dysgenesis, four with true hermaphroditism, and one with complex hypospadias), aged 0.5–46 years (mean 18.3), underwent laparoscopy to remove gonads and/or ductal structures incompatible with the social gender, or for gonadal tumour or a potential risk for malignancy. When necessary, genitoplasty was performed concomitantly.

RESULTS

At the laparoscopic evaluation, 10 gonads of six patients were absent, while four were identified as ‘vanishing’; 72 gonads (46 dysgenetic, 17 normal testes, one normal ovary, one ovotestis, seven gonadoblastomas or dysgerminomas) were removed; two ovotestes were replaced in the scrotum after removing the ovarian segment, as was one normal testis. Twelve patients with a urogenital sinus had its vaginal component removed, 11 including a hysterectomy. Three of these patients had a combined perineal approach to complete its removal, together with masculinizing genitoplasty. There were no intraoperative complications or conversions; two patients had complications after surgery.

CONCLUSIONS

Laparoscopy allows the straightforward identification and removal of gonads. All abnormal ductal structures must be removed, as this increases the chance of resecting unidentified gonads. Removing the uterus and vaginal component of the urogenital sinus in patients with male social sex is feasible, with low morbidity. Genitoplasty, according to the social sex, can be performed in the same procedure.

Abbreviations
MPH

male pseudohermaphroditism

FPH

female pseudohermaphroditism

GD

mixed gonadal dysgenesis

TH

true hermaphroditism

INTRODUCTION

The management of patients with intersexual states is clinically demanding. Only with a careful clinical evaluation can the correct diagnosis be established, therefore reducing the probability of therapeutic misconduct (mainly for sex assignment). The therapeutic objective is to offer the best quality of life, reconciling structural limitations with the functional potential of the external genital organs, and adjusting them to the psychological characteristics and gender preferences of the patient, whenever defined [1,2].

Laparoscopy was recently introduced as a method of evaluating and treating intersex patients with impalpable gonads [1,3]. The objective of the present study was to report the results of what is, to our knowledge, the largest series of laparoscopic procedures in intersex patients.

PATIENTS AND METHODS

From March 1994 to April 2004, 50 patients with intersex disorders (mean age 18.3  years, range 0.5–46) were treated at our institution. The disorders included male pseudohermaphroditism (MPH) in 34 patients, female pseudohermaphroditism (FPH) in two, mixed gonadal dysgenesis (GD) in nine, true hermaphroditism (TH) in four and complex hypospadias in one. Laparoscopy was indicated for evaluation, but mainly to remove unwanted gonads and ductal structures. Whenever necessary, associated genitoplasty was also performed.

The preoperative evaluation included a careful clinical history and detailed physical examination, particularly of the external genitals and perineal orifices, with palpation of the inguinal area [1]. Hormonal, cytogenetic and radiological investigation (pelvic ultrasonography and retrograde genitography) were also used.

The patients had a general anaesthetic with endotracheal and nasogastric intubation. The external genitals were re-evaluated under anaesthesia, to plan the extent of the eventual reconstruction in the same procedure. The entire abdomen and the genital area were cleansed and prepared. When combined genitoplasty was planned, the patient was placed in the semi-lithotomy position, or otherwise supine. The video monitor, insufflator and light source were positioned at the foot of the patient. The surgical technique included the classical steps for laparoscopic surgery, i.e. peritoneal insufflation with a Veress needle inserted infraumbilically, insertion of a 5–10 mm umbilical trocar for laparoscopic evaluation, and two or three additional pelvic trocars for therapeutic procedures. The patient was then placed in a Trendelenburg position. The gonadal structures were evaluated initially, and when necessary, the bowel retracted. In some cases when the gonads are not easily seen, the gonadal vessels may be identified and followed downwards. Most often, the gonads are identified near the inguinal region, eventually with normal testicular or ovarian appearance, but also with a dysplastic or tumoral aspect. In some cases the gonads are not clearly identified because of dysplasia, sometimes leading to confusion with ductal structures. Once identified, the gonads are resected, most often together with the ductal structures. In the presence of a normal testis in a patient with a male social sex, laparoscopic orchidopexy can be performed [2].

MPH represents the most frequent indication for therapeutic laparoscopy [1]. As the patient with an underdeveloped phallus is generally orientated to the female gender, the testes must be resected. When present, the hypoplastic uterus should be left, to allow the possibility of menstruation and pregnancy [4,5]. When the testes are palpable, orchidectomy can be done through inguinotomies, but as most such patients have impalpable testes, a laparoscopic exploration and gonadectomy is indicated [6,7]. When the patient is assigned to or assumes a male role, laparoscopic gonadectomy is still necessary when the gonads are dysgenetic or tumoral, but when the testes are normal, orchidopexy is indicated. In cases of MPH with male gender, resection of Müllerian duct derivatives is always necessary [8]. In the rare cases of FPH with a male social sex, laparoscopic gonadectomy with resection of Müllerian duct derivatives is also always necessary. In patients with TH and female social sex, laparoscopic orchidectomy or resection of testicular tissue from the ovotestis is indicated. In patients with TH and male social sex laparoscopic resection of the Müllerian duct derivatives, ovary or the ovarian tissue from ovotestis is indicated, as well as orchidopexy in selected cases. In patients with GD, particularly those with a Y chromosome, gonadectomy is essential, while resection of Müllerian duct derivatives is indicated in patients with male social sex [8].

Because of social and geographical conditions, most of the present patients were first evaluated as adults, and their treatment adjusted to the already defined sexual role. The laparoscopic procedures, according to clinical diagnosis, social sex and laparoscopic findings, are summarized in Table 1. When necessary, associated genitoplasty is also performed, according to the sexual role of each patient.

Table 1.  Guidelines for the laparoscopic management of the gonads and Müllerian derivatives in intersex patients
Clinical intersex diagnosesPossible findingsSocial sexLaparoscopic management
  1. MDD, Müllerian duct derivatives.

FPHNormal gonads, MDDMaleGonadectomy resection of MDD
MPHNormal gonads and/or dysgenetic gonads; MDDFemaleGonadectomy
MaleGonadectomy (when dysgenetic)/orchidopexy (when normal)
Resection of MDD
THNormal gonads, ovotestis MDDFemaleOrchidectomy/resection of testicular tissue from ovotestis
MaleResection of ovarian tissue from ovotestis/orchidopexy
Resection of MDD
GDDysgenetic gonads, MDDFemaleGonadectomy (Y chromosome)
MaleGonadectomy (Y chromosome), resection of MDD

We evaluated the efficacy of laparoscopy in identifying the gonads, the success of gonadal and ductal resection, the relationship between surgical findings and gonadal histology, the need for conversion to an open procedure, the incidence of complications during or after surgery, and the need for blood transfusion.

RESULTS

All 50 patients had a laparoscopic evaluation that identified the pelvic structures. Ten gonads in six patients were absent on laparoscopic evaluation. Two patients had bilateral ‘vanishing testes’ identified by blind-ending gonadal vessels. We defined laparoscopically 86 structures as gonads; some of these were not confirmed histologically, as described below.

The ductal structures were also very variable, ranging from normal epididymis and Fallopian tubes to vestigial structures associated or not with normal or hypoplastic uteri. Differences were also noted between sides in the same patient. In all, 17 uteri and 12 urogenital sinuses were confirmed laparoscopically.

After laparoscopic evaluation there were 38 bilateral and seven unilateral procedures intended to removed gonads and ductal structures, as well as 12 resections of the vaginal component of the urogenital sinus, six hysterosalpingectomies and five hysterectomies, one unilateral orchidopexy, and one resection of an ovarian segment of ovotestis associated with orchidopexy (Fig. 1). One bilateral herniorrhaphy and one cholecystectomy were also performed.

Figure 1.

A 22-year-old with TH (XX, SrY+) with a male social sex: Left, genitography showing the bladder, urogenital sinus, uterine cavity and tubes. Right, the result of laparoscopic hysterosalpingectomy with unilateral gonadectomy (ovary) and urogenital sinus resection (surgery also included open orchidopexy for palpable right testes and masculinizing genitoplasty).

Only 75 gonads were confirmed histologically, including five that were not identified during surgery, but were resected incidentally together with the ductal structures. These gonads were either normal but contrary to the social sex, dysgenetic or tumoral.

Three of the 12 patients with a urogenital sinus who had its vaginal component removed laparoscopically required a combined perineal approach to complete the removal, which was accomplished together with masculinizing genitoplasty. Associated genital masculinization was done in 12 patients and feminization in seven. One ovotestis was replaced without laparoscopy in the scrotum after removing the ovarian segment.

Five patients had seven neoplastic gonads (represented mainly by gonadoblastoma, a tumour of low malignant potential, and dysgerminoma, a malignant tumour), but only three of these gonads were enlarged (as seen before surgery on imaging, and during laparoscopy), suggesting tumoral involvement (Fig. 2) (Table 2).

Figure 2.

A 14-year-old with GD (46,XY/45,X; female social sex). A laparoscopic view of the increased left gonad; the diagnosis was gonadoblastoma.

Table 2.  Clinical and histopathological information of patients with gonadal tumours; the incidence was seven in 75 gonads (9%) and five of 50 patients (10%)
Age, yearsSocial sexKaryotypeClinical diagnosisHistological finding, right/left
13Male46,XYTHOvotestis with gonadoblastoma/gonad absent
14Female46,XYq+/45XGDHypoplastic ovary/gonadoblastoma
20Female46,XYMPHGonadoblastoma/dysgerminoma
20Female45,X/46,XYGDDysgerminoma + gonadoblastoma both sides
23Male46,XYGDLeydig cell hyperplasia/gonadoblastoma + mixed germinative cell tumour

All procedures were completed with minimal blood loss, except in one patient who had significant bleeding during genitoplasty, receiving one unit of blood. The duration of the procedures was 55–270 min, including associated genitoplasty. There were no complications during surgery nor conversion to laparotomy, but in one patient with TH the resection of the ovarian portion of the ovotestis and subsequent orchidopexy was done through an inguinal incision, after laparoscopic removal of the uterus and urogenital sinus, and the contralateral gonad. Only two patients had complications after surgery, one with an umbilical port infection and another with a pelvic abscess, both successfully treated with antibiotics. When there was only a laparoscopic procedure the hospital stay was 1–3 days, and with associated genitoplasty the stay was 6–11 days.

DISCUSSION

The first laparoscopic bilateral gonadectomy in an intersex patient was reported in 1992 and since then this procedure has gradually become the standard for evaluating and treating the internal genital organs in these patients [9,10]. The classical advantages of laparoscopy include the elimination of a sizeable laparotomy incision, resulting in less discomfort after surgery, less need for analgesia, and a shorter hospital stay, convalescence and return to normal activities [10]. Other advantages include magnification, excellent visibility and illumination, and less venous oozing because of the pressure effect of the pneumoperitoneum. One of the main advantages of this method is the lack of scars, a very important issue for these patients, who need reaffirmation of their body image and self-esteem [1].

The intersexual states for which laparoscopy is more frequently used are MHP, FHP, TH and GD [1]. It is helpful for gonadal evaluation, resection or biopsy, and for identifying internal ductal derivatives [3,11]. It is also used for removing all normal structures contrary to the assigned social sex, as well as gonads that are either dysgenetic, nonfunctional or malignant or of increased malignant potential [12,13].

Laparoscopy gives an excellent view of the pelvic structures, including the genital organs. In most cases identifying these structures is easy and their removal straightforward. However, the accuracy of identifying the gonads is not total. As documented here, there are cases where structures identified as gonads and removed were not confirmed histologically as such. On the other hand, some dysgenetic gonads were not identified, being removed incidentally as ductal structures. This reinforces the need to remove all ductal structures when the gonads are not clearly identified, as most unseen dysplastic and potentially malignant gonads will be thus removed.

The relative risk for testicular germ-cell tumours associated with intersex syndromes is increased more than 100 times, justifying prophylactic gonadectomy as soon as is feasible after the diagnosis is established [14]. The risk of gonadal neoplasia is not confined to patients with a 46,XY karyotype, but extends to patients with GD and any mosaic karyotype containing a Y chromosome or the SRY antigen [15]. In the present patients 9.3% of the gonads in 10% of the patients had tumours, but only three of them had macroscopic abnormalities suggesting tumour.

We treated all patients by laparoscopy, with no conversion to laparotomy, or significant bleeding or intraoperative complications. The most difficult procedures are those associated with resecting the vaginal portion of the urogenital sinus, particularly in those whose distal end extends beyond the urogenital diaphragm. The complication rate after surgery was 4%, including two localized infections treated successfully with conservative measures.

In conclusion, laparoscopy allows easy visualization of impalpable gonads, ductal remnants and urogenital sinus in intersex patients. All procedures necessary for adequately treating intersex disorders can be done with few complications and all the advantages of the laparoscopic procedures, even in small children. All dysgenetic, nonfunctioning and neoplastic gonads, and contradictory ductal structures, can be resected. In cases where the gonad is not clearly identified, ipsilateral ductal derivatives must be resected, as some specimens may include undetected dysgenetic gonads. Laparoscopic resection of urogenital sinus, with or without the uterus, is feasible. Complementary perineal resection of the inferior vaginal segment of the urogenital sinus may be required. Genitoplasty, according to social sex, can be performed simultaneously with laparoscopy.

CONFLICT OF INTEREST

None declared.

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