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Keywords:

  • Total urogenital sinus mobilization;
  • cloaca;
  • urogenital sinus;
  • bladder exstrophy;
  • congenital adrenal hyperplasia;
  • penile agenesis

Abstract

  1. Top of page
  2. Abstract
  3. Introduction
  4. Patients and methods
  5. Results
  6. Discussion
  7. Acknowledgements
  8. References

Objective

To report further applications of total urogenital sinus mobilization, earlier described as an easier method to correct a cloaca.

Patients and methods

Seven children (six girls and one boy, mean age 4 years, range 3 months to 10.5 years) underwent surgery and were followed for a mean of 1 year; their diagnoses included persistent cloaca and congenital adrenal hyperplasia (CAH) in two each, and a urogenital sinus (UGS), bladder exstrophy and penile agenesis in one each. The UGS is approached through a posterior sagittal incision and dissected circumferentially to the retropubic space, allowing the UGS to descend. It is then excised and separate openings of the vagina and urethra created. This technique is applicable to a UGS of ≤3 cm.

Results

In all patients, separate openings for the urethra and vagina were created. In three patients urinary continence was preserved after surgery. The patient with bladder exstrophy remains incontinent. The remaining patients are too young to assess (not yet toilet-trained).

Conclusion

This technique simplifies the surgical correction of UGS malformation; we confirm its usefulness in cases of persistent cloaca. It is also valuable in patients with CAH, primary UGS and in selected patients with bladder exstrophy and penile agenesis. When the UGS is not associated with a cloaca, the procedure can be performed perineally. Despite circumferential mobilization of the UGS, urinary continence is preserved.


Introduction

  1. Top of page
  2. Abstract
  3. Introduction
  4. Patients and methods
  5. Results
  6. Discussion
  7. Acknowledgements
  8. References

The surgical correction of the persistent urogenital sinus (UGS) depends on the length of the sinus, the length of the urethra proximal to the sinus and the presence of anorectal anomalies (persistent cloaca). A UGS with a low confluence can be managed with a simple ‘cutback’ operation, with the addition of an inverted U-flap to prevent introital stenosis [ 1]. A UGS of >3 cm long or those with a short urethra proximal to the confluence require the sinus to be preserved, as urethral and distal vaginal reconstruction with skin flaps or intestine is required [ 2].

In 1997, Peña described the procedure known as total urogenital sinus mobilization (TUM) for sinuses <3 cm long [ 3]. In the original description, the UGS was approached through a posterior sagittal incision, dissected circumferentially to the retropubic space, allowing the urethra and vagina to descend. The entire UGS was then incised or excised and separate openings of the vagina and urethra created. Usually the repair of UGS malformations needs separation of the vagina from the urethra; this step is technically difficult and time-consuming. The main advantage of TUM is to avoid complications of vaginal separations (vaginal stricture, fistula or loss). Peña applied the procedure only to patients with a persistent cloaca [ 3]; we report extended applications for this procedure.

Patients and methods

  1. Top of page
  2. Abstract
  3. Introduction
  4. Patients and methods
  5. Results
  6. Discussion
  7. Acknowledgements
  8. References

The patients were selected based on the length of the UGS (≤3 cm) and an adequate urethra proximal to the confluence. Seven children (six girls and one boy, mean age 4 years, range 3 months to 10.5 years) underwent TUM and were followed for a mean of 1 year. The diagnoses included a persistent cloaca in two, congenital adrenal hyperplasia (CAH) in two, and UGS, bladder exstrophy and penile agenesis in one each.

The urethral length and UGS were assessed endoscopically; the surgical approach was posterior sagittal in the two girls with a cloaca and perineal in the other patients. The sinus, vagina and urethra to the peritoneal reflection and the retropubic area were dissected circumferentiallly (Fig. 1). In two patients, vaginoplasty was completed with a posterior inverted U-flap and in one with labial flaps. In the child with penile agenesis, the vagina was created with a vascularized colonic segment.

image

Figure 1. The technique used to mobilize and advance the urogenital sinus when it is <3 cm long. An incision is made from the meatus of the sinus and it is dissected circumferentially. The dissection should reach the prevesical space anteriorly and the peritoneal reflection posteriorly. a, The sinus is pulled down and excised. The posterior vaginal wall is incised to accommodate the perineal U-flap. b, Separate vaginal and urethral openings are created in the vestibule.

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Results

  1. Top of page
  2. Abstract
  3. Introduction
  4. Patients and methods
  5. Results
  6. Discussion
  7. Acknowledgements
  8. References

In all patients, separate openings for the urethra and vagina were created. In two patients (one CAH, one UGS) urinary continence was preserved after surgery. They were toilet-trained well and had no other risk factors for incontinence. The patient with exstrophy remains incontinent; the remaining patients are too young to be assessed as they are not yet toilet-trained.

Discussion

  1. Top of page
  2. Abstract
  3. Introduction
  4. Patients and methods
  5. Results
  6. Discussion
  7. Acknowledgements
  8. References

TUM simplifies the reconstruction of intermediate-length UGS malformations. In patients with a normal anus and rectum, the procedure can be performed perineally; our experience confirms the usefulness in those with a persistent cloaca. In addition, it was valuable in patients with CAH, a primary UGS, bladder exstrophy and penile agenesis. For example, at presentation, the girl with complications from a neonatal closure of a bladder exstrophy had a single opening of the urethra and the vagina, and massive hydronephrosis. There was distal urethral and vaginal stenosis. After descent and excision of this iatrogenic sinus, separate openings for the urethra and vagina were obtained and the vaginal introitus was located further posteriorly, in a more normal position. The 16-month-old child with an XY karyotype, penile agenesis and recto-urethral fistula presented after detachment of the recto-urethral fistula with severe anorectal stenosis and a colostomy. The perineal opening of the urethra was close to the anus. After correcting the rectal stenosis, total mobilization of the urethra to a more anterior position in the retropubic space was used to create enough room between the urethra and rectum to place a sigmoid neovagina.

Although initially there was concern that the circumferential dissection encompassing the urethra and vagina might compromise continence, the two patients who were continent before the repair remained so. The anatomical justification for this is the presence of adipose tissue between the symphysis pubis and the urethra, already present in the embryo, suggesting that no muscular structures are divided with this dissection [ 4].

Technically, it is important that the dissection is carried to the prevesical space anteriorly and to the peritoneal reflection posteriorly to obtain good caudal mobilization of the sinus. The sinus can be excised, but it can also be incised in the sagittal or in the coronal plane to create mucosal flaps for the vulva when needed.

Acknowledgements

  1. Top of page
  2. Abstract
  3. Introduction
  4. Patients and methods
  5. Results
  6. Discussion
  7. Acknowledgements
  8. References

The authors thank Prof. Dr H. Fritsch (Professor of Anatomy, University of Innsbruck, Austria) for her help with the embryology.

References

  1. Top of page
  2. Abstract
  3. Introduction
  4. Patients and methods
  5. Results
  6. Discussion
  7. Acknowledgements
  8. References
  • 1
    Fortunoff S, Lattimer JK, Edson M. Vaginoplasty technique for the female pseudohermaphrodite. Surg Gyn Obstet 1964; 118: 545 8
  • 2
    González R & Fernandes E. Single-stage feminization genitoplasty. J Urol 1990; 143: 776 8
  • 3
    Peña A. Total urogenital mobilisation — an easier way to repair cloacas. J Pediatr Surg 1997; 32: 263 8
  • 4
    Fritsch HEntwicklung des Beckenbindegewebes. Eine morphologische Studie bei Feten und Neugeborenen. Habilitatiosschrift. Lübeck 1991