An insight into vaginal surgery in a severely masculinized CAH patient.

Reconstruction and exteriorization of the "high" vagina in girls with congenital adrenal hyperplasia (CAH) is technically challenging. Although current surgical thinking favors correction in infancy the evidence from several long-term studies indicates that many patients will require further surgery in adolescence or adult life. Deferred vaginoplasty has been advocated because the procedure may be technically easier as a consequence of changes in the characteristics of the genital tissues and vaginal anatomy. The role of urogenital mobilization is as yet undetermined but carries promise for the short, high vagina. We describe a patient where the radiological and clinical findings support the argument for deferred vaginoplasty.

An objective assessment of the results of hypospadias surgery.

OBJECTIVE: To compare the cosmetic result of tubularized incised-plate urethroplasty (Snodgrass method) with that of two established techniques, the meatal-based flap and onlay island flap repair. SUBJECTS AND METHODS: Photographs of the penis after hypospadias repair in 32 boys were assessed by a panel of five independent health professionals, including four surgeons with variable paediatric urological experience and a urology nurse. Twenty patients had a distal and 12 a proximal meatus. The Snodgrass technique was applied by one paediatric urologist for either distal (10) or proximal (six) hypospadias. A Mathieu repair was used for distal hypospadias (10) and an onlay preputial island flap for proximal hypospadias (six) by a second paediatric urologist. The panel was asked to grade cosmesis as poor, unsatisfactory, satisfactory or very good (points 1-4) for each of the following aspects of penile appearance: meatus, glans, shaft and overall appearance. Photographs were taken in a standard way, with a standard distance, lighting and two views, one of the dorsal surface and one ventral, for each patient. Signed written consent for the study was obtained from each family. RESULTS: The mean assessment score for any aspect of cosmesis was significantly higher for the Snodgrass technique (P < 0.05). The mean score (95% confidence interval) for the meatus was 0.76 (0.4-1.1) points higher for the patients with a Snodgrass repair than those with a Mathieu or onlay island flap repair (P = 0.002). Correspondingly, the values for the glans were 0.67 (0.38-0.97) (P = 0.003), shaft 0.42 (0.16-0.69) (P = 0.01) and overall appearance 0.62 (0.24-1.0) (P = 0.01) points higher for the Snodgrass repair. The Snodgrass technique was more effective in producing a vertically orientated meatus (87.5%) than the Mathieu and Duckett onlay repairs (37.5%; P = 0.009). CONCLUSION: The Snodgrass technique, as assessed by this panel, had a better cosmetic outcome than the Mathieu and Duckett onlay island flap repairs. The assessment of cosmesis in hypospadias surgery is potentially more objective when several health professionals, not involved in the surgery, compared the various methods of repair.

Management of müllerian duct remnants.

OBJECTIVES: To describe the presentation and radiologic investigation of 6 patients who had persistent müllerian duct remnants (MDRs) in association with severe hypospadias and to review the long-term results of surgical management using the transtrigonal approach. METHODS: The hospital records of 6 patients who had MDRs and who underwent transvesical transtrigonal excision were reviewed and long-term assessment of urinary control and continence was made by interview. RESULTS: One patient presented in the newborn period with clinically palpable MDRs and five others escaped detection until after the hypospadias repair, despite ultrasonography of the pelvis specifically looking for MDRs. All had undescended testis with severe hypospadias. The karyotype was 45XO/46XY in five and 46XY in one. The most consistently useful examinations were voiding cystourethrography and magnetic resonance imaging. The excision was transtrigonal in each case. Early or late postoperative complications were not encountered in any of our patients. Gonadal histologic examination showed streak gonad in four and a dysgenetic testis in one. Histopathologic examination showed rudimentary uterus and fallopian tubes in 4 patients and a cystic structure lined with squamous and columnar epithelium in 2 patients. No areas of metaplasia were noted. Bladder emptying was normal after surgery. CONCLUSIONS: Ultrasound examination alone is not always sufficient to diagnose MDRs, which may be responsible for symptoms after hypospadias surgery. The transtrigonal approach provided excellent visualization and access to MDRs and prostatic urethra.