Justin H. Kelly

Vesical exstrophy: repair using radical mobilisation of soft tissues

By more radical mobilisation of both internal and external sphincter muscles of the bladder and urethra than has hitherto been undertaken, physiological continence of urine has been achieved in patients with vesicourethral exstrophy. The purpose of this paper is to describe the surgical technique used in the type regarded as the classic anomaly, though the principles apply to all variants in males and females. The key features are relevant to the second stage of a three-stage procedure. The periosteum on the inner sides of the ischium and pubis with the attachments of the involuntary and voluntary sphincter muscles and the pudendal vessels and nerves is mobilised on both sides so that these outstretched muscles can be converted into a wrap around the newly constructed “membranous” urethra without the need for iliac or pubic osteostomy. This new “membranous” urethra is made from the urethral plate, which is uplifted from its bed on the dorsum of the penis, rotated posteriorly between the crura, hinged proximally at the level of the verumontanum, tubularised, and relocated posteriorly within the grasp of the external sphincter complex. This necessitates the construction of an interim penoscrotal urethrostomy prior to penile urethroplasty. The physiological continence rating achieved so far in 19 patients is 73%. The identification and localisation of a latent but effective external sphincter muscle complex situated deep in the pelvis and totally separated from the dorsal penile plate in the male and from the urethrovaginal outlet in the female formed the basis and rationale of this new radical soft-tissue mobilisation technique.

Female epispadias: are we missing the diagnosis?

To describe three of four female patients with occult epispadias, as usually epispadias is reported to be a rare condition in females (1/480 000), and obvious and easy to diagnose, but less severe variants may still involve urethral sphincter incompetence in the absence of obvious clinical signs.

Balanitis xerotica obliterans with urethral stricture after hypospadias repair

Abstract Three cases of urethral stricture due to balanitis xerotica obliterans (BXO) after hypospadias repair are reported. The first patient showed white, dense scarring on the prepuce before the hypospadias repair and developed a stricture of the urethra after the operation. The second and the third were uneventful for 6 and 2 years, respectively, after the hypospadias repair, and then developed urethral strictures. Pathologic diagnosis of the stenotic lesion is essential. Complete excision of the affected urethra with topical steroid ointment or sublesional triamcinolone injection is recommended for this condition. Although the complication of BXO after hypospadias repair is rare (3 out of 796 cases with hypospadias in our series), surgeons need to be aware of this condition as a cause for late onset of urethral problems.

Urinary tract abnormalities in association with oesophageal atresia: frequency, significance, and influence on management

Urinary tract abnormalities occur in about 24% of infants with oesophageal atresia. It is important in the neonatal period to recognise those that may cause renal damage if untreated so that the long-term complications of renal disease, e. g., reflux-associated nephropathy, can be prevented. Our current practice is to obtain a renal ultrasound and micturating cystourethrogram during the first admission. If the infant has not been observed to pass urine, then renal ultrasound should be performed before repair of the oesophageal atresia, because there is inadequate functioning renal tissue for long-term survival in 3% of oesophageal atresia patients. The absence of Potters syndrome in infants with both oesophageal atresia and bilateral renal agenesis means that these patients often cannot be identified pre-operatively on clinical grounds alone.

Girth-reduction clitoroplasty : a new technique : experience with 37 patients

A new method of clitoroplasty is described that more closely simulates normal clitoral anatomy than previously described methods. The technique has been used successfully over the past 15 years in 37 patients whose underlying conditions were: congenital adrenal hyperplasia (24); mixed gonadal dysgenesis (6); partial androgen insensitivity (5); and exogenous androgen stimulation (2). The key points of this new technique are: (1) minimisation of blood loss; (2) reduction in clitoral girth rather than length; (3) preservation of the vascular and nerve supplies; and (4) creation of a hair-pin bend in the shaft of the clitoris, which simulates normal female anatomy as well as achieving an apparent reduction in length. Irrespective of the cause or severity of the virilisation, all patients obtained a good cosmetic result, 78% being regarded by their parents as appearing like a normal female. Five early patients required further surgery to achieve the desired results. Confident from experience that a good cosmetic result can be achieved without major surgical risk, even in a neonate, we now advocate surgical correction at the earliest possible age: as soon as an accurate diagnosis has been made, the parents have accepted the gender assignment, and the infants metabolic condition has been stabilised.

Inverted Y Duplication of the Ureter in Association with Ureterocele and Bladder Diverticulum

We report a case of inverted Y duplication of the ureter in association with an obstructing ectopic ureterocele and paraureteral diverticulum. Preoperative radiological studies demonstrated the ureterocele and diverticulum but they failed to show the duplex distal ureter. The duplicated section of ureter was excised and a Cohen reimplantation was performed. The presence of a large ureterocele obstructing the bladder neck, a diverticulum, failure of reflux into the ectopic segment and poor function of the affected kidney all contributed to the difficulties in establishing a preoperative diagnosis.

Minimal surgery in the prune belly syndrome

In the past 20 years 14 patients with the prune belly syndrome have been seen. A treatment regimen consisting of minimal operative intervention, prophylactic antibiotics, and regular surveillance has been followed. Four patients died in the first 4 weeks of life from grossly inadequate renal function and other anomalies. Another patient died in early adult life after a late presentation. The remaining 9 patients have recently been plan using minimal surgical intervention is a satisfactory alternative.

Congenital mesoblastic nephroma: treatment options

Congenital mesoblastic nephroma (CMN) is an uncommon tumour in the neonatal period. Many CMNs are of “typical” histology and require no therapy other than nephrectomy. However, some are of “atypical” histology and are known to recur and metastasize. The use of chemotherapy in the atypical variant and in case of recurrence has been a matter of controversy. The presence of tumour at the excision margin seems to be the only important predictor of recurrence. The role of heminephrectomy for localised lesions has not been defined. Five cases of CMN seen at the Royal Childrens Hospital, Melbourne, are discussed. Two of them were atypical histologically. One child had a heminephrectomy and is among the first to have had this procedure for CMN. Current treatment options are reviewed in relation to existing knowledge of the pathology and natural history of CMN.

Posterior ectopia of penis mimics marsupial anatomy

In kangaroos, the penis lies posterior to the scrotum. We report an infant born with a similar arrangement initially thought to be penile agenesis. In addition, there was a dysplastic horseshoe kidney: two ureters and the rectum drained into a primitive cloaca which in turn drained externally through an anal canal. Previous reports of “penile agenesis” have consistently described a perineal skin tag immediately anterior to the anus; postmortem dissection of this patient revealed intact corpora extending from the pubis to the perineal appendage into which ran the urethra, suggesting that the patient had concealed posterior ectopia rather than penile agenesis.

Prenatal intussusception of a vitello-intestinal duct in association with ileal atresia

A complication of patent vitello-intestinal ducts is intussusception producing a double-horned prolapse of small bowel through the duct. We report a previously undescribed variant where prolapse occured prenatally and resulted in complete atresia of the distal horn as far as the transverse colon.