José Carnevale

The exstrophy-epispadias complex: is aesthetic appearance important?

To show the relevance of cosmetic appearance in the adequate treatment of patients with exstrophy‐epispadias complex (EEC), and to indicate that surgery by experienced teams can improve the long‐term treatment forecast and the patients body image.

SIGMOID RECONFIGURED VAGINAL CONSTRUCTION IN CHILDREN

PURPOSE We present a modified technique of sigmoid neovaginal construction in children that protects the sigmoid pedicle from traction, allows easy adjustment of caliber and reorients the mucosal fold in a longitudinal direction. MATERIALS AND METHODS From 1997 to 2000, 10 genetically male (46 XY) children 1 to 13 years old underwent construction of a neovagina with sigmoid, incorporating the Yang-Monti concept of intestinal reconfiguration. The diagnosis was androgen insensitivity in 7 patients, congenital adrenal hyperplasia in 2 due to 17 alpha-hydroxylase deficiency and 3 beta-hydroxysteroid dehydrogenase deficiency, respectively, and bladder exstrophy in 1 who required sex reassignment. RESULTS Eight children had an adequate caliber neovagina after an initial period of systematic dilation. In 1 case a relevant stricture required reoperation using the same technique and the outcome was good. In another child a stricture developed in the middle of the reconfigured sigmoid segment and a regular dilation schedule is still being followed after 23 months of followup. CONCLUSIONS The new sigmoid reconfiguration technique enables the use of smaller dimension intestinal segments and construction of a long vaginal conduit of adequate caliber. Its optimal adequacy for penetration must be assessed in the future after these patients begin sexual activity.

Posterior urethral injuries and the Mitrofanoff principle in children

To report our experience of children with trauma causing posterior urethral injury who at some stage underwent a Mitrofanoff intervention, as post‐traumatic urethral injuries can demand long‐term treatment which (regardless of the surgical intervention) requires a period of dilatation of the reconstructed urethra.

A posterior‐based omega‐shaped flap vaginoplasty in girls with congenital adrenal hyperplasia caused by 21‐hydroxylase deficiency

To describe a technical modification for constructing a vagina in girls with congenital adrenal hyperplasia caused by 21‐hydroxylase deficiency, using a narrower skin base but rich subcutaneous tissue, aiming to obtain both longer and larger vaginal segments with better cosmesis of the external genitalia.

Ectopic opening of the vas deferens into a Müllerian duct cyst.

Two consecutive cases of ectopic opening of the vas deferens into Müllerian duct cysts are presented, with descriptions of the presentation, physical examination, radiographic and ultrasonographic images, surgical findings and treatment undertaken. In both cases, ablation of the cyst and ligature of the vas deferens was the technique employed. Both patients are currently well, with normal urinary flow and no urinary tract infections, 3 years after surgery. Ectopic opening of the vas deferens into Müllerian duct cysts is a rare entity but should always be considered when a child presents with purulent urinary discharge and abdominal tumor. Surgery is the only treatment recommended.

GASTROCYSTOPLASTY AND CHRONIC RENAL FAILURE: AN ACID-BASE METABOLISM STUDY

PURPOSE To verify in an experimental model whether gastrocystoplasty may protect patients with chronic renal failure from acid loading associated acidosis a wedge-shaped portion of the middle stomach was used to improve bladder capacity in animals with chronic renal failure. MATERIALS AND METHODS An experimental model was used to study 112 adult female Wistar rats (EPM-1) weighing between 156 and 259 gm. The animals were randomly assigned to groups, including 41 controls, 24 undergoing five-sixths nephrectomy to create chronic renal failure, 26 undergoing gastrocystoplasty and 21 undergoing gastrocystoplasty and five-sixths nephrectomy to create renal failure. To provide an acid overload a 5% NH(4)Cl diet was administered to a subgroup of each group. Two months after surgery 24-hour urine was collected, and volume and pH were measured as well as the amount of bicarbonate, ammonium, titratable acidity and chloride. The animals were then exsanguinated through an abdominal aorta puncture. The blood was used for blood gas analysis and to measure sodium, potassium, chloride, ionized calcium and creatinine. RESULTS When undergoing an acid overload, the animals with gastrocystoplasty had no acidosis since acid radicals were eliminating in the urine as NH(4)Cl. When given the same acid overload, metabolic acidosis developed in the animals with gastrocystoplasty and chronic renal failure. CONCLUSIONS Gastrocystoplasty protected healthy rats from acidosis when they were given an acid overload but failed to protect the rats with chronic renal failure under the same conditions.

Spinal lipoma associated with urethral duplication

Urethral duplication is a rare anomaly with varied clinical manifestations such as urinary tract infections, serous discharge from sinus, outflow obstruction and urinary incontinence. A spinal cord lipoma is fat within the normally positioned spinal cord without any skin or bony abnormalities. Most commonly these rare lesions are usually present at birth with an obvious lumbosacral mass. Spinal cord compression can cause numbness and tingling, weakness, difficulty, incontinence and stiffness of the extremities. We describe an interesting case of spinal lipoma associated with urethral duplication.

Bladder acontratility after hypospadia repair

Hinman and Bauman brought attention to an entity that causes unilateral or bilateral hydronephrosis, vesicoureteral reflux, and trabeculated or irregular bladder walls. First referred to as non-neurogenic neurogenic bladder, the disease is a detrusor hyperactivity that patients try to suppress by increasing the activity of the external sphincter. Later the entity became known as Hinman Syndrome. The name was first improperly used, not discriminated, for different voiding dysfunction conditions which led the International Childrens Continence Society to reserve the name “Hinman Syndrome” only for more severe cases, of relevant consequences for the patients renal function. Voiding dysfunctions, particularly in the presence of a vesicosphincterian dyssynergia, may develop into hydronephrosis and renal insufficiency if inadequately treated, in which case being referred to as Hinman syndrome. We present the case of a boy who developed full detrusor acontractility after undergoing a distal hypospadias repair, with no mechanical cause associated. As he was treated early by means of an appendicovesicostomy the development of hydronephrosis and of renal insufficiency that characterize Hinman Syndrome was possibly prevented. Material and methods

Cobb's collar and chronic renal failure

Cobbs collar is a membranous stricture of the bulbar urethra, often underestimated and non-diagnosed. Dewan presented evidence of a common morphological diagnosis for patients presenting with congenital obstructive posterior urethral membrane. The term COPUM (Congenital obstructive posterior urethral membrane) has been suggested in that two distinct pathologies should be distinguished. In boys with a COPUM, the obstructing membrane is just distal to the external sphincter and reinforced by folds extending down from the verumontanum, which when split resemble the valve obstruction suggested by Young. The COPUM is probably due to persistence of the attachment of the verumontanum to the anterior wall of the posterior urethra. Cobb described a congenital narrowing of the bulbar urethra that probably represents an embryological process different from that of the COPUM. Cobbs collar is most likely due to a partially persistent urogenital membrane. We report the case of a child with Cobbs collar having had, in other Service, endoscopic fulguration as if being treated for posterior urethral valve. When undergoing cystoscopy, the obstructive ring was found to be fully intact as if never treated before. The child evolved into chronic renal failure.