Objective To review the results of gender reversal in six rearing, with ages ranging from 3 years to 16.5 years (mean 8.5) at the time of review. Two children have patients with 46XX congenital virilizing adrenal hyperplasia (CVAH).normal penises and four have a satisfactory result after two-stage repair of hypospadias/chordee. Patients and methods Fifty-one patients with 46XXCVAH were seen in an 8 year period; 45 were managed Conclusion Most patients with 46XX CVAH are preferably raised as females and require a feminizing by conventional feminizing genitoplasty, but six underwent gender reversal and were managed as males.genitoplasty. However, the clinical decision may be influenced by many factors, including delay in diagThe clinical decision for gender reversal was made after appropriate counselling and was based primarily nosis, social bias and the premium on male rearing in certain communities. When male rearing is chosen, on parental choice, this being influenced significantly by a delayed diagnosis in four patients. Surgical manearly gonadectomy and excision of Mü llerian structures, together with staged hypospadias repair, gives agement consisted of gonadectomy, excision of Mü llerian structures and staged hypospadias repair/ satisfactory results.

No abstract

The tenth occurrence of complete unilateral agenesis of the diaphragm (McKusick 22240) in siblings is reported in this study. It is likely that parents of children with this type of congenital diaphragmatic hernia have a significantly increased recurrence risk for future offspring. Genetic counselling and antenatal ultrasound should be considered for subsequent pregnancies in families with a previous child with complete agenesis of the diaphragm.

Background: Hydronephrosis secondary to pelviureteric junction (PUJ) obstruction is common in infancy and childhood. Pyeloplasty has until recently been the accepted method of management, but alternative endo-urological techniques have evolved in the last decade. Methods: Published results of conventional pyeloplasty for primary PUJ obstruction in children were compared with published results of endo-urological procedures. Results: Sixty-six pyeloplasties were performed in 61 children in a 6-year period. During a similar period, 63 primary endo-urological procedures were reported in the literature. The success rate after pyeloplasty was 95.5% compared with 65% after endo-urology. Conclusions: Conventional pyeloplasty is superior to endo-urology and should remain the gold standard for the treatment of primary PUJ obstruction in children.

Aim:Review of our experience and to develop an algorithm for salvage procedures in the management of hypospadias cripples and treatment of urethral strictures following hypospadias repair.Methods:This is a retrospective review of hypospadias surgeries over a 41-month period. Out of a total 168 surgeries, 20 were salvage/re-operative repairs. In three children a Duplay repair was feasible, while in four others a variety of single-stage repairs could be done. The repair was staged in seven children – buccal mucosal grafts (BMGs) in five, buccal mucosal tube in one, and skin graft in one. Five children with dense strictures were managed by dorsal BMG inlay grafting in one, vascularized tunical onlay grafting on the ventrum in one, and a free tunical patch in one. Three children were treated by internal urethrotomy and stenting for four weeks with a poor outcome.Results:The age of children ranged from 1.5–15 years (mean 4.5). Follow-up ranged from 3 months to 3.5 years. Excellent results were obtained in 10 children (50%) with a well-surfaced erect penis and a slit-like meatus. Glans closure could not be achieved and meatus was coronal in three. Two children developed fistulae following a Duplay repair and following a staged BMG. Three repairs failed completely – a composite repair broke down, a BMG tube stenosed with a proximal leak, and a stricture recurred with loss of a ventral free tunical graft.Conclusions:In salvage procedures performed on hypospadias cripples, a staged repair with buccal mucosa as an inlay in the first stage followed by tubularization 4–6 months later provides good results. A simple algorithm to plan corrective surgery in failed hypospadias cases and obtain satisfactory results is devised.