The management of hypospadias when associated with anorectal malformation (ARM) represents a unique challenge for the surgeon. Often there will be associated malformations and a need for multiple surgical procedures as well as invasive testing. The pediatric urologist must be aware of the immediate and long term needs of these patients prior to the hypospadias repair. We reviewed our experience with hypospadias in this population to propose a rational guideline for the timing of surgical management.
A retrospective chart review was performed of all male patients with the diagnosis of ARM and hypospadias evaluated since 1980. Patients with bladder or cloacal exstrophy were excluded from the analysis. Data was abstracted from the medical record including level of defect, neurogenic bladder, and renal anomalies. Renal anomalies included: vesicoureteral reflux, hydronephrosis, solitary kidney, and renal dysplasia. Proximal hypospadias was defined as a urethral opening located mid-shaft or lower and distal hypospadias included glanular, subcoronal defects, and megalourethra.
A total of 91 patients were identified. There were 34 proximal hypospadias patients. Of these patients, 13 (38%) had a documented neurogenic bladder and 18 (53%) had renal anomalies. 42 patients were identified as having a distal hypospadias. 12 of these patients (29%) had a diagnosed neurogenic bladder and 27 (64%) had renal anomalies. In 15 patients, the hypospadias defect was not recorded. 6 of these patients (40%) had documented renal anomalies and 2 (13%) had a known neurogenic bladder.
Hypospadias when associated with ARM represents a unique problem. The incidence of associated anomalies is higher than seen with hypospadias alone. A poorly timed repair or one that does not allow catheterization for invasive testing may have negative consequences including delay in diagnosis. We propose that, at a minimum, all patients have a renal ultrasound performed before surgery and strongly advise that the repair be done after the initial posterior sagittal pull through in order to ensure that the often present recto-urinary fistula is closed. We obtain a voiding cystourethrogram when necessary a few days before colostomy closure. This allows an understanding of the bladder and may help guide the decision for urodynamic testing. Ideally hypospadias repair can be performed a few months after colostomy closure when the renal anatomy and bladder dynamics are known. Concomitant repair is ill advised because of the severe rash that will develop for several weeks after stool touches the perineum for the first time. When a neurogenic bladder is suspected, hypospadias repair may be delayed until after catheterization has begun unless the surgeon is confident that the neo-urethra can be easily catheterized by the family. This may avoid the need for an early diversion or Mitrofanoff and prevent the potential for pressure on the repair from straining.