One-hundred-fifteen consecutive bladder exstrophies successfully closed in a single nationally commissioned centre

Since 2007 the authors have adopted a staged Delayed Bladder Exstrophy Repair (DER). Because of the creation of two dedicated exstrophy units in the UK, the authors can report their experience with this approach on a large cohort of patients with bladder exstrophy.

Patients with classic bladder exstrophy (CBE) or bladder exstrophy variant (BEV) primarily treated at the authors' institution between 2007 and 2023 were identified from a prospectively maintained database. Patients with cloacal exstrophy were excluded. Exstrophy closure was performed electively by a paediatric urology and orthopaedic team with a dedicated anaesthetic support. First stage of closure included bilateral ureteric reimplantation and bladder closure with tubularisation of the posterior urethra. 'L-shape' bilateral iliac wing osteotomy with (combined) or without (single) posterior unicortical osteotomy was performed at the time of first stage. An external fixator with mermaid bandage was applied without lower limbs traction. Epispadias repair was performed as a separate stage 6-18 months following exstrophy closure. The following parameters were recorded: age at closure, extent of diastasis and timing of external fixators. The primary outcome was to determine the success rate of DER (no abdominal wall dehiscence, suprapubic fistula or prolapse). Secondary outcomes included short-term complications and mid-term outcomes such as renal scarring and persistent hydroureteronephrosis.

One-hundred and fifteen patients were included (103 CBE and 12 BEV). Median age at repair was 7.8 months and pubic diastasis was 4.7 cm in the CBE group and 5.7 in the BEV group. Median external fixator removal time was 27 days. All had successful closure. One patient required subcutaneous tissue and skin debridement and suturing of the median wound because of infection. There were no significant orthopaedic or neurological complications and no penile ischemic injuries. Mild hydronephrosis and distal ureteric dilatation occurred respectively in 7 and 10 patients and were treated conservatively with anticholinergics and clean intermittent catheterisation. Two required eventually early enterocystoplasty due to a small and non-compliant bladder. Median follow-up was 4.1 years.

In a dedicated bladder exstrophy unit, staged DER with anterior oblique 'L-shape' osteotomy, external fixator and mermaid bandage, provides secure abdominal wall and bladder closure, while preserving the upper urinary tract and preventing ischemic penile injuries.