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Association of congenital vertebral malformations with bladder and cloacal exstrophy.

The radiographs of 28 patients with bladder exstrophy and five with cloacal exstrophy were reviewed at an average age of 8 years 3 months to assess congenital vertebral malformations. Fourteen malformations in 11 patients were noted, including abnormal lumbosacral segmentation in five, congenital scoliosis and kyphosis in four, partial sacral agenesis in three, and interpedicular lumbar widenings in two. These findings are explained by an abnormal embryologic development of the spine and cloaca between 4 and 6 weeks of intrauterine life. We recommend entire spinal radiographs in these patients.

Abnormalities, Multiple↗
PubMed1989

Continent urinary diversion and bladder augmentation in children: the Mainz pouch procedure.

The formation of a bowel reservoir of large capacity at low pressure by using small and large bowel (ileocaecal segment) has proved reliable for achieving continent urinary diversion (n = 80), for bladder augmentation (n = 42) as well as for total bladder replacement (n = 24). Encouraged by the results we obtained in our adult patients, we have used this technique during the last 3.5 years in 29 children. Indications for urinary diversions in children have been: neurogenic bladder with diplegia (n = 8), bladder exstrophy (n = 2), traumatic loss of the bladder (n = 1), urogenital sinus (n = 1) and rhabdomyosarcoma of the prostate or bladder (n = 2). Bladder augmentation was indicated in 6 children with iatrogenic bladder loss, in 5 children with neurogenic bladder without diplegia and in 4 boys with incontinent epispadias or exstrophy. In children with bladder exstrophy or incontinent epispadias, continence was achieved using a modified Young-Dees technique with formation of a long intra-abdominal muscular tube made out of the bladder plate or the low-capacity bladder. The capacity of the urinary reservoir was guaranteed by bladder augmentation or bladder replacement with an ileocaecal pouch. During a mean follow-up period of 26 months (bladder augmentation) and 21 months (continent diversion) there was only one postoperative complication (intussusception ileus) which required operative revision. Two children had to undergo reoperation because of nipple problems. Follow-up, with monitoring of biochemical and metabolic parameters, is necessary to show whether this technique will provide a long-term successful solution for these problems.

Adolescent↗