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Cloacal exstrophy and cloacal exstrophy variants: a proposed system of classification.

A coding system that documents the abnormalities within the cloacal exstrophy complex is presented. Analysis allows the classification of these abnormalities into classical and variant series in a logical manner. The application of the coding system to selected reported material is described and it demonstrates the similarities between apparently divergent cases.

Abnormalities, Multiple↗

[Continent urinary reservoir using dilated renal pelvis of non-functioning pelvic kidney in a girl with cloacal exstrophy].

We performed construction of continent urinary reservoir in an 8-year-old girl with cloacal exstrophy who had double stoma of ileostomy and colon conduit. Preoperative evaluation revealed non-functioning right kidney with severely dilated renal pelvis and calyxes in her pelvis. Urinary reservoir was constructed using detubularized colon segment which had been used as the urinary conduit and dilated renal pelvis of non-functioning pelvic kidney. Using Mitrofanoff's principle, continent catheterizable channel was also made of the anterior wall of the renal pelvis. Postoperative course was uneventful. Reservoir capacity increased to 350 ml one and half year postoperatively and she is almost dry with clean intermittent catheterization 5 times a day. Dilated upper urinary tract is one of the ideal material for bladder enlargement that avoids the complication associated with the use of gastrointestinal tract.

Child↗

Surgical treatment of cloacal anomalies.

From 1989-1998 14 patients were treated with cloacal anomalies: 5 typical cloacas (PC), 5 posterior cloacas, and 4 cloacal exstrophies (CE); 12 underwent surgery. Four typical cloacas were resolved with posterior sagittal anorectovagino-urethroplasty (PSARVUP), whereas in the 5th total urogenital mobilization (TUM) was used. Three PCs were managed with transanorectal TUM and 2 with anterior TUM without opening the anal canal and rectum (without a protective colostomy). Two CEs were treated with atypical procedures. Two patients with CE died without surgery and 2 died after surgery due to complex associated anomalies. During postoperative follow-up of 1-8 years, 5 children had voluntary bowel movements and no soiling while the others had soiling with or without enemas; 1 had stress incontinence; 3 were on clean intermittent catheterization due to neurogenic bladder and were dry. PSARVUP provides a satisfactory result if there is no sacral anomaly. TUM makes this operation easier to perform. In patients with a PC it is sometimes possible using TUM to separate the urinary from the genital tract and remove the accessory urethra without opening the anus and rectum.

Child, Preschool↗

Cloacal exstrophy: a report of 34 cases.

A clinical review is presented on 34 patients with cloacal exstrophy who were seen between 1963 and 1986. The patients were separated into 2 main groups: classical cloacal exstrophy (type I) and variant cloacal exstrophy (type II). In the classical cases 3 surface patterns were recognized: A-hemibladders confluent cranial to the bowel, B-hemibladders lateral to the bowel and C-hemibladders confluent caudal to the bowel. Surgical reconstruction was performed in 24 patients, with a 50 per cent survival rate. However, there was marked improvement in survival from 22 per cent between 1963 and 1978 to 90 per cent between 1979 and 1986.

Abnormalities, Multiple↗

Tethered cord associated with anorectal malformation.

Ten children with a tethered cord and also an anorectal malformation are reported in this document. The anorectal malformations comprised 5 vesicointestinal fissures, 2 cloacal exstrophies, 2 rectovesical fistulas and 1 rectobulbar fistula. All of the patients underwent colostomy in advance of surgery for untethering of the spinal cord. Although their neurologic deficits had previously been considered static, they were subjected to radiographic examination of the caudal spine and found to have a tethered cord. These 10 children were among 55 children with a tethered cord surgically treated at the Division of Neurosurgery of the Osaka Medical Center and Research Institute for Maternal and Child Health during the last 11 years. Data were obtained for these 10 children (6 boys and 4 girls, mean age 1.7 years) who underwent surgical untethering. Several hypotheses are offered to explain this association. Anorectal malformations are related to underlying spinal cord anomalies, which may be amenable to neurosurgical correction. Eight of our patients had no skin stigma of the lumbosacral region, in contrast to an ordinary tethered cord. Spinal cord imaging is necessary to closely scrutinize these children.

Abnormalities, Multiple↗

Simultaneous augmentation cystoplasty and artificial urinary sphincter placement: infection rates and voiding mechanisms.

PURPOSE: Simultaneous augmentation cystoplasty and artificial urinary sphincter placement have recently been reported to be associated with a high incidence of infection. We reviewed our results to define the infection rate and outline the mechanisms of voiding in our patient population. MATERIALS AND METHODS: A total of 29 patients underwent a simultaneous procedure. The etiology of lower urinary tract disease was exstrophy in 14 patients, myelomeningocele in 10, lipomeningocele in 3, spinal cord injury in 1 and radical retropubic prostatectomy in 1. We used 19 gastric, 5 ileal and 5 colonic intestinal segments. Average followup was 33 months. All patients were followed for a minimum of 2 years. Preoperatively all cases had mechanical bowel preparation and documented sterile urine cultures or treated bacteriuria. RESULTS: Infection developed in 2 patients (6.9%) necessitating artificial urinary sphincter removal at 1 week and 9 months. There were no infections associated with gastrocystoplasty. Clean intermittent catheterization was required in 21 patients, while the remaining 8 voided spontaneously. Of the 8 patients 4 were catheterized at least once daily to monitor residual urine volumes. Of all patients 5 were catheterized with a gastric tube, 5 with an appendicovesicostomy and 14 per urethra. CONCLUSIONS: A simultaneous procedure was associated with an acceptable prosthetic infection rate and gastric segments were associated with the lowest incidence of infection. The minority of patients voided spontaneously. The combination procedure was effective in achieving continence. However, in the future a nonprosthetic means of providing urethral resistance may provide better treatment.

Adolescent↗