Anorectal atresia with gross terminal colonic distension in Africa.

UNLABELLED: Anorectal malformation (ARM) is a group of significant birth defects with geographic variation in incidence, individual phenotypes and regional geographic subtypes occurring in approximately 1:5,000 live births. Anorectal atresia with gross terminal colonic distension in the presence of an absent anal canal has also been known as 'pouch colon' which is mainly associated with the Indian subcontinent. Its prevalence is unknown but it is rare, possibly representing a fraction of 1 % of ARM. The problem seems to revolve around the gross terminal distension of the distal pouch which remains a significant surgical challenge to surgeons resulting in poorer postoperative continence in many instances. AIM: This study set out to collate data on ARM patients with gross terminal distension of the distal pouch from Africa to evaluate its epidemiology and outcome in African patients. METHODS: Fifteen African paediatric surgical centres (7 South African and 8 African centres) were polled on the occurrence of anorectal atresia with gross terminal distension of the terminal bowel, an ARM variant. Data included ethnic group, age, gender as well as the anatomical pathology, classification and presence or absence of associated anomalies. RESULTS: Of 12 respondents, 8 (67 %) responded and sufficient data to classify and analyse were obtained from six of the eight positive replies (7 new cases). Abdominal X-ray showed a grossly dilated terminal portion of the colon in the presence of an imperforate anus. A colovesical fistula was observed in four (three males and one female cloaca). Three were associated with a colonic atresia, of which two were in the transverse colon and one in the sigmoid colon. Surgical corrective procedures were carried out in six, but one patient, with a cloaca, died prior to surgical correction. CONCLUSIONS: In this survey of 15 African centres, we have attempted to document the occurrence and presentation of anorectal atresia with gross terminal distension in Africa and report seven additional new cases. A possible association with associated conditions like colonic atresia requires further investigation.

Retrospective surveillance of intussusception in South Africa, 1998-2003.

BACKGROUND: Intussusception is a common gastrointestinal emergency in children and appears to have a somewhat different clinical spectrum in developing countries. Its etiology is still unclear, but a link to infective agents and viruses has been highlighted. This study aimed to assess the clinical spectrum and prevalence of intussusception in children from the diverse South African population. METHODS: Retrospective data were obtained from 9 participating pediatric referral units on the occurrence of intussusception in South African children (<14 years old) during a 6-year period (1998-2003). Results were correlated with national population statistics. Intussusception was anatomically classified into ileoileal, ileocolic, and colocolic types. The clinical features, management, outcome, and possible causes were examined. RESULTS: We reviewed the occurrence and clinical spectrum of intussusception in 423 children (age, 0-14 years) presenting with acute intussusception to 9 pediatric surgical centers. The mean duration of symptoms was 1.5 days, but a delayed presentation was common (median delay, 2.3 days). Intussusception occurred throughout the year, with a peak in the summer months. The majority of patients (89%) were <2 years old, and 78% presented at age 3-18 months of age. Crude population estimates indicate an occurrence of 1 case per 3123 population <2 years old. Only 11% of patients presented after 2 years of age, and the age at presentation was significantly lower (P < .05) in black African patients. All ethnic groups were affected. In 84% of patients, intussusception occurred at the ileocolic region junction, in 7% it was ileoileal, and in 9% it was colocolic. Colocolic intussusception appeared more common in black African patients, and associated pathologic conditions (polyps and Burkitt's lymphoma) occurred mainly in older children. Surgical intervention was required in 81% of patients and involved resection of gangrenous bowel in 40%. CONCLUSION: Intussusception appears to be a relatively frequent occurrence in children in South Africa. Although the clinical spectrum appears to vary, there is an apparent link to intestinal infection, which requires further investigation. A collaborative approach is required to ascertain the relationship of intussusception to preventable infections and to improve its diagnosis and management.