No abstract

Anorectal malformations (ARM) remain a significant birth defect with geographic variation in incidence, individual phenotypes and regional geographic subtypes. Although early studies indicated a low incidence in Black patients, there is a great paucity of knowledge as to the types, frequency and incidence of ARMs encountered in the African continent and their associated anomalies. Current evidence suggests a significant clinical load. This study set out to evaluate ARM in Southern and other parts of Africa to define the clinical load of ARM. We retrospectively collected data on 1,401 ARM patients from six South African Paediatric Surgical units plus representative samples from five other African countries from West, Central and Southern Africa. Data included ethnic group, age, gender as well as the anatomical pathology, classification and presence or absence of associated anomalies. ARM lesions classified by the Wingspread classification plus an analysis of fistula position was carried out in evaluable cases for purposes of comparison. South African centres reported a higher prevalence of cloacae and vestibular fistulae, whereas rectovaginal, recto prostatic and anorectal malformation without fistula were more prevalent in the Northern African group. 76% of 1,401 patients were ethnically Black African [gender ratio = 2 (vs gender ratio 1.38 overall)] and 49.8% were "low" lesions (Wingspread classification). High or intermediate lesions were mostly males (72%). Anal stenosis was most prevalent in black males and non-Black females. Fistulae were identified in 95% with 682 (52%) being low (perineal/covered anus/vestibular) fistulae. Perineal fistulae had a male predilection (n = 260; 20%), whereas vestibular fistulas (n = 416; 32%) was strikingly frequent in black females (55%). Of the remainder, 15 fistulae were rectovesical (1.2%), 544 recto-urethral or prostatic (42%), 16 recto-vaginal (1.2%). In addition, there were 43 cloacal lesions (3.3%). Isolated rare ARM anomalies included "Pouch" colon (2) and H-type fistula (2). Isolated lesions occurred in 81% but 163 associated anomalies were identified in 114 patients. These included chromosomal lesions (10), genito-urinary anomalies (50), genital (16), cardiac (31), skeletal anomalies (33), gastro-intestinal malformations (28). Other anomalies included CNS anomalies (14), anterior abdominal wall defects (2) and facial (8) abnormalities and neuroblastoma (1). The ARM is not uncommon in Black African populations and constitutes a significant clinical load to surgical practice in Africa. Ethnic differences appear to exist and vestibular fistulae predominate in black females. Cloaca (3.3%) did appear to be more prevalent. Isolated lesions are frequent but the types of associated anomalies appear similar to other series except chromosomal syndromes. This study illustrates the need for more objective data from developing countries to assess geographical differences.

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.

Macroscopic generalized necrotizing enterocolitis (G-NEC) is associated with a very high mortality in neonates. In some instances, however, multiple bowel segments are necrotic, with most of the remaining small bowel damaged but viable. In these selected patients morbidity can be reduced and survival increased with an aggressive and early surgical approach. We have termed this approach damage control laparotomy (DCL). Over a 5(1/2)-year period, all neonates with G-NEC with adequate length of viable small bowel were subjected to DCL. The procedure is characterized by a resuscitative period of a few hours followed by laparotomy and resection of dead/perforated bowel. The bowel ends are either anastomosed or tied, and the bowel is returned to the abdomen to allow full tissue demarcation. Re-look laparotomy is performed 3-4 days later, at which time any new necrotic bowel is excised and re-joined, to achieve small bowel continuity. In this prospective study, 104 neonates with G-NEC underwent operation; 27 neonates (26%) were considered to have an adequate potential length of viable bowel and were selected for DCL. Nineteen neonates survived in the follow-up period. Early mortality was due to sepsis syndrome in 6 patients, and late mortality in 2 neonates was secondary to the short bowel syndrome. The DCL procedure is another step toward improving survival in surgical G-NEC; this technique avoids proximal stomas and their complications, and at the same time it preserves the best possible bowel length.