Congenital aganglionic mega colon (Hirschsprung's disease) is a motor disorder in the gut, due to a defect in the craniocaudal migration of the neuroblast originating from the neural crest that occurs during the first twelve weeks of gestation, causing a functional intestinal obstruction, with its attendant complications, in infants. Despite modern pediatric practice, with emphasis on early diagnosis, Hirschsprung's disease is seen in adults in regions where perinatal care is limited. We report two cases of Nigerian adults with longstanding, recurrent constipation, getting relieved by laxatives and herbal enemata, and then presented to our Emergency Department with a history of progressive abdominal distention, colicky pain, occasional vomiting, and weight loss. Per rectal examination revealed a gripping sensation in the rectum, 10 cm from the anal verge, with rectal fecal load. Barium enema showed a grossly distended proximal large colon, with high fecal retention, with the transition zone at the middle one-third of the rectum. Due to difficulty in bowel preparation of these patients, emergency laparotomy was done. The first case had a diverting sigmoid colostomy and later had a low anterior resection. The second case had a one-stage procedure. Histology of both the cases showed aganglionosis of the stenotic segment and a normal distal rectum. Both patients had complete resolution of the symptoms, without complications, in a three-year follow-up. The related literatures were reviewed. Hirschsprung's disease should be considered in adults patient presenting with chronic constipation. Low anterior resection of the rectum would be a surgical option for the treatment of short and zonal segment of adult Hirschsprung's disease.
Although sacrococcygeal teratomas are rare, there have been several reports from various parts of Nigeria, outside the Northeast region. This report reviews the experience with this tumour from the northeastern part of Nigeria. A retrospective study of 21 children with histologically confirmed sacrococcygeal teratoma managed in 18 years. Over the 18-year-period, 21 histologically confirmed cases of sacrococcygeal teratoma were seen-80% were girls. Thirteen (60.9%) presented during the neonatal period. Eighteen patients presented with benign sacrococcygeal teratoma while three patients and a recurrence from an initial benign tumour had malignant sacrococcygeal teratoma. All malignant tumours had a significant presacral component. There were associated congenital malformations in 3 (14.3%) of the patients. All the benign tumours were resectable. Using the American Academy of Pediatrics (Altman's) classification, 13 patients were type I, 5 patients type II, 3 patients type Ill and none was for type IV. The commonest post-operative complication was wound infection in six patients. Others were wound dehiscence in three and post-operative diarrhoea in two patients. Three deaths were recorded in this series. The size of mass greater than 5 cm did not correlate with the incidence of post-operative complications (P=0.367). The post-operative follow-up was very poor; but there was a recurrence in a girl, two-years after the initial surgery. Our study revealed that most of our patients with sacrococcygeal teratoma present early to hospital with benign lesions and the immediate post-operative results were excellent. However, the follow-up was poor as such long-term post-operative results are difficult to study. It is to be recommended that sacrococcygeal teratoma presenting early to hospital should have prompt surgical intervention.
Appendicovesical fistulae are rare. Only 112 cases in all ages have been reported previously in the world literature. Our case is that of a 1-year-old boy who presented during the neonatal period with missed appendiceal perforation associated with a long segment Hirschsprung's disease. The case is discussed.
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