1/307. Congenital alimentary tract abnormalities presenting in adolescence and young adulthood. Intestinal atresias and duplications of the alimentary tract commonly present in the neonatal period or early infancy, but in rare cases they can persist and present de novo during adolescence. We report on these two abdominal congenital anomalies, the clinical presentations of which in adolescence and young adulthood are unique. ( info) |
| A 28-year-old woman was referred at 33 weeks of gestation with suspected fetal intestinal atresia. Sonography showed a large extra-abdominal mass on the right of the normal umbilical cord insertion. Following cesarean section at 36 weeks and immediate surgical treatment, the malformation was not definable either as an omphalocele or as gastroschisis. This reported case involves a previously undocumented malformation of the fetal abdominal wall described as a 'hernia' of the fetal abdominal wall. ( info) |
3/307. Intestinal blind pouch- and blind loop- syndrome in children operated previously for congenital duodenal obstruction. A follow-up study of 27 children operated for congenital duodenal obstruction (CDO) in the years 1953--71 is presented. Nine children belonged to the intrinsic and 18 children to the extrinsic group of CDO. A total of 7 retrocolic, isoperistaltic, side-to-side duodeno-jejunostomy, 7 Ladd's operation, 8 duodenolysis, 2 reduction of midgut volvulus, 2 duodenostomy a.m. Morton and one gastro-jejunostomy were performed at the age of 1 day--15 years. The clinical and radiological examinations were performed 3--21 years (mean 10 years 2 months) after these operations. In 3 cases there was a moderate duodenal dilatation, but reoperation was not necessary. During the follow-up period, one boy, now aged 8 years, developed a blind pouch-syndrome in the I portion of the duodenum containing a 5 x 5 cm phytobezoar 4 1/2 years after duodeno-jejunostomy. The frequency of blind pouch-syndrome after duodeno-jejunostomy was thus 1:7 or 14%. One girl, now aged 9 years, developed a blind loop-syndrome in the ileocaecal segment 3 months after side-to-side ileotransversostomy, which was performed from adhesion-obstruction after duodenolysis for malrotation I and CDO. Both the blind pouch- and the blind loop-deformation were resected and the children recovered well. To avoid blind-pouch- and blind loop-deformations in the intestines, the anastomosis must be made wide enough, and especially in the surgery of the jejuno-ileo-colic region an end-to-end anastomosis is preferable. ( info) |
4/307. Multiple atresias in a low-birth-weight twin. This report describes a case of 16 small bowel atresias in a twin who was born at 31 weeks' gestation, weighing 1,690 g. All atresias and intestinal segments of 5 cm or less in length were resected, resulting in nine primary anastomoses, preserving 75% (107 cm) of his initial small bowel length. The baby went home on full oral feedings after 10 weeks. Multiple anastomoses in the low-birth-weight neonate can be tolerated with the functional benefit of maximal bowel length. The time taken to tolerate feedings appears to be independent of the number of anastomoses. Vascular anastomoses associated with monochorionic twinning may place both fetuses at risk of intestinal atresia in the event of an ischemic insult, either concurrently or with the demise of one affecting the other. Prenatal ultrasound scan appears to be useful for monitoring the evolution of intestinal atresia. However, the risks of extreme prematurity preclude the delivery of the affected baby at the time of initial diagnosis, and as yet it is unknown whether early delivery will alter the number, type, or prognosis of multiple atresias. ( info) |
| Recombinant growth hormone (GH) has been used successfully to promote mucosal intestinal adaptation in adults with short bowel syndrome. Here the authors report the case of an infant with extreme short bowel syndrome successfully treated with intestinal lengthening and GH. The patient was born with intestinal atresia leaving only 12 cm of jejunum and 2 cm of ileum with ileocecal valve. A Bianchi procedure extended the length to 25 cm, but 6 months later she still received 50% of her nutritional requirements by parenteral route. A 4-week course of 0.5 U/kg of GH dramatically changed the situation allowing weaning of total parenteral nutrition (TPN) 2 weeks after the end of the treatment with striking nutritional improvement, decrease in the number of stools from 7 to 2 per day, and no secondary effects. The authors believe that GH deserves clinical trial in cases of extreme short bowel. ( info) |
6/307. Duodenojejunal atresia with apple peel configuration of the ileum and absent superior mesenteric artery: observations on pathogenesis. A child with loss of the third and fourth part of the duodenum and of the proximal jejunum was found to have an apple peel configuration of the remaining small bowel. The complete absence of branches from the superior mesenteric artery impaired the blood supply of the distal duodenum. An annular pancreas was found in this patient with Down's syndrome. This anomaly may have impaired the flow through the pancreaticoduodenal arcade, which would normally compensate for the distal vascular occlusion. According to current understanding, duodenal atresia is a primary malformation. The current case suggests, however, that in rare circumstances vascular accidents may be the underlying cause for duodenal atresia. ( info) |
7/307. Choanal and ileal atresia: a new syndrome or association? choanal atresia is a relatively common congenital malformation which is often associated with other anomalies. On the other hand, ileal atresia is very rare, mostly nonsyndromic and occasionally associated with other anomalies. The association of choanal and ileal atresia is unknown. Here we report the first instance of bilateral choanal atresia and ileal atresia in a full term male infant and describe the subsequent surgical treatment of both conditions. The association is unique and may represent a syndrome. ( info) |
8/307. Vanishing gut in infants with gastroschisis. Infants born with gastroschisis (GS) in association with intestinal atresia are well described, however, atresia to the extent where none or very little of the midgut remains has rarely been reported. In this paper we present one infant born with a GS defect in combination with total loss of the midgut and another with a tight GS defect with atresia of the proximal and distal midgut at the regions where the bowel had gone through the abdominal wall. On examining these and other case reports in the literature, we realised that there is a GS spectrum, at one end with simple, localised atresia with the rest of the gut exhibiting minimal vascular compromise and at the other a very small or no defect with absence of the entire midgut. ( info) |
9/307. Pyloric atresia associated with duodenal and jejunal atresia and duplication. A case of congenital pyloric atresia associated with duodenal atresia, jejunal atresia, and a duplication is described. A review of the literature revealed nine previously reported cases of congenital pyloric atresia and multiple intestinal atresias, but this is the first report of such an association and a duplication. ( info) |
10/307. Ulceration in an ileocolic anastomosis treated with ranitidin. The authors report the case of a child born with a gastroschisis and an ileal atresia. After surgery, only 100 cm small bowel and the distal one third of his colon remained. Perianastomotic ulcers developed 6 years later. These were treated successfully with ranitidin, a treatment not previously reported in the literature. The authors conclude that treatment with ranitidin was successful in a patient with an ulcer in an ileocolic anastomosis. ( info) |