JEJUNOILEAL ATRESIA AND STENOSIS- incidence of jejunoileal atresia and stenosis is one in1000 live births.Etiology :Tandler suggested that intestinal atresia was related to a lack of recanalization of the solid stage of the intestine, while others have questioned these theories- Louw suggested that jejuno-ileal atresia was probably due to a vascular accident rather than the result of inadequate recanalization- A localized intrauterine vascular accident can cause ischemic necrosis, liquefaction of tissues and subsequent resorption of the affected devitalized segment.

ClassificationStenosis (11%) is characterized by a short localized narrowing of the bowel without discontinuity or a mesenteric defect. The bowel is of normal length.

Atresia type I (23%) is represented by a transluminalSeptum, membrane or web formed by both mucosa and submucosa while the muscularis and serosa remain intact. The dilated proximal bowel remains in continuity with the collapsed distal bowel, there is no mesenteric defect and the bowel is of normal length.

Atresia type II (10%) has two blind-ending atretic ends connected by a fibrous cord along the edge of the mesentery. There is no mesenteric defect and the bowel length is not foreshortened.

Atresia type III(a) (15%) is similar to type II except that the fibrous connecting cord is absent and there is a V-shaped mesenteric defect. The bowel length may be foreshortened. Cystic fibrosis is commonly associated with this variety.

Atresia type III(b) (19%) (Apple peel or Christmas tree) consists of a proximal jejunal atresia often with associated malrotation, absence of most of the superior mesenteric artery and a large mesenteric defect. The distal bowel is coiled in a helical configuration around a single perfusing artery arisingfrom the right colic arcades. Occasionally, additional type I or type II atresias are found in the distal bowel. There is always a significant reduction in intestinal length. A familial incidence and atresias amongst siblings and identical twins point to a morecomplex genetic transmission with an overall recurrencerate of 18%

Atresia type IV (22%) represents multiple segmentalatresias or a combination of types IIII. Bowel length is always reduced. A rare autosomal recessive pattern of transmission has been documented and pathological findings could support the concept that a developmental process early on, could have affected the whole bowel.

Clinical Manifestationspresent as neonatal intestinal obstructionAbdominal distension and bilious vomiting is observed within 24 hours of birth. The degree of abdominal distension is related directly to the site of obstruction. Abdominal distension is more pronounced with distal small-bowel obstruction.Most infants fail to pass meconium. Although the classic first stools passed by these patients are small, gray and mucoid, normal meconium can occasionally be passedJaundice is present occasionally

DIAGNOSTIC PROCEDURESradiological examination of the abdomen and chest - distended air-filled small intestinal loops proximal to an obstruction in a gasless distal abdomen. In some instances, the first abdominal radiograph can reveal a completely opaque contrastless abdomen due to fluid-filled obstructed bowel.The more distal the obstruction the greater the number of air fluid filled and distended loops of bowelThe bowel proximal to the site of obstruction may have the appearance of a large air-fluid filled loop.a contrast enema is performed to exclude colonic atresia, distinguish between small and large bowel distension, determine whether the colon has the typical microcolon appearance,

TREATMENTPREOPERATIVE MANAGEMENT- Nasogastric suction and intravenous hydration are essential. Dehydration and acidbase and electrolyte imbalances should be corrected.The aim is to establish intestinal continuity and preserve as much normal bowel as possible. The proximal bowel is dilated and a limited resection is usually needed. This resection also facilitates an end-to-end or end-to-oblique anastomosis. A single-layer, end-to-oblique anastomosis is simple and efficacious. If proximal resection is not possible, then tapering or plication of the dilated bowel is indicated. The distal bowel should be evaluated for additional atresias or stenosis by an intraluminal injection of 0.9% saline. If multiple atresias are present, then many anastomoses may be required