jejunal atresia
description
upper small gut atresias probably have a vascular basis. they can be the consequence of a volvulus, a transparietal constriction or a parietal perforation. multiple forms may rarely be familial. the apple-peel type is seen where the bowel is coiled around a vascular axis. it can be secondary to a volvulus being responsible for the occlusion of the superior mesenteric arteries distal to the jejunal branches. other anomalies are rare with jejunal atresia.
diagnosis
the classical appearance is that of the triple bubble. this really is a misnomer since in fact there are several bubbles not just three but the ultrasound cut may produce this. the distended loops usually appear large. as with other upper gut problems there is usually polyhydramnios.
differential diagnosis
other cystic intra-abdominal conditions such as duodenal atresia, can be confusing but here the lesion tends to be more discrete and in the upper abdomen. multicystic dysplastic kidneys and hydronephrosis with hydroureter should be considered and certainly the multiple loops found with hydroureter can be difficult; upper renal tract dilatation will usually be present. distended large bowel can be distinguished from its more formed contents but small and large bowel can often be mistaken for one another even postnatally.
sonographic features
triple bubble noted in the fetal abdominal
polyhydramnios
lesion is usually isolated
associated syndromes
- diabetic embryopathy (including caudal regression/sirenomelia sequence)
- fetal cocaine effects
- multiple intestinal atresias
- oeis complex
- short rib- polydactyly syndrome type 1 (saldino-noonan)
- trisomy 21
references
filkins k, russo j, flowers wk third trimester ultrasound diagnosis of intestinal atresia following clinical evidence of polyhydramnios prenatal diag 5: 215-220
fletman d, mcquown d, kanchana poon v, gyepes mi óapple peelò atresia of the small bowel : prenatal diagnosis of the obstruction by ultrasound pediar radiol 9: 118-119
lai pc, jehng ch, chiang lm, liao ks, chen mh, ho mm, hwang kc jejunal atresia with óapple peelò deformity ; report of one case acta paediatr sin 32: 47-53