MyPACS.net: Radiology Teaching Files > Case 11381487

previously visited 3 DAY OLD WITH SUSPECTED GI ANOMALY
Contributed by: Mohamed Eltomey, Radiologist, Tanta University Hospitals-Radiology & Imaging Dept., Egypt.
Patient: 3 day old
History: A 3 day old male referred to the Pediatric surgery unit at out institution with suspected GI anomaly on prenatal ultrasound
Images:[small]larger

Fig. 1: Prenatal ultrasound showing the dilated bowel loop

Fig. 2: Radiograph showing dilated proximal bowel suggesting a high bowel obstruction

Fig. 3: Upper GI study showing the abnormal oreintation of the duodenal curve and the dilted proximal jejunum

Fig. 4: Water soluble enema showing a small caliber of the colon and abnormal position of the cecum

Fig. 5: Operative Specimen showing the Apple peel type of Jejunoileal atresia

Fig. 6: Figure showing the different types of Jejunoileal atresia (Oldham 1993)
Findings: The patient had a prenatal ultrasound performed at 39 weeks GA showing a dilated bowel loop and polyhdraminous.

A plain radiograph of the abdomen showed 3 air fluid levels raising the suspecion of a proximal jejunal atresia.

An upper GI series "using water soluble contrast" was performed showing dilatation of the proximal jejuneum and non passage of the contrast beyond to the distal bowel loops.

A watersoluble enema was performed on the next day to further verify the level of the obstruction. The enema revealed a somewhat small colon with relatively abnormal position of the cecum.

The patient was found to have combined jejunoileal atresia (type IIIb) or also known as the Apple peel or Maypole type.
Diagnosis: Jejunoileal atresia (Maypole type)
Discussion: Atresia or stenosis of the jejunoileal area is the diagnosis in a significant percentage of neonates who have bowel obstruction. Atresia is more common than stenosis. Reports of its incidence would seem to be 1 in 750 live births. Coexisting anomalies and familial tendencies are uncommon; the incidence of associated extraintestinal anomalies is thought to be about 5%.

Classification of jejunolileal atresias: after Grosfeld et al 1979

1- Type I: the obstruction is caused by a membrane or web formed by mucosa and submucosa. The proximal dilated and distal collapsed bowel are in continuity without a mesenteric defect. The bowel length is not foreshortened.

2- Type II: the proximal bowel terminates in a bulbous blind end, which is connected by a short fibrous cord along the edge of a intact mesentery to the collapsed distal bowel (Blind ends joined by a fibrous cord).

3- Type III (a): the atresia ends blindly both proximally and distally as in type II, but the fibrous connecting cord is absent and there is a V-shaped mesenteric defect of varying size.

- Type III (b): Apple peel, Christmas tree or Maypole atresia all are synonyms for the same condition. It consists of proximal jejunal atresia near the ligament of Trietz, absence of the superior mesenteric artery beyond the origin of the middle colic branch and absence of the dorsal mesentery, significant loss of intestinal length and a large mesenteric defect. The distal small bowel assumes a helical configuration around a single perfusing vessel arising from the ileocolic or right colic arcades, hence the descriptive terminology. Occasionally, further type I or type II atresias are found in the bowel closest to the distal blind end.

4- Type IV: there are multiple segment atresias, of combination of types I to III, often having the morphological appearance of a string of sausages.

In jejunal atresia abdominal radiography shows two or three dilated loops, so called "triple bubble" sign, which is more than would be seen in duodenal atresia and fewer than in ileal atresia or other causes of low bowel obstruction.

In jejunal atresia contrasted studies are seldom required. If contrast is introduced from above, non-ionic water soluble contrast agents are preferable to barium. If an enema is performed and a microcolon is encountered, additional distal atresia(s) is implied, which is considered diagnostic, and present in all cases except in those whom atresia occurred shortly before birth.

References: Ross AJ.: Intestinal obstruction in the newborn. Pediatrics in Review 15(9):338-347,1994.

Grosfeld JL, Ballantine TVN, Shoemaker R: Operative management of intestinal atresia and stenosi based on pathologic findings. Journal of pediatric surgey 14:368, 1979.

Hernanz-Schulman M: Imaging of neonatal gastrointestinal obstruction. Radiological clinics of North America 37(6):1163-1186, 1999.

McAlister WH, Kronemer KA: Emergency gastrointestinal radiology of the newborn. Radiologic clinics of North America 34(4):819-844, 1996.

Oldham KT: Gastrointestinal disorders, neonatal intestinal obstruction. In Greenfield LJ, Mullholand MW, Oldham KT, Zelenk GB (eds.): Surgery, scientific principles and practice. 1st ed.,Lippincot, Philadelphia, PP 1838-1873, 1993.

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Additional Details:

Case Number: 11381487Last Updated: 10-20-2007
Anatomy: Gastrointestinal (GI)   Pathology: Congenital
Modality: Conventional Radiograph, GIAccess Level: Readable by all users
Keywords: jejunoileal atresia

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