SMA syndrome in a 3-year-old child

Clinical History

A 3-year-old boy presented to our facility with loss of appetite, abdominal pain, bloating, nausea and vomiting after receiving 3 cycles of chemotherapy for parailiac retroperitoneal neuroblastoma.

Imaging Findings

Fluoroscopic examination of upper GIS (gastrointestinal system) was performed to exclude a potential problem in gastroduodenal passage. On upper GIS examination, the passage of contrast material from the second to the third duodenal segment was prolonged up to 10-15 minutes with continuous forward-reverse movement of contrast material at this level. Then, contrast material passed to fourth segment and no abnormality was detected at these levels. These findings are compatible with SMA syndrome.

Discussion

Superior mesenteric artery (SMA) syndrome occurs due to compression of the third segment of the duodenum between the aorta and the proximal part of the superior mesenteric artery. It is termed Wilkie syndrome since it was first described by Wilkie as chronic duodenal ileus in 1927 [1]. It is a rare syndrome manifested as epigastric pain after eating, bloating, nausea, vomiting, anorexia and weight loss [2]. Because it is a rare disease and the diagnosis can be confirmed only by invasive examinations, patients receive symptomatic treatment for prolonged periods. Symptoms can be acute or chronic [3]. In acute cases, conservative treatments are given preferentially while surgical treatment is considered in chronic cases [2, 3]. Various techniques have been described for surgical treatment but the most commonly preferred technique is side-to-side duodenojejunostomy [4]. SMA travels inferior/anterior course with an angle of 45° (38-56°) after arising from the aorta [4]. Between these arteries, the third segment of the duodenum passes from right to left. Acute or chronic causes narrowing aorto-mesenteric angle (6-16°) lead to compression of the duodenum in this space and complete or partial narrowing [4]. The reasons for of SMA syndrome may include narrow SMA-aorta space, narrow aorto-mesenteric vascular angle, high insertion of Treitz ligament duodenojejunal flexure, lower SMA origin, loss in mesenteric and retroperitoneal adipose tissue following marked weight loss seen in diseases causing severe deterioration such as cancer or burns, severe injuries such as head injury, peritoneal adhesions due to duodenal malrotation, excessive lumbar lordosis, visceroptosis, laxity of abdominal wall and anorexia nervosa [2, 5]. Our case experienced marked weight loss and SMA syndrome presumably developed secondary to loss of mesenteric and retroperitoneal adipose tissue.

Differential Diagnosis List

Final Diagnosis

Superior mesenteric artery syndrome

URL:	https://www.eurorad.org/case/12922
DOI:	10.1594/EURORAD/CASE.12922
ISSN:	1563-4086