Duodenal involvement of scleroderma

Case contributed by Charlie Chia-Tsong Hsu
Diagnosis almost certain

Presentation

Poor appetite and reduced oral intake. Recent percutaneous gastrostomy tube insertion. Vomiting post PEG feeds with gastric distention. Background of critical limb ischemia with bilateral lower limb ulcers.

Patient Data

Age: 50 years
Gender: Female
Fluoroscopy

Initial supine abdominal radiograph demonstrate paucity of bowel gas. An inconspicuous, distended,  fluid filled,  loop of duodenum can be inferred by the valvulae conniventes. A left lateral decubitus radiograph demonstrate air fluid level within the D2 segment of the duodenum. 

Water soluble contrast was used for the examination. The patient was positioned in the right lateral position with 30 degrees caudal tilt of the examination table to assist contrast passage into the duodenum. The patient was unable to assume an erect position due to bilateral lower limb ulcers. Initial fluoroscopic image take at 30 mins shows minimal contrast passage into the D1 segment of the duodenum (not shown)

Subsequent supine abdominal radiograph performed 1 hour after contrast ingestion demonstrate abrupt termination of the D3 segment of the duodenum as it cross midline at the level of L3/L4. This is associated with dilatation of the proximal portion of the D3 segment. There is paucity of oral contrast in the proximal jejunum with significant quantity of contrast retained in the stomach. 

Supine abdominal radiograph performed 2.5 hours after shows contrast opacification of the remaining small bowel and the ascending colon. 

Note is made of a narrowed D3 segment of the duodenum as it crosses the midline behind the superior mesenteric artery. Coronal reformat demonstrate proximal distention of the duodenum and a grossly distended stomach. 

Case Discussion

Duodenum is the second most commonest site after the esophagus to be affected by scleroderma. Imaging finding is characterize by gastric dilatation and delayed emptying with marked dilatation of the D2 and D3 segment of the duodenum. As the D3 segment cross the physiologic narrowing between the SMA and the vertebral body, the slow transit is further delayed resulting in proximal bowel dilatation. The clinical symptoms, proposed mechanism and imaging appearance overlap with the superior mesenteric artery syndrome. Whether there a common or separate pathogenesis exist between the two condition remained to be elucidated. 

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