Small Bowel Bezoar in a Patient With Noonan Syndrome: Report of a Case

Case Report

A 64-year-old man with Noonan syndrome presented to the emergency department with a 1-day history of abdominal pain, nausea, vomiting, decreased oral intake, and obstipation. His abdomen was distended but he was passing flatus. His past medical history was significant for Noonan syndrome, in addition to congestive heart failure, atrial fibrillation, hypertension, gout, asthma, hypogonadism, chronic renal insufficiency, and 2 surgeries for kidney stone removal.

Abnormal laboratory values included a white blood cell count of 13.3 x 10³ cells/mcL and a creatinine level of 1.5 mg/dL. A radiograph of the abdomen revealed 2 significantly dilated small bowel loops with fold thickening in the left side of the abdomen (Figure 1). A computed tomography (CT) scan of the abdomen demonstrated many dilated loops of fluid-filled distal jejunum with decompressed proximal jejunum and distal ileum (Figure 2). Inferior to the umbilicus near the midline was a 1.5-cm target sign of the small bowel with an outer enhancing wall, a hypodense inner ring, and a central hyperdensity. Because this abnormality was seen at the transition point and was likely located in the ileum, we thought it might represent a very short-segment intussusception or potentially a gallstone ileus. A streaky air pattern in the dilated loops was suspicious for pneumatosis.

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Figure 1.

Abdominal plain film radiograph. Note the multiple dilated loops of small bowel suggestive of a small bowel obstruction.

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Figure 2.

Abdominal CT scan. The arrow points to the phytobezoar in the small bowel.

An emergency exploratory laparotomy was performed and we found that the patient had mild adhesive disease requiring a lysis of adhesions. A segment of hyperemic thickened ileum was also noted to have a large mobile mass within it. The wall of the small bowel was incised to extract the phytobezoar and closed primarily. The patient's postoperative course was characterized by mild congestive heart failure, but there were no other complications. When he was discharged from the hospital he was able to tolerate a regular diet without difficulty.

© 2007  Medscape

Cite this: Small Bowel Bezoar in a Patient With Noonan Syndrome: Report of a Case - Medscape - Feb 21, 2007.