Strong procedure with added anchoring stitches from the jejunum to the abdominal wall for Superior Mesenteric Artery Syndrome in children: A case report

Sanjad-Sakati Syndrome (SSS) is a rare autosomal recessive disorder primarily observed in the Middle East. Superior mesenteric artery syndrome (SMAS) is also a rare gastrointestinal condition that mainly occurs in individuals who experience rapid weight loss and/or have a very low body mass index (BMI). The occurrence of these conditions together is extremely rare. An 8-year-old girl with SSS was admitted to the hospital with flu-like symptoms, shortness of breath, and reduced oral intake over the previous two days. Her medical history included a recent right lobar pneumonia and recurrent vomiting. She was admitted with a diagnosis of recurrent pneumonia and received intravenous antibiotics. We placed a nasogastric tube, which drained bilious fluid. She underwent a plain abdominal film, an upper gastrointestinal study, and a computerized tomography (CT) scan which revealed that she had SMAS. She was started on conservative management, which included parenteral nutrition, metoclopramide, and small volume of enteral feedings. After four weeks without any improvement, we decided to proceed with a modified Strong operation. The modification involved anchoring the first segment of the jejunum to the abdominal wall of the right upper quadrant using two stitches of absorbable material, with the goal of minimizing the chance of a recurrence by preventing the jejunum from slipping back to its normal anatomical position. She tolerated the operation well. After an uneventful recovery, oral feedings were gradually introduced and increased until she reached full volume by postoperative day 12, She was discharged home one day later. She was followed for nine months and to date she has shown adequate weight gain. Even though the association is remarkably rare, patients with SSS who had failure to thrive should undergo testing to rule out SMAS. If SMAS is confirmed and conservative measures are not successful, the modified Strong procedure appears to be a safe and effective surgical option.