הכינוס השנתי של החברה הישראלית לפדיאטריה קלינית - חיפ"ק 2022

SMA Syndrome: Clinical Characteristics, Diagnosis and Outcome in Pediatric Population – 10 years` Experience

לאה אוחנה-סרנה-כאהן 1 Tamar Orgad 1 Maayan Toisano 3 Shimon Reif 1,2 Mordechai Slae 1,2
1Department of Pediatrics, Hadassah-Hebrew University Medical Center, Jerusalem
2Department of Pediatric Gastroenterology, Hadassah-Hebrew University Medical Center, Jerusalem
3Faculty of Mediciner, Hadassah-Hebrew University Medical Center, Jerusalem

Background: Superior mesenteric artery (SMA) syndrome is a rare cause of proximal intestinal obstruction. There is scarce literature data regarding children suffering from SMA syndrome.

Methods: Retrospective data analysis of all pediatric patients aged 0 to 16 years old between the years January 2010- December 2020 with diagnosis of SMA.

Results: Eleven children < 16 years of age were diagnosed with SMA syndrome. The mean age was 13.05 years ± 4.5, 6 were females. Six (54%) originated from multiple children family (≥5 siblings). Thirty-six percent of children with a history of eating disorder, 18.1% with scoliosis. Nine had abdominal pain, 63.6% suffered of vomiting, followed by diarrhea, constipation, nausea, weight loss and weakness (36.6% ,36.6%,27.2%, 27.2%,18.1% respectively). The body weight percentile of 5 (45.4%) was below 3th percentile, and 3 (27.7%) had normal weight gain with average percentile of 28%. No electrolyte abnormalities were found. Upper GI series was done in 7 children. 27.2% underwent CT scan, 18.1% were evaluated with MRI scan. Seven evaluated by gastroscopy all with finding characteristic of gastritis. Average time from symptoms start to diagnosis was 7.5± 6.3 months. The median length of hospitalization was 23.3 days ±38.86. three children were treated with TPN. One child required surgery. Psychiatric care was provided in 63.6% including psychiatric day-ward, in-house psychiatric hospitalization.

Conclusion: SMA syndrome is a rare condition in pediatric population. The diagnosis is challenging due to variety of presenting symptoms. Children mostly necessitate psychiatric intervention and demonstrated low rate of complication and mortality.