Superior mesenteric artery syndrome: a rare complication in a child with Marfan syndrome

• Published in: Archives of disease in childhood Vol. 98; no. 10; pp. 754 - 755
• Main Authors: Jain, Viral, Singal, Arbinder Kumar, Ramu, Chithra, Raghunaathan, Krithiha Devi
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health 01.10.2013; BMJ Publishing Group; BMJ Publishing Group LTD
• Subjects: Abdominal pain; Adolescent; Biological and medical sciences; Blood and lymphatic vessels; Cardiology. Vascular system; Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous; Duodenal obstruction; Duodenojejunostomy; Ethics; Female; General aspects; Humans; Kyphoscoliosis; Literature Reviews; Marfan syndrome; Marfan Syndrome - complications; Medical sciences; Miscellaneous; Prevention and actions; Public health. Hygiene; Public health. Hygiene-occupational medicine; Risk factors; Superior Mesenteric Artery Syndrome - diagnosis; Superior Mesenteric Artery Syndrome - etiology; Superior Mesenteric Artery Syndrome - surgery; Human; Connective tissue disease; Pediatrics; Skin disease; Elastic tissue disease; Superior mesenteric artery syndrome; Cardiovascular disease; Marfan syndrome; Genetic disease; Arteritis; Arterial disease; Vascular disease; Superior mesenteric artery; Systemic disease; Complication; Child; Public health; Abdominal pain; Duodenojejunostomy; Duodenal obstruction; Kyphoscoliosis
• ISSN: 0003-9888; 1468-2044; 1468-2044
• DOI: 10.1136/archdischild-2013-304321

SMAS is a rare cause of duodenal obstruction secondary to compression by the SMA. 1 It has not been reported in Marfan syndrome (MFS) earlier, where gastrointestinal complications are noted to be rare. 2 SMAS can present with acute symptoms, as in the present case, or with chronic nonspecific postprandial abdominal pain or fullness, vomiting or early satiety, leading to a diagnostic dilemma. 3 4 Asthenic build, rapid weight loss and spinal deformities are major risk factors for SMAS. 1 5 In children with scoliosis coupled with adolescent skeletal growth, the aortomesenteric angle may become narrow, leading to a risk of SMAS. 5 Moreover, MFS patients are underweight, and thus, may have less periduodenal fat to cushion and protect the duodenum in the SMA angle. [...]the present case elicits the unusual causal association between these two syndromes.