Familial superior mesenteric artery syndrome

Abstract

Superior mesenteric artery syndrome (SMAS) has an incidence of 0.1-0.3%. It is caused by a narrow angle between the aorta and the superior mesenteric artery, resulting in the clamping of the third portion of the duodenum. It is more common in females (2:1), with no racial differences. It is also more frequent in patients under 30 years and may have a congenital or acquired aetiology. There is only one case of the familial form described in the medical literature. We present a case of a 20-year-old woman, with recurrent episodes of epigastric discomfort and vomiting since she was 10 years old. After clinical suspicion, diagnosis was achieved after digestive endoscopy, upper gastrointestinal transit and abdominal MR. The patient was kept under parenteral nutrition for 17 days before surgery. She was discharged 8 days after surgery and the postoperative course was uneventful.

Co-occurring superior mesenteric artery syndrome and nutcracker syndrome requiring Roux-en-Y duodenojejunostomy and left renal vein transposition: a case report and review of the literature

“…The weight loss may simply provoke a further narrowing of a preexisting abnormally acute aortomesenteric angle, and, as a result, the vomiting and compression become self-perpetuating regardless of the initiating factors [ 40 ], particularly since we know that the compression can progress from irrelevant to complete obstruction [ 3 , 72 ]. Evidence of SMAS in individuals with certain skeletal deformities or who have undergone a surgical intervention to correct a skeletal problem suggests that rather than weight loss, certain congenital anatomic factors or post-surgical changes in skeletal conformation predispose an individual to this disorder [ 3 , 5 , 26 , 28 , 50 – 54 , 61 – 63 ].…”

“…There may also be a congenital explanation for SMAS that is supported by reports of SMAS in neonates [ 61 , 62 ], identical twins [ 26 ], and within families [ 35 , 63 ]. It may be the case that a higher positioned duodenum within a rather more acute aortomesenteric angle may leave enough room for the duodenum during childhood, but as the duodenum grows with age the available space within the angle becomes insufficient [ 1 ].…”

Superior mesenteric artery syndrome – diagnostic difficulties

“…In patients with diagnosed SMAS, the angle of branching of the superior mesenteric artery is below 26 °, the distance between the SMA wall and aortic aorta is 2-8 mm [4][5][6]. A familial susceptibility to the occurrence of SMAS has been demonstrated [7,8]. It mainly affects young children and women aged 18-35 years [9].…”

Síndrome de Arteria Mesentérica Superior o Síndrome de Wilkie. Caso Clínico

“…El origen de la AMS está ubicado a nivel de L1, orientándose de manera caudal, creando un ángulo de 45 a 60º en correlación con la aorta. La tercera porción del duodeno atraviesa por el ángulo formado, a nivel de L3-L4, este síndrome se atribuye esencialmente a la perdida de grasa mesentérica que intercede como cojinete entre dichas estructuras (5,6,7,8,9,10,11,12,13).…”

“…La incidencia exacta de este síndrome no es conocida con exactitud debido a que es poco diagnosticada por su rara frecuencia, además puede existir opresiones duodenales en menor grado que conllevarían a ser un cuadro asintomático. Según revisiones se ha encontrado una incidencia que varía del 0,13% y que no supera el 1% (2,3,6,7,8,9,12,15,16,19,20,21), presentando una mortalidad estimada del 33% (3,18).…”