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Oct 22, 2018

7th Congress of the European Academy of Paediatric Societies

Superior mesenteric artery syndrome in A Child with Sanjad Sakkatti Syndrome: A case report

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superior mesentric artery syndrome

sanajad sakati syndrome

progressive loss of weight

Abstract

Abstract

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Keywords

superior mesentric artery syndrome

sanajad sakati syndrome

progressive loss of weight

Abstract

Introduction Sanjad Sakati syndrome (SSS) is a recently described syndrome mainly from the Middle East and the Arabian Gulf countries. Children affected with this condition are born with IUGR and present with hypocalcaemic tetany or seizures due to hypoparathyroidism at an early stage in their lives. They have typical physical features, namely; long narrow face, deep set small eyes, beaked nose, large floppy ears and micrognathia, severe failure to thrive and mild to moderate mental retardation. Superior mesenteric artery syndrome (SMAS) was described for the first time by Von Rokitansky in 1861. It commonly occurs due to a rare entity in which the third part of duodenum is compressed between the superior mesenteric artery and the aorta causing acute or chronic duodenal obstruction. SMAS mostly develops after a rapid and dramatic weight loss. The most common symptoms of SMAS are epigastric fullness with pain specially after food, eructation, and bilious vomiting. The first proposed surgery for SMAS was described as duodenojejunostomy by Bloodgood. Case Report A 8 year young girl known to have Sanjad Sakati Syndrome since her early life on the maintenance treatment in the form of vitamin D supplementation and calcium. She was presented with recurrent hospital visits with abdominal pain and vomiting with progressive weight loss. Vomiting is non bilious and non projectile since 3 months back associated with abdominal pain which was colicky in nature for which the patient received carminative and more review of her medication as a case of SSS. The pain were usually precipitating by eating food and relieved after bouts of vomiting (undigested food). Her vitals were stable. Abdominal examination revealed epigastric fullness and hyper peristaltic bowel sounds. Routine blood and urine examination were normal. Ultrasonography (USG) of the abdomen was normal. Upper gastrointestinal barium study showed dilated stomach with dilated second part of the duodenum and cut off at the third part of duodenum with no intrinsic mucosal abnormalities (Fig 1). Computed tomography of the abdomen contrast enhanced showed marked distention of the stomach with dilatation of second part of duodenum and constriction/extrinsic compression of the third part of duodenum between aorta and SMA (Figs 2). The clinical symptoms and signs with investigative findings suggested the diagnosis of SMAS. The child referred to pediatric surgery who preferred to start conservative treatment in the form of naso-jejunal (NJ) tube for feeding the child and increase the child`s weight. The parents was advised to give frequent small meals of nutritious liquid and high energy milk, advised to lie on left side/prone following meals. Her initial weight before persistent vomiting was 8 Kg then after the acute illness she became 6 Kg on admission then after 2 weeks of admission and feeding on NJ tube the weight became 6.5 Kg. Her nutrition is under supervision of highly specialized nutrition specialist.

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All rights reserved.