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Superior mesentric artery compression syndrome
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     Dept of Pediatrics- Sir J.J. Group of Hospitals; Lecturer, Dept of Pediatrics- Sir J.J. Group of Hospitals and 3rd year M.B.B.S.- Grant Medical College & Sir J.J. Group of Hospitals, Mumbai, India

    Abstract

    Superior Mesenteric Artery Syndrome is an atypical cause of high intestinal obstruction seen frequently in patients with rapid weight loss or immobilization in a body cast (\also called CAST Syndrome). The SMA impinges on the third part of the duodenum immediately after originating from the anterior aspect of aorta, making an abnormally narrow angle with the later. Intestinal obstruction results causing characteristic symptoms like postprandial epigastric pain, eructations, fullness and vomiting. Here we report such a case of SMA syndrome in a patient with history of rapid loss of weight.

    Keywords: Superior Mesenteric Artery (SMA) syndrome, arteriomesentric duodenal compression, high intestinal obstruction.

    SMA syndrome, also known as arteriomesentric duodenal compression, the Cast syndrome and the Wilkie's syndrome was first described by Von Rokitansky in 1861. He proposed that its cause was obstruction of the third part of the duodenum due to superior mesenteric artery compression. We report a case that presented with symptoms and signs of partial intestinal obstruction and was subsequently diagnosed to be suffering from SMA Syndrome using Barium meal follow through and Colour Doppler studies.

    Case Report

    A 12-year-old male child presented with pain in epigastric region with fullness and voluminous eructations since 3-4 years. The symptoms get relieved on vomiting, which occurs on and off, irregularly after food. Vomitus is fluid- grayish green in colour. There is a history of weight loss evident by loosening of clothings. There is no palpable lump, liver or spleen on abdominal examination.

    Real Time Ultrasonography of abdomen revealed no abnormality. Barium Meal Follow Through revealed a partial obstruction to the passage of barium in third part of duodenum with slight dilatation of the second part Figure1. Also there was a delayed pyloric emptying with well-distended duodenal cap. A vertical linear impression possibly because of superior mesenteric artery is seen in the third part of duodenum in close relation to the site of obstruction. This is confirmed by Colour Doppler studies, which also showed the superior mesenteric vein, placed anteriorly & to the left of superior mesenteric artery Figure2.

    The patient was given strict advice on adequate nutrition. He was recommended proper positioning after eating e.g. left lateral decubitus, prone or knee chest position. He was followed for one year and has now gained adequate weight with no symptoms.

    Discussion

    The Superior Mesenteric Artery usually forms an angle of approx. 45° with the abdominal aorta at its origin, while the third part of the duodenum crosses in between the SMA anteriorly and aorta posteriorly[1]. Any factor that sharply narrows this aortomesentric angle (to less than 25° approx.) can cause entrapment and compression of the third part of the duodenum as it passes between the SMA & aorta, resulting in the Superior Mesenteric Artery Syndrome. Alternatively other causes implicated include high insertion of duodenum at the Ligament of Treitz, a low origin of SMA and compression of duodenum due to peritoneal adhesions Figure3

    Since Von Rokitansky first described in 1861, only about 400 cases have been described in English Language literature[4]. Just like the case described here, patients often present with chronic upper abdominal symptoms such as epigastric pain, nausea, eructations, voluminous vomiting (bilious or partially digested food), postprandial discomfort, early satiety and sometimes as subacute small bowel obstruction. These symptoms are typically relieved when the patient is in the left lateral decubitus, prone or knee-chest position and are often aggravated in supine position. An asthenic habitus can be noted in more than 2/3rd patients[3].

    The diagnosis of SMA Syndrome is difficult. Confirmation usually requires a radiographic study. A Hayes maneuver (pressure applied below the umbilicus in superodorsal direction), which elevates the root of small bowel mesentery, also may relieve the obstruction.

    Conservative initial treatment includes adequate nutrition, G.I. decompression and proper positioning after eating. Prokinetic drugs like metoclopromide, cisapride may be helpful. Enteral feeding through a tube passed distal to the obstruction[5] or total parenteral nutrition can be effective adjunct in treatment of patients with rapid and severe weight loss. Surgical intervention in the form of duodenojejunostomy is indicated only when conservative measures fail. The use of laparoscopic surgery[6] that involves lysis of the ligament of Treitz and mobilization of the duodenum has been reported. Our patient responded immediately to an adequate and balanced diet therapy and is now free of symptoms[8].

    References

    1. Babak Raissi, MD; Brian M. Taylor, MD, FRCSC; Donald H. Taves, MD, FRCPC. Canadian Journal of Surgery 1996; 39: 410-416.

    2. Ulises Baltazar, MD, Julie Dunn, MD, Carlos Floresguerra, MD, Larry Schmidt, MD and William Browder, MD, Johnson City, Tenn: Superior Mesenteric Artery Syndrome: An Uncommon Cause of Intestinal Obstruction: Southern Medical Journal, Vol. 93, No. 6, 2000; 606-608.

    3. Hutchinson DT, Bassett GS. Superior mesenteric artery syndrome in pediatric orthopedic patients. Clin Orthop 1990; 250: 250-257.

    4. Santer R, Young C, Rossi T, Riddles-berger MM. Computed tomography in superior mesenteric artery syndrome. Pediatr Radiol 1991; 21: 154-155.

    5. Smith SJ, Cooney RN. Superior mesenteric artery syndrome in a tube-fed patient. Nutr Clin Pract 1994; 9:151-153.

    6. Massoud WZ. Laparoscopic management of superior mesenteric artery syndrome. Int Surg 1995; 80: 322-327.

    7. Behram, Keligman, Arvin : Nelson's Textbook of Pediatrics, 17th edition: pg 1241

    8. John M. Daly, James T. Adams, Gray A. Fantini, Josef E. Fischer : Schwartz Principles of Surgery, 7th ed.: pg 1560.(Samdani PG, Samdani Vinit)