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WILKIE'S SYNDROME: A RARE CAUSE OF INTESTINAL OBSTRUCTION

INTRODUCTION

Superior mesenteric artery (SMA) syndrome or Wilkie's syndrome is a rare but potentially life threatening gastrointestinal condition. This syndrome is a clinical phenomenon believed to be caused by compression of the third part of the duodenum between the SMA and the aorta, leading to obstruction. Patients may present symptoms of gastrointestinal obstruction, such as with recurrent episodes vomiting, upper abdominal distension and epigastric tenderness88. Shiu JR, Chao HC, Luo CC, Lai MW, Kong MS, Chen SY, Chen CC,Wang CJ. Clinical and nutritional outcomes in children with idiopathic superior mesenteric artery syndrome. J Pediatr Gastroenterol Nutr 2010; 51:177-82.. Various etiology theories, clinical course and treatment options have hitherto been discussed55. Mathenge N, Osiro S, Rodriguez II, Salib C, Tubbs RS, Loukas M. Superior mesenteric arterysyndrome and its associated gastrointestinal implications. Clin Anat. 2014 Nov;27(8):1244-52.. An interdisciplinary teamwork provides the most beneficial diagnostic and therapeutic result in this often underestimated disease.

CASE REPORT

A 27 years old woman was referred to our hospital, with recurrent episodes of profuse vomiting and upper abdominal pain associated with loss of appetite and dyspepsia since two years. She had no other comorbidities. Had been treated at another hospital with proton pump inhibitors, analgesics and intravenous fluids. She had a history of chronic anorexia and progressive loss of weight along with recurrent episodes of vomiting and upper abdominal pain. Clinical examination revealed dehydration, asthenicity (body mass index 19,5 kg/m2, weight: 50 kg, length:160 cm), abdominal distension, epigastric tenderness. Laboratory investigations showed a total white cell count of 9 500 mm33. Hines JR, Gore RM, Ballantyne GH. Superior mesenteric artery syndrome. Diagnostic criteria and therapeutic approaches. Am J Surg 1984; 148:630-2 and hypokalaemia (serum potassium: 3 mEq/l). Plain radiograph of the abdomen revealed gastric dilation. Ultrasonography was unremarkable. Upper gastrointestinal endoscopy showed dilated stomach and duodenum. Contrast-enhanced computerized tomography scan revealed grossly distended stomach and duodenum proximal to the third part of the duodenum at the level of the origin of superior mesenteric artery with abrupt narrowing at this level, suggestive of Wilkie's syndrome. While, normally, the angle between the SMA and the aorta is 22° to 60°, in this case, the aortomesenteric angle was 13,5°(Figure 1). In this case, conservative management was inefficient, so surgical treatment aiming to bypass the obstruction by an anastomosis between the jejunum and the proximal duodenum (duodenojejunostomy) was successful.

FIGURE 1
- CT of the abdomen showing reduced angle between the superior mesenteric artery and the aorta, with compression of the duodenum

DISCUSSION

Wilkie's syndrome occurs when the third portion of the duodenum is compressed between the SMA and the aorta. While, normally, the angle between the SMA and the aorta is 25° to 60°, it is narrowed in this syndrome77. Neri S, Signorelli SS, Mondati E, Pulvirenti D, Campanile E, Di Pino L, Scuderi M, Giustolisi N, Di Prima P, Mauceri B, Abate G, Cilio D, Misseri M,Scuderi R. Ultrasound imaging in diagnosis of superior mesenteric artery syndrome. J Intern Med 2005;257:346-51. The aortomesenteric angle may be narrowed because of congenital anomalies, significant weight loss, lumbar hyperlordosis, restorative proctocolectomy with ileal-anal anastomosis11. Adson DE, Mitchell JE, Trenkner SW. The superior mesenteric artery syndrome and acute gastric dilatation in eating disorders: a report of two cases and a review of the literature. Int J Eat Disord 1997;21:103-14.,22. Goitein D, Gagne DJ, Papasavas PK, Dallal R, Quebbemann B, Eichinger JK, Johnston D, Caushaj PF. Superior mesenteric artery syndrome after laparoscopic Roux-en-Y gastric bypass for morbid obesity. Obes Surg 2004; 14:1008-11,66. Matheus Cde O, Waisberg J, Zewer MH, Godoy AC. Syndrome of duodenal compression by the superior mesenteric artery following restorative proctocolectomy: a case report and review of literature. Sao Paulo Med J 2005;123:151-3. Clinical features of Wilkie's syndrome are entirely vague and non-specific. The most prominent symptoms are post-prandial abdominal pain (59%), nausea (40%), vomiting (50%), early satiety (32%), and anorexia (18%). These symptoms are aggravated by lying supine after eating and are relieved by assuming the left lateral decubitus, prone or knee-chest position33. Hines JR, Gore RM, Ballantyne GH. Superior mesenteric artery syndrome. Diagnostic criteria and therapeutic approaches. Am J Surg 1984; 148:630-2. These symptoms are compatible with more common conditions such as peptic ulcer disease, biliary colic, pancreatitis, and mesenteric ischemia. Physical examination generally reveals an asthenic body habitus.

The diagnosis of Wilkie's Syndrome requires a high degree of clinical suspicion confirmed by radiographic studies demonstrating compression of the third portion of the duodenum. CT of the abdomen typically shows gastric and duodenal dilation and narrowed aortomesenteric angle99. Unal B, Aktas A, Kemal G, Bilgili Y, Güliter S, Daphan C, Aydinuraz K. Superior mesenteric artery syndrome: CT and ultrasonography findings. Diagn Interv Radiol 2005; 11:90-95. Wilkie's syndrome responds to conservative management in the form of adequate nutrition by enteral/parenteral feeding and proper positioning of the patient after feeds. Surgery is resorted to when conservative measures are ineffective or in patients with long history of progressive weight loss or pronounced duodenal dilatation with stasis and complications44. Massoud WZ. Laparoscopic management of superior mesenteric artery syn-drome. Int Surg 1995; 80:322-7..

REFERENCES

  • 1
    Adson DE, Mitchell JE, Trenkner SW. The superior mesenteric artery syndrome and acute gastric dilatation in eating disorders: a report of two cases and a review of the literature. Int J Eat Disord 1997;21:103-14.
  • 2
    Goitein D, Gagne DJ, Papasavas PK, Dallal R, Quebbemann B, Eichinger JK, Johnston D, Caushaj PF. Superior mesenteric artery syndrome after laparoscopic Roux-en-Y gastric bypass for morbid obesity. Obes Surg 2004; 14:1008-11
  • 3
    Hines JR, Gore RM, Ballantyne GH. Superior mesenteric artery syndrome. Diagnostic criteria and therapeutic approaches. Am J Surg 1984; 148:630-2
  • 4
    Massoud WZ. Laparoscopic management of superior mesenteric artery syn-drome. Int Surg 1995; 80:322-7.
  • 5
    Mathenge N, Osiro S, Rodriguez II, Salib C, Tubbs RS, Loukas M. Superior mesenteric arterysyndrome and its associated gastrointestinal implications. Clin Anat. 2014 Nov;27(8):1244-52.
  • 6
    Matheus Cde O, Waisberg J, Zewer MH, Godoy AC. Syndrome of duodenal compression by the superior mesenteric artery following restorative proctocolectomy: a case report and review of literature. Sao Paulo Med J 2005;123:151-3
  • 7
    Neri S, Signorelli SS, Mondati E, Pulvirenti D, Campanile E, Di Pino L, Scuderi M, Giustolisi N, Di Prima P, Mauceri B, Abate G, Cilio D, Misseri M,Scuderi R. Ultrasound imaging in diagnosis of superior mesenteric artery syndrome. J Intern Med 2005;257:346-51
  • 8
    Shiu JR, Chao HC, Luo CC, Lai MW, Kong MS, Chen SY, Chen CC,Wang CJ. Clinical and nutritional outcomes in children with idiopathic superior mesenteric artery syndrome. J Pediatr Gastroenterol Nutr 2010; 51:177-82.
  • 9
    Unal B, Aktas A, Kemal G, Bilgili Y, Güliter S, Daphan C, Aydinuraz K. Superior mesenteric artery syndrome: CT and ultrasonography findings. Diagn Interv Radiol 2005; 11:90-95
  • Financial source: none

Publication Dates

  • Publication in this collection
    Jan-Mar 2016

History

  • Received
    09 Dec 2014
  • Accepted
    19 Nov 2015
Colégio Brasileiro de Cirurgia Digestiva Av. Brigadeiro Luiz Antonio, 278 - 6° - Salas 10 e 11, 01318-901 São Paulo/SP Brasil, Tel.: (11) 3288-8174/3289-0741 - São Paulo - SP - Brazil
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