Perplexing Case of Wilkie's Syndrome: A Rare Presentation in a Young Patient

Waqar, Syed Hamza Bin; Khan, Anosh Aslam; Mohiuddin, Osama

doi:10.7759/cureus.5085

Cited by 5 publications

(13 citation statements)

References 11 publications

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“…Wilkie's syndrome is characterized by a narrowing of the aortomesenteric angle. In a physiologic state and due to the erect position, the aorta-SMA angle ranges from 38º to 65º [ 7 ] and is maintained by the presence of perivascular fat tissue. As for the normal aortomesenteric distance, it lies between 10 and 28 mm [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

Wilkie's Syndrome: An Unexpected Finding

Claro¹,

Sousa²,

Silva³

et al. 2021

Cureus

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Wilkie's syndrome is a rare pathology caused by extrinsic compression of the third part of the duodenum by the superior mesenteric artery (SMA) at its origin. The symptoms are variable and non-specific consisting of postprandial abdominal pain, nausea and vomiting, early satiety, anorexia, and weight loss. A contrastenhanced CT scan is the standard imaging modality. Surgery is reserved for severe cases or those unresponsive to pharmacological treatment.We present a case of a 66-year-old woman with a history of prolonged postprandial abdominal pain, nausea, and substantial weight loss (30 kg in four months). Laboratory tests revealed acute renal failure with hypokalemia due to severe dehydration. She underwent an abdominal-pelvic CT scan that showed enlargement of the second and third parts of the duodenum, without an identifiable cause, followed by an upper gastrointestinal (GI) endoscopy that showed a dilated duodenum until D2 and inability of progression to D3, without mucosal abnormalities. Her MRI revealed considerable gastric and proximal duodenum distension with compression of D3 by the SMA. The patient underwent a laparoscopic duodenojejunostomy with intra-operative findings consistent with the diagnosis. The procedure and the postoperative period were uneventful, and the patient was discharged on the ninth postoperative day. Gastrografin study made at day six postop showed normal progression of the oral contrast. At the outpatient reevaluation one month postop, she remained asymptomatic and with progressive weight gain.Wilkie's syndrome is a rare form of intestinal obstruction, which is commonly disregarded. Its non-specific symptoms make it a challenging diagnosis and imply a high clinical suspicion. Among the different surgical options, duodenojejunostomy presents the best outcomes.

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Section: Discussionmentioning

confidence: 99%

Wilkie's Syndrome: An Unexpected Finding

Claro¹,

Sousa²,

Silva³

et al. 2021

Cureus

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“…However, other conditions (i.e., anatomical, surgical, pubescent) may predispose aortomesenteric angle decreases as well. [2][3][4][5][6] Congenital SMAS is very rare, with only a small number of patients being diagnosed in the medical literature. Congenital SMAS occurs due to an abnormal anatomical placement of the ligament of Treitz.…”

Section: Discussionmentioning

confidence: 99%

“…1 Abnormal gastrointestinal anatomy, corrective spinal surgery, and pediatric growth spurts have all been described causing SMAS as well. [2][3][4][5][6] Conditions causing rapid weight loss (e.g., anorexia nervosa, malabsorption, and cancer) can each decrease the fat pad cushion, leading to SMAS. 2 Common presenting complaints include less specific gastrointestinal obstruction symptoms, such as nausea, vomiting, early satiety (i.e., "feeling full") and postprandial (i.e., after meal) pain.…”

Section: Intoductionmentioning

confidence: 99%

“…[2][3][4][5][6] Conditions causing rapid weight loss (e.g., anorexia nervosa, malabsorption, and cancer) can each decrease the fat pad cushion, leading to SMAS. 2 Common presenting complaints include less specific gastrointestinal obstruction symptoms, such as nausea, vomiting, early satiety (i.e., "feeling full") and postprandial (i.e., after meal) pain. 1 These initial complaints can lead to further cyclical food aversion, poor fluid and food intake, and weight loss that further worsen symptoms.…”

Section: Intoductionmentioning

confidence: 99%

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Wilkie’s Weight Loss Wonder: A Case Series

Haidar

Davies

Hussain

et al. 2020

Spartan Medical Research Journal

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INTRODUCTION Wilkie’s Syndrome, also known as Superior Mesenteric Artery Syndrome (SMAS), is a rare cause of bowel obstruction that can contribute to vague abdominal symptoms on clinical presentation. This syndrome occurs when the aortomesenteric angle decreases, compressing the third portion of the duodenum between the aorta and the superior mesenteric artery. An acute decrease in the mesenteric fat pad cushion between these two blood vessels is the primary etiology, although other causes (e.g., anatomical, postoperative, functional, and pubescent etiologies) have also been described. CASE PRESENTATION In the present cases, 2 females with a common history of recent weight loss presented to our institution with similar symptoms of abdominal pain, nausea and vomiting. Each patient was subsequently diagnosed with SMAS following imaging studies. Both patients experienced successful resolution of symptoms with conservative nutritional management. DISCUSSION Common presenting complaints of SMAS include nausea, vomiting, early satiety and postprandial pain. These symptoms overlap with other gastrointestinal disorders (i.e., mesenteric ischemia, intestinal volvulus, peptic ulcer disease) making diagnosis difficult. SMAS can be identified through imaging modalities including barium studies and computer tomography. First line therapies typically include conservative nutritional support and promotion of weight gain. If conservative therapies fail, various surgical procedures can be pursued. Delayed diagnosis can lead to further pathological sequelae, including duodenal compromise, ischemia and necrosis. As the syndrome progresses, success of conservative nutritional support is less likely, and surgical correction becomes increasingly necessary. CONCLUSION Therefore, a clinical goal for SMAS should include as swift a recognition and diagnosis as possible.

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“…Given its various names and ill-defined diagnosis, the exact incidence of SMA syndrome is difficult to determine. SMA syndrome is reported more frequently among older children and adolescents which could be related to rapid growth without corresponding weight gain [1,5,6].…”

Section: Discussionmentioning

confidence: 99%

Superior Mesenteric Artery Syndrome Secondary to Anorexia Nervosa and Methamphetamine Use

Johnson¹,

Paladugu²

2019

Cureus

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Superior mesenteric artery (SMA) syndrome, or Wilkie syndrome, is a rare cause of small bowel obstruction due to compression of the duodenum between the SMA and aorta. Patients most at risk of SMA syndrome include those with rapid weight loss due to a variety of conditions including chronic illness, malignancy, trauma, HIV, eating disorders, substance abuse, or bariatric surgery. Characteristic radiologic findings include an aortomesenteric angle less than 25 degrees and an aortomesenteric distance of less than 8 mm. Symptoms are typically postprandial and notably include abdominal fullness, voluminous emesis, and abdominal pain. Here we present a case of SMA syndrome in a 19-year-old cachectic female who initially presented with sudden-onset nausea, vomiting, and severe abdominal pain. Imaging revealed a severely distend stomach and proximal duodenum with a transition point in the third portion of the duodenum consistent with SMA syndrome. Her symptoms resolved with nasogastric decompression in addition to fluid and electrolyte management. She later endorsed restrictive eating patterns consistent with anorexia nervosa as well as methamphetamine use for weight loss. She underwent close outpatient follow-up for her anorexia nervosa and substance abuse.

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Perplexing Case of Wilkie's Syndrome: A Rare Presentation in a Young Patient

Cited by 5 publications

References 11 publications

Wilkie's Syndrome: An Unexpected Finding

Wilkie's Syndrome: An Unexpected Finding

Wilkie’s Weight Loss Wonder: A Case Series

Superior Mesenteric Artery Syndrome Secondary to Anorexia Nervosa and Methamphetamine Use

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