Pediatric Wilkie’s Syndrome: Report of two monozygotic siste
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Wilkie’s syndrome (WS), also called the aortomesenteric compass (AMC) or the superior mesenteric artery (SMA) syndrome, refers to the condition in which the third portion of the duodenum is compressed between the SMA, anteriorly, and the aortovertebral plane, posteriorly. The etiology is multifactorial (i.e., weight loss, deformity of the spine), and it is more common in females (2:1).

Two monozygotic twins with nervous anorexia (body mass index [BMI] less than 15), presented with biliary vomiting and abdominal pain, the first at the age of 11-years and the second when she was 14-years old. The patients were in poor general conditions, with diffuse abdominal pain. Both had lost about 15 kg in the previous 12 months. Radiological findings were similar: abdominal ultrasound showed massively distended stomach filled with fluid content, consistent with significant gastrectasia at plain abdominal X-ray. About 3 L of biliary secretions were drained from the stomach via the insertion of nasogastric tube (NGT) in both of them. After abdominal (CT) scans, the suspicions of duodenal obstruction because of aortomesenteric compasses were confirmed.

Both the twins were conservatively managed as inpatients with fasting, maintenance of NGT, and total parenteral nutrition (TPN). The NGTs have been maintained for 20 days; at those stages, upper gastrointestinal contrast studies were obtained, demonstrating patency of the duodenojejunal tracts. Overall, the patients were hospitalized for 1 month each to confirm the weight gain and to consolidate psychological support. Every condition that leads to rapid weight loss causes the reduction of the fatty cushion around the SMA, leading to the sharpening of the aortomesenteric angle. Alert symptoms could be vomiting, failure to thrive, abdominal pain, or constipation.

Treatment should be initially conservative, aiming for weight gain by TPN, and gastric decompression. Surgical treatment should be reserved to cases completely refractory to prolonged conservative approach (more than 1 month without improvement), and laparoscopic duodenojejunostomy is the technique of choice.

The WS in twins pose the suspicion of genetic predisposing factors: a short ligament of Treitz and a low SMA origin, in association to weight loss, could contribute to the development of WS. The placement of NGT associated with prolonged TPN should be considered as a first-line treatment in the WS syndrome. It remains to be clarified whether the hit is a genetic predisposition or an acquired susceptibility.

Source: https://www.pediatr-neonatol.com/article/S1875-9572(20)30172-8/fulltext?rss=yes
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