The superior mesenteric artery (SMA) syndrome is a rare but potentially life-threatening gastrointestinal condition. Over the years, it has been referenced by several names, the most common of which is Wilkie's syndrome. These numerous terminologies have made it difficult to estimate its true frequency in the general population. Common symptoms associated with this syndrome include intermittent postprandial abdominal pain, nausea, and bilious vomiting. Our review revealed that although it is currently well-defined in the literature, the diagnosis of SMA syndrome remains challenging as other disorders can mimic its presentation. However, CT angiography is currently favored in the literature for diagnosis as it can not only show the narrowed aorto-mesenteric angle and distance, but also the extent of duodenal obstruction. In addition, we found no consensus on the preferred mode of therapy once SMA syndrome is diagnosed. The agreement among authors is that the treatment options should be based on severity of the disease, using conservative measures as the first line of therapy in mild SMA syndrome. Duodenojejunostomy is the preferred surgical approach when conservative management fails, or in severe cases.

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Cranial fasciitis (CF) is an uncommon benign myofibroblastic proliferation involving the soft and hard tissues of the cranium. It typically occurs in the pediatric population with a male predilection (male-to-female ratio 1.5:1). The clinical presentation is usually a rapidly expanding, painless nodule. Bone erosion may be appreciated radiographically. Histopathologic sections of CF show plump, fibroblast-like cells with pale, oval shaped nuclei and prominent nucleoli in a fibrous or myxoid background. Growth is self-limited and surgical excision is considered curative. Due to these features, CF is thought to be a variant of nodular fasciitis (NF). As with NF, CF may mimic a sarcomatous process and pose a diagnostic challenge to clinicians and pathologists alike. Erickson-Johnson et al. identified rearrangements of the ubiquitin-specific protease 6 (USP6) gene in 44 of 48 cases of NF. MYH9 was the fusion partner in 12 of these cases. To date, the molecular profile of CF has not been studied. Here we present the molecular findings in three cases of CF identified at our institution. Each case was subjected to fluorescence in-situ hybridization with appropriate negative controls. Two of three cases were positive for the USP6 gene rearrangement. The third case failed to hybridize, likely related to nucleic acid damage secondary to decalcification. Negative controls did not demonstrate the genetic rearrangement. These findings warrant further investigation of the USP6 gene rearrangement in CF, as it may prove helpful as a diagnostic adjunct in challenging cases.

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