Superior mesenteric artery syndrome and its associated gastrointestinal implications

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. Clin. Anat. 27:1244–1252, 2014. © 2013 Wiley Periodicals, Inc.     

Localized malignant mesenteric mesothelioma causing small bowel obstruction

Malignant mesothelioma is an uncommon lethal neoplasm in the serous membrane in which peritoneal mesothelioma is a rarer form. Herein is reported a case of malignant mesothelioma presenting as a localized mass inside the mesentery causing focal luminal obstruction of the small intestine. The diagnosis of malignant mesothelioma was obtained on repeat double balloon endoscopic biopsy. Partial resection of the small intestine along with the mesentery was performed, followed by a course of chemotherapy. No relapse of the disease has been found in the 8 months' follow up radiologically. To the best of the authors' knowledge this is the first reported case of localized malignant mesothelioma arising inside the mesentery. Mesothelioma should be considered as the differential diagnosis when small bowel obstruction occurs with unknown primary neoplasm.     

Superior mesenteric artery aneurysm in a 9-year-old boy with classical Ehlers-Danlos syndrome

A 9-year-old boy with the classical type of Ehlers-Danlos syndrome (EDS) developed a symptomatic aneurysm of the superior mesenteric artery. His EDS diagnosis had been confirmed biochemically and genetically. Vascular complications are known to be associated with the vascular type of EDS, but this is the first report of a child with classical EDS who developed a major vascular complication. Clinicians should be aware that severe vascular complications albeit rare, can also occur in classical EDS.     

Superior mesenteric artery syndrome due to an aortic aneurysm in a renal transplant recipient

Superior mesenteric artery (SMA) syndrome is a rare disease in which the third portion of the duodenum is compressed by SMA. There are many causes leading to the SMA syndrome, however it's extremely rare that aortic aneurysm causes a SMA syndrome. We report a case of a successfully treated SMA syndrome due to an abdominal aortic aneurysm in a renal transplant recipient. The patient was a 52-yr-old woman with a thin stature (weight 40 kg, height 164 cm). She received a renal transplant 8 yr before, and had hypertension and abdominal aortic aneurysm. Her SMA syndrome developed in a prolonged supine position for the accidental rib fractures and was diagnosed by clinical and radiological findings. After a surgical correction (resection of an aneurysm and aortobiiliac bypass with an inverted Y graft), her symptoms relieved without deterioration of the graft function.     

Superior mesenteric origin of the proper hepatic artery: embryological and clinical implications

The hepatic arteries are subject to a great deal of anatomical variation, potentially complicating hepatobiliary surgical procedures as well as general gastrointestinal procedures that involve foregut and midgut structures. We report a case of a rare variant of the proper hepatic artery discovered during dissection of an 84-year-old male cadaver. In this individual, the common hepatic artery was absent and the proper hepatic artery was replaced directly to the superior mesenteric artery. The gastroduodenal artery and the right inferior phrenic artery took origin from the celiac trunk. In addition, there was no identifiable right gastric artery. The celiac trunk gave off three branches: the splenic, left gastric, and gastroduodenal arteries. The entire arterial blood supply to the liver, therefore, was derived from the superior mesenteric artery. Patterns of regression of the ventral branches and the partial disappearance of the ventral anastomotic arteries during embryonic development play a major role in the variations of the gut arteries. An intraoperative encounter with this particular variant carries a significant risk of iatrogenic injury with potentially devastating ischemia and necrotic results. Accurate depiction and definition of the hepatic arterial anatomy are crucial. Variations like the one described here underscore the importance of pre-operative imaging and knowledge of the embryological origins of variation.     

Histological phenotypes of enteric smooth muscle disease causing functional intestinal obstruction in childhood

Aims: Functional intestinal obstruction or chronic idiopathic intestinal pseudo-obstruction is due to defects either in the enteric innervation or in intestinal smooth muscle. We have studied full-thickness intestinal biopsies from 27 patients with functional intestinal obstruction due to enteric smooth muscle disease by routine histology and electron microscopy together with histochemical and immunohistochemical techniques to detect changes in the intestinal smooth muscle. Methods and results: Two patients appeared to have an acquired intestinal myopathy as a result of an autoimmune process. In 25 the disorders were congenital, of these seven had segmental abnormalities limited to the rectum and distal colon and 18 had a diffuse disease affecting both the small and large bowel. We identified five apparent histological phenotypes of enteric muscle disease, three of which represent abnormalities in morphogenesis resulting in alterations in intestinal muscle layering and two exemplify intrinsic myocyte defects and/or changes in the extracellular matrix. Conclusions: Careful phenotyping of these patients is important in devising optimal treatment and in understanding the underlying defect as well as the possible genetic mechanisms resulting in these abnormalities. Recognition of autoimmune smooth muscle disease is helpful, since making the diagnosis influences the patient's management.     

Histological phenotypes of enteric smooth muscle disease causing functional intestinal obstruction in childhood

Aims : Functional intestinal obstruction or chronic idiopathic intestinal pseudo-obstruction is due to defects either in the enteric innervation or in intestinal smooth muscle. We have studied full-thickness intestinal biopsies from 27 patients with functional intestinal obstruction due to enteric smooth muscle disease by routine histology and electron microscopy together with histochemical and immunohistochemical techniques to detect changes in the intestinal smooth muscle. Methods and results : Two patients appeared to have an acquired intestinal myopathy as a result of an autoimmune process. In 25 the disorders were congenital, of these seven had segmental abnormalities limited to the rectum and distal colon and 18 had a diffuse disease affecting both the small and large bowel. We identified five apparent histological phenotypes of enteric muscle disease, three of which represent abnormalities in morphogenesis resulting in alterations in intestinal muscle layering and two exemplify intrinsic myocyte defects and/or changes in the extracellular matrix. Conclusions : Careful phenotyping of these patients is important in devising optimal treatment and in understanding the underlying defect as well as the possible genetic mechanisms resulting in these abnormalities. Recognition of autoimmune smooth muscle disease is helpful, since making the diagnosis influences the patient's management.