This syndrome is variably called as arterial mesenteric duodenal compression, cast syndrome or wilkies syndrome. Superior mesenteric artery sma syndrome is a rare condition that involves the small intestine. Superior mesenteric artery sma syndrome arises from a reduction in the angle formed between the sma and the aorta, thereby compressing the third portion of the duodenum. Total score ranges from 0 to 3,600 being 0 the worst and 3,600 the best. For language access assistance, contact the ncats public information officer.
Health, general arterial occlusions care and treatment case studies diagnosis arterial occlusive diseases gastrointestinal diseases. Superior mesenteric artery syndrome genetic and rare. Superior mesenteric artery sma syndrome clinical presentation. Superior mesenteric artery syndrome research awareness and. However, after extensive research, original data establishing this mortality rate has not been found, indicating that the number is likely to be unreliable. Acute pancreatitis and superior mesenteric artery syndrome. Superior mesenteric artery syndrome or wilkie syndrome is an acquired rare vascular compression disorder due to acute angling of the superior mesenteric artery sma resulting in compression of the 3rd part of the duodenum leading to obstruction. The full text of this article is available in pdf format. This familial clustering raises the possibility of a genetic predisposition to this symptom and radiographic complex. Familial superior mesenteric artery syndrome springerlink. Superior mesenteric artery syndrome is a rare cause of proximal small.
Results for superior mesenteric artery syndrome 21 30 of 74 sorted by relevance date. The four daughters also had radiographic studies supporting that diagnosis. Volume 11 issue 2 computed tomography and ultrasonography in superior mesenteric artery syndrome 93 consisted of 10 females and 3 males, whereas group b consisted of 34 females and 42 males. Superior mesenteric artery sma syndrome is an unusual cause of vomiting and weight loss resulting from the compression of the third part of the duodenum by the sma. Superior mesenteric artery syndrome is suggested the 3 rd part of the duodenum is compressed between the aorta and the superior mesenteric artery with consequential dilatation of the proximal duodenum and stomach. Mesenteric ischemia bowel infarction therapy and ortners. Introduction superior mesenteric artery syndrome smas mostly occurs i n adolescent or young adults and is a rare disorder. Patients seen in acute rehabilitation settings, not uncommonly, have a period of rapid weight loss. Jun 14, 2018 superior mesenteric artery syndrome smas is a digestive condition that occurs when the duodenum the first part of the small intestine is compressed between two arteries the aorta and the superior mesenteric artery.
Laparoscopic management of superior mesenteric artery syndrome. Mean of superior mesenteric artery syndrome is 0 points 36 %. See how superior mesenteric artery syndrome is diagnosed. Aneurysms of the superior mesenteric artery sma and its branches are rare. Which specialists are essential to meet, what tests are needed and other useful information for the diagnosis of superior mesenteric artery syndrome.
This exploration revealed small bowel ischemia with a parietal thickening and, at the early arterial phase, a 90% reduction of the superior mesenteric artery sma lumen 2 cm from its origin fig 1, combined with a slight ectasic aspect of the origin of the celiac artery. Aneurysms of the superior mesenteric artery and its branches. Superior mesenteric artery syndrome associated with rapid. It is a rare but lifethreatening syndrome caused by inadequate flow of blood through the mesenteric vessels resulting in ischemia and gangrene of the bowel wall. Contrast upper gi study was performed that demonstrated an abrupt cutoff in the third portion of the duodenum with a massively distended. The patient was afebrile and vital signs were stable. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 toll free. The patient will present with bloating after meals, nausea and vomiting and a feeling of abdominal fullness. Superior mesenteric artery syndrome is an unusual cause of proximal intestinal obstruction. Superior mesenteric artery syndrome my story youtube.
In five cases in group a, all of the investigated clinical findings i. The superior mesenteric artery syndrome smas is characterized by the compression of the third segment of duodenum by the mesentery at sma level, and a resulting duodenal gastric dilatation 16. Aneurysms of the superior mesenteric artery and its. Superior mesenteric artery syndrome and nutcracker phenomenon. Apr 17, 2015 superior mesenteric artery syndrome 1. Apr 27, 2017 superior mesenteric artery syndrome, gastroparesis and gerd. After 1400 cm 3 of emesis was noted, a nasogastric tube was placed and 2500 cm 3 of gastric fluid was obtained overnight. The syndrome may be imitated or caused by an eating disorder and having to distinguish the two from each other is very important since misdiagnosis can be really dangerous. This compression causes partial or complete blockage of the duodenum. The syndrome is characterized by compression of the third portion of the duodenum due to. Superior mesenteric artery sma syndrome also known as wilkies syndrome is a rare condition caused by the entrapment of the third part of the duodenum between the aorta and the sma. Radiological modalities play a major role in its diagnosis and surgical methods remain the gold standard in its management. This study aims at analyzing the clinical presentation, diagnosis, and management of sma syndrome. Mesenteric artery disease healthcare baylor college of.
The superior mesenteric artery sma syndrome is an atypical cause of high intestinal obstruction in adults. Superior mesenteric artery syndrome causes, symptoms, diagnosis, treatment. The importance of mesenteric aneurysms and different procedures for treatment are discussed. Superior mesenteric artery syndrome, gastroparesis and.
Superior mesenteric artery syndrome coexists with nutcracker. Case report superior mesenteric artery syndrome and its rami. Superior mesenteric artery syndrome smas is a rare condition caused by compression of the third part of the duodenum between the superior mesenteric artery sma and the aorta, causing symptoms. It has been referred to by a variety of other names, including cast syndrome, wilkie syndrome, arteriomesenteric duodenal obstruction, and chronic duodenal ileus. Introductionsuperior mesenteric artery syndrome is a rare condition. Case discussion superior mesenteric artery syndrome. This phenomenon may be caused by a number of factors, one of which being acute weight loss. Superior mesenteric artery syndrome radiology case. The condition occurs when the duodenum the part of the small. Superior mesenteric artery syndrome rani sophia and waseem ahmad bashir yeovil hospital nhs foundation trust, yeovil, somerset united kingdom 1.
We report a case of a female patient presenting with abdominal pain and vomiting who developed superior mesenteric artery sma. Superior mesenteric artery syndrome and its ramifications. Five members of a family of eight, including the father and four daughters, presented with symptoms previously attributed to the superior mesenteric artery syndrome. Superior mesenteric artery syndrome, new advances in the basic and clinical gastroenterology, thomas brzozowski. Its also to explain what happened during this time because most people. The differential diagnosis includes anorexia nervosa and bulimia.
This results in chronic, intermittent, or acute complete or partial duodenal obstruction. Superior mesenteric artery syndrome stacismasnpo on. It is relatively rare and can be hard to distinguish from other causes of intestinal obstruction. Ct or ct angiogram is also superior to ultrasound because it can measure the reduced aortomesenteric angle and sh ow the gastric and duod enal dilatation at. Superior mesenteric artery syndrome statpearls ncbi bookshelf. How is superior mesenteric artery syndrome diagnosed. Superior mesenteric artery syndrome genetic and rare diseases. Read more about symptoms, diagnosis, treatment, complications, causes. History, physical, causes superior mesenteric artery sma syndrome is an uncommon but well recognized clinical entity characterized by compression of the third, or transverse, portion of the duodenum between the aorta and the superior mesenteric artery. The superior mesenteric artery syndrome is characterized by the compression of the third segment of the duodenum by the mesentery at superior mesenteric artery level and a resulting duodenal. Superior mesenteric artery syndrome a rare presentation. Superior mesenteric artery sma syndrome is a form of. Definition of mesenteric ischemia infarction and necrosis through the mesenteric ischemia. Superior mesenteric artery syndrome research awareness and support, bonham, texas.
Sanjadsakati syndrome and its association with superior. Is there a cure for the superior mesenteric artery syndrome. Dec 03, 2017 the purpose of this video is spread awareness about a rare, lifethreatening disease by sharing my story with the condition. Superior mesenteric artery sma syndrome is an uncommon but well recognized clinical entity characterized by compression of the third, or transverse, portion of the duodenum between the aorta and the superior mesenteric artery. Clinical report by journal of evolution of medical and dental sciences. Amazon prime days is coming up and its a great time to help the nonprofit raise a little money. Pals patient airlift services patient airlift services pals arranges free air transportation for individuals requiring medical diagnosis, treatment or followup, for compassionate assistance, f. Superior mesenteric artery sma syndrome is a gastrovascular disorder in which the third and. Occurs when duodenum is compressed between aorta and superior mesenteric artery. In addition, superior mesenteric artery sma syndrome should be differentiated from other causes of megaduodenum or duodenal ileus, including diabetes mellitus, collagen vascular conditions, and chronic idiopathic intestinal pseudoobstruction.
Superior mesenteric artery syndrome smas is a digestive condition that occurs when the duodenum the first part of the small intestine is compressed between two arteries the aorta and the superior mesenteric artery. Superior mesenteric artery syndrome is a rare condition, causing intestinal obstruction. Case report superior mesenteric artery syndrome and its. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. The ctscan was performed to screen the cause of abdominal pain. Superior mesenteric artery sma syndrome, also known as wilkies syndrome and cast syndrome, is a rare gastrointestinal disease that leads to obstruction of the duodenum. Minimally invasive surgery for superior mesenteric artery syndrome.
Mesenteric ischemia bowel infarction diagnosis, therapy, and ortners syndrome. Mesenteric or intestinal artery disease is a condition that develops when the arteries in the abdomen that supply the intestines become narrowed, or blocked, by an accumulation of a fatty substance called plaque. The superior mesenteric artery sma syndrome is a rare entity presenting with upper gastrointestinal tract obstruction and weight loss. Some studies report the incidence of superior mesenteric artery syndrome to be 0.
The purpose of this video is spread awareness about a rare, lifethreatening disease by sharing my story with the condition. Superior mesenteric artery sma syndrome differential diagnoses. Laparoscopic duodenojejunostomy for treatment of superior mesenteric artery syndrome. Sma supplies the midgut up to the spleenic flexure. Patients with sma syndrome, like those with gastroparesis and gerd, present with epigastric pain, bloating, nausea, vomiting and early satiety as well as heartburn and food regurgitation. Aug 06, 2015 superior mesenteric artery syndrome or wilkie syndrome is an acquired rare vascular compression disorder due to acute angling of the superior mesenteric artery sma resulting in compression of the 3rd part of the duodenum leading to obstruction. Statistics of superior mesenteric artery syndrome 23 people with superior mesenteric artery syndrome have taken the sf36 survey. Downloaded free from on thursday, march 03, 2016, ip. An indication of a mesenteric infarction is given by blood on the finger stall during the. Superior mesenteric artery, sma, syndrome is a rare disorder. Superior mesenteric artery sma syndrome is a rare disorder, characterized by compression of the third segment of.
A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for superior mesenteric artery. Ct scan of the abdomen shows hugely distended stomach, first and second part of the duodenum, with transition zone at the origin of the sma, confirming the diagnosis of sma syndrome. Jun 01, 2015 superior mesenteric artery syndrome smas is caused by the compression of the superior mesenteric artery sma against the 3rd part of the duodenum creating a proximal intestinal obstruction. Studies to determine the optimal methods of diagnosis and treatment are required. Superior mesenteric artery syndrome a rare presentation and. Obstruction of the third part of the duodenum d3 is a very rare cause of gastric outflow obstruction. Superior mesenteric artery syndrome smas was diagnosed in a 16yearold boy who presented with a short history of repeated vomiting associated with weight loss to the point of collapse. After resuscitation and investigation, he was treated surgically. Pdf download for minimally invasive surgery for superior mesenteric.
Please share the word and ask your friends to shop through our link. Superior mesenteric artery sma syndrome is a gastrovascular disorder in which the third and final portion of the duodenum is compressed between the abdominal aorta aa and the overlying superior mesenteric artery. Zimmermanc adepartment of radiology, long island college hospital, 339 hicks street, brooklyn, ny 11201, usa bdepartment of pediatric gastroenterology, long island college hospital, 339 hicks street, brooklyn, ny 11201, usa. To access free multiple choice questions on this topic, click here. Superior mesenteric artery syndrome, gastroparesis and gerd. Diagnosis superior mesenteric artery syndrome an abdominal computed tomography ct scan with contrast revealed the narrowing of the third portion of the duodenum compressed by the superior mesenteric artery sma, with dilation of the portion of the duodenum proximal to the compression. Closure of the inferior mesenteric artery usually has a mild course. Its is a difficult syndrome to diagnose as alot of drs. We describe three patients with aneurysms in the sma or its branches found at angiography in our department.
Superior mesenteric artery syndrome symptoms, treatment. Im going to answer in technical terms because i assume you asking the question means either you are a med. Superior mesenteric artery syndrome symptoms, causes, diagnosis, and treatment information for superior mesenteric artery syndrome superior mesenteric artery syndrome with alternative diagnoses, fulltext book chapters, misdiagnosis, research treatments, prevention, and prognosis. Superior mesenteric artery sma syndrome differential. We are a registered nonprofit with the ultimate goals of raising awareness of. A 1in3 mortality rate for superior mesenteric artery syndrome has been quoted by a small number of sources. The laboratory results on presentation revealed a bun 25 and a creatinine 1. Superior mesenteric artery syndrome smas is caused by the compression of the superior mesenteric artery sma against the 3rd part of the duodenum creating a proximal intestinal obstruction. Superior mesenteric artery syndrome evidence search nice. Apr 28, 2014 im going to answer in technical terms because i assume you asking the question means either you are a med. Superior mesenteric artery syndrome 417 lee, mangla 1978. If you have problems viewing pdf files, download the latest version of adobe reader. Jul 05, 2015 inferior mesenteric artery the inferior mesenteric artery is the artery of hindgut it arises from the anterior or left anterolateral aspect of the aorta at about the level of third lumbar vertebra 3 or 4 cm above the aortic bifurcation. Superior mesenteric artery syndrome smas mostly occurs i n adolescent or young adults and is a rare disorder.
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