It is a rare cause of benign duodenal obstruction with a prevalence ranging from 0. Genotropin is a prescription product for the treatment of growth failure in children with a genetic condition called praderwilli syndrome pws. Superior mesenteric artery wilkies syndrome as a result. Apr 25, 2018 wilkie april 25, 2018 as discussed in our previous blog post entitled federal circuit rules veterans can get disability benefits for pain, the u. Traditionally treatment has consisted of conservative measures such as gastric decompression, parenteral nutrition andor postpyloric feeding. Praderwilli syndrome and growth hormone treatment please see important safety information on pages 6, 7, 8, 16, and 17 and accompanying full prescribing information in pocket.
Recent observations have stated a new etiologic concept in the development of this syndrome. The child was diagnosed to be suffering from wilkie s syndrome. Therefore, conservative therapy with nutritional supplementation is the initial approach, and surgery is reserved for those who do not respond to hyperalimentation. Superior mesenteric artery sma syndrome, also known as wilkies syndrome, is extremely rare and is characterized by postprandial epigastric pain, nausea, vomiting and. Superior mesenteric artery wilkies syndrome as a result of. Wilkie wilkinson 19032001, british auto mechanic and racing official. Wilkie syndrome, also referred as superior mesenteric artery syndrome, is an unusual cause of a proximal small bowel obstruction. Introduction the superior mesenteric artery syndrome sma or wilkie syndrome is the extrinsic compression of the third portion of the duodenum between the superior how to cite this paper. Acute dilatation of the stomachgastromesenteric ileus. Wilkies syndrome is a rare variant of small intestinal obstruction resulting from compression of third part of the duodenum by the superior mesenteric artery. The parents and relatives of such a case need to be offered genetic counseling to prevent the recurrence of fragile x syndrome in the family.
A rare cause of vomiting and weight loss ali coskun, deniz ucar, erdem bar. Superior mesenteric artery syndrome, intestinal obstruction, duodenojejunostomy, chad 1. Wilkie rasmussen born 1958, cook islands politician and former cabinet minister. Feb 10, 2003 wilkie s syndrome the patients underwent a laparoscopic duodenojejunal bypass, and resumed a normal diet on the fifth postoperative day. It is distinct from nutcracker syndrome, which is the entrapment of the left renal vein between the aa and the sma.
Superior mesenteric artery sma syndrome, also named as wilkie s syndrome ws, is a rare entity caused by the obstruction of 3rd proportion of the duodenum between sma and aorta. The superior mesenteric artery syndrome sma or wilkie syndrome is the extrinsic compression of the third portion of the duodenum between the superior mesenteric artery and the aorta. Superior mesenteric artery sma syndrome is a very rare. It is caused by a narrowed aortomesenteric angle that leads to compression of the third portion of duodenum between the abdominal aorta aa and superior mesenteric artery sma. We report a case of yearold female who was diagnosed with sma syndrome. Get a printable copy pdf file of the complete article 785k, or click on a page image below to browse page by page. Superior mesenteric artery sma syndrome differential diagnoses. The purpose of this case report is to discuss the diagnosis and conservative management of a patient with both wilkie syndrome and nutcracker syndrome. Superior mesenteric artery sma syndrome, also known as wilkie syndrome or cast syndrome or aortomesentric duodenal compression syndrome is a rare acquired vascular compression disorder in which acute angulation of the superior mesenteric artery sma results in compression of the third part of the duodenum, leading to obstruction. Wilkie requested that the examiner provide a complete rationale for the diagnosis. Melba saunders appeals from a decision of the united states court of appeals for veterans claims the veter. Jul 26, 2012 superior mesenteric artery syndrome superior mesenteric artery sma syndrome, also known as wilkie syndrome, is a rare acquired vascular compression disorder in which acute angulati.
It is defined by a decrease of aortomesenteric distance. Media in category superior mesenteric artery syndrome the following 5 files are in this category, out of 5 total. Sma syndrome is estimated to have a mortality rate of 1 in 3. Wilkes syndrome is a rare cause of partial or complete duodenal obstruction due to exterior compression of the third part of the duodenum by the superior mesenteric artery. Feb 01, 2004 wilkie s syndrome wilkie s syndrome kwan, eugene. Wilkies syndrome is an interesting and infrequent cause of small bowel obstruction. Need awareness prevention research on superior mesenteric. Wilkies syndrome is a rare cause of duodenal obstruction that can easily be. Exploratory laparotomy, performed when conservative management failed, revealed compression of the third part of duodenum by a shortened ligament of trietz and dense peritoneal bands near the third part of duodenum. Wilkie s syndrome is also known as superior mesenteric artery syndrome, cast syndrome, mesenteric root syndrome and chronic duodenal ileus 2. Superior mesenteric artery syndrome wikimedia commons. Wilkie s syndrome has many synonyms which include acute gastroduodenal ob struction, arteriomesenteric duodenal com pression and superior mesenteric artery syndrome. Sma syndrome is also known as wilkie s syndrome, cast syndrome, mesenteric root syndrome, chronic duodenal ileus and intermittent arteriomesenteric occlusion. The patients are still symptomfree with patent anastomosis on gastrointestinal radiographic control at 24 and 6 months, respectively, following their operation.
Links to pubmed are also available for selected references. Superior mesenteric artery syndrome radiology reference. After treatment the vomits and weight loss, to some extent, were. Wilkies syndrome or superior mesenteric artery syndrome. The superior mesenteric artery syndrome also known as wilkie s syndrome or as arteriomesenteric obstruction of the duodenumis a rare condition of upper intestinal obstruction in which the third. The patient continued to have normal renal function and she was managed conservatively. Adobe acrobat reader dc download free pdf viewer for. Media in category superior mesenteric artery syndrome. Clinical and nutritional outcomes in children with idiopathic superior mesenteric artery syndrome. Wilkies syndrome causing persistent vomiting postcolectomy.
Patient with both wilkie syndrome and nutcracker syndrome. The treatment regimen begins with conservative, medical management to provide relief from the symptoms of obstruction. United states court of appeals for the federal circuit. All structured data from the file and property namespaces is available. He had previously been treated for gastritis, but his symptoms persisted. The syndrome is caused by compression of the third part of the duodenum in the angle between the aorta and the superior mesenteric artery.
A 29yearold female was transferred to our hospital for management of duodenal obstruction. Download free adobe acrobat reader dc software for your windows, mac os and android devices to view, print, and comment on pdf documents. If a presumed condition is diagnosed in a veteran in a certain group, they can be awarded disability compensation. This is a temporary file and hence do not link it from a website, instead link the url of this page if you wish to link the pdf file. Chronic vomiting and high ileus caused by intermittent duodenal compression between the aorta and superior mesenteric artery.
Diagnostic value of diminished aortomesenteric distance amd. Sma syndrome also known as arteriomesenteric duodenal compression, chronic duodenal ileus, the cast syndrome, and wilkie s syndrome was first described in the 1800s by rokitansky. Wilkie artery the right colic artery when it occasionally crosses the duodenum. Hence, all cases of mental retardation without obvious cause should be tested for fragile x syndrome, it is often the only way to identify fragile x syndrome cases. It is characterised by the compression of the duodenum in its. The superior mesenteric artery syndromealso known as wilkies syndrome or as arteriomesenteric obstruction of the duodenumis a rare condition of upper intestinal obstruction in which the third. Aug 16, 2011 sma syndrome is also known as wilkies syndrome, cast syndrome, mesenteric root syndrome, chronic duodenal ileus and intermittent arteriomesenteric occlusion. Wilkie, saskatchewan, a town in canada wilkie electoral district wilkie airport, an abandoned aerodrome located adjacent to wilkie, saskatchewan, canada. Get a printable copy pdf file of the complete article 785k, or click on a. Wilkie general counsel, united states department of veterans affairs, washington, dc. This is because of the unique circumstances of a specific veterans military service. It is distinct from nutcracker syndrome, which is the entrapment of the left renal vein between the aa and the sma, although it is possible to be diagnosed with both conditions.
The diagnostic challenges of identifying vascular constriction between the aorta and superior mesenteric artery have been answered by advances in the field of computed tomography. 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. This is usually associated with conditions that cause significant weight loss, such as anorexia nervosa, malabsorption, or hypercatabolic states such as burns, major surgery, severe injuries, or malignancies. Superior mesenteric artery sma syndrome also known as wilkie s syndrome is a rare cause of upper gastrointestinal obstruction. Court of appeals for the federal circuit recently held that va can award disability benefits for pain related to military service. Superior mesenteric artery syndrome smas, wilkie syndrome or cast syndrome is a rare condition that usually presents with symptoms of mid to upper gastrointestinal obstruction due to the compression of the duodenum between the abdominal aorta, posteriorly, and the superior mesenteric artery, anteriorly.
It is characterised by the compression of the duodenum in its third portion due to a narrowing of the space between the superior mesenteric artery and the aorta. The reduced angle compresses the structures situated between the aorta and the superior mesenteric artery, such as the duodenum and left renal vein. Wilkies syndrome, or superior mesenteric artery syndrome smas, is a rare clinical condition characterized by partial or complete duodenal obstruction 1, 2. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis.
Va presumes that certain disabilities were caused by military service. Prolonged gastroparesis after corrective surgery for wilkies. In a supplemental report, the examiner stated there was pathology to no render a diagnosis on saunderss condition, and noted that the theory of causation was based on the chronology of events nduring sau derss service. Full text full text is available as a scanned copy of the original print version. Wilkie disease partial or complete block of the superior mesenteric artery. Superior mesenteric artery sma syndrome is a rare acquired disorder in which acute angulation of sma causes compression of the third part of the duodenum between the sma and the aorta, leading to obstruction.
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