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. To report on our experience with the management of sma syndrome, drawing the attention to its existence. Wilkie requested that the examiner provide a complete rationale for the diagnosis. Superior mesenteric artery sma syndrome differential diagnoses. For additional information, please read the supplement distribution policy. The pain they are feeling, and the atmosphere of a health care setting, can heighten this feeling of anxiety. Wilkie syndrome, also referred as superior mesenteric artery syndrome, is an unusual cause of a proximal small bowel obstruction.
Wilkie artery the right colic artery when it occasionally crosses the duodenum. Minimally invasive duodenojejunostomy for superior mesenteric. This prospective registry aims to describe demographic. Herein, we presented a patient with wilkies syndrome and discussed the diagnostic difficulties and surgical treatment options.
Chronic vomiting and high ileus caused by intermittent duodenal compression between the aorta and superior mesenteric artery. This article analyzes 178 published studies in the ws literature covering the following areas. Wilkies syndrome is due to compression of 3rd part of duodenum d3 between abdominal aorta ao and superior mesenteric artery sma 1. 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 it should not be confused with nutcracker syndrome which. Minimally invasive duodenojejunostomy for superior. Diagnostic value of diminished aortomesenteric distance amd. Simpsongolabibehmel syndrome is a condition that affects many parts of the body and occurs primarily in males. We report a case of yearold female who was diagnosed with sma syndrome. Williams syndrome many patients with williams syndrome have high levels of anxiety. Wilkie syndrome definition of wilkie syndrome by medical.
Simpsongolabibehmel syndrome genetics home reference. Wilkie syndrome and nutcracker syndrome are rare compression syndromes. Diagnostic value of diminished aortomesenteric distance. 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. Aug 12, 2015 superior mesenteric artery sma syndrome is a rare condition of duodenal obstruction, caused by the overlying sma. Loss of fatty tissue as a result of a variety of debilitating conditions is believed to be the etiologic factor causing the acute. The syndrome is caused by compression of the third part of the duodenum in the angle between the aorta and the superior mesenteric artery. See more ideas about superior mesenteric artery, superior mesenteric artery syndrome and interventional radiology. Superior mesenteric artery sma syndrome also known as wilkie syndrome is an 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 of proximal bowel.
The initial management should include gastric andduodenal decompression, treatment of fluid and electrolyte imbalance as well as reversal of weight loss using enteral and parenteral feeding. Genomic screening of fibroblast growthfactor receptor 2. 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. Wilkies syndrome is also known as superior mesenteric artery syndrome, cast syndrome, mesenteric root syndrome and chronic duodenal ileus 2. Specifically, this syndrome affects the first branchial or pharyngeal arch, which is the precursor of the maxilla and mandible.
Association of gastrointestinal motility disorders agmd. This results in chronic, intermittent, or acute complete or partial duodenal obstruction. The current definition of ws was agreed by the williams syndrome guideline development committee at the williams syndrome management consensus meeting held in manchester in may 2009. Melver failed to show that the board clearly erred in its weighing of this evidence. 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, superior mesenteric artery syndrome, intestinal obstruction, weight loss introduction superior mesenteric artery sma syndrome, also called wilkies syndrome or cast syndrome, is a rare disorder in which acute angulation of the sma causes compression of the third part of the duodenum between the sma and the aorta.
Patient with both wilkie syndrome and nutcracker syndrome. The causes of gi symptoms in adults with williams syndrome mirror those seen in the general population. Wilkie disease partial or complete block of the superior mesenteric artery. Pdf merge combine pdf files free tool to merge pdf online. Genomic screening of fibroblast growthfactor receptor 2 reveals a wide spectrum of mutations in patients with syndromic craniosynostosis shihhsin kan,1, navaratnam elanko,1, david johnson,1,2 laura cornejoroldan,3 jackie cook,4 elsa w. Once you merge pdfs, you can send them directly to your email or download the file to our computer and view. It is defined by a decrease of aortomesenteric distance. Printed versions of the nih curriculum supplements are available to qualified people free of charge. Wilkies syndrome, arteriomesenteric duodenal compression syndrome, the cast syndrome and chronic duodenal ileus.
Herein, we presented a patient with wilkie s syndrome and discussed the diagnostic difficulties and surgical treatment options. Definition of stress the word stress is derived from latin and was used in the english language as early as the 17th century to mean hardships, straits, adversity or affliction cited in hinkle, 1971. The condition is named for the areas of the body that are typically affected. Sma syndrome is also known as wilkies syndrome, cast syndrome, mesenteric root syndrome, chronic duodenal ileus and intermittent arteriomesenteric occlusion. Its manifestations are due to compression of the third portion of. It is distinct from nutcracker syndrome, which is the entrapment of the left renal vein between the aa and the sma. However, i need to embed an actual file inside the pdf in certain arias, after clicking on an image or. 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.
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. Chronic vomiting and high ileus caused by intermittent duodenal compression between the aorta. In a narrow and confined anatomic space, the otherwise morphologically normal vein becomes entrapped between rigid or semirigid surfaces, usually related to an underlying anatomic abnormality 2,3. Apr 17, 2008 wilkies syndrome, a rare cause of intestinal obstruction, is related to anatomical and mechanical factors associated with the reduction of retroperitoneal fat padding. The diagnosis and treatment of wilkie syndrome is controversial. Background superior mesenteric artery syndrome smas is a rare cause of duodenal obstruction, resulting from the compres sion of the duodenum between superior mesenteric artery and aorta. Superior mesenteric artery syndrome radiology reference. United states court of appeals for the federal circuit. Venous compression syndromes are caused by extrinsic mass effect on a vein by adjacent structures such as arteries, bones, muscles, or ligaments. This condition is classified as an overgrowth syndrome, which means that affected infants are considerably larger than normal at birth macrosomia and continue to grow and gain weight at an unusual rate.
Sma syndrome is also known as wilkie s syndrome, cast syndrome, mesenteric root syndrome, chronic duodenal ileus and intermittent arteriomesenteric occlusion. 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. Prolonged gastroparesis after corrective surgery for wilkies. Links to pubmed are also available for selected references. Sma syndrome also known as arteriomesenteric duodenal compression, chronic duodenal ileus, the cast syndrome, and wilkies syndrome was first described in the 1800s by rokitansky. Need awareness prevention research on superior mesenteric. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. 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. Superior mesenteric artery syndrome genetic and rare. Wilkie s syndrome is also known as superior mesenteric artery syndrome, cast syndrome, mesenteric root syndrome and chronic duodenal ileus 2. Wilkie syndrome or superior mesenteric artery syndrome is a rare cause of duodenal obstruction in pediatric and adult age groups. Superior mesenteric artery sma syndrome which is also known as wilkies syndrome, is a rare cause of upper gastrointestinal obstruction. Wilkies syndrome, a rare cause of intestinal obstruction, is related to anatomical and mechanical factors associated with the reduction of retroperitoneal fat padding. 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.
Read more about symptoms, diagnosis, treatment, complications, causes. The veterans court, however, determined that the board took account of the veterans reports of pain as part of its analysis and that mr. Sma syndrome is estimated to have a mortality rate of 1 in 3. Craniofrontonasal syndrome is a rare condition characterized by the premature closure of certain bones of the skull craniosynostosis during development, which affects the shape of the head and face. The cause of this syndrome is not known, but there are several factors in common. Wilkies syndrome or superior mesenteric artery syndrome. Sun anesthesiology and perioperative medicine, georgia regents university, augusta, ga 30907, usa email. Once files have been uploaded to our system, change the order of your pdf documents. Since that time many concepts, models and definitions of stress have emerged eisdorfer and wilkie, 1978. Note united states court of appeals for the federal circuit. The causes of gi symptoms in adults with williams syndrome mirror those seen in.
Rearrange individual pages or entire files in the desired order. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. For language access assistance, contact the ncats public information officer. Dec 31, 2018 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. Superior mesenteric artery sma syndrome is a rare condition of duodenal obstruction, caused by the overlying sma. Superior mesenteric artery sma syndrome, also named as wilkies syndrome ws, is a rare entity caused by the obstruction of 3rd proportion of the duodenum between sma and aorta. If you have problems viewing pdf files, download the latest version of adobe reader. Embedding pdf files documents inside a adobe acrobat pdf i am trying to find a way to embed a pdf document into an existing adobe acrobat x pdf. Williams syndrome is a rare condition, that occurs in 1 in 20,000 births. Wilkie s syndrome, a rare cause of intestinal obstruction, is related to anatomical and mechanical factors associated with the reduction of retroperitoneal fat padding. Crouzon syndrome is an autosomal dominant genetic disorder known as a branchial arch syndrome. Get a printable copy pdf file of the complete article 785k, or click on a page image below to browse page by page.