|Year : 2020 | Volume
| Issue : 1 | Page : 88-90
Wilkie's syndrome - An unusual presentation in an elderly female
Lakshmi Venkata Simhachalam Kutikuppala1, MV Sathvika2, T Uma Maheswara Rao2, Hemanth Kumar Chowdary2
1 Final MBBS Part-1, Konaseema Institute of Medical Sciences and Research Foundation (KIMS&RF), Amalapuram, Andhra Pradesh, India
2 Department of General Surgery, Konaseema Institute of Medical Sciences and Research Foundation (KIMS&RF), Amalapuram, Andhra Pradesh, India
|Date of Submission||09-Oct-2019|
|Date of Decision||24-Oct-2019|
|Date of Acceptance||11-Jan-2020|
|Date of Web Publication||08-Jul-2020|
M V Sathvika
Final MBBS Part-2, 148 Batch, Konaseema Institute of Medical Sciences and Research Foundation, Chaitanya Nagar, NH-216, East Godavari District, Amalapuram - 533 201, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
Wilkie's syndrome, also called as superior mesenteric artery syndrome (SMAS), is an extremely uncommon condition, which is evident in only 0.1%–0.3% of the population. This is a case of a 55-year-old female who presented to our hospital complaining of abdominal discomfort for 20 days, vomiting of two episodes per day for 20 days, constipation for 20 days, decreased appetite for 20 days, and significant weight loss. After further examination and investigations, it was diagnosed as SMAS and managed surgically through duodeno-jejunostomy followed by duodeno-jejunal and jejuno-jejunal anastomosis. She was later followed through conservative management and discharged from the hospital in a stable condition.
Keywords: Abdominal discomfort, appetite, duodeno-jejunostomy, superior mesenteric artery, vomiting
|How to cite this article:|
Simhachalam Kutikuppala LV, Sathvika M V, Maheswara Rao T U, Chowdary HK. Wilkie's syndrome - An unusual presentation in an elderly female. BLDE Univ J Health Sci 2020;5:88-90
|How to cite this URL:|
Simhachalam Kutikuppala LV, Sathvika M V, Maheswara Rao T U, Chowdary HK. Wilkie's syndrome - An unusual presentation in an elderly female. BLDE Univ J Health Sci [serial online] 2020 [cited 2020 Oct 24];5:88-90. Available from: https://www.bldeujournalhs.in/text.asp?2020/5/1/88/289201
| Introduction|| |
Superior mesenteric artery Syndrome (SMAS), also called Wilkie's syndrome, is an very rare condition that is evident in only 0.1%–0.3% of the population. It wasfirst explained by Von Rokitansky in the year 1861, who suggested obstruction of the third part of the duodenum due to arterio-mesenteric compression as a cause for Wilkie's syndrome. The aortomesenteric angle and aortomesenteric distance parameters are reduced in SMAS with values of 6°–15° and 2–8 mm. SMAS diagnosis is mostly based on clinical symptoms and radiological evidences. Conservative treatment is generally unsuccessful, and thus, surgical treatment is the only accepted way of treating a case of Wilkie's syndrome. Surgical success rate is 90%, and a total of nearly 500 cases are observed worldwide.
| Case Report|| |
An Indian female aged 55 years presented to the general surgery outpatient department of our hospital, complaining of epigastric pain, nausea, vomiting, constipation, and weight loss for about 20 days [Figure 1]. This was an acute presentation with pain worsening after food intake and with supine position. An episode of vomiting consisting undigested food usually occurred about 3 h after the food intake. Her prior medical history was statistically insignificant.
|Figure 1: Showing image of this elderly female Wilkie's syndrome patient|
Click here to view
She was found highly emaciated due to sudden weight loss on general examination. All her vitals were within the normal range. However, she had a distended abdomen and fullness over the epigastrium. She underwent an upper endoscopy recently, which revealed gastric stasis. Abdominal ultrasonography showed a reduced angle between the abdominal aorta and superior mesenteric artery. Upper GI contrast study disclosed enlarged stomach with hampered gastric emptying and lagging of the contrast at third part of the duodenum. Computed tomography of the abdomen established severe enlargement of the stomach and proximal parts of the duodenum with a narrowing at the third portion of the duodenum between the abdominal aorta and superior mesenteric artery, with a decreased angle (10°) and reduced distance (3–4 mm) between these vessels [Figure 2]. All these findings suggested an aortomesenteric clamp. Based on all the above findings, a final diagnosis of Wilkie's syndrome was made. This condition might be triggered by sudden weight loss due to poor diet history. Sudden weight loss causes emaciation of fat around duodenum that narrows the angle between superior mesenteric artery and aorta. This may cause compression of duodenum leading to duodenal obstruction.
|Figure 2: Showing image of contrast-enhanced computed tomography abdomen with narrowed aorto-superior mesenteric artery angle|
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We opted conservative modality of treatment initially that showed good improvement in patient's nutrition status. As symptoms still persisted, we had to adopt surgical treatment then. Due to temporary problem with laparoscopy equipment and acute condition of the patient, our surgical approach was open duodeno-jejunostomy, followed by duodeno-jejunal and jejuno-jejunal anastomosis. Under general anesthesia, midline incision from xiphisternum to below the umbilicus was made exposing the abdominal structures. Duodeno-jejunostomy, followed by duodeno-jejunal and jejuno-jejunal anastomosis was performed. Postsurgically, the patient was stable and shifted to the ward and put on conservative management and nil by mouth till the 4th day. After this, greenish discharge started from the wound site and anastomotic leak was confirmed. Organism isolated from the leak was Escherichia More Details coli (pus culture sensitivity) and treated specifically. Leak from the wound site decreased by 12th postoperative day. Secondary suturing was done on 16th postoperative day. The patient was managed conservatively at the hospital and discharged after attaining stable condition. The patient was sent home from the hospital following liquid diet and restoration of digestion for 2 weeks. She was advised to continue conservative medication for another 2 weeks and asked to revisit hospital. A regular follow-up was done for 12 weeks. Later, she gained weight and remained asymptomatic.
| Discussion|| |
SMAS is a rare condition, whose diagnosis requires a high index of suspicion. Duodenojejunostomy appears to be superior to gastrojejenostomy (Strong's operation). Imaging techniques (mainly contrast) are more reliable than clinical (examination and symptomatology) basis for the diagnosis of SMAS. Malnutrition and weight loss should also be addressed (ruled out) adequately for definitive and easier diagnosis. A laparoscopic duodenojejunostomy with reduced morbidity and mortality than an open surgery wasfirst described by Gersin and Heniford in 1998. This case report stresses that a minimally invasive surgery may be a comparatively easy approach to a case of Wilkie's syndrome. However, patient's condition, availability of essential resources before the surgery will also play a key role in definitive treatment of SMAS.
The authors wish to thank the dean, principal, and head of the department of general surgery of the institution for all the support and help rendered. We are very much thankful to the subject of this study, without whom this study could not be carried out.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
This study was finacially supported by the Department of General Surgery, Konaseema Institute of Medical Sciences and Research Foundation, Amalapuram, Andhra Pradesh, India.
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]