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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 6  |  Issue : 1  |  Page : 51-53

Wilkie's syndrome with duodenojejunal diverticulosis: A rare combination causing duodenal obstruction


Institute of Surgical Gastroenterology and Liver Transplant, Center for GI Bleed, Division of HPB Diseases, Govt. Stanley Medical College, Chennai, Tamil Nadu, India

Date of Web Publication28-Jul-2016

Correspondence Address:
Dr. Saravanan Janakiraman
Institute of Surgical Gastroenterology and Liver Transplant, Center for GI Bleed, Division of HPB Diseases, Govt Stanley Medical College, Old Jail Road, Chennai, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-9596.187201

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  Abstract 

Wilkie's syndrome is an unusual cause of proximal intestinal obstruction. It is a clinical entity characterized by compression of the third or transverse portion of the duodenum between the aorta and superior mesenteric artery (SMA). This results in chronic, intermittent, or acute complete or partial duodenal obstruction. We present the case of a 40-year-old lady who presented with postprandial abdominal pain at the epigastric region, colic type, without radiation accompanied by nausea, postprandial vomiting, and weight loss. She was evaluated and diagnosed with SMA syndrome with duodenojejunal diverticulosis. She was taken for surgery due to chronic malnutrition that did not improve with conservative management. Duodenojejunostomy with transgastric feeding jejunostomy was done. To alleviate postoperative refractive gastroparesis, kinking, and angulation as is our previous few cases, transgastric feeding jejunostomy was done in contrast to modified Witzel's technique. The patient was free of symptoms and recurrence during follow-up. Wilkie's syndrome is an unusual cause of intestinal obstruction. Prompt diagnosis and appropriate surgical intervention are associated with good outcome.

Keywords: Arteriomesenteric duodenal compression syndrome, cast syndrome, chronic duodenal ileus, duodenojejunal diverticulosis, Wilkie's syndrome


How to cite this article:
Janakiraman S, Sathyanesan J, Palaniypaan R. Wilkie's syndrome with duodenojejunal diverticulosis: A rare combination causing duodenal obstruction. Arch Int Surg 2016;6:51-3

How to cite this URL:
Janakiraman S, Sathyanesan J, Palaniypaan R. Wilkie's syndrome with duodenojejunal diverticulosis: A rare combination causing duodenal obstruction. Arch Int Surg [serial online] 2016 [cited 2021 Sep 16];6:51-3. Available from: https://www.archintsurg.org/text.asp?2016/6/1/51/187201


  Introduction Top


Superior mesenteric artery (SMA) syndrome was first described in 1861 by Von Rokitansky [1] who proposed that its cause was obstruction of the third part of the duodenum as a result of arteriomesenteric compression. It was later popularized by Wilkie.[2] The syndrome is characterized by compression of the third portion of the duodenum due to narrowing of the space between the SMA and aorta and is primarily attributed to loss of the intervening mesenteric fat pad. It has been referred to by a variety of other names including cast syndrome, Wilkie's syndrome, arteriomesenteric duodenal obstruction, and chronic duodenal ileus.[3] Despite the fact that about 400 cases are described in the English language, existence of duodenojejunal diverticulosis makes it the second case to be reported. Furthermore, the diagnosis may be confused with other anatomic- or motility-related causes of duodenal obstruction.


  Case Report Top


A 40-year-old housewife weighing 29 kg presented to our department with complaints of postprandial vomiting for 1 year. Vomiting was bilious, 30-60 min after food intake with increased frequency for past 45 days. She also had colicky postprandial abdominal pain, recurrent abdominal distension, ball rolling movements, and weight loss despite good appetite. There was no history of altered bowel habits, hematemesis, melena, or jaundice. She had no comorbidity. Examination revealed an emaciated woman with mild epigastric fullness. Her laboratory parameters of complete hemogram, liver function, renal function, and viral markers were unremarkable. Upper gastrointestinal (UGI) endoscopy revealed D1 and D2 distensions, narrowing at D3, and duodenal diverticulosis. Contrast-enhanced computed tomography (CECT) revealed reduced aortomesentric distance of 7 mm [Figure 1]. Aortomesentric angle also reduced to 10° with compression of D3 segment of the duodenum between the aorta and SMA [Figure 2] with distention of the proximal duodenum and stomach. The left renal vein was also compressed between the aorta and SMA. A diagnosis of SMA syndrome was made. Due to chronic malnutrition with increased frequency of symptoms, she underwent duoduenojejusotmy bypass procedure. The first and second parts of the duodenum were grossly dilated and hypertrophied. The dilated duodenum abruptly ended at the third part of the duodenum where the SMA crossed the duodenum. Multiple duodenojejunal diverticulosis was found with the largest measuring 3 cm [Figure 3]. SMA was thickened and found to be compressing D3. These findings confirmed the diagnosis of SMA syndrome and side-to-side duodenojejunostomy in two layers was performed along with transgastric feeding jejunostomy [Figure 4]. In our patient, transgastric feeding gastrostomy was done in contrast to modified Witzel's technique. This has avoided postoperative kinking and angulation and gastroparesis. Hence, her postoperative course was uneventful. She had complete relief of the symptoms without any recurrence in the follow-up period of 6 months. She gained 12 kg in weight on follow-up at the 6th month and remained asymptomatic.
Figure 1: CECT showing reduced aortomesentric distance

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Figure 2: CECT showing reduced aortomesentric angle

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Figure 3: Multiple duodenojejunal diverticulosis

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Figure 4: Duodenojejunal anastomosis

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  Discussion Top


SMA syndrome is a rare but well-recognized clinical entity characterized by compression of the third portion of the duodenum between the aorta and the SMA. It was first described by Von Rokitansky in 1861.[1] The reported prevalence in the general population varies between 0.013% and 0.78%.[4] SMA usually forms an angle of approximately 45° (range: 38-56°) with the abdominal aorta. The third part of the duodenum crosses caudal to the origin of the SMA, coursing between the SMA and aorta. Any factor that sharply narrows the aortomesenteric angle to approximately 6-25° can cause entrapment and compression of the third part of the duodenum resulting in SMA syndrome.[5],[6] In the majority of patients, the normal angle between the SMA and the aorta is between 38° and 65° due, in part, to the mesenteric fat pad. This angle correlates with body mass index.[6] The aortomesenteric distance is normally 10-28 mm. Wilkie's syndrome tends to affect young female adults aged 17-39 years. The main symptoms of Wilkie's syndrome are epigastric discomfort and pain followed by vomiting, which as the disease progresses become more severe, frequent, and causes fluid and electrolyte disturbances with weight loss [7] as seen in our patient. An acute presentation is uncommon.[8] The diagnosis is confirmed by radiological imaging. Conservative management is the rule for acute cases. It consists of frequent meals of fluid or blenderized food, diluted to the consistency of soup. Total parenteral nutrition is also used but eventually 50-70% of all cases come to surgery. Surgery is indicated for chronic cases and in case of failure of conservative management. The operative options include duodenojejunostomy, section of the ligament of Treitz and relocation of the duodenojejunal junction (Strong's procedure), gastrojejunostomy, and duodenal anterior replacement.[7] Duodenojejunostomy is the procedure of choice and is effective in 90% of the patients. Successful laparoscopic duodenojejunal bypass has also been described. Although experience is limited, it offers a less invasive surgical option.[9],[10] Gastroparesis after surgery is a frequently encountered problem related to gastric atony and duodenal atony. Although the presence of such persistent symptoms has been described in the literature, there is little information on their management. In our patient, transgastric feeding gastrostomy was done in contrast to modified Witzel's technique. This particular attention has avoided kinking and angulation in addition to the postoperative gastroparesis. Further powered studies are needed to address this technique in alleviating the postoperative refractive gastroparesis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Von Rokitansky C. Lehrburch der Patologische Anatomie. 1st ed. Braumulle: Wien; 1861. p. 187.  Back to cited text no. 1
    
2.
Wilkie, DP. Chronic duodenal ileus. Br J Surg 1921;9:204.  Back to cited text no. 2
    
3.
Dorph MH. The cast syndrome; review of the literature and report of a case. N Engl J Med 1950;243:440-2.  Back to cited text no. 3
[PUBMED]    
4.
Welsch T, Büchler MW, Kienle P. Recalling superior mesenteric artery syndrome. Dig Surg 2007;24:149-56.  Back to cited text no. 4
    
5.
Kyslan K, Barla J, Kyslan K, Stanislayová M. Superior mesenteric artery (SMAS/AMS) syndrome and its management. Rozhl Chir 2008;87:255-8.  Back to cited text no. 5
    
6.
Ozkurt H, Cenker MM, Bas N, Erturk SM, Basak M. Measurement of the distance and angle between the aorta and superior mesenteric artery: Normal values in different BMI categories. Surg Radiol Anat 2007;29:595-9.  Back to cited text no. 6
    
7.
Akin JT, Gray SW, Skandalakis JE. Vascular compression of the duodenum: Presentation of 10 cases and review of the literature. Surgery 1976;79:515-22.  Back to cited text no. 7
    
8.
Kaushik R, Attri AK. Acute superior mesenteric artery syndrome. Indian Pediatr 2003;40:1014-5.  Back to cited text no. 8
[PUBMED]    
9.
Palanivelu C, Rangarajan M, Senthilkumar R, Parthasarathi R, Jani K. Laparoscopic duodenojejunostomy for superior mesenteric artery syndrome. JSLS 2006;10:531-4.  Back to cited text no. 9
    
10.
Bermas H, Fenoglio ME. Laparoscopic management of superior mesenteric artery syndrome. JSLS 2003;7:151-3.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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