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 Table of Contents  
Year : 2022  |  Volume : 2  |  Issue : 4  |  Page : 217-220

Wilke's Syndrome: A Missed Diagnosis in Pulmonary Tuberculosis in an Adolescent

Department of Pediatrics, GGSMCH, Faridkot, Punjab, India

Date of Submission08-Jul-2022
Date of Decision28-Oct-2022
Date of Acceptance28-Oct-2022
Date of Web Publication29-Nov-2022

Correspondence Address:
Dr. Varun Kaul
Department of Pediatrics, GGSMCH, Faridkot, Punjab
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ipcares.ipcares_168_22

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Background: Superior mesenteric artery (SMA) syndrome or Wilke's syndrome is a rare, atypical cause of upper gastrointestinal tract obstruction. It occurs due to acute angulation between SMA and aorta causing compression of the third part of the duodenum inbetween them leading to proximal intestinal obstruction. Clinical Description: SMA syndrome occurs mostly in patients who have significant weight loss. It occurs mostly due to the loss of the retroperitoneal fat pad, which serves as a cushion for the duodenum protecting it from compression between the two arteries. Causes include prolonged supine bed rest, spine surgery, an unusual high insertion of ligament of Treitz, other causes leading to weight loss, and congenital anatomical defects. Symptoms include weight loss, bilious vomiting, pain abdomen, postprandial nausea, early satiety, and anorexia. We report a 14-year-old female, a known case of pulmonary tuberculosis, who developed pneumothorax followed by acute gastric dilatation superadded with septicemia and upper gastrointestinal tract obstructive symptoms during the hospital course. Management: Detailed history and radio imaging such as barium studies or contrast-enhanced computerized tomography abdomen are required for diagnosis. Treatment includes conservative management such as gastric decompression, maintenance of electrolytes, nutritional rehabilitation via nasojejunal feeding, orally or parenterally. Treatment aims to restore the mesenteric fat pad to increases the angle between the two arteries. If it fails, surgery needs to be planned wherein duodenojejunostomy can be performed. Conclusion: An increased index of suspicion should be kept for this rare syndrome in children, especially in cases associated with significant weight loss. This may lead to timely diagnosis and saving a precious life.

Keywords: Intestinal obstruction, superior mesenteric artery syndrome, weight loss

How to cite this article:
Arora N, Kaur G, Kaul V, Singh M. Wilke's Syndrome: A Missed Diagnosis in Pulmonary Tuberculosis in an Adolescent. Indian Pediatr Case Rep 2022;2:217-20

How to cite this URL:
Arora N, Kaur G, Kaul V, Singh M. Wilke's Syndrome: A Missed Diagnosis in Pulmonary Tuberculosis in an Adolescent. Indian Pediatr Case Rep [serial online] 2022 [cited 2023 Apr 2];2:217-20. Available from: http://www.ipcares.org/text.asp?2022/2/4/217/362239

Superior mesenteric artery (SMA) syndrome, also known as Wilke's Syndrome, is an atypical cause of upper gastrointestinal tract obstruction. Acute angulation between the SMA and the aorta causes compression of the third part of the duodenum in between, leading to proximal intestinal obstruction. The incidence of SMA in the general population is 0.013%–0.3%,[1] and it is rare in children. Usually, retroperitoneal fat serves as a cushion for the duodenum along with the surrounding lymphatic tissue and protects it from compression by the two arteries.[2] Thus, Wilke's syndrome occurs mostly in situations when there is significant weight loss. Causes include prolonged supine bed rest, spinal surgery, an unusual high insertion of the ligament of Treitz, along with a congenital anatomical defect involving embryonic duodenal rotation and fixation.

The obstruction can be acute (complete/partial) or chronic. Symptoms vary accordingly and include weight loss, bilious vomiting, pain abdomen, postprandial nausea, early satiety, and anorexia.[3] The severity of symptoms is mainly affected by the degree of compression, depending on the angle between the SMA and the aorta (aortomesenteric angle). These include dehydration, electrolyte imbalance, malnutrition (wasting), perforation, and peritonitis. Treatment includes attempts to cause weight gain and restore mesenteric fat pad. This can be done either orally or parentally. If this strategy fails, surgery involving duodenojejunostomy needs to be planned.[4]

Pediatricians are generally unaware of this condition in children with significant weight loss. We lost one precious life due to delayed diagnosis. Albeit earlier case reports of SMA from India have been after spinal surgery, renal tubular acidosis and Marfan syndrome but none with underlying tuberculosis (TB). The aim of presenting this case is to increase the index of suspicion to increase early diagnosis and timely management.

  Clinical Description Top

A 14-year-old girl was brought to our emergency with complaints of productive, purulent, non-blood-tinged cough for 5 days. This was followed by acute onset of difficulty in breathing for 36 h before presentation, which progressively increased. It was associated with chest pain that was stabbing in nature and got aggravated with movement and cough. There was a significant past history of prolonged cough, fever, and weight loss 9 months back earlier, for which she had been investigated and diagnosed with pulmonary TB. This had been on the basis of chest X-ray and sputum for cartridge-based nucleic acid amplification test (CBNAAT). The patient had been started on anti-TB therapy (ATT). At the time of admission, she was in the continuation phase of ATT (rifampicin, isoniazid, and ethambutol) as per standard protocol (National Tuberculosis Elimination Programme 2020). Drug compliance was satisfactory. The child's respiratory symptoms had resolved, but her appetite was still poor even after the past 8 months. Her diet had a prolonged daily deficit of 35% and 25% calories and proteins, respectively. Besides this, there was no past history of any trauma, or prolonged bed rest/immobilization. No other member of her family had TB (negative sputum CBNAAT and X-ray chest). The child was immunized as per her age and had received the BCG vaccine. She studied in class 8, was an average student and belonged to the upper-lower socioeconomic status.

At the presentation, the patient looked anxious and was oriented to time, place, and person. Her heart rate was 115/min, respiratory rate 38/min, and she had oxygen saturation of 91% in room air. Nasal flaring was evident, with no obvious chest retractions. Her peripheries were cold and peripheral pulses feeble, though the central pulses were palpable. The blood pressure was 114/76 mm of Hg (between 50th and 90th centiles, Indian Academy of Pediatrics [IAP] growth chart). Her body mass index (BMI) was 13.9 kg/m2 (<3rd centile for age, revised IAP growth chart of BMI for 5–18-year-old Indian children, 2015), indicative of severe thinness. There was no pallor, cyanosis, icterus, clubbing, or significant lymphadenopathy. The respiratory system examination revealed a shift of the trachea and apex beat to the left side, with decreased chest expansion on the right side. The percussion note was hyperresonant on the whole of the right side, more anteriorly than posteriorly, with decreased vocal fremitus. Liver dullness shifted downward. The air entry was markedly decreased on the right side with absent added sound (no wheeze, crepitations or whispering pectoriloquy) and absent vocal resonance. The abdomen was soft, nontender, and nondistended abdomen and there was no palpable organomegaly. The bowel sounds were present. The remaining systemic examination was normal. On the basis of history and examination, we kept a differential diagnosis of pulmonary TB (possibly drug resistant), complicated by a right-sided pneumothorax with septicemia and compensated shock. The patient was started on 100% oxygen with a partial rebreathing mask, and a normal saline intravenous (IV) bolus was administered (@10ml/kg). Investigations (routine blood tests, sepsis screen, chest X-ray, CBNAAT, and cultures) were planned accordingly.

Management and outcome

The patient's condition improved hemodynamically. A chest X-ray confirmed our clinical suspicion of right-sided pneumothorax, mediastinal shift to the left side, and multiple heterogeneous opacities bilaterally. An intercostal chest tube was inserted to resolve the pneumothorax. The hemogram showed hemoglobin of 11.2g/dl, white blood cell count of 13,900/mm3 with neutrophilic predominance (62%), and platelet count of 175,000/μL. The C reactive protein was elevated (68.31 mg/L). Serum sodium levels were 131 meq/L, Potassium 4.3 meq/L. The total serum bilirubin was 0.4 mg%, SGOT/SGPT 71, and 45 IU/L, respectively. Her blood urea and serum creatinine levels were 31 mg% and 0.6 mg%, respectively.

Treatment comprised IV fluids, IV antibiotics (ceftriaxone @75 mg/kg/day q 12 h and injection ampicillin and cloxacillin @ 200mg/kg/day of ampicillin base every 6 h) were given for 10 days. ATT was continued. There was gradual symptomatic relief in terms of her respiratory symptoms along with the radiological resolution of the pneumothorax. The gastric aspirate CBNAAT showed the presence of Mycobacterium tuberculosis sensitive to rifampicin. Hence, the ATT was continued. Nasogastric feeding was started on the 3rd day of admission. The patient developed acute hepatic dysfunction on day 15 of hospitalization (SGOT/SGPT increased to 176 IU/L and 237 IU/L, respectively, and serum bilirubin was 1.4 mg%), which was presumed to be ATT induced, since viral serology markers for hepatitis B, hepatitis C, and human immunodeficiency virus were negative. ATT was modified as per standard protocol for 2 weeks.

On the 24th day of hospitalization, the patient developed mild respiratory distress again. There was X-ray showed multiple heterogeneous opacities with no sign of pneumothorax, so IV antibiotics (amoxycillin and potassium clavulanate @ 100mg/kg/day of amoxycillin base every 8 hours) were started. The patient displayed loss of weight as evident from the loosening of clothes and increasing visible wasting. On day 33 of admission, the patient developed mild abdominal distension, bilious vomiting, and non-passage of stools over 3 days. The LFTs and serum potassium were normal. An abdominal X-ray showed a large gastric shadow [Figure 1]. She was kept nil per oral, and nasogastric tube drainage was started with fluid replacement. The pediatric surgery team was consulted who advised to the continuation of conservative management and a contrast-enhanced computerized tomography (CECT) of the abdomen. Injection Meropenem (@ 60mg/kg/day every 8 h) was added, suspecting nosocomial infection. While awaiting the report, the patient developed an acute episode of abdominal pain associated with a change in the nature of draining gastric aspirate from bilious to feculent, loss of consciousness (Glasgow Coma Scale total score 5 with E2V2M1), and peripheral circulatory failure. The patient was intubated, ventilated, and resuscitated as per standard protocol but could not be revived. The CECT report collected posthumously confirmed massive gastric dilatation [Figure 2] and also identified a decreased angle between the SMA and aorta, suggestive of SMA syndrome [Figure 3]. The family refused consent for performing an autopsy. The final diagnosis of pulmonary TB with severe thinness with SMA syndrome was made.
Figure 1: X-ray abdomen showing large gastric shadow

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Figure 2: CECT abdomen (coronal view) showing massive gastric dilatation. CECT: Contrast-enhanced computerized tomography

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Figure 3: CECT abdomen (sagittal view) showing decreased angle between the superior mesenteric artery and aorta. CECT: Contrast-enhanced computerized tomography

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  Discussion Top

The characteristic radiological features of SMA syndrome is the narrowing of the aorta-SMA angle to <20° (in this case, it was <17°), causing the aortomesenteric distance to decrease.[5] [Figure 3]. Here, the distance between the two arteries was <6 mm against the normal range of 8–12 mm.[6] Barium studies show dilatation of the first and second parts of the duodenum with the backflow of the barium proximal to obstruction, and which gets relieved in the prone or lateral positions.[4] Radio imaging-specific modalities such as CECT or magnetic resonance angiography (whichever is available) confirm the diagnosis. We performed the CECT abdomen due to the clinical condition of the patient, as the availability of the report is faster in our center.

Treatment of SMA syndrome includes conservative management like gastric decompression, maintenance of electrolyte balance, and nutritional rehabilitation, which can be done enterally via nasojejunal feeding (thus bypassing the obstruction) or parenterally, followed by oral feeds when the condition permits.[7],[8],[9] Positioning maneuvers like lying on the left side or assuming the knee-chest position can be tried during the feed. The ultimate goal is to increase the weight so that the pad of fat develops at the mesenteric root. If conservative management fails, surgery is considered.[4] This may be by duodenojejunostomy to relieve the obstruction. Gastro-jejunostomy was done previously, but there was a high incidence of complications like blind loop syndrome and recurrence. Strong's procedure, i.e., lysis of the ligament of Treitz with mobilization of the duodenum, can also be performed. However, this is associated with a failure rate of 25%.[5]

Acute massive gastric dilatation is a rare emergency that usually occurs in the setting of a closed-loop obstruction, rarely seen in chronic medical illnesses. It is usually managed conservatively, with only a few cases requiring surgical intervention. In our case, the patient was managed medically, suspecting acute gastric dilatation due to worsening sepsis since an electrolyte imbalance was ruled out.

SMA syndrome secondary to TB has been reported in a 59-year-old patient,[10] but there are no case reports in children or adolescents. Abdominal lymphadenopathy, mucosal edema, ulceration, bowel wall thickening, and scarring in the ileocecal region are usual in abdominal TB resulting in subacute or acute intestinal obstruction. These findings were not seen in our patient. In this patient, the risk factors for causing SMA syndrome were probably cachexia due to TB coupled with prolonged immobilization during her hospital stay. The prognosis depends upon how early the syndrome is suspected and diagnosed, with the mortality rate as high as one in three affected patients. Thus, an increased index of suspicion should be kept for this rare syndrome in children, especially in cases associated with significant weight loss. This may lead to timely diagnosis and saving a precious life.

Declaration of patient consent

The authors certify that they have obtained the appropriate consent from the parent. The legal guardian has given his consent for the images and other clinical information to be reported in the journal. The guardian understands that the name and initials will not be published, and due efforts have been made to conceal the same, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Ylinen P, Kinnunen J, Höckerstedt K. Superior mesenteric artery syndrome. A follow-up study of 16 operated patients. J Clin Gastroenterol 1989;11:386-91.  Back to cited text no. 1
Crowther MA, Webb PJ, Eyre-Brook IA. Superior mesenteric artery syndrome following surgery for scoliosis. Spine (Phila Pa 1976) 2002;27:E528-33.  Back to cited text no. 2
Baltazar U, Dunn J, Floresguerra C, et al. Superior mesenteric artery syndrome: An uncommon cause of intestinal obstruction. South Med J 2000;93:606-8.  Back to cited text no. 3
Mandarry M, Zhao L, Zhang C, et al. A comprehensive review of superior mesenteric artery syndrome. Eur Surg 2010;42:229-36.  Back to cited text no. 4
Merrett ND, Wilson RB, Cosman P, et al. Superior mesenteric artery syndrome: Diagnosis and treatment strategies. J Gastrointest Surg 2009;13:287-92.  Back to cited text no. 5
Jain R. Superior mesenteric artery syndrome. Curr Treat Options Gastroenterol 2007;10:24-7.  Back to cited text no. 6
Reckler JM, Bruck HM, Munster AM, et al. Superior mesenteric artery syndrome as a consequence of burn injury. J Trauma 1972;12:979-85.  Back to cited text no. 7
Tsirikos AI, Anakwe RE, Baker AD. Late presentation of superior mesenteric artery syndrome following scoliosis surgery: A case report. J Med Case Rep 2008;2:9.  Back to cited text no. 8
Welsch T, Büchler MW, Kienle P. Recalling superior mesenteric artery syndrome. Dig Surg 2007;24:149-56.  Back to cited text no. 9
Limaye CS, Karande SP, Aher SP, et al. Superior mesenteric artery syndrome secondary to tuberculosis induced cachexia. J Assoc Physicians India 2011;59:670-1.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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