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Instructive Cases

Superior Mesenteric Artery Stenosis Due to Disseminated Tuberculosis in a Pediatric Patient

Martinson, James MD*; Tronetti, Tara*; Goel, Nidhi MD, MHS; Sarkar, Rajabrata MD, PhD*; Strauch, Eric MD*; Lumpkins, Kimberly MD*

Author Information
The Pediatric Infectious Disease Journal: December 2021 - Volume 40 - Issue 12 - p e501-e503
doi: 10.1097/INF.0000000000003268
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Abstract

Pediatric splanchnic vessel stenosis is very rare, and typically results from a congenital aortic anomaly such as abdominal aortic coarctation or in conjunction with renal artery stenosis.1,2 Although vasculitis of the splanchnic vessels resulting in stenosis is an uncommon but well described condition in adults, there are only a few reports in the pediatric literature.2–7 We present a pediatric patient with superior mesenteric artery (SMA) stenosis due to disseminated Mycobacterium tuberculosis.

REPORT

An 8-year-old woman presented to an outside hospital with right neck swelling and generalized abdominal pain. She was transferred to our institution after the discovery of SMA stenosis on abdominal ultrasound (Fig. 1). She reported a 2-month history of fevers, night sweats, rash and unintentional weight loss down to 20.9 kg (fifth percentile for age) with decreased appetite. There was no history of food fear. She had immigrated from The Gambia to the USA 4 years prior. On examination, she had a 3.3 cm palpable right supraclavicular lymph node. She had scattered papules on the face and trunk that were nonerythematous but with scabbing and umbilication. Laboratory data were pertinent for microcytic anemia and an elevated erythrocyte sedimentation rate but negative for Epstein-Barr virus, HIV, hepatitis B and cytomegalovirus. Complement levels were within normal limits, and antinuclear antibody and antineutrophil cytoplasmic antibodies were negative. Chest radiograph was significant only for paratracheal lymphadenopathy; no parenchymal lung disease was identified. CT of the chest (performed after diagnosis, for evaluation of vascular involvement) showed patchy opacities in the right upper and lower lobes.

FIGURE 1.
FIGURE 1.:
Abdominal US demonstrating focal narrowing and wall thickening of the SMA. SMA indicates superior mesenteric artery.

Focal narrowing with associated wall thickening of the proximal SMA starting 4 mm from its origin and extending 1 cm with poststenotic fusiform dilation was seen on abdominopelvic CT. No other vascular involvement was identified on chest or neck imaging. A mesenteric duplex demonstrated greater than 70% stenosis from a periarterial mass extending from 1 cm beyond the artery origin to 3–4 cm distally, with peak systolic velocities in the proximal and middle SMA measured at 290 cm/s and 401 cm/s, respectively (Table 1). The lymph node pathology demonstrated necrotizing granulomatosis lymphadenitis, and her skin punch biopsy demonstrated perforating necrobiotic granuloma. Lymph node culture was eventually positive for M. tuberculosis (streptomycin resistant; sensitive to isoniazid, rifampin, ethambutol and pyrazinamide). Interferon gamma release assay Quantiferon gold was positive, and directly observed therapy with rifampin, isoniazid, pyrazinamide and ethambutol was initiated for disseminated TB for 6 months. Corticosteroids were considered but not initiated due to a lack of convincing evidence of benefit. At 3-month follow-up, she denied any abdominal discomfort or fear of food and had a good appetite with weight gain up to 24.9 kg (25th percentile for age). Magnetic resonance angiography showed slightly worsened SMA stricture with 1.4 cm in total length and persistent poststenotic dilation (Fig. 2). However, she was well-collateralized by her left colic and superior pancreaticoduodenal arteries providing sufficient inflow downstream of the lesion. The patient and her family were extensively educated about warning signs of mesenteric ischemia and annual magnetic resonance angiography surveillance imaging was initiated.

Table 1. - Mesenteric Duplex Results of the SMA
SMA Region Stenosis (%) PSV (cm/s) EDV (cm/s) RI
Proximal 60–99 290 50.7 0.83
Middle 60–99 401 69.6 0.83
Distal 1–59 52 14.3 0.73
EDV indicates end diastolic velocity; PSV, peak systolic velocity; RI, Resistive Index; SMA, superior mesenteric artery.

FIGURE 2.
FIGURE 2.:
MR angiography of the aorta, with stenosis of 1.4 cm increased from prior evaluation.

DISCUSSION

Few cases of SMA vasculitis in pediatric patients exist in the literature; even fewer have been observed as part of an active TB infection.2,4–6 While TB may cause mesenteric vasculitis from direct infection, this generally affects smaller distal vessels, leading to such pathology as bowel ulceration and peritoneal ascites that were not observed in our patient.8,9 On the other hand, Takayasu arteritis (TA) affecting large vessels has also been described following active TB, and may persist despite appropriate treatment.7,10,11 While the pathogenesis is uncertain, it has been hypothesized to be due to either cross-reactivity between a mycobacterial antigen and a human homolog, an overwhelming inflammatory response, or development of autoimmunity.12 Our patient’s vasculitis is consistent with Type IV TA (Table 2).13

Table 2. - Grading Scheme for Takayasu Arteritis Proposed at the 1994 Takayasu Conference10
Type of Takayasu Arteritis Vessel Involvement
Type I Branches of the aortic arch
Type IIa Ascending aorta, aortic arch and major branches
Type IIb Type IIa region plus thoracic descending aorta
Type III Thoracic descending aorta, abdominal aorta, renal arteries or a combination
Type IV Abdominal aorta, renal arteries or both
Type V Entire aorta and its branches

The persistent SMA stenosis after resolution of inflammation is likely due to fibrosis, the third stage of TA. In our patient’s case, she had sufficient inflow by collateralization to avoid symptoms. Data on SMA revascularization in children are limited and is predominantly limited to patients with congenital lesions such as abdominal aortic coarctation.1 Treatment is indicated in symptomatic cases as well as in asymptomatic children undergoing major aortic or renal reconstruction for coarctation or renal artery stenosis. Endovascular treatment of splanchnic stenosis is not recommended in either pediatric patients or in patients with TA due to a high recurrence rate.1,14 Open reconstruction has shown higher durable patency rates in pediatric patients, with secondary revascularization for stenosis or occlusion more frequently required in initially symptomatic patients and those younger than 5 years old at presentation.1 This patient is asymptomatic, with rapid weight gain after TB therapy and no history of food fear. As there are no other conditions requiring aortic reconstruction, intervention is not currently indicated. Regular surveillance imaging and careful monitoring are required indefinitely; she is at risk for both development of ischemia and for catastrophic outcomes following abdominal surgery if her intestinal blood supply is inadvertently disrupted.

CONCLUSION

We describe a pediatric patient with severe SMA stenosis due to disseminated TB. Systemic symptoms included weight loss, fever and lymphadenopathy. Although rare, large vessel involvement from TA can occur in this setting and should be considered in patients presenting with abdominal pain. Symptomatic SMA stenosis in the pediatric population requires open revascularization, while asymptomatic disease should be closely monitored.

REFERENCES

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Keywords:

pediatric; SMA stenosis; Takayasu arteritis; tuberculosis

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