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| CASE REPORT |
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| Year : 2023 | Volume
: 13
| Issue : 2 | Page : 89-92 |
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Tubercular aortoiliac aneurysm with challenging management
Mansi Shah1, Atul Kakar1, Atul Gogia1, Ambarish Satwik2
1 Department of Internal Medicine, Sir Ganga Ram Hospital, New Delhi, India
2 Department of Vascular and Endovascular Surgery, Sir Ganga Ram Hospital, New Delhi, India
| Date of Submission | 24-Feb-2023 |
| Date of Decision | 12-Mar-2023 |
| Date of Acceptance | 16-Mar-2023 |
| Date of Web Publication | 28-Apr-2023 |
Correspondence Address:
Dr, Atul Kakar
Medident Clinic, 31, South Patel Nagar, New Delhi - 110 008
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/cmrp.cmrp_39_23
A 38-year-old male was diagnosed with aortoiliac aneurysm while evaluating for new-onset hypertension. On further workup, the cause was identified as tubercular aortoiliac aneurysm. His aneurysm had stormy course and disseminated further while ongoing antitubercular therapy with multiple episodes of aneurysmal rupture and endovascular interventions. Management of this case was complicated with several other rarer entities, such as haemophagocytosis and thrombotic microangiopathy with disseminated intravascular coagulation resistant to steroids and plasmapheresis, within a span of few weeks. Moreover, first-line antitubercular therapy had to be regularly modified in view of emerging complications. While case reports for each individual entity exists in literature, this is the first case to the best of our knowledge where such varied complications were present in a patient of tubercular mycotic aneurysm.
Keywords: Disseminated intravascular coagulation, disseminated tuberculosis, extrapulmonary tuberculosis, haemophagocytosis, tubercular mycotic aneurysm
How to cite this article:
Shah M, Kakar A, Gogia A, Satwik A. Tubercular aortoiliac aneurysm with challenging management. Curr Med Res Pract 2023;13:89-92 |
| Introduction | |  |
Even in India, the country with world's highest burden of tuberculosis, mycotic aneurysm caused by tuberculosis remains a rare clinical entity. Constrictive lesions are prevalent in Indian cases of tubercular aortoarteritis compared to aneurysmal destruction noted in Western literature. This obliterative arteritis is hypothesised as a protective mechanism against dissemination caused by repeated infections in developing countries.[1] The incidence of tubercular aortic aneurysms is likely to increase with the increasing prevalence of drug-resistant tuberculosis and infection by atypical mycobacteria.[2] A high index of suspicion is required as patient can have varied clinical manifestations and carries grave prognosis unless diagnosed promptly and treated aggressively.
| Case Report | | |
A 38-year-old male experienced palpitations for 3 days and was diagnosed with hypertension. He gave a history of intermittent lower backache, not associated with any neurological deficit, for the past 6 months. There was no history of fever, weight loss, night sweats or Koch's contact. Routine investigations were unremarkable. Computed tomography (CT) of the abdomen and angiography revealed aortoiliac aneurysm with perivascular collection inseparable from the aneurysm and endovascular abdominal aortic aneurysmal repair (EVAR) was done for the suspected contained rupture of aneurysm. The sample of the aortic wall obtained from the access site for histopathologic evaluation showed no evidence of systemic vasculitis.
About 10 months later, the patient was brought to us in a drowsy state with a history of prolonged fever and significant weight loss. Routine blood chemistries and inflammatory markers were normal. Initial investigations for a case of pyrexia of unknown origins such as blood, urine, and stool cultures, COVID reverse transcription–polymerase chain reaction, COVID total and immunoglobulin G antibodies, spot tests for anti-HIV antibodies, anti-hepatitis C virus antibodies and Hepatitis B surface antigen, VDRL, Mantoux, QuantiFERON gold, Anti-nuclear antibodies, C3 and C4 levels were non-contributory. To investigate unexplained fever and weight loss, positron emission tomography was done, which was suggestive of widely disseminated tuberculosis. Thick, purulent and haemorrhagic fluid was aspirated from the para-aortic collection, which tested positive for TB-GeneXpert with no resistance to rifampicin.
Despite treatment with antitubercular agents for a week, he continued to develop high-grade fever spikes, his sensorium deteriorated further and he had to be intubated. Acid-fast bacilli (AFB) were detected by Zielh–Neilson staining from endotracheal tube secretions. Tubercular meningitis was suspected, for which CT head was done, which was normal. Consent for lumbar puncture was not given by the attendants. Diagnosis of secondary haemophagocytic lymphohistiocytosis was made in view of worsening bicytopenias, elevated S. Ferritin (1596 ng/mL) and soluble CD 25 levels (67.3 ng/mL), and confirmed by bone marrow biopsy, which showed multiple coalescing necrotising epithelioid granulomas along with evidence of haemophagocytosis. Platelet count stabilised eventually with steroid therapy.
About 2 weeks later, he developed sudden hypotension, which was attributed to the rupture of aneurysm. Resuscitative measures were ensued, and fenestrated endovascular aortic repair using surgeon-modified stent graft were placed in situ to maintain visceral flow [Figure 1]a and [Figure 1]b. He was haemodynamically stable post-operative for the following week. | Figure 1: Angiography. (a) Surgeon-modified device fenestrations for superior mesenteric artery (arrow). (b) Superior mesenteric artery was cannulated and stents were deployed (arrow)
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He suffered from fresh episodes of epistaxis with multiple petechiae over his trunk and limbs. Rifampicin was withheld. Laboratories showed recurrence of thrombocytopenia (14 thousand/microL) with giant platelets and schistocyte index of 2.7% on peripheral smear, deranged coagulation profile (prothrombin time 14 s, INR 1.4), raised D-dimer (1.68 μg/mL) and fibrinogen equivalent units (3.36 μg/mL). He was diagnosed to have thrombotic microangiopathy secondary to disseminated intravascular coagulation as per the International Society on Thrombosis and Haemostasis criteria. However, this thrombocytopenia was refractory to steroids and six cycles of plasmapheresis. Eventually, his platelet count stabilised with a course of intravenous immunoglobulins.
About 2 weeks later, the patient complained of a sudden diminution of vision from the left eye. Ethambutol was withheld, and fundoscopic evaluation demonstrated miliary choroidal tubercles involving the macula. He was treated with high-dose steroid pulse therapy.
He developed sudden-onset palpitations and dyspnoea, a week later. Cardiomyopathy was suspected based on T wave inversion in lead V2-V6, I and aVL in electrocardiogram, elevated cardiac biomarkers (NT proBNP >25,000 pg/mL, hsTropI 78.2 pg/mL) and global LV hypokinesia with an ejection fraction of 25% in two-dimensional echo. This cardiomyopathy was treated conservatively. Over the subsequent 2 weeks, he developed non-oliguric pre-renal acute kidney injury, as suggested by worsening renal function tests. Ultrasonography showed normal-sized kidneys. However, the Doppler study confirmed decreased flow in the right renal artery. He was treated conservatively and was discharged on request once stabilised haemodynamically.
An open procedure was attempted at another hospital about a month later as an attempt to treat the sudden new-onset rupture of his aneurysm, during which he succumbed.
| Discussion | | |
Diagnosing tubercular mycotic aneurysm, an extremely rare form of extrapulmonary tuberculosis, even in developing countries with a high burden of tuberculosis, is challenging as it can have presentation varying from constitutional symptoms, pyrexia of unknown origin to massive haemorrhage. Usually, it affects major vessels, such as the aorta, in middle-aged people with disseminated tuberculosis. Our patient had quite an unusual presentation of palpitations and had no features suggestive of Koch's in history or clinical examination. The perivascular collection masqueraded as contained rupture, and thus, the endovascular abdominal aortic repair was done. It was striking that the erythrocyte sedimentation rate was normal; Mantoux and QuantiFERON gold were negative even when he presented to the hospital after 10 months, while the imaging study demonstrated massive dissemination of tuberculosis. As clinical presentation varies, gross characteristics of aneurysm are undistinguishable and AFB staining is unlikely to be done unless there is a high index of suspicion. Diagnosis could not be made at presentation in a third of cases, according to a review.[3]
EVAR is being increasingly preferred as it shortens recovery time and reduces complications associated with an open procedure.[4],[5] Thus, initially, EVAR was done for suspected contained rupture rather than a complex open procedure involving multiple major vessels with a thoracoabdominal approach associated with significant morbidity. However, obtaining biopsy sample from the site of the lesion was not feasible with the endovascular approach. The sample obtained for histopathologic evaluation from the access site showed no evidence of systemic vasculitis.
During the subsequent rupture, in view of multiple concomitant medical complications, an extensive open thoracoabdominal procedure to deal with the extension of the lesion across the pararenal and suprarenal aorta was unviable. Therefore, endovascular approach with a surgeon-modified endograft was preferred to ensure adequate flow across major vessels. Customised endograft was fashioned during the procedure by creating bilateral renal fenestrations, and stented fenestration for the superior mesenteric artery. The position of the stent and patency of the vessels was confirmed with an angiogram. However, the patient developed acute renal injury, which subsequently led to diminished flow across the right renal artery, perhaps due to secondary thrombosis along with insult aggravated by concomitant cardiomyopathy.
Thrombocytopenia was particularly perplexing in our case as even though bone marrow infiltration was present, megakaryocytic thrombocytopenia and the absence of pancytopenia were suggestive of peripheral cause. Furthermore, drug-induced thrombocytopenia should improve within a week after cessation of the offending drug.[6] Haemophagocytic lymphohistiocytosis in the tubercular mycotic aneurysm is rarely described across the literature. Our patient improved with timely diagnosis and treatment with steroids.
Disseminated intravascular coagulation secondary to tuberculosis is infrequent (3.2%) and associated with extremely high mortality (63%).[7] This subsequent thrombocytopenia was perhaps even more challenging as it had undulating response to the first-line therapy of steroids and plasmapheresis. A course of intravenous immunoglobulins was required to induce remission.
The initial diagnostic dilemma challenges among the surgical treatment compounded by emerging rare medical complications warranting frequent modifications in antitubercular therapy causing difficulty in optimising the medical treatment made this an exceptional case. Furthermore, the progression of the lesion, despite combined medical and surgical approach, in the form of recurrent life-threatening ruptures, could be attributed to inadequate penetration of antitubercular therapy through laminated thrombus lining the aneurysm. There is a possibility that the placement of intraluminal stent or angiography may have aggravated the continued dissemination of tuberculosis[8] such as tubercular choroiditis, another rare extrapulmonary manifestation of tuberculosis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Acknowledgements
Dr. Prabhu Kumarappan for providing guidance and angiography images.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Gajaraj A, Victor S. Tuberculous aortoarteritis. Clin Radiol 1981;32:461-6.  |
| 2. | Orimoto Y, Ohta T, Ishibashi H, Sugimoto I, Yamada T, Kamiya M. A case of an infected abdominal aortic aneurysm caused by infectious spondylitis. Vasa 2010;39:94-7. |
| 3. | Long R, Guzman R, Greenberg H, Safneck J, Hershfield E. Tuberculous mycotic aneurysm of the aorta: Review of published medical and surgical experience. Chest 1999;115:522-31. |
| 4. | Canaud L, Marzelle J, Bassinet L, Carrié AS, Desgranges P, Becquemin JP. Tuberculous aneurysms of the abdominal aorta. J Vasc Surg 2008;48:1012-6. |
| 5. | Manika K, Efthymiou C, Damianidis G, Zioga E, Papadaki E, Lagoudi K, et al. Miliary tuberculosis in a patient with tuberculous mycotic aneurysm of the abdominal aorta: Case report and review of the literature. Respir Med Case Rep 2017;21:30-5. |
| 6. | Kalra A, Kalra A, Palaniswamy C, Vikram N, Khilnani GC, Sood R. Immune thrombocytopenia in a challenging case of disseminated tuberculosis: A case report and review of the literature. Case Rep Med 2010;2010:946278. |
| 7. | Wang JY, Hsueh PR, Lee LN, Liaw YS, Shau WY, Yang PC, et al. Mycobacterium tuberculosis inducing disseminated intravascular coagulation. Thromb Haemost 2005;93:729-34. |
| 8. | Colucci V, Moreo A, Passini L, Gordini V, Fedriga E, Pellegrini A. Successful resection of a tuberculous pseudoaneurysm of the descending thoracic aorta: Case report and review of the literature. Tex Heart Inst J 1992;19:142-5. |
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