Sir,
A 2-month-old girl presented to the emergency department with respiratory distress. Chest X-ray showed pulmonary edema with cardiomegaly. The electrocardiogram showed sinus tachycardia with Q-waves in lateral leads. An echocardiogram showed an anomalous origin of the left coronary artery arising from the pulmonary artery (ALCAPA). She underwent repair by direct transfer of the anomalous coronary artery. A large button of the coronary with a portion of the pulmonary artery wall was dissected to create a tube extension for the coronary. This was connected to the left posterior side of the ascending aorta. The postoperative course was significant for Serratia marcescens respiratory infection and bacteremia, treated with intravenous (IV) antibiotics. On postoperative day 32, an echocardiogram showed a large aneurysm arising from the ascending aorta [Figure 1]. A computed tomography angiogram (CT scan) showed a 3.5 cm × 4.2 cm × 3.1 cm ascending aorta aneurysm [Figure 2]. In the operating room, a mucoid-like tissue at the aneurysmal wall junction and sutures was noted. The aneurysmal tissue was resected, and a pulmonary homograft was used to patch the ascending aorta. Tissue bacterial and fungal cultures were negative. She was treated with IV antibiotics for 4 weeks. Repeat echocardiogram showed a recurrent large pseudoaneurysm, 3.5 cm × 3.9 cm × 4.0 cm confirmed by CT scan [Figures 1 and 2]. In the operating room, there was mucoid-like tissue at the base of the aneurysm with dehiscence of left coronary artery sutures. Repair with aortic homograft was used this time for reconstruction and re-implantation of the left coronary artery. Tissue pathology showed fungal hyphae [Figure 3], and 16s ribosomal RNA gene sequencing was positive for Rhizopus microsporus. Extensive immunodeficiency workup was negative, and treatment was started with IV liposomal amphotericin for 3 months, followed by oral posaconazole for 6 months. Repeat echocardiograms and chest CT scan showed no recurrent pseudoaneurysm.
Figure 1.
Long-axis parasternal echocardiogram showing the initial aortic pseudoaneurysm (a) and the recurrent pseudoaneurysm (b)
Figure 2.
(a) Computed tomography angiogram which shows a pseudoaneurysm arising from the anterior wall of the ascending aorta. Dimensions 3.5 cm × 4.2 cm × 3.1 cm. (b) Recurrent pseudoaneurysm. Dimensions 3.5 cm × 3.9 cm × 4.0 cm. AAo: Ascending aorta, Ao: Aorta
Figure 3.
Aortic wall pathology: Irregular structures consistent with PAS stain (a) and GMS stain (b) positive for fungal hyphae (arrows)
DISCUSSION
Mycotic aneurysm (MA) is an abnormal pouching of the wall of an artery secondary to an infection and is reported mostly in adults after surgical repair of aortic dissection.[1] However, it is rarely seen in children. We report the first case of MA due to R. microsporus in an infant following the repair of ALCAPA.
In this case, tissue pathology and 16s ribosomal RNA gene sequencing confirmed the diagnosis. Possibly, a chronic infection seeded into the suture line, resulting in the dehiscence and development of aneurysm. Foreign bodies can lower the immune system causing MA.[2] This unique etiology with R. microsporus has not been reported in children. Fungal causes of aortic aneurysms are rare, but Silva et al. reported Aspergillus terreus with thoracic aortic aneurysm.[3] Pruthi et al. reported five adult patients with pulmonary mucormycosis after SARS-CoV-2 infection. One patient had R. microsporus and was later found to have a pseudoaneurysm of the right pulmonary artery.[4] Few cases of MA caused by bacterial infections are seen in children.[5] We could not find any case of MA caused by R. microsporus in the pediatric age group after cardiac surgery.
There is no well-defined treatment for MA in pediatrics. Surgery remains the treatment of choice, but there is increased morbidity as shown in adult patients with low cardiac output states in 6.7%–17.2% of patients.[1] Common surgical practice is ligation of the artery, removing infected tissues, and putting in a graft. Endovascular aneurysm repair is used in high-risk patients as a bridge to surgery.
CONCLUSION
Aggressive surgical interventions and long-term antifungal therapy resulted in the favorable outcome of MA caused by R. microsporus.
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
Nil.
Conflicts of interest
There are no conflicts of interest.
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