We report the case of a 7-year-old child from Iraq who presented with the complaints of cough and breathing difficulty, with progressive worsening over two months before presentation. Parents had also noticed increasing yellowish discolouration of eyes and skin, loss of appetite and weight in the previous month. The patient had a low-grade, intermittent fever for the past 20 days. The child had been diagnosed to be a case of hepatic failure and had been referred for liver transplant. On examination, the child had tachycardia, tachypnea and mild subcostal, intercostal retractions. Breath sounds were absent on the right side. There was non-tender hepatomegaly, with the liver span of 16 cm and smooth surface. Minimal ascites was present. Liver functions were deranged (serum glutamic oxaloacetic transaminase or SGOT/ serum glutamic pyruvic transaminase or SGPT 145 / 345 U/L, gamma-glutamyl transferase or GGT 528 U/L, total bilirubin and direct 6.4/2 mg/dL, total protein 8.1 g/dL, serum albumin 2.8 g/dL). The international normalized ratio (INR) was 1.37. He also had severe anemia (hemoglobin – 5.8 g/dL), with absolute eosinophil count of 2.45×109/L and high pro-inflammatory markers. Chest radiograph revealed right-sided pleural effusion with underlying collapse and consolidation. Pleural tap revealed almost bile-like pleural fluid with high bilirubin level suggestive of a trans-diaphragmatic extension of the hepatic disease. Evaluation of the fluid for infection was negative. Contrast-enhanced, multiphasic, multi-detector computed tomographic (MDCT) scan of abdomen revealed hepatomegaly with a large hypodense lesion in the liver, invading the inferior vena cava and serosa of the oesophagus with cystic changes, and was reported by the radiologist to possibly be mitotic etiology of the biliary tract or Echinococcus alveolaris. Qualitative Echinococcus (E) IgG was positive. Endoscopy revealed normal esophageal and gastric mucosa. Echocardiography demonstrated inferior vena cava infiltration by a mass extending into the right atrium. Ultrasound-guided liver biopsy revealed an inflammatory pathology with the possibility of mass forming E. multi-locularis. The child was treated with 15 mg/kg/day divided in two doses of continuous albendazole therapy and other supportive treatment, and was under regular follow up. Unfortunately, the child died 2 months later.

Fig. 1 (a) 2-D Echocardiography (modified 4 chamber view) showing mass in IVC (b) Modified subcostal view with mass clearly seen in RA (c) CT: Nature of disease seen here – hypoechoic lesion with no blood/calcification within, unlikely to be carcinoma (d) Venous Phase of Triphasic CT: Trans diaphragmatic spread seen into the adjoining tissue, oesophageal serosa infiltrated. IVC, inferior vena cava; RA, right atrium; CT, computed tomography.

Alveolar echinococcosis, due to E. multilocularis is extremely unusual, accounting for < 5% of all cases of hydatid liver disease and, less frequently, lung disease. The mean age of presentation is 55 years [1,2], with children being rarely affected. Liver is the primary site of cyst development in almost all patients. The characteristic feature of E. multilocularis is that they behave just like malignant tumors with invasion and destruction of surrounding tissue, spread into contiguous areas and metastasis to distant organs, with the most common organ being lung [3]. Lung manifestations always appear after the involvement of the liver [3]. Cardiac echinococcosis is very rare (0.03%-1% of all cases) [2], with the left ventricle being most frequently affected (55–60%).

We diagnosed our patient to be a confirmed case of alveolar echinococcosis based on clinical findings, contrast-enhanced MDCT, histopathology and serology [4]. We further classified the case as per the WHO-IWGE (WHO-Informal Working Group on Echinococcosis) PNM classification as P4N1M1 [5].

The focus of management in these patients is early diagnosis and radical (tumour-like) surgery, which is followed by anti-infective prophylaxis with benzimidazoles [1,3,4]. However, as in our case, most patients are diagnosed at an advanced stage, when radical surgery (a distance of larval to liver tissue of >2 cm) cannot be achieved. Hence, as per current recommendations, the cornerstone of treatment remains the continuous medical treatment with albendazole, with individualized interventional measures at the appropriate time [1,4]. Radical surgery could not be done in our patient as R0 (no residue) resection was not possible. Palliative surgery was not possible as the lesion was unresectable due to invasion into the oesophagus, as well as into a blood vessel, leading to its spread to distant organs (both lungs and heart) [1]. Liver transplant was contraindicated due to the presence of extra-hepatic locations [1].

2. Su L, Yu J, Dai C, Liu Y, Peng L. Echinococcosis in left ventricle: a case report. Medicine (Baltimore). 2019;98:e15267.

3. Morar R, Feldman C. Pulmonary echinococcosis. Eur Respir J. 2003;21:1069-77.

4. Brunetti E, Kern P, Vuitton DA. Writing panel for the WHO-IWGE. Expert Consensus for the Diagnosis and Treatment of Cystic and Alveolar Echinococcosis in Humans. Acta Trop. 2010;114:1-16.

5. Kern P, Wen H, Sato N, et al. WHO classification of alveolar echinococcosis: Principles and application. Parasitol Int. 2006;55:S283-87.

6. Zhang X, Wei X, Ran L, Tang H. A rare case of cardiac alveolar echinococcosis. Eur Heart J. 2020;41:2698.

7. Fiengo L, Bucci F, Giannotti D, Patrizi G, Redler A, Kucukaksu DS. Giant cardiac hydatid cyst in children: Case report and review of the literature. Clin Med Insights Case Rep. 2014;7:111-16.