Echographic findings in a complex and rare vascular malformation: persistance of the primitive hepatic venous plexus, inferior vena cava interruption and Scimitar syndrome

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Aim: To present a 15 yo boy, with bronchial asthma, discovered at the age of 1 with hepatic vascular tumor considered to be hepatic hemangioma, right diaphragmatic relaxation and right inferior pulmonary tumor, negative at minimal pulmonary surgery, followed up many years, in different departments. He was admitted in the cardiology department for a routine cardiac examination. Material and methods: The patient performed a complex cardiac exploration, beginning with clinical examination, ECG, echocardiography, chest X ray, and abdominal ultrasound (US). After that, the diagnosis changed dramatically to a complex and rare congenital vascular malformation, confirmed by angio CT of the heart and abdomen. Results: The clinical exam discovered a continuous murmur in the abdomen and hepatomegaly at 2–3-3cm under the costal border. ECG exploration was normal, but chest X ray revealed a right diaphragmatic relaxation and a Scimitar shape shadow of the right inferior lung field. Echocardiography could not reveal all the 4 pulmonary veins draining in the left atrium and a particular filling of the right atrium, directly from hepatic veins, was evident in subcostal views. Abdominal US completed the investigations, prooving the inferior vena cava interruption, right hepatic vein draining in the inferior vena cava, as well as extremely dilated middle and left hepatic vein that join together, entering directly in the right atrium and multiple hepatic arterio-venous shunts. Angio CT of the heart and abdomen confirmed and completed the diagnosis with right pulmonary hypoplasia, partial abnormal pulmonary venous drainage of the right inferior pulmonary vein in the inferior vena cava, arterial vessel rising from the celiac trunk, directed to the right lung, posterior to the right inferior pulmonary vein. Conclusions: Joining together clinical and paraclinical investigations with cardiac and abdominal US we proved the persistance of the primitive hepatic venous plexus, multiple hepatic arterio-venous shunts, inferior vena cava interruption and Scimitar syndrome, all of them confirmed by angio CT. These are rare associations having only been reported a handful of times. Scimitar syndrome created a „phantom“ tumor, undetected in minimal lung surgery diagnosedat the age of 1 yo. Good US exploration on a good machine and experienced doctors working in a team was enough to establish a real and rare diagnose.

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