Imaging features and enhancement technique to diagnose and classify intrathoracic Lymphatic-venous malformations: A case report and literature review

Abstract

The diagnosis and treatment of pediatric intrathoracic lymphatic-venous malformations (LVM) can be complex due to their rarity, variable presentation and confusing nomenclature in the literature. The International Society for the Study of Vascular Anomalies (ISSVA) has recently (2018) updated their classification to help guide the correct diagnosis, nomenclature and management of such cases. We present the case of a 12-month-old Caucasian female with a lymph-venous malformation (LVM) classified in the updated ISSVA classification as a combined vascular malformation (CLVM) defined as two or more vascular malformations found in one lesion, associated with an underlying “malformation of an individual named vessel”. The patient presented with tachypnea, tachycardia and fever. While all the previous cases underwent surgical treatment, our patient was successfully treated with rapamycin and sclerotherapy. Appropriate imaging can aid in the diagnosis of vascular anomalies and in the proper ISSVA classification, saving the patient the need for a biopsy and allow for proper referral to Multidisciplinary Vascular Anomalies centers. The accurate classification can identify cases that can be treated through Interventional Radiology with sclerosing agents and medical therapy as opposed to surgery.

Keywords

• Lymphatic-venous malformations
• Vascular malformation
• Pediatric radiology
• Sclerosing agents
• ISSVA
• Combined vascular malformation