SURGICAL MANAGEMENT OF A MASSIVE CONGENITAL HEMANGIOMA OF THE TONGUE IN AN INFANT: A RARE CASE REPORT

Lesions involving the tongue may present with life threatening complications such as airway compromise and risk of severe hemorrhage. This paper reports the surgical management of a non-involuting massive congenital hemangioma of the tongue in an infant presenting with difficulty breathing, feeding and inability to close the mouth.

Rapidly involuting congenital hemangioma associated with Kasabach-Merritt Syndrome

Background: Rapidly involuting congenital hemangioma (RICH) is a rare vascular tumor that is present at birth and involutes during the first year of life. Kasabach-Merritt syndrome (KMS) is a complication of some vascular tumors such as kaposiform hemangioendothelioma and tufted angioma associated with thrombocytopenia and coagulopathy. Results: The case of a 2-month-old infant with a diagnosis of RICH with thrombocytopenia and coagulation disorder, successfully treated with surgical excision without complications or recurrence is presented. Conclusion: The association between RICH and KMS is rare. Histopathological study, immunohistochemistry and ultrasound findings are important for the diagnosis. Brief summary: This report covers the rare association between rapidly involuting congenital hemangioma and Kasabach-Merritt syndrome in a 2-months-old female infant.

Repidly involuting congenital hemangioma

Congenital hemangiomas are rare benign vascular tumors that develop in utero and are fully formed by the time of birth. Depending on the ability to involution, there are three subtypes: RICH (repidly involuting congenital hemangioma), NICH (non involuting congenital hemangioma), PICH (partially involuting congenital hemangioma). PICH may be accompanied by thrombocytopenia and consumption coagulopathy. Despite clearly defined clinical and histological characteristics, it can be difficult to make a differential diagnosis between congenital hemangiomas and other vascular tumors (infantile hemangioma, kaposiform hemangioendothelioma/“fascicular” angioma and others). The clinical case in the article of a vascular tumor in a newborn complicated by thrombocytopenia and consumption coagulopathy was regarded as Kazabach-Merritt syndrome, which is based on kaposiform hemangioendothelioma/“fascicular” angioma. Rapid regression of the tumor and recovery of hemogram and coagulogram parameters, as well as anamnesis of the disease and initial characteristics of the tumor forced to reconsider the diagnosis. Based on the histological picture, the diagnosis of congenital hemangioma, RICH, was confirmed. Verification of the diagnosis made it possible to change therapeutic tactics and avoid chemotherapy. A giant hemangioma, accompanied by thrombocytopenia and consumption coagulopathy, may have a very favorable outcome – a complete resolution of the pathological process inherent in its natural course. The patient's parents agreed to use the information, including the child's photo, in scientific research and publications.