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A Rare Case of Diffuse Neonatal Hemangimatosis

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Diffuse neonatal hemangiomatosis (DNH) is a rare neonatal condition in which cutaneous and visceral hemangiomas coexist. If left untreated, DNH is usually fatal at an early age. We report a case of a 6-month-old male infant who was brought to our institution with hepatosplenomegaly and a history of anemia and thrombocytopenia since 1 month of age. Cytogenetic analysis and liver biopsy were normal and bone marrow aspirate was nondiagnostic. Congenital red blood cell abnormality was ruled out. Ultrasound confirmed an increase in size of the spleen from 5 to 15 cm, and magnetic resonance imaging demonstrated intense splenic enhancement consistent with a hemangioma or vascular malformation. Despite severe thrombocytopenia, an exploratory laparotomy was done and the patient underwent a splenectomy and omentectomy. The final pathology confirmed hemangiomatosis of the spleen and omentum. In the neonate with unexplained anemia and thrombocytopenia, DNH should be considered as part of the differential diagnosis. In our case, the patient not only exhibited no obvious cutaneous involvement, but also had rare splenic involvement. Although there are risks involved when operating on a thrombocytopenic patient, the benefits of operating on a patient with DNH far outweigh the risks, and operative intervention should proceed without delay.
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Document Type: Research Article

Affiliations: 1: From the Departments of Surgery 2: Pathology, Seton Hall University School of Graduate Medical Education, General Surgical Program at St. Francis Medical Center, Trenton, New Jersey 3: Department of Pediatric Surgery, St. Joseph's Regional Medical Center, Patterson, New Jersey2

Publication date: April 1, 2006

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