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A rare case of diffuse neonatal hemangimatosis
S M Schulze1, R L Moser, N Bhattacharyya
1Department of Surgery, Seton Hall University School of Graduate Medical Education, General Surgical Program at St. Francis Medical Center, Trenton, New Jersey 08629, USA.
The American Surgeon
|May 9, 2006
Summary
Diffuse neonatal hemangiomatosis (DNH) is a rare, often fatal condition. Early surgical intervention for splenic hemangiomatosis, even with thrombocytopenia, can be life-saving in infants with unexplained anemia.
Area of Science:
- Neonatology
- Pediatric Surgery
- Vascular Malformations
Background:
- Diffuse neonatal hemangiomatosis (DNH) is a rare condition characterized by widespread cutaneous and visceral hemangiomas.
- Untreated DNH typically leads to early mortality.
Observation:
- A 6-month-old male infant presented with hepatosplenomegaly, anemia, and thrombocytopenia since 1 month of age.
- Imaging revealed significant splenic enlargement and enhancement, suggestive of a hemangioma or vascular malformation.
- The patient had no apparent cutaneous hemangiomas but rare splenic involvement.
Findings:
- Splenectomy and omentectomy were performed despite severe thrombocytopenia.
- Pathology confirmed hemangiomatosis of the spleen and omentum.
- Cytogenetic analysis, liver biopsy, and bone marrow aspirate were non-diagnostic; congenital red blood cell abnormalities were excluded.
Implications:
- DNH should be considered in neonates with unexplained anemia and thrombocytopenia.
- Surgical intervention for DNH, including splenectomy, offers significant benefits that outweigh the risks, even in thrombocytopenic patients.
- Prompt operative management is crucial for improving outcomes in DNH.