Hemangiomatosis, neonatal D18.0

Neonatal, non-hereditary, also self-limited in growth, multilocular variant of infant hemangioma with multiple (> 5) hemangiomas of the skin (benign neonatal hemangiomatosis) and possibly also of the internal organs (diffuse neonatal hemangiomatosis). Large growth pressure within the 1st year of life. As haemangiomas express iodothyronine deiodinase, the risk of clinically relevant secondary hypothyroidism increases with the size and number of haemangiomas.

• Benign neonatal hemangiomatosis without extracutaneous involvement
• Diffuse neonatal hemangiomatosis with involvement of 2 other organ systems.

• Type I: Benign neonatal hemangiomatosis with multiple, usually pinhead-sized or up to 20 mm large, red-livid hemangiomas, (exclusively) of the skin; no organ involvement.
• Type II: Diffuse neonatal hemangiomatosis with multiple skin and system involvement (skin: 100%, liver: 64%, brain: 52%, gastrointestinal tract: 52%, oral: 44%, eyes: 32%). Prognosis for systemic infestation significantly reduced. Most frequent cause of death: Congestive heart defects as a result of arteriovenous shunts in liver and lungs. Haemorrhages from haemangiomas in the (upper) respiratory tract or GI tract.

• Oral, genital and conjunctival involvement is more suggestive of diffuse neonatal hemangiomatosis but is not pathognomonic.
• Clinical: exclusion of GI bleeding; hematuria, anemia, petechiae, icterus, hepatomegaly, neurological signs.
• Apparative diagnostics: X-ray chest, abdominal ultrasonography, CT/MRI e.A. of intracranial lesions.
• Exclusion of other diseases with hemangiomas such as Kasabach-Merritt syndrome (consumption coagulopathy) or Maffucci syndrome.

Clarification of organ involvement and regular monitoring. Without early treatment, mortality is very high.

Analogous to the therapy of the infantile hemangioma, a therapy with propanolol (for dosages see there) should be aimed at.

Some patients respond well to glucocorticoids (2-3 mg/kg bw/day prednisolone equivalent).

If necessary, surgical removal of larger or unfavourably localised haemangiomas. Continuous monitoring with regard to internal bleeding is important (intensive care measures!). In case of lack of response, possibly vincristine 2 mg/m2 KO/week i.v. as a single dose.

1. Glick ZR et al (2012) Diffuse neonatal hemangiomatosis: an evidence-based review of case reports in the literature. J Am Acad Dermatol 67:898-903.
2. Höger PH (2005) Pediatric dermatology. Schattauer, Stuttgart New York
3. Leung AKC et al (2003) Benign Neonatal Hemangiomatosis. Pediatric Dermatology 2: 161-163
4. Schulze SM (2006) A rare case of diffuse neonatal hemangiomatosis. At the surgeon's office 72: 359-362
5. Smolinski KN, Yan AC (2005) Hemangiomas of infancy: clinical and biological characteristics. Clin Pediatr 44: 747-766