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The Nasal Crease Sign May Herald Destruction of Septum in Segmental Facial Hemangiomas

Complications in hemangiomas of the nasal tip can be difficult to predict and difficult to manage.

Although most infantile hemangiomas require no treatment, some problems, such as visual obstruction or ulceration, do require intervention. Hemangiomas with a segmental morphology have a higher risk for complications than focal hemangiomas and may be associated with PHACES syndrome (see Journal Watch Dermatology Aug 18 2006).

In this report, the authors describe three full-term infant girls who presented in the first 2 to 4 weeks of life with rapidly proliferating segmental facial hemangiomas involving the columella and varying degrees of the philtrum. At least one patient had PHACES syndrome. To prevent visual obstruction, cutaneous ulceration, and deformity of the nose and lip, clinicians initiated systemic corticosteroid therapy (oral prednisolone at 3 mg/kg/day), but no patient responded, necessitating treatment with interferon alfa-2a. Despite therapy, all three infants experienced destruction of the columella and nasal septum. The authors describe the appearance of a grayish, horizontal transverse crease on the columella underneath the nasal tip, approximately 2 weeks before rapid necrosis and loss of the nasal septum and columella. They also note that reconstruction of an aesthetic, functional, and durable nose in such infants is extraordinarily difficult.

Comment: Predicting the course of hemangiomas involving the nasal tip in young infants is notoriously difficult. Some require only watchful waiting, but others require aggressive systemic management. These three infants suffered serious nasal destruction despite early aggressive therapy. We need further study to determine whether the nasal crease sign is sensitive and specific for impending columella and septal destruction. Furthermore, it is unclear whether the "point of no return" has been crossed once this sign is visible. Infants with segmental hemangiomas are at high risk for complications, and close serial follow-up is mandatory. Ulcers in segmental hemangiomas may be less responsive to therapy than those arising in the nonsegmental type.

— Mary Wu Chang, MD

Published in Journal Watch Dermatology August 3, 2007

Citation(s):

Dakara RW et al. The nasal crease sign in segmental facial hemangioma — An early sign of cartilage destruction. Pediatr Dermatol 2007 May/Jun; 24:241-5.

• Medline abstract (Free)