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Segmental facial hemangioma accompanied by brain anomalies: Report of a case

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Segmental facial hemangioma accompanied by brain anomalies: Report of a case
Thaer Douri
Dermatology Online Journal 9 (5): 22

Ministry of Health, Syria-Hama. s-dori@scs-net.org

Abstract

Hemangioma of infancy (HOI) is the most common tumor of infancy. Initial rapid proliferation is generally followed by gradual involution and resolution. However, some HOI may be associated with congenital structural abnormalities. We describe a case of HOI associated with hypoplasia of the left cerebellum and arachnoid cyst.


Hemangioma of infancy (HOI) is the most common tumor of infancy. The incidence in the newborn nursery is between 1.0 and 2.6 percent in white, black, and Japanese infants. An incidence of 10-12 percent has been reported in white children by age 1 [2]. HOI is characterized by an initial phase of rapid proliferation followed by a phase of slow involution, often leading to complete regression. Although most of the tumors are small and innocuous, some HOI may be life threatening, may inhibit function, or may be associated with structural congenital anomalies [1]. We describe a rare case of HOI which was associated with brain anomalies.


Case report


Figure 1

A 20-day-old female had a sudden onset of a large red macule on the face with a segmental distribution on the cheeks and upper eyelid. The lesion was mainly on the left side of face, but the right side was also involved (Fig. 1). The macule rapidly enlarged to form a vascular plaque on the upper eyelid and cheek, forcing closure of the left eye. Ulceration and bleeding were problematic. Routine blood tests were normal. Ophthalmic examination was normal. A computerized tomography examination revealed hypoplasia of the left cerebellum and formation of a cyst in the archnoid matter (Fig. 2).


Figure 2

The patient was initially treated with prednisolone (1mg/kg/day) without benefit. The hemangioma enlarged and continued to bleed. After two weeks the patient expired.


Discussion

Hemangioma of infancy is characterized by an initial phase of rapid proliferation followed by several years of slow regression. The initial proliferative phase of rapid growth usually lasts for 3-9 months and rarely extends beyond 18 months. The involution phase occurs gradually over 2-6 years and is complete by the age of 7-10 years [2]. Females are affected more commonly than males in a 3:1 ratio [2].

Facial HOI has been associated with midline-ventral defects, aortic anomalies, and central nervous system abnormalities. Frieden and Cohen proposed the acronym PHACE to unify the varying features of this syndrome: posterior fossa malformations, coarctation of the aorta, and cardiac defects [1].

PHACE represents a spectrum of associated anomalies, as 70 percent of affected individuals have only one extracutaneous manifestation. The common feature in all cases is a large facial hemangioma that is plaque like and segmental in nature. Nearly 90 percent of affected infants are female, a much higher prevalence than the 3:1 or 5:1 ratio reported for common HOI.

Dandy-Walker malformation is the most common structural brain abnormality. Its features include posterior fossa cyst, hypoplasia of the cerebellar vermis, and cystic dilation of the fourth ventricle, often resulting in hydrocephalus and increased head circumference. Many other central nervous system lesions have been described. Arterial anomalies of the head and neck, such as aneurysms and anomalous branches of the internal carotid artery, are also common.

Systemic corticosteroid therapy is the treatment of choice for the PHACE syndrome. Zarem and Edgerton treated seven children with enlarged HOI with prednisolone (20-30 mg per day), and noted rapid cessation of growth or partial involution within 2 weeks [3]. The recommended starting dose is 2-3 mg/kg of prednisone or prednisolone given daily as a single morning dose. Higher doses are sometimes used for severe cases such as hemangioma associated with airway obstruction. We treated our patient with prednisolone (1.5 mg/kg) but our treatment failed. The failure was possibly the result of insufficient dose or inadequate absorption.

Others treatments include α-interferon (2a and 2-b, 1-3 million U/m² of body surface area daily). Weekly intravenous vincristine may be used at a dosage of 0.05 mg/kg in infants less than 10 kg or 1.5 mg/m² in children greater than 10 kg. Cyclophosphamide is also reported to be an effective treatment [1].

References

1. Bruckner AL, Frieden IJ. Hemangiomas of infancy. J Am Acad Dermatol 2003;48(4):477-93; quiz 494-6. PubMed

2. Dermatology in General Medicine. Fitzpatrick (CD ROM fifth edition)

3. Zarem HA, Gray GF Jr, Morehead D, Edgerton MT. Heterotopic brain in the nasopharynx and soft palate: report of two cases. Surgery. 1967 Mar;61(3):483-6. PubMed

© 2003 Dermatology Online Journal