Rapidly involuting congenital haemangioma in a term neonate

Description

Following an uncomplicated pregnancy with normal antenatal scans, a term neonate was unexpectedly born with a large mass on the right temporal region (figure 1). This caused extreme anxiety among staff regarding the possibility of encephalocoele or meningocoele. Examination revealed a soft, compressible mass, with no midline defect. Subsequently, cranial ultrasonogram demonstrated no extracranial extension of brain tissue, and normal ventricles. MRI confirmed a haemangioma with no evidence of intracranial extension and normal brain parenchyma. The infant was haemodynamically stable with a normal platelet count. Rapidly involuting congenital haemangioma (RICH) was diagnosed following review by plastic surgeons. The haemangioma completely regressed by 7 months, resulting in redundant skin and distortion of the pinna requiring reconstructive surgery (figure 2).

Figure 1.

Large haemangioma over the right temporal region; taken on day 3 of life showing distortion of the pinna.

Figure 2.

Involuting congenital haemangioma at 6 months of age resulting in redundant skin and distortion of the right pinna.

Congenital haemangiomas are rare lesions that are fully developed at birth. They are classified into two types: RICH and non-involuting congenital haemangiomas. Their combined incidence is less than 3% of the more commonly occurring nascent infantile haemangiomas.¹ The natural course is characterised by spontaneous and complete resolution within 6–14 months.^{1 2} Histopathologically, unlike infantile haemangiomas, congenital haemangiomas stain negative for glucose transporter-1 protein.^{1 2 3} The management of RICH includes expectant observation, assessment of haemodynamic status and wound care. Surgical excision is indicated for persistent ulceration or development of Kasabach Merritt phenomenon in medically resistant lesions. Lesions that do not follow the expected course require biopsy/excision to distinguish from malignant tumours.

This case highlights rare congenital haemangiomas and emphasises the need for multidisciplinary input for diagnosis and management.

Learning points.

• The importance of multidisciplinary teams for the diagnosis and management.

• Identification of rarer haemangiomas from common haemangiomas by their natural history.

• Haemangiomas should be monitored, if they do not follow their expectant course biopsy/excision should be considered to exclude malignancy.

• A platelet count should be checked in large haemangiomas.

• Patients with large haemangiomas should be examined for signs of heart failure.