G145(P) An aggressive and expanding infantile hemangioma: a therapeutic challenge. (May 2019)

• Record Type: Journal Article
• Authors: Dhar, A; Damian, O; Nair, P
• Is Part Of: Archives of disease in childhood. Volume 104:(2019)Supplement 2
• Page Start: A59
• Page End: A59
• Publication Date: 2019-05
• Subjects: Children -- Diseases -- Periodicals; Infants -- Diseases -- Periodicals; 618.920005
• DOI: 10.1136/archdischild-2019-rcpch.141
• Languages: English
• ISSNs: 0003-9888

Abstract

Abstract : Introduction: Infantile haemangiomas (IH), are elevated swellings of the skin usually soft in nature that occur in 3%–10% of infants. These benign, endothelial cellular origins, vascular tumours are mostly uncomplicated and tend to regress spontaneously. However when seen in high risk areas like orbit, nose, airway with functional impairment, or when complications develop, early intervention is required. Background: A 6 month old girl was born at 35+1 gestation by Emergency Caesarean Section due to intrauterine growth restriction. She was born with two small haemangiomas, on lateral aspect of her left eyelid and at back. Her father has Stargardt disease (Juvenile macular degeneration); mother had haemangioma, requiring excision. Presentation: She had haemangiomas over her eyelid and back which were 2 × 2 cm, 1.5 × 1 cm respectively at birth. Haemangioma over left eyelid continued to grow, at 3wks, this was 3.5 × 3 cm with yellow discharge. She subsequently came to hospital for bleeding from hemangioma which by then measured 4 × 4 cm. This was steadily growing in spite of all medical treatment (propranolol). At around 6 months age the haemangioma compressed the nostril to the right side of face. The bulk of haemangioma from left face extended into temporal area and left eye causing complete ptosis (with risk of amblyopia) and compression to bottom of jaw. There was increasing breakdown of skin and dry areas which bled easily. Auditory Brainstem Response suggested right side auditory neuropathy. Management: Ultrasound was consistent with haemangioma. MRI showed a diffuse left facial lesion extending superficially between the temporal fossa, cheek and parotid region down into the submental space. The lesion extended to intraorbital, masticator, para-pharyngeal spaces. Within the orbit it displaced the globe, orbital contents superolaterally. Propranolol was started from 4 week, increased to highest dose, and then switched to Atenolol around 3 months of age having no clinical improvement even with higher dose. She was also commenced on steroids without any response, so was stopped. She is under discussion with craniofacial team for possible resection of lesion. Conclusion: Large aggressive hemangiomas can be very difficult to manage. Currently there are no agreed guidelines on how we manage these aggressive lesions. These hemangiomas cause significant tissue excess, bone compression, amblyopia and presents significant reconstructive challenges.