A 3 month old infant with a “strawberry” red mass on her nose

• Published in: BMJ (Online) 2014-06, Vol.348 (jun11 10), p.g3810-g3810
• Main Authors: Charles, E, Milroy, C, Goldstraw, N K, Giuliani, S
• Format: Article
• Language: English
• Subjects: Babies; Classification; Counseling; Diagnosis, Differential; Female; Hemangioma - diagnosis; Hemangioma - therapy; Humans; Infant; Nose; Nose Neoplasms - diagnosis; Nose Neoplasms - therapy; Patient Education as Topic; Pediatrics; PICTURE QUIZ; Referral and Consultation; Tumors
• ISSN: 0959-8138; 1756-1833; 1756-1833
• DOI: 10.1136/bmj.g3810

The International Society for the Study of Vascular Anomalies (ISSVA) classification system was issued in 1996 with the aim of establishing a common nomenclature, and it is currently the only system to be widely accepted across the different specialties involved. 1 This classification system refers to the original description by Mulliken and Glowacki in 1982 and is based on endothelial characteristics and clinical behaviour. 2 It divides vascular anomalies into two main categories: vascular tumours and vascular malformations. 3 4 Infantile haemangioma is the most common vascular tumour of infancy, being found in about 5% of infants. 5 The incidence is highest in female and premature infants. 6 Unlike congenital vascular malformations, this benign lesion is typically not present at birth but appears in the first few weeks of life as a small red macule. Indications for immediate referral to a tertiary paediatric vascular anomalies team Life or function threatening Periorificial location (eyes, nose, mouth, perineum, or genitalia) Beard or mandibular lesion (risk of airway compromise) Parotid area Breast area Extremely large lesions (>15 cm), especially in a visible area Risk of underlying abnormality Multifocal (>=5) lesions (risk of hepatic haemangiomatosis) Large segmental lesions of the face and neck (associated with PHACES* syndrome) Lumbosacral lesions (spinal dysraphism or structural anomalies) *PHACES: Furthermore, long term deformity or scarring of the nose can have a serious aesthetic impact and is often difficult to correct at a later stage Lesions around the mouth or parotid and perineal lesions have an increased risk of ulceration and long term structural damage Mandibular and beard haemangiomas are associated with underlying airway lesions and a risk of airway compromise; they may need to be assessed in the ear, nose, and throat department with laryngoscopy and bronchoscopy. 15 In girls, lesions in the breast area may lead to impaired development of the mammary glands and result in long term functional, aesthetic, and psychological impairments. The drug is usually taken as an oral liquid three times daily (total dosage 2 mg/kg/day) for three to 12 months. 22 Reported side effects include sleep disturbance, lethargy, gastrointestinal upset, hypoglycaemia, hypotension, bradycardia, and bronchospasm. 23 Pulse dye laser (585-595 nm) treatment is indicated for airway haemangiomas and ulcerating lesions that have not responded to systemic therapeutic