Document Detail

Small facial haemangioma and supraumbilical raphe--a forme fruste of PHACES syndrome?
MedLine Citation:
PMID:  16225625     Owner:  NLM     Status:  MEDLINE    
We report two female infants with congenital midline supraumbilical raphes who subsequently developed haemangiomas on the lower lip and gingiva within the first 2 months of life. One was found to have a subglottic haemangioma during laryngoscopy. The infants were otherwise well and had normal chest X-ray, echocardiogram, cranial ultrasound, magnetic resonance imaging/angiography (head, neck, chest) and ophthalmological examination. Both received oral prednisolone 1-2 mg kg(-1) daily and four sessions of flashlamp pulsed-dye laser therapy to the lip lesions, with significant improvement. The initial presentation of these two infants with supraumbilical raphes, who were otherwise healthy and without other cutaneous stigmata, suggested the diagnosis of isolated congenital sternal malformation. However, lower lip and gingival haemangiomas developed 4-6 weeks later, consistent with the diagnosis of PHACES syndrome. Children with sternal malformation and haemangioma may also have intracranial and/or cardiovascular anomalies. All previously reported patients were females who had either craniofacial and/or multiple haemangiomas. We propose guidelines for the evaluation and management of a neonate presenting with a sternal fusion defect at birth.
Y-C Chan; L F Eichenfield; J Malchiodi; S F Friedlander
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Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  The British journal of dermatology     Volume:  153     ISSN:  0007-0963     ISO Abbreviation:  Br. J. Dermatol.     Publication Date:  2005 Nov 
Date Detail:
Created Date:  2005-10-17     Completed Date:  2006-02-03     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0004041     Medline TA:  Br J Dermatol     Country:  England    
Other Details:
Languages:  eng     Pagination:  1053-7     Citation Subset:  IM    
National Skin Centre, 1 Mandalay Road, Singapore 308205.
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MeSH Terms
Abnormalities, Multiple / diagnosis
Facial Neoplasms / diagnosis*
Gingival Neoplasms / diagnosis
Hemangioma / diagnosis*
Lip Neoplasms / diagnosis
Sternum / abnormalities*

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