Objective: To establish whether the prognosis of bilateral facial capillary malformation (BFCM) is worse compared with that of unilateral facial port-wine stain. Design: Retrospective study. Setting: Paediatric Dermat...
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Objective: To establish whether the prognosis of bilateral facial capillary malformation (BFCM) is worse compared with that of unilateral facial port-wine stain. Design: Retrospective study. Setting: Paediatric Dermatology Department, Great Ormond Street Hospital for Children NHS Trust, a tertiary referral center for vascular anomalies. Patients: A cohort of 350 children who presented with facial CM was seen between January 1, 1994, and June 30, 2004. Twenty-seven children with BFCM were identified. A control group of 27 children with unilateral CM was randomly selected from the total cohort. Main Outcome Measures: Demographic, clinical, and radiographic characteristics were recorded and compared between the 2 groups: age at presentation, sex, distribution, extension, extrafacial lesions, glaucoma, ipsilateral leptomeningeal angiomatosis, and epilepsy. The recorded informationwas collected from the database of the Paediatric Dermatology Department, the hospital records, and the patients’photographs. Results: Compared with the 27 children with unilateral facial CM, the 27 with BFCM showed a higher frequency of association with extrafacial lesions (17 [63%] vs 6 [22%]), glaucoma (21 [78%]-vs 2 [7%]), and ipsilateral leptomeningeal angiomatosis (14[52%] vs 2 [7%]). All patients who had BFCM with bilateral and complete involvement of the ophthalmic area had ipsilateral leptomeningeal angiomatosis. Conclusion: Patients with BFCM must be considered as a group with a worse prognosis compared with patients with unilateral facial CM.
Introduction. Capillary malformations such as benign hereditary telangiectasia are a familial affection, of dominant autosomal transmission, characterized by the progressive development of cutaneous telangiectasia dur...
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Introduction. Capillary malformations such as benign hereditary telangiectasia are a familial affection, of dominant autosomal transmission, characterized by the progressive development of cutaneous telangiectasia during childhood. The association with cutaneous vascular, arteriovenous or lymphatic malformations is exceptional and has only recently been described Case report. A 5 year-old girl presented with widespread erythematous, predominantly telangiectasic, congenital and acquired macules when she was one year-old. Her history was marked by right temporal cerebral hemorrhage at the age of 4, revealing a right temporal cavernoma-like vascular malformation. The familial history of telangiectasic macules and clinical and histological examination led to the diagnosis of benign hereditary telangiectasia. Discussion. This case report raises doubt on the exclusively cutaneous nature of benign hereditary telangiectasic-type capillary malformations. Moreover, it illustrates the possibility of a particular clinical form of this affection, associating classical telangiectasia and post-wine stain-type macules. The recent localization of the locus implied in this affection in 5q14 in the same chromosomic space as the CMCi locus incriminated in familial capillary malformations, suggests that these two affections are in fact phenotype variations of a single and same clinical entity.
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