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一名患有蓝色橡皮疱痣综合征的儿童在口服普萘洛尔治疗期间出现结膜下出血。

Subconjunctival Hemorrhage in a Child with the Blue Rubber Bleb Nevus Syndrome on Treatment with Oral Propranolol.

作者信息

De Loecker Karen, Labarque Veerle, Seynaeve Hilde, Casteels Ingele

机构信息

Department of Ophthalmology, University Hospitals Leuven, Leuven, Belgium.

Department Pediatric Hematology, University Hospitals Leuven, Leuven, Belgium.

出版信息

Case Rep Ophthalmol. 2021 May 19;12(2):451-456. doi: 10.1159/000513504. eCollection 2021 May-Aug.

Abstract

Blue rubber bleb nevus syndrome (BRBNS) is a rare syndrome characterized by venous malformations of mostly skin and gastrointestinal tract. Patients present with multiple venous malformations in various organs including liver, spleen, heart, eye, and central nervous system. Few ophthalmological cases have been published in literature and at present, there are no clear guidelines on the treatment of eye hemorrhages associated with the BRBNS. We report a 3-year-old boy with the BRBNS who developed a spontaneous progressive enlarging subconjunctival hemorrhage in the left eye despite being treated with oral propranolol. The subconjunctival hemorrhage was caused by an underlying conjunctival vascular malformation. With topical treatment with timolol maleate 0.5% once a day in the affected eye, the lesion regressed completely after 4 months. This child represents the first case of the BRBNS associated with a subconjunctival progressive bleeding necessitating topical treatment despite oral propranolol effectively controlling the cutaneous lesions. We recommend ophthalmic screening of patients with BRBNS in early childhood. For patients with BRBNS-related subconjunctival vascular lesions with subsequent hemorrhage, treatment with a topical β-blocker may be an efficient and harmless treatment option.

摘要

蓝色橡皮疱痣综合征(BRBNS)是一种罕见的综合征,其特征主要为皮肤和胃肠道的静脉畸形。患者在包括肝脏、脾脏、心脏、眼睛和中枢神经系统等多个器官出现多发性静脉畸形。文献中报道的眼科病例较少,目前对于与BRBNS相关的眼部出血尚无明确的治疗指南。我们报告了一名3岁患BRBNS的男孩,尽管接受了口服普萘洛尔治疗,但左眼仍出现了自发性进行性增大的结膜下出血。结膜下出血是由潜在的结膜血管畸形引起的。患眼每天使用一次0.5%的马来酸噻吗洛尔局部治疗,4个月后病变完全消退。该患儿是首例尽管口服普萘洛尔有效控制了皮肤病变,但仍出现结膜下进行性出血且需要局部治疗的BRBNS病例。我们建议在儿童早期对BRBNS患者进行眼科筛查。对于患有与BRBNS相关的结膜下血管病变并随后出血的患者,局部使用β受体阻滞剂治疗可能是一种有效且无害的治疗选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0143/8215979/2eebdea265d0/cop-0012-0451-g01.jpg

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