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双侧永存原始玻璃体增生症

Bilateral persistent hyperplastic primary vitreous.

作者信息

Sanghvi D A, Sanghvi C A, Purandare N C

机构信息

Department of Radiology, Tata Memorial Hospital, Mumbai, India.

出版信息

Australas Radiol. 2005 Feb;49(1):72-4. doi: 10.1111/j.1440-1673.2005.01402.x.

Abstract

We report a case of bilateral persistent hyperplastic primary vitreous (PHPV) in a 5-month-old infant who presented with bilateral leukokoria. The child was referred for ocular ultrasound with a clinical suspicion of retinoblastoma. Grey-scale evaluation revealed an echogenic band in the posterior segment of the left globe extending from the posterior surface of the lens capsule to the optic disc. Doppler examination revealed the presence of blood flow in the band. Ultrasound assessment of the contralateral globe showed an elevated mass of echogenic tissue in the posterior segment, in contact with the optic disc. Most cases of PHPV are sporadic and unilateral. Bilateral PHPV is rare. In a study by Pollard of 83 cases, only two patients (2.4%) had bilateral PHPV. The imaging features in this case point toward the diagnosis of bilateral PHPV. We suggest that this entity, although rare, should be considered in the differential diagnosis while evaluating bilateral leukokoria.

摘要

我们报告了一例5个月大的双侧永存原始玻璃体增生症(PHPV)婴儿病例,该婴儿表现为双侧白瞳症。因临床怀疑视网膜母细胞瘤,该患儿被转诊接受眼部超声检查。灰阶评估显示,左眼后段有一条回声带,从晶状体囊后表面延伸至视盘。多普勒检查显示该带内有血流信号。对侧眼球的超声评估显示,后段有一个回声增强的组织团块,与视盘相连。大多数PHPV病例为散发性且单侧发病。双侧PHPV较为罕见。在Pollard对83例病例的研究中,只有2例患者(2.4%)为双侧PHPV。该病例的影像学特征指向双侧PHPV的诊断。我们建议,尽管这种情况罕见,但在评估双侧白瞳症时的鉴别诊断中应考虑到这一疾病。

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