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Abstract：
BACKGROUND: Persistent hyperplastic primary vitreous (PHPV), also known as persistent fetal vasculature (PFV), is a clinical entity that traditionally presents with leukocoria, microphthalmia, retinal dysplasia, or eyeball shrinkage which is associated with poor vision. However, there is a dearth of literature on cases of PHPV in adulthood or with asymptomatic occurrence. This report presents the clinical and pathological findings of a non-typical PHPV case and discuss the current knowledge for this condition.
METHODS: A 68-year-old healthy male was referred to our outpatient department for evaluation of age-related cataract without other visual symptoms. Preoperative fundus examination occasionally detected an isolated stalk-like band extending to the posterior pole of the eye with normal central vitreous and retina. Other ocular examinations including b-mode ultrasonography, optical coherence tomography did not unveil any abnormalities, which caused diagnostic uncertainty. We referred to cataract surgery along with histopathological study, that revealed characteristics of PHPV including fibrous connective tissues mainly composed of fibrocyte proliferation and a very few capillary vessels. Thereafter, a definitive diagnosis of non-typical PHPV was established.
CONCLUSIONS: Our case is unique due to it was not discovered until adulthood, presence with only age-related cataract, and accompanied with normal central vitreous and retina. Histopathological explorations lead to an accurate diagnosis of the condition. Those results broaden the phenotype spectrums of PHPV and further provide clinical clues for the cognition of the disease.
METHODS: A 68-year-old healthy male was referred to our outpatient department for evaluation of age-related cataract without other visual symptoms. Preoperative fundus examination occasionally detected an isolated stalk-like band extending to the posterior pole of the eye with normal central vitreous and retina. Other ocular examinations including b-mode ultrasonography, optical coherence tomography did not unveil any abnormalities, which caused diagnostic uncertainty. We referred to cataract surgery along with histopathological study, that revealed characteristics of PHPV including fibrous connective tissues mainly composed of fibrocyte proliferation and a very few capillary vessels. Thereafter, a definitive diagnosis of non-typical PHPV was established.
CONCLUSIONS: Our case is unique due to it was not discovered until adulthood, presence with only age-related cataract, and accompanied with normal central vitreous and retina. Histopathological explorations lead to an accurate diagnosis of the condition. Those results broaden the phenotype spectrums of PHPV and further provide clinical clues for the cognition of the disease.
摘要：
背景：原发性玻璃体持续增生（PHPV），也称为持续性胎儿脉管系统（PFV），是传统上表现为白细胞增多症的临床实体，小眼症,视网膜发育不良,或与视力不良相关的眼球收缩。然而,关于成年期PHPV或无症状发生的病例的文献很少。本报告介绍了非典型PHPV病例的临床和病理发现，并讨论了这种情况的最新知识。
方法：一名68岁的健康男性被转诊到我们的门诊部进行年龄相关性白内障的评估，但没有其他视觉症状。术前眼底检查偶尔会发现一条孤立的茎状带，延伸到具有正常中央玻璃体和视网膜的眼睛后极。其他眼部检查，包括b型超声检查，光学相干断层扫描没有发现任何异常，这导致了诊断的不确定性。我们提到了白内障手术以及组织病理学研究，这揭示了PHPV的特征，包括主要由纤维细胞增殖和很少的毛细血管组成的纤维结缔组织。此后,确定了非典型PHPV的明确诊断。
结论：我们的案例是独特的，因为它直到成年后才被发现，仅存在与年龄相关的白内障，伴有正常的中央玻璃体和视网膜。组织病理学探索导致对病症的准确诊断。这些结果拓宽了PHPV的表型谱，进一步为疾病的认知提供了临床线索。
方法：一名68岁的健康男性被转诊到我们的门诊部进行年龄相关性白内障的评估，但没有其他视觉症状。术前眼底检查偶尔会发现一条孤立的茎状带，延伸到具有正常中央玻璃体和视网膜的眼睛后极。其他眼部检查，包括b型超声检查，光学相干断层扫描没有发现任何异常，这导致了诊断的不确定性。我们提到了白内障手术以及组织病理学研究，这揭示了PHPV的特征，包括主要由纤维细胞增殖和很少的毛细血管组成的纤维结缔组织。此后,确定了非典型PHPV的明确诊断。
结论：我们的案例是独特的，因为它直到成年后才被发现，仅存在与年龄相关的白内障，伴有正常的中央玻璃体和视网膜。组织病理学探索导致对病症的准确诊断。这些结果拓宽了PHPV的表型谱，进一步为疾病的认知提供了临床线索。
