Facultad de Medicina, Universidad de Antioquia, Medellín, Colombia.
Facultad de Medicina, Universidad de Antioquia, Medellín, Colombia.
Arch Soc Esp Oftalmol (Engl Ed). 2022 Jan;97(1):44-47. doi: 10.1016/j.oftale.2020.11.018. Epub 2021 Nov 14.
The most cases of persistence hyperplastic primary vitreous (PHPV) are unilateral and sporadic, however, bilateral presentation could be present in a small number of patients, in whom other genetic diseases must be ruled out. We describe a case of a 2 months child with bilateral persistence hyperplastic primary vitreous confirmed by ultrasound. In addition, with neurodevelopmental defects, microcephaly, facial dimorphism, axial hypotonia, and without brain abnormalities on MRI, in whom a de novo mutation of the CTNNB1 gene was found during the genetic study, which explains the findings.
大多数永存原始玻璃体增生症 (PHPV) 为单侧和散发性,但少数患者也可能为双侧,在此情况下必须排除其他遗传疾病。我们描述了一例 2 个月大的双侧永存原始玻璃体增生症患儿,该病例经超声检查确诊。此外,患儿还存在神经发育缺陷、小头畸形、面部畸形、轴向张力减退,且 MRI 未见脑部异常,遗传学研究发现 CTNNB1 基因存在新生突变,这可解释患儿的所有临床表现。