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Study of the Genesis of Persistent Fetal Vasculature through Clinical Observations of Two Pediatric Patients
Author(s) -
Dong BaiXia,
Wang Long,
Hong Ying,
Chen Huijin,
Ma Zhizhong
Publication year - 2016
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.30.1_supplement.lb26
Subject(s) - iris (biosensor) , medicine , cornea , pathology , cyst , fetus , anatomy , hamartoma , ophthalmology , biology , pregnancy , genetics , computer security , computer science , biometrics
Purpose Persistent fetal vasculature (PFV) is a rare congenital disease caused by fetal blood vessels remaining in the eyes, with various residues and persistent hyperplasia. A series of observations in 2 typical PFV were conducted to better understand fatal development in normal eyes and to illustrate involved controversies. Methods Case One was an 11‐month girl who was found to have leukocoria accompanied by black spherical goiter in the right eye approximately 50 days after birth. Case Two was a 3‐year old girl who was found to have leukokoria at birth. IN both cases, image data prior to surgery and series of photographs during the procedures were carefully colliaected and reviewed comparatively with surgical video record. Novel tissue samples obtained from surgeries were observed histopathologically. Comprehensive analysis was carried out with literature review, Results Majority of the abnormal signs were concomitant with those that Goldberg described in Jackson Memorial Lecture emphasizing the congenital anomalies caused by different degrees of PFV, which seemingly existed in isolation, but were intrinsically syntrophic. Pigment epithelium layer of iris detoured over stoma through pupil to adhere with the back surface of cornea, which manifested as an iris cyst. Trace of fatal development supplied a hard evidence that corneal endothelium was originated from neuroectodermal cells. Network profile of persistent papillary membrane tended to be formed by anterior tunica vasculosa lentis while the remnants were coated by iris stromal tissues which were believed to be originated from neural crest cells. Conclusions Close relation between corneal endothelium and pigment epithelium of iris at early stage of fatal development was evidenced by clinical observations in the two pediatric cases. Corneal endothelium and Descemet's membrane were most possibly originated from neuroectodermal and spread with the manner migrated from centre to periphery. Neural crest cells went forward to center from periphery via the staggered gap between developing endothelium and anterior surface of optic cup to form stroma of iris. Mechanism of persistent pupil membrane formation could be a result of mutualism between anterior tunica vasculosa lentis and stroma of iris.