PubMed · 14928500
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F WAGNER. 1951. [Persistent pupillary membrane].. https://pubmed.ncbi.nlm.nih.gov/14928500/
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Focal dermal hypoplasia or Goltz syndrome is a rare genodermatosis, characterized by multiple abnormalities of ectodermal and mesodermal origin. It is found predominantly in females and is characterized by hypoplasia of skin and papillomas. Three cases of focal dermal hypoplasia in infancy with unusual inheritance patterns are reported. Cutaneous features were atrophic reticulated scars involving the trunk and extremities following the lines of Blaschko. Papillomas were present on the genitalia and in a periorificial distribution. Skeletal abnormalities included syndactyly, polydactyly and lobster claw deformities. Ophthalmological examination revealed strabismus and retinal colobomas.
AIMS: To report outcomes in cases of persistent hyperplastic primary vitreous (PHPV) and examine factors predictive of visual acuity in patients selected for surgery. METHODS: Retrospective case series of 55 eyes in 50 patients presenting between 1990 and 2001 at the Children's Hospital, Westmead. 33 eyes underwent surgery aimed at visual rehabilitation. RESULTS: In surgical patients, median age at surgery was 77 days, and median age at final follow up was 28 months. Six (18%) achieved a visual acuity at final follow up of 6/60 or better and eight (24%) achieved acuity of less than 6/60 to counting finger vision inclusive. Those undergoing surgery before 77 days were approximately 13 times more likely to obtain a visual acuity of counting fingers or better than those operated later (p = 0.01). Neither posterior segment PHPV nor post-surgical glaucoma was a significant predictor of outcome in this selected group. CONCLUSION: Early surgery aimed at rehabilitation markedly improves the likelihood of useful visual outcome in selected eyes with PHPV.
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