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I read with interest the case reported by Lauer et al.¹ In such cases it is important to obtain a careful, detailed, and accurate history of any drug abuse by the mother during pregnancy, because persistent hyperplastic primary vitreous (PHPV) may be associated with maternal cocaine² and lysergic acid diethylamide³ use. In addition, it is extremely important to examine these cases thoroughly, because many ocular and systemic abnormalities are associated with PHPV. Ocular associations include microphthalmos, megalocornea, Peters anomaly, cataract colobomas, retinal detachment with retinal dysplasia,⁴ intraocular cartilage,³ and homonymous hemianopia.⁵ Important systemic associations include hemiparesis, impaired coordination, ataxia, hypotonia, spastic quadriplegia, microcephaly, and deafness,⁵ as well as arachnoid cysts, syndactyly, congenital heart anomalies, pulmonary atresia, asplenia,⁶ and Aicardi syndrome.⁷

Ajay Tripathi, FRCS(Edin), FRCS(Glasg)
Liverpool, England

1. Lauer AK, Balish MJ, Palmer EA. Persistent hyperplastic primary vitreous associated with septo-optic-pituitary dysplasia and schizencephaly. Arch Ophthalmol. 2000;118:578-580. FREE FULL TEXT 2. Teske MP, Trese MT. Retinopathy of prematurity-like fundus and persistent hyperplastic primary vitreous asociated with maternal cocaine use. Am J Ophthalmol. 1987;103:719-720. ISI | PUBMED 3. Chan CC, Fishman M, Egbert PR. Multiple ocular anomalies associated with maternal LSD ingestion. Arch Ophthalmol. 1978;96:282-284. ABSTRACT 4. Pagon RA, Clarren SK, Milam DF Jr, Hendrickson AE. Autosomal recessive eye and brain anomalies: Warburg syndrome. J Pediatr. 1983;102:542-546. FULL TEXT | ISI | PUBMED 5. Marshman WE, Jan JE, Lyons CJ. Neurological abnormalities associated with persistent hyperplastic primary vitreous. Can J Ophthalmol. 1999;34:17-22. ISI | PUBMED 6. Yamada K, Ozeki H, Ieda M, Shirai S, Majima A. Four cases of persistent hyperplastic primary vitreous. Nippon Ganka Gakkai Zasshi. 1997;101:826-831. PUBMED 7. Weissgold DJ, Maguire AM, Kalin NS, Hertle RW. Persistent hyperplastic primary vitreous in association with Aicardi syndrome. J Pediatr Ophthalmol Strabismus. 1995;32:52-54. ISI | PUBMED

In reply

Dr Tripathi's comments are greatly appreciated. As our report suggests, maternal exposure to a teratogen is indeed a likely etiology for this . . . [Full Text of this Article]