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Ligneous conjunctivitis and holoprosencephaly –a rare association

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Abstract (2. Language): 
Ligneous conjunctivitis is a rare form of bilateral, chronic, recurrent disease in which thick membranes form on the palpebral conjunctiva and other mucosal sites. Ligneous conjunctivitis has been reported to occur occasionally in association with congenital occlusive hydrocephalus. We report the association of ligneous conjunctivitis with holoprosencephaly, a rare cerebral malformation resulting from incomplete division of the embryonic forebrain which is not well known. A five month old infant with developmental delay presented to us with thickened woody membranes on the palpebral conjunctivae of both eyes. Neuroimaging studies revealed fusion of the frontal horns of both lateral ventricles, absent septum pellucidum and thin corpus callosum- suggestive of lobar holoprosencephaly. A clinical diagnosis of ligneous conjunctivitis was made and the infant was managed medically with topical heparin and fresh frozen plasma. At six months follow up there has been significant resolution of membranes and no recurrences have been noted.
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