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Jurnal Oftalmologi Indonesia

ISSN 1693-2587

Vol. 5 / No. 3 / Published : 2007-12

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Original Article :

Dandy walker syndrome in 7 month-old boy

Author :

  1. Dewi Rosalina*1
  2. Lina Puspita Hutasoit*2
  3. Uyik Unari*3
  4. Diany Yogiantoro*4
  1. Department of Ophthalmology, Medical Faculty Airlangga University, Dr.Soetomo Hospital, Surabaya
    Correspondence: Dewi Rosalina, c/o.: Departemen/SMF Ilmu Kesehatan Mata Fakuktas Kedokteran Unair/RSU Dr. Soetomo, Jl. Mayjend. Prof. Dr. Moestopo 6-8 Surabaya 60286
  2. Department of Ophthalmology, Medical Faculty Airlangga University, Dr.Soetomo Hospital, Surabaya
  3. Department of Ophthalmology, Medical Faculty Airlangga University, Dr.Soetomo Hospital, Surabaya
  4. Department of Ophthalmology, Medical Faculty Airlangga University, Dr.Soetomo Hospital, Surabaya

Abstract :

Objective:To report an unusual case of a 7 month old boy with Dandy Walker Syndrome. Method:A case report, a 7 month-old boy was referred to an ophthalmologist by a pediatrician with diagnosis Dandy Walker Syndrome. USG cranium examination revealed a presence of large cyst in the posterior fossa which extends to the fourth ventricle, and also hypoplasia of both cerebellum hemisphere. CT Scan of the head showed multiple craniocerebral anomalies which includes corpus callosum hypogenesis and Dandy Walker malformation. From ophthalmologic examination we found visual acuity of both eyes still responsive to light and environment and no sign of ptosis, sixth nerve paralysis, papilledema or optic atrophy. Result: We keep the patient under observation especially for any sign of papilledema or optic atrophy. Conclusion: Dandy Walker syndrome is a congenital abnormality that affects midline structure of the cerebellum, with hydrocephalus caused by atresia of foramen Luschka and Magendi. Increased intra cranial pressure can cause compression to the optic nerve and chiasma, and leads to papilledema and optic atrophy in chronic cases that left untreated. Therefore, besides the primary management of hydrocephalus, regular visit to ophthalmologist is necessary and awareness should be taken for any ocular abnormalities related to this syndrome, such as ptosis, sixth nerve paralysis, papilledema or optic atrophy.

Keyword :

Dandy Walker Syndrome, hydrocephalus, optic atrophy,


References :



       


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