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Dysencephalia Splanchnocystica

Journal: International Journal of Science and Research (IJSR) (Vol.3, No. 9)

Publication Date:

Authors : ; ; ;

Page : 2301-2302

Keywords : Dysencephalia Splanchnocystica; Occipital encephalomeningocele; Postaxial polydactyly;

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Dysencephalia Splanchnocystica also called as Meckel-Gruber syndrome is an autosomal recessive disorder1. It is characterized by a traid of occipital encephalocele, large polycystic kidneys, and postaxial polydactyly2. The major diagnostic criteria of MGS include at least 2 of these 3 classic manifestations, occipital encephalocele, cystic renal dysplasia, and polydactyly found in 90 %, 100 % and 83.3 % respectively 3. It is a rare syndrome with highest incidence in Gujarati Indians and Finnish population. We are reporting a case of Dysencephalia Splanchnocystica in a non Gujarati Indian from a municipal town, Sirkazhi in Nagapattinam district in Tamil Nadu, India.

Last modified: 2021-06-30 21:07:44