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Research Paper | Medicine Science | India | Volume 3 Issue 9, September 2014

Dysencephalia Splanchnocystica

Dr. Muruganantham Balagurunathan ^[2] | Dr. Ramesh Samikannu | Dr. Ramanathan Ramanathan ^[2]

Abstract: 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.

Keywords: Dysencephalia Splanchnocystica, Occipital encephalomeningocele, Postaxial polydactyly

Edition: Volume 3 Issue 9, September 2014,

Pages: 2301 - 2302