DOI: http://dx.doi.org/10.18203/2349-3291.ijcp20175598

Meckel Gruber Syndrome: a rare case report

Manish Verma, Shruti Sharma, Kiran Suthar, Beena Thada

Abstract


Meckel gruber syndrome or dysencephalia splanchnocystica, is a rare autosomal recessive disorder caused by failure of mesodermal induction. Worldwide incidence of MGS is 1 per 13,500-140,000 live births. It is characterized by triad of occipital Meningoencephalocele, polycystic kidneys and post-axial polydactyly. Most fetuses affected with this syndrome die before birth due to oligohydramnios, renal failure or pulmonary hypoplasia. We report a rare case of MGS who delivered live at birth with classical features.


Keywords


Occipital encephalocele, Polysystic kidney, Polydactyly, Pulmonary hypoplasia

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References


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