Meckel Gruber Syndrome: a rare case report


  • Manish Verma Department of Pediatrics, JLN Medical College, Ajmer, Rajasthan, India
  • Shruti Sharma Department of Pediatrics, JLN Medical College, Ajmer, Rajasthan, India
  • Kiran Suthar Department of Pediatrics, RNT Medical College, Udaipur, Rajasthan, India
  • Beena Thada Department of Anaesthesia, JLN Medical College, Ajmer, Rajasthan, India



Occipital encephalocele, Polysystic kidney, Polydactyly, Pulmonary hypoplasia


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.


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