Conditions / Syndrome

Meckel syndrome 1

info · Syndrome · ICD-10: Q61.9

A Meckel syndrome that has_material_basis_in an autosomal recessive mutation of the MKS1 gene on chromosome 17q22.

Signs and symptoms

  • Camptodactyly of finger
  • Hypotonia
  • Smooth philtrum
  • Molar tooth sign on MRI
  • Delayed ability to sit
  • Polycystic kidney dysplasia
  • Rotary nystagmus
  • Absent speech
  • Dilated fourth ventricle
  • Global developmental delay

Also known as: MKS1; Meckel-Gruber syndrome, type 1