Conditions / Syndrome

Stromme syndrome

info · Syndrome · ICD-10: Q87.8

A primary ciliary dyskinesia that is characterized by autosomal recessive inheritance and ciliopathy with some type of intestinal atresia, variable ocular abnormalities, microcephaly, and has_material_basis_in compound heterozygous mutation in the CENPF gene o

A primary ciliary dyskinesia that is characterized by autosomal recessive inheritance and ciliopathy with some type of intestinal atresia, variable ocular abnormalities, microcephaly, and has_material_basis_in compound heterozygous mutation in the CENPF gene on chromosome 1q41.

Signs and symptoms

  • Jejunal atresia
  • Myopathy
  • Short columella
  • Agenesis of corpus callosum
  • Microcornea
  • Retinal vascular tortuosity
  • Cataract
  • Hypertelorism
  • Iris coloboma
  • Hydrocephalus

Also known as: CILD31; apple peel syndrome with microcephaly and ocular anomalies; jejunal atresia with microcephaly and ocular anomalies; lethal fetal brain malformation-duodenal atresia-bilateral renal hypoplasia syndrome; primary ciliary dyskinesia 31