Conditions / Syndrome

Kabuki syndrome

info · Syndrome · ICD-10: Q89.81

A syndrome characterized by multiple congenital anomalies and mental retardation. Other characteristics include a peculiar facial gestalt, short stature, skeletal and visceral abnormalities, cardiac anomalies, and immunological defects.

Signs and symptoms

  • Focal impaired awareness seizure
  • Renal insufficiency
  • Short nose
  • Type II pneumocyte hyperplasia
  • Lobar holoprosencephaly
  • Finger clinodactyly
  • Large earlobe
  • Downslanted palpebral fissures
  • Midface retrusion
  • Bilateral renal hypoplasia

Also known as: KMS; Kabuki make up syndrome; Niikawa-Kuroki syndrome