9C82.2

ICD-11 MMS 2026-01

Congenital cranial dysinnervation syndrome

Description

Congenital cranial dysinnervation syndrome is a group of congenital cranial dysinnervation disorders (CCDDs). CCDDs are congenital, non-progressive conditions that result from abnormal development of the cranial nerves and their nuclei. They include both sporadic and familial disorders, and since they frequently involve the extraocular and facial musculature and result in abnormal eye movements, they often present to the ophthalmologist.

Index terms

  • Congenital cranial dysinnervation syndrome
  • congenital cranial dysinnervation disorders
  • Congenital fibrosis of extraocular muscles
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  • Congenital fibrosis of extraocular muscles 1A
  • Congenital fibrosis of extraocular muscles 1B
  • Congenital fibrosis of extraocular muscles 2
  • Congenital fibrosis of extraocular muscles 3A
  • Congenital fibrosis of extraocular muscles 3B
  • Congenital fibrosis of extraocular muscles 3C
  • Tukel syndrome
  • Duane's syndrome
  • duane retraction syndrome
  • eye retraction syndrome
  • retraction syndrome
  • stilling-turk-duane syndrome
  • Ocular motor dysinnervation disorders

Exclusions from above levels

  • 01 – Certain infectious or parasitic diseases
  • 05 – Endocrine, nutritional or metabolic diseases
  • 18 – Complications of pregnancy, childbirth and the puerperium
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  • 19 – Certain conditions originating in the perinatal period
  • 22 – Injury, poisoning or certain other consequences of external causes
  • 8A21.0 – Posterior cortical atrophy

Sibling codes

  • 9C82.0 – Progressive external ophthalmoplegia
  • 9C82.1 – Muscular dystrophy affecting extraocular muscle
  • 9C82.3 – Restrictive ophthalmopathy
  • 9C82.4 – Oculomotor apraxia
  • 9C82.Y – Other specified disorders of extraocular muscles
  • 9C82.Z – Disorders of extraocular muscles, unspecified

Foundation entity

Congenital cranial dysinnervation syndrome – http://id.who.int/icd/entity/691350959

Allowed postcoordination axes

Part of: 9C82: Disorders of extraocular muscles › 09: Diseases of the visual system