LA56

ICD-11 MMS 2026-01

Pierre Robin syndrome

Description

Pierre-Robin syndrome (or Pierre-Robin sequence) is characterised by triad of orofacial morphological anomalies consisting of retrognathism, glossoptosis and a posterior median velopalatal cleft. This condition is referred to as a sequence because the posterior cleft palate is a secondary defect associated with abnormal mandibular development: mandibular hypoplasia occurring early in gestation causes the tongue to be maintained high-up in the oral cavity, preventing fusion of the palatal shelves.

Index terms

  • Pierre Robin syndrome
  • Pierre Robin sequence

Exclusions from above levels

  • http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

  • LA30 – Structural developmental anomalies of teeth and periodontal tissues
  • LA31 – Structural developmental anomalies of mouth or tongue
  • LA50 – Congenital velopharyngeal incompetence
  • LA51 – Facial clefts
  • LA52 – Facial asymmetry
  • LA53 – Macrocheilia
  • LA54 – Microcheilia
  • LA55 – Compression facies
  • LA5Y – Other specified structural developmental anomalies of the face
  • LA5Z – Structural developmental anomalies of the face, unspecified

Foundation entity

Pierre Robin syndrome – http://id.who.int/icd/entity/136361299

Part of: 20: Developmental anomalies