ICD-11 code : LA56;
Preferred Label : Pierre Robin syndrome;
ICD-11 definition : 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.;
ICD-11 synonym : Pierre Robin sequence;
Origin ID : 136361299;
Currated CISMeF NLP mapping
- ICD-10 Mapping
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.
