" /> Short rib-polydactyly syndrome - CISMeF





ICD-11 code : LD24.B0;

Preferred Label : Short rib-polydactyly syndrome;

ICD-11 definition : Short rib-polydactyly syndromes are a group of bone malformations characterized by a narrow thorax and polydactyly (usually preaxial). Prevalence as a group is unknown. The group is heterogeneous and includes Jeune syndrome and Ellis Van Creveld syndrome, neither of which are lethal, together with lethal chondrodysplasias: Saldino-Noonan (type 1), Majewski (type 2), Verma-Naumoff (type 3) and Beemer-Langer (Type 4).;

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Short rib-polydactyly syndromes are a group of bone malformations characterized by a narrow thorax and polydactyly (usually preaxial). Prevalence as a group is unknown. The group is heterogeneous and includes Jeune syndrome and Ellis Van Creveld syndrome, neither of which are lethal, together with lethal chondrodysplasias: Saldino-Noonan (type 1), Majewski (type 2), Verma-Naumoff (type 3) and Beemer-Langer (Type 4).

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25/05/2025


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