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LD2F.13 Meckel-Gruber syndrome

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

Meckel syndrome (MKS) is a monogenic disease characterised by a combination of renal cysts and variably associated features, including developmental anomalies of the central nervous system (usually occipital encephalocele), hepatic ductal dysplasia and cysts, and polydactyly, and a lethal course, with death occurring in the perinatal period.

synonyms

• Meckel-Gruber syndrome
• Meckel syndrome

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