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Wiedemann's dysmelia syndrome
Also known as:
Lenz' syndrome
Wiedemann's syndrome I
Wiedemann-Lenz syndrome
Associated persons:
Widukind Lenz
Hans-Rudolf Wiedemann
Description:
An embryopathic malformation syndrome occurring in newborns of mothers who took thalidomide during pregnancy. It is characterised mainly by severe defects of the limbs. Abnormalities are variable and depend on the timing at which the drug was given, the most critical period being between 37th and 50th day after latest menstrual period. The limb deformities vary from amelia to minor thumb anomalies. Deformities prevalent in upper extremities; involvement bilateral but generally asymmetric. Associated anomalies include cranial malformation, hydrocephalus, meningomyelocele, macrophthalmia or anophthalmia, saddle nose, cleft palate, webbed neck, capillary haemangioma of the face, cardiovascular anomalies, and malformation of gastrointestinal and genitourinary tracts. No sex preference. Usually normal intelligence.
Frequent during the years 1958 to 1963. It has rarely been observed after thalidomide drugs were prohibited.
Bibliography:
- A. Weidenbach:
Total Phocomelia.
Zentralblatt für Gynäkologie, Leipzig, 1956, 81: 2048-2052.
- H. R. Wiedemann:
Hinweis auf eine derzeitige Häufung hypo- und aplastischer Fehlbildungen der Gliedmassen.
Die Medizinische Welt, Stuttgart, 1961, 2: 1863-1866.
- W. Lenz, K. Knapp:
Die Thalidomid-Embryopathie.
Deutsche Medizinische Wochenschrift, Stuttgart, 1962, 87: 1232-1242.
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