Aim: Since the thalidomide-catastrophe in the 50’s and 60’s the sawareness of children with limb defects has become more important in the population. An accurate registration of limb defects did not exist in Germany till now. Besides the incidence of limb defects, the aetiology of limb defects is a very important question.

Method: Like the ESPED-Model (Documentation of rare pediatric diseasea in Germany) we send every three months a letter to 1079 gynaecological clinics to ask the number of live births and still births and the number of limb defects. If there are limb defects announced, a second letter is send to ask details about pregnancy, birth and family. The limb defects are registered after the ICD-10-classification.

Results: Time of registration: 18 months (April 2000 – September 2001).Number of all registered births: 411656, number of live births with limb defects: 493 (0,12%), number of still births with limb defects: 16 (1,2%), minor limb defects like polydactylie (26,3%) are more often than major defects of the tibia (1,3%) or of the fibula (2,3%), hereditary in 17%, multiple anomalies in 24%, no correlation to the profession of the parents or their age (mother: 29, father 32), no correlation to nicotine abuses, nothing special concerned pregnancy (amniocentese in 5,7 %; oligohydramnie in 1%, polyhy-dramnie in 1%, etc.) and birth (normal birth in 67%, sectio in 28%, etc.). The data will be update regularly.

Conclusion: It is very important to continue the registration of limb defects in the whole of Germany to detemine the incidence of the different types of limb defects and to define the aetiology of these limb defects.

We analysed the cases of lumbar kyphosis in 151 (21%) of a series of 719 patients with myelomeningocele. Three different types were distinguished: paralytic, sharp-angled and congenital. In a cross-sectional and partly longitudinal study the size and magnitude of the kyphosis, the apex of the curve and the level of paralysis of each group were recorded and statistically analysed.

Paralytic kyphosis (less than 90° at birth) occurred in 44.4% and increased linearly during further development. Sharp-angled kyphosis (90° or more at birth) was present in 38.4% and also showed a linear progression. In both types, progression seemed to depend also on the level of paralysis. Congenital kyphosis occurred in 13.9% and we could find no significant factor which correlated with progression.