CHONDRODYSTROPHY 485 







set forth that it was concerned with an affection of the epiphysial cartilages 

 that sets in in fetal life. Kaufmann in his known monograph lines himself 

 against Virchow's explanation, Kaufmann and later Diederle have done away 

 for good with the conception of a thyrogenic disturbance, until Kaufmann 

 originated the name chondrodystrophy, by which the condition is known in 

 Germany. Kassowitz calls the disease " Mikromelie." But this designa- 

 tion is not precise, as. ihere are different kinds of micromelie (shortness of the 

 extremities) that have nothing to do with chondrodystrophy (osteogenesis 

 imperfecta [Vrolik], phokomelie, etc.), and chondrodystrophy constitutes 

 only one of these types. I refer the reader to the excellent dissertation of 

 Sieger t. 



Definition. Chondrodystrophy is characterized by a growth disturbance 

 of the cartilages at the ossification boundary of the cartilages, especially, those 

 of the long bones setting in the earliest fetal life. Microscopically there is 

 found, according to Kaufmann, either mucoid softening of the cartilages 

 (malacic form) , or only cessation of growth (hypoplastic form) , or even con- 

 siderable, but entirely disorderly, proliferation, which takes place in all 

 directions instead of iri the longitudinal direction, and thus leads to a mod- 

 erate swelling out of the epiphyses (hyper plastic form). In older individuals, 

 the cartilaginous zone of proliferation is bounded at the periphery by strips 

 of connective tissue (Diederle) . Thereby the periosteal bone formation is not 

 inhibited, and may even be excessive, as is also the formation of bone nuclei 

 in the epiphyses, which may even show acceleration. The disturbance affects 

 also the bones of (he skull that are preformed in cartilage, and the vertebral 

 column, while the growth of the bones preformed in membrane is not inhibited. 



Symptomatology. From this growth disturbance there results a skeletal 

 formation characterized by the following points: The base of the skull is 

 markedly shortened by the inhibition of growth and the premature synos- 

 tosis of the os tribasilare occipitalis and the os basilare occipitalis, as first 

 described by Virchow. There is thus brought about a marked retraction 

 of the root of the nose, such as is not observed in any other growth dis- 

 turbance. There are, however, rare cases of chondrodystrophy without 

 this retraction. Eichholz has described three such cases belonging to one 

 family. Then again the retraction of the root of the nose may exist, without 

 there having taken place a coalescence at the synchondrosis (a case of E. 

 Langenbach). The clivus is shortened I here am following the description 

 of Breus and Kolisko the foramen magnum is very small; the base of the 

 skull is usually supernormally large; there occur also hydrocephalic skulls in 

 which ossification is rather retarded so that the f ontanelles persist abnormally 

 long. The form of the sella turcica may be quite abnormal (confer Ob- 

 servation LXIII). If the subject lives after the period of childhood, the 

 development of the brain and the intelligence is normal. The upper jaws 

 are broad, spread far apart, and the upper jaws project markedly. Dentition 



