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Osteogenesis imperfecta
860
THE A~IERICAN JOURNAL OJ!' OBSTETRICS AND GYNECOLOGY
lnt of women hnve cnnw to the Vanderbilt Clinic suffering from the spontaneous n1enop:.m~e. I religiously kepp away :fr·om using it in artificial ra~es, because I hrlieve that the taking of medicine suggests ilhwss. Hut in the spontaneon,; ea:;Ps who already haYc their discomforts (about 130 odd), large amounts of dry whole ovarian substaw:c have l>ern used. The distribution is such that it seems lrng·ely to nlimina.te suggestion. Tho hot ftashe's have hecm, I think, h0lped, but I agree with Dr. Frank that this matter ,, f suggestion is so suhth~ and ;o rli f · Jicnlt to appraise that we must accept thn present commercial pr0parations with eaution. Atropine we have used spasmodically, but because we became &o impressed with the psychic fartors, we gave up the use of all drugs in these cases.
DR. J. H. TELFAm. reported a ease of Osteogenesis Imperfecta noted in a baby born at term in a gravida ii, age 20, with a normal first labor and infant. 1'he present pregnancy "·as nneventful awl the lahor lasted about twenty-three hours. The baby was born in poor eondition, cry weak, and hody disfigured. The hones of the skull were for the most pati. eartilagiuous and very brittle, breaking with extreme ease on slight pressure. Fontanelles and sutures were markedly enlarged and separated. The eyes were slit-like and the features approached the mongoloid type. The verh'brae appeared to be intact but the ribs presented numerous fractures and these likewise involved the long bones of both extremities, resulting in a marked shortening. The baby lived only three hours. 'l'he Wassermann test on mother was negative. Tho autopsy showed characteristic rvidences of fwhondroplasia. The body cavitiPs werr <'RAentially normal. A sectioct of the fc,mur showed the shaft to he of almost paper th>inn08s with a large amount of extravasatec1 blood. 'l'he epiphyses were nomml, although the line was red anc1 congestetl. DISCUSSION DR. HAROLD BAILEY.-I think there is no question that the case presented is typical of the condition. Ballantyne diYides the group into several types: A, B, C, and D, and this specimen woulil possibly correspond to type A, chon· dromalacia micromelia or, more simply, achondropln.sia. As n. matter of fad. abuut a do zAn terms have been applied to this con clition; the original one was eongenital rickets, and cert8Jinly this specimen looks as though it might be of that nature. This radiograph i~ very intrresting, l>P<·ause it shows the peculiar heail dPformity characteristic of th('se -dwarfs. Thl' hmul is flattened from above downward anrl the jaw stands forward, thus giving a curious Rinking in tho rE>gion of the g-labella. This head c1eformity is due to a shortened base of the occipital bono in front of the foramen magnum, RO that the opening is far forward in the base of. the skull. It is comparable to the formation existing in the bulldog. 'l'he skulls of this animal in Stockard's laboratory show plainly that the area in front of the foramen is very much shortened and is perhaps only about one-fourth the length in any other clog. The deformity is inheritable. It is exceedingly difficult to bring up these dwarfs and they usually ·die during pregnancy, labor or shortly after birth. When they do come to adult life they are the strong dwarfs that we see ,jn the cireus and elsewhe,re. The condition pertain!' to teratology rather than to nutritional disturbances of the mother.
THE A~IERICAN JOURNAL OJ!' OBSTETRICS AND GYNECOLOGY
lnt of women hnve cnnw to the Vanderbilt Clinic suffering from the spontaneous n1enop:.m~e. I religiously kepp away :fr·om using it in artificial ra~es, because I hrlieve that the taking of medicine suggests ilhwss. Hut in the spontaneon,; ea:;Ps who already haYc their discomforts (about 130 odd), large amounts of dry whole ovarian substaw:c have l>ern used. The distribution is such that it seems lrng·ely to nlimina.te suggestion. Tho hot ftashe's have hecm, I think, h0lped, but I agree with Dr. Frank that this matter ,, f suggestion is so suhth~ and ;o rli f · Jicnlt to appraise that we must accept thn present commercial pr0parations with eaution. Atropine we have used spasmodically, but because we became &o impressed with the psychic fartors, we gave up the use of all drugs in these cases.
DR. J. H. TELFAm. reported a ease of Osteogenesis Imperfecta noted in a baby born at term in a gravida ii, age 20, with a normal first labor and infant. 1'he present pregnancy "·as nneventful awl the lahor lasted about twenty-three hours. The baby was born in poor eondition, cry weak, and hody disfigured. The hones of the skull were for the most pati. eartilagiuous and very brittle, breaking with extreme ease on slight pressure. Fontanelles and sutures were markedly enlarged and separated. The eyes were slit-like and the features approached the mongoloid type. The verh'brae appeared to be intact but the ribs presented numerous fractures and these likewise involved the long bones of both extremities, resulting in a marked shortening. The baby lived only three hours. 'l'he Wassermann test on mother was negative. Tho autopsy showed characteristic rvidences of fwhondroplasia. The body cavitiPs werr <'RAentially normal. A sectioct of the fc,mur showed the shaft to he of almost paper th>inn08s with a large amount of extravasatec1 blood. 'l'he epiphyses were nomml, although the line was red anc1 congestetl. DISCUSSION DR. HAROLD BAILEY.-I think there is no question that the case presented is typical of the condition. Ballantyne diYides the group into several types: A, B, C, and D, and this specimen woulil possibly correspond to type A, chon· dromalacia micromelia or, more simply, achondropln.sia. As n. matter of fad. abuut a do zAn terms have been applied to this con clition; the original one was eongenital rickets, and cert8Jinly this specimen looks as though it might be of that nature. This radiograph i~ very intrresting, l>P<·ause it shows the peculiar heail dPformity characteristic of th('se -dwarfs. Thl' hmul is flattened from above downward anrl the jaw stands forward, thus giving a curious Rinking in tho rE>gion of the g-labella. This head c1eformity is due to a shortened base of the occipital bono in front of the foramen magnum, RO that the opening is far forward in the base of. the skull. It is comparable to the formation existing in the bulldog. 'l'he skulls of this animal in Stockard's laboratory show plainly that the area in front of the foramen is very much shortened and is perhaps only about one-fourth the length in any other clog. The deformity is inheritable. It is exceedingly difficult to bring up these dwarfs and they usually ·die during pregnancy, labor or shortly after birth. When they do come to adult life they are the strong dwarfs that we see ,jn the cireus and elsewhe,re. The condition pertain!' to teratology rather than to nutritional disturbances of the mother.