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Osteogenesis imperfecta a case discovered in utero
62
JOURNAL
OF
THE
FACULTY
OF
RADIOLOGISTS
OSTEOGENESIS IMPERFECTA A C A S E D I S C O V E R E D IN U T E R O BY G. W. E. AITKEN, A. COHEN, and P. W. VERCO ADELAIDEj AUSTRALIA
OSTEOGENESISimperfecta, named by Vrolik in 1845 , is not an uncommon disease. The characteristic feature of this condition is fragility of the bones. Cases may be grouped, according to Fairbank (1951), as pre-natal and post-natal, but there is no clear-cut distinction between the two groups, except in the date of the first fracture, which depends upon the severity of the disorder. Females are said to be more frequently affected than males. Hereditary influences are apparent in a minority of both groups, blue sclerotics may be present, and otosclerosis may occur in those who live to the third decade. Dwarfing is largely accounted for by the deformities of limb and spine : in some cases an osteomalacic element may be present in addition to the brittleness of the bones. From the radiographic appearance, Fairbank (1951) describes three types : - I. A thich bone type, the severe pre-natal cases, born with limbs which are stunted as in achondroplasia and with numerous fractures. The appearance of the bones suggests that the thickening is not due entirely to callus formation following recent or remote fractures. It may be seen up to three months after birth, but seldom later. 2. The slender, fragile bone type: This type is seen in pre-natal cases which survive more than a few months, and in all post-natal cases. The skeleton as a whole is osteoporotic ; the long bones are slender, and their cortex is thin and of deficient density. 3. Osteogenesis imperfecta cystica. This name has been applied to cases with pronounced honeycombing of bones. It is extremely rare. Brailsford (1948) records a case of osteogenesis imperfecta recognized in utero : " A radiograph of the pregnant woman two days before delivery showed an enlarged uterus with two ill-defined opacities, which were thought to be calcified glands, and the abdominal mass a large ovarian cyst. More careful examination of these radiographs revealed that the opacities showed the characters of the petromastoids, and with this identification it was possible to recognize a very faint outline of the foetal skull. The foetus was in its ninth month and was the first pregnancy. The maternal pelvis revealed areas of cancellous absorption which aroused the suspicion of osteitis fibrosa of the hyperparathyroid type." Films of the fmtus showed typical osteogenesis imperfecta. Frerking and Zink (1952) reported the instance of a 2-para, 3 ° weeks pregnant, sent for examination because of recent abdominal pain, and'sudden increase in the size of the abdomen. The f&tal head could .not be felt, nor could the fmtal heart be heard. Several anteroposterior films were obtained, and no fmtal skeleton could be seen in these films, despite repeated examination with varying techniques. In a left lateral'view a small partially calcified foetal skeleton could be seen lying within a greatly enlarged uterus. The skull and facial bones were invisible except for the petrous pyramids. The spine was fairly well shown, the scapulae were distinguishable, but neither upper extremity could be seen. All the ribs, particularly their spinal ends, were faintly shown. The bones of the lower extremity were broad and short, and one of the femora was sharply angulated at one point, suggesting that it was fractured. A film of the stillborn fmtus showed changes typical of osteogenesis imperfecta : the entire skeleton was poorly calcified, most marked in the skull and facial bones. There were many subperiosteal and pseudo-fractures of the long bones of the limbs, which were broad, short, and deformed. Frerking and Zink (i952) quote a case of this condition diagnosed in utero, reported by Danelius (1933).
OSTEOGENESIS
IMPERFECTA
We have not been able to discover any other report of osteogenesis imperfecta u t e r o i n t h e l i t e r a t u r e a v a i l a b l e to u s , t h o u g h it is k n o w n s u c h r e p o r t s e x i s t .
63 diagnosed in
CASE REPORT ItlSTORY.--Mrs. H . , gravida 4, aged 3I years, was admitted to t h e Q u e e n Victoria M a t e r n i t y Hospital on April i, I952, at 6 a.m., in early labour. T h e m e m b r a n e s h a d r u p t u r e d two h o u r s prior to admission. Medical History.--Poliomyelitis, right leg, as a child. N o recent illnesses. Menstrual taristory.--Last m e n s t r u a l period J u n e 24, I95I. E s t i m a t e d date of confinement, April i, I952.
Fig. 5I.--The deformed foetus in utero,, showing the very short limbs. ON EXAMINATION.~On e x a m i n a t i o n no a b n o r m a l i t y was detected other t h a n on palpation of the a b d o m e n at the last antenatal visit. H y d r a m n i o s was present. T h e r e was also difficulty in palpation of the p r e s e n t i n g part of t h e foetus. I n early labour fcetal distress occurred. T h e ffetal heart-rate was elevated to 16o beats per m i n u t e a n d m e c o n i u m - s t a i n e d liquor was draining f r o m t h e vagina. Postero-anterior a n d lateral films of t h e a b d o m e n (Fig. 51) disclosed calcification of t h e placenta, situated in t h e region of t h e right cornu. T h e presentation was R.O.A., the h e a d was n o t engaged, a n d t h e foetal skeleton was poorly calcified. H y d r a m n i o s was present. T h e skfitl vault appeared relatively large, t h e bone was thin, a n d it was seen to he i n d e n t e d w h e r e t h e vertex rested on t h e s y m p h y s i s in t h e u p r i g h t lateral film. T h e petrous bones a n d t h e semicircular canals were very p r o m i n e n t c o m p a r e d w i t h the rest of t h e poorly calcified skull'and facial bones. Several long b o n e s were visible, one scapula, b o t h h u m e r i , b o t h femora, a n d b o t h tibise. T h e y were short, and broad for their length, with the cortex poorly defined, a n d stippled areas of increased d e n s i t y were p r e s e n t within t h e m . O n e tibia was b e n t at a sharp angle, a n d a stripe o f lessened d e n s i t y across it s u g g e s t e d a p s e u d o fracture.
64
JOURNAL
OF
THE
FACULTY
OF
RADIOLOGISTS
T h e vertebral bodies a n d the pelvis were indistinct, a n d did n o t appear d e f o r m e d . T h e ribs could be distinguished, a n d t h e posterior portions of t h e lower ribs on each side were broader t h a n n o r m a l : each was distinctly distorted into a shallow wave formation. S o m e of t h e bones of t h e h a n d s a n d feet were visible. L a b o u r h a d n o w ceased a n d u t e r i n e contractions were s t i m u l a t e d by the u s e of a pitocin drip. A living female child was b o r n s p o n t a n e o u s l y at 3 p . m . T h e h e a d was grossly m o u l d e d and was delivered with t h e sagittal s u t u r e in t h e transverse diameter of t h e pelvic outlet. T h e b a b y w e i g h e d 3 lb. zz oz. a n d died half an h o u r afte r delivery. Films after delivery s h o w e d scattered, foci of calcification in t h e placenta. T h e f~tal skull was poorly calcified, m a n y W o r m i a n bones were present, a n d t h e p e t r o u s bones were p r o m i n e n t . T o o t h b u d s were developing in t h e u p p e r a n d lower jaws. T h e r e m a i n d e r of t h e skeleton was poorly calcified, with t h i n cortex, relatively large m e d u l l a r y cavities ; t h e long bones were broad a n d shortened, a n d m a n y of t h e m were d e f o r m e d a n d showed fractures and p s e u d o - f r a c t u r e s : several areas of increased bone density were p r e s e n t in s o m e of t h e long bones. W a v y d e f o r m i t y of t h e posterior portions of t h e b r o a d e n e d lower ribs was present, p r e s u m a b l y t h e result of u n e q u a l stresses from the intercostal m u s c u l a t u r e . T h e vertebra, pelvis, and t h e bones of t h e h a n d s a n d feet Were poorly calcified b u t n o t d e f o r m e d (Fig. 5Z)POST-MORTEM FINDrNOs.--The b o d y was that of a female infant Weighing approximately I75 ° g. T h e head h a d a r o u g h l y triangular shape with a broad base u p p e r m o s t , a n d on palpation t h e bones of t h e skull were u n u s u a l l y mobile and underdeveloped, so that the anterior fontanelle h a d no proper borders a n d was widely open, w i t h no enclosure at its angles. T h e limbs were u n u s u a l l y short, a n d tapere d f r o m a b n o r m a l l y wide u p p e r arms and thighs. T h e fingers a n d toes were long and slender. T h e r e were fractures at t h e m i d - p o i n t s of b o t h femora. T h e internal organs s h o w e d no macroscopical abnormality. HISTOPATHOLOGY.--The histological findings in all types of osteogenesis imperfecta have been well described by K n a g g s (I926). W i t h regard to the f~tal type, he draws attention to three i m p o r t a n t criteria : - L T h e stages of cartilaginous ossification are n o r m a l up to t h e f o r m a t i o n of the p r i m a r y areolm. z. T h e periosteal a n d m e d u l l a r y ossification is quite a b n o r m a l in character, deficient in q u a n t i t y a n d inferior in quality. 3- Osteoblast e d g i n g to t h e trabecuhe is either absent altogether or only partially p r e s e n t in places. Fig. 5~.--Post-partum radiograph of the foetus. All of t h e s e features were evident in t h e long bones of t h e p r e s e n t case. T h e epiphysial cartilage was normal, and the c o l u m n a r formation of vesicular cartilage cells at t h e m e t a p h y s i s was typical of early e n d o c h o n d r a l ossification. F r o m this point, however, t h e appearances were abnormal, w i t h an a b r u p t transition to a disorderly type of bone formation (Fig. 53)- P e n e t r a t i o n of the cartilage b y vascular m e s e n c h y m e was scanty, a n d t h e few areas of n e w l y calcified cartilage were thin, pale, a n d tattered, lacking t h e compact, deeply staining, u n i f o r m strands w h i c h characterize t h e n o r m a l (Fig. 54)- Very few osteoblasts ~were seen, a n d these did n o t f o r m an u n b r o k e n chain along t h e m a r g i n s of t h e n e w bone, b u t were patchily collected at various points on one or other side. T h e diaphysis was h i g h l y cellular, with a p r e d o m i n a n c e of m o n o n u e l e a r e l e m e n t s w h i c h could n o t be accurately identified. T h r o u g h o u t t h e sections t h e r e were scattered islands of partly calcified cartilage w i t h deeply staining a n d apparently active cartilage cells within it (Fig. 55). T h e p e r i o s t e u m was thickened a n d there were small i n g r o w t h s of diaphysial bone f o r m a t i o n along its length, t h e s e also being poor in investing osteoblasts a n d rich in deeply staining cartilage cells. T h e whole appearance lacked t h e u n i f o r m i t y a n d corn-
OSTEOGENESIS
IMPERFECTA
65
pacmess of n o r m a l e n d o c h o n d r a l bone formation, a n d the weakness of the b o n e was a p p a r e n t from its h i g h l y cellular and s t r u c t u r e l e s s c o m p o s i t i o n . T h e r e m a i n d e r of the organs e x a m i n e d s h o w e d acute congestion only.
Fig. 53---Abrupt transition from normal endochondral bone formation; ( x 25.)
Fig. 54.---Normal appearance of the tibia in a newborn infant. (× 35.)
Fig. 55.--Island of cartilage with scanty osteoblasts, ( x zoo.)
DISCUSSION The discovery of a major degree of osteogenesis imperfecta at birth is not rare. One of us has seen films of two other instances in this small community in the last febw years, with radiographic features similar to this fcetus. With more frequent use of radiography of the pregnant abdomen, an increasing number of abnormalities of the fcetus are being recorded.
66
JOURNAL
OF
THE
FACULTY
OF
RADIOLOGISTS
Other conditions may resemble osteogenesis imperfecta in utero.- In achondroplasia, calcification of the foetal skeleton is normal, the skull vault is relatively large, the long bones are short and broad, and characteristic spiking of the diaphysis of the long bones may be visible, and there is no tendency to fractures. In cleidocranial dysostosis, the poorly calcified skull vault should contrast with the normally calcified and undeformed skeleton. In the majority of other skeletal dystrophies the calcification of the skull and the major part of the skeleton is normal, and deformities of the long bones are uncommon at this age. Differentiation could conceivably be difficult in an obese woman, particularly if associated with hydramnios. The possibility of foetal osteogenesis imperfecta, with a very poorly calcified skeleton, as reported by Brailsford, should be borne in mind in instances of ovarian or uterine turnouts simulating pregnancy. SUMMARY A case of osteogenesis imperfecta, recognized in utero, is reported.
REFERENCES Bt~AILSFORD,J. F. (I948), The Radiology of Bones and Joints, 4th ed. London : J. & A. Churchill. DANELIUS, G. (I933), Arch. Gyniik, 154, I6I. FAIRBANK, H. A. T. (I95I), An Atlas of General Affections of the Skeleton. Edinburgh : E. & S. Livingstone. FRERKING, H. W., and ZINK, O. C. (I952), Amer. J. Roentgenol., 67, lO3. KNACCS, R. L. (I926), The Inflammatory and Toxic Diseases of Bone, 39o- Bristol : Wright.
JOURNAL
OF
THE
FACULTY
OF
RADIOLOGISTS
OSTEOGENESIS IMPERFECTA A C A S E D I S C O V E R E D IN U T E R O BY G. W. E. AITKEN, A. COHEN, and P. W. VERCO ADELAIDEj AUSTRALIA
OSTEOGENESISimperfecta, named by Vrolik in 1845 , is not an uncommon disease. The characteristic feature of this condition is fragility of the bones. Cases may be grouped, according to Fairbank (1951), as pre-natal and post-natal, but there is no clear-cut distinction between the two groups, except in the date of the first fracture, which depends upon the severity of the disorder. Females are said to be more frequently affected than males. Hereditary influences are apparent in a minority of both groups, blue sclerotics may be present, and otosclerosis may occur in those who live to the third decade. Dwarfing is largely accounted for by the deformities of limb and spine : in some cases an osteomalacic element may be present in addition to the brittleness of the bones. From the radiographic appearance, Fairbank (1951) describes three types : - I. A thich bone type, the severe pre-natal cases, born with limbs which are stunted as in achondroplasia and with numerous fractures. The appearance of the bones suggests that the thickening is not due entirely to callus formation following recent or remote fractures. It may be seen up to three months after birth, but seldom later. 2. The slender, fragile bone type: This type is seen in pre-natal cases which survive more than a few months, and in all post-natal cases. The skeleton as a whole is osteoporotic ; the long bones are slender, and their cortex is thin and of deficient density. 3. Osteogenesis imperfecta cystica. This name has been applied to cases with pronounced honeycombing of bones. It is extremely rare. Brailsford (1948) records a case of osteogenesis imperfecta recognized in utero : " A radiograph of the pregnant woman two days before delivery showed an enlarged uterus with two ill-defined opacities, which were thought to be calcified glands, and the abdominal mass a large ovarian cyst. More careful examination of these radiographs revealed that the opacities showed the characters of the petromastoids, and with this identification it was possible to recognize a very faint outline of the foetal skull. The foetus was in its ninth month and was the first pregnancy. The maternal pelvis revealed areas of cancellous absorption which aroused the suspicion of osteitis fibrosa of the hyperparathyroid type." Films of the fmtus showed typical osteogenesis imperfecta. Frerking and Zink (1952) reported the instance of a 2-para, 3 ° weeks pregnant, sent for examination because of recent abdominal pain, and'sudden increase in the size of the abdomen. The f&tal head could .not be felt, nor could the fmtal heart be heard. Several anteroposterior films were obtained, and no fmtal skeleton could be seen in these films, despite repeated examination with varying techniques. In a left lateral'view a small partially calcified foetal skeleton could be seen lying within a greatly enlarged uterus. The skull and facial bones were invisible except for the petrous pyramids. The spine was fairly well shown, the scapulae were distinguishable, but neither upper extremity could be seen. All the ribs, particularly their spinal ends, were faintly shown. The bones of the lower extremity were broad and short, and one of the femora was sharply angulated at one point, suggesting that it was fractured. A film of the stillborn fmtus showed changes typical of osteogenesis imperfecta : the entire skeleton was poorly calcified, most marked in the skull and facial bones. There were many subperiosteal and pseudo-fractures of the long bones of the limbs, which were broad, short, and deformed. Frerking and Zink (i952) quote a case of this condition diagnosed in utero, reported by Danelius (1933).
OSTEOGENESIS
IMPERFECTA
We have not been able to discover any other report of osteogenesis imperfecta u t e r o i n t h e l i t e r a t u r e a v a i l a b l e to u s , t h o u g h it is k n o w n s u c h r e p o r t s e x i s t .
63 diagnosed in
CASE REPORT ItlSTORY.--Mrs. H . , gravida 4, aged 3I years, was admitted to t h e Q u e e n Victoria M a t e r n i t y Hospital on April i, I952, at 6 a.m., in early labour. T h e m e m b r a n e s h a d r u p t u r e d two h o u r s prior to admission. Medical History.--Poliomyelitis, right leg, as a child. N o recent illnesses. Menstrual taristory.--Last m e n s t r u a l period J u n e 24, I95I. E s t i m a t e d date of confinement, April i, I952.
Fig. 5I.--The deformed foetus in utero,, showing the very short limbs. ON EXAMINATION.~On e x a m i n a t i o n no a b n o r m a l i t y was detected other t h a n on palpation of the a b d o m e n at the last antenatal visit. H y d r a m n i o s was present. T h e r e was also difficulty in palpation of the p r e s e n t i n g part of t h e foetus. I n early labour fcetal distress occurred. T h e ffetal heart-rate was elevated to 16o beats per m i n u t e a n d m e c o n i u m - s t a i n e d liquor was draining f r o m t h e vagina. Postero-anterior a n d lateral films of t h e a b d o m e n (Fig. 51) disclosed calcification of t h e placenta, situated in t h e region of t h e right cornu. T h e presentation was R.O.A., the h e a d was n o t engaged, a n d t h e foetal skeleton was poorly calcified. H y d r a m n i o s was present. T h e skfitl vault appeared relatively large, t h e bone was thin, a n d it was seen to he i n d e n t e d w h e r e t h e vertex rested on t h e s y m p h y s i s in t h e u p r i g h t lateral film. T h e petrous bones a n d t h e semicircular canals were very p r o m i n e n t c o m p a r e d w i t h the rest of t h e poorly calcified skull'and facial bones. Several long b o n e s were visible, one scapula, b o t h h u m e r i , b o t h femora, a n d b o t h tibise. T h e y were short, and broad for their length, with the cortex poorly defined, a n d stippled areas of increased d e n s i t y were p r e s e n t within t h e m . O n e tibia was b e n t at a sharp angle, a n d a stripe o f lessened d e n s i t y across it s u g g e s t e d a p s e u d o fracture.
64
JOURNAL
OF
THE
FACULTY
OF
RADIOLOGISTS
T h e vertebral bodies a n d the pelvis were indistinct, a n d did n o t appear d e f o r m e d . T h e ribs could be distinguished, a n d t h e posterior portions of t h e lower ribs on each side were broader t h a n n o r m a l : each was distinctly distorted into a shallow wave formation. S o m e of t h e bones of t h e h a n d s a n d feet were visible. L a b o u r h a d n o w ceased a n d u t e r i n e contractions were s t i m u l a t e d by the u s e of a pitocin drip. A living female child was b o r n s p o n t a n e o u s l y at 3 p . m . T h e h e a d was grossly m o u l d e d and was delivered with t h e sagittal s u t u r e in t h e transverse diameter of t h e pelvic outlet. T h e b a b y w e i g h e d 3 lb. zz oz. a n d died half an h o u r afte r delivery. Films after delivery s h o w e d scattered, foci of calcification in t h e placenta. T h e f~tal skull was poorly calcified, m a n y W o r m i a n bones were present, a n d t h e p e t r o u s bones were p r o m i n e n t . T o o t h b u d s were developing in t h e u p p e r a n d lower jaws. T h e r e m a i n d e r of t h e skeleton was poorly calcified, with t h i n cortex, relatively large m e d u l l a r y cavities ; t h e long bones were broad a n d shortened, a n d m a n y of t h e m were d e f o r m e d a n d showed fractures and p s e u d o - f r a c t u r e s : several areas of increased bone density were p r e s e n t in s o m e of t h e long bones. W a v y d e f o r m i t y of t h e posterior portions of t h e b r o a d e n e d lower ribs was present, p r e s u m a b l y t h e result of u n e q u a l stresses from the intercostal m u s c u l a t u r e . T h e vertebra, pelvis, and t h e bones of t h e h a n d s a n d feet Were poorly calcified b u t n o t d e f o r m e d (Fig. 5Z)POST-MORTEM FINDrNOs.--The b o d y was that of a female infant Weighing approximately I75 ° g. T h e head h a d a r o u g h l y triangular shape with a broad base u p p e r m o s t , a n d on palpation t h e bones of t h e skull were u n u s u a l l y mobile and underdeveloped, so that the anterior fontanelle h a d no proper borders a n d was widely open, w i t h no enclosure at its angles. T h e limbs were u n u s u a l l y short, a n d tapere d f r o m a b n o r m a l l y wide u p p e r arms and thighs. T h e fingers a n d toes were long and slender. T h e r e were fractures at t h e m i d - p o i n t s of b o t h femora. T h e internal organs s h o w e d no macroscopical abnormality. HISTOPATHOLOGY.--The histological findings in all types of osteogenesis imperfecta have been well described by K n a g g s (I926). W i t h regard to the f~tal type, he draws attention to three i m p o r t a n t criteria : - L T h e stages of cartilaginous ossification are n o r m a l up to t h e f o r m a t i o n of the p r i m a r y areolm. z. T h e periosteal a n d m e d u l l a r y ossification is quite a b n o r m a l in character, deficient in q u a n t i t y a n d inferior in quality. 3- Osteoblast e d g i n g to t h e trabecuhe is either absent altogether or only partially p r e s e n t in places. Fig. 5~.--Post-partum radiograph of the foetus. All of t h e s e features were evident in t h e long bones of t h e p r e s e n t case. T h e epiphysial cartilage was normal, and the c o l u m n a r formation of vesicular cartilage cells at t h e m e t a p h y s i s was typical of early e n d o c h o n d r a l ossification. F r o m this point, however, t h e appearances were abnormal, w i t h an a b r u p t transition to a disorderly type of bone formation (Fig. 53)- P e n e t r a t i o n of the cartilage b y vascular m e s e n c h y m e was scanty, a n d t h e few areas of n e w l y calcified cartilage were thin, pale, a n d tattered, lacking t h e compact, deeply staining, u n i f o r m strands w h i c h characterize t h e n o r m a l (Fig. 54)- Very few osteoblasts ~were seen, a n d these did n o t f o r m an u n b r o k e n chain along t h e m a r g i n s of t h e n e w bone, b u t were patchily collected at various points on one or other side. T h e diaphysis was h i g h l y cellular, with a p r e d o m i n a n c e of m o n o n u e l e a r e l e m e n t s w h i c h could n o t be accurately identified. T h r o u g h o u t t h e sections t h e r e were scattered islands of partly calcified cartilage w i t h deeply staining a n d apparently active cartilage cells within it (Fig. 55). T h e p e r i o s t e u m was thickened a n d there were small i n g r o w t h s of diaphysial bone f o r m a t i o n along its length, t h e s e also being poor in investing osteoblasts a n d rich in deeply staining cartilage cells. T h e whole appearance lacked t h e u n i f o r m i t y a n d corn-
OSTEOGENESIS
IMPERFECTA
65
pacmess of n o r m a l e n d o c h o n d r a l bone formation, a n d the weakness of the b o n e was a p p a r e n t from its h i g h l y cellular and s t r u c t u r e l e s s c o m p o s i t i o n . T h e r e m a i n d e r of the organs e x a m i n e d s h o w e d acute congestion only.
Fig. 53---Abrupt transition from normal endochondral bone formation; ( x 25.)
Fig. 54.---Normal appearance of the tibia in a newborn infant. (× 35.)
Fig. 55.--Island of cartilage with scanty osteoblasts, ( x zoo.)
DISCUSSION The discovery of a major degree of osteogenesis imperfecta at birth is not rare. One of us has seen films of two other instances in this small community in the last febw years, with radiographic features similar to this fcetus. With more frequent use of radiography of the pregnant abdomen, an increasing number of abnormalities of the fcetus are being recorded.
66
JOURNAL
OF
THE
FACULTY
OF
RADIOLOGISTS
Other conditions may resemble osteogenesis imperfecta in utero.- In achondroplasia, calcification of the foetal skeleton is normal, the skull vault is relatively large, the long bones are short and broad, and characteristic spiking of the diaphysis of the long bones may be visible, and there is no tendency to fractures. In cleidocranial dysostosis, the poorly calcified skull vault should contrast with the normally calcified and undeformed skeleton. In the majority of other skeletal dystrophies the calcification of the skull and the major part of the skeleton is normal, and deformities of the long bones are uncommon at this age. Differentiation could conceivably be difficult in an obese woman, particularly if associated with hydramnios. The possibility of foetal osteogenesis imperfecta, with a very poorly calcified skeleton, as reported by Brailsford, should be borne in mind in instances of ovarian or uterine turnouts simulating pregnancy. SUMMARY A case of osteogenesis imperfecta, recognized in utero, is reported.
REFERENCES Bt~AILSFORD,J. F. (I948), The Radiology of Bones and Joints, 4th ed. London : J. & A. Churchill. DANELIUS, G. (I933), Arch. Gyniik, 154, I6I. FAIRBANK, H. A. T. (I95I), An Atlas of General Affections of the Skeleton. Edinburgh : E. & S. Livingstone. FRERKING, H. W., and ZINK, O. C. (I952), Amer. J. Roentgenol., 67, lO3. KNACCS, R. L. (I926), The Inflammatory and Toxic Diseases of Bone, 39o- Bristol : Wright.