1979, British Journal of Radiology, 52, 441-443
JUNE 1979
Metaphyseal fractures in osteogenesis imperfecta By R. Astley, M.D., F.R.C.P.E., F.R.C.R. The Children's Hospital, Birmingham {Received November, 1978) ABSTRACT
Forty-one children with osteogenesis imperfecta have been reviewed. A minority (7/41) showed small metaphyseal fractures, resembling those seen in non-accidental injury, but in all of these there was obvious generalized bone disease so that confusion with non-accidental injury did not occur.
In non-accidental injury of childhood (the "battered baby" syndrome), the possibility of fragile bones may be raised. Therefore the findings in 41 children seen at the Children's Hospital with osteogenesis imperfecta have been reviewed. Special attention has been paid to the occurrence of the type of metaphyseal fracture that is often seen in non-accidental injury, i.e. a small flake detached near the epiphyseal line. The 41 children had received 316 radiological examinations, an indication of the large number of radiographs that was available for review.
three children without wormian bones all showed easily recognized osteoporosis and none had metaphyseal fractures. 3. Small metaphyseal fractures were present in seven of the 41 children (Table I). In these seven there was an obvious generalized skeletal disease with easily recognized osteoporosis, abnormal bone modelling and major fractures in addition to the detached metaphyseal flakes (Fig. 1). In all seven there were wormian bones in the skull. In six of the seven (in one child there was no follow-up), further fractures were seen after the diagnosis of osteogenesis imperfecta had been made and none of these later fractures were of metaphyseal type. COMMENT
In 37 of the 41 children reviewed, there was no radiological difficulty whatsoever in making a diagnosis of osteogenesis imperfecta. Even in the FINDINGS 1. Osteoporosis. In addition to their fractures, all remaining four there was osteoporosis, albeit less 41 children showed osteoporosis, easily detected in obvious, plus the presence of multiple wormian 37 but mild in four. These four all had multiple bones. wormian bones in the skull and none had metaThe presence of wormian bones is clearly a physeal fractures. useful diagnostic sign. In the four children where 2. Wormian bones were present in 33 out of 36 they were not present, osteoporosis was not in children where skull radiographs were available. The doubt; conversely, where osteoporosis was slight, TABLE I Name
Age when MF seen
Sites of MF
General appearance of bones
Wormian bones
A.H.
1 day
Upper L. humerus
Porosis with gross abnormality
Present
R.W.
2 days
Lower R. humerus; Lower R. & L. radius
Porosis with gross abnormality
Present
B.M.
1 week
Upper L. humerus; Lower R. humerus; Lower R. & L. radius
Porosis with gross abnormality
Present
C.S.
1 month
Upper L. radius; Lower L. ulna
Porosis with gross abnormality
Present
S.B.
5 months
Lower L. tibia
Porosis with gross abnormality
Present
R.C.
14 months
Lower L. radius
Porosis with gross abnormality
Present
B.B.
3 years
Lower R. ulna
Porosis with gross abnormality
Present
MF = me taphyseal fracture. 441
VOL.
52, No. 618 R. Astley
FIG. 1. c A T-U R.W., age 2 days. (A and B) There are small metaphyseal fractures at the lower end of the right humerus and at the lower end of each radius.
(c) There is early recognized generalized skeletal disease with major fractures and deformities. (D) The skull has multiple wormian bones.
JUNE 1979
Metaphyseal fractures in osteogenesis imperfecta wormian bones were present to reinforce the diagnosis. A minority of children showed small metaphyseal fractures, resembling those seen in non-accidental injury. In all of these there was an obvious generalized
disease of bone with easily recognized osteoporosis, abnormal bone modelling, deformities, major fractures and wormian bones, in addition to the detached metaphyseal flakes. Confusion with nonaccidental injury did not occur.
Book reviews Telescreen and Radiographic Examination of Urinary Trans- The Diagnostic Limitations of Computerised Axial Tomoport. By Endre Hajds, pp. 117, illus., 1978. (Akademiai graphy. Edited by J. Bories, pp. xi + 220, 1978 (Springer Kiado—Publishing House of the Hungarian Academy of Verlag, Berlin, Heidelberg, New York) DM54/127/£14-50. Sciences—Budapest). $10.50. ISBN 3-540-08593-9 ISBN 963-05-1507-5. The profound changes that have occurred in the diag"Functional radiodiagnostics of the kidney is an oft- nostic management of patients with neurological problems desired and highly desirable ideal which, however, is not since Hounsfield first presented computed tomography to easily achieved. This work has set the aim to get one step the world have inevitably resulted in a vast literature, most nearer to this ideal.. . of which extolled its "virtues" without perhaps enough "In the last 15 years (the) image intensifier has been emphasis placed on its "vices". It is therefore welcome to extensively used for the examination of the urinary tract. In find at last a scientific meeting whose main purpose has been the present study a great number of patients were examined to criticize CT, to define its limitations and perhaps to and the motility changes were recorded over a relatively long "get it in perspective". period of time. This allowed us to make new observations The book edited by Dr. Bories, derives from papers and to draw some conclusions regarding the site and presented at the 6th Congress of the European Society of frequency of the renal pacemaker." Neuroradiology in Dijon in September, 1976. The papers These are two paragraphs from the Preface of this well from many major world centres cover a wide variety of produced monograph. They give a fair indication of the pathology and most accurately demonstrate the limitations range and style of the work. It is a painstaking, sober record of computed tomography as well as giving numerous useful, of 800 IVUs, mostly adult, using fluoroscopy of the upper practical hints. It is divided into five parts—cerebral urinary tracts and only a few radiographs. The author tumours; orbit and skull base; infarcts, oedema and presents evidence that this did not result in high radiation sub-dural haematomas; CT and other techniques; CT accuracy and future aspects. There are however, few papers burdens in these patients. What happens in normal as well as abnormal states is that compare the accuracy of CT with other neuroradiodocumented with care. Such studies of the patterns of logical procedures, indeed in some areas the pendulum has contraction of the upper urinary tract already have a long already begun to swing back to more conventional exhistory. Many radiologists will have learnt for themselves aminations. In this context I was most interested to see, as I how fluoroscopy can often resolve points of diagnostic have always thought, how little extra help is forthcoming as difficulty, particularly, in confirming or refuting a doubtful a result of "gazing at print-outs". ureteric stone. However, there is real value in having so It is over two years since the meeting was held and most of much first hand information on the human system assembled the newer developments referred to by Professor Taveras in one good small book. There is for instance the authori- have already been reached. Scan times of one to two tative finding that contractions commonly begin in the renal seconds, gating for the heart and reconstruction of picture pelvis at a site just above the pelviureteric junction—the slices, both coronally and sagitally, as well as higher human pacemaker is here, not in the calices (bifid systems resolution scans for the spinal canal are now available on a excepted). number of machines. Indeed further technical advances are In work so much concerned with tone, contractility and expected each year. Nevertheless the problem areas (skull movement, the absence of any pressure measurements is base, perichiasmatic region, posterior fossa etc.) still remain perhaps rather a drawback. This is particularly so in the essentially unchanged, as does sometimes the overlap in final part of the book, dealing with "the orthostatic reaction". appearances between those cases which require surgery and This is said to be a reflex contraction of the upper urinary those in which surgery is unwise if not dangerous. It is tract on standing up. The author is aware of alternative apparent that there is no substitute for an experienced explanations for the IVU findings in the erect posture (e.g. radiologist in reducing the false positive and negative rates. gravity), and argues his case skilfully. I found this the most Such experience takes time to acquire, but with the aid of interesting section of the book, nicely provocative even if the this book many of the problem areas have not only been reader may dissent. Why is the ureter below a stone some- highlighted, but elucidated. The book assumes a reasonable knowledge of the subject, but is undoubtedly essential times dilated and idle? I think this is a stimulating contribution to the field of reading to anyone who wishes to become competent in this urodynamics. Perhaps not every X-ray department will subject. wish to own a copy, but larger libraries and anyone interested D. P. E. KlNGSLEY. in the subject should have a look at Hajos. THOMAS SHERWOOD.
443
JUNE 1979
Metaphyseal fractures in osteogenesis imperfecta By R. Astley, M.D., F.R.C.P.E., F.R.C.R. The Children's Hospital, Birmingham {Received November, 1978) ABSTRACT
Forty-one children with osteogenesis imperfecta have been reviewed. A minority (7/41) showed small metaphyseal fractures, resembling those seen in non-accidental injury, but in all of these there was obvious generalized bone disease so that confusion with non-accidental injury did not occur.
In non-accidental injury of childhood (the "battered baby" syndrome), the possibility of fragile bones may be raised. Therefore the findings in 41 children seen at the Children's Hospital with osteogenesis imperfecta have been reviewed. Special attention has been paid to the occurrence of the type of metaphyseal fracture that is often seen in non-accidental injury, i.e. a small flake detached near the epiphyseal line. The 41 children had received 316 radiological examinations, an indication of the large number of radiographs that was available for review.
three children without wormian bones all showed easily recognized osteoporosis and none had metaphyseal fractures. 3. Small metaphyseal fractures were present in seven of the 41 children (Table I). In these seven there was an obvious generalized skeletal disease with easily recognized osteoporosis, abnormal bone modelling and major fractures in addition to the detached metaphyseal flakes (Fig. 1). In all seven there were wormian bones in the skull. In six of the seven (in one child there was no follow-up), further fractures were seen after the diagnosis of osteogenesis imperfecta had been made and none of these later fractures were of metaphyseal type. COMMENT
In 37 of the 41 children reviewed, there was no radiological difficulty whatsoever in making a diagnosis of osteogenesis imperfecta. Even in the FINDINGS 1. Osteoporosis. In addition to their fractures, all remaining four there was osteoporosis, albeit less 41 children showed osteoporosis, easily detected in obvious, plus the presence of multiple wormian 37 but mild in four. These four all had multiple bones. wormian bones in the skull and none had metaThe presence of wormian bones is clearly a physeal fractures. useful diagnostic sign. In the four children where 2. Wormian bones were present in 33 out of 36 they were not present, osteoporosis was not in children where skull radiographs were available. The doubt; conversely, where osteoporosis was slight, TABLE I Name
Age when MF seen
Sites of MF
General appearance of bones
Wormian bones
A.H.
1 day
Upper L. humerus
Porosis with gross abnormality
Present
R.W.
2 days
Lower R. humerus; Lower R. & L. radius
Porosis with gross abnormality
Present
B.M.
1 week
Upper L. humerus; Lower R. humerus; Lower R. & L. radius
Porosis with gross abnormality
Present
C.S.
1 month
Upper L. radius; Lower L. ulna
Porosis with gross abnormality
Present
S.B.
5 months
Lower L. tibia
Porosis with gross abnormality
Present
R.C.
14 months
Lower L. radius
Porosis with gross abnormality
Present
B.B.
3 years
Lower R. ulna
Porosis with gross abnormality
Present
MF = me taphyseal fracture. 441
VOL.
52, No. 618 R. Astley
FIG. 1. c A T-U R.W., age 2 days. (A and B) There are small metaphyseal fractures at the lower end of the right humerus and at the lower end of each radius.
(c) There is early recognized generalized skeletal disease with major fractures and deformities. (D) The skull has multiple wormian bones.
JUNE 1979
Metaphyseal fractures in osteogenesis imperfecta wormian bones were present to reinforce the diagnosis. A minority of children showed small metaphyseal fractures, resembling those seen in non-accidental injury. In all of these there was an obvious generalized
disease of bone with easily recognized osteoporosis, abnormal bone modelling, deformities, major fractures and wormian bones, in addition to the detached metaphyseal flakes. Confusion with nonaccidental injury did not occur.
Book reviews Telescreen and Radiographic Examination of Urinary Trans- The Diagnostic Limitations of Computerised Axial Tomoport. By Endre Hajds, pp. 117, illus., 1978. (Akademiai graphy. Edited by J. Bories, pp. xi + 220, 1978 (Springer Kiado—Publishing House of the Hungarian Academy of Verlag, Berlin, Heidelberg, New York) DM54/127/£14-50. Sciences—Budapest). $10.50. ISBN 3-540-08593-9 ISBN 963-05-1507-5. The profound changes that have occurred in the diag"Functional radiodiagnostics of the kidney is an oft- nostic management of patients with neurological problems desired and highly desirable ideal which, however, is not since Hounsfield first presented computed tomography to easily achieved. This work has set the aim to get one step the world have inevitably resulted in a vast literature, most nearer to this ideal.. . of which extolled its "virtues" without perhaps enough "In the last 15 years (the) image intensifier has been emphasis placed on its "vices". It is therefore welcome to extensively used for the examination of the urinary tract. In find at last a scientific meeting whose main purpose has been the present study a great number of patients were examined to criticize CT, to define its limitations and perhaps to and the motility changes were recorded over a relatively long "get it in perspective". period of time. This allowed us to make new observations The book edited by Dr. Bories, derives from papers and to draw some conclusions regarding the site and presented at the 6th Congress of the European Society of frequency of the renal pacemaker." Neuroradiology in Dijon in September, 1976. The papers These are two paragraphs from the Preface of this well from many major world centres cover a wide variety of produced monograph. They give a fair indication of the pathology and most accurately demonstrate the limitations range and style of the work. It is a painstaking, sober record of computed tomography as well as giving numerous useful, of 800 IVUs, mostly adult, using fluoroscopy of the upper practical hints. It is divided into five parts—cerebral urinary tracts and only a few radiographs. The author tumours; orbit and skull base; infarcts, oedema and presents evidence that this did not result in high radiation sub-dural haematomas; CT and other techniques; CT accuracy and future aspects. There are however, few papers burdens in these patients. What happens in normal as well as abnormal states is that compare the accuracy of CT with other neuroradiodocumented with care. Such studies of the patterns of logical procedures, indeed in some areas the pendulum has contraction of the upper urinary tract already have a long already begun to swing back to more conventional exhistory. Many radiologists will have learnt for themselves aminations. In this context I was most interested to see, as I how fluoroscopy can often resolve points of diagnostic have always thought, how little extra help is forthcoming as difficulty, particularly, in confirming or refuting a doubtful a result of "gazing at print-outs". ureteric stone. However, there is real value in having so It is over two years since the meeting was held and most of much first hand information on the human system assembled the newer developments referred to by Professor Taveras in one good small book. There is for instance the authori- have already been reached. Scan times of one to two tative finding that contractions commonly begin in the renal seconds, gating for the heart and reconstruction of picture pelvis at a site just above the pelviureteric junction—the slices, both coronally and sagitally, as well as higher human pacemaker is here, not in the calices (bifid systems resolution scans for the spinal canal are now available on a excepted). number of machines. Indeed further technical advances are In work so much concerned with tone, contractility and expected each year. Nevertheless the problem areas (skull movement, the absence of any pressure measurements is base, perichiasmatic region, posterior fossa etc.) still remain perhaps rather a drawback. This is particularly so in the essentially unchanged, as does sometimes the overlap in final part of the book, dealing with "the orthostatic reaction". appearances between those cases which require surgery and This is said to be a reflex contraction of the upper urinary those in which surgery is unwise if not dangerous. It is tract on standing up. The author is aware of alternative apparent that there is no substitute for an experienced explanations for the IVU findings in the erect posture (e.g. radiologist in reducing the false positive and negative rates. gravity), and argues his case skilfully. I found this the most Such experience takes time to acquire, but with the aid of interesting section of the book, nicely provocative even if the this book many of the problem areas have not only been reader may dissent. Why is the ureter below a stone some- highlighted, but elucidated. The book assumes a reasonable knowledge of the subject, but is undoubtedly essential times dilated and idle? I think this is a stimulating contribution to the field of reading to anyone who wishes to become competent in this urodynamics. Perhaps not every X-ray department will subject. wish to own a copy, but larger libraries and anyone interested D. P. E. KlNGSLEY. in the subject should have a look at Hajos. THOMAS SHERWOOD.
443
