1990, The British Journal of Radiology, 63, 894-897
Figure 2. Computed tomographic scan showing characteristic whorls of bowel around the superior mesenteric artery.
The distal duodenum lay anterior to and was compressed by the transverse colon lying on the left. The small bowel and proximal colon, on a narrow mesentery on the right, were twisted about the superior mesenteric artery forming the mass seen on computed tomography. This was presumed to have compressed the biliary and pancreatic ducts causing derangement of liver function tests and amylase. Discussion
Disorders of rotation of the gut are all uncommon, those of the fore and hind guts extremely so. Those of
Case reports
the midgut are most often encountered in the paediatric age group, but their presentation in adult life is well recognized. Their characteristics are well described by Dott (1923). The most common is volvulus in which the midgut leaves and returns to the abdomen in the first trimester without rotation. The disposition of small and large bowels is explained by the attachments of the fore and hind guts (Moore, 1988). Malrotation of the gut provides considerable diagnostic difficulty. Computed tomographic scanning may be a useful means of establishing a pre-operative diagnosis in such patients. Although rare, the bizarre CT appearance of volvulus in malrotation showing whorls of bowel around the superior mesenteric artery has been previously described (Fisher, 1981; Mori etal, 1987). Characteristic changes in the relative positions of superior mesenteric artery and vein may also be seen in malrotation (Nichols & Li, 1983). It is important that the characteristic appearances of this rare condition are recognized so that a correct preoperative diagnosis can be made. References DOTT, N. M., 1923. Anomalies of intestinal rotation. British Journal of Surgery, 11, 251-286. FISHER, J. K., 1981. Computed tomographic diagnosis of volvulus in intestinal malrotation. Radiology, 140, 145-146. MOORE, K. L., 1988. The Developing Human—Clinically Orientated Embryology (W. B. Saunders, Philadelphia), pp. 228-236. MORI, H.,
HAYASHI, K.
&
FUTAGAWA, S.,
1987.
Vascular
compromise in chronic volvulus with midgut rotation. Paediatric Radiology, 17, 227-281. NICHOLS, D. M. & Li, D. K., 1983. Superior mesenteric vein rotation: A CT sign of midgut malrotation. American Journal of Roentgenology, 141, 707-708.
The value of computed tomography in the diagnosis of atlanto-axial rotatory fixation By J. B. Murray, MB, ChB, DA, FRCR and M. Ziervogel, MB, ChB, FRCR Department of Radiology, Royal Hospital for Sick Children, Yorkhill, Glasgow G2 {Received July 1989 and in revised form June 1990)
Case report A 10-year-old girl was examined in Casualty complaining of a torticollis with her head looking downwards and to the right, which had developed suddenly that morning as she was drying herself after a bath. She had no neurological signs or symptoms. Interpretation of her cervical spine radiographs was difficult owing to her inability to move her neck for proper radiographic positioning (Fig. 1). She was given a soft collar and sent home. She returned 10 days later with increased para vertebral muscle spasm in her neck. Further radiographs, including oblique views, were taken of her cervical spine. Interpretation was again difficult owing to her inability to move her neck. She was admitted and further investigations including isotope bone
894
scan and routine bloods were normal. She was unable to tolerate a collar support. Analgesia and muscle relaxants were not successful. There were two episodes, one at home and one in the ward, where she was noted to have untwisted her neck during the night; but gradually the torticollis returned. Because of her rather odd posturing and anxiety in the ward it was thought this might be a conversion hysteria. An opinion was therefore sought from a child psychiatrist, who concluded that the child was normal and well adjusted. A consultant neurologist examined her and could find no peripheral or central neurological deficit. Wilson's disease was considered because of possible Kayser-Fleischer rings and a slightly raised bilirubin but an ophthalmological opinion discounted this.
The British Journal of Radiology, November 1990
Case reports
Figure 1. (a) Anteroposterior view of cervical spine showing marked degree of torticollis, (b) Anteroposterior coned view of atlanto-axial joint. Interpretation is difficult as the patient was unable to move her head, (c) Oblique view of atlanto-axial joint. Interpretation is again difficult.
Following a further neurological opinion a computed tomographic (CT) scan of the neck was suggested and, 4 months after initial presentation, an atlanto-axial rotatory fixation was diagnosed (Fig. 2). This was treated by initial traction and subsequent bone grafting. Discussion
Six weeks after initial presentation she was examined by an orthopaedic surgeon with a paediatric interest. It was concluded that she had a spastic torticollis of unexplained aetiology, probably also to some degree hysterical in origin. In order to exclude an intraspinal lesion, a magnetic resonance scan was performed. No intracranial abnormality and no cervical cord abnormality was identified. Vol. 63, No. 755
Atlanto-axial rotatory fixation is one of a spectrum of rotatory abnormalities of the atlanto-axial joint (Hess et al, 1935; Cattell & Filtzer, 1965; El-Khoury et al, 1984). This ranges from bilateral atlanto-axial rotatory dislocation to the atlanto-axial joint fixed in a position of normal rotation. The reason for the atlanto-axial joint becoming fixed in rotation remains obscure, although one theory is that there may be a capsular tear with invagination of torn capsule and synovial fringes (Wortzman & Dewar, 1968). Fielding and Hawkins (1977) classified atlanto-axial rotatory subluxation into four types (Fig. 3). Type I: rotatory fixation without anterior displacement of the axis. Thefixedrotation is within the normal range of atlanto-axial rotation. The transverse ligament is intact so that the dens acts as a pivot. This is the most common. Type II: rotatory fixation with 3-5 mm anterior displacement of the atlas and is the second most common lesion. This is associated with slackness of the transverse ligament and anterior displacement of the lateral mass while the opposite atlanto-axial joint acts as a pivot. 895
Case reports
(c) Figure 2. Axial CT scan through the axis with the base of the odontoid process shown. A Fielding Type II atlanto-axial rotatory fixation with dislocation of the left atlanto-axial joint anteriorly is present, (b) Axial CT scan at a slightly higher level showed the position of the odontoid process fixed on rotation of the head, (c) Coronal reconstruction at this level confirmed the persistent proximity of the odontoid process to the right lateral mass of the atlas.
anatomical structures in a patient who was unable to move her neck to obtain correct radiographic positioning. The value of early diagnosis in relation to outcome has recently been emphasized (Johnson & Fergusson,
Type III: rotatory fixation with more than 5 mm anterior displacement. Both lateral atlantal masses are displaced anteriorly, one more than the other. Type IV: rotatory fixation with posterior displacement. This is exceedingly rare and can occur only if the dens is absent. Plain film examination of atlanto-axial rotatory fixation is well established; anteroposterior radiographs reveal shift of the odontoid peg to one side. The diagnosis is confirmed by demonstrating that the atlanto-axial joint is fixed in rotation and that the atlas and the axis move as one. This is shown on open-mouth anteroposterior radiographs of the upper cervical spine taken in neutral and in 15° of rotation to either side to show a persistent proximity of the odontoid peg to the lateral mass of the atlas on the affected side. Plain film interpretation, as in this case, is often difficult. The problem was demonstrating these complex 896
Figure 3. Diagram illustrating the four types of rotatory fixation. 1. Type I: odontoid acting as the pivot with no anterior displacement. 2. Type II: one lateral articular process acting as the pivot with 3-5 mm anterior displacement. 3. Type III: rotatory fixation with more than 5 mm anterior displacement 4. Type IV: odontoid process absent with rotatory fixation and posterior displacement. The British Journal of Radiology, November 1990
1990, The British Journal of Radiology, 63, 897-899 Case reports
1986). Early treatment with a short period of halter traction increases the prospect of successful reduction and early return of function, avoiding subsequent deformity and instability for which cervical fusion may be required. This case illustrates the difficulty in diagnosing this relatively rare condition and stresses the value of early CT in establishing the diagnosis where plain film interpretation is unclear.
EL-KHOURY, G. Y., CLARK, C. R. & GRAVETT, A. W., 1984.
Acute traumatic rotatory atlanto-axial dislocation in children; a report of three cases. American Journal of Bone and Joint Surgery, 66A, 11^-111. FIELDING, J. W. & HAWKINS,
R. J.,
1977. Atlanto-axial
rotatory fixation (fixed rotatory subluxation of the atlanto-axial joint). American Journal of Bone and Joint Surgery, 59A, 37-44. HESS, J. H., BRONSTEIN, I. P. & ABELSON, S. M., 1935.
Atlanto-axial dislocations unassociated with trauma and secondary to inflammatory foci in the neck. American Journal of Diseases of Children, 49, 1137-1147. JOHNSTON, D. P. & FERGUSSON, C. M., 1986. Early diagnosis of
References CATTELL, H. S. & FILTZER, D. L., 1965. Pseudosubluxation and
other normal variations in the cervical spine in children; a study of one hundred and sixty children. American Journal of Bone and Joint Surgery, 47A, 1295-1309.
atlanto-axial rotatory fixation. British Journal of Bone and Joint Surgery, 68B, 698-701. WORTZMAN, G. & DEWAR, F. P., 1968. Rotatory fixation of the
atlanto-axial joint; rotational atlanto-axial Radiology, 90, 479-487.
subluxation.
Multifocal central nervous system lesions and retropharyngeal lymphadenopathy on magnetic resonance imaging: an association that suggested progressive multifocal leukoencephalopathy in a patient with acute aphasia By J. G. Llewelyn, MRCP, *A. R. Valentine, FRCR, C. Bradley, MRCP, K. King, MB, BCh and M. L. P. Gross, MRCP Departments of Neurological Science and * Radiology, Royal Free Hospital School of Medicine, London {Received December 1989 and in revised form April 1990)
Progressive multifocal leukoencephalopathy (PML) is an uncommon condition associated almost exclusively with impaired host immunity. More recently it has been recognized as a complication of the acquired immunodeficiency syndrome (AIDS), occurring in as many as 3.8% of these patients (Krupp et al, 1985). It may, rarely, be the initial manifestation of primary HIV infection, and in documented cases presents as focal limb weakness, altered mental state, ataxia, visual loss, headache and speech or language disturbance (Berger et al, 1987). We report a case of PML which presented as an acute aphasia, and in whom the diagnosis of an immunodeficient state was suggested by the magnetic resonance imaging (MRI)findingof enlarged retropharyngeal lymph nodes. Case report A 45-year-old married man with one child was admitted with an acute aphasia. He had been at work the day prior to admission, and later that evening became aware of difficulty finding words. The following morning his aphasia had progressed and he was admitted to hospital. General examination was unremarkable with no lymphadenopathy detected. Neurological examination revealed an expressive and receptive aphasia, dysgraphia and dyslexia. There was a mild right upper Address correspondence to Dr J. G. Llewelyn, University Department of Neurology, King's College Hospital and Institute of Psychiatry, Denmark Hill, London SE5 9RS. Vol. 63, No. 755
motor neurone facial weakness with normal limb power and symmetrical reflexes, but an extensor plantar response on the right. Serum investigations including white cell differential count were normal. Computed tomography (CT) showed a well defined non-enhancing low density lesion in the left posteroinferior frontal region, involving grey and white matter, but exerting no appreciable mass effect (Fig. 1) Electroencephalogram showed bifrontal delta activity. Cerebrospinal fluid analysis and digital subtraction arteriography were normal. Despite clinical deterioration, CT brain scan 2 weeks after the first showed very little, if any, progression of the lesion. Magnetic resonance imaging demonstrated an extensive lesion exerting slight but definite mass effect in the left frontal lobe, involving the basal ganglia and corpus callosum. It also revealed other smaller lesions posteriorly in the left cerebral hemisphere and also in the right frontal lobe (Fig. 2). It was also noted that there was a mass in the post-nasal space consistent with adenoidal enlargement (Fig. 3, arrowed) and this latter finding was considered to suggest the possibility of host immunodeficiency. Anti-HIV antibody testing was positive; p24 core antigen titre was negative. Helper (T4): suppressor (T8) lymphocyte ratio was reversed at 0.18. A biopsy taken from the left cerebral cortex showed foci of demyelination with a "snowstorm" appearance in the white matter and grey/white interface, with areas of confluence. Oligodendrocyte nuclei were enlarged, hyperchromatic, with abnormal homogeneous chromatin and some filled with granular material. Bizarre astrocytes were also seen. These appearances were characteristic of PML. Electron microscopy showed uniform viral particles within oligodendrocytes, shown by immunofluorescence to be JC polyoma virus. Information obtained from a male friend of the patient
897
Figure 2. Computed tomographic scan showing characteristic whorls of bowel around the superior mesenteric artery.
The distal duodenum lay anterior to and was compressed by the transverse colon lying on the left. The small bowel and proximal colon, on a narrow mesentery on the right, were twisted about the superior mesenteric artery forming the mass seen on computed tomography. This was presumed to have compressed the biliary and pancreatic ducts causing derangement of liver function tests and amylase. Discussion
Disorders of rotation of the gut are all uncommon, those of the fore and hind guts extremely so. Those of
Case reports
the midgut are most often encountered in the paediatric age group, but their presentation in adult life is well recognized. Their characteristics are well described by Dott (1923). The most common is volvulus in which the midgut leaves and returns to the abdomen in the first trimester without rotation. The disposition of small and large bowels is explained by the attachments of the fore and hind guts (Moore, 1988). Malrotation of the gut provides considerable diagnostic difficulty. Computed tomographic scanning may be a useful means of establishing a pre-operative diagnosis in such patients. Although rare, the bizarre CT appearance of volvulus in malrotation showing whorls of bowel around the superior mesenteric artery has been previously described (Fisher, 1981; Mori etal, 1987). Characteristic changes in the relative positions of superior mesenteric artery and vein may also be seen in malrotation (Nichols & Li, 1983). It is important that the characteristic appearances of this rare condition are recognized so that a correct preoperative diagnosis can be made. References DOTT, N. M., 1923. Anomalies of intestinal rotation. British Journal of Surgery, 11, 251-286. FISHER, J. K., 1981. Computed tomographic diagnosis of volvulus in intestinal malrotation. Radiology, 140, 145-146. MOORE, K. L., 1988. The Developing Human—Clinically Orientated Embryology (W. B. Saunders, Philadelphia), pp. 228-236. MORI, H.,
HAYASHI, K.
&
FUTAGAWA, S.,
1987.
Vascular
compromise in chronic volvulus with midgut rotation. Paediatric Radiology, 17, 227-281. NICHOLS, D. M. & Li, D. K., 1983. Superior mesenteric vein rotation: A CT sign of midgut malrotation. American Journal of Roentgenology, 141, 707-708.
The value of computed tomography in the diagnosis of atlanto-axial rotatory fixation By J. B. Murray, MB, ChB, DA, FRCR and M. Ziervogel, MB, ChB, FRCR Department of Radiology, Royal Hospital for Sick Children, Yorkhill, Glasgow G2 {Received July 1989 and in revised form June 1990)
Case report A 10-year-old girl was examined in Casualty complaining of a torticollis with her head looking downwards and to the right, which had developed suddenly that morning as she was drying herself after a bath. She had no neurological signs or symptoms. Interpretation of her cervical spine radiographs was difficult owing to her inability to move her neck for proper radiographic positioning (Fig. 1). She was given a soft collar and sent home. She returned 10 days later with increased para vertebral muscle spasm in her neck. Further radiographs, including oblique views, were taken of her cervical spine. Interpretation was again difficult owing to her inability to move her neck. She was admitted and further investigations including isotope bone
894
scan and routine bloods were normal. She was unable to tolerate a collar support. Analgesia and muscle relaxants were not successful. There were two episodes, one at home and one in the ward, where she was noted to have untwisted her neck during the night; but gradually the torticollis returned. Because of her rather odd posturing and anxiety in the ward it was thought this might be a conversion hysteria. An opinion was therefore sought from a child psychiatrist, who concluded that the child was normal and well adjusted. A consultant neurologist examined her and could find no peripheral or central neurological deficit. Wilson's disease was considered because of possible Kayser-Fleischer rings and a slightly raised bilirubin but an ophthalmological opinion discounted this.
The British Journal of Radiology, November 1990
Case reports
Figure 1. (a) Anteroposterior view of cervical spine showing marked degree of torticollis, (b) Anteroposterior coned view of atlanto-axial joint. Interpretation is difficult as the patient was unable to move her head, (c) Oblique view of atlanto-axial joint. Interpretation is again difficult.
Following a further neurological opinion a computed tomographic (CT) scan of the neck was suggested and, 4 months after initial presentation, an atlanto-axial rotatory fixation was diagnosed (Fig. 2). This was treated by initial traction and subsequent bone grafting. Discussion
Six weeks after initial presentation she was examined by an orthopaedic surgeon with a paediatric interest. It was concluded that she had a spastic torticollis of unexplained aetiology, probably also to some degree hysterical in origin. In order to exclude an intraspinal lesion, a magnetic resonance scan was performed. No intracranial abnormality and no cervical cord abnormality was identified. Vol. 63, No. 755
Atlanto-axial rotatory fixation is one of a spectrum of rotatory abnormalities of the atlanto-axial joint (Hess et al, 1935; Cattell & Filtzer, 1965; El-Khoury et al, 1984). This ranges from bilateral atlanto-axial rotatory dislocation to the atlanto-axial joint fixed in a position of normal rotation. The reason for the atlanto-axial joint becoming fixed in rotation remains obscure, although one theory is that there may be a capsular tear with invagination of torn capsule and synovial fringes (Wortzman & Dewar, 1968). Fielding and Hawkins (1977) classified atlanto-axial rotatory subluxation into four types (Fig. 3). Type I: rotatory fixation without anterior displacement of the axis. Thefixedrotation is within the normal range of atlanto-axial rotation. The transverse ligament is intact so that the dens acts as a pivot. This is the most common. Type II: rotatory fixation with 3-5 mm anterior displacement of the atlas and is the second most common lesion. This is associated with slackness of the transverse ligament and anterior displacement of the lateral mass while the opposite atlanto-axial joint acts as a pivot. 895
Case reports
(c) Figure 2. Axial CT scan through the axis with the base of the odontoid process shown. A Fielding Type II atlanto-axial rotatory fixation with dislocation of the left atlanto-axial joint anteriorly is present, (b) Axial CT scan at a slightly higher level showed the position of the odontoid process fixed on rotation of the head, (c) Coronal reconstruction at this level confirmed the persistent proximity of the odontoid process to the right lateral mass of the atlas.
anatomical structures in a patient who was unable to move her neck to obtain correct radiographic positioning. The value of early diagnosis in relation to outcome has recently been emphasized (Johnson & Fergusson,
Type III: rotatory fixation with more than 5 mm anterior displacement. Both lateral atlantal masses are displaced anteriorly, one more than the other. Type IV: rotatory fixation with posterior displacement. This is exceedingly rare and can occur only if the dens is absent. Plain film examination of atlanto-axial rotatory fixation is well established; anteroposterior radiographs reveal shift of the odontoid peg to one side. The diagnosis is confirmed by demonstrating that the atlanto-axial joint is fixed in rotation and that the atlas and the axis move as one. This is shown on open-mouth anteroposterior radiographs of the upper cervical spine taken in neutral and in 15° of rotation to either side to show a persistent proximity of the odontoid peg to the lateral mass of the atlas on the affected side. Plain film interpretation, as in this case, is often difficult. The problem was demonstrating these complex 896
Figure 3. Diagram illustrating the four types of rotatory fixation. 1. Type I: odontoid acting as the pivot with no anterior displacement. 2. Type II: one lateral articular process acting as the pivot with 3-5 mm anterior displacement. 3. Type III: rotatory fixation with more than 5 mm anterior displacement 4. Type IV: odontoid process absent with rotatory fixation and posterior displacement. The British Journal of Radiology, November 1990
1990, The British Journal of Radiology, 63, 897-899 Case reports
1986). Early treatment with a short period of halter traction increases the prospect of successful reduction and early return of function, avoiding subsequent deformity and instability for which cervical fusion may be required. This case illustrates the difficulty in diagnosing this relatively rare condition and stresses the value of early CT in establishing the diagnosis where plain film interpretation is unclear.
EL-KHOURY, G. Y., CLARK, C. R. & GRAVETT, A. W., 1984.
Acute traumatic rotatory atlanto-axial dislocation in children; a report of three cases. American Journal of Bone and Joint Surgery, 66A, 11^-111. FIELDING, J. W. & HAWKINS,
R. J.,
1977. Atlanto-axial
rotatory fixation (fixed rotatory subluxation of the atlanto-axial joint). American Journal of Bone and Joint Surgery, 59A, 37-44. HESS, J. H., BRONSTEIN, I. P. & ABELSON, S. M., 1935.
Atlanto-axial dislocations unassociated with trauma and secondary to inflammatory foci in the neck. American Journal of Diseases of Children, 49, 1137-1147. JOHNSTON, D. P. & FERGUSSON, C. M., 1986. Early diagnosis of
References CATTELL, H. S. & FILTZER, D. L., 1965. Pseudosubluxation and
other normal variations in the cervical spine in children; a study of one hundred and sixty children. American Journal of Bone and Joint Surgery, 47A, 1295-1309.
atlanto-axial rotatory fixation. British Journal of Bone and Joint Surgery, 68B, 698-701. WORTZMAN, G. & DEWAR, F. P., 1968. Rotatory fixation of the
atlanto-axial joint; rotational atlanto-axial Radiology, 90, 479-487.
subluxation.
Multifocal central nervous system lesions and retropharyngeal lymphadenopathy on magnetic resonance imaging: an association that suggested progressive multifocal leukoencephalopathy in a patient with acute aphasia By J. G. Llewelyn, MRCP, *A. R. Valentine, FRCR, C. Bradley, MRCP, K. King, MB, BCh and M. L. P. Gross, MRCP Departments of Neurological Science and * Radiology, Royal Free Hospital School of Medicine, London {Received December 1989 and in revised form April 1990)
Progressive multifocal leukoencephalopathy (PML) is an uncommon condition associated almost exclusively with impaired host immunity. More recently it has been recognized as a complication of the acquired immunodeficiency syndrome (AIDS), occurring in as many as 3.8% of these patients (Krupp et al, 1985). It may, rarely, be the initial manifestation of primary HIV infection, and in documented cases presents as focal limb weakness, altered mental state, ataxia, visual loss, headache and speech or language disturbance (Berger et al, 1987). We report a case of PML which presented as an acute aphasia, and in whom the diagnosis of an immunodeficient state was suggested by the magnetic resonance imaging (MRI)findingof enlarged retropharyngeal lymph nodes. Case report A 45-year-old married man with one child was admitted with an acute aphasia. He had been at work the day prior to admission, and later that evening became aware of difficulty finding words. The following morning his aphasia had progressed and he was admitted to hospital. General examination was unremarkable with no lymphadenopathy detected. Neurological examination revealed an expressive and receptive aphasia, dysgraphia and dyslexia. There was a mild right upper Address correspondence to Dr J. G. Llewelyn, University Department of Neurology, King's College Hospital and Institute of Psychiatry, Denmark Hill, London SE5 9RS. Vol. 63, No. 755
motor neurone facial weakness with normal limb power and symmetrical reflexes, but an extensor plantar response on the right. Serum investigations including white cell differential count were normal. Computed tomography (CT) showed a well defined non-enhancing low density lesion in the left posteroinferior frontal region, involving grey and white matter, but exerting no appreciable mass effect (Fig. 1) Electroencephalogram showed bifrontal delta activity. Cerebrospinal fluid analysis and digital subtraction arteriography were normal. Despite clinical deterioration, CT brain scan 2 weeks after the first showed very little, if any, progression of the lesion. Magnetic resonance imaging demonstrated an extensive lesion exerting slight but definite mass effect in the left frontal lobe, involving the basal ganglia and corpus callosum. It also revealed other smaller lesions posteriorly in the left cerebral hemisphere and also in the right frontal lobe (Fig. 2). It was also noted that there was a mass in the post-nasal space consistent with adenoidal enlargement (Fig. 3, arrowed) and this latter finding was considered to suggest the possibility of host immunodeficiency. Anti-HIV antibody testing was positive; p24 core antigen titre was negative. Helper (T4): suppressor (T8) lymphocyte ratio was reversed at 0.18. A biopsy taken from the left cerebral cortex showed foci of demyelination with a "snowstorm" appearance in the white matter and grey/white interface, with areas of confluence. Oligodendrocyte nuclei were enlarged, hyperchromatic, with abnormal homogeneous chromatin and some filled with granular material. Bizarre astrocytes were also seen. These appearances were characteristic of PML. Electron microscopy showed uniform viral particles within oligodendrocytes, shown by immunofluorescence to be JC polyoma virus. Information obtained from a male friend of the patient
897
