1976, British Journal of Radiology, 49, 92-94 Case reports REFERENCES
FARACK, U. M., GORESKY, C. A., JABBARI, M., and KIN-
CHRISTIEN, G., BRANTHAMME, J. M., VOLNY, L., DES-
CHAMPS, P., and MORICE, A., 1971. Pylore double: mal-
formation congenitale. Semaine des hdspitaux de Paris, 40,1485-1488.
NEAR, D. G., 1974. Double pylorus: A hypothesis concerning its pathogenesis. Gastroenterology, 66, 596-600. HANSEN, O. H., KRONBORG, O., and PEDERSEN, T., 1972.
The double pylorus. Scandinavian Journal of Gastroenterology, 7, 695-696.
DRAPKIN, R. L., OTSUKA, A. L., CASTELLANOS, H. L., EL HINDI, S., NOWICKA, H., and LEVITAN, R., 1974. Acquisi-
LECHNER, G., RIEDL, P., and DINSTL, K., 1973. Rontgen-
tion of a pyloric septum or pyloric duodenal fistula. Gastroenterology, 66, 1234-1236.
morphologische und funktionelle Veranderungen am Magenausgang nach Vagotomie und Drainageoperationen. Bruns' Beitrdge zur klinischer Chirurgie, 220, 405-412.
A venous malformation of the cavernous sinus By F. I. Spiro, M.B., B.Ch. (Rand), M.R.C.P. (Lond.), F.R.C.R. (Lond.), and L Meyerson, M.B., B.Ch. (Rand), D.O.R.C.P. and S. (England) Medical Towers, 2nd floor, Jeppe Street, Johannesburg, South Africa {Received April, 1975) The corneal sensation and skin temperature of the face were normal and there was no anhydria. The maxillary and mandibular nerves were normal. The left pupil was larger than the right. However, direct and consensual light reflexes were brisk. Media, fundi, tensions and fields were all normal. Corrected visual acuity both sides = 6/5. Case history A clinical diagnosis was made of a partial right oculoA 13-year-old boy was first seen by an ophthalmologist at associated with one year of age when his mother noticed unequal pupils. motor nerve and right abducens nerve lesion was postulated proptosis. Sympathetic involvement At four years of age, his right eye started to protrude and a slight diagnosis of a cyst behind the right eye was made by a to explain the constricted right pupil. A lesion situated in the anterior aspect of the right caverneurologist and ophthalmologist. sinus was diagnosed and the patient was sent for At four and a half years, the child was referred to an nous radiological investigation. orthoptist and his findings were as follows:— Paresis of the right superior oblique and inferior oblique muscles and overaction of the left superior and inferior oblique muscles. Intermittent left hypertropia or right RADIOLOGICAL FINDINGS hypotropia for near distance. Right ptosis when tired. The A plain occipito-frontal view of the orbits (Fig. 1) right pupil was slightly larger than the left. showed the presence of opacities, varying in size from 1 to 2 mm, superimposed on the right sphenoidal PREVIOUS MEDICAL HISTORY The child was born by Caesarian section. He has recurrent fissure and its margins. The margins of the fissure tonsilitis. There is no history of trauma or fits. There is no as well as the greater wing of the sphenoid showed relevant family history. diminution of bone density compared with the RECENT CLINICAL FINDINGS normal left side. (November 13, 1971) The opacities were not visible on the plain An otherwise healthy, white, 13-year-old male presenting with slight proptosis of the right eye. Exophthalmometer lateral view, but were clearly demonstrable on both readings—right eye, 15 mm; left eye, 13 mm. No bruit was antero-posterior and lateral polytomography (Figs. heard and no abnormality of conjunctival veins was observed. There was drooping of the right lid but it was difficult to 2 and 3) to be lying within the anterior portion of determine whether this was due to levator weakness or to the cavernous sinus. The medial wall of the caverhabit closure of the lid in order to suppress the image coming nous sinus was seen to be intact and the pituitary from this eye. fossa was normal. The clinical features and radiological demonstration of a previously unreported venous malformation of the cavernous sinus are described.
MOVEMENTS OF THE EYE
In the primary position of gaze, fixation was done by the left eye and the right eye was observed in a downward and inward position. There was no movement of the right eye past the midline. Left hyperphoria was seen in laevoversion and elevation. Diplopia was not elicited in any position of gaze. 92
Right common carotid angiography demonstrated normal internal and external carotid arteries. No "tumour" vessels were seen in the right orbit or in the right cavernous sinus.
JANUARY
1976
Case reports
fc
FIG. 1. Opacities overlying the right sphenoidal fissure (black arrow).
FIG. 4. Postero-anterior subtraction view of orbital venogram showing "abnormal" vessels (black arrows) overlying the right orbit.
FIG. 2. Antero-posterior tomography demonstrating the position of the opacities (black arrow) at the level of the anterior clinoid process.
FIG. 5. Lateral subtraction view of orbital venogram showing the position of the abnormal vessels in the cavernous sinus.
of the right cavernous sinus corresponding exactly with the opacities seen in the plain films and on polytomography. DISCUSSION
The radiological appearances in this patient suggest a vascular malformation in the anterior portion of the right cavernous sinus. Fig. 3. The fact that it opacities on orbital venography Lateral tomography demonstrating the position of the opacities below and behind the anterior clinoid process and not on arteriography, together with the opacities (black arrows). which are thought to be phleboliths, would suggest that the lesion is a venous malformation. The shape of the cavernous sinus varies conOrbital venography (Figs. 4 and 5) was carried out siderably. According to Viallefont, Paleirac and via a vein in the right frontal region. No abnormality Boudet (1959), there are three fundamental types: was seen in the orbital veins. There was a large (1) A single large venous trunk on either side of the sella tursica. inferior orbital vein on the left side, but this is probably within normal limits. A collection of small (2) A sinus composed of several individual veins running in a general antero-posterior direction. "haphazard" veins was seen in the anterior portion 93
1976, British Journal of Radiology, 49, 94-96 Case reports
(3) A plexiform type where the sinus is composed of a multitude of small veins with rich anastomoses. The lumen of the sinus is traversed by trabeculae giving it the appearance of spongy tissue. Although the right cavernous sinus in this patient appears to consist of multiple channels, the calcification demonstrated within it must be considered as being pathological. Haemangiomata and varices in the orbit are well described (Viallemonte et al., 1959). In some patients a venous malformation may extend outside the orbit into the middle fossa (Hanafee, Sing and Dayton, 1968), through an enlarged sphenoidal fissure (Brauston and Norton, 1963), onto the scalp, frontal bone or hard palate (Lloyd, Wright and Norman, 1971). Lloyd (1974) also describes two patients in whom a haemangioma within the muscle cone was associated with abnormal veins remote from the site of the haemangioma. In both patients these veins lay on the lateral orbital wall. The patient being described showed no evidence of a venous abnormality within the orbit and there was no evidence of an anomaly elsewhere in the skull or face. The condition at the present time is stable and surgery is not contemplated in the immediate future.
CONCLUSIONS
A hitherto undescribed entity of a venous malformation of the right cavernous sinus is described in a 14-year-old boy with proptosis and evidence of involvement of the right sympathetic and third and sixth cranial nerves. The lesion is confined to the cavernous sinus and there is no evidence of a concomitant intra- or extra-orbital venous malformation or of an intra-cranial arterio-venous anomaly. ACKNOWLEDGMENT
We are indebted to Dr. G. Lloyd for his valuable opinion on the radiological changes in this case.
REFERENCES BRAUSTON, B. B., and NORTON, E. N. D., 1963. Intermittent
exophthalmos. American Journal of Ophthalmology, 55, 701-708. HANAFEE, W. N., SING, P. C , and DAYTON, G. O., 1968.
Orbital venography. American Journal of Roentgenology, 104, 29-35. LLOYD, G. A. S., WRIGHT, J. E., and MORGAN, G., 1971.
Venous malformation in the orbit. British Journal of Ophthalmology, 55, 505-516. LLOYD, G. A. S., 1974. Pathological veins in the orbit. British Journal ofRadiology, 47, 570-578. VIALLEFONT, H., PALEIRAC, R., and BOUDET, C , 1959.
The radiological examination of the cavernous sinuses. Bulletin de la Societe Franc Opthalmology 72, 200-219.
Renal cortical necrosis and papillary necrosis in an infant By M. R. Funston, M.B.B.Ch., D.M.R.D., B. J. Cremin, F.R.A.C.R., F.R.C.R., and I. J. K. Tidbury M.B.Ch.B. Department of Radiology, Groote Schuur Hospital, Observatory 7925, Cape Town, So uth Africa {Submitted March, 1975 and in revised form May, 1975)
Bilateral renal cortical necrosis (BRCN) and renal papillary necrosis have been reported in infancy as a complication of gastro-enteritis, perinatal asphyxia, utero-placental haemorrhage, septicaemia, exsanguination, kernicterus, eclampsia, renal vein thrombosis, fetomaternal transfusion and anoxic shock (Mauer and Nogrady, 1969; Leonidas, Berdon and Gribetz, 1971). We wish to report a case of combined BRCN and papillary necrosis, with some unusual radiological findings. Case history A female infant aged three months presented with a fourday history of severe gastro-enteritis followed by convulsions. She was oliguric with metabolic acidosis. The urine contained protein and red cells. The blood urea was 184 and
the serum creatinine 2-5 mg/100 ml. The patient had a spontaneous diuresis without requiring dialysis. In the diuretic phase fluid, electrolyte and acid-base balance was corrected with difficulty. Four weeks after the onset of the illness, an excretory urogram was performed. At the time of the examination the infant was well hydrated, polyuric, and 20 ml. of sodium iothalamate 54 per cent W/V (Conray 325) was injected intravenously. No calcification was present on the control film of the abdomen. Both kidneys were enlarged, measuring 7-5 cm in length. Prompt opacification of the calyces was seen, and all the calyces were noted to be dilated. The ureters were of normal calibre and there was no ureteric or bladder outlet obstruction. A striking feature was dense opacification of the renal cortex and the columns of Bertin, which occurred five minutes after injection of the contrast. The medullary zones were distinctly less opacified. This difference in opacification of the cortex and medulla persisted for 30 minutes. After one hour there was a steady increase in overall kidney density, with equal opacification of the cortex and medulla, which persisted for four days (Fig. 1).
94
FARACK, U. M., GORESKY, C. A., JABBARI, M., and KIN-
CHRISTIEN, G., BRANTHAMME, J. M., VOLNY, L., DES-
CHAMPS, P., and MORICE, A., 1971. Pylore double: mal-
formation congenitale. Semaine des hdspitaux de Paris, 40,1485-1488.
NEAR, D. G., 1974. Double pylorus: A hypothesis concerning its pathogenesis. Gastroenterology, 66, 596-600. HANSEN, O. H., KRONBORG, O., and PEDERSEN, T., 1972.
The double pylorus. Scandinavian Journal of Gastroenterology, 7, 695-696.
DRAPKIN, R. L., OTSUKA, A. L., CASTELLANOS, H. L., EL HINDI, S., NOWICKA, H., and LEVITAN, R., 1974. Acquisi-
LECHNER, G., RIEDL, P., and DINSTL, K., 1973. Rontgen-
tion of a pyloric septum or pyloric duodenal fistula. Gastroenterology, 66, 1234-1236.
morphologische und funktionelle Veranderungen am Magenausgang nach Vagotomie und Drainageoperationen. Bruns' Beitrdge zur klinischer Chirurgie, 220, 405-412.
A venous malformation of the cavernous sinus By F. I. Spiro, M.B., B.Ch. (Rand), M.R.C.P. (Lond.), F.R.C.R. (Lond.), and L Meyerson, M.B., B.Ch. (Rand), D.O.R.C.P. and S. (England) Medical Towers, 2nd floor, Jeppe Street, Johannesburg, South Africa {Received April, 1975) The corneal sensation and skin temperature of the face were normal and there was no anhydria. The maxillary and mandibular nerves were normal. The left pupil was larger than the right. However, direct and consensual light reflexes were brisk. Media, fundi, tensions and fields were all normal. Corrected visual acuity both sides = 6/5. Case history A clinical diagnosis was made of a partial right oculoA 13-year-old boy was first seen by an ophthalmologist at associated with one year of age when his mother noticed unequal pupils. motor nerve and right abducens nerve lesion was postulated proptosis. Sympathetic involvement At four years of age, his right eye started to protrude and a slight diagnosis of a cyst behind the right eye was made by a to explain the constricted right pupil. A lesion situated in the anterior aspect of the right caverneurologist and ophthalmologist. sinus was diagnosed and the patient was sent for At four and a half years, the child was referred to an nous radiological investigation. orthoptist and his findings were as follows:— Paresis of the right superior oblique and inferior oblique muscles and overaction of the left superior and inferior oblique muscles. Intermittent left hypertropia or right RADIOLOGICAL FINDINGS hypotropia for near distance. Right ptosis when tired. The A plain occipito-frontal view of the orbits (Fig. 1) right pupil was slightly larger than the left. showed the presence of opacities, varying in size from 1 to 2 mm, superimposed on the right sphenoidal PREVIOUS MEDICAL HISTORY The child was born by Caesarian section. He has recurrent fissure and its margins. The margins of the fissure tonsilitis. There is no history of trauma or fits. There is no as well as the greater wing of the sphenoid showed relevant family history. diminution of bone density compared with the RECENT CLINICAL FINDINGS normal left side. (November 13, 1971) The opacities were not visible on the plain An otherwise healthy, white, 13-year-old male presenting with slight proptosis of the right eye. Exophthalmometer lateral view, but were clearly demonstrable on both readings—right eye, 15 mm; left eye, 13 mm. No bruit was antero-posterior and lateral polytomography (Figs. heard and no abnormality of conjunctival veins was observed. There was drooping of the right lid but it was difficult to 2 and 3) to be lying within the anterior portion of determine whether this was due to levator weakness or to the cavernous sinus. The medial wall of the caverhabit closure of the lid in order to suppress the image coming nous sinus was seen to be intact and the pituitary from this eye. fossa was normal. The clinical features and radiological demonstration of a previously unreported venous malformation of the cavernous sinus are described.
MOVEMENTS OF THE EYE
In the primary position of gaze, fixation was done by the left eye and the right eye was observed in a downward and inward position. There was no movement of the right eye past the midline. Left hyperphoria was seen in laevoversion and elevation. Diplopia was not elicited in any position of gaze. 92
Right common carotid angiography demonstrated normal internal and external carotid arteries. No "tumour" vessels were seen in the right orbit or in the right cavernous sinus.
JANUARY
1976
Case reports
fc
FIG. 1. Opacities overlying the right sphenoidal fissure (black arrow).
FIG. 4. Postero-anterior subtraction view of orbital venogram showing "abnormal" vessels (black arrows) overlying the right orbit.
FIG. 2. Antero-posterior tomography demonstrating the position of the opacities (black arrow) at the level of the anterior clinoid process.
FIG. 5. Lateral subtraction view of orbital venogram showing the position of the abnormal vessels in the cavernous sinus.
of the right cavernous sinus corresponding exactly with the opacities seen in the plain films and on polytomography. DISCUSSION
The radiological appearances in this patient suggest a vascular malformation in the anterior portion of the right cavernous sinus. Fig. 3. The fact that it opacities on orbital venography Lateral tomography demonstrating the position of the opacities below and behind the anterior clinoid process and not on arteriography, together with the opacities (black arrows). which are thought to be phleboliths, would suggest that the lesion is a venous malformation. The shape of the cavernous sinus varies conOrbital venography (Figs. 4 and 5) was carried out siderably. According to Viallefont, Paleirac and via a vein in the right frontal region. No abnormality Boudet (1959), there are three fundamental types: was seen in the orbital veins. There was a large (1) A single large venous trunk on either side of the sella tursica. inferior orbital vein on the left side, but this is probably within normal limits. A collection of small (2) A sinus composed of several individual veins running in a general antero-posterior direction. "haphazard" veins was seen in the anterior portion 93
1976, British Journal of Radiology, 49, 94-96 Case reports
(3) A plexiform type where the sinus is composed of a multitude of small veins with rich anastomoses. The lumen of the sinus is traversed by trabeculae giving it the appearance of spongy tissue. Although the right cavernous sinus in this patient appears to consist of multiple channels, the calcification demonstrated within it must be considered as being pathological. Haemangiomata and varices in the orbit are well described (Viallemonte et al., 1959). In some patients a venous malformation may extend outside the orbit into the middle fossa (Hanafee, Sing and Dayton, 1968), through an enlarged sphenoidal fissure (Brauston and Norton, 1963), onto the scalp, frontal bone or hard palate (Lloyd, Wright and Norman, 1971). Lloyd (1974) also describes two patients in whom a haemangioma within the muscle cone was associated with abnormal veins remote from the site of the haemangioma. In both patients these veins lay on the lateral orbital wall. The patient being described showed no evidence of a venous abnormality within the orbit and there was no evidence of an anomaly elsewhere in the skull or face. The condition at the present time is stable and surgery is not contemplated in the immediate future.
CONCLUSIONS
A hitherto undescribed entity of a venous malformation of the right cavernous sinus is described in a 14-year-old boy with proptosis and evidence of involvement of the right sympathetic and third and sixth cranial nerves. The lesion is confined to the cavernous sinus and there is no evidence of a concomitant intra- or extra-orbital venous malformation or of an intra-cranial arterio-venous anomaly. ACKNOWLEDGMENT
We are indebted to Dr. G. Lloyd for his valuable opinion on the radiological changes in this case.
REFERENCES BRAUSTON, B. B., and NORTON, E. N. D., 1963. Intermittent
exophthalmos. American Journal of Ophthalmology, 55, 701-708. HANAFEE, W. N., SING, P. C , and DAYTON, G. O., 1968.
Orbital venography. American Journal of Roentgenology, 104, 29-35. LLOYD, G. A. S., WRIGHT, J. E., and MORGAN, G., 1971.
Venous malformation in the orbit. British Journal of Ophthalmology, 55, 505-516. LLOYD, G. A. S., 1974. Pathological veins in the orbit. British Journal ofRadiology, 47, 570-578. VIALLEFONT, H., PALEIRAC, R., and BOUDET, C , 1959.
The radiological examination of the cavernous sinuses. Bulletin de la Societe Franc Opthalmology 72, 200-219.
Renal cortical necrosis and papillary necrosis in an infant By M. R. Funston, M.B.B.Ch., D.M.R.D., B. J. Cremin, F.R.A.C.R., F.R.C.R., and I. J. K. Tidbury M.B.Ch.B. Department of Radiology, Groote Schuur Hospital, Observatory 7925, Cape Town, So uth Africa {Submitted March, 1975 and in revised form May, 1975)
Bilateral renal cortical necrosis (BRCN) and renal papillary necrosis have been reported in infancy as a complication of gastro-enteritis, perinatal asphyxia, utero-placental haemorrhage, septicaemia, exsanguination, kernicterus, eclampsia, renal vein thrombosis, fetomaternal transfusion and anoxic shock (Mauer and Nogrady, 1969; Leonidas, Berdon and Gribetz, 1971). We wish to report a case of combined BRCN and papillary necrosis, with some unusual radiological findings. Case history A female infant aged three months presented with a fourday history of severe gastro-enteritis followed by convulsions. She was oliguric with metabolic acidosis. The urine contained protein and red cells. The blood urea was 184 and
the serum creatinine 2-5 mg/100 ml. The patient had a spontaneous diuresis without requiring dialysis. In the diuretic phase fluid, electrolyte and acid-base balance was corrected with difficulty. Four weeks after the onset of the illness, an excretory urogram was performed. At the time of the examination the infant was well hydrated, polyuric, and 20 ml. of sodium iothalamate 54 per cent W/V (Conray 325) was injected intravenously. No calcification was present on the control film of the abdomen. Both kidneys were enlarged, measuring 7-5 cm in length. Prompt opacification of the calyces was seen, and all the calyces were noted to be dilated. The ureters were of normal calibre and there was no ureteric or bladder outlet obstruction. A striking feature was dense opacification of the renal cortex and the columns of Bertin, which occurred five minutes after injection of the contrast. The medullary zones were distinctly less opacified. This difference in opacification of the cortex and medulla persisted for 30 minutes. After one hour there was a steady increase in overall kidney density, with equal opacification of the cortex and medulla, which persisted for four days (Fig. 1).
94
