SUDDEN ONSET OF BILATERAL SYMMETRICAL PROPTOSIS IN ACUTE INTRACRANIAL HYPERTENSION ABDUR
R. CHOUDHURY, F.R.C.S. Derby, England respiration became stertorous and periodic, and the plantar responses became extensor. During the next few hours, both eyes became proptosed. On admission here, the patient was unconscious with total left hemiplegia, a left-sided, widely dilated and fixed pupil, obvious bilateral proptosis with conjunctival chemosis, and edematous eye lids. The optic disks were edematous. Exophthalmometric readings, taken in the supine position, were R.E.: 28 mm, and L.E.: 29 mm. There was no vertical or horizontal displace ment of the eyes, which were nonpulsatile and could be pressed back easily into the orbits without resistance. The right arm and leg moved and re sponded to painful stimuli but the left arm and leg exhibited only decerebrate postures. Blood pres sure was 160/90 mm Hg, the pulse rate was 70 beats per minute, and the temperature was 36.7°C. In view of the left-sided, dilated, fixed pupil, the ipsilateral hemiplegia, and a history of leftsided headaches, the diagnosis was chronic subdural hematoma. The left hemiplegia was con sidered to be a false localizing sign. We believed that the sudden deterioration of consciousness and the development of other neurologic signs were the result of acute tentorial coning after the lumbar puncture. Plain radiographs of the skull taken elsewhere were noncontributory. We infused 350 ml of 20% mannitol solution for a short period before cerebral angiography and surgery. The carotid angiogram (Figure) con firmed the presence of a giant left-sided, subdural hematoma that was evacuated through the pos terior parietal and frontal burr holes. The sub dural blood was liquid and of varying colors, presumably due to its long duration. After evacua tion of the hematoma, we found that the surface of the hemisphere was 4 cm deep to the dura mater in the frontal region. Seven postoperative subdural taps were done at increasing time intervals. The aspirated fluid de creased in each successive tap, and no fluid was aspirated on the last occasion. The shift of the midline structures was assessed by serial echo encephalograms. On the day after surgery, the patient moved his left arm and leg but developed a right-sided total hemiplegia. Four days after the operation, the patient was conscious but aphasic. One week after the operation there was improvement in the proptosis, which virtually disappeared by the middle of the second week. During the third week, the patient spoke several words and movement had returned to the right arm and leg. He then made steady progress. By the fifth week, right facial weakness had almost dis-
Sudden onset of bilateral symmetrical proptosis, a rare clinical condition, results from a symmetrical increase in the intraorbital contents. Although malignant exophthalmos, due to generalized deposition of hydrophilic mucopolysaccharide in the orbits, is generally bilateral, its onset is gradual. According to Duke-Elder and Scott, 1 bilateral symmetrical proptosis may develop suddenly in a subarachnoid hemor rhage from a ruptured intracranial aneurysm. It is probably caused by venous con gestion in the orbit, resulting from intra cranial hypertension. C A S E REPORT
A 32-year-old man presented on July 24, 1974, with an eight-month history of early morning vomiting and intense left frontal headache of one month's duration. During recent months, vomiting had increased to two or three times daily. The headache had persisted day and night for two weeks. One week before admission, there was a blood-stained, loose stool. There was no history of fits, visual disturbances, or head injury. He had been treated for cervical tuberculous adenitis for one year. Examination elsewhere showed full conscious ness and noncontributing neurologic signs. Tests for typhoid and infectious hepatitis were negative. On July 27, the patient became confused and drowsy. Examination disclosed bilateral partial sixth cranial nerve palsy. The left pupil was larger than the right but both pupils reacted briskly to light. In order to exclude tuberculous meningitis, a lumbar puncture was done after infusing 200 ml of 10% mannitol solution. The xanthochromic cerebrospinal fluid had a pressure of 160 mm H 2 0, containing 10 lymphocyte cells/mm"; protein was 10 mg/100 ml; and glucose, 53 mg/100 ml. There was a free rise and fall of fluid during the Queckenstedt's sign. The acid-fast stain was negative. After the lumbar puncture, the patient became stuporous, the left pupil became dilated and fixed, From the Department of Neoursurgery, Derby shire Royal Infirmary, Derby, England. Reprint requests to Abdur R. Choudhury, F.R.C.S., Department of Neurosurgery, Derby shire Royal Infirmary, Derby, England. 85
86
AMERICAN JOURNAL OF OPHTHALMOLOGY
JULY, 197S
plegia. Acute tentorial coning probably re sulted in further aggravation of the intra cranial hypertension which, in turn, pro duced increased intracranial venous pres sure. The increased intracranial venous pres sure was transmitted from the intracranial i
Figure (Choudhury). Left carotid angiogram. Anteroposterior view of the late arterial phase, showing the subdural hematoma and a marked shift of the anterior cerebral artery to the right. appeared. The patient walked with support and his speech was greatly improved. Ten weeks later, he had virtually recovered. There were no unusual neurologic signs and the exophthalmometric read ings were R.E.: 19 mm, and L.E.: 18 mm. DISCUSSION
A history of vomiting and increasingly severe headaches indicated the presence of increased intracranial pressure. The tradi tional view is that lumbar puncture is contraindicated in the presence of papilledema. However, papilledema often may be absent in intracranial hypertension, and careful consideration should be given before per forming lumbar puncture in any patient with possible hypertension. Lumbar puncture performed elsewhere in our patient precipitated acute tentorial con ing. A consequence of temporal lobe hernia is displacement of the midbrain toward the opposite side, compressing the opposite cerebral peduncle against the sharp edge of the tentorium, and causing ipsilateral hemi-
cavity to the orbital veins, resulting in or bital venous stasis and the sudden onset of bilateral proptosis. Subsequent venous con gestion and edema of the orbital tissues then caused progression and a further increase in the intraorbital contents. Bilateral sym metrical proptosis then resulted. The gradual disappearance of the proptosis after release of the intracranial hypertension supports this hypothesis. The superior and inferior ophthalmic veins in the orbit drain the posterior orbit and empty into the cavernous sinus while the frontal, nasal, and anterior facial veins drain the anterior orbit and empty into the common facial vein. The two systems com municate freely; they also communicate through the pterygoid venous plexus. In addition, absence of valves in these venous systems provides an alternative drainage be tween them. With such free communication, venous stasis due to increased intracranial venous pressure that produces proptosis is un usual. However, reports that noninflamma tory lesions near the cavernous sinus pro duce ipsilateral proptosis attest to the in adequacy of anterior orbital venous drain age. Dixon 2 and Meadows3 described cases of exophthalmos due to pituitary adenomas. Aneurysms near Willis' circle may produce similar changes, presumably by the same mechanism.2'4 However, because venous stasis develops slowly in these conditions, proptosis is gradual. Association of hemorrhage into the extraocular orbital tissues and optic nerve sheath was reported in cases of sudden intracranial hypertension.5'6 This factor may contribute to minor degrees of bilateral proptosis but it does not explain the presence of such
VOL. 80, NO. 1
BILATERAL SYMMETRICAL PROPTOSIS
extraocular congestive features as conjunctival chemosis and edematous eyelids, the absence of any visual disturbances, and the occurrence of bilateral gross symmetrical proptosis. SUMMARY
A 32-year-old man had the clinical fea tures of progressive increase in intracranial pressure due to a chronic subdural hematoma except for papilledema. Lumbar punc ture, however, precipitated acute tentorial coning and severe neurologic signs. The tentorial coning resulted in an acute aggra vation of intracranial hypertension and precipitated an increase in the intracranial venous pressure that was transmitted to the orbital veins, causing orbital venous stasis, symmetrical increase in the intraorbital con tents, and symmetrical bilateral proptosis.
87
ACKNOWLEDGMENTS
I thank J. C. Taylor, F.R.C.S., for permission to report this case, and R. Whitaker, F.F.R., for permission to reproduce the radiograph. REFERENCES
1. Duke-Elder, S., and Scott, G. I.: NeuroOphthalmology. In Duke-Elder, S. (ed.): System of Ophthalmology, vol. 12. London, Henry Kimpton, 1971, p. 31. 2. Dixon, G. J.: Unilateral exophthalmos (prop tosis). Causation and differential diagnosis. Brain 64:73, 1941. 3. Meadows, S. P.: Orbital tumours. Proc. R. Soc. Med. 38:594, 194S. 4. Jefferson, G.: Compression of chiasma, optic nerves and optic tracts by intracranial aneurysms. Brain 60:444, 1937. 5. Walsh, F. B., and Hedges, T. R.: Optic nerve sheath hemorrhage. Am. J. Ophthalmol. 34: 509, 1951. 6. Muller, P. J., and Deck, J. H. N.: Intra ocular and optic nerve sheath hemorrhage in cases of sudden intracranial hypertension. J. Neurosurg. 41:160, 1974.
O P H T H A L M I C MINIATURE
" . . . W h e r e did it go to?" "We don't know, miss." Harriet looked at the stout and agitated Carrie, whose face was puck ered and twitching and her eyes bolting with imminent hysteria. She had never thought the present head scout any too dependable, and was in clined to put down her abundant energy to an excess of thyroid. Dorothy L. Sayers Gaudy Night
R. CHOUDHURY, F.R.C.S. Derby, England respiration became stertorous and periodic, and the plantar responses became extensor. During the next few hours, both eyes became proptosed. On admission here, the patient was unconscious with total left hemiplegia, a left-sided, widely dilated and fixed pupil, obvious bilateral proptosis with conjunctival chemosis, and edematous eye lids. The optic disks were edematous. Exophthalmometric readings, taken in the supine position, were R.E.: 28 mm, and L.E.: 29 mm. There was no vertical or horizontal displace ment of the eyes, which were nonpulsatile and could be pressed back easily into the orbits without resistance. The right arm and leg moved and re sponded to painful stimuli but the left arm and leg exhibited only decerebrate postures. Blood pres sure was 160/90 mm Hg, the pulse rate was 70 beats per minute, and the temperature was 36.7°C. In view of the left-sided, dilated, fixed pupil, the ipsilateral hemiplegia, and a history of leftsided headaches, the diagnosis was chronic subdural hematoma. The left hemiplegia was con sidered to be a false localizing sign. We believed that the sudden deterioration of consciousness and the development of other neurologic signs were the result of acute tentorial coning after the lumbar puncture. Plain radiographs of the skull taken elsewhere were noncontributory. We infused 350 ml of 20% mannitol solution for a short period before cerebral angiography and surgery. The carotid angiogram (Figure) con firmed the presence of a giant left-sided, subdural hematoma that was evacuated through the pos terior parietal and frontal burr holes. The sub dural blood was liquid and of varying colors, presumably due to its long duration. After evacua tion of the hematoma, we found that the surface of the hemisphere was 4 cm deep to the dura mater in the frontal region. Seven postoperative subdural taps were done at increasing time intervals. The aspirated fluid de creased in each successive tap, and no fluid was aspirated on the last occasion. The shift of the midline structures was assessed by serial echo encephalograms. On the day after surgery, the patient moved his left arm and leg but developed a right-sided total hemiplegia. Four days after the operation, the patient was conscious but aphasic. One week after the operation there was improvement in the proptosis, which virtually disappeared by the middle of the second week. During the third week, the patient spoke several words and movement had returned to the right arm and leg. He then made steady progress. By the fifth week, right facial weakness had almost dis-
Sudden onset of bilateral symmetrical proptosis, a rare clinical condition, results from a symmetrical increase in the intraorbital contents. Although malignant exophthalmos, due to generalized deposition of hydrophilic mucopolysaccharide in the orbits, is generally bilateral, its onset is gradual. According to Duke-Elder and Scott, 1 bilateral symmetrical proptosis may develop suddenly in a subarachnoid hemor rhage from a ruptured intracranial aneurysm. It is probably caused by venous con gestion in the orbit, resulting from intra cranial hypertension. C A S E REPORT
A 32-year-old man presented on July 24, 1974, with an eight-month history of early morning vomiting and intense left frontal headache of one month's duration. During recent months, vomiting had increased to two or three times daily. The headache had persisted day and night for two weeks. One week before admission, there was a blood-stained, loose stool. There was no history of fits, visual disturbances, or head injury. He had been treated for cervical tuberculous adenitis for one year. Examination elsewhere showed full conscious ness and noncontributing neurologic signs. Tests for typhoid and infectious hepatitis were negative. On July 27, the patient became confused and drowsy. Examination disclosed bilateral partial sixth cranial nerve palsy. The left pupil was larger than the right but both pupils reacted briskly to light. In order to exclude tuberculous meningitis, a lumbar puncture was done after infusing 200 ml of 10% mannitol solution. The xanthochromic cerebrospinal fluid had a pressure of 160 mm H 2 0, containing 10 lymphocyte cells/mm"; protein was 10 mg/100 ml; and glucose, 53 mg/100 ml. There was a free rise and fall of fluid during the Queckenstedt's sign. The acid-fast stain was negative. After the lumbar puncture, the patient became stuporous, the left pupil became dilated and fixed, From the Department of Neoursurgery, Derby shire Royal Infirmary, Derby, England. Reprint requests to Abdur R. Choudhury, F.R.C.S., Department of Neurosurgery, Derby shire Royal Infirmary, Derby, England. 85
86
AMERICAN JOURNAL OF OPHTHALMOLOGY
JULY, 197S
plegia. Acute tentorial coning probably re sulted in further aggravation of the intra cranial hypertension which, in turn, pro duced increased intracranial venous pres sure. The increased intracranial venous pres sure was transmitted from the intracranial i
Figure (Choudhury). Left carotid angiogram. Anteroposterior view of the late arterial phase, showing the subdural hematoma and a marked shift of the anterior cerebral artery to the right. appeared. The patient walked with support and his speech was greatly improved. Ten weeks later, he had virtually recovered. There were no unusual neurologic signs and the exophthalmometric read ings were R.E.: 19 mm, and L.E.: 18 mm. DISCUSSION
A history of vomiting and increasingly severe headaches indicated the presence of increased intracranial pressure. The tradi tional view is that lumbar puncture is contraindicated in the presence of papilledema. However, papilledema often may be absent in intracranial hypertension, and careful consideration should be given before per forming lumbar puncture in any patient with possible hypertension. Lumbar puncture performed elsewhere in our patient precipitated acute tentorial con ing. A consequence of temporal lobe hernia is displacement of the midbrain toward the opposite side, compressing the opposite cerebral peduncle against the sharp edge of the tentorium, and causing ipsilateral hemi-
cavity to the orbital veins, resulting in or bital venous stasis and the sudden onset of bilateral proptosis. Subsequent venous con gestion and edema of the orbital tissues then caused progression and a further increase in the intraorbital contents. Bilateral sym metrical proptosis then resulted. The gradual disappearance of the proptosis after release of the intracranial hypertension supports this hypothesis. The superior and inferior ophthalmic veins in the orbit drain the posterior orbit and empty into the cavernous sinus while the frontal, nasal, and anterior facial veins drain the anterior orbit and empty into the common facial vein. The two systems com municate freely; they also communicate through the pterygoid venous plexus. In addition, absence of valves in these venous systems provides an alternative drainage be tween them. With such free communication, venous stasis due to increased intracranial venous pressure that produces proptosis is un usual. However, reports that noninflamma tory lesions near the cavernous sinus pro duce ipsilateral proptosis attest to the in adequacy of anterior orbital venous drain age. Dixon 2 and Meadows3 described cases of exophthalmos due to pituitary adenomas. Aneurysms near Willis' circle may produce similar changes, presumably by the same mechanism.2'4 However, because venous stasis develops slowly in these conditions, proptosis is gradual. Association of hemorrhage into the extraocular orbital tissues and optic nerve sheath was reported in cases of sudden intracranial hypertension.5'6 This factor may contribute to minor degrees of bilateral proptosis but it does not explain the presence of such
VOL. 80, NO. 1
BILATERAL SYMMETRICAL PROPTOSIS
extraocular congestive features as conjunctival chemosis and edematous eyelids, the absence of any visual disturbances, and the occurrence of bilateral gross symmetrical proptosis. SUMMARY
A 32-year-old man had the clinical fea tures of progressive increase in intracranial pressure due to a chronic subdural hematoma except for papilledema. Lumbar punc ture, however, precipitated acute tentorial coning and severe neurologic signs. The tentorial coning resulted in an acute aggra vation of intracranial hypertension and precipitated an increase in the intracranial venous pressure that was transmitted to the orbital veins, causing orbital venous stasis, symmetrical increase in the intraorbital con tents, and symmetrical bilateral proptosis.
87
ACKNOWLEDGMENTS
I thank J. C. Taylor, F.R.C.S., for permission to report this case, and R. Whitaker, F.F.R., for permission to reproduce the radiograph. REFERENCES
1. Duke-Elder, S., and Scott, G. I.: NeuroOphthalmology. In Duke-Elder, S. (ed.): System of Ophthalmology, vol. 12. London, Henry Kimpton, 1971, p. 31. 2. Dixon, G. J.: Unilateral exophthalmos (prop tosis). Causation and differential diagnosis. Brain 64:73, 1941. 3. Meadows, S. P.: Orbital tumours. Proc. R. Soc. Med. 38:594, 194S. 4. Jefferson, G.: Compression of chiasma, optic nerves and optic tracts by intracranial aneurysms. Brain 60:444, 1937. 5. Walsh, F. B., and Hedges, T. R.: Optic nerve sheath hemorrhage. Am. J. Ophthalmol. 34: 509, 1951. 6. Muller, P. J., and Deck, J. H. N.: Intra ocular and optic nerve sheath hemorrhage in cases of sudden intracranial hypertension. J. Neurosurg. 41:160, 1974.
O P H T H A L M I C MINIATURE
" . . . W h e r e did it go to?" "We don't know, miss." Harriet looked at the stout and agitated Carrie, whose face was puck ered and twitching and her eyes bolting with imminent hysteria. She had never thought the present head scout any too dependable, and was in clined to put down her abundant energy to an excess of thyroid. Dorothy L. Sayers Gaudy Night
