A LTHOUGH the first operation for brain-stem

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1 J Neurosurg 56" , 1982 Brain-stem hematoma A report of six surgically treated cases SEAN A. O'LAOIRE, M.D., F.R.C.S.I., F.R.C.S., H. ALAN CROCKARD, F.R.C.S., DAVID G. T. THOMAS, M.R.C.P., F.R.C.S., AND DEREK S. GORDON, M.CH., F.R.C.S. Atkinson Morley's Hospital and National Hospital for Nervous Diseases, London, England, and Royal Victoria Hospital, Belfast, Northern Ireland ~/ Six cases of brain-stem hematoma are described. The clinical presentation in these cases was extremely variable. Operative evacuation of the hematoma resulted in immediate improvement in all cases, and a full or nearly full recovery in five. Untreated brain-stem hematoma is probably always fatal, whereas surgical evacuation is effective and safe. The diagnosis of brain-stem hematoma should be considered in any case of brain-stem lesion. KEY WORDS 9 brain-stem hematoma 9 cryptic arteriovenous malformation 9 surgical evacuation A LTHOUGH the first operation for brain-stem hematoma was carded out by Dandy in 1932, other reports of surgical treatment of these lesions have been confined to a small number of isolated cases. 1,2,4-n,la,15-2~ The authors have had experience with six cases, one of which has been reported previously; 5 these cases form the basis of this report. Case 1 Case Reports This 28-year-old man presented in August, 1971, with a steadily progressive history lasting 2 months which began with tingling around the mouth. He then developed tingling and clumsiness of the right limbs, slurred speech, and double vision. On examination, he was ataxic and dysarthric. He had marked impairment of horizontal eye movements, weakness and sensory loss of the right limbs, and a left seventh nerve palsy. Investigation with a combined air ventriculogram and Myodil myelogram showed expansion of the brain stem with a mass protruding into the fourth ventricle (Fig. 1). At operation through a midline approach, the floor of the fourth ventricle was found to be expanded and discolored blue. A mixture of liquid blood and solid clot extruded when the ependyma was incised. The wall of the clot cavity was yellow but there was no other abnormality. Histological examination of the clot revealed no evidence of tumor or blood vessels. The patient made a slow but steady recovery and returned to his previous job 9 months postoperatively. His facial palsy is unchanged and he remains minireally ataxic. Case 2 This 14-year-old grid presented in November, 1978, with a 2-month history of progressive neurological deterioration, which began with tingling around the right side of the face. She became unsteady on her feet, then drowsy, and she Irmally developed double vision. On examination, she was drowsy with a left ptosis and sixth nerve palsy, a right fifth nerve palsy, nystagmus, and ataxia of the fight limbs. Computerized tomography (CT) scan showed areas of high and low attenuation in the brain stem (Fig. 2). Angiography showed vessel displacement but no abnormal vessels, and an air encephalogram confirmed expansion of the brain stem. At operation through a left cerebeuopontine angle approach, the pons was found to be enlarged and discolored blue. Several cubic centimeters of liquid hematoma was evacuated with a needle. Microscopic examination of the clot revealed no abnormality. The patient improved steadily and has been leading a 222 J. Neurosurg. / Volume 56 / February, 1982

2 Brain-stem hematoma FIG. 2. Case 2. Unenhanced computerized tomography scan demonstrating a mass of high attenuation in the pons, approaching the surface in the floor of the fourth ventricle and in the left cerebellopontine angle. FIG. 1. Case 1. Combined Myodil and air study (by the lumbar route) demonstrating a swollen brain stem with distortion of the floor of the fourth ventricle. normal life without residual deficits since 3 months postoperatively. Case 3 This 15-year-old girl presented in September, 1979, with a rapidly progressive deterioration over a period of 36 hours. She first developed vomiting followed by headache. Twelve hours later she became unsteady, and fmauy she became increasingly drowsy, with slurred speech and incoordinate movements. On examination, she was drowsy and dysarthric. She had marked impairment of all eye movements, bilateral ptosis, bilateral seventh nerve palsies, and bilateral ataxia. ACT scan suggested hemorrhage within the brain stem (Fig. 3), and angiography showed vessel displacement but no abnormal vasculature (Fig. 4). At exploration through the right cerebellopontine angle, a grossly enlarged pons, discolored blue, was found, and 6 cc of liquid hematoma was evacuated with a needle. Microscopic examination of the dot revealed no abnormality. The patient recovered very rapidly and was walking around without ataxia 3 days postoperatively. Within a week she had lost all neurological abnormalities, and remains well. Case 4 This 26-year-old woman presented in March, 1980, with an acute onset of tingling in the left arm, FIG. 3. Case 3. Unenhanced computerized tomography scan demonstrating a large pontine area of nonhomogeneous high attenuation, approaching the surface in the right cerebellopontine angle. which was followed within 15 minutes by headache and vomiting, then drowsiness and confusion. Examination revealed neck stiffness. She was drowsy and dysarthric, had dysconjugate impairment of eye movements, nystagmus, a left-sided hemianalgesia and right-sided ataxia. Lumbar puncture produced uniformly blood-stained fluid. A CT scan showed blood high in the brain stem on the right side and some degree of hydrocephalus (Fig. 5). Angiography was interpreted as showing a small arteriovenous malformation in the region of the superior cerebellar peduncle (Fig. 6). She deteriorated further thereafter J. Neurosurg. / Volume 56 / February,

3 S. A. O'Laoire, et al. FIG. 4. Case 3. Vertebral angiogram demonstrating displacement of the anterior inferior cerebeuar artery (arrows), suggestive of an intrinsic pontine mass. due to increasing hydrocephalus. A ventriculoperitoneal (VP) shunt was inserted, but she did not improve despite evidence of a return to normal ventricular size on sequential CT scans. At operation 10 days following the ictus, the right side of the brain stem was inspected through a combined supra- and infratentorial approach. There was no evidence of an arteriovenous malformation, but there was a subpial hematoma in the superior cerebellar peduncle on the right side. Several cubic centimeters of liquid hematoma was evacuated. Microscopic examination of the clot revealed no abnormality. A slow recovery started immediately. By 3 months postoperatively the patient was fully recovered, and she remains well. Case 5 This 26-year-old man presented in February, 1981, with a 2 89 history of stepwise neurological deterioration. His story began with tingling in the mouth which spread to involve the entire left side. The left limbs then became clumsy. He developed slurred speech, double vision, and finally clumsiness of the right limbs. Each symptom became obvious rather abruptly, the previous ones having been static for several days. On examination, he was dysarthric, with impairment of vertical eye movements, nystagmus, a left seventh nerve palsy, bilateral ataxia, worse on the left, and spasticity of gait. ACT scan was suggestive of extensive hemorrhage within the brain stem, coming nearest to the surface in the right cerebellopontine angle (Fig. 7). Angiography showed no abnormal vessels, but did show appropriate displacement. At operation through the right cerebellopontine angle, the pons was found to be enlarged and discol- FIG. 5. Case 4. Unenhanced computerized tomography scan demonstrating an area of high attenuation inthe region of the right superior cerebellar peduncle. ored blue. Several cubic centimeters of liquid hematoma was evacuated through a needle. Microscopic examination of the clot revealed no abnormality. There was immediate improvement in the patient's eye movements and in the facial weakness. Three months later he was minimally ataxic and exhibited mild impairment of upward gaze. Case 6 This 53-year-old man presented in February, 1981, with a 2-month history of alternating neurological deficit and improvement. He developed acute onset of double vision, followed by unsteadiness which persisted. He collapsed some 2 weeks later, recovered rapidly but remained dysarthric with bilateral partial ptosis, impairment of eye movements, and spasticity and ataxia of limbs. He made a slow partial recovery over a period of 3 weeks, but developed complete ptosis with increasing drowsiness over the next 5 days. Examination then showed him to be drowsy and dysarthric, with complete loss of eye movements, and spasticity and ataxia of his limbs. Eighteen months before, he had had an episode of transient double vision and nystagmus. ACT scan showed areas of low and high attenuation in the brain stem (Fig. 8). The high attenuation areas were suggestive of blood clot which came nearest the surface in the floor of the fourth ventricle. Angiography showed no abnormality. At operation through a midline fourth ventricular approach, a blue, domed swelling was seen in the floor of the fourth ventricle in its upper region. Several cubic centimeters of liquid hematoma was evacuated under pressure when the ependyma was disrupted, and a good flow of cerebrospinal fluid immediately came through the aqueduct. The wall of the clot cavity was comprised of yellowish white matter. Histological 224 J. Neurosurg. / Volume 56 / February, 1982

4 Brain-stem hematoma FIG. 6. Case 4. Vertebral angiogram anteroposterior view (left) and lateral view (right) showing a lesion, interpreted preoperatively as an arteriovenous malformation (arrows), in the region of the right superior cerebeuar peduncle. examination of the clot showed no evidence of tumor or blood vessels. The patient improved immediately, becoming fully alert. There was resolution of his ptosis and return of some eye movements. Five days later, he deteriorated, with further drowsiness, and a CT scan showed increased hydrocephalus. A VP shunt was inserted. He improved but deteriorated again within 2 days due to a severe pulmonary infection, which proved resistant to all treatment. He died 3 weeks later due to multiple lung abscesses. Permission for autopsy was not obtained. Discussion The cause of hemorrhage was not identified in any of our cases (despite the suggestive angiographic findings in Case 4), nor has it been found in the majority of reported cases. Fragments of abnormal blood vessels have been identified in the clot evacuated in one case, however; 19 and, although rupture of a microaneurysm has been suggested as a cause of brainstem hematoma, 16,2~ most operated cases have been attributed to cryptic arteriovenous malformations (AVM's). 1,2,4-11,13,15,17,19 Several major pathological studies have reported on such lesions, 3,12,14,22,2a both as FIG. 7. Case 5. Unenhanced computerized tomography scan demonstrating a mass of high attenuation in the brain stem, approaching the surface high in the right cerebellopontine angle. FIG. 8. Case 6. Computerized tomography scan showing a lesion high in the brain stem nearest the surface in the floor of the fourth ventricle. Note that the lesion is of mixed attenuation on the unenhanced view (left), and that it enhances strongly after injection of intravenous contrast material (righo. J. Neurosurg. / Volume 56 / February,

5 S. A. O'Laoire, et al. incidental findings at autopsy and as causes of fatal hemorrhage. It is noteworthy that the pons is the commonest site of incidental cryptic AVM's24 Although the evidence is circumstantial, rupture of such a lesion appears the most likely explanation of brainstem hematoma. The lesions are frequently so disrupted by the hemorrhage that identification of blood vessels in the clot may be impossible, a,~2,14 Failure to identify angioma vessels in the clot is probably not due to complete destruction of the AVM, but to the difficulty of finding a very small lesion, which is often fragmented, in a relatively large volume of hematoma. a,12 It is conceivable that the angiographically demonstrated lesion in our Case 4 was partly destroyed in a further hemorrhage between the time of angiography and surgery. The natural history of brain-stem hemorrhage is not well documented. Before the introduction of effective hypotensive agents, major brain-stem hemorrhage was a common finding in patients dying of malignant hypertension, z~ In our cases, however, and in all reported cases operated on, the patients have been normotensive and the clinical presentation has not been so catastrophic. Moreover, most reported cases of brain-stem hematoma ascribed to rupture of a cryptic AVM have presented with a deteriorating neurological condition; either steadily progressing over days or weeks, or alternately relapsing and improving over a period of weeks, months, or, rarely years until either surgery, x,2,4-11,1a,15-2~ or death. 12,14,22 Further evidence of the progressive nature of brainstem hematoma is available in the series of Kowada, et al, 1~ of seven cases diagnosed in life, of whom five untreated patients died; two were operated on after 6- week periods of coma (one by evacuation of the hematoma, one by a shunt) without significant recovery. There are no reports of spontaneous recovery. The majority of patients operated on, on the other hand, have done well. 1,2,4-9,x1,13,15-20 With the exception of the case of Kowada, et al, 1~ all patients reported have returned to their previous occupations, the majority with minimal or no residual deficit. Our experience also indicates a good prognosis in these cases. There does not seem to be any typical clinical picture of brain-stem hematoma due to rupture of a cryptic AVM, either in the literature or in our experience. The commonest presentations appear to be either progressive deterioration, suggesting a pontine glioma, 4,x3,15,~7-19 or the relapsing or stepwise deterioration mimicking demyelination. 1,2~~ This entity may also present with the acute onset of a stroke s,1~ or of a subarachnoid hemorrhage, 9,xl or with a subacute onset suggesting brain-stem encephalitis (Case 3). The condition is dearly rare, but may be recognized more frequently as use of the CT scanner becomes more widespread. However, the CT appearance may not be characteristic of hemorrhage (Fig. 8), and we believe that the good prognosis for surgically treated brain-stem hematoma adds to the argument in favor of establishing a histological diagnosis in all cases of suspected pontine glioma. We are aware that our decision not to biopsy the wall of the hematoma cavity carries some risk of missing a hemorrhagic tumor; we believe, however, that careful microscopic examination of the clot is adequate, and that biopsy of the brain-stem tissue would carry an unnecessary risk. The few reported patients in whom biopsy was carded out deteriorated markedly postoperatively, although all recovered eventually.x7.xa Furthermore, four of our patients have been followed postoperatively for more than 2 years without evidence of recurrent disease, and follow-up review has been prolonged and uneventful in most of the reported cases. The clinical presentation is extremely variable, and brain-stem hematoma should therefore be considered in the differential diagnosis of any patient with brainstem signs, particularly children and young adults. Surgical treatment is safe and effective. Although the evidence is circumstantial, an untreated brain-stem hematoma is likely to prove fatal. References 1. Abroms IF, Yessayan L, Shillito J, et al" Spontaneous intracerebral haemorrhage in patients suspected of multiple sclerosis. J Neurol Neurosurg Psychiatry 34: , Arseni C, Stanciu M: Primary haematomas of the brain stem. Acts Neurochir 28: , Crawford JV, Russell DS: Cryptic arteriovenous and venous hamartomas of the brain. J Neurol Neurosurg Psychiatry 19:.1-11, Dandy WE" The Brain. New York: Harper and Row, 1969, pp Doczi T, Thomas DGT: Successful removal of an intrapontine haematoma. J Neurol Neurosurg Psychiatry 42: , Gros C, Giraud G, Latour H: H6matome spontan6 de la calote protub6rantieue oper6 et gueri. Presse Med 56:890, Humphreys RP: Computerized tomographic definition of mesencephalic hematoma with evacuation through pedunculotomy. Case report. J Neurosurg 49: , Kempe LG: Surgical removal of an intramedullary haematoma simulating Wallenberg's syndrome. J Neurol Neurosurg Psychiatry 27:78-80, Koos WT, Sunder-Plassmann M, Salah S: Successful removal of a large intrapontine hematoma. Case report. J Neurosurg 31: , Kowada M, Ito Z, Matsuoka S, et al: Primary pontine haemorrhage revealed by pneumoencephalo-roulette tomography, and a report on surgically treated cases. Acta Neurochir 25: , 1971 l l. La Torte E, Delitala A, Sorano V: Hematoma of the quadrigeminal plate. Case report. J Neurosurg 49: , Margolis G, Odom GL, Woodhall B, et al: The role of small angiomatous malformations in the production of intracerebral hematomas. J Neurosurg 8: , Matson DD: Surgery of posterior fossa tumors in child- 226 J. Neurosurg. / Volume 56 / February, 1982

6 Brain-stem hematoma hood. Clin Neurosurg 15: , McCormick WF, Nofzinger JD: "Cryptic" vascular malformations of the central nervous system. J Neurosurg 24: , Murphy MG: Successful evacuation of acute pontine hematoma. Case report. J Neurosurg 37: , Obrador S, Dierssen G, Odoriz B J: Surgical evacuation of a pontine-medullary hematoma. Case report. J New rosurg 33:82-84, Pak H, Patel SC, Malik GM, et al: Successful evacuation of a pontine hematoma secondary to rupture of a venous angioma. Surg Neurol 15: , Papo I, Pasquini U, Salvolini U: Subependymal brainstem hematomas: a report of two cases. Neuroradiology 11: , Scott BB, Seeger JF, Schneider RC: Successful evacuation of a pontine hematoma secondary to rupture of a pathologically diagnosed "cryptic" vascular malformation. Case report. J Ncurosurg 39: , ScoviUe WB, Poppen JL: Intrapeduncular hemorrhage of the brain. Successful operative approach, with evacuation of clot and a seven and one-fourth year observation period. Arch Neurol Psychiatry 61: , Silverstein A: Primary pontile hemorrhage. A review of 50 cases. Confin Neurol 29:.33-46, Teilmann K: Hemangiomas of the pons. Arch Neuroi Psychiatry 6~ , White R J, Kernohan JW, Wood MW: A study of fifty intracranial vascular tumors found incidentally at necropsy. J Neuropathol Exp Neurol 17: , 1958 Manuscript received June 10, Accepted in final form September 11, This paper was presented in part at a joint meeting of the Society of British Neurological Surgeons and the Soci6t6 Franfaise de Neurochirurgie, at Bendor, France, April 7-11, Address for Mr. Crockard and Mr. Thomas: National Hospital for Nervous Diseases, London, England. Address for Mr. Gordon: Royal Victoria Hospital, Belfast, Northern Ireland. Address reprint requests to: Sean A. O'Laoire, M.D., F.R.C.S., Consultant Neurosurgeon, Atkinson Morley's Hospital, 31 Copse Hill, Wimbledon, London SW20 ONE, England. J. Neurosurg. / Volume 56 / February,