Surgical Excision of Familial Cavernous Angioma in the Pontomedullary —

Hidemitsu

Case

NAKAGAWA, Akira

Department

Report—

Yoshihiro

MURASAWA

of Neurosurgery,

Region

and

FURUTA, Yoshikazu

Shin

NAKAJIMA,

NAKAJIMA

The Center for Adult Diseases,

Osaka, Osaka

Abstract The - old

authors male

lar signs,

report

exhibited without

on a surgical

case of cavernous

a progressive

neurological

ictal episodes,

and computed

angioma

deficit

in the pontomedullary

consisting

tomographic

mainly scans

of bulbar

showed

growth

region. paresis

A 60-year and cerebel

of the lesion

over 3

years. On preoperative assessment, a glial tumor was suspected to coexist with a cavernous angioma. The vascular malformation was totally excised successfully. This case also revealed an unusual familial occurrence. Key words:

cavernous

angioma,

brainstem,

familial

Introduction

Case

An intracranial cavernous angioma is a relatively uncommon congenital vascular malformation. 10,14) Many are clinically asymptomatic and are found only at autopsy.'"') Cavernous angiomas may cause uncontrollable seizures, hemorrhage or neurological deficits.21,21) Therefore, those lesions located in a non-eloquent zone should be excised surgically, and the outcome is usually favorable.""') On the other hand, when the lesion is located in an eloquent zone and the risk of surgery is thought to be unacceptable, conservative therapy is advisable, and the follow-up computed tomography (CT) or magnetic resonance (MR) imaging can give information as to the size of the lesion. When the presence of a tumor cannot be completely excluded, or the lesion has already caused marked neurological deficits and surgical excision itself adds no further neurological deficits, surgical removal or biopsy26' of the lesion is indicated. In this report, we describe a case of the successful surgical removal of a cavernous angioma located in the pontomedullary region. This case also revealed a familial occurrence, which is most unusual, with only 17 familial cases.

Received

March

22,

1989;

Accepted

occurrence

April

10, 1990

Report

A 60-year-old male first noticed a speech disturb ance, swallowing disturbance, and coughing around August, 1985. He consulted a physician, who found an abnormal hyperdense area on a CT scan carried out in October, 1985, and he was then advised to consult a neurosurgeon. He did not seek consulta tion and the symptoms gradually worsened. Two weeks prior to admission he developed tingling and a sense of coldness in the right upper extremity and a moderately ataxic gait. Physical examination was negative, showing no cutaneous or retinal vascular malformations. Neu rological examination showed a marked bulbar paresis, characterized by a bilaterally absent gag reflex, and bilateral weakness of the soft palate, more notably on the left, but with the tongue pro truding along the midline. Bilateral abducens and facial nerve pareses, and a linear horizontal and rotatory nystagmus in the direction of lateral gaze were evident, again more notably on the left. A slight hearing deficit, with midline deviation to the right in the Weber's test, was also present. The gait was moderately ataxic, and speech was severely dysar thric. Bilateral, but predominantly right-sided, cerebellar signs were seen. Deep tendon reflexes were bilaterally hypoactive and symmetrical, without

Fig.

1

Preoperative slightly

postcontrast

enhanced

central spot of hypodense zone

Fig. 2

CT

hyperdense calcification. is visible.

scan,

showing

nodule No

with

a a

surrounding

Preoperative (A) and postoperative (B) sagittal MR images of high definition of T,-weighted sequence obtained for the purpose of confirm ing total excision of the cavernous angioma. Preoperative MR image shows the anatomical relationship of the lesion more clearly and a part of the lesion composed of low and high signals protruding into the fourth ventricular floor. The high signal in the center of the postoperative cavity is considered an effect of the Oxycel® and Avitene®.

pathological reflexes. No abnormal findings were con firmed on sensory examination. Electroencephalo graphy did not show any epileptic patterns, focal signs or asymmetry. Plain skull x-rays failed to reveal any calcified regions, however, a CT scan showed a hyperdense lesion in the fourth ventricular floor, which was slightly enhanced with contrast medium. The center of the lesion contained a small calcifica tion (Fig. 1). A CT scan carried out in February, 1988, suggested growth of the lesion compared with

the CT scan obtained in October, 1985. MR im aging performed in March, 1988, showed lesions consistent with those on the CT scan; the lesion re vealed a multilobular, ring-like high intensity signal on both T, and T2-weighted sequences. Sagittal MR image showed multilobular ring-like high signals with a surrounding high signal, demarcated by a thin low-signal zone, on the T,-weighted sequence. This image also showed part of the lesion to be composed of iso and high intensity signals protruding into the fourth ventricular floor (Fig. 2A). Angiograms were normal, except for marked sclerotic changes of the arteries. On March 9, 1988, with the patient in the left lateral position, a bilateral suboccipital craniectomy was performed with the removal of the arch of atlas and opening of the foramen magnum. The cere bellar tonsils were retracted laterally, and superiorly, to expose the obex and the foramen of Magendie. The vermis was not incised for greater exposure of the fourth ventricle because the left vermian branch of the posterior inferior cerebellar artery ran on the right side of the midline. The vermis was also retracted superiorly, and a raspberry-like mass, ap proximately 1.5 cm in diameter, was seen protruding from the fourth ventricular floor. The surrounding area was brownish, suggesting previous bleeding within the left floor, adjacent to the median sulcus. The left floor of the fourth ventricle, beneath the obex, was bulging moderately, which suggested an intraparenchymal mass. Firstly, this protruding mass was removed, including the intraparenchymal parts of the lesion, and then the old hematoma was found. When the intraparenchymal lesion was ap proached, the old hematoma located around the mass continued to drain out, and two thrombosed aneurysm-like sacs, approximately 1.0-1.2 cm in diameter with a small bleb extending rostrocaudally, were encountered. They were connected to each other and surrounded by the old hematoma and granulation and scar tissue. One sac was found to extend to the right of the fourth ventricular floor, more deeply and caudally than the other, which was located in the left fourth ventricular floor beneath the obex. The borders of the lesion were distinct. Marked bradycardia (40/min), and hypotension (40 mmHg, systolic pressure), occurred intermittently during operative manipulation and removal of the two sacs. The postoperative cavity, after removal of the lesion, measured approximately 2.0 cm in dia meter and 2.0 cm or more in depth. Minor bleeding from the postoperative cavity wall was controlled with Oxycel® (oxy-cellulose) and Avitene® (micro fibrillar collagen hemostat).

He regained consciousness soon after surgery. Chest x-rays taken immediately after operation revealed cloudiness in the left lower lobe of the lung, which disappeared on the fourth postoperative day . His vital signs were stable and normal. Neurological examination on May 10, 1988, showed the disap pearance of the bilateral facial paresis, and improve ment in the cerebellar signs (finger to nose and rapid alternating movements), especially on the right side. Only mild left-sided dysmetria was noted. His gait became smooth. The bulbar paresis , rotatory nystagmus in the direction of lateral gaze and slight abduction paresis of the left eye remained, although slightly improved. The dysarthria was also moderately relieved. Postoperative MR imaging confirmed the total disappearance of the cavernous angioma (Fig. 2B). He was able to return to work in June, 1988, although tube feeding through a small diameter (8 Fr) transnasal tube (Japan Medical Sup

Fig.

3

Photomicrograph

showing

cular spaces varying separated by connective neural

tissue

multilobular

vas

markedly in size and tissue. No intervening

is present.

HE stain,

x 33.

ply Co., Hiroshima) was continued. Histologically, the lesion contained numerous endothelial vascular spaces of various size, with little intervening neural tissue. The vascular spaces consisted primarily of a single layer of endothelial cells. However, the walls of the spaces showed con siderable variation in thickness due to organized thrombi and hyalinization. No internal elastic lami na or smooth muscle was present in the thickened walls of the vascular spaces. The histological diag nosis was cavernous angioma (Fig. 3). Family history The patient's mother also had shown a swallowing disturbance and dysarthria, as seen in this case, and finally died of a cerebral hemorrhage, which may have been due to cavernous angioma. The patient has three sons and two sisters. All were examined by CT scanning. His third son had three vascular lesions in the pons, although none were histologically confirmed. MR imaging allowed accurate diagnosis of cavernous angioma (Fig. 4A). He recently experienced an episode of sudden neurological deficits, which suggested a hemorrhage. These symptoms disappeared within 2 months. He showed no neurological deficit later on our examina tion and he is now being followed with CT scanning and MR imaging. The second sister also had a cavernous angioma in the left posterior parietal region (Fig. 4B). Massive hemorrhage occurred in and around this lesion twice within 1 week. She was admitted to our hos pital on November 5, 1988. The hematoma and cavernous angioma were removed through a crani otomy on November 11. Histological examination

Fig.

4

A: Sagittal revealing image

of the

lesion

with

posterior

confirmed

MR image multiple

of the patient's

lesions

patient's a massive

parietal

the diagnosis

in the pons.

second

sister

hematoma

third

son

B: MR showing in the

a left

region.

of cavernous

angioma.

Discussion Next to varix,22) cavernous angioma is the second least common among the four groups of vascular malformations classified as vascular hamartomas by Russell and Rubinstein21): capillary telangiectases, cavernous angiomas, and venous and arteriovenous malformations. Reports concerning cavernous angi oma appear to have been increasing since CT scan ning was introduced and microsurgical techniques were established. In general, the postoperative prog nosis in cases of cavernous angiomas in the cerebral hemisphere is favorable, whereas deep-seated caver

nous angiomas have a doubtful postoperative prog nosis. Cavernous angiomas in the brainstem are par ticularly inaccessible, and surgery may be justified only for the evacuation of a hematoma. Surgery for intraparenchymal cavernous angiomas of the brain stem might cause fatal neurological deficits, and, usually, they can only be treated by conserva tive therapy, or radiation therapy,25 which has been shown to decrease the angioma size. However, cases of successful radical surgery on the cavernous angi oma of the rostral brainstem have been reported recently,",") and the results have suggested the possi bility of removal of the entire lesion with minimal damage to normal surrounding tissue. But a direct surgical approach to cavernous angioma of the brainstem is still controversial. Our patient developed signs of bulbar paresis, which gradually worsened over 2 years. There was suspicion of growth identified by CT, which may be explained by repeated hemorrhage and worsening infarction due to thrombosis, as speculated by Diamond et al.') He failed to respond to intravenous corticosteroid therapy (dexamethasone 16 mg/day), and finally became unable to swallow anything without vomiting and severe coughing. This clinical course aroused suspicion of a coexisting, glial origin tumor such as an oligodendroglioma, but we could not initially propose surgery as the best course of action. Finally, we decided on an operative ap proach for this lesion because it was confirmed by MR imaging to be protruding from the fourth ven tricular floor, and thus biopsy of the lesion itself might not cause any further neurological deficit. He did improve neurologically following total removal of the lesion. This should be considered a fortuitous result, and we do not always recommend surgical intervention for cavernous angiomas in this region. The fact that it was relatively easy to excise the lesion in this case and the poor general prognosis of sur gical removal of brainstem lesions must be kept in mind. For diagnosis of cavernous angiomas, CT scans and MR images are useful. 11,21) MR imaging has been shown to be superior to CT scanning in terms of visualizing posterior fossa lesions, especially lower brainstem lesions, detecting previous hemor rhage, diagnosing vascular lesions, and confirming more accurate anatomical relationships. Mason et al."' reported cavernous angiomas detected by MR images but not by CT scans. The most common and characteristic findings on CT scans are reported to be slightly hyperdense areas, with or without en hancement, 1,2) although the CT findings of a caver nous angioma are currently thought not to be

specific .21,21) Hyperdense lesions sometimes have lobular components, with or without calcification, and show little mass effect. The MR findings have not been reported in detail. Some authors reported an increased signal on the T2-weighted sequence with a minimal surrounding low signal,', 11,211and a multisignal, septate lesion on both T1 and T2 sequences of MR imaging was suggested by Ondra et al.") The clinical manifestation of cavernous angiomas is classified into three categories: seizure, hemor rhage, and mass effect.26) These vascular malforma tions, however, sometimes do not give rise to any symptoms and are incidentally detected by CT. They can be multiple and cause multifocal neuro logical signs. Vascular malformations of the brain stem are reported to be remarkably variable in their clinical presentation, 5,20,27)and it may therefore be difficult to distinguish them from gliomas, encepha litis, or multiple sclerosis affecting the brainstem26,27) merely on the basis of the clinical picture. Stahl et al.27) reported 3 cases and reviewed 18 cases of brain stem vascular malformation. Of 21 patients, 6 had hemangiomas in the brainstem. They suggested that the triad of cranial nerve, cerebellar, and pyrami dal signs, which fluctuate or are insidiously pro gressive over time, can mimic a brainstem vascular malformation as well as multiple sclerosis. Familial occurrence of cavernous angioma is unusual '26) and to date only 17 families have been reported, as shown in Table 1.2-4,6-9,11-13,15,16,18,28,291 Hayman et al.') reported that familial cavernous angiomas were found in 15 of 43 members of a large family in 1982, and Mason et al. 13) also reported

Table

I

Familial

occurrence

of cavernous

angiomas

cavernous angiomas in 10 out of 22 members of a large Hispanic family in 1988. These two families are the largest among the 17 families, and these authors concluded that the inheritance pattern was autosomal dominant, as in the case of arteriovenous malformation, as shown by Pasyk et al.19) A positive family history may help to establish early diagnosis and treatment of cavernous angioma as in the case of our patient's second sister.

14)

15)

16)

References 1) 2)

3)

4)

5)

6)

7)

8)

9)

10)

11) 12)

13)

Bartlett JE, Kishore PS: Intracranial cavernous angioma. AJR 128: 653-656, 1977 Bicknell JM, Carlow TJ, Kornfeld M, Stovring J, Turner P: Familial cavernous angiomas. Arch Neurol 35: 746-749, 1978 Clark JV: Familial occurrence of cavernous angiomata of the brain. J Neurol Neurosurg Psychiatry 33: 871-876, 1970 Combelles G, Blond S, Biondi A, Combelles-Pruvot M, Szikla G, Christiaens JL: Formes familiales des hémangiomes caverneux intracraniens. A propos de 5 cas dans deux families. Neurochirurgie 29: 263-269, 1983 Diamond C, Torvik A, Amundsen P: Angiographic diagnosis of telangiectases with cavernous angioma of the posterior fossa. Acta Radiol [Diagn] (Stockh) 17: 281-288, 1976 Dobyns WB, Michels VV, Groover RV, Mokri B, Trautmann JC, Forbes GS, Laws ER Jr: Familial cavernous malformations of the central nervous system and retina. Ann Neurol 21: 578-583, 1987 Fukushima T, Ohkawa M, Tomonaga M: Multiple and familial cavernous angiomas in the brain and ex tremities. Case report. No Shinkei Geka 14: 423-428, 1986 (in Japanese) Giombini S, Morello G: Cavernous angiomas of the brain: Account of fourteen personal cases and review of the literature. Acta Neurochir (Wien) 40: 64-82, 1978 Hayman LA, Evans RA, Ferrel RE, Fahr LM, Ostrow P, Riccardi VM: Familial cavernous angi omas: Natural history and genetic study over a 5-year period. Am J Med Genet 11: 147-169, 1982 Jellinger K: The morphology of centrally-situated angiomas, in Pia HW, Gleave JRW, Grote E, Zierski J (eds): Cerebral Angiomas: Advances in Diagnosis and Therapy. New York, Springer, 1975, pp 9-20 Kidd HA, Cumings JN: Cerebral angiomata in an Icelandic family. Lancet 1: 747-748, 1947 Kufs H: Über Heredofamiliare Angiomatose des Gehirns and der Retina, ihre Beziehungen zueinander und zur Angiomatose der Haut. Zeitschrift fur die Gesamte Neurologie and Psychiatrie 113: 651-686, 1928 Mason I, Aase JM, Orrison WW, Wicks JD, Seigel

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reprint

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to: H. Nakagawa,

Neurosurgery, Osaka,

537,

Japan.

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The Nakamichi,

M.D.,

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for

Depart Adult

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