D.A. Gulyaev, P.V. Krasnoshlyk, Yu.M. Zabrodskaya, Z.F. Kuzbekova
Polenov Research Neurosurgical Institute, Saint Petersburg
Cavernous angiomas are referred to angiomatous vascular developmental defects. They are clusters of vascular cavities, separated by connective septa, which have no elements of a normal vascular wall and are covered with endothelium. Virchow was the first to describe these malformations in his Die Krankhafte Geschwulste, published in 1863 . Today the rate of this pathology is 0.45-0.9% [2, 4]. Usually it is revealed in adults (75-80% of all cases) [2, 3] and is more typical of young people. Cavernous angiomas can be characterized by sporadic and isolated genic defects. Besides, there can be multiple genic defects with autosomal-dominant hereditary mutations in the 7q11-22 locus [5, 6]. The most frequent intracranial localization is subcortical white matter, pons, cerebellum and capsula externa [2, 4]. Any other localization is rare [3, 4]. Clinical manifestations include seizures, intracerebral hemorrhages, focal neurological symptoms [2, 4].
A 24 years old man was admitted to the Polenov Research Neurosurgical Institute on October 4, 2006. He complained of sporadic vision impairment, photopsy in the form of light glimmer, narrowing of visual field margins on the right and headache. These manifestations of the disease appeared one and a half years ago. The patient did not visit any medical establishment before June 2006. MR-examination was carried out on the basis of a referral from the out-patient department. It revealed a mass within the brain. On admission the patient’s state was compensated from the point of view of vital functions (the score of 80 according to Karnofsky scale). Neurological examination was indicative of horizontal adaptive nystagmus in extreme abduction, increased muscular tonus of a pyramidal-extrapyramidal type and left-side pyramidal insufficiency.
MR-examination of the brain (Fig.1) demonstrated several nodes of the mass, characterized by active accumulation of a contrast substance. They were located in the area of the left parietal and occipital lobes, invaded the bone and spread along the tentorium, petrous pyramid and in the subtentorial direction. The mass dimensions were 11.3´6.4´13.5 cm. It dislocated median structures from left to right by 10 mm, deformed left segments of the pons, ambient cistern, cerebellar hemisphere and dislocated the left cerebellar tonsil into the great foramen by 4.0-5.0 mm.
Carotid angiography (Fig.2) revealed dislocation of vessels of the MCA region in the forward and downward direction, caused by the mass in the left parietal and occipital lobes. The tumor was supplied by hypertrophied branches of the middle meningeal and occipital arteries. A vascular network of the tumor was chaotic and corresponded to two main nodes of the tumor. Blood from anterior and posterior segments of the brain drained into cavernous and petrosal sinuses and deep cerebral veins respectively. Middle and posterior thirds of the ACA were indistinct due to infiltration by tumor tissue.
The patient was operated on October 13, 2006 (Senior Researcher Gulyaev D.A.). Decompressive osteoplastic trephination of the skull and partial removal (80%) of the tumor, involving the left parietal, temporal and occipital lobes, were performed. A bone flap, which contained the tumor, was removed. Histological examination (Prof. Medvedev Yu.A.) revealed giant multi-node intraoesseous cavernous angioma (Fig.3) of the left parietal and occipital bones, accompanied by hyperostosis. It spread intracranially with depression of the left temporal, parietal and occipital lobes, as well as along the tentorium, petrous pyramid and in the subtentorial direction. There were no signs of malignant degeneration. The tumor was characterized by a growth, causing local destruction.
There were no postoperative augmentation of neurological deficit (the score of 80 according to Karnofsky scale) and vision impairment events. The wound healed by first intention; sutures were removed on the 10th day. The patient was discharged on the 26th day in a satisfactory state. Control postoperative CT (Fig.4) demonstrated a state after craniotomy and partial removal of tumor.
Trephination defect was closed with a titanium network a year after operation (Fig.5).
The goal of the present article has been to demonstrate an extremely rare (casuistic) case of giant cavernous angioma with extensive involvement of bones of the fornix and skull base, compression of cerebral hemispheres and block of big venous collectors.