Nicola Acciarri

Growth, Subsequent Bleeding, and De Novo Appearance of Cerebral Cavernous Angiomas

In a series of 145 patients with brain cavernous angiomas treated at our hospital in the last 16 years, the angiomas of 18 patients exhibited aggressive biological behavior characterized by recurrent overt bleeding, growth, or de novo appearance. The cavernomas were in the cerebellum in three patients, in the brain stem in one, in the thalamus in four, in the caudate nucleus in two, in the diencephalon in one, and in the white matter of the cerebral hemispheres in seven. Three of these patients suffered from the familial or multiple form of the disease, two were pregnant, three had previously been irradiated for other tumors, and one had been treated by radiosurgery in the past. Overall, new cavernous malformations not previously shown were discovered in six patients. In 10 patients (3 male and 7 female) presenting with recurrent hemorrhages, the mean period of time between bleedings was 11 months (range, 1 wk-3 yr). Eleven patients were treated by definitive surgery, and seven were conservatively treated. One patient with a diencephalic cavernoma died from progressive hypothalamic dysfunction; three patients in the nonsurgical group had repeated symptoms and were left with additional neurological deficits. The outcome of the surgical group was the same (seven patients) or improved (four patients). Risk factors favoring an aggressive behavior included pregnancy, familial or multiple form of the disease, previous whole brain or stereotactic radiotherapy, incomplete removal, brain location, and associated venous malformation. The female preponderance (female to male ratio, 13:5) may also suggest some role of hormonal factors in influencing the biological behavior of cavernous malformations.

Long-term follow-up of occlusive cervical carotid dissection.

We retrospectively studied 19 cases of occlusive cervical carotid dissection encountered at our hospital between 1974 and 1984 and followed for 5-13 (mean 8.2) years to assess the long-term prognosis of the disease. Five patients had transient ischemic attacks, seven had minor stroke, six had major stroke, and one had epileptic seizures. Angiography demonstrated the typical string sign in 17 cases, a double lumen with occlusion in one, and multiple scalloped narrowings with distal occlusion in the other. Three patients died within 1 month and three remain severely disabled (overall mortality and major morbidity 32%), five have permanent deficits, and seven are neurologically intact; the remaining patient was lost to follow-up. Five patients were treated surgically (two had extracranial-intracranial bypass and three had cervical carotid exploration), and the other 14 were treated medically. The overall rate of reopening was 47% with eight of 10 patients demonstrating recanalization on control angiography and another patient demonstrating recanalization at surgery. These nine patients remain clinically stable on follow-up evaluations. However, vascular abnormalities in the healed arteries were notable and include kinking, fibromuscular dysplasia, dissecting aneurysms, intracranial occlusion, and a residual mural defect.

Cavernous Malformations of the Central Nervous System in the Pediatric Age Group

Objective: The main clinico-diagnostic features, risk factors and associated diseases of cavernous malformations (CMs), also called cavernous angiomas or cavernomas, of the central nervous system (CNS) in children are described, and the most relevant differences compared to the affected adult population are pointed out, focusing on the management of pediatric patients harboring cranial and spinal CMs. Materials: This was a retrospective study of a series of 42 children symptomatic for CMs of the cranial and spinal compartments (35 supratentorial brain lesions, 5 infratentorial and 2 in the spinal region) operated on between 1975 and 2005, with a clinical follow-up ranging from 12 to 192 months. The results were compared with those found in the most recent literature dealing with pediatric CMs of the CNS. Results: Surgical treatment produced excellent or good results in 69% of our 42 children. Unchanged neurological deficits were observed in 23.8% of cases, while morbidity from surgical procedures was 7.14%. Mortality was absent in this series. These surgical results are comparable with those found in the literature, where morbidity and mortality rates from surgery are 8.8 and 1.13%, respectively, and are mostly associated with procedures for the excision of deep, critically located cavernomas. Conclusion: CMs represent the most common CNS vascular lesion in children, although their incidence is 4 times lower than that of the adult population. The natural history of pediatric CMs throughout the neuraxis seems to be more aggressive than in adult patients; these lesions have higher rates of growth and hemorrhage, larger dimensions and often atypical radiological pictures at diagnosis. Beside the familial form of the disease, which is more often associated with multiple lesions and an earlier age of clinical presentation, the major risk factor for CMs in children seems to be radiotherapy for CNS tumors. Furthermore, a greater number of CMs coexistent with mixed angiomatous lesions have been reported in children than in adults. Surgical results are related to the preoperative neurological status of the children; symptomatic patients who are operated on early, before they develop severe neurological deficits or long-standing seizures, may achieve the best clinical outcome. Radiosurgery does not seem to be advisable in children as an alternative treatment for deep CMs or those causing epilepsy.

CT scan and surgical treatment of traumatic iliacus hematoma with femoral neuropathy: case report.

Iliacus hematoma should be suspected as the cause of femoral neuropathy in cases of minor trauma in teenagers, especially when the onset of paralysis is delayed and therefore an acute traction injury of the nerve is unlikely. In our 14-year-old female patient the condition followed minor trauma. In such cases CT scan should be performed. When this reveals a hematoma and the nerve lesion is rapidly progressing or complete, the hematoma should be promptly evacuated.

Intracranial and orbital cavernous angiomas: a review of 74 surgical cases.

We present a surgical series of 74 patients (30 males and 44 females) with pathologically verified cavernous angiomas of the intracranial and orbital compartments. Patients were admitted between 1975 and 1991; six had a family history of cerebral cavernomas, and two had multiple (two) lesions. The 76 malformations were located as follows: 57 were in the cerebral hemispheres, four in the supratentorial ventricles, one was in the middle cranial fossa, two were in the brain stem, five in the cerebellum and seven in the orbits. Seizures and focal neurological deficits, and decrease of visual acuity with exophthalmus, were the main clinical signs observed in patients with intracranial and orbital cavernomas, respectively. Sixteen patients (21.6%) had a clinically significant haemorrhage attributable to the cavernous angioma. A number of these vascular malformations were misdiagnosed by computed tomography. In the last 10 years magnetic resonance imaging has been the most sensitive method for detecting these lesions. Seventy-four of the 76 diagnosed cavernomas were treated surgically: a complete excision was obtained in 68 patients; in two patients with multiple lesions only those causing symptoms were removed. Surgery for the 10 deep lesions was aided considerably by stereotactic localization. Two patients died in the immediate postoperative course. The overall outcome was good in 66 of the 72 remaining patients, resulting in improved seizure control or lessened neurological deficit.

Spinal cavernous angioma: A rare cause of subarachnoid hemorrhage

A case of cervical intrathecal extramedullary cavernous angioma is presented. The rarity of this lesion in comparison with the more frequent vertebral cavernous angiomas with secondary extension to the epidural space is emphasized. The special features of this case are noted: the acute clinical onset due to recurrent subarachnoid hemorrhages, the visualization only by means of magnetic resonance imaging, and the unusual cervical level. The most debated characteristics of these lesions and the relevant literature are summarized.

Results of surgery in children with cerebral cavernous angiomas causing epilepsy

Epilepsy is the most frequent presenting symptom of cerebral cavernous angiomas or cavernomas, and surgical removal of these vascular malformations is considered the treatment of choice in patients with intractable or long-standing seizures, or in those with poor compliance to medical therapy. In this paper the results of surgical treatment in 11 children with seizures from cerebral cavernomas are reported. Surgery for deep-seated cavernomas was aided in 3 cases by a stereotactic localization technique, that allowed a limited approach with minimal brain damage. Major morbidity and mortality were absent; follow-up ranged between 1 to 16 years. Improved seizure control was seen in all the patients: eight (72%) became seizure-free with the same preoperative therapy; one became seizure-free with a lower drug dosage, and two (18%) were seizure-free without medical therapy. The removal of cavernomas prevented the risk of haemorrhages or further deficits from growth and, above all, avoided spreading and autonomization of the epileptogenic area induced by the cavernoma.

Surgical management of cavernous angiomas in children

Cavernous angiomas are vascular malformations affecting any part of the central nervous system (CNS). The management of asymptomatic cavernous angiomas is still debated due to their poorly understood natural history, although more data are now available regarding results of surgical treatment in symptomatic cases. The authors report their surgical experience with 18 pediatric patients operated on for symptomatic CNS cavernous angiomas. The children ranged in age from 10 months to 17 years, without a relevant sex difference. Cavernous angiomas were intracranial in 17 cases: 15 being in the supratentorial compartments and two in the cerebellum. Clinical manifestations were as follows: seizures in 11 cases, focal neurologic deficits in five, and headache in one. The 18th case was observed in a girl showing paraparesis in the spinal subdural-extramedullary space at T8-T9 level. Excision of four deep cerebral lesions was performed after stereotactic localization through non-eloquent cortex. Pathologic confirmation of cavernous angiomas was obtained in all patients. Mortality from surgical procedures was absent in this series. The follow-up period ranged from 1 to 16 years. All 11 epileptic patients obtained seizure control; improvement or stabilization of neurologic symptoms was observed in the remaining seven patients.

Intraneural ganglion of the peroneal nerve: An unusual presentation: Case report

The authors report an intraneural ganglion of the peroneal nerve at the fibular head, in which the external appearance of the nerve was normal at operation. Only the incision of the epineurium permitted discovery of the lesion.

Langerhans cell histiocytosis of the spine causing cord compression: case report.

A case of Langerhans cell histiocytosis of the spine causing myeloradiculopathy is reported. Almost complete regression of symptoms and signs was observed in the patient after surgical treatment and radiotherapy. The clinical and histological features of the disease are discussed. The literature dealing with the previously reported cases of spinal histiocytosis involving neural structures is reviewed.