Michael Besser

Determinants of Outcome in Melanoma Patients With Cerebral Metastases

PURPOSEnTo analyze prognostic factors, effects of treatment, and survival for patients with cerebral metastases from melanoma.nnnPATIENTS AND METHODSnAll melanoma patients with cerebral metastases treated at the Sydney Melanoma Unit between 1952 and 2000 were identified. From 1985 to 2000, patients were diagnosed and treated using consistent modern techniques and this cohort was analyzed in detail. Multivariate analysis of prognostic factors for survival was performed.nnnRESULTSnA total of 1137 patients with cerebral metastases were identified; 686 were treated between 1985 and 2000. For these 686 patients, the median time from primary diagnosis to cerebral metastasis was 3.1 years (range, 0 to 41 years). A total of 646 patients (94%) have died as a result of melanoma. The median survival from the time of diagnosis of cerebral metastasis was 4.1 months (range, 0 to 17.2 years). Treatment was as follows: surgery and postoperative radiotherapy, 158 patients; surgery alone, 47 patients; radiotherapy alone, 236 patients; and supportive care alone, 210 patients. Median survival according to treatment received for these four groups was 8.9, 8.7, 3.4, and 2.1 months, respectively; the differences between surgery and nonsurgery groups were statistically significant. On multivariate analysis, significant factors associated with improved survival were surgical treatment (P <.0001), no concurrent extracerebral metastases (P <.0001), younger age (P =.0007), and longer disease-free interval (P =.036). Prognostic factors analysis confirmed the important influence of patient selection on treatment received.nnnCONCLUSIONnThis large series documents the characteristics of patients who developed cerebral metastases from melanoma. Median survival was dependent on treatment, which in turn was dependent on patient selection.

Vein of Galen malformation: diagnosis and management

The vein of Galen malformation is a midline arteriovenous fistula with aneurysmal dilatation of the vein of Galen. The clinical details of diagnosis and treatment in 13 patients with such lesions together with a review of 232 cases collected from the literature are presented in this report. There were 132 males, 77 females, and 36 cases in which the sex was not stated. Eighty patients presented as neonates, 82 were 1 to 12 months old, 39 were 1 to 5 years old, 22 were 6 to 20 years old, and 22 were over the age of 20. The most common presenting symptoms were congestive cardiac failure (110 cases), raised intracranial pressure secondary to hydrocephalus (94 cases), cranial bruit (57 cases), focal neurological deficit (37), seizures (26 cases), and hemorrhage (25 cases). The most characteristic vascular supply to the midline fistula involved multiple bilateral vessels, although bilateral posterior cerebral and unilateral posterior cerebral supply was relatively common. The overall figures for treatment and outcome showed that 91 patients (37.1%) were treated by direct operation and 29 patients (11.3%) were treated by other forms of operation, predominantly shunting or remote vessel ligation. Forty-six patients (18.8%) were treated by medical means (digoxin, diuretics, and ventilatory support). In 79 patients (22.2%), there was no treatment or no details of treatment were available. There was an overall series mortality of 55.6% (no details were available in 33 cases) and a 37.4% mortality for surgically treated cases. After operation, there was a 46.3% incidence of significant morbidity in surviving patients. Neonatal patients fared worst, with an overall mortality of 64 of 70 cases (91.4%) where details were available. The outcome was equally bad for surgically and conservatively treated cases. Operation in the 1- to 12-month age group was more successful, but still carried a mortality of 31.7%, with a significant morbidity in approximately half of the surviving patients. Over the age of 1 year, the surgically treated patients had a 25.6% mortality and a 42.3% major morbidity in survivors. Consideration is given to some of the ways in which these figures may be improved, in particular a staged approach during the neonatal period, with the use of selective embolization or occlusion of vessels to reduce the volume of the arteriovenous shunt until the patient is older and better able to tolerate major operation.

The treatment of benign intracranial hypertension: A review of 134 cases

Abstract The treatment of benign intracranial hypertension in 134 patients is reviewed. No treatment was required in 16 patients, although the diagnosis may have been doubtful in a number of these. Serial lumbar punctures had only a 38.8% success rate when used alone. Steroids were effective as primary treatment in 81.1% of the patients, although recurrence (13.5%) and complications of therapy were significant in this group. Subtemporal decompression was relatively ineffectual, particularly on the measured cerebrospinal fluid (CSF) pressure. CSF shunting, especially lumboperitoneal, was very effective, although complications were important. The basis of treatment in benign intracranial hypertension is reviewed in light of the current theories of its pathogenesis.

Intracranial pressure changes in craniostenosis

Intracranial pressure changes occurring in patients with craniostenosis were studied by continuous monitoring of intracranial pressure in 20 children. Although clinical or radiologic evidence of intracranial hypertension was present in only one patient, 13 (65%) had persistent or episodic raised intracranial pressure. Intracranial hypertension occurred with involvement of both single and multiple sutures and in four of six patients with hydrocephalus and craniostenosis. Recordings after surgical treatment in nine patients revealed that several months elapsed before intracranial pressure improved significantly or returned to normal, despite apparently adequate bony decompression. This study confirms that continuous monitoring of intracranial pressure adds significantly to clinical assessment in determining the need for, and the efficacy of, surgical treatment in craniostenosis.

Meningoceles and the tethered cord syndrome

This study shows that there is a relationship between simple meningocele and the tethered cord syndrome. Two groups of patients were examined: the first comprised patients presenting with neurological abnormality at some period after initial closure of a simple meningocele, and the second group (18 patients) had elective myelography after closure of a simple meningocele in the neonatal period (in 17 of the 18 cases). Of these 18 cases, 10 showed an intradural abnormality, and all underwent surgery confirming their myelographic diagnosis. The conclusion is drawn that after closure of a simple meningocele in the neonatal period, patients should have elective myelography in the first 12 months of life to determine whether or not the tethered cord syndrome is likely to develop during the period of growth.

Pseudotumor syndrome in treated arachnoid cysts

We report three patients with arachnoid cysts treated by cyst-peritoneal shunting in whom intracranial hypertension occurred during episodes of shunt malfunction. In one case this was associated with re-expansion of the arachnoid cyst, whilst in the other two cases this did not occur. The similarities between these two cases and patients with pseudotumor cerebri suggest a common pathogenic mechanism — specifically, a disturbance of the cerebrospinal fluid circulation.

Sinus pericranii: a clinical and radiological review of an unusual condition

Sinus pericranii is an unusual and rare condition characterized by an abnormal communication between an intracranial venous sinus and the extracranial venous circulation. Patients are usually children and often asymptomatic, presenting only when cosmetic concerns are raised by the parents or the presence of a scalp lump is noted. The diagnosis is made on clinical grounds with supportive evidence from radiological investigations such as plain skull X-rays, CT scans, MR scans and cerebral angiography. The treatment may be conservative but surgical excision is usually required, whether for cosmetic integrity or for fear of direct trauma with life threatening haemorrhage. The aetiology and pathophysiology of this condition remains poorly understood. We present a case of a 6-year-old boy with sinus pericranii that was treated with surgical excision and repair. The radiological investigations employed to confirm the diagnosis are presented, including a Doppler ultrasound of the lesion. We also present a review of the literature on this condition.

Short latency somatosensory-evoked potentials in children —part 4. Findings with cerebral lesions

Cortical somatosensory-evoked potential studies were performed on seven children with unilateral intracerebral lesions. Three patients had neoplasms, and four had hematomas of various etiology. All the lesions involved either directly or indirectly with the thalamocapsular or perirolandic regions of the brain. Patterns of somatosensory-evoked potential asymmetry included abnormality of the primary cortical complex (N1-P1), loss of intermediate and long latency waveform components, and attenuation of the response on the side of the lesion. The most abnormal somatosensory-evoked potentials were seen in patients with major clinical deficits, whereas patients with minimal neurologic signs had minor somatosensory-evoked potential changes. The location, etiology, and duration of the lesions all appeared to influence the somatosensory-evoked potential waveform. The value of somatosensory-evoked potential studies in the diagnosis and localization of cerebral lesions would seem to be limited because of pathophysiologic phenomena related to the primary cerebral lesion.