Giuseppe Maggi

Endoscopic third ventriculostomy in the treatment of hydrocephalus in posterior fossa tumors in children

ObjectThe purpose of the present study is to assess the effectiveness of endoscopic third ventriculostomy (ETV) in children with hydrocephalus related to posterior fossa tumors.MethodsBetween September 1999 and December 2002, 63 children with posterior fossa tumors were treated at Santobono Hospital in Naples, Italy. Twenty-six patients had severe hydrocephalus. In order to relieve intracranial hypertension before tumor removal, 20 were treated with ETV, and 6 with ventriculo-peritoneal (VP) shunts. Twenty patients with mild hydrocephalus were treated with diuretics, corticosteroid agents, and early posterior fossa surgery, and 17 patients who did not have hydrocephalus were treated by elective posterior fossa surgery. Another 4 ETV were performed in the management of postoperative hydrocephalus.ResultsPreoperative ETV procedures were technically successful. One was complicated by intraventricular bleeding. The successful 19 preoperative ETV resolved intracranial hypertension before posterior fossa surgery in all cases. Three of these 19 patients developed postoperative hydrocephalus and were treated by VP shunt insertion after posterior fossa surgery. Out of the 4 ETV performed after posterior fossa surgery, only 2 were successful, both when the shunt malfunctioned.ConclusionsEndoscopic third ventriculostomy should be considered as an alternative procedure to ventriculo-peritoneal shunting and external ventricular draining for the emergency control of severe hydrocephalus caused by posterior fossa tumors, since it can quickly eliminate symptoms, and hence, can delay surgery scheduling if required. Even though ETV does not prevent postoperative hydrocephalus in all cases, it does protect against acute postoperative hydrocephalus due to cerebellar swelling. In addition, it eliminates the risks of cerebrospinal fluid (CSF) infection related to external drainage and minimizes the risk of overdrainage because it provides more physiological CSF drainage than the other procedures. Since postoperative hydrocephalus is very often physically obstructive, ETV should always be considered a possible treatment procedure.

Current state and future development of intracranial neuroendoscopic surgery

Since the introduction of the modern, smaller endoscopes in the 1960s, neuroendoscopy has become an expanding field of neurosurgery. Neuroendoscopy reflects the tendency of modern neurosurgery to aim towards minimalism; that is, access and visualization through the narrowest practical corridor and maximum effective action at the target point with minimal disruption of normal tissue. Transventricular neuroendoscopy allows the treatment of several pathologies inside the ventricular system, such as obstructive hydrocephalus and intra-/paraventricular tumors or cysts, often avoiding the implantation of extracranial shunts or more invasive craniotomic approaches. Endoscopic endonasal transphenoidal surgery allows the treatment of pathologies of the sellar and parasellar region, with the advantage of a wider vision of the surgical field, less traumatism of the nasal structures, greater facility in the treatment of possible recurrences and reduced complications. However, an endoscope may be used to assist microsurgery in virtually any kind of neurosurgical procedures (endoscope-assisted microsurgery), particularly in aneurysm and tumor surgery. Basic principles of optical imaging and the physics of optic fibers are discussed, focusing on the neuroendoscope. The three main chapters of neuroendoscopy (transventricular, endonasal transphenoidal and endoscope-assisted microsurgery) are reviewed, concerning operative instruments, surgical procedures, main indications and results.

Intracranial pressure monitoring and lumbar puncture after endoscopic third ventriculostomy in children.

OBJECTIVE:The aim of this study is to analyze changes in intracranial pressure (ICP) after endoscopic third ventriculostomy (ETV) performed in children affected by noncommunicating hydrocephalus. METHODS:ICP was continuously recorded for an average of 7 days in 64 children who underwent 68 ETVs for obstructive triventricular hydrocephalus of various etiology. In the first group (44 children), ETV was performed as the primary treatment; in the second group (20 children), the patients presented with shunt malfunction and underwent ETV and shunt removal. Three of the patients in the second group were reoperated for obstruction of the stoma: two were reoperated once and one was reoperated twice. RESULTS:ICP changes after ETV were not homogeneous and varied according to etiology: the highest values were observed in patients affected by posterior fossa tumors and the lowest values were seen in patients operated on during shunt malfunction and who had their shunt removed. After 31 procedures (45.6%), ICP remained normal (<20 mmHg) for the entire duration of the monitoring. After 37 procedures (54.5%), ICP was persistently high on Day 1 (mean, 29.7) and decreased very slowly in the subsequent days, remaining high for 2–9 days (mean, 4.5). After 20 of the 37 procedures with high postoperative ICP, patients presented symptoms of intracranial hypertension that resolved, in most of the cases, with one or two lumbar punctures. Lumbar puncture was noted to be effective in bringing about fast normalization of the ICP and resolution of the symptoms. In 13 patients (19.1%), ETV failed and a ventriculoperitoneal shunt was implanted. After four procedures, the stoma obstructed and the patients were treated, reopening the stoma. Postoperative ICP was not statistically significant higher in the patients in whom ETV failed. CONCLUSION:The high ICP observed in a group of patients in the early postoperative days is probably related to the slow permeation of the subarachnoid spaces by the cerebrospinal fluid flowing out of the third ventriculostomy. Management of intracranial hypertension after ETV remains a matter of controversy. The role of the lumbar puncture in the faster normalization of the ICP is examined in this article. By increasing the compliance and the buffering capacities of the spinal subarachnoid spaces, it probably decreases the cerebrospinal fluid outflow resistance from the ventricular system, facilitating the decrease of the ventricular volume and allowing faster permeation of the intracranial subarachnoid spaces. High postoperative ICP can account for persistent symptoms of intracranial hypertension and ventricular dilatation on computed tomographic scans after third ventriculostomy. A cycle of one to three lumbar punctures should always be performed in patients who remain symptomatic and who show increasing ventricular dilatation after ETV, before ETV is assumed to have failed and an extracranial cerebrospinal fluid shunt is implanted.

Second-line chemotherapy with the association of liposomal daunorubicin, carboplatin and etoposide in children with recurrent malignant brain tumors.

Progressive or recurrent high-grade gliomas are characterized by a very poor prognosis, and the relevance of second-line chemotherapy is still unassessed.Although it has been reported that liposomal anthracyclines and carboplatin show some activity in these patients, their association has never been investigated.We have treated six children with recurrent high-grade glioma after surgery, radiotherapy and chemotherapy, and one child with progressive teratoid/rhabdoid tumor with the combination of liposomal daunorubicin and carboplatin plus etoposide. Five out of seven children showed a major response and the 29 month progression-free survival was 38%. The above regimen was feasible and children showed only little and transient hematological toxicity.In our opinion, these results justify further investigation of the above combination chemotherapy for recurrent or progressive malignant brain tumors in children.

Cytogenetic evaluation of isochromosome 17q in posterior fossa tumors of children and correlation with clinical outcome in medulloblastoma

AbstractBackground.A number of chromosomal abnormalities have been described in the presence of central nervous system tumors; isochromosome 17q, representing a loss of heterozygosity for the short arm of the chromosome 17, is the one most frequently reported in association with medulloblastoma. The purpose of this study was to evaluate the prognostic correlation of this variable, compared with other variables (surgery extent and radiotherapy), with survival. Methods and results.We looked for the presence of i(17q) in 32 children affected by posterior fossa tumors, including 16 medulloblastomas and 2 teratoid/rhabdoid tumors. For our study we used both karyotypic analysis and the fluorescence in situ hybridization (FISH) procedure, both on fresh and on paraffin-embedded tissues. Cytogenetic analysis allowed us to detect a hitherto unreported abnormality in medulloblastoma: ins(1;10)(q31;q23q26). Moreover, 16 of the 32 patients analyzed by FISH were found to be positive for the presence of i(17q): the 2 with teratoid/rhabdoid tumors, 11 of 16 with medulloblastomas, plus 1 with ependymoblastoma and 2 with anaplastic astrocytomas. As far as the outcome of medulloblastoma patients is concerned, we found that 8 out of the 10 children whose tumor had been totally removed had a favorable outcome regardless of the presence of i(17q): 4 were i(17q) positive and 4 i(17q) negative. Conclusions.Although it was impossible to draw any definitive conclusion about detection of i(17q) in central nervous system tumors in infancy, particularly in the case of medulloblastoma, we suggest that this chromosomal abnormality is not an independent prognostic factor, but may be a marker for uncontrolled cell proliferation.

Eosinophilic granuloma of C4 causing spinal cord compression.

A rare case of vertebral eosinophilic granuloma (C4) causing spinal cord compression is reported. The clinical, histological and radiological features of this pathological entity are discussed. After surgery a complete neurological recovery was observed. The value, in selected cases, of surgical treatment with total removal of the tumour and reconstruction of the spine to ensure spinal stability and to prevent irreversible neurological deficit is emphasized.

Efficacy of Sequential Chemotherapy Including Methotrexate and Doxorubicin in an Infant with Partially Resected Choroid Plexus Carcinoma

This report refers to a 3-month-old male, with a residual choroid plexus carcinoma following partial resection, who was successfully treated with sequential chemotherapy without any postoperative radiation therapy. Along with carboplatin, we also used doxorubicin and methotrexate, hypothesizing that, given the patient’s age, the blood-brain barrier should not hamper drug delivery to the tumor. According to this hypothesis, the treatment achieved complete remission of the disease, which lasts 27 months after the diagnosis. This result deserves further studies to assess the possible curative role of chemotherapy in very young patients suffering from choroid plexus carcinoma.

Acute cerebellar ataxia in children.

Abstract Acute cerebellar ataxia is a benign syndrome usually occurring after an acute febrile disease. In a few cases neuroradiological investigations reveal cerebellar alterations. Clinical and neuroradiological involvement of the brain stem has rarely been reported in the literature. We present five cases of acute cerebellar ataxia. In two cases the cerebellar symptomatology was associated with neurological signs of brain stem involvement. CT scans did not show any pathologic findings in three patients. MRI disclosed cerebellar or brain stem alterations in all the patients. Clinical and neuroradiological findings allow differentiation of this pathologic entity from other demyelinating or dysmyelinating diseases. The value of MRI in detection and localization of the lesions and in following their evolution is emphasized.