Gianpiero Tamburrini

Sylvian fissure arachnoid cysts: a survey on their diagnostic workout and practical management

BackgroundThe management of Sylvian arachnoid cysts in children is still a matter of debate. Diagnosis is often incidental, and symptoms are frequently aspecific in symptomatic cases. Suggested diagnostic investigation results have been often unclear. Surgical treatment is also controversial, pure, and assisted endoscopic cyst marsupialization having entered in the traditional debate between craniotomic and shunting approachPurposeThe objective of the present study was to survey if (and, eventually, which) agreement points do actually exist between internationally recognized pediatric neurosurgery centers in the management of children with a controversial type of Sylvian arachnoid cyst (Type II cyst).MethodsContributors were asked to answer to a six-separate-part multiple choice questionnaire related to the case of a 2.7-year-old boy with a Type II left Sylvian arachnoid cyst presented in different clinical situations. If surgery was indicated, it was asked which surgical procedure the authors would have suggested as first option.Results and conclusionsThe option of the mere clinical observation was chosen by the majority of surgeons in case of asymptomatic clinical discovery. On the other hand, a constantly high percentage of participants suggested direct surgical treatment based on clinical manifestations or as a preventive measure justified by the risk of spontaneous or traumatic intracranial bleeding. The only diagnostic investigation result which significantly influenced the surgical indication was a localizing electroencephalography, if the child presented with seizures. The result is that in most cases the surgical indication was based on aspecific clinical manifestations and laboratory data. Craniotomy and arachnoid cyst marsupialization represented the preferred surgical option (66.6%), 28.8% of the participants suggesting pure or assisted endoscopic cyst marsupialization as primary surgical procedure. Cyst shunting was suggested by only three centers.

Antibiotic impregnated external ventricular drainage and third ventriculostomy in the management of hydrocephalus associated with posterior cranial fossa tumours

BackgroundThe effectiveness of antibiotic pre-treated ventricular catheters in reducing the risk of CSF infections (determined on CSF cultures) resulting from the use of per-operative external ventricular drainages (EVD) and the success rate of post-operative endoscopic third ventriculostomy (ETV) in the management of persistent hydrocephalus after posterior cranial fossa tumour removal are assessed.MethodForty-seven children (group I) were prospectively managed by means of per-operative antibiotic impregnated EVD, post-operative ICP monitoring, and ETV. The results of this group were compared with those of a control group composed by 44 children treated with the same protocol as above except for the use of not-impregnated catheters (group II).FindingsThe rate of positive CSF cultures due to EVD resulted significantly lower in group I (2.1% vs 31.8%); there was no clinical evidence of CSF infections. The success rate of ETV was the same in both groups (75%). Failures of ETV occurred in the patients with subarachnoid tumour seeding and/or tumour extension to the basal cisterns. All the children of group II with failed ETV also showed a bacterial growth in the CSF.ConclusionsAntibiotic pre-treated catheters in our experience considerably limited EVD-related bacterial growth in the CSF. Preoperative hydrocephalus resolved in 60% of the cases after tumour removal, thus confirming recent data from the literature against the routine use of preoperative ETV. In our experience postoperative ETV had a high success rate; poor results were obtained in children with tumour seeding and/or the evidence of positive CSF cultures.

Long-term neuropsychological development in single-suture craniosynostosis treated early

OBJECT The authors evaluated the results of complete cognitive function examinations in a series of 65 adolescents who had undergone surgery for sagittal or unicoronal craniosynostosis when they were younger than 1 year old. METHODS Each of the 65 study participants was evaluated individually. The neuropsychological assessment, consisting of a battery of tests tailored to the patients age, cognitive level, and level of cooperation, was conducted in 2 separate sessions on the same day. The main outcome measures included fine motor skills, language, visual motor spatial and visual perceptual skills, working and visual memory, attention, executive function, and verbal fluency. RESULTS Thirty-five children (mean age 13.4 years) were affected by sagittal synostosis and 30 (mean age 14.9 years) by unicoronal synostosis (16 right-sided, 14 left-sided). The mean age at surgery was 7.2 months. All of the children had begun school at a normal age and attended regular classes. Seven percent of those with sagittal craniosynostosis demonstrated visuospatial and constructional ability defects with associated visual memory recall deficits; 17% also exhibited selective and sustained attention deficits. Approximately one-third (30%) of the children with anterior plagiocephaly had processing and planning speech deficits. CONCLUSIONS Data in this study support the hypothesis that children with sagittal or unicoronal craniosynostosis, although they undergo early surgical treatment, may still manifest lower than average results at long-term selective neuropsychological evaluations.

Genetic basis of single-suture synostoses: genes, chromosomes and clinical implications

BackgroundNon syndromic craniosynostoses are the most frequent craniofacial malformations worldwide. They represent a wide and heterogeneous group of entities, in which the dysmorphism may occur in a single (simple forms) or in multiple sutures (complex forms). Simple forms present a higher birth prevalence and are classified according to the involved suture and to the corresponding abnormal cranial shape: scaphocephaly (SC; sagittal suture), trigonocephaly (TC; metopic suture), anterior plagiocephaly (unilateral coronal suture), posterior plagiocephaly (unilateral lambdoid suture). They occur commonly as sporadic forms, although a familiar recurrence is sometimes observed, suggesting a mendelian inheritance. The genetic causes of simple craniosynostosis are still largely unknown, as mutations in common craniosynostosis-associated genes and structural chromosomal aberrations have been rarely found in these cases.AimsThis review is intended to dissect comprehensively the state-of-the art on the genetic etiology of single suture craniosynostoses, in the attempt to categorize all known disease-associated genes and chromosomal aberrations. Possible genotype/phenotype correlations are discussed as useful clues towards the definition of optimized clinical management flowcharts.

Cavernous Angiomas of the Brain Stem in Children

Out of 27 children with intracranial cavernous angiomas observed in a 17-year period of time, 3 presented with their lesion located in the brain stem. A further 19 pediatric cases of brain stem cavernous angiomas sufficiently well described for a reliable analysis were collected from the literature and evaluated for the present study. The widespread use of MR imaging has significantly increased the possibility of recognizing brain stem cavernomas at an early age; indeed, their incidence is nowadays regarded to range between 9 and 15% of pediatric cavernous angiomas. A female predominance was noted in the present study. Focal neurological deficits are the most frequent presenting symptoms; they tend to occur acutely in most cases, although subsequent improvement may then be observed in several patients. Recurrent hemorrhages from the angioma result usually in heavier neurological signs. Surgical approaches have to be planned according to the location of the lesion, and to the site where the angioma is eventually in contact with the pial or ependymal surface. No surgical deaths were observed in these 22 children; only 2 patients presented a mild persistent worsening of their neurological status after the operation. These good results indicate that brain stem cavernous angiomas are actually a surgical lesion in spite of their apparently unfavorable location. The good prognosis is related to the anatomophysiological features of these lesions, which usually displace the brain stem structures rather than invade them, and are characterized by a low-pressure and slow blood flow circle.

Endoscopic Third Ventriculostomy for the Management of Chiari I and Related Hydrocephalus: Outcome and Pathogenetic Implications

BACKGROUND:Hydrocephalus affects 7% to 10% of patients with Chiari I malformation (CIM). It can be successfully treated by endoscopic third ventriculostomy (ETV), possibly improving related CIM and syringomyelia. OBJECTIVE:To confirm the effectiveness of ETV in the management of Chiari-related hydrocephalus and symptoms and to estimate the posterior cranial fossa volume (PCFV) to find the possible reasons for the success or failure of ETV. METHODS:Fifteen patients (11 children and 4 adults) underwent ETV for hydrocephalus associated with CIM (syringomyelia was present in 6 patients). Preoperative PCFV, posterior fossa brain volume (PFBV), and PFBV/PCFV ratio were calculated in the last 12 patients in the series by a magnetic resonance imaging–based computerized method. RESULTS:All patients had symptomatic hydrocephalus (mean third ventricle diameter, 14.1 mm). Mean tonsillar ectopia was 12.7 mm. Postoperatively, hydrocephalus symptoms improved in all cases (mean third ventricle diameter, 8.3 mm); signs and symptoms of CIM and syringomyelia resolved or improved in all patients, although the malformation remained radiologically stable in half of the patients (postoperative mean tonsillar ectopia, 8.8 mm). There were no remarkable differences between cases and controls with regard to PCFV and PFBV. The PFBV/PCFV ratio was comparable in pediatric cases and controls but not among adult patients, suggesting a PCF overcrowding in the controls. CONCLUSION:ETV is an effective treatment for hydrocephalus associated with CIM. It is successful in improving CIM and syringomyelia in patients with no overcrowding (mainly in children) or with reversible overcrowding of the PCF (mainly in adults).

Anterior plagiocephaly: epidemiology, clinical findings,diagnosis, and classification. A review

IntroductionAnterior plagiocephaly due to the early unilateral fusion of the coronal suture is associated to a clinical phenotype characterized by the unilateral flattening of the frontal bone and the orbit and by the distortion and maldevelopment of adjacent cranial and facial bones. Homolateral anterior displacement of the ear and contralateral deviation of the nasal pyramid and mandible are other prominent clinical features. The differential diagnosis includes a variety of conditions characterized by facial asymmetry, namely, due to muscular alterations, anomalies of the visual function, congenital anomalies of the cervical spine and craniovertebral junction, abnormal pregnancy, and birth injuries. Anterior plagiocephaly is the direct cause of oculomotor anomalies, with a characteristic strabismus, and of progressive asymmetrical maldevelopment of the face, craniovertebral junction, and cervical spine.MethodsThe medical literature was reviewed in regard to the epidemiology, clinical features, and radiological findings. Different categorization of the affected infants in specific subtypes according to the severity of the condition was analyzed. The aim was to contribute to a practical clinical classification to be utilized for the surgical indication and for predicting the prognosis according to the severity of the condition at presentation.ConclusionsAnterior plagiocephaly represents the most challenging simple suture craniosynostosis. The clinical differential diagnosis with other forms of cranial asymmetry is possible on the grounds of mere clinical findings. A classification system is necessary not only for the establishment of surgical planning but also to predict the late cosmetic and functional outcomes.

Functional hemispherectomy in children with epilepsy and CSWS due to unilateral early brain injury including thalamus: sudden recovery of CSWS

PURPOSE To try to prove in patients with refractory symptomatic epilepsy due to early brain injury involving thalamus and complicated by CSWS the effects of the isolation of the injured hemisphere, performed with functional hemisperectomy, on epilepsy, namely on CSWS. METHODS Full clinical follow-up before and after surgery of two cases with CSWS onset at four years in whom functional hemispherecomy was performed with resection of inter-hemispheric connections. RESULTS An immediate effectiveness of the surgical treatment was observed on both epileptic evolution (no more seizures) and EEG abnormalities. In particular, CSWS completely disappeared, together with a concurrent progressive improving of the cognitive and behavioural disorders. DISCUSSION The isolation of the injured hemisphere through the section of inter-hemispheric cortico-cortical connections could prevent the contralateral diffusion of discharges coming from the injured cortex and cortico-thalamic network, favouring a normal function of thalamo-cortico-thalamic circuitries in the healthy hemisphere. That could explain the disappearance of CSWS after surgery in our patients and the consequent improvement of cognitive abilities and behaviour.

The role of endoscopic choroid plexus coagulation in the surgical management of bilateral choroid plexuses hyperplasia.

BackgroundBilateral choroid plexus hyperplasia is a rare condition often associated with cerebrospinal fluid (CSF) overproduction. CSF overproduction is usually so high that the placement of a CSF ventriculoperitoneal shunt almost always results in progressive ascites leading to the necessity of removing the inserted shunt device. A direct surgical treatment of the hyperplastic choroid plexuses is then mandatory. Endoscopic coagulation of the choroid plexuses has been recently proposed as an alternative to open surgical plexectomy. However, the effectiveness of the procedure in controlling CSF overproduction is still debated.TechniqueWe report a case of bilateral choroid plexus hyperplasia in which an extensive bilateral endoscopic coagulation of the choroid plexuses failed to reduce the CSF formation rate sufficiently. A one-stage bilateral open surgical plexectomy was performed.ResultsThe procedure succeeded to control CSF overproduction. Intraoperative blood loss during the surgical removal of the choroid plexuses was significantly reduced due to the previous coagulation of their surface.ConclusionOn these grounds, we suggest that endoscopic choroid plexuses coagulation, even when failing to normalize CSF production, may still be considered as a valid adjuvant procedure in the management of this condition.

Trigeminal involvement in intracranial tumours. Anatomical and clinical observations on 73 patients.

SummaryNeoplastic involvement of the trigeminal nerve was observed in 73 patients operated on in our institution for extra-axial tumours of the posterior and middle cranial fossae.It was defined as contact, compression, or infiltration. The nerve root was involved in 58 patients, the ganglion and/or the peripheral divisions in 9, all portions of the Vth nerve system in 6. A clinical trigeminal dysfunction was present in 44 patients (60%).Anatomico-surgical findings are correlated with clinical features and with tumour type. Typical trigeminal neuralgia was the complaint in 7 subjects; all of them presented an involvement of the sensory root.Post-operatively, 11 patients were relieved of their symptoms. The outcome is correlated with the anatomical findings and with the extent of surgical removal of the tumours.The importance of a careful evaluation of patients with trigeminal symptomatology is stressed.