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Object Functional involvement of the thalamus in cognitive processing has been only anecdotally reported in the literature, and these cases are mostly related to thalamic hemorrhages; there is no available information on cognitive development in children with thalamic tumors. Methods All children admitted with a diagnosis of thalamic tumor at the authors' institution between January 2008 and January 2011 were considered for the present study. Exclusion criteria were age less than 18 months and the presence of severe neurological deficits, both of which prevented a reliable neuropsychological evaluation. A complete preoperative neuropsychological evaluation was performed. Results Twenty children were selected (mean age 102.4 months). Total IQ was in the normal range in all patients (mean 90.1, SD 13.87) with a significant difference between verbal IQ (mean 97.70, SD 17.77) and performance IQ (mean 84.82, SD 17.01). A significant correlation was found between global cognitive impairment and a histological finding of low-grade tumors (p < 0.001). Children with a mesial thalamic tumor had a higher working memory deficit and delayed recall disorders (p < 0.001). Naming disorders were related to the presence of a bilateral (p < 0.001) or mesial (p < 0.001) thalamic tumor, without a significant difference between left or right hemisphere involvement. A significant correlation was also found between the presence of neurolinguistic disorders and mesially located tumors (p < 0.001). Children with right-sided tumors more frequently had constructional apraxia and executive function disorders (p < 0.001). Conclusions The present study suggests that thalamic tumors in different locations might have specific neuropsychological profiles.

Palsy of the abducens nerve is a neurological sign that has a wide range of causes due to the nerve's extreme vulnerability. Need of immediate neuroimaging is a matter of debate in the literature, despite the risks of delaying the diagnosis of a skull base tumor. The authors present 2 cases of skull base tumors in which the patients presented with recurrent and self-remitting episodes of sixth cranial nerve palsy (SCNP). In both cases the clinical history exceeded 1 year. In a 17-year-old boy the diagnosis was made because of the onset of headache when the tumor reached a very large size. In a 12-year-old boy the tumor was incidentally diagnosed when it was still small. In both patients surgery was performed and the postoperative course was uneventful. Pathological diagnosis of the tumor was consistent with that of a chondrosarcoma in both cases. Recurrent self-remitting episodes of SCNP, resembling transitory ischemic attacks, may be the presenting sign of a skull base tumor due to the anatomical relationships of these lesions with the petroclival segment of the sixth cranial nerve. Physicians should promptly recommend neuroimaging studies if SCNP presents with this peculiar course.

Chiari malformation Type I (CM-I) is usually suspected in patients with slowly progressing neurological symptoms. However, in some instances, especially if syringomyelia is associated, an abrupt clinical onset is reported and is accompanied by an acknowledged risk of potentially severe clinical signs or even sudden death. Little is known about such a critical course in CM-I/syringomyelia complex. The authors describe 3 challenging cases of the abrupt onset of CM-I/syringomyelia to reveal more information on the clinical presentation and pathogenetic mechanisms of this sudden and potentially severe clinical phenomenon: a 38-year-old man experienced acute respiratory failure requiring intubation following acute decompensation of hydrocephalus associated with Noonan syndrome, a 1-year-old boy had sudden hemiparesis and Horner syndrome after a minor head/neck injury, and a 2.5-year-old boy presented with quickly progressing tetraplegia and dyspnea after a mild flexion and extension neck injury a few hours before. All 3 patients showed a CM-I/syringomyelia complex at diagnosis, and all of them had a good neurological outcome after surgery despite the ominous clinical presentation.

Object Complete ependymoma resection ensures a better prognosis for children with this tumor, but the complete excision of infratentorial ependymomas involves serious risks. Second-look surgery for tumor remnants may be less harmful and enable complete removal. There is a potential, although still unclear, role for neoadjuvant chemotherapy in preparation for further surgery. Methods Since 1994, the authors have adopted two successive protocols for intracranial ependymoma, both including a phase of adjuvant chemotherapy for children with surgical tumor remnants with a plan for potential second-look surgery before radiotherapy. Results In the first protocol, 9 of 63 children underwent further surgery, and 6 became tumor free with no additional sequelae. Their prognosis for progression-free survival and freedom from local relapse was comparable to that of children who were operated on only once. In the second protocol, efforts were made to achieve complete resection and 29 of 110 patients underwent reoperations: 9 after the first surgery, 17 after chemotherapy, and 3 soon after radiotherapy. Fourteen of the 29 patients became tumor free, 1 of them with worsening neurological symptoms. The outcome of the 66 patients who became tumor free after 1 operation was compared with that of the 14 who became tumor free after reoperation. The 3-year progression-free survival of the 66 patients compared with the 14 other patients was 71.4% ± 6.9% and 90% ± 9.5%, respectively; the 3-year freedom from local relapse was 84.7% ± 5.9% and 90% ± 9.5%, respectively; and the 3-year overall survival was 85.9% ± 5.4% and 87.5% ± 11.7%, respectively. Conclusions Second-look surgery proved feasible with no major morbidity, and results improved with time. Local tumor control was comparable in patients undergoing 1 or more resections.

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 patient's 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.

The authors report a case of minimal prenatal trauma producing a large subdural hematoma in the fetus, which was diagnosed in utero by MR imaging. The occurrence of such a complication is extremely rare in the absence of significant maternal trauma. Prenatally diagnosed intracranial hemorrhages, particularly those that are subdural in origin, have a poor prognosis in most cases. After birth, brain compression required a complex neurosurgical intervention because simple hematoma evacuation was not possible. The clinical and neurological outcome at 6 months was excellent, as confirmed by the neuroimaging findings.

Scalp masses are not infrequently encountered in daily clinical practice. They are represented by a wide spectrum of different clinical entities and are usually managed by an excision or by simple observation. Although it happens rarely, head lumps may hide an underlying cranioencephalic malformation that has to be preoperatively diagnosed to perform an appropriate treatment. Cerebral arteriovenous malformations (AVMs) are not included among the intracranial malformations connected with a scalp mass. The authors report on the unusual case of a child harboring a complex intracranial AVM that initially presented as a small scalp mass. Actually, this young boy came to the authors' attention just for a small, soft, pulsatile, and reducible mass of the vertex that produced a circumscribed bone erosion. The presence of macrocranium and venous engorgement of the face, however, suggested the presence of an intracranial “mass.” The neuroimaging investigations pointed out a temporal AVM causing dilation of the intracranial sinuses and ectasia of the vein of the scalp; one of the veins was appreciable as a lump on the vertex.

Accessory human tails are uncommon malformations consisting of meningeal and neural structures and possibly occult spinal dysraphism (pseudotails). The thoracic location of a lipomeningocele constituting a pseudotail is very rare. The authors report the case of a young boy harboring a thoracic dorsal appendage consisting of a lipomeningocele and a dermoid cyst that was complicated by the presence of diastematomyelia, tethered spinal cord, syringomyelia, and mild hydrocephalus. The preoperative moderate ventricular dilation was decompressed by the surgical repair of the spinal malformation and required a specific treatment. The authors discuss the rare association of these clinical entities and conclude that complete spine and brain neuroimaging studies are necessary for the correct surgical planning of this kind of malformation.

Object. Despite progress in the understanding of the causes and pathophysiology of Chiari malformation Type I (CM-I), definitive surgical treatment remains unresolved. Various techniques have been propounded over the years, but there is no general consensus on the most appropriate surgical management for this condition. The authors report their experience with the surgical treatment of 30 pediatric patients with CM-I. Methods. The results obtained in 30 patients who underwent surgery for symptomatic CM-I with a purely extradural procedure were retrospectively reviewed. The patient age at operation ranged from 2 months to 16 years (mean 68 months). In 26 patients the CM-I occurred as an isolated disease, whereas in four a recognized bone anomaly involving the cranial base (achondroplasia) was observed. All patients underwent suboccipital craniectomy, which was accompanied by C-l laminectomy in 22. In all cases a thick fibrous band at the level of foramen magnum was resected; in 11 children serial incisions of the outer layer of the dura mater were performed as well to expand the posterior fossa volume. The follow-up duration varied from a minimum of 12 months to 12.6 years (mean 4.7 years). The most frequent symptoms and signs were head and/or neck pain (56.7%), followed by vertigo (27.7%), upperand lower-extremity weakness (20.0%), and ataxia (20.0%). Syringomyelia was noted in 12 patients (40.0%) on magnetic resonance (MR) images. Improvement in or resolution of clinical symptoms and signs was observed in all patients. However, only minimal or no modifications could be found in the position of cerebellar tonsils on postoperative MR images in most patients (only occasional improvement in tonsillar herniation), whereas syringomyelic cavitations reduced in size in half of the cases. Nevertheless, in only two patients was a reoperation necessary. Conclusions. In the authors’ experience, suboccipital craniectomy and C-l laminectomy (eventually integrated by dural delamination) can represent an effective treatment for symptoms associated with CM-I.

Object. Dermoid and epidermoid cysts are rare space-occupying lesions of the central nervous system. Although characterized by a slow growth rate, they are often associated with serious complications. Surgery is the only effective treatment, and radical resection of the entire cyst, whenever possible, generally succeeds in achieving a cure. Authors of large series have only occasionally reported on pediatric cases, and these reports often lack a specific analysis of those cerebral midline lesions. Methods. The authors report on the treatment of 19 patients (16 with intracranial intradural dermoid and three with epidermoid cysts located along the cerebral midline). All patients underwent surgery at the Catholic University Medical School in Rome. The patients ranged in age from 3 months to 16 years. Nine cysts were located in the posterior cranial fossa, six in the frontobasal subarachnoid spaces, two in the third ventricle, and two in the quadrigeminal plate cistern. In the cases presenting with dermal sinus tracts, attempts at resecting the dermoid cyst and the associated dermal sinus were made in a single stage to achieve an en bloc removal. In the cases without dermal sinus tracts, and in the three with epidermoid cysts, a standard craniotomy was performed to reach the lesion. There were no surgery-related deaths, and the morbidity rate was low. Total and subtotal resections were achieved in 15 and four cases, respectively; however, regrowth of the residual tumor was observed only in two of them. After repeated resection, tumor progression has not been observed. At a mean follow-up period of 92.7 months, excellent clinical results were achieved in 18 patients. Conclusions. Because surgery is the only effective treatment modality for these lesions, radical resection should be performed in all cases to avoid tumor recurrence; however, because the cyst capsule can adhere firmly to vital structures and attempts at its radical removal can be dangerous, subtotal resection may be a wise option in selected cases.