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Editorial. Is the helmet doing most of the job in the endoscopic correction of craniosynostosis?

Concezio Di Rocco

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Natural history of the Chiari Type I anomaly

Harold L. Rekate

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A modified slit-valve shunt prototype for the management of hydrocephalus

Concezio Di Rocco, Rino Mancinelli, Pietro Pola, and Francesco Velardi

✓ The hydraulic properties of a double-slit valve for cerebrospinal fluid (CSF) shunting have been tested in vitro and compared to those of the corresponding standard single-slit valve. The double-valve system proved to compensate better for large transitory increases in CSF pressure than the single valves, without significant variations in the mean pressure levels.

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Continuous intraventricular cerebrospinal fluid pressure recording in hydrocephalic children during wakefulness and sleep

Concezio Di Rocco, David G. McLone, Takeyoshi Shimoji, and Anthony J. Raimondi

✓ Continuous 24-hour recordings of intracranial pressure and electroencephalographic activity were made on five hydrocephalic children in whom, in the resting wakefulness state, the intracranial pressure (ICP) was considered normal. An increase in both the mean ICP and its oscillations related to cardiac systole was recorded during slow-wave sleep. Further episodic increases, up to a factor of 7 compared to wakefulness values, occurred during sleep. In three patients it was possible to correlate such episodic increases to the rapid eye movement phases of sleep. The authors discuss these phenomena and their possible implication in the progression of hydrocephalus.

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Preoperative neuropsychological and behavioral evaluation of children with thalamic tumors

Clinical article

Daniela Chieffo, Gianpiero Tamburrini, Massimo Caldarelli, and Concezio Di Rocco

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.

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Surgical management of cerebellopontine angle arachnoid cysts associated with hearing deficit in pediatric patients

Mario Giordano, Massimo Gallieni, Amir Samii, Concezio Di Rocco, and Madjid Samii

OBJECTIVE

Few cases of cerebellopontine angle (CPA) arachnoid cysts in pediatric patients have been described in the literature, and in only 2 of these cases were the patients described as suffering from hearing deficit. In this article, the authors report on 3 pediatric patients with CPA arachnoid cysts (2 with hearing loss and 1 with recurrent headaches) who underwent neurosurgical treatment at the authors’ institution.

METHODS

Four pediatric patients were diagnosed with CPA arachnoid cysts at the International Neuroscience Institute during the period from October 2004 through August 2012, and 3 of these patients underwent surgical treatment. The authors describe the patients’ clinical symptoms, the surgical approach, and the results on long-term follow-up.

RESULTS

One patient (age 14 years) who presented with headache (without hearing deficit) became asymptomatic after surgical treatment. The other 2 patients who underwent surgical treatment both had hearing loss. One of these children (age 9 years) had recent-onset hypacusia and experienced complete recovery immediately after the surgery. The other (age 6 years) had a longer history (2 years) of progressive hearing loss and showed an interruption of the deficit progression and only mild improvement at the follow-up visit.

CONCLUSIONS

CPA arachnoid cysts are uncommon in pediatric patients. The indication and timing of the surgical treatment are fundamental, especially when a hearing deficit is present.

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Intracranial midline dermoid and epidermoid cysts in children

Massimo Caldarelli, Luca Massimi, Charles Kondageski, and Concezio Di Rocco

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.

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Spinal epidural angiolipoma complicated by an intratumoral abscess

Case report

Gianpaolo Petrella, Gianpiero Tamburrini, Libero Lauriola, and Concezio Di Rocco

✓Spinal angiolipomas are rare, benign lesions representing 0.14 to 1.2% of all spinal axis tumors. They most commonly involve the midthoracic spine and are located in the posterior epidural space. Up to now, six pediatric cases have been reported in the literature; two of them involved an acute clinical onset that was related to a venous infarction of a tumor. The authors report the case of a 16-year-old boy with a midthoracic epidural angiolipoma who was admitted with a clinical history of an acute paraparesis. In contrast to previous descriptions, the acute onset in this case was related to a spontaneous intratumoral abscess within the tumor.

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Midline Dermoid Cysts

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Introduction. Controversies in the management of single-suture craniosynostosis

Concezio Di Rocco, John R. W. Kestle, Richard Hayward, and Jesse A. Taylor