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Neurocutaneous Melanosis

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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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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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Endoscopic transventricular positioning of intracystic catheter for treatment of craniopharyngioma

Technical note

Benedetta L. Pettorini, Gianpiero Tamburrini, Luca Massimi, Massimo Caldarelli, and Concezio Di Rocco

The intracystic injection of chemo- and radiotherapeutic agents was introduced for the treatment of craniopharyngioma to control tumor growth and to delay the potentially harmful effects of surgery or radiation therapy. The positioning of cyst catheters has been performed by means of direct vision, stereotactically guided insertion, and ultrasonographic and ventriculoscopic guidance. The insertion of a catheter into the cyst is not devoid of complications, with an incidence ranging up to 16%, independent of the surgical technique used.

Eight patients (mean age 25.8 years) with symptomatic cystic craniopharyngioma were treated by means of an endoscopic transventricular approach for the insertion of an intracystic catheter for intratumoral therapy with interferon-α.

A single right precoronal bur hole is made, and the frontal horn of the lateral ventricle is accessed under neuronavigation guidance. A ventricular catheter with an inserted stylet was advanced anterior to the endoscope sheath through the same cortical access as the endoscope and was guided under endoscopic view down to the cyst dome wall. The coagulated surface of the craniopharyngioma cyst was punctured and the tip of the ventricular catheter was advanced; the depth was established preoperatively on MR scans and confirmed by neuronavigation guidance. The proximal end of the cystic catheter was connected to an access chamber to be left in the subcutaneous space, and the endoscope was slowly retracted.

The authors' experience favors the use of neuroendoscopic positioning of intracystic catheters as safer than open and stereotactic approaches.

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Sudden onset of Chiari malformation Type I in previously asymptomatic patients

Report of 3 cases

Luca Massimi, Giuseppe Maria Della Pepa, Gianpiero Tamburrini, and Concezio Di Rocco

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.

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Descending transtentorial herniation, a rare complication of the treatment of trapped fourth ventricle: case report

Paolo Frassanito, Georgios Markogiannakis, Rina Di Bonaventura, Luca Massimi, Gianpiero Tamburrini, and Massimo Caldarelli

Descending transtentorial herniation (DTH) is a complication of raised pressure in the supratentorial compartment, usually resulting from mass lesion of several etiologies. The authors report an exceptional case of DTH complicating the implant of a CSF shunting device in the trapped fourth ventricle of a 17-year-old boy in whom a second CSF shunting device had been implanted for neonatal posthemorrhagic and postinfectious hydrocephalus. The insidious clinical and radiological presentation of DTH, mimicking a malfunction of the supratentorial shunt, is documented. Ultimately, the treatment consisted of removal of the infratentorial shunt and endoscopic acqueductoplasty with stenting. The absence of supratentorial mass lesion and other described etiologies of DTH prompted the authors to speculate on the hydrodynamic pathogenesis of DTH in the present case.

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Retrieval of ventricular catheter with the aid of endoscopy

Technical note

Benedetta Ludovica Pettorini, Paolo Frassanito, Gianpiero Tamburrini, Luca Massimi, Massimo Caldarelli, and Concezio Di Rocco

Multilocular hydrocephalus usually requires placement of multiple ventricular catheters for the treatment of secondary cysts and intraventricular septation. The formation of strong adhesions can embed the catheters so that they cannot be removed without a higher risk of intraventricular hemorrhage. Moreover, the devices could represent a nidus for infection and a risk for formation and enlargement of intraventricular secondary cysts. Neuroendoscopy allows the surgeon safely to reach the ventricular catheters that have been left or lost in the ventricular cavity, and to remove them via a minimally invasive approach. In this paper, the authors document another application of ventricular endoscopy in the management of this kind of hydrocephalus. The removal of an unnecessary and no longer useful prosthetic device that is a possible focus of infection justifies endoscopic treatment, which, in this experience, is not associated with morbidity.

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The natural history of the Chiari Type I anomaly

Federica Novegno, Massimo Caldarelli, Antonio Massa, Daniela Chieffo, Luca Massimi, Benedetta Pettorini, Gianpiero Tamburrini, and Concezio Di Rocco

Object

Since the advent of MR imaging, an increasing number of asymptomatic or oligosymptomatic patients have been diagnosed with Chiari malformation Type I (CM-I). The decision of whether or not to operate is more difficult in these patients than in those with clear symptoms because of the lack of information about the natural course of this disease.

Methods

The authors report on their experience in a series of 22 patients with CM-I who were evaluated at the authors' institution, and for whom a conservative approach to treatment was adopted. The patients ranged in age from 1 to 16 years (mean 6.3 years) at diagnosis. Neuroradiological and complete clinical examinations were performed in all patients 6 months after the first observation and every year thereafter. The follow-up period ranged from 3 to 19 years (mean 5.9 years).

Results

Chiari malformation Type I was incidentally detected on MR images in 11 of 22 patients. The remaining 11 patients had minimal clinical signs at presentation that were not regarded as necessitating immediate surgical treatment. Seventeen patients (77.3%) showed progressive improvement in their symptoms or remained asymptomatic at the last follow-up whereas 5 patients (22.7%) experienced worsening, which was mild in 2 cases and required surgical correction in the remaining 3 cases. On MR imaging a mild reduction in tonsillar herniation was appreciated in 4 patients (18.18%), with complete spontaneous resolution in 1 of these. In 16 patients, tonsillar herniation remained stable during follow-up.

Conclusions

The authors' data confirm the common impression that in both asymptomatic and slightly symptomatic patients with CM-I, a conservative approach to treatment should be adopted with periodic clinical and radiological examinations.

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Calcified cephalohematoma as an unusual cause of EEG anomalies: case report

Vera Vigo, Domenica Immacolata Battaglia, Paolo Frassanito, Gianpiero Tamburrini, Massimo Caldarelli, and Luca Massimi

Cephalohematoma, one of the most common neonatal head injuries, generally undergoes spontaneous resorption. When calcified, it may cause cranial vault distortion and depression of the inner skull layer, although it remains asymptomatic. Surgery, indeed, is usually performed for cosmetic purposes. For these reasons, the long-term effects of calcified cephalohematoma (CC) are widely unknown.

The authors report the case of an 11-year-old girl with a persistent calcified CC causing skull deformity and delayed electroencephalography (EEG) anomalies. These anomalies were detected during routine control EEG and were not clinically evident. The young girl underwent surgical removal of the CC for cosmetic purpose. The EEG abnormalities disappeared after surgery, thus reinforcing the hypothesis of a correlation with the brain “compression” resulting from the CC. To the best of the authors' knowledge this is the first time that CC-associated EEG anomalies have been described: even though these anomalies cannot be considered an indication for surgery, they merit late follow-up in case of skull deformity.

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Chiari Malformation Type I

Hector E. James