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Pietro Spennato, Carmela Chiaramonte, Domenico Cicala, Vittoria Donofrio, Manlio Barbarisi, Anna Nastro, Giuseppe Mirone, Vincenzo Trischitta, and Giuseppe Cinalli

OBJECTIVE

Intraventricular choroid plexus cysts are unusual causes of acute hydrocephalus in children. Radiological diagnosis of intraventricular choroid plexus cysts is difficult because they have very thin walls and fluid contents similar to CSF and can go undetected on routine CT studies.

METHODS

This study reports the authors' experience with 5 patients affected by intraventricular cysts originating from the choroid plexus. All patients experienced acute presentation with rapid neurological deterioration, sometimes associated with hypothalamic dysfunction, and required urgent surgery. In 2 cases the symptoms were intermittent, with spontaneous remission and sudden clinical deteriorations, reflecting an intermittent obstruction of the CSF pathway.

RESULTS

Radiological diagnosis was difficult in these cases because a nonenhanced CT scan revealed only triventricular hydrocephalus, with slight lateral ventricle asymmetry in all cases. MRI with driven-equilibrium sequences and CT ventriculography (in 1 case) allowed the authors to accurately diagnose the intraventricular cysts that typically occupied the posterior part of the third ventricle, occluding the aqueduct and at least 1 foramen of Monro. The patients were managed by urgent implantation of an external ventricular drain in 1 case (followed by endoscopic surgery, after completing a diagnostic workup) and by urgent endoscopic surgery in 4 cases. Endoscopic surgery allowed the shrinkage and near-complete removal of the cysts in all cases. Use of neuronavigation and a laser were indispensable. All procedures were uneventful, resulting in restoration of normal neurological conditions. Long-term follow-up (> 2 years) was available for 2 patients, and no complications or recurrences occurred.

CONCLUSIONS

This case series emphasizes the necessity of an accurate and precise identification of the possible causes of triventricular hydrocephalus. Endoscopic surgery can be considered the ideal treatment of choroid plexus cysts in children.

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Giuseppe Cinalli, Daniel T. Aguirre, Giuseppe Mirone, Claudio Ruggiero, Daniele Cascone, Lucia Quaglietta, Ferdinando Aliberti, Serena de’ Santi, Maria Consiglio Buonocore, Anna Nastro, and Pietro Spennato

OBJECTIVE

In the past, the outcome of surgical treatment for thalamic tumor was poor. These lesions were often considered inoperable. However, contemporary microsurgical techniques, together with improvements in neuroimaging that enable accurate presurgical planning, allow resection to be accomplished in a safer way.

METHODS

The medical records, imaging studies, and operative and pathology reports obtained for pediatric patients who were treated for thalamic tumors at the authors’ department were reviewed. Neuronavigation and intraoperative monitoring of motor and somatosensory evoked potentials were used. Preoperative tractography, which helped to identify internal capsule fibers, was very important in selecting the surgical strategy. Postoperatively, an MRI study performed within 24 hours was used to assess the extent of tumor resection as partial (≤ 90%), subtotal (> 90%), or gross total (no residual tumor).

RESULTS

Since 2002, 27 children with thalamic tumors have been treated at the authors’ department. There were 9 patients with unilateral thalamic tumors, 16 with thalamopeduncular tumors, and 2 with a bilateral tumor. These last 2 patients underwent endoscopic biopsy and implantation of a ventriculoperitoneal shunt. Thirty-nine tumor debulking procedures were performed in the remaining 25 patients. Different surgical approaches were chosen according to tumor location and displacement of the posterior limb of the internal capsule (as studied on axial T2-weighted MRI) and corticospinal tract (as studied on diffusion tensor imaging with tractography, after it became available). In 12 cases, multiple procedures were performed; in 7 cases, these were done as part of a planned multistage resection. In the remaining 5 cases, the second procedure was necessary because of late recurrence or regrowth of residual tumor.

At the end of the surgical phase, of 25 patients, 15 (60%) achieved a gross-total resection, 4 (16%) achieved a subtotal resection, and 6 (24%) achieved a partial resection. Eighteen patients harbored low-grade tumors in our series. In this group, the mean follow-up was 45 months (range 4–132 months). At the end of follow-up, 1 patient was dead, 12 patients were alive with no evidence of disease, 4 patients were alive with stable disease, and 1 was lost to follow-up. All patients were independent in their daily lives. The outcome of high-grade tumors in 9 patients was very poor: 2 patients died immediately after surgery, 6 died of progressive disease, and 1 was alive with residual disease at the time of this report.

CONCLUSIONS

This institutional review seems to offer further evidence in favor of attempts at radical resection in pediatric patients harboring unilateral thalamic or thalamopeduncular tumors. In low-grade gliomas, radical resection in a single or staged procedure can be curative without complementary treatment. Recurrences or residual regrowth can be safely managed surgically. In high-grade tumors, the role of and opportunity for radical or partial resection remains a matter of debate.