Management of ventriculomegaly in pediatric patients with syndromic craniosynostosis: a single center experience.

INTRODUCTION: Management of ventriculomegaly in pediatric patients with syndromic craniosynostosis (SC) requires understanding the underlying mechanisms that cause increased intracranial pressure (ICP) and the role of cerebrospinal fluid (CSF) in cranial vault expansion in order to select the best treatment option for each individual patient. METHODS: A total of 33 pediatric patients with SC requiring craniofacial surgery were retrospectively evaluated. Cases of nonsyndromic craniosynostosis and shunt-induced craniosynostosis were excluded. Six syndrome-based categories were distinguished: Crouzon syndrome, Pfeiffer syndrome, Apert syndrome, cloverleaf skull syndrome, and others (Muenke syndrome, Sensenbrenner syndrome, unclassified). All of the patients were treated surgically for their cranial deformity between 2010 and 2016. The presence of ventriculomegaly and ventriculoperitoneal (VP) shunt requirement with its impact in cranial vault expansion were analyzed. Clinical and neuroimaging studies covering the time from presentation through the follow-up period were revised. The mean postoperative follow-up was 6 years and 3 months. A systematic review of the literature was conducted through a PubMed search. RESULTS: Of the total of 33 patients with SC, 18 (54.5%) developed ventriculomegaly and 13 (39.4%) required ventriculoperitoneal (VP) shunt placement. Six patients (18.2%) required shunt placement previous to craniofacial surgery. Seven patients (21.2%) required a shunt after craniofacial surgery. Seven fixed pressure ventriculoperitoneal shunts and six programmable valves were placed as first choice. All patients improved their clinical symptoms after shunt placement. Aesthetic results seemed to be better in patients with programmable shunts. CONCLUSIONS: Unless clear criteria for overt hydrocephalus are present, it is recommended to perform craniofacial surgery as a first step in the management of patients with SC in order to preserve the expansive effect of CSF for cranial vault expansion. In our experience, the use of externally programmable valves allows for the treatment of hydrocephalus while maintaining the expansive effect of CSF for the remodeling of the cranial vault. Prospective evaluations are needed to determine causality.

Leptomeningeal lymphoma in a child with acquired immune deficiency syndrome.

Primary non-Hodgkin lymphoma of the central nervous system is rare in pediatric AIDS patients. We report a seven-year-old HIV-infected boy, in stage C3 of the disease, who developed non-Hodgkin lymphoma in the central nervous system with a leptomeningeal location. The patient started with signs and symptoms of increased intracranial pressure, impaired consciousness and then became blind. The diagnosis was based on brain biopsy, immunophenotypic studies of B cells, and Epstein-Barr virus serology of the cerebrospinal fluid. The boy was treated with intrathecal and systemic chemotherapy. Fifteen months after diagnosis he had clinically improved, but he then relapsed with a thalamic tumor. He was treated with radiotherapy and he died four months later. In the present article, we discuss diagnostic difficulties, evolution, treatment, and the association of this neoplasm with the Epstein-Barr virus.

Complications in paediatric craniopharyngioma treatment.

Forty-eight consecutive children treated for craniopharyngioma at the Juan P. Garrahan National Paediatric Hospital (Buenos Aires, Argentina) from 1988 to 1994 are described. Complications of patients undergoing total resection alone and those undergoing subtotal or partial resection plus radiotherapy were compared. Survival time and quality of life proved more satisfactory in the former group, as there were no recurrences. In contrast, among the latter patients, 53% suffered relapses. Endocrinological complications were similar in the two groups. Postsurgical subdural haematomas were quite frequent and eight patients required treatment for intracranial hypertension. Vascular complications, though less common, led to high morbidity and mortality. There was a considerable incidence of shunt malfunction (80%), arguing against placement of a preoperative shunt, which tended besides to foster postsurgical subdural haematomas.