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Abhaya V. Kulkarni

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Merdas Al-Otibi, Andrew Jea and Abhaya V. Kulkarni

✓The authors describe the novel use of computed tomography (CT) venography in the preoperative evaluation of a child with Crouzon syndrome who was being considered for Chiari decompression. This 18-month-old girl presented with hydrocephalus (treated with a ventriculoperitoneal shunt) and persistent symptomatic Chiari malformation and associated syrinx. A CT venogram was obtained because of the well-described relationship between multisutural craniosynostosis and abnormal intracranial-to-extracranial venous drainage. The CT venogram showed widely dilated vertebral and paravertebral veins located in the paraspinous muscles of the craniocervical junction. Because of the risk of massive intraoperative blood loss and/or occlusion of important collateral draining veins leading to intracranial venous hypertension and intractably raised intracranial pressure, the planned posterior fossa decompression was not performed. Computed tomography venography is an easily obtained study that we recommend in the evaluation of children with multisutural craniosynostosis prior to cranial surgical interventions.

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Abhaya V. Kulkarni and Ruth Donnelly

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Abhaya V. Kulkarni, Ruth Donnelly, Donald J. Mabbott and Elysa Widjaja

OBJECT

Larger-than-normal ventricles can persist in children following hydrocephalus treatment, even if they are asymptomatic and clinically well. This study aims to answer the following question: do large ventricles result in brain injuries that are detectable on diffusion tensor imaging (DTI) and/or in measurable neurocognitive deficits in children with stable, treated hydrocephalus that are not seen in children with small ventricles?

METHODS

For this prospective study, we recruited 23 children (age range 8–18 years) with hydrocephalus due to aqueductal stenosis or tectal glioma who were asymptomatic following hydrocephalus treatment that had been performed at least 2 years earlier. All patients underwent detailed DTI and a full battery of neuropsychological tests. Correlation analysis was performed to assess the relationship between DTI parameters, neurocognitive tests, and ventricular size. The false-discovery rate method was used to adjust for multiple comparisons.

RESULTS

The median age of these 23 children at the time of assessment was 15.0 years (interquartile range [IQR] 12.1–17.6 years), and the median age at the first hydrocephalus treatment was 5.8 years (IQR 2.2 months–12.8 years). At the time of assessment, 17 children had undergone endoscopic third ventriculostomy and 6 children had received a shunt. After adjusting for multiple comparisons, there were no significant correlations between any neurocognitive test and ventricular volume, any DTI parameter and ventricular volume, or any DTI parameter and neurocognitive test.

CONCLUSIONS

Our data do not show an association between large ventricular size and additional white matter injury or worse neurocognitive deficits in asymptomatic children with stable, treated hydrocephalus caused by a discrete blockage of the cerebral aqueduct. Further investigations using larger patient samples are needed to validate these results.

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John R. W. Kestle, Abhaya V. Kulkarni and Benjamin C. Warf

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Abhaya V. Kulkarni, James M. Drake and Maria Lamberti-Pasculli

Object. Hydrocephalus is a common condition of childhood that usually requires insertion of a cerebrospinal fluid (CSF) shunt. Infection is one of the most devastating complications that may arise from the presence of CSF shunts. In this study, the authors prospectively analyzed perioperative risk factors for CSF shunt infection in a cohort of children.

Methods. Between 1996 and 1999, 299 eligible patients underwent CSF shunt operations (insertions and revisions) that were observed by a research nurse at a tertiary care pediatric hospital. Several perioperative variables were recorded. All cases were followed postoperatively for 6 months to note any development of CSF shunt infection. A Cox proportional hazards model was used to analyze the relationship between the variables and the development of shunt infection.

Thirty-one patients (10.4%) experienced shunt infection. Three perioperative variables were significantly associated with an increased risk of shunt infection: 1) the presence of a postoperative CSF leak (hazard ratio [HR] 19.16, 95% confidence interval [CI] 6.96–52.91); 2) patient prematurity (< 40 weeks' gestation at the time of shunt surgery: HR 4.72, 95% CI 1.71–13.06); and 3) the number of times the shunt system was inadvertently exposed to breached surgical gloves (HR 1.07, 95% CI 1.02–1.12).

Conclusions. Three variables associated with an increased incidence of shunt infection have been identified. Changes in clinical practice should address these variables, as follows. 1) Great care should be taken intraoperatively to avoid a postoperative CSF leak. 2) Alternatives to placement of a CSF shunt in premature infants should be studied. 3) Surgeons should minimize manual contact with the shunt system and consider the use of double gloves.

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Abhaya V. Kulkarni, Juan M. Bilbao, Michael D. Cusimano and Paul J. Muller

✓ Ganglioneuroma is generally considered to be a benign tumor and potentially surgically curable. The authors present a case of a 21-year-old woman who underwent resection of a retroperitoneal ganglioneuroma and developed spinal neuroblastoma 11 years later. She has survived 10 more years with only recent development of metastases. To the authors' knowledge, this is the first report of malignant transformation of a ganglioneuroma into a neuroblastoma. Also, such long-term survival in an adult with spinal neuroblastoma has not been reported previously. This case raises the possibility of a dedifferentiating potential for ganglion cells in a ganglioneuroma or the presence of a long-term, quiescent form of neuroblastoma.