Abhaya V. Kulkarni
Abhaya V. Kulkarni and Ruth Donnelly
Detection of important venous collaterals by computed tomography venogram in multisutural synostosis
Case report and review of the literature
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.
Mostafa El Khashab
Benjamin C. Warf, John Mugamba, and Abhaya V. Kulkarni
In Uganda, childhood hydrocephalus is common and difficult to treat. In some children, endoscopic third ventriculostomy (ETV) can be successful and avoid dependence on a shunt. This can be especially beneficial in Uganda, because of the high risk of infection and long-term failure associated with shunting. Therefore, the authors developed and validated a model to predict the chances of ETV success, taking into account the unique characteristics of a large sub-Saharan African population.
All children presenting with hydrocephalus at CURE Children's Hospital of Uganda (CCHU) between 2001 and 2007 were offered ETV as first-line treatment and were prospectively followed up. A multivariable logistic regression model was built using ETV success at 6 months as the outcome. The model was derived on 70% of the sample (training set) and validated on the remaining 30% (validation set).
Endoscopic third ventriculostomy was attempted in 1406 patients. Of these, 427 were lost to follow-up prior to 6 months. In the remaining 979 patients, the ETV was aborted in 281 due to poor anatomy/visibility and in 310 the ETV failed during the first 6 months. Therefore, a total of 388 of 979 (39.6% and [55.6% of completed ETVs]) procedures were successful at 6 months. The mean age at ETV was 12.6 months, and 57.8% of cases were postinfectious in origin. The authors' logistic regression model contained the following significant variables: patient age at ETV, cause of hydrocephalus, and whether choroid plexus cauterization was performed. In the training set (676 patients) and validation set (303 patients), the model was able to accurately predict the probability of successful ETV (Hosmer-Lemeshow p value > 0.60 and C statistic > 0.70). The authors developed the simplified CCHU ETV Success Score that can be used in the field to predict the probability of ETV success.
The authors' model will allow clinicians to accurately identify children with a good chance of successful outcome with ETV, taking into account the unique characteristics and circumstances of the Ugandan population.
Abhaya V. Kulkarni, Ruth Donnelly, Donald J. Mabbott, and Elysa Widjaja
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?
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.
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.
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.
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.
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.