✓作者描述小说使用的计算mography (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
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
Abhaya V. Kulkarni and Iffat Shams
Object
Children with hydrocephalus face several quality of life (QOL) issues that have been poorly studied. The authors' aim was to quantify the QOL for children with hydrocephalus and identify predictors of long-term outcome, using a reliable and validated outcome measure: the Hydrocephalus Outcome Questionnaire (HOQ).
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All children (5–18 years old) with treated hydrocephalus attending the neurosurgery outpatient clinic at the Hospital for Sick Children were asked to participate. The patient's QOL was measured by the parent-completed HOQ. Predictor variables were extracted from the medical records. Multivariable linear regression was used to identify those predictor variables that were significantly associated with outcome.
Results
There was an 89% participation rate, with a total of 346 children participating (mean age 11.7 years, mean duration since diagnosis 9.9 years). Their mean HOQ Overall Health score was 0.68 (on a scale of 0 [worst QOL] to 1.0 [best QOL]). On multivariable analysis, the following predictors were associated with a worse overall QOL: increased seizure frequency, increased length of stay (LOS) in the hospital for the initial treatment of hydrocephalus, increased LOS for treatment of shunt infection and shunt overdrainage, increased number of proximal shunt catheters in situ, and increased distance of the family residence from the pediatric neurosurgical center.
Conclusions
For the first time, these results establish baseline QOL values for a typical large group of children many years after their diagnosis of hydrocephalus, by using a validated and reproducible outcome measure. Many of the factors that adversely impact QOL appear to be related to shunt complications and might, therefore, be modifiable.
Abhaya V. Kulkarni
Mostafa El Khashab
Abhaya V. Kulkarni and Ruth Donnelly
Shelly Wang, James Drake, and Abhaya V. Kulkarni
OBJECTIVE
Spontaneous subaponeurotic fluid collection (SSFC) is an uncommon and newly described entity of unknown etiology, observed in infants less than 1 year of age. The authors report on series of infants who presented to the Hospital for Sick Children (HSC) with SSFC, focusing on the natural history of this condition.
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Data from the Hospital for Sick Children were retrospectively reviewed for the period between January 2004 and June 2015. Patient age and sex, birth history, medical history, laboratory findings, and symptoms were reviewed. SSFC location, imaging characteristics, management, and outcome were also analyzed. A MEDLINE and Embase literature search was performed on the condition, yielding previously reported cases of SSFC in the English language.
RESULTS
Nine cases involving patients who presented with SSFC during the study period were identified. The patients were 4 male and 5 female infants (age range 5 weeks to 11 months). All cases of SSFC developed spontaneously over a period of days, and the infants had no history of injuries, trauma, or hair manipulation in the immediate period preceding the development of the subgaleal collections. Six patients underwent remote forceps- or vacuum-assisted instrumented births, although none of the patients developed scalp collections or skin discoloration immediately after birth. All of the cases were managed conservatively on an outpatient (6 cases) or inpatient (3 cases) basis. In 1 case, the size of the fluid collection fluctuated over 4 months, but in all of the cases, the collections resolved spontaneously without structural or infectious complications.
CONCLUSIONS
This is the largest series describing SSFC to date and summarizes 9 cases managed at a large academic neurosurgical center. Although the specific pathophysiology of SSFC remains unknown, in some cases the condition may be associated with a remote history of instrumented delivery. SSFC occurs spontaneously without immediate preceding trauma, and an extensive hematology or child abuse workup is not necessary. A conservative approach with outpatient follow-up is advocated.
Abhaya V. Kulkarni, Janine Piscione, Iffat Shams, and Eric Bouffet
Object
面对越来越多的生存、生活质量e (QOL) has become an important indicator of treatment success in children with posterior fossa brain tumors (PFBTs). The authors' objective was to assess the long-term QOL in survivors of PFBT.
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The authors conducted a cross-sectional study of children who, between birth and age 18 years at diagnosis, had previously been treated at their institution for a PFBT. At the time of assessment for this study, children were between 5 and 19 years old and had received standard treatment for PFBT ending at least 6 months before the assessment. The QOL was measured with the Pediatric Quality of Life Inventory (PedsQL) generic score scales and the Health Utilities Index Mark 3 (HUI3). Multivariate analyses were used to assess several variables (patient related, treatment related, and socioeconomic) for association with QOL.
Results
共有62名儿童参加了研究(median age at assessment 11.9 years, interquartile range [IQR] 7.8–14.8, and median age at tumor diagnosis of 4.9 years, IQR 2.5–6.9). Median time since active treatment for their PFBT was 5.2 years (IQR 2.4–10.1). Tumor types included cerebellar pilocytic astrocytoma (45.2%), medulloblastoma (30.6%), ependymoma (11.3%), and brainstem astrocytoma (11.3%). Adjuvant therapy included chemotherapy (40.3%) or radiotherapy (14.5% focal and 21.0% craniospinal radiotherapy). Permanent treatment for hydrocephalus was required in 38.7% of the patients. Tumors recurred in 11.3%, requiring repeat treatment in these patients. The median HUI3 utility score was 0.91 (IQR 0.71–1.00) and the median PedsQL total score was 78.3 (IQR 64.1–92.4). Only the following variables were significantly associated with decreased QOL in multivariable model testing (all p < 0.05): need for permanent hydrocephalus treatment, large ventricle size, decreased family functioning, and lower family income.
Conclusions
As a group, long-term survivors of pediatric PFBT appear to have QOL indicators that are similar to those of the general population, although a reasonable minority of patients experience poor outcomes. Although several confounding variables likely remain in this retrospective study, important associations with QOL include the presence of hydrocephalus and socioeconomic factors. The study sample size, however, was limited and the presence of other important factors cannot be excluded.
