Jennifer Strahle, Karin M. Muraszko, Steven R. Buchman, Joseph Kapurch, Hugh J. L. Garton and Cormac O. Maher
Chiari malformation (CM) Type I is frequently associated with craniosynostosis. Optimal management of CM in patients with craniosynostosis is not well-established. The goal of this study was to report on a series of pediatric patients with both craniosynostosis and CM and discuss their management.
The authors searched the medical records of 383 consecutive patients treated for craniosynostosis at a single institution over a 15-year period to identify those with CM. They recorded demographic data as well as surgical treatment and outcomes for these patients. When MR imaging was performed, cerebellar tonsillar descent was recorded and any other associated findings, such as hydrocephalus or spinal syringes, were noted.
A total of 29 patients with both CM and craniosynostosis were identified. Of these cases, 28% had associated occipital venous abnormalities, 45% were syndromic, and 52% also had hydrocephalus. Chiari malformation was more likely to be present in those patients with isolated lambdoid synostosis (55%), multisuture synostosis (35%), and pansynostosis (80%), compared with patients with coronal synostosis (6%) or sagittal synostosis (3%). All patients underwent surgical repair of craniosynostosis: 16 had craniosynostosis repair as well as CM decompression, and 13 patients did not undergo CM decompression. Of the 7 patients in whom craniosynostosis repair alone was performed, 5 had decreased tonsillar ectopia postoperatively and 5 had improved CSF flow studies postoperatively. Both patients with a spinal syrinx had imaging-documented syrinx regression after craniosynostosis repair. In 12 patients in whom CM was diagnosed after primary craniosynostosis repair, 5 had multiple cranial vault expansions and evidence of elevated intracranial pressure. In 5 cases, de novo CM development was documented following craniosynostosis repair at a mean of 3.5 years after surgery.
Chiari malformation is frequently seen in patients with both multi- and single-suture lambdoid craniosynostosis. Chiari malformation, and even a spinal cord syrinx, will occasionally resolve following craniofacial repair. De novo development of CM after craniosynostosis repair is not unusual.
Judith M. Wong, John E. Ziewacz, Allen L. Ho, Jaykar R. Panchmatia, Angela M. Bader, Hugh J. Garton, Edward R. Laws and Atul A. Gawande
As part of a project to devise evidence-based safety interventions for specialty surgery, the authors sought to review current evidence in CSF shunt surgery concerning the frequency of adverse events in practice, their patterns, and the state of knowledge regarding methods for their reduction. This review may also inform future and ongoing efforts for the advancement of neurosurgical quality.
The authors performed a PubMed search using search terms “cerebral shunt,” “cerebrospinal fluid shunt,” “CSF shunt,” “ventriculoperitoneal shunt,” “cerebral shunt AND complications,” “cerebrospinal fluid shunt AND complications,” “CSF shunt AND complications,” and “ventriculoperitoneal shunt AND complications.” Only papers that specifically discussed the relevant complication rates were included. Papers were chosen to be included to maximize the range of rates of occurrence for the adverse events reported.
In this review of the neurosurgery literature, the reported rate of mechanical malfunction ranged from 8% to 64%. The use of programmable valves has increased but remains of unproven benefit even in randomized trials. Infection was the second most common complication, with the rate ranging from 3% to 12% of shunt operations. A meta-analysis that included 17 randomized controlled trials of perioperative antibiotic prophylaxis demonstrated a decrease in shunt infection by half (OR 0.51, 95% CI 0.36–0.73). Similarly, use of detailed protocols including perioperative antibiotics, skin preparation, and limitation of OR personnel and operative time, among other steps, were shown in uncontrolled studies to decrease shunt infection by more than half.
Other adverse events included intraabdominal complications, with a reported incidence of 1% to 24%, intracerebral hemorrhage, reported to occur in 4% of cases, and perioperative epilepsy, with a reported association with shunt procedures ranging from 20% to 32%. Potential management strategies are reported but are largely without formal evaluation.
Surgery for CSF shunt placement or revision is associated with a high complication risk due primarily to mechanical issues and infection. Concerted efforts aimed at large-scale monitoring of neurosurgical complications and consistent quality improvement within these highlighted realms may significantly improve patient outcomes.