Hajdu-Cheney syndrome (HCS) is a rare genetic disorder with autosomal dominant inheritance, although most cases result from de novo mutations. Progressive platybasia and basilar impression (BI) can potentiate obstructive hydrocephalus due to aqueductal stenosis. Limited literature exists on the surgical intervention for hydrocephalus in patients with this condition. The authors present (to their knowledge) the first case of obstructive hydrocephalus due to aqueductal stenosis from BI treated with an endoscopic third ventriculostomy in a patient with the complex anatomy of HCS.
Ryan M. Johnson and Brent R. O’Neill
Hector E. James
Visish M. Srinivasan, Brent R. O'Neill, Diana Jho, Donald M. Whiting and Michael Y. Oh
External ventricular drainage (EVD) is one of the most commonly performed neurosurgical procedures. It was first performed as early as 1744 by Claude-Nicholas Le Cat. Since then, there have been numerous changes in technique, materials used, indications for the procedure, and safety. The history of EVD is best appreciated in 4 eras of progress: development of the technique (1850–1908), technological advancements (1927–1950), expansion of indications (1960–1995), and accuracy, training, and infection control (1995–present). While EVD was first attempted in the 18th century, it was not until 1890 that the first thorough report of EVD technique and outcomes was published by William Williams Keen. He was followed by H. Tillmanns, who described the technique that would be used for many years. Following this, many improvements were made to the EVD apparatus itself, including the addition of manometry by Adson and Lillie in 1927, and continued experimentation in cannulation/drainage materials. Technological advancements allowed a great expansion of indications for EVD, sparked by Nils Lundberg, who published a thorough analysis of the use of intracranial pressure (ICP) monitoring in patients with brain tumors in 1960. This led to the application of EVD and ICP monitoring in subarachnoid hemorrhage, Reye syndrome, and traumatic brain injury. Recent research in EVD has focused on improving the overall safety of the procedure, which has included the development of guidance-based systems, virtual reality simulators for trainees, and antibiotic-impregnated catheters.
Brent R. O'Neill, Alexander K. Yu and Elizabeth C. Tyler-Kabara
The term VACTERL represents a nonrandom association of birth defects including vertebral malformations, anal atresia, cardiac anomalies, tracheoesophageal fistulas (TEFs), renal anomalies, and limb malformations. Clinical experience and a few published case series suggest that a tethered spinal cord (TSC) occurs commonly in children with VACTERL, but to date, no study has defined the prevalence of TSC in patients with VACTERL. Such information would guide decisions about the appropriateness of screening spinal imaging.
The authors reviewed the charts of all patients discharged from the neonatal intensive care unit at Children's Hospital Pittsburgh in the past 14 years with the diagnosis of VACTERL, TEF, or anal atresia. During that period, the authors' protocol has been to use spinal ultrasound to screen this population for TSC. The charts were reviewed for the presence of a TSC requiring surgery and for the features of VACTERL.
Thirty-three patients with VACTERL and adequate spinal imaging studies were identified. In 13 (39%) of these, a TSC requiring surgery was identified. Among patients without VACTERL, the incidence of TSC was 7.9% in those with anal atresia and 2.4% in those with TEF. False-negative ultrasounds were identified in 21.4% of patients with TSC.
Children with VACTERL should undergo MR imaging screening for TSC. In infants with anal atresia without VACTERL, the incidence of TSC is much lower than in those with VACTERL.
Brent R. O’Neill, Michael H. Handler, Suhong Tong and Kevin E. Chapman
Seizures may cause diagnostic confusion and be a source of metabolic stress after traumatic brain injury (TBI) in children. The incidence of electroencephalography (EEG)-confirmed seizures and of subclinical seizures in the pediatric population with TBI is not well known.
A routine protocol for continuous EEG (cEEG) monitoring was initiated for all patients with moderate or severe TBI at a Level 1 pediatric trauma center. Over a 3.5-year period, all patients with TBI who underwent cEEG monitoring, both according to protocol and those with mild head injuries who underwent cEEG monitoring at the discretion of the treating team, were identified prospectively. Clinical data were collected and analyzed.
Over the study period, 594 children were admitted with TBI, and 144 of these children underwent cEEG monitoring. One hundred two (71%) of these 144 children had moderate or severe TBI. Abusive head trauma (AHT) was the most common mechanism of injury (65 patients, 45%) in children with cEEG monitoring. Seizures were identified on cEEG in 43 patients (30%). Forty (93%) of these 43 patients had subclinical seizures, including 17 (40%) with only subclinical seizures and 23 (53%) with both clinical and subclinical seizures. Fifty-three percent of patients with seizures experienced status epilepticus. Age less than 2.4 years and AHT mechanism were strongly correlated with presence of seizures (odds ratios 8.7 and 6.0, respectively). Those patients with only subclinical seizures had the same risk factors as the other groups. The presence of seizures did not correlate with discharge disposition but was correlated with longer hospital stay and intensive care unit stay.
Continuous EEG monitoring identifies a significant number of subclinical seizures acutely after TBI. Children younger than 2.4 years of age and victims of AHT are particularly vulnerable to subclinical seizures, and seizures in general. Continuous EEG monitoring allows for accurate diagnosis and timely treatment of posttraumatic seizures, and may mitigate secondary injury to the traumatized brain.
Ramesh Kumar, Frederic W. B. Deleyiannis, Corbett Wilkinson and Brent R. O'Neill
The authors' goals in this study were to describe a series of dog attacks on children that required neurosurgical consultation and to better understand the pattern of injuries inflicted, the circumstances that place children at risk for attack, and the dog breeds involved. In addition, the authors review the surgical and medical management of these patients.
The authors performed a retrospective review of all children requiring neurosurgical consultation for dog bite at a regional Level 1 pediatric trauma center over a 15-year period.
A total of 124 children with dog bites to the head, face, and neck were evaluated in the emergency department. Of these, 17 children (13.7%) incurred injuries requiring neurosurgical consultation. Fifty-three percent of victims were female. The mean age at the time of attack was 30 months. Twelve (71%) of the attacks were perpetrated by the family pet, and 13 (76%) occurred at the patient's home. Breeds involved in the attacks included German Shepherd, Pit Bull, American Bulldog, large mixed breed, Labrador Retriever, and Akita, with German Shepherds and Akitas being the most frequently involved. Neurosurgical injuries included nondepressed skull fracture in 5, depressed skull fracture in 10, intracranial hemorrhage in 5, cerebral contusions in 4, dural laceration in 4, pneumocephalus in 5, clinically evident CSF leak in 3, spinal fracture with complete spinal cord injury in 1, stroke in 2, vascular injury in 2, and cranial nerve injury (hypoglossal and facial nerve) in 1. Prophylactic antibiotics were administered in 16 patients (94%). Only 1 patient had a confirmed infection involving the site of injury. Neurosurgical intervention was required in 10 patients (59%) and ranged in severity from debridement and closure of a complex scalp wound to decompressive craniectomy. Neurological deficits, all of which were considered catastrophic, developed in 3 patients (18%).
Dog attacks on children requiring neurosurgical consultation commonly involve the family pet, which is usually a large-breed dog with no history of prior aggression. Neurosurgical injuries often involve the cranial vault, with depressed skull fractures being the most common injury pattern. Most patients do not suffer a neurological deficit, although catastrophic neurological injury may occur. Prophylactic antibiotics are commonly used and surgical intervention is required in the majority of cases.
Marlin Dustin Richardson, Nicholas O. Palmeri, Sarah A. Williams, Michelle R. Torok, Brent R. O’Neill, Michael H. Handler and Todd C. Hankinson
NSAIDs are effective perioperative analgesics. Many surgeons are reluctant to use NSAIDs perioperatively because of a theoretical increase in the risk for bleeding events. The authors assessed the effect of routine perioperative ketorolac use on intracranial hemorrhage in children undergoing a wide range of neurosurgical procedures.
A retrospective single-institution analysis of 1451 neurosurgical cases was performed. Data included demographics, type of surgery, and perioperative ketorolac use. Outcomes included bleeding events requiring return to the operating room, bleeding seen on postoperative imaging, and the development of renal failure or gastrointestinal tract injury.
Variables associated with both the exposure and outcomes (p < 0.20) were evaluated as potential confounders for bleeding on postoperative imaging, and multivariable logistic regression was performed. Bivariable analysis was performed for bleeding events. Odds ratios and 95% CIs were estimated.
Of the 1451 patients, 955 received ketorolac. Multivariate regression analysis demonstrated no significant association between clinically significant bleeding events (OR 0.69; 95% CI 0.15–3.1) or radiographic hemorrhage (OR 0.81; 95% CI 0.43–1.51) and the perioperative administration of ketorolac. Treatment with a medication that creates a known bleeding risk (OR 3.11; 95% CI 1.01–9.57), surgical procedure (OR 2.35; 95% CI 1.11–4.94), and craniotomy/craniectomy (OR 2.43; 95% CI 1.19–4.94) were associated with a significantly elevated risk for radiographically identified hemorrhage.
Short-term ketorolac therapy does not appear to be associated with a statistically significant increase in the risk of bleeding documented on postoperative imaging in pediatric neurosurgical patients and may be considered as part of a perioperative analgesic regimen. Although no association was found between ketorolac and clinically significant bleeding events, a larger study needs to be conducted to control for confounding factors, because of the rarity of these events.
Brent R. O'Neill, Danielle Gallegos, Alex Herron, Claire Palmer, Nicholas V. Stence, Todd C. Hankinson, C. Corbett Wilkinson and Michael H. Handler
Cutaneous stigmata or congenital anomalies often prompt screening for occult spinal dysraphism (OSD) in asymptomatic infants. While a number of studies have examined the results of ultrasonography (US) screening, less is known about the findings when MRI is used as the primary imaging modality. The object of this study was to assess the results of MRI screening for OSD in infants.
The authors undertook a retrospective review of all infants who had undergone MRI of the lumbar spine to screen for OSD over a 6-year period (September 2006–September 2012). All images had been obtained on modern MRI scanners using sequences optimized to detect OSD, which was defined as any fibrolipoma of the filum terminale (FFT), a conus medullaris ending at or below the L2–3 disc space, as well as more complex lesions such as lipomyelomeningocele (LMM).
Five hundred twenty-two patients with a mean age of 6.2 months at imaging were included in the study. Indications for imaging included isolated dimple in 235 patients (45%), asymmetrically deviated gluteal cleft in 43 (8%), symmetrically deviated (Y-shaped) gluteal cleft in 38 (7%), hemangioma in 28 (5%), other isolated cutaneous stigmata (subcutaneous lipoma, vestigial tail, hairy patch, and dysplastic skin) in 31 (6%), several of the above stigmata in 97 (18%), and congenital anomalies in 50 (10%).
Twenty-three percent (122 patients) of the study population had OSD. Lesions in 19% of these 122 patients were complex OSD consisting of LMM, dermal sinus tract extending to the thecal sac, and lipomeningocele. The majority of OSD lesions (99 patients [81%]) were filar abnormalities, a group including FFT and low-lying conus.
The rate of OSD ranged from 12% for patients with asymmetrically deviated gluteal crease to 55% for those with other isolated cutaneous stigmata.
Isolated midline dimple was the most common indication for imaging. Among this group, 20% (46 of 235) had OSD. There was no difference in the rate of OSD based on dimple location. Those with OSD had a mean dimple position of 15 mm (SD 11.8) above the coccyx. Those without OSD had a mean dimple position of 12.2 mm (SD 19) above the coccyx (p = 0.25).
The prevalence of OSD identified with modern high-resolution MRI screening is significantly higher than that reported with US screening, particularly in patients with dimples. The majority of OSD lesions identified are FFT and low conus. The clinical significance of such lesions remains unclear.
Seerat Poonia, Sarah Graber, C. Corbett Wilkinson, Brent R. O'neill, Michael H. Handler and Todd C. Hankinson
Postoperative management following the release of simple spinal cord–tethering lesions is highly variable. As a quality improvement initiative, the authors aimed to determine whether an institutional protocol of discharging patients on postoperative day (POD) 1 was associated with a higher rate of postoperative CSF leaks than the prior protocol of discharge on POD 2.
This was a single-center retrospective review of all children who underwent release of a spinal cord–tethering lesion that was not associated with a substantial fascial or dural defect (i.e., simple spinal cord detethering) during 2 epochs: prior to and following the institution of a protocol for discharge on POD 1. Outcomes included the need for and timing of nonroutine care of the surgical site, including return to the operating room, wound suturing, and nonsurgical evaluation and management.
Of 169 patients identified, none presented with CSF-related complications prior to discharge. In the preintervention group (n = 113), the postoperative CSF leak rate was 4.4% (5/113). The mean length of stay was 2.3 days. In the postintervention group, the postoperative CSF leak rate was 1.9% (1/53) in the patients with postdischarge follow-up. The mean length of stay in that group was 1.3 days.
At a single academic children's hospital, a protocol of discharging patients on POD 1 following uncomplicated release of a simple spinal cord–tethering lesion was not associated with an increased rate of postoperative CSF leaks, relative to the previous protocol. The rates identified are consistent with the existing literature. The authors' practice has changed to discharge on POD 1 in most cases.
Michael R. Levitt, Brent R. O'Neill, Gisele E. Ishak, Paritosh C. Khanna, Nancy R. Temkin, Richard G. Ellenbogen, Jeffrey G. Ojemann and Samuel R. Browd
Cerebrospinal fluid shunt placement has a high failure rate, especially in patients with small ventricles. Frameless stereotactic electromagnetic image guidance can assist ventricular catheter placement. The authors studied the effects of image guidance on catheter accuracy and shunt survival in children.
Pediatric patients who underwent placement or revision of a frontal ventricular CSF shunt were retrospectively evaluated. Catheters were placed using either anatomical landmarks or image guidance. Preoperative ventricular size and postoperative catheter accuracy were quantified. Outcomes of standard and image-guided groups were compared.
Eighty-nine patients underwent 102 shunt surgeries (58 initial, 44 revision). Image guidance was used in the placement of 56 shunts and the standard technique in 46. Shunt failure rates were not significantly different between the standard (22%) and image-guided (25%) techniques (p = 0.21, log-rank test). Ventricular size was significantly smaller in patients in the image-guided group (p < 0.02, Student t-test) and in the surgery revision group (p < 0.01). Small ventricular size did not affect shunt failure rate, even when controlling for shunt insertion technique. Despite smaller average ventricular size, the accuracy of catheter placement was significantly improved with image guidance (p < 0.01). Shunt accuracy did not affect shunt survival.
The use of image guidance improved catheter tip accuracy compared with a standard technique, despite smaller ventricular size. Failure rates were not dependent on shunt insertion technique, but an observed selection bias toward using image guidance for more at-risk catheter placements showed failure rates similar to initial surgeries.