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Douglas L. Brockmeyer, Meghan M. Brockmeyer, and Taryn Bragg

Object

Congenital craniovertebral anomalies are relatively common, but anomalies leading to overt craniocervical instability may be difficult to recognize and treat. The authors present a series of patients with atlantal hemi-rings, a disorder resulting in congenital craniovertebral instability. Presentation, treatment, imaging, and follow-up data obtained in patients with atlantal hemi-rings were assessed to identify factors relevant to craniocervical instability.

Methods

Nineteen patients were identified with atlantal hemi-rings, defined as a bony discontinuity of the C-1 ring in conjunction with lateral displacement of the C-1 lateral masses (as seen on coronal CT scans). Clinical and radiological characteristics were analyzed, including patient age at presentation, extent of occipitocervical motion, amount of C-1 lateral mass displacement, associated craniocervical anomalies, integrity of the transverse ligament, and neurological status.

Results

The mean patient age at presentation was 22 months (range birth to 9 years). The mean amount of occipitocervical translation seen on dynamic imaging was 9 mm (range 2–20 mm). Four patients required occipitocervical fusion at presentation. The remaining 15 patients were monitored for a mean of 20 months, and 9 ultimately underwent fusion. Surgery was also recommended for 4 of the remaining 6 children.

Conclusions

This report describes the radiological and clinical characteristics of patients with atlantal hemirings and craniocervical instability. The authors believe that this anomaly is the underlying cause of progressive instability in a significant proportion of patients with craniocervical abnormalities. The presence of atlantal hemi-rings should prompt immediate and thorough neurosurgical evaluation.

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Mark R. Kraemer, Carolina Sandoval-Garcia, Taryn Bragg, and Bermans J. Iskandar

OBJECTIVE

The authors conducted a survey to evaluate differences in the understanding and management of shunt-dependent hydrocephalus among members of the American Society of Pediatric Neurosurgeons (ASPN).

METHODS

Surveys were sent to all 204 active ASPN members in September 2014. One hundred thirty responses were received, representing a 64% response rate. Respondents were asked 13 multiple-choice and free-response questions regarding 4 fundamental problems encountered in shunted-hydrocephalus management: shunt malfunction, chronic cerebrospinal fluid (CSF) overdrainage, chronic headaches, and slit ventricle syndrome (SVS).

RESULTS

Respondents agreed that shunt malfunction occurs most often as the result of ventricular catheter obstruction. Despite contrary evidence in the literature, most respondents (66%) also believed that choroid plexus is the tissue most often found in obstructed proximal catheters. However, free-text responses revealed that the respondents’ understanding of the underlying pathophysiology of shunt obstruction was highly variable and included growth, migration, or adherence of choroid plexus, CSF debris, catheter position, inflammatory processes, and CSF overdrainage. Most respondents considered chronic CSF overdrainage to be a rare complication of shunting in their practice and reported wide variation in treatment protocols. Moreover, despite a lack of evidence in the literature, most respondents attributed chronic headaches in shunt patients to medical reasons (for example, migraines, tension). Accordingly, most respondents managed headaches with reassurance and/or referral to pain clinics. Lastly, there were variable opinions on the etiology of slit ventricle syndrome (SVS), which included early shunting, chronic overdrainage, and/or loss of brain compliance. Beyond shunt revision, respondents reported divergent SVS treatment preferences.

CONCLUSIONS

The survey shows that there is wide variability in the understanding and management of shunt-dependent hydrocephalus and its complications. Such discrepancies appear to be derived partly from inconsistent familiarity with existing literature but especially from a paucity of high-quality publications.

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Sara Saleh, Kyle I. Swanson, and Taryn Bragg

Cervical spine injuries are the most common spine injuries in the pediatric population. The authors present the youngest known patient who underwent cervical spine fusion to repair birth trauma–induced cervical fracture dislocation, resulting in spondyloptosis and spinal cord injury. A 2-week-old boy was found to have spondyloptosis and spinal cord injury after concerns arose from reduced movement of the extremities. The patient’s birth was complicated by undiagnosed abdominal dystocia, which led to cervical distraction injury. At 15 days of age, the boy underwent successful C-5 corpectomy, with anterior C4–6 and posterior C2–7 arthrodesis, using an autologous rib graft for a C-5 fracture dislocation. MRI performed 2 weeks postoperatively revealed significant improvement in the alignment of the spinal canal. The patient was discharged from the hospital in a custom Minerva brace and underwent close follow-up in addition to occupational therapy and physical therapy. At the latest follow-up 4.5 years later, the patient was able to walk and ride a tricycle by himself. The authors describe the patient’s surgery and the challenges faced in achieving successful repair and cervical spine stabilization in such a young patient. The authors suggest that significant neurological recovery after spinal cord injury in infants is possible with appropriate, timely, and interdisciplinary management.

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Taryn McFadden Bragg and Edward A. M. Duckworth

Numerous nuanced approaches have been used to access posterior inferior cerebellar artery (PICA) aneurysms for microsurgical clipping. The authors report the case of a patient with a right vertebral artery (VA)–PICA aneurysm that was reached via a contralateral far-lateral approach. The wide-necked saccular/fusiform aneurysm arose from the lateral aspect of the right V4 segment just proximal to the PICA origin, anterior to the jugular tubercle at the level of the hypoglossal canal. Computed tomography angiograms demonstrated the size and configuration of the aneurysm, and 3D reconstructions revealed the tortuosity of the right VA, defining its location just left of the midline adjacent to the lower clivus.

A contralateral far-lateral approach to VA–PICA aneurysms should be considered when aneurysms cross the midline. Computed tomography angiography with volume rendering and interactive software capabilities can help identify the relationship of such an aneurysm to an individual's particular skull base osseous anatomy and is paramount in selecting the optimal microsurgical approach.

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Joyce Koueik, Brandon G. Rocque, Jordan Henry, Taryn Bragg, Jennifer Paul, and Bermans J. Iskandar

Continuous irrigation is an important adjunct for successful intraventricular endoscopy, particularly for complex cases. It allows better visualization by washing out blood and debris, improves navigation by expanding the ventricles, and assists with tissue dissection. A method of irrigation delivery using a centrifugal pump designed originally for cardiac surgery is presented.

The BioMedicus centrifugal pump has the desirable ability to deliver a continuous laminar flow of fluid that excludes air from the system. A series of modifications to the pump tubing was performed to adapt it to neuroendoscopy. Equipment testing determined flow and pressure responses at various settings and simulated clinical conditions. The pump was then studied clinically in 11 endoscopy cases and eventually used in 310 surgical cases.

Modifications of the pump tubing allowed for integration with different endoscopy systems. Constant flow rates were achieved with and without surgical instruments through the working ports. Optimal flow rates ranged between 30 and 100 ml/min depending on endoscope size. Intraoperative use was well tolerated with no permanent morbidity and showed consistent flow rates, minimal air accumulation, and seamless irrigation bag replacement during prolonged surgery. Although the pump is equipped with an internal safety mechanism to protect against pressure buildup when outflow obstructions occur, equipment testing revealed that flow cessation is not instantaneous enough to protect against sudden intracranial pressure elevation.

A commonly available cardiac pump system was modified to provide continuous irrigation for intraventricular endoscopy. The system alleviates the problems of inconsistent flow rates, air in the irrigation lines, and delays in changing irrigation bags, thereby optimizing patient safety and surgical efficiency. Safe use of the pump requires good ventricular outflow and, clearly, sound surgical judgment.

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Mark R. Kraemer, Joyce Koueik, Susan Rebsamen, David A. Hsu, M. Shahriar Salamat, Susan Luo, Sara Saleh, Taryn M. Bragg, and Bermans J. Iskandar

OBJECTIVE

Ventricular shunts have an unacceptably high failure rate, which approaches 50% of patients at 2 years. Most shunt failures are related to ventricular catheter obstruction. The literature suggests that obstructions are caused by in-growth of choroid plexus and/or reactive cellular aggregation. The authors report endoscopic evidence of overdrainage-related ventricular tissue protrusions (“ependymal bands”) that cause partial or complete obstruction of the ventricular catheter.

METHODS

A retrospective review was completed on patients undergoing shunt revision surgery between 2008 and 2015, identifying all cases in which the senior author reported endoscopic evidence of ependymal tissue in-growth into ventricular catheters. Detailed clinical, radiological, and surgical findings are described.

RESULTS

Fifty patients underwent 83 endoscopic shunt revision procedures that revealed in-growth of ventricular wall tissue into the catheter tip orifices (ependymal bands), producing partial, complete, or intermittent shunt obstructions. Endoscopic ventricular explorations revealed ependymal bands at various stages of development, which appear to form secondarily to siphoning. Ependymal bands are associated with small ventricles when the shunt is functional, but may dilate at the time of obstruction.

CONCLUSIONS

Ventricular wall protrusions are a significant cause of proximal shunt obstruction, and they appear to be caused by siphoning of surrounding tissue into the ventricular catheter orifices.

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Brandon G. Rocque, Bonita S. Agee, Eric M. Thompson, Mark Piedra, Lissa C. Baird, Nathan R. Selden, Stephanie Greene, Christopher P. Deibert, Todd C. Hankinson, Sean M. Lew, Bermans J. Iskandar, Taryn M. Bragg, David Frim, Gerald Grant, Nalin Gupta, Kurtis I. Auguste, Dimitrios C. Nikas, Michael Vassilyadi, Carrie R. Muh, Nicholas M. Wetjen, and Sandi K. Lam

OBJECTIVE

In children, the repair of skull defects arising from decompressive craniectomy presents a unique set of challenges. Single-center studies have identified different risk factors for the common complications of cranioplasty resorption and infection. The goal of the present study was to determine the risk factors for bone resorption and infection after pediatric cranioplasty.

METHODS

The authors conducted a multicenter retrospective case study that included all patients who underwent cranioplasty to correct a skull defect arising from a decompressive craniectomy at 13 centers between 2000 and 2011 and were less than 19 years old at the time of cranioplasty. Prior systematic review of the literature along with expert opinion guided the selection of variables to be collected. These included: indication for craniectomy; history of abusive head trauma; method of bone storage; method of bone fixation; use of drains; size of bone graft; presence of other implants, including ventriculoperitoneal (VP) shunt; presence of fluid collections; age at craniectomy; and time between craniectomy and cranioplasty.

RESULTS

A total of 359 patients met the inclusion criteria. The patients’ mean age was 8.4 years, and 51.5% were female. Thirty-eight cases (10.5%) were complicated by infection. In multivariate analysis, presence of a cranial implant (primarily VP shunt) (OR 2.41, 95% CI 1.17–4.98), presence of gastrostomy (OR 2.44, 95% CI 1.03–5.79), and ventilator dependence (OR 8.45, 95% CI 1.10–65.08) were significant risk factors for cranioplasty infection. No other variable was associated with infection.

Of the 240 patients who underwent a cranioplasty with bone graft, 21.7% showed bone resorption significant enough to warrant repeat surgical intervention. The most important predictor of cranioplasty bone resorption was age at the time of cranioplasty. For every month of increased age the risk of bone flap resorption decreased by 1% (OR 0.99, 95% CI 0.98–0.99, p < 0.001). Other risk factors for resorption in multivariate models were the use of external ventricular drains and lumbar shunts.

CONCLUSIONS

This is the largest study of pediatric cranioplasty outcomes performed to date. Analysis included variables found to be significant in previous retrospective reports. Presence of a cranial implant such as VP shunt is the most significant risk factor for cranioplasty infection, whereas younger age at cranioplasty is the dominant risk factor for bone resorption.