Depressed skull fractures in neonates involve the inward buckling of the skull bones to resemble a cup shape or “ping-pong ball” shape. In the past, several methods have been used to treat these fractures for cosmetic effect, including the use of vacuum extraction. The aim of this study was to demonstrate the use of a novel treatment method for these skull fractures. All patients presented to Hershey Medical Center between May 2007 and March 2010. All underwent treatment of their ping-pong skull fracture using a novel technique of percutaneous screw elevation with self-tapping microscrews (4 or 5 mm) typically used in neurosurgery for cranial plating. Four patients were treated, all between the ages of 2 days and 4 months. Two of the patients were treated under general anesthesia and 2 with local anesthesia only. All patients had good cosmetic results and experienced no adverse events. Percutaneous screw elevation of the ping-pong type, simple depressed skull fracture in neonates is a feasible method of treatment with a low complication rate associated with the procedure and no risk from anesthesia.
Omar Zalatimo, Moksha Ranasinghe, Mark Dias, and Mark Iantosca
Omar Zalatimo, Moksha Ranasinghe, Robert E. Harbaugh, and Mark Iantosca
Accuracy in documenting clinical care is becoming increasingly important; it can greatly affect the success of a neurosurgery department. As patient outcomes are being more rigorously monitored, inaccurate documentation of patient variables may present a distorted picture of the severity of illness (SOI) of the patients and adversely affect observed versus expected mortality ratios and hospital reimbursement. Just as accuracy of coding is important for generating professional revenue, accuracy of documentation is important for generating technical revenue. The aim of this study was to evaluate the impact of an educational intervention on the documentation of patient comorbidities as well as its impact on quality metrics and hospital margin per case.
All patients who were discharged from the Department of Neurosurgery of the Penn State Milton S. Hershey Medical Center between November 2009 and June 2012 were evaluated. An educational intervention to improve documentation was implemented and evaluated, and the next 16 months, starting in March 2011, were used for comparison with the previous 16 months in regard to All Patient Refined Diagnosis-Related Group (APR-DRG) weight, SOI, risk of mortality (ROM), case mix index (CMI), and margin per discharge.
The APR-DRG weight was corrected from 2.123 ± 0.140 to 2.514 ± 0.224; the SOI was corrected from 1.8638 ± 0.0855 to 2.154 ± 0.130; the ROM was corrected from 1.5106 ± 0.0884 to 1.801 ± 0.117; and the CMI was corrected from 2.429 ± 0.153 to 2.825 ± 0.232, and as a result the average margin per discharge improved by 42.2%. The mean values are expressed ± SD throughout.
A simple educational intervention can have a significant impact on documentation accuracy, quality metrics, and revenue generation in an academic neurosurgery department.
Mark S. Dias, Jeffrey S. Sussman, Susan Durham, and Mark R. Iantosca
Research suggests that there may be a growing disparity between the supply of and demand for both pediatric specialists and neurosurgeons. Whether pediatric neurosurgeons are facing such a disparity is disputable, but interest in pediatric neurosurgery (PNS) has waxed and waned as evidenced by the number of applicants for PNS fellowships. The authors undertook a survey to analyze current neurosurgical residents' perceptions of both benefits and deterrents to a pediatric neurosurgical career.
All residents and PNS fellows in the United States and Canada during the academic year 2008–2009 were invited to complete a Web-based survey that assessed 1) demographic and educational information about residents and their residency training, particularly as it related to training in PNS; 2) residents' exposure to mentoring opportunities from pediatric neurosurgical faculty and their plans for the future; and 3) residents' perceptions about how likely 40 various factors were to influence their decision about whether to pursue a PNS career.
Four hundred ninety-six responses were obtained: 89% of the respondents were male, 63% were married, 75% were in at least their 3rd year of postgraduate training, 61% trained in a children's hospital and 29% in a children's “hospital within a hospital,” and 72% were in programs having one or more dedicated PNS faculty members. The residencies of 56% of respondents offered 6–11 months of PNS training and nearly three-quarters of respondents had completed 2 months of PNS training. During medical school, 92% had been exposed to neurosurgery and 45% to PNS during a clinical rotation, but only 7% identified a PNS mentor. Nearly half (43%) are considering a PNS career, and of these, 61% are definitely or probably considering post-residency fellowship. On the other hand, 68% would prefer an enfolded fellowship during residency. Perceived strengths of PNS included working with children, developing lasting relationships, wider variety of operations, fast healing and lack of comorbidities, and altruism. Perceived significant deterrents included shunts, lower reimbursement, cross-coverage issues, higher malpractice premiums and greater legal exposure, and working with parents and pediatric health professionals. The intrinsic nature of PNS was listed as the most significant deterrent (46%) followed by financial concerns (25%), additional training (12%), longer work hours (12%), and medicolegal issues (4%). The majority felt that fellowship training and PNS certification should be recommended for surgeons treating of all but traumatic brain injuries and Chiari I malformations and performing simple shunt-related procedures, although they felt that these credentials should be required only for treating complex craniosynostosis.
The nature of PNS is the most significant barrier to attracting residents, although reimbursement, cross-coverage, and legal issues are also important to residents. The authors provide several recommendations that might enhance resident perceptions of PNS and attract trainees to the specialty.
Mark S. Dias, Michele L. Shaffer, Mark R. Iantosca, and Kenneth L. Hill Jr.
The thresholds for shunting CSF in children with asymptomatic hydrocephalus are unclear; there are neither guidelines nor sufficient research to determine what degree of hydrocephalus should be treated. The authors hypothesize that 1) pediatric neurosurgeons currently have high thresholds for recommending treatment for these children, but 2) there is significant variability among these treatment thresholds.
The authors surveyed attendees of the Joint Pediatric Neurosurgery Section meeting in Spokane, Washington, in December 2008, regarding their treatment thresholds for 22 clinical scenarios. Each participant was provided an illustration of 5 imaging studies (3 slices each) showing progressively larger ventricles. For each scenario, respondents were asked to indicate the minimum ventricular size they would treat, if any. Responses were quantified from 1 to 6 from smaller to larger, with 6 being no treatment, and a mean theoretical treated ventricular size (MTTVS) was calculated for each scenario.
Respondents were relatively conservative in recommending treatment, with MTTVSs of 3.7–6.0; in 13 scenarios, the MTTVS was greater than 5.0 (larger than the largest presented ventricular size). For scenarios in which a mean frontooccipital ratio could be calculated, the value ranged from 0.55 to 0.67 (moderate to severe hydrocephalus). Although there were clear majority responses for each scenario, there was also significant variability. There were no patterns of association with the respondent's age, training, board certification, or type or location of practice.
This study demonstrates that pediatric neurosurgeons' thresholds for treating asymptomatic children with hydrocephalus are generally high, but there is also significant variability.
Christina Huang, Elias Rizk, Mark Iantosca, Andrea L. Zaenglein, Klaus F. Helm, Arabinda K. Choudhary, and Mark S. Dias
An in utero female was found to have a small hemorrhage at the foramen of Monro, hydrocephalus, and what was originally interpreted as a Dandy-Walker variant. At birth she had macrocephaly and numerous cutaneous, multifocal, red-pink blanchable macules. Postnatal MRI demonstrated a hemorrhagic soft-tissue mass involving the upper brainstem, thalamus, and basal ganglia most consistent with in utero complex multifocal intracranial hemorrhage. The skin lesions were thought to be consistent with multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT). The size and location of the hemorrhage precluded operative intervention, although the hydrocephalus was treated with a ventricular shunt. The child continues to have severe developmental delays. Multifocal lymphangioendotheliomatosis with thrombocytopenia is a multifocal vascular disorder most commonly involving the skin and gastrointestinal tract. Intracranial hemorrhages are rare in this context. This case is the third reported instance of MLT with associated intracranial hemorrhage and the only case described in the neurosurgical literature. The authors review the presenting features and pathophysiology of this condition.
Megha Madhukar, Vinod G. Maller, Arabinda K. Choudhary, Mark R. Iantosca, Charles S. Specht, and Mark S. Dias
Primary intramedullary spinal cord germinoma (PISCG) is an exceedingly rare diagnosis, with fewer than 30 cases reported in the literature. It is even less common in the pediatric population. Usually, initial imaging at patient presentation reveals a mass. The authors describe the unique case of a child whose initial imaging showed only focal spinal cord atrophy, which was the earliest sign of a slowly growing intramedullary lesion that was eventually proven via biopsy to represent a PISCG. The authors outline this child's diagnostically challenging presentation, review the events leading up to a diagnosis, briefly discuss PISCG, and summarize their recommendations for other physicians who may encounter a similar case. They assert that PISCG should be considered as a rare entity in the differential diagnosis of progressive spinal cord dysfunction even in the absence of an MRI abnormality of an intrinsic spinal cord mass, especially if there is unexplained focal atrophy of the cord.
Michael J. Gigliotti, Christine Mau, Charles S. Specht, Cynthia Lawson, Sarah McNutt, Shreela Natarajan, Elias B. Rizk, and Mark Iantosca
The WHO Classification of Tumours of the Central Nervous System (2016) classifies nonmeningothelial malignant spindle cell tumors involving the extraaxial tissues of the posterior fossa as melanocytic tumors and malignant mesenchymal tumors (sarcomas). The objective of this study was to conduct a review of the literature pertaining to the management strategies of posterior fossa malignant spindle cell tumors in the pediatric population.
The authors performed an institutional search of their pathology database for patients younger than 18 years of age who presented with posterior fossa malignant spindle cell tumors. A literature review was also performed using the PubMed database, with “posterior fossa” or “spindle cell tumors” or “Ewing sarcoma” or “high-grade” or “spindle cell sarcoma” or “leptomeningeal melanocytoma” as keywords. The database search was restricted to pediatric patients (age ≤ 18 years). Parameters reported from the literature review included patient age, tumor location, presenting symptoms, treatment modalities (resection, chemotherapy, and/or radiotherapy), leptomeningeal spread at or after the time of treatment, and follow-up length and resulting outcome.
The authors report 3 rare cases of posterior fossa malignant spindle cell tumors, including Ewing sarcoma in a 13-year-old male; high-grade spindle cell sarcoma, not otherwise specified in a 10-year-old male; and primary leptomeningeal melanocytoma in a 16-year-old female. All 3 patients underwent resection and radiotherapy and either chemotherapy or targeted immunotherapy. At the last follow-up, all patients were alive with either resolution or stable disease.
A review of these 3 cases and the existing literature support managing patients with intracranial malignant spindle cell tumors with multimodal therapy that can include a combination of resection, radiotherapy, and chemotherapy or immunotherapy to prolong progression-free and overall survival.
Alexander T. Yahanda, P. David Adelson, S. Hassan A. Akbari, Gregory W. Albert, Philipp R. Aldana, Tord D. Alden, Richard C. E. Anderson, David F. Bauer, Tammy Bethel-Anderson, Douglas L. Brockmeyer, Joshua J. Chern, Daniel E. Couture, David J. Daniels, Brian J. Dlouhy, Susan R. Durham, Richard G. Ellenbogen, Ramin Eskandari, Timothy M. George, Gerald A. Grant, Patrick C. Graupman, Stephanie Greene, Jeffrey P. Greenfield, Naina L. Gross, Daniel J. Guillaume, Todd C. Hankinson, Gregory G. Heuer, Mark Iantosca, Bermans J. Iskandar, Eric M. Jackson, James M. Johnston, Robert F. Keating, Mark D. Krieger, Jeffrey R. Leonard, Cormac O. Maher, Francesco T. Mangano, J. Gordon McComb, Sean D. McEvoy, Thanda Meehan, Arnold H. Menezes, Brent R. O’Neill, Greg Olavarria, John Ragheb, Nathan R. Selden, Manish N. Shah, Chevis N. Shannon, Joshua S. Shimony, Matthew D. Smyth, Scellig S. D. Stone, Jennifer M. Strahle, James C. Torner, Gerald F. Tuite, Scott D. Wait, John C. Wellons III, William E. Whitehead, Tae Sung Park, and David D. Limbrick Jr.
Posterior fossa decompression with duraplasty (PFDD) is commonly performed for Chiari I malformation (CM-I) with syringomyelia (SM). However, complication rates associated with various dural graft types are not well established. The objective of this study was to elucidate complication rates within 6 months of surgery among autograft and commonly used nonautologous grafts for pediatric patients who underwent PFDD for CM-I/SM.
The Park-Reeves Syringomyelia Research Consortium database was queried for pediatric patients who had undergone PFDD for CM-I with SM. All patients had tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and ≥ 6 months of postoperative follow-up after PFDD. Complications (e.g., pseudomeningocele, CSF leak, meningitis, and hydrocephalus) and postoperative changes in syrinx size, headaches, and neck pain were compared for autograft versus nonautologous graft.
A total of 781 PFDD cases were analyzed (359 autograft, 422 nonautologous graft). Nonautologous grafts included bovine pericardium (n = 63), bovine collagen (n = 225), synthetic (n = 99), and human cadaveric allograft (n = 35). Autograft (103/359, 28.7%) had a similar overall complication rate compared to nonautologous graft (143/422, 33.9%) (p = 0.12). However, nonautologous graft was associated with significantly higher rates of pseudomeningocele (p = 0.04) and meningitis (p < 0.001). The higher rate of meningitis was influenced particularly by the higher rate of chemical meningitis (p = 0.002) versus infectious meningitis (p = 0.132). Among 4 types of nonautologous grafts, there were differences in complication rates (p = 0.02), including chemical meningitis (p = 0.01) and postoperative nausea/vomiting (p = 0.03). Allograft demonstrated the lowest complication rates overall (14.3%) and yielded significantly fewer complications compared to bovine collagen (p = 0.02) and synthetic (p = 0.003) grafts. Synthetic graft yielded higher complication rates than autograft (p = 0.01). Autograft and nonautologous graft resulted in equal improvements in syrinx size (p < 0.0001). No differences were found for postoperative changes in headaches or neck pain.
In the largest multicenter cohort to date, complication rates for dural autograft and nonautologous graft are similar after PFDD for CM-I/SM, although nonautologous graft results in higher rates of pseudomeningocele and meningitis. Rates of meningitis differ among nonautologous graft types. Autograft and nonautologous graft are equivalent for reducing syrinx size, headaches, and neck pain.
Jennifer M. Strahle, Rukayat Taiwo, Christine Averill, James Torner, Chevis N. Shannon, Christopher M. Bonfield, Gerald F. Tuite, Tammy Bethel-Anderson, Jerrel Rutlin, Douglas L. Brockmeyer, John C. Wellons III, Jeffrey R. Leonard, Francesco T. Mangano, James M. Johnston, Manish N. Shah, Bermans J. Iskandar, Elizabeth C. Tyler-Kabara, David J. Daniels, Eric M. Jackson, Gerald A. Grant, Daniel E. Couture, P. David Adelson, Tord D. Alden, Philipp R. Aldana, Richard C. E. Anderson, Nathan R. Selden, Lissa C. Baird, Karin Bierbrauer, Joshua J. Chern, William E. Whitehead, Richard G. Ellenbogen, Herbert E. Fuchs, Daniel J. Guillaume, Todd C. Hankinson, Mark R. Iantosca, W. Jerry Oakes, Robert F. Keating, Nickalus R. Khan, Michael S. Muhlbauer, J. Gordon McComb, Arnold H. Menezes, John Ragheb, Jodi L. Smith, Cormac O. Maher, Stephanie Greene, Michael Kelly, Brent R. O’Neill, Mark D. Krieger, Mandeep Tamber, Susan R. Durham, Greg Olavarria, Scellig S. D. Stone, Bruce A. Kaufman, Gregory G. Heuer, David F. Bauer, Gregory Albert, Jeffrey P. Greenfield, Scott D. Wait, Mark D. Van Poppel, Ramin Eskandari, Timothy Mapstone, Joshua S. Shimony, Ralph G. Dacey Jr., Matthew D. Smyth, Tae Sung Park, and David D. Limbrick Jr.
Scoliosis is frequently a presenting sign of Chiari malformation type I (CM-I) with syrinx. The authors’ goal was to define scoliosis in this population and describe how radiological characteristics of CM-I and syrinx relate to the presence and severity of scoliosis.
A large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°).
Based on available imaging of patients with CM-I and syrinx, 260 of 825 patients (31%) had a clear diagnosis of scoliosis based on radiographs or coronal MRI. Forty-nine patients (5.9%) did not have scoliosis, and in 516 (63%) patients, a clear determination of the presence or absence of scoliosis could not be made. Comparison of patients with and those without a definite scoliosis diagnosis indicated that scoliosis was associated with wider syrinxes (8.7 vs 6.3 mm, OR 1.25, p < 0.001), longer syrinxes (10.3 vs 6.2 levels, OR 1.18, p < 0.001), syrinxes with their rostral extent located in the cervical spine (94% vs 80%, OR 3.91, p = 0.001), and holocord syrinxes (50% vs 16%, OR 5.61, p < 0.001). Multivariable regression analysis revealed syrinx length and the presence of holocord syrinx to be independent predictors of scoliosis in this patient cohort. Scoliosis was not associated with sex, age at CM-I diagnosis, tonsil position, pB–C2 distance (measured perpendicular distance from the ventral dura to a line drawn from the basion to the posterior-inferior aspect of C2), clivoaxial angle, or frontal-occipital horn ratio. Average curve magnitude was 29.9°, and 37.7% of patients had a left thoracic curve. Older age at CM-I or syrinx diagnosis (p < 0.0001) was associated with greater curve magnitude whereas there was no association between syrinx dimensions and curve magnitude.
Syrinx characteristics, but not tonsil position, were related to the presence of scoliosis in patients with CM-I, and there was an independent association of syrinx length and holocord syrinx with scoliosis. Further study is needed to evaluate the nature of the relationship between syrinx and scoliosis in patients with CM-I.
Andrew T. Hale, P. David Adelson, Gregory W. Albert, Philipp R. Aldana, Tord D. Alden, Richard C. E. Anderson, David F. Bauer, Christopher M. Bonfield, Douglas L. Brockmeyer, Joshua J. Chern, Daniel E. Couture, David J. Daniels, Susan R. Durham, Richard G. Ellenbogen, Ramin Eskandari, Timothy M. George, Gerald A. Grant, Patrick C. Graupman, Stephanie Greene, Jeffrey P. Greenfield, Naina L. Gross, Daniel J. Guillaume, Gregory G. Heuer, Mark Iantosca, Bermans J. Iskandar, Eric M. Jackson, James M. Johnston, Robert F. Keating, Jeffrey R. Leonard, Cormac O. Maher, Francesco T. Mangano, J. Gordon McComb, Thanda Meehan, Arnold H. Menezes, Brent O’Neill, Greg Olavarria, Tae Sung Park, John Ragheb, Nathan R. Selden, Manish N. Shah, Matthew D. Smyth, Scellig S. D. Stone, Jennifer M. Strahle, Scott D. Wait, John C. Wellons, William E. Whitehead, Chevis N. Shannon, David D. Limbrick Jr., and for the Park-Reeves Syringomyelia Research Consortium Investigators
Factors associated with syrinx size in pediatric patients undergoing posterior fossa decompression (PFD) or PFD with duraplasty (PFDD) for Chiari malformation type I (CM-I) with syringomyelia (SM; CM-I+SM) are not well established.
Using the Park-Reeves Syringomyelia Research Consortium registry, the authors analyzed variables associated with syrinx radiological outcomes in patients (< 20 years old at the time of surgery) with CM-I+SM undergoing PFD or PFDD. Syrinx resolution was defined as an anteroposterior (AP) diameter of ≤ 2 mm or ≤ 3 mm or a reduction in AP diameter of ≥ 50%. Syrinx regression or progression was defined using 1) change in syrinx AP diameter (≥ 1 mm), or 2) change in syrinx length (craniocaudal, ≥ 1 vertebral level). Syrinx stability was defined as a < 1-mm change in syrinx AP diameter and no change in syrinx length.
The authors identified 380 patients with CM-I+SM who underwent PFD or PFDD. Cox proportional hazards modeling revealed younger age at surgery and PFDD as being independently associated with syrinx resolution, defined as a ≤ 2-mm or ≤ 3-mm AP diameter or ≥ 50% reduction in AP diameter. Radiological syrinx resolution was associated with improvement in headache (p < 0.005) and neck pain (p < 0.011) after PFD or PFDD. Next, PFDD (p = 0.005), scoliosis (p = 0.007), and syrinx location across multiple spinal segments (p = 0.001) were associated with syrinx diameter regression, whereas increased preoperative frontal-occipital horn ratio (FOHR; p = 0.007) and syrinx location spanning multiple spinal segments (p = 0.04) were associated with syrinx length regression. Scoliosis (HR 0.38 [95% CI 0.16–0.91], p = 0.03) and smaller syrinx diameter (5.82 ± 3.38 vs 7.86 ± 3.05 mm; HR 0.60 [95% CI 0.34–1.03], p = 0.002) were associated with syrinx diameter stability, whereas shorter preoperative syrinx length (5.75 ± 4.01 vs 9.65 ± 4.31 levels; HR 0.21 [95% CI 0.12–0.38], p = 0.0001) and smaller pB-C2 distance (6.86 ± 1.27 vs 7.18 ± 1.38 mm; HR 1.44 [95% CI 1.02–2.05], p = 0.04) were associated with syrinx length stability. Finally, younger age at surgery (8.19 ± 5.02 vs 10.29 ± 4.25 years; HR 1.89 [95% CI 1.31–3.04], p = 0.01) was associated with syrinx diameter progression, whereas increased postoperative syrinx diameter (6.73 ± 3.64 vs 3.97 ± 3.07 mm; HR 3.10 [95% CI 1.67–5.76], p = 0.003), was associated with syrinx length progression. PFD versus PFDD was not associated with syrinx progression or reoperation rate.
These data suggest that PFDD and age are independently associated with radiological syrinx improvement, although forthcoming results from the PFDD versus PFD randomized controlled trial (NCT02669836, clinicaltrials.gov) will best answer this question.