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D. Andrew Wilkinson, Kyle Johnson, Hugh J. L. Garton, Karin M. Muraszko and Cormac O. Maher

OBJECTIVE

The goal of this analysis was to define temporal and geographic trends in the surgical treatment of Chiari malformation Type I (CM-I) in a large, privately insured health care network.

METHODS

The authors examined de-identified insurance claims data from a large, privately insured health care network of over 58 million beneficiaries throughout the United States for the period between 2001 and 2014 for all patients undergoing surgical treatment of CM-I. Using a combination of International Classification of Diseases (ICD) diagnosis codes and Current Procedural Terminology (CPT) codes, the authors identified CM-I and associated diagnoses and procedures over a 14-year period, highlighting temporal and geographic trends in the performance of CM-I decompression (CMD) surgery as well as commonly associated procedures.

RESULTS

There were 2434 surgical procedures performed for CMD among the beneficiaries during the 14-year interval; 34% were performed in patients younger than 20 years of age. The rate of CMD increased 51% from the first half to the second half of the study period among younger patients (p < 0.001) and increased 28% among adult patients between 20 and 65 years of age (p < 0.001). A large sex difference was noted among adult patients; 78% of adult patients undergoing CMD were female compared with only 53% of the children. Pediatric patients undergoing CMD were more likely to be white with a higher household net worth. Regional variability was identified among rates of CMD as well. The average annual rate of surgery ranged from 0.8 surgeries per 100,000 insured person-years in the Pacific census division to 2.0 surgeries per 100,000 insured person-years in the East South Central census division.

CONCLUSIONS

Analysis of a large nationwide health care network showed recently increasing rates of CMD in children and adults over the past 14 years.

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Joseph R. Linzey, James F. Burke, Jeffrey L. Nadel, Craig A. Williamson, Luis E. Savastano, D. Andrew Wilkinson and Aditya S. Pandey

OBJECTIVE

It is unknown what proportion of patients who undergo emergent neurosurgical procedures initiate comfort care (CC) measures shortly after the operation. The purpose of the present study was to analyze the proportion and predictive factors of patients who initiated CC measures within the same hospital admission after undergoing emergent neurosurgery.

METHODS

This retrospective cohort study included all adult patients who underwent emergent neurosurgical and endovascular procedures at a single center between 2009 and 2014. Primary and secondary outcomes were initiation of CC measures during the initial hospitalization and determination of predictive factors, respectively.

RESULTS

Of the 1295 operations, comfort care was initiated in 111 (8.6%) during the initial admission. On average, CC was initiated 9.3 ± 10.0 days postoperatively. One-third of the patients switched to CC within 3 days. In multivariate analysis, patients > 70 years of age were significantly more likely to undergo CC than those < 50 years (70–79 years, p = 0.004; > 80 years, p = 0.0001). Two-thirds of CC patients had been admitted with a cerebrovascular pathology (p < 0.001). Admission diagnosis of cerebrovascular pathology was a significant predictor of initiating CC (p < 0.0001). A high Hunt and Hess grade of IV or V in patients with subarachnoid hemorrhage was significantly associated with initiation of CC compared to a low grade (27.1% vs 2.9%, p < 0.001). Surgery starting between 15:01 and 06:59 hours had a 1.70 times greater odds of initiating CC compared to surgery between 07:00 and 15:00.

CONCLUSIONS

Initiation of CC after emergent neurosurgical and endovascular procedures is relatively common, particularly when an elderly patient presents with a cerebrovascular pathology after typical operating hours.

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Jeffrey L. Nadel, D. Andrew Wilkinson, Hugh J. L. Garton, Karin M. Muraszko and Cormac O. Maher

OBJECTIVE

The goal of this study was to determine the rates of screening and surgery for foramen magnum stenosis in children with achondroplasia in a large, privately insured healthcare network.

METHODS

Rates of screening and surgery for foramen magnum stenosis in children with achondroplasia were determined using de-identified insurance claims data from a large, privately insured healthcare network of over 58 million beneficiaries across the United States between 2001 and 2014. Cases of achondroplasia and screening and surgery claims were identified using a combination of International Classification of Diseases diagnosis codes and Current Procedural Terminology codes. American Academy of Pediatrics (AAP) practice guidelines were used to determine screening trends.

RESULTS

The search yielded 3577 children age 19 years or younger with achondroplasia. Of them, 236 met criteria for inclusion in the screening analysis. Among the screening cohort, 41.9% received some form of screening for foramen magnum stenosis, whereas 13.9% of patients were fully and appropriately screened according to the 2005 guidelines from the AAP. The screening rate significantly increased after the issuance of the AAP guidelines. Among all children in the cohort, 25 underwent cervicomedullary decompression for foramen magnum stenosis. The incidence rate of undergoing cervicomedullary decompression was highest in infancy (28 per 1000 patient-years) and decreased with age (5 per 1000 patient-years for all other ages combined).

CONCLUSIONS

Children with achondroplasia continue to be underscreened for foramen magnum stenosis, although screening rates have improved since the release of the 2005 AAP surveillance guidelines. The incidence of surgery was highest in infants and decreased with age.

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Sravanthi Koduri, D. Andrew Wilkinson, Julius M. Griauzde, Joseph J. Gemmete and Cormac O. Maher

Moyamoya syndrome predisposes patients to ischemic or hemorrhagic stroke due to progressive narrowing of intracranial vessels with subsequent small-vessel collateralization. Dural arteriovenous fistulae (DAVFs) are most commonly noted after venous sinus or cortical vein thrombosis and are believed to be primarily due to venous hypertension and elevated sinus pressures, although there is no known association with moyamoya syndrome, or with surgical treatment for moyamoya disease (MMD). The authors present the case of a 14-year-old girl with Down syndrome treated using pial synangiosis for MMD who subsequently was noted to have bilateral DAVFs. This case provides a new perspective on the origins and underlying pathophysiology of both moyamoya syndrome and DAVFs, and also serves to highlight the importance of monitoring the moyamoya population closely for de novo cerebrovascular changes after revascularization procedures.

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Osama N. Kashlan, D. Andrew Wilkinson, Hal Morgenstern, Siri S. Khalsa and Cormac O. Maher

OBJECTIVE

Thickened or fatty filum terminale is an occult lesion that can cause tethered cord syndrome requiring surgical untethering. This study’s objectives were to estimate the incidence of tethered fibrofatty filum terminale (TFFT) in a large insured pediatric population, identify predictors of surgery among those TFFT patients, and assess a diagnostic algorithm.

METHODS

TFFT was defined according to the ICD-9-CM code for cord tethering (742.59), after excluding codes for diastematomyelia, lipomyelomeningocele, terminal myelocystocele, meningocele, and myelomeningocele. Utilizing the Optum Insight database for 2001–2014, the authors identified pediatric patients (< 21 years) in the US who were diagnosed with a tethered cord and estimated the TFFT incidence rates in that source population and the surgical untethering probability among TFFT patients over the 14-year period. Logistic regression was used to estimate the effects (adjusted OR and 95% CI) of age at diagnosis, sex, Charlson Comorbidity Index (CCI) score, diagnosis of Chiari malformation type I, diagnosis of syrinx, and the probability of surgery by US census region. Lastly, to evaluate their algorithm for identifying TFFT from ICD-9 codes, the authors estimated its positive predictive value (PPV) among 50 children who were diagnosed at their institution and met the ICD-9-CM criteria.

RESULTS

There were 3218 diagnoses of TFFT, with 482 of these pediatric patients undergoing tethered cord release during the study period. The estimated incidence rate was 12.0 per 100,000/year (95% CI 11.6–12.4 per 100,000/year). The incidence rate was slightly higher in females than in males (12.7 vs 11.4 per 100,000/year). The probability of surgery in the total pediatric TFFT population was 15.0% (95% CI 13.8%–16.2%) and was greater in children with a syrinx (OR 2.2, 95% CI 1.6–3.0), children 7–11 years of age at diagnosis versus < 1 year (OR 1.5, 95% CI 1.1–2.0), CCI score ≥ 3 versus 0 (OR 2.3, 95% CI 1.4–3.8), and residents of the Western vs Northeastern US (OR 2.3, 95% CI 1.6–3.5). In the authors’ own institution’s database, the PPV of TFFT was 35/50 (70.0%, 95% CI 57.3%–82.7%) for identifying tethered cord due to fibrofatty filum terminale among childhood positives.

CONCLUSIONS

Patients with comorbidities or an associated syrinx showed a higher risk of untethering procedures for TFFT. Also, surgery was appreciably more frequent in the Western US. These findings signify the need for a collaborative prospective cohort study of long-term outcomes for TFFT patients with and without surgery to determine which patients should have surgery.