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Travis R. Ladner, Jacob K. Greenberg, Nicole Guerrero, Margaret A. Olsen, Chevis N. Shannon, Chester K. Yarbrough, Jay F. Piccirillo, Richard C. E. Anderson, Neil A. Feldstein, John C. Wellons III, Matthew D. Smyth, Tae Sung Park and David D. Limbrick Jr.

OBJECTIVE

Administrative billing data may facilitate large-scale assessments of treatment outcomes for pediatric Chiari malformation Type I (CM-I). Validated International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) code algorithms for identifying CM-I surgery are critical prerequisites for such studies but are currently only available for adults. The objective of this study was to validate two ICD-9-CM code algorithms using hospital billing data to identify pediatric patients undergoing CM-I decompression surgery.

METHODS

The authors retrospectively analyzed the validity of two ICD-9-CM code algorithms for identifying pediatric CM-I decompression surgery performed at 3 academic medical centers between 2001 and 2013. Algorithm 1 included any discharge diagnosis code of 348.4 (CM-I), as well as a procedure code of 01.24 (cranial decompression) or 03.09 (spinal decompression or laminectomy). Algorithm 2 restricted this group to the subset of patients with a primary discharge diagnosis of 348.4. The positive predictive value (PPV) and sensitivity of each algorithm were calculated.

RESULTS

Among 625 first-time admissions identified by Algorithm 1, the overall PPV for CM-I decompression was 92%. Among the 581 admissions identified by Algorithm 2, the PPV was 97%. The PPV for Algorithm 1 was lower in one center (84%) compared with the other centers (93%–94%), whereas the PPV of Algorithm 2 remained high (96%–98%) across all subgroups. The sensitivity of Algorithms 1 (91%) and 2 (89%) was very good and remained so across subgroups (82%–97%).

CONCLUSIONS

An ICD-9-CM algorithm requiring a primary diagnosis of CM-I has excellent PPV and very good sensitivity for identifying CM-I decompression surgery in pediatric patients. These results establish a basis for utilizing administrative billing data to assess pediatric CM-I treatment outcomes.

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Jacob K. Greenberg, Margaret A. Olsen, Chester K. Yarbrough, Travis R. Ladner, Chevis N. Shannon, Jay F. Piccirillo, Richard C. E. Anderson, John C. Wellons III, Matthew D. Smyth, Tae Sung Park and David D. Limbrick Jr.

OBJECTIVE

Chiari malformation Type I (CM-I) is a common and often debilitating pediatric neurological disease. However, efforts to guide preoperative counseling and improve outcomes research are impeded by reliance on small, single-center studies. Consequently, the objective of this study was to investigate CM-I surgical outcomes using population-level administrative billing data.

METHODS

The authors used Healthcare Cost and Utilization Project State Inpatient Databases (SID) to study pediatric patients undergoing surgical decompression for CM-I from 2004 to 2010 in California, Florida, and New York. They assessed the prevalence and influence of preoperative complex chronic conditions (CCC) among included patients. Outcomes included medical and surgical complications within 90 days of treatment. Multivariate logistic regression was used to identify risk factors for surgical complications.

RESULTS

A total of 936 pediatric CM-I surgeries were identified for the study period. Overall, 29.2% of patients were diagnosed with syringomyelia and 13.7% were diagnosed with scoliosis. Aside from syringomyelia and scoliosis, 30.3% of patients had at least 1 CCC, most commonly neuromuscular (15.2%) or congenital or genetic (8.4%) disease. Medical complications were uncommon, occurring in 2.6% of patients. By comparison, surgical complications were diagnosed in 12.7% of patients and typically included shunt-related complications (4.0%), meningitis (3.7%), and other neurosurgery-specific complications (7.4%). Major complications (e.g., stroke or myocardial infarction) occurred in 1.4% of patients. Among children with CCCs, only comorbid hydrocephalus was associated with a significantly increased risk of surgical complications (OR 4.5, 95% CI 2.5–8.1).

CONCLUSIONS

Approximately 1 in 8 pediatric CM-I patients experienced a surgical complication, whereas medical complications were rare. Although CCCs were common in pediatric CM-I patients, only hydrocephalus was independently associated with increased risk of surgical events. These results may inform patient counseling and guide future research efforts.

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R. Shane Tubbs, Matthew D. Smyth, John C. Wellons III and W. Jerry Oakes

Object. The literature contains scant data regarding variations in anatomy at the level of the foramen of Magendie in patients with Chiari I malformation and syringomyelia.

Methods. Based on their operative experience and hospital data, the authors detailed the incidence of arachnoid veils found in juxtaposition to the foramen of Magendie in patients with hindbrain herniation. Additionally, radiological studies were retrospectively reviewed in cases in which such an anomaly was noted intraoperatively.

Of 140 patients with Chiari I malformation who underwent decompressive surgery, an associated syrinx was demonstrated in 80 (57%). The foramen of Magendie was obstructed by an arachnoid veil in 10 (12.5%) of these patients; once the lesion was punctured, the cerebrospinal fluid drained freely from this median aperture. On retrospective review of imaging studies, none of these anomalous structures was evident. In all patients with an arachnoid veil and syringomyelia resolution of syringomyelia was revealed on postoperative imaging.

Conclusions. In the absence of a clear pathophysiology of syrinx production, the authors would recommend that patients with syringomyelia and Chiari I malformation undergo duraplasty so that, if present, these veils can be fenestrated.

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Matthew D. Smyth, Jason T. Banks, R. Shane Tubbs, John C. Wellons III and W. Jerry Oakes

Object. The authors performed a study to evaluate the efficacy of a regimen of scheduled minor analgesic medications in managing postoperative pain in children undergoing intracranial procedures.

Methods. Postoperative pain scores were analyzed in two groups of children who underwent decompressive surgery for Chiari malformation: Group A underwent a scheduled regimen of minor oral analgesic medications (standing doses of acetaminophen [10 mg/kg] and ibuprofen [10 mg/kg] alternating every 2 hours) and Group B received analgesic medication when requested.

Fifty children underwent a standard occipital craniectomy (25 in each group). The pain scores were significantly lower in Group A during most of the postoperative period. Length of stay (LOS) was shorter (2.2 compared with 2.8 days), and narcotic and antiemetic requirements were also lower in Group A patients than in Group B patients. Patients with spinal cord syringes exhibited a similar postoperative status to those without, and similar improvements in pain scores with scheduled minor analgesic medications were also evident.

Conclusions. A regimen of minor analgesic therapy, given in alternating doses every 2 hours immediately after craniotomy and throughout hospitalization, significantly reduced postoperative pain scores and LOS in children in whom suboccipital craniotomy was performed. Narcotic and antiemetic requirements were also decreased in association with this regimen. Application of this postoperative analgesia protocol may benefit children and adults in whom various similar neurosurgical procedures are required.

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R. Shane Tubbs, Matthew D. Smyth, John C. Wellons III, Jeffrey P. Blount, Paul A. Grabb and W. Jerry Oakes

Object. To the best of the authors' knowledge, no quantitative analysis of the atlantoaxial interlaminar distance in flexion (ILD) in children exists in the medical literature. In this study they sought to determine the age-matched relationship between the posterior elements of the atlas and axis in children in cervical spine flexion, to be used as an adjunct to the atlantodental interval in common clinical use.

Methods. Lateral radiographs of the cervical spine in full flexion were analyzed in 74 children. The atlantoaxial ILD was defined as the distance between a midpoint of the anterior cortices of the atlantal and axial posterior arches.

The mean ILD for the entire group was 19 mm (range 8–30 mm). No significant difference was seen between male and female patients (p = 0.084). When stratified by age, the mean ILD was 12.3 ± 3 mm (15 cases) in children age 3 years or younger and 20.5 ± 4.7 mm (59 cases) in children age older than 3 years. Further stratification of the groups yielded a mean ILD of 10.4 ± 1.4 [eight cases]) in children age 1 to 2 years, and 14.4 ± 4.7 mm (seven cases) in children age 3 years. In children older than 3 years of age the mean ILD was consistently approximately 20 ± 5 mm regardless of age.

Conclusions. Rapid, safe, and accurate diagnosis of the cervical spine is essential in critical care. Knowledge of the distance between the posterior elements of the atlas and axis in flexion should enhance the clinicians' (those who clear cervical spines) ability to diagnose accurately atlantoaxial instability on lateral radiographs obtained in flexion.