scholarly journals Chiari malformation Type I surgery in pediatric patients. Part 1: validation of an ICD-9-CM code search algorithm

2016 ◽  
Vol 17 (5) ◽  
pp. 519-524 ◽  
Author(s):  
Travis R. Ladner ◽  
Jacob K. Greenberg ◽  
Nicole Guerrero ◽  
Margaret A. Olsen ◽  
Chevis N. Shannon ◽  
...  

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.

Author(s):  
Giorge Voutsas ◽  
Aaron St-Laurent ◽  
Crystal Hutchinson ◽  
Reshma Amin ◽  
James Drake ◽  
...  

OBJECTIVE Chiari malformation type I (CM-I) involves the herniation of the cerebellar tonsils through the foramen magnum. CM-I is associated with both obstructive sleep apnea (OSA) and central sleep apnea (CSA) in children. The primary management of symptomatic CM-I remains surgical decompression. There is, however, a paucity of data evaluating the efficacy of decompression surgery on outcomes related to sleep-disordered breathing (SDB). The objective of this study was to evaluate SDB outcomes, specifically the need for respiratory support following decompression in pediatric patients with CM-I. METHODS This was a retrospective chart review of all children diagnosed with CM-I when younger than 18 years of age who had polysomnography (PSG) studies pre- and postsurgery, between January 2008 and October 2018 at the Hospital for Sick Children in Toronto. Patient demographics, symptoms, PSG data, ongoing respiratory support, and surgical notes were recorded. Differences in PSG studies obtained pre- and postsurgery were compared using the Wilcoxon test for paired samples. RESULTS A total of 15 children with 15 interventions met inclusion criteria with pre- and postsurgery PSG studies and were considered for statistical analysis. Of the 15 subjects included for analysis, preoperative OSA was present in 2 (13.3%), CSA in 5 (33.3%), mixed SDB (both OSA and CSA) in 4 (26.7%), and no significant SDB in 4 (26.7%). Postoperatively, OSA was present in 3 (20.0%), CSA in 4 (26.7%), mixed SDB in 0 (0%), and no significant SDB in 8 (53.3%). The presence of severe OSA decreased from 4/15 (26.7%) to 2/15 (13.3%) postoperatively, and severe CSA decreased from 5/15 (33.3%) to 2/15 (13.3%) postoperatively. Following decompression surgery, 7/15 subjects (46.7%) required positive airway pressure for management of their SDB. Overall, significant improvements were observed in a number of respiratory parameters following decompression including the following: the total apnea-hypopnea index (AHI) (17.5 ± 48.2 vs 6.1 ± 32.7 events/hour; p = 0.001), obstructive AHI (2.1 ± 16.1 vs 1.0 ± 6.6 events/hour; p = 0.005), central AHI (6.3 ± 48.9 vs 2.7 ± 33.0 events/hour; p = 0.005), and the desaturation index (16.7 ± 49.6 vs 3.8 ± 25.3; p = 0.001). CONCLUSIONS Although decompression surgery led to a significant reduction in obstructive and central events, many children continued to have persistent SDB and required additional positive airway pressure therapy. This information is important and relevant for anticipatory guidance around decompression surgery and the necessity for respiratory support for the management of SDB in pediatric patients with CM-I.


2019 ◽  
Vol 10 ◽  
pp. 242 ◽  
Author(s):  
Jan De Vlieger ◽  
Joost Dejaegher ◽  
Frank Van Calenbergh

Background: We studied patient-reported outcome among patients who underwent posterior fossa decompression (PFD) for Chiari malformation type I (CM-I). Methods: We interviewed patients who underwent PFD for CM-I from 1995 to 2016. Results: A total of 79 patients were interviewed. The median age at surgery was 30 years (range 5–72 years) with 27 pediatric patients. Forty-six patients had syringomyelia (36 adults and 10 pediatric patients). Fifty-four patients (68%) reported at least some improvement, 46 (58%) important improvement, 13 (16%) worsening, and 12 stabilization (15%). Any improvement as well as important improvement were significantly more often reported in the nonsyringomyelia group (85% vs. 57%, P = 0.01 and 76% vs. 46%, P = 0.01, respectively). Of the 47 patients reporting preoperative neck pain, 31 (66%) reported at least some improvement after surgery and 9 (19%) worsening after surgery. Of the 59 patients experiencing headaches before surgery, 45 (76%) reported at least some improvement after surgery and 4 (7%) worsening. Quality of life was mostly affected by pain and discomfort in all groups. Sixty-two patients (78%) were satisfied or very satisfied with the results of surgery and 8 (11%) were unsatisfied or very unsatisfied. Up to 71 patients (90%) would consent to surgery again. Conclusion: In CM-I patients, PFD offers symptom improvement in about two-thirds of patients with high patient satisfaction. Symptom improvement is significantly higher in patients without associated syringomyelia, but patient satisfaction is similar. Symptom worsening is more frequent in the adult than in the pediatric population, with similar rates of postoperative improvement and patient satisfaction.


2017 ◽  
Vol 19 (5) ◽  
pp. 511-517 ◽  
Author(s):  
Siri Sahib S. Khalsa ◽  
Alan Siu ◽  
Tiffani A. DeFreitas ◽  
Justin M. Cappuzzo ◽  
John S. Myseros ◽  
...  

OBJECTIVEPrevious studies have indicated an association of Chiari malformation Type I (CM-I) and a small posterior fossa. Most of these studies have been limited by 2D quantitative methods, and more recent studies utilizing 3D methodologies are time-intensive with manual segmentation. The authors sought to develop a more automated tool to calculate the 3D posterior fossa volume, and correlate its changes after decompression with surgical outcomes.METHODSA semiautomated segmentation program was developed, and used to compare the pre- and postoperative volumes of the posterior cranial fossa (PCF) and the CSF spaces (cisterna magna, prepontine cistern, and fourth ventricle) in a cohort of pediatric patients with CM-I. Volume changes were correlated with postoperative symptomatic improvements in headache, syrinx, tonsillar descent, cervicomedullary kinking, and overall surgical success.RESULTSForty-two pediatric patients were included in this study. The mean percentage increase in PCF volume was significantly greater in patients who showed clinical improvement versus no improvement in headache (5.89% vs 1.54%, p < 0.05) and tonsillar descent (6.52% vs 2.57%, p < 0.05). Overall clinical success was associated with a larger postoperative PCF volume increase (p < 0.05). These clinical improvements were also significantly associated with a larger increase in the volume of the cisterna magna (p < 0.05). The increase in the caudal portion of the posterior fossa volume was also larger in patients who showed improvement in syrinx (6.63% vs 2.58%, p < 0.05) and cervicomedullary kinking (9.24% vs 3.79%, p < 0.05).CONCLUSIONSA greater increase in the postoperative PCF volume, and specifically an increase in the cisterna magna volume, was associated with a greater likelihood of clinical improvements in headache and tonsillar descent in patients with CM-I. Larger increases in the caudal portion of the posterior fossa volume were also associated with a greater likelihood of improvement in syrinx and cervicomedullary kinking.


2007 ◽  
Vol 107 (1) ◽  
pp. 49-52 ◽  
Author(s):  
John C. Wellons ◽  
R. Shane Tubbs ◽  
Cuong J. Bui ◽  
Paul A. Grabb ◽  
W. Jerry Oakes

2019 ◽  
Vol 2 (1) ◽  
Author(s):  
Shawyon Baygani, BS ◽  
Kristin Zieles,BS ◽  
Andrew Jea, MD, MHA

Background and Hypothesis: Chiari malformation type I (CM-I) is a common pediatric disorder in which the cerebellar tonsils herniate the foramen magnum with associated spinal symptoms. The purpose of this study is to determine if the preoperative Pediatric Quality of Life Inventory (PedsQL) score is predictive of intermediate-term PedsQL outcomes following Chiari decompression surgery. The utility of preoperative patient-reported outcomes (PROs) to postoperative PROs has been previously demonstrated in adult spine surgery. However, to our knowledge, there is currently no widely accepted tool to predict short-, intermediate-, or long-term outcomes after pediatric Chiari decompression surgery. Project Methods: A prospectively maintained database was retrospectively reviewed. Patients who underwent first-time decompression for symptomatic Chiari malformation were identified and grouped by their preoperative PedsQL scores: mild disability (score 80–100), moderate disability (score 60–80); and severe disability (score < 60). PedsQL outcomes were collected at 6-week, 3-month, and 6-month follow-up. Preoperative PedsQL subgroups were tested for an association with demographic, perioperative characteristics, and improvements between groups by one-way ANOVA, chi-square, or Wilcoxon signed rank test, where appropriate. Results: The postoperative patient- and parent-reported PedsQL values were significantly different between all three groups at 6-weeks, 3-months, and 6-months after surgery (p < 0.05), except at the 6-month timepoint for parent-reported outcomes (p = 0.111). Patients with higher preoperative disability demonstrated statistically significant greater improvements (compared to preoperative score) in parent-reported PedsQL at all timepoints after surgery, except at the 6-month parent-reported outcomes (p = 0.133). Conclusion: Patients with worse preoperative disability, as assessed by PedsQL, experienced lower absolute PedsQL scores at all timepoints after surgery but had greater improvement in short- and intermediate-term PROs. We conclude that PedsQL is an efficient and accurate tool that can quickly assess patient disability in the preoperative period and predict both short-term and long-term surgical outcomes.


2018 ◽  
Vol 22 (4) ◽  
pp. 426-438 ◽  
Author(s):  
Andrew C. Vivas ◽  
Nir Shimony ◽  
Eric M. Jackson ◽  
Risheng Xu ◽  
George I. Jallo ◽  
...  

OBJECTIVEHydrocephalus associated with subdural hygromas is a rare complication after decompression of Chiari malformation type I (CM-I). There is no consensus for management of this complication. The authors present a series of 5 pediatric patients who underwent CM-I decompression with placement of a dural graft complicated by posterior fossa hygromas and hydrocephalus that were successfully managed nonoperatively.METHODSA retrospective review over the last 5 years of patients who presented with hydrocephalus and subdural hygromas following foramen magnum decompression with placement of a dural graft for CM-I was conducted at 2 pediatric institutions. Their preoperative presentation, perioperative hospital course, and postoperative re-presentation are discussed with attention to their treatment regimen and ultimate outcome. In addition to reporting these cases, the authors discuss all similar cases found in their literature review.RESULTSOver the last 5 years, the authors have encountered 194 pediatric cases of CM-I decompression with duraplasty equally distributed at the 2 institutions. Of those cases, 5 pediatric patients with a delayed postoperative complication involving hydrocephalus and subdural hygromas were identified. The 5 patients were managed nonoperatively with acetazolamide and high-dose dexamethasone; dosages of both drugs were adjusted to the age and weight of each patient. All patients were symptom free at follow-up and exhibited resolution of their pathology on imaging. Thirteen similar pediatric cases and 17 adult cases were identified in the literature review. Most reported cases were treated with CSF diversion or reoperation. There were a total of 4 cases previously reported with successful nonoperative management. Of these cases, only 1 case was reported in the pediatric population.CONCLUSIONSDe novo hydrocephalus, in association with subdural hygromas following CM-I decompression, is rare. This presentation suggests that these complications after posterior fossa decompression with duraplasty can be treated with nonoperative medical management, therefore obviating the need for CSF diversion or reoperation.


2011 ◽  
Vol 7 (4) ◽  
pp. 375-379 ◽  
Author(s):  
David Benglis ◽  
Derek Covington ◽  
Ritwik Bhatia ◽  
Sanjiv Bhatia ◽  
Mohamed Samy Elhammady ◽  
...  

Object The natural history of untreated Chiari malformation Type I (CM-I) is poorly defined. The object of this study was to investigate outcomes in pediatric patients with CM-I who were followed up without surgical intervention. Methods The authors retrospectively reviewed 124 cases involving patients with CM-I who presented between July 1999 and July 2008 and were followed up without surgery. The patients ranged in age from 0.9 to 19.8 years (mean 7 years). The duration of follow-up ranged from 1.0 to 8.6 years (mean 2.83 years). Imaging findings, symptoms, and findings on neurological examinations were noted at presentation and for the duration of follow-up. Results The mean extent of tonsillar herniation at presentation was 8.35 mm (range 5–22 mm). Seven patients had a syrinx at presentation. The syrinx size did not change in these patients on follow-up imaging studies. No new syrinxes developed in the remaining patients who underwent subsequent imaging. The total number of patients with presenting symptoms was 81. Of those 81 patients, 67 demonstrated symptoms that were not typical of CM-I. Of the 14 patients with symptoms attributed to CM-I, 9 had symptoms that were not severe or frequent enough to warrant surgery, and surgery was recommended in the remaining 5 patients. Chiari malformation Type I was also diagnosed in 43 asymptomatic patients who had imaging studies performed for various reasons. No new neurological deficits were noted in any patient for the duration of follow-up. Conclusions The majority of patients with CM-I who are followed up without surgery do not progress clinically or radiologically. Longer follow-up of this cohort will be required to determine if symptoms or new neurological findings develop over the course of many years.


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