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Wright DT, Nguyen SA, Teufel RJ, White DR. Health Care Resource Use in Patients With and Without 22q11.2 Deletion Syndrome Undergoing Sphincter Pharyngoplasty for Velopharyngeal Insufficiency. JAMA Otolaryngol Head Neck Surg. 2017;143(3):286–291. doi:10.1001/jamaoto.2016.3533
Do patients with 22q11.2 deletion syndrome, a population with more medically complex conditions, undergoing sphincter pharyngoplasty for velopharyngeal insufficiency have increased length of stay, total costs, and complications?
Among the 687 patients in this analysis of the 2012 Kids’ Inpatient Database, the median length of stay and total cost of admission were significantly higher for patients with 22q11.2 deletion syndrome. Furthermore, 22qDS alone was significantly associated with both length of stay and total cost of admission.
Patients with 22q11.2 deletion syndrome undergoing sphincter pharyngoplasty for velopharyngeal insufficiency require more healthcare resources than patients without 22qDS, which can be attributed to the presence of the syndrome.
The use of health care resources in patients with velopharyngeal insufficiency undergoing sphincter pharyngoplasty is unknown.
To examine the use of health care resources by patients with velopharyngeal insufficiency who have undergone sphincter pharyngoplasty and investigate whether patients with 22q11.2 deletion syndrome (22qDS) had a longer length of stay, increased cost of admission, and higher number of complications.
Design, Setting, and Participants
Using data from the Kids’ Inpatient Database for January 1 to December 31, 2012, we retrospectively analyzed all patients undergoing pharyngoplasty for velopharyngeal insufficiency. In addition, patients were analyzed according to whether or not they had 22qDS. Data were analyzed from January 1 to December 31, 2012.
Main Outcomes and Measures
Total cost of admission, length of stay, number of procedures, and number of complications.
There were 687 patients: 90 with 22qDS (mean [SD] age, 6.4 [2.7] years; 43 males and 47 females) and 597 without 22qDS (mean [SD] age, 7.5 [4.1] years; 326 males and 271 females). In both groups, patients were predominantly white, had private insurance, were treated in a children’s hospital, and were from either the West or Midwest. The median length of stay (2.0 [interquartile range (IQR), 1.0-3.9] vs 1.0 [IQR, 1.0-2.0] days; 95% CI, 1.0-2.0) and total cost of admission ($9269.24 [IQR, $6800.65-$13 189.25] vs $6936.95 [IQR, $5036.71-$9054.98]; 95% CI, $6791.83-$7542.47) were higher for the group with 22qDS than those without 22qDS, while there was no significant difference in the number of procedures performed. In addition, the median number of diagnoses (5.0 [IQR, 3.0-9.0] vs 3.0 [IQR, 2.0-5.0]; 95% CI, 3.0-4.0) and number of chronic conditions (3.0 [IQR, 2.0-5.0] vs 2.0 [IQR, 1.0-2.0]; 95% CI, 2.0-2.0) were higher for the group with 22qDS than those without 22qDS. Furthermore, results of a multiple regression model showed that 22qDS was positively associated with both length of stay (B = 0.92; 95% CI, 0.59-1.24) and total cost (B = $3458.78; 95% CI, $2051.09-$4866.46).
Conclusions and Relevance
This study demonstrates that patients with 22qDS undergoing sphincter pharyngoplasty for velopharyngeal insufficiency have more complex medical conditions and require more health care resources than patients without 22qDS.
Velopharyngeal insufficiency (VPI) is a disorder characterized by an inability to adequately close the velopharyngeal port. Competency of velopharyngeal closure is necessary for normal speech production. Speech has increased nasal resonance when producing nonnasal phonemes (most of the consonant sounds in English) in patients with inadequate closure of the velopharyngeal port. Treatment of VPI includes speech therapy, palatal lift prosthetic devices that assist in closure of the velopharyngeal port, and surgery. Corrective operations for VPI include such procedures as palatal lengthening, pharyngeal flap surgery, and sphincter pharyngoplasty (SP).
22q11.2 Deletion syndrome (22qDS) is the most common microdeletion syndrome in humans, with a reported prevalence as high as 1 in 2000 individuals, although it is most commonly reported as 1 in 4000 individuals.1 22q11.2 Deletion syndrome demonstrates variable expressivity and incomplete penetrance, with DiGeorge syndrome and velo-cardio-facial syndrome representing 2 classic phenotypes of 22qDS. Patients with 22qDS have varying presentation of disease; congenital heart defects, palatal abnormalities, dysmorphic facies, learning difficulties, and immune deficiencies are the most common abnormalities present.1,2 From 67% to 100% of patients with 22qDS have some palatal abnormality, which may include cleft palate, bifid uvula, submucosal cleft palate, and velopharyngeal dysfunction.3,4 Velopharyngeal insufficiency is the most common speech abnormality found in patients with 22qDS.1,3
Anatomical considerations and surgeon preference dictate which surgical procedure is performed to correct VPI.5-8 When anatomical considerations are applied, patients with sagittal velopharyngeal port closure patterns are preferentially given pharyngeal flap procedures to correct VPI, whereas SP is preferentially performed on patients with coronal or circular velopharyngeal closure patterns. Patients with 22qDS may concurrently have hypodynamic velopharynges or anatomical variants, including medialization of the internal carotid arteries, adding a layer of complexity to surgical repair.9 Furthermore, patients with 22qDS are expected to have other comorbidities that may necessitate increased use of health care resources in their management. Although patients with 22qDS have more complex medical conditions, there are currently no national studies on how this difference in complexity is manifested in the care of children with VPI.
The Healthcare Cost and Utilization Project (HCUP) 2012 Kids’ Inpatient Database (KID) sponsored by the Agency for Healthcare Research and Quality is a national database that contains data generated from hospital billing information and is the only all-payer pediatric inpatient care database in the United States. KID samples pediatric discharges from all community nonrehabilitation hospitals from 44 states and more than 4100 institutions. The criterion for inclusion include all admissions for which the patient age was younger than 21 years at the time of admission. KID allows analysis of national estimates for costs and patient safety, among other possibilities.10
To our knowledge, the current literature lacks any studies demonstrating the national inpatient health care use and adverse surgical events for patients with VPI and 22qDS who undergo SP. The purpose of this study was to determine if patients with 22qDS and VPI undergoing SP had increased length of stay, total costs, and complications compared with patients without 22qDS undergoing SP for VPI. We hypothesized that children with 22qDS would represent a more medically complex population requiring more use of health care resources and higher risk of complications during surgical management of VPI.
The study examined inpatient admissions for pediatric patients from January 1 to December 31, 2012, using KID, which contains more than 6.6 million patients for 2012. This study did not require approval from the Medical University of South Carolina Office of Research Integrity as it is compliant with the Health Insurance Portability and Accountability Act because it is a deidentified, publically accessible database. Patients with International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) procedure code (PC) 29.4 (plastic operation on pharynx) and either ICD-9-CM diagnosis code (DC) 478.29 (other diseases of pharynx or nasopharynx), 528.9 (other and unspecified diseases of the oral soft tissues), or 750.29 (other specified congenital anomalies of pharynx) were analyzed. The ICD-9-CM PC 29.4 was used as an indicator of SP. The ICD-9-CM DCs 478.29, 528.9, and 750.29 were used as an indicator of velopharyngeal insufficiency. Patients were further segregated into 2 groups: 1 group with concurrent ICD-9-CM DC of either 279.11 (DiGeorge syndrome) or 758.32 (velo-cardio-facial syndrome) and 1 group without the aforementioned diagnoses. The ICD-9-CM DCs 279.11 and 758.32 were used to generate a pool of patients with 22qDS because there is no ICD-9-CM DC specifically denoting 22qDS.
Patients with obstructive sleep apnea (OSA; ICD-9-CM DC 327.23) as a primary diagnosis, those with malignant tumors of the head and neck, and patients without a diagnosis of VPI were excluded from analysis since other types of pharyngoplasty procedures are used to address those diagnoses. To exclude patients with malignant tumors of the head and neck, all patients with an ICD-9-CM DC ranging from 140.0 to 239.9 were independently reviewed by their ICD-9-CM DC to determine if they met exclusion criteria. The ICD-9-CM PC 29.4 is used as a code for multiple procedures, necessitating the exclusion of patients with a primary diagnosis of OSA and all patients with a concomitant diagnosis of malignant tumor of the head and neck. In addition, patients undergoing a concurrent surgical closure of a pharyngeal fistula (ICD-9-CM PC 29.53) and those at risk for identification were excluded from the analysis.
In this study, race is reported by the participant. If race and ethnicity were recorded in separate categories, then ethnicity takes precedence over race in setting the HCUP value for race. All procedures by ICD-9-CM PC were summed using all procedure variables to determine the total of other procedures performed.
Total cost was determined using the HCUP Cost-to-Charge Ratio File for the 2012 KID.11 KID provides data on the total charges per patient encounter; however, these charges do not reflect the final cost. The Center for Medicare & Medicaid Services collects hospital accounting reports that were used to generate a hospital-specific cost-to-charge ratio based on all-payer inpatient costs and charges for most of the hospitals in KID. Where all-payer inpatient cost and charge information was not available, group average all-payer inpatient cost and charge ratios are used. The hospital charges were multiplied by the cost-to-charge ratio to compute an estimated cost.
Data were analyzed from January 1 to December 31, 2012, using GraphPad Prism, version 6.0, software (GraphPad Software); SPSS, version 23.0 (IBM SPSS); and SigmaPlot, version 12.5 (Systat Software). Continuous variables were summarized as mean (SD) and median and interquartile range (IQR). Categorical variables were summarized as frequency and percentage. All continuous variables were tested for normal distribution as determined by the D’Agostino-Pearson omnibus normality test. Comparisons of categorical variables were performed using a Fisher exact test or χ2 test. For continuous variables, comparisons between groups were made with a Mann-Whitney test (nonnormal distribution). Effect size estimates were calculated with the SPSS compute variable function from Mann-Whitney test output data.12 A backward, stepwise multiple regression model was performed to determine association of total costs and length of stay with 22qDS and all Agency for Healthcare Research and Quality comorbidity measures10 with F<0.1 as exclusion criteria. The backward, stepwise regression model has more power to improve the prediction performance owing to having a smaller number of independent predictor variables in the model. P < .05 was considered statistically significant. All analyses were weighted by the discharge-level weight to ensure estimates were generalizable to the nation.10
There were 930 patients with an ICD-9-CM PC that could indicate SP; 243 of these patients were excluded because they either had a primary diagnosis of OSA, had a concurrent surgical closure of a pharyngeal fistula, had a malignant tumor of the head and neck, did not have a diagnosis of VPI, or were at risk for identification, leaving 687 patients in the study. Of the patients meeting inclusion criteria, 90 had 22qDS and 597 did not. Basic demographics of the 2 groups were analyzed, and there was no significant difference between groups with regard to sex, median age, race, hospital region, hospital type, and primary payer (Table 1). In addition, the median number of chronic conditions (3.0 [IQR, 2.0-5.0] vs 2.0 [IQR, 1.0-2.0]; U = 5816; 95% CI, 2.0-2.0) and number of diagnoses (5.0 [IQR, 3.0-9.0] vs 3.0 [IQR, 2.0-5.0]; U = 8026; 95% CI, 3.0-4.0) were higher for the group with 22qDS than those without 22qDS.
The median length of stay for the group with 22qDS was longer than for the group without the syndrome (2.0 [IQR, 1.0-3.9] vs 1.0 [IQR, 1.0-2.0] days; U = 103 251; 95% CI, 1.0-2.0) (Table 2). The median total cost for the group with 22qDS was higher than for the group without 22qDS ($9269.24 [IQR, $6800.65-$13 189.25] vs $6936.95 [IQR, $5036.71-$9054.98]; U = 8370; 95% CI, $6791.83-$7542.47). An analysis on the number of iatrogenic events owing to medical care or drugs was conducted; however, there were not enough events to report significance. Table 3 shows a summary of other procedures performed during admission. Myringotomy with insertion of tube (n = 78), other plastic repair of palate (n = 63), and removal of tympanostomy tube (n = 33) were the most common procedures among patients without 22qDS.
Results of multivariable regression models showed that 22qDS alone was significantly independently associated with total cost (B = $3458.78; 95% CI, $2051.09-$4866.46) and length of stay (B = 0.92; 95% CI, 0.59-1.24). Deficiency anemias also were statistically significantly associated with length of stay (B = 1.68; 95% CI, 0.28-3.07).
As the most prevalent microdeletion syndrome in humans, 22qDS is a condition for which the burden of disease warrants further study. To our knowledge, there are no studies to date examining the health care burden of patients with 22qDS undergoing surgical treatment of VPI. In our study, patients with 22qDS had more complex medical conditions than patients without 22qDS, as evidenced by the increased median number of chronic conditions and number of total diagnoses. Increased use of health care resources was observed in the group with 22qDS in the form of increased median length of stay and increased median total cost. The increased use of health care resources was in part owing to the patients’ increased medical complexity and not the total number of procedures, which was not significantly different between the 2 groups. To our knowledge, this study is the first to assess the disparity in health care use of patients with 22qDS and VPI but also the first to evaluate for possible causes of this disparity.
Patients with 22qDS and VPI are known to have a relative increased medical complexity compared with patients without 22qDS who have VPI, as demonstrated in this study by the increased number of chronic conditions in patients with 22qDS. This study demonstrates how that complexity affects use of health care resources in patients undergoing SP. Both length of stay and total cost of admission were statistically significantly higher for patients with 22qDS. When examining the multiple regression models, only patients with 22qDS had a significant contribution to both length of stay and total cost. The observation that 22qDS was positively associated with both length of stay and total cost when all other comorbidities were not significantly associated with length of stay or total cost suggests that 22qDS correlates with health care use in our patient population.
With regard to the difference in use of health care resources between the 2 groups, patients with 22qDS may undergo more diagnostic testing (eg, evaluation for carotid medialization) before pharyngeal surgery. This information would not be captured unless the testing was done during the same admission as the pharyngoplasty.
Myringotomy with tympanostomy tube placement is the most prevalent procedure performed in this cohort after SP. Examining other procedures performed in patients in our cohort provides more information about surgical procedures performed for VPI. The second most common procedure performed in the group without 22qDS is other plastic repair of palate. This finding likely represents concurrent double-opposing Z-plasty palatoplasty for either correction of submucous cleft palate or revision of previous palatoplasty for lengthening or muscle realignment. Analysis of the 22qDS subgroup provided a sample size too small to do further analysis of the most common procedures performed during their admission.
When evaluating demographics of patients in this study, the mean age of patients in both groups (with 22qDS, 6.4 [2.7] years; without 22qDS, 7.5 [4.1] years) provokes some discussion. The mean (SD) age of 6.4 (2.7) years for patients with 22qDS undergoing SP for VPI falls in line with the mean age range of less than 6.1 to 9.3 years reported in one systematic review.8 The younger mean age of repair for the group with 22qDS may represent earlier identification of VPI in this population (possibly owing to more severe VPI being noted), or it may represent a tendency toward a more aggressive surgical approach.
A total of 390 patients (56.8%) in our study had private insurance in contrast to the 2 836 105 of all 6 675 222 patients (42.5%) in KID (3 277 608 of all 6 675 222 patients [49.1%] in KID have Medicaid). 22q11.2 Deletion syndrome is more prevalent in Hispanic individuals compared with white, black, or Asian individuals.1 Despite that fact, 368 patients (53.6%) were white, warranting a question about whether there is a discrepancy in access for patients of minority race/ethnicity who have Medicaid with or without 22qDS undergoing SP for VPI. In addition, 419 patients (61.0%) were treated in a children’s hospital compared with 599 550 of all 6 675 222 patients (9.0%) in KID. This difference is probably owing to the fact that children’s hospitals are positioned to handle children with complex medical conditions and children requiring services from pediatric subspecialists and pediatric surgeons. Whether there is a link between the aforementioned possible disparities in health care use and treatment at children’s hospitals warrants further investigation.
The postoperative course of patients undergoing SP for VPI is well documented in the literature for long-term outcomes, but health care use is poorly studied. Patients who undergo SP for VPI typically are admitted for observation overnight to monitor for apneic events following surgery because patients undergoing both SP and pharyngeal flap surgical procedures for VPI have been reported to have new-onset OSA after surgery.13-25 A previous study that evaluated management after SP for all patients with no delineation of 22qDS status found it appropriate to perform SP as an outpatient procedure because upper airway obstruction was uncommon, yet it did not report whether there was a change in cost or use of health care resources as a result.26
The limitations of this study are primarily those inherent to the use of a national discharge database. Longitudinal follow-up is not available, and we cannot identify patients who required multiple hospitalizations. Furthermore, ICD-9-CM DCs and PCs were used to identify our study cohort and key variables. Limitations of coding deserve special mention. Strictly speaking, 22qDS refers to patients who have been diagnosed with the microdeletion, while DiGeorge syndrome and velo-cardio-facial syndrome nomenclature is dependent on the patient’s phenotype. There is currently no unique ICD-9-CM DC for VPI; instead, multiple codes are used by different individuals and institutions.
Analysis of the database required careful examination and correlation of codes to minimize the inclusion of patients without VPI who were undergoing pharyngoplasty for another reason (eg, OSA). These considerations were undertaken to reduce the confounding nature of the imprecision of ICD-9-CM codes. For patients meeting inclusion criteria, the ICD-9-CM PC for plastic operation on pharynx may include other pharyngoplasty procedures, such as posterior wall augmentation. Although other procedures may be included in the ICD-9-CM PC, the relative prevalence of SP in patients with VPI when compared with other pharyngoplasty procedures ensures that we captured the intended procedure. Furthermore, the ICD-9-CM PCs 29.91 and 29.99 may capture other pharyngoplasty procedures, thus inherently reducing some of the confounding operations. More specific coding identifiers need to be in place to make future analyses more precise. Although the aforementioned issues make such a study more difficult, the validity of the observations should not be discounted and rather should be used to promote studies of health care use in patients with 22qDS and VPI at an institutional level.
An analysis of the number of iatrogenic events owing to medical care or drugs was conducted. There were not enough events in each group to satisfy HCUP reporting criteria despite the large sample size relative to the population we are studying. Overall, 16 of all 687 patients (2.3%) in this study experienced an iatrogenic event, but further comment on the nature of the events is prohibited by HCUP reporting criteria because the number of events was so small.
Despite these limitations, the strengths of our study are largely owing to the large sample size of the administrative data. KID allows for a robust characterization of national trends of low-incidence pediatric conditions. This study provided a unique assessment of the patient demographics, hospital practices, and overall costs using data from regions across the United States and found that patients with 22qDS undergoing SP for VPI have more complex medical conditions and require more health care resources than patients without 22qDS.
Accepted for Publication: September 15, 2016.
Corresponding Author: Darrell T. Wright, MD, Department of Otolaryngology–Head and Neck Surgery, Medical University of South Carolina, 135 Rutledge Ave, Mail Stop Code 550, Charleston, SC 29425 (email@example.com).
Published Online: December 22, 2016. doi:10.1001/jamaoto.2016.3533
Author Contributions: Dr Wright had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.
Study concept and design: Teufel, White.
Acquisition, analysis, or interpretation of data: All authors.
Drafting of the manuscript: Wright, White.
Critical revision of the manuscript for important intellectual content: All authors.
Statistical analysis: Wright, Nguyen, Teufel.
Administrative, technical, or material support: Teufel, White.
Study supervision: White.
Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported.
Additional Contributions: We thank the Healthcare Cost and Utilization Project, Agency for Healthcare Research and Quality and Healthcare Cost and Utilization Project Data partners (https://www.hcup-us.ahrq.gov/db/hcupdatapartners.jsp) for making this study feasible.