Pilot Investigation of Somatosensory Functioning and Pain Catastrophizing in Pediatric Spinal Fusion Surgery.

PURPOSE: Chronic post-surgical pain (CPSP) is a significant concern and contributes to the opioid epidemic; however, little is known about CPSP in young people. DESIGN: This prospective study aimed to identify sensory, psychological, and demographic factors that may increase the risk of CPSP after spinal fusion surgery for children and adolescents with idiopathic scoliosis. METHODS: 32 children and adolescents from two children's hospitals completed quantitative sensory testing (QST) and the Pain Catastrophizing Scale Child (PCS-C) pre-and 4-6 months post spinal fusion surgery. Between-group differences were assessed using an independent samples t-test. Pearson's correlations and stepwise linear regression were used to assess the relationship between variables at both time points. RESULTS: 56% of patients endorsed pain post-surgery. They were more sensitive tomechanical detection on both a control non-pain site (r = -2.87, p = .004) and the back (r = -1.83, p = .04), as well as pressure pain (r=-2.37, p = .01) on the back. This group also reported worse pain scores pre-surgery. Pre-surgery helplessness positively correlated with preoperative pain (r = .67 p < .001), and age was negatively correlated with the post-surgical catastrophizing total score (r =-.39, p = .05), suggesting that younger patients endorsed more pain-related worry after surgery. CONCLUSIONS: Patients who present with pain during their preoperative appointment may need to be monitored with increased vigilance throughout the perioperative period, possibly with bedside QST and psychological questionnaires, which nurses could administer. Biobehavioral interventions targeting pain intensity and feelings of helplessness and anxiety during the preoperative period may alleviate the transition to CPSP.

Dark-adapted threshold and electroretinogram for diagnosis of Usher syndrome.

PURPOSE: To determine the utility of ophthalmology evaluation, dark-adapted threshold, and full-field electroretinogram for early detection of Usher syndrome in young patients with bilateral sensorineural hearing loss. METHODS: We identified 39 patients with secure genetic diagnoses of Usher Syndrome. Visual acuity, spherical equivalent, fundus appearance, dark-adapted threshold, and full-field electroretinogram results were summarized and compared to those in a group of healthy controls with normal hearing. In those Usher patients with repeated measures, regression analysis was done to evaluate for change in visual acuity and dark-adapted threshold with age. Spherical equivalent and full-field electroretinogram responses from dark- and light-adapted eyes were evaluated as a function of age. RESULTS: The majority of initial visual acuity and spherical equivalent results were within normal limits for age. Visual acuity and dark-adapted threshold worsened significantly with age in Usher type 1 but not in Usher type 2. At initial test, full-field electroretinogram responses from dark- and light-adapted eyes were abnormal in 53% of patients. Remarkably, nearly half of our patients (17% of Usher type 1 and 30% of Usher type 2) would have been missed by tests of retinal function alone if evaluated before age 10. CONCLUSIONS: Although there is an association of abnormal dark-adapted threshold and full-field electroretinogram at young ages in Usher patients, it appears that a small but important proportion of patients would not be detected by tests of retinal function alone. Thus, genetic testing is needed to secure a diagnosis of Usher syndrome.

Association of Blood Type With Postsurgical Mucosal Bleeding in Pediatric Patients Undergoing Tonsillectomy With or Without Adenoidectomy.

Importance: Blood type (BT) O has been identified as a risk factor for bleeding complications, while non-O BTs may increase risk for thromboembolic events. Limited data are available in children undergoing tonsillectomy with or without adenoidectomy. Objective: To determine whether BT O is associated with hemorrhage after tonsillectomy with or without adenoidectomy. Design, Setting, and Participants: Retrospective cohort study of patients younger than 22 years who underwent tonsillectomy with or without adenoidectomy at a single institution between January 1, 2008, and August 7, 2017. Statistical analysis was performed from November 2017 to January 2019. Main Outcomes and Measures: Prevalence of hemorrhage following surgery was defined as any bleeding requiring cauterization up to 1 month after the procedure. Data on sex, age, von Willebrand disease (VWD) status, BT, white blood cell counts, and platelet counts closest to date of surgery were collected from an electronic medical record system, and the association of these factors with hemorrhage following surgery was investigated. Results: A total of 14 951 pediatric patients (median [range] age, 5.6 [0.8-21.9] years; 6956 [46.5%] female) underwent tonsillectomy with or without adenoidectomy. Prevalence of hemorrhage following the procedure was 3.9% (578 patients) for the full cohort and 2.8% (362 of 13 065) for patients with no BT identified or preprocedure VWD panel results at baseline. Children who had a BT identified and/or a VWD panel before surgery had higher bleeding rates (BT only, 14.9% [172 of 1156]; preprocedure VWD panel only, 4.6% [28 of 607]; and BT and preprocedure VWD panel, 13.0% [16 of 123]), all of which were significantly different from the baseline bleeding rate (P < .001). While the bleeding rates in children with BT O were not statistically different from those with non-O BT (14.8% and 14.6%, respectively; P > .99), mean von Willebrand factor values were statistically different (mean [SD] von Willebrand factor antigen level in O group, 86.9 [42.4] IU/dL in the O group vs 118.0 [53.8] IU/dL in the non-O group; P = .002; and mean [SD] von Willebrand factor ristocetin-cofactor in the O group, 72.2 [44.3] IU/dL vs 112.6 [68.0] IU/dL in the non-O group; P = .001). In addition, children older than 12 years had increased bleeding rates in the full cohort (8.3% vs 3.2%), in the testing-naive cohort (6.5% vs 2.3%), and in those with a preprocedure VWD panel only (13.5% vs 3.1%) compared with children aged 12 years or younger. Conclusions and Relevance: Type O blood was not a risk factor associated with hemorrhage after tonsillectomy with or without adenoidectomy despite lower baseline von Willebrand factor antigen and von Willebrand factor ristocetin-cofactor values in children with BT O vs those with non-O BT in our study cohort. No association was found between VWD status and bleeding, and there was no difference in VWD panel values in those who experienced hemorrhage vs those who did not within BT groups. Further studies elucidating the utility of von Willebrand factor values for children undergoing tonsillectomy with or without adenoidectomy are needed.

Torticollis in children with enlarged vestibular aqueducts.

OBJECTIVES: To evaluate the association between torticollis and enlarged vestibular aqueduct (EVA). METHODS: An online/phone survey was administered to parents of 133 children diagnosed with the following disorders: EVA, GJB2 (Connexin 26) mutations associated congenital hearing loss and epistaxis (control). The survey included questions regarding symptoms of torticollis, vertigo, and hearing loss. RESULTS: Patients with EVA had a 10-fold greater odds of having torticollis than controls (31% vs. 4%; OR = 10.6; 95% CI: 2.9, 39.2). No patients with GJB2 had a reported history of torticollis. Torticollis preceded the diagnosis of hearing loss in most (87%) patients with EVA who had a reported history of torticollis. EVA patients were more likely to have reported motor delay than controls (40% vs. 15%; p = 0.002). EVA patients with prior torticollis (80%; 12/15) were more likely to have balance impairment than EVA patients without prior torticollis (12%; 4/33; p < 0.001). Twelve patients had a reported history of paroxysmal torticollis, all of whom had EVA. CONCLUSION: Torticollis in infants may be a marker of EVA. Infants with torticollis should be monitored closely for hearing loss and motor delay, especially when the torticollis is paroxysmal.

Perspective on the Development of a Large-Scale Clinical Data Repository for Pediatric Hearing Research.

The use of "big data" for pediatric hearing research requires new approaches to both data collection and research methods. The widespread deployment of electronic health record systems creates new opportunities and corresponding challenges in the secondary use of large volumes of audiological and medical data. Opportunities include cost-effective hypothesis generation, rapid cohort expansion for rare conditions, and observational studies based on sample sizes in the thousands to tens of thousands. Challenges include finding and forming appropriately skilled teams, access to data, data quality assessment, and engagement with a research community new to big data. The authors share their experience and perspective on the work required to build and validate a pediatric hearing research database that integrates clinical data for over 185,000 patients from the electronic health record systems of three major academic medical centers.

Validation of a portable hearing assessment tool: Agilis Health Mobile Audiogram.

OBJECTIVES: To examine if the tablet-based Agilis Health Mobile Audiogram (Agilis Audiogram) is an effective and valid measure of hearing thresholds compared to a pure-tone audiogram in an adult and pediatric population. METHODS: Participants underwent an otologic exam, conventional audiometric evaluation and the self-administered Agilis Audiogram. We examined whether the difference of pure-tone average (PTA) between the two measurement techniques fell within the equivalence range of ±8 dB. The Agilis Audiogram was administered twice for each subject to assess test-retest reliability of the application. RESULTS: A total of 54 ears from 27 participants were evaluated. The average time to complete the self-administered Agilis Audiogram was 10 min. Among participants with normal hearing, the average PTA from conventional audiometric evaluation was 8.9 dB (±3.8) and the average PTA from the Agilis Audiogram was 8.5 dB (±4.5), with mean difference of 0.4 dB (±4.2; 95% CI -1.0 to 1.7 dB) falling within the equivalence range (-8 to 8 dB). Among participants with confirmed hearing loss, the average PTA was 22.5 dB (±17.1) from conventional audiometric evaluation and 24.3 dB (±16.6) from the Agilis Audiogram, with mean difference of -1.8 dB (±5.4; 95% CI -4.9 to 1.3 dB), falling within the equivalence range. Overall, there was a significant correlation between conventional audiometric evaluation and the Agilis Audiogram (Pearson correlation = 0.93; p < 0.001). CONCLUSION: Thresholds obtained by the Agilis Audiogram were found to be a valid measure of hearing among adults with normal hearing and children with hearing loss in the mild-moderate range.

Ambulatory Visits for Otitis Media before and after the Introduction of Pneumococcal Conjugate Vaccination.

OBJECTIVE: To examine the long-term trend of otitis media (OM)-associated ambulatory visits from 1997 to 2014 and to evaluate the impact of 7-valent and 13-valent pneumococcal conjugate vaccines (PCV7 and PCV13) on OM ambulatory visits. STUDY DESIGN: We examined OM-associated ambulatory visits in children, using the National Ambulatory Medical Care Survey and National Hospital Ambulatory Medical Care Survey. RESULTS: A total of 24 148 OM-related visits were identified from 363 240 ambulatory visits. OM visits accounted for 9.5% of all ambulatory visits in 1997-1999, 7.1% in 2002-2009, and 5.5% in 2012-2014. Between the pre-PCV7 and the post-PCV13 period, annual physician's office visits for OM declined from 826 to 387 visits per 1000 children among children younger than 2 years of age. Compared with the pre-PCV7 period, office visit rates for OM significantly declined by 51% (95% CI 42%-58%) among children younger than 2 years of age and by 37% (95% CI 23%-48%) among children 2-4 years of age during the post-PCV13 period. Among children younger than 2 years of age, emergency department visits for OM declined by 47% (95% CI 36%-55%) during the post-PCV13 period, and hospital outpatient visits declined by 30% (95% CI 8%-48%) during the PCV13-transition period. OM-related visits declined across sex, race/ethnicity, health insurance status, and geographic region. CONCLUSION: Our nationwide study shows that ambulatory visits for OM have declined following the introduction of PCV7 and PCV13. Future research on the evolving microbiology of OM and continued monitoring of the epidemiology of pneumococcal disease are needed.

Enlarged Vestibular Aqueduct and Cochlear Implants: The Effect of Early Counseling on the Length of Time Between Candidacy and Implantation.

OBJECTIVE: To determine if discussing cochlear implantation (CI) with patients with enlarged vestibular aqueducts (EVA) and their families before reaching audiological criteria for CI candidacy effects the length of time between reaching audiological candidacy and CI surgery, and to describe the universal newborn hearing screening (UNHS) results and communication modality in this sample. PATIENTS: Forty-two patients (25 females) with confirmed EVA and cochlear implants. INTERVENTION(S): Diagnostic CI visit. MAIN OUTCOME MEASURES: The primary outcome measure is the difference in length of time between reaching audiological candidacy for CI and surgical implantation between those who had preliminary discussions regarding CI with their medical and healthcare providers before reaching audiological candidacy versus who had discussions after reaching candidacy. The secondary outcome measure is the result of the UNHS and primary mode of communication used by each patient. RESULTS: Discussing CI before reaching audiological candidacy was associated with a significantly shorter duration between reaching audiological candidacy and receiving CI (median = 3.1 mo; interquartile range [IQR] = 1.7-5.4) as compared with discussing CI after reaching candidacy (median = 5.8 mo; IQR = 3.2-11.2; p = 0.012). Participants born after the implementation of the UNHS, 16 of 24 patients referred on one or both ears. Communication modalities were evenly divided between utilizing sign-support English and oral/aural communicators only. CONCLUSIONS: Discussion of CI in patients with EVA before reaching audiological candidacy reduces the amount of time the child is without adequate auditory access and contributes to a constructive and interactive preparatory experience.

Otolaryngologic Manifestations of Klippel-Feil Syndrome in Children.

IMPORTANCE: Children with Klippel-Feil syndrome (KFS), characterized principally by abnormal fusion of 2 or more cervical vertebrae, may have many additional congenital anomalies. The overall prevalence of otolaryngologic manifestations among patients with KFS has not been previously characterized. OBJECTIVE: To define the otolaryngologic diagnoses made and procedures performed in 95 patients with KFS, which, to our knowledge, is the largest series of this challenging patient population published to date. DESIGN, SETTING, AND PARTICIPANTS: For this retrospective review, all patients with KFS who underwent otolaryngology consultation at our institution over a 26-year period (January 1989 to December 2015) were included. Patients were identified using International Classification of Diseases, Ninth Revision (ICD-9) codes and were confirmed through individual medical record review. Relevant otolaryngologic diagnoses and procedures were extracted using ICD-9 and Current Procedural Terminology codes, respectively. Selected demographics included age, sex, number of clinic visits, and number of procedures. MAIN OUTCOMES AND MEASURES: The primary outcomes were the otolaryngologic diagnoses and procedures associated with the KFS patient population; the secondary outcome was Cormack-Lehane classification documented during airway procedures. RESULTS: Overall, 95 patients with KFS were included in this study (55 males [58%] and 40 females [42%]); mean (range) age at time of presentation to the otorhinolaryngology clinic was 5.8 (birth-23.0) years. Each patient with KFS averaged 8 visits to the otorhinolaryngology office and 5 otolaryngologic diagnoses. The most common diagnosis was conductive hearing loss (n = 49 [52%]), followed by sensorineural hearing loss (n = 38 [40%]), and dysphagia (n = 37 [39%]). Sixty-two (65%) patients underwent otolaryngologic procedures, with 44 (46%) undergoing multiple procedures. The most common procedure was tympanostomy tube placement (n = 36 [38%]), followed by office flexible endoscopy (n = 23 [24%]). Twelve of the 20 patients who underwent direct laryngoscopy had documented Cormack-Lehane classification; 5 of 12 patients (42%) had a compromised view (grade 2, 3, or 4) of the larynx. Three patients required tracheotomies at this institution for airway stabilization purposes; each had severe upper airway obstruction leading to respiratory failure. CONCLUSIONS AND RELEVANCE: Patients with KFS require consultation for a variety of otolaryngologic conditions. Among these, hearing loss is the most common, but airway issues related to cervical spine fusion are the most challenging. Formulating an appropriate care plan in advance is paramount, even for routine otolaryngology procedures.

Changes in Maternal and Paternal Pain-Related Attitudes, Behaviors, and Perceptions across Pediatric Pain Rehabilitation Treatment: A Multilevel Modeling Approach.

Objectives: This prospective study compared paternal versus maternal factors and their impact on child outcomes in the context of an intensive pediatric pain rehabilitation program. Methods: One hundred four youth with treatment refractory chronic pain and their parents enrolled in an intensive pediatric pain rehabilitation program completed measures of pain, functional disability, and parent pain-related attitudes, perceptions, and behaviors at admission and discharge. Results: Linear mixed models were used. Controlling for significant demographic and clinical characteristics, mothers and fathers who were present for the program typically demonstrated significantly better improvement from admission to discharge compared with nonpresent fathers. Mothers made the most significant gains in protective parent responses. Children also had significant decreases in pain and improvements in functioning over time. Conclusions: Results indicate the efficacy of this treatment model for both children with chronic pain and their parents and highlights the importance of parental presence in treatment.

Predicting Postsurgical Satisfaction in Adolescents With Idiopathic Scoliosis: The Role of Presurgical Functioning and Expectations.

BACKGROUND: There is a need to better assess patient satisfaction and surgical outcomes. The purpose of the current study is to identify how preoperative expectations can impact postsurgical satisfaction among youth with adolescent idiopathic scoliosis undergoing spinal fusion surgery. METHODS: The present study includes patients with adolescent idiopathic scoliosis undergoing spinal fusion surgery enrolled in a prospective, multicentered registry examining postsurgical outcomes. The Scoliosis Research Society Questionnaire-Version 30, which assesses pain, self-image, mental health, and satisfaction with management, along with the Spinal Appearance Questionnaire, which measures surgical expectations was administered to 190 patients before surgery and 1 and 2 years postoperatively. Regression analyses with bootstrapping (with n=5000 bootstrap samples) were conducted with 99% bias-corrected confidence intervals to examine the extent to which preoperative expectations for spinal appearance mediated the relationship between presurgical mental health and pain and 2-year postsurgical satisfaction. RESULTS: Results indicate that preoperative mental health, pain, and expectations are predictive of postsurgical satisfaction. CONCLUSIONS: With the shifting health care system, physicians may want to consider patient mental health, pain, and expectations before surgery to optimize satisfaction and ultimately improve clinical care and patient outcomes. LEVEL OF EVIDENCE: Level I-prognostic study.

Family Beliefs and Interventions in Pediatric Pain Management.

Whether a child has to endure a procedure that incurs acute pain or a child has chronic pain, the impact on the family, especially parents, can be profound. Parents need to be active members of their child's health care team; however, they are often ill equipped to cope with either acute pain stressors or longstanding chronic pain in their children. This article provides an overview of acute and chronic pain, the impact of parent factors on pediatric pain, parental assessments of parent functioning, and parent-based interventions for pediatric pain management. Case examples are used to illustrate the treatments presented.