Search

Objective: Robin sequence (RS) consists of micrognathia and glossoptosis that result in upper airway obstruction (UAO). In RS patients who undergo tracheostomy, long-term goals include natural decannulation (ND) without further surgical airway intervention. The objective of this study was to identify long-term trends in the rate and length of time to ND. Methods: Retrospective chart review on 144 patients with RS treated from 1995 to 2020 at a pediatric tertiary care center. Patients were grouped by year of tracheostomy. Demographic data, UAO management, postoperative care, complications, and time to decannulation were recorded. Results: Thirty-six patients met the inclusion criteria. Tracheostomy was performed at a median age of 45.5 days. 19 (53%) patients experienced ND at a median time of 66.1 months. ND rate was higher in non-syndromic patients (93% non-syndromic vs 27% syndromic; P <.0001) and during the first study period (1995-2006: 78%, 2007-2020: 28%; P =.003). Cox proportional-hazard regression demonstrated that white race [aHR 0.15 (0.03-0.8); P =.023] and higher birthweight [aHR 0.9 (0.8-0.98); P =.018] were associated with a higher likelihood of ND while a syndromic diagnosis had a negative association with ND [aHR 12.5 (3.3-50.0); P <.001]. Conclusions: Our study documented that ND in patients with RS who underwent tracheostomy was significantly associated with ethnicity, birthweight, and syndromic status. The negative impact on successful ND was most observed in patients with syndromic associations. Additionally, ND rates are lower in the 2007 to 2020 subgroup. We suspect this is because alternative management techniques such as tongue lip adhesion and mandibular distraction osteogenesis became primary surgical treatment in severe RS upper airway obstruction at our institution starting in 2007. [ABSTRACT FROM AUTHOR]

Objective: Robin Sequence (RS), characterized by micrognathia, glossoptosis, and upper airway obstruction, is an increasingly recognized diagnosis. An effective surgical intervention is mandibular distraction osteogenesis (MDO). This study analyzes published evidence regarding facial nerve dysfunction (FND) associated with MDO. Design and Setting: According to PRISMA guidelines, a systematic review was carried out with databases queried in June 2019 using MESH terms, or equivalent terms, as follows: "distraction osteogenesis" and "Robin Sequence". A review of original Spanish and English articles, were included. Outcome measures included the prevalence of FND; the affected branches; the rate of permanent vs. transient FND; the use of an internal vs. external device; the daily distraction rate; and finally, the overall distraction length. Subsequently, a meta-analysis was conducted to collate results regarding the prevalence of FND and the factors associated with it. Results: Of 239 unique studies identified, 19 studies with 729 patients met inclusion criteria; 52 patients developed FND after MDO. A random-effects meta-analysis yielded a pooled prevalence of FND of 6.40%, with moderately heterogeneous studies (I2 = 41%, τ2 = 0.006). Marginal mandibular nerve involvement was most commonly noted. Nine studies reported transient FND, six permanent, one both, and two unspecified. Internal distractors were used in 8 studies and external in 3 and both in 2. Distraction rate was 1.00 to 2.00 mm/day and total distraction length ranged from 13.00 to 22.3 mm. Sample size was the only parameter inversely associated with rate of FND (p = 0.04). Conclusion: This analysis of FND associated with MDO for patients with RS demonstrates a lack of consistent documentation. MDO-associated FND does not appear to be uncommon, and permanent dysfunction can occur. This review underscores the importance of thorough documentation to elucidate the mechanism of FND. [ABSTRACT FROM AUTHOR]

Objective: To analyze safety and efficacy of single-dose ketorolac after primary palatoplasty (PP). Design: Consecutive cohort of patients undergoing PP, comparing to historical controls. Setting: A large academic children's hospital. Patients, Participants: A consecutive cohort of 111 patients undergoing PP (study n = 47) compared to historical controls (n = 64). Interventions: All patients received intraoperative acetaminophen, dexmedetomidine, and opioids while the study group received an additional single dose of ketorolac (0.5 mg/kg) at the conclusion of PP. Main Outcome Measures: Safety of ketorolac was measured by significant bleeding complications and need for supplementary oxygen. Efficacy was assessed through bleeding, Face Legs Activity Cry Consolability (FLACC) scale, and opioid dose. Results: Length of stay was similar for both groups (control group 38.5 hours [95% CI: 3.6-43.3] versus study group 37.6 hours [95% CI: 31.3-44.0], P =.84). There were no significant differences in all postoperative FLACC scales. The mean dose of opioid rescue medication measured as morphine milligram equivalents did not differ between groups (P =.56). Significant postoperative hemorrhage was not observed. Conclusions: This is the first prospective study to evaluate the safety and efficacy of single-dose ketorolac after PP. Although lack of standardization between study and historical control groups may have precluded observation of an analgesic benefit, analysis demonstrated a single dose of ketorolac after PP is safe. Further investigations with more patients and different postoperative regimens may clarify the role of ketorolac in improving pain after PP. [ABSTRACT FROM AUTHOR]

Objectives: To explore long-term patient reported outcome (PRO) measures of pediatric paradoxical vocal cord motion (PVCM) including ease of diagnosis, management, symptom duration and effect on quality of life. Methods: All children >8 years of age diagnosed with PVCM at a tertiary pediatric hospital between 2006 and 2017 were invited to complete a survey addressing study objectives. Results: 21/47 eligible participants could be contacted and 18/21 (86%) participated. 78% were female with a mean age at diagnosis of 11.6 and 15.0 years at survey completion. Common PVCM symptoms reported were dyspnea (89%), globus sensation (56%), and stridor (50%). The median time to diagnosis was 3 months (IQR 2-5 months). Nearly all reported being misdiagnosed with another condition, usually asthma, until being correctly diagnosed usually by an otolaryngologist. Participants reported undergoing 3.7 diagnostic studies (range 0-8); pulmonary function testing was most common. Of numerous treatments acknowledged, breathing exercises were common (89%) but only reported helpful by 56%. Use of biofeedback was recalled in 1/3 of subjects but reported helpful in only 14% of them. Anti-reflux, allergy, anticholinergics, inhalers and steroids were each used in >50%, but rarely reported effective. PVCM was reportedly a significant stressor when initially diagnosed but despite 2/3 of participants still reporting ongoing PVCM symptoms, the perceived stress significantly decreased over time (Z = 3.26, P = 0.001). Conclusions: This first PVCM PRO study endorses that diagnosis is often delayed and prescribed treatments often viewed as ineffective. While biofeedback and breathing exercises may be critical for short-term control of PVCM episodes, lifestyle changes and stress reduction are likely necessary for long-term management. Increased awareness and improvements in management are needed for this condition. [ABSTRACT FROM AUTHOR]

Objectives: Chronic recalcitrant cough is present in 2/3 of pediatric patients evaluated in our tertiary-care multidisciplinary aerodigestive clinic (ADC). This study aimed to determine the impact of chronic cough and efficacy of ADC treatment using the validated Pediatric-Cough Quality-of-Life-27 tool (PC-QOL-27). Methods: The PC-QOL-27 survey was administered to ADC patients with chronic cough at initial clinic visit and 6 to 12 weeks after cough management. Pre and post survey scores, demographic data, treatment and evaluation season were collected over 16 months. Results: Twenty parents completed pre and post PC-QOL-27 surveys (mean 12.1 weeks later). Patient median age was 6.04 years (IQR: 2.2-10.44 years). A total of 65% were males and 65% were African American. Management was tailored based on clinical assessment and diagnostic studies, including direct laryngoscopy/bronchoscopy (4), pulmonary function tests (PFT's 9), esophagogastroduodenoscopy (9), and flexible bronchoscopy/lavage (9). Following ADC management, changes in physical, social and psychological domain scores of the PC-QOL-27 each met the threshold for minimal clinical important difference (MCID) indicating a clinically meaningful improvement. Improvements were most notable in the physical domain where post survey scores significantly improved from pre-survey scores (P =.009) regardless of age, gender, ethnicity, history of endoscopy and season. Conclusions: The physical impact of chronic cough in pediatric patients who failed prior management by a single specialist was lessened by an ADC team approach to management. [ABSTRACT FROM AUTHOR]