Pre-Operative Characteristics Helping to Avoid Gastrostomy Tube After Mandibular Distraction in Neonates With Pierre-Robin Sequence: A Institutional Case-Series and Review of the Literature.

OBJECTIVE: to investigate the ability of mandibular distraction osteogenesis (MDO) to avoid gastrostomy tube (G-tube). DATA SOURCES: PubMed, EBSCOhost, Cochrane, and Embase. REVIEW METHODS: We retrospectively reviewed the number of MDO cases performed at our institution for patients with Robin Sequence (RS) over the past 10 years. In our institutional review, patients were excluded if they had a G-tube already placed at the time of surgery. We also performed a systematic review of the literature. Articles were excluded if they did not detail feeding outcomes after MDO, or if MDO was performed on patients that did not have RS. RESULTS: In our systematic review, 12 articles were included that comprised a total of 209 neonates with RS that underwent MDO. A total of 174 (83.3%) patients avoided a G-tube once MDO was performed. A total of 14 patients met the inclusion criteria at our institution. Of the 14 RS patients, 9 (64%) avoided having a G-tube placed and all (14/14) avoided tracheostomy. The average birth weight of patients avoiding a G-tube was 3.11 kg compared to 2.25 kg (P = .045) in the group requiring a G-tube. In the group avoiding a G-tube, the average weight at time of operation was 3.46 kg compared to 2.83 kg (P = .037) in the group requiring a G-tube. CONCLUSION: MDO may be considered as a surgical option to prevent G-tube placement for neonates with non-syndromic RS who have difficulty with PO feeding but whose airway obstruction is not severe enough to require respiratory support. Based on our institutional experience, a minimum weight of 3.00 kg correlated with higher success rates of PO intake and avoiding a G-tube.

National Trends of Otolaryngology Involvement in Cleft Surgical Management over 10-Years.

OBJECTIVE: Surgical management of cleft lip/palate and cleft rhinoplasty have historically been performed by plastic surgeons. No study has addressed temporal trends in cleft-associated surgeries. This study assesses trends in cleft surgical management and complications in a national database. METHODS: Cross-sectional analysis of the National Surgical Quality Improvement Program Pediatric database from 2012 to 2021. Patients receiving cleft lip and/or palate repair were isolated using CPT codes. A subset receiving cleft rhinoplasty was also analyzed. The yearly proportion of otolaryngologists compared to general plastic surgeons performing surgeries was noted. Regression analysis was used to identify trends and predictors of management by OHNS. RESULTS: We identified 46,618 cases of cleft repair, of which 15.6% (N = 7,255) underwent repair with otolaryngology. On univariate Pearson correlation analysis, neither cleft rhinoplasties performed by OHNS over time (R = 0.371, 95% CI -0.337 to 0.811, p = 0.2907) nor all cases (R = -0.26, -0.76 to 0.44, p = 0.465) exhibited a significant change. On multivariable regression, the operative year was not associated with being treated by otolaryngology (p = 0.826) for all cleft cases but was associated with such in cleft rhinoplasties (OR 1.04, 1.01-1.08, p = 0.024). On multivariable analysis, the operative year was correlated with a higher rate of complications overall (OR 1.04, 1.01-1.07, p = 0.002). Surgeon specialty was not associated with complication rates. CONCLUSIONS: In the last 10 years, no change in the proportion of cleft lip/palate repair performed by OHNS was observed. Otolaryngologists are performing more cleft rhinoplasty but at a marginal rate. Otolaryngologists also manage more complex patients with multiple comorbidities compared to their colleagues. Complication rates have increased overall regardless of surgeon specialty, warranting further investigation. LEVEL OF EVIDENCE: 3 Laryngoscope, 134:671-677, 2024.

An Enhanced Audiologic Protocol for Early Identification of Conductive Hearing Loss in Patients with Cleft Palate.

OBJECTIVE: To characterize the onset and prevalence of conductive hearing loss (CHL) in pediatric patients with cleft palate (CP) prior to palatoplasty with an enhanced audiologic protocol. DESIGN: Retrospective cohort study. SETTING: Multidisciplinary cleft and craniofacial clinic at a tertiary care center. PATIENTS: Patients with CP who received audiologic workup pre-operatively. Patients with bilateral permanent hearing loss, expiration prior to palatoplasty, or no pre-operative data were excluded. INTERVENTIONS: Patients with CP born February 2019 to November 2019 who passed newborn hearing screening (NBHS) received audiologic testing at 9 months of age (standard protocol). Patients born December 2019 to September 2020 underwent testing prior to 9 months of age (enhanced protocol). MAIN OUTCOME MEASURES: Age of identification of CHL in patients after implementation of the enhanced audiologic protocol. RESULTS: The number of patients who passed their NBHS in the standard protocol (n = 14, 54%) and the enhanced protocol (n = 25, 66%) did not differ. Infants who passed their NBHS, but demonstrated hearing loss on subsequent audiologic testing did not differ between enhanced (n = 25, 66%) and standard cohort (n = 14, 54%). Of patients who passed NBHS in the enhanced protocol, 48% (n = 12) had CHL identified by 3 months, and 20% (n = 5) by 6 months of age. With the enhanced protocol, patients who did not undergo additional testing post NBHS significantly dropped from 44.9% (n = 22) to 4.2% (n = 2) (P < .0001). CONCLUSION: Even with passed NBHS, CHL is still present for infants with CP pre-operatively. Earlier and more frequent testing for this population is recommended.

Update on Vascular Anomalies of the Head and Neck.

Vascular anomalies of the head and neck is an evolving field, with more recent focus on identifying and understanding the underlying genetic and molecular causes for these lesions. Medical therapies for some of these vascular anomalies have been developed. Many complex vascular anomalies require multimodality therapy, and other lesions could be treated with any of a variety of the available therapies. High-quality outcomes research and establishing clinical practice guidelines to help guide management are essential.

Clinical Response to PI3K-α Inhibition in a Cohort of Children and Adults With PIK3CA-Related Overgrowth Spectrum Disorders.

Objective: The goal of this report is to describe, through a series of 5 cases, the clinical response and safety of alpelisib (BYL719) use in children and adults with PIK3CA-related overgrowth spectrum (PROS) disorders at our center. Methods: We reviewed clinical records of 5 patients from October 2019 through September 2021 followed by the pediatric hematology and multidisciplinary vascular anomalies teams at the Monroe Carell Jr. Children's Hospital at Vanderbilt (MCJCHV). All patients carried a clinical or genetic diagnosis of PROS and were treated with alpelisib provided by a Novartis managed access program. Results: We highlight improvement in reported symptoms, objective overgrowth measurements, and quality of life to varying degrees in all patients. We note dose-dependent hyperglycemia and gastrointestinal side effects in 2 of the 5 patients. No patients experienced any serious side effects. Conclusion: This case series reports on the real-world use of PI3K-α inhibition in the management of PROS. Ongoing clinical trials will provide efficacy and safety data as these drugs become more widely used in patients with vascular anomalies and syndromes secondary to somatic PIK3CA mutations.

How we established a multidisciplinary program for vascular anomalies.

The care of patients with vascular anomalies is quickly becoming a complex field requiring high-quality, coordinated multidisciplinary care. In this article, we review the history of multidisciplinary care in this field, discuss the benefits of this model of care, and outline some of the essential components and structure of a successful vascular anomalies team. We provide an overview of two example programs and a roadmap for other centers to develop their own multidisciplinary vascular anomalies teams.

Utilization of part C early intervention services for patients with cleft palate.

OBJECTIVES: To investigate the utilization of early intervention services under Part C of the 2004 Individuals with Disabilities Educational Act for children with cleft palate and to better understand barriers these patients encounter when receiving services. METHODS: A retrospective chart review was performed on children under the age of 18 who were treated for cleft palate at a single tertiary care center and referred to Tennessee's Early Intervention System (TEIS) between January 2007 and December 2018. RESULTS: For the 61 patients included, developmental therapy was the most common TEIS referral made (n = 408, 28%), followed by speech therapy (n = 371, 26%). Most of these services were delivered as scheduled (n = 513, 80%); some families missed appointments without notifying the provider (i.e., family no-show)(n = 101, 2%). Children referred at a younger age were more likely to receive developmental therapy (p = 0.012) and to attend their services (p = 0.027). Patients with Medicaid were more likely to have absences with prior notification (p = 0.05) and without prior notification (i.e., family no-show)(p = 0.009) than patients with other types of health insurance. CONCLUSIONS: Patients with cleft palate often have complex needs; earlier referral to ancillary services may improve attendance at appointments and impact the services they receive. Socioeconomic factors may hinder patients from accessing these services even once they are referred.

Clinical Significance of Screening Electrocardiograms for the Administration of Propranolol for Problematic Infantile Hemangiomas.

OBJECTIVE: Low-dose nonselective ß blockade is an effective treatment for problematic infantile hemangioma (PIH). Screening electrocardiograms (ECG) are performed prior to the initiation of propranolol to minimize the risk of exacerbating undiagnosed heart block. How ECG results affect subsequent propranolol usage and patient management remains unclear. We examined the value of ECG prior to propranolol therapy in a quaternary pediatric hospital. METHODS: A retrospective chart review was performed on all infants who received propranolol (2 mg/kg/day divided three times daily) to treat PIH at Arkansas Children's Hospital from Sept. 2008 to Sept. 2015. All available demographic, historical, and clinical data were obtained. ECGs and echocardiographic data were reviewed and summarized. A pediatric cardiologist read all ECGs. RESULTS: A total of 333 patients (75% female) received propranolol therapy. ECG information was available for 317 (95%). Abnormal findings were present on 44/317 (13.9%) of study ECGs. The most common abnormal finding was "voltage criteria for ventricular hypertrophy" (n = 35, 76.1%). Two patients had abnormal rhythms; one had first-degree atrioventricular (AV) block, and one had occasional premature atrial contractions. Of the 31 patients who underwent echocardiograms, 20 (35%) were abnormal. 2.9% of infants with PIH treated with propranolol required a follow-up with a cardiologist. No patient was precluded from taking propranolol due to the findings on screening ECG. CONCLUSIONS: Screening ECGs prior to propranolol therapy are abnormal in nearly 14% of patients with PIH but are unlikely to preclude therapy. In the absence of prior cardiac history, this cohort offers further evidence suggesting that screening ECGs may be of limited value in determining the safety of propranolol in otherwise healthy infants with PIH.

Evaluating the Clinical Outcomes of Parotid Hemangiomas in the Pediatric Patient Population.

INTRODUCTION: Infantile hemangiomas (IHs) are the most common tumor of the parotid gland in children; however, there is no standard protocol for the treatment of IH. The generally accepted practice is to begin the patient on ß-blocker therapy if there are no contraindications. OBJECTIVE: The purpose of this study is to better understand the challenges and successes of management of pediatric patients with parotid IH. METHODS: This retrospective study analyzed 15 patients diagnosed with parotid IH from 2009 to 2016 who were cared for at a tertiary care center. Demographic information, lesion characteristics, and treatment course were obtained through patient chart review. RESULTS: Fifteen pediatric patients with parotid IH were evaluated. The female:male ratio was 4:1; the average age of diagnosis was 8.75 months. Most lesions were greater than 3 cm in their widest dimension (73.3%), and 13 patients underwent imaging to further clarify the parotid mass in their clinical workup. Fourteen patients began treatment with propranolol; 10 patients saw complete resolution of their IH (66.7%) and 3 had a partial response to ß-blocker therapy (20%). After discontinuation of propranolol, 2 patients had regrowth 2 to 3 months later after regression and were restarted on therapy. The average duration of treatment was 9.9 ± 8.45 months. The known adverse effects of propranolol-hypoglycemia, hypotension, bradycardia, and bronchospasm-were not observed in any patient. CONCLUSION: In the treatment of parotid IH, propranolol is the generally accepted first-line therapy, as compared to corticosteroid or interferon α injections of years past. Parotid hemangiomas, however, have a lower response rate to propranolol and a similar recurrence rate compared to IH at other sites. The treatment duration necessary tends to be longer. Future studies will aim at identifying and evaluating potential predictors of outcomes to help inform the management of parotid hemangiomas.

Total facial nerve injury during mandibular distraction osteogenesis.

INTRODUCTION: Over the last 10-15 years, usage of internal mandibular distraction systems has increased in the pediatric population, particularly for craniofacial syndromes. Mandibular distraction osteogenesis (MDO) has been shown to be effective in avoiding tracheostomy or achieving early decannulation in patients with micro-retrognathic mandibles in hemifacial microsomia or Pierre Robin sequence. As the frequency of the application of MDO has increased, so has the awareness and management of subsequent complications from the procedure. In this study, we discuss a complication involving paresis and eventual recovery of cranial nerve (CN) VII after the application of an MDO internal device at our institution in two cases. We also review the literature and propose multiple anatomic considerations that can impact more than just the marginal branch of CN VII. METHOD: This study is a retrospective case study from our institution and a review of the literature. Pubmed was queried for terms singularly and in combination including "mandibular distraction osteogenesis", "facial nerve", "cranial nerve", "complications", "micrognathia", "retrognathia". After reviewing the results, studies discussing complications of MDO that involved CN VII were reviewed and included. RESULTS: In the literature review and our retrospective review, CN VII injuries from MDO vary in their length and timing of onset. Management of this complication depended on the timing of onset and ranged from conservative management to removal of the distraction device. Majority (7/9) of the cases resolved to an eventual House-Brackmann of 0/6 with conservative measures. CONCLUSION: Total facial nerve injury in association with MDO, has been scarcely reported, though the facial nerve is at great risk given its intimate location near the mandible particularly in neonates. It is encouraging that though it could be a devastating complication, out of all the cases reported, the large majority resolve with a combination of time and steroids.

Expression of ß-Adrenergic Receptor Subtypes in Proliferative, Involuted, and Propranolol-Responsive Infantile Hemangiomas.

IMPORTANCE: Propranolol hydrochloride has become the primary medical treatment for problematic infantile hemangioma; however, the expression of propranolol's target receptors during growth, involution, and treatment of hemangioma remains unclear. OBJECTIVE: To measure and compare the expression of ß1-, ß2-, and ß3-adrenergic receptors (ADBR1, ADBR2, and ADBR3, respectively) in proliferative (n = 10), involuted (n = 11), and propranolol-responsive (n = 12) hemangioma tissue. DESIGN, SETTING, AND PARTICIPANTS: Infantile hemangioma specimens were harvested for molecular investigation. Messenger RNA (mRNA) expression of the ADBR1, ADBR2, and ADBR3 genes was detected by real-time polymerase chain reaction. Protein level expression was measured by Western blot and standardized with densitometry. A total of 33 specimens were collected from patients in a tertiary pediatric hospital who underwent excision of problematic hemangiomas. This study was conducted from January 18, 2011, to September 24, 2013, and data analysis was performed from February 25, 2015, to June 25, 2016. RESULTS: Of the 33 patients included, 21 were female (64%). The mean (SD) patient age at the time of excision was 7 (2.5) months for the proliferative group lesions, 23.5 (10) months for the involuted group, and 16 (10) months for the propranolol group. The mean level of ADBR1 mRNA expression was significantly higher in proliferative hemangioma than in propranolol-responsive hemangioma (1.05 [0.56] vs 0.52 [0.36]; P = .01; 95% CI, 0.12-0.94). There was no difference in ADBR2 expression among the groups. Protein expression of ADBR3 was significantly higher in involuted (0.64 [0.12] vs 0.26 [0.04]; P < .01; 95% CI, 0.26-0.49) and propranolol-responsive hemangioma (0.66 [0.31] vs 0.26 [0.04]; P = .01; 95% CI, 0.16-0.68) compared with proliferative hemangioma. CONCLUSIONS AND RELEVANCE: These data demonstrate the variable expression of ADBR subtypes among infantile hemangiomas during growth, involution, and response to treatment. These findings may have clinical implications regarding the use of selective vs nonselective ß-blockade. LEVEL OF EVIDENCE: 2.

Subpicometer length measurement using semiconductor laser tracking frequency gauge.

We have demonstrated heretofore unattained distance precision of 0.14 pm (2 pm) incremental and 14 nm (2.9 µm) absolute in a resonant (nonresonant) interferometer at an averaging time of 1 s, using inexpensive telecommunications diode lasers. We have controlled the main source of error, that due to spurious reflection and the resulting amplitude modulation. In the resonant interferometer, absolute distance precision is well under λ/6. Therefore, after an interruption, an absolute distance measurement can be used to return to the same interferometer order.