Robert H. Chun

Initiation and Use of Propranolol for Infantile Hemangioma: Report of a Consensus Conference

Infantile hemangiomas (IHs) are common neoplasms composed of proliferating endothelial-like cells. Despite the relative frequency of IH and the potential severity of complications, there are currently no uniform guidelines for treatment. Although propranolol has rapidly been adopted, there is significant uncertainty and divergence of opinion regarding safety monitoring, dose escalation, and its use in PHACE syndrome (PHACE = posterior fossa, hemangioma, arterial lesions, cardiac abnormalities, eye abnormalities; a cutaneous neurovascular syndrome characterized by large, segmental hemangiomas of the head and neck along with congenital anomalies of the brain, heart, eyes and/or chest wall). A consensus conference was held on December 9, 2011. The multidisciplinary team reviewed existing data on the pharmacologic properties of propranolol and all published reports pertaining to the use of propranolol in pediatric patients. Workgroups were assigned specific topics to propose protocols on the following subjects: contraindications, special populations, pretreatment evaluation, dose escalation, and monitoring. Consensus protocols were recorded during the meeting and refined after the meeting. When appropriate, protocol clarifications and revision were made and agreed upon by the group via teleconference. Because of the absence of high-quality clinical research data, evidence-based recommendations are not possible at present. However, the team agreed on a number of recommendations that arose from a review of existing evidence, including when to treat complicated IH; contraindications and pretreatment evaluation protocols; propranolol use in PHACE syndrome; formulation, target dose, and frequency of propranolol; initiation of propranolol in infants; cardiovascular monitoring; ongoing monitoring; and prevention of hypoglycemia. Where there was considerable controversy, the more conservative approach was selected. We acknowledge that the recommendations are conservative in nature and anticipate that they will be revised as more data are made available.

Standardized Outcome and Reporting Measures in Pediatric Head and Neck Lymphatic Malformations

Objective To develop general and site-specific treatment effect and outcome measures to standardize the reporting of head and neck lymphatic malformation (HNLM) treatments. Study Design Consensus statement/expert opinion. Setting Multiple tertiary academic institutions. Subjects and Methods The modified Delphi method is an iterative process of collecting expert opinions, refining opinions through discussion and feedback, statistically aggregating opinions, and using these aggregates to generate consensus opinion in the absence of other data. The modified Delphi method was used by a multi-institutional group of otolaryngology and interventional radiology experts in the field of vascular anomalies to formulate a list of recommended reporting outcomes for the study and treatment of head and neck lymphatic malformations. Results Through 3 rounds of iteration, 10 expert panelists refined 98 proposed outcome measures and 9 outcome categories to a final consensus set of 50 recommended outcome measures in 3 global categories (general, demographics, and treatment complications) and 5 site-specific categories (orbit, oral cavity, pharynx, larynx, and neck). Conclusions We propose the first consensus set of standardized reporting measures for clinical and treatment outcomes in studies of HNLMs. Consistent outcome measures across future studies will facilitate comparison of treatment options and allow systematic review. We hope that these guidelines facilitate the design and reporting of subsequent HNLM studies.

Mediastinitis in infants from deep neck space infections.

Objective: To describe mediastinitis in infants. Study Design: Case series. Setting: Tertiary-care, academic, pediatric hospital. Subjects: Four infants managed during eight months. Results: Patients were eight months (two patients), nine months, and 18 months old. They had retropharyngeal phlegmons and were started on clindamycin/cephalosporin. Interval imaging scans revealed abscess formation with mediastinal extension. All retropharyngeal abscesses were drained; three patients required video-assisted thoracoscopic debridement. Methicillin-resistant Staphylococcus aureus (two patients) and methicillin-sensitive Staphylococcus aureus (two patients) were cultured. Peak white blood cell counts were 15,000, 18,400, 30,200, and 44,900. The patients survived without long-term morbidity. Conclusions: In infants with pediatric deep neck space infections, increased vigilance should be maintained for progression to mediastinal extension. Optimal management includes imaging of the neck and chest as indicated, broad-spectrum antibiotic coverage, and aggressive surgery as warranted to clear the neck space infections with appropriate surgical consultants to manage the mediastinal process. Despite high morbidity and mortality in adult mediastinitis, infant mediastinitis may be a different disease process as all patients survived without sequelae.

Utility of Bronchoscopy for Recurrent Croup

Objectives: We performed a retrospective review to determine the utility of bronchoscopy in patients with recurrent croup (RC). Methods: Bronchoscopy was performed on 30 patients with a diagnosis of RC (age, 14 months to 13.9 years) over a 2-year period. Results: One third of the patients (33.3%) were found to have airway disorders, including subglottic stenosis (7), subglottic edema (2), and a subglottic cyst (1). Patients with RC who were less than 3 years of age were more likely to have an airway abnormality found on endoscopy (9 of 14 or 64.2%) than were those older than 3 years (1 of 16 or 6.2%; χ2, p < 0.001). There was no statistically significant difference in abnormal findings 1) in patients with RC who had a history of prematurity or prior intubations (χ2, p = 0.17 and p = 0.052, respectively); 2) between infectious and spasmodic croup (χ2, p = 0.794); or 3) by number of croup episodes (χ2, p = 0.300). Two patients required surgical intervention (laryngotracheal reconstruction and marsupialization of a subglottic cyst). Conclusions: Of 30 patients who underwent bronchoscopy for RC, 33% had airway disorders — Mostly children less than 3 years old. We suggest a higher index of suspicion for finding airway disorders in children less than 3 years old with RC and having a lower threshold for performing diagnostic bronchoscopy in this population.

Transient swallowing dysfunction in typically developing children following supraglottoplasty for laryngomalacia.

OBJECTIVE To evaluate early postoperative swallowing function in otherwise typically-developing children following supraglottoplasty. METHODS Retrospective chart review case series. RESULTS Of 37 children identified as having undergone supraglottoplasty for severe laryngomalacia at our institution between January 2007 and October 2011, 24 were identified as otherwise typically developing with no indications/signs of swallowing problems pre-operatively and eligible for inclusion in this study. Twenty-two children underwent bilateral supraglottoplasty and 2 children underwent unilateral supraglottoplasty using the CO2 laser or the laryngeal skimmer microdebrider in combination with cold steel technique, based on the discretion of four Pediatric Otolaryngologists. Seventeen children were seen post-operatively for a clinical swallow evaluation. Six children were found to have swallowing dysfunction. Four of the six children showed variable symptoms, signs, or findings concerning for aspiration with oral feeding. Three of six underwent video fluoroscopic swallow study (VFSS). All 6 children responded to dietary modifications, positioning alterations, and anti-reflux medications. All showed improvement by their 4-week post-operative follow-up visit. The median age of the 24 patients at the time of surgery was 3 months, with a range from 2 weeks to 4 years. The median age of the patients found to have transient post-operative swallowing dysfunction was 1.5 months, with a range of 2 weeks to 4 months. There was no association between post-operative swallowing dysfunction and the surgical technique employed. CONCLUSIONS Supraglottoplasty in otherwise typically developing children carries a risk of transient swallowing dysfunction with a low risk of persistent dysfunction. Most patients can be assessed post-operatively via clinical swallow evaluation without requiring an instrumental swallow study.

Choosing a fellow or fellowship: A survey of pediatric otolaryngologists

IMPORTANCE The numbers of pediatric otolaryngology fellowship programs and applicants have increased over the past 5 years. However, the qualities desired in programs and applicants have not been explored. OBJECTIVE To determine the factors that fellowship program directors and applicants believe to be most important in choosing a fellow and the factors most important to fellowship applicants in choosing a program. DESIGN, SETTING, AND PARTICIPANTS Cohort study using an anonymous online survey of 2012 pediatric otolaryngology fellowship program directors and applicants. Respondents were asked to rank a list of 10 qualities from most to least important for judging the strength of a fellowship applicant. Applicants also assessed the importance of factors in choosing a fellowship. MAIN OUTCOMES AND MEASURES Rank of each factor by members of each group. RESULTS Thirty-two of 47 applicants (68%) and 15 of 31 fellowship directors (48%) completed the survey. For applicants, the most important factors when choosing a fellowship program were gaining strong experience in airway management and otology, faculty reputation, and location, whereas Accreditation Council for Graduate Medical Education (ACGME) accreditation, fellowship longevity, and salary were less important. For choosing an applicant, applicants indicated that the interview, prior applicant knowledge (trusted recommendation), and letters of recommendation, sequentially, should be given the greatest weight. Directors reported that they used the same top 3 factors to rank applicants, but knowledge or trusted recommendation of the applicant ranked first. Applicants who successfully matched interviewed at (mean, 9.5 vs 3.0; P = .003), applied at (mean, 11.6 vs 4.3; P = .02), and ranked (mean, 8.3 vs 2.3; P < .001) more fellowship programs than those who did not. United States Medical Licensing Examination scores higher than 230 and AΩA membership status did not significantly affect fellowship match. CONCLUSIONS AND RELEVANCE Personal knowledge or a trusted colleagues recommendation may be the most important determinant when pediatric otolaryngology fellowship programs choose an applicant. When fellows choose a program, the opportunity to gain surgical experience in both otology and airway management is crucial, but ACGME accreditation status seems less important. Successful applicants ranked and interviewed at more fellowship programs than nonmatching applicants.

Endoscopic cricopharyngeal myotomy for management of cricopharyngeal achalasia (CA) in an 18–month–old child†‡

A 6–month–old patient presented with dysphagia and failure to thrive. Video fluoroscopic swallow study (VFSS), esophagogastroduodenoscopy, and manometry were diagnostic for CA. A gastrostomy tube was placed at 8 months. Botulinum toxin injection improved symptoms, but within 10 weeks symptoms returned.

Otolaryngologic Surgeries Are Frequent in Children With Eosinophilic Esophagitis

Objective: The aim was to study the prevalence of otolaryngologic surgeries in pediatric patients with eosinophilic esophagitis (EoE). Methods: Retrospective cohort study at a tertiary care center. The type of otolaryngologic surgeries performed in patients with diagnosis of EoE was recorded during a 5-year period. Results: Seventy-five percent of patients were male, with average age of EoE diagnosis at 7.5 years with an 83% incidence of atopy. Cohort analysis revealed that 33% (119/362) had a total of 275 otolaryngologic surgeries. Surgeries performed on 119 patients are as follows: 20% bilateral myringotomy with tubes, 14% tonsillectomy, 18.5% adenoidectomy, 1.4% sinus irrigation, 3.3% bronchoscopy, and 1.4% laryngotracheoplasty (LTP); 63% of patients underwent multiple procedures. Thirty percent of patients undergoing bilateral myringotomy with tube placement (BMT) needed additional tubes. Four of 5 LTP patients had successful operations. Twelve percent of patients had EoE diagnosis prior to an otolaryngologic surgery. Conclusion: Thirty-three percent of children with EoE required otolaryngologic surgical intervention and nearly one-third who underwent BMT required additional ear tubes. A large fraction of children with EoE will undergo an otolaryngologic surgery, only a minority with a preoperative EoE diagnosis. Until the nature of this relationship is clarified, the high coincidence with otolaryngologic surgeries dictates that otolaryngologists should be familiar with diagnosis of EoE in patients.

Surgical treatment of infantile hemangioma in a multidisciplinary vascular anomalies clinic

OBJECTIVE (1) Discuss indications for surgical treatment of infantile hemangioma (IH); (2) describe outcomes, management of complications and long term surveillance of surgically treated IH. STUDY DESIGN Retrospective. SUBJECTS AND METHODS The charts of children seen in a dedicated vascular anomalies center at a tertiary pediatric hospital were reviewed. RESULTS Out of 1012 patients diagnosed with IH over 27 months, 92 patients, predominantly caucasian female, with an average age of 36 months, underwent surgery for 94 lesions. Head and neck lesions accounted for 67% of the population and 51 lesions were located on the face. Surgical indications included bleeding, functional impairment, ulceration and cosmetic disfigurement. Mean surface area was 7.75 cm². Although lesions requiring additional procedures were larger (median 5.5 cm²) than single-stage excisions, this difference was not statistically significant (P=0.09). Head and neck lesions were more likely to require multiple modality treatment (P=0.003). There was no identified objective criteria to predict head and neck lesions that are more likely to be associated post-operative complications. CONCLUSIONS Most IH do not require treatment. The majority of infantile hemangioma occurred on the head and neck. When significant functional impairment, ulceration, bleeding, cosmetic deformity is encountered or anticipated, surgical therapy can be performed at any clinical phase with few complications and favorable outcomes.

Management of Plastic Bronchitis in a Child with Mild Intermittent Asthma

Objectives: We describe the clinical presentation of a 7 1/2-year-old boy with a history of mild intermittent asthma who presented with left lung collapse and was found to have plastic bronchitis. Methods: We reviewed the patient chart and imaging results and performed a literature review of plastic bronchitis and its management. Results: Bronchoscopy in our patient demonstrated a large white, friable, cast-like material that obstructed the entire left main stem bronchus and could not be easily suctioned. The cast was removed in a piecemeal fashion by means of serial rigid bronchoscopy over a 6-month period with use of both optical forceps and flexible suction catheters. Microscopic examination of the cast-like material showed a predominance of eosinophils along with neutrophils encased in proteinaceous material. Conclusions: Plastic bronchitis in children is a rare condition that can mimic foreign body aspiration and can be associated with underlying pulmonary inflammatory disorders or cardiovascular disease. Aggressive bronchoscopic management of the airway obstruction and medical management of the underlying disease process are important for the successful treatment of plastic bronchitis.