Stephen R. Hoff

Supraglottoplasty outcomes in relation to age and comorbid conditions

OBJECTIVE To determine if age and comorbid conditions effect outcomes in children undergoing supraglottoplasty for severe laryngomalacia. DESIGN Retrospective study. SETTING Urban tertiary-care childrens hospital. PATIENTS Children undergoing supraglottoplasty for severe laryngomalacia between February 2004 and July 2008. 56 patients were identified. OUTCOME MEASURES Persistence of upper airway obstruction, revision surgery (supraglottoplasty), and additional surgery (tracheostomy). RESULTS 33/56 (58.9%) patients had no comorbid conditions and 23/56 (41.1%) patients had comorbid conditions. In noncomorbid patients, 36.4% of those less than 2 months of age at the time of surgery required revision supraglottoplasty, compared to 5.3% of patients between 2 and 10 months (p<0.05). Compared to the 2-10-month age group, there was a significantly higher percentage of patients with comorbid conditions in the >10-month group (32.1% vs. 79%, p<0.01). Patients with comorbid conditions were diagnosed at a significantly later age than those without (6 mo vs. 2 mo, respectively), and had significantly higher rates of revision supraglottoplasty (47.8% vs. 18.2%) and tracheostomy (39.1% vs. 0.0%). 70% of children with neurological conditions required revision surgery, with 60% requiring tracheostomy. The revision surgery and tracheostomy rates were significantly higher compared to the noncomorbid group (p<0.01 and p<0.0001). Children with cardiac conditions had a higher rate of tracheostomy than noncomorbid children (30% vs. 0%, p<0.01). 16.7% of children with genetic conditions required supraglottoplasty, and none required tracheostomy. CONCLUSIONS In noncomorbid patients, those undergoing supraglottoplasty less than 2 months of age had a significantly higher rate of revision supraglottoplasty. Patients with neurologic and cardiac comorbidities require tracheostomy at a significantly higher rate than noncomorbid patients.

Aspiration before and after Supraglottoplasty regardless of Technique

Objective. To determine the incidence of preoperative and postoperative aspiration in infants who undergo supraglottoplasty. To determine the effect of cold steel and CO2 laser supraglottoplasty on aspiration in infants with severe laryngomalacia. Design. Retrospective study. Setting. Tertiary pediatric hospital. Patients. Thirty-nine patients who underwent CO2 laser-assisted supraglottoplasty (CLS) or cold steel supraglottoplasty (CSS) for severe laryngomalacia. Main Outcome Measures. Aspiration and upper-airway obstruction. Results. Thirty-nine patients met inclusion criteria (18 males, 21 females). Eighteen patients underwent CSS and 21 patients underwent CLS. 10/39 (25.6%) of the patients had preoperative aspiration, and 2/10 (20%) resolved after supraglottoplasty. New onset aspiration was found in 4/13 (30.8%) in the CSS group and 9/16 (56.3%) in the CLS group. Conclusions. There is no significant difference in the rate of postoperative new-onset aspiration or relief of upper-airway obstruction in the CLS or CSS, is temporary and can be managed with thickened diet or temporary tube feedings. The rate of persistent postoperative aspiration was statistically similar regardless of the method of surgery.

Head and Neck Vascular Lesions

Vascular lesions of the head and neck are complex and diverse. These include infantile hemangioma, venous malformations, lymphatic malformations, and arteriovenous malformations, among others. Vascular malformations and tumors display different growth patterns and require different approaches to treatment. Therefore, accurate diagnosis is of utmost importance. This article is a guide for the diagnosis and management of vascular lesions of the head and neck.

Management of acute complicated mastoiditis at an urban, tertiary care pediatric hospital

To describe the presentation and management of acute complicated mastoiditis in children.

Endoscopic transcanal approach to the middle ear for management of pediatric cholesteatoma

To describe outcomes for endoscopic ear surgery (EES) for pediatric cholesteatoma at a tertiary pediatric hospital.

Management of hearing loss and the normal ear in cases of unilateral Microtia with aural atresia

To identify the rate of hearing loss related to middle ear disease and the frequency of tympanostomy tube (TT) insertion in the contralateral ear of patients with unilateral microtia/aural atresia.

Transcutaneous Osseointegrated Implants for Pediatric Patients With Aural Atresia

Importance Patients with aural atresia typically have maximal conductive hearing loss, which can have negative academic and social consequences. Transcutaneous osseointegrated implants (TOIs) can potentially restore hearing on the affected side. Objectives To review the demographic, audiological, and surgical outcomes of TOI placement in pediatric patients with aural atresia and to describe a modification in incision technique in anticipation of later auricular reconstruction. Design, Setting, and Participants This retrospective case series reviewed 41 cases of TOI placement in pediatric patients between January 1, 2014, and September 30, 2016, at Lurie Children’s Microtia and Aural Atresia Clinic. Patients, all younger than 18 years and with atresia or microtia, received at least 6 months of follow-up and underwent testing before and after surgery. Main Outcomes and Measures Patient age, indication for procedure, ear sidedness, case length, incision type, complications, and other postoperative events. Audiological outcomes before and after implantation were measured using pure-tone averages and the Hearing In Noise Test for Children, presented in variable signal to noise ratios. Results In total, 46 TOIs were performed in 38 pediatric patients, but only 41 implantations in 34 patients were included in this study. Of the 34 patients, 13 (38%) were males and 21 (62%) were females, with a mean age of 8.9 (range, 5-17) years at the time of TOI placement. Microtia on the implanted side was present in 39 cases (95%). A modified posterior-superior scalp incision technique was used in 30 (73%) of 41 ears, all in cases of microtia. One perioperative surgical complication occurred: a seroma requiring drainage. Two patients developed minor skin irritation and erythema at the magnet site related to the overnight use of the processor, which resolved when removed while sleeping. The mean (SD; range) score for the Speech In Noise test at 5 dB signal to noise ratio improved from 75.3% (14.4%; range, 50%-92%) correct in unaided/preoperative condition to 93.6% (6.95%; range, 80%-100%) correct in the aided/postoperative condition. The mean improvement in score was 18.3% (95% CI, 10.8%-25.9%), with an effect size of 1.62 (95% CI, 0.95-2.29). The mean pure-tone averages (SD; range) similarly improved from 63.7 (13.2; range, 25-11) dB to 9.6 (4.9; range, 5-15) dB. Conclusions and Relevance Transcutaneous osseointegrated implantation has a low complication rate among pediatric patients with atresia or microtia and can provide excellent audiological results. It should be included as a treatment option for this population of patients who meet audiological criteria.

Outcomes and Time to Emergence of Auditory Skills after Cochlear Implantation of Children with Charge Syndrome

OBJECTIVE Review perioperative complications, benefits, and the timeframe over which auditory skills develop in children with CHARGE syndrome who receive a cochlear implant (CI). STUDY DESIGN IRB-approved retrospective chart review of children with CHARGE syndrome who had at least 12 months of cochlear implant use. SETTING Tertiary care childrens hospital. PATIENTS Twelve children, seven males and five females. Mean age implant = 3.5 years (1.7-8.2 yr); mean duration follow-up = 4.7 years (1.5-10.1 yr). INTERVENTION Cochlear implantation. MAIN OUTCOME MEASURES Auditory skills categorized into four levels, temporal bone imaging findings, perioperative complications, time to emergence of speech perception, expressive communication mode. RESULTS All children imaged with magnetic resonance imaging had cochlear nerve deficiency in at least one ear. Speech awareness threshold improved with the CI compared with aided preoperative in 83% of children, with means of 51.7 dB SAT preoperative and 27.1 dB with the CI (p ≤ 0.002). Overall, four children improved to auditory Level 2 (improved detection), three obtained Level 3 (closed-set speech perception), and three had open-set speech perception with their CIs (Level 4) that was first evident at 3.5, 3.3, and 0.8 years postimplant testing. Two children had minimal or limited improvement. One child with hypoplasia of the cochlear nerve obtained open-set levels. CONCLUSION Auditory skills may develop slowly in children with CHARGE syndrome who receive a CI but most can achieve at least improved detection. In our series, half acquired some speech perception ability. Cochlear nerve deficiency is frequent, but should not be a contraindication to implantation.

The transpalatal approach to repair of congenital Basal skull base cephaloceles.

Basal skull base herniations, including meningoceles and encephaloceles, are rare and may present with characteristic facial and neurologic features. The traditional craniotomy approach has known morbidity, and nasal endoscopy may not allow for control of large posterior basal defects, especially in newborns. We present two cases of successful repair of basal transsphenoidal meningoceles using an oral-transpalatal approach. The first patient with an intact palate presented with respiratory distress, and a palatectomy was performed for access to the skull base. The second patient had a large basal herniation that was reduced through a congenital midline cleft palate, and a calvarial bone graft was used to repair the defect. A literature search revealed 10 previous successful cases using the transpalatal repair, which allows for excellent access, low morbidity, and a team-oriented method to skull base surgery.

The Association between Sleep Duration and Metabolic Variables in Obese Children with Obstructive Sleep Apnea

Objectives: Sleep loss is problematic in adults and children. Decline in sleep duration has paralleled an increase in the prevalence of obesity and diabetes. The aim of this study was to analyze the association between sleep duration, severity of obstructive sleep apnea (OSA), and metabolic variables in obese children. Methods: In this study, patients between the ages of 2 and 12 years who had overnight polysomnography (PSG) at a tertiary care medical center were included. Patients were identified from a xenobase search of medical records. All patients had lipid levels, blood glucose, and insulin evaluated within 3 months of PSG. The demographics, laboratory data, body mass index (BMI), and blood pressure were collected from the medical records. Results: A total of 31 patients were studied. Twenty-one (67.7%) were male, and the mean age of patients was 8.5 ± 1.9 years. The mean BMI z score was 2.7 ± 0.81. All patients were obese. Patients were categorized into groups with total sleep duration >6 hours and <6 hours. Sixteen patients slept <6 hours and 15 patients slept >6 hours. There was no statistical difference in the lipid variables and blood pressure in these patients. A nonsignificant trend of decreased sleep duration with an increase in severity of OSA was observed. Blood sugar was significantly increased in patients with sleep <6 hours (100.2 ± 8.1 mg/dL) when compared with patients with sleep >6 hours (95 ± 4.4 mg/dL; P = .05). The homeostasis model assessment (HOMA) values were significantly higher in children with sleep <6 hours (1.81 ± 0.51) when compared with patients with sleep >6 hours (1.33 ± 0.55; P = .04). Conclusions: Short sleep duration is associated with insulin resistance in obese children with OSA.