Marc C. Thorne

Pediatric laryngeal neurofibroma: Case report and review of the literature

Presentation of a case of pediatric laryngeal neurofibroma (LNF) and review of the world literature. Comprehensive review of the world literature using Pubmed and Google scholar. Pediatric LNF was identified in 62 cases reported in the world literature. The most common presenting symptom is stridor and the most common location of the tumor in the larynx is the aryepiglottic fold. Recent reports demonstrate increased utilization of endoscopic resection with reduced need for tracheostomy. Pediatric LNF is a rare disorder. Review of the world literature since 1940 suggests a recent trend away from aggressive open resection and toward more conservative endoscopic resection with excellent functional results.

The Relationship between Jugular Bulb–Vestibular Aqueduct Dehiscence and Hearing Loss in Pediatric Patients

Objective. To determine the prevalence of jugular bulb and vestibular aqueduct dehiscence (JBVAD) in pediatric patients undergoing temporal bone computed tomography (CT) scans and to assess the relationship between JBVAD and hearing loss. Study Design. Cross-sectional study with chart review. Setting. Tertiary academic medical center. Subjects and Methods. All patients 18 years of age or younger who had undergone temporal bone CT scans and audiometric testing between 2004 and 2009 were retrospectively reviewed. JBVAD was determined by blinded review of CT images. Hearing loss was determined by review of audiometric data and was correlated with imaging findings. Results. CT images and audiometric data were available for review in 927 patients (1854 ears). Overall prevalence of JBVAD was 8.6%, with a prevalence of 6.6% in right ears and 3.6% in left ears. JBVAD was present in 8.3% and 7.1% of patients with and without sensorineural or mixed hearing loss, respectively (95% confidence interval [CI], -2.3% to 4.6%; P = .51). Similarly, JBVAD was present in 5.5% of ears with and 4.6% of ears without sensorineural or mixed hearing loss (95% CI, -1.1% to 2.9%; P = .37). Conclusion. The prevalence of JBVAD is 8.6% in pediatric patients undergoing temporal bone CT scans, 65% of which occur in the right ear. We were unable to identify any relationship between JBVAD and hearing loss. A major contribution to pediatric sensorineural hearing loss from JBVAD is therefore extremely unlikely.

Risk Indicators for Congenital and Delayed-Onset Hearing Loss

Objective To evaluate risk indicators for congenital and delayed onset hearing loss in a cohort of newborns who underwent newborn hearing screening, and to evaluate the impact of use of the Joint Committee on Infant Hearing (JCIH) recommendations on requirements for ongoing monitoring of infants identified as at risk for hearing loss. Patients and Methods Cohort of 26,341 newborns entered in a prospectively collected database as part of the University of Michigan Universal Newborn Hearing Screening program, with 90 patients identified. Logistic regression analysis was used to evaluate putative risk indicators for congenital and delayed onset hearing loss. An estimate of the cost burden of ongoing monitoring imposed by the use of differing risk indicators was performed. Results After controlling for the impact of other risk indicators, intensive care unit length of stay greater than 5 days and exposure to loop diuretics are not associated with an increased risk of congenital or delayed onset hearing loss. Inclusion of these risk indicators as a requirement for ongoing audiologic monitoring results in a high monitoring cost per additional case identified. Discussion This study confirms that the majority of the risk indicators currently recommended by the JCIH are effective at identifying infants at increased risk of congenital and delayed onset hearing loss. However, use of neonatal intensive care unit length of stay greater than 5 days and exposure to ototoxic medications are associated with small gains in the number of infants correctly identified as at risk of hearing loss. Further evaluation of the utility of these risk indicators, preferably with a diversity of patient population and healthcare settings, is warranted.

Pediatric tracheostomy in conjoined twins: multidisciplinary approach.

Tracheostomy for definitive airway management in conjoined twins is a challenge for the surgeon, anesthesiologist, and support staff. A carefully choreographed multidisciplinary approach is required. A literature search finds few reports on tracheostomy in conjoined twins. We report our experience with tracheostomy with 5.5-month-old conjoined omphalopagus twins who were attached facing each other. Initial planning led to a careful rehearsal of infant positioning focused on optimizing anterior cervical exposure. A postoperative tracheostomy care plan was also addressed. Each twin was assigned his own anesthetic team and equipment early in his life to provide consistent care for the anticipated multiple surgical procedures throughout his hospital stay. Issues addressed in this report include transportation, intubation, intraoperative airway management, the mechanical ventilatory pattern of two individuals sharing a common diaphragm, and the pharmaceutical management of two children with partially shared vascular supply.

Pediatric hearing loss and radiographic pericochlear hypoattenuation.

Objective To assess the relationship of sensorineural hearing loss to pericochlear radiographic hypoattenuation in the pediatric population. Study Design Retrospective case review. Setting Tertiary referral center. Patients Children, younger than 5 years, with both computed tomographic imaging of the temporal bone and audiometric analysis between 2004 and 2008 at the University of Michigan. Intervention Diagnostic. Main Outcome Measure(s) The prevalence of pericochlear hypoattenuation in ears with and without hearing loss. Results One hundred seventy-six patients (98 male and 78 female patients) were completely reviewed. The prevalence of cochlear hypoattenuation was 39.2% (107/273) in ears with hearing loss, compared with 40.5% (32/79) in ears without hearing loss (p = 0.83). Similarly, the prevalence of hypoattenuation was not different in ears with sensorineural hearing loss (36.8%, 70/190) and those without hearing loss (40.3%, 31/79, p = 0.60). Patients with hypoattenuation (mean, 24 mo) were significantly younger than patients without hypoattenuation (mean, 30 mo). The prevalence of hypoattenuation was higher in the ears of children born prematurely (35/64, 54.7%) compared with the ears of children born at full term (90/230, 39.1%, p = 0.026; 95% confidence interval, 1.88%–29.3%). Conclusion There does not seem to be a relationship between sensorineural hearing loss and the presence of decreased density of the bony cochlea in the pediatric population. The density of pericochlear bone increases with age. Prematurity is associated with a higher risk of hypoattenuation. This radiographic finding is common and may represent a normal developmental process.

Patient and Family Satisfaction in a Pediatric Otolaryngology Clinic

Objective: Identify factors related to patient and family satisfaction in an academic pediatric otolaryngology clinic. Suggest strategies to improve patient and family satisfaction in this setting. Method: Review of an ongoing continuous quality improvement (CQI) effort. Patients/families were surveyed following each visit between May and December 2010. Linear and multiple linear regression were used to evaluate relationships between wait times in the reception area and examination room, assessment of the provider, and clinic volume with overall satisfaction. Results: During the study interval, 754 clinical encounters in the pediatric otolaryngology clinic resulted in 528 responses for a response rate of 70%. Linear regression showed a significant relationship between each of the independent variables and overall satisfaction, with the greatest variance in overall satisfaction explained by satisfaction with the provider, followed by wait time in the examination room, wait time in the reception area, and clinic volume (r2 0.53, 35, 26, .01, respectively). Multiple linear regression showed that clinic volume was not associated with overall satisfaction independent of the other variables. Conclusion: Variations in satisfaction with the provider accounted for the greatest amount of variance in overall satisfaction. The identification that variation in examination room wait times explained a larger portion of the variance in overall satisfaction has led to efforts to minimize this aspect of the patient and family experience.

Jugular Bulb: Vestibular Aqueduct Dehiscence and Hearing Loss

Objective: Determine the prevalence of dehiscence of the jugular bulb and vestibular aqueduct (JBVAD) in pediatric patients undergoing temporal bone computed tomography (CT) scans and assess the correlation between JBVAD and hearing loss. Method: All patients 18 years of age or younger who underwent temporal bone CT scans and audiometric testing between 2004 and 2009 were retrospectively reviewed. JBVAD was determined by blinded review of CT images. Hearing loss was determined by review of audiometric data and correlated with imaging findings. Results: CT images and audiometric data were available for review in 927 patients (1854 ears). Overall prevalence of JBVAD was 8.6%, with a prevalence of 6.6% in right ears and 3.6% in left ears. JBVAD was present in 8.3% of patients with sensorineural or mixed hearing loss and 7.1% of patients without sensorineural or mixed hearing loss (P = .51). Similarly, JBVAD was present in 5.5% of ears with and 4.6% of ears without sensorineural or mixed hearing loss (P = .37). Conclusion: The prevalence of JBVAD is 8.6% in pediatric patients undergoing temporal bone CT scans, 65% of which occur in the right ear. We were unable to identify any correlation between JBVAD and hearing loss. A major contribution to pediatric sensorineural hearing loss from JBVAD is excluded.

Pathology Quiz Case 3

A HEALTHY 11-YEAR-OLD GIRL WAS REferred with a gradually enlarging, asymptomatic facial mass. She had no history of infection, feline scratches, tuberculosis exposure, or prior lesions. A solitary, nontender, nonfluctuant mass was evident between the sternocleidomastoid muscle and the angle of the mandible. The overlying skin was normal. The findings of a complete head and neck examination, including cranial nerves, were otherwise normal. Computed tomography revealed a 4-cm, hypodense, heterogeneous mass involving the left parotid gland, displacing the retromandibular vein laterally, and extending toward the parapharyngeal and prestyloid spaces, without significant enhancement (Figure 1). The results of fine-needle aspiration were nondiagnostic. A Gram stain and cultures were negative for organisms. The patient underwent a total parotidectomy with facial nerve preservation. A cystic mass in the deep lobe of the parotid gland was excised contiguously, with a solid component extending into the parapharyngeal space. On pathologic gross evaluation, the cyst was unilocular, with an adjacent solid component of adherent fibrous and yellow lobulated tissue. Histologically, the cyst lining was mostly denuded and replaced by a foreign body reaction. The better-preserved areas were lined by a mixture of stratified keratinizing squamous epithelium with associated hair follicles, adjacent sebaceous glands, and smooth muscle bundles (Figure 2). There was a plate of cartilage with an underlying foreign body reaction made up of giant cells containing yellow pigment and a portion of salivary gland tissue (Figure 3). The adjacent solid tissue showed exuberant fat necrosis, chronic inflammation, foreign body reaction, and calcification. What is your diagnosis?

Outcomes of Pediatric Posterior Graft Laryngotracheoplasty

Objective 1) Determine the operation specific decannulation rate of patients who required posterior costal cartilage grafting (PCCG) in the management of laryngeal stenosis. 2) Describe the complications associated with PCCG laryngotracheoplasty. 3) Describe voice outcomes for selected patients. Methods A retrospective review was performed on all cases of posterior graft laryngotracheoplasty (LTP) performed by the 2 senior authors over a 9-year period at a tertiary care childrens hospital. Results There were 42 total patients. The operation specific decannulation rates were 100% (3/3) for Myer-Cotton grade II SGS, 75% (20/26) for grade III, and 60% (3/5) for grade IV. For posterior glottic stenosis (PGS), it was 57% (4/7) and for PGS with grade II SGS, it was 60% (6/10). There was no significant difference (p=0.727) among these groups. The overall decannulation rates were 100% (3/3), 96% (25/26), and 100% (5/5) for Myer-Cotton grades II, III and IV. For PGS, it was 83% (5/6), and for PGS with grade II SGS, it was 100% (8/8). There was no significant difference (p=0.527) among these groups. The relative risk for complications was higher among children who had a sutured vs. a sutureless posterior graft (RR= 2.7, p<.01). The most commonly seen compensatory vocal behavior was supraglottic dysphonia. Conclusions Operation-specific decannulation rates are not significantly different with increasing disease severity, although the power to detect small differences in this study is low. PCCG via a sutureless technique is associated with a lower complication rate. Supraglottic dysphonia is a common compensatory vocal behavior after PCCG LTP.