Faculty Bibliography

OBJECTIVES/HYPOTHESIS: To validate a clinical assessment score for pediatric sleep-disordered breathing. STUDY DESIGN: Prospective instrument validation. METHODS: One hundred children scheduled for overnight polysomnography were evaluated by a standardized history and physical examination and assigned a clinical assessment score. Parents completed the Obstructive Sleep Apnea (OSA)-18, the Pediatric Quality of Life Inventory (PedsQL) 4.0, and the Child Behavior Checklist questionnaires. Children with positive polysomnography underwent adenotonsillectomy or adenoidectomy. The identical assessments were performed at a mean follow-up of 8 months. RESULTS: Item reduction yielded a score of 15 items (Clinical Assessment Score-15 [CAS-15]) that demonstrated the best internal consistency and predictive utility (Cronbach alpha = .80). Intraclass correlation (ICC) demonstrated good intrarater (ICC, 0.78; 95% confidence interval [CI], 0.58 to 0.89) and inter-rater agreement (ICC, 0.65; 95% CI, 0.26 to 0.84). All change scores were significantly improved after surgery. Effect sizes were large for the CAS-15 (2.6), OSA-18 (2.4), and apnea-hypopnea index (1.4), and moderate for the Child Behavior Checklist (0.7) and PedsQL 4.0 (-0.5). Moderate to strong correlation was found between the initial CAS-15 scores and the external measures (|r| between 0.32 and 0.65). Receiver operating characteristic curves were constructed to determine the optimal initial CAS-15 score for predicting positive polysomnography. The area under the curve was 0.77 (95% CI, 0.67 to 0.87); and a score >/=32 yielded a sensitivity of 77.3% (95% CI, 65.3 to 86.7) and a specificity of 60.7% (95% CI, 40.6 to 78.5). CONCLUSIONS: The CAS-15 proved useful in an office setting and correctly diagnosed 72% of referred children when compared to polysomnography. It correlated well with external measures and demonstrated a good response to clinical change.

OBJECTIVES/HYPOTHESIS: To analyze a single surgeon's experience with endoscopic CO(2) laser and stapler repair of Zenker diverticulum (ZD) by comparing dysphagia and regurgitation outcomes. STUDY DESIGN: Retrospective chart review of 148 patient charts. METHODS: Medical records of all patients receiving endoscopic repair of ZD with either CO(2) laser (61 patients) or stapler (67 patients) were reviewed. Additional data included demographics (age and sex), size (cm), preoperative and postoperative symptoms, need for revision, and complications. Symptoms of dysphagia were graded based on a modified Functional Oral Intake Scale 1 to 4 scale (1 = normal intake; 4 = severely limited/G-tube dependent). Regurgitation was also graded on a 1 to 4 scale (1 = no regurgitation; 4 = aspiration events). RESULTS: We noted no difference in patient age or defect size (laser, 3.26 cm; stapler, 3.53 cm; P .135). Significant differences were noted in return trips to the operating room for failed procedures (laser, 0; stapler, 7; P = .009), length of stay (laser, 3.19 days; stapler, 1.29 days; P < .001), time to oral intake (laser, 3.01 days; stapler, 1.22 days; P < .001). Significant improvement occurred in laser and staple patient symptom scales following surgery (P < .001). Laser dysphagia and regurgitation scores showed greater improvement when compared to stapler scores (P < .001). CONCLUSIONS: Endoscopic CO(2) laser and staple methods are effective in treating ZD. The laser can have greater efficacy and result in lower recurrence rates. Both methods are analyzed and compared.

This single-institution phase II study was performed to estimate the response rate to lapatinib in neurofibromatosis type 2 (NF2) patients with progressive vestibular schwannoma (VS). Twenty-one eligible patients were enrolled. Brain and spine MRIs, including 3-dimensional volumetric tumor analysis, and audiograms were performed once at baseline and again every 12 weeks. The primary response end point was evaluable in 17 patients and defined as >/=15% decrease in VS volume. Hearing was evaluable as a secondary end point in 13 patients, with responses defined as an improvement in the pure tone average of at least 10 dB or a statistically significant increase in word recognition scores. Four of 17 evaluable patients experienced an objective volumetric response (23.5%; 95% confidence interval [CI], 10%-47%), with median time to response of 4.5 months (range, 3-12). In responders, reduction in VS volumes ranged from -15.7% to -23.9%. Four of 13 patients evaluable for hearing met hearing criteria for response (30.8%; 95% CI, 13%-58%). One sustained response exceeded 9 months in duration. Median time to overall progression (ie, volumetric progression or hearing loss) was 14 months. The estimated overall progression-free survival and volumetric progression-free survival at 12 months were 64.2% (95% CI, 36.9%-82.1%) and 70.6% (95% CI, 43.1%-86.6%), respectively. Toxicity was generally minor, and no permanent dose modifications were required. Lapatinib carries minor toxicity and has objective activity in NF2 patients with progressive VS, including volumetric and hearing responses. Future studies could explore combination therapy with other molecular targeted agents such as bevacizumab.

OBJECTIVES: Laryngotracheal involvement in relapsing polychondritis (RP) is rare. However, it is one of the most common causes of death in this patient population. We present three patients who primarily presented with laryngeal manifestations of RP and a novel treatment option for bamboo nodules. STUDY DESIGN: Retrospective chart review and comprehensive review of the literature. RESULTS: Two patients first presented to an otolaryngologist because of hoarseness and chronic cough that eventually progressed to dyspnea upon exertion. Laryngeal examination revealed subglottic stenoses. Upon rheumatologic workup both were diagnosed with RP. After treatment with steroids and immunosuppressive drugs, one of the patient's laryngeal symptoms improved, whereas the other required dilation procedures. Neither patient had classic auricular or nasal symptoms upon initial presentation. The third patient was being treated for spasmodic dysphonia and was noted to have bamboo nodules with accompanying dysphonia. Rheumatologic workup revealed RP and systemic treatment ensued. Unfortunately, her symptoms of hoarseness persisted despite systemic treatment. A pulsed-potassium-titanyl-phosphate (KTP) laser was applied to the bilateral bamboo nodules, which eventually caused resolution of her vocal fold lesions and dysphonia. CONCLUSIONS: We present three patients with RP, all of whom sought health care by an otolaryngologist primarily. Awareness of this disease entity and the possibility for early laryngeal involvement is crucial for proper care of those with this life-threatening disease

Extensive and complex defects of the head and neck involving multiple anatomical and functional subunits are a reconstructive challenge. The purpose of this study is to elucidate the reconstructive indications of the use of simultaneous double free flaps in head and neck oncological surgery. This is a retrospective review of 21 consecutive cases of head and neck malignancies treated surgically with resection and reconstruction with simultaneous use of double free flaps. Nineteen of 21 patients had T4 primary tumor stage. Eleven patients had prior history of radiotherapy or chemo-radiotherapy. Forty-two free flaps were used in these patients. The predominant combination was that of free fibula osteo-cutaneous flap with free anterolateral thigh (ALT) fascio-cutaneous flap. The indications of the simultaneous use of double free flaps can be broadly classified as: (a) large oro-mandibular bone and soft tissue defects (n = 13), (b) large oro-mandibular soft tissue defects (n = 4), (c) complex skull-base defects (n = 2), and (d) dynamic total tongue reconstruction (n = 2). Flap survival rate was 95%. Median follow-up period was 11 months. Twelve patients were alive and free of disease at the end of the follow-up. Eighteen of 19 patients with oro-mandibular and glossectomy defects were able to resume an oral diet within two months while one patient remained gastrostomy dependant till his death due to disease not related to cancer. This patient had a combination of free fibula flap with free ALT flap, for an extensive oro-mandibular defect. The associated large defect involving the tongue accounted for the swallowing difficulty. Simultaneous use of double free flap aided the reconstruction in certain large complex defects after head and neck oncologic resections. Such combination permits better complex multiaxial subunit reconstruction. An algorithm for choice of flap combination for the appropriate indications is proposed.

Statement of Problem: The microvascular free fibula flap is widely used to reconstruct complex craniomaxillofacial defects following ablative surgery. Since its popularization for mandibular bony reconstruction in 1989, many permutations of the fibula flap have been applied to composite head and neck defects. Several authors describe endosseous implantation of the fibula post operatively or at the time of surgery to aid in dental reconstruction, but this can leave a patient partially edentulous for up to 1 year after initial surgery. Many patients are lost to follow up and do not go on to complete dental rehabilitation. This may contribute to suboptimal nutritional status, poor cosmetic outcomes, and decreased patient satisfaction. We will discuss how these problems can be circumvented by single stage surgery that incorporates dental implants and a prosthesis to allow for complete jaw reconstruction. Methods: A retrospective chart review at NYU Langone Medical Center and Bellevue Hospital Center was completed to identify patients undergoing extirpative surgery of the maxilla or mandible with immediate reconstruction with a free fibula flap, dental implants, and dental prosthesis from 2011-2012. A total of 5 patients were treated for ameloblastoma (n=3), intraosseus hemangioma (n=1), and odontogenic myxoma (n=1) of the maxilla (n=1) and mandible (n=4). Virtual surgical planning was implemented in all cases. During the computer assisted design phase, a virtual dental construct of an implant supported prosthesis was applied to the planned resection site and the fibula flap was designed to support the desired prosthesis. The cutting jigs for the jaw and fibula were manufactured according to plan as well as the implant borne dental prosthesis. In addition to osteotomy cutting slots, the fibular jig had implant drill guides to aid in correct placement. The prosthesis was secured to the fibular implants with custom abutments and then placed into temporary maxillomandibular fixation with the native dentition prior to plate osteosynthesis of the fibula. Post operative physical examination and computed tomography was used to evaluate occlusion and flap position. Results: All patients were reconstructed successfully with this method without any flap or implant failures. Longest follow up time was 12 months with a mean of 6 months. A total of 23 implants were planned and placed with no implant failures. 1 implant was not used due to suboptimal position in relation to the prosthesis. All patients required post operative guiding elastics with all patients achieving a reproducible desired occlusion by 2 (Figure psented) weeks. All patients tolerated a soft diet by postoperative week 3 without the need for supplemental enteral or parenteral feeding. All patients reported satisfaction in their reconstruction. The mean operative time was 8 hours. One patient needed revision surgery for a mobile locking screw in the plate hardware. Conclusion: The fibula flap continues to be the workhorse of jaw reconstruction, and with proper patient selection and pre-operative planning, it can be used to successfully reconstruct complex maxillofacial defects from bone to teeth in a single operation. In our experience, computer assisted design and virtual planning is essential in achieving the above described results while maintaining appropriate operative times

BACKGROUND: The treatment for upper aerodigestive tract malignancy with external beam radiation therapy and chemotherapy can result in a "cure," yet result in pharyngoesophageal (PE) stenosis. To improve communication, compare therapeutic results, and assist in treatment planning, we developed a new classification system to describe PE stenoses. Additionally, we analyzed our 6-year experience. METHODS: The new classification system was defined and used to assess patient management. RESULTS: Thirty-one patients underwent treatment for PE stenoses using: 4 rendezvous dilations, 3 regional, and 24 free tissue transfers. Overall success was based on airway status, speech, and swallowing outcomes. CONCLUSION: Classification of posttreatment stenoses has been applied to this patient group. It is effective in defining the problem and considerations related to treatment planning and surgical access when augmentation pharyngoplasty is required. Patients with an intact larynx have many issues that make recovery of function less consistent, despite segment restoration.

Congenital cytomegalovirus infection is the most frequent nongenetic cause of pediatric hearing loss in the United States, affecting approximately 8,000 children each year. Due in part to variable cytomegalic involvement of the auditory cortex, cochlear implantation outcomes have varied widely. Functional magnetic resonance imaging (fMRI) has the potential to assist in determining candidacy for cochlear implantation through the detection of intact auditory pathways including the cortex. We report a case of a 21-month-old girl with cytomegalovirus-related deafness and diffuse white matter involvement in which fMRI was a determining factor for cochlear implantation and side selection.

OBJECTIVE: Jugular bulb abnormalities (JBA), such as jugular bulb diverticula (JBD) or large jugular bulbs, rarely present in the middle ear. We review a large series of temporal bone histopathologic specimens to determine their prevalence and present a series of cases of JB abnormalities involving the middle ear (JBME) that shed light on the probable mechanism for their development. PATIENTS: 1,579 unique temporal bone specimens and individuals with radiographically-diagnosed JBME. INTERVENTION: Histopathologic and clinical review of temporal bone specimens and patient presentations, radiographic findings, treatments and outcomes. MAIN OUTCOME MEASURE: Shared characteristics of JBME. RESULTS: There were 17 cases of JBME in 1,579 temporal bone (1.1%), of which, 15 involved the inferior mesotympanum below the level of the round window membrane (RWM), whereas 2 encroached upon the RWM or ossicles. In addition, 4 clinical cases of large JBME extending above RWM were encountered; these occurred in both sexes with ages spanning from young to old (7-66 yr). They presented with conductive hearing loss (n = 3), ear canal mass (n = 1), and intraoperative bleeding (n = 1). Radiologically, they had multiple diverticula of the JB on the side with JBME, with 1 patient demonstrating growth on serial imaging studies. All patients who underwent additional imaging had marked hypoplastic contralateral transverse sinus. CONCLUSION: JBME abnormalities are rare, present across age groups, and may demonstrate serial growth over time. They are usually associated with multiple other diverticula within the same JB. Our clinical series suggests that JBME's development and uniquely aggressive behavior results from contralateral transverse sinus outflow obstruction.

The surgical robot has been demonstrated to have useful applications in urologic, gynecologic, cardiac, general, and endocrine surgery. The development of robotic surgery has enhanced the precision and control of the surgeon in minimally invasive surgical situations specific to these specialties and, more recently, has been applied to the treatment of oropharyngeal tumors in the form of transoral robotic surgery (TORS). The elimination of the need for lip- and mandible-splitting approaches has allowed a reassessment of surgical options for the treatment of tumors that have until recently been primarily addressed nonoperatively with chemoradiation. The TORS approach has created the need to adapt current reconstructive options to robotic technology to manage the resultant tissue defects and to assess and compare the effectiveness of these procedures. This report details our early experience with the use of robot-assisted free tissue transfer for management of soft tissue defects of the oropharynx.