Faculty Bibliography

Importance: Septal deviation commonly occurs in patients with cleft lip and palate (CLP); however, the contribution of the cartilaginous and bony septum to airway obstruction in skeletally mature patients is poorly understood. Objectives: To describe the internal nasal airway anatomy of skeletally mature patients with CLP and to determine the contributors to airway obstruction. Design, Setting, and Participants: This single-center retrospective review included patients undergoing cone-beam computed tomography (CBCT) from November 1, 2011, to July 6, 2015, at the cleft lip and palate division of a major academic tertiary referral center. Patients met inclusion criteria for the study if they were at least 15 years old at the time of CBCT, and images were used only if they were obtained before Le Fort I osteotomy and/or formal septorhinoplasty. Twenty-four skeletally mature patients with CLP and 16 age-matched control individuals were identified for the study. Main Outcomes and Measures: Septal deviation and airway stenosis were measured in the following 3 coronal sections: at the cartilaginous septum (anterior nasal spine), bony septum (posterior nasal spine), and midpoint between the anterior and posterior nasal spine. The perpendicular plate of the ethmoid bone and vomer displacement were measured as angles from the vertical plane at the coronal section of maximal septal deviation. The site of maximal septal deviation was identified. Results: Among the 40 study participants, 26 were male. The mean (SD) age was 21 (5) and 23 (6) years for patients with CLP and controls, respectively. Septal deviation in patients with CLP was significantly worse than that of controls at the anterior nasal spine (2.1 [0.5] vs 0.8 [0.2] mm; P < .05) and posterior nasal spine (2.9 [0.5] vs 1.0 [0.3] mm; P < .01) and most severe at the midpoint (mean [SD], 4.4 [0.6] vs 2.1 [0.3] mm; P < .01). The point of maximal septal deviation occurred in the bony posterior half of the nasal airway in 27 of 40 patients (68%). The CLP bony angular deviation from the vertical plane was significant in the CLP group compared with the control group (perpendicular plate of the ethmoid bone, 14 degrees [2 degrees ] vs 8 degrees [1 degrees ]; vomer, 34 degrees [5 degrees ] vs 13 degrees [2 degrees ]; P < .05 for both), and vomer deviation was significantly associated with anterior nasal airway stenosis (r = -0.61; P < .01). Conclusions and Relevance: Skeletally mature patients with CLP have significant septal deviation involving bone and cartilage. Resection of the bony and cartilaginous septum should be considered at the time of definitive cleft rhinoplasty. Level of Evidence: NA.

OBJECTIVE: To report on the prevalence of voice disturbances in the pediatric craniofacial population and to prove that the pediatric Voice Handicap Index (pVHI) is a useful questionnaire for this unique population. STUDY DESIGN: Case series with chart review. SETTING: Tertiary care center. SUBJECTS: Pediatric patients seen by a pediatric otolaryngologist in a craniofacial clinic. METHODS: Consecutive patients (N = 366) seen by a single pediatric otolaryngologist in a craniofacial clinic from July 2011 to September 2012 were included. Any patient specifically referred for airway problems or voice difficulties completed a pVHI questionnaire. Patients each underwent an evaluation including flexible fiberoptic laryngoscopy and videostroboscopy. Voice disturbance was further characterized into dysphonia, hypernasality, or hyponasality. RESULTS: Of all the patients evaluated, 280 (77%) were specifically referred for airway problems or voice disturbance. Of those referred, 39 (10.7%) were found to have an organic vocal fold pathology causing dysphonia, as seen on the videostroboscopic examination; 53.7% of these lesions were attributable to potential iatrogenic causes. Hypernasality and hyponasality were seen in 116 (31.7%) and 78 (21.3%) patients, respectively. The pVHI was 3.95, 26.3, 11.34, and 10.53 for those with no voice disturbance, dysphonia, hypernasality, and hyponasality, respectively. CONCLUSION: Pediatric patients with craniofacial disorders have a higher prevalence of dysphonia than the general pediatric population. The majority of causes of dysphonia in these patients are possibly iatrogenic in origin. The pVHI serves as a useful questionnaire in this unique patient cohort to quantify the disability from not only dysphonia but also hypernasality and hyponasality.

INTRODUCTION: Congenital maxillomandibular syngnathia, or fusion of the jaws, is a rare condition that has a broad spectrum of presentations. The restricted mouth opening can lead to issues with feeding, swallowing, and respiration resulting in failure to thrive and temporomandibular joint ankylosis. Early recognition and treatment is necessary for proper growth and development. CASE REPORT: We report a 1-day-old male with isolated bilateral soft tissue alveolar fibrous bands. He presented with difficulty feeding secondary to trismus. No bony or muscular involvement in the synechiae was noted and the remainder of the physical exam was unremarkable. The bilateral alveolar synechiae were divided under local anesthesia using surgical scissors. The patient immediately showed improvement in mouth opening and had resolution of his feeding problems. He is now gaining weight and developing appropriately. DISCUSSION: The accompanying review of the literature demonstrates only 11 cases worldwide of isolated maxillomandibular fusion. Depending upon the composition of the synechiae, simple surgical division under local anesthesia can be curative.

Background Tracheobronchopathia osteochondroplastica is a rare benign and often indolent disease. We report the first case of tracheobronchopathia osteochondroplastica (TBO) presenting as acute hypercarbic respiratory failure due to superimposed subglottic submucosal abscess. Case Report A 27-year-old man presented to the emergency department in respiratory distress that required mechanical ventilation for acute hypercarbic respiratory failure. Upon extubation the next day, stridor was elicited with ambulation. Spirometry revealed fixed upper airway obstruction. Neck imaging showed a 2.8x2.0x4.0 cm partially calcified subglottic mass with cystic and solid component obstructing 75% of the airway. Surgical exploration revealed purulent drainage upon elevation of the thyroid isthmus and an anterolateral cricoid wall defect in communication with a subglottic submucosal cavity. Microbiology was negative for bacteria or fungi. Pathology showed chondro-osseous metaplasia compatible with tracheobronchopathia osteochondroplastica (TBO). The patient received a course of antibiotics and prophylactic tracheostomy. Since tracheostomy removal 3 days later, the patient remains asymptomatic. Conclusions Tracheobronchopathia osteochondroplastica is a rare disease with usually benign clinical course and incidental diagnosis. It may present as acute hypercarbic respiratory failure when subglottic infection is superimposed.

AIM: To evaluate the relationship between intraoperative blood loss and juvenile nasopharyngeal angiofibroma (JNA) vascular supply and tumour stage in patients who underwent superselective external carotid artery (ECA) embolization. This series is unique in that all embolizations were performed by dedicated paediatric interventional radiologists at a tertiary referral paediatric centre. MATERIALS AND METHODS: Seventeen male patients treated from January 2002 to August 2009 underwent preoperative angiography and embolization using polyvinyl alcohol (PVA) particles. Tumours were graded using three different staging systems based on preoperative imaging and correlated to surgical blood loss. All patients underwent bilateral internal and external carotid angiography, with embolization of ECA tumour supply via microcatheter delivery of PVA particles. Particle size ranged from 150-500 mum with a mean size of 250-355 mum. Surgical resection was performed with either endoscopic or open techniques within 24 h and intraoperative blood loss was reported. RESULTS: Seven lesions were supplied strictly by the ECA circulation and had mean surgical blood loss of 336 ml. Twelve lesions had both ECA and internal carotid artery (ICA) supply and had mean surgical blood loss of 842 ml. The difference in blood loss in these two groups was statistically significant (p = 0.03). There was no case of inadvertent intracranial or ophthalmic embolization. There were statistically significant correlations between estimated surgical blood loss and the Andrews (p = 0.008), Radkowski (p = 0.015), and University of Pittsburgh Medical Center (UPMC; p = 0.015) preoperative tumour staging systems, respectively. CONCLUSION: Preoperative embolization of JNA tumours can be safely performed without neurological complications. The present study identified a statistically significant difference in intraoperative blood loss between those lesions with a purely ECA vascular supply and a combination of ECA and ICA vascular supply. Angiography is helpful in delineating ICA supply and can help guide surgical planning.

OBJECTIVES/HYPOTHESIS: To characterize the anatomic distribution of lymphatic malformations of the upper airway. STUDY DESIGN: Case series with chart review. SETTING: Tertiary care referral center specializing in the diagnosis and treatment of vascular anomalies. METHODS: A 7-year (2004-2011) retrospective chart review of patients with lymphatic malformations was performed at a tertiary care referral center. Patients with airway lymphatic malformations were identified. The anatomic distribution of lymphatic malformations within the airway was reviewed. RESULTS: A total of 141 patients with lymphatic malformations of the upper aerodigestive tract (UADT) were studied. Of these, 15 (11%) had laryngeal (supraglottic) involvement. In all of these patients, the disease was above the true vocal folds. Seventy-four (52%) patients had involvement of 1 anatomic zone (most common was the oral cavity), and 67 (48%) had involvement of multiple zones. With regard to each zone, 105 (75%) patients had involvement of the oral cavity, 50 (36%) the oropharynx, 8 (6%) the hypopharynx, 42 (30%) the parapharynx, and 12 (9%) had retropharygeal disease (some patients had multiple zones involved). No patients were identified with glottic, subglottic, or tracheal involvement. CONCLUSIONS: Based on our large series, airway involvement in head and neck lymphatic malformations may occur at multiple sites above the glottis. A high percentage of these patients have involvement of the oral cavity (75%) and oropharynx (35%). None involve the glottis, subglottis, or trachea.

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