Pharyngeal flap and obstructive apnea: maximizing speech outcome while limiting complications.

OBJECTIVE: To assess speech results and rate of obstructive sleep apnea using a modified, superiorly based pharyngeal flap performed after staged adenotonsillectomy in a group with velopharyngeal insufficiency. METHODS: In this nonrandomized, retrospective case series (July 1, 1996, through June 30, 2003), patients were mainly children referred to a multispecialty craniofacial clinic. Patients underwent staged adenotonsillectomy 2 months before width-customized pharyngeal flap surgery. Short flaps were created high above the level of the palate, just long enough to reach the nasal surface. Donor sites were closed by superior advancement of the inferior posterior pharyngeal wall tissue. Cardiopulmonary and oximetry data were analyzed for immediate obstructive apnea. Speech results and airway symptoms were assessed at 6-month and yearly follow-up examinations. RESULTS: In the 54 consecutive patients who underwent staged adenotonsillectomy, no apnea occurred immediately after surgery. Long-term clinical examination revealed 4 cases of loud snoring. Polysomnographic results were negative in all cases. Complications included 3 cases of bleeding, 1 requiring transfusion. Velopharyngeal insufficiency was eliminated in 94% of patients. CONCLUSION: Complications related to obstructive sleep apnea have been significantly reduced while maintaining excellent speech results by a staged approach of removing tonsils and adenoids and by creating a short, high, wide, superiorly based pharyngeal flap with superior advancement of the inferior posterior wall to close the donor site.

Upper airway asymmetry in velo-cardio-facial syndrome.

OBJECTIVE: Various forms of asymmetry have been recognized as a feature of velo-cardio-facial syndrome (VCFS). This study was implemented to determine the frequency of anatomic and functional asymmetry of the velum, pharynx and larynx in children with VCFS. METHODS: Individuals with VCFS underwent prospective, blinded analysis by an expert panel who assessed the velum, pharynx and larynx with multi-view videofluoroscopy (MVF) and nasopharyngolaryngoscopy (NPL). The VCFS group was compared to an age-matched group of normal individuals. Eight different parameters were assessed in both groups for functional and anatomic symmetry including: velar elevation, adenoid size, posterior pharyngeal wall size, carotid pulsations, epiglottis size and shape, arytenoid size, true vocal cord size and true vocal cord motion. RESULTS: One hundred and twenty-one subjects with VCFS and 20 normal individuals underwent examination. Children with VCFS showed significantly more asymmetry compared to the normal group (69% versus 20%, P=0.01) with greatest differences seen with palatal motion, posterior pharyngeal wall size and epiglottis shape. On average, subjects with VCFS had three asymmetric parameters versus one parameter in the normal group. CONCLUSION: Asymmetric development of the pharynx and larynx in children with VCFS appears to be a distinct clinical feature of this syndrome. This finding may provide an important diagnostic clue for patients presenting with subtle features of the 22q11.2 microdeletion. These developmental abnormalities may increase the risk of speech impairment, aspiration and airway obstruction in affected individuals.

Wegener's granulomatosis presenting as unilateral parotid enlargement.

OBJECTIVE: Major salivary gland enlargement is a rare presenting symptom of Wegener's granulomatosis. The unrecognized occurrence of this entity can delay diagnosis, leading to increased morbidity from disease progression. This report discusses the clinical features and diagnostic testing of salivary gland enlargement secondary to Wegener's granulomatosis to differentiate it from other, more common, salivary gland diseases. STUDY DESIGN: A case report of a single subject with unilateral parotid gland enlargement secondary to Wegener's granulomatosis. METHODS: A review of the clinical course, diagnostic studies, and histopathology related to the presenting disease. RESULTS: A 54-year-old male was evaluated for a 6-week history of progressive right parotid enlargement and pain unresponsive to antimicrobial therapy. Computed tomography scans showed diffuse, unilateral parotid swelling without enhancement and without a mass lesion or sialolith. Multiple open biopsies from the gland were necessary to demonstrate the presence of necrotizing granulomatous inflammation with vasculitis. Elevated antineutrophil cytoplasmic autoantibody, cytoplasmic pattern (c-ANCA) titers confirmed Wegener's granulomatosis. Hemoptysis and acute renal failure requiring hemodialysis developed shortly after diagnosis but eventually resolved after the initiation of corticosteroids and cyclophosphamide. CONCLUSIONS: Unilateral parotid enlargement is a rare presentation of Wegener's granulomatosis. A high level of clinical suspicion should prompt biopsy and testing of c-ANCA when initial studies or empirical treatment fails to lead to a proper diagnosis. Early treatment may prevent the development of other serious systemic complications such as renal failure.

Bilateral vocal cord paralysis secondary to esophageal compression.

Bilateral vocal cord paralysis is most commonly caused by trauma, malignancy, and neurologic disorders. Cases secondary to esophageal compression of the recurrent laryngeal nerves are rare. We report a patient admitted with an exacerbation of achalasia who developed acute respiratory distress from bilateral immobile vocal cords. Imaging studies revealed impressive dilation of the cervical esophagus causing compression of both recurrent laryngeal nerves. After securing the airway and decompression of the esophagus, mobility of the vocal cords returned within 1 week. This case shows the importance of a careful airway workup in patients with esophageal distention. Early decompression may prevent permanent recurrent laryngeal nerve injury and airway obstruction.