Robert Y. Huang

Adenoid cystic carcinoma of the submandibular gland: a 35-year review.

OBJECTIVES: To evaluate the treatment results of adenoid cystic carcinoma (ACC) of the submandibular gland at a single institution during a 35-year period. STUDY DESIGN & SETTING: A retrospective review was performed by examining the records and reviewing the pathology of 22 patients with ACC of the submandibular gland treated at UCLA Medical Center from June 1963 to December 1997. RESULTS: Seven men and 15 women with an age range of 23 to 85 years (median, 48 years) were treated. Surgical intervention was performed in 21 patients. All patients with advanced tumor size, perineural invasion, microscopically positive surgical margins, or regional neck metastases received postoperative adjunctive therapy, primarily radiotherapy. Follow-up varied from 6 months to 181 months (median, 67 months). Disease-free survival at 3, 5, and 10 years was 66%, 57%, and 41% respectively, whereas overall survival was 76%, 70%, and 37%, respectively (note: 5- and 10-year survival rates are not statistically conclusive due to the small sample size). CONCLUSIONS: We report fairly high disease-free survival rates in this patient population and a number of prognostic trends are evident. Early diagnosis, wide surgical intervention, and postoperative radiation are associated with a favorable prognosis. Advanced tumor size, positive surgical margins, perineural invasion, and local recurrence of the tumor are associated with an unfavorable prognosis. EBM rating: C.

Adenotonsillar histopathology after organ transplantation

OBJECTIVES: The increasing number of surviving pediatric organ transplant recipients has resulted in a new clinical controversy surrounding the significance of adenotonsillar hypertrophy. The objective of this study is to evaluate adenotonsillar specimens, understand characteristic histopathology, and to examine the frequency and significance of this finding in this population. METHODS: Twenty-one cases of pediatric transplant recipients with adenoidal and/or tonsillar hypertrophy were reviewed retrospectively in a tertiary-care setting. Particular attention was given to the histopathology of their surgical specimens, including any evidence of posttransplantation lymphoproliferative disorders (PTLD). RESULTS: Using morphologic, immunohistochemical, and molecular genetic analyses, 15 (71%) of 21 patients were noted to have Epstein-Barr virus (EBV)-related lymphoid hyperplasia, including 1 case (4.7%) of PTLD. Six (29%) of 21 had evidence of reactive follicular hyperplasia not related to EBV. B-cell and T-cell markers were nearly uniformly positive when tested for, except in the single patient with PTLD, who exhibited polymorphic, polyclonal B-cell morphology. Kappa and lambda light-chain clonality markers were positive in 11 (92%) of 12 patients. CONCLUSIONS: EBV-related lymphoid hyperplasia is frequently associated with adenotonsillar hypertrophy in pediatric organ transplant patients (71% of our cases); 92% of those cases tested exhibit polyclonal B-cell populations. PTLD, an important cause of morbidity and mortality in this population, represented approximately 5% of our cases. The remainder of cases represent follicular hyperplasia unrelated to EBV or lymphoproliferative abnormalities. Characteristic histopathologic findings are presented.

Surgical Anatomy of the Recurrent Laryngeal Nerve: Implications for Laryngeal Reinnervation

Functional laryngeal reinnervation depends upon the precise reinnervation of the laryngeal abductor and adductor muscle groups. While simple end-to-end anastomosis of the recurrent laryngeal nerve (RLN) main trunk results in synkinesis, functional reinnervation can be achieved by selective anastomosis of the abductor and adductor RLN divisions. Few previous studies have examined the intralaryngeal anatomy of the RLN to ascertain the characteristics that may lend themselves to laryngeal reinnervation. Ten human larynges without known laryngeal disorders were obtained from human cadavers for RLN microdissection. The bilateral intralaryngeal RLN branching patterns were determined, and the diameters and lengths of the abductor and adductor divisions were measured. The mean diameters of the abductor and adductor divisions were 0.8 and 0.7 mm, while their mean lengths were 5.7 and 6.1 mm, respectively. The abductor division usually consisted of one branch to the posterior cricoarytenoid muscle; however, in cases in which multiple branches were seen, at least one dominant branch could usually be identified. We conclude that the abductor and adductor divisions of the human RLN can be readily identified by an extralaryngeal approach. Several key landmarks aid in the identification of the branches to individual muscles. These data also indicate the feasibility of selective laryngeal reinnervation in patients who might be candidates for laryngeal transplantation after total laryngectomy.

Third branchial cleft anomaly presenting as a retropharyngeal abscess

Branchial cleft anomalies are congenital developmental defects that typically present as a soft fluctuant mass or fistulous tract along the anterior border of the sternocleidomastoid muscle. However, branchial anomalies can manifest atypically, presenting diagnostic and therapeutic challenges. Error or delay in diagnosis can lead to complications, recurrences, and even life-threatening emergencies. We describe a case of an infected branchial cleft cyst that progressed to a retropharyngeal abscess in a 5-week-old female patient. The clinical, radiographic, and histologic findings of this rare presentation of branchial cleft cyst are discussed.

Tracheal stenosis and congenital heart disease in patients with Down syndrome : diagnostic approach and surgical options

Upper airway obstruction is a prevalent feature in patients with Down syndrome. However, these patients may be completely asymptomatic in the early months of life. The recognition of a problem within the airway quite often occurs when these children present for cardiac surgery, diagnostic radiography with sedation, or during the induction and intubation for anesthesia. Tracheal stenosis is rare in the general population, but is seen somewhat more frequently in patients with Down syndrome. The incidence of tracheal stenosis in children with congenital heart disease, which is seen in 40% of patients with Down syndrome, has been reported to be 1.2%. Patients with Down syndrome also tend to have other upper airway obstructive pathology such as nasopharyngeal, oropharyngeal, and subglottic compromise. These entities, combined with the high incidence of cardiac disease, put these children at risk for acute and chronic cardiopulmonary compromise. We present two patients with Down syndrome and congenital heart disease who were found to have significant tracheal stenosis at the time of their cardiac surgery. The perioperative management of their airway defects including diagnostic evaluation and treatment modalities are discussed.

Effect of Salivary Fistulas on Free Flap Failure: A Laboratory and Clinical Investigation

Objectives: Infection is associated with free flap failure in patients undergoing microvascular flap reconstruction. This study investigates the association between infection arising from salivary fistulas, microvascular thrombosis, and free flap failure.

Lack of Evoked Laryngeal Electromyography Response in Patients with a Clinical Diagnosis of Vocal Cord Paralysis

There has been recent debate about whether patients with vocal cord immobility have a neurologic paralysis or whether synkinesis, the misdirection of axons to competing laryngeal muscles, is responsible for the lack of voluntary vocal cord motion. This issue was studied in 15 patients with vocal cord paralysis who underwent laryngeal reinnervation. Evoked electromyography was performed with a surface electrode endotracheal tube. The recurrent laryngeal nerve (RLN) was identified and stimulated with constant current. Of the 15 patients, only 1 produced a compound muscle action potential upon nerve stimulation. The remaining 14 patients had no evoked response during RLN stimulation. a control group of 8 patients with normal vocal cord mobility was studied, and each had a normal evoked electromyography response after RLN stimulation. These results support the assertion that patients who require treatment for vocal cord paralysis do not have synkinesis produced by RLN reinnervation.

Endoscopic diagnosis of sarcoidosis in a patient presenting with bilateral exophthalmos and pansinusitis.

Sarcoidosis is a chronic granulomatous disease of unknown etiology. Otolaryngologic and ophthalmologic manifestations occur in 15 to 55% of afflicted individuals, respectively. Neck masses, parotid enlargement, and facial nerve palsy are the most common presenting otolaryngologic complaints, while lacrimal gland enlargement, uveitis, and upper eyelid masses often call the attention of the ophthalmologist. Biopsy reveals non-caseating granulomas, while the angiotensin converting enzyme (ACE) level may be elevated. We report an unusual case of a patient who presented with severe bilateral exophthalmos as the sole initial complaint. A prior workup included a negative conjunctival biopsy. On magnetic resonance imaging (MRI) and computed tomography (CT), the patient had pansinusitis. Endoscopic ethmoidectomies with tissue analysis revealed sarcoidosis. Further evaluation revealed no evidence of systemic disease, and all symptoms resolved with a course of oral steroids. Thus, nasal endoscopy and biopsy of affected paranasal sinus mucosa may prove a useful adjunct to the diagnosis of sarcoidosis, particularly in atypical cases.

Microvascular Reconstruction of Composite Defects of the Mandible and Lip: Aesthetic and Functional Considerations†

INTRODUCTION Reconstruction of composite defects involving the mandible and lip is challenging. Successful restoration of oral continence relies on creation of a soft tissue oral sphincter and simultaneous establishment of skeletal support to maintain upper and lower lip opposition. Failure to achieve these goals results in severe functional and aesthetic disability evident in chronic drooling, inability to eat or drink, impaired speech, and facial disfigurement. An effective approach to reconstruction of this formidable oromandibular defect has not been discussed in detail in the literature. Successful reconstruction in patients with composite defects of the mandible and lip was difficult before the application of free flaps for head and neck reconstruction. Although the value of local flaps for lip reconstruction has been recognized for many years, vascularized bonecontaining free flaps have allowed for reliable restoration of anterior mandibular skeletal support. In addition, soft tissue free flaps have found a role in total lip reconstruction, where the results of local flap lip reconstruction have been disappointing. The current series analyzes aesthetic and functional outcome in 16 patients who underwent microvascular oromandibular reconstruction for composite defects of the mandible and lip. PATIENTS AND METHODS Sixteen patients underwent microvascular oromandibular reconstruction for composite defects of the mandible and lip. Fifteen patients underwent immediate reconstruction after surgical resection of advanced cancer arising in the lower lip, the buccal mucosa, the alveolar ridge, the floor of mouth, and/or the tongue. One patient underwent delayed reconstruction after sustaining a shotgun wound to the submental region. Fourteen patients had segmental mandibular defects; two patients underwent marginal mandibulectomy. The extent of lower lip resection varied from 20% to 100%. Three patients also required partial upper lip resection, with the upper lip defect ranging from 30% to 50%. Defects of the adjacent oral mucosa as well as defects of the chin, cheek, and upper neck skin were common. Previous therapy impacted the method of reconstruction in 11 of the 16 patients. Specifically, nine patients had undergone previous surgery and radiation therapy, and two patients had undergone previous surgery. The impact of previous therapy was manifested primarily by the effect of pre-existing surgical scars and irradiation on the design and anticipated reliability of local flaps considered for lip and external cutaneous reconstruction. Reconstruction was accomplished using fibula osteocutaneous flaps (n 5 8), radial forearm fasciocutaneous flaps (n 5 6), and latissimus dorsi-rib flaps (n 5 2). Two fasciocutaneous radial forearm flaps were used in patients with marginal mandibulectomies, and four fasciocutaneous radial forearm flaps were used in conjunction with bridging mandibular reconstruction plates after segmental mandibulectomy. Both latissimus dorsi-rib free flaps were performed in patients who were found to be poor candidates for fibula flap reconstruction on the basis of lower extremity angiography that showed severe peripheral vascular disease. Soft tissue lip reconstruction was accomplished using remaining lip tissues in 11 patients, and the soft tissue component of a free flap was used for lip reconstruction in 5 patients. Primary soft tissue closure of the lips or local lip flaps were selected in all patients in whom reconstruction could be accomplished without causing severe microstomia. This included all patients who underwent resection of up to 75% of the lip. Primary wedge Presented at the Meeting of the Western Section of the American Laryngological, Rhinological and Otological Society, Inc., San Francisco, California, January 9, 2000. From the Division of Head and Neck Surgery, the Department of Surgery, University of California Los Angeles School of Medicine, Los Angeles, California. Editor’s Note: This Manuscript was accepted for publication February 9, 2000. Send Correspondence to Keith E. Blackwell, MD, Box 951624 University of California Los Angeles Medical Center, Los Angeles, CA 900951624, U.S.A.