Significant laryngeal destruction in a northern European cohort of Behçet's disease patients.

OBJECTIVES: Behçet's disease (BD) is a multisystem autoimmune disease of unknown origin typically affecting the triad of oral and genital mucosa and the eye. Limited data are available in the literature regarding the otolaryngology-related manifestations of BD, particularly in northern Europeans. This is a novel study detailing surprising and significant laryngeal structural changes in a northern European cohort of BD. METHODS: Patients meeting the International Study Group for Behçet's Disease (ISGBD) and the International Criteria for Behçet's Disease (ICBD) criteria for diagnosis were identified from an institutional database. Patients underwent examination with an otolaryngologist, including flexible laryngoscopy. Intra-oral, pharyngeal and laryngeal manifestations of BD were documented and characterised. Patients underwent hearing assessment with pure-tone audiometry. RESULTS: Fifteen patients with BD were identified (4 male, 11 female; median age 36 years). 60% (n=9) showed evidence of disease on examination and flexible laryngoscopy. 33% (n=5) showed laryngeal changes related to BD. 13% (n=2) demonstrated bilateral sensorineural hearing loss. The 5 cases demonstrating laryngeal manifestations of disease are described in detail with photographic records. CONCLUSIONS: Limited data has been published regarding the laryngeal manifestations of BD, particularly in a northern European population. Our cohort of BD patients demonstrate significant laryngeal structural changes. It would appear that these clinically relevant changes may be more common than was previously thought. Raised awareness of the risk of laryngeal pathology in BD patients, often in the absence of overt clinical symptomatology, may result in earlier diagnosis and treatment. Rheumatologists and otolaryngologists should consider closer multi-disciplinary co-operation in the management and follow up of patients with BD.

The role of surgery for anaplastic thyroid carcinoma in the era of targeted therapeutics: A scoping review.

Background: Questions exist regarding patient selection for surgery in anaplastic thyroid carcinoma (ATC), particularly with the advent of neoadjuvant-targeted therapeutics. The present scoping review sought to evaluate what extent of surgical resection should be performed in ATC. Methods: A scoping review was carried out in accordance with Joanna Briggs Institute and the preferred reporting items for systematic reviews and meta-analyses extension for scoping reviews (PRISMA-ScR) protocols. Included studies were required to provide clear description of the surgery performed for ATC. Results: The final search identified 6901 articles. Ultimately only 15 articles including 1484 patients met inclusion criteria. A total of 765 patients (51.5%) underwent attempted curative intent surgery. The approach to resection of adjacent tissues varied between studies. Eight studies considered laryngeal ± pharyngeal resection (8/15, 53.3%), eight studies (53.3%) considered tracheal resection and again eight studies (53.3%) considered esophageal resection. More extensive resections increased morbidity without improving overall survival (OS) (<9 months in the 12 studies using a combination of surgery and chemoradiotherapy). In the three studies utilizing targeted therapy in addition to surgery, OS was notably improved while surgical resection following neoadjuvant therapy was less extensive. Conclusions: There is no clear agreement in the literature regarding the limits of surgical resection in locoregionally advanced ATC. A definition of surgically resectable disease will be required to guide surgical decision making in ATC, particularly with the potential to reduce tumor burden using neoadjuvant targeted treatment in suitable patients. Level of evidence: III.

Bilateral stapedectomy: association between first- and second-ear surgical findings and their effects on the second-ear outcome.

OBJECTIVES: We assessed the association between first-ear and second-ear surgical findings in patients undergoing second-ear stapedectomy for bilateral otosclerosis and the impact of such findings on the audiometric outcome of the second ear. METHODS: A retrospective chart review of all stapedectomy patients who underwent stapes surgery by one of two surgeons in a single tertiary referral institution from 1962 to 2001 was performed, and those patients who underwent bilateral stapedectomy were identified. Patient demographic data, surgical findings, procedure performed, and preoperative and postoperative audiometric data were recorded. RESULTS: A total of 459 patients (918 ears) underwent bilateral stapedectomy for bilateral otosclerosis during the study period, of whom 426 had complete data for analysis. The finding of a white or obliterated footplate in the second ear was significantly higher if the first ear had this disease manifestation (p < .001, chi2 test). The association between a second drill-out's being performed and a drill-out in the first operation was significant (p < .001, chi2 test). Statistical analysis identified that those who underwent a drill-out procedure had a 2.9-fold increase in unsuccessful outcome in comparison to those who did not have a drill-out (odds ratio, 2.89; 95% confidence interval, 1.41 to 5.89). Facial nerve anomalies were infrequently encountered, affecting only 23 patients, of whom 3 had bilateral abnormalities. The finding of an overhanging or dehiscent facial nerve in the second ear was significantly more likely if such an abnormality was identified during the first procedure (23% versus 2.5%; p = .005, Fisher's exact test). CONCLUSIONS: Second-ear hearing results are poorer in those who require a drill-out of this ear, and this is more likely to be required if a drill-out was required in the first ear, regardless of a successful outcome of the first procedure. Patients should be aware of the reduced likelihood of success in these cases and be counseled regarding risks and benefits of second-ear surgery based, in part, on the findings from the first ear. This study confirms that bilateral advanced footplate obliteration and overhanging or dehiscent facial nerves may be anticipated in patients found to have these abnormalities during first-ear stapedectomy.

Adenotonsillectomy for obstructive sleep apnea in obese children: effects on respiratory parameters and clinical outcome.

OBJECTIVE: To assess the efficacy of adenotonsillar surgery on respiratory sleep parameters and avoiding continuous positive airway pressure (CPAP) treatment in morbidly obese children with obstructive sleep apnea syndrome (OSAS). DESIGN: Retrospective. SETTING: Tertiary referral institution. PATIENTS: Children aged 2 to 18 years, with a body mass index (BMI) at or higher than the 95th percentile (adjusted for age and sex), undergoing adenotonsillar surgery for OSAS. INTERVENTIONS: Adenotonsillectomy. MAIN OUTCOME MEASURES: Preoperative and postoperative respiratory disturbance index, oxygen saturation nadir, overall severity of OSAS (mild, moderate, or severe) and candidacy for CPAP treatment were assessed and compared. Variables such as age, severity of disease, adenotonsillar size, and BMI z scores were compared between responders and nonresponders to surgical treatment. RESULTS: A total of 19 patients with full preoperative and postoperative data for evaluation were identified. The median (SD) age was 78 months (53.3 months). The median (SD) BMI z score was 2.84 (0.94). Eighteen patients (95%) had OSAS preoperatively to warrant CPAP treatment. Surgery reduced the overall median (SD) respiratory disturbance index from 20.7 (24.5) to 7.3 (14.9) (P<.001) and improved the median (SD) oxygen saturation nadir from 77.5% (16.3%) to 88.5 (13.1%) (P<.01). A total of 7 patients (37%) were cured by surgery. Ten patients (53%) had postoperative disease of sufficient severity to require CPAP. Surgery obviated the need for further treatment in only 8 (44%) of the 18 patients with preoperative disease warranting CPAP. No differences were identified between responders and nonresponders to surgical treatment. CONCLUSIONS: Adenotonsillar surgery improves sleep respiratory parameters in morbidly obese children with OSAS. Most patients have residual OSAS requiring further treatment.

Congenital cervical teratomas: diagnostic, management and postoperative variability.

OBJECTIVE: To review the diagnosis, management and outcomes of congenital cervical teratomas presenting to a tertiary referral centre. METHODS: Retrospective chart review of three cases presenting within an 18-month period. RESULTS: Of the three patients in this series, one was diagnosed antenatally. The remaining cases were diagnosed at birth. The antenatally diagnosed patient underwent an EXIT procedure whereby the airway was secured by tracheostomy. This patient subsequently died 30 min after separation from the materno-foetal circulation. Neither of the other two cases had any neonatal respiratory distress, despite having large tumours. Both patients had neonatal surgical excision of the teratomas performed. Both patients had postoperative respiratory distress, requiring intervention. Both patients made a full recovery. No recurrence has been reported. CONCLUSION: The antenatal diagnosis of large congenital cervical teratomas allows for planned intervention by experienced personnel. A successful outcome may not be obtained. All patients that undergo surgical excision of these tumours must be closely observed for post-operative respiratory distress, even in the absence of pre-operative symptoms.

Takayasu's arteritis and saddle nose deformity: a new association.

Aortitis and saddle nose deformity are extremely unusual manifestations of a variety of systemic diseases. The concurrent appearance of these apparently disparate clinical features is a clinical rarity. A case of saddle nose deformity in a patient with confirmed Takayasu's arteritis is presented. The relevant literature is reviewed with reference to the possible differential diagnosis of patients with aortitis and saddle nose deformity.

Parotid lymphomas--clinical and computed tomographic imaging features.

OBJECTIVE: To review the clinical presentation and computed tomography (CT) imaging characteristics of all parotid lymphomas diagnosed at the study institution over a 7-year period. DESIGN: Retrospective chart review of parotid lymphomas diagnosed between 1997 and 2004. SUBJECTS: A total of 121 patients with parotid lesions were identified. After retrospective chart review, a total of 10 patients with histologically proven parotid lymphoma were included in the study, 8 of whom had CT scans available for assessment. RESULTS: Ten patients with histologically proven lymphoma of the parotid gland were identified from among 121 patients with parotid neoplasms, an incidence in this series of 8.3%. All lymphomas were of non-Hodgkin's type. All patients presented with a painless unilateral parotid swelling. Most patients had a short history of less than 4 months' duration, of whom 3 presented with a rapidly evolving swelling of less then 1 month's duration. No patient had a background of Sjögren's disease or any other autoimmune disorders. The commonest finding noted on CT was of a unilateral, single mass of relative soft-tissue homogeneity with poorly defined, indistinct tumour margins. Associated loco-regional lymphadenopathy was identified in 2 cases, 1 clinically and another radiologically; multiple ipsilateral lesions were noted in 2 cases. No cases of contralateral disease were observed. CONCLUSION: Lymphoma has a clinical presentation similar to other neoplasms arising within the parotid gland. A unilateral, non-tender swelling was a universal finding. A history of less than 4 months may suggest the possibility of lymphoma. CT scanning is a useful adjunctive investigation to determine the site and extent of the disease, loco-regional nodal status and contralateral gland and neck status. Multifocality and associated adenopathy are associated with, but not exclusive to, parotid lymphoma. Although poor tumour boundary definition on CT imaging is a strong predictor of malignancy, no pathognomonic finding specific for lymphoma has been identified. The potential diagnosis of parotid lymphoma should be considered in all patients who present with a parotid mass.

Collet-Sicard syndrome: a rare presentation of metastatic prostate adenocarcinoma.

Metastatic spread of prostate adenocarcinoma to the temporal bone is rare. Involvement the jugular foramen may result in Collet-Sicard syndrome. This extremely uncommon clinical entity is characterized by paralysis of the lower four cranial nerves. A case of metastatic prostate adenocarcinoma involving the temporal bone causing a Collet-Sicard syndrome associated with an ipsilateral lower motor facial palsy and a mixed sensorineural and conductive hearing loss is presented. This case highlights the potential of prostate adenocarcinoma to cause symptoms referable to the temporal bone region and histopathologic analysis of biopsy material should include immunohistochemical staining for prostate specific antigen.

Spontaneous retropharyngeal and cervical emphysema: a rare singer's injury.

Spontaneous retropharyngeal and cervical emphysema is rare. We describe a case that was unusual in its etiology: the result of singing. Although this condition is usually benign, hospital admission for close observation and supportive therapy is prudent.

Extranodal lymphoma: clinical presentation and diagnostic pitfalls.

Non-Hodgkin's lymphomas (NHL) are known to present extranodally in 25% of cases, in contrast to Hodgkin's disease which rarely involves extranodal sites. In this article, the authors will to review the presentation of extranodal head and neck NHL and the difficulties that can be encountered in making the diagnosis in these cases.

Airway management of three cases of congenital cervical teratoma.

Cervical teratomas are rare congenital tumors derived from all three germ cell layers. The vast majority are histologically benign, but the significant size they may attain can potentiate life-threatening upper airway obstruction. All cases require the specialist airway skills of the pediatric anesthetist. This may be planned, in the case of antenatally diagnosed lesions, when the pediatric anesthetist is part of a multidisciplinary team involved in an EX utero Intrapartum Treatment (EXIT) or Operation On Placental Support (OOPS) procedure, or when a neonate is undergoing elective excision in the early neonatal period as definitive treatment. Alternatively the anesthetist may be called upon urgently to secure a compromised airway immediately postpartum when no antenatal diagnosis has been made. Furthermore, after elective surgical excision, airway compromise is possible, which may again require anesthetic intervention. The aim of this study is to report the authors' experience in managing the airway in three cases of congenital cervical teratoma in the study institution over the last 24 months. These cases highlight the possible airway scenarios that may confront the anesthetist in the immediate postpartum, elective surgery and postoperative stages and the variety of techniques that may be employed in order to overcome the potential difficulties encountered.