The role of vascular laboratory in the management of Eagle syndrome.

Eagle syndrome is caused by an elongated styloid process or calcified stylohyoid ligament. The stylocarotid variant with neurologic symptoms is rare and presents a diagnostic challenge. Patients may present with transient ischemic attacks, syncope, or less well defined symptoms like episodic dizziness. We report use of vascular laboratory testing in the management of Eagle syndrome. In one patient, on Doppler ultrasound examination of the ipsilateral temporal artery, the signal was lost with provocative neck flexion. In another patient, transcranial Doppler ultrasound showed blunting of the middle cerebral artery with provocative maneuvers. We used perioperative transcranial Doppler ultrasound to assess the effectiveness of styloid resection.

Tubeless Total Intravenous Anesthesia Spontaneous Ventilation for Adult Suspension Microlaryngoscopy.

INTRODUCTION: Maintaining spontaneous ventilation (SV) under total intravenous anesthesia (TIVA) without an endotracheal tube provides uninterrupted and unobstructed surgical access for suspension microlaryngoscopy (SML). This study describes the method and outcome of adults who underwent SML under tubeless TIVA-SV. METHODS: Retrospective review of adults who underwent SML between June 2014 and September 2016 using TIVA-SV without an endotracheal tube. RESULTS: Sixty-six cases in 36 patients were included with mean age of 50.6 years and 52.7% were female. Airway pathology included 41.6% subglottic or tracheal stenosis, 19.4% laryngeal lesion or mass, 16.7% glottic stenosis, 13.9% recurrent respiratory papilloma, and 8.3% supraglottic stenosis. Anesthesia was most commonly provided by continuous infusion of propofol and remifentanil (57.6%). Approximately half (53%) of cases received superior laryngeal nerve block. Average operative time was 72.9 minutes (range, 27-166 minutes). Eight cases required supplemental ventilation: 6 cases required transient endotracheal ventilation or mask ventilation, and 2 cases were converted to alternative ventilation. CONCLUSIONS: Total intravenous anesthesia is an attractive alternative to traditional endotracheal tube intubation, jet ventilation, or intermittent apneic ventilation for adult SML. During episodes of hypoventilation or desaturation, endotracheal ventilation, mask ventilation, or jet ventilation can effectively recover oxygenation.

Endoscopic management of congenital anterior glottic stenosis.

INTRODUCTION: Congenital anterior glottic stenosis (web) is a rare condition usually presenting with airway obstruction, stridor or dysphonia. Symptomatic infants may require tracheotomy to bridge the neonatal period. Early open surgical reconstruction may have significant risks and failure may still result in tracheotomy. We introduce an endoscopic surgical approach with balloon dilation for primary management of congenital anterior glottic stenosis. METHODS: We present three cases of congenital anterior glottic stenosis in children 7, 14, and 90 days old presenting with stridor, dyspnea, and dysphonia. The larynx was exposed by suspension microlaryngoscopy. The glottic stenosis was incised from a posterior to anterior direction using a laryngeal sickle knife. Subsequently, an airway balloon was guided through the stenotic lumen. Once the balloon was inflated, the balloon pressure was maintained for 30s or until the patient's oxygen saturation dropped below 92%. The dilation was repeated two or three times. The patients were kept intubated with an uncuffed endotracheal tube and monitored in the pediatric intensive care unit following surgery. RESULTS: All three patients were extubated within 72h without complications. One patient failed the first extubation attempt and was reintubated and successfully extubated 24h later. Patients were re-evaluated with direct microlaryngoscopy within two weeks. All patients had symptomatic relief and did not require further surgical intervention. CONCLUSION: Endoscopic balloon dilation laryngoplasty may be a safe and effective primary procedure for pediatric patients with congenital anterior glottic stenosis. It is technically simple and obviates the potential morbidities associated with an open surgical procedure or tracheotomy.

Transoral Sclerotherapy for Deep Space Cervical Lymphatic Malformations in Children With Acute Airway Compromise.

INTRODUCTION: Cervical lymphatic malformations in children rarely present with acute airway compromise. During an acute exacerbation or hemorrhage, lymphatic malformations involving the deep neck spaces may precipitate critical airway obstruction. These are rare clinical entities and tracheotomy is the standard procedure to bypass impending airway obstruction. METHODS: We present our recent experience with 2 children presenting with acute airway compromise resulting from deep space cervical lymphatic malformations and describe our technique and success with transoral sclerotherapy. RESULTS: Direct laryngoscopy-assisted transoral sclerotherapy with doxycycline may be considered an alternative to tracheotomy to address retropharyngeal and parapharyngeal space lymphatic malformations. CONCLUSIONS: Direct laryngoscopy-assisted transoral sclerotherapy is an excellent treatment option for children with deep space cervical lymphatic malformations with airway compromise. It is effective, provides direct access, and can be an alternative to a tracheotomy.

Facial nerve outcomes in facial nerve schwannomas.

OBJECTIVE: To better understand the characteristics and outcomes of facial nerve schwannomas (FNSs) over a 30-year period. STUDY DESIGN: Retrospective study. SETTING: Subspecialty practice at a tertiary hospital. PATIENTS: Fifty-six patients diagnosed with FNS over a 30-year period. METHODS: Preoperative data (audiologic data, facial nerve [FN] function, and patient symptoms), intraoperative data (tumor location, total versus subtotal resection, and FN status), and postoperative data (audiologic data, FN function, and recurrence) were collected. Mann-Whitney and χ2 analyses were done to determine which factors correlated with poor FN outcomes (defined as House-Brackmann ≥ 4). RESULTS: Of the 56 patients in this study, 53 (94.6%) underwent surgical resection of their FNS. Of those patients, 45 (84.9%) underwent total resection, and 8 (15.1%) underwent subtotal resection. Subtotal resection was associated with a statistically significant decreased risk of having postoperative HB grade ≥ 4 (odds ratio, 0.09; 95% confidence interval, 0.01-0.77; p = 0.028). Of those undergoing a subtotal resection, no patient had further tumor growth seen on postoperative magnetic resonance imaging (average time of last magnetic resonance imaging since operation, 44.9 mo). Tumor location was not statistically associated with poor FN outcome (all p > 0.05). Preoperative FN paralysis was the only preoperative clinical finding statistically associated with poor FN outcomes (p = 0.004). CONCLUSION: We have identified multiple characteristics of FNS as well as multiple factors associated with increased statistical risk of poor FN outcomes.

Management of cerebrospinal fluid leaks after vestibular schwannoma surgery.

OBJECTIVE: To evaluate the incidence and treatment of cerebrospinal fluid (CSF) leaks after resection of vestibular schwannomas and to propose a treatment algorithm for their management. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: Review of 1,922 subjects who underwent resection of vestibular schwannomas from 1970 to 2010. INTERVENTION: Surgical resection of vestibular schwannoma. MAIN OUTCOME MEASURES: Patient demographics, surgical approach used, CSF leak incidence, meningitis, treatment, and success in the management of CSF leaks. RESULTS: Postoperative CSF leaks were observed in 12.9% of our patients. There was no significant difference between the type of approach and the presence of CSF leak with translabyrinthine, suboccipital and middle fossa CSF leak rates of 12%, 12%, and 13%, respectively (p = 0.07). Patients presented with a wound leak or rhinorrhea almost equally. Ultimately, 92% of patients with rhinorrhea underwent surgical intervention. The probability of a patient with rhinorrhea requiring a second intervention was higher when the initial intervention was conservative rather than surgical. However, the probability of a patient with a wound leak requiring a second intervention was essentially the same when initially treated conservatively or surgically. CONCLUSION: Our data suggests that there is no difference in CSF leak rates between the different surgical approaches. The appropriate treatment strategy is dependent on the presentation of the CSF. Although conservative treatment is effective for managing wound leaks, it is less effective in managing patients with rhinorrhea. Therefore, surgical treatments should play an early role in the treatment algorithm of patients with CSF rhinorrhea.

Natural progression of dysplasia in adult recurrent respiratory papillomatosis.

OBJECTIVES: Recurrent respiratory papillomatosis (RRP) is often described as a benign disease. However, the natural progression of dysplasia and transformation to squamous cell carcinoma has not been elucidated for RRP. This study delineates our extensive experience with dysplasia in RRP. STUDY DESIGN/SETTING: Case series with chart review. SUBJECTS AND METHODS: Demographic data and surgical pathology were analyzed for patients diagnosed with RRP at greater than 18 years of age who underwent operative intervention without cidofovir treatment for RRP between 2004 and 2009. RESULTS: Fifty-four patients were identified. Dysplasia was identified in 27 of 54 patients (50%). Of the 54 patients, 50% had no dysplasia, 26% had mild dysplasia (grade 1), 11% had moderate dysplasia (grade 2), 4% had severe dysplasia (grade 3), 7% had carcinoma in situ, and 2% had squamous cell carcinoma as the highest documented degree of dysplasia. Thirty of 54 patients (55.6%) had 2 or more operative interventions. Nine of the 30 patients (30%) developed a higher dysplastic grade during the course of treatment. Time to progression averaged 16.2 ± 8.7 months for patients with initially benign disease. Of those patients with dysplasia progression, only 1 of 9 (11.1%) developed squamous cell carcinoma. Patients presenting with benign or mild dysplasia typically did not progress beyond mild dysplasia (22 of 24, 91.7%). CONCLUSIONS: Dysplasia is common in RRP. Progression of dysplasia, especially with an initial dysplastic grading of benign or mild disease, is rare.

Regenerative effects of basic fibroblast growth factor on extracellular matrix production in aged rat vocal folds.

OBJECTIVES: We investigated acute changes in extracellular matrix (ECM) gene expression and histologic changes in the deposition of collagen and hyaluronan (hyaluronic acid; HA) after basic fibroblast growth factor (bFGF) treatment of the aged rat vocal fold. METHODS: For the polymerase chain reaction (PCR) experiments, we divided ten 18-month-old Sprague-Dawley rats into two groups that received serial injections of sham (saline solution) or bFGF (2 ng/microL) and euthanized them 2 weeks after the initial injection to investigate acute changes in ECM gene expression. We treated a separate group of 5 animals unilaterally and sacrificed them 4 weeks after the initial injection to investigate histologic changes in the deposition of collagen and HA. RESULTS: Real-time PCR revealed significantly up-regulated HA synthase (HAS)-2, HAS-3, matrix metalloproteinase (MMP)-2, and procollagen type I gene expression in the bFGF treatment group as compared to the sham treatment group. Histologic staining revealed significantly increased deposition of HA in the bFGF-treated vocal fold as compared to the sham-treated vocal fold. No differences in ECM collagen levels were observed between treatment sides. CONCLUSIONS: Basic fibroblast growth factor induced the up-regulation of HAS-2, HAS-3, MMP-2, and procollagen type I. Histologically, aged vocal folds treated with bFGF revealed increased deposition of HA as compared to sham-treated vocal folds.

Regeneration of aged rat vocal folds using hepatocyte growth factor therapy.

OBJECTIVES/HYPOTHESIS: We investigated acute changes in extracellular matrix gene expression and histologic changes in the deposition of collagen and hyaluronan (HA) from hepatocyte growth factor (HGF) treatment of the aged rat vocal fold. We hypothesized that: 1) HGF induces matrix metalloproteinase gene expression, which might contribute to the downregulation of collagen; and 2) HGF induces hyaluronan synthase (HAS) gene expression, which might play a role in the upregulation of extracellular matrix HA. STUDY DESIGN: Prospective animal study. METHODS: Fifteen, 18-month-old, Sprague-Dawley rats were involved in this study. For gene expression analyses, 10 rats were divided into two groups and received serial injections of sham (saline) or HGF (2 ng/microL) and sacrificed 2 weeks after the initial injection to investigate acute changes in extracellular matrix gene expression. A separate group of five animals received the above treatment and were sacrificed 4 weeks after the initial injection to investigate histologic changes in the deposition of collagen and HA. RESULTS: Real-time polymerase chain reaction revealed significantly upregulated matrix metalloproteinase(MMP)-2, -9, and HAS-3 messenger RNA (mRNA) expression and significantly downregulated procollagen type I mRNA expression in the HGF-treatment group, compared to the sham-treatment group. Histologic staining revealed significantly reduced collagen deposition and increased deposition of HA in the HGF-treated vocal fold, compared to the sham-treated vocal fold. CONCLUSIONS: HGF induced the upregulation of MMP-2, -9, and HAS-3, and downregulated the expression of procollagen type I. Histologically, aged vocal folds treated with HGF revealed decreased collagen deposition, and increased deposition of HA, compared to sham-treated vocal folds.

Hemangiosarcoma in a South American sea lion (Otaria byronia).

A 10-yr-old male South American sea lion (Otaria byronia) died after several weeks of depression, anorexia, weight loss, and progressive respiratory distress. At necropsy, three confluent, lobulated, dark-red masses were noted in the mesentery. Similar masses were also observed in the lung and both kidneys. Hemangiosarcoma was diagnosed based on gross findings, histopathology, and immunohistochemistry. This is the first case of hemangiosarcoma reported in pinnipeds.