Chondroblastoma-like mass of the temporal bone, secondary aneurysmal bone cyst, and intracerebral hemorrhage in a patient with cardiofaciocutaneous syndrome: case report.

Cardiofaciocutaneous syndrome (CFCS) is a rare developmental disorder that is phenotypically similar to Noonan syndrome and is associated with mutations in BRAF, MEK1, MEK2, and KRAS. The relationship between malignancy risk and CFCS is unclear with few cases published in the literature. The purpose of this paper is to describe the case of a patient with CFCS presenting in extremis as a result of a large intracerebral hemorrhage arising from a temporal bone mass with histopathology most consistent with chondroblastoma and secondary aneurysmal bone cyst. This is the first case to document an association between CFCS and chondroblastoma.

Fluorescence Imaging of Nerves During Surgery.

OBJECTIVE: This review details the agents for fluorescence-guided nerve imaging in both preclinical and clinical use to identify factors important in selecting nerve-specific fluorescent agents for surgical procedures. BACKGROUND: Iatrogenic nerve injury remains a significant cause of morbidity in patients undergoing surgical procedures. Current real-time identification of nerves during surgery involves neurophysiologic nerve stimulation, which has practical limitations. Intraoperative fluorescence-guided imaging provides a complimentary means of differentiating tissue types and pathology. Recent advances in fluorescence-guided nerve imaging have shown promise, but the ideal agent remains elusive. METHODS: In February 2018, PubMed was searched for articles investigating peripheral nerve fluorescence. Key terms used in this search include: "intraoperative, nerve, fluorescence, peripheral nerve, visualization, near infrared, and myelin." Limits were set to exclude articles exclusively dealing with central nervous system targets or written in languages other than English. References were cross-checked for articles not otherwise identified. RESULTS: Of the nonspecific agents, tracers that rely on axonal transport showed the greatest tissue specificity; however, neurovascular dyes already enjoy wide clinical use. Fluorophores specific to nerve moieties result in excellent nerve to background ratios. Although noteworthy findings on tissue specificity, toxicity, and route of administration specific to each fluorescent agent were reported, significant data objectively quantifying nerve-specific fluorescence and toxicity are lacking. CONCLUSIONS: Fluorescence-based nerve enhancement has advanced rapidly over the past 10 years with potential for continued utilization and progression in translational research. An ideal agent would be easily administered perioperatively, would not cross the blood-brain barrier, and would fluoresce in the near-infrared spectrum. Agents administered systemically that target nerve-specific moieties have shown the greatest promise. Based on the heterogeneity of published studies and methods for reporting outcomes, it appears that the development of an optimal nerve imaging agent remains challenging.

Koos Classification of Vestibular Schwannomas: A Reliability Study.

BACKGROUND: The Koos classification of vestibular schwannomas is designed to stratify tumors based on extrameatal extension and compression of the brainstem. While this classification system is widely reported in the literature, to date no study has assessed its reliability. OBJECTIVE: To assess the intra- and inter-rater reliability of the Koos classification system. METHODS: After institutional review board approval was obtained, a cross-sectional group of the Magnetic Resonance imagings of 40 patients with vestibular schwannomas varying in size comprised the study sample. Four raters were selected to assign a Koos grade to 50 total scans. Inter- and intrarater reliability were calculated and reported using Fleiss' kappa, Kendall's W, and Intraclass correlation coefficient (ICC). RESULTS: Inter-rater reliability was found to be substantial when measured using Fleiss' kappa (.71), extremely strong using Kendall's W (.92), and excellent as calculated by ICC (.88).Intrarater reliability was perfect for 3 out of 4 raters as assessed using weighted kappa, Kendall's W and ICC, with the intrarater agreement for the fourth rater measured as extremely high. CONCLUSION: We have demonstrated that the Koos classification system for vestibular schwannoma is a reliable method for tumor classification. This study lends further support to the results of current literature using Koos grading system. Further studies are required to evaluate its validity and utility in counseling patients with regard to outcomes.

The Cleating Stitch: An Adjunctive Technique for Percutaneous and Revision Osseointegration Screws.

OBJECTIVE: The bone anchored hearing aid (BAHA) has become a widely used and successful option in treatment of conductive and mixed hearing loss, and single sided deafness. Despite improvements in technique and cosmesis, complications remain that can result in implant revision or removal. Herein we describe a unique adjunctive technique, the cleating stitch, in placement of osseointegration screws and examine its impact on complication rates. STUDY DESIGN: Retrospective case review. SETTING: Tertiary academic medical center. PATIENTS: A total of 66 implants in 65 patients (35 men, 30 women) with an average age of 54 years (15-81 yr). Average follow up 10.8 months. INTERVENTION: All patients underwent BAHA implant placement by a single surgeon between April 2012 and June 2017 using the linear incision or punch techniques with soft tissue reduction and placement of a cleating stitch. MAIN OUTCOME MEASURE: Main outcome measures include rates of revision surgery, overgrowth, extrusion, and Holgers reaction ≥2. Secondary outcome measures include associations between main outcome measures and outlying factors (obesity, smoking, diabetes mellitus, coronary artery disease, age). RESULTS: The overall rate of revision was 3%, rate of overgrowth 1.5%, rate of extrusion 1.5%, and Holgers reaction ≥2 10.6%. Overgrowth and extrusion both required revision. Older age was associated with decreased risk of Holgers reaction ≥2 (p = 0.03) with a hazard ratio of 0.95 (confidence interval 0.9-1.0). There were no other statistically significant associations between primary outcome measures and outlying factors. CONCLUSION: The cleating stitch is an effective adjunctive technique in placement of osseointegration screws associated with low rates of overgrowth and overall revision surgery.

Current practices in vestibular schwannoma management: a survey of American and Canadian neurosurgeons.

OBJECTIVES: Comprehensive therapy for vestibular schwannomas has changed dramatically over the past fifty years. Previously, neurosurgeons were most likely to treat these tumors via an independent surgical approach. Currently, many neurosurgeons treat vestibular schwannomas employing an interdisciplinary team approach with neuro-otologists and radiation oncologists. This survey aims to determine the current treatment paradigm for vestibular schwannomas among American and Canadian neurosurgeons, with particular attention to the utilization of a team approach to the surgical resection of these lesions. METHODS: A seventeen part survey questionnaire was sent by electronic mail to residency trained members of the American Association of Neurological Surgeons currently practicing in Canada or the United States. Questions were divided into groups regarding physician background, overall practice history, recent practice history, opinions on treatment paradigms, and experience with an interdisciplinary team approach. RESULTS: Seven hundred and six responses were received. The vast majority of neurosurgeons surgically resect vestibular schwannomas as part of an interdisciplinary team (85.7%). Regional variations were observed in the use of an interdisciplinary team: 52.3% of responding neurosurgeons who surgically treat vestibular schwannomas without neuro-otologists currently practice in the South (no other region represented more than 15.4% of this group, p=0.02). Surgeons who have treated >50 vestibular schwannomas show a trend towards more frequent utilization of an interdisciplinary approach than less experienced surgeons, but this did not reach statistical significance. CONCLUSIONS: The majority of neurosurgeons in the United States and Canada surgically resect vestibular schwannomas via an interdisciplinary approach with the participation of a neuro-otologist. Neurosurgeons in the South appear more likely to surgically treat these tumors alone than neurosurgeons in other regions of the U.S. and Canada.

Outcomes following temporal bone resection.

OBJECTIVES/HYPOTHESIS: To evaluate survival outcomes in patients undergoing temporal bone resection. STUDY DESIGN: Retrospective review. METHODS: From 2002 to 2009 a total of 65 patients underwent temporal bone resection for epithelial (n = 47) and salivary (n = 18) skull base malignancies. Tumor characteristics, defect reconstruction, and postoperative course were assessed. Outcomes measured included disease-free survival and cancer recurrence. RESULTS: The majority of patients presented with recurrent (65%), advanced stage (94%), cutaneous (72%), and squamous cell carcinoma (57%). Thirty-nine patients had perineural invasion (60%) and required facial nerve resection; 16 (25%) had intracranial extension. Local (n = 6), regional (n = 2), or free flap (n = 46) reconstruction was required in 80% of patients. Free flap donor sites included the anterolateral thigh (31%), radial forearm free flap (19%), rectus (35%), and latissimus (4%). The average hospital stay was 4.9 days (range, 1-28 days). The overall complication rate was 15% and included stroke (n = 4), cerebrospinal fluid leak (n = 2), hematoma formation (n = 1), infection (n = 1), flap loss (n = 1), and postoperative myocardial infarction (n = 1). A total of 22 patients (34%) developed cancer recurrence during the follow-up period (median, 10 months), 17 (77%) of whom presented with recurrent disease at the time of temporal bone resection. Two-year disease-free survival was 68%, and 5-year disease-free survival was 50%. CONCLUSIONS: Aggressive surgical resection and reconstruction is recommended for primary and recurrent skull base malignancies with acceptable morbidity and improved disease-free survival.

Evolution of a paradigm for free tissue transfer reconstruction of lateral temporal bone defects.

BACKGROUND: Tumors of the lateral skull base are best treated with surgery plus or minus radiation therapy. Surgical ablation may involve cutaneous structures, the auricle, the parotid, and the lateral temporal bone. These composite soft tissue defects are best reconstructed with composite tissue. Multiple pedicled flaps have been used to reconstruct these defects. Free flaps have been shown to provide the best tissue for these reconstructions. We review our experience and present an algorithm for their reconstruction. METHODS: A case series of consecutive patients treated between 1999 and 2006 at 2 tertiary care institutions, Oregon Health and Science University and University of Alabama at Birmingham were reviewed. There were 73 patients who had periauricular defects requiring 74 free tissue transfers in this retrospective chart review. All defects had extensive cutaneous loss and underwent some form of parotidectomy. There were 57 lateral temporal bone defects and 16 periauricular defects where the external auditory canal was preserved. The majority of patients had nonmelanoma skin malignancies (65%). Eighty percent of patients had undergone previous treatment (radiation therapy, surgery, or a combination therof). RESULTS: Early on, reconstruction was performed using a radial forearm (RFFF, n=29), evolving to lateral arm (n=6), rectus (n=11), and finally an anterolateral thigh (ALT, n=28) free flap. The average hospital stay was 6 days, and the overall complication rate was 22%. The rectus flap needed debulking in 34% of patients, and the anterolateral thigh in 9%. Periauricular defects were classified based on preservation of the external auditory canal (class I), lateral temporal bone resection with preservation of the auricle (class II), or lateral temporal bone with total auriculectomy (class III). CONCLUSION: Class I defects were best managed by RFFF reconstruction, class II defects were managed well with the ALT flap, and class III defects required the ALT or rectus flap.

Pretreatment, preoperative swallowing exercises may improve dysphagia quality of life.

OBJECTIVES: Dysphagia is commonly associated with head and neck cancer treatment. Traditional dysphagia management strategies focus on post-treatment therapy. This study evaluated the utility of pretreatment swallowing exercises in improving post-treatment swallowing quality of life (QOL). STUDY DESIGN: Prospective cohort study and cross-sectional QOL analysis. METHODS: This study includes 37 patients who underwent primary radiation or combined chemoradiation treatment for newly diagnosed hypopharyngeal, laryngeal, or oropharyngeal primary tumors at the University of Alabama at Birmingham. Of the 37, 25 patients underwent swallowing exercises beginning 2 weeks prior to the start of radiation. The M.D. Anderson Dysphagia Inventory (MDADI) was administered an average of 14 months after treatment to assess the success of the protocol. Analysis of QOL scores related to gender, primary site, stage, and race were obtained. RESULTS: Patients who performed pretreatment swallowing exercises (n = 25) showed improvement in the overall MDADI score (P = .0002) compared to the control population (n = 12) who underwent post-treatment therapy. Furthermore, a separate analysis of individual domains of the MDADI (global, emotional, functional, and physical) demonstrated improved quality of life. CONCLUSIONS: Implementation of pretreatment swallowing education and exercise may improve dysphagia-specific QOL in head and neck cancer patients undergoing radiation and/or chemoradiation therapy.

Impact of mastoidectomy on simple tympanic membrane perforation repair.

OBJECTIVES/HYPOTHESIS: Mastoidectomy has long been identified as an effective method of treatment for chronic ear infection. The effect of mastoidectomy on patients without evidence of active infectious disease remains highly debated and unproven. The objective in the study was to examine the impact of mastoidectomy on the repair of uncomplicated tympanic membrane perforations. STUDY DESIGN: Retrospective study of patients at tertiary referral center. METHODS: Four hundred eighty-four patients who underwent surgical repair of simple tympanic membrane perforations were identified and reviewed in a retrospective manner. Simple tympanic membrane perforations were defined as tympanic membrane perforations of any size and location without any of the following confounding variables: 1). active infection (active otorrhea, abnormal middle ear mucosa, or granulations tissue); 2). ossicular abnormalities (ossicular fixation, ossicular discontinuity, ossicular malformation, or ossicular absence); 3). cholesteatoma; or 4). prior attempt at tympanic membrane repair (prior tympanoplasty or mastoidectomy). Surgical outcome and clinical course were assessed to compare results of tympanic membrane perforation repair with and without canal wall up mastoidectomy. RESULTS: Tympanic membrane repair was equally effective in both groups at 91%. Hearing results were comparable. Development of persistent ipsilateral otological disease requiring a subsequent ipsilateral procedure was approximately twice as common in the tympanoplasty group. In the tympanoplasty group, 14.1% of patients underwent subsequent ipsilateral otological procedures, and 6.1% of patients in the tympanoplasty with mastoidectomy intact canal wall group underwent subsequent ipsilateral procedures (P <.05). The most common subsequent ipsilateral procedures were tympanoplasty, tympanostomy tube placement, tympanoplasty with mastoidectomy canal wall up, and tympanoplasty with mastoidectomy canal wall down, in that order. After including untreated tympanic membrane perforations as subsequent procedures, the adjusted rate of subsequent procedures was 15.5% in the tympanoplasty group and 12.2% in the tympanoplasty with mastoidectomy group (P >.05). CONCLUSION: Mastoidectomy was not necessary for successful repair of simple tympanic membrane perforations. However, mastoidectomy impacted the clinical course in patients by reducing the number of patients requiring future procedures and by decreasing disease progression. This suggests that even in the absence of active evidence of infection, mastoidectomy improved the underlying disease process. Combining mastoidectomy with tympanoplasty during repair of simple perforations in patients with no active evidence of infection remains an appropriate option and may be valuable in reducing the need for future surgery.

Reconstruction of the entire ossicular conduction mechanism.

OBJECTIVES/HYPOTHESIS: Stapes fixation combined with fixation, absence, or malformation of the malleus-incus complex requires an uncommon surgical reconstruction and offers a unique combination of challenges and hazards. This situation may occur in the presence of severe tympanosclerosis, otosclerosis, congenital ossicular malformations, and revision surgery for either stapedectomy or chronic ear disease. In previous reports, this procedure has been grouped with total ossicular reconstruction without much distinction. However, the challenges unique to this problem deserve special consideration. The present report offers a treatment plan for a group of patients requiring reconstruction of the entire ossicular conduction mechanism including removal of the stapes footplate. STUDY DESIGN: Retrospective review. METHODS: Three thousand three hundred fifty (3350) charts of patients requiring total ossicular replacement prostheses (TORPs) were reviewed. Of this group of patients, only 21 of 3350 patients from 1977 to 1999 required TORP placement and removal of the stapes footplate. The patients were followed for an average period of 50 months. RESULTS: Hearing results indicated an overall improvement in the air-bone gap of 10 dB, with 52% achieving an air-bone gap of less than 20 dB. Of the 21 cases, 5 revision surgeries were performed. Three were performed because of a displaced TORP (14.2%). and 2 were performed because of extruded TORPs (9.5%). CONCLUSIONS: Reconstruction of the entire ossicular conduction mechanism including removal of the stapes footplate can be successfully achieved with improvement of the air-bone gap of less than 20 dB. Hearing results and extrusion rates are comparable to reported results of TORP placement on a mobile footplate. Successful stapedectomy and simultaneous ossicular chain reconstruction can be performed as a single or staged procedure. Special attention is paid to avoid intrusion of the prosthesis into the vestibule.

Lateral skull base malignancies.

OBJECT: Historically poor outcomes have been characteristic in patients with lateral skull base malignancies. As advances in skull base surgical techniques have been made, complete resection has increasingly been achieved. This has resulted in improved survival rates and local tumor control. METHODS: The authors performed a retrospective review of 95 patients treated for lateral skull base malignancies. The mean age of the patients was 49.4 years. There were 44 females and 51 males. The mean follow-up period was 50 months. Resection was performed in all patients, and postoperative radiotherapy was undertaken in 54% of the cases. Local disease control was maintained in 73% of the patients. Tumor involvement of the facial nerve and intracranial tumor extension did not jeopardize the rate of local control. CONCLUSIONS: Despite the fact that technical advances in skull base surgery have resulted in a higher incidence of complete tumor resection and improved survival rates, a respect for the poor prognosis historically associated with lateral skull base malignancies should be maintained and treatment should be appropriately aggressive.