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A 3-month-old male with a chief complaint of episodic choking with feeds and a hoarse cry is presented. Left eye ptosis and asymmetric soft palate elevation were detected on physical examination. Fiberoptic examination showed a left vocal fold paresis and pooling of secretions in the pyriform sinuses. MRI demonstrated an ill-defined lesion at the left jugular foramen extending into the left carotid sheath. A fine needle biopsy revealed spindle shaped cells consistent with fibromatosis. The histopathology of fibromatosis and the differential diagnosis of jugular foramen masses in children will be described. To our knowledge, this represents the earliest reported case of fibromatosis in the jugular foramen

OBJECTIVES/OBJECTIVE:Congenital anterior skull base defects with meningoencephaloceles causing nasal obstruction are rare clinical entities. Nasal obstruction in children may also be a symptom of multiple benign nasal and allergic disorders, making the initial diagnosis of meningoencephalocele difficult. Traditionally, skull base defects have been repaired via a bifrontal craniotomy approach. With the advent of pediatric endoscopic instrumentation, more of these lesions are accessible via an intranasal endoscopic approach, even in the infant population. However, owing to the rarity of these lesions, there is a paucity of data demonstrating successful adaptation of endoscopic skull base techniques to the pediatric population. METHODS:Retrospective review of two cases of endoscopic repair of anterior skull base defects with meningoencephaloceles at a tertiary care medical center. RESULTS:Two children, ages 15 months and 6 years, underwent successful endoscopic closure of their anterior skull base defects and resection of their intranasal meningoencephalocele with resolution of their nasal obstruction and cerebrospinal fluid rhinorrhea. CONCLUSIONS:Pediatric nasal meningoencephaloceles with anterior skull base defects can be successfully repaired via a transnasal endoscopic technique, thus minimizing the complications associated with craniotomy and frontal lobe retraction. Triplanar computed tomographic and magnetic resonance imaging is paramount to evaluate the caliber of the skull base defect, consistency of the herniated intracranial contents, as well as the presence of cerebral vasculature.

BACKGROUND: It is unclear whether there is a benefit to resection of primary gastrointestinal carcinoid neoplasm with hepatic metastases. We investigated whether primary tumor resection in this setting led to a significant difference in outcomes. METHODS: A retrospective review of patients with abdominal carcinoid neoplasms between 1995 and 2006 was performed. Data collected on patients with proven carcinoid liver metastases at initial diagnosis included whether the primary neoplasm was resected, time to progression of liver metastases, and status at last follow-up. Progression-free survival and survival were calculated by the method of Kaplan-Meier and compared by the log-rank test. RESULTS: There were 84 patients, 60 of whom had their primary neoplasm resected. The resected group had a greater median progression-free survival of 56 months, compared with 25 months for the primary nonresected group (P < .001). Median survival time for the resected group was longer at 159 months, compared with 47 months for the nonresected group (P < .001). CONCLUSIONS: Resection of the primary neoplasm is associated with better progression-free survival and overall survival in patients with abdominal carcinoid neoplasms. Therefore, localization and resection of the primary neoplasm should be considered, even among patients in whom the primary neoplasm is asymptomatic.

OBJECTIVE: To evaluate with a new otologic database the results of primary stapes surgery for otosclerosis with up to 14 years of follow-up in a consecutive series of 2,525 patients operated on by the same surgeon with the same technique (stapedotomy and vein graft interposition) and to provide online access to the complete data of this study for the reviewers. To study the effect of specific operative findings (obliterative otosclerosis and simultaneous malleus ankylosis) and age at the time of surgery on the long-term outcome. STUDY DESIGN: Prospective clinical study using a new computerized otologic database. SETTING: : Tertiary referral center. PATIENTS: Two thousand five hundred twenty-five patients who underwent 3,050 stapedotomies for otosclerotic stapes fixation were enrolled in this study from January 1991 to December 2004. Separate analyses were made for two unique pathologies (92 cases of obliterative otosclerosis and 19 cases of simultaneous malleus ankylosis) diagnosed during surgery and for patients in two age brackets (or=65 yr [302 patients]). INTERVENTION: Stapedotomy with vein graft interposition and reconstruction with either a Teflon piston, a bucket handle prosthesis, or a total prosthesis. MAIN OUTCOME MEASURES: Preoperative and postoperative audiometric evaluation using conventional audiometry. Air-bone gap (ABG), bone-conduction thresholds, and air-conduction thresholds were all assessed. Postoperative audiometry was performed at 3, 6, 9, 12, 18, and 24 months and then annually for 14 years. RESULTS: Overall, the postoperative ABG was closed to 10 dB in 94.2% of cases. The mean four-frequency postoperative ABG was 1.7 dB compared with 25.6 dB preoperatively. The mean four-frequency bone-conduction thresholds were unchanged postoperatively. A significant postoperative sensorineural hearing loss (SNHL; >15 dB) was seen in 0.5% of cases in this series. Postoperative ABG was achieved to within 10 dB in 95% of cases of obliterative otosclerosis and in 64.7% of cases of simultaneous malleus ankylosis. A significant postoperative SNHL (>15 dB) was seen in 4.8% of cases of obliterative otosclerosis and was not observed in any cases of simultaneous malleus ankylosis. Postoperative ABG was achieved to within 10 dB in 93.5% of cases in the pediatric series and in 94.5% of cases in the senior series. A significant postoperative SNHL (>15 dB) was seen in 0.7% of cases in the senior group but was not observed in the children. CONCLUSION: Using a new otologic database, our series confirms that stapedotomy with vein graft interposition for otosclerotic stapes fixation is a safe and successful treatment for long-term hearing improvement. The deterioration in hearing with time after stapedotomy did not exceed the rate of hearing loss because of presbyacusis. Therefore, argon laser stapedotomy with vein graft interposition is our preferred surgical technique in the treatment of otosclerosis. Obliterative otosclerosis and simultaneous malleus ankylosis may be encountered during stapedotomy. Our study shows that reasonable success rates can still be expected in these situations. Stapedotomy results in the elderly and in children are comparable to those obtained in patients of other groups of age undergoing surgery for otosclerosis without an increased risk for complications.