Faculty Bibliography

This single-institution phase II study was performed to estimate the response rate to lapatinib in neurofibromatosis type 2 (NF2) patients with progressive vestibular schwannoma (VS). Twenty-one eligible patients were enrolled. Brain and spine MRIs, including 3-dimensional volumetric tumor analysis, and audiograms were performed once at baseline and again every 12 weeks. The primary response end point was evaluable in 17 patients and defined as >/=15% decrease in VS volume. Hearing was evaluable as a secondary end point in 13 patients, with responses defined as an improvement in the pure tone average of at least 10 dB or a statistically significant increase in word recognition scores. Four of 17 evaluable patients experienced an objective volumetric response (23.5%; 95% confidence interval [CI], 10%-47%), with median time to response of 4.5 months (range, 3-12). In responders, reduction in VS volumes ranged from -15.7% to -23.9%. Four of 13 patients evaluable for hearing met hearing criteria for response (30.8%; 95% CI, 13%-58%). One sustained response exceeded 9 months in duration. Median time to overall progression (ie, volumetric progression or hearing loss) was 14 months. The estimated overall progression-free survival and volumetric progression-free survival at 12 months were 64.2% (95% CI, 36.9%-82.1%) and 70.6% (95% CI, 43.1%-86.6%), respectively. Toxicity was generally minor, and no permanent dose modifications were required. Lapatinib carries minor toxicity and has objective activity in NF2 patients with progressive VS, including volumetric and hearing responses. Future studies could explore combination therapy with other molecular targeted agents such as bevacizumab.

OBJECTIVES To assess the cross-sectional area and angle of the internal nasal valve more accurately by reformatting computed tomography (CT) scans of the nasal airway according to a more appropriate orientation than scans traditionally sectioned in the coronal plane and then to compare the results with clinical data on the nasal valve obtained from physical examination. METHODS We performed a retrospective review of the medical records of 24 rhinoplasty patients treated at a private practice facial plastic surgery office affiliated with a tertiary care university hospital. The patients had fine-cut (0.75-mm section) CT scans ordered for nasal airway obstruction or nasal valve compromise at the same institution. These patients were evaluated from January 1, 2000, through December 31, 2010. The previously acquired CT scans were reformatted to obtain sections through the internal nasal valve at a more appropriate orientation. The internal nasal valve cross-sectional area and valve angle were measured through a standardized section (1 cut immediately anterior to the head of the inferior turbinate) from the reformatted scans. The cross-sectional area was also measured through the same point on the traditionally oriented CT scan, and the values were compared. The results from each patient's scan were compared with data from the patient's medical record and analyzed against the patient's preoperative modified Cottle examination findings. RESULTS The CT scans oriented in the reformatted plane through the internal nasal valve provided a narrower valve angle than the traditionally oriented CT scans and more closely approximated the hypothesized true value of the internal nasal valve of 10 degrees to 15 degrees (P < .001). In a comparison of the same-side internal nasal valve angle and cross-sectional nasal valve area between the 2 different CT scan orientations, a statistically significant difference in the internal nasal valve angles between the 2 scan orientations was discovered, but this finding did not reach significance when distinguishing the nasal valve cross-sectional area. Finally, no correlation was found with regard to the preoperative modified Cottle maneuver scores for the internal nasal valve angle and cross-sectional valve area values in either scan orientation. CONCLUSIONS Precise preoperative evaluation of the internal nasal valve is critical to the workup for reconstruction or repair of problems that involve this area. Although tools such as acoustic rhinometry exist to evaluate the cross-sectional area of the nasal valve, many rhinoplasty surgeons do not have access to this expensive equipment. A CT scan with reformatting in the proper plane of the internal nasal valve can provide the surgeon with improved anatomical information to assess that region. With this in mind, however, the surgeon should always perform a thorough preoperative physical examination and treat the patient and his or her symptoms, not the imaging studies, when considering a candidate for a surgical intervention.

Approximately half of head and neck carcinomas arise from the oral cavity. Imaging plays an essential role in the preoperative evaluation of oral cavity carcinomas. MR imaging is particularly advantageous in the evaluation of the oral cavity, with better depiction of the anatomy in this region and reduction of dental artifacts compared with CT. MR is also the preferred imaging modality for the evaluation of bone marrow invasion and perineural tumor spread, which are findings critical for treatment planning. Advanced MR imaging techniques may potentially better delineate true tumor extent, determine lymph node metastases, and predict treatment response.

Langerhans cell histiocytosis (LCH) encompasses a range of clinical presentations. Pure osseous involvement is referred to as eosinophilic granuloma (EG), whereas systemic involvement can either be classified as Hand-Schuller-Christian disease or Letterer-Siwe syndrome. It is estimated that of the total incidence of LCH (0.5 per 100,000 children per year in the United States), nearly 70% are categorized as EG (1). We describe a case of clinically occult calvarial eosinophilic granuloma brought to medical attention only after a traumatic event led to scalp and epidural hemorrhage at the site of the lesion. Osseous EG initially presents as a painful or tender mass, at times even mimicking osteomyelitis. Computed tomography (CT) reveals a lytic lesion with beveled edges. Magnetic resonance (MR) imaging is often non-specific, but usually demonstrates a marrow-replacing process that is T1 hypointense, T2 hyperintense, with homogenous enhancement. The complex MR imaging findings in our case provided a unique perspective regarding the presentation of EG. Once pathology established eosinophilic granuloma, skeletal survey confirmed the lesion was solitary, despite a compelling history of present illness.

BACKGROUND: Sarcoidosis affects the central nervous system more frequently than previously appreciated. The diagnosis of neurosarcoidosis is often delayed, potentially leading to serious complications. Symptoms, when present, are not specific, may be subtle and resemble those of other neurologic diseases. REVIEW SUMMARY: During the past decade, significant progress has been made in understanding the epidemiology and pathophysiology of neurosarcoidosis, as well as the ability to diagnose and treat this disease. Studies have shown that the optimal diagnostic imaging modality for neurosarcoidosis is magnetic resonance imaging with gadolinium as it enhances visualization of granulomatous infiltration in neural tissue. Subclinical neurosarcoidosis may not be uncommon in patients with sarcoidosis. It is now evident that neurosarcoidosis does not invariably present as a catastrophic event. Adverse effects associated with high-dose systemic corticosteroids, the standard therapy, have discouraged practitioners from initiating treatment in the absence of significant symptomatic neurologic disease. However, other immunosuppressive agents as well newer biologic agents have emerged as an effective, well-tolerated therapeutic alternative to corticosteroids, which are often effective in corticosteroid-recalcitrant cases. CONCLUSION: Neurologists should be aware of the varying presentations of neurosarcoidosis since early recognition of neurologic involvement in patients with undiagnosed or proven sarcoidosis is currently possible and critical to the prevention of disabling complications

Although the pathogenic mechanisms of inflammatory bowel diseases are not fully understood, colonic microbiota may affect the induction of colonic inflammation, and some probiotics and prebiotics have been reported to suppress colitis. The inhibitory effects of brown rice fermented by Aspergillus oryzae (FBRA), a fiber-rich food, on the induction of acute colitis by dextran sulfate sodium (DSS) were examined. Feeding a 5% and 10% FBRA-containing diet significantly decreased the ulcer and erosion area in the rat colon stained with Alcian blue. In another experiment, 10% FBRA feeding decreased the ulcer index (percentage of the total length of ulcers in the full length of the colon) and colitis score, which were determined by macroscopic observation. It also decreased myeloperoxidase activity in the colonic mucosa. Viable cell numbers of Lactobacillus in the feces decreased after DSS administration and was reversely correlated with severity of colitis, while the cell number of Enterobacteriaceae increased after DSS treatment and was positively correlated with colitis severity. These results indicate that FBRA has a suppressive effect on the induction of colitis by DSS and suggest FBRA-mediated modification of colonic microbiota.