High-risk human papillomavirus 16/18 associated with improved survival in sinonasal squamous cell carcinoma.

BACKGROUND: There has been conflicting evidence on the independent prognostic role of human papillomavirus (HPV) status in sinonasal cancer. The objective of this study was to assess whether the survival of patients with sinonasal cancer differs based on various HPV statuses, including HPV-negative, positive for the high-risk HPV-16 and HPV-18 (HPV16/18) subtypes, and positive for other high-risk and low-risk HPV subtypes. METHODS: In this retrospective cohort study, data from the National Cancer Database were extracted from the years 2010-2017 for patients who had primary sinonasal cancer (N = 12,009). The outcome of interest was overall survival based on HPV tumor status. RESULTS: Study included an analytic cohort of 1070 patients with sinonasal cancer who had confirmed HPV tumor status (732 [68.4%] HPV-negative; 280 [26.2%] HPV16/18-positive; 40 [3.7%] positive for other high-risk HPV; and 18 [1.7%] positive for low-risk HPV). HPV-negative patients had the lowest all-cause survival probability at 5 years postdiagnosis (0.50). After controlling for covariates, HPV16/18-positive patients had a 37% lower mortality hazard than HPV-negative patients (adjusted hazard ratio, 0.63; 95% confidence interval [CI], 0.48-0.82). Patients aged 64-72 years (crude prevalence ratio, 0.66; 95% CI, 0.51-0.86) and 73 years and older (crude prevalence ratio, 0.43; 95% CI, 0.31-0.59) presented with lower rates of HPV16/18-positive sinonasal cancer than those aged 40-54 years. In addition, Hispanic patients had a 2.36 times higher prevalence of non-HPV16/18 sinonasal cancer than non-Hispanic White patients. CONCLUSIONS: These data suggest that, for patients with sinonasal cancer, HPV16/18-positive disease may confer a significant survival advantage compared with HPV-negative disease. Other high-risk and low-risk HPV subtypes have survival rates similar to the rates for HPV-negative disease. HPV status might be an important independent prognostic factor in sinonasal cancer that could be used in patient selection and clinical decisions.

The relationship between round window and ear canal Cochlear microphonic.

Hypothesis: Cochlear microphonic recorded at ear canal (CM-EC) can be a substitute for the one recorded at round window (CM-RW). Background: Almost all clinics do not measure tone-burst evoked CM due to technical difficulty although it can provide more information than click evoked CM. Moreover, clinicians like the CM-EC more than that measured at CM-RW because CM-EC is non-invasive. There is difference between CM-RW and CM-EC, for example, CM-EC is less prominent than CM-RW, therefore, studying tone-burst evoked CM-EC and its relationship with CM-RW are highly significant and can promote the clinical application of CM-EC. Method: Nine guinea pigs were randomly allocated into three groups, group 1 was not exposed to noise, called normal control. group 2 and group 3 were exposed to the low- (0.5-2 kHz) and high-frequency band-noise (6-8 kHz) at 120 dB SPL for 1 h, respectively. It was difficulty to record low-frequency CM due to severe environmental interruption, in current study the recording technology of tone-burst evoked CM was optimized so that tone-burst evoked CM was measured across full speech frequency (0.5-8 kHz) in the presence of normal hearing and noise induced hearing loss (NIHL). Results: CM-RW and CM-EC were successfully recorded across speech frequency. Significant reduction in CM amplitude was observed at 0.5 and 2 kHz in group 2, at 6 and 8 kHz in group 3 as compared to group 1, p < .05, indicating that CM amplitude was sensitive to band-noise exposure. Significant correlation between CM-RW and CM-EC was also verified, p < .05. Conclusion: CM-EC is a useful objective test for evaluation of hearing function; the result of current study supports the clinical application of non-invasive CM-EC.

Optimizing the Measurement of 0.5-kHz Cubic Distortion Product Otoacoustic Emission.

BACKGROUND: The measurement of low-frequency cubic distortion product otoacoustic emission, for example, 0.5-kHz cubic distortion product otoacoustic emission, is often severely affected by background noise, and currently 0.5-kHz cubic distortion product otoacoustic emission is not commonly applicable in clinical setting. METHODS: The fundamental part of current study was the optimization of recording technology to reduce noise interference with the measurement of 0.5-kHz cubic distortion product otoacoustic emission and to establish the response patterns of cubic distortion product otoacoustic emission across speech frequencies from 0.5 to 8kHz in the presence of normal hearing and noise-induced hearing loss. RESULTS: After a series of optimization, a clinically applicable technology of measuring 0.5-kHz cubic distortion product otoacoustic emission was successfully completed via animal model. Cubic distortion product otoacoustic emission was recorded in 6 guinea pigs across speech frequencies from 0.5 to 8kHz before and after exposure to white bandnoise between 0.5 and 2 kHz. After noise exposure, significant reduction in the signal-to-noise ratio of cubic distortion product otoacoustic emission was found at 0.5 and 2 kHz, indicating our recording technology was sensitive and accurate. Other interesting finding was the reduction in cubic distortion product otoacoustic emiss ion-s ignal -to-n oise ratio at 4 and 6 kHz although the reduction was not statistically significant probably because of short exposure time. The result implied that the damaging effect induced by low-frequency noise exposure might spread upward to high-frequency region. CONCLUSIONS: Our recording technology was stable and reliable and had the great potentiality to be used in clinical setting.

Extensive hearing loss induced by low-frequency noise exposure.

Background: With little attention given to low-frequency traffic noise and our understanding that cochlear function may be highly susceptible to low-frequency noise, there is an urgent need to determine traffic noise-induced hearing loss (NIHL), not only the hearing loss at low frequency but also the possible high-frequency hearing loss. Methods: The current study aims to investigate the potential for extensive hearing loss induced by exposure to 0.063 kHz octave band noise (OBN), which is an important component of low-frequency traffic noise. The threshold of auditory brainstem response (ABR) was used to evaluate hearing function before and after noise exposure. Chinchillas were randomly assigned into seven different groups. Group 63-3 h/6 h, Group 2 k-3 h/6 h, and group 4 k-3 h/6 h were exposed for either 3 or 6 h to 0.063, 2, and 4 kHz OBN at 90 dB SPL, respectively. The control group was not exposed to noise. Results: Significant ABR threshold-shifts (TS) were observed at 0.88, 2, 4, and 5.7 kHz in Group 63-6 h, and at 2.8 and 4 kHz in Group 2 k-6 h, and at 5.7 kHz in Group 4 k-6 h. ABR-TS were consistent with outer hair cell (OHC) losses, exposure to 0.063 kHz OBN at 90 dB SPL for 6 h induced large-scale losses of OHC both in low- and high-frequency region. Conclusions: Exposure to 0.063 kHz low-frequency OBN at 90 dB SPL for 6 h leads to significant hearing loss over an extensive range from low to high frequencies.

Comorbidity burden and nonclinical factors associated with sinonasal cancer all-cause mortality.

OBJECTIVE: To describe comorbidity burden and nonclinical factors associated with all-cause mortality of sinonasal cancer in the United States. METHODS: The National Cancer Database (2004-2013) was queried for adult cases of sinonasal cancer (n = 10,518). Outcome of interest was all-cause mortality. Independent variables included comorbidity score and nonclinical factors such as age, gender, race, facility type, distance to facility, insurance, and income. Survival analysis was conducted via multivariable extended Cox regression with Heaviside adjustments. RESULTS: Patients were mostly (79%), male (61%), and mean age of diagnosis was 63.5 years. Approximately one in five patients (18.7%) had a major comorbid condition (Charlson-Deyo score ≥ 1) at diagnosis. After adjusting for clinical factors, increasing comorbidity score was associated with a corresponding increase in hazard of mortality (aHR comorbidity score of 1 = 1.25; 95% CI, 1.16, 1.35), (aHR score of 2+ = 1.61; 95%, CI 1.41, 1.83). Hazard of mortality was also associated with being male (aHR = 1.11; 95% CI, 1.04, 1.17); black (aHR = 1.13, 95% CI, 1.03, 1.24); uninsured (aHR = 1.45; 95% CI, 1.25, 1.68) or on Medicaid (aHR = 1.50; 95% CI, 1.33, 1.69); residence in zip codes with lower median income quartile (aHR < $30,000 = 1.17; 95% CI, 1.06, 1.29); and treatment at community cancer programs (aHR = 1.14, 95% CI 1.01, 1.28). CONCLUSION: Comorbid disease is associated with all-cause sinonasal cancer mortality, and after accounting for known clinical factors, significant differences in mortality persist based on disparity-driven, nonclinical factors. LEVEL OF EVIDENCE: NA Laryngoscope, 130:1443-1449, 2020.

Evaluating Simulator-Based Teaching Methods for Endoscopic Sinus Surgery.

A multitude of simulator systems for endoscopic sinus surgery (ESS) are available as training tools for residents preparing to enter the operating room. These include human cadavers, virtual reality, realistic anatomic models, and low-fidelity gelatin molds. While these models have been validated and evaluated as independent tools for surgical trainees, no study has performed direct comparison of their outcomes. To address this deficiency, we aimed to evaluate the utility of high-fidelity and low-fidelity trainers as compared to a traditional control (no simulator exposure) for novice trainees acquiring basic ESS skills. Thirty-four first-year medical students were randomized to 3 groups and taught basic sinus anatomy and instrumentation. Two groups received training with either the high-fidelity or low-fidelity trainer, while 1 group served as control. These groups were then tested with cadaveric specimens. These sessions were recorded and graded by an expert. There was no statistical difference in performance between the 3 study groups with regard to identification of anatomy, endoscopic competency, or completion of basic tasks. When the high-fidelity and low-fidelity arms were grouped into a single "trained" cohort, they demonstrated significantly improved time to completion for basic anatomy (P = .043) and total time (P = .041). This is the first study to perform a direct comparison of performance between high-fidelity and low-fidelity ESS simulators and controls. Although we found no difference in performance of novice trainees with regard to basic anatomical identification or procedural tasks associated with ESS, the use of ESS simulators may improve time to completion.

Survival differences in nasopharyngeal carcinoma among racial and ethnic minority groups in the United States: A retrospective cohort study.

OBJECTIVE: The literature on nasopharyngeal carcinoma survival in the United States has focused mostly on Whites or Asians and not much is known about survivorship in other minority racial and ethnic groups. We aimed to determine the disease-specific survival rate and prognostic factors for nasopharyngeal carcinoma survival across the minority United States population. DESIGN: A retrospective cohort study. SETTING: The Surveillance, Epidemiology and End Results (SEER) 13 database from 1992 to 2014 was queried for adult cases of nasopharyngeal carcinoma (n = 2549). PARTICIPANTS: Eligible cases were Blacks, Hispanics, Asians/Pacific Islanders, American Indians/Alaska Natives; White patients were excluded. MAIN OUTCOMES MEASURE: A multivariable competing risk survival analysis yielded hazard ratios (HR) for competing mortality and was used to identify independent prognostic factors for survival. RESULTS: Non-Hispanic American Indians/Alaska Natives consistently had the worst cause-specific survival of any group and that non-Hispanic Asians/Pacific Islanders consistently had the best survival (P < 0.001). Even after adjusting for other poor prognostic factors in the study, including older age, keratinising histology, and lack of radiation treatment, non-Hispanic American Indians/Alaska Natives had more than double hazards of death from nasopharyngeal cancer compared with non-Hispanic Asians/Pacific Islanders (aHR = 2.63, 95% CI 1.67, 4.13). CONCLUSIONS: There are disparities in nasopharyngeal carcinoma survival among racial and ethnic minority groups in the United States, with American Indians/Alaskan Natives faring worst. It is critical that future research focuses on nasopharyngeal carcinoma among this population to improve survivorship and mitigate cancer-related health disparities.

40-year incidence trends for oropharyngeal squamous cell carcinoma in the United States.

OBJECTIVES: To determine differences in oropharyngeal squamous cell carcinoma (OPSCC) incidence between 1975 and 2014 stratified by race, sex, and age. MATERIALS AND METHODS: We obtained age-adjusted OPSCC incidence rates for race and sex groups from 1975 to 2014 using the Surveillance, Epidemiology, and End Results 9 database. We defined OPSCC as cancers of the base of tongue, lingual/palatine tonsil, oropharynx, soft palate, uvula, and Waldeyer's ring. We used Joinpoint analyses to determine incidence trends for race/sex/age groupings. RESULTS: There were 38,624 oropharyngeal primary tumors in the analyses. Males accounted for 74% of sample population, and whites accounted for 84% of tumors. Overall, there was a 57.3% increase in incidence of oropharyngeal between 1975 and 2014. For blacks and whites, average incidence was lower for females than males. Rates for black males aged ≥50years was highest for most of the follow-up time but decreased sharply around 1988 and were surpassed by the significant increase in incidence in white males aged 50-59 (1995-2014 APC=4.07, p<0.001) and ≥60years (2002-2014 APC=4.25, p<0.001). For males aged ≥60, whites had higher rates than blacks starting in 2010. OPSCC incidence in White males (10.99 per 100,000 person-years) surpassed rates in Blacks (10.14 per 100,000 person-years) beginning in 2008. CONCLUSION: OPSCC has significantly increased in the United States in the last 40 years. This overall increase in OPSCC can primarily be attributed to white males. OPSCC prevention and early detection efforts could target these demographic factors to decrease rising OPSCC incidence.

Acute Vision Loss Following Endoscopic Sinus Surgery.

A 41-year-old female with a history of uterine cancer and Celiac and Raynaud's Disease presented to our institution with frequent migraines and nasal congestion. She underwent functional endoscopic sinus surgery (FESS) and experienced acute unilateral vision loss postoperatively. Rapid recognition of the etiology and effective treatment are paramount given the permanent and irreversible vision loss that can result. Arterial vasospasm following FESS is rare. Patients with autoimmune diseases have perhaps an increased risk for vasospasm secondary to an increased vasoreactive profile. We present the first documented case of nitroglycerin sublingual therapy to successfully treat ophthalmic artery vasospasm following FESS. Nitroglycerin sublingual therapy is a promising treatment for ophthalmic vasospasm secondary to its ability to cross the blood-ocular barrier, its rapid onset of action, and its ability to promote relaxation of vascular smooth muscle.

Penetrating Ocular Trauma from a Bean Bag Gun: A Case Report and Review of Less-Lethal Force and Their Consequences.

Although bean bag guns are considered a "less-lethal" form of law enforcement, these blunt projectiles have risk. The purpose of this study was to perform a literature review of morbidity and mortality associated with less-lethal munitions and present a case report of a bean bag injury leading to a traumatic globe evisceration and skull base fracture. Patients presenting with bean bag gun associated injuries warrant a high clinical suspicion for injury to deeper structures.

An update on endoscopic orbital decompression.

PURPOSE OF REVIEW: Endoscopic orbital decompression is an ever-evolving surgical procedure with modifications as well as new indications for the procedure. The purpose of this review is to update the reader on optimizing patient selection, surgical timing, highlight the latest modifications to surgical technique and to evaluate surgical outcomes that can be achieved. RECENT FINDINGS: Patient selection, disease pathology, and optimization of technology can lead to improved outcomes. Changes in technology continue to modify surgical techniques and surgical training, working towards decreased surgical complications with improved outcomes. Historically multiple approaches have been used for orbital decompression and this highlights that a balanced orbital decompression usually leads to the best outcome. SUMMARY: Orbital decompression has evolved significantly since its inception over 100 years ago. Identifying the right patient, the ideal timing, and the indication for the procedure with utilization of technology can lead to improved outcomes and decreased complications.

Proposed clinical internal carotid artery classification system.

INTRODUCTION: Numerical classification systems for the internal carotid artery (ICA) are available, but modifications have added confusion to the numerical systems. Furthermore, previous classifications may not be applicable uniformly to microsurgical and endoscopic procedures. The purpose of this study was to develop a clinically useful classification system. MATERIALS AND METHODS: We performed cadaver dissections of the ICA in 5 heads (10 sides) and evaluated 648 internal carotid arteries with computed tomography angiography. We identified specific anatomic landmarks to define the beginning and end of each ICA segment. RESULTS: The ICA was classified into eight segments based on the cadaver and imaging findings: (1) Cervical segment; (2) cochlear segment (ascending segment of the ICA in the temporal bone) (relation of the start of this segment to the base of the styloid process: Above, 425 sides [80%]; below, 2 sides [0.4%]; at same level, 107 sides [20%]; P < 0.0001) (relation of cochlea to ICA: Posterior, 501 sides [85%]; posteromedial, 84 sides [14%]; P < 0.0001); (3) petrous segment (horizontal segment of ICA in the temporal bone) starting at the crossing of the eustachian tube superolateral to the ICA turn in all 10 samples; (4) Gasserian-Clival segment (ascending segment of ICA in the cavernous sinus) starting at the petrolingual ligament (PLL) (relation to vidian canal on imaging: At same level, 360 sides [63%]; below, 154 sides [27%]; above, 53 sides [9%]; P < 0.0001); in this segment, the ICA projected medially toward the clivus in 275 sides (52%) or parallel to the clivus with no deviation in 256 sides (48%; P < 0.0001); (5) sellar segment (medial loop of ICA in the cavernous sinus) starting at the takeoff of the meningeal hypophyseal trunk (ICA was medial into the sella in 271 cases [46%], lateral without touching the sella in 127 cases [23%], and abutting the sella in 182 cases [31%]; P < 0.0001); (6) sphenoid segment (lateral loop of ICA within the cavernous sinus) starting at the crossing of the fourth cranial nerve on the lateral aspect of the cavernous ICA and located directly lateral to the sphenoid sinus; (7) ring segment (ICA between the 2 dural rings) starting at the crossing of the third cranial nerve on the lateral aspect of the ICA; (8) cisternal segment starting at the distal dural ring. CONCLUSIONS: The classification may be applied uniformly to all skull base surgical approaches including lateral microsurgical and ventral endoscopic approaches, obviating the need for 2 separate classification systems. The classification allows extrapolation of relevant clinical information because each named segment may indicate potential surgical risk to specific structures.

Cerebral Vasospasm Following Transsphenoidal Hypophysectomy in the Treatment of Lymphocytic Hypophysitis.

Cerebral vasospasm following transsphenoidal hypophysectomy is an exceedingly rare but serious complication with high morbidity and mortality. The initial signs and symptoms of cerebral vasospasm following transsphenoidal hypophysectomy are changes in mental status, motor deficits, and speech difficulties. Symptomatic vasospasm is difficult to reverse despite treatment and often only resolves with time. Furthermore, most transsphenoidal hypophysectomy patients have been discharged before the onset of vasospasm, so when they do present it is often in a fulminant form and recalcitrant to available treatments.All previously reported patients of cerebral vasospasm following transsphenoidal hypophysectomy were status postresection of a pituitary macroadenoma. The authors report the first patient of vasospasm following transsphenoidal hypophysectomy in the treatment of lymphocytic hypophysitis; a rare inflammatory disorder characterized by lymphocytic infiltration of the pituitary gland causing destruction glandular tissue.

Avascular necrosis after oral corticosteroids in otolaryngology: Case report and review of the literature.

OBJECTIVE: In this report, we present a rare case of avascular necrosis (AVN) in an otherwise healthy 42-year-old male patient treated with low dose oral corticosteroids for his bronchitis. A systematic review of the literature related to AVN and corticosteroids was performed. CASE REPORT: Forty-two-year-old male with no underlying conditions predisposing him to AVN who had been treated four years before for chronic bronchitis with two courses of oral prednisone therapy presented with bilateral AVN of the hips. METHODS: An OVID database search of the terms "low total dose," "corticosteroids," and "avascular necrosis" was performed. Two PubMed searches of various permutations of "low-dose," "corticosteroids," "avascular necrosis," and "osteonecrosis" were also performed. Results were then narrowed to relevant articles. RESULTS: Median total dose of oral corticosteroids in patients with AVN in reviewed articles was 981 mg, with lowest reported association at 105 mg. Median duration of therapy was 16 days with shortest course of six days. CONCLUSION: There is emerging data linking AVN with corticosteroid doses previously thought to be safe. After reviewing the relevant literature, it is our consensus to inform all patients regarding AVN before oral corticosteroid use.

Product comparison model in otolaryngology: Equivalency analysis of absorbable hemostatic agents after endoscopic sinus surgery.

OBJECTIVES/HYPOTHESIS: Evidence-based medicine in otolaryngology literature continues to be lacking, especially with regard to new products brought to market. The marketing of products often includes statements of benefit that have limited objective support in research or literature. To address this, and to adequately determine product equivalency/superiority, careful evaluation must be made. In order to establish standards for this process in rhinology products, we directly compare three different absorbable hemostatic agents in patients with chronic rhinosinusitis (CRS) after undergoing endoscopic sinus surgery (ESS), using both objective and subjective outcomes. STUDY DESIGN: Double-blinded prospective (level 1) comparison and equivalency analysis of three plant-based absorbable hemostatic agents (carboxymethylcellulose [CMC] gel, mucopolysaccharide hemospheres (MPH), and potato starch wafer) in patients undergoing bilateral ESS. METHODS: Patients with medically refractory CRS who underwent bilateral ESS were recruited and prospectively followed. At the conclusion of ESS, one of three different hemostatic agents was applied to each nasal passage. Subjective patient data was obtained using rated symptoms compared between the two sides (nasal obstruction, bleeding, pain, and nasal discharge) at baseline and on postoperative days 1, 7, and 14. Objective data was obtained by blinded endoscopic scoring to rate mucosal edema, inflammation, granulation, crusting, infection, and synechiae formation on postoperative weeks 1, 3, and 6. RESULTS: Forty-eight patients who underwent ESS for CRS were included. There is no statistical difference in subjective scores for any of the variables measured, although (MPH) nearly reached statistical significance at postoperative day 7 for increased pain (P = 0.06) and obstruction (P = 0.22). Objective measures showed equivalency between all products, except the CMC gel approached significance at week 3 for increased crusting (P = 0.10), granulation (P = 0.24), and debridement (P = 0.07). At 6 weeks, increased debridement (P = 0.14) also approached significance. CONCLUSION: Careful and deliberate consideration should be taken when choosing products to assist our surgical endeavors. Subjectively, patients treated with MPH showed near-significant increases in pain and obstruction. In objective measures, CMC gel nearly reaches significance for more postoperative debridement, with increased crusting and inflammation. Product choice could consider these factors, although it remains at the discretion of the surgeon. This model of comparison allows careful product comparison and should be applied to other hemostatics, as well as other materials in use in otolaryngology. LEVEL OF EVIDENCE: 1b. Laryngoscope, 126:S5-S13, 2016.

Endoscopic surgery of the orbital apex: Outcomes and emerging techniques.

OBJECTIVES/HYPOTHESIS: To review our experience with endoscopic orbital apex surgery. STUDY DESIGN: Retrospective review. METHODS: All cases with Current Procedural Terminology codes for endoscopic orbital decompression between 2002 and 2011 at two institutions were reviewed. Patients with a diagnosis of Graves orbitopathy or an orbital complication of sinusitis were excluded. Presenting symptoms, lesion location, pathology, surgical outcomes, and complications were examined. RESULTS: A total of 27 patients were identified. Seventeen (63%) of the patients were men, and the average age was 56 (range = 14-82) years. Eighteen patients had primary orbital apex lesions, and nine patients had sinonasal lesions that predominantly involved the medial orbital apex. The lesions were found to be on the right in 59% of cases. The etiologies include benign (40.7%), malignant (44%), infectious (7.4%), and metastatic (7.4%) lesions. Obtaining a pathologic diagnosis was successful endoscopically in all but two (7.4%) patients, both with lateral lesions. The two-surgeon, four-handed technique and intraoperative image guidance employing fused computed tomography/magnetic resonance imaging were used in the majority of intraconal cases. Complications occurred in three patients (11%) and included myocardial infarction, deep venous thrombosis, and vision loss. There were no cerebrospinal fluid leaks or postoperative hemorrhages. Notably, vision remained stable or improved in all but one patient (3.7% risk of vision decline). Average follow-up was 4 years (range = 1 month-8 years). CONCLUSIONS: The endoscopic approach to the orbit apex offers significant advantages over traditional external approaches, and should be the preferred approach for all medial and inferior lesions. A two-surgeon multihanded technique can help facilitate difficult cases.

Bilateral meningoencephaloceles with cerebrospinal fluid rhinorrhea after facial advancement in the Crouzon syndrome.

BACKGROUND: Cerebrospinal fluid (CSF) rhinorrhea and encephaloceles are rare complications of craniofacial advancement procedures performed in patients with craniofacial dysostoses (CD) to address the ramifications of their midface hypoplasia including obstructed nasal airway, exorbitism, and impaired mastication. Surgical repair of this CSF rhinorrhea is complicated by occult elevations in intracranial pressure (ICP), potentially necessitating open, transcranial repair. We report the first case in otolaryngology literature of a patient with Crouzon syndrome with late CSF rhinorrhea and encephalocele formation after previous LeFort III facial advancement surgery. OBJECTIVES: Describe the case of a patient with Crouzon syndrome who presented with CSF rhinorrhea and encephaloceles as complications of Le Fort III facial advancement surgery. Review the literature pertaining to the incidence and management of post-operative CSF rhinorrhea and encephaloceles. Analyze issues related to repair of these complications, including occult elevations in ICP, the utility of perioperative CSF shunts, and the importance of considering alternative repair schemes to the traditional endonasal, endoscopic approach. METHODS: Review of the literature describing CSF rhinorrhea and encephalocele formation following facial advancement in CD, focusing on management strategies. RESULTS: CSF rhinorrhea and encephalocele formation are rare complications of craniofacial advancement procedures. Occult elevations in ICP complicate the prospect of permanent surgical repair, potentially necessitating transcranial repair and the use of CSF shunts. Though no consensus exists regarding the utility of perioperative CSF drains, strong associations exist between elevated ICP and failed surgical repair. Additionally, the anatomic changes in the frontal and ethmoid sinuses after facial advancement present a challenge to endoscopic repair. CONCLUSION: Otolaryngologists should be aware of the possibility of occult elevations in ICP and sinonasal anatomic abnormalities when repairing CSF rhinorrhea in patients with CD. Clinicians should consider CSF shunt placement and carefully weigh the advantages of the transcranial approach versus endonasal, endoscopic techniques.