The morphometrical evaluation after uvulopalatopharyngoplasty.

OBJECTIVES: The aim of this study was to evaluate the efficacy of uvulopalatopharyngoplasty (UPPP) and the corresponding postoperative morphometrical changes. METHODS: Patients diagnosed with obstructive sleep apnea syndrome who complained of snoring and apnea were enrolled in this study. Pre- and postoperative tests, including CTs, pharyngeal volume (PV), cross-sectional area (CSA), and six-category morphometrical studies, were performed. RESULTS: Of 11 patients included, 10 showed improvement of symptoms. BMI correlated with the respiratory event index (REI). In terms of PV, there was a significantly wider postoperative area. The rate of change between preoperative REI and postoperative REI (ΔREI) correlated with the amount of change of PV between preoperative PV and postoperative PV (ΔPV). CSA increased postoperatively and correlated with REI. Uvula space (UV) and distance between the hyoid bone and the base of the tongue (HB) increased postoperatively, and posterior airway space (PAS) and epiglottic space (Epi) decreased postoperatively. UV and PAS were significant (p = 0.046, 0.014). UV was related to REI. CONCLUSION: Widening the PV, increasing CSA, and the posterior movement of the tongue base after UPPP surgery were found. The improvement of REI did not depend only on volume. These results suggest that it was important not only to widen the PV but also to improve the shape of the pharynx.

Combination therapy with von Willebrand factor concentrate plus recombinant factor VIII during cesarean section in a patient with type 3 von Willebrand disease and a low inhibitor titer: a case report.

Type 3 von Willebrand disease (VWD), a rare and severe subtype, can produce inhibitors in roughly 5% to 10% of cases. We present a case of type 3 VWD with inhibitors in late pregnancy, which was successfully managed with a combination of neutralization and factor (F)VIII replacement during cesarean delivery. The patient, a 30-year-old woman, had no history of inhibitors despite over 100 exposures to VWF/FVIII. She developed inhibitors after 28 weeks of weekly pd VWF/FVIII prophylaxis for recurrent urolithiasis-associated hematuria during pregnancy. Genetic analysis detected two novel frameshift mutations: VWF Exon7 c.777_784dup and Exon14 c.1625_1646del. Titers of inhibitors to factors VIII and VWF using the Bethesda assay were 1.2 and 1.1 BU/mL, respectively. Pharmacokinetics revealed significantly low in vivo recovery of FVIII:C and VWF:Rcof and shortened half-life. During cesarean delivery, a combination of bolus pd VWF/FVIII once daily for neutralizing inhibitors plus continuous infusion of recombinant FVIII Fc fusion protein resulted in minimal bleeding without allergic reactions. Both VWF:Rcof and FVIII:C levels increased transiently during the 7-h of combination therapy without thrombotic events. In conclusion, combination therapy with neutralization and continuous FVIII replacement was effective for hemostasis with a low VWD inhibitor titer, though further optimization is required.

New Modified Transoral Approach to Remove a Hilar Stone of the Submandibular Gland: A Case Report.

Nerve damage and intraoperative bleeding for the removal of the hilar stones are possible. We used the new modified lateral oral floor approach with a 2-3 cm longitudinal mucosal incision outside of the Wharton's. There were no complications and our technique seemed to be effective.

Postoperative functional evaluation of obstructive sleep apnea syndrome by computational fluid dynamics.

The aim of this study is to evaluate the efficacy of uvulopalatopharyngoplasty and the corresponding postoperative airflow. Eleven patients diagnosed with obstructive sleep apnea syndrome who complained of snoring and apnea were enrolled in this study. Computational fluid dynamics (CFD) was implemented. CFD could be accomplished in nine cases. Airflow analysis was not possible in cases with a high respiratory event index (REI) score. Before surgery, stenosis was identified in the oropharynx and epiglottic area. And the airflow velocity and pressure were found to have significantly decreased in the oropharynx postoperatively, while in the epiglottic area, those data had increased postoperatively in some cases. The velocity and pressure of the oropharynx are related to REI score. From the CFD analysis, airflow analysis is important for evaluating the apnea state. It is suggested that the postoperative function can now be predicted preoperatively.

Congenital External Auditory Canal Cholesteatoma Resected by a Transcanal Endoscopic Approach.

OBJECTIVE: To describe the first case of congenital external auditory canal cholesteatoma resected via a transcanal endoscopic approach and describe the possible pathologenesis of cholesteatoma. PATIENT: A 2-year-old female patient presented with a cholesteatoma that extended from the floor of the external auditory bony canal to the inferior quadrant of her right tympanic membrane. INTERVENTION: Otological examinations and computed tomography were performed. The cholesteatoma was resected using a transcanal endoscopic approach. RESULTS: Pathologic examination confirmed the diagnosis of external auditory canal cholesteatoma. The patient's postoperative hearing was observed to be normal. Cholesteatoma did not recur during the 2-year follow-up period. Computed tomography scan revealed an isodense mass, partly surrounded by bony tissue, on the floor of the external auditory canal, outside of the inferior quadrant of an intact tympanic membrane. A minor malformation of the bony tissue, covering the inferiolateral surface of the cholesteatoma, was observed. The proposed mechanisms of pathogenesis are: 1) the embryonic developmental deficits of the meatal plug, during its differentiation into squamous epithelium, cause the arrest of ectodermal tissues, 2) a remnant of the squamous epithelium becomes trapped in the niche of the foramen tympanicum. CONCLUSION: A rare case of congenital external auditory canal cholesteatoma, located on the inferior external auditory canal, was diagnosed and resected using a transcanal endoscopic approach. It was possibly caused by a minor anomaly of the first branchial cleft or by a remnant of the squamous epithelium trapped in the foramen tympanicum.

Successful treatment of non-HIV progressive multifocal leukoencephalopathy: case report and literature review.

BACKGROUND: Progressive multifocal leukoencephalopathy (PML) is a subacute onset demyelinating disease caused by JC virus and characterized by multifocal involvement of the subcortical white matter and cerebellar hemispheres or peduncles on magnetic resonance imaging (MRI). However, non-HIV PML patients with brain lesions limited to the cerebellum and brainstem have not been well characterized. METHODS: We report a 68-year-old man with systemic lupus erythematosus under treatment with immunosuppressants who developed non-HIV PML with brain lesions limited to the cerebellum and brainstem and successfully treated with a combination of mefloquine and mirtazapine. We performed a literature review to characterize patients with non-HIV PML with brain lesions limited to the cerebellum and brainstem. RESULTS: Eight cases with non-HIV brainstem/cerebellar form PML were identified including our case. All cases had compromised status related underlying diseases. Four (50%) had a good prognosis. Five cases were treated, including 3 with favourable outcomes. Between the good prognosis group (n = 4) and the poor prognosis group (n = 4), treatment status for PML and the interval between the initial manifestation and diagnosis did not differ. Among those who performed contrast-enhanced brain imaging, lesion enhancement was related to good prognosis (good prognosis group vs. poor prognosis group; 100% vs. 0%). CONCLUSION: PML should be considered in the differential diagnosis of brain lesions limited to the cerebellum and brainstem in immunocompromised patients. The presence of immune response against JC virus and inflammatory reactions may indicate good prognosis in non-HIV brainstem/cerebellar form PML.

Sinonasal Inverted Papilloma: Efficacy of Demucosation to Reduce Recurrence After Surgical Managements.

To compare the outcomes of various surgical approaches to resect sinonasal inverted papilloma and to discuss their advantages and disadvantages. A retrospective chart review of 61 consecutive patients with sinonasal inverted papilloma was performed. Surgical treatment included non-demucosation endoscopic sinus surgery (ESS), demucosation ESS, endonasal medial maxillectomy (EMM), Draf type 3, Caldwell-Luc surgery, Denker, Killian, and lateral rhinotomy. Recurrence rates were compared between endonasal and external approaches and between demucosation and non-demucosation. After the first curative surgery, the non-demucosation ESS, endonasal demucosation (demucosation ESS, EMM, and Draf type 3), and external surgery showed recurrence rates of 61.5%, (8/13), 0.0% (0/21), and 7.4% (2/27), respectively. A significantly lower recurrence rate was observed in the endonasal demucosation (p < 0.001) and in the demucosation ESS group (p < 0.001) in comparison with the non-demucosation ESS. However, as for recurrence rate, no statistically significant difference was observed between endonasal surgery and external surgery (p = 0.162). Demucosation is a better strategy for the treatment of inverted papilloma than is non-demucosation. Demucosation is the key procedure for preventing recurrence.

A case of neck xanthogranulomatous inflammation-suspected malignant tumor.

Xanthogranulomatous inflammation (XGI) is an uncommon chronic inflammatory disease. A 59-year-old male presented with a neck mass which had been diagnosed as an undifferentiated carcinoma. From CD68 staining, XGI was confirmed. It is important to consider the possibility of XGI for a neck mass mimicking a malignant tumor.

A Small Glomus Tympanicum Tumor Resected by Minimally Invasive Transcanal Endoscopic Approach.

We present a case of the transcanal endoscopic resection of a glomus tympanicum tumor. A 51-year-old woman presented with pulsatile tinnitus of the right ear persisting for 6 months. A reddish mass was observed through her tympanic membrane. A computed tomography scan revealed a small mass in the mesotympanum. She was diagnosed with a right-sided glomus tympanicum tumor. The glomus tympanicum tumor was classified as type 1 using the Glasscock-Jackson classification, class A using the Fisch classification, and class A1 using the modified Fisch and Mattox classification. The tumor was transcanally and completely resected by endoscopy without any complication. Before and after the surgery, pure-tone audiometry showed a normal hearing level. Preoperative right-sided pulsatile tinnitus resolved after the surgery. Transcanal endoscopic ear surgery is a favorable surgical method for small localized glomus tympanicum tumors.

Apocrine Adenoma of the External Auditory Canal with Pseudoepitheliomatous Hyperplasia.

The tumors derived of the ceruminous gland in the external auditory canal are rare. Here, we report a case of a ceruminous adenoma (apocrine adenoma) with refractory chronic inflammation in the external auditory canal. A 46-year-old man presented with otorrhea, itching, and a foreign body sensation in his right ear. A soft reddish protruding lesion was revealed at the posterosuperior portion of the entry to the right external auditory canal by otoscopy. The skin lesion was endaurally resected; histopathology showed luminal structures in the middle to deep layer of the epidermis and inflammatory granulation below pseudoepitheliomatous hyperplasia. The walls of the luminal structures consisted of inner luminal secretory cells featuring apical decapitation secretion and outer myoepithelial cells. The patient was diagnosed with an apocrine adenoma. Three years after surgery, there has been no evidence of recurrence. Complete resection, including the deep layer of the epidermis, is necessary.

Endoscopic Type 1 Tympanoplasty in Chronic Otitis Media: Comparative Study with a Postauricular Microscopic Approach.

OBJECTIVE: To compare surgical outcomes after tympanoplasty without ossiculoplasty for chronic otitis media between transcanal endoscopic ear surgery (TEES) and postauricular microscopic ear surgery (PAMES). STUDY DESIGN: Case-control study. SETTING: Tertiary care university hospital. SUBJECTS AND METHODS: Consecutive patients (N = 122) who had undergone tympanoplasty without ossiculoplasty for chronic otitis media were enrolled in this retrospective study and divided into 2 groups: TEES (n = 47) and PAMES (n = 75). Middle ear condition was graded with the middle ear risk index. Hearing, repair of tympanic membrane perforation, and surgical time were assessed. RESULTS: The surgical success rate for hearing (air-bone gap ≤20 dB) was 95.7% in the TEES group and 84.0% in the PAMES group. Lower middle ear risk resulted in similar mean (95% CI) closure of air-bone gaps (TEES: 9.6, 6.5-12.6; PAMES: 8.0, 6.4-9.7; P = .333), whereas higher middle ear risk demonstrated significantly larger closure of air-bone gaps for the TEES group (10.1, 3.3-16.9) than the PAMES group (-0.2, -4.5 to 4.2; P = .009). The surgical success rate for repair of tympanic membrane perforation and surgical time were equivalent between TEES and PAMES. CONCLUSION: Under favorable conditions of the middle ear, TEES and PAMES resulted in similar hearing improvement by tympanoplasty without ossiculoplasty. However, under adverse conditions of the middle ear, TEES was a more beneficial approach for hearing improvement than PAMES.

Effects of nasal septum perforation repair on nasal airflow: An analysis using computational fluid dynamics on preoperative and postoperative three-dimensional models.

OBJECTIVE: The purpose of this research is to examine the changes in nasal airflow dynamics before and after the nasal perforation repair. METHODS: Three dimensional (3D) models of the nasal cavity before and after septal perforation repair was reconstructed using preoperative and postoperative computed tomography (CT) images of a patient. The numerical simulation was carried out using ANSYS CFX V15.0. Pre- and post-operative models were compared by their velocity, pressure (P), pressure gradient (PG), wall shear (WS), shear strain rate (SSR) and turbulence kinetic energy (TKE) in three plains. RESULTS: In the post-operative state, the cross flows disappeared. In preoperative state, there were areas showing high PG, WS, SSR at the posterior border of the perforation, which exactly correspond to the area showing erosive mucosa on endoscopic inspection of the patient. In postoperative state, such high PG, WS and SSR areas disappeared. High TKEs also disappeared after surgery. CONCLUSION: The effects of septal perforation repair on airflow dynamics were evaluated using computer fluid dynamics (CFD). High WS, PG and SSR observed at the edge of the septal perforation may be related to the clinical symptom such as nasal bleeding and pain. TKE was considered to cause nasal symptom.

Nasal, oral, and pharyngolaryngeal manifestations of pemphigus vulgaris: Endoscopic ororhinolaryngologic examination.

Pemphigus vulgaris is an autoimmune blistering disorder that involves the skin and mucous membranes. Few reports have described nasal and oropharyngolaryngeal lesions in pemphigus vulgaris using an endoscopic ororhinolaryngologic examination. We retrospectively reviewed the clinical records of 11 patients with pemphigus vulgaris between 2001 and 2013 with respect to their symptoms, lesion sites, lesion features, and treatments received. All patients had undergone an endoscopic ororhinolaryngologic examination. Their mucosa-related symptoms were sore throat, oral pain, odynophagia, gingival bleeding, hoarseness, and epistaxis. The most frequent sites were the oral cavity (gingiva and buccal mucosa), larynx (epiglottis and vocal fold), oropharynx (soft palate), and nasal cavity (nasal septum). Lesions were typically characterized by erosion, erosion with a whitish exudate, and erythematous patches. Thus, our study findings reveal that pemphigus vulgaris involves both the nasal and oropharyngolaryngeal regions. Patients with pemphigus vulgaris should undergo an endoscopic ororhinolaryngologic examination to determine the range of their lesions.

Features of oral, pharyngeal, and laryngeal lesions in bullous pemphigoid.

Bullous pemphigoid is an autoimmune bullous disease characterized by skin lesions, with or without oral lesions. The occurrence of pharyngolaryngeal lesions is very rare in affected patients. We conducted a study to investigate the characteristics of oral and pharyngolaryngeal lesions in bullous pemphigoid. Our study population was made up of 6 consecutively presenting outpatients-2 men and 4 women, aged 40 to 83 years (mean: 68.2)-who had been referred to our department over an 11-year period. Presenting symptoms included sore throat in all 6 patients and oral pain in 3. The sites of mucosal lesions included the soft palate, epiglottis, gingiva, hypopharynx, tongue, nasal cavity, and buccal mucosa. These lesions appeared as erosions, erosions with white coating, erythematous patches, and/or blisters. Mucosal lesions preceded skin lesions in 2 patients, appeared after skin lesions in 1 patient, and appeared simultaneously with skin lesions in 3 patients. We conclude that bullous pemphigoid sometimes involves the mucosa, such as that of the laryngopharynx and the oral cavity, and it can manifest as skin lesions. In the differential diagnosis of refractory pharyngolaryngeal lesions, bullous pemphigoid should be considered.

Potentially lethal pharyngolaryngeal edema with dyspnea in adult patients with mumps: A series of 5 cases.

In this article we describe 5 rare cases of mumps-associated pharyngolaryngeal edema. To the best of our knowledge, this report includes the first case of mumps-associated pharyngolaryngeal edema in a patient who had previously received mumps vaccination, and these cases represent the sixth report of mumps-associated pharyngolaryngeal edema in the English literature. All 5 of our patients with mumps infection were adults and manifested airway stenosis due to pharyngolaryngeal edema. This edema responded favorably to steroid treatment without tracheotomy. We conclude that a pharyngolaryngeal examination is recommended for patients with mumps infection. Steroid treatment is usually effective against pharyngolaryngeal edema; however, in certain cases tracheotomy may be inevitable.

[A Clinical Study of Acute Epiglottitis].

We carried out a retrospective review of 285 cases of acute epiglottitis (180 males, 105 females, mean age 49.6 years) that required inpatients hospital care between 1998 and 2014. All the patients complained of sore throat, and 62 patients complained of respiratory discomfort; 17 patients had severe dyspnea, and 27 patients required airway management (tracheotomy in 25, cricothyroidotomy in 2 patients). All the patients survived. As acute epiglottitis can cause rapidly progressive airway obstruction and death, emergent airway management should be undertaken in patients with dyspnea. However, it is difficult to determine the indications for prophylactic respiratory management in patients without dyspnea. Therefore, the disease severity of the epiglottitis was evaluated on a five-grade scale according to the degree of swelling of both the epiglottis and the arytenoids. Although prospective evaluation is necessary, this scoring system may be beneficial to determine the indication for airway management, because all of the patients who complained of severe dyspnea or underwent airway management had grade 4 or 5 disease, while none of the patients with grade 1-3 disease required tracheotomy or cricothyroidotomy. Moreover, we compared the white blood cell count, body temperature, serum CRP and the interval from the onset between the group that required airway management and the group that did not require airway management. The white blood-cell count and body temperature were significantly higher, and the interval from the onset was significantly shorter in the group that required airway management than in the group that did not require airway management; however, the serum CRP level did not differ between the two groups.

Periodic alternating nystagmus caused by a medullary lesion in acute disseminated encephalomyelitis.

OBJECTIVE: To document a patient with periodic alternating nystagmus (PAN) caused by acute disseminated encephalomyelitis (ADEM) and suggest a mechanism to explain her PAN. PATIENT: A 34-year-old woman with PAN caused by ADEM. INTERVENTION: Diagnostic. RESULTS: The patient complained of severe disequilibrium from the disease onset. Four years after onset, when she visited us, the patient exhibited prominent PAN consisting of alternating rightward and leftward components, which cycled about every 90 seconds and were accompanied by a 5-second translating phase with downbeating nystagmus. Eye movement analysis that separated the horizontal and vertical components revealed the presence of downbeating movements throughout all phases of the PAN. ENG recordings revealed slightly saccadic pursuit, slightly impaired optokinetic eye movement and an absence of visual suppression of the caloric response. MRI recorded at the onset of the disease revealed lesions in the medulla, the spinal cord at the C2 level, and the frontal horn of the left lateral ventricle, but not the cerebellum. CONCLUSION: We attribute this patient's PAN to impairment of the nucleus prepositus hypoglossi in the medulla, which plays a role in the velocity storage system. In addition, cerebellar dysfunction is indicated by the occurrence of PAN while fixating.

Thrombophlebitis of the head and neck caused by eosinophilic otitis media.

OBJECTIVE: To describe the clinical course and treatment of a patient presenting with thrombophlebitis of the head and the neck with eosinophilic otitis media (EOM). PATIENT: The patient was a 32-year-old woman with bronchial asthma and chronic sinusitis. She was treated for EOM, which presented as intractable otitis media with viscous effusion. Subsequently, she experienced sudden onset of nausea and headache. No signs of improvement were observed after the intravenous administration of several antibiotics. Magnetic resonance imaging (MRI) revealed right thrombophlebitis extending from the sigmoid sinus to the jugular vein. INTERVENTIONS: Systemic administration of adrenocortical steroid hormone after intravenous antibiotic therapy. MAIN OUTCOME MEASURES: Radiologic features and patient symptoms. RESULTS: Intravenous antibiotic therapy was ineffective, but the patient's symptoms were relieved less than 1 day after the initiation of prednisolone treatment (30 mg/d for 5 d). MRI performed 3 days after treatment revealed that thrombophlebitis of the head and neck had disappeared. CONCLUSION: Adrenocortical steroid hormone therapy may be an effective treatment for thrombophlebitis with EOM. Chemoattractants activated by EOM might be associated with the pathogenesis of thrombophlebitis of the head and neck.

Optokinetic stimulation can break Listing's law without induction of eye movement.

OBJECTIVE: Pseudo-images of three-dimensional eye movements captured on an infrared video oculogram can be translated onto a rotational expression around axial vector. This provides a subject's Listing's plane, which moves according to the head's orientation relative to gravity. Optokinetically induced changes in the cognitive gravitational reference frame will affect the context of Listing's plane. The purpose of this study was to estimate the effect of OKS on Listing's plane. METHODS: In this study, we presented vertical optokinetic visual stimulation with fixation targets, which are thought to induce pseudo-inclination of the head, and evaluated changes in the subjects' Listing's plane. RESULTS: We observed no stimulus-induced movement of Listing's plane that corresponded to the assumed pseudo-recognition of a change in verticality. On the other hand, we did observe vergence movement of Listing's plane (in the yaw plane), which corresponded to exposure to diminished and increased gravitational circumstance. In addition, the thickness of Listing's plane significantly increased with the load of each stimulation. CONCLUSION: Vertical OKS leads to a rotation of Listing's plane mainly around a vertical axis. This may represent false exhibition of central compensatory re-weighting with respect to inherent otoconial mass asymmetry resulting from the OKS-mediated loss of the gravity reference. In addition, a OKS-mediated thickening of Listing's plane suggests to us that confusing visual input can reduce the stability of the internal model, which would likely manifest itself as a thickening of Listing's plane. In other words, fluctuation between the build-up and drop-out of vection induced by optokinetic stimulation will cause a thickening of Listing's plane. The thickness of Listing's plane could be a novel clinical parameter for quantitatively evaluating static vestibular function and accuracy of the internal model.