Usefulness of endoscopic endonasal underwater surgery using a combination of coblation and a lens-cleaning system for hemostasis in hereditary hemorrhagic telangiectasia.

Hemostatic procedures for controlling nasal bleeding in refractory diseases such as hereditary hemorrhagic telangiectasia (HHT) can be challenging. In this report, we present a novel technique for underwater endoscopic endonasal hemostatic surgery, which was performed on a 69-year-old man with HHT. The patient had been experiencing frequent episodes of nasal bleeding and had many telangiectasias in the nasal cavity, which were the cause of the bleeding. These telangiectasias were effectively treated using a coblation device in combination with an endoscope lens-cleaning system that supplied saline to create stable underwater conditions. There are several advantages to this technique, including provision of a stable and clear endoscopic field of view, allowing for better visualization of the surgical site. This makes it easier to identify bleeding points and ensure accurate hemostasis. Additionally, the hydrostatic pressure created by the underwater environment helps to reduce bleeding during the procedure. However, it is important to take careful precautions to prevent water from entering the lower airway. With this precautionary measure, this technique is particularly useful in managing bleeding in patients with HHT.

Practical guide for the diagnosis and management of primary ciliary dyskinesia.

OBJECTIVE: Primary ciliary dyskinesia (PCD) is a relatively rare genetic disorder that affects approximately 1 in 20,000 people. Approximately 50 genes are currently known to cause PCD. In light of differences in causative genes and the medical system in Japan compared with other countries, a practical guide was needed for the diagnosis and management of Japanese PCD patients. METHODS: An ad hoc academic committee was organized under the Japanese Rhinologic Society to produce a practical guide, with participation by committee members from several academic societies in Japan. The practical guide including diagnostic criteria for PCD was approved by the Japanese Rhinologic Society, Japanese Society of Otolaryngology-Head and Neck Surgery, Japanese Respiratory Society, and Japanese Society of Pediatric Pulmonology. RESULTS: The diagnostic criteria for PCD consist of six clinical features, six laboratory findings, differential diagnosis, and genetic testing. The diagnosis of PCD is categorized as definite, probable, or possible PCD based on a combination of the four items above. Diagnosis of definite PCD requires exclusion of cystic fibrosis and primary immunodeficiency, at least one of the six clinical features, and a positive result for at least one of the following: (1) Class 1 defect on electron microscopy of cilia, (2) pathogenic or likely pathogenic variants in a PCD-related gene, or (3) impairment of ciliary motility that can be repaired by correcting the causative gene variants in iPS cells established from the patient's peripheral blood cells. CONCLUSION: This practical guide provides clinicians with useful information for the diagnosis and management of PCD in Japan.

Allergic Fungal Otomastoiditis: A Case Report.

Otomastoiditis caused by an allergic reaction to fungi in the middle ear is rare, with only four cases reported in the English literature. We report the case of a patient with allergic fungal otomastoiditis. A 28-year-old man presented with otalgia, hearing loss, and vertigo. Exploratory tympanotomy revealed mucin with a peanut butter-like consistency and containing eosinophils and Candida parapsilosis, but no evidence of direct tissue invasion by fungi. The patient was treated with a combination of surgery and medication. Subtotal petrosectomy was finally performed to remove the middle ear mucosa and separate the middle ear from the external environment. Short-term prednisolone and long-term fluconazole were administered without satisfactory therapeutic results. The inflammatory condition has improved but continues without complete remission. Allergic fungal otomastoiditis is an extremely rare condition that may share pathophysiological features with allergic fungal rhinosinusitis, so a thorough examination combining bacterial cultures, histopathological examination with fungal staining, and serum antigen-specific immunoglobulin E against multiple fungi is essential. Optimal treatment probably comprises appropriate surgery and long-term administration of systemic corticosteroids. Definitive diagnostic criteria and therapeutic strategies need to be established, based on the accumulation of similar cases.

Retrospective study of cochlear implantations at a single facility focusing on postoperative complications.

OBJECTIVE: Although cochlear implantation (CI) is a relatively safe operation, postoperative complications sometimes occur. We reviewed the frequency and severity of complications of CI at our hospital. We compared our results with previously reported complications and considered measures to improve patient outcomes. METHODS: This retrospective study examined the medical records of 70 patients who received CI between March 2005 and December 2018. We collected the following data: age at the time of the first surgery, etiology of hearing impairment, date of implantation, type of implanted devices, and complications. Surgical complications were divided by time into perioperative, early, and late, and by severity into major or minor. RESULTS: Records of 38 adults and 32 children were analyzed. Bilateral CI was performed in 16 patients, 8 of whom were sequential, and unilateral CI was performed in 54 patients. The total number of operations was 78 for 86 CI. Complications were observed in 15 of 78 operations (19%), and the rates of minor and major complications were 15% and 4%, respectively. Complication rates were 21% (8/39) for children and 10% (4/39) for adults. All of the perioperative and early complications were minor. There were three major complications, all of which were infections presenting with mastoiditis and subcutaneous or subperiosteal abscesses. One case required reimplantation twice because of recurrent mastoiditis and temporal abscess. CONCLUSIONS: There was no significant difference in the incidence of complications between children and adults, but all major complications were infection in pediatric cases. Careful attention is needed to prevent postoperative infection.

Successful Balloon-assisted Coil Embolization of Right Subclavian Artery Aneurysm: A Case Report.

Objective: Subclavian artery aneurysms are relatively rare, and have been treated by open surgery and/or endovascular treatment using a stent graft. In this article, we report a case of unruptured right subclavian artery aneurysm successfully treated using balloon-assisted coil embolization. Case Presentation: A 77-year-old man was diagnosed with an asymptomatic unruptured right subclavian artery aneurysm of 8 mm in diameter by follow-up CTA after surgery for thoracoabdominal aortic aneurysms. He also had a history of cerebral infarction and clipping of an unruptured cerebral aneurysm. The subclavian artery aneurysm was treated by balloon-assisted coil embolization because its diameter increased to 17.6 mm in 2 years. Balloon assistance was mainly used to prevent protrusion of the framing coil into the parent artery, and satisfactory framing was achieved. Subsequently, the aneurysm was obliterated using filling and finishing coils. The postoperative course was uneventful, and the follow-up MRI at 18 months after treatment revealed no recanalization of the aneurysm. Conclusion: Balloon-assisted coil embolization may be an effective treatment for subclavian artery aneurysms, but further long-term follow-up and case accumulation are needed.

Significance of novel subcortical low intensity score on transient neurological events after revascularization surgery for moyamoya disease.

OBJECTIVES: Transient neurological events (TNEs) are frequently observed after revascularization surgery for moyamoya disease (MMD). Recently, two signs on fluid-attenuated inversion recovery magnetic resonance images, a cortical hyperintensity belt (CHB) sign possibly reflecting vasogenic edema and a transient subcortical low intensity (SCLI) sign possibly reflecting cytotoxic edema, were reported associated with TNEs. The purpose of this study was to create a SCLI score and to investigate the significance of the score in TNEs. PATIENTS AND METHODS: The authors retrospectively analyzed 18 cerebral hemispheres in 16 consecutive patients with revascularization surgery for MMD. The SCLI sign was defined as a transient SCLI in surgically treated hemispheres, and blindly graded as the SCLI score (0-4) based on the extent. The relationships among SCLI, CHB signs and TNEs were evaluated. RESULTS: Postoperative TNEs, SCLI and CHB signs were detected in 8 (44.4%), 9 (50.0%) and 12 (66.7%) hemispheres, respectively. Patients with SCLI and CHB signs had a significantly higher TNE occurrence rate than those without these signs (p = 0.015, and p = 0.013, respectively). Patients with TNEs showed significantly higher SCLI scores than those without TNEs (p = 0.009), while the difference of CHB scores did not reach significance between patients with and without TNEs. For the occurrence of postoperative TNEs, SCLI score with a cut-off value of 1.0 resulted in a specificity of 80.0% and a sensitivity of 87.5%. CONCLUSION: The novel SCLI score may be useful for diagnosing TNEs after revascularization surgery for MMD, although both vasogenic and cytotoxic edema may be involved in postoperative TNEs.

A case of arteriovenous fistula of the cauda equina fed by the proximal radicular artery: anatomical features and treatment precautions.

PURPOSE: Arteriovenous fistula (AVF) of the cauda equina (CE) fed by the proximal radicular artery (PRA) is very rare, and the differentiation from that of the filum terminale (FT) is important to avoid treatment-related injury to the CE when endovascular treatment is selected. The authors describe a case of AVF of the CE fed by the PRA, demonstrate the anatomical features and discuss the treatment precautions. METHODS: A 69-year-old man presented with a transient weakness of lower limbs. Spinal angiography and magnetic resonance (MR) imaging revealed AVF, of which the feeding artery arose from the anterior spinal artery (ASA), forming the fistula at L2 level to be drained into the longitudinal venous trunk. Under a tentative diagnosis of AVF of the FT, endovascular treatment was attempted but failed due to impossible catheterization into the ASA. Therefore, surgery was performed. RESULTS: Intraoperative finding revealed that the feeding artery and draining vein were not on the FT but on the CE, resulting in the proper diagnosis of AVF of the CE. Surgical clips were applied to the draining vein closest to the fistula, and postoperatively the symptom improved gradually. Although we thoroughly reevaluated spinal angiography and MR images postoperatively, AVF of the CE fed by the PRA and that of the FT were not distinguishable. CONCLUSIONS: The authors described a case of AVF of the CE fed by the PRA and demonstrated the difficulty of the differentiation from that of the FT. The utmost precautions are necessary when endovascular treatment is selected.

Endoscopic electrocauterization of pyriform sinus fistula.

To determine the efficacy of endoscopic electrocauterization for pyriform sinus fistula (PSF) using a flexible Bugbee cautery electrode. From 2009 to 2016, a total of eight patients with acute suppurative thyroiditis or cervical abscess secondary to PSF were retrospectively registered in our study (three males, five females; median age 6.5 years). All patients underwent endoscopic electrocauterization as treatment for PSF. Six of eight patients had no recurrence after the initial endoscopic electrocauterization of PSF. One patient with recurrence developed symptoms 9 days after cauterization and another experienced recurrence after 2 years. Mean follow-up for the eight patients was 50 months (range 5-96 months). No post-operative complication was reported. Endoscopic electrocauterization appears to be a less-invasive, safe, and effective method for the treatment of PSF.

Atlantoaxial rotatory fixation as a rare complication from head positioning in otologic surgery: Report of two cases in young children.

BACKGROUND: Atlantoaxial rotatory fixation is a condition in which the first and second vertebrae of the cervical spine become interlocked in a rotated position. This condition can result in serious consequences and thus have a significant impact on patients, especially when diagnosis and treatment are delayed. Some cases of atlantoaxial rotatory fixation have been described in association with otologic surgery or plastic surgery involving the ear. We present the cases of two pediatric patients who developed atlantoaxial rotatory fixation following otologic surgery and we review the relevant literature. CASE PRESENTATION: One patient was a 7-year-old boy who underwent tympanoplasty for cholesteatoma. The other patient was a 5-year-old girl with profound sensorineural hearing loss who underwent cochlear implantation. Both patients developed atlantoaxial rotatory fixation on the day after surgery, and they were treated conservatively. Our literature search using relevant terms identified 12 similar published cases. Thus, a total of 14 patients, including our 2 patients, were evaluated. Most of the patients were children and typically they complained of painful torticollis and exhibited a characteristic posture called the "cock-robin" position on the day after surgery. Mostly, the direction of torticollis was opposite to the side of surgery. Most of the patients received conservative treatment alone, but three underwent surgical treatment. CONCLUSION: The correlation between the direction of torticollis and the side of surgery suggests that rotation of the head during surgery has an impact on development of postoperative atlantoaxial rotatory fixation. Thus, children undergoing otologic surgery are thought to be at a risk of postoperative atlantoaxial rotatory fixation. Although rare, the surgical team needs to be aware of this adverse event and pay close attention to this possibility throughout the perioperative period. Perioperative management should include informed consent, preoperative assessment of the range of head and neck motion, proper intraoperative positioning and monitoring of the position, and postoperative follow-up. Postoperative atlantoaxial rotatory fixation is not completely preventable, but good perioperative management can minimize the damage resulting from this condition.

Preventive effects of cilostazol against the development of shunt-dependent hydrocephalus after subarachnoid hemorrhage.

OBJECTIVE Chronic hydrocephalus develops in association with the induction of tenascin-C (TNC), a matricellular protein, after aneurysmal subarachnoid hemorrhage (SAH). The aim of this study was to examine if cilostazol, a selective inhibitor of phosphodiesterase Type III, suppresses the development of chronic hydrocephalus by inhibiting TNC induction in aneurysmal SAH patients. METHODS The authors retrospectively reviewed the factors influencing the development of chronic shunt-dependent hydrocephalus in 87 patients with Fisher Grade 3 SAH using multivariate logistic regression analyses. Cilostazol (50 or 100 mg administered 2 or 3 times per day) was administered from the day following aneurysmal obliteration according to the preference of the attending neurosurgeon. As a separate study, the effects of different dosages of cilostazol on the serum TNC levels were chronologically examined from Days 1 to 12 in 38 SAH patients with Fisher Grade 3 SAH. RESULTS Chronic hydrocephalus occurred in 12 of 36 (33.3%), 5 of 39 (12.8%), and 1 of 12 (8.3%) patients in the 0 mg/day, 100 to 200 mg/day, and 300 mg/day cilostazol groups, respectively. The multivariate analyses showed that older age (OR 1.10, 95% CI 1.13-1.24; p = 0.012), acute hydrocephalus (OR 23.28, 95% CI 1.75-729.83; p = 0.016), and cilostazol (OR 0.23, 95% CI 0.05-0.93; p = 0.038) independently affected the development of chronic hydrocephalus. Higher dosages of cilostazol more effectively suppressed the serum TNC levels through Days 1 to 12 post-SAH. CONCLUSIONS Cilostazol may prevent the development of chronic hydrocephalus and reduce shunt surgery, possibly by the inhibition of TNC induction after SAH.

Pharyngolaryngeal ulcers associated with the improper use of alendronate.

Bisphosphonates can cause mucosal irritation. Although esophageal ulceration is a well-recognized adverse effect of bisphosphonates, pharyngolaryngeal ulcers associated with the improper use of oral bisphosphonates have rarely been described. A previously healthy 78-year-old woman presented with refractory pharyngolaryngeal ulcers. Extensive evaluation, including biopsy, bacterial culture, and blood tests did not identify any findings that indicated a specific disease diagnosis. Antibiotics and oral prednisolone were ineffective. Ultimately, it was found that the patient regularly took a tablet of alendronate, a type of bisphosphonate, by dissolving it in the oral cavity. Within 2 weeks after withdrawal of the use of the medication, her symptoms were eliminated, and the lesions were completely healed. This case illustrates the importance of correct administration of bisphosphonates. Given the widespread use of bisphosphonates, physicians need to be aware that their improper use can cause pharyngolaryngeal ulcers.

Successful application of endoscopic modified medial maxillectomy to orbital floor trapdoor fracture in a pediatric patient.

Although surgical treatment of orbital floor fractures can be performed by many different approaches, the application of endoscopic modified medial maxillectomy (EMMM) for this condition has rarely been described in the literature. We report on a case of a 7-year-old boy with a trapdoor orbital floor fracture successfully treated with the application of EMMM. The patient suffered trauma to the right orbit floor and the inferior rectus was entrapped at the orbital floor. Initially, surgical repair via endoscopic endonasal approach was attempted. However, we were unable to adequately access the orbital floor through the maxillary ostium. Therefore, an alternative route of access to the orbital floor was established by EMMM. With sufficient visualization and operating space, the involved orbital content was completely released from the entrapment site and reduced into the orbit. To facilitate wound healing, the orbital floor was supported with a water-inflated urethral balloon catheter for 8 days. At follow-up 8 months later, there was no gaze restriction or complications associated with the EMMM. This case illustrates the efficacy and safety of EMMM in endoscopic endonasal repair of orbital floor fracture, particularly for cases with a narrow nasal cavity such as in pediatric patients.

Management of inadvertent vertebral artery injury due to central venous catheterization in a coagulopathic patient.

Case: A 72-year-old man was admitted to the intensive care unit for severe pancreatitis with coagulopathy. He underwent hemodialysis catheter insertion into the internal jugular vein that subsequently leaked arterial blood; vertebral artery cannulation was suspected following a computed tomography scan. Outcome: Under angiographic guidance, the catheter was removed, and an arteriovenous fistula was identified. The patient was successfully treated with endovascular embolization of the affected vertebral artery with detachable coils and N-butyl-2-cyanoacrylate. Conclusion: Despite ultrasound guidance, vertebral cannulation can occur, which can result in serious complications. Prompt management is needed to prevent further sequelae. Endovascular embolization with detachable coils and N-butyl-2-cyanoacrylate appears to be an effective option for vertebral artery injury in patients with coagulopathy.

Analysis of factors influencing sensitization of Japanese cedar pollen in asymptomatic subjects.

OBJECTIVE: Japanese cedar pollinosis is increasing rapidly in Japan. Although analysis has been made concerning the factors influencing the development of the cedar pollinosis, analysis concerning the risk factors influencing the sensitization in asymptomatic subjects has not been done. METHODS: Risk factors for sensitization to Japanese cedar pollen were analyzed among 73 subjects (32 men and 41 women) who do not develop symptoms of pollinosis at the time of Japanese cedar pollen scattering. Their ages ranged from 18 to 60 years with the mean of 34.1 years. Possible factors influencing sensitization were investigated through a written questionnaire and doctors' questioning. Japanese cedar-specific IgE titers and Dermatophagoides pteronyssinus-specific IgE titers in the serum were measured by CAP-FEIA (fluorescent enzyme immunoassay). RESULTS: Of the 73 subjects, 26 were sensitized to the Japanese cedar pollen, for a 36% sensitization rate. Among the eleven factors examined, only one factor was shown to significantly influence the sensitization rate to Japanese cedar pollen. It was sensitization to house dust mites (56.5% vs. 26.0% χ(2) value=6.27, p=0.012). The sensitization rate to the pollen did not correlate to the presence of other allergic diseases, history of rhinosinusitis, family history of Japanese cedar pollinosis, food preference, presence or absence of cedar trees in the surroundings, present living circumstances, childhood circumstances, age, sex, or smoking habits. We calculated odds ratios in order to estimate how much those factors influence the sensitization to Japanese cedar pollen. Significantly high odds ratio for sensitization to house dust mite (6.63; 95% confidence interval (CI): 1.76-32.2) was found. CONCLUSION: The present study indicates that sensitization to the pollen in the subjects without pollinosis is influenced by sensitization to house dust mite.

Descending necrotizing mediastinitis from deep neck infection.

This study aims to identify predisposing characteristics of descending necrotizing mediastinitis (DNM) arising from deep neck infection (DNI) and to determine appropriate therapeutic intervention strategies. We retrospectively reviewed 54 patients (male, n = 34; female, n = 20; mean age, 64.5 years) who had been treated at Mie University Hospital for DNI between April 2001 and October 2011. Eight of nine patients who developed DNM confirmed by computed tomography of the neck and chest, underwent mediastinal drainage (video-assisted thoracic surgical drainage, n = 6; mediastinoscopy-assisted drainage, n = 2). A patient developed uncontrolled acute respiratory distress syndrome after aggressive surgery, resulting in a mortality rate of 12 %. High blood CRP values, and the pharynx and tonsils as origins of infection were factors involved in the development of DNM arising from DNI. In conclusion, DNM remains a destructive and fatal disease that requires aggressive treatment including mediastinal exploration.

The role of transforming growth factor-α on mucin overproduction in eosinophilic chronic rhinosinusitis.

OBJECTIVES: Eosinophilic chronic rhinosinusitis (ECRS) is considered a refractory and intractable disease with thick mucus production, long-term nasal congestion, loss of sense of smell and intermittent acute exacerbations secondary to bacterial infections. In this study, we investigated which growth factor is deeply involved in the mucin overproduction in ECRS. METHOD: We employed fluorescence immunohistochemical analysis to evaluate whether or not TGF-α expression was upregulated in the nasal tissue of ECRS patients. We also examined MUC5AC transcription using a luciferase reporter plasmid in HM3-MUC5AC cells and A549 cells in order to assess the role of TGF-α in human epithelial cells. RESULTS: TGF-α immunoreactivity was found markedly increased in the submucosal tissue in the ECRS patient compared with that of a normal patient and with noneosinophilic CRS. TGF-α synergized with TNF-α to upregulate MUC5AC expression in human epithelial cells through the ERK signaling pathway. CONCLUSION: Our results demonstrated that TGF-α was highly expressed in the upper airway tract in ECRS patients and is deeply involved in mucus hypersecretion.