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Papillon-Lefèvre syndrome (PLS) is an extremely rare autosomal recessive disorder characterized by palmoplantar keratoderma and periodontitis and occuring with an estimated incidence of 1-4 cases per million. Patients with PLS are highly susceptible to infection. The etiology of an infective susceptibility is unknown; however, an association with defects in neurophil dysfunction, insufficient lymphocyte response to pathogens, defects in monocyte functions and impairment of NK cell cytotoxic function has been suggested. To our knowledge, this is the first case report of atypical maxillary sinusitis accompanied by PLS, and we represent the case with a review of the related literatures.
Palatal myoclonus (palatal tremor) was reported to be presented with a sole symptom of objective pulsating tinnitus and could be controlled by botulinum toxin injection alone. However, there were 3 cases reported in Korea of palatal myoclonus tinnitus controlled with botulinum toxin injection through the dual administration route of the mouth and nasal cavity. We present an 11-year-old boy of essential palatal myoclonus tinnitus, which was controlled by intraoral botulinum toxin injection to the tensor veli palatini muscle alone. Intraoral injection of botulinum toxin to the anatomical location of tensor veli palatini muscle with the guidance of electromyography was effective and safe for the child of objective tinnitus caused by palatal myoclonus.
Paraganglioma is an uncommon and slowly-growing benign neuro-endocrine tumor originating from the extra-adrenal paraganglia. The most common site of head and neck paraganglioma is the carotid body. Paraganglioma of thyroid gland is a rare disease, and is hard to diagnose preoperatively since the pathologic features are similar to thyroid neoplasm, especially to medullary carcinoma. Occasionally, multiple paragangliomas can be found in the body. To our knowledge, there are two cases of synchronous carotid body tumor with thyroid paraganglioma that have been reported to date. We report a case of 34-year-old female with carotid body paraganglioma synchronous with intra-thyroid paragangloma, which was misdiagnosed as papillary thyroid carcinoma in the preoperative evaluation. After the tumors were successfully excised, there were no signs of complications and the patient has survived the 2-year-follow-up period without recurrence.
Parapharyngeal abscess is relatively common but it can cause life-threatening complications like internal jugular vein thrombophlebitis, septic shock, airway obstruction, and mediastinitis. The diagnosis is dependent on physical examination, endoscopic view, and CT and MR imaging. The treatment is antibiotics but sometimes incision and drainage are necessary. Pseudoaneurysm in deep neck space is very rare and mainly caused by trauma, postoperative state of head and neck surgery and radiation therapy. We herein report a case of massive oropharyngeal bleeding from pseudoaneurysm of the superior thyroid artery associated with parapharyngeal abscess with a review of related literature.
Inflammatory pseudotumor is a pathologically benign condition, but it demonstrates a wide range of clinical features ranging from silent small sized tumors to aggressive features mimicking malignancy. Pseudotumors most commonly occur in the orbital area, and the involvement of the middle ear cavity is extremely rare. Several modalities are known for the treatment of pseudotumors, including complete surgical excision, oral steroid therapy, and radiation therapy. We describe a 35-year-old woman with inflammatory pseudotumor involving the middle ear cavity. The patient was treated with canal wall up tympanomastoidectomy and additional treatments with steroid and radiation therapy. However, she showed side effects to high dose steroid treatment and no response to radiation therapy. Therefore, we decided to use methotrexate with low dose steroid. After treatment, symptoms were completely resolved and there was no evidence of recurrence 1 year after maintaining immunosuppressant treatment.
Injection laryngoplasty is a simple and noninvasive method for treating unilateral vocal cord paralysis. Lately hyaluronic acid is widely used as injection material, because it is spontaneously resolved after few months and can be injected superficially compared to other materials. However, if hyaluronic acid is injected in the superficial layer of lamina propria (Reinke’s space), it can cause inflammation or granuloma, interrupt the vibration of the vocal cord and worsen the quality of voice. We here present a case of granuloma that occurred as a complication of injection laryngoplasty, where simple surgical method was employed to improve voice quality. Korean J Otorhinolaryngol-Head Neck Surg 2012;55:782-6
Intracranial lipomas are unfrequent tumors developed from mesenchymatous cells. The corpus callosum is the most frequent location (50%) in the intracranial regions, and these tumors are rarely present in the cerebellopontine angle, the internal acoustic meatus, or intravestibular lesions. With a review of literature, authors report a rare case of the left-sided intravestibular lipoma presented as sudden hearing loss in 17-year-old female. Furthermore, differential diagnostic magnetic resonance imaging characteristics of lipomas are discussed in detail.