The epithelial lining of odontogenic keratocysts exhibits either parakeratosis or orthokeratosis. In 2005, the WHO classified odontogenic keratocysts with parakeratosis as keratocystic odontogenic tumors (KCOT). Odontogenic keratocysts with orthokeratosis were not classified as odontogenic tumors, but instead referred to as orthokeratinized odontogenic cysts (OOC). To clarify the difference between these two lesions, we investigated their biological characteristics using immunohistochemical studies for cytokeratins (CK) in KCOT and OOC as well as in dentigerous cysts (DC), radicular cysts (RC) and dermoid cysts (DMC). We examined twenty-five cases of KCOT, fifteen cases each of OOC, DC and RC, and ten cases of DMC. We studied the immunohistochemical expression of CK10, 13, 17 and 19. To evaluate the immunohistochemical staining pattern, we divided the epithelial lining of the lesions into three layers (surface layer: su, spinous layer: sp, basal layer: ba). For CK10, most OOC and DMC specimens of su and sp were positive. For CK13 and 19, most KCOT, DC and RC specimens of su and sp were positive. For CK17, most KCOT specimens of su and sp were positive. The percentages of total CK expression of su and sp, and ba of CK19 differed significantly between the lesions (P < 0.001). These results support the hypothesis that OOC originate from not the odontogenic apparatus, but the oral epithelial component.

Charcot-Marie-Tooth disease (CMTD) is a hereditary peripheral neuropathy and is characterized by progressive muscle atrophy and motor-sensory disorders in all 4 limbs. Most reports have indicated that major challenges with general anesthetic administration in CMTD patients are the appropriate use of nondepolarizing muscle relaxants and preparation for malignant hyperthermia in neuromuscular disease. Moderate sedation may be associated with the same complications as those of general anesthesia, as well as dysfunction of the autonomic nervous system, reduced perioperative respiratory function, difficulty in positioning, and sensitivity to intravenous anesthetic agents. We decided to use intravenous sedation in a CMTD patient and administered midazolam initially and propofol continuously, with total doses of 1.5 mg and 300 mg, respectively. Anesthesia was completed in 3 hours and 30 minutes without adverse events. We suggest that dental anesthetic treatment with propofol and midazolam may be effective for patients with CMTD.

We describe the case of a 37-year-old woman who had been diagnosed with EhlersDanlos syndrome (EDS) 4 years earlier and was scheduled to undergo removal of synovial chondromatosis in the temporomandibular joint. EDS is a heritable connective tissue disorder and has 6 types. In this case, the patient was classified into EDS hypermobility type. The major clinical feature of this type is joint hypermobility. The patient had sprain or subluxation of the elbows and ankles and dislocation of the knees. Anticipated problems during general anesthesia would be affected by the disease type. For this patient, extra attention was directed to positional injury-induced neuropathy and articular luxation, cutaneous injuries, injuries related to intubation and ventilation, and postoperative pain. Anesthesia was induced with propofol, remifentanil, and rocuronium and maintained with oxygenair-desflurane, propofol, remifentanil, fentanyl, and rocuronium. In this case, the patient was safely managed without adverse events.Key Words: Ehlers-Danlos syndrome; Hypermobility type; Joint hypermobility and dislocation. E hlers-Danlos syndrome (EDS) is a heritable connective tissue disorder classified into 6 types: classic, hypermobility, vascular, kyphoscoliosis, arthrochalasis, and dermatosparaxis.1 This case is classified into EDS hypermobility type (EDS-HT), and the chief manifestation was joint hypermobility and dislocation. In the branch of dentistry, temporomandibular joint (TMJ) disorder may be one of the complaints. 2 We report the case of a patient with EDS-HT who underwent removal of synovial chondromatosis in the TMJ under general anesthesia. CASE REPORTA 37-year-old woman (173 cm [68 in], 100 kg, body mass index ¼ 33) with EDS-HT was scheduled for removal of synovial chondromatosis in the TMJ space under general anesthesia.The patient was a native-born American and had been living in Japan for about 20 years. Her thumb showed joint hypermobility, and her elbow showed hyperextension. Her thumb in conjunction with flexion and extension of her wrist could touch her forearm ( Figure A and B). Her skin was not hyperextensible but smooth and velvety. She had suffered sprain or subluxation of the elbows and ankles many times. She had also undergone operations for dislocation of the knees. At present, she had pain into the TMJ, both knees, thumb of the hand, and the iliotibial band. Her medical history included gastroesophageal reflux without current medication use. Thinning hair was being treated with cepharanthine, carpronium chloride hydrate, and deprodone propionate. Electrocardiography revealed sinus arrhythmia with irregular R-R interval and inverted T waves in leads III and V1. Screening echocardiography revealed normal systolic and diastolic function.

A rare case of adenoid cystic carcinoma of the external auditory canal with magnetic resonance imaging appearances is reported. Both T1 weighted and T2 weighted MR images showed the tumour as a hypointense mass, although there was marked contrast enhancement. Microscopic examination of the resected tumour showed a preponderance of solid tumour cell nests. According to previous reports, these pathological and radiological findings indicate a poor prognosis.

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