• Journal of Korean Neurosurgical Society
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• 제29권2호
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• pp.265-269
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• 2000

Traumatic aneurysm is a rare complication of head trauma. Unfortunately, it is rarely recognized until their presence is heralded by delayed deterioration or intracranial hematoma, etc,. Early diagnosis and surgical treatment are most important for good outcome. We recently encountered a patient who developed a pericallosal aneurysm after major trauma. With a high index of suspicion about traumatic aneurysm, brain MRI and MRA were performed. Definitive diagnosis was made by transfemoral carotid angiography, revealing a aneurysm of the right proximal pericallosal artery. The aneurysm was explored through right interhemispheric approch. A $15{\times}15mm$ sized traumatic aneurysmal sac, located in proximal portion of right pericallosal artery was noted. It was dissected from the falx of cerebri and its neck was clipped. The postoperative course was uneventful. Traumatic intracranial aneurysm should be suspected in any patient who deteriorates after the trauma. Early recognition and prompt treatment are warranted in this setting. Definitive surgical treatment of all traumatic intracranial aneurysm is recommended.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제42권5호
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• pp.284-287
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• 2016

Nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin syndrome, is characterized by various embryological deformities and carcinoma formation. It is caused by PTCHI gene mutations and is autosomal dominantly inherited. Some of the main symptoms of NBCCS are multiple basal cell carcinomas, multiple keratocystic odontogenic tumors (KCOTs) of the mandible, hyperkeratosis of the palmar and plantar, skeletal deformity, calcification of the falx cerebri, and facial defomity. Recurrent KCOT is the main symptom of NBCCS and is present in approximately 90% of patients. In NBCCS, KCOTs typically occur in multiples. KCOTs can be detected in patients under the age of 10, and new and recurring cysts develop until approximately the age of 30. The postoperation recurrence rate is approximately 60%. This case report presents a 14-year-old female patient with a chief complaint of a cyst found in the maxilla and mandible. The patient was diagnosed with NBCCS, and following treatment of marsupialization and enucleation, the clinical results were satisfactory.

• Clinical and Experimental Pediatrics
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• 제50권8호
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• pp.789-793
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• 2007

기저세포 모반 증후군은 피부의 기저세포 암종, 악골의 치성 각화낭종, 손, 발바닥의 소와, 이소성 석회화, 기저세포 모반 증후군의 가족력 등을 주요 특징으로 하는 상염색체 우성 유전 질환이다. 저자들은 갑자기 커지는 좌측 안면부 종물을 주소로 내원한 9세 남자 환아에서 외관상 특징적인 소견 관찰되고 분자유전학적 검사에서 PTCH 유전자의 결손 확인되어 기저세포 모반 증후군 확진된 1례를 경험하였기에 보고하는 바이다.

• 대한소아치과학회지
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• 제41권1호
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• pp.34-39
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• 2014

기저세포모반증후군은 상염색체 우성의 유전 질환이며 주요소견으로는 다발성 기저세포암, 악골의 다발성 각화낭종, 늑골이상, 손과 발바닥의 소와, 대뇌겸의 석회화 등이 있다. 기저세포모반증후군에서 호발 하는 각화낭성 치성종양은 치성기원의 골내종양으로 착각화된 중층 편평상피에 의해 특징적으로 이장되어 있고 공격적이고 침습적인 성향을 갖는 것으로 정의된다. 본 증례는 감별진단이 필요한 상악 견치부위의 단방성의 낭종을 주소로 내원한 환아가 조직검사를 통해 각화낭성 치성종양으로 밝혀진 후 임상적, 방사선학적 검사를 통해 기저세포모반 증후군으로 진단된 경우이다. 종양은 적출술로 치료되었고 증후군과 연관된 추가적인 질환들의 조기진단을 위해 정기적인 검진이 필요할 것이다.

• Asian Pacific Journal of Cancer Prevention
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• 제14권11호
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• pp.6337-6340
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• 2013

Through retrospective analysis of 13 cases of magnetic resonance image (MRI) manifestations of atypical meningiomas confirmed by operation and pathology in the First Affiliated Hospital of Xinxiang Medical University, the objective of this study was to evaluate the diagnostic value of MRI in order to improve the accuracy rate of preoperative diagnosis. In this retrospective analysis of MRI findings for atypical meningiomas in First Affiliated Hospital of Xinxiang Medical University from January to July in 2012, the location, morphology and tumor signals and other tumor imaging characteristics were covered. In 13 cases of atypical meningioma patients of this group, most tumors were located at typical sites (10/13), mainly the falx cerebri, parasagittal, convexity, saddle area. Only two cases were at atypical locations, 1 in the cerebellar hemisphere and 1 in a lateral ventricle. Most of the tumors showed T1 and T2 isointensity signals, and necrosis, calcification, and peritumoral edema were always featured. DWI showed isointensity in 11 cases (11/13), and hyperintensity in 2. Some 9 cases had dural tail signs, 12 had accurate positioning (12/13), and 2 were postoperative recurrences. MRI has high value in the diagnosis of atypical meningiomas, with important roles in early clinical diagnosis, treatment and prognosis evaluation.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제36권1호
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• pp.7-12
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• 2014

Nevoid basal cell carcinoma syndrome (NBCCS) is inherited as an autosomal dominant trait with variable conditions, including multiple basal cell carcinoma, numerous keratocystic odontogenic tumors (KOTs) in the jaws, ectopic calcification of the falx cerebri, bifid ribs, macrocephaly, kyphoscoliosis, cleft palate, frontal and temporal bossing, mild ocular hypertelorism, mild mandibular prognathism, vertebral fusion, and so on. A 16-year-old boy visited the Dong-A University Medical Center, requiring diagnosis and treatment of multiple cystic lesions. He presented with many conditions related to NBCCS, including multiple KOTs, bifid rib, cleft lip, frontal bossing, mild ocular hypertelorism, and mild mandibular prognathism. No characteristic cutaneous manifestations (nevoid basal cell carcinoma) were observed in this patient. We report on a case of multiple KOTs associated with NBCCS with a review of the literature.

• Journal of Korean Neurosurgical Society
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• 제54권2호
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• pp.100-106
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• 2013

Objective : To investigate the cases of intracranial abnormal brain MRI findings even in the negative brain CT scan after mild head injury. Methods : During a 2-year period (January 2009-December 2010), we prospectively evaluated both brain CT and brain MRI of 180 patients with mild head injury. Patients were classified into two groups according to presence or absence of abnormal brain MRI finding even in the negative brain CT scan after mild head injury. Two neurosurgeons and one neuroradiologist validated the images from both brain CT scan and brain MRI double blindly. Results : Intracranial injury with negative brain CT scan after mild head injury occurred in 18 patients (10.0%). Headache (51.7%) without neurologic signs was the most common symptom. Locations of intracranial lesions showing abnormal brain MRI were as follows; temporal base (n=8), frontal pole (n=5), falx cerebri (n=2), basal ganglia (n=1), tentorium (n=1), and sylvian fissure (n=1). Intracranial injury was common in patients with a loss of consciousness, symptom duration >2 weeks, or in cases of patients with linear skull fracture (p=0.00013), and also more frequent in multiple associated injury than simple one (35.7%>8.6%) (p=0.105). Conclusion : Our investigation showed that patients with mild head injury even in the negative brain CT scan had a few cases of intracranial injury. These findings indicate that even though the brain CT does not show abnormal findings, they should be thoroughly watched in further study including brain MRI in cases of multiple injuries and when their complaints are sustained.

• 대한두개안면성형외과학회지
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• 제13권1호
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• pp.50-53
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• 2012

Purpose: The Dyke-Davidoff-Masson syndrome is a rare disease entity that was first reported in 1993, and it is characterized by not only the cerebral hemiatrophy that is accompanied by the ipsilateral ventriculomegaly and ipsilateral compensatory osseous hypertrophy, but also the overgrowth of the paranasal sinuses. No studies have attempted to examine it from perspectives of the skull deformity and plastic surgery. Here, we report our case with a review of the literatures. Methods: A 45-year-old man with Dyke-Davidoff-Masson visited our medical institution with nasal bone fracture. Based on the previously taken brain MRI scans, we measured the degree of craniofacial deformity, and the horizontal distance, which is based on the margin of the skull, as well as the falx cerebri. Results: We made a comparison of the degree of craniofacial deformity. This showed that the mean horizontal distance on the axial view was shorter by approximately 28.46%, as compared with that of the left unaffected side. Conclusion: The Dyke-Davidoff-Masson is characterized by a concurrent presence of the atrophy of the cerebral hemisphere, with the cranial deformity. For the reconstruction of the bone and soft-tissue deformity with Dyke-Davidoff-Masson syndrome, it is needed to perform objective assessments.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제34권4호
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• pp.485-489
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• 2008

Basal cell nevus syndrome is a hereditary disease of an autosomal dominant trait with variable conditions such as basal cell carcinomas of the skin, deformity of rib, fusion of vertebrae, mental retardation, hypertelorism, and multiple odontogenic keratocysts. A 32 years old man with pus discharge from fistula on the vestibule of left upper 1st molar visited to Chosun University Dental Hospital. Radiographic evaluation revealed multiple maxillary and mandibular cysts that had multilocular radiolucency on left mandibular body area, thining of inferior border of left border of ramus and well defined unilocular radiolucency above right upper 1st and 2nd molar and from left upper 1st premolar to 2nd molar. In chest PA view, he had a forked rib in the left 4th rib and in skull PA view the calcification of falx cerebri was observed. There was not any skin lesion. After the preliminary evaluation, the patient was diagnosed with basal cell nevus syndrome and he underwent marsupialization for decreasing the size of cystic lesion and came to hospital for dressing 3days a week. As time goes by, the size of lesion decreased. So, one and half year after marsupialization, he underwent cyst enucleation and iliac bone graft for the mandibular lesion and buccal fat pad grafts for the maxillary lesions. After the surgery, the patient experienced normal healing without any complications and he is on long-term follow-up.