• 제목/요약/키워드: malformation

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전두개와 경막에 발생한 동정맥 기형 - 증례보고 - (Dural Arteriovenous Malformation on the Anterior Cranial Fossa - Case Report -)

  • 박태일;황금;변진수;허철;홍순기;한용표
    • Journal of Korean Neurosurgical Society
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    • 제30권2호
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    • pp.244-249
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    • 2001
  • Dural arteriovenous malformations(AVM) are not uncommon. Reports of intracranial dural AVM have been increasing but most of them deal with dural AVM in the region of the cavernous sinus, posterior fossa and tentorium, but those of the anterior cranial fossa are very rare. Recently, we experienced two cases of right frontal dural arteriovenous malformation fed mainly by both ethmoidal arteries. The angiographic appearance in these two cases is quite uniform. The nidus was located in the frontal dura, although their main feeders were dural arteries. They were drained through an intracerebral cortical vein associated with aneurysmal dilatation of proximal portion into superior sagittal sinus. Spontaneous intracerebral hematoma was the cause of the clinical symptoms. We report two cases of intracerebral hematoma, caused by dural AVM, which was successfully managed by surgical treatment.

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Type 1.5 Split Cord Malformation : A New Theory of Pathogenesis

  • Sun, Mengchun;Tao, Benzhang;Luo, Tianbao;Gao, Gan;Shang, Aijia
    • Journal of Korean Neurosurgical Society
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    • 제65권1호
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    • pp.138-144
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    • 2022
  • To report two cases of type 1.5 split cord malformation (SCM), a subtype of SCM with combined characteristics of types I and II and to review the relevant literature and propose a new possible pathogenetic theory for type 1.5 SCM. A 52-year-old woman had hemicords within a single dural sac with a dorsal bony septum at the L5 level. A 9-year-old boy had hemicords within a single dural sac with a ventral bony septum and fibrous extension at the L3 level. Both patients underwent microsurgical treatments for removing the bony septum, detethering the spinal cord, and sectioning the filum terminale. The surgical procedure revealed an extradural partial bony septum and hemicords within an intact single dural sac in each patient. Both patients were discharged from the hospital without de novo nerve dysfunction. Published cases have validated that types I and II SCM can overlap. We recommend recent type 1.5 SCM as a normative terminology for this overlapping SCM and report two rare cases of this SCM. We propose an associated pathogenesis consisting of uneven distribution and regression to explain type 1.5 SCM. Furthermore, we postulate that the amount of condensing meninx primitiva might determine whether the left bony septum has fibrous extensions to the opposite dura in type 1.5 SCM.

Dental Management of First Permanent Molars in Molar-incisor Malformation Patients: A Case Report

  • Seung-Hyun, Kim;Gi-Min, Kim;Jae-Sik, Lee;Hyun-Jung, Kim
    • Journal of Korean Dental Science
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    • 제15권2호
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    • pp.181-189
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    • 2022
  • Molar incisor malformation (MIM) has been introduced as a new type of dental anomaly. Currently, the morphological and histological characteristics of MIM are known; however, its etiology has not been clearly identified. To date, the long-term prognosis of first permanent molars (FPM) affected by MIM has rarely been reported, and few treatment guidelines have been established. The purpose of this case report was to present guidelines for the extraction of FPM affected by MIM, depending on the presence of the third molar. In patients with a third molar, spontaneous mesial shift of the posterior molars might be induced by extracting the FPM at an appropriate time, that is, when the second permanent molar is at an early furcation stage of the tooth. However, it is recommended that FPM be preserved for as long as possible if a third molar does not exist. When an FPM needs to be extracted, it is suggested to consider space maintenance.

Intramuscular epidermal cyst in the masticator space: a case report

  • Sang Woo Han;Jiye Kim;Sug Won Kim;Minseob Eom;Chae Eun Yang
    • 대한두개안면성형외과학회지
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    • 제24권4호
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    • pp.193-197
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    • 2023
  • An epidermal cyst, also known as an epidermoid cyst or epidermal inclusion cyst, is the most prevalent type of cutaneous cyst. This non-cancerous lesion can appear anywhere on the body, typically presenting as an asymptomatic dermal nodule with a visible central punctum. In the case presented herein, an epidermal cyst with uncommon features was misdiagnosed as a lymphatic malformation based on preoperative magnetic resonance imaging (MRI). A 61-year-old man came to us with a swollen left cheek that had been present for 11 months. The preoperative MRI revealed a 3×3.8×4.6 cm lobulated cystic lesion with thin rim enhancement in the left masticator space. The initial differential diagnosis pointed toward a lymphatic malformation. We proceeded with surgical excision of the lesion via an intraoral approach, and the specimen was sent to the pathology department. The pathological diagnosis revealed a ruptured epidermal cyst, indicating that the initial diagnosis of a lymphatic malformation based on preoperative MRI was incorrect. Epidermal cysts located under the muscle with no visible central punctum are uncommon, but should be considered if a patient presents with facial swelling.

Late side effects of bleomycin injection into the lower lip mucosa of a nine-year-old patient with venous malformation

  • Kezia Rachellea Mustakim;Soung Min Kim
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • 제49권5호
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    • pp.304-307
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    • 2023
  • Venous malformation (VM) is a benign lesion of blood vessels caused by an error in vascular morphogenesis during the embryologic phase. This entity mostly affects the head and neck region, including the lips, tongue, buccal mucosa, gingiva, or palate. VM may cause functional and aesthetic impairments. The anatomical structure and shape of the lips provide an important aesthetic accent for an individual. Therefore, management of VM in the lip area without postoperative defects or scarring is challenging. In this brief communication article, we present a conservative approach to lip VM in a nine-year-old boy using a bleomycin injection that had good aesthetic and functional outcomes. Injection of 2 mL of 1/10 of 15 mg bleomycin in a saline dilution into the lip mucosa may present a drug reaction as a white plaque and reddish owl eye lesion that takes up to three weeks to resolve without a scar. It is important to recognize the characteristics and self-limiting nature of postoperative bleomycin complications to avoid unnecessary treatment.

Intraosseous vascular malformation of the skull: a case report and literature review

  • Donghyun Lee;Chul Hoon Chung;Seong Jin Cho
    • 대한두개안면성형외과학회지
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    • 제25권4호
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    • pp.187-191
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    • 2024
  • A 59-year-old woman presented to our clinic with a 3.5×3-cm protruding mass on her forehead. A skull X-ray revealed a radiolucent osteolytic lesion on the left side of the frontal bone. Additionally, computed tomography showed a 3.1×1.7×3.6-cm mass exhibiting a "sunburst" pattern situated between the outer and inner tables of the skull, just superior and lateral to the left frontal sinus. This pattern suggested the presence of an intraosseous vascular malformation (IVM). The lesion was approached via a bicoronal incision. En-bloc resection was performed, removing the mass along with approximately 0.5 cm of the surrounding normal bone without injury to the exposed frontal sinus mucosa. The exposed mucosa was reinforced with a galeal flap, and cranioplasty with bone cement was performed to repair the resulting bony defect. Pathological examination confirmed a diagnosis of intraosseous cavernous-type malformation with mixed cavernous and capillary histological features. We report this case of IVM and review the existing literature, highlighting the satisfactory functional and aesthetic outcomes after surgery.

선형가속기 방사선 수술을 이용한 뇌동정맥기형의 치료 (Linac Based Radiosurgery for Cerebral Arteriovenous Malformations)

  • 이성열;손은익;김옥배;최태진;김동원;임만빈;김인홍
    • Journal of Korean Neurosurgical Society
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    • 제29권8호
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    • pp.1030-1036
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    • 2000
  • 본 논문에서 뇌동정맥기형에 대해 포톤나이프로 정위적 방사선 수술을 시행한 후 방사선학적으로 10명의 환자중 6례에서 완전 폐색, 4례에서 부분적 폐색을 보였으며, 특히 3cm이하의 작은 뇌동정맥기형 7례중 6례에서 완전 폐색을 보였다(완전 폐색율 : 85.7%). 모든 환자에서 방사선 수술로 인한 방사선학적 합병증의 발병은 없었다. 신경학적으로도 모든 환자에서 방사선 수술 전에 보이던 증상들이 호전을 보였다. 포톤나이프 방사선 수술 시스템은 이미 실험적으로 안정성이 검증된 것으로 이 논문에서 임상적으로 방사선 수술의 안정성 및 정확성을 다시 확인할 수 있었다. 정위적 방사선 수술은 지금까지 치료 불가능했던 부위에 생긴 병소, 수술이나 다른 치료방법으로 완전 제거에 실패한 경우, 노인환자나 다른 내과적 질환으로 수술이 어렵거나 수술적 치료를 거부하는 환자에서도 좋은 치료 방법으로 사용되어지며, 특히 뇌 중요부위나 심부병변에 위치한 경우 또는 최대직경이 3cm 이하의 소 동정맥기형으로 발견 당시 출혈량이 많지 않고 신경학적 결손이 경미한 환자에서 좋은 적응증이 되리라 사료된다.

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아프리카발톱개구리의 시각계 발생에 관여하는 식물추출물 3종에 관한 연구 (Study of Effects of Crude Extracts of Three Plants Concerned on Optic Development of African Clawed Frog, Xenopus laevis)

  • 황용기;이유화;윤춘식;박용욱;김덕훈;정선우
    • 한국안광학회지
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    • 제12권3호
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    • pp.151-158
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    • 2007
  • 본 연구는 한약재로 널리 시용되는 원지 (Polygala tenuifolia), 구기자(Lycium chinensis), 산수유(Cornus officinalis) 추출물을 아프리카발톱개구리(Xenopus laevis)의 초기 배시기에 1, 10, $100{\mu}g/ml$의 농도로 처리하여 96시간 $24^{\circ}C{\pm}0.5^{\circ}C$에서 배양하였을 때 배 발생 중 나타나는 전반적인 기관 이상을 조사하였으며 특히 시각계의 발생이상에 초점을 맞추었다. 각 식물 추출물의 농도가 증가할수록 성장이 저해되고 시각계의 발생이상, 체축의 뒤틀림, 수종, 소화관의 꼬임이상, 체색이상 등의 기형의 발생률이 증가하였다. 시각계 이상이 가장 뚜렷이 나타난 군은 $100{\mu}g/ml$ 농도로 구기자 추출물을 처리한 것이었으며 각 실험군의 150개체 중 27% 정도가 시각계 발생이상을 나타내었다. 조직학적 연구에서는 뇌의 뇌실은 비정상적으로 확대되었고 눈에서는 유리체방의 형성부전이 나타났으며 망막세포층의 구분이 뚜렷하지 않았다.

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큐라리노 증후군의 임상 경험 (Clinical Experience of Currarino Syndrome)

  • 김태훈;조민정;김대연;김성철;김인구
    • Advances in pediatric surgery
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    • 제17권1호
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    • pp.65-71
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    • 2011
  • Currarino syndrome is a hereditary syndrome characterized by the triad of a sacral bony defect, presacral mass and anorectal malformation. We retrospectively reviewed 13 Currarino syndrome patients who were treated in our center between 1997 and 2010. Demographic data, initial symptoms, initial diagnosis. pathologic diagnosis of presacral mass, associated anomalies and managements were analyzed. There were 8 boys and 5 girls. Four patients were diagnosed as Currarino syndrome immediately after birth with failure of passage of meconium and abdominal distension. Four patients underwent surgery for imperforate anus immediately after birth and W8re diagnosed as Currarino syndrome later and underwent reoperation. Three patients were diagnosed during work-up and management with of the tentative diagnosis of Hirschsprung's disease. Diagnosis of the remaining two patients was at the age of 26 months and 9 years and anorectal malformation was not associated. Twelve patients showed hemi-sacrum and one patient showed bilateral sacral subtotal agenesis. Two patients without anorectal malformation underwent presacral mass excision, untethering of spinal cord and repair of myelomeningocele. Six out of 8 patients, excluding 3 that expired or were lost to follow up, with anorectal malformation underwent colostomy, presacral mass excision, untethering of spinal cord, repair of myelomeningocele, posterior sagittal anorectoplasty and colostomy repair. One patient underwent only posterior sagittal anorectoplasty after colostomy. One waits the scheduled operation only with Hegar dilatation. Pathologic examation of presacral masses showed myelomeningoceles in 4 patients, lipomyelomeningoceles in 3 patients and dermoid Cyst in one patient. Teratoma was combined in 2 patients. Eight patients needed neurosurgical operation for spinal cord problems. Seven patients had urologic anomalies and two of them underwent operation. Currarino syndrome should be considered as a differential diagnosis in pediatric patients with abdominal distension, constipation and anorectal malforlnation. For proper evaluation and treatment, a multi-disciplinary approach is recommended.

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살균제 Tebuconazole이 한국산 개구리류(두꺼비, 청개구리, 참개구리) 배아 발달에 미치는 영향 (Effects of fungicide tebuconazole on the embryonic development of Korean domestic frogs (Bufo gargarizans, Hyla japonica, and Pelophylax nigromaculatus))

  • 이해범;고선근
    • 환경생물
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    • 제39권3호
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    • pp.311-318
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    • 2021
  • 국내에 서식하는 개구리들의 배아를 이용하여 살균제인 tebucoanzole의 독성을 파악하기 위해 FETAX (Frog Embryo Teratogenesis Assay-Xenopus) 기법에 따라 두꺼비(Bufo gargarizans), 청개구리(Hyla japonica), 참개구리(Pelophylax nigromaculatus)의 배아를 배양하면서 tebuconazole의 효과를 probit 분석법으로 조사하였다. 그 결과, tebuconazole의 농도에 의존하여 유생의 체장 길이는 감소하고 치사율과 기형률은 증가하였으며 tebuconazole의 teratogenic concentration (EC50)은 각각 34.4, 10.6, 14.9 mg kg-1을 나타내었고 embryo lethal concentrations (LC50)은 75.4, 38.2, 39.6 mg kg-1 을 나타내었다. Teratogenic index (TI=LC50/EC50)는 각각 2.19, 3.58, 2.65을 나타내어 두꺼비, 청개구리, 참개구리 배아 발달에 최기형성 물질로 작용함을 알 수 있었다. 이상의 결과들로 보아 tebuconazole은 낮은 농도에서 개구리 배아의 발달에 민감하게 반응하였으며 치사율, 기형률, 성장률, 기형양상 등을 기존의 연구들과 비교하였을 때 유사한 결과를 나타내어 국내 서식하는 개구리류 배아발달에 영향을 미칠 수 있는 것으로 여겨지며, 종에 따라 치사율 및 기형률, 기형양상 등의 차이를 나타내는 원인 등을 명확히 파악하기 위해서 종 특이적 특성 등을 규명하는 연구가 더 필요할 것으로 여겨진다.