• 제목/요약/키워드: Malformation

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Megalencephaly-capillary malformation-polymicrogyria syndrome: the first case report in Korea

  • Choi, Yeon-Chul;Yum, Mi-Sun;Kim, Min-Jee;Lee, Yun-Jung;Ko, Tae-Sung
    • Clinical and Experimental Pediatrics
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    • 제59권sup1호
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    • pp.152-156
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    • 2016
  • Megalencephaly-capillary malformation-polymicrogyria syndrome (MCAP), previously known as macrocephaly-cutis marmorata telangiectatica congenita and macrocephaly-capillary malformation syndrome, is a rare multiple-malformation syndrome that is characterized by progressive megalencephaly, capillary malformations of the midline face and body, or distal limb anomalies such as syndactyly. Herein, we report a female infant case that satisfies the recently proposed criteria of MCAP and describe the distinctive neuroradiological and morphological features. We have also reviewed recently published reports and the diagnostic criteria proposed by various authors in order to facilitate the clinical diagnosis of these children in pediatric neurology clinics.

초음파(超音波)에 대한 ICR Mouse 착상전기(着床前期)의 개체(個體) Level 영향(影響)(기형(奇形).배사망.(胚死亡))으로부터 초음파(超音波)의 물리학적(物理學的) 특성(特性)에 대한 연구(硏究) (Embryonic Effects of Ultrasound Irradiation on Preimplantation Stage of ICR Mouse Embryos - About embryonic death and malformation of ultrasound mechanisms -)

  • 송재관;김예현
    • 대한방사선기술학회지:방사선기술과학
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    • 제18권2호
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    • pp.75-86
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    • 1995
  • Embryos and fetuses are more sensitive to various environmental agents than adults of children biological effects following the exposure, such as intrauterin, malformation, have intimate conception with the prenatal exposure. There have been many studies on radiation and other agent. However, imformation about the ultrasound effects is limited. It is very important to study the effect of ultrasound with these kinds of fatera in consideration of ultrasound protection and safty. In this study, embryonic and fefal effects of ICR mouse embryos irradiated on 24, 48, 12 and 192 hpc of preimplantation and organogenesis period at the intensity of $0.5{\sim}3\;W/cm^2$ were investigated. Many type of external malformation observed in mouse irradiated on 72 hpc and 192 hpc. However, the embryos irradiated on 24 hpc and 48 hpc, at witch embryos had less then 6 cells and were pre-compaction stage, had no sensitivity for external malformation. The threshold doses of external malformation in mouse irradiated on 72 hpc and 192 hpc, at which embryos were consisted of $16{\sim}32$ cells and neural formation stage, were $1\;W/cm^2$ and $0.5\;W/cm^2$.

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Brown-Sequard 증후군과 Horner씨 증후군으로 발현된 동정맥 기형 파열로 인한 경추 경막외 혈종 - 증례보고 - (A Cervical Epidural Hematoma Caused by Ruptured Arteriovenous Malformation Presenting as a Brown-Sequard and Horner's Syndrome - A Case Report -)

  • 전인호;송준혁;박향권;신규만;김성학
    • Journal of Korean Neurosurgical Society
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    • 제30권sup1호
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    • pp.144-148
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    • 2001
  • We report a rare case of Brown-Sequard syndrome associated with Horner's syndrome in cervical epidural hematoma caused by a ruptured arteriovenous malformation. A 54-year-old man developed sudden sharp neck pain, radiating to the interscapular area. Within hours, left side hemiplegia and decreased tactile sense and loss of contralateral pain sense ensued. Emergency cervical magnetic resonance image showed an epidural hematoma over the cervico-thoracic junction. The hematoma was located in the left posterolateral area of the cervical spinal canal. Emergent decompressive laminectomy and an evacuation of the hematoma were performed. A tangled soft tissue mass found in the hematoma was proven to be an arteriovenous malformation. To the authors, knowledge, this might be the first case of a Brown-Sequard syndrome associated with Horner's syndrome caused by ruptured cervical epidural arteriovenous malformation.

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동정맥 기형의 증례보고 (Arteriovenous Malformation : A Case Report)

  • 정연화
    • 치과방사선
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    • 제29권2호
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    • pp.561-569
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    • 1999
  • The present case illustrates an arteriovenous malformation of the cheek in a 25-year-old male. The clinical presentation. radiographic findings, differential diagnosis, treatment and histopathologic description are presented. Diagnosis of the lesion was confirmed by angiography, and the lesion was treated by angiographically controlled vascular embolization followed by complete surgical excision.

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Multiple Spinal Intramedullary Cavernous Malformation with Multiple Intracranial Involvement

  • Cho, Jae-Ik;Cho, Young-Dae;Kim, Young-Don
    • Journal of Korean Neurosurgical Society
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    • 제42권1호
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    • pp.64-66
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    • 2007
  • Intraspinal cavernous malformation (CM) accounts for 5% to 16% of all spinal vascular abnormalities. Multiple spinal cord CMs are very rare and only a few cases have been described. We report a patient presented with right chest paresthesia and seizure, and diagnosed as multiple spinal intramedullary CM and intracranial involvement.

선천성 낭포성 선양기종: 1례 보고 (Congenital Cystic Adenomatoid Malformation of The Lung - A Case Report -)

  • 김명인
    • Journal of Chest Surgery
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    • 제24권8호
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    • pp.819-823
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    • 1991
  • The congenital cystic adenomatoid malformation of the lung is a rare disease, and is one of the most common congenital lung diseases which require prompt surgical intervention. The prognosis depends on its tissue type, prompt diagnosis and surgical intervention. The lesion consists of enlarged, variable sized multiple cyst with overgrowth of terminal bronchioles, like hamartoma. This disease can be associated with other vascular anomalies or other congenital defect especially in type II lesion We recently experienced one case of congenital cystic adenomatoid malformation The patient was 2 months old infant who showed respiratory distress without associated anomaly. After right upper lobe lobectomy, the patient was recovered uneventfully.

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항문직장기형의 교정 (Repair of Anorectal Malformation)

  • 김성철
    • Advances in pediatric surgery
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    • 제12권1호
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    • pp.107-114
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    • 2006
  • Major advances in the management of anorectal malformations have been achieved during the last 20 years. Alberto Pena introduced the posterior sagittal anorectoplasty (PSARP) in 1982. He divided all the sphincteric muscles at the exact posterior midline and fully exposed the crucial anatomy. He was able to manage the associated fistula under direct vision with minimal urinary tract injury. The rectum could be placed in the middle of the sphincteric muscle complex. Even with PSARP by Alberto Pena himself, only 37.5% of all cases were considered totally continent. Anorectal malformation is still acontinuing challenge for the pediatric surgeons.

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직장항문기형의 해부와 분류 (Anatomy and Classification of Anorectal Malformation)

  • 한석주
    • Advances in pediatric surgery
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    • 제12권1호
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    • pp.91-98
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    • 2006
  • Artificial classification of anorectal malformation (ARM) is difficult because it is a spectrum of embryologic maldevelopment. However, the establishment of an ideal classification of ARM should be established in order to choose the adequate treatment and evaluate the prognosis. The classification system of ARM has followed the development of medicine, and the pediatric surgeons, who manage the ARM, should understand the change of this classification system.

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Treatment of Syringomyelia due to Chiari Type I Malformation with Syringo-Subarachnoid-Peritoneal Shunt

  • Akakin, Akin;Yilmaz, Baran;Eksi, Murat Sakir;Kilic, Turker
    • Journal of Korean Neurosurgical Society
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    • 제57권4호
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    • pp.311-313
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    • 2015
  • Chiari type I malformation is a tonsillar herniation more than 3 mm from the level of foramen magnum, with or without concurrent syringomyelia. Different surgical treatments have been developed for syringomyelia secondary to Chiari's malformations: craniovertebral decompression with or without plugging of the obex, syringo-subarachnoid, syringo-peritoneal, and theco-peritoneal shunt placement. Shunt placement procedures are useful for neurologically symptomatic large-sized syrinx. In this paper, authors define the first successful treatment of a patient with syringomyelia due to Chiari type I malformation using a pre-defined new technique of syringo-subarachnoid-peritoneal shunt with T-tube system.

선천성 낭종성 기형의 외과적치료;1례 보고 (Surgical Treatment of Congenital Cystic Adenomatoid Malformation; 1 Case Report)

  • 이정희;임진수;최형호
    • Journal of Chest Surgery
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    • 제26권4호
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    • pp.320-324
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    • 1993
  • Congenital cystic adenomatoid malformation [CCAM] of the lung is extremely rare. The patient was 10 year old female and had no specipic signs and symptoms except right lower chest pain for 5 days ago before admission.On simple chest X-ray and thoracic CT scan, about 9x8x8cm sized, heterogenous marginal enhanced multiseparated hypodence lesion with air fluid level and gas bubbles in posterior pleural space in right lower chest .The culture result of needle aspiration of cavity was apergillus flavus. Right lower lobectomy was carried out and the result of biopsy was congenital cystic adenomatoid malformation.

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