• 대한영상의학회지
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• 제84권2호
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• pp.489-497
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• 2023

두개내 경막 동정맥루는 모든 두개내 혈관 기형의 약 10%-15%를 차지하는 비정상적인 동정맥 단락이다. 대부분의 두개내 경막동정맥루는 단독형이며, 다른 부위에 여러 경막동정맥루가 있는 경우는 드물다. 여러 경막동정맥루에 대한 대부분의 증례 보고에서는 동기형 동정맥루를 기술하였고, 이시성 경막동정맥루에 대한 보고는 상대적으로 적다. 이에 저자들은 경막 동정맥루의 색전술 후 추적 영상에서 이시성 경막 동정맥루가 발견된 75세 여성의 증례를 보고 하고자 한다.

• Journal of Chest Surgery
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• 제29권12호
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• pp.1306-1315
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• 1996

본연구는 서울대학교병원 어린이병원 흉부외과에서 1992년 2월부터 1995년 12월까지 양방향성 체정맥-폐동맥단락술을 시행받은 환자 54례를 대상으로 하였다. 환아의 평균연령 및 체중은 각각 36.8+37.7개월, 8.0+3.0kg이 었다. 환아의 89%는 폰탄(Fontan)수술의 위험 인자를 2개이상 동반하고 있었으며 병원 사망율은 16.6%(9154)였다. 생존 환아를 대상으로 양방향성 체정맥-폐동맥 단락술전과 평균 16.3+14.3개월후의 혈역학 및 심혈관 조영술 검사 소견을 비교하였다. 동맥혈의 평균 산소포화도는 술전 71.9+10.1 %에서 79.8+8.5%로 향상되었으나(n:4), p< 0.05). 양방향성 체정 맥-폐동맥단락의 혈역학으 로 생존하는 환아들은 나이가 많을수록 동맥혈의 산소포화도가 낮은 경향을 보였다(n=22, R2=0.34, p=0.004). 환아들의 평균 폐동맥압은 31+17mrnHg에서 13.5+3.5mmHg로 유의한 감소를 보였으며 (n=22, p<0.05), 폐혈관저항 역시 감소하는 경향을 보였으나 자료의 개수가 적어 통계적으로 유의한 차이는 발견할 수 없었다. 폐동맥의 술전후 크기 변화에 관해서는 폐동맥의 절대 크기는 동측의 경우 유의한 증가가 있었으나(n: 14, p<0.05) 반대측의 경우 차 隔\ulcorner없었다(n=14 p<0.05). 그러나 환아의 체표면적을 고려한 폐동맥크기 변화는 동측이건 반대측이건 모두 현저한 감소를 보여 통계적으로 유의하였다(동측의 경우 16.8, 감소, n=14, p<0.05, 반대측의 경우 25.1% 감소, n= 14, p<0.05). 좌,우 폐동맥 단면적에 대한 폐동맥지수는 동기간 평균 9.3+13.8%의 감소를 보였으며 술후 추적 기간이 길면 길수록 폐동맥 진수의 % 감소가 커지는 경향을 보였다(n=24, R2=0.34, p=0.002). 저자등은 본 연구를 통하여 양방향성 체정맥-폐동맥 단락술이 단계적 폰탄 수술의 중단기 고식술로서 우수함을 입증하였다. 그러나 환아가 성장함에 따라 상대적으로 폐동맥 크기가 감소하며 추적 기간이 길면 길수록 그런 현상이 더욱 심화된다는 사실은 양방향성 체정맥-폐동맥 단락술후 가능하면 빠른시기에 완전 폰탄을 시행하는 것이 바람직하다는 가설을 뒷받침한다.

• Journal of Chest Surgery
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• 제40권8호
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• pp.569-573
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• 2007

기능성 단 심실, 폐동맥 폐쇄, 주 대동맥-폐동맥 측부 혈관, 총폐정맥 환류 이상이 동반된 우심방 이성체의 진단을 받은 여아가 청색증의 악화로 생후 3개월째 좌측 주 대동맥-폐동맥 측부 혈관 단일화 수술 및 좌측 변형 Blalock-Taussig 단락술을 시행하였다. 수술 후 흉부 x-ray 상 폐정맥 울혈의 소견을 보여 시행한 심장 초음파 검사 상 폐정맥 협착이 발견되어 총폐정맥 환류 이상에 대한 무 봉합 술식 및 우측 주 대동맥-폐동맥 측부 혈관에 대한 단일화 수술을 같이 시행하였다. 수술 후 저산소증으로 인한 심폐기 이탈 실패로 8일간 체외 막형 산화기로 보조하였으며, 전신-폐 단락술을 추가하면서 체외 막형 산화기를 이탈할 수 있었다. 환아는 장기간의 기관 삽관으로 유발된 기관 협착으로 기관지 절개를 한 상태에서 1차 수술 후 104일째 퇴원하였고, 산소 투여 없이 산소 포화도 80% 정도로 유지하면서 2개월째 외래 관찰 중이다.

• Journal of Chest Surgery
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• 제51권3호
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• pp.167-171
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• 2018

Background: Heart transplantation (HTx) can be a life-saving procedure for patients in whom single ventricle palliation or one-and-a-half ($1\text\tiny{1/2}$) ventricle repair has failed. However, the presence of a previous bidirectional cavopulmonary shunt (BCS) necessitates extensive pulmonary artery angioplasty, which may lead to worse outcomes. We sought to assess the post-HTx outcomes in patients with a previous BCS, and to assess the technical feasibility of leaving the BCS in place during HTx. Methods: From 1992 to 2017, 11 HTx were performed in patients failing from Fontan (n=7), BCS (n=3), or $1\text\tiny{1/2}$ ventricle (n=1) physiology at Asan Medical Center. The median age at HTx was 12.0 years (range, 3-24 years). Three patients (27.3%) underwent HTx without taking down the previous BCS. Results: No early mortality was observed. One patient died of acute rejection 3.5 years after HTx. The overall survival rate was 91% at 2 years. In the 3 patients without BCS take-down, the median anastomosis time was 65 minutes (range, 54-68 minutes), which was shorter than in the patients with BCS take-down (93 minutes; range, 62-128 minutes), while the postoperative central venous pressure (CVP) was comparable to the preoperative CVP. Conclusion: Transplantation can be successfully performed in patients with end-stage congenital heart disease after single ventricle palliation or $1\text\tiny{1/2}$ ventricle repair. Leaving the BCS in place during HTx may simplify the operative procedure without causing significant adverse outcomes.

• 한국산학기술학회논문지
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• 제19권6호
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• pp.275-279
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• 2018

뇌농양은 심각한 신경학적 후유증을 일으킬 수 있는 중추신경계 감염이다. 신경집중치료 발달에도 불구하고, 여전히 뇌농양은 특정 위험 환자에게 높은 사망률을 보이고 있다. 특히 세균성 뇌농양은 즉각 진단 및 적절한 항생제 치료가 필요한 응급 상황이다. 또한 드물게 뇌농양이 재발되는 경우도 있다. 본 연구에서는 두통으로 내원한 59세 남자환자를 대상으로 증례보고를 통해 동일 분야 연구에 활용하고자 자료분석을 하였다. 59세 남자가 두통을 주소로 내원하였고, 연속적으로 시행한 뇌자기공명영상과 뇌척수액 검사에서 우측 전두엽에 뇌농양을 확인하였고, 항생제 치료 및 수술적 치료 후 완치하였다. 퇴원 후 5개월 뒤 두통 및 경기 증상 있어 다시 촬영한 뇌자기공명영상에서 뇌농양의 재발이 확인되었다. 뇌농양 재발은 특정 조건, 즉, 농양 부위에 이물질이 남아 있거나 만성 부비동염, 동정맥루, 좌우션트 등이 있을 때 생길 수 있다.

• 대한두개안면성형외과학회지
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• 제13권1호
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• pp.54-56
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• 2012

Purpose: Dandy-Walker syndrome is a rare congenital brain malformation that occurs one in every 25,000-35,000 live births, mostly in females. It is characterized by cystic enlargement of the fourth ventricle, agenesis or hypogenesis of the cerebellar vermis and enlargement of the posterior fossa. In this report, the authors aimed to address a rare case of a 14-months-old female Dandy-Walker syndrome patient that is presented with submucous cleft palate. Methods: A 14-months-old female patient admitted to our outpatient clinic, via the department of pediatrics, with the complaints of nasal regurgitation, choking and breathing difficulties. She was diagnosed as Dandy-Walker syndrome by magnetic resonance imaging evaluation, at another hospital and underwent a shunt operation for the hydrocephalus continuing treatments. On physical examination, she had structural abnormality of bifid uvula, and palpable notch in the posterior surface of the hard palate. Her submucous cleft palate was corrected, which used a double opposing Z-plasty under general anesthesia. Results: In a follow-up period of 2 months, no complications, such as wound dehiscence, necrosis and infection occurred, which shows satisfactory results. She consulted with pediatric neurologists and physical therapists for further evaluation and management of the abnormalities in the central nervous system. Conclusion: Dandy-Walker syndrome patient with a cleft palate is a very rare case to find, which only a few cases are reported around the world. Authors would like to share this case of Dandy-Walker syndrome patient, with submucous cleft palate, who underwent a double opposing Z-plasty that shows satisfactory results.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제18권1호
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• pp.61-68
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• 1996

1. 첫 번째 증례에서 관상 절개를 이용한 부분골 절제술과 Le Fort씨 1급 골절단술을 시행한 결과, 골절단술을 시행한 부위에 원활한 골 치유가 일어났다. 2. 두 번째 증례에서 관상 절개를 이용하여 두개안면부에 광범위한 부분골 절제술을 시행하여 만족할만한 결과를 얻었으며, 병소 부위 말초 혈관 과다는 병소의 성장에 따른 생리적 변화로 추정된다.

• 대한핵의학회지
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• 제34권2호
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• pp.154-158
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• 2000

This is a case report of a 5-month-old male who was brought in to hospital for evaluation of jaundice from birth. The baby had a history of ileal atresia operated 2 days after birth. At the age of one month, Tc-99m DISIDA hepatobiliary scintigraphy was performed at other hospital and reported to show good hepatic uptake of the tracer but no uptake in the biliary tree, gall bladder, or intestine for 24 hours post injection. He was judged to have biliary atresia. However, subsequent exploratory laparotomy revealed that the hepatobiliary tree appeared intact and that there was a gall bladder. Additionally, the patient had central aorto-pulmonary shunt for the right ventricular septal defect with pulmonary stenosis of a peripheral type at the age of 4 months. The second hepatobiliary scintigraphy was performed on admission at the age of 5 months, showing a gall bladder but no intestinal uptake up to 24 hours. Retrospectively, the histological specimen of the liver obtained at the exploratory laparotomy was re-evaluated, and by the histological findings coupled with clinical data, arteriohepatic dysplasia (Alagille's syndrome) was diagnosed. In this report, we emphasize the diagnostic limitation of hepatobiliary scintigraphy and the importance of overall clinical and histologic evaluation in a case of Alagille's syndrome.

• Journal of Korean Neurosurgical Society
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• 제38권3호
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• pp.184-189
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• 2005

Objective : Hemangioblastomas are highly vascular and benign neoplasm of the central nervous system[CNS]. They can often be found as multiple lesions, as is commonly observed in von Hippel-Lindau[VHL] disease. The aim of this study is to determine the proper management for multiple hemangioblastomas. Methods : Since 1990, 78cases of hemangioblastoma have been encountered. Among these, 9cases were multiple hemangioblastomas that were treated with surgical resection with or without radiosurgery. The medical, radiological, surgical and histological records were reviewed retrospectively and analyzed statistically. Results : Nine patients presented with multiple hemangioblastomas and were diagnosed as VHL disease. The mean follow-up duration was 75.7months [$6.6{\sim}159.2months$] after the first surgical treatment. Three patients were treated with surgical resection alone and six patients were treated by both surgical resection and radiosurgery. Twenty-one surgical procedures [13 surgical resections and 8 radiosurgery] were performed. One patient required ventriculoperitoneal shunt and a posterior fossa decompressive craniectomy because of post-radiation brain swelling. Another patient refused additional treatment for the newly developed lesions after the successful treatment of initial lesions. The other patient who presented with numerous lesions in the whole brain and spine underwent cranio-spinal irradiation. Remaining patients showed good results. Conclusion : The surgical outcomes for the patients with a single lesion of the CNS hemangioblastoma are favorable. However. the treatment of multiple hemangioblastoma is more difficult, and should be treated by surgical resection and radiosurgery with careful consideration.

• Archives of Pharmacal Research
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• 제22권2호
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• pp.219-224
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• 1999

In our search for the anticonvulsant consitutent of Gastrodia elata repeated column chromatographies guided by activity assay led to isolation of an active compound, which was identified as gastrodin on the basis of spectral data. Brain succinic semialdehyde dehydrogenase (SSADH) was inactivated by preincubation with gastrodin in a time-dependent manner and the reaction was monitored by absorption and fluorescene spectroscopic methods. The inactivation followed pseudo-first-order kinetics with the second-rate order constant of $1.2{\times}10^{3} M^{-1} min^{-1}$. The time course of the reaction was significantly affected by the coenzyme NAD^{+}$, which affected complete protection against the loss of the catalytic activity, whereas substrate succinic semialdehyde failed to prevent the inactivation of the enzyme. It is postulated that the gastrodin is able to elevate the neurotransmitter GABA levels in central nervous system by inhibitory action on one of the GABA degradative enzymes, SSADH.