• Title/Summary/Keyword: Congenital anomaly

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The Evaluation of Donor Site after Transfer of Free Osseous and Osteocutaneous Flap of Fibula (유리 생비골 및 생비골 피부편 이식 후 공여부의 평가)

  • Lee, Kwang-Suk;Han, Seung-Bum;Hwang, In-Churl;Song, Hyung-Suk
    • Archives of Reconstructive Microsurgery
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    • v.10 no.2
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    • pp.75-80
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    • 2001
  • Purpose : We have evaluated the morbidity of donor site after transfer of free fibular osseous and osteocutaneous flap to defect site of bone and soft tissue due to chronic osteomyelitis of long tubular bone, open fracture with bony defect, bone or soft tissue tumor and congenital anomaly. Materials and methods : The 54 cases of 79 cases to be carried out from May, 1982 to May, 2001 which could be followed up were reviewed. There were forty nine in male and five in female. The mean age was 35(4 to 66)years old and mean follow up period is 21.3 month(12 to 72). We have retrospectively analyzed the various postoperative complications such as compartment syndrome, donor site infection, skin defect, hypesthesia, hammer toes, ankle instability and activity of daily living by help of questionnaire, telephone, physical examination, follow up x-ray study and chart. Results : In the total 54 cases the medication period for pain control after operation were classified into three groups under 2 weeks(49 cases), from 2 weeks to 6 weeks(3 cases) and over 6 weeks(2 cases). The postoperative morbidity were occurred in total 12 cases(compartment syndrome: 0, infection : 2, skin defect: 1, hypesthesia: 5, hammer toe: 2 ankle pain: 2 discomfort in activity of daily living: 0), and also the morbidity rates of donor site were 23.5% in osseous flap and 21.6% in osteocutaneous flap were occurred. There was no statistical significonce in morbidity between osseous and osteocutaneous free fibular flap transfer(P>0.05). Discussion : In general the morbidity of free fibular flap transfer was relatively high but it did not have any effect on daily activity of living. We think that the meticulous operation technique, detailed wound care and early range of motion exercise will reduce the morbidity of donor site of flap.

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Retroperitoneal Pulmonary Sequestration in a Neonate - A case report - (신생아에서 발견된 후복막강 폐격리증 - 1예 보고 -)

  • Lee, Hyung-Chae;Cho, Kwang-Hyun;Choi, Kwang-Ho;Yoon, Young-Chul;Lee, Yang-Haeng;Hwang, Youn-Ho
    • Journal of Chest Surgery
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    • v.42 no.3
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    • pp.364-367
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    • 2009
  • Retroperitoneal pulmonary sequestration is an extremely rare congenital malformation. It is more frequently diagnosed in the antenatal period due to routine ultrasonic examinations that are conducted for a fetus or during the first 6 months of life, although retroperitoneal pulmonary sequestration is incidentally discovered in adults on rare occasions. Because the location and radiological findings of retroperitoneal pulmonary sequestration are very similar to those of another retroperitoneal masses, retroperitoneal pulmonary sequestration, although they are very rare, should be included in the differential diagnosis of a retroperitoneal suprarenal mass. Although fine needle aspiration may be considered as an aid for making the preoperative diagnosis, surgery remains the treatment of choice for symptomatic lesions and this surgery is associated with excellent results and a good prognosis.

Congenital Polydactyly of the Foot (선천성 족부 다지증)

  • Hwang, Kuhn-Sung;Kim, Tae-Seung;Sung, Il-Hoon;Park, Jun-Sic
    • Journal of Korean Foot and Ankle Society
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    • v.5 no.2
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    • pp.120-128
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    • 2001
  • Purpose: This study was undertaken to review the precise morphological patter'ns of polydactyly of the foot and to evaluate outcomes of surgical interventions. Materials and Methods: The records of patients who had polydactyly of foot from 1983 to 2000 were reviewed retrospectively. 29 duplicated toes(24 patients) were analyzed. All cases were evaluated and classified with Temtamy and McKusick's axial pattern, Watanabe's morphologic pattern and level of duplication. Clinical evaluation was performed according to Phelps and Grogan's. Mean age at surgery was 5.7 years(range: 10 months$\sim21$ years). All the patients were subsequently evaluated during mean 17 months(range: 13 months-$\sim5$ years). Results: Preaxial polydactyly was seen in 3 cases, central polydactyly in 3 cases, postaxial polydactyly in 22 cases and remaining 1 case was multiple my involvement. Only 2 patients have familial history and the most common associated anomaly was hand deformity(20.8%). Clinical results were excellent in 14 cases, good in 5 cases, poor in one. 17 patients(70%) were operated before 6 years olds and they had better results than those of who were operated after 6 years olds. Conclusion: The Method of surgical correction for the polydactyly should be individualized by its morphological pattern because the purpose of operation was to give comfort in wearing shoes and patient's psychological relief. This study showed that polydactyly of the foot could be corrected surgically with good results in most cases, and the better results would be achieved if the operation is performed before preschool age according to its individualized pattern of duplication.

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A rare type of single coronary artery with right coronary artery originating from the left circumflex artery in a child

  • Kim, Jong Min;Lee, Ok Jeong;Kang, I-Seok;Huh, June;Song, Jinyoung;Kim, Geena
    • Clinical and Experimental Pediatrics
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    • v.58 no.1
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    • pp.37-40
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    • 2015
  • The presence of a single coronary artery is a rare congenital anomaly; such patients often present with severe myocardial ischemia. We experienced the case of a 13-year-old girl with the right coronary artery originating from the left circumflex artery. She visited our Emergency Department owing to severe chest pain; her cardiac enzyme levels were elevated, but her initial electrocardiogram (ECG) was normal. Echocardiography showed normal anatomy and normal regional wall motion. When she presented with recurrent chest pain on admission, the ECG showed significant ST-segment elevation in the left precordial leads and inferior leads with ST-segment depression in aVR lead, suggesting myocardial ischemia, and her cardiac enzyme levels were also elevated. We performed coronary angiography that showed a single right coronary artery originating from the left circumflex artery without stenosis. We confirmed the presence of a single coronary artery using coronary computed tomography. In addition, the treadmill test that was performed showed normal results. She was discharged from the hospital without any medications but with a recommendation of a regular followup.

A Case of Oligomeganephronia of the Solitary Kidney, Presented with Isolated Proteinuria (단독 단백뇨로 발현된 단일신의 oligomeganephronia 1례)

  • Park Soeun;Lee Jung Won;Cho Su Jin;Yoo Eun Sun;Kim Hae Soon;Sung Soon Hee;Lee Seung Joo
    • Childhood Kidney Diseases
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    • v.6 no.1
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    • pp.92-96
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    • 2002
  • Oligomeganephronia is rare congenital anomaly characterized by striking reduction of the number of nephrons, which are markedly hypertrophied in renal hypoplasia. Since the first description of oligomeganephronia in bilateral renal hypoplasia in 1962, dozens of cases were reported. Van Acker reported the first case of oligomeganephronia developed in the solitary kidney and 8 cases were searched in the literature. We report a case of oligomeganephronia in the solitary kidney in 13 years old boy, presented with isolated proteinuria as a first case in Korea. (J Korean Soc Pediatr Nephrol 2002 ; 6:92-6)

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Terminology of Developmental Abnormalities in Common Laboratory Animals (실험동물의 발생이상 용어집)

  • Kim, Jong-Choon;Yang, Young-Su;Ahn, Tai-Hwan;Kim, Sung-Ho;Chung, Soo-Youn;Rhee, Gyu-Seek;Chung, Na-Young;Chung, Moon-Koo
    • Toxicological Research
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    • v.22 no.3
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    • pp.157-220
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    • 2006
  • This paper presents the first version of a Korean glossary of terms for structural developmental abnormalities in common laboratory animals, mainly rats, mice and rabbits. This is a translation of the glossary entitled Terminology and Developmental Abnormalities in Common Laboratory Mammals that was edited by the International Federation of Teratology Societies(IFTS) Committee on International Harmonization of Nomenclature in Developmental Toxicology. The purpose of the Korean glossary is to provide a common vocabulary that will reduce confusion and ambiguity in the description of developmental effects, particularly in submissions to regulatory agencies worldwide. The glossary contains a primary term or phrase, a definition of the abnormality, and notes, where appropriate. Selected synonyms or related terms, which reflect a similar or closely related concept, are noted. Non-preferred terms are indicated where their usage may be incorrect. Modifying terms used repeatedly in the glossary(e.g., absent, branched) are listed in Appendix A, and syndrome names are generally excluded from the glossary, but are listed separately in Appendix B. The glossary is organized into broad sections for external, visceral, and skeletal observations, then subdivided into regions, structures, or organs in a general overall head to tail sequence. Numbering is sequential, and not in any regional or hierarchical order, Uses and misuses of the glossary are discussed. Updates of the Korean glossary are planned based on the comments received.

Clinical study on Renal Replacement Therapy for Acute Renal Failure following Cardiopulmonary Bypass (체외순환후 급성 심부전에 대한 신대체요법의 임삼적 검토)

  • 서경필
    • Journal of Chest Surgery
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    • v.25 no.3
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    • pp.232-239
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    • 1992
  • Acute renal failure is a well known serious complication following open heart surgery and is associated with a significant increase in morbidity and mortality rate. From 1984 to 1990, 33 patients who had acute renal failure following cardiopulmonary bypass received renal replacement therapy. PD[Peritonial dialysis] was employed in 11 patients and CAVH[continous arteriovenous hemofiltration] was employed in 22 patients. Their age ranged from 3 months to 64 years[mean 25.5$\pm$7.8 years]. The disease entities included congenital cardiac anomaly in 18, valvular heart disease in 15 and aorta disease in 2 cases. Low cardiac output was thought as a primary cause of ARF except two redo valve cases who showed severe Aemolysis k depressed renal function preoperatively. Mean serum BUN and creatinine level at the onset renal replacement therapy were 65$\pm$8 mg/dl and 3.5$\pm$0.4 mg/dl respectively, declining only after reaching peak level 7&10 days following the onset of therapy. Overall hospital mortality was 72.7%[24/33]; 81%[9/11] in PD group and 68.2% [15/22] in CAVH group respectively. The primary cause of death was low cardiac output & hemodynamic depression in all the cases. The fatal complications included multiorgan failure in 7, disseminated intravascular coagulation and sepsis in 6, neurologic damage in 4 and mediastinitis in 3 cases. No measurable differences were observed between CAVH and PD group upon consequence of acute renal failure and disease per se. The age at operation, BUN/Cr level at the onset of bypass and highest BUN/Cr level and the consequence of low output status were regarded as important risk factors, determining outcome of ARF and success of renal replacement therapy. Thus, we concluded that althoght the prognosis is largely determined by severity of low cardiac output status and other organ complication, early institution of renal replacement therapy with other intensive supportive measures could improve salvage rate in established ARF patients following CPB.

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A Clinical Study on SGOT,SGPT and LDH in the Patients of Open Heart Operation with Cardiopulmonary Bypass (개심술환자의 혈청효소치 변동에 관한 연구)

  • Park, Kang-Sik;Jee, Heng-Ok;Kim, Kun-Ho
    • Journal of Chest Surgery
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    • v.15 no.1
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    • pp.46-52
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    • 1982
  • Activities of serum glutamic pyruvic transaminase [SGPT], serum glutamic oxaloacetic transaminase [SGOT] and serum lactic dehydrogenase [LDH] were determined during and after surgery In a total of 18 patients with heart diseases Including 8 cases of congenital heart anomaly and 10 cases of acquired valvular diseases who undergone open heart surgery under cardiopulmonary bypass. The results obtained are as follows: 1]The average value of the activity of SGPT before the surgery was $27.94{\pm}12.24$ units. The value was increased following the operation and reached to the maximum value of $43.83{\pm}37.02$ units 2 days after the operation, which was slightly deviated from the normal range. The enzyme activity returned to the normal range of $32.82{\pm}23.61$ units 4 days after the operation. 2]The average value of the activity of SGOT before the surgery was observed to be $30.5{\pm}11.29$ units. The value increased during the operation and reached to the maximum value of $95.56{\pm}43.38$ units. 2 days after the operation, the enzyme activity was gradually decreased thereafter and returned to the normal range 6 days after the operation. 3]The average value of the activity of serum LDH before the surgery was found to be $258.0{\pm}88.96$ units. The value was increased during the operation and reached to the maximum value of $930.55{\pm} 258.23$ units 2 days after the operation. The enzyme activity was gradually decreased thereafter and returned to normal range 7 days after the operation. 4]It was observed that the longer the time required for the extracorporeal circulation in the open heart surgery became, the greater the changed in activity of SGOT, SGPT and serum LDH were found.

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Corrected transposition of the great arteries: surgical treatment of associated anomalies (교정형 대혈관전위증동반된 심혈관기형의 수술요)

  • Kim, Gi-Bong;No, Jun-Ryang;Seo, Gyeong-Pil
    • Journal of Chest Surgery
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    • v.17 no.3
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    • pp.371-380
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    • 1984
  • Corrected transposition of the great arteries [C-TGA] is one of the rare congenital heart disease in which there is both a discordant atrioventricular relationship and transposition of the great vessels. With this arrangement, systemic venous blood passes through the right atrium into the morphologic left ventricle and out the pulmonary artery. Pulmonary venous blood returns to the left atrium, flows into the morphologic right ventricle and out the aorta. Thus, in the rare case when no additional cardiac anomaly is present, a hemodynamically normal heart exists. But more often they are symptomatic as a result of one or several of the commonly associated defects. This paper describes 13 patients who underwent repair of one or more cardiac anomalies associated with corrected transposition at SN UH, from June 1976 through June 1984. 1.8 were males and 5 females, with ages ranging from 3 years to 27 years. 2. Segmental anatomy was {S,L,L} in 12, or {I,D,D} in 1. 3.Associated anomalies were ventricular septal defect in 10, pulmonary outflow tract obstruction in 6, tricuspid insufficiency in. 4, atrial septal defect in 3, subaortic stenosis in 1, mitral insufficiency in 1, and patent ductus arteriosus in 1. 4.None had complete heart block preoperatively, and 3 developed complete heart block intraoperatively. But one of them recovered sinus rhythm on the postoperative 7th day spontaneously. 5.There were 3 cases of hospital morality. But there was no morality since Dec. 1980. 6.Patients with single ventricle, hypoplastic ventricle or those who had palliative surgery alone are not included in this review.

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Operative Correction of Total Left Anomalous Pulmonary Venous Return - A Report of one case - (좌측 총폐정맥 환류이상의 수술 교정 - 1례 보고 -)

  • 류한영
    • Journal of Chest Surgery
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    • v.23 no.5
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    • pp.962-967
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    • 1990
  • The anomalous pulmonary venous return of the entire left lung was an extremely rare congenital anomaly. The reported surgical experience with correction of this disorder was limited. The 3-year-old female patient underwent an operation upon the unilateral total anomalous pulmonary venous return from the left lung, in which the left superior pulmonary vein drained into innominate vein and the left inferior pulmonary vein into the coronary sinus, in Yeungnam University Hospital. The symptoms were nonspecific except frequent upper respiratory infection. Cyanosis was not seen. On auscultatory findings, a grade 2/6 systolic ejection murmur was audible over left second intercostal space of left sternal border and second heart sound had an increased pulmonary component which was widely splitted. The electrocardiogram demonstrated a right ventricular hypertrophy and right axis deviation and chest X-ray showed slightly increased pulmonary vascularity and bulged pulmonary conus. The echocardiogram demonstrated increased right atrial, ventricular, and pulmonary arterial dimension, and also secundum atrial septal defect and enlarged coronary sinus. The cardiac catheterization confirmed the left-to-right with a Qp/Qs of 2.0: 1 and oxygen step-up was seen in pulmonary artery, right ventricle, right atrium, and left innominate vein, and the catheter was not been introduced into the left pulmonary vein. A median sternotomy incision was done. Left superior pulmonary vein was drained to the innominate vein through anomalous vertical vein and the left inferior pulmonary vein drained to right atrium through the coronary sinus. The diversion of the left inferior pulmonary vein to posterior wall of left atrium was done after division in the proximity of coronary sinus. The anomalous vertical vein was diverted to base of left atrial auricle and then a atrial septal defect was sutured directly. The postoperative course was uneventful and she was discharged on the eleventh postoperative day. In the postoperative follow-up-2 months, she has been well without specific problems.

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