• Title/Summary/Keyword: 선천성 기형

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A CASE REPORT OF NEUROCRISTOPATHY THAT SHOWS OBLIQUE FACIAL CLEFT, MAXILLARY DUPLICATION AND OTHER FACIAL MALFORMATIONS (사선안면열, 상악돌기 중복 등 복합 기형을 유발한 신경능병변 환자의 치험례)

  • Ryu, Dong-Mok;Lee, Sang-Chull;Kim, Yeo-Gab;Lee, Baek-Soo;Choi, You-Sung
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.21 no.4
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    • pp.407-413
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    • 1999
  • Maxillary duplication is a kind of proliferative neurocristopathy and considered to arise from bifurcation of neural crest elements soon after migration into mandibular arch. Sometimes this malformation is accompanied with oblique facial cleft. Usually this type of maxillofacial malformation requires multiple surgical intervention and the results are far from ideal. It became more troublesome if it had not been properly corrected on time, because secondary deformities could be developed from growth and development of abnormal tissues. This is a case of a 25-year-old-female patient who showed severe facial asymmetry thought to secondary deformity of maxillary duplication and masticatory disturbance due to multiple supernumerary teeth on posterior part right maxilla. We successully treated these deformities through four times of surgery of bone resection, orthodontic treatment, zygomatic and orbital reconstruction, orthognathic surgery and scar revision... ect. So we reported this rare case with review of literatures.

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Two Cases of Fourth Branchial Anomaly: Pyriform Sinus Fistula Treated by Chemical Cauterization (제 4 새낭 기형 2 례: 화학소작에 의한 이상동누공 치험)

  • 김광현;신진성;성명훈
    • Proceedings of the KOR-BRONCHOESO Conference
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    • 1993.05a
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    • pp.109-109
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    • 1993
  • Fourth branchial pouch anomaly is an extremely rare condition involving the pharyngeal apparatus with only about 30 cases having been reported in the last 20 years. It almost invariably occurs on the left side and typically presents with repeated episodes of neck swelling, recurrent thyroiditis, or repeated bouts of retropharyngeal abscess. This is a presentation of 2 patient with pyriform sinus fistula, presumably of fourth branchial origin, which, were successfully closed with 10ole trichloroacetic acid chemical cautery. Details of the cases along with the summary of embryology and fourth branchial anomaly are given.

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Classification of Congenital Urinary Tract Anomalies Diagnosed by Antenatal Ultrasonogram (산전 초음파로 발견된 선천성 요로계 기형의 분류)

  • Choi Jin-Ho;Hahn Hye-Won;Won Hye-Sung;Kim Kun-Suk;Yoon Chong-Hyun;Park Young-Seo
    • Childhood Kidney Diseases
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    • v.6 no.2
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    • pp.227-236
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    • 2002
  • Purpose : A study was done to assess the incidence and classification of congenital urinary tract anomalies detected by antenatal ultrasonogram. Methods : We reviewed 558 cases of urinary tract anomaly which were detected by antenatal ultrasonogram and postnatally confirmed between June 1989 and May 2002. We investigated the incidence and classified congenital urinary tract anomalies by review of medical records, antenatal and postnatal radiologic studies retrospectively. Results : In 558 cases of congenital urinary tract anomalies, 292 cases of hydronephrosis were found and the most common. Another anomalies were composed of 65 cases of multicystic dysplastic kidney, 32 cases of hydroureteronephrosis, 31 cases of duplication of kidney, 25 cases of renal agenesis, 21 cases of simple renal cyst, 20 cases of polycystic disease, 13 cases of ureterocele, 11 cases of renal hypoplasia, 10 cases of horseshoe kidney, 9 cases of vesicoureteral reflux, 8 cases of posterior urethral valve, 7 cases of bladder diverticulum, 6 casts of megaureter, 5 cases of ectopia, 2 cases of megacystis, and 1 case of medullary cystic disease. In 82 of the 558 cases, there were two or more combined urinary tract anomalies. Associated diseases other than urinary tract were observed in 13 cases, of which the congenital heart disease was the most common. Conclusion : The congenital urinary tract anomaly is frequently found and diverse during the antenatal ultrasonography. The multicenter study is needed to investigate precise incidence and distribution of each anomalies in general population.

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Coronary Artery Transfer for Anomalous Origin of Left Coronary Artery from Right Coronary Sinus (좌 관상동맥 이상기시의 수술적 치료)

  • 이준완;이재원;김종우
    • Journal of Chest Surgery
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    • v.36 no.7
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    • pp.514-517
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    • 2003
  • Anomalous aortic origin of left coronary artery is a rare congenital coronary anomaly that can cause clinical manifestations such as ischemic chest pain, arrhythmic syncope or even sudden cardiac death. We describe a case of anomalous aortic origin of left main coronary artery presented as a cardiogenic shock which was successfully treated by coronary artery transfer.

Sprengel's deformity associated with a de novo balanced translocation involving chromosome 3 and 17 (선천성 고위 견갑골을 동반한 3번과 17번 염색체의 균형전좌 1례)

  • Jung, On;Lee, Jung-Hyun;Chun, Chung-Sik
    • Clinical and Experimental Pediatrics
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    • v.50 no.3
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    • pp.311-315
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    • 2007
  • This is the first case of a de novo balanced translocation 46, XY, t(3;17)(p12.2;q25) associated with multiple musculoskeletal abnormalities, including Sprengel's deformity (congenital undescended scapula to be reported). This translocation has not been described previously with this congenital anomaly in Korea.