• 제목/요약/키워드: Congenital Abnormality

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Current Treatments for Congenital Aural Atresia

  • Lee, Min Young;Cho, Yang-Sun;Han, Gyu Cheol;Oh, Jeong-Hoon
    • 대한청각학회지
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    • 제24권4호
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    • pp.161-166
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    • 2020
  • Congenital aural atresia is an ear malformation evident at birth, involving various degrees of failed external ear canal development. A true external ear canal is desirable, as devices that replace the canal are inconvenient and expensive. Therefore, an optimal surgical technique is required. Here, we review useful preoperative and operative techniques. Surgical correction is often not the preferred treatment; the hearing outcome is no better than the outcomes afforded by bone-conduction devices, and surgery may be associated with recurrence or complications such as meatal stenosis. Preoperative evaluation and appropriate management are important. Several means of preventing meatal stenosis are discussed in this review.

Current Treatments for Congenital Aural Atresia

  • Lee, Min Young;Cho, Yang-Sun;Han, Gyu Cheol;Oh, Jeong-Hoon
    • Journal of Audiology & Otology
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    • 제24권4호
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    • pp.161-166
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    • 2020
  • Congenital aural atresia is an ear malformation evident at birth, involving various degrees of failed external ear canal development. A true external ear canal is desirable, as devices that replace the canal are inconvenient and expensive. Therefore, an optimal surgical technique is required. Here, we review useful preoperative and operative techniques. Surgical correction is often not the preferred treatment; the hearing outcome is no better than the outcomes afforded by bone-conduction devices, and surgery may be associated with recurrence or complications such as meatal stenosis. Preoperative evaluation and appropriate management are important. Several means of preventing meatal stenosis are discussed in this review.

개의 선천성 제4형 항문폐쇄증과 요도직장루의 외과 교정술 (Surgical Correction of Congenital Type IV Atresia Ani with a URethrorectal Fistula in a Dog)

  • 김종민;이재영;조기래;한태성;김소섭;한규보;김근형;최석화
    • 한국임상수의학회지
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    • 제22권4호
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    • pp.404-407
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    • 2005
  • A 4-week-old 0.5 kg male Shih Tzu with history of congenital abnormality, abnormality, was referred to Veterinary Teaching Hospital, Chungbuk National University for further evaluation and treatment. During physical examination, the dog revealed mild depression and dyschezia. In plain radiographs, a digital thermometer put in the anus and grasped blind end of the rectum. In contrast radiographs, a urethrorectal fistula was confirmed. Urine specimens were collected with cystocentesis. Bacteria of the urine were detected using an auto microorganism analyzer. According to history taking, physical examination, radiographic signs and urinalysis, it was diagnosed as type IV atresia ani with a urethrorectal fistula. The dog was treated by fistulectomy and anoplasty, and discharged with instruction. Three days after operation, mild dehiscence was appeared. Wound was left to heal by second intention. During the follow-up of eight weeks, wound showed it to be healed and defecation was normal.

성인 무증상 폐동맥 슬링 (An adult asymptomatic pulmonary artery sling)

  • 정한희;백주열;이원익;장지혜;정민영;우기현;박성일;김일규
    • Journal of Yeungnam Medical Science
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    • 제31권2호
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    • pp.109-112
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    • 2014
  • A pulmonary artery sling is a very rare congenital abnormality in which the left pulmonary artery rises from the posterior surface of the right pulmonary artery and then passes between the trachea and the esophagus, causing tracheal compression. It is associated with tracheo-bronchial abnormalities (50%) and cardiovascular abnormalities (30%). It may produce respiratory symptoms through the airway compression of the abnormal left pulmonary artery and congenital abnormalities associated with it. Because most (90%) pulmonary artery sling patients present symptoms during infancy, their condition is often diagnosed in the first year of life. However, a pulmonary artery sling is occasionally found in adults. It is usually asymptomatic and found incidentally. This is a very rare case of an asymptomatic pulmonary artery sling in an adult. A 38-year-old man presented symptoms of mild exertional dyspnea. His spiral computed tomography showed a pulmonary artery sling. He was discharged without specific treatment because his symptoms improved without specific treatment and might not have been associated with a pulmonary artery sling. We report an adult case of an asymptomatic pulmonary artery sling diagnosed via spiral computed tomography, accompanied by a literature review.

Case of mucinous adenocarcinoma of the lung associated with congenital pulmonary airway malformation in a neonate

  • Koh, Juneyoug;Jung, Euiseok;Jang, Se Jin;Kim, Dong Kwan;Lee, Byong Sop;Kim, Ki-Soo;Kim, Ellen Ai-Rhan
    • Clinical and Experimental Pediatrics
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    • 제61권1호
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    • pp.30-34
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    • 2018
  • Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation, is a rare developmental lung abnormality associated with rhabdomyosarcoma, pleuropulmonary blastoma, and mucinous adenocarcinoma of the lung. We report an unusual case of a 10-day-old male newborn with a left lower lobe pulmonary cyst who underwent lobectomy, which revealed type II CPAM complicated by multifocal mucinous adenocarcinoma. KRAS sequencing revealed a somatic mutation in Codon12 ($GGT{\rightarrow}GAT$), suggesting the development of a mucinous adenocarcinoma in the background of mucinous metaplasia. Mucinous adenocarcinoma is the most common lung tumor associated with CPAM, but it generally occurs in older children and adults. Further, all cases in the literature are of type I CPAM. This case in a neonate indicates that malignant transformation can occur very early in type II CPAM.

Kartagener 증후군을 동반한 Immotile Cilia Syndrome 의 외과적 치험 1례 (Surgical Treatment of Immotile Cilia Syndrome Associated with Kartagener`s Syndrome (Report of one case))

  • 김주현;박승일
    • Journal of Chest Surgery
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    • 제21권2호
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    • pp.383-388
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    • 1988
  • Immotile cilia syndrome is a congenital structural abnormality of cilia. The structural abnormality is lack of dynein arm or defective radial spoke or microtubular transposition. In this syndrome, ciliary movement is completely absent or dyskinetic and half of this syndrome shows Kartagener`s triad. We report a 13-year-old girl who had immotile cilia syndrome with Kartagener`s triad. She had been suffering from frequent respiratory infection, hemoptysis, large amount of sputum, and sinusitis. Bronchography revealed tubular bronchiectasis in right lower lobe and that lobe was resected for treatment of bronchiectasis. Histological examination of resected bronchus showed chronic bronchiectasis and electronmicroscopically complete lack of both inner and outer dynein arms. Hospital course was uneventful and symptoms were much improved.

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유아 엽성 폐기종 -1례 보고- (Infantile Lobar Emphysema -A Case Report-)

  • 신재승
    • Journal of Chest Surgery
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    • 제27권11호
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    • pp.965-969
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    • 1994
  • Infantile lobar emphysema is a pulmonary hyperinflation state that has the clinical features of an air block syndrome characterized by bronchial cartilaginous abnormalities or unknown origin. Left upper lobe was affected in most of the reported infantile lobar emphysema. Infantile lobar emphyema is divided into two categories. e.g., congenital and acquired. We have experienced a case of left lower lobe involved infantile lobar emphysema which had undergone left pneumonectomy. She had progressive signs of tension accompanied by mediastinal displacement, ventilatory and circulatory failure in infant period. Because of the combined left upper lobe hypoplasia, left pneumonectomy was performed. And there was no cartiliginous abnormality in pathologic finding. This is the first domestic case which was affected in the lower lobe and successful surgical repaired.

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Congenital Defect of the Posterior Arch of Cervical Spine : Report of Three Cases and Review of the Current Literature

  • Song, Kyo-Chang;Cho, Kyoung-Suok;Lee, Sang-Bok
    • Journal of Korean Neurosurgical Society
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    • 제48권3호
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    • pp.294-297
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    • 2010
  • Abnormalities of the posterior arch, including congenitally absent cervical pedicle and cervical spondylolysis, are rare entities that are usually found incidentally on neck radiographs. It is important to recognize these characteristic radiological features because their radiographic appearance may cause them to be confused with more serious entities such as fractures, locked facets, and tumor-induced bony erosions. Also, it is important to distinguish these abnormalities from similar pathologies to prevent the use of inappropriate treatment We report the relevant clinical and radiological findings seen in three cases of posterior arch defect after trauma with review of pertinent literature.

Newborn English Bulldogs with Multiple Malformation Syndrome and Lethal Characters

  • Jang, Si-Jung;Kim, Minkyung;Lee, Hyeon-Jeong;You, Young-Sung;Lee, Jaehoon;Lee, Sung-Lim
    • 한국동물생명공학회지
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    • 제34권3호
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    • pp.253-258
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    • 2019
  • A female English bulldog was gave birth two neonates by cesarean section on the sixty one days after mating, but both neonates were died soon after birth. The bodies of neonates were diagnosed using radiography, ultrasonography, computed tomography and necropsy immediately after death. Both neonates had caudal regression syndrome, butterfly vertebra, hydrocephalus, umbilical hernia, cleft palate and bow-legged hind-limb. At necropsy, neonates had mild fetal anasarca, cleft lip and the skull was remained non-union. At thoracic cavity, only three ribs and thoracic spines were existed and patent ductus arteriosus was found. At abnormal cavity, the renal ectopia was found with abnormal morphology. In the present case, those English bulldog neonates with multiple congenital malformation syndromes seriously suffered vertebral column anomalies and that may induced by neural tube defects in during embryonic period. To prevent congenital malformation occurring in English bulldog, further in depth studies are needed for the breed specific genetic diversity and for the reason of behind genetic abnormality in these breed.