• Korean Journal of Radiology
• /
• 제22권9호
• /
• pp.1441-1450
• /
• 2021

Anomalous origin of the coronary artery from the pulmonary artery is a rare and potentially fatal congenital heart defect. Up to 90% of infants with an anomaly involving the left coronary artery die within the first year of life if left untreated. Patients who survive beyond infancy are at risk of sudden cardiac death. Cardiac CT and MRI are increasingly being used for the accurate diagnosis of this anomaly for prompt surgical restoration of the dual coronary artery system. Moreover, life-long imaging surveillance after surgery is necessary for these patients. In this pictorial review, multimodal cardiac imaging findings of this rare and potentially fatal coronary artery anomaly are comprehensively discussed, and representative images are provided to facilitate the understanding of this anomaly.

• Journal of Chest Surgery
• /
• 제35권1호
• /
• pp.68-72
• /
• 2002

선천성 기관지식도루는 매우 드문 질환으로 진단이 늦어질 경우 치명적인 격과를 초래할 수 있다. 이러한 기형은 호흡기 감염, 음식섭취나 물을 마실 때의 발작적 기침, 혈담 등의 증상을 나타낼 수 있다. 수술적 절제는 거의 모든 환자에서 근치적 치료이디-. 본 병원에서는 만성 농흉으로 오진된 제 1 형 선천성 기관지 식도루를 문헌 고찰과 함께 보고하는 바이다.

• Journal of Chest Surgery
• /
• 제18권1호
• /
• pp.24-35
• /
• 1985

Between January 1980 and July 1984, 321 cases of open heart surgery for infants or small children under 10Kg of body weight were performed, which occupied the great part of total open heart surgery done in the same period. The mean age was 16.58.7 months [ranging from 2 days to 51 months], and the mean body weight was 7.8a1.8Kg [from 2.8 to 10Kg] which was below the third percentile compared with the mean age. The technique of deep hypothermia with total circulatory arrest, which contributed to great improvement in operative condition, was used increasingly and widespreadly in this period. For each anomaly, the number of patients and operative deaths were as follows: VSD, 11 of 184 [6.0%]; TOF, 8 of 47 [17.8%]; TGA, 13 of 30 [43.3%]; ASD, none of 9; TAPVR, 1 of 8 [12.7%]; C-ECD, 3 of 6 [50%]; Tricuspid Atresia, 4 of 5 [80%]; Pulmonary Atresia, 2 of 4 [50%]; Congenital Mitral Anomaly, 1 of 3 [33.3%]; P-ECD, none of 3

• Journal of Chest Surgery
• /
• 제12권3호
• /
• pp.247-258
• /
• 1979

Two hundred and six cases of open heart surgery were done with over all mortality of 8.3% during 1978 in this Department. There were 130 congenital and 76 acquired cases. In congenital anomaly 58 cases were acyanotic and 72 cyanotic, which showed 3.4% and 12.5% of operative mortality respectively. Thirty-six cases of ventricular septal defect and 10 atrial septal defect were repaired without mortality but in 60 cases of tetralogy of Fallot it was 10%. In 76 cases of acquired lesions 74 were valvular cases. There were 75 cases of valve replacement with over all operative mortality of 8%. In 75-valve replacement one case of Ebstein anomaly was found. There were 62 cases of single valve and 13 double valve replacement. In every case tissue valve was utilized and coumadin and Persantin 75 were administered as anticoagulant treatment for one year.

• 한국임상수의학회지
• /
• 제35권6호
• /
• pp.279-281
• /
• 2018

This report describes a case of newly recognized congenital $M{\ddot{u}}llerian$ duct anomaly in a bitch. A fourteen-year-old, mixed breed bitch was presented with mild hematuria and mammary gland tumor. Complementary examinations were performed and abdominal ultrasonography revealed distended uterine horns and uterine tumor-like mass around the right ovarian area. Macroscopic examination showed the mass to be on the level of insertion of the right round ligament. The mass was cystic in nature, and had no communication with the uterine cavity. Histopathologically, the mass was identified as an accessory cavity lined by functional endometrium, which confirmed the diagnosis of accessory and cavitated uterine mass.

• Journal of Korean Neurosurgical Society
• /
• 제40권1호
• /
• pp.44-46
• /
• 2006

Although congenital anomalies of the atlas have been well-documented, atlas anomalies of clefts and aplasia are rare. Anterior and posterior midline clefts of the atlas have been reported separately in some series. However, combined congenital anterior and posterior midline clefts of the atlas are reported rarely. Hence, we report a very rare case of combined congenital anterior and posterior midline clefts of the atlas associated with asymptomatic lateral atlantoaxial subluxation.

• Archives of Plastic Surgery
• /
• 제47권3호
• /
• pp.272-276
• /
• 2020

Anorectal malformation or imperforate anus is a congenital anomaly of rectum and anus. Mullerian duct anomalies are abnormal development of uterus, cervix, and vagina. Imperforate anus with double uterus is extremely rare and cannot explain by normal embryologic development. Moreover, guideline in treatment is inconclusive. We report an extremely rare case of a young adult female who presented with recurrent pelvic inflammatory disease caused by rectovaginal fistula in congenital imperforate anus and didelphys uterus, and successfully neoanal reconstruction with gracilis muscle flap. Aims for treatment are closed rectovaginal fistula, and anal sphincter reconstruction. To our best knowledge, the imperforate anus with double uterus is extremely rare anomaly. Furthermore, successfully anal sphincter reconstruction with functional gracilis muscle in the imperforate anus with double uterus has never been reported in English literature.

• Maxillofacial Plastic and Reconstructive Surgery
• /
• 제38권
• /
• pp.43.1-43.4
• /
• 2016

Background: Supernumerary nostril is a congenital anomaly that contains additional nostril with or without accessory cartilage. These rare congenital nasal deformities result from embryological defects. Since 1906, Lindsay (Trans Pathol Soc Lond. 57:329-330, 1906) has published the first research of bilateral supernumerary nostrils, and only 34 cases have been reported so far in the English literature. Case presentation: A 1-year-old female baby was brought to our department group for the treatment of an accessory opening above the left nostril which had been presented since her birth. Medical history was non-specific and her birth was normal. The size of a supernumerary nostril was about 0.2 cm diameter and connected to the left nostril. The right one was normal. Minimal procedure was operated for the anomaly. After 1 year, rhinoplasty was performed for the nostril asymmetry. Conclusions: At 1 year follow-up, the functional and cosmetic result was satisfactory. In this case, it is important that we have early preoperative diagnosis. Also, it is desirable that we should perform a corrective surgery as soon as possible for the patient's psychosocial growth.

• Journal of Korean Neurosurgical Society
• /
• 제46권6호
• /
• pp.522-527
• /
• 2009

Objective : Atlantal arch defects are rare. Few cadaveric and imaging studies have been reported on the variations of such anomalies. Our goal in this study was to examine the incidence and review the clinical implications of this anomaly. Methods : A retrospective review of 1,153 neck or cervical spine computed tomography (CT) scans was performed to identify patients with atlantal arch defects. Neck CT scans were performed in 650 patients and cervical spine CT scans were performed in 503 patients. Posterior arch defects of the atlas were grouped in accordance with the classification of Currarino et al. In patients exhibiting this anomaly, special attention was given to defining associated anomalies and neurological findings. Results : Atlantal arch defects were found in 11 (11/1153, 0.95%) of the 1,153 patients. The type A posterior arch defect was found in nine patients and the type B posterior arch defect was found in two patients. No type C, D, or E defects were observed. One patient with a type A posterior arch defect had an anterior atlantal-arch midline cleft (1/1153, 0.087%). Associated cervical spine anomalies observed included one $C_{6-7}$ fusion and two atlantal assimilations. None of the reviewed patients had neurological deficits because of atlantal arch anomalies. Conclusion : Most congenital anomalies of the atlantal arch are found incidentally during investigation of neck mass, neck pain, radiculopathy, and after trauma.

• 대한영상의학회지
• /
• 제81권6호
• /
• pp.1511-1516
• /
• 2020

허리근뒤 이상 온엉덩동맥은 엉덩허리 혈관 이상 중에서도 굉장히 희귀한 선천적 이상이다. 51세 여성이 응급실에 점점 심해지는 오른쪽 하지 통증과 쇠약으로 내원하였다. 하지 CT 혈관조영술에서 하지 동맥들에 협착 소견 없었으며 이 검사 중에 오른쪽 허리근뒤 온엉덩동맥을 우연히 발견하였다. 우리는 희귀한 허리근뒤 이상 온엉덩동맥 증례를 보고하자고 한다. 외과의사와 임상의는 이 선천적 이상을 알고 있어야 골반 쪽이나 기타 정형외과적 수술 시 발생할 수 있는 심각한 합병증을 예방할 수 있다.