• Journal of Chest Surgery
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• 제15권3호
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• pp.325-330
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• 1982

A case of a 7 year old girl with rare congenital anomalies is reported. The anomalies as called Cantrell`s pentalogy is consisted of defect in supraumbilical abdominal wall, ventral diaphragm, adjacent pericardium, and lower sternum associated with cardiac malformation. Her cardiac lesion was muscular diverticulum of left ventricle. The diverticulum was resected and the other defects were repaired successfully.

• Journal of Chest Surgery
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• 제17권4호
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• pp.625-631
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• 1984

A modified Fontan procedure was performed on two patients with Univentricular heart. The first patient had UVH of right ventricular type with trabecular pouch and had various associated anomalies, such as common atrium, common atrioventricular valve and combined pulmonary stenosis. The second patient had UVH of left ventricular type with outlet chamber and the associated anomalies were atrial septal defect, tricuspid stenosis and combined pulmonary stenosis. Postoperative hemodynamic insufficiency, fluid retention and renal insufficiency were occurred in the first patient, but relieved with the aid of inotropics and vasodilators. We thought that the good postoperative course and surgical result were gained from the widely patent atriopulmonary anastomosis.

• Journal of Korean Neurosurgical Society
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• 제39권6호
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• pp.467-470
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• 2006

Aneurysmal ruptures associated with middle cerebral artery[MCA] anomalies, such as a duplicated MCA and an accessory MCA, are quite rare. The authors reviewed the clinical relevance and possible etiology of these anomalies.

• 대한소아치과학회지
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• 제51권1호
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• pp.66-79
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• 2024

This study aimed to assess the prevalence and distribution pattern of dental anomalies in the permanent teeth of patients with non-syndromic cleft lip with or without cleft palate. Additionally, it aimed to compare differences in dental anomalies between cleft and non-cleft areas, considering gender and cleft phenotype. Panoramic radiographs of 164 patients diagnosed with non-syndromic orofacial clefts were retrospectively analyzed by a single examiner to confirm dental anomalies. The dental anomalies investigated included tooth agenesis, supernumerary teeth, microdontia, rotation, ectopic eruption, and enamel hypoplasia. Cleft phenotypes were categorized into 7 types based on medical and dental records. A significantly higher prevalence of supernumerary teeth was observed in males than females within non-cleft areas (p = 0.017), with no significant differences in other dental anomalies. In non-cleft area, patients with cleft palate exhibited a high prevalence of tooth agenesis (p < 0.0001) and microdontia (p = 0.012) compared to other cleft phenotypes. Maxillary incisor rotation was closely associated with adjacent tooth agenesis in unilateral cleft lip and palate cases (p = 0.034). This study suggests that the additional subphenotype based on dental anomalies in patients with orofacial cleft may serve as applicable clinical markers.

• 자원환경지질
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• 제32권3호
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• pp.281-291
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• 1999

This study presents the gravity data with GPS survrying and the geophysical profiles at the Choogaryeong Rift Valley. And in determing geoid by GPS measurement, survey control points (SCP) whoch built by the Republic of Korean Army are used. Seventy nine SCP and the two triangulation stations are reviewd by GPS. Digital terain model is under for terrain analysis. The analyses of the gravity surveying with GPS are as follows. The low values of the negative Bouguer anomalies represent the high elevation terrain. The Bouguer anomalies show the decrrasing trend toward the eastern part of the study area. Characteristics of free-air anomalies are related with terrain elevation. The regional gravity anomalies decreas toward the eastern part of the study area. The trends of variations are associated with the thermotectonic and geologic structure beneath the Choogaryeong Rift Valley. The most parts of the study area represent negative residual gravity anomalies due to the low dencity of sedimentary cover in the Rift Valley. There are three valleys and four mountains in the direction of NE-SW or NNE-SSW which are structured by the geological features.

• Journal of Chest Surgery
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• 제17권2호
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• pp.177-183
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• 1984

From July 1975 to March 1984, 16 patients of complex congenital cardiovascular anomalies associated with left superior vena cava were seen at Han-Yang University Hospital. The age of patients was ranged from 2 to 15 years-old. The distribution of Sex was 7 patients in male, 9 patients in female. Persistent Left Superior Vena Cava [L.S.V.C.] was classified according to the proximal connection of L.S.V.C. into 3 groups. Group I which L.S.V.C. connected to coronary venous sinus was in 9 patients, Group II which L.S.V.C. connected to Left atrium was in 5 patients, Group III which L.S.V.C. hemodynamically connected to right atrium was in 2 patients. Pathoanatomical findings of complex congenital cardiovascular anomalies associated with L.S.V.C. in 16 cases were generally show unsystematic irregularity. In group I, A.S.D. were only in 3 cases, but in highest incidence and in group III, all two cases were supracardiac type of total anomalous drainage of pulmonary veins. Post-operatively, 3 patients among 14 patients of total correction, were died immediately, 1 patient of palliative shunt operation was died after 2 and half years, and Follow-up results of other remaining patients were excellent.

• Clinical and Experimental Pediatrics
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• 제62권2호
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• pp.68-74
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• 2019

Purpose: To investigate the prevalence of occult spinal dysraphism (OSD) and subsequent neurosurgery in pediatric patients with isolated or combined dorsal midline cutaneous stigmata with or without other congenital malformations. Methods: We carried out a retrospective review of patients who underwent sonography or magnetic resonance imaging (MRI) for OSD because of suspicion of dorsal midline cutaneous stigmata (presumed to be a marker for OSD) between January 2012 and June 2017. Information about patient characteristics, physical examination findings, spinal ultrasound and MRI results, neurosurgical notes, and accompanying congenital anomalies was collected. Results: Totally 250 patients (249 ultrasound and one MRI screening) were enrolled for analysis. Eleven patients underwent secondary MRI examinations. The prevalence of OSD confirmed by an MRI was 2.4% (6 patients including one MRI screening). Five patients (2%) had tethered cord and underwent prophylactic neurosurgery, 3 of whom had a sacrococcygeal dimple and a fibrofatty mass. Prevalence of tethered cord increased as markers associated with a sacrococcygeal dimple increased (0.5% of the isolated marker group, 8.1% of the 2-marker group, and 50% of the 3-marker group). Incidence of OSD with surgical detethering in 17 other congenital anomaly patients was 11.8%, which was higher than the 1.3% in 233 patients without other congenital anomalies. Conclusion: Our results suggest that the presence of dorsal midline cutaneous stigmata, particularly fibrofatty masses, along with a sacrococcygeal dimple is associated with OSD or cord tethering requiring surgery. OSD should be suspected in patients with concurrent occurrence of other congenital anomalies.

• Childhood Kidney Diseases
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• 제3권2호
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• pp.180-186
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• 1999

목적 : 요로감염이 있을 경우 방광요관역류를 포함한 선천성 요로계 이상을 25-55%에서 발견할 수 있다. 이 중 방광요관역류는 8-52%이나 이러한 역류성 병변이 일차성 역류인지,선천성 기형과 동반되는 이차성 역류인지 구별하기 어렵고 역류 자체에 의해 기존의 선천성 병변은 무시되기 쉽다. 이에 저자들은 방광요관역류 여부와 관계없이 선천성 요로계 이상이 요로감염과 동반된 경우에 대하여 성별 연령별 빈도, 선천성 요로계 이상의 종류 및 방광요관역류와의 관련성 여부 등에 대해서 알아보고자 본 연구를 실시하였다. 대상 및 방법 : 1996년 3월부터 1998년 2월까지 삼성서울병원에 요로감염증으로 입원치료받은 환아로 신초음파검사 단독 또는 초음파검사 및 배뇨중 요도조영술검사(voiding cystourethrography)를 모두 시행하여 적어도 한 가지 이상의 방사선학적 검사를 시행하였던 65례를 그 대상으로 하였다. 59례에서 초음파검사를 시행하였고 배뇨중 방광요도조영술은 51례에서, $^{99m}Tc-DMSA$ scan은 45례에서 시행하였다. 결과 : 1) 방광요관역류 여부와 관계없이 선천성 요로계 이상이 요로감염과 동반된 경우는 요로감염환아 65례 중 9례(13.8%)로 남아 7명 여아 2명이었다. 2) 연령별 분포를 보면 1세 미만이 1명, 1-2세 5명, 3-5세 1명, 5세 이상 2명이었다. 3) 선천성 요로계 이상의 종류는 각각 양측이소성신, 방광요관이행부폐쇄, 다낭신, 방광신우이행 부협착, 방광신우이행부폐쇄 및 수신증, 후부요도판막, 방광격막, 요막관잔유물, hutch diverticula 등이었다. 4) 역류 이외의 선천성 요로계 이상 9례 중 배뇨중 방광요도조영술은 8례에서 시행되었고 이 중 4례에서 방광요관역류가 발견되었다. 5) 요배양 검사에서는 E. coli가 3례(33.4%)에서 동정되어 가장 많았다. 결론 : 본 연구는 선천성 요로계 이상을 방광요관역류 여부와 관계없이 고찰한 것으로 13.8%에서 관찰되었다. 연구 대상의 예가 적어서 그 결과를 단정짓기는 어려우나, 다른 요로계 기형도 적지않게 동반되는 것으로 관찰되는바 요로감염증과 연관된 역류 이외의 다른 기형의 중요성도 간과되어서는 안될 것으로 사료된다.

• Anatomy and Cell Biology
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• 제56권4호
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• pp.469-473
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• 2023

Anomalies of anterior cerebral artery (ACA) include aplasia, hypoplasia and variations in number. Magnetic resonance angiography (MRA) is a non-invasive diagnostic technique for assessment of anomalies of cerebral arteries. The aim of the study was to determine the role of MRA in detection of variants of ACA in adults. This study is an observational retrospective study. This study included forty-nine adult cases (28 males and 21 females), mean age 48±12.9 SD with anomalies of ACA in MRA. Magnetic resonance imaging of the brain and MRA were done to all patients. Cerebral MRA and magnetic resonance images were evaluated for frequency and distribution of variants of anterior cerebral arteries, associated aneurysms and infarctions. Odds ratios (ORs) and relative risk were calculated to determine risk of occurrence of cerebral infarctions in patients with anomalies of ACA. Hypoplasia of ACA was the commonest anomaly of ACA (51% of cases). Risk of occurrence of cerebral infarctions was higher in cases with azygos variant (OR, 3.3; P=0.35) than in those with hypoplastic ACA (OR, 2; P=0.58). MRA was highly reliable in identification of different variants of ACA and concomitant vascular changes.

• Journal of Chest Surgery
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• 제28권6호
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• pp.565-570
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• 1995

Seventy-three patients with isolated total anomalous pulmonary venous connection the patients associated with other major cardiac anomalies such as single ventricle, DORV[Double Outlet Right Ventricle and large VSD[Ventricular Septal Defect were excluded were underwent surgical repair from January 1980 through October 1993. There were 45 boys and 28 girls. The mean age at operation was 19.9 months[range 6 days to 24 years and mean body weight was 7.1kg[range 2.6kg to 45kg . The anomalous locations of connection were supracardiac in 38, cardiac in 21, infracardiac in 5, and mixed in 9. In 38 patients[52% , the venous drainage was obstructed. The obstruction ratios according to the connection type were as follows: 53%[28/38 in supracardiac, 52%[11/21 in cardiac, 100%[5/5 in infracardiac, 22%[2/9 in mixed type. The associated cardiac anomalies were persistent left SVC[2 , tricuspid valve regurgitation[3 , cor triatriatum[1 , and mitral cleft[1 . And associated noncardiac anomalies were imperforate anus[1 and Neil Weightman syndrome[1 . The operative mortality was 23%. The causes of death were pulmonary hypertensive crisis, perioperative myocardial failure, pneumonia with sepsis, arrhythmia and etc. The statistically significant factors in postoperative mortality were the pulmonary venous obstruction and age [p<0.01 . The operative mortality was high in groups of age under 1 month and pulmonary venous obstruction. The mean follow-up was 27.1 months. There were two late deaths. The first patient was three months old boy with supracardiac type and severe obstructive symptoms. The postoperative echocardiography was showed anastomotic stenosis and reoperations were performed twice but the patients expired due to pneumonia and sepsis. The second patient was three month old boy with supracardiac type and total correction was done and was doing well postoperatively. Eight years later, he expired suddenly due to arrhythmia. But all the other patients were in NYHA Fc I and received no medications. The 5-year survival rate excluding early expired patients is 97.1 $\pm$ 0.03 %. In conclusion, although the operative mortality of total anomalous pulmonary venous connection was relatively high compared to other major cardiac anomalies, we could expect excellent long-term results by early surgical correction.