Kinky Hair Syndrome -1예 보고 -

연경모 대한영상의학회 1986 대한영상의학회지 Vol.22 No.6

Kinky hair syndrome is a sex-linked recessively inherited copper metabilic disorder with severe neurodegenerative change and infant death. In 1962, Menkes and associates described five boys of a related pedigree with severe psychomotor retardation, seizures and widespread cerebral and cerebellar degeneration. In 1796,. Wesenberg and associates specified the raioligical characterization of the syndrome. Symmetrical metaphyseal spurring and diaphyseal periosteal reactio of thelong bones. anterior rib flaring, am malformed cerebral arterial system and subdural effusion. In 1972, Danks and associates found the disease to be associated with a defect of copper metabilism, confirmed by studies with labelled Cu. Authors experienced a case with characteristics clinical picture, and report cerebral and abdominal arteriorgrpahic changes and plain raidographic findings with brain CT, DSA and post-mortem angiogrpahy.

만성 가성 장폐쇄 증후군

연경모 대한영상의학회 1992 대한영상의학회지 Vol.28 No.2

Chronic intestional pseudoobstruction syndrom is a rare clinical condition in which impaired intestinal peristalsis causes recurrent symptoms of bowel obstruction in the abvsence of a mechanical occlusion. This syndrome may involve variable segments of small or large bowel, and may be associated with urinary bladder retention. This study included 6 children(3 boys and 3 girls) of chronic intestinal obstruction. Four were symptomatic at birth and two were of the ages of one month and one year. All had abdominal distention and defication difficulty. Five had urinary bladder distention. Despite parenteral nutrition and surgical intervention(ileostomy or colostomy), bowel obstruction persisted and four patients expired from sepsis within one year. All had gaseous distention of small and large bowel on abdominal films. In small bowel series, consistent findings were variable degree of dilatation, decreased peristalsis (prolonged transit time) and microcolon or microrectum. This dis ase entity must be differentiated from congenital megacolon, ileal atresia and megacystis syndrome.

폐동맥 판막협착증의 심장영화 촬영 소견과 바룬카테타 성형술에 관한 연구

연경모 대한영상의학회 1987 대한영상의학회지 Vol.23 No.4

Transluminal balloon valvuloplasty was performed in the treatment of congenital pulmonic valvular stenosis in 55 children, aged 4 months to 15 years. The right ventricular outflow tract pressure gradient decreased significantly immediately after the procedure from 87.18$\pm$56mmHg to 29.62$\pm$26.48mmHg. Technical success rate was 98% and failed case was due to severe fibrous thickening of valve. Complication occurred in one case, that is tricuspid regurgitation due to suspected rupture of chordae tendinae. THe degree of pulmonary conus dilatation was closely related with age than the pressure gradient.

활로사증후군의 심장영화촬영의 소견

연경모 대한영상의학회 1985 대한영상의학회지 Vol.21 No.3

The total 223 cases of tetralogy of Fallot is analysed angiographically, in which selective biplane cinecardioangiography with axial projection has been done at the Department of Radiology, Seoul National University Hospital, in recent 2 year and 4 months, with specific refrence to the location of pulmonic stenosis, and associated cardiac anomalies. The summary of the analysis is as follow; 1. Left ventriculogram (long axial oblique view) was necessary to detect the ventricular septal defects including anterior marginal VSD, and was helpful to identify the coronary artery anatomy and presence of PDA and evaluate the size of left ventricle. 2. Right ventriculogram (elongated RAO view) was the best projection for the demonstration of pulmonic infundibulum and was essential to differenciate and localize the ventricular septal defects. 3. In the right ventriculogram (4 chamber view), well demonstrated the right ventricular outflow tract, pulmonary valve, and pulmonary arteries (ma n, right, left and peripheral), and also perimitted the detection of ASD in levophase. 4. The position of ventricular septal defects was perimembranous in 199 cases (89.2%0, subarterial in 75 cases (6.8%), and infundibular (intracristal) in 9 cases (4%0. Multiple vintircular septal defects(perimem-branous & marginal) were found in 11 cases (4.5%) 5. In 35 cases (15.5%), pulmonary stenosis is found only in pulmonary infundibulum. The combination of its components was most commonly anterior wall and conus septum hypertrophy in 24 cases (68.6%). 6. Combined pulmonary stenosis was identified in 188 cases (84.3%). The most common combination was infundibular and pulmonary valvular stenosis in 56 cases (29.8%0. 7. Associated cardiac anomalies were right aortic arch in 53 cases (23.8%0, persistent left SVC in 17 cases (7.7%0, ASD in 13 cases (5.9%), PDA in 9 cases (4.1%), aberrant subclavian artery in 5 cases (2.2%0, IVC interruption with azygos continuation in 2 cases, single coronary arte y in 2 cases, and anterior descending artery from right coronary artery in 1 cases, etc. 8. Selective biplane cinecardioangiography with axial projection is essential to evaluate the size of left ventricle and to localize the ventricular septal defects and pulmonary stenosis, and to detect associated cardiac anomalies in T.O.F. Sometimes aortography is necessary when anomaly of coronary arteries is suspected.

신동맥색전증에서의 미세혈관조영상에 관한 연구

연경모,한만청 대한영상의학회 1981 대한영상의학회지 Vol.17 No.1

Renal artery embolization was induced experimentally in rabbits and microvascular changes were studied angiographically, mocroangiographically and histopathologically. The results were as follows : 1. The main microangiograhic findings of renal artery embolization were arterial occlusion and collateral vessels are characterized by spiralling, dilatation, irregular lumen with abrupt caliber change, disoriented course and increased number. 2. Collarteral vessel formation was demonstrated in all cases of renal artery embolization by microangiography. 3. REcanalization of embolized vessels was better visualized by renal angiography than microangiograhy. It was considered that microangiography is a valuable method for the observation of microvascular changes in renal artery embolization and other renla diseases.

小兒 腹部疾患 診斷

延敬模 한국의학사 1984 診斷과治療 Vol.4 No.2

염색체이상의 골 X-선진단

延敬模 한국의학사 1985 診斷과治療 Vol.5 No.5

Balloon catheter를 이용한 소아 식도협착의 치료

연경모 대한영상의학회 1986 대한영상의학회지 Vol.22 No.5

Severe postoperative strictures in two children and an infant and mild postoperative stricture in a child were treated with balloon catheter. A child with post-fundoplication stricture showed symptomatic improvement.Anastomotic stricutes after esophageal atresia repair in an infant and a child were successfully dilated withimproved lumina diameter and symptoms. In a child with mild postoperative stricture, balloon dilatation was performed to prevent stricture of the anastomotic site. Raiological esophageal dilatation using balloon catheters is a safe and effective method for dilating symptomatic esophageal strictures which obviates surgery and allows subsequent standard bougienage.

선천성 요도 판막증의 방사선학적 고찰

연경모 대한영상의학회 1990 대한영상의학회지 Vol.26 No.6

Congenital Urethral Valve is the common cause of hydronephrosis in newborn infants and the most common cause of bladder outlet obstruction in male children. We reviewed and analysed radiological findings and associated anomalies of 16 cases of congenital urethral valve which were examined during the period from January 1985 to December 1989. The most frequent age was under one year old(56%). The main symptoms were urinary dribbling (37.5%), weak stream (25%) and urinary frequency & incontinence 925%). Anterior urethral valve (AUV) was 5 cases (31%) and posterior urethral valve (PUV) was 11 cases (69%), in which 10 cases were Type Iand one case was Type III. Bladder wall thickening was seen in all cases and its severity was partly correlated with the degree of vesicoureteral reflux (VUR). VUR was observed in 12 cases (75%), and relatively severe in older age group. The degree of VUR was milder in AUV than PUV. Hydronephrosis was more severe in PUV than in anterior one, and its egree was correlated with the severity of VUR. Associatd anomalies were ectopic urethral opening 92 cases0, PDA (1 case), congenital megacolon (1 case) and patent urachus (1 case) in PUV. So early diagnosis and treatment of cogenital urethral valve is essential to the prevention of renal damage.

폐동맥판막 풍선 카테타 성형술에 관한 실험적 연구 : 단일풍선과 복수풍선카테타의 비교

연경모 대한영상의학회 1993 대한영상의학회지 Vol.29 No.3

Balloon pulmonary valvuloplasty is an established method in the treatment of congenital valvular pulmonary stenosis. Balloon valvuloplasty was performed by using one or two balloons in a total of 19 mongrel dogs. Cross sectional area ratio ranging 145-406% was used to compare the relative sizes of the pulmonary annulus and the balloon. Hemodynamic changes and cardiac damages in single and double balloon groups were analyzed. Survival time of double balloon group was longer than that of single balloon group (p<0.05) Increment of the right ventriclular systolic pressure in single balloon group (mean 38 mmHg)was greater than that in double balloon group (mean 87 mmHg) was greater than that in double balloon gourp (mean 41 mmHg) (p<0.05). The locations of trauma were mainly the right ventricular free wall, the main pulmonary artery, the anterior leaflet of the pulmonary valve and in a less degree, anterior wall of the right atrium, the right ventricular outflow tranct and the left pulmonary artery int he order of frequency. There were no significant differences in cardiac damages between single and double balloon groups(p<0.05) Major microscopic findigns were sloughing of the endotheliu, fibrin deposition, hemorrhae, localized myocardial infarction and so on. In conclusion, double balloon pulmonary valvuloplasty is superior to single balloon technique in survival time and hemodynamics. Therefore, double balloon technique is recommended for the pulmonary valvuloplasty.