Clinical Observation of Myocardial Bridge

BACKGROUND AND OBJECTIVE: A myocardial bridge(MB) is an anatomical arrangement in which an epicardial coronary artery becomes engulfed for a limited segment by myocardial fibers. Although it has generally been felt that most instance of bridge are benign. Recent reports have suggested that MB can be associated with evidence of myocardial ischemia, myocardial infarction, arrhythmia and sudden death. This study investigated clinical characteristics of myocardial bridge and significance of treadmill test(TMT). METHOD: Among 4317 consecutive coronary angiograms performed from November 1995 to June 1999, 52 patients had a myocardial bridge. For the patients with MB, the clinical data, coronary angiography and the results of treadmill tests were reviewed. RESULT: The overall prevalence of myocardial bridge was 1.22%. Stable angina, atypical chest pain, variant angina, AMI were 33(63%), 15(29%), 2(4%), 2(%) cases, respectively. Electrocardiographic finding were normal in 31 cases(59%), ST-T change in 20 cases(38%), OMI in 1 case(3%). Mean systolic stenosis of MB was 54%, Mean length of segment of MB was 11.96 4.96mm and all patients had MBs of left anterior descending(LAD) coronary artery. Among 23 cases which had been performed TMT, 17 were positive(77%). There was no significant statistical difference between TMT(+) and TMT(-) in clinical characteristic and coronary angiographic data. We divided the patients with MB into two groups [group I(34 cases): systolic compression < 50%(mean 35.1 10.7%), group II(18 cases): systolic compression 50%(mean 63.6 14.7%)] and there were no statistical difference in clinical characteristics, TMT and angiographic data. CONCLUSION: The patients with MB present variable clinical characteristics of stable angina, atypical chest pain, variant angina, acute myocardial infarction. There is no relationship between the degree of systolic compression and TMT positive in MB. We think that symptoms of MB are not developed only by mechanical compression but concerned with other variable mechanism.

Mitral-aortic Intervalvular Fibrosa Pseudoaneurysm with Dual Fistula that Occurred in Prosthetic Aortic Valve Endocarditis

The Iinvolvement of subaortic structures in the aortic valve endocarditis appears more commonly than previously recognized. These subaortic complications are most commonly located in the mitral-aortic intervalvular fibrosa and may be presented as abscess, or as pseudoaneurysm with or without perforation. Perforated pseudoaneurysm can lead to the development of communication between the left ventricular outflow tract and various cardiac chambers, most commonly the left atrium. These complications are related with poor prognosis. Early and precise recognition of these complications is important for optimal treatment. At present, transesophageal echocardiography (TEE) has been validated as the technique of choice. We describe a case of infectious pseudoaneurysm of mitral-aortic intervalvular fibrosa featuring the connection of the fistulous simultaneously to the left atrium and aorta. In our case, accurate interpretation of TEE imaging revealing the subaortic structures was not so easy due to interference of both aortic and mitral prosthetic valves. We expect the further development of (Ed-confirming that here you don't intend, "We expect to further develop") TEE and other imaging modalities to substantially improve the future diagnosis of these undesirable complications.

Clinical Observation of Infective Endocarditis

BACKGROUND AND OBJECTIVES: The clinical pattern of infective endocarditis is constantly changing. Diagnosis and treatment of infective endocarditis were developed by recent diagnostic strategy (Duke criteria) and introduction of transesophageal echocardiography. The aim of this study was to compare the clinical character-istics of infective endocarditis in Sejong hospital to the previous report and was to investigate risk factor of heart failure, embolism and death. MATERIAL AND METHODS: All episodes hospitalized of infective endocarditis from January of 1990 to October of 1999 in Sejong hospital were reviewed retrospectively. The total cases of infective endocarditis was 80 cases in 78 patients. RESULTS: The male to female ratio was 1:1.05 (39 male, 41 female). The mean age was 42 years. Rheumatic heart disease was the most common predisposing heart disease in 28.8%, followed by prosthetic valve endocarditis. Streptococci were the most commonly isolated micro-organisms in 18 cases (45.0%), followed by staphylococci in 11 cases. Transesophageal echocardiography has a higher sensitivity than transthoracic echocardiography for detection of vegetation, abscess and paravalvular complication in endocarditis. In patients with echocardiographic vegetation and involvement of aortic valve, there was a statistically significant increase in the risk of heart failure. The factor that was associated with a statistically significant increase in the overall risk of embolization was not exist. Ten patients died (12.5%). Risk factor for death was left ventricular dysfunction. The incidence of death was more higher in patients with abscess and non-streptococcal endocarditis. CONCLUSION: Compared to the 1980's report, we observed increased mean age of patients, the decreasing trend of rheumatic heart disease as a predisposing heart disease and the decreasing incidence of streptococci as causative microorganism. Risk factors for congestive heart failure were aortic valve endocarditis and endocarditis with vegetation. Risk factor for death was left ventricular dysfunction.

Two cases of ruptured congenital sinus of Valsalva aneurysms dissecting into the interventricular septum in patients with cerebral infarction

Sinus of Valsalva aneurysms are rare cardiac anomalies and are usually caused by the separation of the aortic wall media from the valve ring tissue. These aneurysms frequently rupture into the low-pressure areas like the right ventricle and right atrium, rarely do they rupture into the left atrium, left ventricle, pericardial sac, or pulmonary artery. Cerebral infarction has been reported as a rare complication of unruptured sinus of Valsalva aneurysm. We experienced very rare two cases of Valsalva aneurysms of right coronary sinus dissecting into the interventricular septum in patients with cerebral infarction. In two cases these aneurysms ruptured into the left ventricle. These aneurysms were excised and the defect was closed with autopericardium. At the end of the surgical repair, coaptation was found to be insufficient and aortic valve replacement was undertaken.

One case of left anterior descending artery fistula-right ventricle complicating rotablator atherectomy with spontaneous occlusion in a following coronary angiogram

No abstract available.

Congenital Esophageal Stenosis: with Special Reference to Diagnosis and Postoperative Complications

PURPOSE: Congenital esophageal stenosis(CES) is one of the rare causes of recurrent vomiting during infancy and childhood. We studied the diagnostic and therapeutic tools and postoperative complications for early diagnosis and adequate management of CES. METHODS: Fourteen cases of CES were evaluated for clinical manifestations, findings of esophagogram and esophagoscopy, classification of pathologic findings and postoperative complications. RESULTS: Most common clinical manifestations at onset were non-projectile vomiting(14), dysphagia to solids(13). Age at onset of symptoms corresponded with the introduction of solids in 11 cases. Esophagogram showed segmental stenosis of variable length in the lower portion of the esophagus in all cases with marked proximal dilatation in 11 cases. Esophagoscopy revealed no signs of esophagitis or ulcer at the area of stenosis. Segmental resection and primary anastomosis were performed as a definitive treatment modality in all cases except one with fibromuscular stenosis. Bronchial cartilage were present in all cases of tracheobronchial remnants(10). Abnormal arrangement and thickening of muscularis mucosae and inner circular muscle were found in all cases of fibromuscular stenosis(4). Postoperative complications were gastroesophageal reflux(5), stricture of anastomotic sites, reflux esophagitis, and so on. CONCLUSION: CES is rare but should be considered as a cause of recurrent vomiting and dysphagia to solid food beginning in infancy and childhood especially in the weaning period. Esophagogram and esophagoscopy are useful tools for diagnosis and differential diagnosis. The stricture of anastomosis site, gastroesophageal reflux and esophagitis need to be evaluated in the follow-up postoperative periods.

A Result of Treatment of 'Eight Drugs in a Day' in Medulloblastoma: Comparison between Pre- vs Post-Irradiation Chemotherapy / 대한소아혈액종양학회지

BACKGROUND: 'Eight drugs in a day' was one of the widely used regimen in medulloblastoma. Result of treatment of this regimen and comparison between pre-RT chemotherapy and post-RT chemotherapy were presented. METHODS: Medical records of children who were diagnosed as medulloblastoma and treated with 8 in 1 therapy in Seoul National University Children's Hospital from January 1986 to June 1997 were reviewed. RESULTS: 1) Forty nine cases(male: 30, female: 19) were analyzed. The age at diagnosis was between 3 months and 15 years 3 months and median age was 7 years 10 months. 2) The T stage by Chang classification revealed T1(2%), T2(26%), T3a(9%), T3b(56%), and T4(7%) in 43 cases. M stage revealed M0(46%), M1(15%), M3(37%), and M4(2%) in 41 cases. The surgical results revealed gross total resection(36%), near total resection(18%), subtotal resection(38%), and partial resection(9%). 3) The 5-yr disease free survival(DFS) rate of all tumors was 53%. There was no difference in DFS about sex, age, pathology, T stage, M stage, and surgical result. 4) Difference of 5-year DFS between pre-RT chemotherapy group(53%) and post-RT chemotherapy group(83%) was significant[-2Log(LR), P=0.023], excluding the patient relapsed or too young(below 18 month, who had more chemotherapy to delay radiation) to be received radiation therapy. CONCLUSIONS: DFS in post-RT chemotherapy was better than pre-RT chemotherapy. As medulloblastoma has the property to confine in CNS system, prior treatment with radiation as a intensive local therapy may be more effective than the systemic chemotherapy. Progression or relapse were the main causes of treatment failure. Result of treatment may be improved by more intensive therapy.

Periventricular Leukomalacia and Cerebral Palsy in Preterm Infants

As the survival rate of preterm infants increasing, much perinatologic concern has been paid to the possibility of their getting into cerebral palsy. The purpose of this study is to determine the relationship between the early periventricular abnormalities and the incidence of cerebral palsy among preterm infants, and then to estimate the value of periventricular leukomalacia(PVL) as the prognostic factor by verifying the clinicopathological correlation of it with cerebral palsy. We have selected 174 cases with periventricular abnormality in early ultrasonographic findings among the survival preterm infants. 163 of them were followed up successfully to verify the incidence of cerebral palsy. Sequential ultrasonographic observations had been made at the interval of 7 or 10 days. Periventricular abnormalities could be classified into three types, which consist of the PVL(including cystic PVL), suspicious PVL, and periventricular echodensity without tissue loss. Associated lesions such as intraventricular hemorrhage, ventriculomegaly, brain atrophy were evaluated. Diagnosis of cerebral palsy was confirmed after following up more than 2 years of age, and the correlations of periventricular abnormalities with the clinical type and severity of cerebral palsy were analyzed. The 24.5% (40/163) of our samples revealed periventricular leukomalacia and the 17.8% (29/163) of them were diagnosed as cerebral palsy. The 65% (26/40) of preterm infants with definite PVL were cerebral palsy and their relative risk ratio for cerebral palsy is 26.7. Without PVL, there were no explicit correlations between cerebral palsy and associated abnormalities. Most cases with cerebral palsy turned out to be the spastic diplegia. In the cases with cystic lesions especially, the clinical outcome appear to be more severe. We've reached the point that the presence of PVL in preterm infants had a strong correlation with the development of cerebral palsy. Moreover, the association with cystic lesions is not only a strong correlation with cerebral palsy, but also a poor prognostic value for functional outcome.

A Pediatric Case of MELAS Syndrome Associated with Insulin-dependent Diabetes Mellitus

MELAS(mitochondrial myopathy, encephalopathy, lactic acidosis, and sroke like episodes) is a major subgroup of heterogeneous mitochondrial encephalopathy. Recent advances in molecular genetics revealed specific mutations in the mitochondrial DNA causing MELAS. We described clinical and molecular genetic findings of a 13-year-old boy with MELAS syndrome associated IDDM(insulin dependent diabetes mellitus). For molecular genetic studies, DNAs from peripheral blood nucleated cells were used. And the A-->G substitution at the nt position 3,243 in the mitochodrial tRNAleu(UUR) gene in a heteroplasmic fashion was confirmed in the patient and his mother, which supporting maternal transmission. The mother had no neuromuscular syndromes but was diabetic. The islet cell antibody was abscent in both the patient and his mother, proving an indirect evidence of beta cell destruction was caused by the definite mitochondrial DNA itself. The association of MELAS syndrome and IDDM has been reported very rarely, and this is the first case report in Korea.

Four Cases of Congenital Esophageal Stenosis Due to Tracheobronchial Remnants

Esophageal stenosis due to tracheobronchial remnants is a rare cause of congenital esophageal stenosis. The cause is thought to be esophageal sequestration of tracheobronchial remnants during embryonic separation. Errors in diagnosis are common and high index of suspicion is required for accurate diagnosis, and resection of primary site with anastomosis is recommanded. Although a rare entity, esophageal stenosis due to tracheobronchial remnants should be considered in patients with esophageal stenosis, when esophageal stenosis fails to respond to standard therapy including bougienage and balloon dilatation, and esophagoscopy shows normal mucosa on the stenotic segment. Four cases of congenital esophageal stenosis due to tracheobronchial remnants were reported, the first case without accompanied anomaly, the second case accompanied by tracheoesophageal fistula, the third case accompanied by Down syndrome, and the last case accompanied by tracheoesophageal fistula and congenital heart disease.

Cystinuria 3 Cases

We report three cases of cystinuria, presenting with urinary stones. A 2-year-old girl presented with urinary difficulty, hematuria, dysuria of sudden onset, and her 7-month-old younger brother also was presented with urinary difficulty, irritability on urination & stone passage. Other 6-month-old boy was admitted due to sudden onset anuria. They had radioopague renal & ureter stones and stone analysis revealed mixed cystine stones. The diagnosis of cystinuria was confirmed metabolic studies and stone analysis. Lrinary amino acid analysis showed excessive excretion of dibasic amino acids(cystine, ornithine, lysine, arginine). And they all had hypercalciuria and hyperuricosuria. They were treated with combination of percutaneous lithotripsy for large obstructing senes a nd an oral drug therapy with sodium bicarbonate for rendering the urine more alkaline, and alpha-mercaptopropionylglycine(ThiolaR). This form of treatment was sucessful in our three cases with elimination of recurrent nephrolithiasis, but in one patient, nephrotic syndrome possibly caused by ThiolaR was developed. The nephrotic syndrome was recovered spontaneously after cessation of Thiola. A review of literatures was also attempted briefly.

Infantile Choledochal Cyst Presenting with Neonatal Cholestasis; Review of Anatomical and Clinical Aspect

No abstract available.

Magnetic resonance imaging in Legg-Calve-Perthes' disease / 대한정형외과학회잡지

No abstract available.

Hallervorden-Spatz syndrome in two siblings diagnosed by the clinical features and magnetic resonance imaging (MRI)

Hallervorden-Spatz syndrome (HSS) is a heredodegenerative disorder characterized by both progressive pyramidal and extrapyramidal signs, dysarthric speech, and mental deterioration. No diagnostic biochemical test is yet available, and diagnosis of HSS can be confirmed only at autopsy by the characteristic neuropathology including abnormal iron storage, disordered myelination, and loss of brain substance. We present two siblings with clinical features consistent with HSS, in whom magnetic resonance imaging (MRI) demonstrated the deposition of iron in the globus pallidus and the substantia nigra thus allowing an antemortem diagnosis of HSS.

Three cases of acute lobar nephronia in children / 대한신장학회잡지

No abstract available.

Clinical observation on brainstem glioma in childhood

No abstract available.

The Urologic Abnormalities of Urinary Tract Infection in Children and their Detection / 대한비뇨기과학회지

We have reviewed 251 children with urinary tract infection who were seen in our hospital during the last 5 years. Underlying abnormalities of the urinary tract were found in 154 patients(61 %). This figure is high because referred patients from other physicians for further treatment are included here. Of these 154 patients, surgical treatments were required in 85. The most frequent finding was vesicoureteral reflux(110 pts). Obstructive lesions were found in 44 pts ; UPJ obstruction in 9, megaureter in 6, obstruction associated with duplication in 6, urethral valve in 5, neurogenic bladder in 11, and others in 7. Abnormal findings without clinical significance were found in additional 17 pts. To evaluate the role of ultrasonography(US), we have compared findings of intravenous pyelography(IVP) and US. Of 58 pts who had both studies, 52 showed identical findings. US failed in detection of small renal scar and duplication of collecting system. US in combination with voiding cystourethrography(VCU) identified abnormal findings in most cases. It is our impression that US can replace IVP as a screening procedure which should be performed with VCU in every child with proven UTI. And as IVP is superior in imaging anatomical details than US, we recommend IVP before any surgical attempt.

Mitral Atresia Associated with Corrected Transposition of Great Arteries

A case of congenital absence of right atriovntricular (A-V) connection of heart is reported. Patient was 81 day old male infant with chief complaints of dyspnea and irritability. Clinical evaluation revealed increased pulmonary vascularity on chest x-ray, absence of right A-V connection, regurgitation of left A-V valve, right-sided ventricular hypoplasia and L-TGA. Clinical assessment of the case was tricupsid atresia with LTGA and mitral regurgitation. Pulmonary artery banlding and artial septectomy was performed. Parenteral alimentation through the femoral veins resulted in obstruction of IVC and subsequent multiorgan failure. The autopsy findings of the heart were corrected transposition of great arteries with associated anomallies of right-sided mitral atresia, Ebstein's malformation of left-sided tricuspid valve, ventricular septal defect, muscular hypertrophy of right-sided left ventricle with luminal obliteration. Long segment of IVC was completely occluded due to occlusive thrombi with fungal infection. Bilateral kidneys were infarcted and azygos vein was markedly engorged. Microscopic examination revealed candidal colonization in the inferior vena cava and septic embolzation in brain.

Mitral Atresia Associated with Corrected Transposition of Great Arteries

A case of congenital absence of right atriovntricular (A-V) connection of heart is reported. Patient was 81 day old male infant with chief complaints of dyspnea and irritability. Clinical evaluation revealed increased pulmonary vascularity on chest x-ray, absence of right A-V connection, regurgitation of left A-V valve, right-sided ventricular hypoplasia and L-TGA. Clinical assessment of the case was tricupsid atresia with LTGA and mitral regurgitation. Pulmonary artery banlding and artial septectomy was performed. Parenteral alimentation through the femoral veins resulted in obstruction of IVC and subsequent multiorgan failure. The autopsy findings of the heart were corrected transposition of great arteries with associated anomallies of right-sided mitral atresia, Ebstein's malformation of left-sided tricuspid valve, ventricular septal defect, muscular hypertrophy of right-sided left ventricle with luminal obliteration. Long segment of IVC was completely occluded due to occlusive thrombi with fungal infection. Bilateral kidneys were infarcted and azygos vein was markedly engorged. Microscopic examination revealed candidal colonization in the inferior vena cava and septic embolzation in brain.

Pheochromocytoma and Renal Artery Stenosis

In hypertensive patients it is very important to detect renal artery stenosis or pheochromocytoma, since both diseases are curable causes of hypertension. However, renal artery stenosis can be induced by pheochromocytoma, when the diagnosis of the two simultaneous disease is very difficult. We experienced two cases of pheochromocytoma presented as renal artery stenosis. Pheochromcytoma was overlooked when renovascular hypertension was diagnosed. Pheochromocytoma was found during surgery in one patient and after angioplasty in the other. In both cases, BP returned to normal after surgical removal of pheochromocytoma without repair of the stenosis. Prevention of ineffective and unnecessary renal artery angioplasty of surgery requires knowledge of this unusal association between pheochromocytoma and renal artery stenosis and a high degree of clinical alertness for pheochromocytoma.