No abstract available.
Aneurysm* ; Hydrocephalus* ; Hypothalamus* ; Subarachnoid Hemorrhage*

The VSD in TOF is usually large and unrestrictive with an equal to or greater than that of the aortic annulus. Typically shunting through the VSD is bidirectional or right-to-left component. Restrictive VSD in TOF caused by ingrowing fibrotic tissue is very rare. We report a case of restrictive VSD and LVOTO in TOF caused by ingrowing fibrotic tissue with the review of literature.
Constriction, Pathologic* ; Fibrosis ; Heart Septal Defects, Ventricular* ; Tetralogy of Fallot*

The causes of aortic dissection are usually hypertension, connective tissue disease such as Marfan syndrome, congenital valvular abnormality such as bicuspid aortic valve, iatrogenic injury, pregnancy and drugs. Previous studies have shown that 50% of all dissections in women less than 40 years age were associated with pregnancy. Almost all aortic dissections during pregnancy occur during the third trimester or during labor and delivery. Marfan's syndrome is a particularly important predisposing factor for aortic dissection during pregnancy. We report here on a case of surgical treatment for acute type II aortic dissection in a Marfan syndrome patient who was 24 weeks pregnant, and we include a review of literature.
Aortic Valve ; Bicuspid ; Causality ; Connective Tissue Diseases ; Female ; Humans ; Hypertension ; Marfan Syndrome* ; Pregnancy ; Pregnancy Trimester, Third

This retrospective case series of prospective data aims to describe the transaxillary approach for the treatment of upper thoracic spine pathology. Various surgical techniques and approaches have been reported across the literature to address upper thoracic spine pathology, including the cervicothoracic approach, anterior transsternal approach, posterolateral approach, supraclavicular approach, and lateral parascapular approaches. These techniques are invasive. A minimally invasive, less morbid, and direct access approach to the pathology of the upper thoracic spine has not been reported in the literature. Patients with pathology affecting the first thoracic vertebra up to the sixth thoracic vertebra were classified into the upper thoracic spine group. Patients with pathology below the sixth thoracic vertebra were excluded. Patients not having a minimum follow-up of 12 months were also excluded. The study analyzed 18 patients. The mean preoperative modified Japanese Orthopedic Association score was 7.2±1.44, which improved to 10.16±1.2 (p<0.05). The majority (14/18) of the patients had an excellent outcome. Three patients had good outcomes, and one patient had a fair outcome. Five cases of intraoperative dural leak were recorded, and one patient had postoperative neurological deficit. The transaxillary approach is a safe, viable, muscle-sparing, and minimally invasive approach for ventral pathologies of the upper thoracic spine.

BACKGROUND: We analyzed our experience of arrhythmia surgery in patients with congenital heart disease. MATERIAL AND METHOD: A retrospective review was performed on 43 consecutive patients with congenital heart disease, who underwent arrhythmia surgery between June 1998 and June 2006. RESULT: The median age at surgery was 52 years (4~75 years). The most frequent cardiac anomaly was an atrial septal defect (23/43, 53.5%). The types of arrhythmias were atrial flutter-fibrillation, intermittent non-sustainable ventricular tachycardia and others in 37, 2 and 4, respectively. Arrhythmia surgery consisted of a bi-atrial maze operation in 18 patients (modified cox maze III procedure in 5 patients, and a right side maze plus pulmonary vein cryo-isolation in 13), right side maze operation in 18 patients, cavo-tricuspid isthmus cryoablation for benign atrial flutter in 4 patients, right ventricular endocardial cryoablation in 2 patients and extranodal cryoablation for atrioventricular node re-entry tachycardia in 1 patient. The median follow-up was 23.8 months (1~95.2 months). There was no early mortality, and one late non-cardiac related death. The overall rates of restored sinus rhythm before discharge and 3~6 months after surgery were 79% and 81%, respectively (bi-atrial maze group: 72% and 83%, right-side maze group: 77%, 77%). CONCLUSION: Arrhythmias associated with congenital heart disease can be safely treated surgically with an excellent intermediate-term outcome.
Arrhythmias, Cardiac* ; Atrial Fibrillation ; Atrial Flutter ; Atrioventricular Node ; Cryosurgery ; Follow-Up Studies ; Heart Defects, Congenital* ; Heart Septal Defects, Atrial ; Humans ; Mortality ; Pulmonary Veins ; Retrospective Studies ; Tachycardia ; Tachycardia, Ventricular

A 3 month old female baby, who had been diagnosed with right atrial isomerism associated with total anomalous pulmonary venous return (TAPVR), a functional single ventricle and major aortopulmonary collateral arteries (MAPCA), underwent left MAPCA unifocalization and left Blalock-Taussig shunt (3.5 mm) at 3 months of age. The postoperative course was complicated by pulmonary venous congestion, and the drainage site of the TAPVR was found to be stenotic on echocardiography. We performed sutureless repair of the TAPVR along with unifocalization of the right MAPCA. She was put on an extracorporeal membrane oxygenator for 8 days after the 2nd operation, and she was able to come off the oxygenator with the placement of a central shunt (3 mm). She developed tracheal stenosis, which was presumably due to longstanding endotracheal intubation, and she then underwent tracheostomy. She was discharged to home on day 104 after the 1st operation, and she has been followed up for 2 months in a good clinical condition.
Arteries ; Drainage ; Echocardiography ; Extracorporeal Membrane Oxygenation ; Female ; Heterotaxy Syndrome* ; Humans ; Hyperemia ; Infant ; Intubation, Intratracheal ; Oxygen ; Oxygenators ; Oxygenators, Membrane ; Scimitar Syndrome ; Tracheal Stenosis ; Tracheostomy

PURPOSE: The aim of this study was to characterize Korean patients with Fanconi anemia (FA), which is a rare but very challenging genetic disease. METHODS: The medical records of 12 FA patients diagnosed at Chonnam National University Hospital from 1991 to 2012 were retrospectively reviewed. RESULTS: The median age at diagnosis was 6.2 years. All patients showed evidence of marrow failure and one or more physical stigmata. Chromosome breakage tests were positive in 9 out of 11 available patients. The median follow-up duration was 69.5 months. The Kaplan-Meier (KM) survival of all patients was 83.3% at 10 years and 34.7% at 20 years, respectively. Seven patients underwent 9 stem cell transplantations (SCTs). Among them, 5 were alive by the end of the study. Ten-year KM survival after SCT was 71.4% with a median follow-up of 3.4 years. All 5 patients treated with supportive treatment alone died of infection or progression at the median age of 13.5 years, except for one with short follow-up duration. Acute leukemia developed in 2 patients at 15.4 and 18.1 years of age. Among 6 patients who are still alive, 3 had short stature and 1 developed insulin-dependent diabetes mellitus. CONCLUSION: We provide information on the long-term outcomes of FA patients in Korea. A nation-wide FA registry that includes information of the genotypes of Korean patients is required to further characterize ethnic differences and provide the best standard of care for FA patients.
Bone Marrow ; Christianity ; Chromosome Breakage ; Diabetes Mellitus, Type 1 ; Diagnosis ; Fanconi Anemia* ; Follow-Up Studies* ; Genotype ; Humans ; Jeollanam-do ; Korea ; Leukemia ; Medical Records ; Retrospective Studies ; Standard of Care ; Stem Cell Transplantation ; Treatment Outcome*

OBJECTIVE: The anterior interbody fusion for cervical spine disorder may not provide adequate immediate stabilization, whereas anterior cervical spine plating has overcome this main disadvantage. Recently, several types of hardware were introduced and the complications related to these were also reported. In case of degenerative cervical spine disorders, the adequate intervertebral disc space height and lordotic curvature are the major factors for preventing recurrence of symptoms. So we used the PCB instrumentation(cervical plate cage system) for these purpose. MATERIAL AND METHOD: From April 1998 to October 1998, 16 patients with degenerative cervical spine disorders who had radiculopathy or myelopathy were underwent anterior cervical spine fusion with the PCB instrumentation(cervical plate cage system). Single level fusion was accomplished in 8 patients and two level fusion in 8. The mean follow-up period was 4 month. RESULT: All patients show some recovery at discharge. There were no hardware problems. The intervertebral disc space height and lordotic curvature were not changed during follow up. CONCLUSION: These finding suggest that PCB instrumentation can be considered to be one of the useful hardwares for anterior cervical spine fusion in degenerative cervical spine disorders.
Follow-Up Studies ; Humans ; Intervertebral Disc ; Radiculopathy ; Recurrence ; Spinal Cord Diseases ; Spine

Absorbed dose to water based protocols recommended that plane-parallel chambers be calibrated against calibrated cylindrical chambers in a high energy electron beam with R50>7 g/cm2 (E> or =16 MeV). However, such high-energy electron beams are not available at all radiotherapy centers. In this study, we are compared the absorbed dose to water determined according to cross-calibration method in a high energy electron beam of 16 MeV and in electron beam energies of 12 MeV below the cross-calibration quality remark. Absorbed dose were performed for PTW 30013, Wellhofer FC65G Farmer type cylindrical chamber and for PTW 34001, Wellhofer PPC40 Roos type plane-parallel chamber. The cylindrical and the plane-parallel chamber to be calibrated are compared by alternately positioning each at reference depth, zref=0.6R50-0.1 in water phantom. The DW of plane-parallel chamber are derived using across-calibration method at high-energy electron beams of 16, 20 MeV. Then a good agreement is obtained the DW of plane-parallel chamber in 12 MeV. The agreement between 20 MeV and 12 MeV are within 0.2% for IAEA TRS-398.
Electrons ; Water

For treatment of Total Skin Electron beam Therapy (TSET), measurement of dose at various conditions is need on the contrary to usual radiotherapy. When treating TSET with modified Stanford technique based on linear accelerator, the energy of treatment electron beam, the spatial dose distribution and the actual doses deposited on the surface of the patient were measured by using EBT2. The measured energy of the electron beam was agreed with the value that measured by ionization chamber, and the spatial dose distribution at the patient position and the doses at several point on the patient's skin could be easily measured by EBT2 film. The dose on the patient that was measured by EBT2 film showed good agreement with the data measured simultaneously by TLD. With the results of this study, it was proven that the EBT2 film can be one of the useful dosimeter for TSET.
Electrons ; Humans ; Particle Accelerators ; Skin