• Journal of Chest Surgery
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• v.38 no.8 s.253
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• pp.529-537
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• 2005

Background: Historically the Fontan operation in patients with single ventricle and heterotaxy syndrome has been associated with high mortality because of systemic or pulmonary anomalous venous drainage, incompetent common atrioventricular valve, right ventricle type univentricular heart, and arrhythmia. Material and Method: A retrospective review of 62 patients $(age:\;54.79\pm33.97\;months)$ with heterotaxy syndrome who underwent a extracadiac Fontan operation between 1996 and 2005 was performed. Twenty one patients had left atrial isomerism, and 41 had right isomerism. The Fontan procedure was staged in all but 2 patients, and a fenestration was less placed in left isomerism. Result: Left isomerism was associated more with interrupted inferior vena cava and pulmonary arteriovenous fistula, and right isomerism was associated more with anomalous pulmonary venous drainage, common atrioventricular valve and morphologic right ventricle. There were 3 hospital deaths$(4.8\%)$ and 3 late deaths $(5.2\%)$ with a follow-up duration of $48.8\pm31.0$ months. Eight-year survivals were $90.5\pm6.4\%$ in left isomerism and $88.6\pm5.4\%$ in right isomerism (p=0.94). At 8 years, freedom from reoperation was $73.9\pm11.3\%$ in left isomerism, and $82.3\pm6.7\%$ in right isomerism (p=0.87). Atrioventricular valve regurgitation progressed after Fontan operation in heterotaxy syndrome, and reoperation for pulmonary arteriovenous fistula and permanent pacemaker implantation for sinus node dysfunction were required more in left isomerism. Conclusion: The extracardiac Fontan operation can now be performed in patients with heterotaxy syndrome with excellent survival. However, morbidity in terms of postoperative atrioventricular valve regurgitation, arrhythmia, and pulmonary arteriovenous fistula remains significant.

• Journal of Korean Neurosurgical Society
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• v.29 no.12
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• pp.1555-1562
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• 2000

Objects : Surgical management of the distal anterior cerebral artery(DACA) aneurysms presents several unique problems to surgeons, such as difficulty in early identification of parent arteries, high incidence of rebleeding and premature rupture, and requirement of unfamiliar approach other than conventional frontotemporal craniotomy. Therefore, preoperative anatomical knowledge of anterior interhemispheric fissure and entry point of dissection is prerequisite. Authors utilized a frontobasal approach for DACA aneurysms by using consistent external landmark for guidance to the deep structure. Materials and Methods : From Nov. 1995 to Jun. 1999, a surgical clipping of DACA aneurysms was carried out in 9 patients among a total 131 patients with intracranial aneurysms. In each case, the clinical and aneurysmal features were carefully reviewed through the angiograms, medical records, and intraoperative findings. Results : The incidence of DACA aneurysms was 6.9% from our series. All cases were arisen from juxtacallosal por-tion ; 6 cases from pericallosal-callosomarginal(PC-CM) junction and 3 from pericallosal-frontopolar(PC-FP) junction. Associated vascular anomalies were noted in 3 cases and multiple aneurysms in 3 cases, respectively. The preoperative clinical grades were generally poor. An early surgery was performed in 7 cases and frontobasal interhemispheric approaches in 7 cases. Postoperatively, two patients died of complications ; one delayed ischemic vasospasm and one aspiration pneumonia but remaining patients recovered well. Conclusion : The frontobasal interhemispheric approach was useful for DACA aneurysms in early surgery. Division of superior sagittal sinus(SSS) enabled a minimal retraction of brain on both sides, and prevention of intraoperative rupture was possible. Authors suggest the frontopolar(first frontal bridging) vein as a constant external landmark for approaching the genu of the corpus callosum and juxtacallosal DACA aneurysms.

• Archives of Plastic Surgery
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• v.34 no.6
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• pp.671-678
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• 2007

Purpose: Venous malformation(VM) which often causes pain and discomfort is the most common type of vascular malformations. Although it is presented with disfigured appearance and associated soft tissue or skeletal hypertrophy, the molecular bases of VMs are poorly understood. Differentially expressed genes(DEGs) of VMs were investigated to illuminate the molecular mechanism of the disease entity. Methods: Gene expressions of VM patients' subcutaneous tissue were studied in comparison with normal persons' by $GeneFishing^{TM}$ technique using the annealing control primers (ACPs) to identify DEGs. Candidate genes were sequenced and screened by basic local alignment search tool (BLAST) afterwards. Results: Among seventy DEGs identified, forty DEGs which had shown significantly different expression pattern were sequenced. Twenty eight out of 40 were up-regulated while 12 were down-regulated. BLAST searches revealed that 37 were known genes and 3 were unknown genes. Many genes were involved in the differentiation and remodeling of smooth muscle cells, opposed to the previous hypothesis that a lot of angiogenetic genes would be involved. Furthermore, several transcription factors and related genes, as well as cell signaling and metabolism regulators, were up regulated. Conclusion: It suggests that analysis of DEGs in VMs provide basic knowledge about its pathophysiology. and new therapeutic approaches.

• Tuberculosis and Respiratory Diseases
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• v.66 no.4
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• pp.314-318
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• 2009

Hereditary hemorrhagic telangiectasia (HHT, also called Osler-Weber-Rendu Disease) is a rare systemic fibrovascular dysplasia characterized by recurrent epistaxis, cutaneous telangiectasia, and visceral arteriovenous malformations (AVMs). HHT is an autosomal dominant disease with a prevalence of 1 in 5,000~8,000. Recurrent epistaxis is often the first and most common manifestation, and about 30% of patients reveal pulmonary AVM. Presently, we report a familial case of HHT. A 61-year-old male with asymptomatic multiple pulmonary AVMs was successfully treated with embolization. His older brother who presented with recurrent epistaxis and multiple telangiectasias was treated with laser ablation. Their pedigree revealed a family history of recurrent epistaxis.

• Clinical and Experimental Pediatrics
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• v.53 no.4
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• pp.603-606
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• 2010

Paraneoplastic limbic encephalitis, a remote effect of cancer without nervous system metastasis, is rare, especially in childhood. Here, we report a case of paraneoplastic limbic encephalitis associated with an ovarian mature teratoma in an adolescent girl. The 15-year-old girl developed neuropsychiatric symptoms, memory loss, seizures, and unconsciousness. Cerebrospinal fluid analysis and brain magnetic resonance imaging (MRI) findings were normal, while single photon emission computed tomography imaging showed hypoperfusion in both temporal lobes. Ultrasound and MRI of the abdomen revealed a left ovarian cystic mass. The patient experienced a significant recovery of cognitive function after surgical resection of the tumor, which was pathologically identified as a mature ovarian teratoma, and treatment with intravenous immunoglobulin.

• Journal of Chest Surgery
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• v.39 no.11 s.268
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• pp.868-871
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• 2006

Nuss operation as a method for correction of pectus excavatum is safe and satisfactory, but its complication presents pneumothorax, displaced bar, wound infection, pericarditis, pleural effusion, hemothorax, cardiac injury etc. We report a rare case of acute ascending aortic rupture after displaced and adhered Nuss bar removal. The patient was a 30-years old man who received Nuss operation 3 years ago. Nuss bar was removed without resistance but massively bled at both operation wound, so immediately femoro-femoral CPB and median sternotomy was done and repaired proximal aortic arch under deep hypothermic total circulatory arrest. The patient was discharged without complication at postoperative 13 day.

• Journal of Chest Surgery
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• v.5 no.2
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• pp.107-112
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• 1972

Recently we experienced a case of the portal hypertension, extrahepatlc origin in the National Medical Center, Seoul. The case was a male aged 19 who was undergone the elective splenorenal shunt with splenectomy 9 years ago and emergency ligation of the coronary vein because of recurred variceal rupture 6 years later and had recurring esophageal varices with bleeding this time.At the age of 10 he had been occasionally suffering from nasal bleeding and visited to our Pediatric department, when there was encountered for the first time the splenomegaly, esophageal varices in the lower third esophagus on the esophagogram, and stenosis and kinking of the portal vein with rich collateral circulation on the splenoportography without hepatic functional impairment.The elective splenorenal shunt with splenectomy was undergone under the diagnosis of portal hypertension due to congenital anomaly of the portal vein and postoperatlvely no troubles had been obtained until postoperative 1st attack of massive hematemesis due to esophagenl variceal rupture recurred about 6 years later which was confirmed by control esophagogram and it was resulted by stenosis of previous anastomotic site of the splenorenal shunt.Then emergency ligation of the coronary vein was only made for bleeding control and no episodes of hematemesis had been encountered thereafter until April 1972 about 3 years after the 2nd operation, when hematemesis recurred again. In this time, recurring esophageal varices were noted in the lower third esophagus on the control esophagogram and he was employed side to end mesocaval shunt as the final step of portal decompression,and following results were obtained. 1] No postoperatlve troubles as leg edema or pain: Postoperatively leg elevation and elastic bandage on the both legs were employed until discharge. 2] During operation the portal pressure was 300 mm $H_2O$ and immediately lowered to 170 mm $H_2O$ after shunt.

• Journal of the Korean Society of Radiology
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• v.83 no.2
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• pp.372-377
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• 2022

Unilateral pulmonary vein atresia (PVA) is a rare congenital cardiovascular anomaly occurring after the common pulmonary vein fails to incorporate into the left atrium. It is most commonly diagnosed in childhood, and diagnosis after reaching adulthood is extremely rare. Dyspnea on exertion and hemoptysis are common clinical features in adult PVA patients, whereas lung parenchymal abnormalities are indirect signs of PVA, which can manifest as interstitial lung disease. Herein, we present the case of a 62-year-old female diagnosed with unilateral PVA presenting as unilateral interstitial lung disease and report the changes in her chest radiographs over 12 years.

• Journal of Chest Surgery
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• v.40 no.12
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• pp.811-816
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• 2007

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 ($4{\sim}75$ 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 ($1{\sim}95.2$ months). There was no early mortality, and one late non-cardiac related death. The overall rates of restored sinus rhythm before discharge and $3{\sim}6$ 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.

• Archives of Reconstructive Microsurgery
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• v.7 no.2
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• pp.146-156
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• 1998

Microvascular surgery has been widely used clinically for over 30 years. Although many types of free skin and myocutaneous flap are being used at present, surgeons are still looking for new flaps to suit the specific requirements of different recipient sites, to reduce the deformity at the donor site, to ease the management of the flap and to increase the success rate of those operations. The lateral thigh free flap was designed and reported simultaneously with the medial thigh free flap by Baek in 1983. The flap, based on the third perforator of the profunda femoris artery. is designed on the posterolateral aspect of the distal thigh. Clinically, the vascular variations and the locations of perforators of this system can be determined preoperatively with simple angiograms and Dopper audiometry. The lateral thigh free flap is suitable for reconstruction of defects in an oral floor with tongue and esophageal deficits, scalp defects with dural defects, and large full thickness defects of the lip. The advantages of this flap are safe elevation, a long vascular pedicles with a large lumen, skin that is generally thin, and good pliability. Furthermore, the skin territory is very wide and long. The donor site is hidden and therefore more acceptable to the patient. The disadvantage of this flap is that the anatomy of the pedicle vessels has irregular derivation from the main vessel. We had reconstructed lateral thigh free flap to the nine patients from January, 1997 to July, 1998 and got satisfactory results. In this paper we illustrate the arterial anatomy of the thigh and usefulness of this flap for the reconstruction of the head and neck.