• Archives of Craniofacial Surgery
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• v.13 no.1
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• pp.54-56
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• 2012

Purpose: Dandy-Walker syndrome is a rare congenital brain malformation that occurs one in every 25,000-35,000 live births, mostly in females. It is characterized by cystic enlargement of the fourth ventricle, agenesis or hypogenesis of the cerebellar vermis and enlargement of the posterior fossa. In this report, the authors aimed to address a rare case of a 14-months-old female Dandy-Walker syndrome patient that is presented with submucous cleft palate. Methods: A 14-months-old female patient admitted to our outpatient clinic, via the department of pediatrics, with the complaints of nasal regurgitation, choking and breathing difficulties. She was diagnosed as Dandy-Walker syndrome by magnetic resonance imaging evaluation, at another hospital and underwent a shunt operation for the hydrocephalus continuing treatments. On physical examination, she had structural abnormality of bifid uvula, and palpable notch in the posterior surface of the hard palate. Her submucous cleft palate was corrected, which used a double opposing Z-plasty under general anesthesia. Results: In a follow-up period of 2 months, no complications, such as wound dehiscence, necrosis and infection occurred, which shows satisfactory results. She consulted with pediatric neurologists and physical therapists for further evaluation and management of the abnormalities in the central nervous system. Conclusion: Dandy-Walker syndrome patient with a cleft palate is a very rare case to find, which only a few cases are reported around the world. Authors would like to share this case of Dandy-Walker syndrome patient, with submucous cleft palate, who underwent a double opposing Z-plasty that shows satisfactory results.

• Journal of Yeungnam Medical Science
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• v.19 no.2
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• pp.99-106
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• 2002

Background: Simple ventricular septal defect(VSD) is the most common congenital heart disease. Although closure of VSD is currently associated with a relatively low risk, experience with younger and smaller infants has been variably less satisfactory. We assessed the results of surgical closure of VSD in infant. Materials and Methods: Between 1996 and 2000, 45 non-restrictive VSD patients underwent patch repair and retrospective analysis was done. Patients were divided into two groups based on weight: group I infants weighed 5kg or less(n=16), and group II infants weighed more than 5kg(n=29). Both groups had similar variation in sex, VSD location, aortic cross clamp time and total bypass time. But combined diseases (ASD, PDA, MR) were more in group I. We closed VSD with patch and used simple continuous suture method in all patients. Results: There were no operative mortality, no reoperation for hemodynamically significant residual shunt and no surgically induced complete heart block. As a complication, pneumonia(group I: 2 cases, group II: 2 cases), transient seizure(group II: 2), wound infection(group I: 1, group II: 1), urinary tract infection(group I: 1) and chylopericardium(group I: 1) developed, and there was no significant difference between two groups(p>0.05). Conclusion: Early primary closure with simple continuous suture method was applicable in all patients with non-restrictive VSD without any serious complications.

• Journal of Chest Surgery
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• v.31 no.10
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• pp.1004-1008
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• 1998

We report a case of heart-lung transplantation in a 32 year-old female with Eisenmenger syndrome secondary to patent ductus arteriosus. She has been suffered from congestive heart failure since June 1996 and repeatedly treated at Intensive Care Unit with intravenous inotropic support since July 1997. Preoperative echocardiography showed a patent ductus arteriosus with right to left shunt, severe regurgitation of tricuspid valve and estimated right ventricular systolic pressure of 100mmHg. The brain-dead donor was an 18 year-old male with head trauma from traffic accident 3 days ago. Heart-lung block procurement was performed at another general hospital and was transported to the Seoul National University Hospital by ambulance. Total ischemic time of the transplanted heart and lung were 249 minutes and 270 minutes, respectively. The immunosuppressive therapy was commenced preoperatively with cyclosporine and azathioprine. Corticosteroid was not used until postoperative 3 weeks in order to avoid infection and delayed healing at the tracheal anastomotic site. The patient was discharged at 31st postoperative day, and has been regularly followed up at outpatient clinic without specific complication. The follow-up bronchoscopy, performed 2 weeks and 4 months after surgery, revealed no evidence of cellular rejection.

• Advances in pediatric surgery
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• v.1 no.1
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• pp.18-26
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• 1995

For the prevention of later contralateral hernia as well as unnecessary contralateral exploration in pediatric patients with unilateral inguinal hernias, a reasonable indication of contralateral exploration is required. To examine the contralateral positivity, a prospective selective contralateral exploration has been performed by the author from Sept. 1985 to Dec. 1993, at Pediatric Surgical Section of the Department of Surgery, Kangnam St. Mary's Hospital, Catholic University Medical College. Among the total 1200 cases of pediatric inguinal hernias, 580 cases of contralateral side were explored at hernia operations, by the indications as; male with infant onset, 2)female of all age, 3)prematurity, 4)profuse ascites due to cirrhosis, nephrotic syndrome, and ventriculoperitoneal shunt, and 5)remarkable silk sign. Overall positive rate was 71.4%, and positive rates of each indication were 80.7%, 70.4%, 73.1%, 66.7%, and 72.0%, respectively. Right side hernia showed 67.0%, left s ide 75.7%, and positive familial history 71.8% of contralateral positivities. In male, getting older revealed lower positive rates and the rate suddenly dropped after 12 years of age. Birth order, mother's age at delivery, postmaturity did not show any significant differences between the rates. Recurrence was seen in 3(0.5%) ipsilateral and 2(0.3%) contralateral, both of which were negative esplorations on previons operations. Overall complication rate was 3.8%, including 1 infection, 14 fluid or blood accumulation, 5 edemas, 3 temporary testicular edemas, 2 persisting fevers, 2 enures is and one delayed recovery from anesthesia. Among 38 cases with contralateral hernias developed after unilateral surgery by authors(6 cases) or surgeons in other institutions, 14 were males with infant onset, 4 were prematurities and 9 were females. Therefore, 27(71.7%) cases were originally under the contralateral exploration indications. The primary site of the hermia was right in 25 and left in 13. With above results, the following indications for contralateral exploration could be suggested ; 1)under one year of age, both sex, 2)prematurity, 3) remarkable silk sign, 4)in the double checked suspicions among males with infant onset, all age females, ascites, left hernia and familial history. After 12 years of age, exploration is not required. Considering complications, contralateral explorations could be considered only in the following situations; 1)expert, experienced pediatric surgeon, 2)experienced pediatric anesthesiologist, 3)operations could be done smoothly in an hour, 4)good general condition of the patient.

• Journal of Chest Surgery
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• v.25 no.11
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• pp.1318-1326
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• 1992

Backgroud. To determine the influence of age at operation upon surgical outcome in patients with isolated secundum atrial septal defect, retrospective clinical analysis was done. Material and method. From June, 1976 to December, 1991, 146 patients, 63 male and 83 female patients ranging in age from 13 months to 56 years, were operated on for isolated secundum atrial septal defect. The patients were divided into 3 groups according to their age at operation: Group I [<20 years old], 91 patients[62.3%]; Group II [21 to 40 years old], 44 patients[30.1%]; GroupIII[>41 years old], 11 patients[7.6%]. Significant differences in clinical features, hemodynamic function, early and late results between age groups were speculated. Results. One hundred thirty-one patients[89.6%] were symptomatic at the time of operation, the most common symptoms being dyspnea on exertion, recurrent respiratory infection, palpitation and chest pain. Patients in NYHA class III or IV were 3.3% in group I, 25% in groupIII, and 54.5% in group Ill. Hemodynamic data was available for 138 patients [94.5%]. Significant pulmonary hypertension [MPA systolic pressure $^3$ 40mmHg] was noted in 22 patients [15.9%]. Patients with pulmonary vascular disease [Rp/Rs>1.25] were 2% in group I, 7.3% in group Il, and 9.1% in groupIII. But there were no significant differences between the age groups in the size of the shunt or the ratio of pulmonary to systemic flow. Atrial septal defects were closed with direct suture in 144 patients and patch repair was performed in 2 patients with high defect. Atrial arrhythmia [8.2%] was the most common postoperative complication. The mean [LSD] duration of follow-up in all patients was 16$\pm$22 months [range, 1~96 months]. Functional result was excellent regardless of the age groups. During follow-up period, late cardiovascular events were arrhythmia [7 cases], reoperation for recurrent ASD [2 cases], and premature late death due to bacterial endocarditis [1 case]. Incidence of preoperative and late atrial fibrillation was significantly higher in older age group. Conclusion. Age at operation is one of the most important predictor of early and late surgical outcome with its impact on the following factors : 1] hemodynamic alterations and ventricular dysfunction due to longstanding volume and pressure overload, 2] pulmonary vascular disease, and 3] atrial arrhythmia including atrial fibrillation as a result of atrial dilatation. Therefore, among patients with surgically repaired atrial septal defects, those operated on over the age of 20 require careful supervision on the long-term basis.

• Clinical and Experimental Pediatrics
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• v.50 no.7
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• pp.665-671
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• 2007

Purpose : The purpose of this study was to investigate the clinical features and outcome in newborns undergoing cardiac surgery. Methods : Eighty two neonates underwent heart surgery for congenital heart defect at Kyungpook National University Hospital between March 2000 and February 2006. Patient characteristics (sex, age, diagnosis), pre-operative conditions, operation type, postoperative complications and mortality were reviewed retrospectively. Results : In 82 patients, 41 (50%) were male. The mean age and weight at operation were 12 days and 3.2 kg, respectively. The common cardiac anomalies were complete transposition of the great arteries (TGA), Tetralogy of Fallot (TOF), pulmonary atresia with intact ventricular septum, and single ventricle variants. Fifty seven operations were performed with cardiopulmonary bypass and corrective surgery was done on 54 patients. Arterial switch operation and modified Blalock-Taussig shunt were most frequently performed as corrective and palliative operations, respectively. The early hospital mortality rate was 7%; the late mortality was 3.9%. Complications were acute renal insufficiency, delayed sternal closure, wound infection, arrhythmia, and brain hemorrhage. Conclusion : During the last 6 years, the outcomes of cardiac surgery for congenital heart defects in neonates improved by progress in perioperative, anaesthetic, surgical, and postoperative care.

• Journal of Biomedical Engineering Research
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• v.19 no.2
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• pp.163-170
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• 1998

The ultimate goal of total artificial heart is permanent substitute for a failed heart in a patient without any other therapeutic modality. Until now, infection has been the main problem related to the mechanical circulatory support system. The best way to solve this catastrophic complication and to improve the quality of life of TAH patients in terms of tethering must be implantation of TAH totally. The EH-TAH has been developed in NCVC from 1987 for this purpose. The system consists of an energy converter and pumps, which are designed to be placed in abdomen and pericardial space separately for a good anatomical fit. To evaluate the anatomical fit and hemodynamic performance of the EH-TAH, in vivo test was done. General condition of the animal and hemodynamic status had been stable until the TAH stopped on the 11th pumping day. The estimated cardiac output was about 7.7L/min. The values of mean aortic pressure, left and right atrial pressure were 93$\pm$10, 19$\pm$3 and 15$\pm$4 mmHg, respectively. The correlation coefficient between left and right atrial pressure was 0.96, which represents the dynamic function of the interatrial shunt in controlling left-right imbalance of cardiac output. During pumping days, the temperature on the surface of actuator had been maintained at 39.7$\pm$0.4$^{\circ}C$, less than 1$^{\circ}C$ higher than the rectal temperature. The TAH stopped on the 11th day due to mechanical problems. We concluded that the EH-TAH possessed satisfactory basic performance including anatomic fit and hemodynamic adequacy, although there were several mechanical problems to be solved yet.

• Journal of Chest Surgery
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• v.36 no.8
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• pp.559-565
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• 2003

By improving the flow pattern in Fontan circuit, total cavopulmonary connection (TCPC) could result in a better outcome than atriopulmonary connection Fontan operation. For the patients with impaired hemodynamics after atriopulmonary Fontan connection, conversion to TCPC can be expected to bring hemodynamic and functional improvement. We studied the results of the revision of the previous Fontan connection to TCPC in patients with failed Fontan circulation. Material and method: From October1979 to June 2002, eight patients who had failed Fontan circulation, underwent revision of previous Fontan operation to TCPC at Yonsei University Hospital. Intracardiac anomalies of the patients were tricuspid atresia (n=4) and other functional single ventricles (n=4). Mean age at TCPC conversion was 14.0$\pm$7.0 years (range, 4.6~26.2 years) and median interval between initial Fontan operation and TCPC was 7.5 years (range, 2.4~14.3 years). All patients had various degree of symptoms and signs of right heart failure. NYHA functional class was 111 or IV in six patients. Paroxysmal atrial fibrillation (n:f), cyanosis (n=2), intraatrial thrombi (n=2), and protein losing enteropathy (PLE) (n=3) were also combined. The previous Fontan operation was revised to extracardiac conduit placement (n=7) and intraatrial lateral tunnel (n=1). Result: There was no operative death. Major morbidities included deep sternal infection (n=1), prolonged pleural effusion over two weeks (n=1), and temporary junctional lachyarrhythrnia (n=1). Postoperative central venous Pressure was lower than the preoperative value (17.9$\pm$3.5 vs. 14.9$\pm$1.0, p=0.049). Follow-up was complete in all patients and extended to 50,1 months (mean, 30.3$\pm$ 12.8 months). There was no late death. All patients were in NYHA class 1 or 11. Paroxysmal supraventricular tachycardia developed in a patient who underwent conversion to intraatrial lateral tunnel procedure, PLE was recurred in two patients among three patients who had had PLE before the convertsion. There was no newly developed PLE. Conclusion: Hemodynamic and functional improvement could be expected for the patients with Fontan circulatory failure after atriopulmonary connection by revision of their previous circulation to TCPC. The conversion could be performed with low risk of morbidity and mortality.

• Journal of Chest Surgery
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• v.29 no.2
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• pp.177-184
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• 1996

Between 1987 and 1994, 21 patients were treated surgically for aortic aneurysm involving the thoracoabdominal aorta. There were 11 males and 10 females, and their age ranged from 20 to 67 years old and mean age was 41.5 years. Many complained of back pain, chest pain or discomfort, and flank pain, but three patients were asymptomatic. 15 patients had chronic dissection (71.4%) and 6 had nondissecting fusiform or saccular aneurysm(28.5%), and of those 15 patients with chronic dissection, 6(28.5%) had atherosclerosis assniated with hypertension, 5 (23.8%) were Martian syndrome, and 2 (9.5%) were associated with pregnancy. The diameter of an aneurysm ranged from 6cm to 12cm, and their extent was classified as type I in 7(33.3%), type II in 8(38.1%), type III in 3(14.3%), and type IV in 3(14.3%) patients based on Crawford classification for TAA . Diseased aorta was replaced with artificial vascular graft in all but one patient. In whom the aortic tear site due to pseudoaneurysm was closed by primary suture. For the spinal cord protection during the operation, we used partial cardiopulmonary bypass (FV-FA or PA-FA bypass) in 12 patients (57.1%), Biopump (LA-FA bypass) in 4(19.0%), total circulartory arrest and CPB in 2 (9.5%), Gott's heparinized shunt in 1(4.7%), and simple aortic cross clamping in 2 (9.5%). The most common complication after the operation was hoarseness due to unilateral vocal cord palsy which onured in 5 patients (23.8%), and the next common complication was wound infection in 4 patients(19.0%), paraplegia in 2 patients (9.5%), chylothorax in 1 patient(4.7%). The hospital mortality rate was 9.5% (2deaths), and there was no late death. Our experience shows that the graft replacement of TAAA had reasonable rate of mortality, low rate of serious complication, and provided good post operative state of the pati nts, and since the thoracoabdominal aortic operation is not a high risky procedure anymore, we recommend a radical operation for the indicated patients.