• Clinical and Experimental Pediatrics
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• 제48권6호
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• pp.660-664
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• 2005

Infantile hemangioendothelioma(IHE) of the liver is the most common vascular tumor in infants before the age of 6 months. It is a histologically benign tumor with potentially life-threatening complications. The clinical manifestations are variable, ranging from asymptomatic forms to intractable high-output heart failure. In addition, abdominal mass, intraperitoneal hemorrhage due to rupture of mass, respiratory distress, hematologic abnormalities and jaundice can occur. Diagnostic work-up is through doppler ultrasound sonography, computed tomography scan, magnetic resonance imaging and angiography. Treatment consists of medical treatment, interventional therapy, surgical resection and liver transplantation. We experienced symptomatic IHE in a premature neonate who presented with high output heart failure and respiratory distress. Initial medical treatment and steroid therapy failed to improve his condition. Coil embolization of left hepatic artery resulted in improvement of respiratory symptoms. However, a left lobectomy was performed because the mass size was not decreased with development of collateral vessels. The infant was well, after a successful discharge from the hospital.

• Journal of Chest Surgery
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• 제27권12호
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• pp.977-983
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• 1994

From April 1989 to December 1993, total 39 patients who were unable to be weaned off CPB or expected fatal immediate postoperiatively, were treated with ventricular assist device [VAD] or extracorporeal membrane oxygenator[ECMO] at the Royal Children`s Hospital, Melbourne. Ages ranged from 3 day to 19.4 year old and body weights from 2.0Kg to 70Kg. Twenty-seven[69.2%] of 39 patients were weaned to be decannulated successfully and sixteen[41.0%] survived to hospital discharge and late survival rate was twelve[30.8%] of 39 patients. The total follow-up period was 4 to 56 months [32.92$\pm$20.77months] and most of the late survivals showed good myocardial recovery state. From the viewpoint of the assist modality, 29 patients were treated with VAD and among them, 23 were weaned from assist successfully, but among the 8 ECMO patients, only 3 could be weaned, and both modalities were performed to the 2 patients with one weaned. The total duration of assist was from 8 to 428 hours and there was a significant difference between hospital discharged group and hospital death group, which were 83.13$\pm$31.29 hours vs 147.52$\pm$112.03 hours[P=0.032]. Conclusively, at the critical postcardiotomy situation of the paediatrtic patients including various congenital complex disease and procedures, we can choose this VAD or ECMO treatment strategy as the reasonable life saving way except transplantation.

• Journal of Chest Surgery
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• 제30권3호
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• pp.247-252
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• 1997

Ten acute and chronic in vivo studies were utilized to develop a pneumatic ventricular assist device (VAD) as a bridge to heart transplantation or a circulatory assist device for patients with end-stage heart disease or poor myocardial funct on after cardiac surgery. Two sizes of blood pump of 70cc for adult patients and 34cc for pediatric patients were implanted in the animals. Ventricle of the blood pump was made from the polyurethaae to enhance antithrombogenecity. The VAD was implanted between the left atrium and the descending aorta. Average flow rate was 2.38 L/min for adult and 0.41 L/min for pediatric VAD at the rate of 60 bum. The duration of support ranged from 1 to 26 hours. The most frequent complication was bleeding. Main causes of death were heart failure and respiratory failure. The device function was good for short term use. Studies to date suggest that, with further refinement, a reliable long term VAD that will have clinical application can be developed.

• Archives of Plastic Surgery
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• 제39권5호
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• pp.483-488
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• 2012

Background Pediatric hand burns are a difficult problem because they lead to serious hand deformities with functional impairment due to rapid growth during childhood. Therefore, adequate management is required beginning in the acute stage. Our study aims to establish surgical guidelines for a primary full-thickness skin graft (FTSG) in pediatric hand burns, based on long-term observation periods and existing studies. Methods From January 2000 to May 2011, 210 patients underwent primary FTSG. We retrospectively studied the clinical course and treatment outcomes based on the patients' medical records. The patients' demographics, age, sex, injury site of the fingers, presence of web space involvement, the incidence of postoperative late deformities, and the duration of revision were critically analyzed. Results The mean age of the patients was 24.4 months (range, 8 to 94 months), consisting of 141 males and 69 females. The overall observation period was 6.9 years (range, 1 to 11 years) on average. At the time of the burn, 56 cases were to a single finger, 73 to two fingers, 45 to three fingers, and 22 to more than three. Among these cases, 70 were burns that included a web space (33.3%). During the observation, 25 cases underwent corrective operations with an average period of 40.6 months. Conclusions In the volar area, primary full-thickness skin grafting can be a good indication for an isolated injured finger, excluding the web spaces, and injuries of less than three fingers including the web spaces. Also, in the dorsal area, full-thickness skin grafting can be a good indication. However, if the donor site is insufficient and the wound is large, split-thickness skin grafting can be considered.

• Journal of Chest Surgery
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• 제32권3호
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• pp.299-302
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• 1999

Heart transplantation was planned for a 10-year old boy who had dilated cardiomyopathy with severe congestive heart failure and had been on dopamine for 1month. However, partial left ventriculectomy and mitral annuloplasty were performed instead, because there was no donor heart of the adequate size and the symptoms were aggravated. The clinical symptoms were markedly improved after the surgery. Comparing the postoperative echocardiographic results with the preoperative results, there were remarkable changes in the left ventricular ejection fraction(preoperative LV EF 17% to postoperative 3 months 29%, 6 months 35%, 1 year 36%) and the left ventricular end-diastolic dimension(preoperative 72 mm to postoperative 3 months 59 mm, 6 months 61 mm, 1 year 61 mm). Partial left ventriculectomy and mitral annuloplasty reduced the cardiac loading in the dilated cardiomyopathy. Partial left ventriculectomy and mitral annuloplasty may be considered as one of the alternative surgical metho s to carry over until a heart transplantation can be performed, especially for children.

• Journal of Chest Surgery
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• 제56권3호
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• pp.162-170
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• 2023

Background: Extracorporeal cardiopulmonary resuscitation (E-CPR) plays an indispensable role when resuscitation fails; however, extracorporeal life support (ECLS) in infants is different from that in adults. The objective of this study was to evaluate the outcomes of E-CPR in infants. Methods: A single-center retrospective study was conducted, analyzing 51 consecutive patients (age <1 year) who received E-CPR for in-hospital cardiac arrest between 2010 and 2021. Results: The median age and body weight was 51 days (interquartile range [IQR], 17-111 days) and 3.4 kg (IQR, 2.9-5.1 kg), respectively. The cause of arrest was cardiogenic in 45 patients (88.2%), and 48 patients (94.1%) had congenital cardiac anomalies. The median conventional cardiopulmonary resuscitation (C-CPR) time before the initiation of ECLS was 77 minutes (IQR, 61-103 minutes) and duration of ECLS was 7 days (IQR, 3-12 days). There were 36 in-hospital deaths (70.6%), and another patient survived after heart transplantation. In the multivariate analysis, single-ventricular physiology (odds ratio [OR], 5.05; p=0.048), open sternum status (OR, 8.69; p=0.013), and C-CPR time (OR, 1.47 per 10 minutes; p=0.021) were significant predictors of in-hospital mortality. In a receiver operating characteristic curve, the optimal cut-off of C-CPR time was 70.5 minutes. The subgroup with early E-CPR (C-CPR time <70.5 minutes) showed a tendency for lower in-hospital mortality tendency (54.5% vs. 82.8%, p=0.060), albeit not statistically significant. Conclusion: If resuscitation fails in an infant, E-CPR could be a life-saving option. It is crucial to improve C-CPR quality and shorten the time before ECLS initiation.

• Clinical and Experimental Pediatrics
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• 제58권10호
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• pp.392-397
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• 2015

Purpose: Alagille syndrome is a complex hereditary disorder that is associated with cardiac, hepatic, skeletal, ocular, and facial abnormalities. Mutations in the Notch signaling pathway, such as in JAG1 and NOTCH2, play a key role in embryonic development. A cardiac or hepatic presentation is a critical factor for determining the prognosis. Methods: We conducted a retrospective study of 41 patients with Alagille syndrome or a JAG1 mutation between 1983 and 2013. Results: The first presentations were jaundice, murmur, cyanosis, and small bowel obstruction at a median age of 1.0 months (range, 0-24 months). The JAG1 mutation was found in 27 of the 28 genetically-tested patients. Cardiovascular anomalies were identified in 36 patients, chronic cholestasis was identified in 34, and liver transplantation was performed in 9. There was no significant correlation between the severity of the liver and cardiac diseases. The most common cardiovascular anomaly was peripheral pulmonary stenosis (83.3%), with 13 patients having significant hemodynamic derangement and 12 undergoing surgical repair. A total bilirubin level of >15 mg/dL with a complex surgical procedure increased the surgical mortality (P=0.022). Eight patients died after a median period of 2.67 years (range, 0.33-15 years). The groups with fetal presentation and with combined severe liver and heart disease had the poorest survival (P<0.001). Conclusion: The group with combined severe liver and heart disease had the poorest survival, and a multidisciplinary approach is necessary to improve the outcome.

• Journal of the Korean Society of Radiology
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• 제83권5호
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• pp.1014-1031
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• 2022

The Kasai portoenterostomy is the first-line treatment for the restoration of the flow of bile to the small intestine in patients with biliary atresia. Various complications can occur after Kasai portoenterostomy, including ascending cholangitis, biliary cirrhosis, and portal hypertension. Of these potential complications, ascending cholangitis in the most common. In cases of patients having uncontrolled complications due to progressive liver cirrhosis, portal hypertension, or progressive hyperbilirubinemia, liver transplantation is the indicated as treatment plan. Lifelong follow-up, particularly involving imaging studies, is important for the identification of various complications arising from biliary atresia after Kasai portoenterostomy. Additionally, imaging studies play a crucial role in the evaluation of potential liver donors and recipients. US is a key imaging modality utilized in the management of patients who undergo Kasai portoenterostomy, while CT and MRI are imperative to obtaining an accurate diagnosis.