• Advances in pediatric surgery
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• v.13 no.2
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• pp.144-154
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• 2007

Liver tumors in children are rare, relatively complex, and encompass a broad spectrum of disease processes. This study reviews our experience of liver tumors during the last 10 years. Medical records of 36 cases of liver tumors in children, treated at Samsung Medical Centers, from October 1994 to December 2005, were reviewed in this study. We analyzed disease characters and survival rates as a whole and by specific disease. The median age was 3.6 years. Male and female ratio was 1:1. The most common symptom was the palpable mass in 15 cases. Others were abdominal distension in 9 cases, jaundice in 2, vomiting in 2, weight loss in 2, and pubic hair growth in 1. CT or US and liver biopsy were performed for diagnosis. There were 28 malignant tumors: malignant rhabdoid tumor (1 case), hepatocellular carcinoma (3 cases), hemangioendothelioma type II (3 cases), angiosarcoma (1 case), and hepatoblastoma (20 cases). Eight tumors were benign; hepatic adenoma (1 case), focal nodular hyperplasia (2 cases), hemangioendothelioma type I (2 cases), mesenchymal hamartoma (3 cases). In this study the clinical characteristics were not different from the other reports. Liver transplantation was performed in 3 cases-1 with hepatoblastoma and 2 with hepatocelleular carcinoma. Accurate and early diagnosis, and individualized multimodality therapeutic approaches might be important for better outcome.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.4
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• pp.358-368
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• 2019

Purpose: Pediatric Crohn's disease (CD) is directly related to growth and has a high probability of requiring surgical intervention(s); therefore, more active treatment for CD is required for children. This study investigated the impact of biologics on growth and disease course associated with surgery. Methods: This was a retrospective cohort study involving patients diagnosed with CD at the Seoul National University Children's Hospital (Seoul, Korea) between January 2006 and October 2017. The aim was to determine the characteristics of pediatric patients with CD and whether biologics affected growth and the surgical disease course. Results: Among patients who underwent surgery for CD, the mean number of operations per patient was 1.89. The mean time from initial diagnosis to surgery was 19.3 months. The most common procedure was fistulectomy (34%), followed by incision and drainage (25%). In all patients, the use of biologics increased the height (p=0.002) and body mass index (BMI) (p=0.005). Among patients who underwent surgery, height (p=0.004) and BMI (p=0.048) were increased in the group using biologics. Patients who used biologics exhibited a low operation rate only within 2 years after diagnosis, with no differences thereafter (p=0.027). Conclusion: Although biologics could not mitigate the operation rate in pediatric patients who underwent surgery for CD, biological therapy delayed disease progression within 2 years of disease onset. Additionally, biologics conferred growth and BMI benefits in this window period. Therefore, it may be helpful to use biologics for optimal growth in pediatric patients with a high probability of undergoing future surgery.

• Journal of Chest Surgery
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• v.57 no.1
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• pp.79-86
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• 2024

Background: This study investigated the surgical outcomes associated with coronary artery fistulas (CAFs) in children. Methods: We retrospectively reviewed the medical records of 23 pediatric patients who underwent surgical closure of CAFs between 1995 and 2021. At presentation, 7 patients (30.4%) exhibited symptoms. Associated cardiac anomalies were present in 8 patients. Fourteen fistulas originated from the right coronary artery and 9 from the left. The most common drainage site was the right ventricle, followed by the right atrium and the left ventricle. The median follow-up duration was 9.3 years (range, 0.1-25.6 years) Results: The median age and body weight at repair were 3.1 years (range, 0-13.4 years) and 14.4 kg (range, 3.1-42.2 kg), respectively. Cardiopulmonary bypass was used in 17 cases (73.9%), while cardioplegic arrest was employed in 14 (60.9%). Epicardial CAF ligation was utilized in 10 patients (43.5%), the transcoronary approach in 9 (39.1%), the endocardial approach in 2 (8.7%), and other methods in 2 patients (8.7%). The application of cardioplegic arrest during repair did not significantly impact the duration of postoperative intensive care unit stay or overall hospital stay. One in-hospital death and 1 late death were recorded. The overall survival rate was 95.7% at 10 years and 83.7% at 15 years. A residual fistula was detected in 1 patient. During the follow-up period, no surviving patient experienced cardiovascular symptoms or coronary events. Conclusion: Surgical repair of CAF can be performed safely with or without cardioplegic arrest, and it is associated with a favorable prognosis in children.

• Journal of Chest Surgery
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• v.48 no.4
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• pp.272-276
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• 2015

Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder that is characterized by aggressive arterial and aortic disease, often involving the formation of aortic aneurysms. We describe the cases of two children with LDS who were diagnosed with aortic root aneurysms and successfully treated by valve-sparing aortic root replacement (VSRR) with a Valsalva graft. VSRR is a safe and suitable operation for children that avoids prosthetic valve replacement.

• Journal of Chest Surgery
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• v.54 no.3
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• pp.214-217
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• 2021

In rare cases, levoatrial cardinal vein may occur as an isolated condition without additional congenital anomalies. Depending on the direction and flow of the shunt, this pathology may produce symptoms; alternatively, it may be asymptomatic, as in the case presented in this study. In asymptomatic cases, complications, such as paradoxical embolism and brain abscess, can arise later. In the 11-year-old patient whose case is presented here, the levoatrial cardinal vein was asymptomatic and incidentally detected. The percutaneous closure method was applied first. However, by 16 hours after the procedure, the occluder device had embolized to the iliac artery. Emergency surgery was performed; first, the occluder device was removed, and levoatrial cardinal vein ligation was then performed via a mini-thoracotomy. The symptoms, diagnosis, and treatment modalities of isolated levoatrial cardinal vein are discussed in the context of this case described herein.

• Advances in pediatric surgery
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• v.1 no.2
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• pp.177-180
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• 1995

A 2-day-old male (Premie, Large for gestational age(LGA), Intrauterine period(IUP) 33 weeks, birth weight 2,955 gram) was transferred with marked abdominal distention, bilious return via the orogastric tube, respiratory difficulty, and generalized edema (hydrops fetalis). He was born by cesarean section to a 36 year-old mother. Antenatal ultrasonogram at IUP 31 weeks demonstrated multiple dilated bowel loops suggestive of intestinal obstruction. There was no family history of cystic fibrosis. Simple abdominal films disclosed diffuse haziness and suspicious fine calcifications in the right lower quadrant. Barium enema demonstrated a microcolon. Sweat chloride test was not available in our institution. At laparotomy, there noted 1) a segmental volvulus of the small bowel with gangrenous change, associated with meconium peritonitis, 2) an atresia of the ileum at the base of the volvulus, and 3) the terminal ileum distal to the volvulus was narrow and impacted with rabbit pellets-like thick meconium. These findings appeared to be very similar to those of a complicated meconium ileus. In summary, the ileal atresia and meconium peritonitis seemed to be caused by antenatal segmental volvulus of the small intestine in a patient with probable meconeum ileus.

• Advances in pediatric surgery
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• v.5 no.2
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• pp.111-115
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• 1999

The results of hepatic portojejunostomy in 34 patients with biliary atresia operated upon by one surgeon between May 1989 and December 1997 were analyzed. Eleven (32.3 %) patients were 60 days or younger, 14 patient (41.2 %) were between 60 and 90 days, and 9 (26.5 %) were over 90 days of age. Jaundice cleared in 20 cases (58.8 %). Three patients died of liver insufficiency, 2 were anicteric but died from esophageal variceal bleeding. Three patients died as a result of sepsis, heart failure and left kidney agenesis. Five patients were lost to follow-up. The five-year survival rate was 73.8 %. Two patients over 90 days of age, survived more than 5 years. Survival rates were not significantly related to the age at operation. We conclude that hepatic portojejunostomy should be considered as a primary surgical modality for biliary atresia, even at age 90 days or more. Early detection of esophageal varices and sclerotherapy may be necessary. Liver transplantation is necessary if hepatic failure develops.

• Advances in pediatric surgery
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• v.1 no.1
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• pp.46-52
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• 1995

Percutaneous infraclavicular subclavian catheterization has been widely used for a total parenteral nutrition, hemodynamic monitoring and for venous access in difficult clinical situations. Many authors have claimed the infraclavicular cannulation of the subclavian vein in the tiniest infants can be performed with safety and ease, but there are always possibility of serious complications in this method. We present our experiences of peripheral venous cutdown with Broviac catheter. Author routinely introduced Broviac catheter into central vein via peripheral venous cutdown. There was no life threatening complications and no catheter related death. The complication rate was very low. The catheter related sepsis was documented in only two patient(4.7%). The average catheter longivity was 19.59 days. In view of the safety and low rate of complication, we think that peripheral venous cutdown with Broviac catheter should be the method of choice when central venous access is necessary in infants. The infraclavicular subclavian catheterization should be reserved in infants with few accessible peripheral vein.

• Advances in pediatric surgery
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• v.4 no.1
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• pp.74-78
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• 1998

Early recognition and surgical treatment of Hirschsprung's disease prevents serious mortality and morbidity from enterocolitis and obstruction. Usually this disease is characterized by a single aganglionic segment of the colon extending distally to the anal margin. In surgical treatment, the surgeon performs a frozen section biopsy to confirm whether there are ganglion cells. If there are intervening ganglionic sites in aganglionic bowel, there may be confusion in diagnosis and treatment. The authors experienced one case of total colonic aganglionosis with skip area. A transverse loop colostomy was performed on a 7 day-old male baby with colon perforation due to Hirschsprung's disease. But intestinal obstruction persisted and required two more operations to find the true nature of the disease. There were aganglionic segments from the anal margin to the terminal ileum 3.7cm proximal to the ileocecal valve. The entire transverse colon and appendix were normally ganglionated.

• Journal of Chest Surgery
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• v.48 no.4
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• pp.277-280
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• 2015

We report the case of a 37-year-old man who suffered from biventricular failure due to left isomerism, inferior vena cava interruption with azygos vein continuation, bilateral superior vena cava, double outlet of right ventricle, complete atrioventricular septal defect, pulmonary stenosis, and isolated dextrocardia. Heart transplantation in patients with systemic venous anomalies often requires the correction and reconstruction of the upper & lower venous drainage. We present a case of heart transplantation in a patient with left isomerism, highlighting technical modifications to the procedure, including the unifocalization of the caval veins and reconstruction with patch augmentation.