Joong Kee Youn

Hepatocellular carcinoma after the Fontan procedure in a 16-year-old girl: A case report

Introduction: The Fontan procedure (FP) has become the standard operation for patients with single ventricle physiology. However, a long period of elevated systemic venous pressure and low cardiac output after the procedure result in chronic inflammation and liver cirrhosis, which may eventually lead to the occurrence of hepatocellular carcinoma (HCC). Clinical Findings: We described the case of a 16-year-old female who developed HCC after the FP. At 21 months, the patient received a lateral tunnel FP, and 14 years later, she began complaining of abdominal distension, telangiectasia, and fatigue. Imaging studies revealed a large hepatic mass involving most of the right lobe and multiple masses in the left lobe. Evidence suggested severe liver cirrhosis, and the presence of ascites, hepatosplenomegaly, paraesophageal gastric varices reflecting the severity of the disease. In addition, tumor thrombosis was found in the right hepatic vein, middle hepatic vein, and inferior vena cava, as well as multiple metastatic nodules in both lungs. The patient received an incisional biopsy and the diagnosis of HCC was pathologically confirmed. After treatment with 1 cycle of systemic chemotherapy, she received ongoing supportive care for disease-related complications, and died 2 months after chemotherapy due to hematemesis. Conclusion: With the advances in medicine, the incidence of Fontan physiology-related complications is likely to increase, and the incidence of HCC will also increase accordingly. As early diagnosis of HCC results in better patient outcomes, a surveillance guideline for HCC after the FP should be developed.

Surgical Treatment of Infected Aortoiliac Aneurysm.

Purpose: Infected aneurysms of the abdominal aorta or iliac artery (IAAA) are rare but fatal and difficult to treat. The purpose of this study was to review the clinical presentations and outcomes of IAAA and to establish a treatment strategy for optimal treatment of IAAA. Materials and Methods: Electronic medical records of 13 patients treated for IAAA at Seoul National University Hospital between March 2004 and December 2012 were retrospectively reviewed. Results: Mean age was 64.2 (median 70, range 20–79) years. Aneurysms were located in the infrarenal aorta (n=7), iliac arteries (n=5), and suprarenal aorta (n=1). Seven patients underwent excision and in situ interposition graft, 3 underwent extra-anatomical bypass, and 1 underwent endovascular repair. One patient with endovascular repair in an outside hospital refused resection, and only debridement was done, which revealed tuberculosis infection. One staphylococcal infection was caused by iliac stenting. Mycobacterium was the most common pathogen, followed by Klebsiella, Salmonella, and Staphylococcus. There were 3 in-hospital mortalities and the causes were sepsis in 2 and aneurysm rupture in 1. The 3 extra-anatomic bypasses were all patent after 5-year follow-up. Conclusion: IAAA develops from various causes and various organisms. IAAA cases with gross pus were treated with extra-anatomic bypass, which was durable. In situ reconstruction is favorable for long term-safety and efficacy, but extensive debridement is essential.

Pediatric split liver transplantation after Fontan procedure in left isomerism combined with biliary atresia: A case report

LI is a subset of the heterotaxy syndrome and a rare birth defect that involves the heart and other organs. It can be combined with extracardiac abnormalities, especially BA. CHD can be associated with LI in up to 15% of cases, although it is rare in BA. Pediatric LT for a child with ESLD due to BA combined with LI and CHD is a challenging issue for a transplant surgeon. Herein, we report a successful split LT on a three‐yr‐old boy with LI who survived after a Fontan procedure due to single ventricle, but who suffered from HPS associated with BA.

Isolated cystic duct cyst with associated stones in a 4-month-old boy

Isolated cystic duct cysts are rare entities, with few cases having been reported. We present the case of a 4-month-old male patient presenting with abdominal pain and vomiting. Ultrasonography and magnetic resonance cholangiopancreatography revealed an isolated cystic duct cyst with associated stones. The patient underwent open cholecystectomy with complete cyst excision and cystic duct transection; there were no postoperative complications. While lesions like the one described herein are extremely rare, they should be included as a separate category in classifications of choledochal cysts.

Abdominal tumors in children: Comparison between minimally invasive surgery and traditional open surgery.

AbstractThe use of minimally invasive surgery (MIS) in pediatric patients has been steadily increasing in recent years. However, its use for diagnosing and treating abdominal tumors in children is still limited compared with adults, especially when malignancy is a matter of debate. Here, we describe the experience at our center with pediatric abdominal tumors to show the safety and feasibility of MIS.Based on a retrospective review of patient records, we selected for study those pediatric patients who had undergone diagnostic exploration or curative resection for abdominal tumors at a single center from January 2010 through August 2015.Diagnostic exploration for abdominal tumors was performed in 32 cases and curative resection in 173 cases (205 operations). MIS was performed in 11 cases of diagnostic exploration (34.4%) and 38 cases of curative resection (21.9%). The mean age of the children who underwent MIS was 6.09 ± 5.2 years. With regard to diagnostic exploration, patient characteristics and surgical outcomes were found to be similar for MIS and open surgery. With regard to curative resection, however, the mean age was significantly lower among the patients who underwent open surgery (4.21 ± 4.20 vs 6.02 ± 4.99 for MIS, P = 0.047), and the proportion of malignancies was significantly higher (80% vs 39.4% for MIS, P < 0.001). MIS compared favorably with open surgery with respect to the rate of recurrence (6.7% vs 35.1%, P = 0.035), the rate of intraoperative transfusions (34.2% vs 58.5%, P = 0.01), the median amount of blood transfused (14 vs 22 mL/kg, P = 0.001), and the mean number of hospital days (4.66 ± 2.36 vs 7.21 ± 5.09, P < 0.001). Complication rates did not differ significantly between the MIS and open surgery groups. The operation was converted to open surgery in 3 cases (27.2%) of diagnostic MIS and in 5 cases (13.1%) of curative MIS.MIS was found to be both feasible and effective for the diagnosis and curative treatment of pediatric abdominal tumors. However, to determine the surgical role and guidelines for MIS for each specific tumor, a multicenter prospective study with a long-term follow-up is warranted.

Right iliac arterial aneurysm in a 4-year-old girl who does not have a right external iliac artery

Pediatric arterial aneurysm is rare disease. Among them, idiopathic-congenital arterial aneurysm is extremely rare. This is a case report of right common iliac artery idiopathic aneurysm with absence of right external iliac artery. A 4-year-old girl who had been complaining of intermittent abdominal pain since 2 years prior presented with a right lower abdominal mass that had been palpable since 6 months prior. Abdominal CT revealed a 5.2 cm × 4.5 cm × 5.1 cm, right-sided, partially thrombosed, saccular, iliac artery aneurysm. She underwent to operation, aneurismal resection. A pathological examination confirmed that it was a true aneurysm, considering that all layers of the vascular wall were stretched with no deficit. The patient was discharged 3 days after the surgery without any complication. Five months passed since the surgery, and the patient is doing well without any abdominal or leg pain.

The Optimal Timing of Enterostomy Closure in Extremely Low Birth Weight Patients for Acute Abdomen

There are few reports on enterostomy closure (EC) timing for acute abdomen in extremely low birth weight (ELBW) patients. We retrospectively reviewed ELBW patients who underwent enterostomy formation (EF) and subsequent EC. We investigated baseline characteristics, surgical outcomes, and follow-up data of 55 patients and analyzed optimal timing by age at EC, enterostomy duration, and body weight (Bwt) at EC. The minimum p-value approach (MPA) using the Chi-squared test was used to determine each cut-off value. Mean gestational age was 25+3 weeks, while mean age and Bwt at EF were 10 days and 660 g. Enterostomy duration and Bwt at EC were 102 days and 2400 g. Fourteen surgical complications were related to EC. The MPA identified a cut-off of 2100 g (p = 0.039) at EC but no significant cut-off age or enterostomy duration. The 18 patients <2100 g had more enterostomy-related problems at EC than the >2100 g group (66.7% vs 10.8%, p < 0.001). No other characteristics were significantly different. Operation time, ventilator period, hospital stay, parenteral nutrition duration, and full feeding day were significantly longer in <2100 g patients. Follow-up Bwt did not differ (11.55 kg vs 13.95 kg, p = 0.324). Our findings suggest EC can be safely performed when Bwt is over 2100 g.

Inguinal hernia in preterms in neonatal intensive care units: Optimal timing of herniorrhaphy and necessity of contralateral exploration in unilateral presentation

BACKGROUND We sought to determine the optimal timing of IH repair in preterms and the need for routine contralateral exploration. METHODS Medical records of 3690 pediatric patients who underwent unilateral IH repair between January 1998 and December 2009 were reviewed. We assessed medical record review and telephone interviews. In total, 1990 patients were enrolled in the study. Early, early-delayed, and late repair were defined as herniorrhaphy performed within 7 days of diagnosis, later than 7 days of diagnosis, and after discharge from the NICU, respectively. RESULTS Of 1990 patients, 90 preterms and 1900 full-terms were included. Among these, 7, 11 and 72 preterm patients received early, early-delayed and late IH repairs, respectively. Preoperative incarceration and postoperative complication rates were not different, but the recurrence rate was higher in the early repair group. Two group analysis of early and early-delayed vs. late repairs indicated similar results. The rates of synchronous and metachronous bilateral IH (SBIH, MBIH) were observed to be higher and the diagnostic interval of MBIH was shorter in preterms than in full-terms (35.6% vs. 15.9%, P < 0.001; 12.2% vs. 6.3%, P < 0.001; 5.2 vs. 41.8 months, P = 0.003). CONCLUSION Our results indicate that IH repair is safe to perform in preterm babies in the NICU at a delayed or late stage since the preoperative incarceration and recurrence rates were not different. Contralateral exploration could be considered in preterms because the rates of SBIH and MBIH were significantly higher and the MBIH diagnosis interval was shorter than in full-terms. LEVEL OF EVIDENCE III, treatment study.

Why Do the Patients with Hirschsprung Disease Get Redo Pull-Through Operation?

INTRODUCTION  The treatment of Hirschsprung disease (HD) is pull-through (PT) surgery. Redo PT can be performed in 1 to 10% of patients after initial PT. In this study, we reviewed the causes and associated factors of redo PT. MATERIALS AND METHODS  We retrospectively reviewed medical charts of 657 patients with HD who underwent surgeries between September 1979 and January 2016. The indications for redo PT are as follows. First, there were persistent obstructive symptoms after the first operation, (1) with transition zone shown definitely on contrast study, (2) with anatomic problems, and (3) obstructive symptoms persist despite conservative or nonredo surgical treatment without (1) and (2). We analyzed the causes and associated factors of redo PT. RESULTS  A total of 49 (7.5%) patients underwent redo PT. Among them, 41 and 8 patients underwent PT twice and three times, respectively. Among 57 cases of redo, the causes of redo included pathologic problem (n = 28)-aganglionosis (n = 20), hypoganglionosis (n = 4), immature ganglion cell (n = 4)-or anatomic problem (n = 21)-stricture (n = 13), fistula and/or abscess (n = 8) at anastomosis. Comparing associated factors between the nonredo and redo groups, the redo group had longer initial PT operation time (p = 0.001), more postoperative complications (p < 0.001), and more transanal endorectal PT (TERPT) approach as initial PTs (p < 0.001). According to causes of redo, the anatomic problem group underwent more third PTs than the pathologic problem group (p = 0.010). CONCLUSION  Approximately 7.5% of patients experienced redo PT. The cause of redo included pathologic (n = 28) or anatomic problem (n = 21). Longer operation time, more complications, and TERPT were associated with redo. The anatomic problem group underwent more third PTs than the pathologic problem group.

Analysis of clinical features of lipoblastoma in children

ABSTRACT Objective: A lipoblastoma is pathologically benign but often recurs. Due to its rarity, studies are scarce. The purpose of this study was to investigate the clinical characteristics of lipoblastoma occurring in children and to detect any correlations with the expression of Ki-67. Participants: From 1998 to 2010, 33 patients were diagnosed with lipoblastoma at Seoul National University Childrens Hospital. Methods: Ki-67 immunohistochemistry staining of the tumor tissue was performed. Results: A total of 33 patients (64% males) were enrolled in the study, with a mean age of 28 month. Eleven and 22 lesions were deep and superficial, respectively. Complete excisions were performed for 30 patients, and three underwent incomplete excisions. Two patients who underwent incomplete excision subsequently underwent a second operation due to tumor regrowth, and one patient had a recurrence despite complete excision. There was no statistically significant correlation observed between the tumor size or recurrence and the expression of Ki-67. Conclusions: Lipoblastoma requires an accurate diagnosis and operative resection to alleviate the symptoms induced by its growth. Incompletely resected tumor may regrow; therefore, complete excision is the treatment of choice. Continuous follow-up is needed to monitor for recurrence of disease, even after a complete excision.