Won Kyoung Jhang

CT findings of plastic bronchitis in children after a Fontan operation

BackgroundPlastic bronchitis is a rare cause of acute obstructive respiratory failure in children. Life-threatening events are much more frequent in patients with repaired cyanotic congenital heart disease, and most frequent following a Fontan operation. Commonly, the diagnosis is not made until bronchial casts are expectorated. Detailed CT findings in plastic bronchitis have not been described.ObjectiveTo describe the CT findings in plastic bronchitis in children after a Fontan operation.Materials and methodsThree children with plastic bronchitis after a Fontan operation were evaluated by chest CT. Bronchial casts were spontaneously expectorated and/or extracted by bronchoscopy. Airway and lung abnormalities seen on CT were analyzed in the three children.ResultsCT demonstrated bronchial casts in the central airways with associated atelectasis and consolidation in all children. The affected airways were completely or partially obstructed by the bronchial casts without associated bronchiectasis. The airway and lung abnormalities rapidly improved after removal of the bronchial casts.ConclusionCT can identify airway and lung abnormalities in children with plastic bronchitis after a Fontan operation. In addition, CT can be used to guide bronchoscopy and to monitor treatment responses, and thereby may improve clinical outcomes.

Perioperative Evaluation of Airways in Patients With Arch Obstruction and Intracardiac Defects

BACKGROUND Patients with arch obstruction and intracardiac defects have a high probability of abnormal aortopulmonary space geometry, which provides airway compression. The tissue-to-tissue technique arch repair could result in real airway problems. This report describes our experience with the perioperative evaluation and management of airway problems. METHODS We retrospectively reviewed the medical records of 90 patients with arch obstruction and intracardiac defects who underwent computed tomography (CT) and corrective surgery in our institution between January 2000 and January 2007. RESULTS Of the 77 patients who underwent preoperative CT (group 1), 21 were found to have airway compression (27.2%). Of those 21 patients, 5 underwent concomitant airway relieving procedures. In group 1, 2 patients required subsequent secondary surgery for airway problems after the initial arch repair. Of the 13 patients who underwent postoperative CT only (group 2), 6 underwent subsequent secondary surgery for airway relief. For airway relief, several procedures were additionally performed (eg, right pulmonary artery translocation anterior to the aorta, aortopexy, peribronchial dissection, and tissue augmentation). In terms of the type of arch repair, 48 patients underwent end-to-side anastomosis, 39 underwent extended end-to-end anastomosis, and 3 underwent end-to-end anastomosis. End-to-side was the repair type most commonly associated with airway compression requiring additional procedure (10 of 15, 66.6%). CONCLUSIONS Patients with arch obstruction and intracardiac defects had a rather high incidence of airway compression preoperatively and postoperatively. Preoperative CT and intraoperative complementary bronchoscopy were useful for identifying and fixing the airway problems. Additional procedures for relieving airway compression were required more frequently after end-to-side type arch repair than after extended end-to-end anastomosis. More meticulous intraoperative evaluation and management are recommended in this type of repair.

Toxic Inhalational Injury-Associated Interstitial Lung Disease in Children

Interstitial lung disease in children (chILD) is a group of disorders characterized by lung inflammation and interstitial fibrosis. In the past recent years, we noted an outbreak of child in Korea, which is possibly associated with inhalation toxicity. Here, we report a series of cases involving toxic inhalational injury-associated chILD with bronchiolitis obliterans pattern in Korean children. This study included 16 pediatric patients confirmed by lung biopsy and chest computed tomography, between February 2006 and May 2011 at Asan Medical Center Childrens Hospital. The most common presenting symptoms were cough and dyspnea. The median age at presentation was 26 months (range: 12-47 months), with high mortality (44%). Histopathological analysis showed bronchiolar destruction and centrilobular distribution of alveolar destruction by inflammatory and fibroproliferative process with subpleural sparing. Chest computed tomography showed ground-glass opacities and consolidation in the early phase and diffuse centrilobular nodular opacity in the late phase. Air leak with severe respiratory difficulty was associated with poor prognosis. Although respiratory chemicals such as humidifier disinfectants were strongly considered as a cause of this disease, further studies are needed to understand the etiology and pathophysiology of the disease to improve the prognosis and allow early diagnosis and treatment.

The CCR5 (−2135C/T) Polymorphism may be Associated with the Development of Kawasaki Disease in Korean Children

BackgroundKawasaki disease (KD) is an acute vasculitis syndrome of unknown etiology that frequently affects small to medium size arteries. C-C chemokine receptor 5 (CCR5) is a chemokine receptor that binds C-C chemokines. This study investigated the association of the CCR5 (−2135C/T) polymorphism with KD in Korean children.MethodsThe study population consisted 189 Korean children with KD and 194 Korean children with congenital heart disease (CHD). CCR5 (−2135C/T) polymorphism genotypes were determined using the single-base extension method.ResultsThe allele frequencies of the CCR5 (−2135C/T) polymorphism differed significantly between CHD children and KD children (−2135T/T, 16.75% vs. 30.05%, aOR 2.14, 95% CI 1.31–3.51). The tested laboratory parameters differed significantly between the KD and CHD groups. The development of coronary artery aneurysm in KD patients was not associated with the CCR5 polymorphism.ConclusionsOur findings suggest that the T allele at the CCR5 (−2135C/T) polymorphism might be associated with the development of KD in Korean children but does not appear to be associated with the development of coronary artery aneurysm.

Impact of Delayed Sternal Closure on Postoperative Infection or Wound Dehiscence in Patients With Congenital Heart Disease

BACKGROUND We sought to determine whether longer duration before delayed sternal closure (DSC) increases the risk of mortality, postoperative infection, or wound dehiscence. METHODS A retrospective review was performed of 154 patients who underwent DSC between January 1999 and April 2009. Median body weight and age at operation were 3.6 kg (1.5 to 80 kg) and 25 days (2 days to 20 years), respectively. Palliative procedures were performed in 58 patients (58/154, 37.7%). Sternal wound problems were categorized according to the consensus-based definitions. Multivariate analysis was conducted encompassing various preoperative and intraoperative variables to identify risk factors for adverse surgical outcomes. The mean comprehensive Aristotle score (CAS) was 13.2±3.1. RESULTS There were 28 hospital mortalities (28/154, 18.2%). Excluding patients who died before sternal closure (4/154, 2.6%), the median duration of sternal opening was 3.5 days (1 to 182 days). Postoperative infection (sternal wound infection or systemic infection) and sterile wound dehiscence (SWD) occurred in 17 and 14 patients, respectively. Multivariate analysis revealed that duration of ventilatory support increased the risk of mortality (p=0.004), and postoperative infection/SWD (p=0.001). CAS also correlated with postoperative infection/SWD (p=0.026). Duration of sternal opening however was associated with none of the outcome variables. CONCLUSIONS Long ventilatory support and complexity of the cardiac anomaly increase the risk of adverse outcomes after procedures to repair congenital cardiac anomalies . After adjusting these variables, longer duration before DSC does not seem to be a risk factor for surgical mortality, postoperative infection, or wound dehiscence.

Extracorporeal membrane oxygenation support in a patient with status asthmaticus.

Status asthmaticus is a rare, fatal condition, especially in children. Sometimes respiratory support is insufficient with a mechanical ventilator or medical therapy for patients with status asthmaticus. In such situations, early extracorporeal membrane oxygenation application is a useful method for treating refractory respiratory failure. We report on a case of a six-year-old, male child who underwent venovenous extracorporeal membrane oxygenation support for refractory status asthmaticus.

Left Ventricular Function After Left Ventriculotomy for Surgical Treatment of Multiple Muscular Ventricular Septal Defects

BACKGROUND Optimal management of muscular ventricular septal defects (MVSD) is still not determined in the current era. Moreover, long-term left ventricular function after closure of MVSD is not well known. Thus, we investigated surgical outcomes including long-term left ventricular function after closure of MVSD through left ventriculotomy. METHODS We conducted a retrospective review of medical records of 20 children who underwent MVSD closure between March 1993 and August 2010. There were 10 boys (50%) and 10 girls (50%). Patient age ranged from 1.6 to 103.4 months (median, 26.4 months), and body weight from 2.8 to 31.5 kg (median, 11.9 kg). Electrocardiogram results were normal sinus rhythm in all except 1 patient with congenital complete atrioventricular block. There were 16 patients who previously had palliative pulmonary artery banding procedures before closure of MVSD. There were 13 patients (65%) with Swiss-cheese type VSD. RESULTS There was 1 hospital death of a patient with congenital complete atrioventricular block with pacemaker malfunction (5%). There was 1 late death of a patient with del 22q with adenoviral pneumonia. There was no reoperation. Median follow-up duration was 85.9 months (range, 4.7 to 166.7). The location of MVSD was apical portion in 10 patients (50%) and midtrabecular portion in 9 patients (45%). There were 6 Dacron patch closures and 13 direct closures of MVSD through left ventriculotomy. There was no complete atrioventricular block. Last follow-up echocardiographic data showed normal ejection fraction with 65.2% ± 8.2% after closure of MVSDs. There was no leakage in 8 patients; 11 patients had insignificant leakage, which disappeared spontaneously in 4 patients 17.9 months (median value) after operation. CONCLUSIONS Our acceptable long-term results of left ventricular function after left ventriculotomy proved that this technique might be a viable option in the management of MVSD.

Successful liver transplantation following veno-arterial extracorporeal membrane oxygenation in a child with fulminant Wilson disease and severe pulmonary hemorrhage: a case report.

Son SK, Oh SH, Kim KM, Lee YJ, Jhang WK, Park SJ, Shin HJ, Park J‐J, Kim TH, Kim DY, Hwang S, Park K‐M, Lee Y‐J, Lee S‐G. Successful liver transplantation following veno‐arterial extracorporeal membrane oxygenation in a child with fulminant Wilson disease and severe pulmonary hemorrhage: A case report. 
Pediatr Transplantation 2011.

High prevalence of neonatal presentation in Korean patients with citrullinemia type 1, and their shared mutations.

Type 1 citrullinemia (CTLN1) often presents as a hyperammonemic encephalopathy in the neonatal period, but it can also develop in the late-infantile period and in adults. In addition, some patients can be identified in the presymptomatic period by neonatal or family member screening. In this study, twenty Korean patients with CTLN1 (19 families) were examined; fourteen patients with neonatal-onset, three with late-onset, and three that were identified presymptomatically. The 13 patients with hyperammonemic encephalopathy received continuous venovenous hemofiltration (CVVH) or peritoneal dialysis (PD). Although the hyperammonemia was relieved more effectively in the six patients on CVVH than the seven on PD, most of these patients suffered from severe neurologic deficits. Recurrent hyperammonemic episodes (7 pts, 35%), recurrent and reversible acute hepatic dysfunction (5 pts, 25%), and focal cerebral infarction (2 pts, 10%) were noted. The neonates with hyperammonemic encephalopathy had extensive brain injuries at the onset of hyperammonemia, followed by encephalomalacia and brain atrophy at quite an early age. Genetic testing for the ASS1 gene revealed a different mutation spectrum from those of other ethnicities; Three common mutations, c.421-2A>G (37.8%), c.1128-6_1188dup67 (18.9%), and p.Gly324Ser (16.2%), accounted for 73% of the mutations. The poor outcome was expected in patients with the peak ammonia level at onset over 600μmol/L, whose proportion was higher in the neonatal presentation group than in the presymptomatic/late presentation group. Our findings add to the current understanding of the ethnic diversity of CTLN1 from both clinical and genetic perspectives.

Factors Associated With Mortality in Continuous Renal Replacement Therapy for Pediatric Patients With Acute Kidney Injury

Objectives: To analyze the epidemiology of pediatric acute kidney injury requiring continuous renal replacement therapy and identify prognostic factors affecting mortality rates. Design: Retrospective analysis. Setting: PICU of a tertiary medical center. Patients: One hundred-twenty three children diagnosed with acute kidney injury requiring continuous renal replacement therapy. Interventions: None. Measurements and Main Results: Vasoactive-Inotropic Score, arterial blood gas analysis, blood chemistry at continuous renal replacement therapy initiation, the extent of fluid overload 24 hours prior to continuous renal replacement therapy initiation, Pediatric Risk of Mortality III score at admission, and need for mechanical ventilation during continuous renal replacement therapy were compared in survivors and nonsurvivors. Out of 1,832 patient admissions, 185 patients (10.1%) developed acute kidney injury during the study period. Of these, 158 patients were treated with continuous renal replacement therapy, and finally, 123 patients were enrolled. Of the enrolled patients, 50 patients died, corresponding to a mortality rate of 40.6%. The survivor group and the nonsurvivor group were compared, and the following factors were associated with an increased risk of mortality: higher Pediatric Risk of Mortality III score at admission and Vasoactive-Inotropic Score when initiating continuous renal replacement therapy, increased fluid overload 24 hours before continuous renal replacement therapy initiation, and need for mechanical ventilation during continuous renal replacement therapy. The percentage of fluid overload difference between the survivors and the nonsurvivors was 1.2% ± 2.2% versus 4.1% ± 4.6%, respectively. Acidosis, elevated lactic acid and blood urea nitrogen, and lower serum creatinine level were laboratory parameters associated with increased mortality. On multivariate analysis, Vasoactive-Inotropic Score, need for mechanical ventilation, blood urea nitrogen, and creatinine level were statistically significant. (Odds ratio: 1.040, 6.096, 1.032, and 0.643, respectively.) Conclusions: A higher Vasoactive-Inotropic Score, need for mechanical ventilation, elevated blood urea nitrogen, and lower creatinine level were associated with increased mortality in pediatric acute kidney injury patients who underwent continuous renal replacement therapy. Lower creatinine levels may be associated with increased mortality in the context of fluid overload, which is correlated with a reduced chance of survival.