Investigational lymphatic imaging at the bedside in a pediatric postoperative chylothorax patient.

Chylothorax is a rare but serious complication in children who undergo heart surgery. Its pathogenesis is poorly understood, and invasive surgical treatments are considered only after conservative management fails. Current diagnostic imaging techniques, which could aid decision making for earlier surgical intervention, are difficult to apply. Herein, we deployed near-infrared fluorescence (NIRF) lymphatic imaging to allow the visualization of abnormal lymphatic drainage in an infant with postoperative chylothorax to guide the choice of surgical management. A 5-week-old male infant, who developed chylothoraces after undergoing Norwood surgery for hypoplastic left heart syndrome, was intradermally administered trace doses of indocyanine green in both feet and the left hand. NIRF imaging was then performed at the bedside to visualize lymphatic drainage patterns. Imaging results indicated impeded lymphatic drainage from the feet toward the trunk with no fluorescence in the chest indicating no leakage of peripheral lymph at the thoracic duct. Instead, lymph drainage occurred from the axilla directly into the pleural cavity. As a result of imaging, left pleurodesis was performed to stop the pleural effusion with the result of temporary decrease of left chest tube drainage. Although additional studies are required to understand normal and abnormal lymphatic drainage patterns in infants, we showed the potential of using NIRF lymphatic imaging at the bedside to visualize the lymphatic drainage pathway to guide therapy. Timely management of chylothorax may be improved by using NIRF imaging to understand lymphatic drainage pathways.

Systemic hypertension requiring treatment in the neonatal intensive care unit.

OBJECTIVES: To determine the difference in the risk factors for systemic hypertension in preterm and term infants in the neonatal intensive care unit (NICU). STUDY DESIGN: Data were collected from an existing database of NICU children and confirmed by chart review. Systemic hypertension was defined when 3 separate measurements of systolic and/or diastolic blood pressure were >95th percentile and an antihypertensive medication was administered for >2 weeks in the NICU. RESULTS: Of 4203 infants, we identified 53 (1.3%) with treated hypertension, of whom 74% were preterm, 11% required surgical intervention, and 85% required medications on discharge. The presence of a patent ductus arteriosus, umbilical catheterization, left ventricular hypertrophy, hypertensive medication at discharge, and mortality was similar between the term and preterm infants. The major risk factors for preterm infants, especially those <28 weeks' gestation, were bronchopulmonary dysplasia and iatrogenic factors, but, in term infants, they were systemic diseases. Term infants were diagnosed with hypertension earlier during hospitalization, had a shorter duration of stay in the NICU, and had a higher incidence of hypertension needing >3 medications than preterm infants. CONCLUSIONS: Perinatal risk factors are significant contributors to infantile hypertension. Term infants were diagnosed with hypertension earlier, had a shorter duration of stay, and had a higher incidence of resistant hypertension than preterm infants.

Sudden unexplained cardiac arrest in apparently healthy children: a single-center experience.

This study aimed to determine the causes of sudden cardiac arrest (SCA) in apparently healthy children at a single center in the era of primary prevention (screening questionnaire [SQ]) and secondary prevention (automated external defibrillator [AED] and the automated implantable cardioverter defibrillator [AICD]). Any child 0 to 18 years of age without prior known disease, except for attention deficit disorder, who underwent out-of-the hospital cardiopulmonary resuscitation was included in the study as a SCA subject. A retrospective chart review was used to evaluate the efficacy of the SQ, electrocardiogram (ECG), chest roentgenogram (CXR), and echocardiogram. The findings showed that for 44 of 6,656 children admitted to intensive care with SCA, an AED was used for 39%, an AICD was placed in 18%,and survival to hospital discharge was 50%. The etiology for SCA was identified in 57% of the cases, mostly in those older than 1 year, and the majority of these had a cardiac etiology (50%), whereas 7% had rupture of an arteriovenous malformation. Stimulant medication use was seen in 11% of the SCA subjects. In the best-case scenario of hypothesized primary prevention, a prior SQ, CXR, ECG, or echocardiogram may have detected respectively 18, 9, 23 and 16% of the at-risk cases, and 32% of the cases may have been detected with ECG and SQ together. Based on a historical control cohort, a positive ECG was significantly higher in the children with SCA (p = 0.014). An ECG together with a screening SQ may be more effective in identifying children potentially at risk for SCA than an SQ alone.

Immunoglobulin Resistance in Kawasaki Disease.

Background: The aim of this study was to identify risk factors for immunoglobulin resistance, including clinical symptoms such as arthritis and the pH of intravenous immunoglobulin. Methods: The data of children with Kawasaki disease who had received immunoglobulin were evaluated. Data regarding the brand of immunoglobulin administered were abstracted from the pharmacy records. Results: Eighty consecutive children with Kawasaki disease were evaluated (Mdnage=28 months, 66% male). The prevalence of immunoglobulin resistance was 30%. Arthritis was a presenting symptom in the acute phase of Kawasaki disease in 8% (6/80, all male) and was seen in significant association with immunoglobulin resistance in comparison to those without arthritis (16.7% vs. 0.2%, p=0.008). Next, the immunoglobulin brand types were divided into two groups: the relatively high pH group (n=16), including Carimune (pH 6.6±0.2), and the low pH group (n=63), including Gamunex (pH 4-4.5) or Privigen (pH 4.6-5). Overall, no significant difference in immunoglobulin responsiveness was found between the low pH and the high pH groups (73% vs. 56%, p=0.193), although the low pH group showed a trend toward a larger decrease in erythrocyte sedimentation rate (p=0.048), lower steroid use (p=0.054), and lower coronary involvement (p=0.08) than those in the high pH group. Conclusions: Children presenting with arthritis in the acute phase of Kawasaki disease may be at risk for immunoglobulin resistance.

Detection of early diastolic alterations by tissue Doppler imaging in untreated childhood-onset essential hypertension.

The aim of the study was to determine the presence of preclinical diastolic dysfunction in hypertensive children relative to normotensive children by Tissue Doppler Imaging (TDI). We prospectively enrolled children with untreated essential hypertension in absence of any other disease and a matched healthy control group with normal blood pressure (BP); both groups confirmed by clinic BP and a 24-hour ambulatory BP monitoring. Echocardiographic diastolic parameters were determined using spectral transmitral inflow Doppler, flow propagation velocity, TDI, and systolic parameters were determined via midwall shortening fraction and ejection fraction. A total of 80 multiethnic children were prospectively enrolled for the study: 46 hypertensive (median age, 13 years; 72% males) and 34 control (median age, 14 years; 65% males). The only echocardiography parameters that had a statistically significant change compared with the control children, were regional mitral Ea, Aa, and the E/Ea ratio by TDI. In comparison with controls, hypertensive children had lower Ea and Aa velocities of anterior and posterior walls and higher lateral wall E/Ea ratio. The decrease in posterior wall Ea and Aa remained significant after adjustment for gender, age, body mass index, ethnicity, and left ventricular hypertrophy on multivariate analysis. The lateral and septal wall E/Ea ratios correlated significantly with fasting serum insulin levels on similar multivariate analysis. Decreased regional TDI velocities were seen with preserved left ventricular systolic function even when other measures of diastolic dysfunction remained unchanged in untreated hypertensive children. Hypertension and serum insulin levels had strong associations with preclinical diastolic alterations in children.

Neonatal coarctation repair with a long isthmus: the isthmus patch.

This case report illustrates a ductal-dependent coarctation repair in a neonate whose long isthmus was believed to make conventional end-to-end repair problematic. The isthmus and left subclavian artery were isolated and augmented with a homograft while flow to the descending aorta was preserved through the ductus. After patch augmentation of the isthmus, ductal tissue was resected and an end-to-end anastomosis was performed using the length of the augmented isthmus. Angiography 18 months later showed excellent growth of the arch despite homograft tissue comprising the majority of the isthmus at the time of repair.