Laparoscopic excision of choledochal cysts with total intracorporeal reconstruction.

Purpose: There are numerous published reports of laparoscopic resection of choledochal cysts (CDCs), but almost all involve extracorporeal reconstruction of a biliary drainage system. We describe and evaluate the technique of laparoscopic CDC resection with total intracorporeal reconstruction.

Methods: We reviewed all patients who underwent a laparoscopic CDC resection from March 2005 to January 2010 at Rocky Mountain Children's Hospital and Children's Hospital of New York-Presbyterian.

Nonneurogenic voiding disorders in children and adolescents: clinical and videourodynamic findings in 4 specific conditions.

Purpose: We determined if there were any unique findings regarding specific clinical manifestations and videourodynamics among our patients with nonneurogenic voiding disorders.

Materials And Methods: A cohort of 237 normal children with lower urinary tract symptoms were evaluated by videourodynamics and uroflow/electromyogram, and divided into 4 groups based on the specific urodynamic findings 1) dysfunctional voiding (active electromyogram during voiding with or without detrusor overactivity), 2) idiopathic detrusor overactivity disorder (detrusor overactivity on urodynamics but quiet electromyogram during voiding), 3) detrusor underutilization disorder (willful infrequent but otherwise normal voiding) and 4) primary bladder neck dysfunction. Association of lower urinary tract symptoms, urinary tract infection, vesicoureteral reflux and abnormal urodynamic parameters within each condition was compared.

Variability of expert opinion in treatment of early-onset scoliosis.

Background: In contrast with treatment recommendations for adolescent idiopathic scoliosis, there are no clear algorithms for treating patients with early-onset scoliosis. There has been rapid expansion of treatment options for children with early-onset scoliosis, including casting, growth rods, the vertical expandable prosthetic titanium rib, and anterior vertebral stapling.

Questions/purposes: Given the range of treatment options, we assessed variability in decision making regarding treatment of patients with early-onset scoliosis.

Adolescent varicocele: influence of Tanner stage at presentation on the presence, development, worsening and/or improvement of testicular hypotrophy without surgical intervention.

Purpose: Testicular asymmetry in adolescents with varicocele can worsen, remain unchanged or decrease on followup. We determined the incidence of testicular asymmetry at presentation by Tanner stage and the correlation between Tanner stage at presentation and subsequent changes in percent asymmetry (ability for catch-up growth or progressive asymmetry) without surgical intervention.

Materials And Methods: We retrospectively studied the records of 115 boys with a mean age of 14.

Ethical considerations in live liver donation to children.

Transplant professionals are often faced with ethical situations in practice. In the field of pediatric transplantation, these ethical dilemmas can be even more profound than in adults. Transplant professionals must have a firm foundation of professional and personal ethical principles in order to handle ethical situations they encounter.

Pericardial effusion post-SCT in pediatric recipients with signs and/or symptoms of cardiac disease.

The objective of this study was to assess the incidence, risk factors, outcome and impact on OS of pericardial effusion (PEF) in a cohort of 156 pediatric SCT recipients. The mean age was 8.15±6.

BNP levels predict outcome in pediatric heart failure patients: post hoc analysis of the Pediatric Carvedilol Trial.

Background: The ability of serum B-type natriuretic peptide levels (BNP) to predict outcomes in children with heart failure (HF) has not been well demonstrated. This study was designed to determine whether BNP levels predict outcomes in patients with moderate symptomatic HF.

Methods And Results: We investigated whether enrollment BNP levels for the Pediatric Carvedilol Trial were associated with baseline characteristics.

The sieving coefficient and clearance of vasopressin during continuous renal replacement therapy in critically ill children.

Hypothesis: As vasopressin is a small peptide, its sieving coefficient (SC) and clearance (CL) during continuous renal replacement therapy may be intermediate to those for urea and β2 microglobulin (commonly used markers for small- and middle-molecular weight solutes, respectively).

Methods: A prospective, minimal-risk study was undertaken of the SC and CL of vasopressin in critically ill children on the first day of continuous renal replacement therapy using AN69 membrane filters and prefilter replacement fluid. All prefilter plasma (vasopressin) samples were drawn from the arterial port after predilution.

Cutaneous vesicostomy for palliative management of hemorrhagic cystitis and urinary clot retention.

We present the case of a 9 year old boy with hemorrhagic cystitis and urinary clot retention in the setting of chemotherapy refractory pre-B cell acute lymphocytic leukemia. The patient was undergoing palliative care which was complicated by severe discomfort from urinary clot retention. The decision was made to perform a cutaneous vesicostomy for clot removal and urinary drainage given the goals of care.

Cryoablation with an 8-mm tip catheter for pediatric atrioventricular nodal reentrant tachycardia is safe and efficacious with a low incidence of recurrence.

Background: Cryoablation with 4- and 6-mm tip ablation catheters has been demonstrated to be safe and effective in the treatment of atrioventricular nodal reentrant tachycardia (AVNRT) in pediatric patients, albeit with a higher rate of clinical recurrence. Limited information is available regarding efficacy, mid-term outcomes, and complications related to the use of the 8-mm Freezor Max Cryoablation catheter (Medtronic, Minneapolis, MN, USA) in pediatric patients.

Methods: We performed a retrospective review of all pediatric patients with normal cardiac anatomy who underwent an ablation procedure for treatment of AVNRT using the 8-mm tip Cryoablation catheter at three large pediatric academic arrhythmia centers.

Staphylococcus aureus Septicemia with a fatal transmural myocardial infarction in a 27-week-gestation twin infant: a case study.

Septicemia, one of the major causes of morbidity and mortality in the neonatal period, often has a rapid and fulminant course. Low-birth-weight infants with persistent Staphylococcus aureus septicemia, possibly associated with percutaneous central venous catheters, may develop metastatic infections including endocarditis with large vegetations. This article describes a neonate with S.

Baseline correction of phase-contrast images in congenital cardiovascular magnetic resonance.

Background: One potential source of error in phase contrast (PC) congenital CMR flow measurements is caused by phase offsets due to local non-compensated eddy currents. Phantom correction of these phase offset errors has been shown to result in more accurate measurements of blood flow in adults with structurally normal hearts. We report the effect of phantom correction on PC flow measurements at a clinical congenital CMR program.

Transition from pediatric to adult health care for adolescents with congenital heart disease: a review of the literature and clinical implications.

As medical and surgical techniques advance in modern health care, children and adolescents with congenital heart disease (CHD) are living well into their adult years. Their complex cardiac anatomy, physiology, and medical histories present a challenge to adult health care providers who are not traditionally educated and trained in CHD. Growing evidence demonstrates that adolescents and young adults with CHD are at risk for extensive complications and sequelae from their heart condition and surgical treatments.

Hepatic pulmonary fusion in an infant with a right-sided congenital diaphragmatic hernia and contralateral mediastinal shift.

Hepatic pulmonary fusion is extremely rare with only 9 previous cases reported in the literature. In typical cases, the clinician should be alerted to the possibility of hepatic pulmonary fusion if the chest radiograph shows a large opacity on the right side without a contralateral mediastinal shift. The authors present a case of right-sided diaphragmatic hernia and hepatic pulmonary fusion with associated contralateral mediastinal shift discovered beyond the neonatal period.

Reversal of intestinal failure-associated liver disease in infants and children on parenteral nutrition: experience with 93 patients at a referral center for intestinal rehabilitation.

Purpose: Intestinal failure (IF)-associated liver disease (IFALD) complicates the treatment of children with IF receiving parenteral nutrition (PN). We hypothesized that prevention or resolution of IFALD was possible in most children and that this would result in improved outcomes.

Methods: We reviewed prospectively gathered data on all children referred to the intestinal rehabilitation and transplantation center at our institution.

Risk factors for spinal cord injury during surgery for spinal deformity.

Background: Spinal cord monitoring is now considered standard care during surgery for spinal deformity. Combined somatosensory and motor evoked potential monitoring allows the detection of early spinal cord dysfunction in most patients. The purpose of the current study was to identify clinical factors that increase the risk of intraoperative electrophysical changes and to provide management recommendations.

Testicular asymmetry and adolescent varicoceles managed expectantly.

Purpose: Adolescent varicocele is often associated with testicular asymmetry. Depending on the degree of asymmetry, some physicians will recommend surgery. However, given the possibility that asynchronous growth may be transient, others advocate for a period of observation.

Primary bladder neck dysfunction in children and adolescents III: results of long-term alpha-blocker therapy.

Purpose: Primary bladder neck dysfunction is a nonneurogenic voiding disorder frequently overlooked in pediatrics. The diagnosis classically is made by videourodynamics but can also be made with noninvasive uroflow studies with pelvic floor electromyography. We report our long-term results using alpha-blocker therapy in patients with primary bladder neck dysfunction.

Massive pulmonary embolism in children.

We present 3 children with massive pulmonary embolism and review 17 recent pediatric reports. Malignancies were a frequent cause (40%), and sudden death was common (60%). Compared with adults, diagnosis was more likely to be made at autopsy (P < .

Indices of body and brain size at birth and at the age of 2 years: relations to cognitive outcome at the age of 16 years in low birth weight infants.

Objective: To determine in low birth weight infants the relations of being small for gestational age at birth, microcephalic at birth, low weight for age at 2 years, and microcephalic at 2 years to full scale intelligence quotient (FSIQ) at the age of 16 years.

Methods: A prospective observational study of 422 of 837 eligible nondisabled low birth weight (<2000 g) adolescents from the newborn brain hemorrhage cohort with weight and head circumference at birth and at the age of 2 years in whom FSIQ was assessed with the Wechsler Abbreviated Scales of Intelligence at the age of 16 years.

Results: In a multiple regression analysis, being small for gestational age (beta = -0.

Incidence of Viral and fungal infections following busulfan-based reduced-intensity versus myeloablative conditioning in pediatric allogeneic stem cell transplantation recipients.

Reductions in the duration and nadir of neutropenia have translated into a significant decrease in bacteremia in adult recipients of allogeneic stem cell transplantation (allo-SCT) with reduced-intensity conditioning (RIC) during the first 30 days after transplantation. It remains to be determined whether RIC allo-SCT also will result in a decrease in systemic viral infections (SVIs) and invasive fungal infections (IFIs), which are more dependent on alterations in cellular immunity. We compared the incidence of SVIs and IFIs in children receiving busulfan-based RIC allo-SCT and in children receiving myeloablative conditioning (MAC) allo-SCT for various malignant and nonmalignant diseases.

Favorable outcome of juvenile dermatomyositis treated without systemic corticosteroids.

Objective: To describe the course of patients with juvenile dermatomyositis (JDM) treated effectively without systemic corticosteroids.

Study Design: A retrospective study of 38 patients with JDM treated at a tertiary care children's hospital identified 8 patients who had never received corticosteroids. Disease presentation and course, pharmacologic, and ancillary treatments were recorded.