Short pelvic floor electromyographic lag time: a novel noninvasive approach to document detrusor overactivity in children with lower urinary tract symptoms.

Purpose: Noninvasive uroflow with simultaneous electromyography can measure electromyographic lag time, ie the interval between the start of pelvic floor relaxation and the start of urine flow (normally 2 to 6 seconds). Intuitively one would expect that in patients experiencing urgency secondary to detrusor overactivity the lag time would be short or even a negative value. We studied whether short electromyographic lag time on uroflow with electromyography actually correlates with documented detrusor overactivity on urodynamics.

Intact right ventricle-pulmonary artery shunt after stage 2 palliation in hypoplastic left heart syndrome improves pulmonary artery growth.

For patients with hypoplastic left heart syndrome who have undergone the Norwood procedure with a right ventricle-pulmonary artery (RV-PA) shunt, the shunt can either be removed or left intact at the time of the stage 2 procedure. This study aimed to determine the effects of an intact shunt on pulmonary artery growth and clinical outcomes after the stage 2 procedure. A retrospective review of patients who underwent Norwood with an RV-PA shunt from 2005 to 2010 was performed.

Intratesticular varicoceles: are they significant?

Objective: Varicoceles occur in 15% of adult and adolescent males and are generally considered to be an extratesticular phenomenon. However, an intratesticular component has been reported in up to 2% of adult and 2% of adolescent varicoceles. We sought to determine the incidence of intratesticular varicoceles (ITV) in adolescents in our practice, its significance, associated findings and response to treatment.

Adolescent varicocele-is the 20/38 harbinger a durable predictor of testicular asymmetry?

Purpose: Part of the management of adolescent varicocele is trying to prognosticate who with testicular asymmetry will have catch-up growth with observation and who will have persistent asymmetry. We previously reported that catch-up growth is rare when peak retrograde flow greater than 38 cm per second is associated with 20% or greater asymmetry (ie the 20/38 harbinger). We sought to determine if this 20/38 cutoff held true with a larger series, and what peak retrograde flow value should be used when 15% instead of 20% asymmetry is chosen as the cutoff.

Nitric oxide-associated pulmonary edema in children with pulmonary venous hypertension.

Nitric oxide (NO)-associated pulmonary edema is rarely reported in children; in adults, it is often associated with left-sided heart failure. We report a case series of children with NO-associated pulmonary edema, which was defined as new multilobar alveolar infiltrates and worsening hypoxemia within 24 h of initiation or escalation of NO and radiologic or clinical improvement after NO discontinuation. We identified six patients (0.

Outcomes of the infant Ross procedure for congenital aortic stenosis followed into adolescence.

Objectives: The Ross procedure is used to treat aortic valve disease in children. The advantages include autograft growth, long-term durability, and avoidance of anticoagulation. Long-term follow-up of the Ross procedure in infancy is limited.

Prenatal diagnosis of congenital heart disease and birth outcomes.

This study was undertaken to examine the impact that prenatal diagnosis of congenital heart disease (CHD) has on birth and early neonatal outcomes. The prevalence of prenatally diagnosed CHD has risen over the past decade, but the effect that prenatal diagnosis of CHD has on peripartum decisions remains unclear. No consensus exists on the effect of prenatal diagnosis on neonatal outcomes.

Pediatric pulmonary arterial hypertension and hyperthyroidism: a potentially fatal combination.

Context: Patients with pulmonary arterial hypertension (PAH) who develop hyperthyroidism are at risk for acute cardiopulmonary decompensation and death.

Cases And Setting: We present a series of eight idiopathic PAH/heritable PAH pediatric patients who developed hyperthyroidism between 1999 and 2011. Institutional Review Board approval was obtained; informed consent was waived due to the retrospective nature of the series.

Severe Mycoplasma pneumoniae infection requiring extracorporeal membrane oxygenation with concomitant ischemic stroke in a child.

Mycoplasma pneumoniae is one of the most common agents causing respiratory disease in children. The most common extra-pulmonary manifestations of M. pneumoniae include central nervous system involvement, with stroke being an uncommon but devastating consequence.

Valve-sparing options in tetralogy of Fallot surgery.

Given late outcomes of patients with tetralogy of Fallot repaired in the 1970s and 1980s, as well as a better understanding of the late deleterious effects of pulmonary regurgitation, there is a tendency toward preservation of the pulmonary valve function during primary repair of tetralogy of Fallot. The bar keeps moving downward, to include smaller and more dysmorphic pulmonary valves. This article reviews some useful indications and techniques for valve-sparing options, including intraoperative balloon dilation and cusp reconstruction using a patch.

Laparoscopic excision of an antenatally diagnosed large simple hepatic cyst in the newborn.

Non-parasitic hepatic cysts are rare in infants and children. Advances in antenatal imaging have improved their detection, while development of laparoscopic techniques has expanded options for treatment. We present a case of an antenatally diagnosed non-parasitic hepatic cyst in a neonate, which was successfully excised laparoscopically with no recurrence.

Fetal cerebrovascular resistance and neonatal EEG predict 18-month neurodevelopmental outcome in infants with congenital heart disease.

Objectives: The purpose of this study was to investigate early markers of risk for neurobehavioral compromise in survivors with congenital heart disease (CHD).

Methods: Pregnant women in whom a fetal CHD had been diagnosed before 24 weeks' gestational age (GA) were enrolled in this prospective pilot study for serial Doppler ultrasound assessment of the fetal middle cerebral artery (MCA) and umbilical arteries. The cerebral-to-placental resistance ratio (CPR) and MCA pulsatility index (PI) Z-scores for GA were calculated.

Recommendations for treatment of nonclassic congenital adrenal hyperplasia (NCCAH): an update.

Congenital adrenal hyperplasia (CAH) is a family of autosomal recessive disorders. 21-Hydroxylase deficiency, in which there are mutations in CYP21A2 (the gene encoding the adrenal 21-hydroxylase enzyme), is the most common form (90%) of CAH. In classic CAH there is impaired cortisol production with diagnostic increased levels of 17-OH progesterone.

Observations on hydroceles following adolescent varicocelectomy.

Purpose: There is wide variation in the reported incidence of hydrocele after varicocelectomy (0% to 29%). We determined the incidence of hydroceles and hydrocelectomy following adolescent varicocelectomy, the time it took for them to manifest, and the results of aspiration and surgical correction.

Materials And Methods: Our adolescent varicocele registry was reviewed to identify patients with a post-varicocelectomy hydrocele.

Outcomes in pediatric cardiac transplantation with a positive HLA cross-match.

Previous studies have shown poor outcomes in pediatric heart transplant recipients with a high PRA or a positive direct donor-recipient cross-match. This study describes outcomes in patients with a positive cross-match at a large pediatric program. Pediatric heart transplant patients at a large single center between January 1993 and July 2009 were reviewed; those with cross-match data were analyzed.

The use of recombinant tissue-type plasminogen activator in a newborn with an intracardiac thrombus developed during extracorporeal membrane oxygenation.

Extracorporeal membrane oxygenation (ECMO) support is often used to support infants and children with hemodynamic or respiratory failure. One of the major obstacles of safely treating a child with ECMO is balancing the risk of hemorrhage with the potential for thrombus development. Managing thrombosis in the setting of ECMO is challenging and has no defined algorithm.

The triad of bridging bronchus malformation associated with left pulmonary artery sling and narrowing of the airway: the legacy of Wells and Landing.

Although noted in the 19th century, it was not until 1938 that Scheid published the combination of left pulmonary artery sling and narrowing of the airway due to annular tracheal cartilages. Unaware of these prior descriptions, and without a precise preoperative diagnosis, Willis Potts in Chicago performed the first successful sling repair in 1953. In 1976, Cohen and Landing described Scheid's combination of left pulmonary artery sling and stenosis caused by complete tracheal rings, and the term "ring-sling complex" was introduced by Berdon in 1984.