Longitudinal Analysis of Pulmonary Function in Survivors of Congenital Diaphragmatic Hernia.

Objective: To analyze longitudinal trends of pulmonary function testing in patients with congenital diaphragmatic hernia (CDH) followed in our multidisciplinary clinic.

Study Design: This was a retrospective cohort study of CDH patients born between 1991 and 2013. A linear mixed effects model was fitted to estimate the trends of percent predicted forced expiratory volume in 1 second (FEVpp), percent predicted forced vital capacity (FVCpp), and FEV/FVC over time.

Longitudinal Analysis of Ventilation Perfusion Mismatch in Congenital Diaphragmatic Hernia Survivors.

Objective: To determine the natural history of pulmonary function for survivors of congenital diaphragmatic hernia (CDH).

Study Design: This was a retrospective cohort study of survivors of CDH born during 1991-2016 and followed at our institution. A generalized linear model was fitted to assess the longitudinal trends of ventilation (V), perfusion (Q), and V/Q mismatch.

Does the ex utero intrapartum treatment to extracorporeal membrane oxygenation procedure change morbidity outcomes for high-risk congenital diaphragmatic hernia survivors?

Purpose: In high-risk congenital diaphragmatic hernia (CDH), significant barotrauma or death can occur before extracorporeal membrane oxygenation (ECMO) can be initiated. We previously examined ex utero intrapartum treatment (EXIT)-to-ECMO in our most severe CDH patients, but demonstrated no survival advantage. We now report morbidity outcomes in survivors of this high-risk cohort to determine whether EXIT-to-ECMO conferred any benefit.

Cardiac Findings in the Fetus with Cerebral Arteriovenous Malformation Are Associated with Adverse Outcome.

Objectives: To assess cardiac sequelae of fetal cerebral arteriovenous malformations (CAVMs) and evaluate any association with outcomes.

Methods: We retrospectively analyzed cardiac structure and function in fetuses with CAVMs who underwent fetal echocardiography (October 1999 to August 2015, n = 11), and compared them with normal controls.

Results: The median gestational age was 36 weeks (range 18-38).

Human fetal sacrococcygeal extension or 'tail' in the second trimester: prenatal diagnosis, associated findings, and clinical outcome.

Background: There are over 30 cases of prenatally diagnosed sacral extensions or human 'tails' in the literature, including isolated and syndromic etiologies. Most cases were reported to resolve by the second trimester and postnatal course was benign. Our objective was to describe the prenatal findings, associated anomalies, and clinical outcome of a series of seven fetuses diagnosed prenatally with fetal sacrococcygeal extension.

In utero valvuloplasty for pulmonary atresia with hypoplastic right ventricle: techniques and outcomes.

Background: Prenatal intervention for fetuses with pulmonary atresia with an intact ventricular septum (PA/IVS) has the potential to alter right heart physiologic features in utero, facilitating right heart growth and improving the prospect of a biventricular outcome after birth.

Methods: Since 2002, we have considered prenatal intervention for fetal PA/IVS in patients with (1) membranous pulmonary atresia, with identifiable pulmonary valve (PV) leaflets or membrane; (2) an intact or highly restrictive ventricular septum; and (3) right heart hypoplasia, with a tricuspid valve annulus z score of -2 or below and an identifiable but small right ventricle. Intervention was performed through direct cardiac puncture under ultrasound guidance, with percutaneous access or access through a limited laparotomy.

Results of in utero atrial septoplasty in fetuses with hypoplastic left heart syndrome.

Objectives: Neonates with hypoplastic left heart syndrome and intact or highly restrictive atrial septum have a high rate of mortality. We sought to assess the effect of prenatal intervention intended to create atrial septal defects in fetuses with this diagnosis.

Methods: We reviewed the medical records and imaging of all fetuses undergoing intervention for atrial septal defect creation in the setting of hypoplastic left heart syndrome and intact atrial septum.

How accurately does current fetal imaging identify posterior fossa anomalies?

Objective: The first objective of our study was to describe the prevalence and spectrum of posterior fossa anomalies over 5 years in a major fetal care center where the referral diagnosis (by fetal sonography) was investigated by fetal MRI and, if confirmed, by postnatal MRI if possible. The second objective was to assess the accuracy with which fetal MRI predicts postnatal MRI findings in this population.

Materials And Methods: We retrospectively identified all cases of suspected fetal posterior fossa anomalies referred to our center from 2002 through 2006.

Ex utero intrapartum treatment with extracorporeal membrane oxygenation for severe congenital diaphragmatic hernia.

Purpose: The purpose of this study was to determine whether ex utero intrapartum treatment with extracorporeal membrane oxygenation (EXIT to ECMO) is a reasonable approach for managing patients antenatally diagnosed with severe congenital diaphragmatic hernia (CDH).

Methods: A 6-year retrospective review was performed on fetuses with severe CDH (liver herniation and a lung/head ratio <1.4, percentage of predicted lung volume <15, and/or congenital heart disease).

An interdisciplinary project that changed practice in feeding methods after pyloromyotomy.

Postoperative feeding methods for infants with hypertrophic pyloric stenosis (HPS) have remained unchanged for several decades. Conventional feeding methods include a prolonged NPO period immediately after pyloromyotomy, followed by a slow, incremental increase in volume and strength of feedings. Recently, some surgeons have ascribed to a more rapid or ad lib feeding advancement.

Ad libitum feeding decreases hospital stay for neonates after pyloromyotomy.

Purpose: The optimal feeding regimen for neonates after pyloromyotomy for hypertrophic pyloric stenosis (HPS) remains controversial. This study sought to compare ad libitum feeding to a Conventional feeding regimen with regard to time to full diet, length of hospital stay, and readmission rates.

Methods: A 6-month review of 36 consecutive patients who underwent pyloromyotomy for HPS was undertaken.