N Scott Adzick

BACKGROUND/PURPOSE: Cystic adenomatoid malformation of the lung (CAM) diagnosed in utero has a variable natural history that may result in hydrops in up to 40% or regress in up to 15%. No criteria have been available to determine which lesions would grow and develop hydrops versus those whose growth would stabilize or regress. To better understand the natural history of CAM the authors developed a measure of tumor volume normalized for gestation age, the CAM volume ratio, or CVR. The ...

BACKGROUND: Since the results of the Management of Myelomeningocele Study were published, maternal-fetal surgery for the in utero treatment of spina bifida has become accepted as a standard of care alternative. Despite promise with fetal management of myelomeningocele repair, there are significant complications to consider. Chorioamniotic membrane separation and preterm premature rupture of membranes are known complications of invasive fetal procedures. Despite their relative frequency ...

OBJECT: The Management of Myelomeningocele Study (MOMS) was a multicenter randomized trial comparing the safety and efficacy of prenatal and postnatal closure of myelomeningocele. The trial was stopped early because of the demonstrated efficacy of prenatal surgery, and outcomes on 158 of 183 pregnancies were reported. Here, the authors update the 1-year outcomes for the complete trial, analyze the primary and related outcomes, and evaluate whether specific prerandomization risk factors ...

BACKGROUND/PURPOSE: Prenatal tracheal occlusion (TO) has been shown to accelerate lung growth in animal models and models of pulmonary hypoplasia. However, these models may not mimic early events in human congenital diaphragmatic hernia (CDH). The authors previously have developed a model of TO in the rat. The purpose of this study was to apply this technique to characterize TO-induced lung growth in the early onset nitrofen-induced model of CDH, and to address the clinically important ...

Most fetuses with congenital diaphragmatic hernia (CDH) diagnosed before 24 weeks' gestation die despite optimal postnatal care. In fetuses with liver herniation into the chest, prenatal repair has not been successful. In the course of exploring the pathophysiology of CDH and its repair in fetal lambs, the authors found that obstructing the normal egress of fetal lung fluid enlarges developing fetal lungs, reduces the herniated viscera, and accelerates lung growth, resulting in improved ...

The objective of this paper is to evaluate the efficacy of various magnetic resonance imaging (MRI) sequences and the general usefulness of prenatal MRI in determining the position of the fetal liver and visualizing lung tissue in fetuses who have congenital diaphragmatic hernia (CDH). This was a retrospective review of prenatal MRI of fetuses with a confirmed diagnosis by surgery or autopsy of CDH. MRI was performed in a 1.5-Tesla magnet using fast gradient echo, half-Fourier ...

Congenital cystic adenomatoid malformation and bronchopulmonary sequestration are congenital lung tumors that are classically described as having distinct embryology, pathology, and natural history. The authors treated six patients who had prenatally diagnosed lung masses that displayed clinicopathologic features of both lesion types. At prenatal diagnosis (19 to 30 weeks' gestation), all six lesions were classified sonographically as congenital cystic adenomatoid malformation, and none ...

PURPOSE: The optimal timing of repair for congenital diaphragmatic hernia (CDH) in patients requiring extracorporeal membrane oxygenation (ECMO) is controversial. Repair during ECMO may improve respiratory function by restoring normal anatomy. However, there is increased risk of complications including surgical bleeding. The purpose of this study was to examine the impact of timing of CDH repair on outcomes in a large cohort of patients treated at a single institution.

METHODS: We ...

OBJECTIVE: Our purpose was to review our experience with fetal congenital cystic adenomatoid malformation and extralobar pulmonary sequestration emphasizing natural history, management, and outcome.

STUDY DESIGN: We conducted a retrospective review of 175 fetal lung lesions diagnosed by antenatal ultrasonography at 2 fetal treatment centers.

RESULTS: There were 134 congenital cystic adenomatoid malformation cases. Fourteen women underwent elective abortion, 101 women were managed ...

BACKGROUND/PURPOSE: In utero repair of myelomeningocele (MMC) in humans spares distal neurologic function, reverses the hindbrain herniation component of the Arnold-Chiari II malformation (ACM), and reduces the rate of postnatal shunt placement. The authors hypothesized that extravasation of cerebrospinal fluid (CSF) from the lumbar spinal cord results in herniation. This hypothesis was tested by assessing the impact of a spinal cord myelotomy on hindbrain anatomy in fetal ...

OBJECTIVE: Fetal skin wounds heal without scarring. To determine the role of TGF-beta 1 in fetal wound healing, mRNA expression of TGF-beta 1 was analyzed in human fetal and adult skin wounds.

METHODS: Human fetal skin transplanted to a subcutaneous location on an adult athymic mouse that was subsequently wounded heals without scar, whereas human adult skin heals with scar formation in that location. In situ hybridization for TGF-beta 1 mRNA expression and species-specific ...

BACKGROUND: Ultrasound (US) evaluation of some fetal anomalies provides limited information. Anatomic details that affect prognosis and selection for fetal therapy, such as liver herniation and pulmonary hypoplasia in congenital diaphragmatic hernia (CDH) and airway patency in giant neck masses, may be difficult to delineate using conventional sonographic methods. The authors evaluated the utility of prenatal magnetic resonance imaging (MRI) with new ultrafast imaging sequences in the ...

BACKGROUND/PURPOSE: The high mortality rate of congenital diaphragmatic hernia (CDH) is ascribed generally to pulmonary hypoplasia and persistent pulmonary hypertension characterized by associated pulmonary arterial structural changes. Prenatal tracheal occlusion (TO) accelerates lung growth, but the effect of TO on pulmonary arterial structure in CDH has not been well defined. The authors hypothesized that TO could reverse the pulmonary arterial structural changes observed in CDH. To ...

OBJECTIVE: The purpose of this study was to determine the ability of liver position and lung-to-head ratio to predict outcome in isolated left congenital diaphragmatic hernia.

STUDY DESIGN: We reviewed prenatal studies and postnatal outcomes of congenital diaphragmatic hernia between January 1996 and January 2006.

RESULTS: Eighty-nine patients received prenatal and postnatal care at 1 institution. In fetuses with liver up, extracorporeal membrane oxygenation was required in 39 of 49 ...