Persistent cloaca: a 10-year review of prenatal diagnosis.

OBJECTIVE: The purpose of this study was to review antenatal sonographic findings in children born with persistent cloaca. METHODS: Infants (n =145) with persistent cloaca followed at a center for colorectal congenital anomalies were identified by a retrospective chart review. Fifty female infants with a persistent cloaca met inclusion criteria and had prenatal records and imaging studies available for review. Sonographic data were retrospectively abstracted from charts. RESULTS: Anomalies were detected in 27 of 50 cases (54%). A correct antenatal diagnosis of persistent cloaca occurred in 3 of 50 (6%). Common findings misinterpreted on antenatal sonography include urinary tract anomalies, dilated bowel, and a cystic pelvic mass (representing hydrocolpos). CONCLUSIONS: Antenatal diagnosis of persistent cloaca is difficult. Persistent cloaca should be considered in the differential diagnosis if urinary tract malformations, dilated bowel loops, or cystic pelvic masses are visualized by prenatal diagnosis.

Prenatal MRI findings of fetuses with congenital high airway obstruction sequence.

OBJECTIVE: To define the MRI findings of congenital high airway obstruction sequence (CHAOS) in a series of fetuses. MATERIALS AND METHODS: Prenatal fetal MR images were reviewed in seven fetuses with CHAOS at 21 to 27 weeks of gestation. The MRI findings were reviewed. The MRI parameters evaluated included the appearance of the lungs and diaphragm, presence or absence of hydrops, amount of amniotic fluid, airway appearance, predicted level of airway obstruction, and any additional findings or suspected genetic syndromes. RESULTS: All the fetuses viewed (7 of 7) demonstrated the following MRI findings: dilated airway below the level of obstruction, increased lung signal, markedly increased lung volumes with flattened or inverted hemidiaphragms, massive ascites, centrally positioned and compressed heart, as well as placentomegaly. Other frequent findings were anasarca (6 of 7) and polyhydramnios (3 of 7). MRI identified the level of obstruction as laryngeal in five cases and tracheal in two cases. In four of the patients, surgery or autopsy confirmed the MRI predicted level of obstruction. Associated abnormalities were found in 4 of 7 (genetic syndromes in 2). Postnatal radiography (n = 3) showed markedly hyperinflated lungs with inverted or flattened hemidiaphragms, strandy perihilar opacities, pneumothoraces and tracheotomy. Two fetuses were terminated and one fetus demised in utero. Four fetuses were delivered via ex utero intrapartum treatment procedure. CONCLUSION: MRI shows a consistent pattern of abnormalities in fetuses with CHAOS, accurately identifies the level of airway obstruction, and helps differentiate from other lung abnormalities such as bilateral congenital pulmonary airway malformation by demonstrating an abnormally dilated airway distal to the obstruction.

High-risk fetal congenital pulmonary airway malformations have a variable response to steroids.

BACKGROUND/PURPOSE: Anecdotal reports suggest that maternal steroids may arrest the growth of congenital pulmonary airway malformations (CPAMs), preventing or reversing hydrops. We reviewed our experience with CPAMs to determine the fetal response to steroid therapy. METHODS: This study is a retrospective review of all fetal CPAMs from 2004 to 2008. Fetuses with high-risk CPAMs that received at least one course of steroids were identified. Fetal magnetic resonance imaging and ultrasound data were used to classify the CPAMs, identify hydrops fetalis and follow the fetuses poststeroid dosing. RESULTS: Forty-four fetuses with CPAM were identified. Fifteen patients were found to have received at least one course of steroids. Thirteen were hydropic and 2 were nonhydropic. Seven of the 13 hydropic fetuses (54%) showed an initial response to steroid administration, whereas the 2 nonhydropic high-risk fetuses progressed to birth without developing hydrops. Seven of the 15 patients, however, resulted in fetal demise or early postnatal death, giving a survival rate of 53%. CONCLUSIONS: High-risk CPAMs have a variable response to steroids. This variable response demonstrates the need for a placebo-controlled randomized study to more accurately determine the effect of steroids on hydrops and CPAM growth rates. Repeated steroid courses may not be helpful, and progression in CPAM volume to head circumference ratio (CVR) or hydrops should prompt open fetal surgery to prevent irreversible fetal insult.

Concentrations of estrogens in patients with preeclampsia.

The role of estrogens in the pathophysiology of preeclampsia remains to be determined. The aim of our study was to compare serum concentrations of 17 beta-estradiol and estriol in women with preeclampsia to normotensive pregnant controls. Serum concentrations of estrogens were measured in women with mild (n = 24) and severe (n = 24) preeclampsia as well as is normotensive pregnant controls (n = 24). Patients were matched for gestational age. Pregnancies complicated by early onset severe preeclampsia are associated with increased rates of maternal and fetal morbidity. Subsequently, we created further subgroups before and after 34 weeks of gestation (34 + 0). Serum estrogen concentrations were determined by standard ELISA technique. Compared to normotensive controls, the differences between the overall median serum concentrations of 17 beta-estradiol in women with mild (3811 v. 3730 pg/ml, P = 0.9) and severe (3811 v. 3630 pg/ml, P = 0.1) preeclampsia were statistically not significant. The differences between the overall median serum concentrations of estroil in controls and in patients with mild (121 v. 76 ng/ml, P = 0.6) and severe (121 v. 79 ng/ml, P = 0.4) preeclampsia were similar. The differences between the median concentrations of 17 beta-estradiol in patient with early onset severe preeclampsia compared to patients with mild preeclampsia (3061 v. 3715 pg/ml, P = 0.004) and controls (3061 v. 3807 pg/ml, P = 0.006) were statistically significant. In addition, the differences between the median concentrations of estriol in women with early onset severe preeclampsia compared to controls were statistically significant (20 v. 92 ng/ml, P = 0.02). The differences between the median concentrations of estrogens in those with late onset severe preeclampsia compared to women with mild preeclampsia were not significant. We found significantly lower concentrations of estrogens in women with early onset severe preeclampsia.