Prediction of Pediatric Outcome after Prenatal Diagnosis and Expectant Antenatal Management of Congenital Cystic Adenomatoid Malformation

Abstract

Objective: To determine whether the congenital cystic adenomatoid malformation (CCAM) volume ratio (CVR) is associated with fetal and postnatal outcome after prenatal diagnosis and antenatal expectant management in a provincial tertiary referral center that does not offer fetal surgery. Methods: Retrospective cohort of 71 consecutive cases of prenatally diagnosed CCAM meeting study criteria (1996–2004). CVR was calculated on the initial ultrasound at the referral center, and associated with hydrops (Fisher’s exact test) and a composite adverse postnatal outcome consisting of death, intubation for respiratory distress, extracorporeal membrane oxygenation, non-elective surgery for symptomatology, or respiratory infection requiring hospital admission (Mann-Whitney test). Results: A CVR >1.6 was significantly associated with hydrops (p = 0.003). In addition, the CVR was significantly associated with the composite adverse postnatal outcome (p = 0.004) at a mean age of follow-up of 41 months (range <1–117 months). For CVR and postnatal outcome, the area-under-the-curve receiver operating characteristic was 0.81 (95% CI 0.69–0.93, p = 0.006), and choosing a CVR cut-off of <0.56, the negative predictive value was 100% (95% CI 0.85–1.00). Conclusion: In a provincial referral center with antenatal expectant management of CCAM, the CVR was associated with hydrops and postnatal outcome, with a CVR <0.56 predictive of good prognosis after birth.

Neonatal Outcomes of Prenatally Diagnosed Congenital Pulmonary Malformations

“…Previously published values are highly variable, probably due to major differences in the definitions used and case selection bias. Respiratory symptoms at birth were noted in 13% to 49% of cases in previous studies, [7][8][9][10][11][12] with neonatal ventilatory support required Data are presented as n (%), median (IQR), or n/N (%). a Two cases for which data were unavailable in the subgroup without oxygen supplementation.…”

“…1 This frequency is consistent with previously published evaluations, which ranged from 3% to 23%. [5][6][7]11,12 Of interest, early surgical removal of the malformation (ie, within the first 30 days after birth) was required in only a minority of children with severe respiratory symptoms at birth. Spontaneous improvement was observed in 2 of the 6 children on conventional mechanical ventilation and in 3 of the 4 children with noninvasive ventilation.…”

“…3 Many of the previous studies evaluating neonatal respiratory morbidity in children with CPM used crude indicators, such as mortality or the need for immediate surgery. 2,[4][5][6] Only a few have focused on the prevalence of respiratory symptoms at birth, [7][8][9][10][11][12] and the findings of these studies are often limited by an imprecise definition of the respiratory symptoms, the use of a single-center retrospective design, or a small number of patients. The heterogeneity of these studies probably contributed to the wide range of percentages of neonates with respiratory distress reported (13%-49%).…”

“…The heterogeneity of these studies probably contributed to the wide range of percentages of neonates with respiratory distress reported (13%-49%). Very few of these studies attempted to identify factors predictive of respiratory symptoms at birth, 8,10,12 and conflicting results have been published concerning the predictive value of the size of the antenatal mass. Some of these studies have identified congenital pulmonary malformation volume ratio (CVR) as a significant predictor of neonatal respiratory distress, but discordant cutoff points have been proposed.…”

“…Some of these studies have identified congenital pulmonary malformation volume ratio (CVR) as a significant predictor of neonatal respiratory distress, but discordant cutoff points have been proposed. 8,12 Nevertheless, an understanding of these predictors is essential to optimize the care pathway after the prenatal diagnosis of lung malformations and to ensure that the appropriate level of care is provided at delivery. In 2008, a prospective registry of children born with CPM was established in France, under the auspices of the national health authority.…”