Cystic adenomatoid malformation volume ratio predicts outcome in prenatally diagnosed cystic adenomatoid malformation of the lung
Abstract
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 results of an initial retrospective review of CVR at presentation suggested its usefulness as a predictor of outcome in CAM. The authors now report the results of prospective use of the CVR both to track tumor growth and regression during gestation and confirm its predictive value in fetuses with CAM. Methods: In the retrospective review performed between November 1998 and August 1999, 32 fetuses with CAM were reviewed and divided into those with hydrops and those in whom hydrops never developed. The CVR was determined by measuring 3 dimensions of the CAM using the formula for the volume of an ellipse and dividing by the head circumference to correct for differences in gestational age. Of the 32 fetuses in the retrospective study, the 8 that had hydrops had a significantly higher CVR (3.1 [plusmn] 1.1) compared with hydropic fetuses (0.74 [plusmn] 0.48; P [lt ] .001). The mean of the nonhydropic fetus's CVR plus 2 standard deviations (0.74 + 0.96 = 1.7) was used as a cutoff in the subsequent prospective study. From September 1, 1999 through March 1, 2001, the authors evaluated prospectively 58 patients with CAM by CVR measurement. These patients were followed up with serial ultrasound scans, and CVR at presentation correlated with the development of hydrops, survival, need for fetal intervention, and the need for ventilatory support or extracorporeal membrane oxygenation (ECMO), and length of hospital stay postnatally. The indication for fetal intervention was the development of hydops. Results: The fetuses with CVR [le ]1.6 (n = 42) were considered to be at low risk for the development of hydrops, and those with CVR greater than 1.6 (n = 16) were considered at increased risk for developing hydrops. Of the 42 fetuses in the low-risk group, 7 (16.7%) developed hydrops, and all but 1 had a dominant cyst. If CAMs with a dominant cyst are excluded, only 1 of 36 (2.8%) of CAMs with CVR [le ] 1.6 developed hydrops (P [lt ] .001). In fetuses with CVR at presentation more than 1.6, 12 of 16 (75%; P [lt ] .005) developed hydrops. Seventeen fetuses underwent fetal treatment (8 CVR [le ] 1.6; 9 CVR [gt ] 1.6): 7 patients required open fetal surgery (survival rate, 2 of 7), 6 patients thoracoamniotic shunting (survival rate, 6 of 6); and 4 patients cyst aspiration (survival rate, 4 of 4). All survivors of fetal intervention required at a least brief period of ventilatory support; none required ECMO. Conclusions: A CVR of greater than 1.6 at presentation accurately predicts increased risk of hydrops developing in CAM. A CVR of [le ]1.6 at presentation suggests that the risk of hydrops developing in the absence of a dominant cyst is less than 3%. The CVR is a useful sonographic indicator of fetuses at risk for hydrops who require close ultrasound observation and possible fetal intervention.
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Management and Outcomes of Patients With High-Risk (Congenital Lung Malformation Volume Ratio≥ 1.6) Congenital Lung Malformations
2024, Journal of Surgical ResearchCongenital lung malformations (CLMs) have a variable natural history. Larger lesions with CLM volume ratio (CVR) ≥ 1.6 are associated with hydrops and fetal mortality. The purpose of this study is to describe the management and outcomes of high-risk (CVR ≥ 1.6) CLM patients.
A retrospective cohort study was performed for all fetuses evaluated between May 2015 and May 2022. Demographics, prenatal imaging factors, prenatal and postnatal treatment, and outcomes were collected. Descriptive statistics were used to compare the cohorts.
Of 149 fetal CLM patients referred to our fetal center, 21/149 (14%) had CVR ≥ 1.6. One CLM patient had intrauterine fetal demise, and 2 patients were lost to follow-up. Of the remaining 18 patients, 11/18 (67%) received maternal steroids. Seven out of 18 patients (39%) underwent resection at the time of delivery with 1/7 (14%) undergoing exutero intrapartum treatment (EXIT)-to-resection, 5/7 (71%) undergoing EXIT-to-exteriorization-to-resection, and 1/7 (14%) undergoing a coordinated delivery to resection; among those undergoing resection, there were 2 fatalities (28.5%). Seven out of 18 (39%) patients required urgent neonatal open lobectomies, and the remaining 4/18 (22%) patients underwent elective thoracoscopic lobectomies with no mortality.
The natural history and outcomes of severe CLM patients remain highly variable. The EXIT-to-exteriorization-to-resection procedure may be a safe and effective approach for a subset of CLM patients with persistent symptoms of mass effect and severe mediastinal shift due to the observed decreased operative time requiring placental support observed in our study.
Antenatal diagnosis of bronchopulmonary sequestration: A case report and review of the literature
2024, Radiology Case ReportsCongenital lung malformations are a constellation of pathologies that can be diagnosed antenatally by ultrasound and fetal MRI. Ultrasound is considered the modality of choice for a routine assessment of second-trimester scans worldwide. Bronchopulmonary sequestration (BPS) and congenital pulmonary airway malformation (CPAM) are the 2 most common echogenic chest masses discovered incidentally during routine ultrasound scans in the second trimester. This paper describes BPS and differentiates it from CPAM sonographically in utero. An extensive literature search involving antenatal ultrasound is undertaken to review the most up-to-date understanding of the BPS. Furthermore, a case study at our institution and the literature review will help better describe the salient features of BPS. A 41-year-old female G3P1 visits our department for a routine second-trimester ultrasound. An echogenic lesion with a cystic component is visualized in this scan. Based on the grayscale and color imaging, this complex echogenic lesion was reported as CPAM and was referred to fetal assessment for confirmation. The fetal assessment diagnosed the lesion as BPS because of the pathognomonic feeding vessel from the thoracic aorta. Regardless of the congenital lung mass, any large mass compromising fetal well-being is an indication for intervention. The prognosis of BPS in the absence of fetal hydrops is excellent. A robust collaboration among radiologists, obstetricians, and pediatricians is required for the best outcome for the pregnancy and the neonate.
Foetal MRI for cardiovascular and thoracic diseases
2024, RadiologiaLa ecografía es la técnica de elección en el estudio de la patología cardiotorácica fetal, pero incluso en manos expertas, tiene algunas limitaciones, sobre todo en casos de mala ventana acústica. La resonancia magnética (RM) torácica fetal es una técnica complementaria en el estudio de la patología torácica que permite, por su caracterización tisular y carácter multiplanar, diagnosticar las diferentes lesiones, valorar el grado de afectación y detectar posibles anomalías asociadas, aportando información útil para el manejo, pronóstico y tratamiento pre y posnatal.
La RM cardiovascular fetal es una nueva herramienta diagnóstica para estudiar el corazón y la circulación fetal que puede ser útil como modalidad de imagen alternativa cuando la ecografía no es concluyente. Además, puede aportar información anatómica y fisiológica útil para establecer un pronóstico o tratamiento médico y para planificar intervenciones cardiovasculares invasivas prenatales.
Ultrasound is the main imaging technique used to assess foetal cardiovascular and thoracic diseases. Nonetheless, even when performed by senior physicians, it has some limitations, particularly in cases of suboptimal acoustic window. Foetal chest magnetic resonance imaging is a complementary technique for studying thoracic pathology. Due to its ability to provide tissue characterisation and its multiplanar capabilities, it can diagnose multiple lesions, assess the degree of involvement and detect possible associated anomalies, providing valuable information for management, prognosis and both prenatal and postnatal treatment.
Foetal cardiac magnetic resonance is a novel diagnostic tool for studying the foetal heart and circulation and it is useful as an alternative imaging modality when ultrasound is inconclusive. Furthermore, it can provide useful anatomical and physiological information for establishing a prognosis or medical treatment and for planning invasive prenatal cardiovascular interventions.
Novel Clinical Algorithm for Prenatal Monitoring of Congenital Lung Malformations
2024, Journal of Surgical ResearchCongenital lung malformations (CLMs) are readily identified early in pregnancy with a variable natural history. Monitoring for lesion size and mediastinal shift (MS) is recommended following diagnosis. The purpose of this study is to propose a risk-stratified clinical algorithm for prenatal monitoring of CLM.
After ethical approval, all fetuses with CLMs evaluated at our fetal center from January 2015 to June 2022 were retrospectively reviewed. Patient demographics, imaging characteristics, and fetal interventions were collected. Lesions were stratified by congenital lung malformation volume ratio (CVR) and the presence of MS. Descriptive statistics and receiver operating characteristic curves were employed in the analysis.
We analyzed 111 patients with a mean of 23.4 wk gestational age, a median CVR of 0.5 (interquartile range, 0.3-1.2), and MS in 76 of 111(68%) patients on initial evaluation. Among low-risk patients (CVR ≤1.1), 96% remained low-risk on final evaluation. No patients transitioned from low to high risk during the growth period. Patients with CVR >1.1 often had persistent MS (P < 0.001). Hydrops (5/111, 5%) and fetal intervention (4/111, 4%) only occurred in patients with CVR >1.1 (P < 0.001, P = 0.002) and MS (P = 0.144, P = 0.214). On receiver operating characteristic curve analysis, initial CVR >1.1 had 100% sensitivity and negative predictive value for hydrops and fetal intervention.
CLMs with initial CVR ≤1.1 are low risk for hydrops and fetal intervention. We propose a risk-stratified algorithm for the monitoring of CLM during the growth period based on CVR. While our experience suggests that patients with CLM and MS are at higher risk, the current subjective assessment of MS is not adequately predictive. Incorporating an MS grading system may further refine risk stratification in the management of CLM.
Prenatal Measurements of Congenital Lung Malformations: Can They Predict Postnatal Size?
2024, Journal of Surgical ResearchPrenatally diagnosed congenital lung malformations (CLMs) are monitored via ultrasound and measured by congenital pulmonary airway malformation volume ratios (CVRs) which can predict postnatal respiratory symptoms. This study compared CVR to postnatal lesion size to help guide prenatal counseling.
A retrospective chart review evaluated the prenatal imaging and postnatal outcomes for patients who were prenatally diagnosed with CLMs and had a postnatal computed tomography (CT) scan at one institution.
Fifty-seven patients were included. Four had symptoms requiring urgent resection. The remaining were discharged and had clinic follow-up with CT scan to determine next steps: five had no identified lesions, eight had lesions whose diagnosis did not warrant an operation, and 40 had lesions whose diagnosis rendered size a factor in operative decision-making. Of these 40, 26/40 patients (65%) underwent elective resection (median maximum CVR 0.97; median lesion size 4 cm) and 14/40 patients (35%) were observed without resection (median maximum CVR 0.5; median lesion size 3 cm). There was a positive correlation between prenatal CVR and postnatal lesion size, with R-squared = 0.46. Maximum CVRs were better than last CVRs when predicting whether postnatal CT size would fall above or below our institution's level of recommended resection, with an area under the curve of 0.85 and a CVR cut-point of 0.61.
For newborns with asymptomatic CLMs, higher maximum CVRs correlated with larger size on postnatal CT. A maximum CVR ≤0.6 was correlated with a smaller postnatal CT size that may be eligible for nonoperative management. While these results are not intended to recommend surgery based on higher CVRs alone, this information could potentially be used to reassure expectant parents whose babies’ prenatal imaging demonstrate lower maximum CVRs.
Prenatally Diagnosed Large Lung Lesions: Timing of Resection and Perinatal Outcomes
2023, Journal of Pediatric SurgeryFetuses with large lung lesions including congenital cystic adenomatoid malformations (CCAMs) are at risk for cardiopulmonary compromise. Prenatal maternal betamethasone and cyst drainage for micro- and macrocystic lesions respectively have improved outcomes yet some lesions remain large and require resection before birth (open fetal surgery, OFS), at delivery via an Ex Utero Intrapartum Treatment (EXIT), or immediately post cesarean section (section-to-resection, STR). We sought to compare prenatal characteristics and outcomes in fetuses undergoing OFS, EXIT, or STR to inform decision-making and prenatal counseling.
A single institution retrospective review was conducted evaluating patients undergoing OFS, EXIT, or STR for prenatally diagnosed lung lesions from 2000 to 2021. Specimens were reviewed by an anatomic pathologist. Lesions were divided into “CCAMs” (the largest pathology group) and “all lung lesions” since pathologic diagnosis is not possible during prenatal evaluation when care decisions are made. Prenatal variables included initial, greatest, and final CCAM volume-ratio (CVR), betamethasone use/frequency, cyst drainage, and the presence of hydrops. Outcomes included survival, ECMO utilization, NICU length of stay (LOS), postnatal nitric oxide use, and ventilator days.
Sixty-nine percent (59 of 85 patients) of lung lesions undergoing resection were CCAMs. Among patients with pathologic diagnosis of CCAM, the initial, largest, and final CVRs were greatest in OFS followed by EXIT and STR patients. Similarly, the incidence of hydrops was significantly greater and the rate of hydrops resolution was lower in the OFS group. Although the rate of cyst drainage did not differ between groups, maternal betamethasone use varied significantly (OFS 60.0%, EXIT 100.0%, STR 74.3%; p = 0.0378). Notably, all OFS took place prior to 2014. There was no difference in survival, ventilator days, nitric oxide, NICU LOS, or ECMO between groups. In multiple variable logistic modeling, determinants of survival to NICU discharge among patients undergoing resection with a pathologic diagnosis of CCAM included initial CVR <3.5 and need for <3 maternal betamethasone doses.
For CCAMs that remain large despite maternal betamethasone or cyst drainage, surgical resection via OFS, EXIT, or STR are viable options with favorable and comparable survival between groups. In the modern era there has been a shift from OFS and EXIT procedures to STR for fetuses with persistently large lung lesions. This shift has been fueled by the increased use of maternal betamethasone and introduction of a Special Delivery Unit during the study period and the appreciation of similar fetal and neonatal outcomes for STR vs. EXIT and OFS with reduced maternal morbidity associated with a STR. Accordingly, efforts to optimize multidisciplinary perinatal care for fetuses with large lung lesions are important to inform patient selection criteria and promote STR as the preferred surgical approach in the modern era.
Level IV.