Obstetrician patterns of steroid administration for the prenatal management of congenital pulmonary airway malformations

Abstract

BACKGROUND:

Congenital pulmonary airway malformation (CPAM) is the most common prenatally-diagnosed lung malformation. This lesion, classified as macrocystic or microcystic, can lead to significant fetal compromise. Management options include observation, maternal antenatal steroid administration, and fetal surgical intervention. Current evidence suggests that microcystic (but not macrocystic) lesions and those with a cyst volume ratio (CVR) >1.6 are responsive to steroid therapy. The objective of this study was to identify patterns of prenatal steroid administration for the management of CPAMs and to identify characteristics of CPAMs prompting steroid administration.

METHODS:

An 18-question survey was distributed to obstetricians from the Pregnancy-Related Care Research Network (PRCRN) and the North American Fetal Therapy Network (NAFTNet), from January to April 2019, to capture antenatal steroid prescribing patterns.

RESULTS:

Response rates were 28.3% (138/487) for PRCRN and 63.3% (19/30) for NAFTNet. Among PRCRN members, 16.8% administered prenatal steroids, with most (77.2%) doing so for both microcystic and macrocystic CPAMs; corresponding percentages for NAFTNet members were 90.9% and 52.6%. Two thirds (65.6%) of obstetricians who administer steroids do so for a CVR > 1.6, without evidence of mediastinal shift or hydrops fetalis.

CONCLUSIONS:

There is a lack of consensus among obstetricians as to the CPAM characteristics that should prompt administration of prenatal steroids. Many surveyed obstetricians do not use cyst type or CVR to guide decision-making regarding steroid therapy.

Keywords

Survey CPAM steroids CVR

1 Introduction

Congenital pulmonary airway malformations (CPAMs) are cystic hamartomas resulting from abnormal branching morphogenesis in the lung. They arise from tracheal, bronchial, bronchiolar or alveolar tissue [1], and represent the most common congenital lung lesion [2, 3]. With the widespread availability of ultrasound imaging, CPAMs are now frequently diagnosed prenatally [2, 4]. During fetal life, the lesion typically grows rapidly from diagnosis at 18–20 weeks gestation until approximately 28 weeks’ gestation, followed by a period of stabilization, and in many cases, spontaneous regression [5, 6]. In severe cases, however, the lesion continues to grow and ultimately may cause severe lung compression, mediastinal shift, hydrops fetalis, and/or fetal demise [7, 8].

Prenatal treatment options for CPAMs range from close observation with serial ultrasounds to open fetal surgery for lesion resection. Historically, the rate of spontaneous regression for congenital lung lesions ranged from 8–49% [9, 10], depending on associated features. However, lesions associated with hydrops fetalis had a universally fatal prognosis [7]. The early 2000’s saw the advent of open fetal surgery with lesion resection for CPAMs causing fetal hydrops, which reduced mortality by 50% [11 –14]. Because of the risk of preterm labor associated with open fetal surgery, women undergoing the procedure were given prophylactic steroids to promote fetal lung maturation. Incidentally, it was noted that the fetuses exposed to prenatal steroids and not undergoing lesion resection demonstrated a remarkably high incidence of CPAM regression and resolution of fetal hydrops [11]. The discovery that a noninvasive, relatively benign medical intervention could dramatically reduce fetal morbidity and mortality led to the rapid clinical adoption of prenatal steroid administration, and was further reinforced by subsequent retrospective studies documenting a mortality rate of <20% among hydropic fetuses with microcystic CPAMs receiving antenatal steroids. It is important to note, however, that macrocystic CPAMs did not respond as favorably to antenatal steroid administration, and there was no evidence of a survival benefit for this sub-population [7 , 13]. This relatively rapid practice change in the absence of prospective studies evaluating the optimal timing, dosing, and patient population most responsive to steroid therapy has left it to the individual practitioner to determine when, and for whom, steroids should be prescribed. A cyst volume ratio (CVR) >1.6 and presence of mediastinal shift or hydrops fetalis are associated with poorer fetal and neonatal outcomes [8 , 16], and are often, though not universally, used as criteria to prompt steroid administration. We hypothesized that steroid prescribing practices would vary significantly among obstetricians, and sought to evaluate this hypothesis by analyzing survey data from a national sample of general and subspecialty-trained obstetricians.

2 Methods

2.1 Survey development and piloting

We developed an 18-question survey to characterize obstetrical practice patterns in the prenatal management of CPAMs [Appendix 1]. The goal of the survey was to determine the extent of practice variability or consensus among a national sample of obstetricians, representing both general practitioners and subspecialists. The survey collected information about (1) obstetrician level of training, (2) CPAM characteristics prompting prenatal steroid administration), (3) earliest gestational age at which steroids would be administered, and (4) how additional prenatal findings, including CVR and presence of mediastinal shift or hydrops fetalis, influence the decision to administer steroids.

The survey was piloted among practicing obstetricians at Johns Hopkins University School of Medicine, in Baltimore, Maryland, for accuracy, clarity, and consistency. The pilot group included general obstetricians (residents, fellows, and attending physicians) and specialists (maternal fetal medicine and fetal therapy). Once the pilot group approved the survey, we sought and received approval from the Institutional Review Board at Johns Hopkins School of Medicine (IRB 00186426).

2.2 Survey administration

The survey was administered from January 4, 2019 through April 4, 2019 to members of two national organizations – the Pregnancy-Related Care Research Network (PRCRN) and the North American Fetal Therapy Network (NAFTNet) – which represent general obstetricians and fetal medicine subspecialists respectively. The PRCRN is a research network of academic and non-academic institutions throughout the United States conducting collaborative research examining pregnancy-related care. The group maintains a contact list of approximately 500 practicing obstetricians who have agreed to be surveyed regarding their practice patterns. NAFTNet is a consortium of medical centers in the United States and Canada with expertise in fetal surgery and other fields related to the multidisciplinary care of complex fetal disorders. A large percentage of the membership are obstetricians with additional training in maternal fetal medicine (MFM).

The survey was sent to members of the PRCRN via the network’s administrative staff, based at the University of Washington. Members received a cover letter explaining the research study and an email link to the survey, which was administered via Qualtrics. Three subsequent email reminders were sent to non-responders over a one-month period. The survey was also distributed to the subset of NAFTNet members who were obstetricians, at the organization’s annual conference in Chicago, Illinois, on April 4, 2019. Individuals received a cover letter explaining the research study, and a paper version of the survey.

2.3 Statistical analysis

Results were analyzed using descriptive and exploratory statistics with SPSS, version 24 (Armonk, NY).

3 Results

3.1 Survey response

A total of 138 PRCRN members and 19 NAFTNet members returned the survey, yielding response rates of 28.3% (138/487) and 63.3% (19/30), respectively. Within the PRCRN group, there were seven incomplete surveys which were discarded, leaving 131 completed surveys for analysis. Within the NAFTNet group, there were four incomplete surveys; however, respondents wrote in their answers (with their preferred answer choice being easily discernable), or only neglected to answer demographic questions, and thus were included in the analysis.

When comparing demographic characteristics between respondents, we noted three salient differences: (i) all NAFTNet members were MFM specialists, whereas only 15% of PRCRN members were MFM specialists, (ii) NAFTNet members predominately practiced in academic medical settings, while PRCRN members predominately practiced in community settings, and (iii) most NAFTNet members were male (74%), whereas most PRCRN members were female (70%) (Table 1).

Table 1
Comparison of professional profiles and demographics between general practitioners (PRCRN) and sub-specialists (NAFTNet). Data presented are numbers and percentages

PRCRN (n = 131) NAFTNet (n = 19)

Professional profile

Years in practice

<5 6 (4.6%) 2 (10.5%)

5–10 28 (21.4%) 5 (26.3 %)

11–15 13 (9.9%) 5 (26.3%)

>15 84 (64.1%) 7 (36.9%)

Practice setting

Academic 43 (32.8%) 16 (84.2%)

Community 74 (56.5%) 1 (5.3%)

Mixed 14 (10.7%) 2 (10.5%)

Subspecialty training

Yes - MFM 20 (15.3%) 19 (100%)

Yes - other 11 (8.4%) 0

No 100 (76.3%) 0

Demographics

Gender

Male 40 (30.5%) 14 (73.7%)

Female 91 (69.5%) 4 (21.0%)

Not reported 0 1 (5.3%)

Race

White 111 (84.7%) 16 (84.2%)

Black 7 (5.3%) 0

Asian 12 (9.2%) 2 (10.5%)

Other 1 (0.8%) 0

Not reported 0 1 (5.3%)

		PRCRN (n = 131)	NAFTNet (n = 19)
Professional profile
Years in practice
	<5	6 (4.6%)	2 (10.5%)
	5–10	28 (21.4%)	5 (26.3 %)
	11–15	13 (9.9%)	5 (26.3%)
	>15	84 (64.1%)	7 (36.9%)
Practice setting
	Academic	43 (32.8%)	16 (84.2%)
	Community	74 (56.5%)	1 (5.3%)
	Mixed	14 (10.7%)	2 (10.5%)
Subspecialty training
	Yes - MFM	20 (15.3%)	19 (100%)
	Yes - other	11 (8.4%)	0
	No	100 (76.3%)	0
Demographics
Gender
	Male	40 (30.5%)	14 (73.7%)
	Female	91 (69.5%)	4 (21.0%)
	Not reported	0	1 (5.3%)
Race
	White	111 (84.7%)	16 (84.2%)
	Black	7 (5.3%)	0
	Asian	12 (9.2%)	2 (10.5%)
	Other	1 (0.8%)	0
	Not reported	0	1 (5.3%)

3.2 Prevalence of antenatal steroid administration

Among PRCRN members, 17% (22/131) report administering antenatal steroids for the management of CPAM, and all of whom are MFM physicians. For those PRCRN members who do not administer steroids, 15/109 (14%) report continuing to manage patients with CPAMs; the rest either refer these patients (56/109, 51%) or otherwise do not manage their prenatal care (38/109, 35%).

Conversely, nearly all NAFTNet members (17/19) administer steroids for fetal management of CPAMs, and all report subspecialty training in maternal-fetal medicine. With respect to choice of steroids, all 22 PRCRN members report administering betamethasone, while 68% (13/19) of NAFTNet members administer betamethasone (Table 3).

Table 2
Comparison of administration of maternal steroids for management for CPAMs between general practitioners (PRCRN) and sub-specialists (NAFTNet)

Do you ever administer maternal steroids for the management of fetal CPAMs? PRCRN¹ (n = 131) NAFTNet¹ (n = 19)

Yes

For all CPAMs 17 (13%) 11 (57.9%)

Only for microcystic CPAMs, but not macrocystic CPAMs 2 (1.5%) 6 (31.5%)³

Only for macrocystic CPAMs, but not microcystic CPAMs 3 (2.3%) 0

No

I do not administer steroids, and I continue to manage these patients 15 (11.5%)² 1 (5.3%)

I do not administer steroids, and I refer these patients 56 (42.7%)² 1 (5.3%)

I do not see these types of patients 38 (29.0%)² 0

Do you ever administer maternal steroids for the management of fetal CPAMs?	PRCRN¹ (n = 131)	NAFTNet¹ (n = 19)
Yes
For all CPAMs	17 (13%)	11 (57.9%)
Only for microcystic CPAMs, but not macrocystic CPAMs	2 (1.5%)	6 (31.5%)³
Only for macrocystic CPAMs, but not microcystic CPAMs	3 (2.3%)	0
No
I do not administer steroids, and I continue to manage these patients	15 (11.5%)²	1 (5.3%)
I do not administer steroids, and I refer these patients	56 (42.7%)²	1 (5.3%)
I do not see these types of patients	38 (29.0%)²	0

¹Data presented are numbers and percentages. ²If the participant answered “no”, the survey ended, as all subsequent survey questions pertained to steroid administration. ³One participant hand wrote they would administer steroids only for hydrops.

Table 3

Comparison of decision making about prenatal management of CPAMs between general practitioners (PRCRN) and sub-specialists (NAFTNet)

		PRCRN¹ (n = 22²)	NAFTNet¹ (n = 19)
Which steroid do you administer for the management of CPAM?
	Betamethasone	22 (100%)	13 (68.4%)
	Dexamethasone	0	2 (10.5%)
	Other	0	1 (5.3%)
	No answer	0	3 (15.8%)
Do you use CVR measurements when deciding to administer steroids?
	Yes	7 (31.8%)	13 (68.4%)
	No	15 (68.2%)	5 (26.3%)
	No answer	0	1 (5.3%)
Does the CVR factor into your decision for administering steroid if there is evidence of:
hydrops?
	Yes	7 (31.8%)	8 (42.1%)
	No	15 (68.2%)	10 (52.6%)
	No answer	0	1 (5.3%)
mediastinal shift?
	Yes	10 (45.5%)	7 (36.8%)
	No	12 (54.5%)	11 (57.9%)
	No answer	0	1 (5.3%)
Do you ever administer a second course of steroids?
	Yes	15 (68.2%)	12 (63.2%)
	No	7 (31.8%)	6 (31.5%)
	No answer	0	1 (5.3%)
Do you use MRI in the third trimester to evaluate/follow CPAMs?
	Yes	10 (45.5%)	11 (57.9%)
	No	12 (54.5%)	8 (42.1%)

¹Data presented are numbers and percentages. ²n = 22 based on built-in skip pattern for electronic survey.

3.3 Lesion characteristics used for decision-making

Among PRCRN members who administer steroids, most (68%) report they do not use CVR in their decision-making regarding steroid administration (Table 3). Consistent with this finding, nearly three quarters (16/22) report that they would administer steroids for a CVR < 1 without evidence of mediastinal shift or hydrops fetalis (Fig. 1). This percentage did not change for larger CPAMs (CVR 1–1.6 or >1.6) without evidence of mediastinal shift or hydrops fetalis, (16/22), or in the presence of mediastinal shift alone (15/22).

Fig.1

Variations in earliest GA for steroid administration, according to health of the child and between general obstetricians (PRCRN) and national experts (NAFTNet). Note that PRCRN responses are consistent across increasing CVR measurements. NAFTNet members are more likely to administer steroids for a CVR > 1.6. There is no consensus in gestational age for steroid administration.

NAFTNet members were twice as likely as PRCRN members to use CVR measurements when deciding whether to administer steroids (68% versus 32%) (Table 3). Among NAFTNet members, 26% (5/19) would administer steroids for a CVR of <1.6 without evidence of mediastinal shift or hydrops fetalis, and 63% would administer steroids for a CVR of >1.6 without evidence of mediastinal shift or fetal hydrops.

Both PRCRN and NAFTNet members were less likely to use CVR in their decision-making for steroid administration when there was evidence of hydrops or mediastinal shift (Table 3). Of the 22 PRCRN members who report administering steroids, 77% would give steroids for both microcystic and macrocystic CPAMs (Table 2). In comparison, among the 19 NAFTNet members who administer steroids, 11 (53%) would give steroids for a CPAM regardless of cyst type, and six (32%) would administer steroids for microcystic CPAMs alone (Table 2). Three PRCRN members and zero NAFTNet members report administering steroids exclusively for macrocystic lesions.

3.4 Earliest gestational age for steroid administration, number of steroid courses, and use of MRI

Steroids were administered at gestational ages ranging from 23.0 to 28.6 weeks among PRCRN and NAFTNet members; there was no gestational age at which antenatal steroids were most frequently administered (Fig. 1). If necessary, both PRCRN and NAFTNet members would administer a second course of steroids for microcystic CPAMs (68% and 63% of respondents, respectively). The NAFTNet members were more likely to use third trimester MRIs to evaluate CPAMs than were the PRCRN members (58% versus 45% respectively).

4 Discussion

Our survey results demonstrate marked variation in steroid prescribing patterns for the prenatal management of CPAMs among PRCRN and NAFTNet members. We speculate that this variability has been driven by three key factors: (1) the absence of guidelines delineating when and under what circumstances steroids should be administered, (2) lack of familiarity with the literature supporting the efficacy of and indications for antenatal steroid administration in the management of prenatally diagnosed CPAM, and (3) an assumption that regression of the CPAM after steroid administration reflects its expected (untreated) trajectory, and would have occurred regardless of steroid administration.

Our results indicate that most obstetricians who prescribe antenatal steroids agree that the presence of hydrops fetalis should prompt steroid administration. However, cyst type does not appear to influence the decision to administer steroids, as many obstetricians will prescribe them for either type, despite a paucity of evidence demonstrating efficacy in macrocystic lesions. There does not appear to be provider consensus regarding the use of CVR or the presence of mediastinal shift in determining which women have the highest likelihood of deriving therapeutic benefit from antenatal steroids. This finding was surprising given that mediastinal shift traditionally occurs in the presence of an enlarging CPAM, which may subsequently progress to hydrops fetalis or fetal death [15 , 18].

We noted marked differences between PRCRN and NAFTNet physicians in their use of CVR measurements in informing prenatal management strategies. Approximately one third of PRCRN physicians and two thirds of NAFTNet physicians used CVR measurements to inform their decision to administer antenatal steroids. Among PRCRN physicians, steroid administration practices remain consistent as CVR increases, whereas for NAFTNet physicians, steroid administration more than doubles when the CVR is >1.6 without evidence of hydrops fetalis or mediastinal shift.

The CVR, a measure of the relative size of the CPAM, is a useful tool in predicting the risk that a lesion will progress to the development of mediastinal shift or hydrops fetalis. A CVR of >1.6 is associated with an increased risk of hydrops fetalis and subsequent fetal demise, and thus, many obstetricians will use this value as an indication for steroid administration. However, once hydrops or mediastinal shift have occurred, it is unclear whether CVR measurements remain relevant to clinical decision-making. We found that obstetricians do consider CVR when deciding whether to administer steroids in the presence of mediastinal shift (17/41) or hydrops fetalis (15/41), which suggests that many, but not all obstetricians will administer steroids independent of CVR when hydrops fetalis or mediastinal shift are present. However, this also highlights differences in the extent to which CVR informs medical decision-making, and how it can increase or mitigate clinician perceptions of risk of poor neonatal outcome in the presence of other independent risk factors [5, 19].

There is no consensus regarding the optimal number of steroid courses to be administered and the earliest gestational age at which steroid administration should begin. Published studies report that for high risk CPAM lesions (CVR > 1.6, presence of mediastinal shift or hydrops fetalis) where an initial course of steroids has failed, a second course of steroids may be effective in improving outcomes (reduction in CVR, resolution of mediastinal shift or hydrops fetalis) [8, 12]. This demonstration of potential benefit, coupled with a relatively benign side effect profile and high morbidity and mortality associated with open fetal surgery, mediastinal shift or hydrops fetalis, may make some providers more amenable to administration of additional steroid courses.

We also found that there was no clear consensus as to the use of third trimester fetal MRI. MRI offers better tissue characterization, spatial resolution, and enhanced morphologic and volumetric measurements of the fetal lungs, as well as more clearly delineating the CPAM margins compared to ultrasound, especially during the third trimester [20]. The lack of consensus most likely stems from the variable availability, higher costs and longer study times associated with fetal MRIs compared to ultrasound, and advances in ultrasound quality and availability that make diagnosis and monitoring of CPAM lesions throughout pregnancy accurate, informative, and relatively easy [2].

Most of the 131 PRCRN members surveyed, 83%, did not administer steroids for the management of fetal CPAMs. The low frequency of steroid administration among the PRCRN members is most likely because these patients are largely referred or managed elsewhere.

Interestingly, this study identified a subset of providers (∼11%) who would not administer steroids for prenatally diagnosed CPAMs yet continue to manage their prenatal care. Although this represents a minority of practitioners, we hypothesize that the willingness to expectantly manage prenatally-diagnosed CPAMs arises from their known natural evolution, and the significant proportion of lesions that spontaneously regress without intervention. As the rate of spontaneous regression ranges from 8–49% [9, 10], some obstetricians may feel that most patients will have an uncomplicated prenatal course with a low risk of fetal compromise, and therefore feel comfortable providing ongoing obstetrical management. The decision to continue expectant management or refer to another provider may be influenced by individual obstetrician experience, parental preferences, and ease of access to subspecialty care.

Overall, the results of this study highlight a national lack of consensus among obstetricians, regardless of training background, as to the circumstances under which steroids should be administered for the prenatal management of CPAM lesions. The study describes the prevalence of obstetrician practices regarding maternal steroid administration, and highlights some differences in management strategies between general obstetricians and those with additional training in maternal fetal medicine. Furthermore, it emphasizes the lack of data to inform clinical practice decisions. Because of this lack of data, the ability of obstetricians to develop evidence-based clinical management algorithms and guidelines for the prenatal management of CPAMs is severely curtailed, and results in marked variability in practice patterns. However, our survey also indicates that the results of clinically-relevant observational studies may not be widely known and have not been uniformly incorporated into routine obstetrical care. This suggests that educational interventions informing obstetricians of the current evidence addressing antenatal steroid use for management of CPAMs, the utility of serial CVR measurements in assessing and managing CPAMs, and the importance of cyst type in predicting response to steroids could have a significant impact on standardizing provider prescribing practices and the prenatal management of CPAMs.

A key strength of this study is the inclusion of members from both PRCRN and NAFTNet, which allows for comparisons between practices prevalent within the general obstetric community versus practices prevalent among specialists specifically trained in the prenatal management of congenital malformations, including CPAMs. Other strengths of the study included physician participants representing a range of years in practice and type of practice (academic vs community).

This study has several limitations. Although the response rate for the PRCRN and NAFTNet members was 28.3% and 63.3% respectively, the sample size of obstetricians reporting administration of antenatal steroids was small – only 41 respondents. Because we were specifically interested in the practices surrounding steroid administration, we did not investigate other modes of prenatal management such as shunts and resection. Additional concerns include partially completed surveys and technical difficulties in obtaining primary data for one question from the PRCRN group.

There are three key conclusions from our study: (i) there is considerable variability in the application of CVR to inform clinical decision-making in the prenatal management of CPAM, which may reflect a knowledge gap or lack of confidence in the clinical utility of the measurement, (ii) among obstetricians who administer antenatal steroids for CPAM, most use the presence of hydrops fetalis as an indication for administration, and (iii) a large proportion of physicians will administer antenatal steroids for macrocystic CPAMs despite a paucity of evidence to support this practice.

Our study highlights the need for collaborative prospective clinical trials to answer important clinical questions that directly inform provider management decisions in caring for fetal CPAMs. Specifically, there is very little information available to assist clinicians in identifying the patient population for whom steroid administration is the most effective, and to identify the optimal timing of steroid administration to achieve the best postnatal outcomes. Future studies should prioritize addressing this knowledge gap, with the goal of generating the evidence necessary to create rigorous, evidence-based consensus guidelines for the prenatal management of congenital lung lesions.

Disclosure statements

Funding

No funding was received for this work.

Ethical approval

This study was approved by the Institutional Review Board at Johns Hopkins School of Medicine (IRB 00186426).

Conflicts of interest

The authors have no conflicts of interest.

Footnotes

Appendix 1

Thank you for participating in this study. We are conducting a survey to help understand current practice patterns with regard to the maternal administration of steroids for fetal congenital pulmonary airway malformations (CPAMs). This study and survey has been approved by the institutional review board (IRB). This survey should take approximately 5 minutes to complete. Participants, their responses, and their individual practices will be kept confidential. Your completion of the survey will serve as your consent to be in this research study.

How many years have you been practicing as an OB/GYN physician?

<5 years

5 years to 10 years

11 years to 15 years

>15 years

Do you have any subspecialty training in Obstetrics/Gynecology?

Yes – Maternal Fetal Medicine

Yes – Other

In what Health Resources and Services Administration (HRSA) region do you practice?

HRSA Region 1: Maine, New Hampshire, Vermont, Massachusetts, Rhode Island, and Connecticut

HRSA Region 2: New York and New Jersey

HRSA Region 3: Pennsylvania, Maryland, Delaware, Virginia, and West Virginia

HRSA Region 4: Kentucky, Tennessee, North Carolina, South Carolina, Georgia, Florida, Alabama, and Mississippi

HRSA Region 5: Minnesota, Wisconsin, Illinois, Indiana, Michigan, and Ohio

HRSA Region 6: New Mexico, Texas, Oklahoma, Arkansas, and Louisiana

HRSA Region 7: Nebraska, Kansas, Iowa, and Missouri

HRSA Region 8: Montana, North Dakota, South Dakota, Wyoming, Colorado, and Utah

HRSA Region 9: Nevada, California, Arizona, and Hawaii

HRSA Region 10: Washington, Oregon, Idaho, and Alaska

How would you describe your practice setting?

Academic institution

Community practice

Mixed

Please specify your sex

Female

Male

I do not wish to answer

Please specify your ethnicity or race

White or Caucasian

Black or African American

Hispanic or Latino

Asian or Asian American

American Indian or Alaska Native

Native Hawaiian or other Pacific Islander

Another race

Do you ever administer maternal steroids for the management of fetal congenital pulmonary airway malformations (CPAMs)?

Yes, for all CPAMs

Yes, only for microcystic CPAMs, but not macrocystic

Yes, only for macrocystic CPAMs, but not microcystic

No, I do not administer steroids for CPAMs and I continue to manage these patients

No, I do not administer steroids for CPAMs and I refer these patients to another institution/practice, or provider

No, I do not see these patients

Which steroid do you administer for the management of CPAM?

Betamethasone

Dexamethasone

Other

Do you use cyst volume ratio (CVR) measurements when deciding to administer maternal steroids for the management of CPAMs?

Yes

Does the CVR factor into your decision for administering steroids if there is evidence of hydrops?

Yes

Does the CVR factor into your decision for administering steroids if there is evidence of mediastinal shift?

Yes

Do you ever administer a second course of steroids for the management of microcystic CPAM?

Yes

What is the earliest gestational age at which you would administer steroids for fetal microcystic CPAM with a CVR of < 1 WITHOUT evidence of hydrops or mediastinal shift?

I would not administer steroids

<23 weeks

23.0–23.6 weeks

24.0–24.6 weeks

25.0–25.6 weeks

26.0–26.6 weeks

27.0–27.6 weeks

28.0–28.6weeks

29.0–29.6weeks

30.0–30.6weeks

31.0–31.6weeks

>32 weeks

What is the earliest gestational age at which you would administer steroids for fetal microcystic CPAM with a CVR > 1 but less than 1.6 WITHOUT evidence of hydrops or mediastinal shift?

I would not administer steroids

<23 weeks

23.0–23.6 weeks

24.0–24.6 weeks

25.0–25.6 weeks

26.0–26.6 weeks

27.0–27.6 weeks

28.0–28.6 weeks

29.0–29.6 weeks

30.0–30.6 weeks

31.0–31.6 weeks

>32 weeks

What is the earliest gestational age at which you would administer steroids for fetal microcystic CPAM with a CVR > 1.6 WITHOUT evidence of hydrops or mediastinal shift?

I would not administer steroids

<23 weeks

23.0–23.6 weeks

24.0–24.6 weeks

25.0–25.6 weeks

26.0–26.6 weeks

27.0–27.6 weeks

28.0–28.6 weeks

29.0–29.6 weeks

30.0–30.6 weeks

31.0–31.6 weeks

>32 weeks

What is the earliest gestational age at which you would administer steroids for fetal microcystic CPAM WITH evidence of hydrops?

I would not administer steroids

<23 weeks

23.0–23.6 weeks

24.0–24.6 weeks

25.0–25.6 weeks

26.0–26.6 weeks

27.0–27.6 weeks

28.0–28.6 weeks

29.0–29.6 weeks

30.0–30.6 weeks

31.0–31.6 weeks

>32 weeks

What is the earliest gestational age at which you would administer steroids for fetal microcystic CPAM WITH evidence of mediastinal shift?

I would not administer steroids

<23 weeks

23.0–23.6 weeks

24.0–24.6 weeks

25.0–25.6 weeks

26.0–26.6 weeks

27.0–27.6 weeks

28.0–28.6 weeks

29.0–29.6 weeks

30.0–30.6 weeks

31.0–31.6 weeks

>32 weeks

Do you use MRI in the 3rd trimester to evaluate/follow for CPAM?

Yes

Thank you for completing this survey. Your responses will help us understand practices for CPAMs. Our goal is to share this information with the scientific and medical community through publication.

Acknowledgments

We would like to thank all the survey participants. The Pregnancy-Related Care Research Network is supported by the Health Research and Services Administration Grant UA6MC31609.

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