Prospective analysis of the prognostic value of prenatal MRI measurement of cystic volume ratio in fetal congenital cystic adenomatoid malformation.

BACKGROUND: The cystic volume ratio (CVR) has been used to predict the prognosis of fetal lung disease, but most of them are reported on ultrasound. The purpose of this study was to investigate the prognostic value of prenatal magnetic resonance imaging (MRI) measurement of CVR in fetal congenital cystic adenomatoid malformation (CCAM). METHODS: To collect follow-up data on postnatally confirmed CCAM fetuses. According to the size of CVR, they were divided into a CVR ≥1.26 group and a CVR <1.26 group. The lesions were divided into macrocystic (diameter ≥5 mm) and microcystic (diameter <5 mm) types according to the size of cysts in the lesions. The collected contents also included the location of the lesions, prenatal symptoms, postpartum clinical prognosis, and pregnancy outcomes. RESULTS: In all, 51 cases were collected. Of these, 11 were placed into the CVR ≥1.26 group, and 40 were placed into the CVR <1.26 group; meanwhile 34 were classified as macrocystic lesions and 17 as microcystic lesions; 7 cases involved a terminated pregnancy. In the CVR ≥1.26 group, 81.82% (9/11) and 36.36% (4/11) had displacement of large vessels/heart and increased amniotic fluid, respectively; meanwhile, in the CVR <1.26 group, these phenomena occurred in 35.00% (14/40) and 2.50% (1/40) of cases (P=0.006, 0.010). Additionally, 83.33% (5/6) of live infants in the CVR ≥1.26 group had dyspnea, a significantly higher proportion than the 21.05% (8/38) in the CVR <1.26 group (P=0.011). Moreover, 45.45% (5/11) of the cases in the CVR ≥1.26 group were terminated pregnancies, a significantly higher proportion than the 5.26% (2/38) in the CVR <1.26 group (P=0.001). Finally, the CVR of macrocystic lesions was 0.55 (0.34-1.31), which was significantly greater than the 0.34 (0.17-0.57) of microcystic lesions (P=0.022). Logistical regression analysis identified that CVR is an independent factor associated with the postpartum prognosis of CCAM. CONCLUSIONS: Prenatal symptoms and postpartum prognosis were worse than CVR <1.26 when fetal CVR ≥1.26 measured by prenatal MRI; and the measurement of CVR of CCAM through prenatal MRI has considerable practical value in prenatal consultation, evaluation, and postpartum treatment.

Use of Corticosteroids in Prenatal Treatment of Congenital Pulmonary Adenomatoid Malformation: Integrative Review.

OBJECTIVE: To review data on the use of corticosteroids for the treatment of fetuses with high-risk congenital pulmonary adenomatoid malformation (CPAM). METHODS: Integrative review based on the literature available on MEDLINE and LILACS, including articles published until November, 2020. RESULTS: The initial search resulted in 87 articles, 4 of which were selected for analysis, with all of them being retrospective descriptive observational studies. In the group of fetuses that received only a single corticosteroid cycle, the hydrops resolution rate was 70%, and the survival rate was 83.8%. In fetuses treated with 2 or more cycles of corticosteroids, there was an improvement in the condition of hydrops or edema in a single body compartment in 47%, and survival of 81.8% of the fetuses. CONCLUSION: The use of corticosteroids for the prenatal treatment of high-risk CPAM appears to be associated with an improvement in perinatal outcomes.

OBJETIVO: Revisar os dados sobre o uso de corticoide no tratamento de fetos com malformação adenomatoide pulmonar congênita (MAPC) de alto risco. MéTODOS: Revisão integrativa com base na literatura disponível no MEDLINE e LILACS, incluindo artigos publicados até novembro de 2020. RESULTADOS: A busca inicial resultou em 87 artigos, dos quais 4 foram selecionados para análise, todos tratando-se de estudos observacionais descritivos retrospectivos. No grupo de fetos que recebeu apenas um único ciclo de corticosteroide, a taxa de resolução da hidropsia foi de 70% e a taxa de sobrevida de 83,8%. Em fetos tratados com 2 ou mais ciclos de corticosteroides, houve melhora do quadro de hidropsia ou edema em um único compartimento corporal em 47% dos fetos e taxa de sobrevida de 81,8%. CONCLUSãO: O uso de corticosteroides para o tratamento pré-natal da MAPC de alto risco parece estar associado à melhora dos resultados perinatais.

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

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.

Effect of single and multiple courses of maternal betamethasone on prenatal congenital lung lesion growth and fetal survival.

PURPOSE: Administration of maternal betamethasone (BMZ) is a therapeutic option for fetuses with large microcystic congenital lung lesions at risk for, or causing, hydrops. Not all fetuses respond to a single course of BMZ. We review our experience with the use of single and multiple courses of maternal BMZ for the management of these patients. METHODS: A retrospective review of fetuses with congenital lung lesions managed with maternal BMZ from 2003 to 2014 was performed. RESULTS: Forty-three patients were managed with prenatal steroids (28 single course, 15 multiple courses). Single course recipients demonstrated a reduction in lesion size and resolution of hydrops in 82% and 88% of patients respectively compared to 47% and 56% in recipients of multiple steroid courses. Survival of multiple course patients (86%) was comparable to that of single course patients (93%) and improved compared to non-treated historical controls. Multiple course recipients demonstrated an increased need for open fetal surgery and postnatal surgery at a younger age. CONCLUSION: Fetuses who fail to respond to a single course of BMZ may benefit, as indicated by hydrops resolution and improved survival, from additional courses. However, failure to respond is indicative of a lesion which may require fetal or immediate neonatal resection.

Favorable outcomes in high-risk congenital pulmonary airway malformations treated with multiple courses of maternal betamethasone.

BACKGROUND/PURPOSE: Congenital pulmonary airway malformations (CPAMs) are rare congenital lung lesions often diagnosed by prenatal ultrasound. High-risk cases can result in hydrops and prenatal or postnatal demise. Antenatal betamethasone has resulted in improved survival but it is unclear how to manage patients who do not respond to a single course. METHODS: We present a bi-institutional retrospective review of patients treated with multiple courses of prenatal steroids for high-risk CPAMs between 2007 and 2013. RESULTS: Nine patients met inclusion criteria. All but one either had an increased CPAM volume ratio (CVR) or number of fluid-containing compartments involved after a single course of antenatal betamethasone, prompting additional courses. Four patients stabilized, three improved and two progressed after the second course. The two cases with disease progression underwent an in utero resection. There were one in utero fetal demise and two deaths within the delivery room. Both fetuses that underwent a fetal resection died. All but one mother who delivered a viable fetus had complications of pregnancy. CONCLUSIONS: Multiple courses of antenatal betamethasone for high-risk fetal CPAMs often result in favorable short-term outcomes without the need for open fetal resection. Pregnancy complications are common and women within this cohort should be monitored closely.

In utero resolution of microcystic congenital cystic adenomatoid malformation after prenatal betamethasone therapy: A report of three cases and a literature review.

Fetal congenital cystic adenomatoid malformation (CCAM) can progress to nonimmune hydrops, and the mortality rate of CCAM with hydrops is reported to be nearly 100%. We describe three microcystic CCAM cases in which the fetal condition improved after maternal betamethasone therapy. The median gestational age at steroid administration was 23 5/7 weeks' gestation. The CCAM decreased in size in all cases. Our series showed a 100% hydrops resolution rate (2/2) and a 100% survival rate (3/3). Our experience suggests the efficacy of betamethasone treatment on fetuses with microcystic CCAM who have fluid collection or are at risk of developing hydrops.

Prenatal steroids for microcystic congenital cystic adenomatoid malformations.

OBJECTIVE: The purpose of this study is to evaluate the effect of prenatal steroid treatment in fetuses with sonographically diagnosed congenital cystic adenomatoid malformations (CCAMs). METHODS: This was an institutional review board-approved retrospective review of 372 patients referred to the University of California, San Francisco (UCSF), for fetal CCAM. Inclusion criteria were (1) a predominately microcystic CCAM lesion sonographically diagnosed at our institution, (2) maternal administration of a single course of prenatal corticosteroids (betamethasone), and (3) no fetal surgery. CCAM volume-to-head ratio (CVR), presence of hydrops, mediastinal shift, and diaphragm eversion were assessed before and after administration of betamethasone. The primary end points were survival to birth and neonatal discharge. RESULTS: Sixteen patients with predominantly microcystic CCAMs were treated with prenatal steroids. Three were excluded because of lack of follow-up information. All remaining fetuses (13/13) survived to delivery and 11/13 (84.6%) survived to neonatal discharge. At the time of steroid administration, all patients had CVR greater than 1.6, and 9 (69.2%) also had nonimmune hydrops fetalis. After a course of steroids, CVR decreased in 8 (61.5%) of the 13 patients, and hydrops resolved in 7 (77.8%) of the 9 patients with hydrops. The 2 patients whose hydrops did not resolve with steroid treatment did not survive to discharge. CONCLUSION: In high-risk fetal patients with predominantly microcystic CCAM lesions, betamethasone is an effective treatment. This series is a pilot study for a prospective randomized trial comparing treatment of CCAM with betamethasone to placebo.

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.

Effect of maternal betamethasone administration on prenatal congenital cystic adenomatoid malformation growth and fetal survival.

OBJECTIVE: To evaluate the effect of prenatal steroid treatment on the growth of congenital cystic adenomatoid malformations (CCAM) and survival in affected fetuses not amenable to other percutaneous ultrasound-guided prenatal interventions. METHODS: A retrospective review of patients with a CCAM or hybrid lesion treated with two maternal prenatal betamethasone injections was performed. Patients receiving cyst aspiration or thoracoamniotic shunting at the time of or after steroid administration were excluded. Growth rates and survival data were compared to historical non-steroid treated controls. RESULTS: Eleven patients were treated with prenatal steroids (10 microcystic and 1 macrocystic). Survival was 100% in fetuses with hydrops (5/5) or a CCAM volume ratio (CVR) >1.6 (7/7) at the time of steroid administration. This compares to a mortality of 100 and 56.2% respectively in historical non-treated controls. Resolution of hydrops was seen in 80% (4/5) of steroid-treated patients. CCAM growth rates were variable after steroid administration. However, when compared to historical data where CVR and CCAM volume have been documented to increase until 28 weeks' gestation, the CVR and CCAM volume growth rates decreased in 72.73% and 50% of patients respectively from the time of steroid administration to 28 weeks' gestation. CONCLUSIONS: In the fetus with a CCAM, the presence of hydrops fetalis or a CVR >1.6 is indicative of poor fetal outcome without prenatal intervention. The observed effect of antenatal steroid treatment on CCAM growth is variable, but its potential to improve survival in these high-risk groups is encouraging and warrants further controlled evaluations.

Malformación quística adenomatoidea pulmonar: a propósito de un caso. Año 2006 / Lung adenomatoid cystic malformation: a propos of a case, year 2006

Se presenta el caso de un lactante, parto fuera de la maternidad, ingresado en el Servicio de Pediatría por tos, discreta dificultad respiratoria, fiebre y disminución del peso para su edad, que luego del interrogatorio a la madre sobre sus síntomas a partir del nacimiento y las radiografías simples y contrastadas se llega a la conclusión de tratarse de una anomalía congénita del pulmón llamada malformación quística adenomatoidea, entidad muy rara caracterizada por quistes pulmonares únicos o múltiples limitándose a un lóbulo del pulmón y que se presentan en niños desde el período neonatal, se describe el cuadro clínico y radiológico del paciente.(AU)

We present a case report of a breast-fed whild which delivery was out of a lying in hospital admitted to Pediatric service suffering from cough, a discrete respiratory distress, fever, and decreased weight for its age. After the mother´s interrogatpry about its symptoms from delivery, and simple and contrasted X rays, it is concluded it is a lung congenital anomaly colled adenomatoid cystic malformation, a very rare entity characterized by unique on multiple lung cysts, limited to a lung lobule, which is presented in children from neonatal period on. Clinical and radiologic findings of the patient are described.(EU)

Unexpected intrauterine death following resolution of hydrops fetalis after betamethasone treatment in a fetus with a large cystic adenomatoid malformation of the lung.

Without intervention, the development of hydrops fetalis secondary to cystic adenomatoid malformation of the lung (CAML) implies a 100% mortality rate. Conversely, four CAML cases with in-utero resolution of hydrops fetalis after prenatal betamethasone therapy with good clinical outcome have been reported in the literature. The mechanism is speculated to be the effect of corticosteroid in improving lung maturation. Here we present another CAML case with resolution of hydrops fetalis after prenatal betamethasone therapy but which was followed by later intrauterine death. We speculate that the resolution of the CAML lesion itself may be more important in determining the clinical outcome than the resolution of hydrops. The association between prenatal betamethasone therapy and resolution of hydrops may be related to a different mechanism other than improvement of lung maturation.

Resolution of hydrops fetalis in congenital cystic adenomatoid malformation after prenatal steroid therapy.

BACKGROUND/PURPOSE: Development of hydrops fetalis in fetuses with congenital cystic adenomatoid malformations (CCAM) is a significant risk factor for fetal or neonatal demise. In rare cases, resolution of CCAM has occurred, presumably owing to lesion maturation or involution. Steroid therapy, utilized for lung maturity, has been postulated to accelerate this process. The natural history of hydropic fetuses with CCAM after receiving steroid therapy is presented. METHODS: The authors prospectively followed up with 3 patients who had antenatally diagnosed CCAM and nonimmune hydrops fetalis with predicted mortality. All patients declined or were not candidates for fetal intervention and were treated with standard prenatal betamethasone to increase lung maturity. RESULTS: Three fetuses had CCAM and nonimmune hydrops fetalis diagnosed prenatally. After a course of prenatal steroids during the second trimester, all 3 patients had resolution of their hydrops and were delivered at term without respiratory distress. CONCLUSIONS: Nonimmune hydrops fetalis in fetuses with congenital cystic adenomatoid malformation is a harbinger for fetal demise. The resolution of hydrops in these patients after receiving steroid therapy is an interesting and compelling observation. Because the mechanism of this process is speculative, further studies are needed to elucidate the relationship between antenatal steroids and maturation of congenital cystic adenomatoid malformation.

[Use of nitric oxide in a newborn child with pulmonary cystic adenomatoid malformation]. / Utilización del óxido nítrico en un neonato con una malformación adenomatoidea quística pulmonar.

Nitric oxide (NO) inhalation therapy relaxes preconstricted pulmonary blood vessels without causing concomitant systemic hypotension and increases oxygen uptake into the blood. NO inhalation is a new treatment for various disorders of neonates (respiratory distress syndrome, persistent pulmonary hypertension, congenital heart disease and congenital diaphragmatic hernia). There is no references of its use in congenital cystic adenomatoid malformation (CCAM). We report a case of a newborn of 33 week's gestation. The infant underwent resection of the CCAM that had occupied the right middle lobe. At the end of the operation, arterial blood gases at a fractional inspired oxygen concentration (FiO2) of 1.0 reveled acidosis and severe hypoxemia probably due to persistent pulmonary hypertension. NO therapy was used for 16 hours with decreased oxygen need and increase of arterial blood oxygen data. According to the extent of the adenomatoid lesion, likely due to the compression of the surrounding tissue, these patients at times postoperatively develop difficulty in oxygenation and ventilation and the changes are similar to those patients with congenital diaphragmatic hernia. The use of NO in these disorders are successfull.

Congenital cystic adenomatoid malformation of the lung and fetal hydrops--a case with favourable outcome.

We present a case of congenital cystic adenomatoid malformation of the lung (CCAM) diagnosed at 23 weeks of gestation with concomitant fetal hydrops. The sonographical picture of CCAM disappeared in the third trimester of pregnancy and fetal hydrops resolved under medication with digitalis to the mother. The neonate showed mild dyspnea; the prenatal diagnosis of CCAM was confirmed by chest X-ray and computed tomography. The affected lung segments were dissected at 5 days of age. The diagnosis of CCAM type III was confirmed histologically.