Survival after CCAM associated with ascites: a report of a case and review of the literature

A congenital cystic adenomatoid malformation (CCAM) is a congenital lung lesion that, when associated with hydrops, is thought to have a dismal prognosis without fetal intervention. The authors report a case of a fetus with a CCAM associated with ascites as the only manifestation of hydrops, which was diagnosed at 19 weeks gestation. The CCAM regressed without intervention between 23 and 29 weeks of gestation with resolution of the ascites. The baby was born asymptomatic at term, and the infant underwent elective resection of the CCAM at 1 year of age. A review of the literature from 1980 to 2000 yielded 9 other cases of spontaneous resolution of hydrops associated with CCAM, suggesting that although rare, this outcome is a possibility in fetuses diagnosed with the condition. Because most fetuses diagnosed with hydrops undergo termination of pregnancy, the true incidence of hydrops resolution is potentially higher than predicted. This has important implications for prenatal counseling particularly in centers in which fetal intervention is not available.     

Fetal intervention for mass lesions and hydrops improves outcome: a 15-year experience

Purpose

The natural history of certain prenatally diagnosed masses is well known. Large thoracic mass lesions can evolve one of 2 ways, either to regress and cause minimal morbidity, or to progress and enlarge, often resulting in hydropic changes in the fetus. This nonimmune hydrops carries a dismal prognosis, with nearly all fetuses expiring before or shortly after birth. However, hydrops associated with fetal mass lesions can be halted and even reversed with fetal intervention and treatment of the underlying defect. We examined our patients with fetal mass lesions to evaluate survival after intervention.

Methods

Institutional approval was obtained by the Committee on Human Research. A retrospective review was performed of 294 fetuses evaluated over 15 years with large mass lesions. All patients were evaluated for evidence of fetal hydrops using ultrasound criteria. Patients were divided according to type of intervention. Primary outcome measure was 30-day survival after birth.

Results

(1)

Patients without fetal hydrops did not undergo fetal intervention and survived to 30 days after birth (167/172, 97%).

(2)

Patients with fetal mass lesions that developed hydrops fared poorly with no intervention (1/33 survival, 3%), whereas fetuses undergoing prenatal intervention fared much better (15/30 open, 50%; 3/10 percutaneous, 30%).

(3)

Four patients with hydropic congenital cystic adenomatoid malformation (n = 3) or pulmonary sequestration (n = 1) received steroids in preparation for surgery but underwent no intervention, and the patients survived the neonatal period.

Conclusion

Fetuses with prenatal diagnoses of masses not associated with hydrops have excellent prognosis with survival higher than 95%. Nonimmune hydrops associated with prenatal diagnosis of a fetal mass is a devastating complication with less than 5% survival. Open resection of a mass causing hydrops resulted in 50% survival, with reversal of hydrops in a group with near-uniform fatality. Further investigation is warranted regarding the use of minimally invasive prenatal therapies including steroid administration for hydropic fetuses.     

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.     

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. J Pediatr Surg 38:508-510.     

Microcystic congenital pulmonary airway malformation with hydrops fetalis: steroids vs open fetal resection

Background/PurposeCongenital pulmonary airway malformations (CPAM) are rare lesions often diagnosed during routine prenatal ultrasound. The presence of hydrops fetalis is an indicator of poor prognosis. Here we present a retrospective review of fetuses undergoing either open fetal surgery or steroids for predominantly microcystic CPAM with hydrops fetalis.MethodA retrospective review of patients undergoing open fetal surgery or steroids for CPAM at our institution was performed. The primary outcome was survival.ResultsA retrospective review of all patients referred to our institution with the diagnosis of CPAM was performed. Fetuses with predominantly microcystic CPAM and the presence of hydrops fetalis treated with steroid or surgery were included. Thirteen patients were treated with steroids, and 11 patients underwent open fetal surgery. In the steroid group 12 (92%) of 13 fetuses survived to delivery versus 9 (82%) of 11 in the open fetal surgery group. Only 5 (56%) of 9 of the patients in the open fetal surgery group survived to neonatal discharge compared to 10 (83%) of 12 in the steroid group.ConclusionsIn the present retrospective study, improved survival was seen in fetuses with hydrops fetalis and predominantly microcystic CPAM treated with steroids when compared with open fetal surgery. Steroids should be considered for first-line therapy in these cases.     

Importance of fetal fluid imbalance in congenital cystic adenomatoid malformation of the lung.

Congenital cystic adenomatoid malformation (CCAM) is a regional overgrowth of bronchioles with suppression of alveolar development in fetal and newborn lung. Twelve patients with CCAM were treated. Six premature infants had acute respiratory distress. Prenatal ultrasound was performed in only five patients and was abnormal in all five. Five premature neonates also had patent ductus arteriosus complicating their courses. Six older children presented with recurrent pneumonitis. Radiographs showed asymmetry of the chest and radiolucent masses in all 12 patients. Seven had type I lesions, two had type II lesions, and three had type III lesions. Hydramnios and hydrops were present in three, and hydrops alone was present in one of the six neonates. These four patients died. One other neonate died of respiratory failure and persistent fetal circulation. Seven patients survived for an extended period. Ultrasound makes the prenatal diagnosis of hydramnios and hydrops possible. It is in this group that fetal interventions can be considered. For the remaining patients, surgical intervention is indicated at the time of diagnosis.     

Primary fetal hydrothorax: natural history and management

Primary fetal hydrothorax presents a wide spectrum of severity ranging from small, harmless effusions, to life-threatening thoracic compression. To define natural history and management, we reviewed 32 cases seen at two large perinatal centers from 1980 to 1987. Spontaneous resolution of the effusions was seen in three fetuses, all of whom survived. Three fetuses were electively terminated. The overall mortality was 53%. In the 24 untreated fetuses, sex and the presence of polyhydramnios did not influence mortality, but hydrops, gestational age less than 35 weeks at delivery, and bilateral effusions were associated with a poor prognosis. Five fetuses underwent in utero decompression. In four, thoracentesis was performed, with rapid reaccumulation of the effusion. All four died from pulmonary insufficiency. In the fifth fetus, a thoracoamniotic shunt permanently decompressed the effusion, with resolution of the hydrops, and delivery of a normal viable infant. We conclude that (1) primary fetal hydrothorax may resolve or progress to hydrops, necessitating close follow-up with ultrasound; (2) pulmonary hypoplasia as a result of undrained large pleural effusions may result in neonatal mortality; (3) the gestational age at both diagnosis and delivery, the development of hydrops, and bilaterality of effusions are important prognostic predictors; and (4) the fetus with large effusions and hydrops has a poor prognosis, and thoracic decompression with a thoracoamniotic shunt may prove life saving.     

Large fetal congenital cystic adenomatoid malformations: growth trends and patient survival

Purpose

The prognosis for fetuses with large congenital cystic adenomatoid malformations (CCAMs) remains uncertain. This study examined the natural history of large fetal CCAMs managed expectantly at a major referral center.

Methods

A 5-year retrospective review was conducted on fetuses diagnosed with a thoracic lesion (n = 59). Large CCAMs were identified on prenatal imaging and followed longitudinally. Perinatal outcomes were assessed.

Results

Twelve (20.3%) fetuses had large CCAMs in the absence of other congenital anomalies. Peak CCAM size occurred at 25.3 ± 3.6 weeks' gestation. Serial magnetic resonance volumetry demonstrated a trend toward decreasing CCAM mass volume relative to thoracic cavity volume over time. Overall, 6 patients, including 3 with signs of early hydrops, showed a marked regression of their lesions relative to estimated fetal weight. Five fetuses required an emergent intervention postnatally, including extracorporeal membrane oxygenation support (n = 1), cyst aspiration (n = 1), and lung resection (n = 5). Overall survival was 75%, with severe hydrops before 30 weeks seen in all 3 deaths.

Conclusion

Large fetal CCAMs tend to peak in size at 25 weeks' gestation and are characterized by in utero diminution relative to overall fetal growth. The prognosis for most fetuses with large CCAMs remains quite favorable under careful perinatal management.     

Management of the fetus with a cystic adenomatoid malformation

Prenatal detection and serial sonographic study of fetuses with congenital cystic adenomatoid malformation (CCAM) now makes it possible to define the natural history of this lesion, determine the pathophysiologic features that affect clinical outcome, and formulate management based on prognosis. In a series of 22 prenatally diagnosed cases, the overall prognosis depended on the effects of the lung mass and the consequent physiologic derangement: A large mass may cause mediastinal shift, hypoplasia of normal lung tissue, polyhydramnios, and cardiovascular compromise leading to fetal hydrops. Two types of CCAM were distinguished by gross anatomy, ultrasonographic findings, and prognosis. Microcystic lesions (cysts < 5 mm; solid appearance) are often associated with fetal hydrops and have a poor prognosis. Macrocystic lesions (single or multiple cysts > 5 mm) are not usually associated with hydrops and have a more favorable prognosis. Four initially large lesions partially disappeared on serial prenatal sonography, suggesting that improvement can occasionally occur during fetal life. The finding that fetuses with hydrops are at high risk for fetal or neonatal demise led to fetal surgical resection of the massively enlarged pulmonary lobe (fetal lobectomy) in two cases. Fetuses with CCAM but without hydrops have a good chance for survival with maternal transport, planned delivery, and immediate neonatal resuscitation and surgery.

---

Resumen La detección prenatal y el estudio sonográfico seriado de fetos con malformación adenomatosa quística congénita (MAQC) actualmente permite definir la historia natural de esta lesión, determinar las características patofisiológicas que afectan el resultado clínico y formular un manejo basado en pronóstico. En una serie de 22 casos diagnosticados en el período prenatal, el pronóstico global dependió de los efectos de las masas pulmonares y la consecuente alteración fisiológica: una gran masa puede producir desviación mediastinal, hipoplasia del tejido pulmonar normal, polihidramnios y compromiso cardiovascular resultante en hidrops fetal. Los dos tipos de MAQC pudieron ser distinguidos por la anatomía macroscópica, los hallazgos ultrasonográficos y el pronóstico. Las lesiones microquísticas (quiste <5 mm; apariencia sólida) generalmente están asociadas con hidrops fetal y tienen un pronóstico pobre. Las lesiones macroquísticas (quistes únicos/múltiples >5 mm) usualmente no están asociados con hidrops y exhibieron un pronóstico más favorable. Cuatro lesiones inicialmente de gran tamaño desaparecieron subsiguientemente bajo observación con sonografía seriada prenatal, lo cual sugiere que ocasionalmente se presenta mejoría en el curso del desarrollo fetal. El hallazgo de que los fetos con hidrops conllevan un alto riesgo de muerte fetal o neonatal ha llevado a la resección quirúrgica prenatal del lóbulo pulmonar de volumen masivo (lobectomía fetal) en dos casos. Los fetos con MAQC pero libres de hídrops tienen una buena probabilidad de sobrevivir mediante el transporte materno, un parto programado y resucitación y cirugía neonatales inmediatas.

---

Résumé L'étude échographique répétée des foetus ayant la maladie kystique (adénomateuse) congénitale du poumon (MKACP) et la détection prénatale ont contribué à augmenter nos connaissances de l'histoire naturelle de la lésion, ont aidé à préciser les données physiopathologiques qui peuvent influencer l'évolution clinique et déterminer une démarche thérapeutique basée sur le pronostic. Dans cette série de 22 cas, diagnostiqués avant la naissance, le pronostic était basé sur l'effet de masse pulmonaire et les conséquences physiologiques: une masse importante peut en effet être responsable d'une déviation du médiastin, de l'hypoplasie des tissus pulmonaires normaux, d'une hydramnios, et d'une décompensation cardiovasculaire dont le résultat global est une anasarque foetale. Selon les données anatomiques macroscopiques, échographiques et pronostiques, on peut distinguer deux types de MKACP. Les kystes microscopiques (kystes < 5 mm, allure solide) sont souvent associés à une anarsaque foetale et un mauvais pronostic. Les lésions macroscopiques (kystes solitaires ou multiples > 5 cm) ne sont pas habituellement associés à une anasarque et le pronostic est généralement plus favorable. Dans cette série, quatre lésions larges initialement, ont diminué de taille au cours de la surveillance échographique, suggérant qu'une amélioration spontanée est toujours possible pendant la vie foetale. En raison du mauvais pronostic associé à une anasarque foetale avec mort foetale ou néonatale, on a été amené à pratiquer une résection chirurgicale élargie des lobes pulmonaires dans deux cas. Les foetus avec MKACP mais sans anasarque ont de bonnes chances de survie à condition d'entourer la maternité, la naissance et les premiers jours de l'enfant avec des soins chirurgicaux et de réanimation adaptés.

     

Outcomes of fetal intervention for primary hydrothorax

Objective

Primary hydrothorax is a rare congenital anomaly with outcomes ranging from spontaneous resolution to fetal demise. We reviewed our experience with fetuses diagnosed with primary hydrothorax to evaluate prenatal management strategies.

Methods

We reviewed the records of patients evaluated for fetal pleural effusions at our Fetal Treatment Center between 1996 and 2013. To define fetuses with primary hydrothorax, we excluded those with structural or genetic anomalies, diffuse lymphangiectasia, immune hydrops, and monochorionic diamniotic twin gestations.

Results

We identified 31 fetuses with primary hydrothorax, of whom 24 had hydrops. Hydropic fetuses were more likely to present with bilateral effusions. Of all fetuses with primary hydrothorax, 21 had fetal interventions. Survival without hydrops was 7/7 (100%), whereas survival with hydrops depended on whether or not the patient had fetal intervention: 12/19 (63%) with intervention and 1/5 (20%) without intervention. Premature delivery was common (44%) among those who had fetal intervention.

Conclusions

Fetal intervention for primary hydrothorax may lead to resolution of hydrops, but preterm birth and neonatal demise still occur. Understanding the pathophysiology of hydrops may provide insights into further prenatal management strategies, including targeted therapies to prevent preterm labor.     

Residual congenital cystic adenomatoid malformation and thoracic scar deformation after fetal surgery: a case report

Fetal surgery is a treatment option for fetuses with congenital cystic adenomatoid malformation (CCAM) of the lung who develop hydrops before 32 weeks of gestation. We report on a fetus with CCAM and hydrops who underwent subtotal resection of a huge right, lower lobe CCAM at 20 weeks of gestation. Postnatally, the infant developed a thoracic scar deformation and was suspected to have residual CCAM. The residual CCAM was resected and the chest wall deformity was corrected at 3 1/2 years of age.     

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.     

Defining hydrops and indications for open fetal surgery for fetuses with lung masses and vascular tumors

PurposeThe aim of this study was to identify the most accurate prenatal predictors of outcomes and need for fetal surgery for fetuses with high-risk lung masses and vascular tumors.MethodsThe records of all fetuses with high-risk lung mass (congenital cystic adenomatoid malformation-volume ratio > 1.6 or findings of hydrops) and vascular tumor evaluated between July 2001 and March 2011 were reviewed retrospectively. Hydrops was defined as accumulation of fluid in 2 or more compartments.ResultsOf fetuses with high-risk lung mass, hydrops was identified in 46% (11/24). Fetuses with hydrops and an abnormal echocardiogram (n = 8) demonstrated poor survival without fetal surgery (13%) compared with 100% survival in fetuses with hydrops and a normal echocardiogram (n = 3; P = .02). Of 21 fetuses with vascular tumor (11 sacrococcygeal and 8 cervical teratomas; 2 hemangioendotheliomas), hydrops was identified in 29% and an abnormal echocardiogram in 57%. All fetuses with hydrops had an abnormal echocardiogram and either died (n = 5) or required fetal surgery (n = 1). However, all fetuses with abnormal echocardiograms alone (n = 7) survived without fetal intervention.ConclusionsFor fetuses with lung mass, an abnormal echocardiogram in the setting of hydrops is the best predictor of mortality and need for fetal surgery. For fetuses with vascular tumor, hydrops in the setting of high-output physiology best predicts demise and need for fetal surgery.     

Predicting the severity of congenital high airway obstruction syndrome

Background

Congenital high airway obstruction syndrome (CHAOS) is caused by complete or near-complete obstruction of the fetal airway. Obstruction sets in motion a sequence of events that can ultimately lead to fetal demise. However, on rare occasions in utero airway decompression occurs, reversing syndromic findings and improving the prognosis. In our relatively large series of CHAOS patients, we have observed a spectrum of clinical severity. The aim of this study was to identify the prenatal characteristics of CHAOS predictive of a milder postnatal course.

Methods

The medical charts of all fetuses observed at our institution with the diagnosis of CHAOS were reviewed for radiologic findings, delivery information, perinatal course, autopsy or discharge report, and long-term follow-up.

Results

Between 1996 and 2008, 12 fetuses with CHAOS were identified. Four fetuses had no evidence of hydrops on initial imaging. Of the 8 fetuses displaying hydrops, 3 were terminated, 2 died in utero, and 1 with multiple anomalies died at birth. Six fetuses were delivered via the ex utero intrapartum therapy procedure for attempted salvage, and 5 of the 6 survived the neonatal period including all 4 fetuses without hydrops. Serial prenatal imaging demonstrated less severe signs of CHAOS in 3 fetuses, and in 2 of them, direct laryngoscopy revealed a tiny opening in the airway. All 3 fetuses that showed improvement on serial imaging survived the neonatal period and were discharged home by 2-10 weeks of age.

Conclusions

Although the natural history of CHAOS is variable, trends in prenatal ultrasound findings are highly predictive of postnatal outcome and are a valuable guide to prenatal counseling.     

Congenital diaphragmatic hernia and hydrops: a lethal association?

Background/Purpose: Nonimmune hydrops in the fetus is a finding that often portends death. The association and prognosis of fetuses with congenital diaphragmatic hernia (CDH) and hydrops is not known. Methods: A retrospective review of all prenatally diagnosed cases and referrals of CDH was performed. Variables analyzed included gestational age at diagnosis and delivery, side of hernia, presence of associated anomalies and hydrops, and neonatal outcome. Results: Since 1993, 474 prenatal referrals for CDH have been made. One hundred seventy-five were evaluated; 15 fetuses had hydrops (9%). Five patients had CDH, hydrops, and associated lethal anomalies. In the remaining 10 patients, 6 of the diaphragmatic defects were right-sided and 4 were left-sided. All except one had a major portion of the liver herniated into the chest. Six fetuses had prenatal intervention. Five neonates died shortly after birth. There were 5 long-term survivors; all received prenatal intervention. Conclusions: The association of CDH and hydrops is rare but often results in fatality. Hydrops appears to be associated with liver in the hernia, right-sided lesions, and lethal anomalies. Fetal intervention can be performed successfully in patients with CDH and hydrops, and may improve long-term survival rate in this group.     

Survival of a profoundly hydropic fetus with a sacrococcygeal teratoma delivered at 27 weeks of gestation for maternal mirror syndrome

The development of hydrops fetalis and maternal mirror syndrome in the setting of fetal sacrococcygeal teratoma (SCT) carries a grave prognosis. Fetal surgery is not typically offered for hydropic fetuses beyond 26 weeks of gestational age. Few options exist for these families, and they are typically counseled to continue pregnancy until delivery is necessary for maternal indications or the fetus is 30 weeks old. Although a number of series report the survival of hydropic SCT infants delivered after 30 weeks of gestation, a paucity of information is available regarding the outcome of hydropic SCT infants delivered between 26 and 30 weeks of gestation. We now report successful postpartum resection and survival of a hydropic fetus delivered at 27 weeks for maternal mirror syndrome that may assist in counseling families in similar situations.     

Cystic adenomatoid malformation volume ratio predicts outcome in prenatally diagnosed cystic adenomatoid malformation of the lung

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.     

Current outcome of antenally diagnosed cystic lung disease

Background/purpose

The natural history of parenchymal lung lesions such as congenital cystic adenomatoid malformation (CCAM) and pulmonary sequestration (PS) has been altered by the advent of antenatal ultrasonography. Initial reports were characterized by a high (about 30%) incidence of adverse features (eg, hydrops) and a poor outcome and did not accord with our recent experience. The authors have reviewed the outcome of fetuses that had been diagnosed in a large tertiary referral fetal medicine unit with the aim of delineating current experience. The term cystic lung disease was used throughout to avoid unjustifiable histologic precision.

Methods

The scans of all fetuses that had been diagnosed with cystic lung disease between January 1995 and July 2001 were reviewed. The outcome of each pregnancy was established, and, where possible, all infants underwent appropriate investigations, including thoracic computed tomography (CT) scans.

Results

Sixty-seven fetuses had a cystic lung abnormality diagnosed from January 1995 to July 2001. The median (range) age at diagnosis was 21 (19 to 28) weeks. The lesion was right sided in 29 (43%), left in 36 (54%), and bilateral in 2 (3%); it was characterized as dominantly macrocystic in 27 (40%), microcystic in 35 (52%), and mixed in 5 (8%). Mediastinal shift was present in 30 fetuses (45%). Severe signs of fetal distress (eg, hydrops) were present in 5 fetuses (7%). Antenatal intervention was performed in 4 fetuses (thoraco-amniotic shunts [n = 3] and percutaneous intrauterine laser therapy [n = 1]). Sixty-four (96%) of the fetuses were born alive. There was 1 termination of pregnancy and 2 intrauterine deaths (all severe microcystic lesions). Forty-two infants (63%) underwent thoracotomy and appropriate excisional surgery at a median of 7.5 months (range, 1 day to 34 months). Two infants (which included the fetus having intrauterine laser therapy) died early in the postnatal period. Both were large microcystic lesions and had antenatal features of severe fetal distress. Twelve infants were investigated in the postnatal period but did not undergo surgery. Ten infants were not appropriately investigated or were lost to follow-up. Histologic examination showed definitive diagnostic features of CCAM (n = 25) or PS (n = 6). Other lesions with hybrid features of both were also seen (n = 11). There was a degree of correlation between antenatal ultrasound features (size of cyst [P = .03], in-utero behavior [P = .06], mediastinal shift [P = 0.05]) and the need for surgery but not with the final histologic diagnosis. Surgical excision was required in 45% of lesions showing late-gestation “resolution.”

Conclusions

Antenatally diagnosed “cystic lung disease” has an excellent prognosis in the absence of signs of severe fetal distress. The need for surgery should be based on appropriate postnatal investigations (eg, CT scans), rather than on antenatal behavior.     

Prenatal Therapy for Thoracic and Mediastinal Lesions

Most prenatally diagnosed lung lesions can be managed successfully during the neonatal period. Prenatal imaging and experimental models have provided a comprehensive understanding of the pathophysiology, natural history, and prognosis of intrathoracic and mediastinal lesions. Clinical experience has demonstrated that progression to nonimmune hydrops fetalis and pulmonary hypoplasia is a harbinger of fetal or neonatal demise. Advances in fetal anesthesia, tocolysis, and surgical techniques have made fetal surgery a viable in utero option to ameliorate life-threatening masses. Congenital cystic adenomatoid malformation, bronchopulmonary sequestration, and congenital hydrothorax are the most common abnormalities amenable to surgical intervention. The natural history, evaluation, and treatment of intrathoracic and mediastinal lesions are discussed.     

Long-term outcome in patients with prenatally diagnosed cystic lung disease: special reference to ventilation and perfusion scan in the affected lung

Background/Purpose

The natural history of cystic lung disease (CLD) such as congenital cystic adenomatoid malformation (CCAM) and pulmonary sequestration has been altered by the advent of prenatal diagnosis. Although recent advances including fetal therapy have gradually improved outcome, the long-term course and the function of the residual lung have not been well clarified.

Methods

Twenty-two patients with CLD who had been prenatally diagnosed and treated between 1990 and 2004 were reviewed. The clinical outcome and growth measurements were established, and, where possible, all infants underwent ventilation and perfusion lung scan.

Results

Mediastinal shift was present in 14 fetuses. Fetal hydrops was present in 5 fetuses. Antenatal intervention was performed for hydrops in 2 fetuses (cyst-amniotic shunt and aspiration). Twenty-one infants underwent appropriate excisional surgery. Final diagnosis included CCAM (n = 12) and pulmonary sequestration (n = 7). No late death was observed. Common complications were failure to thrive (n = 5), frequent respiratory tract infection (n = 4), and asthmatic attack (n = 4). A significant decrease in lung ventilation and perfusion on the affected side was observed in patients with hydrops, lobectomy, and CCAM.

Conclusion

Long-term follow-up including respiratory care and growth assessment should be performed in prenatally diagnosed patients with CLD, especially those who present with hydrops.