Thoracoamniotic Shunting Research Articles

Fetal thoraco-amniotic shunting for large macrocystic congenital cystic adenomatoid malformations (CCAM) of the lung

Fetal thoraco-amniotic shunting for large macrocystic congenital cystic adenomatoid malformations (CCAM) of the lung

Expanding the differential diagnosis of fetal hydrops: an unusual prenatal presentation of megalencephaly‐capillary malformation syndrome

What's already known about this topic? Megalencephaly‐capillary malformation (MCAP) syndrome is a rare disorder characterized by growth dysregulation, central nervous system and vascular anomalies, and distal limb malformations. Disruption of PI3K‐AKT signaling has recently been implicated as the cause of MCAP.What does this study add? This study reports a case of MCAP with bilateral pleural effusions and fetal hydrops managed with thoracoamniotic shunt placement, highlights characteristic features of MCAP that may facilitate prenatal diagnosis, and discusses the possibility of prenatal molecular diagnosis in future cases.

Malformations adénomatoïdes kystiques du poumon : diagnostic, prise en charge, hypothèses physiopathologiques

Congenital cystic adenomatoid malformations (CCAM) of the lung are the most frequent congenital lung malformations. Their diagnosis is based on histological features. CCAM consist of bronchopulmonary cystic lesions which are classified according to the presence and cysts size. Type I CCAM are composed of large cysts (>2 cm) lined by a columnar pseudostratified epithelium. Type II CCAM contain multiple small cystic lesions (<1 cm) lined by a flattened cuboidal epithelium. Type III CCAM are more solid and contain immature structures resembling the pseudoglandular stage of lung development. Ultrasonography (US) allows early detection during the second trimester of pregnancy as cystic, and/or hyperechoic fetal lung lesions. Although most CCAM remain asymptomatic, CCAM can cause polyhydramnios or fetal hydrops, respiratory distress at birth, infections and pneumothoraces during infancy, and may give rise to malignancies. Serial US allow detection of complications, and planification of delivery. Complicated forms require an urgent treatment. In fetuses with a macrocystic life-threatening lesion, a thoraco-amniotic shunt can be placed. Microcystic compressive forms may respond to prenatal steroids. Post-natal symptomatic lesions require early surgery. The treatment of asymptomatic forms remains controversial. Some recommend a non-operative approach with a long-term clinical and radiological following, whereas other favour a preventive surgical excision. The origin of CCAM remains unknown. Recent advances suggest a transient and focal abnormality in lung development which may result from an airway obstruction. This article reviews the diagnosis, treatment, and pathophysiology of CCAM.

Diagnosis and Prevention of Abnormal Antenatal Lung Growth

Abnormal antenatal lung growth can result in a high mortality and morbidity, thus accurate diagnosis and effective preventative measures are essential. We have critically reviewed the literature to identify whether current diagnostic tools yield results predictive of outcome and whether antenatal interventions do prevent abnormal lung growth. Three-dimensional (3D) ultrasound examination provides more accurate information than 2D ultrasound on fetal lung volume prior to 34 weeks of gestation, but can be inaccurate if there is oligohydramnios. In addition, fetal lung volumes obtained by 3D ultrasound analysed by Virtual Organ Computer Aided analysis (VOCAL) can predict neonatal respiratory outcomes. Fetal lung volume assessments by magnetic resonance imaging (MRI) correlate with lung to head ratio results obtained by 2D ultrasound results, with an observed/expected ratio of 0.25 predicting poor survival. MRI using quantitative signal analysis gives a functional assessment of the lung parenchyma. MRI, however, is expensive and has limited patient acceptability. Antenatal interventions attempting to prevent pulmonary hypoplasia include amnioinfusion which can relieve oligohydramnios in preterm, premature rupture of the membranes, but there are insufficient data on longer term outcomes to recommend it for routine practice. In severe idiopathic oligohydramnios, amnio-infusion might prolong the duration of the pregnancy, but there is no evidence to date that it improves neonatal mortality. Thoracoamniotic shunting results in effective drainage of pleural effusions facilitating resuscitation, but it is usually performed too late in pregnancy to influence lung growth and there are complications. Pleural effusions can also be managed by pleurodesis using either OK-432 or autologous blood. In fetuses with a congenital diaphragmatic hernia (CDH) obstruction of the normal egress of fetal lung fluid by placing a balloon in the trachea (fetal tracheal obstruction (FETO)) in non randomized studies, appear to improve survival. The results of randomised trials of FETO with long term outcomes are eagerly awaited.

Thoracoscopy-assisted removal of a thoracoamniotic shunt double-basket catheter dislodged into the fetal thoracic cavity: report of three cases

The indications for and timing of surgical removal of a dislodged thoracoamniotic shunt double-basket catheter are not established, and the side effects of the dislodged into the thoracic cavity remain controversial. The double-basket catheter was designed to reduce the incidence of catheter dislodgement; however, we have encountered four cases of thoracoamniotic shunt double-basket catheter dislodgement into the fetal thorax. The dislodged shunt catheters were removed safely with thoracoscopic assistance within several days of birth, when additional treatments for pleural effusion were needed, such as thoracic drainage tube insertion and adhesion treatment of the thorax. We report the clinical courses of three of these cases of thoracoamniotic shunt tube dislocation. By waiting several days postnatally for stabilization of respiratory and circulatory status and the effective use of thoracoscopic assistance, the dislodged catheter was safely removed from the neonatal thorax. The accumulation of case reports will help establish suitable treatments, and their indication, for a dislodged thoracoamniotic shunt catheter within the fetal thoracic cavity.

Term Infant With Respiratory Distress and Desaturations

On day 2 after birth, a term male infant began having episodes of tachypnea and oxygen desaturation to 60% to 70% during feeding, which self-resolved after removing the nipple. A chest radiograph showed a hazy opacity in the right lower lung field. Physical examination revealed an unlabored breathing pattern with equal air entry in all lung fields. (Figure 1). Figure 1. Figure 2. Right lung multicystic lesion. Figure 3. Right lung cystic lesion, with possible feeding vessel (anterior view). Figure 4. MRI repeated at 34 weeks’ gestation showed minimal regression of the mass, with continued multicystic appearance but no definitive mass effect (posterior view). ### Prenatal and Birth Histories The patient was not to have anything by mouth, but he continued to have frequent oxygen desaturation episodes without associated respiratory distress.

Thoracoamniotic shunting for fetal pleural effusions using a double‐basket shunt

To describe the safety and efficacy of thoracoamniotic shunting for fetal pleural effusion using a double-basket catheter with a very small diameter (1.47 mm). In this 2-year multicenter, prospective single-arm clinical study registered with the University hospital Medical Information Network (UMIN) Clinical Trials Registry (UMIN00001095); shunting was performed between 18w0d and 33w6d of gestation with this catheter in cases of fetal pleural effusions reaccumulating after thoracocentesis. The primary endpoint measures were maternal and fetal adverse effects and survival in the neonatal period. A total of 24 cases were included, of which 17 had hydrops (71%). The median gestational ages at shunting and delivery were 27.4 and 34.8 weeks, respectively. There were no fetal deaths, lung injuries, or severe maternal complications. Preterm rupture of the membranes occurred in 7/24 (29%) cases at a median of 62 days after the shunting. Preterm rupture of the membranes within 28 days of the procedure occurred in 1/24 (4%) cases. Catheter displacement towards the fetal thoracic cavity occurred in 4/42 (10%) cases. The overall survival rate was 79% (19/24), whereas it was 71% (12/17) in the cases with hydrops. Drainage of fetal pleural effusions with a double-basket shunt is safe and effective, and the shunt could be an alternative device.

OC25.05: Percutaneous laser ablation under ultrasound guidance for fetal hyperechogenic microcystic lung lesions with hydrops

Objectives: To determine the perinatal outcome of fetuses with congenital cystic lung lesions complicated by hydrops, who underwent thoracoamniotic shunting. Methods: A retrospective study of 7 hydropic fetuses with prenatally diagnosed congenital cystic lung malformation (4) or bronchopulmonary sequestration (3), who underwent thoracoamniotic shunting at a single tertiary center. Results: The median gestational age at diagnosis was 23 weeks (15–29) and the median gestational age at shunt insertion was 25 weeks (19–31). In 6 cases, the hydrops resolved following shunt insertion, whereas in one fetus, who underwent shunting at 19 weeks, the hydrops persisted despite the shunting and the patient elected to terminate the pregnancy. All 6 fetuses whose hydrops resolved following shunting were born alive at a median gestational age of 36.5 weeks (33–38) resulting in perinatal survival of 86%. None of the patients delivered prior to 32 weeks of gestation. Conclusions: Hydropic fetuses with congenital cystic lung lesions can benefit from thoraco-amniotic shunting resulting in perinatal survival of 86%. Therefore, hydropic fetuses with macrocystic CCAM or BPS should be offered thoraco-amniotic shunting as the treatment of choice.

OC25.04: Should fetuses with congenital cystic lung lesions complicated by hydrops be treated by thoraco‐amniotic shunting?

Objectives: To determine the perinatal outcome of fetuses with congenital cystic lung lesions complicated by hydrops, who underwent thoracoamniotic shunting. Methods: A retrospective study of 7 hydropic fetuses with prenatally diagnosed congenital cystic lung malformation (4) or bronchopulmonary sequestration (3), who underwent thoracoamniotic shunting at a single tertiary center. Results: The median gestational age at diagnosis was 23 weeks (15–29) and the median gestational age at shunt insertion was 25 weeks (19–31). In 6 cases, the hydrops resolved following shunt insertion, whereas in one fetus, who underwent shunting at 19 weeks, the hydrops persisted despite the shunting and the patient elected to terminate the pregnancy. All 6 fetuses whose hydrops resolved following shunting were born alive at a median gestational age of 36.5 weeks (33–38) resulting in perinatal survival of 86%. None of the patients delivered prior to 32 weeks of gestation. Conclusions: Hydropic fetuses with congenital cystic lung lesions can benefit from thoraco-amniotic shunting resulting in perinatal survival of 86%. Therefore, hydropic fetuses with macrocystic CCAM or BPS should be offered thoraco-amniotic shunting as the treatment of choice.

A Review of Fetal Thoracoamniotic and Vesicoamniotic Shunt Procedures

Fetal anomalies such as lower urinary tract obstructions and fluid-filled space-occupying lesions in the fetal chest can result in severe morbidity or mortality if left untreated. In-utero fetal shunt placement offers the potential to improve outcomes in infants with these conditions. The role of the nurse is paramount in the clinical and psychosocial management of the mother and family.

Fetal thoracoamniotic shunting for large macrocystic congenital cystic adenomatoid malformations of the lung

To evaluate fetal thoracoamniotic shunting for isolated large macrocystic congenital cystic adenomatoid malformations (CCAM) of the lung. This was a retrospective study of 11 fetuses with macrocystic CCAM who underwent thoracoamniotic shunting. This procedure was offered if fetal hydrops or signs of evolving hydrops (such as ascites or polyhydramnios) were present, or when there were very large lesions or lesions rapidly increasing in size. If there were multiple large cysts within the lesion, a single shunt was used, aiming to traverse several cysts. Shunts were inserted at a mean gestational age of 24.6 (range, 17-32) weeks. Marked mediastinal shift was present in all cases. Six fetuses were hydropic and, of the remaining five, one had severe polyhydramnios, three had lesions that were rapidly increasing in size and one had a very large lesion at initial presentation. In total, four cases had polyhydramnios. Shunting one cyst always decompressed the entire lesion and hydrops and/or polyhydramnios resolved in all surviving fetuses. One hydropic fetus that underwent the procedure at 17 weeks died 1 day later. The shunt dislodged in one case and the lesion did not re-expand. No mother went into labor or had ruptured membranes before 35.6 weeks. Mean gestational age at delivery was 38.2 weeks (n = 10). All pregnancies were delivered vaginally, with no maternal complications. All newborns had uneventful lobectomies, and pathology confirmed CCAM in all cases. Fetal thoracoamniotic shunting for large macrocystic CCAM is associated with favorable outcome in most cases, and should be considered in severe cases even before hydrops develops.

Prenatal management and thoracoamniotic shunting in primary fetal pleural effusions: a single centre experience

Primary fetal pleural effusions are rare. If severe, thoracoamniotic shunting is needed. Our objective was to study the management and outcomes of pleural effusions in our unit. Retrospective analysis of primary fetal hydrothorax between 1991 and 2010. Of 41 cases, 23 (56%) were hydropic, and 27 (66%) required shunting. Overall, 2 (4.8%) were diagnosed with a chromosomal condition and 4 (9.6%) with a congenital condition (3 Noonan syndrome, 1 mild structural cardiac defect). There were 5 terminations of pregnancy (TOP), 3 in utero deaths and 33 liveborn neonates (80%). Intact survival rate was 44% (12/27) among those shunted, 56% (23/41) among all cases and 70% (23/33) among all liveborn neonates. Most (87.5%) neonatal deaths occurred in newborns delivered before 34 weeks of gestation. The survival rate was higher in nonhydropic compared with hydropic fetuses (85% vs 47%). There were no procedure-related fetal losses. One in utero death was complicated by fatal maternal amniotic embolism. Fetuses with pleural effusions should undergo expert prenatal workup. Hydropic fetuses and those with massive effusions are candidates for thoracoamniotic shunting.

Masked anemia due to cardiac tamponade in a hydropic fetus caused by placental chorioangioma

A 23-year-old woman in her third pregnancy was referred at 28 + 2 weeks because of fetal hydrops and a suspected placental tumor. Ultrasound examination confirmed severe polyhydramnios, placentomegaly and a large chorioangioma of 104 × 84 × 88 mm. The fetus had hydrops with severe bilateral hydrothorax (Figure 1), generalized skin edema, ascites and holosystolic tricuspid insufficiency. Doppler examination of the middle cerebral artery (MCA) revealed a peak systolic velocity (PSV) of 61 cm/s corresponding to 1.5 multiples of the median1 and normal pulsatility. Umbilical Doppler examination and further investigations (chromosomal analysis, TORCH serology and search for HbF cells in maternal blood) were unremarkable. At 28 + 4 weeks bilateral thoraco–amniotic shunts (Harrison fetal bladder stents) were inserted. Only a few hours after successful drainage and cardiac decompression ultrasound examination revealed an increase of MCA-PSV to 100 cm/s, indicating fetal anemia (Figure 2). Fetal blood sampling confirmed severe anemia (hemoglobin concentration, 5.8 g/dL) and intrauterine transfusion was performed. After the procedure MCA-PSV normalized to 67 cm/s and signs of hydrops started to regress. One week later a second transfusion of packed erythrocytes was necessary. At 30 + 3 weeks of gestation secondary Cesarean section was performed because of preterm rupture of membranes followed by labor. The female newborn (weight, 1610 g; Apgar scores 7, 9 and 10 at 1 min, 5 min and 10 min, respectively; hemoglobin concentration, 13 g/dL) was referred to the neonatal intensive care unit. She was discharged in healthy condition at 6 weeks of age and development at the age of 1.5 years appeared to be normal. Placental histology confirmed a chorioangioma of 115 × 95 mm. Bilateral severe hydrothorax and skin edema in a 28-week fetus. Middle cerebral artery Doppler velocimetry following shunt insertion for bilateral hydrothorax, showing a peak systolic velocity of 100 cm/s. In the presented case chorioangioma led to severe but masked anemia2, 3, which was correctly diagnosed by measuring MCA-PSV4, 5 only after drainage of the severe bilateral hydrothorax. A likely explanation is cardiac compression due to the hydrothorax, leading to impaired ventricular filling and low cardiac output, resulting in relatively normal MCA-PSV despite severe anemia. After successful drainage of fluid the intrathoracic pressure decreased and the cardiac tamponade disappeared, leading to increased ventricular filling, stroke volume and cardiac output which resulted in higher MCA-PSV, correctly indicating the presence of fetal anemia. The pathomechanism of fetal cardiac compression leading to diminished cardiac output and hydrops has been investigated in the context of different thoracic anomalies6, 7. Swast et al.8 measured diminished cardiac output in fetuses with congenital cystic adenomatoid malformation of the lung. Bigras et al.9 found that fetuses with hydrothorax had smaller dimensions of the left ventricle and the peak velocity of the ascending aorta was lower than that of the normal population. Both authors hypothesized that these findings may represent lower preload of the left ventricle caused by increased intrathoracic pressure. In addition, Van Mieghem et al.10 recently found decreased MCA-PSV in fetuses with prenatally diagnosed congenital diaphragmatic hernia. The authors' explanation was similar to our observation, with a smaller dimension of the left ventricle and decreased left ventricular output due to intrathoracic compression. In summary, elevated intrathoracic pressure may generate distinct alterations in fetal hemodynamics, which are likely to affect MCA-PSV. Therefore, evaluation of MCA-PSV to exclude associated fetal anemia should be interpreted with caution in the presence of bilateral hydrothorax. In these cases drainage of bilateral pleural effusions may normalize intrathoracic pressure and increase cardiac output. A. Hellmund*, C. Berg* , B. Rösing*, U. Gembruch*, A. Geipel*, * Department of Obstetrics and Prenatal Medicine, University Medical School Bonn, Bonn, Germany, Division of Prenatal Medicine and Gynecologic Sonography, University Medical School Cologne, Cologne, Germany

Squamous metaplasia in the cyst epithelium of type 1 congenital pulmonary airway malformation after thoracoamniotic shunt placement

Thoracoamniotic shunting is the treatment of choice for management of the fetus with type 1 congenital pulmonary airway malformation. Thoracoamniotic shunting has been performed to reduce life-threatening risks such as fetal hydrops. However, caution is needed because of possible complications. Here, we report that thoracoamniotic shunting can cause histologic changes in the cyst epithelia. In 5 of 8 patients treated prenatally with thoracoamniotic shunting, squamous metaplasia in the cyst epithelia was seen; whereas squamous metaplasia was not found in 6 patients who were not treated with this procedure. Our results reveal that long-term exposure to the intrauterine environment could possibly lead to the change in the nature of cyst epithelium and consequent squamous metaplasia.

Abstract No. 404: Percutaneous in utero thoracoamniotic shunt creation for congenital cystic adenomatoid malformations: a novel technique

In a fetus, congential cystic adenomatoid malformations (CCAM) are often large, can cause mediastinal shift and compression of adjacent lung parenchyma ultimately leading to lung hypoplasia, hydrops and intra-uterine death. Traditionally, thoracoamniotic shunts are created using a Trocar technique. We will describe a novel trans-abdominal, trans-uterine Seldinger based percutaneous approach to create a shunt for treatment of CCAMs.

Experimental treatment of bilateral fetal chylothorax using in‐utero pleurodesis

To assess the use and efficacy of in-utero pleurodesis for experimental treatment of bilateral fetal chylothorax. This was a study of 78 fetuses with bilateral pleural effusion referred to three tertiary referral centers in Taiwan between 2005 and 2009. Fetuses were karyotyped following amniocentesis and the lymphocyte ratio in the pleural effusion was determined following thoracocentesis. Forty-nine (62.8%) fetuses had a normal karyotype and were recognized to have fetal chylothorax; of these, 45 underwent intrapleural injection of 0.1KE OK-432 per side per treatment. We evaluated clinical (hydrops vs. no hydrops) and genetic (mutations in the reported lymphedema-associated loci: VEGFR3, PTPN11, FOXC2, ITGA9) parameters, as well as treatment outcome. Long-term survival was defined as survival to 1 year of age. The overall long-term survival rate (LSR) was 35.6% (16/45); the LSR for non-hydropic fetuses was 66.7% (12/18) and for hydropic fetuses it was 14.8% (4/27). If we included only fetuses with onset of the condition in the second trimester, excluding those with onset in the third trimester, the LSR decreased to 29.4% (10/34). Notably, 29.6% (8/27) of hydropic fetuses had mutations in three of the four loci examined. OK-432 pleurodesis appeared to be an experimental alternative to the gold-standard technique of thoracoamniotic shunting in non-hydropic fetal chylothorax. In hydropic fetuses, pleurodesis appeared less effective.

Grayscale and Doppler sonographic evaluation of response to in utero treatment of hydrops fetalis caused by extralobar pulmonary sequestration

Pulmonary sequestration is defined as nonfunctional lung tissue that lacks communication with the bronchial tree and that is supplied by an anomalous systemic vessel. In comparatively rare cases, pulmonary sequestration may lead to hydrothorax or hydrops fetalis, which is nearly universally fatal. In this report, we describe a case of pulmonary sequestration with hydrops fetalis, which was successfully treated by thoracoamniotic shunting. A sonographic Doppler study in this case suggested that the underlying mechanism of the hydropic change in a fetus with extralobar pulmonary sequestration may have differed from that in fetuses with primary hydrothorax not associated with a structural anomaly.

P21.13: Prognostic factors for fetuses with pleural effusions after thoracoamniotic shunting

P21.13: Prognostic factors for fetuses with pleural effusions after thoracoamniotic shunting

Formation of a membrane from repeated thoracostomy and thoracoamniotic shunting

Formation of a membrane from repeated thoracostomy and thoracoamniotic shunting

444: Perinatal outcomes in low risk and high risk congenital pulmonary airway malformations (CPAMs) as indicated by CPAM volume ratio (CVR)

CPAMs are bronchopulmonary malformations frequently identified on routine prenatal ultrasound. Complications related to rapid growth and mass effect in the chest include polyhydramnios, pulmonary hypoplasia, and hydrops. Hydrops in CPAM is usually fatal without intervention. Prenatal treatment options range from steroid administration, open fetal surgery, thoracoamniotic shunting, or third trimester ex-utero intrapartum treatment by EXIT-to-resection. We reviewed a single center experience with CPAMs and the impact on perinatal outcome.