Pleuroamniotic Shunt Research Articles

Congenital chylothorax in a preterm infant with non-immune hydrops: A case report with literature review on treatment options

Congenital chylothorax is rare with an incidence of 1:10,000-24,000 live births. Congenital chylothorax occurs in lymphatic disorders, heart diseases, cancers, chromosomal abnormalities, tracheoesophageal fistula, etc. Acquired causes are due to surgical thoracic duct trauma. Many cases of chylothorax are idiopathic. The case fatality rate is 15% to 57% and reaches 98% in hydrops foetalis. There is no set standard of treatment for congenital chylothorax. Prenatally, thoracentesis or pleuro-amniotic shunts are done. Postnatally, management of cause, drainage, dietary changes, Octreotide, and Sildenafil have variable results. Pleurodesis, pleuroperitoneal shunt, abrasion, thoracic duct ligation, and anastomoses are surgical options.

Fetal Hydrothorax Treated with Pleuro-Amniotic Shunting: Fetal and Maternal Complications and Long-Term Outcomes

Introduction: We aimed to identify maternal and fetal complications and investigate postnatal and long-term outcomes of fetal hydrothorax (FHT) treated with pleuro-amniotic shunting (shunt). Methods: Single-center retrospective observational cohort of shunt cases performed from 2000 to 2021. Risk factors for maternal complications, fetal demise, neonatal death (NND), and postnatal outcomes were identified. Results: Out of 88 cases, 70 (79.5%) were complicated by hydrops, with an average gestational age (GA) at diagnosis of 27 weeks (range 16–34). In 16 cases, definitive etiology of FHT was identified; five cases of Noonan syndrome and three cases of monogenic disorders diagnosed by whole-exome sequencing (EPHB4, VEGFR3, RASA1). Shunt was performed at an average GA of 28 weeks (20–34), with a dislodgement in 10 cases (11.4%). Maternal: Complications occurred in three cases; survival rate was 76.1% (67/88). Follow-up data were available for 57/67 (85.1%) children. Incidence of severe neurodevelopmental impairment and pneumopathy (broncho dysplasia, persistent pulmonary hypertension of newborn, and asthma) was 5.3% and 8.8%, respectively. Post-treatment persistence of hydrops, FHT associated with genetic syndromes, and GA at birth were risk factors for fetal demise, NND, and postnatal complications. Conclusion: In truly isolated FHT, whenever indicated, pleuro-amniotic shunting is a safe procedure associated with good survival rate and long-term outcome.

VP52.07: Single percutaneous uterine access for bilateral pleuroamniotic shunting in fetuses with severe hydrothorax and hydrops

VP52.07: Single percutaneous uterine access for bilateral pleuroamniotic shunting in fetuses with severe hydrothorax and hydrops

The Use of Somatex Shunt for Fetal Pleural Effusion: A Cohort of 8 Procedures

Introduction: Fetal pleural effusion may require in utero shunting which is associated with procedure-related complications. Objective: To evaluate the efficacy and complications of the newly designed Somatex shunt in treating fetal pleural effusion. Methods: Consecutive cases with primary fetal pleural effusion who were treated with the Somatex shunt between 2018 and 2019 were evaluated. Perinatal outcomes and complications were retrospectively analyzed. Results: There were 6 cases of unilateral and 1 case of bilateral pleural effusion, and hence a total of 8 pleuroamniotic shunting procedures were performed. The median gestational age at diagnosis and shunting was 20.7 and 22.6 weeks, respectively. All 8 procedures were successful, achieving complete in utero drainage. All but one were live births (85.7%) with a median gestational age of 38 weeks. The single case of in utero death occurred 4.7 weeks after successful shunting, and no cause could be identified after autopsy. The rates of preterm birth and premature rupture of membranes were 33.3% (2/6) and 16.7% (1/6), respectively. Four of the 8 procedures (50%) had minor shunt-related complications such as dislodgement and entrapment, occurring at a median of 7.7 weeks after shunting. None of the shunts became blocked. Conclusions: The Somatex shunt is effective in relieving fetal pleural effusions with good survival rate. Overall, it was a safe instrument, though minor shunt complications occurred.

Preoperative Cervical Length Predicts the Risk of Delivery within One Week after Pleuroamniotic Shunt in Fetuses with Severe Hydrothorax

Objective: The objective of this study was to assess the predictive performance of preoperative cervical length (CL) for delivery within 1 week after pleuroamniotic shunting (PAS) in fetuses with severe hydrothorax. Methods: A prospective cohort of fetuses with severe hydrothorax referred to our fetal surgery center in Querétaro, Mexico from January 2012 to July 2020. Severe fetal hydrothorax was diagnosed as an accumulation of fluid within the fetal pleural space accompanied with severe bilateral lung compression, mediastinal shift, polyhydramnios, and/or hydrops. Transvaginal CL was measured immediately before PAS, and a short cervix was defined as that <25 mm. The interval from fetal intervention to delivery, prevalence of preterm prelabor rupture of membranes (PPROMs), and associations with delivery within the first week after PAS according to a short or a normal CL, were evaluated. Results: Thirty-five pregnancies with severe fetal hydrothorax treated with PAS were evaluated. Median gestational age at PAS was (weeks + days) 31⁺² (range, 26⁺⁰–36⁺¹). Two (5.7%) and 7 (20.0%) cases delivered within the first 24 h and 1 week after PAS, respectively. Ten (28.6%) women had a short cervix before PAS, while 25 (71.4%) had normal preoperative CL. Women with a short cervix showed lower mean interval between fetal intervention and delivery (2.4 vs. 5.5 weeks, p = 0.01), and higher prevalence of PPROM (50 vs. 12%, p = 0.01), as compared to women with a nonshort cervix. Preoperative short cervix was associated with significantly higher risk of delivery within the first 24 h (20.0 vs. 0%, respectively, p < 0.05) and 1 week after PAS (50.0 vs. 8.0%, respectively, p < 0.01) compared with pregnancies with normal preoperative CL. Conclusion: In pregnancies with severe fetal hydrothorax candidates for pleuroamniotic shunt, identification of a short cervix before fetal intervention can predict delivery within 1 week after the surgical procedure.

Single Uterine Access for Bilateral Pleuroamniotic Shunting in Fetuses with Severe Hydrothorax by an Internal Rotational Maneuver: Feasibility and Outcomes between Successful and Failed Procedures

Objective: The objective of this study was to describe the feasibility of single percutaneous uterine access for bilateral pleuroamniotic shunting (PAS) in fetuses with severe hydrothorax by using an internal rotational maneuver and to compare perinatal outcomes between successful and failed procedures. Methods: A prospective cohort of 25 fetuses with isolated bilateral hydrothorax and hydrops were referred to our fetal surgery center in Queretaro, Mexico during an 8-year period. Bilateral PAS was first attempted through a percutaneous single uterine access by internal rotation of the fetus, which was achieved by using the blunt tip of the same cannula, and in case of a failed procedure, a second uterine port was used to place the second shunt. The perinatal outcomes between successful (single uterine port) and failed (2 uterine ports) fetal procedures were compared. Results: Placing of bilateral shunts through a percutaneous single uterine access was feasible in 15/25 (60%) cases. Overall, median GA at delivery was 35.2 weeks with a survival rate of 64.0% (16/25). Three cases were excluded due to shunt dislodgement, leaving a final population of 22 fetuses; 13/22 (59.1%) and 9/22 (40.9%) managed using 1 and 2 uterine ports, respectively. The group with bilateral PAS placement through a successful single uterine port showed a significantly higher GA at birth (36.5 vs. 32.8 weeks, p = 0.001), lower surgical time (11.0 vs. 19.0 min, p = 0.01), longer interval between fetal intervention and delivery (5.7 vs. 2.7 weeks, p = 0.01), lower risk of preterm delivery (46.2 vs. 100%, p < 0.01), and lower rate of perinatal death (15.4 vs. 55.6%, p < 0.05) than the failed procedures requiring 2 uterine ports. Conclusion: In fetuses with severe bilateral hydrothorax and hydrops, bilateral pleuroamniotic shunting through a successful single percutaneous uterine access is feasible in up to 60% of cases and is associated with better perinatal outcomes.

Non-immune fetal hydrops: etiology and outcome according to gestational age at diagnosis.

Fetal hydrops is associated with increased perinatal morbidity and mortality. The etiology and outcome of fetal hydrops may differ according to the gestational age at diagnosis. The aim of this study was to evaluate the cause, evolution and outcome of non-immune fetal hydrops (NIFH), according to the gestational age at diagnosis. This was a retrospective cohort study of all singleton pregnancies complicated by NIFH, at the Fetal Medicine Unit at St George's University Hospital, London, UK, between 2000 and 2018. All fetuses had detailed anomaly and cardiac ultrasound scans, karyotyping and infection screening. Prenatal diagnostic and therapeutic intervention, gestational age at diagnosis and delivery, as well as pregnancy outcome, were recorded. Regression analysis was used to test for potential association between possible risk factors and perinatal mortality. We included 273 fetuses with NIFH. The etiology of the condition varied significantly in the three trimesters. Excluding 30 women who declined invasive testing, the cause of NIFH was defined as unknown in 62 of the remaining 243 cases (25.5%). Chromosomal aneuploidy was the most common cause of NIFH in the first trimester. It continued to be a significant etiologic factor in the second trimester, along with congenital infection. In the third trimester, the most common etiology was cardiovascular abnormality. Among the 152 (55.7%) women continuing the pregnancy, 48 (31.6%) underwent fetal intervention, including the insertion of pleuroamniotic shunts, fetal blood transfusion and thoracentesis. Fetal intervention was associated significantly with lower perinatal mortality (odds ratio (OR), 0.30 (95% CI, 0.14-0.61); P < 0.001); this association remained significant after excluding cases with a diagnosis of anemia or infection (OR, 0.29 (95% CI, 0.13-0.66); P = 0.003). In 104 fetuses not undergoing active fetal intervention, the gestational age at diagnosis was the only parameter that was significantly associated with the risk of perinatal mortality (OR, 0.92 (95% CI, 0.85-0.99); P = 0.035), while the affected body cavity and polyhydramnios were not (P > 0.05). An earlier gestational age at diagnosis of NIFH was associated with an increased risk of aneuploidy and worse pregnancy outcome, including a higher risk of perinatal loss. Fetal therapy was associated significantly with lower perinatal mortality. © 2020 Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

OC23.02: In utero treatment of prenatal thoracic abnormalities by pleuro‐amniotic shunts (PAS): short‐ and long‐term outcome

OC23.02: In utero treatment of prenatal thoracic abnormalities by pleuro‐amniotic shunts (PAS): short‐ and long‐term outcome

Resolution of maternal Mirror syndrome after succesful fetal intrauterine therapy: a case series

BackgroundMirror syndrome (MS) is a rare obstetric condition usually defined as the development of maternal edema in association with fetal hydrops. The pathogenesis of MS remains unclear and may be misdiagnosed as pre-eclampsia.Case presentationWe report a case series of MS in which fetal therapy (intrauterine blood transfusion and pleuroamniotic shunt) resulted in fetal as well as maternal favourable course with complete resolution of the condition in both mother and fetus.ConclusionsOur case series add new evidence to support that early diagnosis of MS followed by fetal therapy and clinical maternal support are critical for a good outcome.

OP23.09: Perinatal outcome in fetuses with dislodged pleuroamniotic shunts (PAS)

OP23.09: Perinatal outcome in fetuses with dislodged pleuroamniotic shunts (PAS)

Perinatal features of congenital cystic lung diseases: results of a nationwide multicentric study in Japan.

The current study aimed to assess the perinatal risk and clinical features of congenital cystic lung diseases (CCLD). Of the 874 CCLD patients identified in a nationwide survey, 428 patients born between 1992 and 2012 and treated at 10 high-volume centers, were retrospectively reviewed. Fetal hydrops was visualized using MRI in 9.2% of the patients. Prenatal interventions were described for 221 of the 428 patients, including the maternal administration of steroid and pleuro-amniotic shunting. Postnatally, a right-to-left shunt flow through a persistent ductus arteriosus was observed in 7.8% of the patients. The fetal lung lesion volume ratio (LVR) was significantly higher among these symptomatic patients (2.04±1.71 vs. 0.98±0.50, P<0.00071), and decreased to a greater degree in non-CCAM patients compared with CCAM patients during the late gestational period (from 1.37±1.28 to 1.14±0.84 in CCAM and from 1.08±0.47 to 0.46±0.64 in non-CCAM). An estimated 8-9% of prenatally diagnosed patients carry the highest risk of perinatal respiratory distress. Fetal LVR remaining at a high level during the late gestational period seems to predict a high risk.

Unusual pleuroamniotic shunt complication managed using a two‐port in‐CO2 fetoscopic technique: technical and ethical considerations

Unusual pleuroamniotic shunt complication managed using a two‐port in‐<scp>CO₂</scp> fetoscopic technique: technical and ethical considerations

Toracocentesis intrauterina de quilotórax congénito: reporte de un cas o

El quilotórax congénito es una anomalía rara que se asocia a una alta mortalidad perinatal, cuando se acompaña de hidropesía fetal. Tanto la toracocentesis como la derivación pleuroamniótica han mostrado ser alternativas exitosas de tratamiento antenatal; pero, aún existe controversia sobre cuál es la primera elección. En el Perú no se ha comunicado casos similares tratados in útero hasta la fecha. Se presenta el caso de un embarazo de 37½ semanas afectado con quilotórax masivo de aparición tardía, tratado exitosamente mediante toracocentesis intrauterina. Se logró una adecuada expansión pulmonar, permitiendo el nacimiento de un bebe que no presentó insuficiencia respiratoria y no requirió soporte ventilatorio posterior. Se demostró que la toracocentesis intrauterina es una alternativa viable de tratamiento para casos similares en nuestro medio.

First Case of Congenital Myeloproliferative Disorder in a Newborn Diagnosed With Noonan Syndrome

Noonan syndrome (NS) is one of the most common genetic syndromes, but its diagnosis is difficult antenatally because prenatal ultrasound findings are unspecific. Infants with NS are predisposed to developing juvenile myelomonocytic leukaemia (JMML) or myeloproloferative disorders. We report a case of severe polyhydramnios and hydrops fetalis at 32+6 weeks gestation, complicated by preterm labour. Tocolysis, amnioreduction and pleuroamniotic shunt were performed. Fetal blood sampling showed: 1127 monocytes/mm3 and 245 metamyelocytes/mm3. The patient gave birth at 33 weeks and 4 days to a 2780 g male baby. Absolute monocyte count was maximum at 8000/mm3, without blasts in peripheral blood. Study of the PTPN11 gene identified a de novo heterozygous missense mutation. Chemotherapy could not be started due to the severity of the multiple organ failure. The patient died at 2 months old. The prenatal monocytosis >1000/μL is one of the criteria for JMML. We suggest performing a cordocentesis, including white cell blood count in order to search for myelomonocytic disorders, especially in cases of hydropic fetuses and severe pleural effusions, before placing pleuroamniotic shunts. This could help evoking the diagnosis of NS and anticipating the postnatal clinical course.

Bronchopulmonary sequestration with massive pleural effusion: pleuroamniotic shunting vs intrafetal vascular laser ablation

To assess the incidence of complications among a relatively large cohort of fetuses with bronchopulmonary sequestration (BPS) and the success of two different intrauterine treatment modalities. All cases with a prenatal diagnosis of BPS detected in a 10-year period (2002-2011) in two tertiary referral centers were reviewed retrospectively for intrauterine course and outcome. Up to May 2010 severe pleural effusions were treated with pleuroamniotic shunting. Thereafter, they were treated with ultrasound-guided laser coagulation of the feeding artery. A total of 41 fetuses with BPS were included in the study. In 29 (70.7%) there was no pleural effusion or hydrops and they were treated conservatively. In 19/29 (65.5%) there was partial or complete regression of the lesion during the course of pregnancy. All were born alive (median age at delivery, 38.3 (interquartile range (IQR), 34.0-39.6) weeks) and 16 (55.2%) required sequestrectomy. Intrauterine intervention was performed in all 12 (29.3%) fetuses with pleural effusion. Seven fetuses were treated with pleuroamniotic shunting. One fetus with severe hydrops died in utero. There was no complete regression in any case of BPS in this group. Six infants were born alive (median age, 37.2 (IQR, 30.3-37.4) weeks), of which five (83.3%) required sequestrectomy. Five fetuses were treated with laser ablation of the feeding vessel. In all cases of BPS there was regression after laser ablation. All infants were delivered at term (median age, 39.1 (IQR, 38.0-40.0) weeks). One (20.0%) neonate required sequestrectomy after birth. Following intrauterine shunt placement complete regression of the lesion was significantly less frequent (0/7 (0%) with shunt placement vs 4/5 (80%) with intrafetal laser treatment) and gestational age at birth was significantly lower, compared to treatment with intrafetal laser. Complete regression of the lesion was also significantly more frequent in the laser group compared to cases without intervention. In the absence of pleural effusion, the likelihood of spontaneous regression of BPS is high and the prognosis is therefore favorable. In cases with massive pleural effusion, treatment by laser ablation of the feeding vessel seems to be more effective than is pleuroamniotic shunting, with fewer complications. It might also reduce the need for postnatal surgery.

Successful treatment of fetal bilateral primary chylothorax – report of the two cases

Primary fetal chylothorax is an uncommon complication, associated with high perinatal morbidity and mortality. In our report, we describe two cases of fetal bilateral primary chylothorax successfully treated with pleuro-amniotic shunting. In both cases, ultrasound scans showed bilateral, hypoechoic fluid in the pleural space without any associated structural malformations and features of infection and aneuploidy Laboratory analysis of pleural fluids revealed 79% and 92% of lymphocytes, respectively confirming chylothorax in both fetuses. In the first case, pleuro-amniotic shunts were successfully inserted at 31 weeks and 6 days of gestation. Ultrasound scan after two weeks showed expansion of the left lung and lack of fluid in both pleural cavities. At 39 weeks of gestation, a 2660 g baby boy was delivered by cesarean section (Apgar score: 9). The child did not require surgical intervention and was discharged home on day 16 of life. In the second case, the insertion of shunts (at 24 weeks and 6 days of gestation) also significantly reduced the amount of the fluid in the pleural cavities, but one shunt had to be surgically removed after birth. At 30 weeks and 2 days of gestation, a cesarean section was performed due to maternal cholestasis. A female weighing 1400 g was delivered (Apgar score: 7). The chest X-ray revealed only a small amount of fluid in the left pleural cavity The infant was discharged on postnatal day 26, in good condition and with body weight of 2150 g. Pleuro-amniotic shunt insertion is a method of choice in the treatment of confirmed primary fetal chylothorax.

Recurrent Congenital Chylothorax: A Case Report

We report a case of fetal chylothorax and hydrops. Chylothorax is a very rare condition, occurring in approximately 1 in 10000-15000 pregnancies, with an overall mortality rate of 25% to 50%. It is the most common form of pleural effusion in the prenatal period. Available treatments include thoracocentesis, pleuro-amniotic shunting and pleurodesis and the optimal antenatal management and timing are still controversial. We report a case diagnosed at 30 weeks' gestation that was submitted to bilateral pleural-amniotic shunt, but turned in to increased bilateral pleural effusion, generalized hydrops and polyhydramnios. Attempted intervention allowed to achieve good obstetric outcome No etiology was found, revealing the diagnostic challenge this entity can be.

Pulmonary lymphangiomatosis as a cause of first trimester nuchal cysts in a euploid fetus

Abstract Increased nuchal and perinuchal translucency in euploid fetuses can be due to a wide range of congenital malformations, metabolopathies or syndromes. We present a case of transient increased perinuchal fluid accumulation in a euploid female fetus that showed no anomalies in the morphological ultrasound scan on the 20th week of gestation. The lymphatic fluid accumulation resolved spontaneously but hydrothorax developed at week 29 of pregnancy. The hydrops recurred despite the placement of a pleuro-amniotic shunt, and the gestation ended in premature delivery and early neonatal death due to massive hydrops. Necropsy revealed pulmonary lymphangiomatosis. This disease had not been previously related to increased perinuchal translucency in a euploid fetus.

胸壁内に遺残した胎児胸腔羊水腔シャントチューブに対し，2 方向より剥離を行い抜去した 1 例

胸壁内に遺残した胎児胸腔羊水腔シャントチューブに対し，2 方向より剥離を行い抜去した 1 例

1278 Outcome Following Fetal Pleuroamniotic Shunting in 114 Large Hydrothoraces

IntroductionUntreated, fetal hydrothorax is associated with significant morbidity and a mortality rate of approximately 80–90%.Population114 fetuses with isolated large pleural effusions underwent pleuroamniotic shunting at our perinatal centre. All had...