Outcome of Monochorionic Diamniotic Twins with Twin Reversed Arterial Perfusion Sequence: Interstitial Laser versus Endoscopic Cord Occlusion.

Twin reversed arterial perfusion sequence (TRAP) is a rare complication of monochorionic twins (MC). This study aimed to describe and compare the short- and long-term outcomes of MC pregnancies with the TRAP sequence treated with two different techniques: interstitial fetal laser (IFL) (n = 22) versus endoscopic cord occlusion (CO) (n = 24). The study population included 46 MC pregnancies with TRAP. Pregnancy loss within 2 weeks after the procedure occurred in 27% of cases (6/22) in the group treated with IFL and in 8% of cases (2/24) in the group treated with CO. The survival rate of the pump twin was 73% (16/22) in the IFL group and 83% (20/24) in the group treated with CO. The median gestational age at birth was 38 weeks in the group treated with IFL and 35 weeks in the group treated with CO. The rate of preterm birth before 34 weeks was 12.5% (2/16) in the group treated with IFL and 32% (7/22) in the group treated with CO. In the group treated with IFL, there were no cases of neurological disabilities reported by the parents compared to three cases in the CO group. IFL is associated with a higher risk of early pregnancy loss; however, if the pregnancy progresses, it is associated with lower risks of preterm birth and neurological disabilities in the survivors.

The Polish Society of Gynecologists and Obstetricians Guideline for the diagnostic assessment and management of multiple-gestation pregnancy complicated by fetal growth restriction.

The aim of the Guideline is to unify the diagnostic-therapeutic management of multiple-gestation pregnancies complicated by fetal growth restriction in at least one fetus.

Management Options for Fetal Bronchopulmonary Sequestration.

To evaluate the prenatal course and perinatal outcome of fetuses with bronchopulmonary sequestration (BPS) managed expectantly or using minimally invasive methods. This was a retrospective study of 29 fetuses with suspected BPS managed between 2010 and 2021 in three fetal medicine centers in Poland. Medline was searched to identify cases of BPS managed expectantly or through minimally-invasive methods. In 16 fetuses with BPS, there was no evidence of cardiac compromise. These fetuses were managed expectantly. Thirteen hydropic fetuses with BPS qualified for intrauterine intervention: a thoraco-amniotic shunt (TAS) was inserted in five fetuses, laser coagulation of the feeding vessel was performed in seven cases, and one fetus had combined treatment. In the combined data from the previous and the current study of various percutaneous interventions for BPS associated with hydrops, the survival rate was 91.2% (31/34) for TAS, 98.1% (53/54) for laser coagulation, and 75% (3/4) for intratumor injection of sclerosant. After taking into account cases with available data, the rate of preterm birth before 37 weeks in the group treated with laser coagulation was 14.3% (7/49) compared to 84.6% (22/26) in the group treated with TAS. The need for postnatal sequestrectomy was lower in the group of fetuses treated with laser coagulation 23.5% (12/51) in comparison to fetuses treated with TAS 84% (21/26). In fetuses with BPS without hydrops, progression of the lesion's volume, leading to cardiac compromise, is unlikely. In hydropic fetuses with BPS, intrauterine therapy using minimally invasive methods prevents fetal demise. Both, the rate of preterm birth and the need for postnatal surgery is significantly lower in the group treated with laser coagulation compared to the group treated with TAS.

Percutaneous Intratumor Laser Ablation for Fetal Sacrococcygeal Teratoma.

OBJECTIVE: To evaluate the efficiency of percutaneous intratumor laser ablation for fetal solid sacrococcygeal teratoma (SCT). SUBJECTS AND METHODS: We carried out percutaneous ultrasound-guided intratumor laser ablation through a 17-gauge needle using an output of 40 W in 7 fetuses with large solid SCT and reviewed the literature for minimally invasive therapy for this condition. RESULTS: Laser ablation was carried out at a median gestational age of 20 (range 19-23) weeks, and in all cases there was elimination of obvious vascularization within the tumor and improvement in cardiac function. Three (43%) babies survived and had surgical excision of the tumor within 2 days of birth, 3 liveborn babies died within 5 days of birth and before surgery, and 1 fetus died within 2 weeks after the procedure. In previous series of various percutaneous interventions for predominantly solid SCT the survival rate was 33% (2/6) (95% CI 9.7-70%) for endoscopic laser to superficial vessels, 57% (4/7) (95% CI 25-84%) for intratumor laser, 67% (8/12) (95% CI 39-86%) for intratumor radiofrequency ablation, and 20% (1/5) (95% CI 3.6-62%) for intratumor injection of alcohol. CONCLUSIONS: In solid SCT, the reported survival from intratumor laser or radiofrequency ablation is about 50%, but survival does not mean success, and it remains uncertain whether such interventions are beneficial or not because the number of fetuses is small and there were no controls that were managed expectantly.

Thoracoamniotic Shunts in Macrocystic Lung Lesions: Case Series and Review of the Literature.

OBJECTIVE: To evaluate the efficiency of thoracoamniotic shunts for drainage of macrocystic-type congenital cystic adenomatoid malformation (CCAM). SUBJECTS AND METHODS: This was a retrospective study of 12 fetuses with a large thoracic cyst treated with thoracoamniotic shunting between 2004 and 2014 in a tertiary fetal therapy center. Medline was searched to identify cases of CCAM treated with thoracoamniotic shunting. RESULTS: In all cases the thoracic cyst was associated with major mediastinal shift, the CCAM volume ratio (CVR) was >1.6, and in eight cases there was associated hydrops. Shunt insertion was successfully carried out in all cases at a median gestational age of 24 weeks (range 18-34). In 10 cases there was live birth at a median age of 38 weeks (range 35-41), but in two hydropic fetuses there was intrauterine death. A literature search identified a total of 98 fetuses with CCAM treated with thoracoamniotic shunting between 1987 and 2016. In the combined data from the previous and the current study, the survival rate was 77% (53 of 69) for hydropic and 90% (37 of 41) for nonhydropic fetuses. CONCLUSIONS: The role of thoracoamniotic shunting in macrocystic lung lesions associated with hydrops is well accepted. Intrauterine intervention is also likely to be beneficial in the subgroup of nonhydropic fetuses with a CVR >1.6.

Wspólczesne metody monitorowania dobrostanu plodu w ciazy powiklanej wewnatrzmacicznym zahamowaniem wzrastania.

Intrauterine growth restriction (IUGR) is one of the most important problems in current perinatology. The number of complications such as intrauterine fetal hypoxia, preterm and operative labours, intrauterine demises and neonatal deaths are signifcantly higher among pregnant women with IUGR. The proper monitoring and assesement of the fetal well-being are crucial to make the right decision about optimal time and mode of delivery.

[Fetal therapy--evaluation of intrauterine therapy in congenital cystic adenomatoid malformation of the lung (CCAM)]. / Terapia plodu--ocena skutecznosci leczenia wewnatrzmacicznego wrodzonego zwyrodnienia gruczolakowato-torbielowatego pluc (CCAM).

OBJECTIVES: The aim of the study was to evaluate the efficiency of intrauterine treatment of large cysts in fetal lungs using thoracoamniotic shunts. MATERIAL AND METHODS: Our observational retrospective study was carried out on a series of 8 fetuses who under went thoracoamniotic shunting after sonographic statement of large macrocystic lesions in the lungs at the Department of Gynecology Fertility and Therapy of the Fetus, Polish Mother's Research Institute, between 2009-2014. RESULTS: Mean gestational age at shunt insertion was 26.6 (range 18-33) weeks. Marked mediastinal shift in the echocardiographic examination was observed in all of the investigated cases. Five fetuses had polyhydramion, with 4 hydropic cases. Out of the remaining 4 fetuses without impaired cardiac function, 3 had very large lesions at initial presentation and 1 had a lesion that was rapidly increasing in size. Shunt insertion was successful in all cases. Only one patient went into premature labor (at 36 weeks of gestation). Mean gestational age at delivery was 38.2 weeks. Cesarean section was necessary in the half of the patients due to obstetric complications. All newborns underwent resection of the lesions. Three of them were operated in the first month after birth. The rest of the operations were postponed. Prenatal diagnosis of congenital cystic adenomatoid malformations was confirmed by pathologists in all cases. CONCLUSION: Intrauterine therapy of macrocystic lesions in fetal lungs enables to achieve good perinatal outcome It needs to be considered in every case of a fetus with developing impaired cardiac function.

[Intrauterine therapy for nonimmune hydrops fetalis (NIHF)--analysis of 38 cases]. / Terapia wewnatrzmaciczna w nieimmunologicznym obrzeku plodu (NIHF)--analiza 38 przypadków.

OBJECTIVE: The aim of the study was to perform an audit the results of fetal therapy in cases of nonimmune hydrops fetalis (NIHF), isolated hydrothorax and isolated ascites. METHODS: A total of 38 fetuses (17-35 weeks of pregnancy) were included in the study whereas 6 patients were excluded due to abnormal karyotype. NIHF was diagnosed in 24 cases, hydrothorax in 4 cases, and ascites in 4 cases. Shunts were implanted in 26 (81%) cases and 7 (19%) participants underwent therapeutic cordocentesis. RESULTS: After therapy anterior-posterior diameter of the right and the left lung increased to 9.6 mm (27%) and 12.4 mm (35%), respectively. Early complications were observed in 5 (16%) cases. PROM 2 (40%), fetal death 1 (20%), infection 1 (20%), and preterm delivery 1 (20%). Out of the 27 patients, 65% had a caesarian section without early complications and 35% had a vaginal delivery with 58% at term and 42% pre-term. CONCLUSIONS: Preceding results show that intrauterine therapy significantly improves prognosis of fetuses with NIHF.

[Fetal therapy--evaluation of ventriculo-amniotic shunts in the treatment of hydrocephalus]. / Terapia plodu--ocena zastosowania shuntu komorowo-owodniowego w leczeniu wodoglowia.

OBJECTIVE: The aim of the study was to establish optimal diagnostic and therapeutic scheme and to assess the efficacy of intrauterine therapy of hydrocephalus. MATERIAL AND METHODS: The study was carried out between 1992-2012 on the total of 222 fetuses with hydro- cephalus, using Orbis-Sigma and ACCU-Flow valves (168 cases) and Cook8 shunts, according to a strictly defined diagnostic and therapeutic scheme. RESULTS: In the first stage of the study (between 1992-2001), a total of 168 fetuses with prenatally diagnosed hydrocephalus received intrauterine therapy In 91.6% of the cases the therapy resulted in a decreased size of cerebral ventricles. The valve dislocated in 23 cases (13.6%). Preterm delivery occurred in 44% of the affected neonates. Severe mental impairment occurred in 17.76%, average in 36.8%, and slight in 32.9% of the infants. Normal mental development at the age of 3 was observed in 12.5% of the children. A total of 11.2% of chldren did not require further neurosurgical treatment. In the second stage of the study (between 2006-2012) after therapy the size of the right lateral cerebral ventricle decreased by 54.76% (average of27.54 mm to 12.46 mm) and the left lateral cerebral ventricle decreased by 53.12% (average of 26.41 mm to 12.38 mm) (p=0.0018). The maximum and minimum width of the cerebral cortex increased by 23.06% and 27% (average of 9.04 mm to 11.75 mm vs. 3.65 mm to 5 mm), respectively Early complications were observed in 22% of the cases: PROM (6), intrauterine fetal death (4), intrauterine infection (1), and premature detachment of the placenta (1). Average gestational age at delivery was 34 weeks, and 24% of the patients delivered at term. CONCLUSIONS: Implantation of ventriculoamniotic shunts proved to be an effective form of therapy resulting in normalization of intracranial pressure. In both stages of therapy reduction of ventricular size in patients with hydrocephalus and good neurological outcome (45.4% in I stage, 60% in II stage) were observed. In the second stage of therapy the size of lateral brain ventricles after fetal therapy was significantly lower (54%). A total of 18% of the neonates did not require neurosurgical treatment.

[Successful intrauterine therapy in a fetus with a large lung cyst in the third trimester of pregnancy--case report]. / Skuteczna interwencja wewnqtrzmaciczna u plodu z olbrzymiq torbiela pluca w trzecim trymestrze ciazy--opis przypadku.

Congenital cystic adenomatoid malformation is a rare disorder of the respiratory system which occurs with an incidence of 1/25,000-35,000. Depending on the size of the cysts, CCAM is classified into microcystic and macrocystic. Very large lesions carry a significant risk of causing both, pulmonary hypoplasia due to compression of the lung tissue and fetal hydrops, probably due to impaired cardiac function as a result of mediastinal shift and compression of the vena cava. In this report, we present a case of a fetus with prenatally diagnosed large cyst of the left lung. Despite advanced gestational age, the baby was treated by thoracoamniotic shunting owning to developing impaired cardiac function and polyhydramion. Spectacular effects of the therapy have been achieved.

[Treatment of twin to twin transfusion syndrome - comparison of two therapeutic methods - amnioreduction and lasertherapy]. / Leczenie zespolu przetoczenia miqdzy plodami /TTTS/ - porównanie dwóch terapeutycznych metod - amnioredukcji i laseroterapii.

BACKGROUND: Twin to twin transfusion syndrome occurs in 15% of monochorionic twin pregnancies. Untreated, TTTS has been reported to have a mortality of nearly 100%. Two main therapies include serial amnioreduction and fetoscopic laser coagulation for the vascular anastomoses. OBJECTIVES: The aim of the project was to investigate the optimal diagnostic and therapeutic procedure in pregnancies complicated by TTTS. Additionally the study was supposed to compare non-invasive and invasive methods of treatment and to show antenatal and postnatal follow - up to 4 months of age. METHODS: 42 pregnant women with twin-to-twin transfusion syndrome were assigned to laser therapy using diode laser and 33 pregnant women underwent only several amnioreductions. Selected parameters characterizing the pregnancy were compared in both groups. RESULTS: In the amnioreduction group, the perinatal survival rate seven days after the delivery was 31.8%. The survival rate of at least one twin was 39.4%. As compared to the amnioreduction group, in the laser group the survival rate of at least one twin was observed in 31 cases (31/42) and it was equal to 74%. Neurological complications in the amninoreduction group were observed in 19% (4/21) of cases, in the laser group and in 5% (2/40) of neonates at 4 months of age. CONCLUSIONS: Currently the preferred and only method that addresses the cause of the disease is the endoscopic laser coagulation of anastomoses. Comparison of the two treatments shows better outcomes with higher survival rates and minor neurological defects in cases treated with laser coagulation.

Ultrasound and echocardiographic findings obtained in the second and third trimesters of gestation in fetuses with normal karyotype and increased nuchal translucency.

INTRODUCTION: Numerous papers have proven that an increased nuchal translucency is connected with a raised risk of chromosomal aberrations, but few analyses are related to the further state of fetuses with a normal karyotype. THE AIM OF THE STUDY: The aim of the study was to estimate the risk of cardiac defects and other developmental disorders in fetuses with increased nuchal translucency and normal findings of a standard cytogenetic examination. METHODS: The authors carried out a retrospective analysis of 5183 examinations of 3376 patients who reported to the Department of Diagnosis and Prophylaxis of Congenital Malformations in the Polish Mother's Memorial Hospital in Lódz in the period from January 2008 to March 2011 for prenatal ultrasound and echocardiographic examinations. The authors analyzed the results of the examinations performed in the second and third trimesters of gestation in fetuses with an increased nuchal translucency of ≥3 mm in the first trimester and with a normal karyotype. RESULTS: Fifty-seven patients (1.7% of the examined group) fulfilled the criteria necessary to be included in the study. In 31 pregnant women (54%) structural defects or anomalies of the fetus were found. Cardiac anomalies were detected in 17 fetuses (29.8%). The authors detected various types of cardiac defects such as tetralogy of Fallot, ventricular septal defect, atrioventricular septal defect, transposition of the great arteries and hypoplastic left heart syndrome. CONCLUSIONS: In more than half of the fetuses with an increased nuchal translucency (NT ≥ 3 mm) and a normal karyotype, developmental defects of various organs appeared in the further course of pregnancy: mainly heart defects that were either isolated, or accompanied other anomalies.

The three-vessel view in the fetal mediastinum in the diagnosis of interrupted aortic arch.

Interruption of the aortic arch (IAA) is difficult to detect and diagnose in utero. However, prenatal diagnosis may be beneficial because IAA is rapidly fatal (median age, 10 d) if left uncorrected. Our objective was to review the direct and indirect echocardiographic markers associated with IAA, focusing on the importance of the three-vessel view (3VV), which is obtained during routine ultrasound examination to rule out malformations. We analyzed the fetal echocardiograms of nine fetuses and compared them with 56 normal controls. In each fetus, there was a large discrepancy between the diameter of the larger, dilated pulmonary artery (PA) and smaller, narrow aortic arch (Ao). The calculated ratio of PA/Ao in fetuses with IAA was 2.6 ± 0.4 compared with 1.1 ± 0.09 in normal controls (p < 0.0001). The calculated ratio of PA/Ao in fetuses with IAA type A was 2.1 ± 0.09 and IAA type B 2.9 ± 0.2 (p = 0.0007). Discrepancy between PA/Ao diameters should raise the suspicion of aortic arch anomalies and a large discrepancy is a nearly pathognomonic sign of IAA type B.

Prenatal diagnosis of major aortopulmonary collateral arteries.

BACKGROUND: The presence of foetal major aortopulmonary collateral arteries (MAPCAs) is associated with adverse outcome, therefore early diagnosis is essential. AIM: To evaluate the usefulness of foetal echocardiography in the diagnosis and evaluation of MAPCAs in foetuses with pulmonary atresia, as well as to assess the effects of prenatal diagnosis on the management of neonates with pulmonary atresia. METHODS: From 11,678 examined foetuses, we retrieved 15 cases of patients with MAPCAs and congenital heart defects which had been diagnosed by foetal echocardiography (1994-2008), using 2D echocardiography + color-Doppler (CD) + pulsed Doppler (2DD) and spatio-temporal image correlation (STIC) techniques. In 13 patients, MAPCAs were confirmed after birth based on angiography. RESULTS: In all cases, vessels corresponding to MAPCAs were visible in longitudinal view with CD, and in three cases were additionally confirmed by STIC technique. In nine cases one, in four cases two, and in two cases three MAPCAs were suspected. In two cases, MAPCAs were not confirmed after birth; one due to misdiagnosis secondary to aberrant right subclavian artery, and one because of abnormal ductus arteriosus course coexistent with right aortic arch. CONCLUSIONS: In foetuses with pulmonary atresia, it is possible to find MAPCAs with current technology (both 2D + CD, power angiography and real time-3D echocardiograophy [4D]). The differential diagnosis (MAPCAs or other vessels) should be included. Although prenatal diagnosis does not change the obstetrical management, it is important information for a paediatric cardiologist. Early neonatal angiography might be of great value not only in confirming MAPCAs, but also in performing cardiac intervention and in some cases preventing future heart failure.

[Maternal hyperoxygenation test in prediction of fetal lung hypoplasia--preliminary report]. / Przydatnosc testu hiperoksygenacji w prognozowaniu hipoplazji pluc u plodów--donlesienie wstepne.

OBJECTIVES: The aim of our study was to present the detailed execution and interpretation of the hyperoxygenation test in the fetus. MATERIAL AND METHODS: This was a retrospective investigation of fetuses examined in The Department for Diagnosis and Prophylaxis of Congenital Malformations (Polish Mothers Memorial Hospital Institute and Medical University in Lodz) between January 2006 and December 2009, in whom in addition to the routine echocardiographic examination the hyperoxygenation test was performed. Indications for such an extended evaluation were suspected fetal malformations and pulmonary hypoplasia. Changes in the fetal pulmonary circulation before and after maternal exposure to hyperoxygenated air together with the newborn follow-up were analyzed. RESULTS: Clinical outcome was available for 42 of 52 cases: 16 patients died (including 11 cases with negative hyperoxygenation test), whereas 24 patients were discharged home (including 17 cases with positive hyperoxygenation test). The probability of survival for fetuses with the positive test was significantly higher than for fetuses with the negative one (p = 0.016, Fischer's exact test). CONCLUSIONS: Based on changes evoked in the fetal pulmonary circulation, the hyperoxygenation test seems helpful in predicting impaired fetal lung development Functional assessment of the fetal pulmonary circulation may be useful in predicting fetal lung hypoplasia and respiratory failure in the neonate.

Prenatal diagnosis and follow-up of 23 cases of cardiac tumors.

OBJECTIVE: To evaluate the prenatal characteristics and postnatal outcome of cardiac tumors diagnosed at two prenatal Polish cardiology centers. METHODS: Descriptive analysis of 23 fetuses with cardiac tumors (12 multiple and 11 single) diagnosed over 16 years (from 1993 to 2009). Congestive heart failure was diagnosed when the cardiovascular profile score was seven or less. RESULTS: Associated structural congenital heart defects were present in three fetuses, extracardiac anomalies in three, and chromosomal anomalies in two. Congestive heart failure developed in five cases. Perinatal survival was not different between cases with and without cardiac failure (2/5 vs 12/18, p = 0.28). The main ultrasonographic signs observed prenatally in association with cardiac tumors were cardiomegaly, left ventricular outflow tract obstruction, pericardial effusion, and hypokinesis. A diagnosis of tuberous sclerosis was eventually made in all 12 fetuses with multiple tumors. Perinatal death occurred in 4/11 cases with single tumors and in 5/12 with multiple tumors (p = 0.57). Surgical resection of the tumor was performed in 3/11 neonates with single tumors (histopathologically: rhabdomyoma, teratoma, and fibroma) and in 2/12 with multiple tumors (both rhabdomyomas). CONCLUSIONS: Survival is not different between neonates with single and multiple tumors and between those with and without congestive heart failure.