Long-term Outcomes, Including Fetal and Neonatal Prognosis, of Renal Oligohydramnios: A Retrospective Study over 22 Years.

OBJECTIVE: To assess the long-term outcome of renal oligohydramnios and risk factors for fetal, neonatal, and postneonatal death. STUDY DESIGN: This retrospective cohort study included fetuses with prenatally detected renal oligohydramnios between 2002 and 2023. Patients who were lost to follow-up were excluded. Fetal, neonatal, and long-term outcomes were evaluated, and their risk factors were analyzed. RESULTS: Of 131 fetuses with renal oligohydramnios, 46 (35%) underwent a termination of pregnancy, 11 (8%) had an intrauterine fetal death, 26 (20%) had a neonatal death, nine (7%) had a postneonatal death, and 39 (30%) survived. Logistic regression analyses showed that an earlier gestational age at onset (odds ratio 1.16, 95% confidence interval (CI) 1.01-1.37) was significantly associated with intrauterine fetal death; anhydramnios (odds ratio 12.7, 95% CI 1.52-106.7) was significantly associated with neonatal death as a prenatal factor. Although neonatal survival rates for bilateral renal agenesis, bilateral multicystic dysplastic kidney (MCDK), and unilateral MCDK with contralateral renal agenesis were lower than for other kidney diseases, one case of bilateral renal agenesis and two of bilateral MCDK survived with fetal intervention. Kaplan-Meier overall survival rates were 57%, 55%, and 51% for 1, 3, and 5 years, respectively. In the Cox proportional hazards model, birth weight <2000 g (hazard ratio 7.33, 95% CI 1.48-36.1) and gastrointestinal comorbidity (hazard ratio 4.37, 95% CI 1.03-18.5) were significant risk factors for postneonatal death. CONCLUSION: Long-term survival following renal oligohydramnios is a feasible goal and its appropriate risk assessment is important.

Clinical features of fetal hydrothorax associated with mucopolysaccharidosis-VII.

Mucopolysaccharidosis (MPS)-VII, called Sly disease, is a lysosomal storage disorder that can cause fetal hydrops, including fetal hydrothorax (FHT). We describe two fetal cases that received thoracoamniotic shunting for FHT, which was later found to be associated with MPS-VII by exome sequencing. Bilateral FHT accompanied by skin edema and ascites was found before 20 weeks of gestation in both cases. One fetus died in utero at 35 weeks of gestation, and the other survived with preterm delivery at 30 weeks of gestation. Both cases inherited compound pathogenic variants of GUSB from parents. Comparison with previously reported primary FHT cases revealed distinct clinical features in MPS-VII-associated FHT: early gestational age at diagnosis (<26 weeks), bilateral effusion, skin edema with ascites, and poor survival. A genetic analysis would be considered for FHT cases, with consideration of shunting when they show early-onset bilateral effusions with skin edema and ascites.

Ultrasound measurement of umbilical venous flow volume at the intra-abdominal portion in normal fetuses.

PURPOSE: Umbilical venous flow volume (UVFV) measured using ultrasound can be used to assess placental circulation in a fetus. UVFV measured at the intra-abdominal portion using half the maximum flow velocity of the umbilical vein (UV) has good reproducibility with low variance. However, reference values in previous reports were based on a small number of cases with a wide reference range. In the present study, we evaluated UVFV standard values measured at the intra-abdominal portion in normal Japanese fetuses. METHODS: Measurements were performed on normal pregnant women during routine ultrasound screening at around 20 or 30 weeks of gestation. The diameter and flow velocity of the UV were measured at the fetal abdomen point between the insertion of the UV and branches of the portal vein. UVFV (ml/min) was calculated as follows: (UV diameter [cm]/2)2 × maximum velocity [cm/s] × 0.5 × 3.14 × 60). RESULTS: A total of 278 pregnant women were included in the study. UVFV increased with gestational weeks, and UVFV per estimated fetal weight (EFW) slightly decreased with increasing gestational weeks. The 50th (10th-90th) percentiles of UVFV per EFW at 20, 25, and 30 weeks of gestation were 130 (105-165), 123 (94-147), and 104 (80-131) ml/min/kg, respectively. CONCLUSION: New UVFV reference values measured at the intra-abdominal portion of fetuses using large-scale samples were established. Future studies should assess fetuses under pathologic conditions using UVFV reference values.

A fetus with large mediastinal cystic lymphatic malformation managed with prenatal serial thoracocentesis and postnatal sclerotherapy.

We report a very rare case of large fetal mediastinal cystic lymphatic malformation (cLM), formerly called lymphangioma, that was managed with prenatal serial thoracocentesis and postnatal sclerotherapy. A fetal pericardial effusion-like lesion gradually increased the size until it occupied more than half of the thoracic cavity. Thoracocentesis was performed three times, namely at 31 and 35 weeks of gestation, and also just before the delivery for decompression. The characteristic shape changes of the lesion were observed after thoracocentesis. A boy delivered by planned cesarian section at term required respiratory support after birth. Postnatal magnetic resonance imaging (MRI), which showed a large cystic lesion with septa and hemorrhage without solid components, led to the diagnosis of cLM. The fluid from the cyst continued to be drained. Neonatal sclerotherapy using OK-432 was effective and the infant recovered well. Fetal thoracocentesis may be useful for the diagnosis and management of a large mediastinal cLM.

Long-term neurodevelopmental outcomes of the pump twin in twin reversed arterial perfusion sequence treated by radiofrequency ablation.

OBJECTIVES: To assess long-term neurodevelopmental outcomes in children after radiofrequency ablation (RFA) for twin reversed arterial perfusion (TRAP) sequence. METHODS: This cross-sectional study included children who underwent RFA for the TRAP sequence between 2012 and 2018. We assessed neurodevelopment in children using the Kinder Infant Development Scale, a validated questionnaire. The developmental quotient (DQ) assessed in nine subscales was calculated as the developmental age divided by the chronological age. Neurodevelopmental delay (NDD) was defined as a DQ of <70 points. RESULTS: In total, 38 children from 37 pregnancies underwent RFA for the TRAP sequence during the study period; 6 fetuses died in utero. We sent the questionnaire to the parents of the 32 surviving children and obtained answers for 27 (84%). The median age at the assessment was 2 years and 5 months old. The median total DQ was 111 (80-150). Most median DQs in the nine subscales were above 70. The incidence of NDD was 0% (0/27). There were no marked differences in DQ by chorionicity. CONCLUSIONS: Children who survived after RFA for TRAP sequence showed favorable long-term neurodevelopmental outcomes. Radiofrequency ablation seems to rarely affect fetal neurodevelopment. Pregnant women with TRAP sequence are encouraged to be treated by RFA.

Prenatally diagnosed lower urinary tract obstruction: A 15-year experience at two tertiary centers in Japan.

AIM: To examine the outcomes of prenatally diagnosed lower urinary tract obstruction (LUTO) with current management using vesicoamniotic shunting (VAS). METHODS: A retrospective study of prenatally diagnosed LUTO before 26 weeks of gestation at two tertiary centers in Japan between March 2002 and September 2017. LUTO was diagnosed by ultrasound demonstration of an enlarged fetal bladder associated with hydronephrosis and/or hydroureters. VAS was offered for fetuses with LUTO at ≤26 weeks of gestational age, in the presence of oligohydramnios or decreasing amniotic fluid and a favorable fetal urinary analysis. RESULTS: Among 87 fetuses with LUTO, 46 (53%) were terminated before 22 weeks of gestation. Eight cases (9%) underwent VAS and one underwent fetoscopic urethrotomy. The live birth rates in the VAS and expectant groups were 100% (8/8) and 56% (18/32), respectively (p = 0.034), and the survival rates at 6 months old with a normal renal function were 38% (3/8) and 16% (5/32), respectively (p = 0.608). The etiology varied with six cases of associated anomalies among 23 diagnosed cases. Among the nine cases of posterior urethral valve (PUV), only one fetus underwent VAS at 25 weeks of gestation, ultimately surviving with mild renal dysfunction. Among the other eight cases of PUV that were managed expectantly, two died, and only one of the six survivors showed a normal renal function. CONCLUSIONS: More than half of the prenatally diagnosed LUTO cases were terminated. VAS seemed effective for achieving a perinatal survival, regardless of etiology. The outcomes were poor in cases of expectantly managed PUV.

Risk Profiling of the Solomon Technique versus Selective Technique of Fetoscopic Laser Surgery for Twin-Twin Transfusion Syndrome.

We evaluated the outcomes and adverse events after fetoscopic laser surgery (FLS) for twin-twin transfusion syndrome (TTTS) using the Solomon technique in comparison to the selective technique. A retrospective analysis of a single-center consecutive cohort of FLS-treated TTTS using the selective (January 2010 to July 2014) and Solomon (August 2014 to December 2017) techniques was performed. Among 395 cases, 227 underwent selective coagulation and 168 underwent the Solomon technique. The incidence rates of recurrent TTTS (Solomon vs. selective: 0% vs. .9%, p = .510) and twin anemia-polycythemia sequence (.6% vs. .4%, p = .670) were very low in both groups. The incidence rates of placental abruption (Solomon vs. selective: 10.7% vs. 3.5%, p = .007) and preterm premature rupture of the membranes (pPROM) with subsequent delivery before 32 weeks (20.2% vs. 7.1%, p < .001) were higher in the Solomon group. The median birth recipient weight was significantly smaller in the Solomon group (1790 g vs. 1933 g, p = .049). The rate of survival of at least one twin was significantly higher in the Solomon group (98.2% vs. 93.8%, p = .046). The Solomon technique and total laser energy were significant risk factors for pPROM (odds ratio: 2.64, 1.07, 95% CI [1.32, 5.28], [1.01, 1.13], p = .006, p = .014, respectively). These findings suggest that the Solomon technique led to superior survival outcomes but increased risks of placental abruption, pPROM and fetal growth impairment. Total laser energy was associated with the occurrence of pPROM. Close attention to adverse events is required for perinatal management after FLS to treat TTTS using the Solomon technique.

Feasibility and outcomes of fetoscopic endoluminal tracheal occlusion for severe congenital diaphragmatic hernia: A Japanese experience.

AIM: To present the feasibility, safety and outcomes of fetoscopic endoluminal tracheal occlusion (FETO) for the treatment of severe congenital diaphragmatic hernia (CDH). METHODS: This was a single-arm clinical trial of FETO for isolated left-sided CDH with liver herniation and Kitano Grade 3 stomach position (>50% stomach herniation into the right chest). FETO was performed at 27-29 weeks of gestation for cases with observed/expected lung to head ratio (o/e LHR) <25% and at 30-31 weeks for cases with o/e LHR ≥25%. RESULTS: Eleven cases were enrolled between March 2014 and March 2016, and balloon insertion was successful in all cases. The median o/e LHR at entry was 27% (range, 20-33%). The median gestational age at FETO was 30.9 (range, 27.1-31.7) weeks. There were no severe maternal adverse events. One fetus died unexpectedly at 33 weeks of gestation due to cord strangulation by the detached amniotic membrane. There were 3 cases (27%) of preterm premature rupture of membranes. In all 10 cases, balloon removal at 34-35 weeks of gestation was successful. The median gestational age at delivery was 36.5 (range, 34.2-38.3) weeks. The median duration of occlusion and the median interval between balloon insertion and delivery were 26 days (range: 17-49 days) and 43 days (range, 21-66 days), respectively. Both the survival rate at 90 days of age and the rate of survival to discharge were 45% (5/11). CONCLUSION: The FETO is feasible without maternal morbidity in Japan and could be offered to women whose fetuses show severe isolated left-sided CDH to accelerate fetal lung growth.

Neurodevelopmental impairment at 3 years of age after fetoscopic laser surgery for twin-to-twin transfusion syndrome.

BACKGROUND: Data on neurodevelopmental outcomes of children surviving after fetoscopic laser surgery (FLS) for twin-to-twin transfusion syndrome (TTTS) are scarce. METHODS: We retrospectively investigated children surviving after FLS for TTTS at 16 to 26 weeks' gestation between 2003 and 2014. Children were evaluated by standardized neurologic examinations using the Kyoto Scale 2001 at a corrected age of 3 years ± 6 months. Neurodevelopmental impairment (NDI) was defined as cerebral palsy (CP), bilateral blindness, bilateral deafness or a developmental quotient (DQ) < 70 points. Brain magnetic resonance imaging (MRI) was performed at term-equivalent age. RESULTS: A total of 188 children from 110 twin pregnancies were evaluated. NDI was detected in 16/188 (8.5%) children, including six cases of CP (3.2%). No children had bilateral blindness or deafness. An earlier gestational age at delivery was associated with a higher incidence of NDI (P < .001). Abnormal brain MRI findings were detected in 9/16 (56%) of children with NDI, including 6/6 (100%) with CP. CONCLUSION: The incidence of NDI in children following FLS at 3 years old was 8.5%. Prematurity is a strong risk factor for NDI. Brain MRI may predict the development of CP.

Nationwide survey of fetal myelomeningocele in Japan: Background for fetal surgery.

BACKGROUND: Fetal surgery for myelomeningocele (MMC) has yet not been performed in Japan, and the clinical background of fetal MMC in Japan remains poorly described. We examined the prenatal characteristics and perinatal outcomes of fetal MMC to prepare for the introduction of fetal surgery. METHODS: A nationwide questionnaire survey was conducted with regard to fetuses with MMC between January 2012 and December 2014 at perinatal centers in Japan. RESULTS: In 50 tertiary centers, 188 cases of MMC were identified, of which 126 (67%) were isolated cases. Only half of the cases involved referral to tertiary centers with a diagnosis of MMC. The median time point for a prenatal diagnosis was 26 weeks' gestation (range, 12-38 weeks); in 54% of cases the diagnosis occurred after 26 gestational weeks, which is over the limit for fetal surgery for MMC. Furthermore, in 22% of cases the diagnosis was made before 22 gestational weeks, and in three-quarters of these cases termination of pregnancy was selected. No fetal or neonatal deaths were observed in the isolated MMC group. MMC repair, ventriculoperitoneal shunt and clean intermittent catheterization were required after birth in 100%, 73% and 55% of isolated MMC cases, respectively. In total, 96% of the tertiary centers cared for <5 cases of fetal MMC per year. CONCLUSIONS: Gestational age at MMC diagnosis was late mid-gestation, therefore earlier detection is essential when considering fetal treatment of MMC in Japan. Although the survival rate was excellent, in three-quarters of isolated MMC cases ventriculoperitoneal shunt was required. Early detection and centralization of MMC cases at specialized centers should be considered.

Umbilical Cord Ulcer and Intrauterine Death in Fetal Intestinal Atresia.

OBJECTIVE: To examine the incidence of umbilical cord ulcer (UCU) that causes intrauterine fetal death (IUFD) in fetal duodenal or jejunoileal atresia and the association between UCU and bile acid concentrations in amniotic fluid. METHODS: Perinatal outcomes were evaluated in cases of fetal intestinal atresia between 2003 and 2017. A pathological examination of the umbilical cord was performed, and bile acid concentrations in the amniotic fluid were measured. RESULTS: Among the 46 cases included in this study, there were 27 with duodenal atresia and 19 with jejunoileal atresia. There were 4 cases (8.7%) of IUFD and 1 (2.2%) neonatal death with multiple structural anomalies. UCUs were found in 37.5% (15/40) of cases, and severe UCUs with exposed vessels were significantly more common in IUFD (3/4) than in livebirth (0/42) cases (p < 0.01). The incidences of chromosomal abnormality and structural anomalies were not markedly different between livebirth (9/30 and 11/42, respectively) and IUFD (1/3 and 1/4, respectively) cases. Bile acid concentrations in amniotic fluid were significantly higher in cases of UCUs than in those without (p < 0.01). CONCLUSION: UCUs were not rare in fetal intestinal atresia and were associated with high bile acid concentrations in amniotic fluid. UCUs with exposed vessels were associated with IUFD in intestinal atresia.

An investigation on clinical differences between congenital pulmonary airway malformation and bronchial atresia.

BACKGROUND/PURPOSE: Differences in clinical features between congenital pulmonary airway malformation (CPAM) and bronchial atresia (BA) have not yet been clearly described. METHODS: We retrospectively reviewed 112 patients with a pathological diagnosis of CPAM or BA. The clinical parameters were statistically analyzed between these diseases. RESULTS: Seventy-one patients received prenatal diagnosis and 41 received postnatal diagnosis. The percentage of prenatal diagnosis was significantly higher in CPAM patients (84% vs 50%, p < 0.001). Among patients with prenatal diagnosis, the backgrounds were not different between the two diseases except for the number of Caesarean sections (81% vs 9%, p < 0.0001). The numbers of patients that underwent fetal interventions and emergent neonatal surgery were higher in CPAM (51% vs 15%, p < 0.01 and 76% vs 12%, p < 0.0001), although there was no statistical difference in survival rate (86% vs 97%, p = 0.2). In patients receiving postnatal diagnosis, pneumonia was the primary symptom in most BA patients, whereas respiratory distress was the major symptom in patients with CPAM. Age at presentation of the primary symptom was significantly older in BA patients (4.2 years vs 1.2 years, p < 0.005). CONCLUSION: CPAM and BA have distinct clinical features in terms of therapeutic and natural history. Careful imaging evaluation and pathological analysis can lead to an accurate diagnosis of BA. TYPE OF STUDY: Prognostic study. LEVEL OF EVIDENCE: Level II. This study is categorized as a "Prognostic Study" with LEVEL III of Evidence.

Stomach herniation prior to nonreassuring fetal status in a case of fetal gastroschisis.

The neonatal prognosis in gastroschisis is generally good; however, intrauterine fetal death (IUFD) and nonreassuring fetal status (NRFS) sometimes occur in fetal cases of gastroschisis. Previously, we reported stomach herniation as a possible risk factor for IUFD or NRFS in fetuses with gastroschisis. We report a case of fetal gastroschisis showing stomach herniation and increased velocity of the umbilical venous (UV) flow the day before NRFS by electronic fetal monitoring (EFM). A 27-year-old pregnant woman was followed up from 16 weeks' gestation due to fetal gastroschisis. At 33 + 1 weeks' gestation, EFM displayed a reassuring fetal state, while the ultrasound examination revealed fetal stomach herniation and an increased flow velocity of the UV in the abdomen. One day later, EFM displayed NRFS, and the patient underwent emergency caesarean section. This case showed stomach herniation preceding the occurrence of NRFS. Stomach herniation may be a predictor of NRFS in fetuses with gastroschisis.

Fetal lung size after thoracoamniotic shunting reflects survival in primary fetal hydrothorax with hydrops.

AIM: To assess the role of lung size and abnormal Doppler findings in the umbilical artery (UA) in determining the outcomes of fetuses with primary fetal hydrothorax (FHT) associated with hydrops who underwent thoracoamniotic shunting (TAS). METHODS: This was a retrospective study at a single center. We included cases of primary FHT with hydrops who underwent TAS at our hospital between 2004 and 2016. We assessed the relationship between mortality until 28 days after birth and ultrasound findings, including absent or reversed end-diastolic velocity (AREDV) in the UA and the lung-to-thorax transverse area ratio (LTR), before and after TAS. RESULTS: Forty-one cases of primary FHT with hydrops underwent TAS. The median (range) gestational age at TAS was 28.5 (19.3-33.8) weeks. Bilateral pleural effusion was observed in 39 cases (95.1%). Among the 41 cases, 19 (46.4%) survived, 11 (26.8%) died in utero, and 11 (26.8%) died in the neonatal period. AREDV in the UA before and after TAS were not associated with mortality (P = 0.32 and 0.47, respectively). The odds ratio for mortality in LTR 0.2-0.3 before TAS was 0.62 (vs LTR < 0.2, P = 0.45) and that in LTR 0.2-0.3 and > 0.3 after TAS were 0.27 and 0.06, respectively (vs LTR < 0.2, P for trend <0.01). CONCLUSION: A larger LTR after TAS was significantly associated with a better prognosis in hydropic primary FHT. The fetal lung size after the procedure may be a prognostic factor of primary FHT.

Fetoscopic laser photocoagulation for twin-twin transfusion syndrome.

The aim of this study was to review fetoscopic laser photocoagulation (FLP), which ablates placental vascular anastomoses to treat twin-twin transfusion syndrome (TTTS). A review of studies reporting on the procedures, outcomes, complications and nonconventional applications of FLP for TTTS was conducted. FLP has been established as the primary treatment for monochorionic twin pregnancy associated with TTTS at 16-26 weeks. FLP is the only therapy that directly addresses the underlying pathophysiology. The recent technique modification of FLP, referred to as the 'Solomon technique', induces selective coagulation to connect the anastomoses ablation sites and has been introduced to reduce residual anastomoses. The perinatal survival following FLP improved significantly with advances in the technique after its introduction. The recent survival rates of both twins and at least one twin are 70% and more than 90%, respectively. However, there is still an 11-14% risk of long-term neurodevelopment impairment. The premature rupture of membranes that leads to preterm labor is a common complication after FLP. FLP is a valuable treatment option for feto-fetal transfusion syndrome in triplets and for TTTS after 26 weeks. FLP for selective intrauterine growth restriction may be potentially beneficial when accompanied by abnormal Doppler findings and oligohydramnios. FLP is the optimal treatment option for TTTS at 16-26 weeks of gestation. FLP appears to be applicable in triplets, TTTS after 26 weeks and cases of selective intrauterine growth restriction with abnormal Doppler findings and oligohydramnios. FLP is the most common and successful fetal intervention. Improvement in the neurodevelopmental outcomes after FLP is a future focus.

Fetal Thoracoamniotic Shunting in a Case of Congenital Pulmonary Airway Malformations with Hydrops Fetalis.

Aim We report a case of congenital pulmonary airway malformation (CPAM) with hydrops in which the fetus underwent thoracoamniotic shunting. Case Report A 40-year-old (G1P1) woman was diagnosed with a macrocystic CPAM. Thoracoamniotic shunting was performed at 19 weeks of gestation but not well drained and was successfully performed again at 23 weeks. However, the CPAM volume ratio, abdominal circumference, and amniotic fluid index started increasing from 28 weeks and hydrops worsened. The insufficient shunting and the fetal cardiac failure had to be considered. At 32 weeks, a male infant with general edema and massive ascites was born weighing 3,362 g (+4.79 SD) with Apgar scores of 2 and 4. The infant was intubated and high-frequency oscillation and nitric oxide therapies were instituted. The resection of CPAM was performed on day 2. Nasal continuous positive airway pressure was instituted on day 16. The infant was discharged and prescribed with home oxygen therapy (HOT) on day 65. The infant was able to leave the HOT at 30 months and is currently 34 months of age in good condition. Conclusion Fetal thoracoamniotic shunting may be life-saving in CPAM complicated by hydrops and that this treatment might be sufficient to cure the child.

Chronic histiocytic intervillositis in three consecutive pregnancies in a single patient: Differing clinical results and pathology according to treatment used.

Chronic histiocytic intervillositis (CHI) is an extremely rare pathological condition but is strongly associated with severe obstetric complications and has a high recurrence rate. The management of this condition has not yet been established. We describe herein the occurrence of CHI in the late second-third trimester in each of three consecutive pregnancies in a single patient with four previous consecutive early miscarriages. In this patient, each of the three complicated pregnancies was managed with one of the following, respectively: low-dose aspirin; heparin plus low-dose aspirin; and prednisolone plus low-dose aspirin. CHI was histologically confirmed in all three pregnancies, but the clinical results and pathology (e.g. extent of histiocytic infiltration) in each pregnancy clearly differed with treatment. Both combination treatments eventuated in a live birth. Immunosuppressive therapy seemed to produce better clinical results by restricting the extent of the affected areas. The elevated alkaline phosphatase associated with the CHI was assumed to have no clinical prognostic value.

Fetoscopic laser photocoagulation for amniotic fluid discordance bordering on twin-twin transfusion syndrome: Feasibility, perinatal and long-term outcomes.

AIM: This study investigated the feasibility and safety of fetoscopic laser photocoagulation (FLP) for amniotic fluid discordance (AFD) bordering on twin-twin transfusion syndrome (TTTS) with an absent or reverse end-diastolic velocity (AREDV) in the umbilical artery (UA), and evaluated the perinatal and long-term outcomes. METHODS: A prospective intervention study was performed between 20 + 0 and 25 + 6 weeks of gestation (UMIN000004165). AFD bordering on TTTS was defined as maximum vertical pocket (MVP) of amniotic fluid in one twin's sac ≤3 cm and amniotic fluid MVP in the other twin's sac ≥7 cm excluding TTTS. Neurodevelopmental outcome was evaluated at 6 months and at 3 years of age. RESULTS: Eleven women were treated without complications between September 2010 and July 2011. In all cases amnioinfusion was required, with a median surgical time of 70 min. There were nine cases of selective intrauterine growth restriction in which the growth discordant rate was >25%. The survival rates of the donor and recipient twins were 27.3% (3/11) and 100% (11/11), respectively. None of the surviving donor twins and two of the 11 recipient twins had hemiplegia at 6 months of age. One additional recipient twin had developmental delay at 3 years of age. CONCLUSIONS: Fetoscopic laser photocoagulation for AFD bordering on TTTS with AREDV in the UA was feasible without complications but frequently resulted in donor twin death and a high survival rate of the recipient twin, albeit with neurodevelopmental abnormalities in some cases. FLP does not seem to be a promising treatment option for AFD bordering on TTTS.

Functional total anomalous pulmonary venous connection via levoatriocardinal vein.

We report a fetal case of double outlet right ventricle, mitral atresia, and intact atrial septum. Although the pulmonary veins were connected to the left atrium, pulmonary venous blood drained into the right superior vena cava via the stenotic levoatriocardinal vein (LACV), which resulted in a circulation resembling total anomalous pulmonary venous connection (TAPVC) with pulmonary venous obstruction. Since the pulmonary veins were connected to both the stenotic LACV and the "dead-end" left atrium, the pulmonary venous flow had a to-and-fro pattern along with atrial relaxation and contraction. Postnatal echocardiography and computed tomography confirmed the diagnosis of normally connected but anomalously draining pulmonary veins via the LACV. Surgical creation of an atrial septal defect on the day of birth successfully relieved pulmonary venous obstruction. Normally connected but anomalously draining pulmonary veins via the LACV should be considered for TAPVC differential diagnosis in fetuses with a left-side heart obstruction.