Management of monochorionic twins discordant for structural fetal anomalies.

OBJECTIVE: To review the perinatal management and outcomes of monochorionic twin pregnancies (MC) discordant for congenital anomalies (DCA). METHODS: Retrospective, study of all MC DCA cases referred to our tertiary referral center from 1997 to 2018. We excluded cases complicated with twin-to-twin transfusion syndrome, twin anemia-polycythemia sequence, twin reversed arterial perfusion sequence or selective intra-uterine growth restriction. Patients were counseled about the possibility of expectant (EM) or interventional management (selective feticide [SF] or termination of the entire pregnancy [TOP]). RESULTS: One hundred eight of 4157 (2.6%) MC pregnancies were discordant for anomaly. Fifty two of 108 n(48.1%) underwent SF at a mean gestational age of 31.4 ± 5.9 weeks while 52/108(48.1%) opted for EM. Livebirth rate of the healthy co-twin was similar between the two groups (SF: 88.5% vs EM: 82.7%, P = .87). In the SF group, six healthy co-twins (6/52, 11.5%) died 5.3 ± 3.1 days after SF of the abnormal co-twin. In the EM group, in-utero demise of the abnormal twin occurred in 9 of 52 (17.3%) of the cases and was followed by the spontaneous demise of the healthy co-twin in 4 of 9 (44.4%) of these cases. CONCLUSION: Selective feticide does not seem to significantly alter survival of the healthy co-twin compared to EM.

Meconium peritonitis in both fetuses with early twin-to-twin transfusion syndrome.

Twin-to-twin transfusion syndrome (TTTS) is due to unbalanced inter-twin bloodflow through placental vascular anastomoses. We present a TTTS case treated with fetoscopic laser photocoagulation (FLP) that was complicated by perinatal meconium peritonitis in both twins. Ten weeks following laser treatment, the two fetuses showed intra-abdominal hyperechogenicity and ascites. After birth, the two newborns were surgically managed for peritonitis. We discuss the pathogenesis of this double insult. The present case highlights the role of end-circulation bowel thrombi as the potential cause of subsequent intestinal perforation.

Long-term developmental follow-up of infants who participated in a randomized clinical trial of amniocentesis vs laser photocoagulation for the treatment of twin-to-twin transfusion syndrome.

OBJECTIVE: We sought to assess long-term neurodevelopment of children who were treated prenatally as part of the Eurofoetus randomized controlled trial. STUDY DESIGN: The study population was composed of 128 cases of twin-to-twin transfusion syndrome (TTTS) included and followed up in France. Survivors were evaluated by standardized neurological examination and by Ages and Stages Questionnaires (ASQ). Primary outcome was a composite of death and major neurological impairment. RESULTS: A total of 120 children (47%) were alive at the age of 6 months and were followed up to the age of 6 years. At the time of diagnosis, only treatment and Quintero stage were predictors of a poor outcome (hazard ratio, 0.61; 95% confidence interval, 0.41-0.90; P = .01 and hazard ratio, 3.23; 95% confidence interval, 2.19-4.76; P < .001, respectively). Children treated by fetoscopic selective laser coagulation (FSLC) had higher ASQ scores at the end of follow-up (P = .04). CONCLUSION: FSLC was significantly associated with a reduction of the risk of death or long-term major neurological impairment at the time of diagnosis and treatment.

Pseudoamniotic band syndrome: a rare complication of monochorionic twins with fetofetal transfusion syndrome treated by laser coagulation.

OBJECTIVE: The purpose of this study was to assess the incidence and risk factors of limb constriction defects that are related to pseudoamniotic band syndrome (PABS) after selective fetoscopic laser surgery (FLS) in fetofetal transfusion syndrome (FFTS). STUDY DESIGN: All consecutive cases of FFTS that were treated by selective FLS between 1999 and 2006 were examined prospectively for PABS at the time of delivery. Incidence and characteristics of PABS were reported. Univariate analysis was conducted to look for potential risk factors of developing PABS. RESULTS: The 438 consecutive FFTS cases were treated at 15-26 weeks of gestation; PABS developed in 8 cases (1.8 %). The affected twin was always the former recipient. The diagnosis was made prenatally in 2 of 8 cases (25%). All cases survived the perinatal period. PABS affected fetal leg, arm, and foot in 3, 4, and 1 cases, respectively. In 5 (62.5%) and 7 (87.5%) cases, PABS occurred after premature rupture of membranes and intrauterine death of the donor, respectively. In 4 cases (50%), there was both premature rupture of membranes (PROM) and intrauterine fetal death; in 3 cases (37.5%), there was intrauterine fetal death alone, and in 1 case (12.5%), there was PROM alone. In the remaining 430 cases, PROM occurred in 62 cases (14.4%) and 66 cases (15.3%) within and after 3 weeks after surgery, respectively. PROM was significantly more frequent within the group that was complicated with PABS than within the rest of the cohort (P = .05). No maternal, fetal, or perioperative risk factor could be identified. CONCLUSION: Awareness and targeted serial ultrasound evaluation in this high-risk group may improve prenatal diagnosis, counseling, and management of PABS after FLS.