Outcomes of late open fetal surgery for intrauterine spina bifida repair after 26 weeks. Should we extend the Management of Myelomeningocele Study time window?

OBJECTIVE: To evaluate maternal and perinatal outcomes of late open fetal repair for open spina bifida (OSB) between 26+0 -27+6  weeks. METHODS: A cohort of fetuses with OSB who underwent open surgery in two fetal surgery centers (Argentina and Mexico). Two groups were defined based on the gestational age (GA) at intervention: Management of Myelomeningocele Study (MOMS) time window group: GA 19+0 -25+6 , and late intervention group: GA 26+0 -27+6 . RESULTS: Intrauterine OSB repair was successfully performed in 140 cases, either before (n = 57) or after (n = 83) 26 weeks, at on average 25+0 (22+6 -25+6 ) and 26+5 (26+0 -27+6 ) weeks + days, respectively. There were no significant differences in the rate of premature rupture of membranes, chorioamnionitis, oligohydramnios, preterm delivery, perinatal death and maternal complications. The late intervention group showed a significantly lower surgical times (112.6 vs. 124.2 min, p = 0.01), lower interval between fetal surgery and delivery (7.9 vs. 9.2 weeks, p < 0.01) and similar rate of hydrocephalus requiring treatment (30.6% vs. 23.3%, p = 0.44) than the MOMS time window group. CONCLUSION: Late fetal surgery for OSB repair between 26+0 -27+6  weeks is feasible and was associated with similar outcomes than that performed before 26 weeks. These findings may allow an extension of the proposed time window for cases with late diagnosis or referral.

Cirugía fetal de Mielomeningocele: evolución obstétrica y resultados perinatales a corto plazo de una cohorte de 21 casos / Fetal surgery for myelomeningocele: obstetric evolution and short-term perinatal results of a cohort of 21 cases

Objetivo: Describir la técnica quirúrgica, evolución obstétrica y resultados perinatales de una cohorte de pacientes sometidas a cirugía intrauterina para reparación de una disrafia fetal abierta (DFA). Métodos: Análisis prospectivo de 21 casos consecutivos de DFA con diagnóstico y tratamiento quirúrgico prenatal en nuestra institución entre 2015 y 2017. La técnica de la cirugía fetal (QF) fue similar a la descripta en el estudio MOMS, excepto que se utilizó histerotomía con asa bipolar. Los cuidados postoperatorios, nacimiento y cuidados neonatales se realizaron en la misma institución. Se analizaron complicaciones perioperatorias de la QF, la evolución obstétrica y los resultados perinatales. Resultados: La QF se realizó a una edad gestacional media de 25.8 semanas (24.1-27.6). La edad gestacional media al nacer fue 34.2 semanas (29.2-37.1). El tiempo quirúrgico medio fue 138 min (101-187) con tendencia descendente y el tiempo de internación medio, 7.1 días (4-32). El 52% (11/21) de las pacientes experimentó rotura prematura de membranas (RPM). Ninguna paciente requirió transfusiones postcesárea. No hubo casos de desprendimiento placentario, rotura uterina ni muertes maternas. La cicatrización de la histerorafia fue normal en 95% de las pacientes. La sobrevida perinatal fue del 95% (20/21, una muerte intrauterina secundaria a banda amniótica). La necesidad de cierre cutáneo postnatal (CCP) fue del 5%. Ninguno de los casos (20) reparados con tejidos fetales requirió CCP. El 70% (14/20) de los pacientes no requirió ningún tratamiento para hidrocefalia. Cuatro pacientes (20%) requirieron una derivación ventriculoperitoneal (DVP) y dos más fueron sometidos adicionalmente, a una tercer ventriculostomía endoscópica (ETV) (10%). El nivel funcional motor neonatal (NFN) fue mejor que el nivel anatómico prenatal (NAP) en 45% (9/20), igual en 50% (10/20) y peor en 5% (1/20). Conclusiones: El presente estudio confirma que la cirugía fetal de disrafias abiertas se asocia a un mayor riesgo de parto prematuro y rotura prematura de membranas, pero reduce significativamente la necesidad de tratamiento postnatal de hidrocefalia y mejora la función motora a corto plazo. Nuestros resultados son similares a los publicados en el ensayo aleatorizado MOMS.

Objective: To describe the surgical technique, obstetrical evolution and perinatal outcomes of a cohort of fetuses undergoing intrauterine surgery to repair open spina bifida (OSB). Methods: We performed a prospective analysis of 21 consecutive fetuses with OSB at our institution between 2015 and 2017. The surgical technique was similar to that described in the MOMS trial, except that the hysterotomy was performed using a bipolar dissector. Post-operative maternal and infant care both were provided at the same institution. There were no losses to follow-up. Surgical and obstetrical complications and perinatal outcomes were analyzed. Results: Fetal surgery was performed at a mean gestation of 25.8 weeks (24.1-27.6). The mean gestational age at birth was 34.2 weeks (29.2-37.1). The mean surgical time was 138 min (101-187), the duration of surgery trending downward over time; while the average admission length was 7.1 days (4-32). Fifty two percent (11/21) of the patients experienced pre-term premature rupture of membranes (pPROM). No patient required any post-cesarean transfusions. There were no instances of placental abruption, uterine rupture, or maternal death. Uterine scar healing was normal in 95% of the patients. All but one of the 21 fetuses (95%) survived, the one fetal death due to an amniotic band. The need for postnatal skin closure (PSC) was 5%, with one of 20 repaired prenatally with a synthetic skin patch. No case (19) repaired with fetal tissues required PSC. Seventy percent (14/20) of the infants required no further treatment for hydrocephalus over their first year of life; four patients (20%) required a ventriculoperitoneal shunt (VPS), while two others underwent an endoscopic third ventriculostomy (ETV) (10%). Neonatal motor function (NMF) was better than the prenatal anatomical level (PAL) in 45% (9/20), equal in 50% (10/20), and worse in 5% (1/20). Conclusions: Our data confirm that fetal surgery for OSB is associated with an increased risk of preterm delivery and PROM, but significantly reduces the need for postnatal treatment of hydrocephalus and improves short-term motor outcomes. Our results are similar to those published for the randomized MOMS trial.

[Fetal surgery for myelomeningocele: Obstetric evolution and short-term perinatal outcomes of a cohort of 21 cases]. / Cirugía fetal de mielomeningocele: Evolución obstétrica y resultados perinatales a corto plazo de una cohorte de 21 casos.

OBJECTIVE: The aim of this study is to describe the surgical technique, obstetrical evolution, and perinatal outcomes of a cohort of fetuses undergoing intrauterine surgery to repair open spina bifida (OSB). METHODS: We performed a prospective analysis of 21 consecutive fetuses with OSB at our institution between 2015 and 2017. The surgical technique was similar to that described in the management of myelomeningocele study (MOMS) (Funded by the National Institutes of Health; ClinicalTrials.gov number, NCT00060606) trial, except that the hysterotomy was performed using a bipolar dissector. Postoperative maternal and infant care both were provided at the same institution. There were no losses to follow-up. Surgical and obstetrical complications and perinatal outcomes were analyzed. RESULTS: Fetal surgery was performed at a mean gestation of 25.8 weeks (24.1-27.6). The mean gestational age at birth was 34.2 weeks (29.2-37.1). The mean surgical time was 138 min (101-187), the duration of surgery trending downward over time; while the average admission length was 7.1 days (4-32). Fifty-two percent (11/21) of the patients experienced preterm premature rupture of membranes. No patient required any postcesarean transfusions. There were no instances of placental abruption, uterine rupture, or maternal death. Uterine scar healing was normal in 95% of the patients. All but one of the 21 fetuses (95%) survived; the one fetal death was due to an amniotic band. The need for postnatal skin closure was 5%, with one of 20 repaired prenatally with a synthetic skin patch. No case (19) repaired with fetal tissues required postnatal skin closure. Seventy percent (14/20) of the infants required no further treatment for hydrocephalus over their first year of life; four patients (20%) required a ventriculoperitoneal shunt, while two others underwent an endoscopic third ventriculostomy (10%). Neonatal motor function was better than the prenatal anatomical level in 45% (9/20), equal in 50% (10/20), and worse in 5% (1/20). CONCLUSIONS: Our data confirm that fetal surgery for OSB is associated with an increased risk of preterm delivery and premature rupture of membranes, but significantly reduces the need for postnatal treatment of hydrocephalus and improves short-term motor outcomes. Our results are similar to those published for the randomized MOMS trial.

Successful orthotopic liver transplantation and delayed delivery of a healthy newborn in a woman with fulminant hepatic failure during the second trimester of pregnancy.

Severe liver dysfunction during pregnancy implies a serious risk for both mother and fetus, and represents a technical and ethical challenge for treating physicians. We report a case of a previously healthy 32-year old woman who was admitted to our hospital with idiopathic fulminant hepatic failure and underwent successful orthotopic liver transplantation (OLT) at gestation week 21. Patient's and fetus' immediate postoperative course were relatively uneventful until week six after OLT, when the mother developed oligohydramnios and preeclampsia. At pregnancy week 27, after inducing baby's lung maturation, a cesarean section was performed with the delivery of an otherwise healthy girl. After 3 years of follow-up, mother and child are leading normal lives with no complications related either to pregnancy or to OLT. We describe the case of a successful emergency liver transplant in a woman during the second trimester of pregnancy, demonstrating that OLT can be a viable option to preserve the life of the mother and an otherwise unviable fetus. Intrauterine baby's growths until the attainment of a viable gestational age was feasible despite the mother's fulminant hepatic failure and liver transplant surgery.