Randomized controlled trial of twin-twin transfusion syndrome laser surgery: the sequential trial.

BACKGROUND: Intraoperative blood transfer between twins during laser surgery for twin-twin transfusion syndrome can vary by surgical technique and has been proposed to explain differences in donor twin survival. OBJECTIVE: This trial compared donor twin survival with 2 laser techniques: the sequential technique, in which the arteriovenous communications from the volume-depleted donor to the volume-overloaded recipient are laser-occluded before those from recipient to donor, and the selective technique, in which the occlusion of the vascular communications is performed in no particular order. STUDY DESIGN: A single-center, open-label, randomized controlled trial was conducted in which twin-twin transfusion syndrome patients were randomized to sequential vs selective laser surgery. Nested within the trial, a second trial randomized patients with superficial anastomoses (arterioarterial and venovenous) to ablation of these connections first (before ablating the arteriovenous anastomoses) vs last. The primary outcome measure was donor twin survival at birth. RESULTS: A total of 642 patients were randomized. Overall donor twin survival was similar between the 2 groups (274 of 320 [85.6%] vs 271 of 322 [84.2%]; odds ratio, 1.12 [95% confidence interval, 0.73-1.73]; P=.605). Superficial anastomoses occurred in 177 of 642 cases (27.6%). Donor survival was lower in the superficial anastomosis group vs those with only arteriovenous communications (125 of 177 [70.6%] vs 420 of 465 [90.3%]; adjusted odds ratio, 0.33 [95% confidence interval, 0.20-0.54]; P<.001). In cases with superficial anastomoses, donor survival was independent of the timing of ablation or surgical technique. The postoperative mean middle cerebral artery peak systolic velocity was lower in the sequential vs selective group (1.00±0.30 vs 1.06±0.30 multiples of the median; P=.003). Post hoc analyses showed 2 factors that were associated with poor overall donor twin survival: the presence or absence of donor twin preoperative critical abnormal Doppler parameters and the presence or absence of arterioarterial anastomoses. Depending on these factors, 4 categories of patients resulted: (1) Category 1 (347 of 642 [54%]), no donor twin critical abnormal Doppler + no arterioarterial anastomoses: donor twin survival was 91.2% in the sequential and 93.8% in the selective groups; (2) Category 2 (143 of 642 [22%]), critical abnormal Doppler present + no arterioarterial anastomoses: donor survival was 89.9% vs 75.7%; (3) Category 3 (73 of 642 [11%]), no critical abnormal Doppler + arterioarterial anastomoses present: donor survival was 94.7% vs 74.3%; and (4) Category 4 (79 of 642 [12%]), critical abnormal Doppler present + arterioarterial anastomoses present: donor survival was 47.6% vs 64.9%. CONCLUSION: Donor twin survival did not differ between the sequential vs selective laser techniques and did not differ if superficial anastomoses were ablated first vs last. The donor twin's postoperative middle cerebral artery peak systolic velocity was improved with the sequential vs the selective approach. Post hoc analyses suggest that donor twin survival may be associated with the choice of laser technique according to high-risk factors. Further study is needed to determine whether using these categories to guide the choice of surgical technique will improve outcomes.

Temporal ultrasound findings in the first trimester of a dual fetal demise occurring in a monochorionic diamniotic twin gestation.

Single fetal demise in monochorionic gestations in the 2nd and 3rd trimester is associated with adverse outcomes for the co-twin. We present a case of single demise in a monochorionic gestation in the 1st trimester with evidence of subsequent hemodynamic aberrations in the co-twin, supportive of feto-fetal hemorrhage occurring early in gestation.

Third-trimester fetoscopic ablation therapy for types II and III vasa previa.

BACKGROUND: Vasa previa is an obstetrical condition in which fetal vessels located near the cervix traverse the fetal membranes unprotected by underlying placenta. Type I vasa previa arises directly from a velamentous cord root, whereas types II and III arise from an accessory lobe or a distal lobe of the same placenta, respectively. Fetoscopic laser ablation for types II and III vasa previa is a novel therapeutic option with benefits that include surgical resolution of the vasa previa, avoidance of prolonged hospitalization, and opportunity for a term vaginal delivery. The potential risks of fetoscopy can be mitigated by delaying laser surgery until a gestational age of 31 to 33 weeks, immediately before anticipated hospitalized surveillance. OBJECTIVE: This study aimed to assess feasibility and outcomes of types II and III vasa previa patients treated via fetoscopic laser ablation in the third trimester. STUDY DESIGN: This is a retrospective study of singleton pregnancies with types II and III vasa previa treated with fetoscopic laser ablation at a gestational age ≥31 weeks at a single center between 2006 and 2022. Pregnancy and newborn outcomes were assessed. Continuous variables are expressed as mean±standard deviation. RESULTS: Of 84 patients referred for vasa previa, 57 did not undergo laser ablation: 19 either had no or resolved vasa previa, 25 had type I vasa previa (laser-contraindicated), and 13 had type II or III vasa previa but declined laser treatment. Of the remaining 27 patients who underwent laser ablation, 7 were excluded (laser performed at <31 weeks and/or twins), leaving 20 study patients. The mean gestational age at fetoscopic laser ablation was 32.0±0.6 weeks, and total operative time was 62.1±19.6 minutes. There were no perioperative complications. All patients had successful occlusion of the vasa previa vessels (1 required a second procedure). All patients were subsequently managed as outpatients. The mean gestational age at delivery was 37.2±1.8 weeks, the mean birthweight was 2795±465 g, and 70% delivered vaginally. Neonatal intensive care unit admission occurred in 3 cases: 1 for respiratory distress syndrome and 2 for hyperbilirubinemia requiring phototherapy. There were no cases of neonatal transfusion, intraventricular hemorrhage, sepsis, patent ductus arteriosus, or death. CONCLUSION: Laser ablation for types II and III vasa previa at 31 to 33 gestational weeks was technically achievable and resulted in favorable outcomes.

Diagnosis and Management of 2 Cases of Spontaneous Septostomy: Monochorionic/Dichorionic Hybrid Twin Gestation and Dichorionic Triamniotic Triplet Gestation.

INTRODUCTION: Spontaneous septostomy is a rare complication of multiple gestations. Related complications include cord entanglement and preterm delivery. Limited data exist to guide the management of these high-risk patients. The majority of spontaneous septostomy cases have been reported in monochorionic diamniotic twins. We present 2 cases of spontaneous septostomy occurring in a monochorionic/dichorionic hybrid twin gestation (chorionicity transitions from dichorionicity to monochorionicity within the placenta) and in a dichorionic triamniotic triplet gestation. CASE PRESENTATION: Case 1 was a monochorionic/dichorionic hybrid twin gestation with a septostomy complicated by fetal parts of one twin protruding into the co-twin's sac as well as symptomatic polyhydramnios. Fetal magnetic resonance imaging confirmed the septostomy. Case 2 was a dichorionic triamniotic triplet gestation with septostomy and cord entanglement. Both patients were managed akin to a pseudo-monoamniotic gestation with serial ultrasound surveillance and eventual inpatient admission for heightened fetal monitoring. Case 1 underwent elective scheduled cesarean delivery at 33 weeks, and case 2 underwent emergent cesarean delivery for fetal heart rate decelerations at 28 weeks. CONCLUSION: With a high degree of clinical suspicion, spontaneous septostomy can be diagnosed in uncommon settings such as hybrid twin gestations and higher order multiples. Management of such patients is individualized and may include a combination of heightened outpatient and inpatient surveillance.

Subclassification of fetal growth restriction type IIa vs type IIb applied to twin-twin transfusion syndrome.

BACKGROUND: Subclassification of monochorionic twins with selective fetal growth restriction type II into IIa vs IIb has been proposed because of differing neonatal survival outcomes of the fetus with growth restriction after laser surgery based on preoperative Doppler findings in the middle cerebral artery and ductus venosus. There is substantial clinical overlap between selective fetal growth restriction and twin-twin transfusion syndrome. OBJECTIVE: This study aimed to compare donor twin neonatal survival after laser surgery in cases of twin-twin transfusion syndrome with concomitant donor fetal growth restriction type IIa vs IIb. STUDY DESIGN: This was a retrospective study of monochorionic multifetal pregnancies treated with laser surgery for stage III twin-twin transfusion syndrome and concomitant donor twin fetal growth restriction type II at a referral center from 2006 to 2021. Donor fetal growth restriction type II was defined as having an estimated fetal weight <10th percentile with persistent absent and/or reversed end-diastolic velocity in the umbilical artery. Moreover, patients were subclassified as type IIa (having normal middle cerebral artery peak systolic velocities and ductus venosus Doppler waveforms) vs type IIb (having middle cerebral artery peak systolic velocities ≥1.5 multiples of the median and/or ductus venosus with persistent absent or reversed atrial systolic flow). This study compared 30-day neonatal survival of the donor twin by fetal growth restriction type IIa vs IIb using logistic regression to adjust for relevant preoperative covariates (P<.10 in bivariate analysis). RESULTS: Of 919 patients who underwent laser surgery for twin-twin transfusion syndrome, 262 had sstage III donor or donor and recipient twin-twin transfusion syndrome; of these patients, 189 (20.6%) had concomitant donor fetal growth restriction type II. Moreover, 12 patients met the exclusion criteria, yielding 177 patients (19.3%) who composed the study cohort. Patients were subclassified as donor fetal growth restriction type IIa (146 [82%]) vs type IIb (31 [18%]). Donor neonatal survival for fetal growth restriction type IIa vs IIb was 71.2% vs 41.9% (P=.003). Recipient neonatal survival did not differ between the 2 types (P=1.000). Patients classified with twin-twin transfusion syndrome and concomitant donor fetal growth restriction type IIb were 66% less likely to have neonatal survival of the donor after laser surgery (adjusted odds ratio, 0.34; 95% confidence interval, 0.15-0.80; P=.0127). The logistic regression model was adjusted for gestational age at the procedure, estimated fetal weight percent discordance, and nulliparity. The c-statistic was 0.702. CONCLUSION: For patients with stage III twin-twin transfusion syndrome and concurrent donor fetal growth restriction with persistent absent or reversed end-diastolic velocity in the umbilical artery (ie, fetal growth restriction type II), subclassification into fetal growth restriction type IIb based on elevated middle cerebral artery peak systolic velocity and/or abnormal ductus venosus flow in the donor conveyed poorer prognosis. Although donor neonatal survival after laser surgery was lower for patients with stage III twin-twin transfusion syndrome with donor fetal growth restriction type IIb than patients with stage III twin-twin transfusion syndrome with donor fetal growth restriction with type IIa, laser surgery for fetal growth restriction type IIb in the setting of twin-twin transfusion syndrome (as opposed to pure selective fetal growth restriction type IIb) still allows for the possibility of dual survivorship and should be offered with shared decision-making when counseling patients on management options.

Multimodal Management of Cervical Insufficiency Complicated by Intra-amniotic Candida albicans Infection.

INTRODUCTION: Bacteria are the most common pathogens implicated in ascending infections in patients with cervical insufficiency. However, Candida albicans is a rare and serious cause of intra-amniotic infection that should be considered on the differential diagnosis. Upon diagnosis following cerclage placement, patients are generally advised to undergo immediate cerclage removal and discontinuation of the pregnancy due to the high risk of maternal and fetal morbidity. However, some patients decline and instead elect to continue the pregnancy with or without treatment. Limited data exist to guide management of these high-risk patients. CASE PRESENTATION: We describe a case of previable intra-amniotic C. albicans infection diagnosed following physical examination-indicated cerclage placement. The patient declined pregnancy termination and subsequently underwent systemic antifungal therapy as well as serial intra-amniotic fluconazole instillations. Fetal blood sampling confirmed transplacental transfer of maternal systemic antifungal therapy. The fetus delivered preterm and without evidence of fungemia, despite persistently positive amniotic fluid cultures. CONCLUSION: In a well-counseled patient with culture-proven intra-amniotic C. albicans infection declining termination of pregnancy, multimodal antifungal therapy in the form of systemic and intra-amniotic fluconazole administration may prevent subsequent fetal or neonatal fungemia and improve postnatal outcomes. KEY POINTS: · Candida is an uncommon cause of intra-amniotic infection in the setting of cervical insufficiency.. · Multimodal antifungal therapy may prevent fetal fungemia related to intra-amniotic Candida infection.. · Fetal blood sampling confirmed transplacental passage of fluconazole after maternal administration..

Advances in Fetal Surgical Repair of Open Spina Bifida.

Spina bifida remains a common congenital anomaly of the central nervous system despite national fortification of foods with folic acid, with a prevalence of 2-4 per 10,000 live births. Prenatal screening for the early detection of this condition provides patients with the opportunity to consider various management options during pregnancy. Prenatal repair of open spina bifida, traditionally performed by the open maternal-fetal surgical approach through hysterotomy, has been shown to improve outcomes for the child, including decreased need for cerebrospinal fluid diversion surgery and improved lower neuromotor function. However, the open maternal-fetal surgical approach is associated with relatively increased risk for the patient and the overall pregnancy, as well as future pregnancies. Recent advances in minimally invasive prenatal repair of open spina bifida through fetoscopy have shown similar benefits for the child but relatively improved outcomes for the pregnant patient and future childbearing.

Percutaneous/mini-laparotomy fetoscopic repair of open spina bifida: a novel surgical technique.

Open spina bifida is the most common congenital anomaly of the central nervous system compatible with life. Prenatal repair of open spina bifida via open maternal-fetal surgery has been shown to improve postnatal neurologic outcomes, including reducing the need for ventriculoperitoneal shunting and improving lower neuromotor function. Fetoscopic repair of open spina bifida minimizes the maternal risks while providing similar neurosurgical outcomes to the fetus. The following 2 fetoscopic techniques are currently in use: (1) the laparotomy-assisted approach, and (2) the percutaneous approach. The laparotomy-assisted fetoscopic technique appears to be associated with a lesser risk of preterm birth than the percutaneous approach. However, the percutaneous approach avoids laparotomy and uterine exteriorization and is associated with lesser anesthesia risk and improved maternal postsurgical recovery. The purpose of this article was to describe our experience with a modified surgical approach, which we call percutaneous/mini-laparotomy fetoscopy, in which access to the uterus for one of the ports is done via a mini-laparotomy, whereas the other ports are inserted percutaneously. This technique draws on the benefits of both the laparotomy-assisted and the percutaneous techniques while minimizing their drawbacks. This surgical approach may prove invaluable in the prenatal repair of open spina bifida and other complex fetal surgical procedures.

Outcomes of laser surgery for stage I twin-twin transfusion syndrome.

OBJECTIVE: A recent randomized controlled trial (RCT) demonstrated no difference in 6 month survival in expectantly managed stage I twin-twin transfusion syndrome (TTTS) patients and those undergoing immediate laser surgery. We aimed to describe outcomes following immediate laser surgery at a single fetal surgery center. METHODS: A retrospective study of monochorionic diamniotic twins diagnosed with stage I TTTS who underwent laser surgery between 16 and 26 gestational weeks from 2006 to 2019. The primary outcome was 6 month survivorship. Intact survival was also assessed. Secondarily, outcomes were compared to the RCT expectant management group. RESULTS: Of 126 consecutive stage I TTTS patients, 114 (90.5%) met inclusion criteria. Median (range) gestational age at delivery was 34.1 (20.6-39.4) weeks. At 6 months, the proportion of patients with at-least-one survivor in the single-center-laser cohort was 97.4%, with 88.6% dual survivorship. Neurological morbidity outcomes were available in 110 pregnancies (220 fetuses). Severe neurological morbidity occurred in 2.7% (6/220), and 6 month survival without severe neurological morbidity was 90.0%. Outcomes compared favorably with the RCT expectant management group. CONCLUSIONS: Given favorable survival and neurological outcomes, laser surgery is a reasonable treatment option for stage I TTTS at experienced fetal surgery centers. Further study is warranted to optimize treatment strategies.

Selective intrauterine growth restriction (SIUGR) type II: proposed subclassification to guide surgical management.

BACKGROUND: Optimal surgical management of monochorionic diamniotic twins complicated by selective intrauterine growth restriction (SIUGR) type II is unknown. Surgical management may involve selective laser photocoagulation of communicating vessels (SLPCV), which offers the possibility of dual twin survivors versus umbilical cord occlusion (UCO) of the SIUGR twin. OBJECTIVE: To identify patient characteristics associated with SIUGR twin survival for those undergoing SLPCV. STUDY DESIGN: All patients studied were those who underwent fetal treatment for SIUGR type II at our center from 2006-2018. SIUGR type II was defined as an estimated fetal weight <10th percentile with persistent absent and/or reversed end diastolic flow in the umbilical artery of the SIUGR twin, in the absence of twin-twin transfusion syndrome. Patients were offered SLPCV versus UCO, and those undergoing SLPCV, patient characteristics associated with 30-day survival of the SIUGR twin were examined using bivariate analysis and multiple logistic regression models. RESULTS: Fifty-four consecutive SIUGR type II patients were treated, 45 via SLPCV and nine via UCO. Of the 45 SLPCV cases, there were 16 (35.6%) with SIUGR twin (and dual) survival. SIUGR twin survival appeared associated with middle cerebral artery (MCA) peak systolic velocity (psv) <1.5 multiples of the median, and forward atrial systolic flow in the ductus venosus (DV). In a post hoc analysis, we subsequently categorized patients as: SIUGR type IIa (N = 32 (71.1%)): normal MCA psv, and normal DV waveform, versus SIUGR type IIb (N = 13 (28.9%)): MCA psv ≥1.5 multiples of the median, and/or DV with absent or reversed atrial systolic flow. Thirty-day survival of the SIUGR twin was 50% for type IIa and 0% for type IIb. CONCLUSION: Over one-third of SIUGR type II patients experienced dual survival after treatment with laser surgery. Normal MCA psv and normal DV waveforms were associated with SIUGR type II survival of the SIUGR twin. Post hoc exploration and subclassification of SIUGR type II patients by preoperative Doppler indices created two groups, one (type IIa) with 50% survival and one (type IIb) with 0% survival of the SIUGR twin after laser surgery. Upon further confirmation, these findings may provide guidance for counseling patients and conducting fetal therapy.

Selective Fetal Growth Restriction Type III: Application of a Recent Expert Consensus Definition.

OBJECTIVES: Per a recent expert definition, diagnosis of selective fetal growth restriction (sFGR) in monochorionic diamniotic twins is based on an estimated fetal weight (EFW) <3% as sole criterion and/or combinations of 4 contributory criteria (1 twin EFW <10%; 1 twin abdominal circumference <10%; EFW discordance ≥25%; and smaller twin umbilical artery [UA] pulsatility index >95th percentile). We assessed these criteria in sFGR Type III (intermittent absent or reversed end-diastolic flow of the UA [iAREDF]) patients to test whether meeting the more stringent parameters of the consensus definition had worse outcomes, that is, progression to sFGR Type II (persistent AREDF) or twin-twin transfusion syndrome; or secondarily, decreased dual survivorship. METHODS: This was a retrospective study of referred sFGR Type III patients (2006-2017). Patients were retrospectively categorized using consensus criteria for 2 comparisons: 1) EFW <3% versus remaining cohort; 2) EFW <3% or met all 4 contributory criteria versus remaining cohort. RESULTS: Forty-eight patients were studied. Comparison 1: EFW <3% patients (58.3%) were not more likely to demonstrate disease progression (46.4% versus 65.0%, P = .2489) or worse dual survivorship (78.6% versus 85.0%, P = .7161). Comparison 2: EFW <3% or met all 4 contributory criteria (75.0%) patients were not more likely than the others to demonstrate progression (44.4% versus 83.3%, P = .0235) or worse dual survivorship (80.6% versus 83.3%, P = 1.0000). CONCLUSIONS: In a referred cohort of sFGR Type III patients, there was no evidence that meeting more stringent parameters of the consensus definition was associated with disease progression or dual survivorship.

Dual demise following laser surgery for twin-twin transfusion syndrome: Analysis of 52 cases at a single fetal surgery center.

OBJECTIVE: To evaluate all individual cases of dual twin demise following laser surgery for twin-twin transfusion syndrome (TTTS). METHOD: This is an analysis of all monochorionic diamniotic twin gestations with TTTS complicated by dual demise following laser surgery from 2006 to 2019. Cases were reviewed by (1) a fetal surgeon researcher and (2) a panel of independent experienced maternal-fetal medicine specialists to code an etiology of demise for the donor and recipient, and to assess for possible preventability. RESULTS: Of 753 twins that underwent laser surgery for TTTS, 52 (6.9%) had postoperative dual demise. In this subgroup, gestational age at surgery was 19.5 (16.1-24.9) weeks, and 36 (69.2%) patients were Quintero stage III and IV. The most common etiology was the spectrum of disorders leading to preterm delivery, which included cervical insufficiency, preterm premature rupture of membranes, and preterm labor (44.2% and 48.1%, donor and recipient, respectively). Some degree of preventability was estimated for 23.1% of dual demises. CONCLUSIONS: The most common cause of dual demise post laser surgery for TTTS was preterm birth, reinforcing the need for studies regarding the etiology and prevention of post-fetoscopy prematurity. Nearly one-quarter of dual demise cases were deemed potentially preventable.

Twin-twin transfusion syndrome and the definition of recipient polyhydramnios.

BACKGROUND: Controversy exists regarding the threshold of recipient twin polyhydramnios required to diagnose twin-twin transfusion syndrome at a gestational age of ≥20 weeks. One criterion set (Quintero staging) requires the amniotic fluid maximum vertical pocket for the recipient twin to measure ≥8 cm, whereas another (European) system uses a maximum vertical pocket for the recipient twin of ≥10 cm. OBJECTIVE: This study aimed to characterize the patients with twin-twin transfusion syndrome who were treated with laser surgery and would be excluded from laser surgery according to the European criteria. STUDY DESIGN: A total of 366 monochorionic diamniotic twins diagnosed with twin-twin transfusion syndrome from 20 to 26 weeks' gestation who underwent laser surgery at our center were studied. A maximum vertical pocket for the recipient twin of ≥8 cm was used to diagnose twin-twin transfusion syndrome. Patients were retrospectively divided into the following 2 groups: group A with a maximum vertical pocket for the recipient twin of ≥8 cm and <10 cm and group B with a maximum vertical pocket for the recipient twin of ≥10 cm. The association of each of the groups with the survivorship outcomes was tested. Bivariate associations between the patient characteristics and the 30-day donor twin and dual survivorship outcomes were evaluated. Tests used in the analysis were chi-square or Fisher exact tests as appropriate for categorical variables and Kruskal-Wallis tests for continuous variables. Multiple logistic regression models for each of the survivorship outcomes were then assessed. The results are reported as mean±standard deviation. RESULTS: Of the 366 studied patients, 53 (14.5%) had a maximum vertical pocket for the recipient twin of ≥8 and <10 cm (group A) and 313 (85.5%) had a maximum vertical pocket for the recipient twin of ≥10 cm (group B). Groups A and B did not differ in the Quintero stage. Notably, 60.4% (32 of 53) of group A patients were stage III or IV. When compared with group B, group A was diagnosed with twin-twin transfusion syndrome at an earlier gestational age (21.7±1.6 vs 22.3±1.6 weeks; P=.0037) and had a higher prevalence of donor growth restriction (81.1% [43 of 53] vs 65.5% [205 of 313]; P=.0260). Rates of at least 1 twin and dual twin survival between group A and B were similar (98.1% [52 of 53] vs 95.8% [300 of 313]; P=.7023, and 79.2% [42 of 53] vs 83.4% [261 of 313]; P=.4369, respectively). Logistic regression models adjusted for perioperative characteristics showed no difference in the outcomes between the groups (group B as reference) (donor twin survival odds ratio, 0.64; 95% confidence interval, 0.29-1.42; P=.2753; and dual survivor odds ratio, 0.90; 95% confidence interval, 0.42-1.91; P=.7757). CONCLUSION: Restriction of the definition of twin-twin transfusion syndrome to a maximum vertical pocket for the recipient of ≥10 cm beyond 20 weeks gestational age would potentially exclude 14.5% of patients from laser surgery, the majority of whom had advanced stage twin-twin transfusion syndrome. A unifying criterion of a maximum vertical pocket for the recipient of ≥8 cm regardless of gestational age would allow inclusion of these patients and access to surgical management.

Fetal blood gases after in utero carbon dioxide insufflation for percutaneous fetoscopic spina bifida repair.

BACKGROUND: Prenatal repair of open spina bifida via the percutaneous fetoscopic approach does not require maternal laparotomy, hysterotomy, or exteriorization of the uterus. This technique requires intrauterine partial CO2 insufflation. Limited data exist on the physiological effects of CO2 insufflation on human fetuses, with no data on open spina bifida repair performed using the entirely percutaneous fetoscopic surgical technique. OBJECTIVE: Our aim was to examine the effects of intrauterine partial CO2 insufflation on fetal blood gases after percutaneous fetoscopic open spina bifida repair. STUDY DESIGN: This was a prospective study of patients who underwent percutaneous fetoscopic open spina bifida repair from February 2019 to July 2020. Fetal cordocentesis of the umbilical vein was performed in cases with favorable access to the umbilical cord. The umbilical vein cord blood samples were obtained under ultrasound guidance immediately at the conclusion of the open spina bifida repair. Simultaneous maternal arterial blood gas samples were also obtained. The results are reported as median (range). RESULTS: Of the 20 patients who underwent percutaneous fetoscopic open spina bifida repair during the study period, 7 patients (35%) underwent fetal blood sampling. The gestational age at the time of surgery was 27.4 (24.0-27.9) weeks and the operative time was 183 (156-251) minutes. The CO2 exposure time was 122 (57-146) minutes with maximum pressure of 13.5 (12.0-15.0) mm Hg. Fetal umbilical vein results were as follows: pH 7.35 (7.30-7.39), partial pressure of O2 56.2 (47.1-99.9) mm Hg, partial pressure of CO2 43.8 (36.2-53.0) mm Hg, HCO3 23.9 (20.1-25.6) mmol/L, and base excess -2.2 (-4.5 to -0.4) mmol/L. Simultaneous maternal arterial blood gas results were as follows: pH 7.37 (7.28-7.42), partial pressure of O2 187.5 (124.4-405.2) mm Hg, partial pressure of CO2 36.6 (30.7-46.0) mm Hg, HCO3 21.3 (18.0-22.8) mmol/L and base excess -3.2 (-5.9 to -1.8) mmol/L. CONCLUSION: Despite prolonged CO2insufflation of the uterus, fetal umbilical vein pH and base excess values did not approach those associated with potentially pathologic fetal acidemia.

Antenatal course of referred monochorionic diamniotic twins complicated by selective intrauterine growth restriction (SIUGR) type III.

OBJECTIVE: To describe the antenatal course of selective intrauterine growth restriction (SIUGR) type III patients. STUDY DESIGN: Retrospective study of monochorionic diamniotic twins with SIUGR type III. Patients were divided into those who did and did not progress to SIUGR type II or twin-twin transfusion syndrome (TTTS) (Groups A and B, respectively). Patient characteristics and perinatal survival were compared by Group, and continuous data are reported as median (range). RESULTS: Forty-eight patients were studied; Group A [26 (54.2%)] and Group B [22 (45.8%)]. The difference in 30-day survivorship for the appropriate for gestational age twin (88.5 vs. 100%, p = .2394) and for the SIUGR twin (73.1 vs. 95.5%, p = .0551) was not statistically significant. However, dual survivorship was lower in Group A compared to Group B (69.2 vs. 95.4%, p = .0276). CONCLUSIONS: Approximately half of the SIUGR type III patients had antenatal progression. Lack of antenatal progression was associated with 95% dual survivorship. RATIONALE: The antenatal course of monochorionic diamniotic twins complicated by SIUGR type III is not well-understood and antenatal management remains a clinical dilemma. We provide pregnancy outcomes in a referred group of SIUGR type III patients, including the rate of progression to SIUGR type II and TTTS.

Child Temperament Outcomes After Laser Surgery for Twin-Twin Transfusion Syndrome.

OBJECTIVE: Twin-twin transfusion syndrome (TTTS) is a severe pregnancy complication of monochorionic (identical) twins that results in markedly discordant in utero environments for each twin. The aim of this study was to test for differences in temperament between former donor (hypovolemic, smaller) and recipient (hypervolemic, larger) twins with TTTS. METHODS: Parents of dual survivors aged 3 to 7 years who underwent in utero laser surgery for TTTS were surveyed using the Children's Behavior Questionnaire-Very Short Form (CBQ-VSF). The CBQ-VSF assessed 3 traits: Surgency, Negative Affect, and Effortful Control. Differences in traits between donor and recipients were assessed using a paired t test. RESULTS: We studied 85 twin pairs treated in utero for TTTS. There were no statistically significant differences in Surgency, Negative Affect, or Effortful Control between recipients and donors, although score differences for each trait varied widely among sibling pairs. CONCLUSION: Despite varied in utero environments, no temperament differences between donor and recipient monozygotic twins were found for the population as a whole. This finding may be of some reassurance to parents whose pregnancies are complicated by TTTS.

Fetal neurodevelopmental recovery in donors after laser surgery for twin-twin transfusion syndrome.

BACKGROUND: Fetal magnetic resonance imaging (MRI) and spectroscopy (MRS) provide a unique opportunity to non-invasively measure markers of neurodevelopment in survivors of twin-twin transfusion syndrome (TTTS). OBJECTIVE: To characterize fetal brain maturation after laser surgery for TTTS by measuring brain volumes and cerebral metabolite concentrations using fetal MRI + MRS. STUDY DESIGN: Prospective study of dual surviving fetuses treated with laser surgery for TTTS. At 4-5 postoperative weeks, fetal MRI was used together with novel image analysis to automatically extract major brain tissue volumes. Fetal MRS was used to measure major metabolite concentrations in the fetal brain. RESULTS: Twenty-one twin pairs were studied. The average (±SD) gestational age at MRI was 25.89 (±2.37) weeks. Total brain volume (TBV) was lower in the donors, although cerebral volumes were not different between twin pairs. Recipients showed lower proportions of cortical and cerebellar volumes, normalized to TBV and cerebral volumes. MRS data showed that biochemical differences between twin brains were related to discrepancy in their brain volumes. CONCLUSION: Although donors have a smaller TBV compared to recipients, proportionality of brain tissue volumes are preserved in donors. MRS maturational markers of fetal brain development show that recovery in donors persists 4 weeks after surgery.

Risk Factors for Co-Twin Fetal Demise following Radiofrequency Ablation in Multifetal Monochorionic Gestations.

BACKGROUND: Umbilical cord occlusion via radiofrequency ablation (RFA) is utilized to maximize outcomes of the co-twin in complicated multifetal monochorionic (MC) gestations. However, post-procedure co-twin fetal demise is of concern. OBJECTIVE: The aim of this study was to determine risk factors for co-twin fetal demise following RFA. METHODS: This is a retrospective study of MC multiples that underwent RFA. Indications for RFA included twin reversed arterial perfusion (TRAP) sequence, selective fetal growth restriction (sFGR) type II, discordant lethal anomalies, and twin-twin transfusion syndrome (TTTS) with proximate placental cord insertion sites. The primary outcome was co-twin fetal demise. Bivariate analyses and multiple logistic regression modeling of identified risk factors were conducted. RESULTS: Of 36 patients studied, surgical indications were: TRAP (n = 15, 41.7%), sFGR (n = 10, 27.8%), discordant anomalies (n = 9, 25.0%), and TTTS (n = 2, 5.6%). Nine patients (25.0%) experienced a co-twin fetal demise. In multiple logistic regression analysis, fetal growth restriction (FGR) of one co-twin was associated with increased risk of co-twin fetal demise (OR = 10.85, 95% CI 1.03-114.48, p = 0.0474) and a preoperative diagnosis of TRAP was protective against fetal demise (OR = 0.06, 95% CI 0.00-0.84, p = 0.0368). CONCLUSION: Co-twin FGR was associated with an increased risk of post-RFA demise. When compared to other indications, patients with TRAP sequence were less likely to have a co-twin demise.

Twin-Twin Transfusion Syndrome Complicated by Proximate Placental Cord Insertion Sites: Endoscopic Clip-Assisted Laser Occlusion.

INTRODUCTION: Severe twin-twin transfusion syndrome (TTTS) with a large vascular communication between proximate placental cord insertion sites is a therapeutic dilemma because laser ablation may cause thermal injury to the cord roots and subsequent fetal demise. CASE PRESENTATION: Stage IV TTTS with placental cord insertion sites 1.3 cm apart and with an intervening large arterio-arterial (AA) anastomosis presented for treatment. The application of endoclips onto the large AA anastomosis between the cord roots allowed for successful laser occlusion using minimal energy. Both the donor and recipient twins were alive and well at 6 months of age. CONCLUSION: Endoscopic clip-assisted laser occlusion of a placental vessel is technically feasible and may be a useful therapeutic option in select cases.

Survival Outcomes by Fetal Weight Discordance after Laser Surgery for Twin-Twin Transfusion Syndrome Complicated by Donor Fetal Growth Restriction.

INTRODUCTION: Management options for treatment of twin-twin transfusion syndrome (TTTS) with severe donor intrauterine growth restriction (IUGR) include fetoscopic laser surgery and umbilical cord occlusion (UCO). We studied perinatal survival outcomes in this select group after laser surgery, stratifying patients by preoperative estimated fetal weight (EFW) discordance. METHODS: In this retrospective study of monochorionic diamniotic twin gestations with TTTS and selective donor IUGR who underwent laser surgery (2006-2017), preoperative EFW discordance was calculated ([(larger twin - smaller twin)/(larger twin)] × 100) and cases were divided into discordance strata. Severe EFW discordance was defined as >35%. The primary outcome was 30-day donor twin neonatal survival. RESULTS: The 371 cases were distributed by discordance strata: ≤20% (74 [19.9%]), 21-25% (49 [13.2%]), 26-30% (68 [18.3%]), 31-35% (53 [14.3%]), 36-40% (51 [13.7%]), 41-45% (38 [10.2%]), >45% (38 [10.2%]). Donor 30-day survival declined as the discordance strata increased: 86.5, 85.7, 83.8, 75.5, 64.7, 63.2, and 65.8% (p = 0.0046); 30-day survival was inversely associated with severe discordance (>35%) (64.6 vs. 83.2%, p < 0.0001). DISCUSSION: In TTTS cases complicated by donor IUGR with severe growth discordance, laser surgery was associated with donor survivorship greater than 60% suggesting that, in this setting, laser surgery remains a reasonable alternative treatment to UCO.