Neurodevelopmental risks in twin-to-twin transfusion syndrome: preliminary findings

doi:10.1053/ejpn.2001.0400

European Journal of Paediatric Neurology

Volume 5, Issue 1, January 2001, Pages 21-27

https://doi.org/10.1053/ejpn.2001.0400 Get rights and content

Abstract

The purpose of this study was to determine the neurodevelopmental risks in patients with twin-to-twin transfusion syndrome, a rare but serious complication of monochorionic twin gestations. From a total sample of 94 twins with twin-to-twin transfusion syndrome, admitted during 1989 and 1993, 49 patients survived and 40 patients were followed to a mean age of 24 months. Neurological status and psychomotor development (Denver and Griffiths Developmental Tests) were determined. Parameters of the neonatal period were evaluated for their potential prediction.Of the 40 tested patients 18 showed a normal psychomotor development. Thirteen patients exibited a specific delay in language development and/or showed minor neurological dysfunctions. Nine twins had severe psychomotor retardation in combination with cerebral palsy. Major neurological sequelae were found, more common in recipients than in donors (6/19 vs 3/21). Correspondingly, neonatal ultrasound showed more pathological results (especially periventricular leucomalacia) in recipients. Neither anaemia nor polycythaemia at birth can predict developmental outcome.Apart from a high prenatal mortality rate, both twins, donators as well as recipients, are highly at risk for brain damage of different aetiology, associated with abnormal neonatal cerebral ultrasound.

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Cited by (110)

Management of short cervix in twin-to-twin transfusion syndrome: a role for pessary placement following fetoscopic laser surgery?
2024, American Journal of Obstetrics and Gynecology
Preterm labor and delivery is a major concern for patients with twin-to-twin transfusion syndrome undergoing fetoscopic laser surgery. A preoperative short cervix is a risk factor for preterm labor. Pessary placement is a short-acting intervention that may be useful to reduce this adverse event.
This study aimed to investigate the relationship between pessary placement and preterm delivery in monochorionic twin pregnancies with twin-to-twin transfusion syndrome and a short cervix before fetoscopic laser surgery.
This was a retrospective study in 2 centers, including all pregnancies affected by twin-to-twin transfusion syndrome that underwent fetoscopic laser surgery with the Solomon technique between 2013 and 2022 (center A) and 2014 and 2022 (center B) with a preoperative cervical length below 25 mm. This study explored the correlation between cervical length and fetoscopic laser surgery–to–delivery interval following active or expectant management and compared perinatal outcomes between patients managed expectantly and patients managed with pessary placement, using multivariate analysis to control for potential confounders. Patients with a cervical length below 5 mm were not included in the comparative analysis.
Of 685 patients, 134 met the inclusion criteria. Moreover, 21 patients were treated with a cervical cerclage and excluded from the analysis, leaving 113 patients for the final analysis. There was a significant negative correlation between cervical length at fetoscopic laser surgery and the risk of early delivery (adjusted odds ratio, 0.66; 95% confidence interval, 0.49–0.81; P<.001). The use of a pessary correlated with fewer patients delivering before 28 weeks of gestation (adjusted odds ratio, 0.28; 95% confidence interval, 0.09–0.75), fewer double neonatal demise (adjusted odds ratio, 0.2; 95% confidence interval, 0.05–0.75). Posthoc subgroup analysis suggested that these improvements were essentially noticeable for cervical lengths between 5 and 18 mm, where pessary placement was associated with an increased fetoscopic laser surgery–to–delivery interval (+24 days; 95% confidence interval, 0.86–42; P=.042) and later gestational age at delivery (+3.3 weeks; 95% confidence interval, 0.86–42; P=.035).
Patients with a moderately shortened cervix, between 5 and 18 mm, may benefit from pessary placement after fetoscopic surgery for twin-to-twin transfusion syndrome, resulting in a reduction of adverse neonatal outcomes, double neonatal demise, and severe preterm delivery.
Mortality, morbidity and 2-years neurodevelopmental prognosis of twin to twin transfusion syndrome after fetoscopic laser therapy: a prospective, 58 patients cohort study
2018, Journal of Gynecology Obstetrics and Human Reproduction
To describe the morbidity and mortality and long-term neurodevelopmental outcomes in children born from a Twin-to-Twin Transfusion Syndrome (TTTS) pregnancy treated using laser fetoscopy in Toulouse.
All pregnancies with TTTS treated by laser fetoscopy in our centre were included. Antenatal and postnatal morbidity and neonatal morbidity were identified in the medical records retrospectively. The neurodevelopmental outcome was studied using Ages and Stages Questionnaires (ASQ) sent to the parents, an oral interview with the parents and with the child's doctor (last objective neurological examination, rehabilitation needs, learning disabilities).
Seventy-one pregnancies, or 144 foetuses, were included from 2008 to 2014, overall survival at 2 years was 72.9% and the survival of at least one child at 2 years was 91.5%, severe neonatal morbidity was 11.2% of the children, severe brain damage accounted for 3.6% of children admitted to the hospital unit. It was possible to study the neurodevelopmental outcome for 58 children (55.3%), with a corrected age from 24 to 95 months. Five children (8.6%) had severe neurological abnormalities and 17 (29.3%) had moderate neurological abnormalities.
The mortality, neonatal morbidity and long-term neurological morbidity of our cohort are higher than those of premature infants (EPIPAGE-2 French cohort), which confirms the severity of this syndrome, and are comparable to those of the TTTS cohorts described in the literature.
Fetal therapy and fetoscopy: A reality in clinical practice in 2015
2015, Journal de Gynecologie Obstetrique et Biologie de la Reproduction
L’amélioration des connaissances physiopathologiques mais aussi des outils diagnostiques et thérapeutiques permet aujourd’hui de proposer dans certaines malformations une thérapie in utero. Dans ce travail, nous discuterons des techniques et des progrès récents de la prise en charge par fœtoscopie de 4 pathologies : le syndrome transfuseur transfusé, la hernie de coupole diaphragmatique, la myéloméningocèle et enfin les pathologies obstructives du bas appareil urinaire. Aujourd’hui, le traitement laser par voie fœtoscopique est le traitement de référence pour le syndrome transfuseur transfusé. En termes de thérapie fœtale, les deux indications principales sont la hernie diaphragmatique congénitale et la myéloméningocèle, le gold standard de cette dernière restant à ce jour la chirurgie par laparotomie. De nouvelles techniques de cystoscopie fœtale sont en cours de développement. Même si la morbidité maternelle de la fœtoscopie est très faible, la rupture des membranes et l’accouchement prématuré reste un problème important. Le devenir à long terme des enfants ayant bénéficié d’un traitement in utero devra être évalué.
Both the improvement of pathophysiological knowledge of major fetal anomalies and the development of therapeutic tools have allowed in some specific cases in utero therapy by foetoscopy. We discuss the state of art and recent advances for four major anomalies: twin-to-twin transfusion syndrome, congenital diaphragmatic hernia, myelomeningocele and lower urinary tract obstruction. Fetoscopic laser surgery for twin-to-twin transfusion syndrome has become the gold standard for treatment of TTS. In terms of fetal surgery, severe congenital diaphragmatic hernia and myelomeningocele are the two main indications, even if open fetal surgery is still the gold standard for management of myelomeningocele. New techniques using fetal cystoscopy are currently under development. Although the maternal morbidity associated with foetoscopy is low, preterm rupture of membranes and preterm delivery remain an important problem. Long-term evaluation of those neonates remains mandatory.
Outcome following selective fetoscopic laser ablation for twin to twin transfusion syndrome: An 8 year national collaborative experience
2015, European Journal of Obstetrics and Gynecology and Reproductive Biology
With the recognition of the role of fetoscopic laser ablation for twin to twin transfusion syndrome (TTTS), there is a requirement for auditable standards for this technically challenging and specialized treatment. The purpose of this study is to report on the perinatal and medium-term neurodevelopmental outcomes following an 8-year national single center experience in the management of TTTS using the selective fetoscopic laser ablation technique.
An audit of all cases of TTTS treated with selective laser ablation by a single national fetal medicine team was performed. Overall perinatal survival and medium-term neurodevelopmental outcomes were reported and correlated with gestational age at diagnosis, placental location, volume of amnio-reduction, Quintero staging and percentage inter-twin growth discordance. Procedure-related complications were recorded.
The overall fetal survival for the first 105 consecutive cases of TTTS was 61% (128/210 fetuses). Dual survival occurred in 47% (49/105) of cases, and with a single survival rate of 28% (30/105), perinatal survival of least one infant was achieved in 75% (79/105) of cases. No correlation was found between any clinical or sonographic marker and perinatal outcome, although dual survival was noted to be significantly decreased with increasing Quintero stage (p = 0.041). Currently, 86% of survivors have been reported to have a normal medium-term neurological outcome.
Fetoscopic laser ablation is the established optimal treatment for severe twin to twin transfusion syndrome (TTTS). We report comparable short and medium-term outcomes following the selective fetoscopic technique comparing results from our national program with internationally published single-center outcomes, supporting the efficacy and safety of this treatment at our center.
Long-term neurologic outcomes after common fetal interventions
2015, American Journal of Obstetrics and Gynecology
Citation Excerpt :
Unfortunately, the data on long-term follow-up after conservative management of conditions are scarce. There is only one available study addressing the long-term follow-up of monochorionic twin pregnancies with twin-twin transfusion syndrome (TTTS) in patients who did not undergo intervention.38 In that study of 94 patients, the perinatal mortality rate was 48%.
Fetal interventions have clearly decreased mortality, but the neurological outcomes of survivors are of critical concern. Here we consolidated available data on long-term neurological outcomes after common fetal interventions to guide counseling, management, and future research.
Published studies assessing long-term neurological outcomes after common fetal interventions from 1990 through 2014 were collected. We included all studies with a cohort of more than 5 patients and with follow-up of 1 year or longer. We divided procedures into those performed for singletons and for multiples. Singleton procedures included amnioinfusion for preterm premature rupture of membranes, intrauterine transfusion for red cell alloimmunization–associated anemia, intrauterine transfusion for parvovirus-associated anemia, vesicoamniotic shunts, thoracoamniotic shunts, ventriculoamniotic shunts, fetal endoscopic tracheal occlusion for congenital diaphragmatic hernia, and open fetal cases by myelomeningocele and others. Multiple procedures included those done for monochorionic twins including serial amnioreduction, selective fetoscopic laser photocoagulation, and selective termination.
Of 1341 studies identified, 28 met the inclusion criteria. We combined available literature for all procedures. Studies varied in their length of follow-up and method of assessing neurological status. Neurological outcome after intervention varied by procedure but was normal in 40-93%, mildly impaired in 3-33%, and severely impaired in 1-40%. Follow-up to school age was rare with the exception of procedures for monochorionic twins.
Fetal treatments have been successful in achieving survival in previously hopeless cases, but success should also be determined by the outcomes of survivors. Except for monochorionic twins, there is a dearth of reported long-term outcomes. Standardized reporting of long-term neurological sequelae is imperative so that meaningful analysis and study comparisons can be made.
Type IV intestinal atresia, congenital bilateral perisylvian syndrome, and chronic pulmonary hypertension secondary to multiple vascular disruption syndrome in a monochorionic twin
2012, Journal of Pediatric Surgery
Citation Excerpt :
If one twin dies, the incidence of neurologic injury is higher due to the sudden decrease in vascular resistance in the placenta, which acutely shunts blood flow away from the surviving twin causing anemia and ischemia [4-7,18]. It has been reported that the recipient is more vulnerable to severe psychomotor impairments in TTTS than the donor because recipients are more prone to thromboembolism, which may lead to ischemic brain damage [19,20]. In addition to neurologic complications, the recipient twin in TTTS is also susceptible to pulmonary hypertension.

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^f1: Correspondence: F Haverkamp MD, Zentrum f. Kinderheilkunde der Universität Bonn, Adenauerallee 119, D-53113 Bonn, FRG e-mail:[email protected]

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Original articleNeurodevelopmental risks in twin-to-twin transfusion syndrome: preliminary findings

Abstract

Original article
Neurodevelopmental risks in twin-to-twin transfusion syndrome: preliminary findings