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Sonographic capture of acute exsanguination in a case of developing monochorionic cotwin demise
Abstract
INTRODUCTION: Single fetal demise in monochorionic twin gestations represents a significant risk for co-twin demise and neurodevelopmental morbidity. Indirect observations have led to a theory of acute exsanguination of the normal twin into the dying twin as the proposed mechanism. CASE: A 22-year-old G3P2 (2002) female with Quintero Stage IV twin-twin transfusion was referred for evaluation. During the course of the examination, bradycardia with impending fetal demise was noted in the recipient twin. Sudden and rapid development of tachycardia and elevation of the peak systolic velocity of the middle cerebral artery in the donor twin were witnessed. The demise of the donor twin was detected 24 hours later. Placental examination revealed one AV and two VA anastomoses. CONCLUSIONS: These observations support the "vascular sink" hypothesis for co-twin demise and neurodevelopmental abnormalities following single fetal demise in monochorionic twins. Rapid fetal exsanguination can occur even in the presence of a minimal number of placental vascular anastomoses.
... Monochorionic (MC) twins are at increased risk of in utero fetal death (IUD), as a consequence of typical complications such as twin-to-twin transfusion syndrome (TTTS) and selective intrauterine growth restriction (sIUGR), or even in absence of any clear cause [1]. Single fetal demise exposes the co-twin survivor to exsanguination in the empty placental territory through vascular anastomoses, with subsequent acute hypovolemia [2,3], which is responsible for multi-organ damage, including Fetal Diagn Ther 2020;47:66-73 DOI: 10.1159/000500774 cerebral injury, or death. It is assumed that the risk of cerebral injury in MC twin survivors after single IUD is four times higher than in dichorionic ones [4]. ...
Objectives:
To evaluate incidence of cerebral injury and outcome in a large series of monochorionic (MC) twin survivors after spontaneous single fetal demise.
Methods:
Retrospective analysis of all MC pregnancies with single fetal demise diagnosed at, or referred to, the Fetal Therapy Unit "U. Nicolini," V. Buzzi Children's Hospital, Milan, Italy, from 2004 to 2015. Survivors evaluation protocol included detailed ultrasound (US) of intracranial anatomy, Doppler investigation of peak systolic velocity in the middle cerebral artery (MCA-PSV), and magnetic resonance (MR). Data were collected on pregnancy characteristics, postnatal brain scan, and MR and neurological follow-up.
Results:
Seventy-eight consecutive MC pregnancies were analyzed. Median gestational age (GA) at single fetal demise was 22 weeks (range 15-36); median interval between single demise and live birth was 105 days (range 1-175), with a median GA at birth of 36 weeks (range 23-41). Prenatal MR was performed in 57 of 78 cases (73%). Cerebral injury affected 14/78 (18%) co-twins, 2 of whom were born immediately after single demise, with postnatal diagnosis of cerebral injury; of the other 12 fetuses that were studied before birth, 10 had a prenatal diagnosis of lesion both with US and MR, one only with MR, and in one case, a grade III intraventricular hemorrhage was reported only after delivery, which occurred at 25 weeks, 5 weeks after the single demise. Signs of fetal anemia (MCA-PSV value above 1.55 MoM) were related to a higher risk of prenatal cerebral injury; cases with postnatal diagnosis of lesion were delivered at lower GA.
Conclusions:
Cerebral injury affects 18% of co-twin survivors after single fetal demise in MC twin pregnancies, and evaluation and follow-up of these cases can improve detection rate of such damage.
... 9,10 Additionally, a recently reported case appeared to capture video-graphically the precise moment of fetal exsanguination at 19 weeks following a monochorionic cotwin death. 11 It is widely accepted that MC twin pregnancies complicated by single twin demise warrant close fetal monitoring in an experienced fetal medicine unit. Different patterns of survivor neurological injury may evolve, which may be detectable on ultrasound, fetal MRI or both. ...
It is well established that the death of one fetus in a monochorionic twin pregnancy places the surviving twin at significant risk for neuro-developmental delay or death. Although the early 1st trimester “vanishing twin” has not traditionally been considered a major risk, the precise gestational threshold beyond which a surviving twin is at risk remains uncertain. Most experts recommend serial ultrasounds and fetal MRI in the survivor, to look for evidence of ischaemic brain injury. We present a case of early monochorionic twin demise at 14–16 weeks, with evolving ventriculomegaly and ischaemic changes on fetal MRI in the co-twin, leading to termination of pregnancy at 28 weeks.
The objective of this study was to determine the relationship between the type of placentation, vascular anatomy of the monochorionic (MC) placenta and the perinatal outcome of the surviving twin following a single intrauterine fetal death (IUFD). In this retrospective study, 92 twin pregnancies complicated by a single intrauterine death were identified from three tertiary referral centres [50 MC and 42 dichorionic (DC)]. Antenatal and neonatal data as well as information on the chorionicity, vascular anastomoses, and autopsy findings were also obtained. The percentage risk of IUFD (26 versus 2.4; P < 0.001), anaemia (51.4 versus 0; P < 0.001) and intracranial lesions at birth (46 versus 0; P < 0.001) was greater in MC than in DC twins. In MC twins without twin-twin transfusion syndrome (TTTS), perinatal mortality was higher in the group with superficial arterioarterial (AA)/venovenous (VV) channels than those with only multiple bidirectional arteriovenous (AV) anastomoses (12/15 versus 0/8; P < 0.001). However, in the TTTS pregnancies (n = 26), perinatal outcome of the surviving twin was dependent on whether the recipient (n = 16) or the donor twin (n = 10) died first. Incidence of IUFD (9/16 versus 0/10; P < 0.001), severe anaemia (7/7 versus 1/10; P < 0.001) and intracranial lesions at birth (6/7 versus 2/10; P < 0.001) was common in pregnancies where the recipient twin died first. In the TTTS group, unidirectional AV anastomotic channels were found in all but two placentae. In conclusion, this study suggests that the outcome of twin pregnancies complicated by IUFD is dependent on the type of vascular anastomoses and TTTS.
The objective of this study was to determine the relationship between the type of placentation, vascular anatomy of the monochorionic
(MC) placenta and the perinatal outcome of the surviving twin following a single intrauterine fetal death (IUFD). In this
retrospective study, 92 twin pregnancies complicated by a single intrauterine death were identified from three tertiary referral
centres [50 MC and 42 dichorionic (DC)]. Antenatal and neonatal data as well as information on the chorionicity, vascular
anastomoses, and autopsy findings were also obtained. The percentage risk of IUFD (26 versus 2.4; P < 0.001), anaemia (51.4 versus 0; P < 0.001) and intracranial lesions at birth (46 versus 0; P < 0.001) was greater in MC than in DC twins. In MC twins without twin–twin transfusion syndrome (TTTS), perinatal mortality
was higher in the group with superficial arterioarterial (AA)/venovenous (VV) channels than those with only multiple bidirectional
arteriovenous (AV) anastomoses (12/15 versus 0/8; P < 0.001). However, in the TTTS pregnancies (n = 26), perinatal outcome of the surviving twin was dependent on whether the recipient (n = 16) or the donor twin (n = 10) died first. Incidence of IUFD (9/16 versus 0/10; P < 0.001), severe anaemia (7/7 versus 1/10; P < 0.001) and intracranial lesions at birth (6/7 versus 2/10; P < 0.001) was common in pregnancies where the recipient twin died first. In the TTTS group, unidirectional AV anastomotic
channels were found in all but two placentae. In conclusion, this study suggests that the outcome of twin pregnancies complicated
by IUFD is dependent on the type of vascular anastomoses and TTTS.
Intrauterine death of one twin in monochorionic pregnancies is associated with increased mortality and morbidity for the survivor. This has been attributed to the consequences of intrauterine disseminated intravascular coagulation (DIC) initiated by the dead twin. We describe a case in which the fetal cerebral and renal lesions typically found in survivors occurred without any derangement in coagulation. Instead, acute twin-twin transfusion was suggested by the presence of severe anemia in the surviving fetus at delivery. We suggest that the lesions frequently found in the survivors are often due to acute hemodynamic and ischemic changes resulting from acute twin-twin transfusion at the time of intrauterine death, rather than to late-onset DIC. This hypothesis has an important implication for future management: Intervention must occur before intrauterine death if neurologic sequelae in the survivor are to be prevented.
In multiple gestations, intrauterine death of one fetus occurs frequently. Sonographic studies indicate that many twin pregnancies are converted in early pregnancy to singletons. The "vanished" twin is sometimes recognized as a fetus papyraceous (compressus) incorporated into the placenta of the survivor. Death of one twin later in pregnancy may have serious implications for the survivor, especially in cases of monochorionic twins. One postulated mechanism has been that thromboplastic proteins from the dead twin are transfused into the survivor's circulation, resulting in disseminated intravascular coagulation. More recently it has been proposed that massive blood loss may occur from the survivor into the more relaxed circulation of a dead monochorionic twin through vascular anastomoses. These mechanisms may explain the higher frequency of cerebral palsy in monochorionic twins. These concepts emphasize the importance of careful examination and thoughtful interpretation of twin placentas.
Our goal was to investigate the mechanisms that play a role in intrauterine death in monochorionic twins and that contribute to the high perinatal mortality and morbidity in the survivors.
In 8 monochorionic twin pregnancies complicated by the intrauterine death of a single twin, we took samples from 5 twin fetuses immediately before death and from 4 of their cotwins and also from 4 surviving fetuses within 24 hours after death of the cotwin.
Four of the 5 fetuses sampled who subsequently died were acidemic and 3 were hypoxemic. None of these fetuses or their cotwins were anemic at that time. All 4 survivors sampled within 24 hours of the death of each cotwin had low hematocrits.
Fetal anemia, probably the consequence of acute blood loss just before the time of death of the cotwin, may play a role in the high mortality and morbidity found in the surviving twin. It is unlikely that immediate delivery of the surviving twin after death could affect the outcome.