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Color Doppler ultrasonography in the identification of communicating vessels in twin-twin transfusion syndrome and acardiac twins.

This study evaluates the role of color Doppler ultrasonography in the identification of the communicating placental vessels in pregnancies with twin-twin transfusion syndrome and acardiac twins. In 18 pregnancies with twin-twin transfusion syndrome and two with an acardiac twin, color Doppler studies of the placental vasculature were performed before fetoscopy for laser coagulation of the communicating vessels. In six cases of twin-twin transfusion syndrome the placental attachment of the intertwin membrane could be visualized. Pulsatile arterial blood flow was observed from the donor to the recipient twin that disappeared after laser therapy. In both cases of acardiac twins, one communicating vessel with pulsatile and another vessel with nonpulsatile blood flow in the opposite direction could be identified. Color Doppler imaging is unlikely to play a major role in assisting endoscopic laser separation of chorioangiopagus in patients with acute polyhydramnios, but it may prove to be useful in the early identification of pregnancies at risk of developing twin-twin transfusion syndrome.

Acute Disease

Agenesis of the ductus venosus in a case of monochorionic twins which mimics twin-twin transfusion syndrome.

We describe a pair of monochorionic twins with clinical manifestation of twin-twin transfusion syndrome, including discordant fetal growth, hydrops of the larger twin, and the 'stuck twin' phenomenon of the smaller twin. In the hydropic twin, we found agenesis of the ductus venosus and an abnormal umbilical vein draining directly into the inferior vena cava. There was also marked enlargement of the right atrium, representing the volume overload on it. We believe that the agenesis of the ductus venosus in the larger twin may have contributed to the 'unbalanced shunting' between the monochorionic twins. This then led to a clinical picture mimicking that seen in the authentic twin-twin transfusion syndrome.

Adult

Extrauterine twin-twin transfusion syndrome after birth in conjoined twins.

Cross-circulation of conjoined twins in utero has been demonstrated and the extent of its severity has varied. Significant 'extrauterine' twin-twin transfusion syndrome after birth, however, caused by an unbalanced shunting within the circulation has not been reported. In a case of omphalopagus conjoined twins complicated by hypovolemia in one twin and hypervolemia in the other, we demonstrated by color Doppler imaging an aberrant vessel from the hepatic artery of one twin to the hepatic vein of the other. Unbalanced shunting through this aberrant vessel was suspected and urgent separation of the conjoined twins was undertaken. Circulatory changes after birth leading to 'extrauterine twin-twin transfusion syndrome' are discussed. A possible explanation of the pathophysiology of intrauterine twin-twin transfusion syndrome is also given.

Adult

[Significance of twin to twin transfusion syndrome in the prognosis of twin pregnancies and its prenatal diagnosis by ultrasonography].

The reason why TTS worsen the prognosis of twin pregnancies was discussed with reference to case results for perinatal death, and antenatal diagnostic criteria for TTS by ultrasonography was established. Of 12 perinatal deaths in 71 twin pregnancies, eight fetuses were affected with TTS (1 acardia, 4 donors, 3 recipients). Perinatal mortality rate (39.3%), rate of preterm lobar (62.2%), rate of polyhydramnion (50.0%), mean amniotic fluid volume (3.310 ml) and cord cross section area ratio (2.38) in monochorionic discordant twins were higher than in the other three groups (monochorionic concordant twins, dichorionic discordant twins and dichorionic concordant twins). Eight fetuses among 12 monochorionic discordant twins were affected with TTS. All twins which showed a single GS in early pregnancy were monochorionic twins. Therefore TTS was considered to have a poorer prognosis than usually reported for all TTS, and to find monochorionic discordant twins with a high cord cross section area ratio must be the key in the screening of TTS. Antenatal diagnosis of TTS by ultrasonography is summarized as follows: 1) A single GS in early pregnancy. 2) Estimated body weight difference between the twins/estimated body weight of the larger twins greater than or equal to 0.2. 3) Cord cross sectional area ratio greater than or equal to 2.0.

Adult

Twin-to-twin transfusion syndrome: a subset of the twin oligohydramnios-polyhydramnios sequence.

OBJECTIVE: Our purpose was to assess the adequacy of currently accepted criteria for the diagnosis of twin-to-twin transfusion syndrome. STUDY DESIGN: Between April 1991 and February 1992 nine consecutive women with twin gestations and ultrasonographic findings of marked growth discordance, coexistent oligohydramnios-polyhydramnios, monochorionic placenta, and same sex of the fetus in the second trimester were evaluated. Mean gestational age at diagnosis was 19 weeks 5 days +/- 5 weeks, and mean growth discordance was 34% +/- 9%. Seven women underwent attempted sequential amniocentesis and cordocentesis at 23 weeks 6 days +/- 2 weeks 4 days to obtain amniotic fluid and cord blood from each fetus. Type O negative, leucocyte-poor, washed adult red blood cells were transfused into the small, oligohydramnic twin during cordocentesis; immediately afterward blood from the polyhydramnic twin was tested with the Kleihauer-Betke stain. RESULTS: The combined procedure was successful in six (86%) of the cases attempted. True twin-to-twin transfusion was confirmed in only four (44%) of those initially identified by ultrasonographic criteria. Hemoglobin difference > 5 g/dl was present in only one of the four cases, and in this case the recipient was anemic. CONCLUSIONS: Currently accepted criteria are insufficient for the diagnosis of true twin-to-twin transfusion. Ultrasonographic findings of marked growth discordance, coexistent oligohydramnios-polyhydramnios, monochorionic placenta, and same sex identify the twin oligohydramnios-polyhydramnios sequence.

Amniocentesis

Clinical and echographic features of in utero cardiac dysfunction in the recipient twin in twin-twin transfusion syndrome.

OBJECTIVE: Fetal twin-twin transfusion syndrome (TTTS) presenting in the second trimester has been associated with almost no perinatal survival until recently, when serial drainage of amniotic fluid has improved the prognosis to 70%-80%. Most recipient twins now survive but develop cardiac dysfunction. The study was undertaken to evaluate the abnormal echocardiographic features and clinical complications of cardiac disease in the recipient twin of TTTS. DESIGN: Antenatal and postnatal echocardiographic and clinical observational study. SETTING: Antenatal studies in a tertiary referral centre. Postnatal management and follow up were performed by the same paediatric cardiologist, either at the obstetric hospital or at the regional referral centre. PATIENTS: Twin pregnancies complicated by TTTS with severe polyhydramnios diagnosed earlier than 25 weeks that proceeded until viability (n = 5). INTERVENTION: Serial fetal echocardiography with colour Doppler. Postnatal echocardiography in the first week and between two and seven months. Serial amnioreduction was performed in all pregnancies. Digoxin treatment, pericardiocentesis, paracentesis, or laser ablation of placental anastomoses was undertaken when there was hydrops. RESULTS: Increased cardiothoracic ratio and tricuspid regurgitation were seen in all recipient twins. High pulmonary artery velocities developed in three. One recipient twin died a week after delivery of endocardial fibroelastosis and infundibular pulmonary stenosis. Two other had balloon dilatation for pulmonary stenosis, one shortly after birth and one at four months. A further twin has apical thickening of the right ventricle at six months. The remaining recipient twin had normal echocardiographic findings at follow up. CONCLUSION: This report characterises for the first time a cardiac disease acquired in utero in the recipient twin in pregnancies complicated by TTTS. Clinical manifestations in utero range from mild to critical pulmonary stenosis or lethal cardiomyopathy. Although perinatal prognosis seems to be related to the severity of dysfunction when first diagnosed in utero, follow up in infancy is recommended in view of the possibility of progressive pulmonary stenosis.

Echocardiography, Doppler

Intrauterine death of co-twin in the third trimester: a case report of twin-to-twin transfusion syndrome and cord accident.

Intrauterine death of one twin in a monochorionic twin pregnancy is associated with increased mortality and morbidity of the survivor. The exact cause is usually difficult to determine even after delivery. The choice of management for the mother and the viable co-twin is difficult. We report on a case of intrauterine death of one twin with monochorionic placentation. Twin-to-twin transfusion syndrome was suggested by the sonographic findings and evidence of a vascular communication. The co-twin survived for another 39 days without complications after the diagnosis of fetal death. Postmortem examination showed severe torsion and constriction of the umbilical cord at the fetal end with focal deficiency of Wharton's jelly. It is likely that torsion and constriction of the cord was responsible for the one intrauterine fetal death; this so-called "twist amputation" of the cord, on the other hand, contributed to the favorable outcome in the surviving co-twin.

Adult

Congenital toxoplasmosis in twins: a series of 14 pairs of twins: absence of infection in one twin in two pairs.

Fourteen pairs of twins with congenital toxoplasmosis were observed. In two pairs, one twin was infected and had symptoms of chorioretinitis or encephalomyelitis, while the other had a negative dye test upon repeated examination: this phenomenon has not previously been reported. In three of the other twelve pairs of twins, one of each pair died; two of them had evidence of toxoplasmosis; in the cotwin the diagnosis was based on serologic data. Clincial and biologic follow-up extended from 19 months to 8 years in five pairs. Marked discrepancies in the individual clinical patterns of the two children in each of six sets of twins were observed, with evidence of infection in one twin and subclinical infection in the other. These clinical findings correlated well with serologic data in the children in whom sufficient follow-up was permitted. The clinical pattern of congenital toxoplasmosis in twins is extremely similar in monochorial pregnancies, but discrepancies are almost the rule in bichorial pregnancies. The importance of placental lesions in determining the extent of fetal involvement is well illustrated by studies of toxoplasmosis in twins.

Child

Twin transfusion: pathological studies of the monochorionic placenta in liveborn twins and of the perinatal autopsy in monochorionic twin pairs.

Vascular injections of 21 monochorionic placentas from liveborn twins were scored for chorionic surface vessel anastomoses and for venous return areas. The results were correlated with neonatal birth weight and first hematocrit. Weight and hematocrit differences did not correlate with each other or with placental anastomoses. There was a relationship between weight and placental area to the individual twin. In some cases, weight differences between monochorionic twins may be the result of placental differences rather than chronic transfusion. Seventeen perinatal autopsies of monochorionic twin pairs were reviewed for plethora/pallor and for organ and body weights. Cardiac hypertrophy was present in three autopsies. Cardiac hypertrophy may be a measure of chronic twin transfusion in the recipient. The lighter twin was as likely to be plethoric as the heavier. Plethora postmortem is a poor measure of chronic transfusion because of confounding factors, particularly acute twin transfusion. As a result of these studies, placental anatomy is speculated to be complexly rather than directly related to clinical outcome.

Adolescent

Successful external version of B-twin after the birth of A-twin for vertex--non-vertex twins.

OBJECTIVE: To determine the optimal intrapartum management of twins, especially those in vertex--non-vertex presentation. STUDY DESIGN: The study population consisted of 408 sets of twins delivered between 1988 and 1992, weighing at birth over 1500 g. According to our protocol, vaginal delivery was attempted in the vertex-vertex presentations. If non-vertex presentation was first, we performed cesarean section. In vertex--non-vertex cases, the first was delivered vaginally and external cephalic version or breech delivery was performed on the second. Apgar scores were compared according to presentation and delivery mode. RESULTS: Vaginal delivery was accomplished in 122 (73%) of 169 vertex--vertex presentations, 96 (68%) of 142 vertex--non-vertex presentations, and 4 (5%) of 99 non-vertex--other presentations. External cephalic version (ECV) of the second twin and subsequent vaginal delivery in vertex--non-vertex was successful in 75% of cases, and internal podalic version and assisted breech delivery were performed in 20 cases, and the remaining two were delivered by cesarean section. Apgar scores were not significantly different among the various groups, and no complications arose from external cephalic version performed on second non-vertex twins. CONCLUSIONS: External cephalic version was found safe for delivering second non-vertex twins in cases where the first twin is in vertex presentation. In our opinion routine cesarean section is thus no longer justified in such cases.

Delivery, Obstetric

Acute twin-twin transfusion: a possible mechanism for brain-damaged survivors after intrauterine death of a monochorionic twin.

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.

Brain Diseases

Twin-twin transfusion syndrome: the surgical removal of one twin as a treatment option.

The twin-twin transfusion syndrome, associated with acute polyhydramnios in the 18th to 28th week of gestation, has a high perinatal mortality rate. Patients managed without intervention have essentially a 100% mortality rate for the involved twins. Different methods of intervention have been described, including therapeutic amniocentesis, selective feticide, and placental vessel puncture. In this case report we describe selective removal of one twin at 21 weeks of gestation by hysterotomy in a patient with the twin-twin transfusion syndrome.

Abortion, Eugenic

Conversational patterns of two-year-old twins in mother-twin-twin triads.

This study investigated the general nature of conversational interaction in mother-twin-twin triads. Four sets of firstborn twins (2;3) were videotaped during 15 minutes of free play with their mothers. Analyses revealed that triadic conversations were approximately five times longer, and elicited more turns from all speakers, than dyadic conversations between mother and a single twin. Twins also responded as often to comments and requests made to another person as to those directed to themselves, but responded more often to questions directly to them than to others, indicating reliable monitoring of language not addressed to them. These results largely parallel those reported for mother-infant-sibling triads and indicate the unique effects of triadic exchanges are not due to the relative linguistic levels of the child partners.

Child, Preschool

Neutropenia in donor (anemic) twins involved in the twin-twin transfusion syndrome.

Neutropenia is common in neonates with sepsis and in those born to women with pregnancy-induced hypertension. Neutropenia has not previously been described, however, as a result of the twin-twin transfusion syndrome. We observed neutropenia of 4 to 8 days' duration in each of five "donor" (anemic) twins affected with the twin-twin transfusion syndrome. No evidence of infection was observed. Like neutropenia of pregnancy-induced hypertension, no left shift was seen. Neutrophil kinetic studies were performed on one of the neutropenic patients. Assessment of the marginal, storage, proliferative, and progenitor cell pools indicated that neutropenia resulted from diminished neutrophil production.

Anemia

In utero resolution of hydrops fetalis following the death of one twin in twin-twin transfusion.

A case of massive hydramnios involving a twin-twin transfusion syndrome is presented with death of the larger twin and the development of hydrops fetalis in the surviving smaller twin. The amniotic fluid volume of the surviving twin became normal and the hydrops fetalis resolved spontaneously in utero. Concomitant with the resolution of the hydrops fetalis, the umbilical cord systolic to diastolic ratio improved. At the time of delivery, the hydrops had resolved. The liveborn infant died 11 days later secondary to pulmonary hypertension and cardiorespiratory failure. Although the fetal status improved in utero, the pulmonary hypertension postdelivery resulted in neonatal death.

Adult

Fetal death of one twin in a monochorionic pregnancy with twin-twin transfusion syndrome.

BACKGROUND: Twin-twin transfusion syndrome (TTTS) occurs in 5-30% of all monochorionic pregnancies. The mortality and morbidity rates are high and therapeutic options limited. CASE: TTTS in a monochorionic twin pregnancy was initially diagnosed by differences in amniotic fluid volume at 26 weeks. No signs of atrioventricular valve insufficiency or nonimmune hydrops fetalis were present in the recipient. Despite this, the recipient died in utero, whereupon the condition of the donor improved markedly. After spontaneous delivery at 30 weeks' gestation, coarctation of the umbilical cord at its insertion was observed in the recipient, probably secondary to severe torsion. CONCLUSION: Unexpected obstetric events may occur in already-complicated pregnancies that are not related to a specific condition, its diagnosis or its therapy.

Adult

Twin-twin transfusion with abrupt onset of microcephaly in the surviving recipient following spontaneous death of the donor twin.

We describe a case in which twin-twin transfusion with fetal death of the donor at 23 weeks was complicated by suspected intracranial embolic insult of the surviving recipient twin. Dynamic transient intracranial ultrasonographic findings were noted with complete resolution by 28 weeks. Subsequent severe microcephaly of the survivor with no further growth of any of the cranial parameters beyond 25 weeks was thought to be a result of this very unusual pathophysiologic mechanism.

Adult

Angioarchitecture of monochorionic placentas in relation to the twin-twin transfusion syndrome.

OBJECTIVE: Twin-twin transfusion syndrome in the midtrimester is associated with a perinatal mortality rate exceeding 80%. Although attributed to intertwin transfusion along vascular anastomoses, these occur in all monochorial placentas, not just the 10% with twin-twin transfusion syndrome. We compared fetoplacental angioarchitecture in monochorionic twin placentas with and without twin-twin transfusion syndrome. STUDY DESIGN: The fetoplacental circulations of both twins in 20 monochorial placentas were perfused immediately after delivery under optimal physiologic conditions and anastomoses delineated by dye-contrast injection. Ten were from pregnancies with evidence of midtrimester twin-twin transfusion syndrome and 10 were from pregnancies without twin-twin transfusion syndrome. RESULTS: Placentas from pregnancies with twin-twin transfusion syndrome had significantly fewer anastomoses than did those without twin-twin transfusion syndrome, both overall (median one versus six, respectively; p < 0.001) and for each of the different types (arterioarterial, venovenous, and arteriovenous, p < 0.001). Whereas multiple anastomoses were present in all controls, only one twin-twin transfusion syndrome placenta had more than a single communication. Anastomoses in the twin-twin transfusion syndrome group were more likely to be of the deep than the superficial type (80% vs 36% in controls, p < 0.01). CONCLUSIONS: Placental vascular anastomoses in monochorial pregnancies complicated by twin-twin transfusion syndrome are both fewer in number and of a different type than those without twin-twin transfusion syndrome. These differences seem implicated in the underlying pathophysiologic features of twin-twin transfusion syndrome and are of relevance to the development of newer therapies such as placental laser surgery.

Arterio-Arterial Fistula