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Atrial natriuretic peptide mediated polyuria: pathogenesis of polyhydramnios in the recipient twin of twin-twin transfusion syndrome.

We studied the role of atrial natriuretic peptide (ANP) in the pathophysiology of polyhydramnios in monochorionic (MC) twins with and without twin-twin transfusion syndrome (TTTS). Matched maternal, fetal blood samples and amniotic fluids (AF) were obtained in utero (n=12) and at birth (n=20) from MC twins with TTTS. Blood and amniotic fluid samples were also collected from non-TTTS MC twin pairs in utero (n=6) and at birth (n=20). In both groups cellular localization of ANP in the fetal kidney and heart was performed using anti ANP rabbit polyclonal antibody. Concentrations of ANP in pg/ml were determined by radioimmunoassay.In recipient fetuses, ANP levels were higher than the donors both in utero (P< 0.001) and at birth (P< 0.001). No such differences were found between the non-TTTS twins. In the TTTS group maternal ANP levels were lower than the non-TTTS group (P< 0.05). A linear relationship was found between fetal ANP levels and the AF volumes removed at fetal blood sampling (r(2)=0.68;P< 0.01, n=12). ANP was localized predominantly to the cytoplasm of the distal convoluted tubules of the fetal kidney and heart, and the intensity of immunostaining for ANP in kidney and heart were markedly greater in the recipient than the donor twin. No such differences were found between the twin pairs. These data suggest that polyhdramnios in the recipient twin occurs as a consequence of ANP mediated increase in fetal urine output and raises the possibility of direct fetal therapy with ANP blocking agents.

Amniotic Fluid↗

Pulmonary artery calcification in recipient twins of twin to twin transfusion syndrome: a report of three cases.

Twin to twin transfusion syndrome may rarely complicate monochorionic twin gestation, resulting in discordant size of the twins. Cardiac dysfunction and various types of cardiac defects have been described in the recipient twin of this syndrome. Pulmonary artery calcification has been described earlier in one case only. We report three recipients of twin to twin transfusion syndrome with pulmonary artery calcification resulting in obstruction of pulmonary trunk in two of these. One case with progressive severe narrowing was successfully operated at four months of age with patch repair of pulmonary artery. He is doing well three years later. The obstruction is moderate in the other case. Calcification in the wall of pulmonary artery may be secondary to vascular injury sustained as a result of volume overloading of the circulation of the recipient twin.

Adult↗

Impact of selective laser ablation of placental anastomoses on the cardiovascular pathology of the recipient twin in severe twin-twin transfusion syndrome.

OBJECTIVES: We investigated the impact of selective laser ablation on the cardiovascular pathology of the recipient twin in twin-twin transfusion syndrome. STUDY DESIGN: Fetal echocardiograms and medical records were reviewed from 22 pregnancies with severe twin-twin transfusion syndrome where echocardiography was performed before and after laser. RESULTS: Before laser, cardiomegaly associated with right and/or left ventricular hypertrophy without ventricular dilatation, was observed in most cases. Right ventricular and left ventricular systolic dysfunction (shortening fraction <28%) was present in 59% and 27%, respectively, and diastolic dysfunction (based on inflow and venous Dopplers) in 73%. Shortly after laser, biventricular systolic function improved significantly and diastolic function tended to improve (50%, P = .06). Functional pulmonary atresia, secondary to right ventricular systolic dysfunction, resolved in 2 of 2 cases at post-laser echocardiography. On serial assessment, diastolic function was normal in 7 of 10, hydrops regressed in 4 of 5, and neither progressive myocardial hypertrophy nor anatomical right ventricular outflow obstruction were found. CONCLUSIONS: Selective laser ablation in severe twin-twin transfusion syndrome acutely improves biventricular systolic function and tends to improve diastolic function in the recipient twin.

Adult↗

Stuck twin syndrome without signs of twin-to-twin transfusion.

Twin-to-twin transfusion syndrome represents a dilemma for both diagnosis and management. It has recently been suggested that a difference in contrast on B-mode ultrasound between the placentas is pathognomonic for the syndrome. We describe a case of monochorionic diamniotic twins with normal growth in one twin, the other showing features of stuck twin syndrome. In this case, although the contrast difference between the placentas was marked, twin-to-twin transfusion syndrome was ruled out, rendering this sign unreliable for diagnosis. Furthermore, evaluation of the stuck twin showed excessive adrenal artery flow, a feature that has been described in connection with poor antenatal and neonatal outcome. In this case, serial Doppler ultrasound evaluation of both fetuses enabled optimal timing of delivery.

Adrenal Glands↗

Endothelin concentrations in monochorionic twins with severe twin-twin transfusion syndrome.

The objective of this study was to determine endothelin (ET-1) concentrations in monochorionic twin fetuses with and without twin-twin transfusion syndrome (TTTS). Fourteen monochorionic twin pregnancies complicated by TTTS and six without TTTS were studied. Matched maternal and fetal blood samples were obtained both in utero and at birth. Amniotic fluid samples were also collected from twin pairs. ET-1 concentrations were measured by radio-immunoassay. ET-1 concentrations in recipient fetuses were higher than in the donors both in utero(P < 0.001) and at birth (P < 0.01). Fetal concentrations of ET-1 in donors were similar to non-TTTS twins. Plasma ET-1 concentrations were significantly higher (P < 0.01) in recipient fetuses with severe hydrops than those with mild/no hydrops. Maternal concentrations of ET-1 were comparable in the two groups. Endothelin concentrations in recipient twins were 2(1/2) times higher than in their co-twins and this was related to the severity of hydrops.

Amniotic Fluid↗

Iatrogenic monoamniotic twin gestation with progressive twin-twin transfusion syndrome.

OBJECTIVE: Intentional puncture of the membrane has been reported to be a promising new method for the management of twin-twin transfusion syndrome. CASE REPORT: Treatment of twin-twin transfusion syndrome with serial amniocenteses resulted in unintentional puncture of the dividing membrane at 24 weeks of gestation. Fetal growth discordance increased, and twin-twin transfusion did not improve following the puncture. Intrauterine death of both fetuses at 27 weeks of gestation occurred. CONCLUSION: Amniotic septostomy for the treatment of twin-twin transfusion syndrome should be performed with serious consideration.

Adult↗

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↗

Acquired right heart outflow tract anomaly without systemic hypertension in recipient twins in twin-twin transfusion syndrome.

Significant hemodynamic changes are commonly observed in both fetuses in twin-twin transfusion syndrome. In the recipient twin there is cardiac dysfunction with hypertrophy of both ventricles and overall enlargement of the heart. We describe five cases of recipient twins in twin-twin transfusion syndrome with right ventricle hypertrophy, pulmonary stenosis and tricuspid regurgitation acquired in utero. These symptoms developed with no signs of systemic hypertension. Three of the five recipient twins survived and were developing normally at 3-30 months of age. Postnatal outcome is likely to have improved as a result of prenatal diagnosis of right ventricle outflow tract obstruction and timed delivery. These acquired anomalies of the right heart might be related to the particular hemodynamic conditions of the recipient twin. A global heart dilatation is logically expected, but this hypertrophy without dilatation is probably related to the shared plasma of fetuses with opposite hemodynamic conditions. This is what we consider as the hemodynamic-hormonal paradox. These acquired anomalies, though severe, are accessible to neonatal treatment if treated early.

Adult↗

Haemodynamic model of twin-twin transfusion syndrome in monochorionic twin pregnancies.

Twin-twin transfusion syndrome in monochorionic twin pregnancies is not understood completely and is controversial which hampers development of acceptable diagnostic and rational treatment strategies. A haemodynamic model was developed that relates fetal growth with (1) fetoplacental blood flow and fetomaternal effects, and (2) net twin-twin transfusion from donor to recipient twin. Fluid balance mechanisms were neglected. Placental vascular anastomoses (arteriovenous, venoarterial, arterioarterial, venovenous) were modelled as straight blood vessels connecting the placental cord insertions that grow during pregnancy. Poiseuille's law predicts significantly decreasing anastomosing resistances, and when placental sharing is unequal it is assumed that smaller placental fractions cause smaller blood volumes and pressures. Two coupled first-order differential equations describing each twin's blood volume were determined and analysis showed that placental and anastomotic development cause anastomotic blood flow to increase faster than fetal growth. Hence, it is proposed as the syndrome's underlying pathophysiology that fetal discordance increases progressively, beyond fetal compensatory capacity. Fewer anastomoses cause larger discordance, but its onset can vary widely during pregnancy. Arteriovenous plus compensating anastomoses produce dynamic steady-state growth patterns with large, opposite, measurable anastomotic blood flows. Clinical study of fetal growth patterns may identify the syndrome's underlying placental anatomy. Predicted trends depend only weakly on implemented fetal physiology and are most likely realistic. This knowledge could improve future management of the syndrome.

Adult↗

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↗

Myocardial hypertrophy of the recipient twins in twin-to-twin transfusion syndrome and cerebral palsy.

OBJECTIVES: This study was performed to determine risk factors for cerebral palsy (CP) in monochorionic twins, especially with twin-to-twin transfusion syndrome (TTTS). METHODS: In 33 pathologically confirmed monochorionic pregnancies, we analyzed the incidence of cardiovascular and neurological complications. RESULTS: Seventeen cases were diagnosed as TTTS. Myocardial hypertrophy was detected in seven recipient twins of TTTS, but not found in the donor twins of TTTS or in non-TTTS. CP developed in six out of 29 in TTTS and one out of 32 in non-TTTS. In TTTS, all the cases with CP were seen in the recipient twins. The development of CP was significantly associated with cardiovascular complications such as myocardial hypertrophy and hydropic changes. CONCLUSIONS: Myocardial hypertrophy found in the recipient twins in TTTS seems to be a risk for developing CP later on, which may have implications in understanding the pathogenesis of CP.

Adult↗

Umbilical cord cyst in a monochorionic twin pregnancy: an experiment of nature for the treatment of twin-twin transfusion syndrome.

A patient with a monochorionic twin pregnancy developed signs of twin-twin transfusion syndrome from 18 weeks of gestational age. At 23 weeks, an umbilical cord cyst and absent diastolic umbilical artery flow were noted in the donor twin. The pregnancy progressed uncomplicated until term and resulted in vaginal delivery of 2 healthy male babies at 38 weeks of gestational age. We hypothesize that the compression on the umbilical arteries of the donor twin by the umbilical cord cyst has resulted in a partial "compensation" of the twin-twin transfusion phenomenon.

Adult↗

Twin-to-twin transfusion syndrome, vein of galen malformation, and transposition of the great arteries in a pair of monochorionic twins: coincidence or related association?

The pathogenetic mechanisms leading to the development of congenital heart disease and congenital intracranial arteriovenous malformation are still unclear. We report on a monochorionic twin pregnancy with twin-to-twin transfusion syndrome (TTTS), in which vein of Galen malformation (VGM) was diagnosed in the donor twin and transposition of the great arteries (TGA) in the recipient twin. The development of TTTS, VGM, and TGA in a single monochorionic pregnancy could be pure coincidence, but there might also be a causative link. We discuss the possible contribution of genetic factors, fetal flow fluctuations, vascular endothelial growth factors, and the process of twinning itself to the development of these congenital anomalies.

Abnormalities, Multiple↗

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↗

Neuroblastoma in monozygotic twins--a case of probable twin-to-twin metastasis.

Concordance for neuroblastoma in monozygotic twins has been reported only rarely, and the cause of the shared pathology has not been established. We describe a case of infant monozygotic twins developing tumours that were morphologically, clinically and molecularly indistinguishable, but with a delay of 6 months between times of presentation. Both tumours were metastatic and had amplification of MYCN and deletion at 1p36. Twin 1, who developed neuroblastoma first, had constitutional karyotype abnormalities in at least 5% of peripheral blood mononuclear cells involving 1p and 3p, and a deletion of 1q44 in 21% of cells. Twin 2 had a normal constitutional karyotype and lacked rearrangement or deletion of these regions. We propose an acquired neuroblastoma predisposition specific for twin 1, and in utero metastatic spread of tumour cells to twin 2 via the shared placental circulation.

DNA, Neoplasm↗

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↗