Cases reported "Polyhydramnios"

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1/13. Ultrasound in the diagnosis of twin-to-twin transfusion syndrome--a preliminary report.

    OBJECTIVE: The aim of this study was to show the diagnostic possibilities of ultrasound in twin-to-twin transfusion syndrome (TTTS), to demonstrate its usefulness in cases of hemoglobin difference < 5 g/dL in neonates as well as in cases complicated by intrauterine death of one or both twins. STUDY DESIGN: The study included 4 pairs of twins. In two cases 3 fetuses died in utero. In the other two the hemoglobin difference in newborn infants was < 5 g/dL. The ultrasound examination, evaluating the fetal anatomy and biometry, amniotic fluid volume, placentation, was performed. The special attention was paid to polyhydramnios/oligohydramnios, discordant fetal growth (fetal weight, abdominal circumference), the presence of fetal hydrops, ascites, pericardial effusion. Doppler blood flow velocimetry has been done in umbilical vessels, middle cerebral artery. The data was compared with normal reference values for singleton pregnancies. The waveforms from both uterine arteries were also evaluated to exclude placental insufficiency. Additionally the blood flow through the AV valves was measured to exclude or confirm its regurgitation. Postnatally the hemoglobin difference was evaluated if available and compared with sonographic findings. RESULTS: In all cases inter-twin difference in abdominal circumference was greater than 18 mm and inter-twin weight difference of more than 15%. We observed polyhydramnios/oligohydramnios in all twin pregnancies. It has been found decreased PI in middle cerebral artery in larger fetus (recipient) in all our cases (comparing to single pregnancy). PI values for this vessel in smaller fetuses were within normal range (for single pregnancy). PI values in umbilical arteries of recipients were abnormal (increased PI or absent end-diastolic flow). COMMENTS: The sonographic criteria seem to be very useful in diagnosis of twin-to-twin transfusion syndrome, especially in cases where the measurement of hemoglobin level is impossible. Doppler blood flow velocimetry in fetal vessels helps to explain the changes in fetal hemodynamics in TTTS.
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2/13. Severe polyhydramnios in twin reversed arterial perfusion sequence: successful management with intrafetal alcohol ablation of acardiac twin and amniodrainage.

    We present two cases of twin reversed arterial perfusion (TRAP) sequence complicated by severe polyhydramnios during the second trimester. Both cases were successfully managed at 27 and 26 weeks with ultrasound-guided intrafetal alcohol ablation of the acardiac twin and amniodrainage. The pump twins were delivered at 35 and 37 weeks, respectively, and both had uneventful postnatal courses. These cases suggest that, in the setting of severe polyhydramnios, prenatal intervention in TRAP sequence could certainly improve the otherwise poor prognosis of the pump twin. Targeting the main intra-abdominal vessel of the acardiac twin rather than its umbilical cord seems to be a good alternative in the prenatal treatment of these cases. The option of percutaneous intrafetal alcohol injection is widely available and less invasive, simpler and easier to perform than recently advocated endoscopic techniques.
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3/13. Alcoholization: the choice of intrauterine treatment for chorioangioma.

    Chorioangioma is a vascular tumor of the placenta. Most are small and asymptomatic, whereas the large tumors are clinically significant and often associated with polyhydramnios and fetal heart failure. To prevent fetal loss from these complications, many interventions have been proposed, including intrauterine transfusion in anemic cases and fetoscopic surgery to ablate the feeding vessels. The case presented herein had large chorioangiomas, 8 and 4cm in diameter, associated with polyhydramnios and early signs of hydrops fetalis, diagnosed at 27 weeks gestation. After extensive counseling, we performed alcohol ablation of the feeding vessel of the larger tumor. Signs of fetal heart failure and hydrops fetalis disappeared dramatically. The pregnancy was extended for 2 weeks, followed by premature rupture of the membranes and spontaneous labor at 32 weeks gestation and a surviving female baby, weighing 1360g, was delivered uneventfully. This preliminary experience suggests that alcoholization may be one of the best choices for this condition due to its high efficacy, simplicity, safety and very low cost. To our knowledge, this is the first report using alcoholization for the treatment of hydrops fetalis secondary to chorioangioma.
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4/13. Monocephalus diprosopus, a rare form of conjoined twins, and associated congenital anomalies.

    Craniofacial duplication (diprosopus) is a rare form of conjoined twins. A case of monocephalus diprosopus with anencephaly, cervicothoracolumbar rachischisis, and duplication of the respiratory tract and upper gastrointestinal tract is reported. The cardiovascular system remained single but the heart showed transposition of the great vessels. We present this case due to its rarity, and compare our pathologic findings with those already reported.
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5/13. Percutaneous ultrasonographically guided ablation of an acardiac twin.

    Acardiac twinning is caused by twin reversed arterial perfusion (TRAP). Normal "pump" twins may face early delivery and cardiac decompensation and have a high perinatal mortality. A primagravid patient had serial evaluation of a TRAP pregnancy beginning early in the second trimester. Rapid growth of the acardiac sibling, high diastolic velocity Doppler waveforms in the perfusing vessel, and early hydramnios prompted ablation of blood flow by a percutaneous ultrasonographically guided infusion of absolute alcohol. A term birth of a normal pump twin was the outcome. Ablation of blood flow into an acardiac sibling of a TRAP pregnancy may be indicated in cases with a poor prognosis by use of an effective percutaneous ultrasonographically guided technique.
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6/13. lymphedema in noonan syndrome: clues to pathogenesis and prenatal diagnosis and review of the literature.

    The noonan syndrome (NS) is a true multiple congenital anomalies (MCA) syndrome with numerous manifestations. An association with lymphedema has been noted, but its pathogenesis is not fully understood. Nine new cases and a review of the literature explore the role of lymphedema in NS, including its pathogenesis, presentations, and phenotypic effects. Consideration is given to developmental stage at time of onset, chronicity, resolution, and anatomic site. It appears likely that lymphedema is a much more frequent concomitant in NS than previously realized. The major source of lymphedema in NS appears to be a presently undefined dysplasia of lymphatic vessels of unknown cause. Further study of lymphedema may provide an understanding of its role in shaping the NS phenotype. Comparison with other MCA syndromes and animal models is made in this regard. Relevance to prenatal diagnosis and treatment is discussed.
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7/13. Acute polyhydramnios complicating twin pregnancies.

    Acute polyhydramnios in the second trimester is a typical complication in monozygous twin pregnancies. It is caused by a feto-fetal transfusion with anemia on the donor and polycythemia on the recipient twin. Contrary to the chronic hydramnios, there is no increase in malformations. In view of the high mortality rate (100%, according to most authors), the clinical management has to be reconsidered. During the years 1979 to 1983, 10 cases of acute polyhydramnios have been observed at the University Hospital in Zurich. This corresponds to an incidence of 9% in our twin population. All cases investigated were MZ twin pregnancies. With the exception of one patient, who underwent an abortion, all women were hospitalized, had bed rest and received recurrent removals of amniotic fluid and prophylactic tocolysis. The mean gestational age at the time of diagnosis was 23 4/7 weeks and at delivery 30 3/7 weeks. In two cases--one of which is presented in detail--with an unintentional puncture of a placental vessel, the recurrence of the hydramnios did not appear. Eight of 18 newborns survived. No malformations were found. bed rest, tocolysis and recurrent amniocenteses seem to have a positive influence on the prolongation and outcome of the gestation in acute polyhydramnios.
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8/13. Aorto-pulmonary calcification: an unusual manifestation of idiopathic calcification of infancy evident antenatally.

    BACKGROUND: Idiopathic arterial calcification of infancy represents a clinical spectrum involving calcification of large and medium-sized blood vessels with an unknown etiology. Its complications include severe systemic hypertension and cardiomyopathy. CASE: A twin infant with a variant of idiopathic arterial calcification was diagnosed antenatally by the detection of hyperechogenicity of the proximal aorta and central pulmonary vessels. The calcification was apparently isolated to these vessels and was associated with polyhydramnios and hypertrophic cardiomyopathy. CONCLUSION: Because antenatal diagnosis is possible, we suggest that there should be a high index of suspicion for idiopathic arterial calcification when there is sonographic hyperechogenicity of vessel walls, evidence of polyhydramnios, cardiomyopathy, or a family history of idiopathic arterial calcification.
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9/13. Chorioangioma with hydramnios and intra-uterine fetal death.

    Chorioangiomas are benign placental tumours derived from fetal vessels, that occur with a frequency of approximately 1%. Often they are associated with hydramnios and intra-uterine fetal death. Based on a case of multiple chorioangiomas with a fatal course for the baby, aspects of the pathogenesis of hydramnios are discussed. Future development in ultrasounc technology may facilitate antenatal diagnosis.
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10/13. Non-immune hydrops fetalis and bilateral pulmonary hypoplasia in a newborn infant with extralobar pulmonary sequestration.

    Extralobar pulmonary sequestration was found in a newborn premature infant that presented with non-immune hydrops fetalis, massive bilateral hydrothorax and polyhydramnios in utero. The baby died of severe respiratory insufficiency 15 h after birth. Postmortem examination revealed distended lymphatic vessels in the sequestered lung tissue probably due to impeded lymph drainage. We suggest that not extralobar pulmonary sequestration itself but a subsequent massive unilateral hydrothorax due to severe obstruction of lymph drainage was the cause of the non-immune hydrops fetalis, pulmonary hypoplasia and polyhydramnios. If these symptoms are diagnosed before delivery, a search for extralobar pulmonary sequestration is indicated.
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