1/34. Acardiac acephalus: a case report and implications on expectant management.Acardiac acephalus is a rare occurrence resulting from extensive anastomoses between the vessels of the monochorionic twins which invariably leads to death of the "perfused" twin and an estimated 50% perinatal mortality rate in the "pump" twin, due mainly to premature delivery or congestive heart failure. Once diagnosis has been documented, blocking the vascular flows between the twins has been the principle goal of treatment with the assumption that a more favorable chance of survival in the pump twin can be achieved. However, hasty surgical intervention without a well-designed preoperative evaluation may result in unfavorable outcomes. A 21-year-old nullipara was found to carry an acardiac parabiotic twin in a monochorionic twin pregnancy at 28 weeks of gestation and was referred to us from a local clinic. Expectant management based on the close monitoring of the well-being of the healthy co-twin is presented in this article along with review of published works on the management of this rare condition.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/34. Chronic intrauterine meconium aspiration causes fetal lung infarcts, lung rupture, and meconium embolism.Three neonates with chronic intrauterine meconium aspiration are reported. All had distinctive subpleural plate-infarcts of the lungs caused by meconium-induced vasoconstriction of peripheral preacinar arteries. These vessels showed plexogenic arteriopathy with medionecrosis and obliterative hyaline sclerosis. Organized thrombi and systemic-pulmonary arterial anastomoses were numerous. The infarcts contained inspissated meconium with a granulomatous reaction.In one case, lung rupture occurred, causing meconiumthorax and meconium embolism to hilar lymphatics and lymph nodes; this suggests that particulate meconium may enter the circulation. This fetus had rubella and probable acute twin-twin transfusion following the intrauterine death of the co-twin. The cause of the hypoxia that led to intrauterine passage of meconium in the other cases is unknown. meconium-stained amniotic fluid was noted in only one case.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/34. Nystagmus mimicking spasmus nutans as the presenting sign of bardet-biedl syndrome.PURPOSE: To investigate the nystagmus of twin brothers presenting with spasmus nutans later diagnosed as bardet-biedl syndrome. methods: The twins presented at the age of 14 months with a presumed diagnosis of spasmus nutans. They were followed clinically and with quantitative electro-oculographic eye movement recordings until the age of 6 years. RESULTS: polydactyly, truncal obesity, mild delay in cognitive development, visual acuity of 20/100, attenuated retinal vessels and pale disks, and bilaterally almost extinguished scotopic and photopic electroretinograms were found in both brothers. They had fine, fast, pendular, disconjugate, intermittent, oblique nystagmus. No head nodding was observed. CONCLUSION: As described in patients with other retinal diseases such as achromatopsia and congenital stationary night blindness, nystagmus of patients with bardet-biedl syndrome can mimic spasmus nutans.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/34. In utero development of hypertensive necrotizing pulmonary arterial lesions: report of a case associated with premature closure of the ductus arteriosus and pulmonary hypoplasia.Premature closure of the ductus arteriosus (PCDA) is an uncommon defect in which pulmonary hypertension (PH) has been documented by echocardiography in patients and by direct measurement after experimental PCDA in animals. The pulmonary vascular histology in human cases has received little attention but in the few recorded observations the vessels were either normal or showed increased muscularity. We report the case of a 31 week hydropic female stillborn monozygotic twin in whom postmortem examination disclosed PCDA and hypoplasia of the lungs. Atypical plexiform lesions with necrotizing pulmonary arteritis were present. These lesions represent vascular consequences of severe pulmonary hypertension produced by greatly enhanced blood flow through a restricted vascular bed resulting from the combined effects of these two abnormalities. The findings in this case of PCDA with presumed severe PH indicate that severe pulmonary vascular changes can develop in utero and that the interval of time needed for development of such chances in secondary PH is relatively short.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/34. 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.- - - - - - - - - - ranking = 3keywords = vessel (Clic here for more details about this article) |
6/34. Monozygotic twins with chromosome 22q11 microdeletion and discordant phenotypes in cardiovascular patterning.Monozygotic twins with chromosome 22q11 microdeletions offer an ideal situation to observe the association of microdeletion and disrupted cardiovascular patterning. We report monozygotic twins concordant for 22q11.2 microdeletion but discordant for cardiovascular patterning. Both twins showed identical intracardiac defects including tetralogy of fallot with pulmonary atresia. Nevertheless, their great vessel patternings were variable. These twins show that the mispatterning of the great vessels may not correlate with intracardiac morphogenesis. The discordant development of the great vessels, especially in the pulmonary vascular system, has clinical significance for prognosis. The phenotypic variability of cardiovascular anomalies seen in 22q11 microdeletion cannot be explained on the basis of genotypic difference.- - - - - - - - - - ranking = 3keywords = vessel (Clic here for more details about this article) |
7/34. An intracranial aneurysm in one identical twin, but no aneurysm in the other.Only seven pairs of identical twins with intracranial aneurysms have been reported in the literature. In six of these, both twins had aneurysms, but in one pair only one twin had an aneurysm. We present another such pair of identical twins 21 years of age of whom only one twin had an aneurysm. She presented with a subarachnoid haemorrhage. Four-vessel angiography showed one aneurysm on the internal carotid artery; the aneurysm was clipped. Four-vessel angiography in her identical twin sister revealed no aneurysms. We have reviewed the seven previous reports of identical twins with intracranial aneurysms. We conclude that in case of an aneurysm in one identical twin, the other twin should be examined.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
8/34. Two-dimensional and three-dimensional ultrasound of fetal anasarca: the glass baby.Fetal edema was recognized in the past as a complication of alloimmune disease. More recently, fetal edema is frequently seen in conditions that cause non-immune fetal hydrops with increased soft tissue thickness. Classically there is a halo pattern around fetal head, neck, thorax, and abdomen. Fetal edema is associated with karyotype abnormalities, with multiple congenital anomalies, and with certain fetal infections like parvovirus B19 that cause severe fetal anemia. In the present case there was no evidence of infection or karyotype abnormality, but there was hypoplasia of umbilical cord vessels, pulmonary hypoplasia, and pericardial effusion. The etiology of fetal hydrops may remain unknown in up to 30% of cases.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/34. Renal failure caused by eyedrops containing phenylephrine in a case of retinopathy of prematurity.The patient was a low birth weight infant with a history of renal failure. She was referred to our department 29 days after birth to undergo fundus examination. She experienced renal failure after undergoing a mydriatic test and needed medical treatment. Eyedrops containing phenylephrine were instilled several times and additional drops were also instilled during the fundal examination using an eyelid retractor, therefore the blood concentration of the drug was elevated sufficiently to contract the renal vessels, ultimately inducing renal failure. The present case suggests that since the use of mydriatic eyedrops in low birth weight infants could induce renal failure, the following points should be considered: 1) Mydriatic eyedrops should be used with caution by monitoring mydriasis and avoiding excessive instillation; 2) After instillation, the lacrimal region should be compressed to prevent the flow of mydriatic drops to the nasolacrimal canal; and 3) vital signs should be monitored to check the onset of any adverse reactions for 12 hours after fundal examination.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/34. Vascular anastomoses in dichorionic diamniotic-fused placentas.A case of fetal twin-to-twin cytomegalovirus infection through a dichorionic diamniotic (DiDi)-fused placenta prompted our search for possible vascular anastomoses in this type of placenta. This case and three additional DiDi-fused placentas were studied with gross (macro) sections and a three-dimensional (3D) stereomicroscopic technique. Two twins were dizygotic (they differed in gender and blood groups) and the other two were probably monozygotic. Macrosections and 3D-image analysis demonstrated side-to-side connections between small subchorionic vessels. These findings demonstrate that vascular anastomoses are present in DiDi-fused placentas.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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