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Vein of Galen aneurysmal malformation (VGAM) is a rare foetal anomaly associated with neurodevelopment delay, cardiac failure, and even perinatal death. We aimed to assess prenatal features of VGAM and describe postnatal outcomes. This was a retrospective study involving six foetuses diagnosed with VGAM prenatally in two centres. All of the cases underwent foetal neurosonography and echocardiography. The presence of ventriculomegaly, intracranial haemorrhage and cardiac failure was recorded. Pregnancy and neonatal outcome information were obtained from medical records. The mean gestational age at diagnosis was 31.1 ± 5.1 weeks, and the mean size of VGAM was 29.2 ± 5.2 × 26.4 ± 3.3 mm. Ventriculomegaly was detected in five of six (83.3%) cases. Intracranial haemorrhage was present in five (83.3%) cases. Cardiac failure was shown in four (66.6%) foetuses. Three foetuses underwent termination of pregnancy (TOP); in two cases, neonatal death occurred. One patient was treated with endovascular embolisation, and there was no cardiac problem or neurodevelopment delay. Prenatally diagnosed VGAM have a poor prognosis, mainly if a cardiac failure or neurological consequences (intracranial haemorrhage, hydrocephaly) are present in utero. What is already known on this subject? VGAM is the most common cerebral arteriovenous malformation detected prenatally, and it can lead to severe consequences in the perinatal period. What do the results of this study add? The accuracy of foetal neurosonography is excellent for detecting VGAM and associated brain abnormalities. Foetal echocardiography is mandatory for the prediction of prognosis What are the implications of these findings for clinical practice and/or further research? VGAM is associated with severe brain injury, cardiac failure, and the prognosis is generally poor. We need predictors to identify those expected to benefit from postnatal therapy. [ABSTRACT FROM AUTHOR]

Objective: We aimed to consider Galen vein aneurysm(GVA) in cases that may often get a diagnosis later due to the pre-diagnoses of transient tachypnea of newborn and congenital heart disease accompanied by moderate-severe pulmonary hypertension in the postpartum period. Method: The study included cases that were followed and treated with the diagnosis of GVA in our clinic between 2016-2020. Types of delivery, week of delivery, birthweight/height/head circumference, medical treatments, and imagings were recorded from patient files. The risk evaluation was performed according to Bicetre and the scoring was recorded. Results: Of our cases; 80% (n=4) were female, and 80% (n=4) had been delivered via spontaneous vaginally. Their week of delivery was 39.20±.83 (min:38, max:40), birth weight was 3488±415 gr, head circumference was 36.20±1.30 and height was 51.20±1.30 cm. Firstly, all cases had tachypnea, dyspnea, cyanosis, and tachycardia. Of our cases, three were intubated, others (4.5) received non-invasive support only. The patient (infant 3) with a lower Bicetre score did not stabilize and died. One of our cases (infant 4) whose Bicetre score was above 12, was operated electively. In all cases the GVA was accompanied by persistent pulmonary hypertension (PPHT). The cases that had severe PPHT and did not improve despite inotrop treatment died. Conclusion: Galen vein aneurysms should certainly be considered in PPHT cases in the early stage of those that don't respond to multiple treatment options. [ABSTRACT FROM AUTHOR]

Objective: Prenatal diagnosis of Galen vein aneurysm using color Doppler has become relatively easy. Management and timing of delivery is however challenging because of cardiac function deterioration. Ultrasound follow-up is essential to detect signs of fetal cardiac failure which must be weighed against risks of prematurity. Postnatal deterioration of the vital signs can follow a short period of improvement after birth. This report illustrates the many pitfalls that could affect the final outcome, raising the issue of the proper timing for delivery. Case: A 23-year-old G1P0 spontaneous pregnancy was referred at 28 WA for a Galen vein aneurysm. The ultrasound finding included a 35 by 14 mm vascular structure with a turbulent flow, occupying the central part of the head. After 48 hours in-hospital surveillance through non-stress tests twice daily, she had a cesarean section due to a persistent non-reactive fetal heart rate, with absent variability. Unfortunately, despite a stable state for the first ten days, the baby's cardiac function deteriorated abruptly on day 11, and the baby died of heart failure despite medical management. Conclusion: The timing of delivery in cases with Galen vein aneurysm is challenging mainly in the case of premature babies, as the prognosis is globally bad. Postnatal deterioration of the vital signs can follow a short period of improvement after delivery. [ABSTRACT FROM AUTHOR]

Background. Hemangiondothelioma is a rare vascular tumor that can occur in the bone. Temporal bone involvement has been reported extremely rare in the literature. Case. Radiological examination of a one-year-old girl who was admitted due to facial paralysis revealed vascular tumor of the temporal bone and Galen vein aneurysm. Pathological examination showed retiform hemangioendothelioma. She was treated with propranolol, prednisolone, vincristine, and endovascular embolization followed by oral sirolimus. With sirolimus treatment, a partial response was obtained first, then the tumor remained stable and sirolimus treatment was discontinued. No progression was observed in the disease after discontinuation of treatment. Conclusion. In this article, a case of hemangioendothelioma originating from the temporal bone is discussed in the light of other case reports in the literature. [ABSTRACT FROM AUTHOR]

Abstract Objective To describe the prenatal diagnosis of Galen vein aneurysm (GVA) based on ultrasonography and magnetic resonance imaging (MRI) in a series of cases, as well as its postnatal outcomes and follow-up until 4 years of age. Methods A retrospective longitudinal study was performed, analyzing a database comprising seven cases of prenatal diagnosis of GVA at two Brazilian institutions from February of 2000 to May of 2012. The following data were evaluated: gestational age at diagnosis, GVA dimensions on ultrasonography, associated fetal changes, findings on fetal echocardiography, gestational age at delivery, type of delivery, birth weight, Apgar score at the 1st and 5th minutes, neonatal outcomes, and survival with follow-up until 4 years of age. Results The mean gestational age ± standard deviation on the prenatal diagnosis of GVA based on ultrasonography was 25±4.9 weeks. The mean length of GVA was 3.2±0.4 cm. The mean gestational age at birth was 37.5±0.7 weeks, and a cesarean section was performed in 85.7% of the cases (6/7). The mean birth weight was 3,070±240.4 g. The total survival rate was 42.8% (4/7), with three neonatal deaths. Of the four survivors, three presented with normal neuropsychomotor development until 4 years of age and only one showed serious neurological sequelae. Ultrasonography and MRI showed similar findings for all seven cases. Conclusions Galen Vein Aneurysm is associated with a high neonatal death rate. Therefore, its prenatal diagnosis is essential for parent counseling and follow-up at tertiary care institutions.

The vein of Galen aneurysmal malformation (VGAM) is an extremely rare arteriovenous malformation. The most frequent clinical finding in the neonatal period is high-flow congestive cardiac failure that causes high morbidity and mortality. Here, we present a case diagnosed as VGAM presenting with strong retrograde flow in the aortic arch as the first manifestation. [ABSTRACT FROM AUTHOR]