RESUMEN
Vein of Galen aneurysmal malformation (VGAM) is a rare vascular anomaly originating during embryonic development, specifically between the 6th and 11th weeks of gestation. This malformation results from abnormal arteriovenous connections between primitive choroidal arteries and the median prosencephalic vein (MPV) of Markowski. Typically, the MPV regresses by the 11th week, but in VGAM, this regression is hindered, leading to persistent abnormal flow and the formation of arteriovenous shunts. We present a case of successful prenatal detection, as well as a comprehensive literature review that summarizes current knowledge, emphasizes the importance of prenatal detection, detailed imaging techniques, understanding clinical presentations, and outlines treatment options. Prenatal detection, crucial for early intervention, has become feasible through ultrasonography and MRI. Fetal MRI has emerged as the gold standard, offering detailed insights into arterial feeders, nidus presence, fistula position, venous drainage, and potential complications. The clinical presentation of VGAM varies with age, and neonates diagnosed in utero may exhibit signs of high-output cardiac failure. Early detection is critical for timely intervention, as untreated VGAMs often result in high mortality rates. Prognosis depends on the severity of heart failure, the number of arteriovenous shunts, and the presence of accompanying fetal abnormalities. Various imaging modalities, including CT angiography and digital subtraction angiography (DSA), aid in the assessment and treatment of VGAM. DSA remains the gold standard for evaluating angioarchitecture and guiding endovascular interventions. The optimal treatment for VGAM is transarterial embolization, offering significant improvements in prognosis. Surgical interventions are limited due to high morbidity and mortality. Management decisions should consider the balance between minimizing neurological damage and achieving maximum embolization effectiveness.
RESUMEN
Twin pregnancies with a complete hydatidiform mole and a coexisting live fetus are rare. The incidence is estimated to be 1 in 20,000-100,000 pregnancies. Prenatal diagnosis can be made with ultrasound findings, abnormally elevated ß-hCG levels, and fetal karyotype. There are various complications following these pregnancies which include hyperemesis gravidarum, vaginal bleeding, spontaneous abortion, pre-eclampsia, intrauterine growth retardation, preterm delivery, and persistent trophoblastic disease. We report an interesting case of twin pregnancy consisting of a complete hydatidiform mole and a normal fetus achieved with in-vitro fertilization in a primary infertile couple. Suspicion of molar pregnancy was made on ultrasound examination, but the couple refused other prenatal testing and wanted to continue the pregnancy. Although the pregnancy was at high risk because of the patient's age and complications associated with a molar pregnancy, a vigorous female baby was delivered at term. The purpose of this report is to present a case of a rare obstetric condition, give evidence that gestational trophoblastic disease is occurring more commonly in multiple gestations and in-vitro fertilization pregnancies, and highlight the importance of ultrasound in prenatal diagnostics and monitoring of high-risk pregnancies.
RESUMEN
OEIS complex is an acronym for a specific, extremely rare, grouping of more commonly occurring congenital malformations consisting of an omphalocele (O), exstrophy of the cloaca (E), imperforate anus (I) and spinal defects (S). It is a midline defect occurring in early blastogenesis or in mesodermal migration. The incidence is not known, it has been estimated to be 1 in 200,000-400,000 pregnancies. The etiology is thought to be heterogeneous. Most cases occur sporadically but several reports show occurrence in siblings, concurrent occurrence in monozygotic twins, and more frequent occurrence in IVF pregnancies. We report two cases of OEIS complex. Case I was recognized postnatally in twin gestation. Case II, an IVF pregnancy, was diagnosed prenatally at 35 weeks gestation by ultrasound (US) and further confirmed by magnetic resonance imaging (MRI). The purpose of this report is to present two additional cases of this rare malformation, give further evidence of the OEIS complex occurring more commonly in multiple gestations (monozygotic twins) and IVF pregnancies and illustrate the importance of MRI in prenatal diagnostics in addition to US providing better prenatal counseling, perinatal care, and planning of reconstructive surgical management.