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Vein of Galen aneurysmal malformation: rationalizing medical management of neonatal heart failure
Neonates who present in high output heart failure secondary to vein of Galen aneurysmal malformation can be difficult to manage medically due to the complex physiology that results from the large shunt through the malformation. Though the cardiac function is often normal, right ventricular dilation, severe pulmonary hypertension, and systemic steal can result in inadequate organ perfusion and shock. This report recommends medical management for stabilization of neonates prior to definitive management with endovascular embolization.
Impact
Vein of Galen aneurysmal malformation (VGAM) is a rare intracranial arteriovenous malformation, which can present in the neonatal period with high output heart failure.
Heart failure secondary to VGAM is often difficult to manage and is associated with high mortality and morbidity. Despite optimal medical management, many patients require urgent endovascular embolization for stabilization of their heart failure.
This report offers discrete recommendations that can be used by clinicians as guidelines for the medical management of heart failure in newborns with VGAM.
Journal Article
Abū Bakr al-Rāzī, Doutes sur Galien : introduction, édition et traduction
\"This book provides a critical edition as well as a French translation of the Doubts About Galen, a polemical treatise written by Abū Bakr al-Rāzī, one of the most innovative clinicians and philosophers of the Arabo-Islamic Middle Ages. His Doubts stand at the cross-roads of metaphysics, natural philosophy and medicine. The edition includes a comprehensive introduction which discusses the treatise most significant philosophical features.\" Publisher's website.
Book
Ultrasound-guided transuterine coil embolization of a Vein of Galen malformation
Vein of Galen malformations (VOGMs) are rare and complex congenital vascular anomalies that can lead to severe morbidity and mortality. Management predominantly focuses on postnatal endovascular interventions. However, these may not be feasible for fetuses with hemodynamic instability and high-output cardiac failure and may fail to prevent irreversible brain damage induced by prolonged compression by the venous varix, hemodynamic alterations and resultant potential ischemic injury. In utero endovascular embolization in high-risk VOGM fetuses may decrease mortality, prevent cardiac decompensation, and improve neurological and cognitive outcomes, thereby potentially establishing a novel standard of care for these challenging cases. We present a case of a fetus with VOGM, managed via a multidisciplinary approach through ultrasound-guided, in utero endovascular embolization. The procedure was successfully performed without complications, and the mother was discharged in good condition. At birth and a 3-month follow-up, the newborn demonstrated normal heart and respiratory function. The newborn is scheduled for diagnostic angiography and potential embolization at 6 months post-delivery
•This is the fourth reported case demonstrating the feasibility of in-utero embolization for VOGM.•The procedure demonstrated a significant reduction in vascular shunting and improved fetal cardiac function, preventing irreversible neurological damage that can be encountered in conventional post-natal treatment.•The study provides a foundation for advancing in-utero interventions as a potential standard of care for select high-risk VOGM cases.
Journal Article
Hygiene
\"Galen of Pergamum (AD 129-?199/216), physician to the court of the emperor Marcus Aurelius, was a philosopher, scientist, medical historian, theoretician, and practitioner who wrote forcefully and prolifically on an astonishing range of subjects and whose impact on later eras rivaled that of Aristotle. Galen synthesized the entirety of Greek medicine as a basis for his own doctrines and practice, which comprehensively embraced theory, practical knowledge, experiment, logic, and a deep understanding of human life and society. His treatise Hygiene, also known as \"On the Preservation of Health\" (De sanitate tuenda), was written during one of Galen's most prolific periods (170-180) and ranks among his most important and influential works, providing a comprehensive account of the practice of preventive medicine that still has relevance today. Also included in this two-volume edition are two shorter treatises on the relationship between health and wellness. Thrasybulus explores the theoretical question of whether hygiene is part of medicine or gymnastics, and in so doing delineates the interrelated roles of doctors and physical therapists. On Exercise with a Small Ball strenuously advocates that activity's superiority to all other forms of exercise.\"-- Provided by publisher.
BOOK
Outcome of vein of Galen malformation presenting in the neonatal period
ObjectiveVein of Galenaneurysmal malformation (VGAM) is a rare but important congenital malformation presenting to neonatal intensive care units (NICUs), and with a change from surgical to endovascular management, survival for this condition has improved. However, there is little reported about the medical management decisions of infants with this condition and the associated long-term neurodevelopmental outcomes. We aim to report a single centre experience of both acute treatment and long-term outcomes of VGAM for those infants admitted to our NICU soon after birth.DesignRetrospective cohort study over a 15-year period from 2001 to 2015 inclusive.SettingA quaternary NICU at The Royal Children’s Hospital, Melbourne, Australia.Participants24 newborn infants referred for management of VGAM. There were no eligibility criteria set for this study; all presenting infants were included.InterventionsNone.Main outcomes measuresClinical neuroimaging data were gathered. Surviving children were formally assessed with a battery of tests administered by a neuropsychologist and occupational therapist/physiotherapist at various ages across early to middle childhood.ResultsFifteen neonates with VGAM did not survive beyond their NICU admission. 10 of these were not offered endovascular intervention. Of the nine surviving infants, only one had a normal neurodevelopmental outcome.ConclusionsThe mortality of VGAM presenting in the neonatal period was high, and rates of normal neurodevelopmental outcome for survivors were low. These findings contribute to our understanding of which neonates should be treated and highlights the importance of providing clinical neurodevelopmental follow-up to survivors beyond their infant years.
Journal Article
Galen and the world of knowledge
\"This volume of new essays is based on a conference with the same title held at the University of Exeter in 2005. All those speaking on that occasion have written chapters in this volume, along with Riccardo Chiaradonna whose chapter has been specially prepared for the volume. The aim of this volume, like the conference on which it is based, is to contribute to the upsurge of new research on Galen by focusing on a topic that bridges the interests of specialists in ancient medical history and Classicists and philosophers more generally. The conference also represents the convergence of two current focuses of research in the Department of Classics and Ancient History at Exeter, on ancient medicine especially Galen and on Hellenistic and Imperial Greek culture more generally\"--Provided by publisher.
Book
Vein of Galen Aneurysmal Malformation: Advances in Management and Endovascular treatment
Abstract
BACKGROUND
Vein of Galen aneurysmal malformation (VGAM) is a rare congenital vascular malformation representing <1% of all arteriovenous malformations. The knowledge and strategies in the management are constantly evolving.
OBJECTIVE
To review our series of postneonatal VGAM patients treated over 11-yr period.
METHODS
Retrospective analysis of 113 VGAM treated between January 2004 and April 2015. After exclusions, 45 patients were included: 33 choroidal and 12 mural types.
RESULTS
Presenting symptom in the order of frequency: enlarged head circumference, antenatal diagnosis, mild CHF, and PHT at birth. Older patients were diagnosed following trauma, headache, cognitive decline, and incidentally during workup for other diseases. Hydrocephalus due to hydrodynamic disorder was present in 70% of choroidal and 58% of mural types. Only a quarter needed cerebrospinal fluid diversion procedure. Radiological cure was achieved in 82%; the outcome graded on a 5-point scale: 0 (death) to 4 (normal). A total of 66.6% are neurologically and developmentally intact with outcome score 4, 20% had outcome score of 3, and 8.9% had outcome score of 2. There was 4.4% mortality. Dural feeders to VGAM were found either in the initial or during the treatment in 22.2% in the current series. Angiogenesis from pial vessels developed after partial embolization in 17.7% that resolved completely following complete obliteration of VGAM.
CONCLUSION
Technical and technological advancements in endovascular embolization along with better understanding of clinical, anatomic, and pathophysiological aspects have resulted in significantly improved outcome and prognosis in VGAM. Most patients with proper treatment can now survive and most develop normally following appropriately timed treatment.
Journal Article
Cardiovascular profile score (CVPS) and selected cardiac parameters in fetuses with Vein of Galen malformation
OBJECTIVES: Vein of Galen Malformation (VGAM) is a rare congenital cerebrovascular anomaly. Early detection and monitoring of concurrent fetal heart failure in VGAM are crucial for improving outcomes. The study aims to evaluate heart anatomy, systolic and diastolic heart function, and indicators of heart failure in fetuses referred to a tertiary center due to VGAM detected in the second or third trimester. MATERIAL AND METHODS: This single-center retrospective study of echocardiographic data from five fetuses with VGAM examined between 2008 and 2023. Parameters analyzed included gestational age, reason for referral, cardiovascular profile score (CVPS), systolic and diastolic heart function and selected cardiac parameters RESULTS: Cardiomegaly as a sign of congestive heart failure was the main reason for referral most of the fetuses (4/5 — 80%) diagnosed later as VGAM in our center. Abnormal cerebral vessel flow was visualized in all cases, with normal umbilical arterial flow. Three fetuses were treated prenatally with digoxin due to congestive heart failure. Monophasic tricuspid valve inflow and decreased shortening fraction (SF) were observed in three fetuses (60%) at the time of diagnosis. CVPS scores ranged from 2 to 10, correlating with the severity of cardiac compromise. All five patients demised, three died in utero, and two shortly after birth. CONCLUSIONS: This study emphasizes the severe cardiac implications of VGAM detected in utero and the need for early and comprehensive fetal assessment. Despite early diagnosis, outcomes remain poor, necessitating further research into effective prenatal treatments and management protocols to enhance survival for affected fetuses.
Journal Article