History of Management of Vein of Galen Malformations in Children

Understanding of Disease

• 1937 — First described: The first case report of a child with a VOGM was published by Jaeger, Forbes, and Dandy in 1937 (2). More cases were reported in the 1940s and 1950s (3).

Walter Dandy: Image shows Walter Dandy, a founding father of neurosurgery. Dandy is accredited with the first detailed case report of a child with a VOGM. (Image originally from the Johns Hopkins Chesney Archives and derived from Kretzer et al. [1].)

• 1960 — Anatomical description achieved: Litvak et al. were the first to provide a robust anatomical description of VOGMs, differentiating between true VOGMs and other midline intracranial vascular malformations (4).
• 1970s and 1980s — Early hypotheses of pathogenesis: Normal and Becker (5) and Kempe (6) were the first to propose an embryological hypothesis of VOGM genesis.
• 1989 — Current understanding of VOGM pathogenesis described: Raybaud, Strother, and Hald identified that VOGMs represent a persistent median prosencephalic vein of Markowski, rather than the vein of Galen itself (7). Thus, the term VOGM is a misnomer.
• 1980s and 1990s — Two classification systems: Yaşargil (10) and Lasjaunias (11) published the first classifications of VOGM. Yaşargil’s classification differentiates between pure AV fistulas and AVMs that drain into the vein of Galen. Lasjaunias’s classification divides VOGMs into choroidal and mural types, depending on the origin of the arterial feeders.

Gazi Yaşargil (left) and Pierre Lasjaunias (right): Yaşargil, pioneer of microneurosurgery, and Lasjaunias, father of pediatric neurointervention, both developed classification systems of VOGM. (Images adapted from Picard and Stienen et al. [8,9].)

• 21st century — Exploring the molecular underpinnings of VOGM: In the 21st century, several gene variants associated with VOGM have been discovered and characterized in an attempt to elucidate the molecular pathogenesis of VOGM (12). This remains an active area of investigation.

Technological Development

• 1940s and 1950s — Cerebral angiography: Visualization of VOGM was assisted by advances in cerebral angiography in the 1940s and 50s (15).
• 1970s and 1980s — Advanced angiography, CT, and microsurgical technology: Further advances in cerebral angiography, CT, and microsurgical technologies allowed for the development of microsurgical techniques for VOGM in the late 1970s and early 1980s (16,17).
• 1990s — Endovascular embolization becomes possible: The development of selective angiography, coil embolization, acrylic glues, and other neurointerventional technologies allowed for treatment of VOGM using endovascular techniques (18).

Surgical Technique

• Late 1930s and 1940s — Carotid artery ligation used: Carotid artery ligation was the first, and rather unsuccessful, surgical technique used in the treatment of VOGM (2,13).
• 1940s and 1950s — Open surgical procedures used: The first open surgical procedures for VOGM involved clipping of the arterial feeders (14,19).
• 1980s — Open microsurgery led to poor outcomes: Open microsurgical techniques in the 1980s involved surgical ligation, although these procedures had exceptionally high mortality rates (16,20).
• Mid to late 1980s — Advent of endovascular embolization: Neurointerventional techniques for VOGM became standard in the late 1980s. In 1986, Mickle and Quisling described a method of transtorcular embolization (21). Several transfemoral, transarterial, and transvenous embolization techniques using coils and various embolic agents were developed in the wake of their report (18, 22-25). Endovascular embolization remains the first-line therapy for VOGM.