Management und Outcome der Vena-Galeni-Malformation im Kindesalter

Zusammenfassung

Die Vena-Galeni-Malformation (VGM) ist eine seltene angeborene zerebrale Gefäßmalformation, welche heute überwiegend pränatal diagnostiziert wird. Die Prognose hängt maßgeblich davon ab, ob der arteriovenöse Shunt zu einer Herzinsuffizienz führt oder nicht. Dabei ist die Prognose der neonatal symptomatischen VGM mit frühzeitiger Herzinsuffizienz besonders schlecht. Da das Krankheitsbild selten ist und die Behandlung eine interdisziplinäre Zusammen-arbeit erfordert, ist es besonders wichtig, Patienten frühzeitig in einer Klinik zu behandeln, welche über ein Perinatalzentrum und eine interventionelle Neuroradiologie sowie über eine Neurochirurgie verfügt und in der Behandlung der VGM geübt ist. Dargestellt werden die Genese, die Pathophysiologie, die Diagnostik, das therapeutische Vorgehen und die Prognose der VGM.

Abstract

The vein of Galen malformation (VGM) is a rare congenital cerebral vascular malformation. In most cases it already has been diagnosed prenatally. The impact of heart failure due to the arteriovenous shunt is relevant for the prognosis.Prognosis of neonatal symptomatic VGM with early heart failure is severe. Therefore an interdisciplinary cooperation is necessary and it is important to treat patients in a clinic with a perinatal center and a neuroradiology and neurosurgery that is experienced in treating such patients. The genesis, the pathophysiology, the diagnostic agents, the therapeutic agents and the prognosis of VGM will be described.

Schlüsselwörter

Vena-Galeni-Malformation - kongestive Herzinsuffizienz - Therapieoptionen - Outcome

Key words

vein of Galen malformation - congestive heart failure - therapeutic options - outcome

Literatur

• 1 Berenstein A, Masters LT, Nelson PK, Setton A, Verma R. Transumbilical catheterization of cerebral arteries.  Neurosurgery. 1997;  41 846-850
• 2 Blondin D, Turowski B, Mödder U, Schaper J. Prä- und postnatale MRT des Fetus bei komplexer intrakranieller vaskulärer Malformation.  Radiologe. 2006;  46 144-149
• 3 Boldrey E, Miller ER. Arteriovenous fistula (aneurysm) of the great cerebral vein (of Galen) and circle of Willis: Report on two cases treated by ligation.  Arch Neurol Psychiatry. 1949;  62 77-83
• 4 Borthne A, Carteret M, Baraton J, Courtel J-V, Brunelle F. Vein of Galen vascular malformations in infants: clinical, radiological and therapeutic aspect.  Eur Radiol. 1997;  7 1252-1258
• 5 Breuer J, Schmaltz AA, Barth H, Steil E, Grunert D, Petersen D, Haßler WE, Apitz J. Aneurysma der Vena Galeni als mögliche Ursache für eine kongestive Herzinsuffizienz.  Monatsschr Kinderheilkd. 1989;  137 726-732
• 6 Brunelle F. Arteriovenous malformation of the vein of Galen in children.  Pediatr Radiol. 1997;  27 501-513
• 7 Casasco A, Lylyk P, Hodes JE, Kohan G, Aymard A, Merland JJ. Percutaneous transvenous catheterization and embolization of vein of Galen aneurysms.  Neurosurgery. 1991;  28 260-266
• 8 Chevret L, Durand P, Alvarez H, Lambert V, Caeymax L, Rodesch G, Devictor D, Lasjaunias P. Severe cardiac failure in newborns with VGAM.  Intensive Care Med. 2002;  28 1126-1130
• 9 Choi JH, Mohr JP. Brain arteriovenous malformations in adults.  Lancet Neurol. 2005;  4 299-308
• 10 Ciricillo SF, Edwards MSB, Schmidt KG, Hieshima GB, Silverman NH, Higashida RT, Halbach V Van. Interventional Neuroradiological Management of Vein of Galen Malformations in the Neonate.  J Neurosurgery. 1990;  27 22-28
• 11 Dahdah NS, Alesseh H, Dahms B, Saker F. Severe Pulmonary Hypertensive Vascular Disease in Two Newborns with Aneurysmal Vein of Galen.  Pediatr Cardiol. 2001;  22 538-541
• 12 Deeg KH, Lode HM. Transfontanelläre Doppler-Sonographie der Hirnvenen im Säuglingsalter Teil II.  Ultraschall in Med. 2006;  27 164-175
• 13 MacElhinney DB, Halbach V Van, Silvermann NH, Dowd CF, Hanley FL. Congenital cardiac anomalies with vein of Galen malformations in infants.  Arch Dis Child. 1998;  78 548-551
• 14 Frawley GP, Dargaville PA, Mitchell PJ, Tress BM, Loughnan P. Clinical course and medical management of neonates with severe cardiac failure related to vein of Galen malformation.  Arch Dis Child Fetal Neonatal Ed. 2002;  87 F144-149
• 15 Friedmann DM, Verma R, Madrid M, Wisoff JH, Bernstein A. Recent Improvement in Outcome Using Transcatheter Embolization Techniques for Neonatal Aneurysmal Malformations of the Vein of Galen.  Pediatrics. 1993;  91 583-586
• 16 Fullerton HJ, Aminoff AR, Ferriero DM, Gupta N, Dowd CF. Neurodevelopmental outcome after endovascular treatment of vein of Galen malformations.  Neurology. 2003;  61 1386-1390
• 17 Van Halbach V, Dowd CF, Higashida RT, Balousek PA, Ciricillo SF, Edwards MSB. Endovascular treatment of mural-type vein of Galen malformations.  J Neurosurg. 1998;  88 74-80
• 18 Hamasaki T, Kai Y, Hamada J-I, Miura M, Kondo Y, Ushio Y. Successful Treatment of a Neonate with Vein of Galen Aneurysmal Malformation.  Pediatr Neurosurg. 2000;  32 200-204
• 19 Hartung J, Heling KS, Rake A, Zimmer C, Chaoui R. Detection of an aneurysm of the vein of Galen following signs of cardiac overload in a 22-week old fetus.  Prenatal Diagn. 2003;  23 901-903
• 20 Henrich W, Fuchs I, Bührer C, Landeghem FKH van, Albig M, Stoever B, Dudenhausen JW. Isolated Cardiomegaly in the Second Trimester as an Early Sign of Fetal Hydrops Due to Intracranial Arteriovenous Malformation.  J Clin Ultrasound. 2003;  31 445-449
• 21 Hernesniemi J. Arteriovenous malformations of the vein of Galen: report of three microsurgically treated cases.  Surg Neurol. 1991;  36 465-469
• 22 Hirose M, Yomo H, Akiyama M, Kimura T, Noda Y. In utero diagnosis of an aneurysm of the vein of Galen causing hydrocephalus and heart failure.  J Obstet Gynaecol Res. 2003;  29 343-346
• 23 Hoffmann HJ, Chuang S, Hendrick EB, Humphreys RP. Aneurysms of the vein of Galen.  J Neurosurg. 1982;  57 316-322
• 24 Houdart E, Gobin YP, Casasco A, Aymard A, Herbreteau D, Merland JJ. A proposed angiographic classification of intracranial arteriovenous fistulae and malformations.  Neuroradiology. 1993;  35 381-385
• 25 Jaeger JR, Forbes RP, Dandy WE. Bilateral congenital cerebral arteriovenous communication aneurysm.  Trans Am Neurol Assoc. 1937;  63 173-176
• 26 Johnston IH, Whittle IR, Besser M, Morgan MK. Vein of Galen malformation: diagnosis and management.  Neurosurgery. 1987;  20 747-758
• 27 Jones BV, Ball WS, Tomsick TA, Millard J, Crone KR. Vein of Galen aneurysmal malformation: diagnosis and treatment of 13 children with extended clinical follow-up.  Am J Neuroradiol. 2002;  23 1717-1724
• 28 Kleindienst A, Hildebrandt G, Klug N, Schön R. Management of Vein of Galen malformations. A review based on five neurosurgically treated cases and literature reports.  Zentralbl Neurochir. 1999;  60 172-182
• 29 Koning TJ de, Gooskens R, Veenhoven R, Meijboom EJ, Jansen GH, Lasjaunias P, Vries LS de. Arteriovenous malformation of the vein of Galen in three neonates: emphasis on associated early ischemic brain damage.  Eur J Pediatr. 1997;  156 228-229
• 30 Lasjaunias P, Rodesch G, terBrugge K, Pruvost P, DeVictor D, Comoy J, Landrieu P. Vein of Galen aneurysmal malformations. Report of 36 cases managed between 1982 and 1988.  Acta Neurochir. 1989;  99 26-37
• 31 Lasjaunias P, Garcia-Monaco R, Rodesch G, Ter Brugge K, Zerah M, Tardieu M, de Victor D. Vein of Galen malformation. Endovascular management of 43 cases.  Childs Nerv Syst. 1991;  7 360-367
• 32 Lasjaunias P, Hui F, Zerah M, Garcia-Monaco R, Malherbe V, Rodesch G, Tanaka A, Alvarez H. Cerebral arteriovenous malformations in children. Management of 179 consecutive cases and review of the literature.  Child`s Nerv Syst. 1995;  11 66-79
• 33 Lasjaunias P, Alvarez H, Rodesch G, Garcia-Monaco R, ter Brugge K, Burrows P, Taylor W. Aneurysmal malformations of the vein of Galen. Follow up of 120 children treated between 1984 and 1994.  Interventional Neuroradiology. 1996;  2 15-26
• 34 Lasjaunias P. Vascular Diseases in Neonates, Infants and Children. Springer, 1.Auflage 1999
• 35 Lylyk P, Vinuela F, Dion JE, Duckwiler G, Guglielmi G, Peacock W, Martin N. Therapeutic alternatives for vein of Galen vascular malformations.  J Neurosurg. 1993;  78 438-445
• 36 Meyers PM, Halbach V Van, Phatouros CP, Dowd CF, Malek AM, Lempert TE, Lefler JE, Higashida RT. Hemorrhagic Complications in Vein of Galen Malformations.  Ann Neurol. 2000;  47 748-755
• 37 Mitchell PJ, Rosenfeld JV, Dargaville P, Loughnan P, Ditchfield MR, Frawley G, Tress BM. Endovascular Management of Vein of Galen Aneurysmal Malformations Presenting in the Neonatal Period.  Am J Neuroradiol. 2001;  22 1403-1409
• 38 Nakano S, Agid R, Klurfan P, Peruzzo dos Santos Souza M, Armstrong D, terBrugge KG. Limitations and technical considerations of endovascular treatment in neonates with high-flow arteriovenous shunts presenting with congestive heart failure: report of two cases.  Childs Nerv Syst. 2006;  22 13-17
• 39 Nass R, Kramer E, Molofsky W, Melnick J, Hollisey M de, Madrid M, Wisoff JH, Berenstein A. Perfusion brain scinitigraphy studies in infants and children with malformations of the vein of Galen.  Childs Nerv Syst. 2001;  17 519-523
• 40 Paternoster DM, Manganelli F, Moroder W, Nicolini U. Prenatal Diagnosis of Vein of Galen Aneurysmal Malformations.  Fetal Diagn Ther. 2003;  18 408-411
• 41 Rayboud CA, Strother CM, Hald JK. Aneurysma of the vein of Galen: embryonic considerations and anatomical features relating to the pathogenesisof the malformation.  Neuroradiology. 1989;  31 109-128
• 42 Reith W, Shamdeen MG. Vaskuläre Erkrankungen bei Neugeborenen, Säuglingen und Kindern.  Der Radiologe. 2003;  43 934-947
• 43 Rodesch G, Hui F, Alvarez H, Tanaka A, Lasjaunias P. Prognosis of antenatally diagnosed vein of Galen aneurysmal malformations.  Childs Nerv Syst. 1994;  10 79-83
• 44 De Victor D, Le Pommelet C. Medical Treatment of Congestive Cardiac Failure from Cerebral Arteriovenous Fistulas at Bicetre Hospital.  Interventional Neuroradiology. 1996;  2 49-52
• 45 Vijayaraghavan SB, Vijay S, Kala MR, Neha D. Prenatal diagnosis of thrombosed aneurysm of vein of Galen.  Ultrasound Obstet Gynecol. 2006;  27 81-83
• 46 Yanaka K, Hyodo A, Nose T. Venous malformation serving as the draining vein of an adjoining arteriovenous malformation.  Surg Neurol. 2001;  56 170-174
• 47 Zerah M, Garcia-Monaco R, Rodesch G, terBrugge K, Tardieu M, DeVictor D, Lasjaunias P. Hydrodynamics in vein of Galen malformations.  Childs Nerv Syst. 1992;  8 111-117

Korrespondenzadresse