Download PDF
Am J Perinatol 2000; 17(8): 405-410
DOI: 10.1055/s-2000-13456
Copyright © 2000 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

PERSISTENT PULMONARY HYPERTENSION OF THE NEWBORN PRESENTING AS THE PRIMARY MANIFESTATION OF INTRACRANIAL ARTERIOVENOUS MALFORMATION OF THE VEIN OF GALEN

Leonora Hendson1 , Derek J. Emery2 , Ernest Z. Phillipos1 , Ravi Bhargava2 , Peter M. Olley3 , Robert P. Lemke1
  • 1Neonatal Intensive Care Unit, University of Alberta Hospital, Edmonton, Alberta, Canada
  • 2Department of Radiology, University of Alberta Hospital, Edmonton, Alberta, Canada and
  • 3Department of Pediatric Cardiology, University of Alberta Hospital, Edmonton, Alberta, Canada
Further Information

Publication History

Publication Date:
31 December 2000 (online)

    Permissions and Reprints

ABSTRACT

Arteriovenous malformations of the Vein of Galen continue to present diagnostic and therapeutic challenges in the neonatal period. Approximately 40-50% of all malformations of the Vein of Galen present in the neonatal period, usually with congestive heart failure. These neonates represent the most severe cases and are also the most difficult to manage. We report a case of a neonate with a Vein of Galen Malformation who presented with cyanosis, a cardiac murmur, and severe persistent pulmonary hypertension of the newborn. Cardiac failure developed later in the patient's course. The degree of pulmonary hypertension on echocardiography was used to time endovascular embolization of the Vein of Galen Malformation. Following embolization, his pulmonary hypertension subsided dramatically. We speculate that pulmonary hypertension associated with Vein of Galen Malformations has been underestimated in the morbidity and demise of these neonates, and should be more aggressively monitored and treated.

KEYWORD

Arteriovenous malformation - Vein of Galen - persistent pulmonary hypertension of the newborn - endovascular embolization

REFERENCES

  • 1 Volpe J J. Brain tumors and Vein of Galen malformations. In: Volpe JJ, ed. Neurology of the Newborn 3rd ed. Philadelphia: WB Saunders 1995: 802-806
    Google Scholar
  • 2 Halbach V V, Barkovich A J. Anomalies of the cerebral vasculature. In: Barkovich AJ, ed. Pediatric Neuroimaging 2nd ed. New York: Raven Press 1995: 619-627
    Google Scholar
  • 3 Horowitz M B, Jungreis C A, Quisling R G, Pollack I. Vein of Galen aneurysms: a review and current perspective.  AJNR . 1994;  15 1486-1496
    PubMedGoogle Scholar
  • 4 Raybaud C A, Strother C M, Hald J K. Aneurysms of the Vein of Galen: embryonic considerations and anatomical features relating to the pathogenesis of the malformation.  Neuroradiology . 1989;  31 109-128
    CrossrefPubMedGoogle Scholar
  • 5 Cumming G R. Circulation in neonates with intracranial arteriovenous fistula and cardiac failure.  Am J Cardiol . 1980;  45 1019-1024
    CrossrefPubMedGoogle Scholar
  • 6 Seidenwurm D, Berenstein A, Hyman A, Kowlaski H. Vein of Galen Malformation: correlation of clinical presentation, arteriography, and MR Imaging.  AJNR . 1991;  12 347-354
    PubMedGoogle Scholar
  • 7 Burrrows P E, Robertson R L. Neonatal central nervous system vascular disorders.  Neurosurg Clin N Am . 1998;  9 155-180
    PubMedGoogle Scholar
  • 8 Lasjaunias P, Rodesch G, Terbrugge K. Vein of Galen aneurysm malformations: report of 36 cases managed between 1982 and 1988.  Acta Neurochir . 1989;  99 26-37
    CrossrefPubMedGoogle Scholar
  • 9 Dowd C F, Halbach V V, Barnwell S L. Transfemoral venous embolization of vein of Galen malformations.  AJNR . 1990;  11 643-648
    PubMedGoogle Scholar
  • 10 Mickle J P. The transtorcular embolization of vein of Galen aneurysms and update on the use of this technique in twenty-four patients. In: Marlin AE, ed. Concepts in Pediatric Neurosurgery Vol. 11. Basel: Karger 1991: 69-78
    Google Scholar
  • 11 Lylyk P, Vinuela F, Dion J E. Therapeutic alternatives for vein of Galen vascular malformations.  J Neurosurg . 1993;  78 438-445
    CrossrefPubMedGoogle Scholar
  • 12 Friedman D M, Verma R, Madrid M. Recent improvement in outcome using transcatheter embolization techniques for neonatal aneurysmal malformation of the Vein of Galen.  Pediatrics . 1993;  91 583-586
    PubMedGoogle Scholar
  • 13 Stanbridge R DL, Westaby S, Smallhorn J, Taylor J FN. Intracranial arteriovenous malformation with aneurysm of the Vein of Galen as a cause of heart failure in infancy: echocardiographic diagnosis and results of treatment.  Br Heart J . 1983;  49 157-163
    PubMedGoogle Scholar
  • 14 Starc T J, Krongrad E, Bierman F Z. Two-dimensional echocardiography and Doppler finding in cerebral arteriovenous malformation.  Am J Cardiol . 1989;  64 252-254
    CrossrefPubMedGoogle Scholar
  • 15 Holden A M, Fyler D C, Shillito J, Nadas A S. Congestive heart failure from intracranial arteriovenous fistula in infancy.  Pediatrics . 1972;  49 30-39
    PubMedGoogle Scholar
  • 16 Pellegrino P A, Milanes O, Saia O, Carollo C. Congestive heart failure secondary to cerebral arteriovenous fistula.  Childs Nerv Syst . 1987;  3 141-144
    CrossrefPubMedGoogle Scholar
  • 17 Selzer G, Rettwitz-Volk W, Schloesser R. Persistent pulmonary hypertension in the newborn caused by aneurysm of the vein of Galen.  Klin Padiatr . 1993;  205 190-193
    Article in Thieme ConnectPubMedGoogle Scholar
  • 18 Huh J, Noh C I, Choi J Y, Yun Y S. Pulmonary hypertensive crisis as an initial manifestation of intracranial arteriovenous malformation with aneurysm of the Vein of Galen.  Int J Cardiol . 1998;  66 107-109
    CrossrefPubMedGoogle Scholar
      >