Neuropediatrics 2004; 35 - P44
DOI: 10.1055/s-2004-819419

Prenatal multicystic encephalomalacia caused by an anomaly of the aortic arch

S Ehehalt 1, M Kehrer 1, 2, M Hofbeck 3, W Küker 4, I Krägeloh-Mann 1, M Schöning 1
  • 1Department of Child Neurology, University of Tuebingen
  • 2Department of Neonatology, University of Tuebingen
  • 3Department of Cardiology, Children's Hospital; University of Tuebingen
  • 4Department of Neuroradiology, University of Tuebingen, Germany

Introduction: Multicystic encephalomalacia (ME) is the typical clinical manifestation of severe hypoxic-ischemic brain damage during the late 3rd trimenon. The subclavian steal syndrome (SSS) is only rarely found in childhood; it may be caused by an anomaly of the aortic arch. This is the first report on a prenatal ME due to SSS.

Methods: Colour duplex sonography (CDS) of the aortic arch, the intracranial and extracranial vessels; MRI and MRA of the brain.

Case report: 5-day-old full term neonate, appropriate for gestational age. Microcephaly, overlapping cranial sutures. Both arms rosy and developed normally. Decreased blood pressure in the left arm. MRI of the brain: Bilateral ME, infratentorial parts normal. CDS: Atresia of the left subclavian artery; stenosis at the origin of left common carotid artery; left-sided SSS. Cerebral Blood Flow (CBF) volume: 22ml/min (reduced).

Conclusion: SSS allowed normal development of the left arm despite of atresia of the left subclavian artery. Consequently, however, cerebral perfusion was markedly reduced leading to extensive ME of brain parenchyma supplied by the anterior cerebral circulation; cerebellum and brain stem remained intact because of retrograde perfusion (“steal“) of the basilar artery. Postnatal CBF volume was severely reduced (normal data for term newborns, 69±14ml/min; cf., for preterms between 32 and 34 weeks, 33±9ml/min; Kehrer et al., in press). The characteristic pattern of ischemic brain lesions of the late 3rd trimenon points to an initially normal development and organisation of the brain before CBF volume fell below a critical level.

Keywords: subclavian steal syndrome, multicystic encephalomalacia, Doppler sonography