Pediatrics & Neonatology
Volume 49, Issue 2 , Pages 43-47, April 2008

Hydranencephaly Associated with Interruption of Bilateral Internal Carotid Arteries

  • Yeak-Wun Quek

      Affiliations

    • Division of Genetics, Department of Pediatrics, Chung Shan Medical University Hospital, Taichung, Taiwan
    • Division of Neonatology, Department of Pediatrics, Chung Shan Medical University Hospital, Taichung, Taiwan
    • ,
  • Pen-Hua Su

      Affiliations

    • Division of Genetics, Department of Pediatrics, Chung Shan Medical University Hospital, Taichung, Taiwan
    • Division of Neonatology, Department of Pediatrics, Chung Shan Medical University Hospital, Taichung, Taiwan
    • Institute of Medicine, Chung Shan Medical University, Taichung, Taiwan
    • Corresponding Author InformationCorresponding author. Division of Genetics, Department of Pediatrics, Chung Shan Medical University Hospital, 110 Chien-Kuo North Road, Section 1, Taichung 402, Taiwan
    • ,
  • Teng-Fu Tsao

      Affiliations

    • Department of Diagnostic Radiology, Chung Shan Medical University Hospital, Taichung, Taiwan
    • ,
  • Jia-Yuh Chen

      Affiliations

    • Division of Genetics, Department of Pediatrics, Chung Shan Medical University Hospital, Taichung, Taiwan
    • Division of Neonatology, Department of Pediatrics, Chung Shan Medical University Hospital, Taichung, Taiwan
    • ,
  • Yan-Yan Ng

      Affiliations

    • Division of Genetics, Department of Pediatrics, Chung Shan Medical University Hospital, Taichung, Taiwan
    • Division of Neonatology, Department of Pediatrics, Chung Shan Medical University Hospital, Taichung, Taiwan
    • ,
  • Jui-Ming Hu

      Affiliations

    • Division of Genetics, Department of Pediatrics, Chung Shan Medical University Hospital, Taichung, Taiwan
    • Division of Neonatology, Department of Pediatrics, Chung Shan Medical University Hospital, Taichung, Taiwan
    • ,
  • Suh-Jen Chen

      Affiliations

    • Division of Genetics, Department of Pediatrics, Chung Shan Medical University Hospital, Taichung, Taiwan
    • Division of Neonatology, Department of Pediatrics, Chung Shan Medical University Hospital, Taichung, Taiwan

Received 30 August 2007; received in revised form 26 January 2008; accepted 25 March 2008.

Article Outline

Hydranencephaly is a rare and fatal central nervous system disorder where all or nearly all of the bilateral cerebral hemispheres are absent. The extensive hollow cerebrum is replaced with cerebrospinal fluid. Clinically, the differential diagnoses of hydranencephaly include severe hydrocephalus and alobar holoprosencephaly. Nearly all cases are sporadic, involving approximately 1 in 5000 continuing pregnancies. The exact main cause is still unknown, but hydranencephaly is usually found to develop secondarily to the occlusion of cerebral arteries above the supraclinoid level. We present the case of a 1-month-old male infant with hydranencephaly initially thought to be severely hydrocephalus via routine antenatal intrauterine sonography performed at 35 weeks of gestation. Hydranencephaly was confirmed by brain sonography, brain magnetic resonance imaging and magnetic resonance angiography postnatally. We discuss several imaging features that are helpful in distinguishing hydranencephaly from extreme hydrocephaly. Different theories that have been recently proposed regarding the origin of hydranencephaly are reviewed.

KEY WORDS:  hydranencephaly , hydrocephalus , magnetic resonance angiography , magnetic resonance imaging , sonography

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References 

  1. Romero R , Pilu G , Jeanty P , Ghidini A , Hobbins JC . Prenatal Diagnosis of Congenital Anomalies . Norwalk, CT: Appleton & Lange; 1988;
  2. Mori F , Nishie M , Tanno K , Sannohe S , Kuroda N , Wakabayashi K . Hydranencephaly with extensive periventricular necrosis and numerous ectopic glioneuronal nests . Neuropathology . 2004;24:315–319
  3. Csabay L , Szabo I , Papp C , Toth-Pal E , Papp Z . Central nervous system anomalies . Ann NY Acad Sci . 1998;847:21–45
  4. McAbee GN , Chan A , Erde EL . Prolonged survival in hydranencephaly: report of two patients and literature review . Pediatr Neurol . 2000;23:80–84
  5. Barozzino T , Sgro M . Transillumination of the neonatal skull: seeing the light . CMAJ . 2002;167:1271–1272
  6. Jordan L , Raymond R , Lin D , Gailloud P . Imaging casebook. CT angiography in a newborn child with hydranencephaly . J Perinatol . 2004;24:565–567
  7. Hochmuth A , Wentz K-U . MR-angiographic findings in a case of hydranencephaly: correlation of vascular and MR-tomographic features . Clin Neuroradiol . 2003;13:84–87
  8. La Torre E , Occhipinti E . Cerebral angiography in hydranencephaly with report of three cases . Eur Neurol . 1969;2:184–191
  9. Levin A , Kutznetova L , Kahana R , Rubinstein-Guini M , Stram Y . Highly effective inhibition of Akabane virus replication by siRNA genes . Virus Res . 2006;120:121–127
  10. Mittelbronn M , Beschorner R , Schittenhelm J , et al.   Multiple thromboembolic events in fetofetal transfusion syndrome in triplets contributing to the understanding of pathogenesis of hydranencephaly in combination with polymicrogyria . Hum Pathol . 2006;37:1503–1507
  11. Halsey JH , Allen N , Chamberlin HR . The morphogenesis of hydranencephaly . J Neurol Sci . 1971;12:187–217
  12. Halsey JH , Allen N , Chamberlin HR . Hydranencephaly . In:  Vinken PJ ,  Bruyn GW ,  Klawans H editor. Handbook of Clinical Neurology . Volume 30: Amsterdam: Elsevier Science; 1987;p. 337–353

PII: S1875-9572(08)60011-X

doi:10.1016/S1875-9572(08)60011-X

Pediatrics & Neonatology
Volume 49, Issue 2 , Pages 43-47, April 2008

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