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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Definitions
Destruction of cerebral hemispheres
IMAGING
General Features
Ultrasonographic Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Cecchetto G et al: Looking at the missing brain: hydranencephaly case series and literature review. Pediatr Neurol. 48(2):152-8, 2013
Ghosh PS et al: Fetal magnetic resonance imaging in hydranencephaly. J Paediatr Child Health. 49(4):335-6, 2013
Sepulveda W et al: Prenatal sonography in hydranencephaly: findings during the early stages of disease. J Ultrasound Med. 31(5):799-804, 2012
Meyer E et al: Mutations in FLVCR2 are associated with proliferative vasculopathy and hydranencephaly-hydrocephaly syndrome (Fowler syndrome). Am J Hum Genet. 86(3):471-8, 2010
Thomas S et al: High-throughput sequencing of a 4.1 Mb linkage interval reveals FLVCR2 deletions and mutations in lethal cerebral vasculopathy. Hum Mutat. 31(10):1134-41, 2010
Vaneckova M et al: Post-mortem magnetic resonance imaging and its irreplaceable role in determining CNS malformation (hydranencephaly)--case report. Brain Dev. 32(5):417-20, 2010
Bae JS et al: Prolonged survival to adulthood of an individual with hydranencephaly. Clin Neurol Neurosurg. 110(3):307-9, 2008
Merker B: Life expectancy in hydranencephaly. Clin Neurol Neurosurg. 110(3):213-4, 2008
Quek YW et al: Hydranencephaly associated with interruption of bilateral internal carotid arteries. Pediatr Neonatol. 49(2):43-7, 2008
Tsai JD et al: Hydranencephaly in neonates. Pediatr Neonatol. 49(4):154-7, 2008
Watts P et al: Chorioretinal dysplasia, hydranencephaly, and intracranial calcifications: pseudo-TORCH or a new syndrome? Eye (Lond). 22(5):730-3, 2008
Werth R: Cerebral blindness and plasticity of the visual system in children. A review of visual capacities in patients with occipital lesions, hemispherectomy or hydranencephaly. Restor Neurol Neurosci. 26(4-5):377-89, 2008
Jordan L et al: CT angiography in a newborn child with hydranencephaly. J Perinatol. 24(9):565-7, 2004
Covington C et al: Prolonged survival in hydranencephaly: a case report. Tenn Med. 96(9):423-4, 2003
Hahn JS et al: Hydranencephaly owing to twin-twin transfusion: serial fetal ultrasonography and magnetic resonance imaging findings. J Child Neurol. 18(5):367-70, 2003
Laurichesse-Delmas H et al: First-trimester features of Fowler syndrome (hydrocephaly-hydranencephaly proliferative vasculopathy). Ultrasound Obstet Gynecol. 20(6):612-5, 2002
Wellons JC 3rd et al: Choroid plexectomy reduces neurosurgical intervention in patients with hydranencephaly. Pediatr Neurosurg. 36(3):148-52, 2002
Greco F et al: Hemihydranencephaly: case report and literature review. J Child Neurol. 16(3):218-21, 2001
Stevenson DA et al: Hydranencephaly in an infant with vascular malformations. Am J Med Genet. 104(4):295-8, 2001
Kavaslar GN et al: The novel genetic disorder microhydranencephaly maps to chromosome 16p13.3-12.1. Am J Hum Genet. 66(5):1705-9, 2000
Lam YH et al: Serial sonographic features of a fetus with hydranencephaly from 11 weeks to term. Ultrasound Obstet Gynecol. 16(1):77-9, 2000
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Definitions
Destruction of cerebral hemispheres
IMAGING
General Features
Ultrasonographic Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Cecchetto G et al: Looking at the missing brain: hydranencephaly case series and literature review. Pediatr Neurol. 48(2):152-8, 2013
Ghosh PS et al: Fetal magnetic resonance imaging in hydranencephaly. J Paediatr Child Health. 49(4):335-6, 2013
Sepulveda W et al: Prenatal sonography in hydranencephaly: findings during the early stages of disease. J Ultrasound Med. 31(5):799-804, 2012
Meyer E et al: Mutations in FLVCR2 are associated with proliferative vasculopathy and hydranencephaly-hydrocephaly syndrome (Fowler syndrome). Am J Hum Genet. 86(3):471-8, 2010
Thomas S et al: High-throughput sequencing of a 4.1 Mb linkage interval reveals FLVCR2 deletions and mutations in lethal cerebral vasculopathy. Hum Mutat. 31(10):1134-41, 2010
Vaneckova M et al: Post-mortem magnetic resonance imaging and its irreplaceable role in determining CNS malformation (hydranencephaly)--case report. Brain Dev. 32(5):417-20, 2010
Bae JS et al: Prolonged survival to adulthood of an individual with hydranencephaly. Clin Neurol Neurosurg. 110(3):307-9, 2008
Merker B: Life expectancy in hydranencephaly. Clin Neurol Neurosurg. 110(3):213-4, 2008
Quek YW et al: Hydranencephaly associated with interruption of bilateral internal carotid arteries. Pediatr Neonatol. 49(2):43-7, 2008
Tsai JD et al: Hydranencephaly in neonates. Pediatr Neonatol. 49(4):154-7, 2008
Watts P et al: Chorioretinal dysplasia, hydranencephaly, and intracranial calcifications: pseudo-TORCH or a new syndrome? Eye (Lond). 22(5):730-3, 2008
Werth R: Cerebral blindness and plasticity of the visual system in children. A review of visual capacities in patients with occipital lesions, hemispherectomy or hydranencephaly. Restor Neurol Neurosci. 26(4-5):377-89, 2008
Jordan L et al: CT angiography in a newborn child with hydranencephaly. J Perinatol. 24(9):565-7, 2004
Covington C et al: Prolonged survival in hydranencephaly: a case report. Tenn Med. 96(9):423-4, 2003
Hahn JS et al: Hydranencephaly owing to twin-twin transfusion: serial fetal ultrasonography and magnetic resonance imaging findings. J Child Neurol. 18(5):367-70, 2003
Laurichesse-Delmas H et al: First-trimester features of Fowler syndrome (hydrocephaly-hydranencephaly proliferative vasculopathy). Ultrasound Obstet Gynecol. 20(6):612-5, 2002
Wellons JC 3rd et al: Choroid plexectomy reduces neurosurgical intervention in patients with hydranencephaly. Pediatr Neurosurg. 36(3):148-52, 2002
Greco F et al: Hemihydranencephaly: case report and literature review. J Child Neurol. 16(3):218-21, 2001
Stevenson DA et al: Hydranencephaly in an infant with vascular malformations. Am J Med Genet. 104(4):295-8, 2001
Kavaslar GN et al: The novel genetic disorder microhydranencephaly maps to chromosome 16p13.3-12.1. Am J Hum Genet. 66(5):1705-9, 2000
Lam YH et al: Serial sonographic features of a fetus with hydranencephaly from 11 weeks to term. Ultrasound Obstet Gynecol. 16(1):77-9, 2000
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