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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Definitions
- Absence of cerebral hemispheres with intact thalamus, brainstem, cerebellum, skull/meninges
- Associated space filled with CSF
- Probably caused by in utero vascular occlusion, infection, or trauma after 1st trimester
- Hemihydranencephaly: Rare unilateral form
- Absence of cerebral hemispheres with intact thalamus, brainstem, cerebellum, skull/meninges
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic Findings
Other Modality Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
- Omar AT 2nd et al: Hydranencephaly complicated by central diabetes insipidus: report of two cases and systematic review of literature. Childs Nerv Syst. 35(7):1165-1171, 2019
- Kline-Fath BM et al: Fowler syndrome and fetal MRI findings: a genetic disorder mimicking hydranencephaly/hydrocephalus. Pediatr Radiol. 48(7):1032-34, 2018
- Pedrosa HAR et al: Choroid plexus cauterization on treatment of hydranencephaly and maximal hydrocephalus. Childs Nerv Syst. 33(9):1509-16, 2017
- Kim SY et al: Endoscopic coagulation of choroid plexus in hydranencephaly. J Korean Neurosurg Soc. 55(6):375-8, 2014
- Pavone P et al: Hydranencephaly: cerebral spinal fluid instead of cerebral mantles. Ital J Pediatr. 40(1):79, 2014
- Cecchetto G et al: Looking at the missing brain: hydranencephaly case series and literature review. Pediatr Neurol. 48(2):152-8, 2013
- Sepulveda W et al: Prenatal sonography in hydranencephaly: findings during the early stages of disease. J Ultrasound Med. 31(5):799-804, 2012
- Meuwissen ME et al: Sporadic COL4A1 mutations with extensive prenatal porencephaly resembling hydranencephaly. Neurology. 76(9):844-6, 2011
- 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
- Jordan L et al: CT angiography in a newborn child with hydranencephaly. J Perinatol. 24(9):565-7, 2004
- Sutton LN et al: Hydranencephaly versus maximal hydrocephalus: an important clinical distinction. Neurosurgery. 6(1):34-8, 1980
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
- Absence of cerebral hemispheres with intact thalamus, brainstem, cerebellum, skull/meninges
- Associated space filled with CSF
- Probably caused by in utero vascular occlusion, infection, or trauma after 1st trimester
- Hemihydranencephaly: Rare unilateral form
- Absence of cerebral hemispheres with intact thalamus, brainstem, cerebellum, skull/meninges
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic Findings
Other Modality Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
- Omar AT 2nd et al: Hydranencephaly complicated by central diabetes insipidus: report of two cases and systematic review of literature. Childs Nerv Syst. 35(7):1165-1171, 2019
- Kline-Fath BM et al: Fowler syndrome and fetal MRI findings: a genetic disorder mimicking hydranencephaly/hydrocephalus. Pediatr Radiol. 48(7):1032-34, 2018
- Pedrosa HAR et al: Choroid plexus cauterization on treatment of hydranencephaly and maximal hydrocephalus. Childs Nerv Syst. 33(9):1509-16, 2017
- Kim SY et al: Endoscopic coagulation of choroid plexus in hydranencephaly. J Korean Neurosurg Soc. 55(6):375-8, 2014
- Pavone P et al: Hydranencephaly: cerebral spinal fluid instead of cerebral mantles. Ital J Pediatr. 40(1):79, 2014
- Cecchetto G et al: Looking at the missing brain: hydranencephaly case series and literature review. Pediatr Neurol. 48(2):152-8, 2013
- Sepulveda W et al: Prenatal sonography in hydranencephaly: findings during the early stages of disease. J Ultrasound Med. 31(5):799-804, 2012
- Meuwissen ME et al: Sporadic COL4A1 mutations with extensive prenatal porencephaly resembling hydranencephaly. Neurology. 76(9):844-6, 2011
- 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
- Jordan L et al: CT angiography in a newborn child with hydranencephaly. J Perinatol. 24(9):565-7, 2004
- Sutton LN et al: Hydranencephaly versus maximal hydrocephalus: an important clinical distinction. Neurosurgery. 6(1):34-8, 1980
