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HYDRANCEPHALY HYDROCEPHALIC ANENCEPHALY, HYDROENCEPHALODYSPLASIA, HYDROMERCENCEPHALY,  CYSTENCEPHALY MERCURY IMAGING INSTITUTE  SCO 172-173 SEC 9C  CHANDIGARH MERCURY IMAGING CENTRE  SCO 16-17 SEC 20D CHANDIGARH
HYDRANENCEPHALY ETIOLOGY Occlusion of  Bilateral ICA Supraclinoid part  or MCA on either side. Infection sequael Diffuse hypoxic ishemicsequael. Thromboblastic  material  from the deceased co twin   DIAGNOSTIC FEATURES  Cortical mantle abscent Normal brain parenchyma  is liquefied and lined by leptomeninges. Basal ganglia / brain stem  intact ( may be atrophic). Cerebellum intact  in most  of the  cases. (Primarily structures supplied with Posterior circulation).
OCCLUSION OF   SUPRACLIONID  ICA -ACL / MCL  LEADING TO LIQUEFACTION  OF BRAIN IN THE  MENTIONED ARTERIAL TERRITORIES  SPARING  OF PCL AND TERRITORY SUPPLIE.D BY IT
FALX INTACT / HYPOPLASTIC CEREBELLUM INTACT BRAIN STEM – INTACT / HYPOPLASTIC THALAMI – USUALLY INTACT. NO CORTICAL MANTLE  BRAIN STEM INTACT  THALAMI INTACCT LIQUEFIED BRAIN WITH LEPTOMENINGES LINING
DIFFERENTIALS      In all the mentioned cases cortical mantle is preserved. Hydrancephaly – Liquefied brain lined with  leptomeninges.      Hydrocephalus : Csf lining is of ependyma. DIFFERENTIALS Extreme Hydrocephalus Alobarholoprosencephaly Severe form of porencephaly
Hydro is water compressing the brain, as noted on the brain.Hydra is the existence of water, where brain matter is non existent.  Most patients do however have a brain stem allowing them to function normally.  The prognosis is that a slim percent will see their first birthday.  The majority of babies born with this disorder, expire.
PRESENT  CASE  2  & ½ MONTH                 MALE CHILD                                    WITH MACROCRANIA
INTACT CEREBELLUM/ATROPHIC BRAIN STEM  LIQUEFACTION OF THE BRAIN PARENCHYMA
NO CORTICAL MANTLE PERIPHERAL MENINGEAL COVERING SEEN  IHF INTACT
NO CORTICAL MANTLE PERIPHERAL MENINGEAL COVERING PRESENT   CEREBELLUM INTACT  BRAINSTEM PRESENT
VAULT SECTIONS IHF PRESENT

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Hydrancephaly

  • 1. HYDRANCEPHALY HYDROCEPHALIC ANENCEPHALY, HYDROENCEPHALODYSPLASIA, HYDROMERCENCEPHALY, CYSTENCEPHALY MERCURY IMAGING INSTITUTE SCO 172-173 SEC 9C CHANDIGARH MERCURY IMAGING CENTRE SCO 16-17 SEC 20D CHANDIGARH
  • 2. HYDRANENCEPHALY ETIOLOGY Occlusion of Bilateral ICA Supraclinoid part or MCA on either side. Infection sequael Diffuse hypoxic ishemicsequael. Thromboblastic material from the deceased co twin DIAGNOSTIC FEATURES Cortical mantle abscent Normal brain parenchyma is liquefied and lined by leptomeninges. Basal ganglia / brain stem intact ( may be atrophic). Cerebellum intact in most of the cases. (Primarily structures supplied with Posterior circulation).
  • 3. OCCLUSION OF SUPRACLIONID ICA -ACL / MCL LEADING TO LIQUEFACTION OF BRAIN IN THE MENTIONED ARTERIAL TERRITORIES SPARING OF PCL AND TERRITORY SUPPLIE.D BY IT
  • 4. FALX INTACT / HYPOPLASTIC CEREBELLUM INTACT BRAIN STEM – INTACT / HYPOPLASTIC THALAMI – USUALLY INTACT. NO CORTICAL MANTLE BRAIN STEM INTACT THALAMI INTACCT LIQUEFIED BRAIN WITH LEPTOMENINGES LINING
  • 5. DIFFERENTIALS In all the mentioned cases cortical mantle is preserved. Hydrancephaly – Liquefied brain lined with leptomeninges. Hydrocephalus : Csf lining is of ependyma. DIFFERENTIALS Extreme Hydrocephalus Alobarholoprosencephaly Severe form of porencephaly
  • 6. Hydro is water compressing the brain, as noted on the brain.Hydra is the existence of water, where brain matter is non existent.  Most patients do however have a brain stem allowing them to function normally.  The prognosis is that a slim percent will see their first birthday.  The majority of babies born with this disorder, expire.
  • 7. PRESENT CASE 2 & ½ MONTH MALE CHILD WITH MACROCRANIA
  • 8. INTACT CEREBELLUM/ATROPHIC BRAIN STEM LIQUEFACTION OF THE BRAIN PARENCHYMA
  • 9. NO CORTICAL MANTLE PERIPHERAL MENINGEAL COVERING SEEN IHF INTACT
  • 10. NO CORTICAL MANTLE PERIPHERAL MENINGEAL COVERING PRESENT CEREBELLUM INTACT BRAINSTEM PRESENT