Porencephaly
ABDUL GOFAR SASTRODININGRAT
NEUROSURGERY
LECTURE NOTES
2012
USU Press
Art Design, Publishing & Printing
Gedung F
Jl. Universitas No. 9, Kampus USU
Medan, Indonesia
Telp. 061-8213737; Fax 061-8213737
Kunjungi kami di:
http://usupress.usu.ac.id
¤ USU Press 2012
Hak cipta dilindungi oleh undang-undang; dilarang memperbanyak, menyalin, merekam sebagian atau
seluruh bagian buku ini dalam bahasa atau bentuk apapun tanpa izin tertulis dari penerbit.
ISBN: 979 458 641 2
Perpustakaan Nasional: Katalog Dalam Terbitan (KDT)
Abdul Gofar Sastrodiningrat
Neurosurgery Lecture Notes / Abdul Gofar Sastrodiningrat – Medan: USU Press, 2012
Chief Editor :
Prof. Dr. Abdul Gofar Sastrodiningrat, SpBS(K)
Ilustrator :
Donny Luis
Ahmad Brata Rosa
A Tok
Cover Designer :
Gatot Aji Prihartomo
xiii, 928 p.: ilus. ; 29 cm.
Bibliografi, Indeks.
ISBN: 979-458-641-2
Percetakan:
USU PRESS - MEDAN
Isi buku diluar tanggung jawab percetakan
Sponsor Pencetakan:
PT. KALBE FARMA TBK
DAFTAR ISI
PART I: ADVANCED TOPICS IN NEUROSURGERY
Neurotransmitter
Beny Atmadja Wiryomartani ............................................................................................................................... 3
Excitatory Amino Acid Excitotoxicity
Abdul Gofar Sastrodiningrat.............................................................................................................................. 19
Endoscopic Third Ventriculostomy
Sri Maliawan...................................................................................................................................................... 31
Neuroendoscopy
Julius July ........................................................................................................................................................... 37
Epilepsy Surgery in Indonesia: Achieving Better Result with Limited Resources
Zainal Muttaqin ................................................................................................................................................. 54
Indications and Presurgical Evaluation For Epilepsy Surgery
Zainal Muttaqin ................................................................................................................................................. 62
Neuroimaging in Epilepsy : MRI evaluation in Refractory Complex Partial Epilepsy
Zainal Muttaqin ................................................................................................................................................ 72
Overview Meningioma: Histology and Molecular Biology
Iskandar Japardi ................................................................................................................................................ 80
Supraclavicular Approach on Brachial Plexus Injury
Adril Arsyad Hakim .......................................................................................................................................... 103
Cerebral Revascularization. Extracranial – Intracranial by-pass Surgery
Rr.Suzy Indharty .............................................................................................................................................. 109
PART II: BASIC NEUROSURGERY
CEREBRAL TRAUMA
Traumatic Brain Injury : Primary Brain Damage, Secondary Brain Damage, Management and Neuro
Critical Care
Abdul Gofar Sastrodiningrat............................................................................................................................ 125
Chronic Subdural Hemorrhage
Sonny G. R Saragih, Abdurrahman Mouza, Marsal Risfandi ......................................................................... 185
ix
SPINE
SPINE TRAUMA
SPINE TRAUMA : Arguments toward better care and patients safety
Abdul Gofar Sastrodiningrat .............................................................................................................. 205
Cervical Spine Trauma
Donny Luis, Abdul Gofar Sastrodiningrat .......................................................................................... 210
Thoracolumbar Trauma
Donny Luis, Abdul Gofar Sastrodiningrat .......................................................................................... 243
SPINE TUMOR
Spine Tumors
Donny Luis, Sabri Ibrahim .................................................................................................................. 267
Extradural Benign Tumor
Donny Luis, Gatot Aji Prihartomo ..................................................................................................... 271
Epidural Malignant Tumors
Donny Luis, Abdul Gofar Sastrodiningart .......................................................................................... 278
Intradural Extramedullary Benign Tumors
Donny Luis, Sabri Ibrahim .................................................................................................................. 293
Intradural Extramedullary Malignant Tumors
Donny Luis, Gatot Aji Prihartomo ...................................................................................................... 319
Intramedullary Tumors
Donny Luis, Abdul Gofar Sastrodiningrat .......................................................................................... 312
DEGENERATIVE DISEASE OF THE SPINE
Concept of Disc Degeneration and Regeneration
Donny Luis, Abdul Gofar Sastrodiningrat .......................................................................................... 327
Ossification Of The Posterior Longitudinal Ligament
Donny Luis, Abdul Gofar Sastrodiningrat .......................................................................................... 345
Cauda Equina Syndrome
Sonny G. R Saragih, Gatot Aji Prihartomo, Michael Norman Jusman .............................................. 353
Degenerative Disorder of the Cervical Spine
Donny Luis, Sabri Ibrahim, Michael Norman Jusman, Abdul Gofar Sastrodiningrat ......................... 368
Degenerative Disorders of the Thoracic Spine
Donny Luis, Sabri Ibrahim, Michael Norman Jusman, Abdul Gofar Sastrodiningrat ......................... 396
Degenerative Disorder of The Lumbar Spine
Donny Luis, Abdul Gofar Sastrodiningrat .......................................................................................... 409
x
CEREBRAL TUMOR
En Plaque Meningioma
Sonny G.R.Saragih ........................................................................................................................................... 439
Parasagital and Falx Meningioma
Sonny G.R.Saragih, Iskandar Japardi ............................................................................................................... 447
Petroclival Meningioma
Sonny G. R. Saragih, Abdul Gofar Sastrodiningrat ........................................................................................ 459
Tentorial Meningioma
Sonny G. R Saragih, Iskandar Japardi ............................................................................................................. 480
Low Grade Glioma
Andre Marolop Siahaan, Sony G.R.Saragih, Abdul Gofar Sastrodiningrat ...................................................... 488
High Grade Glioma
Andre Marolop Siahaan, Adril Arsyad Hakim .................................................................................................. 497
Fibrous Dysplasia
Ahmad Brata Rosa, Iskandar Japardi .............................................................................................................. 505
Medulloblastoma
Sabri Ibrahim, Donny Luis, Muhammad Fadhli, Adril Arsyad Hakim .............................................................. 511
Oligodendroglioma
Ahmad Brata, Sonny G.R.Saragih, Abdul Gofar Sastrodiningrat ..................................................................... 523
Ependymoma
Thomas Tommy, Abdul Gofar Sastrodiningrat ................................................................................................ 528
Pontine Glioma
Sonny G.R.Saragih, Abdul Gofar Sastrodiningrat ............................................................................................ 537
Metastatic Cerebral Tumor
Sabri Ibrahim, Iskandar Japardi ....................................................................................................................... 586
Histiocytosis X
Sonny G. R. Saragih, Abdul Gofar Sasrodiningrat, ....................................................................................... 598
CEREBRAL INFECTION
Cerebral Abscess
Sonny G. R. Saragih, Steven Tandean, Marshal Risfandi, Abdul Gofar Sastrodiningrat ............................... 597
xi
Cerebral Tuberculoma
Sabri Ibrahim, Sonny G.R.Saragih, Abdul Gofar Sastrodiningrat .................................................................... 619
Toxoplasmosis
Michel Norman Jusman, Sonny G.R.Saragih, Rr.Suzy Indharty ...................................................................... 638
Cerebral Aspergillosis
Donny Luis, Muhammad Fadhli, Alvin Abrar Harahap, Abdul Gofar Sastrodiningrat .................................... 649
Neurocysticercosis
Michael Norman Jusman, T. Yose Mahmuddin Akbar, Rr.Suzy Indharty ....................................................... 660
PEDIATRIC NEUROSURGERY
Hydrocephalus In Children
Sabri Ibrahim, Ahmad Brata Rosa, Ade Ricky Harahap .................................................................................. 671
Hydrancephaly
Gatot Aji Prihartomo,Disfahan Sinulingga ..................................................................................................... 683
Porencephaly
Thomas Tommy, Rr.Suzy Indharty .................................................................................................................. 687
Dandy Walker Malformation
Andre MP.Siahaan,Thomas Tommy, Disfahan Sinulingga, Adril Arsyad Hakim ............................................ 691
Chiari Malformation
Donny Luis, Disfahan Sinulingga, Adril Arsyad Hakim .................................................................................... 704
Craniosynostosis
Ahmad Brata Rosa, Sonny G.R. Saragih, Adril Arsyad Hakim ......................................................................... 721
Neural Tube Defect: Schizencephaly, Lissencephaly, Holoprosencephaly
Thomas Tommy, Donny Luis, Iskandar Japardi .............................................................................................. 735
Encephalocele, Myelomeningocele, Spina Bifida Oculta
Thomas Tommy, Rr.Suzy Indharty .................................................................................................................. 740
Occult Spinal Dysraphism
Sonny G. R Saragih, Donny Luis, Abdul Gofar Sastrodiningrat ....................................................................... 747
NEUROVASCULAR
Carotid-Cavernous Fistula
Muhammad Chairul, Rr. Suzy Indharty........................................................................................................... 767
xii
Arterio Venous Malformation
Sabri Ibrahim, Sonny G.R. Saragih, Abdul Gofar Sastrodiningrat .................................................................... 773
Intracranial Aneurysm
Muhammad Chairul, Sabri Ibrahim, Rr.Suzy Indharty ..................................................................................... 807
Spontaneous Intracerebral Hemorrhage
Michael Norman Jusman, Muhammad Fadhli, Abdul Gofar Sastrodiningrat ................................................. 820
PERIPHERAL NERVE
Carpal Tunnel Syndrome
Disfahan Sinulingga, Adril Arsyad Hakim ........................................................................................................ 833
Peripheral Nerve Injury
Marsal Risfandi, Ade Ricky Harahap, Adril Arsyad Hakim............................................................................... 846
Peripheral Nerve Tumor
Sonny G.R.Saragih, Ahmad Brata Rosa, Andre Marolop Siahaan, Abdul Gofar Sastrodiningrat .................... 855
MISCELLANEOUS
Intracranial Pressure
Donny Luis, Michael Norman Jusman ............................................................................................................. 887
Normal Pressure Hydrocephalus
Gatot Aji Prihartomo ....................................................................................................................................... 896
Pain Syndrome
Marshal Risfandi, Abdul Gofar Sastrodiningrat.............................................................................................. 908
Neurocutaneous Syndrome (Phakomatoses)
Sonny R. G. Saragih, Donny Luis, Michael Norman Jusman, Abdul Gofar Sastrodiningrat ............................. 922
INDEKS ............................................................................................................................................................. 958
xiii
Neurosurgery Lecture Notes
PORENCEPHALY
Thomas Tommy, Rr.Suzy Indharty
Pendahuluan
Porencephaly pertama kali dikenalkan oleh
Heschl1, dengan definisi :
the cerebral substance is lacking through the entire
thickness of brain and so, if one disregards the purely
membranous parts which fill the defect, there is a
canal through the brain which begins on the outer
surface of the brain and ends in the cerebral
e t i les .
Porencephaly adalah kejadian neurologis
yang langka pada anak-anak2 yang dikenal dengan
porecephalic cyst dan secara sederhana
didefinisikan sebagai suatu kecacatan dimana
terjadi sebuah hubungan antara ventrikel dengan
ruang subarachnoid atau suatu lesi yang terisi
cairan pada parenkim otak3. Pada saat ini,
terminologi porencephaly digunakan untuk
mendeskripsikan lesi intrakranial yang terisi cairan
(encephalomalacia) pada otak fetus atau neonatus
yang dapat berhubungan secara langsung dengan
sistem ventrikel atau dipisahakan oleh sebuah
membran tipis dan pada bagian luar dilapisi oleh
membran arachnoid4. Kista porensefali dapat
terjadi tunggal maupun multipel. Kista tersebut
kadang terobservasi sebagai ensefalomalacia
multikistik atau sebagai hidraensefali5.
Porensefali termasuk kedalam patologi
neural tube defects post neurulasi6 dan dapat
dibedakan menjadi tipe developmental dan tipe
kongenital. Tipe developmental dapat dibagi lebih
lanjut
menjadi
:
schizencephaly
(true
porencephaly), simple porencephaly, congenital
midline porencephaly. Tipe kongenital dapat terjadi
congenital encephaloclastic porencephaly.
Patogenesis
Porencephaly seringkali diakibatkan oleh
infark vaskular atau kejadian setelah perdarahan
intraserebral atau trauma tembus (termasuk
pungsi
ventrikel
berulang)6,
perdarahan
periventrikular, perdarahan plasenta, toxemia
maternal, leukomalacia periventrikular kistik,
perdarahan intrakranial dan atrofi serebral,
ensefalitis prenatal dan postnatal, meningitis,
penyakit paru kronis, penggunaan steroid untuk
penyakit paru kronis, necrotizing enterocolitis,
infeksi virus, endotoksin dan penyalahgunaan
obat5, serta defisiensi faktor C dan mutasi faktor V
Leiden7.
Terdapat dua lesi otak kongenital yang
berhubungan dengan porensefali. Pertama adalah
porensefali
genetik
akibat
gangguan
perkembangan sewaktu migrasi neuronal awal dan
yang kedua adalah porensefali encephaloclastic
(Tipe I) yang terjadi pada masa prenatal akhir atau
lesi vaskular perinatal (ensefalopati hipoksikiskemik) akibat stroke iskemik atau trombosis
sinus5 yang dikarakterisasi oleh lesi substansia alba
serebral dan keberadaan kavitas degeneratif2.
Penyebab dari porencephaly kongenital
sebagian besar disebabkan oleh infark iskemik yang
kemudian membentuk suatu rongga, dan biasanya
berada pada daerah yang diperdarahi oleh arteri
serebri media. Lesi serupa dapat terjadi akibat
nekrosis otak setelah infeksi prenatal.
Hipoksia yang terjadi sekitar 1-3 jam dapat
menyebabkan kerusakan otak dan defisit pada bayi
baru lahir, khususnya pasien dengan asidosis berat
(pH
NEUROSURGERY
LECTURE NOTES
2012
USU Press
Art Design, Publishing & Printing
Gedung F
Jl. Universitas No. 9, Kampus USU
Medan, Indonesia
Telp. 061-8213737; Fax 061-8213737
Kunjungi kami di:
http://usupress.usu.ac.id
¤ USU Press 2012
Hak cipta dilindungi oleh undang-undang; dilarang memperbanyak, menyalin, merekam sebagian atau
seluruh bagian buku ini dalam bahasa atau bentuk apapun tanpa izin tertulis dari penerbit.
ISBN: 979 458 641 2
Perpustakaan Nasional: Katalog Dalam Terbitan (KDT)
Abdul Gofar Sastrodiningrat
Neurosurgery Lecture Notes / Abdul Gofar Sastrodiningrat – Medan: USU Press, 2012
Chief Editor :
Prof. Dr. Abdul Gofar Sastrodiningrat, SpBS(K)
Ilustrator :
Donny Luis
Ahmad Brata Rosa
A Tok
Cover Designer :
Gatot Aji Prihartomo
xiii, 928 p.: ilus. ; 29 cm.
Bibliografi, Indeks.
ISBN: 979-458-641-2
Percetakan:
USU PRESS - MEDAN
Isi buku diluar tanggung jawab percetakan
Sponsor Pencetakan:
PT. KALBE FARMA TBK
DAFTAR ISI
PART I: ADVANCED TOPICS IN NEUROSURGERY
Neurotransmitter
Beny Atmadja Wiryomartani ............................................................................................................................... 3
Excitatory Amino Acid Excitotoxicity
Abdul Gofar Sastrodiningrat.............................................................................................................................. 19
Endoscopic Third Ventriculostomy
Sri Maliawan...................................................................................................................................................... 31
Neuroendoscopy
Julius July ........................................................................................................................................................... 37
Epilepsy Surgery in Indonesia: Achieving Better Result with Limited Resources
Zainal Muttaqin ................................................................................................................................................. 54
Indications and Presurgical Evaluation For Epilepsy Surgery
Zainal Muttaqin ................................................................................................................................................. 62
Neuroimaging in Epilepsy : MRI evaluation in Refractory Complex Partial Epilepsy
Zainal Muttaqin ................................................................................................................................................ 72
Overview Meningioma: Histology and Molecular Biology
Iskandar Japardi ................................................................................................................................................ 80
Supraclavicular Approach on Brachial Plexus Injury
Adril Arsyad Hakim .......................................................................................................................................... 103
Cerebral Revascularization. Extracranial – Intracranial by-pass Surgery
Rr.Suzy Indharty .............................................................................................................................................. 109
PART II: BASIC NEUROSURGERY
CEREBRAL TRAUMA
Traumatic Brain Injury : Primary Brain Damage, Secondary Brain Damage, Management and Neuro
Critical Care
Abdul Gofar Sastrodiningrat............................................................................................................................ 125
Chronic Subdural Hemorrhage
Sonny G. R Saragih, Abdurrahman Mouza, Marsal Risfandi ......................................................................... 185
ix
SPINE
SPINE TRAUMA
SPINE TRAUMA : Arguments toward better care and patients safety
Abdul Gofar Sastrodiningrat .............................................................................................................. 205
Cervical Spine Trauma
Donny Luis, Abdul Gofar Sastrodiningrat .......................................................................................... 210
Thoracolumbar Trauma
Donny Luis, Abdul Gofar Sastrodiningrat .......................................................................................... 243
SPINE TUMOR
Spine Tumors
Donny Luis, Sabri Ibrahim .................................................................................................................. 267
Extradural Benign Tumor
Donny Luis, Gatot Aji Prihartomo ..................................................................................................... 271
Epidural Malignant Tumors
Donny Luis, Abdul Gofar Sastrodiningart .......................................................................................... 278
Intradural Extramedullary Benign Tumors
Donny Luis, Sabri Ibrahim .................................................................................................................. 293
Intradural Extramedullary Malignant Tumors
Donny Luis, Gatot Aji Prihartomo ...................................................................................................... 319
Intramedullary Tumors
Donny Luis, Abdul Gofar Sastrodiningrat .......................................................................................... 312
DEGENERATIVE DISEASE OF THE SPINE
Concept of Disc Degeneration and Regeneration
Donny Luis, Abdul Gofar Sastrodiningrat .......................................................................................... 327
Ossification Of The Posterior Longitudinal Ligament
Donny Luis, Abdul Gofar Sastrodiningrat .......................................................................................... 345
Cauda Equina Syndrome
Sonny G. R Saragih, Gatot Aji Prihartomo, Michael Norman Jusman .............................................. 353
Degenerative Disorder of the Cervical Spine
Donny Luis, Sabri Ibrahim, Michael Norman Jusman, Abdul Gofar Sastrodiningrat ......................... 368
Degenerative Disorders of the Thoracic Spine
Donny Luis, Sabri Ibrahim, Michael Norman Jusman, Abdul Gofar Sastrodiningrat ......................... 396
Degenerative Disorder of The Lumbar Spine
Donny Luis, Abdul Gofar Sastrodiningrat .......................................................................................... 409
x
CEREBRAL TUMOR
En Plaque Meningioma
Sonny G.R.Saragih ........................................................................................................................................... 439
Parasagital and Falx Meningioma
Sonny G.R.Saragih, Iskandar Japardi ............................................................................................................... 447
Petroclival Meningioma
Sonny G. R. Saragih, Abdul Gofar Sastrodiningrat ........................................................................................ 459
Tentorial Meningioma
Sonny G. R Saragih, Iskandar Japardi ............................................................................................................. 480
Low Grade Glioma
Andre Marolop Siahaan, Sony G.R.Saragih, Abdul Gofar Sastrodiningrat ...................................................... 488
High Grade Glioma
Andre Marolop Siahaan, Adril Arsyad Hakim .................................................................................................. 497
Fibrous Dysplasia
Ahmad Brata Rosa, Iskandar Japardi .............................................................................................................. 505
Medulloblastoma
Sabri Ibrahim, Donny Luis, Muhammad Fadhli, Adril Arsyad Hakim .............................................................. 511
Oligodendroglioma
Ahmad Brata, Sonny G.R.Saragih, Abdul Gofar Sastrodiningrat ..................................................................... 523
Ependymoma
Thomas Tommy, Abdul Gofar Sastrodiningrat ................................................................................................ 528
Pontine Glioma
Sonny G.R.Saragih, Abdul Gofar Sastrodiningrat ............................................................................................ 537
Metastatic Cerebral Tumor
Sabri Ibrahim, Iskandar Japardi ....................................................................................................................... 586
Histiocytosis X
Sonny G. R. Saragih, Abdul Gofar Sasrodiningrat, ....................................................................................... 598
CEREBRAL INFECTION
Cerebral Abscess
Sonny G. R. Saragih, Steven Tandean, Marshal Risfandi, Abdul Gofar Sastrodiningrat ............................... 597
xi
Cerebral Tuberculoma
Sabri Ibrahim, Sonny G.R.Saragih, Abdul Gofar Sastrodiningrat .................................................................... 619
Toxoplasmosis
Michel Norman Jusman, Sonny G.R.Saragih, Rr.Suzy Indharty ...................................................................... 638
Cerebral Aspergillosis
Donny Luis, Muhammad Fadhli, Alvin Abrar Harahap, Abdul Gofar Sastrodiningrat .................................... 649
Neurocysticercosis
Michael Norman Jusman, T. Yose Mahmuddin Akbar, Rr.Suzy Indharty ....................................................... 660
PEDIATRIC NEUROSURGERY
Hydrocephalus In Children
Sabri Ibrahim, Ahmad Brata Rosa, Ade Ricky Harahap .................................................................................. 671
Hydrancephaly
Gatot Aji Prihartomo,Disfahan Sinulingga ..................................................................................................... 683
Porencephaly
Thomas Tommy, Rr.Suzy Indharty .................................................................................................................. 687
Dandy Walker Malformation
Andre MP.Siahaan,Thomas Tommy, Disfahan Sinulingga, Adril Arsyad Hakim ............................................ 691
Chiari Malformation
Donny Luis, Disfahan Sinulingga, Adril Arsyad Hakim .................................................................................... 704
Craniosynostosis
Ahmad Brata Rosa, Sonny G.R. Saragih, Adril Arsyad Hakim ......................................................................... 721
Neural Tube Defect: Schizencephaly, Lissencephaly, Holoprosencephaly
Thomas Tommy, Donny Luis, Iskandar Japardi .............................................................................................. 735
Encephalocele, Myelomeningocele, Spina Bifida Oculta
Thomas Tommy, Rr.Suzy Indharty .................................................................................................................. 740
Occult Spinal Dysraphism
Sonny G. R Saragih, Donny Luis, Abdul Gofar Sastrodiningrat ....................................................................... 747
NEUROVASCULAR
Carotid-Cavernous Fistula
Muhammad Chairul, Rr. Suzy Indharty........................................................................................................... 767
xii
Arterio Venous Malformation
Sabri Ibrahim, Sonny G.R. Saragih, Abdul Gofar Sastrodiningrat .................................................................... 773
Intracranial Aneurysm
Muhammad Chairul, Sabri Ibrahim, Rr.Suzy Indharty ..................................................................................... 807
Spontaneous Intracerebral Hemorrhage
Michael Norman Jusman, Muhammad Fadhli, Abdul Gofar Sastrodiningrat ................................................. 820
PERIPHERAL NERVE
Carpal Tunnel Syndrome
Disfahan Sinulingga, Adril Arsyad Hakim ........................................................................................................ 833
Peripheral Nerve Injury
Marsal Risfandi, Ade Ricky Harahap, Adril Arsyad Hakim............................................................................... 846
Peripheral Nerve Tumor
Sonny G.R.Saragih, Ahmad Brata Rosa, Andre Marolop Siahaan, Abdul Gofar Sastrodiningrat .................... 855
MISCELLANEOUS
Intracranial Pressure
Donny Luis, Michael Norman Jusman ............................................................................................................. 887
Normal Pressure Hydrocephalus
Gatot Aji Prihartomo ....................................................................................................................................... 896
Pain Syndrome
Marshal Risfandi, Abdul Gofar Sastrodiningrat.............................................................................................. 908
Neurocutaneous Syndrome (Phakomatoses)
Sonny R. G. Saragih, Donny Luis, Michael Norman Jusman, Abdul Gofar Sastrodiningrat ............................. 922
INDEKS ............................................................................................................................................................. 958
xiii
Neurosurgery Lecture Notes
PORENCEPHALY
Thomas Tommy, Rr.Suzy Indharty
Pendahuluan
Porencephaly pertama kali dikenalkan oleh
Heschl1, dengan definisi :
the cerebral substance is lacking through the entire
thickness of brain and so, if one disregards the purely
membranous parts which fill the defect, there is a
canal through the brain which begins on the outer
surface of the brain and ends in the cerebral
e t i les .
Porencephaly adalah kejadian neurologis
yang langka pada anak-anak2 yang dikenal dengan
porecephalic cyst dan secara sederhana
didefinisikan sebagai suatu kecacatan dimana
terjadi sebuah hubungan antara ventrikel dengan
ruang subarachnoid atau suatu lesi yang terisi
cairan pada parenkim otak3. Pada saat ini,
terminologi porencephaly digunakan untuk
mendeskripsikan lesi intrakranial yang terisi cairan
(encephalomalacia) pada otak fetus atau neonatus
yang dapat berhubungan secara langsung dengan
sistem ventrikel atau dipisahakan oleh sebuah
membran tipis dan pada bagian luar dilapisi oleh
membran arachnoid4. Kista porensefali dapat
terjadi tunggal maupun multipel. Kista tersebut
kadang terobservasi sebagai ensefalomalacia
multikistik atau sebagai hidraensefali5.
Porensefali termasuk kedalam patologi
neural tube defects post neurulasi6 dan dapat
dibedakan menjadi tipe developmental dan tipe
kongenital. Tipe developmental dapat dibagi lebih
lanjut
menjadi
:
schizencephaly
(true
porencephaly), simple porencephaly, congenital
midline porencephaly. Tipe kongenital dapat terjadi
congenital encephaloclastic porencephaly.
Patogenesis
Porencephaly seringkali diakibatkan oleh
infark vaskular atau kejadian setelah perdarahan
intraserebral atau trauma tembus (termasuk
pungsi
ventrikel
berulang)6,
perdarahan
periventrikular, perdarahan plasenta, toxemia
maternal, leukomalacia periventrikular kistik,
perdarahan intrakranial dan atrofi serebral,
ensefalitis prenatal dan postnatal, meningitis,
penyakit paru kronis, penggunaan steroid untuk
penyakit paru kronis, necrotizing enterocolitis,
infeksi virus, endotoksin dan penyalahgunaan
obat5, serta defisiensi faktor C dan mutasi faktor V
Leiden7.
Terdapat dua lesi otak kongenital yang
berhubungan dengan porensefali. Pertama adalah
porensefali
genetik
akibat
gangguan
perkembangan sewaktu migrasi neuronal awal dan
yang kedua adalah porensefali encephaloclastic
(Tipe I) yang terjadi pada masa prenatal akhir atau
lesi vaskular perinatal (ensefalopati hipoksikiskemik) akibat stroke iskemik atau trombosis
sinus5 yang dikarakterisasi oleh lesi substansia alba
serebral dan keberadaan kavitas degeneratif2.
Penyebab dari porencephaly kongenital
sebagian besar disebabkan oleh infark iskemik yang
kemudian membentuk suatu rongga, dan biasanya
berada pada daerah yang diperdarahi oleh arteri
serebri media. Lesi serupa dapat terjadi akibat
nekrosis otak setelah infeksi prenatal.
Hipoksia yang terjadi sekitar 1-3 jam dapat
menyebabkan kerusakan otak dan defisit pada bayi
baru lahir, khususnya pasien dengan asidosis berat
(pH