ABSTRAK

Kasus: Diagnosis: Dandy-Walker Syndrome

Ny H, 31 tahun : G5P3A1 gravid 29-30 minggu belum inpartu + suspek dandy walker. Dari
hasil screening didapatkan hasil USG obstetric yang abnormal berupa ventricolomegaly
communicans, macroglia, polihidramion. Dilakukan induksi persalinan dan tidak berhasil.
Dilanjutkan dengan operasi sesar dan didapakan hasil luaran bayi perempuan, Berat Badan 1200
gram, Panjang Badan 37 cm, Apgar score 0/0
Diskusi: Sindrom Dandy Walker adalah malformasi intrakranial bawaan langka yang
terdiri dari spektrum kelainan fossa posterior yang diklasifikasikan sebagai (a) Dandy-Walker
malformasi (dilatasi kistik ventrikel 4, lengkap atau agenesis sebagian dari vermis serebelar dan
fossa posterior yang membesar) (b) Dandy-Walker varian (massa kistik fossa posterior dengan
hipoplasia variabel vermis serebelar dan tidak ada pembesaran fossa posterior) dan (c)
Megacisterna magna (cisterna magna yang membesar dengan vermis serebelar normal dan
ventrikel keempat normal) (d) Blakes Pouch Cyst adalah membesar seperti balon ke bagian
posterior dari bagian superior velum medular kedalam sisterna magna. Diagnosis prenatal
sonografi klasik sindrom Dandy-Walker sederhana, namun diagnosis definitif Dandy-Walker
varian dan Megacisterna magna sulit sebelum 18 minggu kehamilan. Malformasi Dandy-Walker
sering dikaitkan dengan anomali intrakranial lain seperti agenesis corpus callosum,
holoprosencephaly, encephaloceles oksipital dan kelainan okular. Anomali ekstra-kranial
termasuk ginjal polikistik, cacat jantung, polydactyly dan sumbing langit-langit.
Kesimpulan: Manajemen bedah Sindrom Dandy Walker dianggap sebagai linipertama,
yang terdiri dari shunting cystoperitoneal dan endoscopic third ventriculostomy (ETV). Janin
yang memiliki ciri seperti sindrom Dandy Walker berdasarkan hasil pemeriksaan penunjang dan
memiliki kemungkinan hidup sebaiknya dilakukan tindakan bedah dan dilanjutkan tindakan
konservatif.
Kata kunci: Sindrom Dandy-Walker; Hidrosefalus; Ventrikulomegali; Makroglia;
Polihidramion

ABSTRACT
Case: Diagnose: Dandy-Walker Syndrome
Mrs H, 31 yo : G5P3A1 gravid 29-30 weeks yet inpartu + suspected dandy walker. From
the results of screening ultrasound obstetric showed abnormal form of ventricolomegaly
communicans, macroglia, polihidramion. Induction of labor performed and did not succeed.
Followed by cesarean section and then the outcome baby girls, Weight 1200 gram, Body Length
37 cm, Apgar score 0/0.
Discussion: The Dandy-Walker complex is a rare congenital intracranial malformation that
comprises a spectrum of abnormalities of the posterior fossa which are classified as (a) DandyWalker malformation (cystic dilatation of the 4th ventricle, complete or partial agenesis of the
cerebellar vermis and an enlarged posterior fossa) (b) Dandy-Walker variant (cystic posterior
fossa mass with variable hypoplasia of the cerebellar vermis and no enlargement of the posterior
fossa) and (c) Megacisterna magna (enlarged cisterna magna with normal cerebellar vermis and
fourth ventricle) (d) Blakes Pouch Cyst is a posterior ballooning of the superior medullary
velum into the cisterna magna. Classic sonographic prenatal diagnosis of Dandy-Walker
syndrome is a simple, yet definitive diagnosis of Dandy-Walker variant and Megacisterna magna
difficult before 18 weeks of gestation. Dandy-Walker malformation is often associated with other
intracranial anomalies such as agenesis of the corpus callosum, holoprosencephaly,
encephaloceles occipital and ocular disorders. Extra-cranial anomalies including polycystic
kidney, heart defects, polydactyly and cleft palate.
Conclusion: Surgical Management of Dandy Walker Syndrome is considered as fisrt line,
which consists of shunting cystoperitoneal and endoscopic third ventriculostomy (ETV). Fetus
has a characteristic such as Dandy Walker syndrome is based on the results of the investigation
and have the possibility of living should be performed surgery and continued conservative
management.
Keywords: Dandy-Walker Syndrome; Hydrocephalus; Ventriculomegaly; Macroglia;
Polyhidramion.