1.A Case of Dandy-Walker Syndrome with Chromosomal Abnormality.
Hyui Sung CHANG ; Seok Kyu LEE ; Gi Chung LEE ; Woo Ki LEE ; Kwang Woo KIM
Journal of the Korean Pediatric Society 1994;37(12):1784-1788
The Dandy-Walker syndrome is a developmental disorders of the brain characterized by cystic deformity of the 4th ventricle and agensis of the cerebellar vermis. Other systemic anomalies and chromosomal abnormalities are associated with this syndrome. We are experienced a case in a 9 months old male infant who presented initially with frequent vomiting, low birth weight, On the physical examination, a prominent occiput, palpable mass below the right upper quadrant, pulmonary valve stenosis, congenital dislocation of the hips, ventral flexion of fingers, clubfoots and the rocker-bottom deformities of feet were present. On the chromosomal study, there were chromosomal polymorphisms in a thickened C-band of chromosome No. 1 by C-banding method. The brain CT revealed a large, thin-walled, low density mass of CSF without enhancement in the posterior fossa, showing upward displacement of cerebellar hemisphere with absent inferior vermis(or associated with dysplastic cerebellar hemisphere). A brief review of the related literatures were included in this report.
Brain
;
Chromosome Aberrations*
;
Clubfoot
;
Congenital Abnormalities
;
Dandy-Walker Syndrome*
;
Dislocations
;
Fingers
;
Foot
;
Hip
;
Humans
;
Infant
;
Infant, Low Birth Weight
;
Infant, Newborn
;
Male
;
Physical Examination
;
Pulmonary Valve Stenosis
;
Vomiting
2.Two cases of Arnold-Chiari malformation type II.
Woo Ki LEE ; Hyui Sung CHANG ; Seok Kyu LEE ; Ewng Won PARK ; Kwang Woo KIM
Journal of the Korean Pediatric Society 1993;36(2):287-291
Arnold-Chirai malformation type II is congenital disorder which consists of downward displacement into upper cervical spinal canal of parts of the cerebellum, 4th ventricle, and medulla oblongata. We experienced two cases of Arnold-Chiari malformation with lumbar meningomyelocele, hydrocephalus. We confirmed the cases by brain C-T and report with brieft review of the lilterature.
Arnold-Chiari Malformation*
;
Brain
;
Cerebellum
;
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
;
Hydrocephalus
;
Medulla Oblongata
;
Meningomyelocele
;
Spinal Canal