Ann Rehabil Med.  2014 Jun;38(3):401-404. 10.5535/arm.2014.38.3.401.

Arnold-Chiari Malformation Type III With Meningoencephalocele: A Case Report

• Jeong DH
• Kim CH
• Kim MO
• Chung H
• Kim TH
• Jung HY

• KMID: 2165760
• DOI: http://doi.org/10.5535/arm.2014.38.3.401

Abstract

Arnold-Chiari malformation type III (CM III) is an extremely rare anomaly with poor prognosis. An encephalocele with brain anomalies as seen in CM II, and herniation of posterior fossa contents like the cerebellum are found in CM III. The female infant was a twin, born at 33 weeks, weighing 1.7 kg with a huge hydrocele on the craniocervical junction. After operations were performed, she was referred to the department of rehabilitation medicine for poor motor development, swallowing dysfunction, and poor eye fixation at 22 months. The child was managed with neurodevelopmental treatment, oromotor facilitation, and light perception training. After 14 months, improvement of gross motor function was observed, including more stable head control, rolling, and improvement of visual perception. CM III has been known as a condition with poor prognosis. However, with the improvement in operative techniques and intensive rehabilitations, the prognosis is more promising than ever before. Therefore, more attention must be paid to the rehabilitation issues concerning patients with CM III.

Keyword

Chiari III malformation; Developmental disabilities; Cortical blindness; Rehabilitation

MeSH Terms

Arnold-Chiari Malformation*
Blindness, Cortical
Brain
Cerebellum
Child
Deglutition
Developmental Disabilities
Encephalocele
Female
Head
Humans
Infant
Prognosis
Rehabilitation
Twins
Visual Perception

Figure

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  Fig. 1 (A) T1-weighted axial view and (B) T2-weighted sagittal view of brain magnetic resonance imaging at birth show a large sac with cerebellar herniation through the occipital area.

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  Fig. 2 (A) Photograph and (B) T1-weighted sagittal view of brain magnetic resonance imaging at 22 months show meningocele at the craniocervical junction with hydrocephalus after removal of the hydrocele.

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  Fig. 3 Visual perception training with penlight for cortical visual impairment in progress.

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