J Korean Neurosurg Soc.  2022 Nov;65(6):841-845. 10.3340/jkns.2021.0234.

Chiari Type III : Experience of Outcome for 15 Cases

• Gabr M
• Elmataeshy M
• Abdullah AA

• KMID: 2535844
• DOI: http://doi.org/10.3340/jkns.2021.0234

Abstract

Objective
: Chiari III malformations are extremely rare hindbrain malformations that are associated with a high early mortality rate, or severe neurologic deficits in the survivors. The treatment is early operative closure and cerebrospinal fluid diversion (CSF) shunting.
Methods
: We operated on 15 patients by repair and excision between July 2014 till June 2020 and retrospective data collection was done. Only one patient doesn’t need ventriculoperitoneal (VP) shunt and the other 14 patients need a VP shunt. We described stepwise dissection and untethering of the cerebellum from the bony edge to regrowth and herniation of cerebellum again into this potential space and simple dural closure or repair with graft was done.
Results
: We started with VP in eight patients (53%) and the other seven patients (46.7%) started with excision and then six patients need VP shunt later on because four patients developed CSF leak and two patients developed increased high intracranial tension. Only four patients (26.7%) needed a blood transfusion.
Conclusion
: There are variations in the outcome and not all cases of Chiari malformation III will die and severe developmental delay is not a must. Proper management and repair carry a good prognosis.

Keyword

Chiari type III; Outcome; Experience

Figure

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  Fig. 1. A : computed tomography brain showing bony defect in posterior fossa wall with herniated cerebellum in sac. b : Magnetic resonance imaging (MRI) showing the sac. Arrows indicate herniated cerebellum. c : Axial MRI showing the encephalocele. Arrow indicates herniated cerebellum.

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  Fig. 2. Intraoperative photo showing the stepwise dissection in (A) and (b) showing reflected dura 1 and excision of contused cerebellum in 2. A : Arrow refer to the capsule of the sac asterisk showing the plane of dissection. b : 1 refer to dura and 2 refer to cerebellar tissue, arrow; refer to bony defect and origin of encephalocele, asterisk refer to dissected capsule.

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