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Review
. 2020 Oct-Dec;15(4):358-364.
doi: 10.4103/jpn.JPN_135_19. Epub 2021 Jan 19.

Outcomes of Chiari Malformation III: A Review of Literature

Mohammad Elbaroody  1 , Hossam Eldin Mostafa  1 , Mohamed F M Alsawy  1 , Mohamed E Elhawary  2 , Ahmed Atallah  1 , Mohamed Gabr  1
Affiliations
Review

Outcomes of Chiari Malformation III: A Review of Literature

Mohammad Elbaroody et al. J Pediatr Neurosci. 2020 Oct-Dec.

Abstract

Purpose: Chiari malformation type III (CM III) is the rarest type compared to other types of CMs. CM III usually reported as sporadic case reports which reflect the rarity of this anomaly. We report two cases of operated CM III at our institute with a reasonable outcome and reviewed the literature to illustrate the variability of prognosis and related hydrocephalus.

Materials and methods: We operated two cases of CM III in our hospital followed by ventriculoperitoneal shunt (VPS) placement with an accepted neurological outcome at 10 and 6 months follow-up. We reviewed the literature for other cases of CM III with focusing on prognosis to illustrate the real image of reported prognosis and related hydrocephalus.

Results: After follow-up for 10 and 6 months, respectively, both cases had mild developmental delays. In this review, we report 51 cases of CM III over the last 30 years since 1989, there was slight male predilection, hydrocephalus was evident in 27 cases which was almost managed with VPSs and was evident in seven deaths.

Conclusion: CM III is a rare anomaly which usually carries a bad prognosis, but death is not ultimate, and there may be a minority who carry good prognosis. This bad prognosis pushes some parents to refuse surgery otherwise repair should be done. With good pre- and postoperative care, physical therapy, and follow-up, the outcome is reasonable.

Keywords: Chiari 3 malformation; Chiari III; encephalocoele; hindbrain; prognosis.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
(A) Occipital mass in first 4-month-old child. (B) MRI T2 sagittal view for the child showing herniation of cerebellum surrounded by CSF through low occipital defect
Figure 2
Figure 2
(A) Occipital mass in second 6-month-old child. (B) MRI T2 sagittal view showing herniation of cerebellum surrounded by CSF through low occipital defect and downward displacement of brain stem
Figure 3
Figure 3
Describe the cranial part of surgery. * indicates the cranial part, # indicates the caudal part toward the cervical spine, (1) points to normal cerebellum, (2) points to the bone of posterior fossa from inside, the red arrow points to the bony edges of posterior fossa after coagulation and removal of herniated cerebellum, which is the cut off point for our removal
See this image and copyright information in PMC

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