Outcomes of Chiari Malformation III: A Review of Literature

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.