The failure rate of ventriculoperitoneal shunts (VPSs) for hydrocephalus in patients with achondroplasia is greater than in other hydrocephalus populations. However, the cause of hydrocephalus in this group is considered “communicating,” Hence, endoscopic third ventriculostomy may not be an option (ETV). Despite this, ETV is effective in a small proportion of achondroplasia patients. The researchers wanted to see how ETV affected this demographic in the long run. Achondroplasia patients who had received hydrocephalus surgical therapy (ETV or VPS placement) were identified. Medical records and neuroimages were reviewed in patients who had undergone ETV to estimate ventricular sizes and frontal and occipital horn ratios (FOHRs) before and after surgery, as well as the prevalence of surgical complications and reoperation. Patients who had VPS implants were included in the study for historical comparison. They examined medical records for baseline demographic information, surgical complications, and reoperation prevalence.
About 19 (17%) of the 114 pediatric patients with achondroplasia who were referred for neurosurgical consultation were treated for hydrocephalus; 10 patients received only ETV, 7 patients received only VPS placement, and 2 patients received VPS implantation followed by ETV. If assessed at birth, ventricular volume and FOHRs in patients treated with ETV were normal but rose dramatically until the ETV. With observation lasting up to 15 years after ETV, all patients showed significant and sustained reductions in ventricular measures. Between the ETV and VPS cohorts, there was a statistically significant difference in rates of repeat CSF surgery (0/12 vs 7/9, p < 0.001). ETV was found to be effective, safe, and long-lasting in treating hydrocephalus in achondroplasia patients. Although several studies have shown that these individuals’ hydrocephalus is “communicating,” some of them may develop an “obstructive” component that is progressive and sensitive to ETV.
