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Purpose: Hydrocephalic macrocephaly is exceeding rare. When macrocephaly occurs, it can be associated with motor developmental delays (primarily from an increased head weight), which can challenge caregivers and impair quality of life. Prior publications (largest series = 4 patients) have described subtotal cranial vault reduction procedures, but no studies have addressed postoperative functional outcomes. We sought to evaluate our series of macrocephaly repairs, which entailed a more limited cranioplasty approach (designed to provide the maximum benefit with the smallest possible procedure), in order to assess outcomes and improve care.
Methods: Medical records were retrospectively reviewed for outcome data and complications. Pre- and postoperative anthropologic measurements were reviewed. A six-point functional scale was designed to assess gross functional outcomes. Data from our series was compared with previously published data.
Results: Over a 12-year period, 10 patients (mean age of 18-months) underwent reduction cranioplasties utilizing a limited reduction technique. Average EBL = 530cc (vs. 1014cc, literature average), the mean PICU and hospital length of stays were 1.3 and 2.6 days, respectively (vs. 10 and 16 days, literature average). The mean length of follow up was 41.5 months. There were 3 complications (30%), one fluid collection and two infections (one requiring shunt revision). The average head circumference reduction was 1.6 cm, with greater reductions in posterior skull height noted. The mean functional assessment scores increased from 2.3 to 3.9 postoperatively, with 100% of patients who were unable to lift their heads unsupported preoperatively, were independently able to do so postoperatively. In comparison with previously reported series, our average length of hospitalization was over a week shorter.
Conclusions: Performance of a limited reduction cranioplasty can result in measurable functional improvements postoperatively. This smaller procedure was associated with marked reductions in transfusion amounts, lengths of both surgery and hospitalization, and shunt revision rates, but it did not appear to reduce complication rates. In this largest series presented to date, we found that more limited procedure effectively improved postoperative function, while reducing hospital length of stay. Nevertheless a longer-term assessment of both function and secondary operations will be needed to fully evaluate the efficacy of performing a limited cranioplasties for the treatment of hydrocephalic macrocephaly.