BACKGROUND: Hydrocephalic macrocephaly is a rare problem that may result in cranial vault instability, difficulties with posture and movement, and hindrance in neurological development. The authors studied the outcomes of hydrocephalic macrocephalic cases in which patients underwent single-stage total cranial vault remodeling and postoperative helmet therapy. METHODS: Consecutive patients with hydrocephalic macrocephaly treated at University of California, Los Angeles from 2000 to 2007 were studied (n = 8). Perioperative examinations (head circumference), lateral cranial radiographs (anteroposterior, lateral, and cranial height measurements) and three-dimensional computed tomography scans (cranial volume) were used to access cranial reduction. Developmental testing, physician evaluations (Whitaker score), and parental surveys were used. RESULTS: Improvements in mean head circumference (from 58.8 cm to 48.9 cm, or 17 percent reduction), and cranial volume measurements (from 1924 cc to 1212 cc, or 35 percent reduction) were seen in patients after the procedure. In addition, developmental testing scores improved in half, or four of eight patients. Whitaker score was 1.9 with only one case requiring revision but five needing subsequent shunts. Surveys showed a high satisfaction rate with final appearance and ease of childcare. CONCLUSION: For the rare patient with hydrocephalic macrocephaly, treatment with total cranial vault remodeling with postoperative helmet therapy may be successful, but follow-up monitoring and subsequent ventriculoperitoneal shunting may be necessary.
BACKGROUND:Hydrocephalic macrocephaly is a rare problem that may result in cranial vault instability, difficulties with posture and movement, and hindrance in neurological development. The authors studied the outcomes of hydrocephalic macrocephalic cases in which patients underwent single-stage total cranial vault remodeling and postoperative helmet therapy. METHODS: Consecutive patients with hydrocephalic macrocephaly treated at University of California, Los Angeles from 2000 to 2007 were studied (n = 8). Perioperative examinations (head circumference), lateral cranial radiographs (anteroposterior, lateral, and cranial height measurements) and three-dimensional computed tomography scans (cranial volume) were used to access cranial reduction. Developmental testing, physician evaluations (Whitaker score), and parental surveys were used. RESULTS: Improvements in mean head circumference (from 58.8 cm to 48.9 cm, or 17 percent reduction), and cranial volume measurements (from 1924 cc to 1212 cc, or 35 percent reduction) were seen in patients after the procedure. In addition, developmental testing scores improved in half, or four of eight patients. Whitaker score was 1.9 with only one case requiring revision but five needing subsequent shunts. Surveys showed a high satisfaction rate with final appearance and ease of childcare. CONCLUSION: For the rare patient with hydrocephalic macrocephaly, treatment with total cranial vault remodeling with postoperative helmet therapy may be successful, but follow-up monitoring and subsequent ventriculoperitoneal shunting may be necessary.