Three tenets for staged correction of Kleeblattschädel or cloverleaf skull deformity

Abstract

Background: Patients with cloverleaf skull deformity are known to have high morbidity and poor outcome. Physical anomalies include a misshapen, trilobar skull, with a high "bossed" forehead, a bulging temporal region, and a flat posterior skull from multiple cranial suture fusion.

Methods: Patients with cloverleaf skull deformity treated at the University of California, Los Angeles from 1990 to 2006 (n = 14) underwent early cranial vault remodeling (group 1) or staged correction with ventriculoperitoneal shunt (neonate), fronto-orbital advancement (3 to 6 months), and posterior vault remodeling (1 year) (group 2). Morbidity, necessary revisions, and neurologic (developmental testing) and aesthetic (Whitaker score) outcomes were assessed at follow-up.

Results: Diagnoses included Apert syndrome, Crouzon syndrome, Pfeiffer syndrome, Saethre-Chotzen syndrome, and nonsyndromic. Early cranial vault remodeling patients had more complications than staged correction patients [pneumonia, meningitis, and excessive bleeding (each 66 percent versus 9 percent); wound infection (66 percent versus 18 percent); and seizure (100 percent versus 0 percent)]. Early cranial vault remodeling patients had prolonged intensive care unit and hospital stays compared with staged correction patients (13 versus 2 days and 27 versus 5 days, respectively). The Whitaker score showed acceptable results at 18 months in group 2 (1.4, no revisions necessary) but not in group 1 (2.8, minor to major bony recontouring). Developmental tests showed that all early cranial vault remodeling patients had lower scores in both preschool tests and global evaluations compared with normative data (mean preschool receptive scores, 95 versus 85; mean preschool expressive scores, 94 versus 87).

Conclusion: Staged correction of cloverleaf skull provided acceptable neurologic and aesthetic outcomes in the authors' series of patients.