Management of isolated sagittal synostosis in the absence of scaphocephaly: a series of eight cases.

Background: The diagnosis of isolated sagittal synostosis is usually made at birth, with a boat-shaped deformity of the skull (scaphocephaly). The Oxford Craniofacial Unit has successfully managed eight cases of isolated sagittal synostosis without scaphocephaly. To the authors' knowledge, there have been no reports of this in the literature.

Methods: The Oxford Craniofacial Unit database (set up in 2004) was searched. This identified 193 cases of isolated sagittal synostosis, eight of which did not have a scaphocephalic head shape (i.e., a cephalic index <76).

Results: Patients with isolated sagittal synostosis in the absence of scaphocephaly represent 4.1 percent of all isolated sagittal synostosis patients in the authors' database. Intracranial pressure was measured in six of the eight cases and found to be high in four of these (67 percent).

Conclusions: It is unclear why the secondary features of sagittal synostosis did not develop in these cases. Possible explanations include fusion of the suture late after the majority of skull growth has taken place and a patent metopic suture allowing growth to occur anteriorly; other sutures appeared patent radiologically but may not have been functioning normally. Although difficult to recognize, patients with isolated sagittal synostosis in the absence of scaphocephaly are important to identify because they are at high risk of developing raised intracranial pressure. A new management algorithm for treatment of these cases is described that has been used successfully in the Oxford Craniofacial Unit.