Long-term outcome in 415 operated cases with single suture scaphocephaly

There are multiple options available for the surgical management of non-syndromic scaphocephaly. The goals of surgery are to correct the cranial deformity in order to optimize overall cosmetic appearance and to prevent raised intracranial pressure (ICP). At Alder Hey Children's Hospital NHS Trust, we offer a range of surgical procedures for single-suture scaphocephaly (SSS) from endoscopic strip craniectomy (ESC) with helmet therapy, sagittal suturectomy, and barrel staving - known as passive vault remodeling (PassiveVR), to an active subtotal (SVR) or total vault remodeling (TVR).

We conducted a retrospective analysis of all operated scaphocephaly patients at Alder Hey. All consecutive patients from 2012 to 2023 were included. Syndromic or multisuture synostosis cases were excluded. Age, type of surgery, issues of ICP at diagnosis, cephalic index (CI) pre- and postoperative, type and indication for reoperation, and length of follow-up were gathered in the database.

Four hundred fifteen patients were included; 200 underwent PassiveVR, 206 underwent SVR/TVR, 8 underwent ESC + helmet, and one was treated with springs. The median follow-up period was 46 months. Eighteen patients underwent treatment due to high ICP related to synostosis, with the median age at surgery being 54 months in this group. Reoperations for intracranial hypertension or persistent abnormal shape were done for 17 patients treated initially with PassiveVR (8.5%), for 4 patients initially treated with SVR/TVR (1.9%), and for 1 patient initially treated with ESC + helmet (12.5%). Postoperative CI was significantly higher than preoperative CI in all operated patients (paired t-testing, p < 0.01).

PassiveVR and SVR/TVR are the most common procedures in our centre to treat scaphocephaly, with PassiveVR being performed earlier in life. In less than 10% of PassiveVR cases, reoperation due to increased ICP or abnormal head shape is required, with the benefit of PassiveVR being less invasive than SVR/TVR. Our experience with ESC is limited at present, but preliminary findings from this study suggest the need for long-term follow-up in order to identify early evidence of raised ICP or persistent deformity in this patient subgroup.