Establishing Standards of Care in Craniosynostosis: Results from a Survey of ERN CRANIO Member Institutions.

BACKGROUND

European Reference Networks (ERNs) are networks involving hospitals with particular expertise in rare conditions. ERN-CRANIO focuses on rare disorders of the skull and face including craniosynostosis.

METHODS

We undertook a pilot study in the form of an electronic survey to understand current practice in craniosynostosis management across ERN-CRANIO, which at the time consisted of 29 member institutions across 11 countries. Most (19 of 29) units replied; however, some answered the survey only partially.

RESULTS

The majority (87.5%) of units have specific management protocols. For single-suture cases, 8 of 15 units see >50 new cases per year, 4 of 15 see 21-50 cases, and 3 of 15 see fewer than 20 cases. Duration of follow-up ranges from age 10 years or less (3 of 6) to indefinite (2 of 6). A variety of surgical techniques are used. For sagittal synostosis, the endoscopic and helmet technique is the most common (5 of 14), and for metopic and unicoronal synostosis, preferred by 9 of 14 centers was the frontoorbital remodeling (bandeau) technique. For multisutural syndromic craniosynostosis, 2 of 16 centers see >20 new cases per year, 4 of 16 see 11-20 cases, 5 of 16 see 6-10 cases, and 4 of 16 see fewer than 5 cases. Most centers (12 of 15) either never discharge syndromic patients or follow until adulthood. Eleven of 14 units perform prophylactic vault expansion, while 3 of 14 wait for clinical indication. Nine of 13 units operate at 6-12 months. Again, a wide variety of techniques are used, most commonly frontal advancement (4 of 13) initially.

CONCLUSIONS

This study provides a useful snapshot of current standards of care in craniosynostosis across the high-volume centers of the ERN. Going forward, these results can be used to direct more detailed analysis of current practice, which will then be useful for constructing a management guideline for patients presenting with both single-suture and multisutural craniosynostosis.