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.

Removal of a penetrating orbital and anterior fossa foreign body using an eyebrow incision.

In this case, we present an underutilised eyebrow approach for removing penetrating foreign bodies of the orbit extending into the anterior fossa floor. Excellent visualisation of the sub-frontal region is achieved and a large trauma craniotomy is avoided, but care must be taken to preserve the supra-orbital and fronto-temporal nerves.