Endoscopic surgery for patients with syndromic craniosynostosis and the requirement for additional open surgery

Author:

Hersh David S.1,Hoover-Fong Julie E.23,Beck Natalie23,Dorafshar Amir H.4,Ahn Edward S.56

Affiliation:

1. Department of Neurosurgery, University of Maryland School of Medicine; and

2. McKusick-Nathans Institute of Genetic Medicine,

3. Greenberg Center for Skeletal Dysplasias,

4. Department of Plastic and Reconstructive Surgery, and

5. Department of Pediatrics,

6. Division of Pediatric Neurosurgery, Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland

Abstract

OBJECTIVERecent reports have described early endoscopic suturectomy as a treatment option for patients with syndromic craniosynostosis, but such patients often require subsequent calvarial remodeling. The authors describe their experience with this patient population and seek to identify predictors of sufficiency of endoscopic surgery alone.METHODSThe medical records of patients with syndromic craniosynostosis who underwent endoscopic repair were retrospectively reviewed. Demographic data, operative details, and follow-up data were collected.RESULTSA total of 6 patients with syndromic craniosynostosis underwent endoscopic surgery followed by helmet therapy during the study period. Of these, 3 patients were male. The involved syndromes included Crouzon, Pfeiffer, Jackson-Weiss, Muenke, Saethre-Chotzen, and craniosynostosis-3 (n = 1 each). The patients underwent endoscopic surgery at a median age of 2.1 months (range 0.9–4.1 months). The median estimated blood loss was 30 ml (range 20–100 ml), with 2 patients requiring a transfusion. The median length of stay in the hospital was 1.5 days (range 1–4 days), and the median follow-up was 29.0 months (range 16.8–81.7 months), with 1 patient (16.7%) requiring an open revision. Three patients (50%) were classified as Whitaker Category I at the last follow-up. The patients for whom additional open surgery was performed or recommended (Whitaker Category IV) were the oldest patients in the cohort, ranging from 2.6 to 4.1 months at the time of surgery.CONCLUSIONSThis series demonstrates that endoscopic surgery can be sufficient to treat syndromic craniosynostosis without subsequent open calvarial remodeling over a median follow-up period of at least 2 years. The findings suggest that younger age at the time of endoscopic surgery may be an important factor in determining the sufficiency of this procedure. Even among patients who require subsequent open calvarial remodeling, early endoscopic surgery may allow for growth and development of the brain and skull while delaying the need for open remodeling until the patient is older and can better tolerate the procedure.

Publisher

Journal of Neurosurgery Publishing Group (JNSPG)

Subject

General Medicine

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