2015 HSC Section 1 Book of Articles

Reprinted by permission of Curr Opin Otolaryngol Head Neck Surg. 2012; 20(4):310-317.

C URRENT O PINION

Current approaches to management of nonsyndromic craniosynostosis

Haruko Okada and Arun K. Gosain

Purpose of review Surgical alternatives to traditional cranial vault remodeling for the treatment of craniosynostosis are being discussed in recent plastic and neurosurgical literature. This review highlights recent developments and discusses the risks as well as benefits of each. Recent findings Surgical treatment of craniosynostosis has evolved from simple suturectomy, to extensive cranial vault remodeling, and now back to the minimally invasive. Options today include endoscopic suturectomies, spring-mediated cranioplasties, and distraction osteogenesis as well as cranial vault remodeling. There are disagreements among centers on the most optimal timing and best operative procedure. Summary Clinicians should be aware that different surgical treatments are rapidly being developed for nonsyndromic craniosynostosis. Keywords cranial suture, cranial vault remodeling, cranioplasty, craniosynostosis, spring-mediated cranioplasty, strip craniectomy, suture

INTRODUCTION Craniosynostosis is the premature fusion of a cranial suture, which can occur in isolation or with an associated syndrome. Its prevalence is approxi- mately one in 2500 births [1]. The most commonly affected sutures are sagittal (40–55%) followed by coronal (20–25%), metopic (5–15%), and lambdoid (0–5%) [2]. More than one suture is affected in 5–15% of cases. In sagittal craniosynostosis, there is a four to one male to female predominance, whereas women outnumber men three to one in unilateral coronal synostosis [3]. There is no sex predominance in metopic craniosynostosis. Although the pathology is in the cranial vault, clinically the disorder affects the cranial base and facial bones as well. Patients with craniosynostosis can have elevated intracranial pressure, learning disabilities, midface hypoplasia, and speech impair- ments. The operative treatment is demanding and requires teamwork between neurosurgery, plastic surgery and anesthesiology. Therefore, such com- plex patients are best cared for in a craniofacial center with multidisciplinary coordination. PATHOGENESIS The human skull develops from a neural crest and mesodermal origin, at 23–26 days of gestation [4].

The cranial base, namely the occipital, sphenoid, ethmoid, and petrous temporal bones, is formed by endochondral ossification. The cranial vault is formed bymembranous ossification. Cranial growth is a passive response to the expanding brain and cerebrospinal fluid compartments. Cranial sutures allow head compression and bony overlap during birth, causing a deformation that may last weeks afterward, but quickly normalizes with rapid brain growth and subsequent cranial expansion. These patent cranial sutures are active sites of bone depo- sition and growth, accommodating rapid brain volume expansion in the first 3 years of life. The metopic suture is the first to close physiologically at 9 months and the sagittal suture closes last at 16 years [3]. Premature closure of sutures was first described by Sommering [5], and the concept that a single Department of Plastic Surgery, Case Western Reserve University, Cleveland, Ohio, USA Correspondence to Arun K. Gosain, MD, Department of Plastic Surgery, Case Western Reserve University, University Hospitals of Cleveland, MS 5044, 11100 Euclid Avenue, Cleveland, OH 44106, USA. Tel: +1 216 844 4780; e-mail: arun.gosain@uhhospitals.org Curr Opin Otolaryngol Head Neck Surg 2012, 20:310–317 DOI:10.1097/MOO.0b013e328355a869

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