2015 HSC Section 1 Book of Articles

URRENT

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

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