**1. Introduction**

Craniosynostosis is a "developmental craniofacial anomaly, resulting in impairment of brain development and abnormally shaped skull" [1]. It involves premature fusion of the sutures of cranial vault in-utero. If untreated, over time craniosynostosis can cause significant cognitive and neurological impairments. Various diagnostic, surgical, and post-operative considerations should be appreciated in order to avoid unnecessary long-term systemic issues in patients with this condition.

This disorder can be classified into three different categories depending upon its formation:


Certain conditions can increase the risk at which a child can develop craniosynostosis such as "family history of abnormal head shapes, in utero exposure to teratogenic drugs, intrauterine restrains, or an abnormal fetal position, as well as any complications during pregnancy and any delayed milestones" [1]. As a result of this, it is essential to perform a thorough examination on the medical history of the patient.

Typically, craniosynostosis is a disorder that is diagnosable within the first year of life. Diagnosis is performed by a "clinical assessment that checks for its presence, whether or not the development of this disorder is a result of an associated syndrome, and if elective or urgent management is required" [1]. Common characteristics of the various classifications of craniosynostosis are summarized in **Table 2**. If surgical treatment is to be taken, then the diagnosis will be confirmed radiologically, either by computed tomography (CT), radiography, or magnetic resonance imaging (MRI). Radiography is the most common method used to confirm the presence of craniosynostosis as it is cost effective, does not require the use of anesthesia, and mitigates the risk of radiation exposure. One exception to the use of radiology to confirm the presence of craniosynostosis is when this disorder is classified as syndromic craniosynostosis. Genetic testing is used for "patients presenting coronal or multi-suture synostosis, since these two types are often genetically determined" [1].


#### **Table 1.**

*Gene involvements in syndromic craniosynostosis [2].*

*A Review of Current Surgical Approaches and Diagnostic Features Associated… DOI: http://dx.doi.org/10.5772/intechopen.108685*


#### **Table 2.**

*Common characteristics of types of craniosynostosis [1].*

Once the type of craniosynostosis is identified, there are two treatment options available:

One option is to perform an endoscopic suturectomy which is only performed on "patients less than six months of age because the bone is more flexible and manageable by an endoscope. The postoperative recovery is faster, there is less blood loss, and the surgery is shorter compared to open craniotomy. The only downside is that most times, there is a need to combine the surgery with the postoperative use of a remodeling helmet for 4 to 6 months" [3].

A second option that involves surgery is an open craniotomy. This procedure is performed on "patients older than six months because the bones are more rigid and cannot be manipulated as well with an endoscope. This modality allows for a better

#### **Figure 1.**

*Visual depictions of the various types of simple synostoses (Kadjic et al).*

remodeling of the skull and decreases the need for helmet use postoperatively" [3]. Ideally, surgery should be done "between 6 and 12 months of age when there are no signs of increased ICP or airway compromise" [3].

The objective that open craniotomies and endoscopic suturectomies aim to accomplish is to "create enough space in the cranial vault for the brain to grow and develop properly as well as to provide the child with a more decent-looking appearance" [3]. While these procedures tend to be conclusive in their ability to resolve this disorder, continuous treatment and follow-ups are often required in cases where the patient has syndromic craniosynostosis (**Figure 1**).
