A Review of Current Surgical Approaches and Diagnostic Features Associated with Craniosynostosis Patients and the Relation to Oral and Maxillofacial Surgery

Abstract

The chapter will describe etiology of craniosynostosis and the management in the young child. Included will be classification of various forms of craniosynostosis and surgical management. Diagnostic imaging including CT scan, MRI, etc. will be mentioned as a tool in the treatment considerations of the patient with Craniosynostosis. Initial diagnosis, and consultation with appropriate surgical service, and treatment options will be discussed in the Chapter. Surgical options will include surgical plan and fixation methods. Further discussion of combined orthodontic and surgical treatment planning is presented. Complications will be discussed and summarized including reasonable expectations with both short and long term outcomes.

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:

• “If a craniosynostosis develops due to a primary defect of the ossification process it is called primary craniosynostosis” [1].

• Secondary craniosynostosis develops as a “result of known systemic diseases with hematologic or metabolic dysfunction” [1].

• It can also be divided into either syndromic or non-syndromic craniosynostosis which involves the development of this disorder as a result of another existing condition such as Pfeiffer syndrome or an isolated disorder, respectively. Syndromic craniosynostosis accounts for approximately 40% of reported cases and tends to have a genetic involvement (Table 1). Furthermore, this disorder can also be classified as either simple or complex where “simple craniosynostosis is a term used when only one suture fuses prematurely, while complex craniosynostosis is used to describe a premature fusion of multiple sutures” [1].

Genes Involved in Syndromic Craniosynostosis
Clinical diagnosis	Genes to be investigated
Apert	FGFR2
Crouzon	FGFR2
Crouzon with acanthosis nigricans	FGFR3
Pfeiffer	FGFR2 (FGFR1)
Carpenter	RAB23
Muenke	FGFR3 (TWIST1)
Saethre-Chotzen	TWIST1 (FGFR3)
Craniofrontonasal dysplasia	EFNB1

Table 1.

Gene involvements in syndromic craniosynostosis [2].

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].

Type of craniosynostosis	Typical characteristics
Scaphocephaly	Premature fusion of the sagittal suture Elongated head in the anterior-posterior and shortened in the bilateral direction Frontal bossing is present Boys more frequently affected (3.5:1) Premature fusion of the coronal suture Forehead flattened on the affected side High supraorbital margins (Harlequin sign)
Anterior plagiocephaly	Forehead pushed forward on the unaffected side Nasal septum deviation towards the normal side More common in girls (2:1) Unilateral lambdoid synostosis
Posterior plagiocephaly	Frontal and occipital bossing Ipsilateral ear and mastoid displaced downward Head shape from above may resemble a trapezoid Ipsilateral ear and forehead displaced anteriorly Parallelogram shape of the head
Positional plagiocephaly	Ipsilateral occipital flattening accompanied by contralateral occipital bossing Male to female ratio 3:1 Premature fusion of the metopic suture
Trigonocephaly	Occipital part is broad, forehead is narrow and pointed Triangular shape of the head Hypotelorism Bilateral coronal synostosis Short skull Forehead and occipital part flattened
Brachycephaly	Frontal bone prominent and elongated in vertical direction Hypertelorism Harlequin malformation of the orbits

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 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).

Figure 1.

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

1.1 Scaphocephaly

This type of synostosis occurs due to a premature fusion of the sagittal suture and is more commonly found in premature infants. It can be detected due to the elongation of the head in the anterior-posterior direction along with the shortening of the head in the bilateral direction. Another observable indicator of the presence of scaphocephaly is the possibility of frontal bossing along with a noticeable ridge along the sagittal suture. “Boys are more frequently affected than girls, with a ratio of 3.5:1” [1].

1.2 Anterior Plagiocephaly

Anterior plagiocephaly occurs when the coronal suture fuses prematurely. Observable signs of this type of synostosis involve a flattening of the forehead on the affected side, a protruding forehead on the unaffected side, and a nasal septum deviation towards the normal side. If a radiograph is performed, anyone that has anterior plagiocephaly will display a characteristic reading that is more commonly known as a Harlequin sign due to the high supraorbital margins that the affected have. In some cases where the “bicoronal fusion” closes prematurely, the condition is called brachycephaly” [1]. This type of synostosis affects girls more than boys in a 2:1 ratio.

1.3 Posterior Plagiocephaly

“Posterior plagiocephaly is a unilateral lambdoid synostosis” [1]. Distinguishable signs to look out for include frontal or occipital bossing, a displacement of the ipsilateral ear and mastoid downwards, and depending on the severity of the posterior plagiocephaly, the head may look like a trapezoid when viewed from above.

1.4 Trigonocephaly

“Trigonocephaly results from a premature fusion of the metopic suture” [1]. Prominent features that are unique to the presence of trigonocephaly are a broad back of the head, a pointed forehead that appears to be triangular when viewed from above. The orbits are also closer together than normal which is commonly referred to as hypotelorism. The ratio of boys to girls affected by this type of synostosis is 3.3:1.

1.5 Brachycephaly

“Brachycephaly is a bilateral coronal synostosis” [1]. It is common for those affected by brachycephaly to display signs such as a flattened forehead or occipital bone along with a “frontal bone [that] is prominent and elongated in a vertical direction” [1]. The orbits are also further apart than normal which is commonly referred to as hypertelorism. This separation can be picked up on radiographs. Studies also tend to show that brachycephaly is typically a type of synostosis that is heavily influenced by genetics or develops as a result of syndromic craniosynostosis.

1.6 Deformational Plagiocephaly

Deformation or non-synostotic plagiocephaly is unlike any other type of plagiocephaly as it is not synostotic. Table 3 summarizes the key differences between non-synostotic and synostotic plagiocephaly. It develops as a result of continuous pressure to one area of the head. It is hard to distinguish between the two as some of the physical changes that come about as a result of deformational plagiocephaly can be similar to those found in synostotic plagiocephaly. However, the physical changes that arise as a result of deformational plagiocephaly can be resolved with time and by changing the sides that the baby sleeps on whereas synostotic plagiocephaly requires surgery to remedy any physical changes. Due to the number of babies that sleep in a supine position in order to avoid the risk of cot death, the amount of deformational plagiocephaly cases rose from “20% to 48% since the early 1990’s” [2, 6] which makes it harder to detect cases in which craniosynostosis is present.

2. Approaches to the management of craniosynostosis

There are various approaches a surgeon should consider for treatment planning a patient with craniosynostosis.

2.1 Surgical approaches

Most occurrences require timely surgical correction. Various articles highlight the importance of the patient’s age as the predominant factor when determining the surgical approach. Per Chong et al.: for patients younger than 6 months, a minimally invasive procedure should be considered. This most commonly involves a minimally invasive suturectomy with postoperative helmet therapy.

The goal of minimally invasive suturectomy is to release a fused suture with small exposure. Chong et al. outlines their approach to a case of sagittal craniosynostosis:

“The patient is prepared with the head extended in a prone position. Skin preparation is done with povidone-iodine. Two transverse incisions are made of 3–4 cm length at 1 cm behind the anterior fontanelle and 1 cm in front of the lambdoid suture. An additional incision may be needed between the two sites to manipulate safely in a patient with a longer head. A subperiosteal dissection is made along the desired craniectomy site. Burr holes are placed over the fused suture at both incision sites. The dura is dissected and carefully detached from the fused bone. During these procedures, a fiber optic suction tip or endoscope is used for the safe and accurate manipulation of the compromising space. Strip craniectomy is performed using curved Mayo scissors, sternal scissors and straight rongeurs. The fused bone is removed from the anterior fontanelle anteriorly to the lambda posteriorly. The width of the craniectomy site is targeted to be between 3 cm to 4 cm. After the strip craniectomy, additional lateral wedge osteotomies or barrel stave osteotomies might be conducted according to the surgeon’s preference. Bleeding from the diploic space is controlled by bone wax and monopolar electrocautery. With the insertion of a drain, the wound is closed layer by layer” [7].

Postoperative helmet therapy is typically initiated after subgaleal swelling is reduced and all stitches are removed. The helmet is recommended to be worn for 12–18 months to account for this important rapid brain and skull growth period. One helmet may be sufficient depending on the procedure (such as following sagittal craniosynostosis) or the patient may require two or more helmet adjustments. An orthotist fits patients for the helmet.

2.1.1 Fronto-orbital advancement

The goal of the fronto-orbital advancement (FOA) surgical technique is to create space in the skull while reshaping the forehead and the orbit. FOA is indicated in unicoronal, bicoronal, or metopic craniosynostosis.

In this procedure, a coronal incision is performed using a sinusoidal pattern with hemostatic clips. Subperiosteal dissection is performed to raise the anterior scalp flap to expose the superior orbital rim and orbital roof bilaterally. Caution must be taken with the release of the supraorbital neurovascular bundle from its notch and retraction with gentle pressure on the globe. The subperiosteal dissection is then directed laterally to expose the fronto-zygomatic suture and lateral orbital wall and medially to expose the nasal root and medial orbital walls. The lateral canthus is released while the medial canthus must remain intact. A frontal craniotomy is performed, diligently avoiding the patent sutures, and frontal bone flap is removed with care to avoid damage to the sagittal sinus and the dura. Any bleeding from inadvertent damage to the dura must be controlled immediately after removal of the frontal bone. Superior osteotomy from anterior to posterior is created, followed by the lateral osteotomy through the zygomatic-frontal suture [8].

Intracranially, an osteotomy of the orbital roof is performed through the anterior cranial fossa while protecting the frontal lobe and globe, as well as the temporal lobe when extending laterally to the lateral sphenoid. A nasal frontal osteotomy is performed starting laterally and extending it medially to avoid damage to the dura. Once the osteotomies are released, the bones, except for the frontal bone, are gently bent to the desired contour, rearranged with overcorrection to avoid relapse. The frontal bone flap is transected, molded and reattached with absorbable hardware and/or sutures in an overcorrected anterior to posterior position to avoid relapse. The sites are irrigated with copious amounts of saline and closed in a layered fashion (Figure 2) [8].

Figure 2.

Bilateral fronto-orbtal advancement [A, B]. Total vault remodeling [C, D] [9, 10].

2.1.2 Posterior cranial vault distraction

The posterior cranial vault distraction (PCVD) technique has been used with the goal of increasing intracranial volume while achieving desired cosmetic results. With low perioperative complications, this procedure allows for 25–30% increase in intracranial volume. Salokorpi et al. describes their method of PCVD with virtual surgical planning preoperatively for osteotomy lines, size of bone flap, direction of distraction and location of the distraction devices.

A coronal incision is performed in a weave fashion and the location of the incision should be placed to allow for front-orbital expansion procedure, if needed. The dissection in the occipital area is performed subperiosteally. Once marked, the osteotomy is performed using the preoperative plan. Burr holes are made with a ball drill with caution to avoid dural damage. The burr holes are also placed, bilaterally, on the lambdoid sutures. The dura is dissected through the burr holes and further craniotomy is performed starting from the temporal and extending to the sagittal suture. The craniotomy is then extended occipitally over the posterior sinus structures. Bleeding in the occipital area from the emissary veins must be managed with bone wax and hemostatic matrix with thrombin. A short bone bridge is left to ensure stability during fixation of the distractors. After fixation, this bone bridge is cut [11].

Distractors are placed, parallel to each other, in the locations that were planned preoperatively. Salokorpi et al. preferred to position the devices with anterior positioning of the activation arms. The occipital movement of the flap is confirmed by activating the devices for a few millimeters. The bone is left attached to the dura as detachment increases the risk of bleeding from venous sinuses. Salokorpi reports that a full mobilization of the bone flap does not affect the ossification. Postoperatively, after a latency period of 5–7 days, the rate of distraction is performed at 0.5–1 mm/day in one to two sessions per day. Once sufficient ossification is obtained, the devices can be removed with small skin cuts perpendicular to the coronal incision [11].

2.1.3 Endoscopic strip craniectomy

The goal of endoscopic strip craniectomy is to perform a strip craniectomy using an endoscope to minimize scalp incision, blood loss, operative times and postoperative recovery periods. The use of the endoscope to perform the strip craniectomy was described by Barone and Jiminez, published in Plastic and Reconstructive Surgery in 1999.

Two 2 cm incisions are placed; one of the posterior aspect of the anterior fontanelle and the other over the lambda. With the use of an endoscope, dissection is performed in the subgaleal plane between these two incisions and extending to the bitemporal regions. With endoscopic visualization, electrocautery is performed for hemostasis to create a dry subgaleal dissection. Then, blunt dissection is performed to separate the dura from the bony edge of the fontanelle. A rongeur is used to remove a thin strip of bone anterior to the lambdoid suture to avoid injury to the sagittal sinus. Dura is dissected off the lambda and posterior aspect of the sagittal suture. With direct visualization, the sagittal sinus is carefully peeled off the synostosed sagittal suture. Epidural dissection is extended laterally to the level of the squamosal suture. Once complete subgaleal and epidural exposure is obtained, a lateral paramedian osteotomy is made using bone cutting scissors and a midline strip measuring 1.5–7 cm wide and 7–12 cm long is removed. Wedges of bone are removed and the surgical field is irrigated with antibiotic solution prior to closure. Postoperatively, Barone and Jiminez describe that the patients were placed in custom-made helmets with continued molding therapy for upto 8 months [12].

Lambdoid, metopic and coronal synostosis follow similar techniques except for some modifications. In lambdoid sutures, incisions are planned preoperatively using ECG and plain x-ray film and the patient is placed in a full prone position. In metopic and coronal synostosis, the patient is placed in supine position and an incision is made at the hairline centrally. Additionally, the subgaleal dissection is performed with the endoscope to the nasofrontal suture (Figure 3) [12].

Figure 3.

Patient with scaphocephaly treated with endoscopic-assisted suturectomy and osteotomies. Patient in modified prone position [A]. Surgical patties showing subcutaneous dissection [B]. Sagittal suture excision [C]. Excised sagittal suture [D] [10].

2.1.4 Spring-mediated cranioplasty

Spring-mediated cranioplasty is a surgical technique employed for the correction of sagittal craniosynostosis to expand the parietal bones transversely to reverse midvault disproportion.

Two transverse incisions are made posterior to the anterior fontanelle and anterior to the posterior fontanelle allowing easier access to the most distal anterior and posterior aspects of the sagittal suture to perform the craniectomy and placing the distractor springs. After identifying and elevating the subgaleal plane, burr holes are placed at the midline of the inferior and superior incisions. The inferior and superior incisions depend on the prone or supine positioning of the patient. A craniectomy is performed and three strips, 1.5 cm or less in width, are removed. Following this, cranial springs, that were selected preoperatively with the criteria of length, thickness and U bend, are placed in the anterior, mid-vault and posterior positions. The springs are placed 1 cm posterior to the anterior fontanelle, 1 cm anterior to the lambdoid sutures and at the parietal bone midpoint. Following copious irrigation, the incisions are closed in a layered fashion (Figure 4) [14].

Figure 4.

Image of craniosynostosis surgery being performed on a patient with anterior plagiocephaly. Bicoronal flap exposing defect [A]. Completed skull reconstruction with resorbable plates [B] [13].

2.2 Combined surgical and orthodontic approach

Treatment of craniosynostosis involves a care team that includes but is not limited to an oral maxillofacial surgeon, neurosurgeon, pediatric dentist, plastic surgeon, pediatric neurologist, geneticist, plastic surgeon, dentist, and orthodontist. As a result of the various medicinal disciplines that are involved in the care of a patient that has craniosynostosis, treatments that the patients undergo are a joint effort that requires the cooperation of those involved. A common collaboration that is vital to providing care for patients afflicted by craniosynostosis is that of dentists and surgeons. While surgeons perform operations on a patient, dentists can monitor the patient and keep record of the recovery process using “photographs, diagnostic models, and imaging records” [1]. Dentists are also heavily involved in ensuring that the teeth of the patient develop properly. Table 4 below illustrates the reliance of providers on the various types of recommended interventions to manage orthodontic patients:

Characteristic	Non-synostotic (deformational) plagiocephaly	Synostotic plagiocephaly
Cause/definition	Abnormally shaped head due to external forces applied to the skull, not due to craniosynostosis	Premature fusion of one or more cranial sutures; exact cause unknown; genetic and environmental factors may play a role
Common types	Lateral deformational (positional) plagiocephaly; posterior deformational plagiocephaly (positional brachycephaly)	Bilateral coronal craniosynostosis (synostotic brachycephaly); sagittal cranosynostosis (dolichocephaly/>scaphocephaly); metopic craniosynostosis (trogonocepnaly)
Distinguishing features	Round, symmetrical head shape at birth; parallelogram or brachycephalic head shape: ear may be anteriorly displaced; no palpable bony ridges	May have abnormal head shape at birth; trapezoid head shape; ear may be posteriorly displaced; palpable bony ridges
Management	Repositioning and physical therapy; helmet in some cases	Usually surgery; helmet in some cases

Table 3.

Comparison of non-synostotic (deformational) plagiocephaly and synostotic plagiocephaly [4, 5].

Note: Adapted from Tables 1 and 2 in Nield et al., 2007.

Age (y)	Dentition Stage	Interventions	Providers involved
<1	Primary dentition	Establish dental home	Pediatric dentist
1–6	Primary dentition	Periodic oral examinations Assessments for growth Supervised oral hygiene practices/aids Maxillary expansion when possible to facilitate incisor and molar eruption	Pediatric dentist, orthodontist, and oral and maxillofacial surgeon
7–12	Mixed dentition	Oral hygiene assessments and prophylaxis as needed Phase I orthodontic treatment (e.g., maxillary expansion to correct posterior crossbites, limited maxillary arch orthodontic treatment to correct anterior crossbites, limited orthodontic treatment to facilitate eruption of permanent dentition, and reverse-pull headgear treatment) Sequential extractions of primary teeth to facilitate eruption of permanent teeth Midface advancement (as needed)	Pediatric dentist, periodontist, orthodontist, and oral and maxillofacial surgeon
13–21	Permanent dentition	Periodic oral examinations, hygiene assessments, and prophylaxis Comprehensive phase of orthodontic treatment with or without orthognathic surgery (depending on degree of skeletal imbalance) Restorative treatment (eg, implants, crowns, veneers) following completion of comprehensive phase of orthodontic treatment	Orthodontist, oral and maxillofacial surgeon, periodontist, and prosthodontist
>21	Permanent dentition	Retention checks Periodic observations to assess long-term stability of surgical corrections Periodic oral hygiene visits	Orthodontist, oral and maxillofacial surgeon, and periodontist

Table 4.

Outline of a treatment plan for a patient with craniosynostosis [1].

Severe cases of craniosynostosis (typically syndromic craniosynostosis) often present an array of problems that need to be dealt with in a timely manner to ensure that the patient does not suffer from any mortal symptoms. While the table above outlines a general treatment plan for a patient that has craniosynostosis, those that suffer from syndromic craniosynostosis require certain steps in their treatment plan to be performed earlier or later. For example, patients with syndromic craniosynostosis tend to develop severe midface hypoplasia and suffer from “sleep apnea as a result of retropalatal airway collapse” [1]. In cases such as these, it is imperative that patients undergo premature midface advancement surgery in order to remedy the discomfort associated with sleep apnea along with fixing the patient’s facial profile. In conjunction with the midface advancement surgery, orthodontic treatment is also performed in order to ensure that the patient can attain a normal occlusion. Typically, this process involves maxillary expansion to allow for incisors and molars to have space to grow in the mouth. “Depending on the severity of maxillary arch constriction, several rounds of expansion may be required” [1]. This process is outlined by Azoulay et al. in a case where they deal with a patient that has syndromic craniosynostosis as a result of Pfeiffer syndrome.

“It is best to use a 4-banded expansion appliance if adequate anterior (primary first molars or primary canines) and posterior abutments (permanent first molars) are present, and overexpansion (by about 30%) should be achieved to account for expected relapse. The expansion appliance (usually hyrax, W arch, or quad helix) should be in place for at least 3 months and a fixed transpalatal arch with mesial extension arms should be placed at the time of device removal. Hawley appliances (with acrylic covering of the palate) can also be used, but these need to be periodically adjusted as the primary teeth exfoliate and permanent teeth emerge. It is most efficient to correct transverse maxillary deficiency during the mixed dentition phase when the circum-maxillary and palatal sutures are patent. As the patient ages, the palatal suture becomes fused and there is a considerable amount of resistance from the circum-maxillary sutures to maxillary expansion. In such situations, a surgically assisted maxillary expansion may be required” [1].

The procedure described above outlines one of the phases of orthodontic treatment that a patient with craniosynostosis goes through. In addition to this treatment there are other steps that can be divided into presurgical orthodontics, orthognathic surgery, and postsurgical orthodontics. Before undergoing maxillary/mandibular surgery to fully correct the patient’s occlusion it is necessary for some pre-surgery setup to be done. The objectives of this stage are to align and level both maxillary and mandibular arches, obtain compatible arch forms, remove dental compensations, and resolve crowding/spacing issues [1]. After the patient has undergone the necessary prerequisite treatments, single jaw or bimaxillary surgery is performed “to correct anterior/posterior, transverse, and vertical maxillary/mandibular discrepancies” [1]. Finally, the patient is left with a normal occlusion that needs minor touchup and detailing which is accomplished in the postsurgery phase of orthodontics.

3. Complications

There are a wide range of surgical complications that can present following the above mentioned procedures. The primary complications can be summarized in Table 5.

Complications of surgery

Wound infection

Intraoperative bleeding

Postoperative hematoma (subgaleal, subdural)

Intraoperative/postoperative hyperthermia

Dural tear

Cerebrospinal fluid leak

Meningitis

Postoperative mortality: 2.6%

Postoperative morbidity: 12%

Table 5.

Surgical complications associated with surgical treatment of craniosynostosis.

Esparza et al. discuss the various complications they found in a review of 283 cases. Mortality was very low with only 2 of their 283 patients reported death 1 year following the procedure with the cause of death owing more to the patient’s syndrome than the actual procedure itself. The most common complications involved postoperative hyperthermia (13.43%) and cranial infection (7.3%). Other less frequent complications included CSF leaking, dura tears, and hematomas. Relapses occured in 11.56% of their cases which is a similar figure reported in other case studies. There is a significantly higher infection rate associated with relapse patients. Overall, 85% of their patients had successful outcomes, while negatively reported outcomes associated with those patients who had craniofacial syndromes. Figure 5 portrays a case of a patient with Pfieffer syndrome who unfortunately died due to postoperative infection from the tracheostomy performed [13].

Figure 5.

3 month old with Pfeiffer syndrome. Cloverleaf skull [A]. GLobe herniation on Valsalva maneuver [B]. Post Op following fronto-facial distraction [C] [D]. Unfortunately, the patient died 18 months later due to infection related to tracheostomy [13].

4. Conclusion

As craniosynostosis is a disorder that affects the development of the skull, the entire face is negatively impacted, which is evidenced by the treatment plan and treatment length that patients must face. The diagnostic and surgical approaches that we can take in order to remedy this disorder are wide and varied such as the joint surgical orthodontic approach. Overall, it is important to recognize the diagnostic features associated with craniosynostosis in order to plan the patient for the optimal form of care. While mortality is low, all the various craniosynostosis surgical techniques offer a wide range of complications that need to be understood and managed by the operator. Regular post-operative and long term followup can help ensure success of the operation.

Conflict of interest

The authors declare no conflict of interest.