Orthodontics Diagnosis and Treatment Plans of Craniosynostosis Syndromes, Report of Three Cases


Tahereh Hosseinzadeh Nik 1 , 2 , Abolghasem Bahador 3 , * , Mohammad Sadegh Ahmad Akhoundi 1 , 2 , Shima Sam 4

1 Dental Research Center, Dentistry Research Institute, Tehran University of Medical Sciences, Tehran, Iran

2 Department of Orthodontics, School of Dentistry, Tehran University of Medical Sciences, Tehran, Iran

3 Department of Orthodontics, Tehran University of Medical Sciences, Tehran, Iran

4 Dentist, Tehran, Iran

How to Cite: Hosseinzadeh Nik T , Bahador A, Ahmad Akhoundi M S, Sam S. Orthodontics Diagnosis and Treatment Plans of Craniosynostosis Syndromes, Report of Three Cases, Iran J Ortho. Online ahead of Print ; 14(1):e65275. doi: 10.5812/ijo.65275.


Iranian Journal of Orthodontics: 14 (1); e65275
Published Online: December 22, 2018
Article Type: Case Report
Received: December 17, 2017
Revised: April 21, 2018
Accepted: May 4, 2018


Craniosynostosis is the result of premature fusion of one or more craniofacial sutures, in which the sutural involvement generally includes the cranial vault, cranial base, and midfacial skeletal structures. Principally, treatment of craniosynostosis syndrome is challengeable and difficult. It is essential to recognize these syndromes, their symptoms and all the therapeutic choices. Crouzon and Apert syndromes are prevalent craniosynostosis which are associated with midface deficiency, exophthalmus, skeletal class III, skull malformation and mandibular prognathism. According to the severity and type of the malocclusion found in these syndromes, there are treatment option including: Removable appliance, face mask therapy + palatal expansion, high Le Fort II surgery, segmental distraction, Le Fort III surgery and distraction ostegenesis. In the present article, we introduced diagnosis of these syndromes, their therapeutic choices and finally we also presented two craniosynostosis patients and discuss about diagnosis and treatment plan procedure of them.

1. Introduction

According to Virchow’s law, craniosynostosis or premature fusion of a cranial vault suture results in prevention of skull growth perpendicular to the fused suture, and a compensatory growth at the open sutures; so that, the general direction of growth becomes parallel to the fused suture (1).

Syndromic craniosynostosis known as craniofacial dysostosis (CFD) are familial forms of synostosis involving various cranial base and midface sutures other than cranial vault sutures (2). Crouzon syndrome is most common craniosynostosis syndrome, differential diagnosis of this syndrome include: Apert, Pfeiffer, Saethre Chotzenin, Jackson Weiss syndrome, Carpenter syndrome (1).

1.1. Crouzon Syndrome

Crouzon syndrome mostly occurs in children of parents with either the menifestus disorder or carrier of the gene and old aged fathers (3). It is an autosomal dominant disorder with complete penetrance and variable expressivity which is caused by mutation in the gene of fibroblast growth factor receptor 2 (FGFR2) (4). The feature of the syndrome is premature synostosis of sagittal and coronal sutures initiating in the first year of life. When the sutures become closed, growth potential to those sutures is restricted. Nevertheless, multiple sutural synostoses regularly result in premature fusion of skull base causing midfacial and maxillary hypoplasia, shallow orbits, and infrequent upper airway obstruction (5). Furthermore, a triad of cranial deformity, maxillary hypoplasia and exophthalmos is seen in Crouzon (4). The intraoral symptoms consist of mandibular prognathism (usually pseudo prognathism), severe crowding of upper teeth, high, narrow or cleft palate, V-shaped maxillary dental arch, and bifid uvula. Also, peg-shaped teeth oligo- or macrodontia, and wide space between teeth is observed occasionally (5).

1.2. Apert Syndrome

Among the five craniosynostosis syndromes Apert syndrome is caused by one or two point allelic mutations of the fibroblast growth-factor receptor 2 (FGFR2) (6). Despite the fact that it is mostly periodic, many are related with high paternal age, with mean father age 34.1 + 6.2 (7, 8).

The syndrome is described by uneven craniosynostosis, midface hypoplasia, and fingers and toes syndactyly. In comparison to other craniosynostosis syndromes, Apert usually causes more severe craniofacial dysmorphology; such as cleft palate which happens in both Apert and Crouzon syndromes but is more frequent in Apert syndrome (8).

1.3. Pfeiffer Syndrome

Pfeiffer syndrome is a result of mutations in two fibroblast growth factor receptors (FGFRs): FGFR1 and FGFR2 (9). On the basis of the severity of craniofacial manifestations, significant clinical variability is observed in patients (10).

Commonly, this syndrome is characterized by fluctuating degrees of craniosynostosis, broad high forehead, small nose with depressed nasal bridge, eyes hypertelorism, proptosis, midface hypoplasia, and high arched palate. In addition, limb anomalies mostly consist of radially deviated broad thumbs and broad great toes and less frequently, partial syndactyly in fingers and toes may also be existed (11).

1.4. Treatment of Craniosynostosis Syndromes

Craniosynostosis syndromes could be treated surgically and non-surgically, especially mild Crouzon syndrome can be treated non-surgically. Non-surgical treatment include: Removable appliance, face mask therapy plus palatal expansion.

Reports have declared three periods of surgical and non-surgical reconstruction for Craniosynostosis syndromes:

1. Infancy: For primary decompression, releasing of the craniosynostosis (commonly bilateral coronal) and also, reshaping of the cranial vault (repeat if required).

2. Childhood: Correction of the total midface deficiency (surgical or non-surgical).

3. Adolescence: Definitive orthognathic surgeries to finalize the position of the lower face and occlusion.

1.4.1. Craniosynostosis Treatment During Childhood

Treatment in this age can be surgical or non-surgical. Non-surgical treatment has been used in mild case especially for Crouzon syndrome. As a treatment, orthopedic devices for class III malocclusions have not been successful in the appert syndrome (12).

In this review, we have gathered the treatments from varying articles: Removable Appliance

According to a research by Padmanabhan et al., a 7 y/o black boy with minor signs of Crouzon syndrome was treated. He was in early mixed dentition, and had no intra cranial pressure (ICP). As an early management, to correct the anterior crossbite, a removable expansion appliance activating twice a week was settled in maxilla. Besides, a posterior bite plane was combined to open the bite and provide correction of crossbite. To correct patient’s mid-face deficiency, authors stated that they would exert interceptive orthodontics so that to enhance his facial profile and to avert many malocclusions from progression (5). Face Mask Therapy Plus Palatal Expansion

Two sisters affected by Crouzon syndrome were treated by Maspero et al. with a non-surgical approach. An orthopedic approach with orthodontic devices was adopted; because of normal head circumference, and normal mental development and also, the limits of intracranial pressure which did not cause hydrocephalus. A rapid palatal expansion with a modified Hyrax expander which was activated a quarter turn twice per day for 15 days was used in his treatment plan. This appliance was kept passively in place for 4 - 6 months. After removing the palatal expander, a facemask (posteroanterior) was applied in order to relieve the maxillary sutures and stimulate maxillary anterior growth. The authors concluded that a combination of orthopedic and orthodontic treatment would be effective for improving the appearance and occlusion of patients with mild Crouzon syndrome, without surgery (13). High Le Fort II Surgery

A combination of orthodontics and high Le Fort II surgery was applied to treat an 11 year old boy by Ahmed and Afzal. Initially, the posterior crossbite was corrected by maxillary expansion and fixed brace. Pre-surgical orthodontics included decompensating of both maxillary and mandibular arches. In the end, for the correction of maxillary hypoplasia and malar deficiency, orthognathic surgery was planned which involved high Le Fort II osteotomy (3). In spite of that the author has applied this method as a phase of surgery to treat the patients we can adopt this method as the first phase of childhood and subsequently, perform the final orthodontics and Le Fort I surgery if required. Segmental Distraction Approach

Matsumoto et al. treated a 20-year-old woman presented with exophthalmos and retrusion of the midface with combination of orthodontics and segmental distraction surgery. The treatment plan was advancement of midface on two different levels, the malar level and the occlusal level and also, necessary preoperative orthodontic treatment. After 1 year of orthodontic procedures, occlusion reverted from class I to class III. A Le Fort III osteotomy with pterygomaxillary disjunction was then performed. By Le Fort I osteotomy the maxilla was divided into upper and lower segments via mucogingival sulcus incision. In order to be distracted separately, each fragment was connected to the rigid external distraction device (Figure 1). Distraction was begun after 1 day at 1 mm/day over 17 (malar level) and 12 days (occlusal level). The patient’s occlusion was fully corrected, and her facial contour was significantly improved. Finally, the distraction device was removed after 3 weeks of consolidation (14). In spite of that the author has applied this method as a phase of surgery to treat the patients we can adopt this method as the first phase of childhood and subsequently, perform the final orthodontics and Le Fort I surgery if required. Le Fort III Surgery or Distraction Ostegenesis

Maxillofacial advancement with Le Fort III osteotomy, for several decades, was conducted to treat severe cases of Crouzon syndrome; this surgical procedure provided good results with long-term stability (15-17).

Recently, as a new surgical technique, distraction osteogenesis (after its first application of mandibular lengthening report in 1992) has evolved to treat patients with jaw deformities. In 1993, the application of distraction osteogenesis for maxillary advancement was started and it is now excessively used in treating skeletal class III malocclusion caused by maxillary hypoplasia (18). there are two types of internal and external distraction devices which have been applied for maxillary advancement (Figures 2 and 3) (19).

Figure 2. External distractor for treatment of midface hypoplasia
Figure 3. Internal Le Fort I distraction device installed on the patient for treatment of maxillary hypoplasia

Distraction osteogenesis is now popular for the treatment of craniosynostosis and for advancing the midfacial. several articles have reported acceptable consequences (20-22) and some others indicated that distraction was more suitable than conventional osteotomy (18). As an example, according to a research by Fearon, the maxilla remained stable after Le Fort III halo distraction with no relapse for up to 5 years postoperatively (22).

Distraction osteogenesis can prevail over the natural soft-tissue resistance by gradual stretching and accordance, and engendering new soft tissues concurrently with skeletal augmentation. Active craniofacial development in childhood can assist both new bone generation and successively soft-tissue accommodation. Besides, distraction is less invasive physically and psychologically; for instance, it involves decreased blood loss and postoperative pain as well as shorter surgery time and little hospitalization (23, 24).

As mentioned, at this period of development, surgical treatment often includes Le Fort III or distraction osteogenesis for midface advancement. The maxilla can be advanced into approximately an ideal anteroposterior relationship with the mandible (25). Nevertheless, because the degree of horizontal deficiency at the orbits is seldom uniform, the surgical objective is to correct the fronto-orbital relationship, not necessarily to achieve a corrected overbite and overjet at the incisor teeth (1). Coordinately, during the mixed dentition, orthodontic treatment is performed to correct the dental problems -which consist of: crowding, ectopic eruption, posterior crossbite, and delayed tooth eruption (26) and also, enable the placement of an acrylic occlusal splint in the time of surgery to fix the monobloc segment (25).

For treating midface hypoplasia, there are varying viewpoints on the best time of performing the surgical advancement. The surgery may be carried out in 5 to 8 years old children or deferred until skeletal maturity.

In the opinion of Waitzman et al., Crouzon syndrome is best managed in early ages of 5 to 7 years old for ultimate reconstruction of the cranial vault dysplasia and orbital dystopia; so that, 85% to 90% of their adult size will be achieved (27).

Posnick and Ruiz, also, concurs with this age; he claims that performing the basic midface and final cranial vault surgeries at or after this age (5 to 7) results in approximate adult dimensions in cranio-orbito-zygomatic region and consequently, a stable long-term aesthetic after healing. This age is also supported by psychosocial considerations; because by early surgery, the child will have a higher self-confidence and body image so that he may make progression in school grades.

To preclude the risk of injury to the permanent maxillary first molars and subsequently reducing the risk of injury to second molars, the procedure is better to be postponed until full eruption of the maxillary first molars (around 7 years of age) (1).

On the contrary, some others suggest different times for the surgery:

Nout et al. believes that midface advancement can be accomplished in the first years of life for particular symptoms, such as severe obstructive sleep apnea or severe exophthalmia (24).

As stated by the craniofacial team of the University of California at San Francisco, if functional demands have not dictated earlier intervention, midface advancement could be done during 9 to 12 years old. The reason is, at that age, the midface can be advanced enough for the adult face, and the procedure does not require to be repeated later.

Bellow, there is a summarizing of the protocol for craniosynostosis syndromes by the University of California at San Francisco:

(A) Instantly after birth:

1. Physician counseling.

2. Feeding recommendation.

3. Team assessment consisting of: neurosurgery, orthopedics, pulmonology, audiology, otorhinolaryngology.

4. Radiography as indicated.

5. Shunt placement.

6. Tracheostomy if required.

(B) Six to twelve months:

1. Advancing fronto-orbital region by a team of craniofacial and neurosurgeons.

2. Correcting the syndactyly.

(C) One to two years:

1. Team monitoring to consider:

-Development of skull and cognitive skills.

-Progression in speaking and language.

oral hygiene.

2. Determining the time of cleft palate correction if existing.

(D) Two to seven years:

1. Team monitoring to consider:

-Development of teeth and their eruption.

-Development of jaw and face.

2. Behavioral and medical treatments for speech, middle ear disease, and so on.

3. Radiography or CT scans if required.

(E) Seven to 9 years:

1. Team monitoring

2. Providing orthodontic records.

3. Extracting teeth.

4. Applying phase 1 orthodontic treatment with maxillary expansion to get prepared for midface advancement.

(F) Nine to twelve years:

1. Team monitoring.

2. A standard Le Fort III osteotomy for midface advancement incorporating with bone grafts or rigid external distraction in case of patient’s cooperation.

(G) Twelve to twenty one years:

1. Team monitoring.

2. Providing orthodontic records.

3. Applying phase 2 orthodontic treatment to get prepared for orthognathic surgery after finalization of development.

4. Postsurgical orthodontic procedures and long-period orthodontic retention with ultimate records.

5. Shaping the forehead.

6. Revision the nose.

7. Genioplasty if required (28).

Table 1 includes common treatment conception for Apert syndrome which enables individual adaptions. The table is presented by the University Hospital of Münster (12).

Table 1. Interdicciplinary Treatment Concept for Patients with Apert Syndrome at the University Hospital of Münster
13 - 6 monthsEradicative-osteoclastic method
27 - 10 years
Le Fort III osteotomy +
Frontal advancement +
Protraction face mask immediately postoperatively for 6 - 12 month (rapid stenosis, problems with anchorage of the orthodontic appliance due to exfoliation of deciduous teach, and eruption of permanent teeth)
Early Le Fort III osteotomy might help to avoid a (second) Le Fort III osteotomy at stage 5, and thus reduce surgical risks
3> 9 yearsExtraction therapy
4> 10 yearsOrthodontic treatment (preparation for orthognathic surgery), retention
5> 16 yearsOrthodontic surgery

After finishing the first stage of orthodontic treatment (with or without initial surgery), a removable retainer may be applied to maintain the achieved dental alignment and prevent dental relapse. Subsequently, patients are followed up for development and eruption of the residual permanent teeth to commence the presurgical orthodontic phase preparatory to the orthognathic operation.

1.4.2. Orthodontic Treatment During Adolescence

Surgical treatment in this phase nearly all the time involves a Le Fort I osteotomy. Le Fort I osteotomy enables transverse expansion, horizontal advancement, and vertical extension. This surgery frequently, is incorporated with a genioplasty for additional correction of lower face malformation and occlusion (25). The presurgical orthodontic phase is started when the entire permanent teeth are existed and at the primary time of skeletal maturity (almost in girls 13 - 15 years old and in boys 15 - 17 years old) (1).

2. Case Presentation

2.1. Case One

2.1.1. Diagnosis and Etiology

Two homozygote twin girls (Figures 4 - 7), aged 8 years, were referred to Dental School of Tehran University of Medical Sciences for treating the maxillary deficiency. They had chief complaints of growth deficiency in upper jaw and overlapping the upper teeth by lower teeth.

Figure 4. Pretreatment of first’s twin case photography
Figure 5. Pretreatment of first’s twin case radiography
Figure 6. Pretreatment of second twin case photograph
Figure 7. Pretreatment of second twin case radiograph

Their medical history showed that their mother had a normal parturition. Their father aged 37 years and their mother aged 26 years at birth time. There were no familial history for the symptoms nevertheless, parents had familial marriage. Therefore, old age of father at their birth time and parents familial marriage could be the possible reasons for the craniosynostosis.

According to the medical history, they had no other disease; in addition, cranial reshaping at 1 years old and cranial sutures releasing operations had been done at 6 years old.

Extra oral examination indicated maxillary deficiency, mild exophthalmos and convex profile.

In intra oral examination reverse overjet, posterior crossbite and high narrow palates were observed. Patients were at early mixed dentition.

In their panoramic radiographies, all permanent teeth were existed but they had delayed eruption.

According to characteristics triad of symptoms of cranial deficiency, maxillary hopoplasia and exophthalmus are manifestations of Crouzon, so that; the patients were diagnosed as having a mild Crouzon syndrome.

2.1.2. Treatment Objective

The objective of the treatment were (1) to correct midface deficiency (2) to correct posterior and anterior cross bite (3) to improve their facial appearance (4) to evaluate delayed teeth eruption and manage eruption of the teeth.

2.1.3. Treatment Progress

We consulted with endocrinologist regarding delayed permanent teeth eruption. Moreover, concerning pervious skull complication we took advice from neurologist on exerting force by face mask. A face mask connecting to a maxillary removable appliance was applied for treating the maxillary deficiency. Because the eruption of the first molars was uncompleted or they had no eruption, primary teeth were applied for appliance retention. Expansion screw was exerted for correcting the posterior crossbite. The purpose of utilizing face mask was to reduce the severity of maxillary deficiency and to enable an easier and a more effective orthognathic surgery at higher ages. If face mask did not respond successfully, the possibility of applying midface distraction osteogenesis was considered.

2.2. Case Two

2.2.1. Diagnosis and Etiology

A girl, aged 30 years had a chief complaint of protrusion of eyes and the lower jaw referred to Dental School of Tehran University of Medical Sciences (Figures 8 and 9). Her medical history showed no allergy. She had undergone cranial reshaping and cranial sutures releasing operations at 9 months age. Her parents had no familial marriage, and her mother had no problem at parturition; also, there were no familial history for the mentioned problem. At birth time, father age was 37 years and mother age was 30 years.

Figure 8. Pretreatment photograph of second case
Figure 9. Pretreatment radiograph of second case

Extra oral examination showed maxillary deficiency, exophthalmos, straight profile, small and deformed nose and open bite.

Moreover, intra oral examination indicated anterior and posterior crossbites, high narrow palate, upper and lower teeth crowding, buccally erupted both canines and also the history of extracting the maxillary left first molar.

Considering exophthalmos, maxillary hopoplasia and cranial deficiency, which are characteristics of Crouzon, the patient was diagnosed with Crouzon syndrome.

2.2.2. Treatment Objective

The objective of the treatment were to (1) correct the facial appearance and the midfacial hypoplasia and, (2) correct the crossbite (anterior and posterior ) and establish ideal overjet and overbite, (3) achieve an acceptable and functional class I occlusion.

2.2.3. Treatment Progress

Regarding severe midface deficiency, anterior and posterior open bites and class III skeletal relationships, orthognathic surgery was considered. Furthermore, SARPE operation was conducted to correct the posterior crossbite. In order to prepare the patient for the surgery, maxillary decompensation, extracting maxillary first premolars and correction of the inclination of anterior teeth were performed.

Maxillary first premolars were extracted after SARPE and achieving sufficient expansion. In the period of preparation for the surgery, a Nance appliance was exerted in maxilla for the expansion maintenance. Due to severe crowding in mandible, single extraction treatment was applied therefore, mandibular left first incisor was extracted. After aligning and leveling maxillary arch and arch coordination, the patient would be referred to hospital for performing Le Fort III osteotomy, maxillary advancement, maxillary impaction, advancement reduction genioplasty and rhinoplasty.

3. Discussion

In case one, since the patients had been referred to us at this age, we were not capable of doing the previous procedures of the University of California. We will treat the patients according to the protocol of the University of California.

If the patient had been presented to us earlier, we would have consulted with physician and different specialists according to first stage of the protocol (instantly after birth).

Fronto-orbital advancement must be done at 6 - 12 months of age as we did for our patient.

Team evaluation from skull development and cognitive and speech skills must be done at 1 - 2 years old (our patient had normal skull development and speech). Teeth development and eruption and also, maxillofacial growth must be evaluated at 2 - 7 years old. Our patient indicated delayed primary and permanent teeth eruption.

After consultation with endocrinologist, orthodontic treatment was performed. During treatment we and the endocrinologist supervised teeth eruption. In case of necessity growth hormone was prescribed for stature and teeth eruption.

We provided records of the first stage of orthodontic treatment. The purpose of this stage (stage 4), is to prepare the patient for Le Fort III surgery. Also, the management of permanent teeth eruption would be undertaken at this stage, this could be included primary teeth extraction.

According to this protocol, patient settles in stage 5. Because of lower developmental age than chronological age, we decided to apply face mask palatal expansion.

In a patient with normal head circumference, normal intracranial pressure, and normal mental development, an orthopedic approach with orthodontic devices can be used (as shown in the cases reported in this paper) (13). The distraction approach to the midface deformity at an early age with orthopedic devices (RME and the face mask) can accomplish horizontal advancement and vertical lengthening of the midface and the transverse maxillary increase. According to these studies, the transverse widening would stimulate remodeling at lateral and posterior maxillary sutures. This will increase anteroposterior response (29).

The orthopedic force of the RME-facemask therapy stimulates cellular activity in sutures (circummaxillary) and maxillary tubercula, and this facilitates maxillary forward displacement (10 m). Younger than 8 y/o patients are better to obtain successful forward movement (29).

Accordingly we provided the required records and we commenced the treatment by expanding the maxilla to correct the maxillary constriction and also by applying face mask to correct the anterior-posterior deficiency. We plan to monitor the patient in this duration and if the adequate response did not obtain, the patient would be prepared for distraction osteogenesis.

Second stage of orthodontic treatment will be performed at 12 - 20 years old in order to prepare the patient for orthognathic surgery. Moreover, rhinoplasty, genioplasty and shaping of forehead should be done if required.

In second case, as well as the first patient, the patient also was referred to us too late so that we could not accomplish the first stages of the University of California at San Francisco protocol.

Ideally, physician and team consultation would have been done in the first stage if the patient had been presented to us.

Fronto-orbital advancement operation must be done at 6 - 12 months of age (this patient had undergone cranial suture releasing surgery by 9 months of age). Moreover, team evaluation was done at 1 - 2 years of age surveying the development of cranium, cognitive skills and also speech (patient has now normal speech).

Evaluation of oral hygiene must be done at this age and be continued in all the stages. The patient had the history of extracting left upper first molar which indicates poor oral hygiene in past.

Teeth eruption and maxillofacial development must be evaluated at 2 - 7 years old. If teeth eruption and development had been evaluated at this stage and subsequent stages, we could have prevented the buccaly eruption of both canines.

The first stage of orthodontic treatment must be evaluated at 7 - 9 years of age. At this age primary teeth is required to be extracted in case of necessity and patient must be prepared for Le Fort II or DO at 9 - 12 years old.

Our patient was in stage 7 (12 - 21 years old). After providing orthodontic records in this stage, phase II orthodontic treatment must be performed to conduct Le Fort I surgery. Our patient had maxillary deficiency, exophthalmos, straight profile, small and deformed nose and open bite, anterior and posterior crossbites, high narrow palate, upper and lower teeth crowding. Therefore, surgical treatment was proposed to improve both functional and morphologic problems with their associated psychosocial issues. Le Fort III advancement is usually indicated for hypoplasia of the upper midfacial structures (malar, orbital, and nasal bones). It is sometimes inadequate to correct the discrepancy at the dentoalveolar level when considered alone (23). The treatment of this patient has been commenced by our college doctor Akhoundi and is it still continuing. He decided to perform Le Fort I accompanied by Le Fort III surgeries for impaction and advancement of maxilla to improve the esthetic of midface. Before performing orthognatic surgery, he accomplished SARPE in order to correct the maxillary constriction. Subsequently, maxillary first premolars and mandibular right central were extracted to correct the inclination of maxillary teeth and the crowding of the lower teeth. After the treatment procedure, patient will refer to the hospital for surgery. Moreover, rhinoplasty, genioplasty and shaping of forehead should be done if required.

3.1. Conclusions

Treatment of craniosynostosis syndromes is difficult and it requires team working. In order to facilitate treatment of these syndromes, we must recognize their diagnosis and also all the therapeutic choices.




  • 1.

    Posnick JC, Ruiz RL. The craniofacial dysostosis syndromes: Current surgical thinking and future directions. Cleft Palate Craniofac J. 2000;37(5):433. doi: 10.1597/1545-1569(2000)037<0433:TCDSCS>2.0.CO;2. [PubMed: 11034022].

  • 2.

    Posnick JC. The craniofacial dysostosis syndromes. Current reconstructive strategies. Clin Plast Surg. 1994;21(4):585-98. [PubMed: 7813158].

  • 3.

    Ahmed I, Afzal A. Diagnosis and evaluation of Crouzon syndrome. J Coll Physicians Surg Pak. 2009;19(5):318-20. [PubMed: 19409168].

  • 4.

    Hlongwa P. Early orthodontic management of Crouzon syndrome: A case report. J Maxillofac Oral Surg. 2009;8(1):74-6. doi: 10.1007/s12663-009-0018-7. [PubMed: 23139476]. [PubMed Central: PMC3454018].

  • 5.

    Padmanabhan V, Hegde AM, Rai K. Crouzon's syndrome: A review of literature and case report. Contemp Clin Dent. 2011;2(3):211-4. doi: 10.4103/0976-237X.86464. [PubMed: 22215936]. [PubMed Central: PMC3214529].

  • 6.

    Ibrahimi OA, Chiu ES, McCarthy JG, Mohammadi M. Understanding the molecular basis of Apert syndrome. Plast Reconstr Surg. 2005;115(1):264-70. [PubMed: 15622262].

  • 7.

    Cohen MM Jr, Kreiborg S. New indirect method for estimating the birth prevalence of the Apert syndrome. Int J Oral Maxillofac Surg. 1992;21(2):107-9. [PubMed: 1602157].

  • 8.

    Tolarova MM, Harris JA, Ordway DE, Vargervik K. Birth prevalence, mutation rate, sex ratio, parents' age, and ethnicity in Apert syndrome. Am J Med Genet. 1997;72(4):394-8. [PubMed: 9375719].

  • 9.

    Jezela-Stanek A, Krajewska-Walasek M. Genetic causes of syndromic craniosynostoses. Eur J Paediatr Neurol. 2013;17(3):221-4. doi: 10.1016/j.ejpn.2012.09.009. [PubMed: 23062756].

  • 10.

    Hackett A, Rowe L. FGFR1 Pfeiffer syndrome without craniosynostosis: An additional case report. Clin Dysmorphol. 2006;15(4):207-10. doi: 10.1097/01.mcd.0000220608.40155.d4. [PubMed: 16957473].

  • 11.

    Junior HM, de Aquino SN, Machado RA, Leao LL, Coletta RD, Burle-Aguiar MJ. Pfeiffer syndrome: Clinical and genetic findings in five Brazilian families. Med Oral Patol Oral Cir Bucal. 2015;20(1):e52-8. [PubMed: 25129254]. [PubMed Central: PMC4320421].

  • 12.

    Hohoff A, Joos U, Meyer U, Ehmer U, Stamm T. The spectrum of Apert syndrome: phenotype, particularities in orthodontic treatment, and characteristics of orthognathic surgery. Head Face Med. 2007;3:10. doi: 10.1186/1746-160X-3-10. [PubMed: 17286873]. [PubMed Central: PMC1821014].

  • 13.

    Maspero C, Giannini L, Galbiati G, Kairyte L, Farronato G. Non surgical treatment of Crouzon syndrome. Stomatologija. 2014;16(2):72-80. [PubMed: 25209230].

  • 14.

    Matsumoto K, Nakanishi H, Koizumi Y, Seike T, Okazaki M, Yokozeki M, et al. Segmental distraction of the midface in a patient with Crouzon syndrome. J Craniofac Surg. 2002;13(2):273-8. [PubMed: 12000885].

  • 15.

    McCarthy JG, La Trenta GS, Breitbart AS, Grayson BH, Bookstein FL. The Le Fort III advancement osteotomy in the child under 7 years of age. Plast Reconstr Surg. 1990;86(4):633-46. discussion 647-9. [PubMed: 2217577].

  • 16.

    Meazzini MC, Mazzoleni F, Caronni E, Bozzetti A. Le Fort III advancement osteotomy in the growing child affected by Crouzon's and Apert's syndromes: Presurgical and postsurgical growth. J Craniofac Surg. 2005;16(3):369-77. [PubMed: 15915099].

  • 17.

    Phillips JH, George AK, Tompson B. Le Fort III osteotomy or distraction osteogenesis imperfecta: Your choice. Plast Reconstr Surg. 2006;117(4):1255-60. doi: 10.1097/01.prs.0000204865.97302.5c. [PubMed: 16582797].

  • 18.

    Kuroda S, Watanabe K, Ishimoto K, Nakanishi H, Moriyama K, Tanaka E. Long-term stability of LeFort III distraction osteogenesis with a rigid external distraction device in a patient with Crouzon syndrome. Am J Orthod Dentofacial Orthop. 2011;140(4):550-61. doi: 10.1016/j.ajodo.2009.12.038. [PubMed: 21967944].

  • 19.

    Polley JW, Figueroa AA. Management of severe maxillary deficiency in childhood and adolescence through distraction osteogenesis with an external, adjustable, rigid distraction device. J Craniofac Surg. 1997;8(3):181-5. discussion 186. [PubMed: 9482064].

  • 20.

    Meling TR, Hans-Erik H, Per S, Due-Tonnessen BJ. Le Fort III distraction osteogenesis in syndromal craniosynostosis. J Craniofac Surg. 2006;17(1):28-39. doi: 10.1097/01.scs.0000194177.21916.f1. [PubMed: 16432404].

  • 21.

    Shetye PR, Boutros S, Grayson BH, McCarthy JG. Midterm follow-up of midface distraction for syndromic craniosynostosis: A clinical and cephalometric study. Plast Reconstr Surg. 2007;120(6):1621-32. doi: 10.1097/01.prs.0000267422.37907.6f. [PubMed: 18040197].

  • 22.

    Fearon JA. Halo distraction of the Le Fort III in syndromic craniosynostosis: A long-term assessment. Plast Reconstr Surg. 2005;115(6):1524-36. [PubMed: 15861055].

  • 23.

    Fearon JA. The Le Fort III osteotomy: To distract or not to distract? Plast Reconstr Surg. 2001;107(5):1091-103. discussion 1104-6. [PubMed: 11373547].

  • 24.

    Nout E, Cesteleyn LL, van der Wal KG, van Adrichem LN, Mathijssen IM, Wolvius EB. Advancement of the midface, from conventional Le Fort III osteotomy to Le Fort III distraction: Review of the literature. Int J Oral Maxillofac Surg. 2008;37(9):781-9. doi: 10.1016/j.ijom.2008.04.006. [PubMed: 18486452].

  • 25.

    Nurko C, Quinones R. Dental and orthodontic management of patients with Apert and Crouzon syndromes. Oral Maxillofac Surg Clin North Am. 2004;16(4):541-53. doi: 10.1016/j.coms.2004.08.003. [PubMed: 18088753].

  • 26.

    Kaloust S, Ishii K, Vargervik K. Dental development in Apert syndrome. Cleft Palate Craniofac J. 1997;34(2):117-21. doi: 10.1597/1545-1569_1997_034_0117_ddias_2.3.co_2. [PubMed: 9138505].

  • 27.

    Waitzman AA, Posnick JC, Armstrong DC, Pron GE. Craniofacial skeletal measurements based on computed tomography: Part II. Normal values and growth trends. Cleft Palate Craniofac J. 1992;29(2):118-28. doi: 10.1597/1545-1569_1992_029_0118_csmboc_2.3.co_2. [PubMed: 1571345].

  • 28.

    Oberoi S, Hoffman WY, Vargervik K. Craniofacial team management in Apert syndrome. Am J Orthod Dentofacial Orthop. 2012;141(4 Suppl):S82-7. doi: 10.1016/j.ajodo.2012.01.003. [PubMed: 22449603].

  • 29.

    Maspero C, Giannini L, Iazzetti F, Tronca S, Farronato G. Eziopatogenesi e manifestazioni cliniche della sindrome di Crouzon: Revisione della letteratura. Mondo Ortodontico. 2010;35(5):249-60. doi: 10.1016/j.mor.2010.01.002.

  • Copyright © 2018, Iranian Journal of Orthodontics. This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License (http://creativecommons.org/licenses/by-nc/4.0/) which permits copy and redistribute the material just in noncommercial usages, provided the original work is properly cited.