OBJECTIVE: To summarize the application and recent development of orthognathic surgery in treating syndromic craniosynostosis. METHODS: The related literature at home and abroad in recent years was extensively reviewed, and the indications, routine procedures, and protocols of orthognathic surgery in the treatment of syndromic craniosynostosis were summarized and analyzed. RESULTS: Craniosynostosis is a common congenital craniofacial malformation. Syndromic craniosynostosis usually involves premature fusion of multiple cranial sutures and is associated with other deformities. Orthognathic surgery is the necessary and effective means to improve the midfacial hypoplasia and malocclusion. Le Fort I osteotomy combined with sagittal split ramus osteotomy are the common surgical options. Orthognathic surgery should combine with craniofacial surgery and neurosurgery, and a comprehensive long-term evaluation should be conducted to determine the best treatment plan. CONCLUSION Orthognathic surgery plays an important role in the comprehensive diagnosis and treatment of syndromic craniosynostosis. The development of digital technology will further promote the application and development of orthognathic surgery in the treatment of syndromic craniosynostosis.
Humans ; Orthognathic Surgery ; Craniosynostoses/surgery* ; Osteotomy ; Osteotomy, Sagittal Split Ramus

Image-guided surgery potentially enhances intraoperative safety and outcomes in a variety of craniomaxillofacial procedures. We explore the efficiency of one intraoperative navigation system in a single complex craniofacial case, review the initial and recurring costs, and estimate the added cost (e.g., additional setup time, registration). We discuss the potential challenges and benefits of utilizing image-guided surgery in our specific case and its benefits in terms of educational and teaching purposes and compare this with traditional osteotomies that rely on a surgeon's thorough understanding of anatomy coupled with tactile feedback to blindly guide the osteotome during surgery. A 13-year-old presented with untreated syndromic multi-suture synostosis, brachycephaly, severe exorbitism, and midface hypoplasia. For now, initial costs are high, recurring costs are relatively low, and there are perceived benefits of imaged-guided surgery as an excellent teaching tool for visualizing difficult and often unseen anatomy through computerized software and multi-planar real-time images.
Adolescent ; Craniosynostoses ; Humans ; Osteotomy ; Surgery, Computer-Assisted* ; Synostosis

Craniofacial malformation caused by premature fusion of cranial suture of infants has a serious impact on their growth. The purpose of skull remodeling surgery for infants with craniosynostosis is to expand the skull and allow the brain to grow properly. There are no standardized treatments for skull remodeling surgery at the present, and the postoperative effect can be hardly assessed reasonably. Children with sagittal craniosynostosis were selected as the research objects. By analyzing the morphological characteristics of the patients, the point cloud registration of the skull distortion region with the ideal skull model was performed, and a plan of skull cutting and remodeling surgery was generated. A finite element model of the infant skull was used to predict the growth trend after remodeling surgery. Finally, an experimental study of surgery simulation was carried out with a child with a typical sagittal craniosynostosis. The evaluation results showed that the repositioning and stitching of bone plates effectively improved the morphology of the abnormal parts of the skull and had a normal growth trend. The child's preoperative cephalic index was 65.31%, and became 71.50% after 9 months' growth simulation. The simulation of the skull remodeling provides a reference for surgical plan design. The skull remodeling approach significantly improves postoperative effect, and it could be extended to the generation of cutting and remodeling plans and postoperative evaluations for treatment on other types of craniosynostosis.
Child ; Computer Simulation ; Cranial Sutures/surgery* ; Craniosynostoses/surgery* ; Humans ; Infant ; Skull/surgery*

OBJECTIVETo discuss the early surgical treatment of fronto-orbital retrusion in craniosynostosis.

METHODSOperative techniques including "C" fronto-orbital osteotomies and advancement, frontal radially oriented osteotomies and remoulding were performed to correct the fronto-orbital retrusion. Ten patients aged from 6-9 months were included. There were two metopic synostosis, three unilateral coronal synostosis, three bilateral coronal synostosis, one Apert syndromic synostosis and one Crouzon syndromic synostosis.

RESULTSFronto-orbital deformities of all patients were corrected. There were no complications happened in these patients. The post-operation results were kept well for 3-11 months follow-up in 8 cases.

CONCLUSIONSFronto-orbital osteotomies and advancement and frontal reshaping were effective to correct fronto-orbital retrusion in craniosynostosis.

Craniosynostoses ; surgery ; Female ; Humans ; Infant ; Male ; Orbit ; abnormalities ; surgery ; Reconstructive Surgical Procedures ; methods ; Treatment Outcome

OBJECTIVETo sum up three types of plastic procedures for frontal plagiocephaly Based on the principle of floating forehead, the plagiocephaly (unilateral coronal synostosis).

METHODSwas corrected and orbito-frontal reconstruction was performed by orbito-frontal advancement, 'or temporoparietal osteotomy, or fronto-parietal osteotomy. The absorbable plates and microscrews composed of From January 2008 to polylactide polymers, were used to immobilize calvarial segments.

RESULTSDecember 2010, 9 cases of plagiocephaly were treated with satisfactory result and no severe complication. The patients were followed up for 1-2 years with no recurrence of suture fusion and deformity. The brain development was not restricted. The absorbable plates were applied to avoid the metal fixation moving into The three types of plastic procedures, orbito-frontal advancement, or the cranial bone.

CONCLUSIONStemporo-parietal osteotomy, or fronto-parietal osteotomy, can correct the plagiocephaly with satisfactory orbito-frontal appearance. Long-term follow-up is needed to observe the long-term effect of the orbito-frontal reconstructive operation on mental and calvarial development as well as visual function.

Absorbable Implants ; Bone Plates ; Craniosynostoses ; surgery ; Forehead ; Humans ; Osteotomy ; methods ; Plagiocephaly ; surgery ; Reconstructive Surgical Procedures ; methods

Plagiocephaly is a premature unilateral coronal craniosynostosis consisting of flattening of the involved frontal region and low-set orbit in affected side and compensatory occipital bulging in contralateral side with resultant facial asymmetry. The authors experienced a 20-year-old male having frontal and posterior palgiocephaly with asymmetric mid-facial appearance in left side who had undergone the strip craniectomy of the coronal suture when he was 100 days old. We performed total intracranial total calvarial remodeling including occipital region followed by two-jaw operation seven months later. Cranial vault was cut into three transverse bone flaps; anterior bi-frontal bone flap, posterior bi-fronto-parietal bone flap, and bi-parieto-occipital bone flap. Unilateral supra-orbital bar advancement with tongue-in-groove arrangement was performed. To restore contour of occipital region, the transposition cranioplasty was done after mid-sagittal osteotomy of posterior bone flap. Seven months later, Le Fort I osteotomy and BSSRO was performed to correct jaw asymmetry and cross-bite. We believe that total correction of orbito-fronto-occipital vault including jaw surgery in plagiocephaly is mandatory for more excellent aesthetic result and effectiveness.
Craniosynostoses ; Facial Asymmetry ; Humans ; Jaw ; Male ; Orbit ; Orthognathic Surgery ; Osteotomy ; Plagiocephaly* ; Sutures ; Young Adult

Apert syndrome is well known condition with craniosynostosis, midface hypoplasia, exorbitism, and typical symmetric syndactyly of hands and feet. Surgical correction method should be selected according to the type and the severity of the deformity and the patient's age. We have experienced an adult atypical Apert syndrome patient who has brachycephaly and midface hypoplasia, without exorbitism, and has limb deformities such as brachymetatarsia and ectrodactyly without syndactyly of hands and feet, which are not typical in Apert syndrome patients. For the simultaneous correction of the cranial vault and the facial deformities, we combined Le Fort II advancement osteotomy and advancement genioplasty as well as transposition cranioplasty at the same time, and obtained successful result. Through our limited experience, we concluded our modified combined methods could be safely applied to the adult patients with various types of craniofacial deformity.
Acrocephalosyndactylia* ; Adult ; Congenital Abnormalities ; Craniosynostoses ; Extremities ; Foot ; Genioplasty ; Hand ; Humans ; Osteotomy ; Surgery, Oral* ; Syndactyly

Craniosynostosis occurs in approximately 1:2000 live births. It may affect the coronal, sagittal, metopic and lambdoid sutures in isolation or in combination. Although non-syndromic synostoses are more common, over 150 genetic syndromes have been identified. Recent advances in genetic mapping have linked chromosomal mutations with craniosynostotic syndromes. Despite the identification of these genetic mutations, the fundamental biomolecular mechanisms mediating cranial suture biology remain unknown. Today, many laboratories are investigating murine cranial suture biology as a model for human cranial suture development and fusion. Normal murine cranial suture biology is very complex, but evidence suggests that the dura mater provides the biomolecular blueprints (e.g. the soluble growth factors), which guide the fate of the pleuripotent osteogenic fronts. While our knowledge of these dura-derived signals has increased dramatically in the last decade, we have barely begun to understand the fundamental mechanisms that mediate cranial suture fusion or patency. Interestingly, recent advances in both premature human and programmed murine suture fusion have revealed unexpected results, and have generated more questions than answers.
Animal ; Craniosynostoses/*etiology/genetics/surgery ; Fetal Development ; Fetus/*physiology ; Human ; Mutation

Crouzon syndrome is one of the craniofacial syndromes characterized by craniosynostosis, midfacial hypolpasia and ocular proptosis. Distraction osteogenesis is becoming important technique to treat craniofacial dysplaisa. It has many advantages compared with standard orthognathic surgery. Maxillary distraction osteogenesis after Le Fort III osteotomy with the RED system presents successful maxillary protraction to Crouzon syndrome patient with severe maxillary deficiency. It also allows remarkable improvement of facial esthetics and respiratory functions.
Craniofacial Dysostosis* ; Craniosynostoses ; Esthetics ; Exophthalmos ; Humans ; Orthognathic Surgery ; Osteogenesis, Distraction* ; Osteotomy

Objective: To summarize the preliminary efficacy, perioperative management and complications of Le Fort Ⅲ osteotomy and midface distraction in patients with syndromic craniosynostosis by retrospective analysis, and to provide clinical experience for reference. Methods: From October 2017 to January 2020, 20 patients with syndromic craniosynostosis underwent Le Fort Ⅲ osteotomy and distraction in The Department of Oral and Maxillofacial Surgery of Peking University International Hospital, including 11 males and 9 females, were involved. The median age was 7 years (1.5 to 15 years). Preoperative risk prevention plan was put forward by multidisciplinary evaluation, and preoperative intervention was carried out. The diagnostic data of SNA, airway volume, polysomnography (PSG), ophthalmology and occlusal relationship were obtained through specialized examination, and osteotomy and distraction surgical plan was formulated through virtual surgical planning. CT was taken 1 week and 3, 6, 12 months after operation, PSG and eye protrudence examination were conducted to evaluate the therapeutic effect, syndrome type, multiple disciplinary treatment (MDT) intervention, occurrence and outcome of complications were summarized. Results: There were 15 cases of Crouzon syndrome and 5 cases of Pfeiffer syndrome. Sleep apnea was the first complaint in 18 cases and exophthalmia in 2 cases. Preoperative interventional therapy included 4 cases of adenoid surgery, 2 cases of continuous positive airway pressure and 2 cases of maxillary expansion. The most common surgical complications were accidental fracture (14/20 cases, 70%), cerebrospinal fluid fistula (2 cases), internal carotid cavernous sinus fistula (1 case), postoperative hyponatraemia (5 cases), crying syndrome (2 cases), wound infection (2 cases), trichiasis of lower eyelid (4 cases), and nasal malformation (1 case). Three cases underwent unplanned secondary surgery. SNA, airway volume and mean percutaneous arterial oxygen saturation (SpO₂) six months after operation were significantly higher than those before operation (F=10.09, P=0.001; F=5.13, P<0.001; F=10.78, P=0.001), and the protrusion and apnea hypopnea index were significantly lower than those before surgery (F=6.73, P=0.010; F=18.47, P<0.001). There were no significant differences in SNA, airway volume, mean SpO₂, ophthalmology between 6 months after surgery and 1 year after surgery (P>0.05). Conclusions: Perioperative safety assessment and early intervention of MDT is an effective diagnosis and treatment model of Le Fort Ⅲ osteotomy and distraction for syndromic craniosynosis. The operative complications are mainly local, and systemic complications are controllable.
Cephalometry ; Child ; Craniosynostoses/surgery* ; Female ; Humans ; Male ; Osteogenesis, Distraction ; Osteotomy, Le Fort ; Retrospective Studies ; Syndrome