Crouzon Syndrome Spanning Three Generations: Advances in the Treatment of Syndromic Midface Deficiency.

Background: Crouzon syndrome is an autosomal dominant genetic disorder characterized by craniosynostosis, midface retrusion, and exophthalmos. Over the past century, the treatment of craniofacial disorders like Crouzon syndrome has evolved significantly.

Methods: An institutional review board-approved retrospective study was conducted to ascertain the treatment of three individuals with Crouzon syndrome from one family, complemented with a series of literature searches to examine the evolution of craniofacial surgical history.

The Role of Digital Surgical Planning and Surgical Guides in the Treatment of Unilateral Condylar Hyperplasia.

Unilateral condylar hyperplasia (UCH) results in facial asymmetry, malocclusion, and temporomandibular joint dysfunction. Treatment consists of both surgical and orthodontic intervention. A review was performed for 4 patients with UCH who underwent digital surgical planning (DSP)-assisted condylectomy.

Long-Term Outcomes After External Maxillary Distraction Surgery in Patients With Down Syndrome.

Background: Patients with Down syndrome have severe facial deformities that can precipitate functional consequences and social stigmatization. Craniofacial surgical intervention can play a role in improving these symptoms and patient quality of life. The objective of this study was to investigate the long-term outcomes of distraction osteogenesis and orthognathic surgical intervention in patients with Down syndrome.

Spontaneous Closure of Congenital Cranial Defect: Is Early Surgical Intervention Warranted?

Infantile cranial development typically occurs in a predictable sequence of events; however, less is known about how the development occurs in isolated, nonsyndromic congenital craniofacial anomalies. Furthermore, the timing of pediatric cranioplasty has been extrapolated from adult studies. Thus, the management of nonsyndromic congenital craniofacial anomalies presents with unique challenges to the craniofacial surgeon.

Developing a core outcome set for anthropometric evaluation for presurgical infant orthopaedics for unilateral cleft lip and palate: e-Delphi consensus.

Introduction: Presurgical infant orthopaedics (PSIO) in infants with cleft lip and palate focuses on improving the anatomical conditions of the lip, palate and nose before the first lip surgery; however, its effectiveness has not been proven.

Objective: To develop a core outcome set for reporting anthropometry-based outcomes in studies appraising the PSIO before primary cleft lip repair in unilateral cleft lip palate (UCLP).

Method: Literature search to identify anthropometric measures.

Monobloc Differential Distraction Osteogenesis.

Midface advancement at the monobloc level can be the seminal life event for patients with craniofacial dysostosis. Monobloc reconstruction, when planned appropriately, can simultaneously and definitively address multiple functional and aesthetic deficiencies in these patients. The application of distraction has reduced the morbidity experienced with traditional monobloc surgery.

Intra-Lesional Injection of Triamcinolone to Palatoplasty Scar to Aid Reversal of Transverse Maxillary Relapse after Orthognathic Surgery.

A 32-year-old female with a repaired right unilateral cleft lip and palate underwent several surgical and orthodontic procedures during the rehabilitation process of her condition. Nine years after this extensive treatment she underwent transverse relapse of her maxilla and requested a consultation for its correction as she felt her speech and chewing were negatively affected. She presented with a transverse maxillary arch collapse on the cleft side with significant palatal scarring secondary to multiple palate procedures.

Cleft Lip and Palate: LeFort I Distraction with Halo and Hybrid Internal Maxillary Distractors.

Distraction osteogenesis is a viable treatment option for patients with a cleft associated with severe maxillary retrusion. A rigid external distraction device and a hybrid internal maxillary distractor have been used to advance the maxilla allowing for predictable and stable results. These techniques can be applied by itself or as an adjunct to traditional orthognathic procedures.

Skeletal Stability and Airway Changes After Maxillary Advancement Using a Rigid External Distraction System in Non-Growing Cleft Patients.

Distraction osteogenesis (DO) is a highly effective technique for correction of severe maxillary hypoplasia, especially in patients with orofacial clefts and craniofacial syndromes. The purpose of this retrospective, longitudinal study was to assess long-term airway alterations after maxillary advancement using a rigid external distraction system (RED) in non growing cleft patients. Fifteen cleft patients (8 males and 7 females) aged from 14 to 25 years were included in this study.

Custom Alloplastic Temporomandibular Joint Reconstruction: Expanding Reconstructive Horizons.

Custom alloplastic temporomandibular joint (TMJ) reconstruction has been well established for the management of end-stage TMJ disease. However, its use in congenital TMJ deformities is limited. Here, the authors present initial outcomes of skeletally mature patients who underwent custom alloplastic TMJ reconstruction and simultaneous orthognathic surgery.

NAM Therapy-Evidence-Based Results.

Many orthodontists working on patients with cleft lip and palate (CLP) have shown great enthusiasm for presurgical infant orthopedics (PSIO) to improve surgical outcomes with minimal intervention. Even though every clinician aims to use the best treatment modality for their patients, PSIO effects can be confounded by surgical type and timing of the primary repair, as is discussed in many studies. In such cases, one should be cautious when evaluating the particular outcomes for patients with CLP since it is difficult to differentiate the sole effect of an individual surgical or orthodontic intervention.

Scar Management After Surgical Repair of Lateral Facial Clefts.

The prevention of scar contracture after surgery is an important aspect of lateral facial cleft repair. Maintaining adequate mouth opening is essential for speech, expression, chewing, oral hygiene and psychosocial well-being. Although there have been extensive reviews of non-surgical microstomia management in patients with oral electrical burns, there have been no reports on the use of oral commissure splints to manage the surgical scar in patients with lateral facial clefts.

Combined maxillary and mandibular distraction osteogenesis in patients with hemifacial microsomia.

Introduction: Hemifacial microsomia is a deformity of variable expressivity with unilateral hypoplasia of the mandible and the ear. In this study, we evaluated skeletal soft tissue changes after bimaxillary unilateral vertical distraction.

Methods: Eight patients (4 preadolescents 4 adolescents) each with a grade II mandibular deformity underwent a LeFort I osteotomy and an ipsilateral horizontal mandibular ramus osteotomy.

Treatment of midfacial hypoplasia in syndromic and cleft lip and palate patients by means of a rigid external distractor (RED).

Introduction: Distraction Osteogenesis (DO) became an alternative for the treatment of severe craniofacial skeletal dysplasias. The rigid external distraction device (RED) is successfully used to advance the maxilla and all the maxillary-orbital-frontal complex (monobloc) in children, adolescents and adults. This approach provides predictable and stable results, and it can be applied alone or with craniofacial orthognathic surgical procedures.

Orthognathic positioning system: intraoperative system to transfer virtual surgical plan to operating field during orthognathic surgery.

Purpose: To introduce the concept and use of an occlusal-based "orthognathic positioning system" (OPS) to be used during orthognathic surgery.

Materials And Methods: The OPS consists of intraoperative occlusal-based devices that transfer virtual surgical planning to the operating field for repositioning of the osteotomized dentoskeletal segments. The system uses detachable guides connected to an occlusal splint.

Parameters of care for craniosynostosis: dental and orthodontic perspectives.

Introduction: A multidisciplinary conference was convened in March 2010 with the charge to develop parameters of care for patients with craniosynostosis. The 52 participants represented 16 medical specialties and 16 professional societies. Herein, we present the dental, orthodontic, and surgical care recommendations for those with craniosynostosis, with special emphasis on craniosynostosis syndromes.

Parameters of care for craniosynostosis.

Background: A multidisciplinary meeting was held from March 4 to 6, 2010, in Atlanta, Georgia, entitled "Craniosynostosis: Developing Parameters for Diagnosis, Treatment, and Management." The goal of this meeting was to create parameters of care for individuals with craniosynostosis.

Methods: Fifty-two conference attendees represented a broad range of expertise, including anesthesiology, craniofacial surgery, dentistry, genetics, hand surgery, neurosurgery, nursing, ophthalmology, oral and maxillofacial surgery, orthodontics, otolaryngology, pediatrics, psychology, public health, radiology, and speech-language pathology.

Nasal changes after presurgical nasoalveolar molding (PNAM) in the unilateral cleft lip nose.

Purpose: Nasal reconstruction for patients with unilateral cleft lip and palate (UCLP) is a challenge for the reconstructive surgeon. Presurgical nasoalveolar molding (PNAM) was introduced to reshape the cleft nasal structures prior to lip repair. This study analyzed two-dimensional nasal changes before and after PNAM in patients with complete UCLP.

Biomechanical considerations for distraction of the monobloc, Le Fort III, and Le Fort I segments.

Background: Distraction osteogenesis is effective for correction of severe maxillary and midface hypoplasia. The vectors controlling the segment to be moved must be planned. This requires knowledge of the physical characteristics of the osteotomized bone segment, including the location of the center of mass (free body) and the center of resistance (restrained body).

Evaluation of maxillary permanent molars in patients with syndromic craniosynostosis after monobloc osteotomy and midface advancement with rigid external distraction (RED).

Objective: This retrospective study was conducted to analyze changes in the maxillary permanent molars after monobloc advancement with rigid external distraction (RED).

Setting: University hospital-based craniofacial center.

Materials And Methods: Fourteen patients, three in primary, eight in mixed, and three in permanent dentition underwent monobloc advancement with RED.

Introduction of a new removable adjustable intraoral maxillary distraction system for correction of maxillary hypoplasia.

Distraction osteogenesis has become a treatment alternative to treat severe craniofacial skeletal dysplasias. A rigid external distraction device has been successfully used to advance the maxilla as well as the maxillary, orbital, and forehead complex (monobloc) in children as young as 2 years, adolescents, and adults. For this severe group of patients, the technique has been found to be simpler and safer than traditional surgical methods.