Simultaneous Le Fort III and Le Fort I Osteotomy: Surgical Outcomes and Clinical Parameters.

INTRODUCTION: Simultaneous Le Fort III/I (LF III/I) osteotomies are often performed when a differential advancement of the upper and lower midface is needed. This study aims to evaluate midface position preoperative and 1 week postoperative in patients with severe midface hypoplasia. In addition, this study aims to compare the planned surgical movements to the actual postoperative movements. MATERIALS AND METHODS: A retrospective review was conducted using cephalometry for patients treated with a simultaneous LF III/I osteotomy at a single institution. Osteotomies were performed during 1980-2018 on skeletally mature patients with a craniofacial syndrome, with clinical and radiographic follow-up available. RESULTS: Twelve patients met the inclusion criteria with a mean age of 20.2±6.4 years. Treatment resulted in statistically significant anterior movements related to Orbitale, anterior nasal spine, A Point, and the upper incisor tip, and inferior movements related to anterior nasal spine, A Point, upper and lower incisor tips, B point, and pogonion. Stability after 1 year showed only statistically significant changes at ANB. The predictable error for planned movements versus actual movements was greater in the vertical plane than the horizontal plane. CONCLUSIONS: A simultaneous LF III/I osteotomy significantly improved the midface position and occlusal relationship in syndromic patients with midface hypoplasia in a predictable manner. Further multicenter studies with larger sample sizes are needed to validate the conclusions.

Longitudinal Skeletal Growth Analysis of Mandibular Asymmetry in Unoperated Patients With Unilateral Craniofacial Microsomia (UCFM).

PURPOSE: To examine the growth rate discrepancy of the affected and unaffected ramus heights in Pruzansky Type I and Type II mandibles. METHODS: This is a serial retrospective longitudinal growth study of 30 untreated patients (21 males and 9 females) with UCMF (age range from 5 years to 14 years). The mean age of patients was 8.5 years, and the mean follow-up records were 3.7 years. There were 13 patients in group I with a Pruzansky Type I mandible and 17 patients in group II with a Pruzansky Type II. The unaffected side of the mandible served as a control. Eighteen cephalometric parameters were examined at each of the two-time intervals. RESULTS: In patients with Pruzansky Type I mandible, the affected ramus grew on average 1.41 mm per year; the unaffected ramus grew 1.66 mm per year during the same period. In patients with Pruzansky Type II mandible, the affected ramus grew on average 0.84 mm per year; during the same period, unaffected ramus grew 1.79 per year. When the growth rate of the ramus height on the affected side was compared to the unaffected side, there was no statistically significant difference in Pruzansky Type I mandibles (p > .05); however, there was a statistically significant difference in the Pruzansky Type II mandibles (p < .05). CONCLUSION: The growth rate discrepancy of the affected and unaffected ramus heights was more severe in Pruzansky Type II mandibles than Pruzansky Type I mandibles explaining the progressive nature of facial asymmetry in Pruzansky II mandibles.

Correction of Severe Facial Asymmetry in Patients With Unilateral Craniofacial Microsomia Using Computer-Aided Design/Computer-Aided Manufacturing Technology: An Evaluation of Postsurgical Results.

ABSTRACT: This is a retrospective study to evaluate the postsurgical position of the maxilla and mandible in 5 matured craniofacial patients with unilateral craniofacial microsomia who underwent 2 jaw surgical procedures using computerized surgical planning. The craniofacial surgeon and orthodontist completed the virtual surgical treatment plan with a biomedical engineer's assistance via a web meeting. The treatment plan of each patient included 2 jaw surgery with genioplasty. At the maxillary dental midline, the planned mean advancement was 4 mm; yaw, a rotational correction towards the unaffected side was 4.96 mm; and impaction was 2.74 mm. The mean advancement measured at point B was 10.5 mm, and the rotational correction towards the unaffected side was 6.58 mm. The mean advancement following genioplasty was 8.43 mm, and the mean transverse correction was 6.33 mm towards the midsagittal plane. The intermediate surgical splint, final surgical splint, bone graft templates, and cutting guides were constructed utilizing computer-aided design/computer-aided manufacturing technology. The surgeon executed the treatment plan in the operating room using appropriate computer-generated guides and splints. A postsurgical cone-beam computed tomography scan was obtained and superimposed on the surgical treatment plan using Simplant OMS 10.1 software. The cranial base was used as a reference for superimposition. Three-dimensional color-coded displacement maps were generated to visually and quantitatively assess the surgical outcome. There was a mean error of 0.88 mm (+0.30) for the position of the maxillary anatomical structures from the planned position, and the anterior mandibular anatomical structures were on average 0.96 mm (+0.26) from the planned position.

Maxillomandibular and occlusal relationships in preadolescent patients with syndromic craniosynostosis treated by LeFort III distraction osteogenesis: 10-year surgical and phenotypic stability.

INTRODUCTION: LeFort III distraction osteogenesis may be indicated in the treatment of syndromic craniosynostosis with severe midface retrusion. This study investigates long-term changes in patients undergoing distraction as children, and compares outcomes to an unaffected, untreated control. METHODS: Fifteen patients (9 males, 6 females) with syndromic craniosynostosis treated by LeFort III distraction at an average age of 4.9 ± 1.5 years were identified. Lateral cephalograms at predistraction, immediate, 1-, 5-, and 10-year postdistraction were superimposed using the best-fit of cranial base details. An untreated, unaffected matched control was obtained from the American Association of Orthodontists Foundation Legacy Collection. Differences in landmark location and cephalometric relationships were assessed between time points and between treatment and control groups. RESULTS: LeFort III distraction produced an average advancement of 14.86 ± 5.14 mm at A-point and 10.54 ± 3.78 mm at orbitale. This advancement produced overcorrection of anteroposterior occlusal relationships and phenotypic correction of midface position. Surgical stability over a 10-year follow-up was excellent. Posttreatment growth was characterized by absent anteroposterior maxillary growth, preservation of dentoalveolar development and maxillary remodeling, and delayed mandibular growth. Subsequent growth resulted in a long-term phenotypic relapse of pretreatment Class III maxillomandibular relationship and negative overjet. CONCLUSIONS: LeFort III distraction osteogenesis produces stable advancement of the midface. Overcorrection is required for long-term phenotypic stability because of deficient postdistraction sagittal midface growth. Late mandibular growth contributes to underestimation of the amount of distraction required to produce long-term phenotypic correction.

Treacher Collins Syndrome and Tracheostomy: Decannulation Using Mandibular Distraction Osteogenesis.

INTRODUCTION: Treacher Collins syndrome (TC) and Pierre Robin sequence (RS) are associated with hypoplastic mandible, glossoptosis, and consequent airway obstruction. Although TC and RS are often grouped together, airway outcomes of bilateral mandibular distraction osteogenesis (MDO) have not been specifically studied in TC. The purpose of this study is to report on the clinical outcomes of MDO in the TC patient population. MATERIALS AND METHODS: A twenty-year single-institution retrospective review of all patients with TC who underwent bilateral MDO was performed. Twenty-four patients were identified after exclusion due to different diagnoses or insufficient medical records. Data on comorbidities, airway status, MDO operations, and complications were collected. Data were compared with published clinical outcomes in RS and data for 13 RS patients from our institution. RESULTS: Surgical success, defined as prevention of imminent tracheostomy or successful decannulation within 1 year after primary distraction, was observed in 21% of TC patients and 65% of RS patients (P = 0.01). Repeat distraction was necessary for 11 TC patients (46%) and 1 RS patient. Complications were divided into minor, moderate, and major based on need for invasive management. Overall, 67% of TC patients had complications, 20% of which were major. CONCLUSIONS: Clinical outcomes to airway function after MDO are significantly inferior in patients with TC compared with patients with RS. Repeat MDO and longer course to decannulation are more prevalent in patients affected by TC.

Unusual Craniofacial Distraction.

A retrospective Institutional Review Board-approved review was performed at the Institute of Reconstructive Plastic Surgery, NYU Langone Medical Center, of patients undergoing craniofacial distraction osteogenesis procedures using the rigid external distractor device between 2000 and 2010. Three particularly challenging cases were identified and are presented here.

Proptosis Correction in Pre-Adolescent Patients With Syndromic Craniosynostosis by Le Fort III Distraction Osteogenesis.

Le Fort III distraction osteogenesis may be indicated in the treatment of syndromic craniosynostosis with severe midface retrusion and proptosis. This study assesses the stability of proptosis correction over 10-years.A retrospective review identified 15 patients with syndromic craniosynostosis treated by Le Fort III distraction prior to age 10 (9 males, 6 females; age 4.9 ±â€Š1.5 years). Untreated, non-craniosynostotic age- and gender-matched controls were obtained from historical growth records. Lateral cephalometric tracings at pre-surgery (T1), immediate (T2), 1 year (T3), 5 years (T4), and 10 years (T5) (n = 11) post-distraction were superimposed using the best-fit of cranial base. Proptosis severity was defined as the horizontal distance between the Ant. Globe cephalometric point and orbital rim landmarks Orbitale and Lat. Orbit.The orbital rim advanced 10.54 ±â€Š3.78 mm (P < 0.001) at Orbitale and 9.73 ±â€Š4.54 mm (P > 0.001) at Lat. Orbit from T1 to T2; Ant. Globe advanced 3.13 ±â€Š3.02 mm (p 0.001). Proptosis decreased 7.41 ±â€Š5.29 mm (P < .001) from Orbitale and 6.60 ±â€Š6.50 mm (p 0.002) from Lat. Orbit. Comparison to controls demonstrated phenotypic correction. In craniosynostotic patients from T2 to T5, the bony orbital rim demonstrated non-significant remodeling posteriorly and inferiorly. Anterior Globe moved 3.79 ±â€Š1.47 mm anteriorly (P < .001), which did not differ significantly from controls. Proptosis increased by 4.18 ±â€Š2.94 mm in craniosynostotic patients from T2 to T5.Le Fort III distraction was stable, with no significant anteroposterior relapse of the maxilla or bony orbit. Phenotypic relapse of proptosis to pre-treatment levels occurred through deficient growth of the midface, surface resorption at the orbital rim, and preservation of normal forward movement of Ant. Globe.

Unilateral Craniofacial Microsomia: Unrecognized Cause of Pediatric Obstructive Sleep Apnea.

Bilateral craniofacial microsomia causes obstructive sleep apnea (OSA). We hypothesize that unilateral craniofacial microsomia (UCFM) is an underappreciated cause of OSA. The records of all pediatric UCFM patients from 1990 to 2010 were reviewed; only complete records were included in the study. UCFM patients with OSA (apnea hypopnea index >1/hr) were compared to UCFM patients without OSA. Univariate and multivariate Fisher and χ(2) tests were performed. Of the 62 UCFM patients, 7 (11.3%) had OSA. All OSA patients had Pruzansky IIB or III mandibles. OSA patients presented with snoring (71.4%), failure to thrive (FTT) (57.1%), and chronic respiratory infections (42.8%). Snoring (P < 0.001), Goldenhar syndrome (P = 0.001), and FTT (P = 0.004) were significantly associated with OSA, but race, obesity, clefts, respiratory anomalies, adenotonsillar hypertrophy, and laterality were not. The prevalence of OSA in UCFM patients is up to 10 times greater than in the general population. Snoring, Goldenhar syndrome, and FTT are significantly associated with the presence of OSA.

Maxillary mucocele with proptosis and visual impairment: a late complication of Le Fort III distraction.

Maxillary mucoceles are a relatively rare entity especially following surgical procedures involving osteotomies of the maxilla. The etiology of maxillary mucoceles has been ascribed to facial trauma (fractures), sinus surgery, and chronic inflammatory diseases or infections. Mucoceles can follow injury to the sinus mucosa and/or sinus outflow tract with a resulting expansile cystic mass. The clinical presentation ranges from swelling, pain, a palpable mass, proptosis, enophthalmos, and diplopia. The treatment involves either open or endoscopic incision and drainage of the cyst, mucosal resection, and an antrostomy for drainage.We report the case of a patient with Pfeiffer syndrome who underwent Le Fort III distraction osteogenesis and developed a symptomatic mucocele 15 years postoperatively.

Cephalometric assessment of craniofacial morphology in patients with treacher Collins syndrome.

BACKGROUND AND PURPOSE: Treacher Collins syndrome is an autosomal dominant craniofacial disorder affecting derivatives of the first and second branchial arches. Given the conflicting reports in the literature regarding the extent of anterior-posterior and vertical (superior-inferior) dysplasia of the cranial base, maxilla, and mandible, this study was designed to provide a comprehensive lateral cephalometric assessment of the craniofacial morphology of unoperated patients with Treacher Collins syndrome. METHODS: The records of 45 patients with Treacher Collins syndrome registered at the Institute of Reconstructive Plastic Surgery of the NYU Langone Medical Center from the period of 1975 to 2008 were reviewed. Inclusion criteria included (1) patients between with the ages of 5 and 17 years, (2) no history of prior surgical intervention to correct skeletal deformity, and (3) diagnostic quality lateral cephalograms. Twenty patients satisfied the inclusion criteria with 12 male and 8 female patients in the sample and a mean age at the time of the lateral cephalogram of 8.8 years (range 5.2 to 16.7 years). Thirty angular and linear variables were measured to assess anterior-posterior and vertical (superior-inferior) position of the cranial base, maxilla, and mandible. Each patient's measurements were compared to age-matched and sex-matched control samples derived from the Moyers growth studies. The mean and standard deviation for each variable was then determined. RESULTS: Lateral cephalometric analysis showed decreased anterior, posterior, and total cranial base lengths and a reduced cranial base angle. The anteroposterior dimension or length of the maxilla is deficient and the maxilla is positioned posteriorly with respect to the cranial base. Both anterior and posterior facial heights (superior-inferior dimension) are decreased. Lower face height is increased, whereas total face is decreased. The maxillary and functional occlusal planes are tipped upwards posteriorly. The mandibular morphology is characteristically small in both body length and total mandibular length, and the maximum ramus width is also deficient. The mandibular plane angle and gonial angle are increased. The mandible is retropositioned. CONCLUSIONS: A lateral cephalometric analysis is described that provides a skeletal basis for the hallmark clinical findings associated with Treacher Collins syndrome. This is of importance because an understanding of the underlying skeletal dysmorphology may shed light on the etiology and growth pattern, and impacts the overall treatment planning for skeletal correction.

Pfeiffer syndrome: analysis of a clinical series and development of a classification system.

Among the craniosynostosis syndromes, Pfeiffer syndrome is notable because of high mortality and the need for multiple surgical interventions. However, it is variable in severity. We propose a new classification of Pfeiffer Syndrome to define pathology and function. A retrospective review was conducted of 42 patients with Pfeiffer syndrome treated from 1975 to 2010, the largest series reported to date. The classification was based on a functional assessment of patients in terms of respiratory, ocular, otological, and neurological status. This classification was tested by scoring and stratifying patients as follows: type A (mild problems), B (moderate problems), or C (severe problems). Patients were scored both at the time of presentation and after all surgical interventions to assess change in functional outcome. The functional classification system was compared to another previously reported. Type A patients did not have any change in postoperative functional outcomes (mean preoperative score 1.6, mean postoperative score 1.6); type B patients showed functional improvement (mean preoperative score 4.1, mean postoperative score 3.4) but type C patients (mean preoperative score 7.7, mean postoperative score 4.8) demonstrated the greatest improvement in functional scores after surgical intervention. Suture pathology did not indicate the clinical severity of phenotype, a variance from a previously published classification. The proposed classification is useful to assess severity of phenotype: respiratory, ocular, otologic, and neurologic problems are key indicators of the need for treatment. The classification can provide a helpful guide in multidisciplinary treatment planning, in reporting outcomes, and in the sharing of data among craniofacial anomalies centers.

Craniofacial surgery on a personal view.

Henry Kawamoto Jr and I go back a long way. Our friendship began at the Columbia-Presbyterian Medical Center. I had just returned from my service obligation after interrupting my general surgery residency. I heard that a new resident from California had been admitted to our class, and, of course, it was Henry.

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. These attendees also represented 16 professional societies and peer-reviewed journals. The current state of knowledge related to each discipline was reviewed. Based on areas of expertise, four breakout groups were created to reach a consensus and draft specialty-specific parameters of care based on the literature or, in the absence of literature, broad clinical experience. In an iterative manner, the specialty-specific draft recommendations were presented to all conference attendees. Participants discussed the recommendations in multidisciplinary groups to facilitate exchange and consensus across disciplines. After the conference, a pediatric intensivist and social worker reviewed the recommendations. RESULTS: Consensus was reached among the 52 conference attendees and two post hoc reviewers. Longitudinal parameters of care were developed for the diagnosis, treatment, and management of craniosynostosis in each of the 18 specialty areas of care from prenatal evaluation to adulthood. CONCLUSIONS: To our knowledge, this is the first multidisciplinary effort to develop parameters of care for craniosynostosis. These parameters were designed to help facilitate the development of educational programs for the patient, families, and health-care professionals; stimulate the creation of a national database and registry to promote research, especially in the area of outcome studies; improve credentialing of interdisciplinary craniofacial clinical teams; and improve the availability of health insurance coverage for all individuals with craniosynostosis.

Le Fort III distraction: controlling position and path of the osteotomized midface segment on a rigid platform.

BACKGROUND: Precise control of the position of the midface through distraction with the rigid external distraction (RED) device has been a challenge. The present RED device with wire attachments to the intraoral dental splint and to the skeletal bone plates allows for flexibility in the vertical plane of the osteotomized Le Fort III segment. This tends to rotate the midface in a counterclockwise direction with inferior movement of the posterior nasal spine. OBJECTIVES: To report the development of a rigid distraction splint attachment to the RED device that permits precise control of the position of the midface during the latency period and through the activation and consolidation phases. METHODS: This paper describes the appliance design and the clinical application of a new device in controlling the position of the midface during distraction. Placement of the device and application of the desired force vectors are discussed. Patients treated by this modified device are illustrated to document the planned midface position after Le Fort III midface advancement. RESULTS: Examination of predistraction and postdistraction cephalograms of 2 patients treated with the new device showed advancement with minimum inferior displacement of the midface during all phases of the distraction process. CONCLUSIONS: The new device prevents undesired inferior movement of the posterior midface immediately after osteotomy and helps to stabilize the midface during the latency period. The device enables directional control of the distraction vectors, resulting in more predictable midface position at the end of treatment.

Eruption of Maxillary Posterior Permanent Molars following Early Conventional Le Fort III Advancement and Early Le Fort III Distraction Procedures Compared to Late Surgical Intervention.

BACKGROUND: Le Fort III advancement and/or distraction involve osteotomies and dysjunction in the region of the maxillary tuberosity in proximity to the maxillary posterior tooth buds. The purpose of this study was to determine the effect of early conventional Le Fort III advancement and/or distraction on development and eruption of the maxillary posterior permanent molars. METHODS: A retrospective review of patients diagnosed with syndromic craniosynostosis, who underwent early Le Fort III or early midface distraction and late surgical intervention, was analyzed. RESULTS: In the early conventional Le Fort III surgery group, 93 percent of maxillary second molars and 28 percent of maxillary first molars experienced a disturbance in eruption. In the early distraction group, 82 percent of maxillary second molars and 20 percent of maxillary first molars experienced a disturbance in eruption. In the control group, the late conventional Le Fort III and the late distraction groups, only 26 percent of maxillary second molars and none of maxillary first molars experienced a disturbance in eruption. CONCLUSIONS: A common disruption seen postoperatively in the early Le Fort III and distraction groups was displacement of the second molars. The majority of the displaced tooth buds were located in the maxillary sinus. Overall, the early Le Fort III surgery groups experienced more frequent disturbances for both first and second molars, with the common sequela of displacement in the maxillary sinus, leading to the question of whether presurgical planning in cases of early intervention to address midface retrusion should include extraction/enucleation of the second molar tooth buds. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Onlay frontal cranioplasty using wire reinforced methyl methacrylate.

INTRODUCTION: Methyl methacrylate is a biologically inert alloplastic material that is commonly used to rebuild the calvarial vault. Since methyl methacrylate does not permit tissue incorporation it is susceptible to displacement and/or fracture. In order to increase the tensile strength of methyl methacrylate onlay cranioplasties, we use wire reinforced masonry techniques. PATIENT: A 56-year-old female presents with forehead asymmetry due to displacement and fracture of a silicone alloplastic implant. This patient, treated with onlay wire reinforced methyl methacrylate, demonstrates the utility of this novel technique. RESULTS: Wire is passed through 2-mm outer cortex tunnels like spokes on a wheel, around the perimeter of the defect to form a rebar grid. Methyl methacrylate is poured onto the rebar grid and contoured after it solidifies. CONCLUSIONS: Wire reinforced methyl methacrylate is a simple technique to improve the tensile strength of calvarial alloplastic reconstructions.