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.

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.

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.

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.

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.

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.

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.

Five-Year Follow-Up of Midface Distraction in Growing Children with Syndromic Craniosynostosis.

BACKGROUND: Maxillary position in patients with syndromic craniosynostosis after midface distraction has been shown to be stable 1 year postoperatively. The purpose of this study is to assess midfacial position in the growing child with craniosynostosis 5 years after Le Fort III advancement with a rigid external device. METHODS: Seventeen consecutive patients were identified to have the diagnosis of syndromic craniosynostosis and had undergone midface advancement [corrected]. There were 10 boys and seven girls, seven patients had Crouzon syndrome, five had Apert syndrome, and five had Pfeiffer syndrome. A standard subcranial Le Fort III osteotomy was performed. Cephalometric analysis was performed to assess the position of the maxilla. RESULTS: After device removal, orbitale advanced 13.67 mm along the x axis and downward 1.70 mm along the y axis. The A point advanced 15.97 mm along the x axis and downward 1.14 mm along the y axis. At 1 year after distraction, both orbitale and A point had advanced an additional 0.47 mm and 0.24 mm along the x axis and downward 0.58 mm and 1.78 mm along the y axis, respectively. At 5 years after distraction, the orbitale moved posterior 0.58 mm and the A point advanced an additional 2.08 mm along the x axis. Orbitale and A point descended 3.23 mm and 5.2 mm along the y axis, respectively. CONCLUSION: After Le Fort III advancement with distraction, the maxillary position remains stable and continues to advance minimally along the x axis and demonstrates more growth along the y axis over the long term. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Early Distraction for Mild to Moderate Unilateral Craniofacial Microsomia: Long-Term Follow-Up, Outcomes, and Recommendations.

BACKGROUND: There is controversy regarding the treatment of young patients with unilateral craniofacial microsomia and moderate dysmorphism. The relative indication for mandibular distraction in such patients poses several questions: Is it deleterious in the context of craniofacial growth and appearance? This study was designed to address these questions. METHODS: A retrospective review of patients undergoing mandibular distraction by a single surgeon between 1989 and 2010 was conducted. Patients with "moderate" unilateral craniofacial microsomia (as defined by Pruzansky type I or IIa mandibles) and follow-up until craniofacial skeletal maturity were included for analysis. Patients were divided into two cohorts: satisfactory and unsatisfactory results based on photographic aesthetic evaluation by independent blinded observers at the initial presentation and at the age of skeletal maturity. Clinical variables were analyzed to detect predictors for satisfactory distraction. RESULTS: Nineteen patients were included for analysis. The average age at distraction was 68.2 months and the average age at follow-up was 19.55 years. Thirteen patients (68.4 percent) had Pruzansky type IIA and six patients (31.6 percent) had Pruzansky type I mandibles. Twelve patients (63.2 percent) had satisfactory outcomes, whereas seven patients (36.8 percent) had unsatisfactory outcomes. Comparing the two cohorts, patients with satisfactory outcomes had distraction at an earlier age (56.4 months versus 89.8 months; p = 0.07) and a greater percentage overcorrection from craniofacial midline (41.7 percent versus 1.8 percent; p = 0.003). CONCLUSION: Mandibular distraction is successful in patients with mild to moderate dysmorphism, provided that there is a comprehensive clinical program emphasizing adequate mandibular bone stock, proper vector selection, planned overcorrection, and comprehensive orthodontic management. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Computer Simulation and Digital Resources for Plastic Surgery Psychomotor Education.

Contemporary plastic surgery residents are increasingly challenged to learn a greater number of complex surgical techniques within a limited period. Surgical simulation and digital education resources have the potential to address some limitations of the traditional training model, and have been shown to accelerate knowledge and skills acquisition. Although animal, cadaver, and bench models are widely used for skills and procedure-specific training, digital simulation has not been fully embraced within plastic surgery. Digital educational resources may play a future role in a multistage strategy for skills and procedures training. The authors present two virtual surgical simulators addressing procedural cognition for cleft repair and craniofacial surgery. Furthermore, the authors describe how partnerships among surgical educators, industry, and philanthropy can be a successful strategy for the development and maintenance of digital simulators and educational resources relevant to plastic surgery training. It is our responsibility as surgical educators not only to create these resources, but to demonstrate their utility for enhanced trainee knowledge and technical skills development. Currently available digital resources should be evaluated in partnership with plastic surgery educational societies to guide trainees and practitioners toward effective digital content.

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.

Soft-tissue profile changes following early Le Fort III distraction in growing children with syndromic craniosynostosis.

BACKGROUND: The purpose of this study was to characterize soft-tissue profile changes following Le Fort III (midface) distraction in growing patients with syndromic craniosynostosis. METHODS: The cohort consisted of 20 syndromic patients who underwent Le Fort III osteotomy with midface advancement using a rigid external distraction device. The mean age at surgery was 5.7 years (range, 3 to 12.5 years). Lateral cephalograms were obtained preoperatively (time 1), after distraction device removal (time 2), and 1 year after distraction (time 3). Ten skeletal hard-tissue and 11 soft-tissue profile landmarks were identified and digitized at time points 1, 2, and 3. The x and y displacement of each landmark was studied to determine the ratios for soft- to hard-tissue change. RESULTS: The horizontal ratio of soft- to hard-tissue change for nasal dorsum to orbitale was 0.73:1 and the soft-tissue tip of nose to the anterior nasal spine was 0.86:1. The horizontal ratio of soft-tissue A point to hard-tissue A point was 0.88:1. The horizontal ratio of the upper lip position to the labial surface of maxillary incisor was 0.88:1. The ratio for nasal tip elevation to the anterior nasal spine advancement was 0.27:1. CONCLUSIONS: The result of this study supported the hypothesis that there exists a linear relationship between soft- and hard-tissue changes in the horizontal direction for the midface landmarks following Le Fort III distraction. However, there was a nonlinear relationship between soft- and hard-tissue changes in the vertical direction. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Surgical management of patients with a history of early Le Fort III advancement after they have attained skeletal maturity.

BACKGROUND: The classic Le Fort III procedure was recommended in syndromic craniosynostotic children to reduce exorbitism, improve airway function, and decrease dysmorphism. This study reports on a cohort of syndromic craniosynostosis patients who have undergone early primary subcranial (classic Tessier) Le Fort III advancement and who have been followed longitudinally through skeletal maturity and beyond. METHODS: In this study, the Le Fort III advancements all occurred between the ages of 3 to 5 years, with a mean age of 4.6 years. Subsequently, these early Le Fort III patients were followed throughout development with longitudinal dental, medical, radiographic, and photographic evaluations conducted through skeletal maturity and beyond. For study inclusion, the patients had to have preoperative medical photographs and cephalometric studies at 6 months and 1, 5, and 10 years postoperatively after the primary Le Fort III advancement as well as cephalometric documentation 6 months and 1 year after the secondary midface advancement after skeletal maturity. RESULTS: After early or primary Le Fort III advancement, there was no evidence of relapse and only minimal anterior or horizontal postoperative growth of the midface. However, there was also a return of occlusal disharmony from "anticipated" mandibular growth, approaching a maximum at skeletal maturity. The dysmorphic concave facial profile and malocclusion, and airway and ocular considerations, provided the impetus for secondary midface surgery after skeletal maturity was attained. CONCLUSION: The data demonstrate that early Le Fort III advancement performed before the age of mixed dentition does not obviate the need for a secondary advancement after skeletal maturity is reached. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

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.