Modified Anterior Z Le Fort III Osteotomy.

Introduction: Surgery of the midface for non-syndromic patients with maxillo-malar hypoplasia exclusively has had many breakthroughs lately. The surgical techniques for midface surgery are varied and must be selected according to the patient's problem. Purpose: The technique is a modification of the Z Osteotomy, the Modified Anterior Z Le Fort III Osteotomy (MAZLFIIIO), is used to correct posteroanterior midface deficiency of 6 mm at most. It can be combined simultaneously with a Le Fort I osteotomy if a greater maxillary advance is needed. MAZLFIIIO advances midface without grafts or distraction osteogenesis. The anterior reposition of the midface is calculated in millimetres and depends on the relation of the eye's most inferior point, as well as the orbital's hard and soft tissues. It is a predictable osteotomy, only for patients without vertical excess of the midface. The osteotomy planned is drawn over a stereolithographic model, and then Z-shaped surgical splints are fabricated over it. Transverse midface deficiencies of no more than 4 mm can also be corrected during the malar osteotomy. A great number of patients are operated exclusively with Le Fort I osteotomies, leaving behind an undesirable step between the upper part of the midface and the Le Fort I osteotomy. Methods: The Modified anterior Z Le Fort III Osteotomy (MAZLFIIIO) was performed in seven patients, from 2016 to 2018. Results: The results obtained with the proposed osteotomy notably improve the appearance of the patient and provide a better protection of the lower part of the eye, while widening the maxillary sinus. Conclusion: We consider that maxillo-malar hypoplasias are very common; the surgeon pretends not to give importance to the deformity, exclusively using an advance Le Fort type osteotomy, with both functional and aesthetic treatment being limited. In this article, we give all the elements to perform this Le Fort III Modified Osteotomy in anterior Z, and that it is a combined and routine procedure in midface surgery.

Subcranial Midface Advancement in Patients with Syndromic Craniosynostosis.

Patients with syndromic craniosynostosis can present with midface hypoplasia, abnormal facial ratios, and obstructive sleep apnea. These symptoms can all be improved with midface advancement, but it is essential to evaluate the specific morphologic characteristics of each patient's bony deficiencies before offering subcranial advancement. Midface hypoplasia in Crouzon syndrome is evenly distributed between the central and lateral midface and reliably corrected with Le Fort III distraction. In contrast, the midface hypoplasia in Apert/Pfeiffer syndromes occurs in both an axial and a sagittal plane, with significantly more nasomaxillary hypoplasia compared with the orbitozygomatic deficiency.

Osteotomía Le Fort III modificada en un paciente no sindrómico / Modified Le Fort III osteotomy in a non-syndromic patient

Las discrepancias dento-esqueletales severas presentan un reto para el cirujano maxilofacial, existe una versatilidad de osteotomías para las diferentes anomalías del crecimiento y desarrollo, sin embargo, son pocas las que permiten mejorar la proyección malar. La osteotomía Le Fort III modificada fue utilizada en un principio en pacientes con algún síndrome craneofacial, pero actualmente es una alternativa útil para discrepancias severas en pacientes no sindrómicos, ya que permite un avance cigomático-maxilar, favorece la proyección malar y disminuye la exposición escleral con un número limitado de complicaciones. Se presenta el caso de un paciente masculino de 33 años con antecedente de fractura orbitocigomática y piso orbitario izquierda con una discrepancia dento-esqueletal severa, la que fue tratada mediante osteotomía Le Fort III modificada, osteotomías sagitales mandibulares para un avance máxilo-malar y retroceso mandibular respectivamente y una genioplastia de avance; logrando un resultado funcional y estético adecuado.

The dento-skeletal severe discrepancies present a challenge for the maxillofacial surgeon, there is a versatility of osteotomies for the different growth and development anomalies, however, a few of them make possible to improve malar projection. The modified Le Fort III osteotomy was originally used in patients with some craniofacial syndrome, but now it is a useful alternative for severe discrepancies in non-syndromic patients, since it allows a zygomatic-maxillary advance, favors malar projection and reduces scleral exposure with a limited number of complications. The case of a 33-year-old male patient with a history of orbitozygomatic fracture and left orbital floor with a severe dento-skeletal discrepancy is presented, who was treated by modified Le Fort III osteotomy, mandibular sagittal osteotomies for maxillo-malar advancement and retrogression mandibular respectively and geniplasty for advance; achieving a suitable functional and aesthetic result.

Virtual planning in Le Fort III distraction osteogenesis: A case series.

The objective of this study is to determine the value of using 3D planning tools and 3D printed cutting guides in Le Fort III osteotomies with external frame distraction osteogenesis. The process of planning and transfer of the virtual planning to the operating room is illustrated with 5 case. The virtual planning is transferred to the operating room using a 3D-printed supra-orbital reference bar with puzzle connections for the planned osteotomy guides. Different systems are presented to transfer the vector of distraction and the position of the external midface distractor. Three-dimensional planning tools and cutting guides help to design the Le Fort III osteotomy and the distraction vector, to anticipate possible difficulties, and to avoid adverse events.

Long-term Management of a Patient with Apert Syndrome.

AIM AND OBJECTIVE: To present an Apert syndrome patient with midfacial growth deficiency treated with Le Fort III distraction osteogenesis and subsequent two-jaw surgery. BACKGROUND: Apert syndrome is expressed as a severe and irregular craniosynostosis, midfacial hypoplasia, and symmetric syndactyly in the fingers and toes. For craniosynostosis syndromes, treatment planning is complex due to the disharmony between facial profile and occlusion. CASE DESCRIPTION: A 4-year-and-5-month-old boy, diagnosed with Apert syndrome, showed a concave profile accompanied with midfacial hypoplasia, moderate exorbitism, a reversed occlusion of -10.0 mm, an anterior open bite of -5.0 mm, and skeletal class III jaw-base relationship. The patient, aged 15 years and 4 months, underwent a Le Fort III osteotomy, and subsequent osteodistraction was performed via a rigid external distraction (RED) device. His midfacial bone was advanced by approximately 7.0 mm. One year after the distraction, preoperative treatment with 0.018-in preadjusted edgewise appliances was initiated. Two-jaw surgery with a Le Fort I osteotomy and bilateral sagittal split ramus osteotomy was performed after 42 months of preoperative orthodontic treatment. At the age of 20 years and 9 months, his facial profile dramatically changed to a straight profile, and an acceptable occlusion with an adequate interincisal relationship was obtained. A functional occlusion with an excellent facial profile was maintained throughout the 2-year retention period, although the upper dental arch width was slightly decreased, resulting in the recurrence of the left posterior crossbite. CONCLUSION: Our report indicates the necessity of long-term follow-up in patients with craniosynostosis because of syndrome-specific growth and methodologically induced relapse. CLINICAL SIGNIFICANCE: The two-stage operation combining early distraction osteogenesis and postgrowth orthognathic surgery proves to be an effective therapy for correcting midfacial hypoplasia and skeletal mandibular protrusion caused by Apert syndrome.

Midface distraction osteogenesis using a modified external device and 3D virtual simulation: technical note.

INTRODUCTION/BACKGROUND: Distraction osteogenesis (DO) with an external distraction device such as the rigid external distraction frame has become an established method for treating midface hypoplasia in faciocraniosynostosis. It allows for greater advancement of the midface in comparison with traditional Le Fort III osteotomies, associated or not with fronto-orbital osteotomies (Le Fort IV). However, the forward movement of the bone segments may not always be performed obeying an ideal distraction vector, resulting in asymmetries, anterior open bite, and loosening of screws. In addition, the cost of the distraction devices is significant and may preclude their routine use in developing countries. METHOD: We present an alternative device and method for craniofacial advancement in a clinical case of Crouzon's syndrome. RESULTS: A 3D virtual simulation of the distraction vector and a modified external device were used in the current case. CONCLUSION: The alternative external device in this case proved to be safe, effective, and reliable.

Surgical-Orthodontic Considerations in Subcranial and Frontofacial Distraction.

Subcranial and frontofacial distraction osteogenesis have emerged as powerful tools for management of hypoplasia involving the upper two-thirds of the face. The primary goal of subcranial or frontofacial distraction is to improve the orientation of the upper face and midface structures (frontal bone, orbitozygomatic complex, maxilla, nasal complex) relative to the cranial base, globes, and mandible. The various techniques used are tailored for management of specific phenotypic differences in facial position and may include segmental osteotomies, differential vectors, or synchronous maxillomandibular rotation.

Retropalatal Cross-Sectional Area Is Predictive of Obstructive Sleep Apnea in Patients With Syndromic Craniosynostosis.

OBJECTIVE: There is a high rate of obstructive sleep apnea (OSA) in patients with syndromic craniosynostosis (SCS). Little is known about the airway anatomy in this population. The purpose of this study is to characterize the 3 dimensional (3D) upper airway in patients with SCS with and without OSA. DESIGN: This is a retrospective study of patients with SCS treated at Boston Children's Hospital from 2000 to 2015. Patients were divided into OSA and no-OSA groups based on polysomnography. Predictor variables included age, sex, body mass index (BMI), and 3D upper airway measurements. The primary outcome variable was the presence or absence of OSA. Secondary outcome variables were apnea-hypopnea index and oxygen saturation nadir. Descriptive and bivariate statistics were computed, and significance was set as P < .05. RESULTS: There were 24 patients: 16 in the OSA group and 8 in the no-OSA group. The 2 groups did not differ significantly by age, BMI, or syndromic diagnosis. The presence of OSA was associated with a smaller minimum retropalatal cross-sectional area (minRPCSA; P < .001). In a logistic regression model controlling for age, sex, and upper airway length, minRPCSA was the primary predictor of OSA (P ≤ .002). Receiver operating characteristic analysis determined minRPCSA = 55.3 mm2 to be the optimal diagnostic threshold for OSA, with sensitivity = 100% and specificity = 87.5% (P < .001). CONCLUSION: A minRPCSA ≤55.3 mm2 is predictive of the presence of OSA in patients with SCS.

Midface correction in patients with Crouzon syndrome is Le Fort III distraction osteogenesis with a rigid external distraction device the gold standard?

INTRODUCTION: Le Fort III distraction osteogenesis with a rigid external distraction device is a powerful procedure to correct both exorbitism and impaired airways in faciocraniosynostosis. The aim of this study was to investigate treatment effect, perioperative parameters and volumetric outcomes after Le Fort III distraction osteogenesis in patients with Crouzon syndrome in a retrospective study design and to explore potential strengths and weaknesses of this procedure. MATERIALS AND METHODS: From June 2013 to February 2015, a total of nine children with Crouzon syndrome underwent Le Fort III distraction osteogenesis with a rigid external distraction device (RED device, KLS Martin, Tuttlingen, Germany). Along with perioperative parameters, sleep study reports, traditional cephalometric analysis, three-dimensional imaging and photographs were evaluated for severity of disease and therapeutic effect and structural and functional changes of the upper airway preoperatively, after device removal and one year postoperatively. RESULTS: Surgery for Le Fort III distraction was performed at a median age of 12.5 years (SD 2.5 months) with an average weight of 43.0 kg (SD 12.9 kg). Mean estimated blood loss was 535.7 ml (SD 128.1 ml), not requiring any red blood cell transfusions. Mean duration of surgery was 240 min (SD 30.6min), average hospital stay eight days (SD 0.5 days) with a planned median ICU stay of 1.7 days (SD 0.4 days) for all patients. There were a total of five minor complications. Exorbitism and Angle class III malocclusions were corrected in all patients. No patient showed velopharyngeal problems postoperatively. The average amount of distraction was 18.4 mm (14-26 mm). Average length of the distraction period was 18.3 days (SD 0.4 days), with a total distraction plus consolidation time of three months (SD 0.25 months). In two patients, vector correction was performed during distraction. A counterclockwise movement despite vector correction, clinically resulting in an open bite, was observed in one of these two patients. Eight of the nine patients showed a frontal overbite at the end of the distraction period. Cephalometric analysis revealed a significant increase of Sella-Nasion-Point A angle (SNA) from 76.0° (+/- 2.9; T1) to 86.0° (+/- 3.4; T2) (p = 0.006) and growth-related point A-Nasion-point B angle (ANB) from -4.8° (+/-3.7) to 5.7° (+/-4.8) (p = 0.001) from preoperatively to device removal and stable results one year postoperatively. Upper airway structure and respiratory function were improved clinically after the Le Fort III DO treatment in all cases with an average posterior airway space increase from 3199 mm3 (+/- 229.6 mm3) to 8917,7 ml (+/-415.1 mm3) (T1 to T2). Surgical outcome was judged good to excellent both by patients and families and the craniofacial team. CONCLUSION: Le Fort III DO with a rigid external distraction device in patients with Crouzon syndrome is a powerful and reliable surgical procedure that reliably produces a more significant change of appearance than most other single procedures routinely performed by craniofacial surgeons. It effectively treated sleep apnea in the affected patients. In our collective, the maxilla remained stable after advancement without any relapse, but there was no subsequent anterior growth on one year follow-up. Careful vector planning was able to avoid frontal open bite in eight patients. Complication rates were acceptably low and patients' functional and esthetic outcome was high.

Modified Le Fort III osteotomy: A simple solution to severe midfacial hypoplasia.

PURPOSE: There are multiple conditions that may affect the development of the middle third of the face and with varying degrees of severity. The surgical treatment alternatives for major midfacial sagittal deficiencies consist in Le Fort I, II, or III with conventional osteotomies or with distraction osteogenesis (DO). Both techniques have advantages and disadvantages that should be evaluated specifically in each case. The aim of this report is to present a group of patients with severe hypoplasia of the middle third of the face, with different origins, and their treatment with a Modified Le Fort III osteotomy and distraction osteogenesis, using a minimally invasive surgical approach. MATERIALS AND METHODS: The surgical technique was performed in a group of patients with severe hypoplasia of the middle third of the face, through a transconjunctival approach with lateral canthotomy and a trans-oral approach. The osteotomy consisted of a Le Fort III without the nasofrontal component. A rigid external distractor (RED) type II or internal distractor was installed. The amount of distraction, surgical time, blood loss, and complications were evaluated. RESULTS: A total of 7 patients underwent operation, 5 men and 2 women with an average age of 20.8 (range 11-41) years; 3 patients with Crouzon syndrome, 2 with Pfeiffer syndrome, 1 patient with cleft lip and palate sequel, and 1 with a severe non-syndromic class III. The average follow-up was 3.14 years. All patients achieved stable occlusion without postoperative changes, positive overbite and overjet, without relapse in the skeletal position. The average advancement was 14.7 (±4.07) mm, in 1.1 incisors, and 15.2 (±3.19) in point A. The average time of surgery was 2.78 (±0.64) hours, with an average blood loss of 240 (±48.6) ml. Four patients required a rhinoplasty in a secondary surgery. CONCLUSION: This technique shows a surgical approach with low morbidity, short surgery time, and low blood loss. It allows optimal resolution of severe hypoplasia of the middle third of the face with long-term stability. It avoids the use of grafts and osteosynthesis material. By not including the nasal pyramid in the osteotomy design, the size, position, and nasofrontal angle in patients with adequate facial balance is maintained. If nasal correction is necessary, a second surgery may be done. In cases of asymmetrical hypoplasia of the middle third, this osteotomy shows great versatility and can be done unilaterally and/or simultaneously combined with other distractions.

Is subcranial Le Fort III plus Le Fort I osteotomy stable?

The purpose of this study was to test whether associated subcranial Le Fort III (sLF III) and Le Fort I (LF I) osteotomies are stable after large advancements of the middle third of the face and maxilla. The authors designed a retrospective study and enrolled a sample of consecutive patients with midface hypoplasia treated with associated sLF III and LF I osteotomies in this IRB-approved study between September 2013 and February 2015. To test whether the long-term stability was satisfactory, the authors compared cephalometric changes from immediately after surgery to 18 months after surgery taken from multi-slice computed tomography using two different third-party imaging software programs. Statistical significance was set as P ≤ 0.05. The sample comprised 11 patients (mean age 23.84 ± 4.17 yr; 54% men). The mean advancement of the upper incisor immediately after surgery was 10.03 ± 1.6 mm. After 18 months, the position of the upper incisor did not vary significantly (10.18 ± 2.35 mm). All other cephalometric landmarks did not present statistically significant differences between immediately after and 18 months after surgery, with horizontal and vertical variations of less than one millimetre. This study supports that sLF III and LF I osteotomies are effective in maintaining stable horizontal and vertical skeletal positioning after surgery.

Management of nasolacrimal duct injuries in mid-facial advancement.

Nasolacrimal duct (NLD) may be injured following trauma. During certain surgical procedure, inadvertent damage to the nasolacrimal apparatus may occur as well. Such instances should be immediately managed to prevent any subsequent complications. The management of such inadvertent injuries may be accomplished only with a thorough anatomic comprehension and surgical expertise in the field of operation. It also requires the presence of armamentarium required for its management in situations when complications arise. The case report discusses one such rare complication of NLD injury during Le Fort III osteotomy and advancement effectively managed by bicanalicular stenting.

Upper airway outcomes following midface distraction osteogenesis: a systematic review.

The objective of this paper is to systematically review the airway outcomes following distraction osteogenesis of midface with the goal of (1) deriving clinically oriented insights and (2) identifying gaps in knowledge to stimulate future research. Medline, EMBASE and Cochrane databases were searched and studies were included if subjects of any age had midface retrusion/hypoplasia and underwent midface distraction osteogenesis. Outcome measures of interest were any respiratory or airway associated measures, and reports of adverse events. A total of 368 abstracts were generated from the literature searches; 16 studies met the criteria for data extraction and analysis. All 16 studies were observational. Generally, midface distraction osteogenesis was reported to improve respiratory status and was well tolerated. Specifically, favorable outcomes in cephalometry (9 studies), polysomnography (9 studies), and decannulation rates (8 studies) were reported. In conclusion, upper airway status was improved in most patients who underwent midface distraction osteogenesis, yet long-term results and consistent objective measures are lacking. Studies reviewed were retrospective case series and details regarding patients who did not improve were deficient. A standardized prospective multicenter cohort trial with long-term patient follow up is required.

A Case Report of RED II Distraction Osteogenesis and Early Rigid Fixation by Minimal Invasive Approach Le Fort III Osteotomy in Crouzon's Disease

PURPOSE: Rigid external distraction(RED) is a highly effective technique for correction of maxillary hypoplasia in patients with cleft or syndromic craniosynostosis. Despite many advantages of RED, it also has the problem of relapse as the conventional advancement surgery. Bicoronal approach, that is the common approach to gain access to the craniofacial skeleton, had some morbidity, such as hair loss, sensory loss, wide scar and temporal hollowing. We present our clinical experience of RED distraction with minimal invasive approach and early rigid fixation to overcome these disadvantages. METHODS: A 27-year-old female patient with Crouzon's disease underwent Le Fort III osteotomy and RED device application through the minimal invasive direct skin incisions. After the latent period of 5 days, distraction was undertaken until proper convexity and advancement were obtained. During the rigid retention period, inflammation occurred on the right cheek, and proper conservative managements were done including continuous irrigation. To maintain the stability of distraction, early rigid fixation was undertaken on the osteotomy sites through another skin incisions. Preoperative and postoperative orthodontic treatments were performed. Serial photographs and cephalometric radiographs were obtained preoperatively, after distraction and 6 months after distraction. RESULTS: The cephalometric analysis demonstrated postoperatively significant advancement of the maxilla and improvement of facial convexity. After 6-month follow-up period, the maxilla was stable in the sagittal plane and no relapse was found. Facial scars were not noticeable and other deformity and morbidity did not occur. CONCLUSION: This effective and stable technique will be a good alternative for the patients who need large amount of distraction and for adult patients with severe maxillary hypoplasia or syndromic craniosynostosis.

A Case Report of RED II Distraction Osteogenesis and Early Rigid Fixation by Minimal Invasive Approach Le Fort III Osteotomy in Crouzon's Disease

PURPOSE: Rigid external distraction(RED) is a highly effective technique for correction of maxillary hypoplasia in patients with cleft or syndromic craniosynostosis. Despite many advantages of RED, it also has the problem of relapse as the conventional advancement surgery. Bicoronal approach, that is the common approach to gain access to the craniofacial skeleton, had some morbidity, such as hair loss, sensory loss, wide scar and temporal hollowing. We present our clinical experience of RED distraction with minimal invasive approach and early rigid fixation to overcome these disadvantages. METHODS: A 27-year-old female patient with Crouzon's disease underwent Le Fort III osteotomy and RED device application through the minimal invasive direct skin incisions. After the latent period of 5 days, distraction was undertaken until proper convexity and advancement were obtained. During the rigid retention period, inflammation occurred on the right cheek, and proper conservative managements were done including continuous irrigation. To maintain the stability of distraction, early rigid fixation was undertaken on the osteotomy sites through another skin incisions. Preoperative and postoperative orthodontic treatments were performed. Serial photographs and cephalometric radiographs were obtained preoperatively, after distraction and 6 months after distraction. RESULTS: The cephalometric analysis demonstrated postoperatively significant advancement of the maxilla and improvement of facial convexity. After 6-month follow-up period, the maxilla was stable in the sagittal plane and no relapse was found. Facial scars were not noticeable and other deformity and morbidity did not occur. CONCLUSION: This effective and stable technique will be a good alternative for the patients who need large amount of distraction and for adult patients with severe maxillary hypoplasia or syndromic craniosynostosis.

Midfacial distraction osteogenesis of Crouzon syndrome with RED(Rigid External Distraction) system / 대한치과교정학회지

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.

Modification of Le Fort III Osteotomy and Fronto-orbital Advancement for the Correction of Crouzon's Disease

In 1912, Crouzon reported one of craniofacial dysostosis, which was inherited as an autosomal dominant trait and characterized by craniosynostosis, midface hypoplasia, and exorbitism. In 1950, the first midface advancement was performed by means of a total facial osteotomy by Sir Harold Gillies. In 1967, Tessier accomplished complete craniofacial dysjunction by performing Le Fort III-Tessier I osteotomy on the basis of Le Fort III fracture and laid the foundation for modern craniofacial surgery. Variable combinations of the surgeries and osteotomies are performed considering functional disabilities as well as patient's age, general condition, and aesthetic problems. The nineteen-year-old female patient complained of frog-like face and showed severe exorbitism and midface hypoplasia, while the occlusion, being relatively normal. We applied modified Le Fort III osteotomy and fronto- orbital advancement. Over the follow-up period of six months, her previous normal occlusion was preserved and the exorbitism and midface deformity largely improved aesthetically.

A case report of surgical correction of mandibular prognathism with midfacial deficiency using Le Fort III osteotomy

True midfacial deficiency is defined as a hypoplasia of various components of midface such as maxilla, orbit, zygoma and nasal bone. For treatment of these anomalies Le Fort III osteotomy and its modifications have been used traditionally. Le Fort III osteotomy is the method which advances maxilla with nasal bone and zygomatic bone at a time. At first midfacial osteotomy was introduced by Gillies to treatment of dentofacial deformity in 1950. In 1967 Tessier designed Le Fort III osteotomy according to Le Fort III midfacial fracture line and popularized to treat midfacial deficiency using coronal incision to appoach osteotomy sites. This is a case of patient who had mandibular prognathism with midfacial deficiency with severe discrepancy in maxillomandibular interrelation. First we performed Le Fort III osteomomy for zygomaticomaxillary advancement, and then carried out simultaneous two jaw surgery with Le Fort I osteotomy and BSSRO three months after first surgery.