Correction of facial deformity using a Red III device in a patient with Antley-Bixler syndrome

Antley-Bixler syndrome was first described in 1975 and to date at least 44 cases have been reported. In addition to brachycephaly, this syndrome is associated with midface hypoplasia, bilateral radiohumeral synostosis, multiple joint contractures, long bone fractures, dysplastic ears, "pear shaped" nose, and occasionally urogenital or cardiac defects. The authors report a case in a 23-year-old man with severe midface deficiency treated using distraction osteogenesis. A modified Le Fort III osteotomy was performed, and nasal subunit and remaining Le Fort III segment was distracted separately. The classic RED II design was found to be insufficient, so the authors added an extra horizontal bar to the system and converted it to a RED III design. Midface advancement was 11 mm from the porion to the orbitale and 31 mm at point A. Nasal advancement at the nasal root level was 5 mm. There were no complications, and at 4 months after surgery, advancement was stable without any relapse.     

Distraction osteogenesis in the surgical management of syndromic craniosynostosis: a comprehensive review of published papers

Our aim was to summarise current published evidence about the prognosis of various techniques of craniofacial distraction osteogenesis, particularly its indications, protocols, and complications. Published papers were acquired from online sources using the keywords “distraction osteogenesis”, “Le Fort III”, “monobloc”, and “syndromic craniosynostosis” in combination with other keywords, such as “craniofacial deformity” and “midface”. The search was confined to publications in English, and we followed the guidelines of the PRISMA statement. We found that deformity of the skull resulted mainly from Crouzon syndrome. Recently craniofacial distraction has been achieved by monobloc distraction osteogenesis using an external distraction device during childhood, while Le Fort III distraction osteogenesis was used in maturity. Craniofacial distraction was indicated primarily to correct increased intracranial pressure, exorbitism, and obstructive sleep apnoea in childhood, while midface hypoplasia was the main indication in maturity. Overall the most commonly reported complications were minor inflammatory reactions around the pins, and anticlockwise rotation when using external distraction systems. The mean amount of bony advancement was 12.3 mm for an external device, 18.6 mm for an internal device and 18.7 mm when both external and internal devices were used. Treatment by craniofacial distraction must be validated by long-term studies as there adequate data are lacking, particularly about structural relapse and the assessment of function.     

牵引成骨术治疗青少年上颌骨严重发育不足的初步报告

目的 通过青少年上颌骨严重发育不足,特别是继发于唇腭裂术后严重畸形者行改良Ｌｅ Ｆｏｒｔ Ｉ型截骨,并通过颅骨外固定上颌骨牵引成骨前移术,对牵引成骨后硬组织改建及腭咽闭合功能进行初步探讨。方法 采用颅骨外固定牵引装置（ＫＬＳ－ＭＡＲＴＩＮ公司提供）对６例１１～１５岁唇腭裂术后继发上颌骨严重发育不足及发育性上颌骨严重后缩畸形的青少年实施牵引成骨术。运用正颌外科电脑分析软件对术前、术后头颅定位Ｘ线侧位     

Respiratory outcome of midface advancement with distraction: a comparison between Le Fort III and frontofacial monobloc

Upper airway stenosis in patients with faciocraniosynostosis is very common and often severe. Midface advancement, either with a Le Fort III or concomitantly to a monobloc frontofacial advancement, may prevent a tracheotomy or result in its ablation. The amelioration of respiratory function appears to be much better if the midface advancement is combined with distraction osteogenesis, although large studies with long-term follow-up are rare. In this study, we reviewed the respiratory outcome between Le Fort III with distraction and monobloc advancement with distraction in 54 faciocraniosynostotic patients. Early respiratory results of both procedures were very good and stable at long-term follow-up. The choice between a Le Fort III and a monobloc procedure is made on the basis of presenting morphology, previous surgery, and age. Both can be expected to give a long-lasting improvement of upper airway obstruction.     

Le Fort III distraction osteogenesis: modification of the intraoral splint in the rigid external distraction

Le Fort III distraction osteogenesis (DO) is a useful procedure for treating midface hypoplasia. The external distraction system has the advantage of allowing modification of the distraction vector during the activation period. This system needs an intraoral splint for the anchorage points. The intraoral splint has an important role in conducting the distraction force to the midface and in forming the advancement vector. The authors developed a modified type of splint to improve the outcome of Le Fort III DO.The intraoral splint is composed of a modified headgear face bow, molar bands, and a palatal acrylic plate without covering the teeth. Four suspension wires were applied to reinforce the stability. The zygomatic bones and the loops of the splint are selected as 4 anchorage points for Le Fort III DO. This procedure was applied to 2 patients.The midface had an advancement of more than 20 mm in both patients. The dental movement of the upper incisors and the upper first molar was minimal.The dental effect was nearly absent. The amount and vector of distraction could be decided under direct observation without blocking the dental occlusion. The vector of distraction could be adjusted during the activation period. The results of DO were satisfactory.     

Segmental distraction of the midface in a patient with Crouzon syndrome

We treated midface hypoplasia in a 20-year-old woman with Crouzon syndrome using a rigid external distraction device. The patient showed severe exophthalmos and maxillary retrusion, although relatively good occlusion had been achieved by long-term orthodontic procedures. We considered that our patient's particular condition could not be resolved by the usual Le Fort III osteotomy/midface distraction procedure, so we devised a segmental approach. The midface, mobilized by Le Fort III osteotomy, was divided into two segments by Le Fort I osteotomy; each fragment was connected to the rigid external distraction device to be distracted separately. Distraction was begun after 1 day at 1 mm/day. The upper and lower segments were distracted over 17 and 12 days, respectively. The patient's occlusion was fully corrected, and her facial contour was significantly improved. After 3 weeks of consolidation, we removed the distraction device. The clinical course was without complication, and no relapse was observed on the cephalogram or computed tomography scan obtained 1 year after the procedure. Our modified technique was helpful in increasing the usefulness of the external distraction system and in refining the midface distraction procedure.     

Le Fort III external midface distraction: surgical outcomes and skeletal stability

A retrospective study on the stability of Le Fort III midface advancement with distraction in craniofacial dysostosis. Twenty-six surgical procedures for midface advancement were performed between 2000 and 2009. Subjects were 14 consecutive patients who underwent Le Fort III osteotomy with midface advancement using a rigid external distraction device (Synthes) in the Dep of Maxillo-Facial Surgery of the University "Sapienza" of Rome. In this study, 8 patients (n = 3 females, n = 5 males) affected by craniofacial dysostosis (Crouzon, n = 5; Apert, n = 3; Pfeiffer syndrome, n = 1; and other, n = 1) were selected on the basis of age and radiologic documentation. The mean age at the time of the procedure was 8.6 years, and all the patients had at least 1 year of follow-up after distraction.The radiologic documentation of each case was composed of posteroanterior and lateral cephalograms preoperatively (T1), at the time of removal of the distractor device (T2), and at follow-up (T3).The average latency period before activation of the distractor was 5 days. The device was activated on an average of 15 mm (range, 7-22 mm) as recorded on the device at a rate of 1 mm/d. Activation period ranged from 7 to 15 days, and the consolidation period was 8 to 12 weeks.Seven anatomic landmarks and 5 cephalometric measurements were identified on the lateral cephalograms at each of the 3 time periods. The cephalograms underwent digital analysis via Dolphin imaging digitalization software by 2 operators independently. The displacement of each identified landmark was recorded and examined in relation to their position at each time point.Cephalometric analysis revealed stability of Le Fort III midface advancement during follow-up. However, bone growth after midface advancement is limited, and as reported in the literature, almost no spontaneous growth is present because of the intrinsic nature of malformations. Patients treated in the growing age benefit from midface advancement, with resolution of exophthalmos, ocular bulb exposure, airway impairment, and good aesthetic outcome.     

Midface distraction to alleviate upper airway obstruction in achondroplastic dwarfs.

OBJECTIVE: The use of midface distraction in patients with achondroplasia and upper airway obstruction secondary to midface hypoplasia has not been reported. In this report, we review the treatment of two patients with severe midface hypoplasia and obstructive sleep apnea secondary to achondroplasia using midface distraction osteogenesis. DESIGN, SETTING, AND PATIENTS: Two patients with achondroplastic dwarfism and midface hypoplasia with airway obstruction were treated in a tertiary referral center for craniofacial disorders. RESULTS AND CONCLUSIONS: Both patients had their tracheostomies decannulated after midface distraction of 25 mm. Midface distraction osteogenesis is useful to alleviate upper airway obstruction from midface hypoplasia seen in achondroplasia.     

Successful Treatment of Postoperative Mouth Opening Limitation Following Le Fort III Distraction with Bilateral Coronoidectomies

Introduction

Distraction osteogenesis is a powerful tool in craniomaxillofacial surgery, allowing for large advancements of osteotomized segments in the setting of a restrictive soft tissue envelope. Despite its benefits, distraction can have negative functional consequences. We present a case of a patient with Crouzon syndrome who developed reduced mouth opening capability after a Le Fort III midfacial advancement with rigid external distraction.

Technique

Radiographic evaluation revealed that the coronoid process was restricting the normal excursion of the mandible by contacting the posterior zygoma. The patient was subsequently treated with a bilateral coronoidectomy via an intraoral approach, which improved his interincisal opening. Maximum interincisal distance was improved from 18 mm to 33 mm following bilateral cornoid resection.

Conclusion

We report coronoid impingement as a potential complication after Le Fort III distraction. Such a finding suggests the need for a detailed vector analysis in cases undergoing midface advancement with distraction. Post-distraction coronoidectomy is a useful surgical procedure to treat mouth opening limitation due to coronoid impingement against the zygoma after midfacial advancement.

     

Upper airway changes in syndromic craniosynostosis patients following midface or monobloc advancement: Correlation between volume changes and respiratory outcome

In syndromic craniosynostosis patients, respiratory insufficiency may be a pressing indication to surgically increase the patency of the upper airway by midface or monobloc advancement. In this study the volume changes of the upper airway and the respiratory outcome following midface (Le Fort I or III) or monobloc advancement in ten syndromic craniosynostosis patients are evaluated. Pre- and postoperatively, the airway volume was measured using a semi-automatic region growing method. Respiratory data were correlated to the volume measurements.In nine patients the outcome of upper airway volume measurements correlated well to the respiratory outcome. Three of these patients showed a minimal airway volume gain or even volume loss, and no respiratory improvement was found. In one monobloc patient improvement of the respiratory outcome without an evident volume gain of the upper airway was found.The majority of patients with Le Fort III advancement showed respiratory improvement, which for the greater part correlated to the results of the volume analysis. In monobloc patients the respiratory outcomes and volume measurements were less obvious. Preoperative endoscopy of the upper airway is advocated to identify the level of obstruction in patients with residual obstructive sleep apnoea.     

Simultaneous hybrid of maxillary Le Fort I halo distraction and mandibular set-back for patients with severe cleft jaw deformity

One of the surgical tactics and retrospective chart review of clinical cases are described for severe maxillo-mandibular discrepancy. The recently developed Le Fort I Halo distraction combined with mandibular sagittal splitting osteotomy is initially carried out simultaneously. Materials include six adult patients revealing severe jaw deformity with mandibular prognathism somehow ranging from 17-19 years of age. The required adjustment of the maxillo-mandibular discrepancy ranged from 14-23 mm to obtain the preferred occlusion. The simultaneous combination of over 10-mm maxillary Le Fort I Halo distraction with mandibular set-back secured rigidly by sagittal splitting was accomplished. The amount of mandibular set-back ranged from 4-6 mm. The amount of maxillary Le Fort I halo distraction ranged from 10-17 mm (Table I). The retention period of the halo brace was 21-22 days. In addition, the Delair type of face mask was used for 3-4 months as a night splint for consolidation after removal of the halo brace. Satisfactory maxillary distraction and mandibular set-back as planned preoperatively was obtained in all six cases. No particular postoperative complications were noticed. Compared with standard Le Fort I advancement for cleft patients, more advancement can be obtained easily with halo distration, particularly in cases where a large amount of advancement > 10 mm is required. This combination is worthwhile for a severe cleft jaw deformity, and is an alternative for standard double jaw osteotomy.     

Additional orthognathic surgery following Le Fort III and monobloc advancement

Severe midface hypoplasia in patients with various craniofacial anomalies can be corrected with Le Fort III or monobloc advancement. Often additional corrective orthognathic surgery is indicated to achieve Class I occlusion and a normal inter-jaw relationship. This study evaluated the incidence of, and the surgical indications for, secondary orthognathic surgery following Le Fort III/monobloc advancement. The total study group consisted of 41 patients: 36 patients with Le Fort III advancement and 5 patients with monobloc advancement. Seven patients underwent additional orthognathic surgery. Of the resulting 18 non-operated patients older than 18 years at the end of follow-up, Class I occlusion was observed in 11 patients. In the remaining patients malocclusions were dentally compensated with orthodontic treatment. None of the patients was scheduled for additional orthognathic surgery due to the absence of functional complaints and/or resistance to additional surgery. Le Fort III and monobloc advancement aim to correct skeletal deformities on the level of zygoma, orbits, nasal area and forehead, but Class I occlusion is frequently not achieved. Additional orthognathic surgery is often indicated in patients undergoing Le Fort III or monobloc advancement. Naso-endoscopic analysis of the upper airway and the outcomes of sleep studies may influence the orthognathic treatment plan.     

Midface distraction osteogenesis: internal vs. external devices

This study compares internal and external distraction devices in the treatment of midface retrusion. 20 patients were treated with midface distraction (12 Crouzon, 4 Apert, 4 others); 12 with internal distraction (MID device), 8 with external distraction (Red or Blue device). The two groups were compared regarding operation time, peroperative blood loss and complications. The groups were comparable regarding patient age, sex, weight and diagnosis. In the MID-group, 7 of 12 patients (58%) underwent Le Fort III, 5 underwent 12 monobloc (32%). In the Blue device group, three of eight patients underwent Le Fort II (38%), three of eight underwent Le Fort III (38%), and two of eight underwent monobloc (25%). Operation time was shorter in the Blue device (mean 298 min) than in the MID group (mean 354 min). Peroperative blood loss and complication rates were similar. The internal distraction device is the ‘gold standard’ for treating midface retrusion. The use of an external distraction device in midface distraction osteogenesis is associated with a shorter operation time; peroperative blood loss and complications were similar. An external device affords better 3-dimensional control during the distraction process, so external distraction is preferable in patients who will tolerate this treatment.     

Le Fort III osteotomy: a new internal positioned distractor.

PURPOSE: The treatment of midfacial retrusion, due to congenital midfacial hypoplasia or trauma, is a challenging problem. Conventional Le Fort III osteotomies, with the surgical mobilisation of the entire midface, offers one solution. Distraction osteogenesis has become an accepted, and often preferred, technique for the treatment of severe midfacial hypoplasia. Existing distraction devices have several disadvantages. To overcome some of these disadvantages, an internal and temporal positioned distraction device was developed. PATIENTS AND METHODS: The new, internal positioned distractor device was used in 5 patients (age range, 8 to 15 years). Nine distractors were used in 4 patients with midfacial retrusion and 1 patient with hemifacial microsomia. RESULTS: The preoperative planned position of the osteotomized Le Fort III segment was obtained without any complications in all cases. Midface advancement, measured as the anterior displacement of the infraorbital rim, ranged from 14 to 20 mm. The distractor was almost invisible and did not disturb daily activities of the patient. Activation of the distractor was performed by rotation of a small lateral activation rod. CONCLUSION: These preliminary results encourage the further use of the new distractor device in a larger series of patients.     

Respiratory outcome of mid-face advancement with distraction: a comparison between Le Fort III and frontofacial monobloc

Upper airway stenosis in patients with faciocraniosynostosis is very common and often severe. Mid-face advancement, either with a Le Fort III or concomitantly to a monobloc frontofacial advancement, may prevent a tracheotomy or result in its ablation. The amelioration of respiratory function appears to be much better if the mid-face advancement is combined with distraction osteogenesis, although large studies with long-term follow-up are rare. In this study we reviewed the respiratory outcome between Le Fort III with distraction and monobloc advancement with distraction in 55 faciocraniosynostotic patients. Early respiratory results of both procedures were very good and stable at long-term follow-up. The choice between a Le Fort III and a monobloc procedure is made based on presenting morphology, previous surgery, and age. Both can be expected to give a long-lasting improvement of upper airway obstruction.     

New osteotomies for midface advancement in patients with Crouzon syndrome

In some patients with Crouzon's syndrome the typical midface advancement can produce an excessive nasal protrusion which calls for subsequent rhinoplasty. Proposed modification of classical Le Fort III procedure consists in midface osteotomy and external distraction without median orbital walls and nasal component. Limited surgical approach through vestibular incision and split lower eyelids makes the operation quite easy and less extensive. So far, three adult patients were successfully treated according to described modality.     

Rigid external distraction of the midface with direct wiring of the distraction unit in patients with craniofacial dysplasia

The development of craniofacial distraction techniques represents a significant advancement in the management of craniofacial dysplasias. For distraction of the midface after Le Fort III osteotomy, two levels of anchorage to the moving segments are necessary. In this report, the authors connected the distraction forces directly to the infraorbital rims, using a surgical wire for superior anchorage. They performed this technique in four patients with craniofacial dysplasia for the management of severe maxillary hypoplasia using RED II system. This configuration not only simplified the placement of the system but also made the removal of the device much easier. The authors did not have any problems with the wire traction unit, such as wire breakdown or bony tunnel disruption. It is a cheap, simple, and effective configuration that can be easily performed without any complication.     

LeFort III internal distraction in syndromic craniosynostosis

Distraction osteogenesis of the mid-face alleviates the requirements of substantial autogenous bone grafts and donor site morbidity and alleviates the restriction of the soft-tissue envelope in gaining advancement of the mid-face. A prospective study, over a 14-month period, was initiated to evaluate the results of seven consecutive patients undergoing mid-facial advancement who were treated with Le Fort III internal distraction using the MID device. All patients had syndromic craniosynostoses and six patients had previously undergone fronto-orbital advancement. Four patients had symptoms of airway obstruction and one patient was tracheostomy dependent. Six of the patients completed the distraction as planned. Lateral cephalograms were analyzed and the mean linear distraction was 18 mm with a range of 8 mm to 23 mm. Complications included infection (one patient), mechanical failure (one patient), intraoperative fragment dysjunction (three patients), velopharyngeal insufficiency (one patient), conjunctivitis (two patients), trismus (six patients), and bony irregularities. It was concluded that in our Unit's future protocol for managing infant syndromic synostoses, synostectomies and cranial vault remodeling will be undertaken in infants as before, but that in early childhood, patients with severe mid-facial hypoplasia and/or respiratory compromise will be offered distraction osteogenesis as a substitute for the traditional Le Fort III advancement and bone grafting.     

Maxillary distraction resulting in facial advancement at Le Fort III level in cleft lip and palate patients: a report of two cases

An 18-year-old female and a 14-year-old male who had previously received surgery for primary repair of a nonsyndromic cleft lip and palate (including alveolar defect bone grafting) unintentionally developed facial advancement at the Le Fort III level after surgical correction of their maxillary hypoplasia. The Le Fort I osteotomy, originally performed for their maxillary dentoalveolar hypoplasia, was an incomplete osteotomy. It was performed without down-fracture, leaving the pterygomaxillary and septal junctions intact. The gradual advancement of the maxilla during distraction osteogenesis was planned to correct the hypoplastic maxilla, and also prevent subsequent hypernasality; however, during the distraction procedure by means of a rigid external device both patients developed an unintentional facial advancement at the Le Fort III level.     

A new Le Fort I internal distraction device in the treatment of severe maxillary hypoplasia.

PURPOSE: The purpose of this pilot study was to test a new Le Fort I internal distraction device. PATIENTS AND METHODS: A new internal Le Fort I distraction device designed by 1 of the authors was used in 3 patients with cleft lip and palate and severe maxillary hypoplasia who needed maxillary advancements in excess of 12 mm. Presurgical planning used CASSOS (SoftEnable Technology, Ltd, Hong Kong SAR, China) prediction tracing software and a stereolithographic model to calculate the distraction vector. The distractors were pre-bent and installed on the stereolithographic model and activated to advance the maxilla. Surgery was performed in a conventional manner, and distraction was started after a 7-day latency phase at the rate of 1 mm/day and continued until the presurgical plan was achieved. The distractor was removed after a 3-month consolidation phase. Cephalometric radiographs were taken at the completion of each phase. RESULTS: This new Le Fort I internal distraction device successfully distracted the maxillae as planned in all 3 patients. At the end of the distraction phase, the maxillary advancement was measured at 15.8 mm, 15.8 mm, and 13.5 mm, respectively. In each patient, a clockwise rotation of the maxilla was observed with a tendency to a posterior open bite. Postoperative radiographs also showed that the actual distraction vectors differed from the planned vectors. After the consolidation phase, radiographs showed a relapse of 2.6 mm, 0 mm, and 5.0 mm, respectively. There was no further relapse on 3-month follow-up radiographs. Each case showed radiographic evidence of excellent new bone formation at the osteotomy sites. CONCLUSION: The new Le Fort I internal distraction device produced the necessary advancement in all 3 patients. The study also showed that the actual distraction vector differed from the planned vector. This discrepancy was caused by a clockwise rotation of the maxilla during the distraction. Finally, the study showed a variable relapse rate not previously reported in maxillary distraction.