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Objective: To evaluate patency of circummaxillary sutures in children with Apert, Crouzon, and Pfeiffer Syndromes and to compare it to a nonsyndromic matched control group. Design: Case–control study. Setting: Tertiary care public hospital. Materials and Methods: Thirty-eight computed tomography (CT) scans of patients affected by syndromic craniofacial synostosis (13 patients with Apert syndrome, 20 patients with Crouzon syndrome, and 5 patients with Pfeiffer syndrome), average age 5 ± 2.8 years, range 1.9 to 12 years, were compared to age- and sex-matched control CTs of 38 nonsyndromic children. Computed tomography scans of the study group had to be performed prior to any midfacial surgery. Main Outcome Measures: Midpalatal suture, zygomaticomaxillary sutures, and pterigomaxillary sutures were evaluated and scored. Results: The syndromic group showed a significant earlier ossification of all sutures compared to the nonsyndromic group. Significant differences were already present in early childhood and continued through adolescence. Conclusions: Based on the differences in terms of maxillary sutural ossification identified, midfacial hypoplasia does not seem to be only secondary to premature cranial base ossification, but also to primary synostosis of facial sutures, thus providing new insights into the pathogenesis of midface deficiency in children with craniofacial-synostosis. Care should be taken when planning any maxillary orthopedics, such as expansion or maxillary protraction, given the high frequency of early fusion of circummaxillary sutures.

Background: The goal of this study was to evaluate with a three-dimensional method the long-term quality of alveolar ossification in unilateral cleft lip and palate patients who underwent early secondary gingivoalveoloplasty according to the Milan surgical protocol. Methods: The sample consisted of 63 computed tomographic scans of unilateral cleft lip and palate patients in permanent dentition. The average age at the time of assessment was 15.7 years. Alveolar thickness, nasoalveolar height, nasal floor ossification, and hard palate morphology were evaluated using dental, axial, and coronal cuts on computed tomographic scans and three-dimensional models. All measurements were normalized and ratios of the affected side versus the nonaffected side were provided. Volume measurements and ratios of each hemimaxilla were added. The presence or absence of the permanent lateral incisor on the cleft side was also recorded. Results: Alveolar thickness and height were ideal or good, respectively, in 89.5 and 91.4 percent of the sample. Insufficient ossification (

Aim: Comparison of the long-term follow-up until the completion of growth of two homogeneous samples of children affected by hemifacial microsomia (HFM), one treated by mandibular distraction osteogenesis (DO) in the deciduous or early mixed dentition, the other not subjected to any treatment until adulthood. Material: Fourteen patients affected by vertically severe type I or II HFM were operated at an average age of 5.9 years with an average follow-up of 11.2 years. They were compared to a sample of eight patients who were never treated until the completion of growth. Methods: Mandibular vertical changes were measured on panoramic radiographs taken at different time points. Ratios between affected and non affected ramal heights were calculated and compared. Results: In the DO sample, after correction, mandibular vertical changes showed a gradual return of the asymmetry with growth in all patients. The ratio in the non treated sample was unchanged between the initial and the long term panoramic x-rays. Conclusion: The facial proportions of HFM patients are maintained, when not treated, throughout growth. The same proportions return to their original asymmetry after DO. Even though short term aesthetic and psychological advantages of distraction osteogenesis are well accepted, early surgery should only be applied after careful patient selection and honest clarification of the long term recurrence by genetically guided craniofacial growth pattern. © 2011 European Association for Cranio-Maxillo-Facial Surgery.

Summary Background Maxillary distraction osteogenesis (DO) in cleft lip and palate patients has been described by several authors, but most studies have a relatively short follow-up and do not clearly separate growing patients from non-growing patients. Method The records of 22 consecutive patients affected by cleft lip and palate, who underwent Le Fort I osteotomy and maxillary distraction with a rigid external distractor (RED), were reviewed. The sample was subdivided into a growing and a non-growing group. All patients had pre-DO cephalometric records, immediately post DO, 12 months post DO and long-term records with a long-term follow-up of >5 years (range 5–13 years). As a control sample for the growing group, cleft children with a negative overjet not subjected to distraction or any protraction treatment during growth were followed up until the completion of growth. Results The average maxillary advancement in the growing group was 22.2 ± 5.5 mm (range: 15–32 mm); in the non-growing group, it was 17.7 ± 6.6 mm (range: 6–25 mm). Excellent post-surgical stability was recorded in the adult sample. On the other hand, growing children had an average 16% relapse in the first year post DO and an additional 26% relapse in the long-term follow-up. Conclusions This study seems to point out that early Le Fort I DO allows for the correction of very severe deformities. It is followed by a relatively high amount of true skeletal relapse in children with cleft lip and palate. Prognosis should be discussed in depth with the family and true aesthetic and psychological needs assessed.

Background: Midface distraction osteogenesis (DO) in craniofacial synostosis (CFS) patients has been described by several authors. However, very few cephalometric and computed tomography (CT) long-term follow-up studies are available. Method: A total of 40 consecutive patients affected by CFS subjected to Le Fort III and rigid external distraction (RED) were examined. All patients had pre-DO cephalometric records, immediately post-DO and 6-12 months post-DO. Twenty-seven patients had mid-term records (3 years post-DO) and 20 patients had long-term records (5-10 years post-DO). Fourteen patients had CT data within 1-year of DO, while 10 patients had long-term CT data (range 5-9 years). Results: Excellent post-surgical stability was recorded. Short- and long-term CT data demonstrated excellent ossification at the osteotomy sites post-DO. In the growing patients, surface resorption in the zygomatic-temporal and in the subspinal area (p < 0.05) was observed in the long-term follow-up, as well as a mild increment of the corrected exorbitism (p < 0.05), as only appositional and no sutural growth occurs post Le Fort III, whereby orbital volume does not increase after surgery. Conclusion: Significant advancement of the midface can be achieved and maintained through Le Fort III and RED. In the long term, in growing patients, in general a class III malocclusion does not re-occur, but physiological remodelling processes at the maxillary-zygomatic level, not coupled with sutural growth, tend to mildly re-express the original midfacial phenotype and the exorbitism. © 2011 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

Bilateral cleft lip and palate patients may present a vertical excess of the premaxilla, which is a severe aesthetic and functional problem. Early surgical correction may lead to secondary growth impairment. We present a suggested protocol based on the severity of the vertical excess and on the age of the patient, which includes orthopedic, orthodontic, and surgical corrections. Patients are presented to elucidate each different approach.

Summary Aim After mandibular unilateral distraction osteogenesis (DO) a gradual reappearance of the vertical asymmetry during growth is observed. A pre- and post-surgical functional-orthodontic treatment was added to our distraction protocol in the attempt to increase long-term stability. In order to evaluate the actual efficacy of such a combined treatment, two samples of children affected by hemifacial microsomia were compared long-term. Material Ten children were treated by a combined orthodontic–distraction treatment, seven by distraction only. Method Only the vertical changes in the mandible and maxilla in the panoramic and postero-anterior cephalometric X-rays were measured. Results All of the patients showed a gradual return of the asymmetry with growth. Occlusal plane correction and, to a much lesser extent, mandibular vertical ramus height correction were better maintained over 5 years post-DO in the orthopaedic group. Conclusion Although orthopaedic treatment allows for a more stable occlusal plane and for a slower return of the mandibular vertical asymmetry, it has mainly a dento-alveolar effect. Therefore, the decision of applying an orthopaedic treatment associated with distraction, should be taken by surgeon and orthodontist together, considering both the advantages and the disadvantages of this treatment.

This is a longitudinal cephalometric study of patients with craniofacial synostosis (Crouzon's and Apert's) syndromes who underwent Le Fort III advancement during early childhood. The objectives of the study were to quantify presurgical growth, surgical results, and surgical stability as well as long-term postsurgical growth. Of the 31 patients operated on during their growth period (less than 10 years of age), 17 had sufficient records to be included in the study: at infancy, before the Le Fort III osteotomy (average age=7.3 years, range: 4.8-10 years), and immediately after surgery. Ten of the patients were followed radiographically long term for an average of 6.1+/-2.7 years (range: 4.5-10.8 years). The presurgical abnormal midfacial vertical growth pattern was accurately described with a progressive class III discrepancy and an increasing exophthalmos. Remarkable postoperative stability of the maxillary segment was observed. After surgery, growth of the midface was measured, together with minimal, if any, anterior growth, similar to the presurgical growth pattern and rate. No actual detrimental or beneficial effect of surgery on subsequent growth was seen. This study quantitatively confirms that the standard Le Fort III osteotomy is a stable and effective but generally not definitive procedure in childhood. This study might also serve as a control sample to compare with groups of patients undergoing distraction osteogenesis to verify the actual advantages and shortcomings of this alternative technique.

Summary Aim Prospective, longitudinal, clinical long-term follow-up study of a homogeneous sample of children affected by hemifacial microsomia and treated by mandibular distraction osteogenesis. Material Eight patients affected by types I and II hemifacial microsomia were operated on at an average age of 5.6 years with an average follow-up of 5.8 years. Methods Vertical changes were measured on postero-anterior cephalometric and panoramic radiographs taken sequentially. Results Angular changes of the infraorbital and nasal floor planes were not significant, showing that distraction osteogenesis starting after 5 years of age did not influence the maxillary skeletal base. Occlusal (plane) cant was reduced by 7° on average following distraction osteogenesis, showing good dentoalveolar plasticity. Mandibular vertical changes showed a gradual return of the asymmetry, with growth in all patients (the ratio between affected and non-affected rami returned by 77% of the correction obtained by means of distraction 5 years postoperatively). Conclusion Although aesthetic and psychological advantages of distraction osteogenesis are well accepted it should only be applied after careful patient selection and honest explanation of the long-term recurrence by genetically determined craniofacial growth patterns.