A Survey of Orthodontic Treatment in Team Care for Patients With Syndromic Craniosynostosis in Japan.

OBJECTIVE: To understand the actual condition of orthodontic treatment in team care for patients with syndromic craniosynostosis (SCS) in Japan. DESIGN: A nationwide collaborative survey. SETTING: Twenty-four orthodontic clinics in Japan. PATIENTS: A total of 246 patients with SCS. MAIN OUTCOME MEASURE: Treatment history was examined based on orthodontic records using common survey sheets. RESULTS: Most patients first visited the orthodontic clinic in the deciduous or mixed dentition phase. Midface advancement was performed without visiting the orthodontic clinic in about a quarter of the patients, and more than a half of the patients underwent "surgery-first" midface advancement. First-phase orthodontic treatment was carried out in about a half of the patients, and maxillary expansion and protraction were performed. Tooth extraction was required in about two-thirds of patients, and the extraction of maxillary teeth was required in most patients. Tooth abnormalities were found in 37.8% of patients, and abnormalities of maxillary molars were frequently (58.3%) found in patients who had undergone midface surgery below the age of 6 years. CONCLUSIONS: Many patients underwent "surgery-first" midface advancement, and visiting the orthodontic clinic at least before advancement was considered desirable. First-phase orthodontic treatment should be performed considering the burden of care. Midface advancement below the age of 6 years had a high risk of injury to the maxillary molars. This survey is considered useful for improving orthodontic treatment in team care of patients with SCS.

Clinical Experience of Treatment of Facial Malformations in Oto-Palato-Digital Syndrome: A Familial Patient.

Oto-palato-digital syndrome type 1 (OPD1) is an X-linked recessive disorder comprising characteristic facial appearances and skeletal alterations. The authors report OPD1 in a mother and her 2 sons who had multiple common congenital anomalies. Both of the brothers were born with mild hearing impairment, frontal bossing with prominent supraorbital ridges, downslanting palpebral fissures, dental malocclusion, and palatal clefts. They underwent a series of aesthetic surgeries for their facial malformations with good cosmetic results. The mother had a milder phenotype with less prominent craniofacial defects that did not require surgical correction. The older brother required a 2-jaw surgery whereas the younger brother required a surgically assisted rapid palatal expansion. In the second series of operations, both brothers underwent scraping of their prominent supraorbital ridge. They have been free of complications throughout their 16-year follow-up. The authors also screened the family for possible genetic etiologies and identified mutations in the causative gene of OPD1 on Xq28 in all 3 patients. In conclusion, the authors have identified 3 patients with OPD1, performed surgical treatments on the affected brothers and have obtained good reconstructive results. There are no reports involving OPD1 patients who have received good surgical treatment. With careful examination and identification of patients with characteristic facies and skeletal abnormalities, it is our opinion that the authors can help more patients with this disease through surgical management.

Backward distraction osteogenesis in a patient with severe mandibular micrognathia.

Maxillary skeletal prognathism can involve severe mandibular micrognathia with marked mandibular retrognathism or hypoplasia. For patients with such a condition, a conventional treatment is mandibular advancement by sagittal split ramus osteotomy (SSRO). This procedure has problems such as insufficient advancement, instability of jaw position, and postoperative relapse. Thus, in recent years, mandibular distraction osteogenesis has been used in some patients. Mandibular distraction has many advantages, but an ideal occlusion is difficult to achieve using this procedure. That is, 3-dimensional control cannot be attained using an internal device that is unidirectional. This report describes a case of severe mandibular micrognathia in a 14-year-old girl treated using backward distraction osteogenesis. This procedure was first reported by Ishii et al (Jpn J Jaw Deform 2004; 14:49) and involves a combination of SSRO and ramus distraction osteogenesis. In the present study, intermaxillary fixation in centric occlusion was performed after osteotomy, and proximal bone segments were distracted in a posterosuperior direction. This procedure is a superior surgical technique that avoids the drawbacks of SSRO and conventional mandibular distraction. However, it applies a large load to the temporomandibular joints and requires thorough management. Thus, careful evaluation needs to be made of the indication for backward distraction osteogenesis.

Dislocation of temporomandibular joint: complication of sagittal split ramus osteotomy.

Sagittal split ramus osteotomy (SSRO) has wide-ranging indications and results in simultaneous improvements in occlusion and facial appearance. Thus, it is the most frequently used osteotomy for jaw deformities. Its main intraoperative and postoperative complications are massive bleeding, atypical fractures, inferior alveolar nerve paralysis, and relapse. This report describes a case of temporomandibular joint dislocation, a rare complication of SSRO. The patient was a 23-year-old man with mandibular prognathism which was treated by bilateral SSRO. Postoperative x-ray showed that the left temporomandibular joint was dislocated anteriorly. Thus, the patient underwent reoperation, including reduction and re-fixation. Subsequently, it was discovered that the patient had a previous history of recurrent dislocation of the temporomandibular joint. This case illustrates the need for practitioners to be mindful of temporomandibular joint dislocation as a rare complication of SSRO.

Le Fort I distraction using internal devices for maxillary hypoplasia in patients with cleft lip, palate, and alveolus: complications and their prevention and management.

In recent years, advancement has been made in distraction osteogenesis in the facial area. It has been applied actively to treat maxillary hypoplasia in patients with cleft lip, palate, and alveolus. Le Fort I distraction using internal devices does not lead to disability in daily living, including during the retention period, and has low surgical invasiveness, facile management, and superior aesthetics. Thus, there have been occasional reports on this procedure in recent years. Because an internal device is unidirectional, the greatest disadvantage is the inability to adjust the direction of distraction after its placement. However, past reports have emphasized its advantages, and its complications have rarely been discussed. We performed Le Fort I distraction using internal devices on 15 young patients. Our results showed notable complications in a few cases. When serial cephalometric analysis was performed after maxillary distraction, it demonstrated that the maxilla assumes various three-dimensional distraction morphologies. In particular, cases with insufficient bone grafting of alveolar clefts developed collapse mainly in the alveolar cleft region during postoperative distraction. Mobility and deviation of the maxillary bone fragment occurred. We implemented preventative measures against complications such as using modifications to place the devices parallel on the left and right sides and using a bite splint for distraction. These measures produced improved outcomes, and we describe here the details.

Atypical Apert syndrome: sequential and segmental distraction osteogenesis of the skull, midface, and mandible.

We describe a boy with Apert syndrome, including cranial deformities and syndactyly (acrocephalosyndactyly), though intracranial hypertension, exophthalmos, and midfacial hypoplasia were mild. We treated him by mandibular distraction, in addition to fronto-orbital distraction, and Le Fort III midfacial distraction, with good results.

Special distraction osteogenesis before bone grafting for alveolar cleft defects to correct maxillary deformities in patients with bilateral cleft lips and palates: distraction osteogenesis performed separately for each bone segment.

INTRODUCTION: Patients with bilateral cleft lips and palates have premaxillary protrusion and characteristic jaw deformities involving three-dimensional malposition of the premaxilla and bilateral maxillary bone segments. This study examined patients with bilateral cleft lips and palates who had deviation and hypoplasia of the premaxillas and bilateral maxillary segments. Before bone grafting, the patients were treated with special distraction performed separately for each bone segment using a halo-type external device. This report describes this novel treatment method which produced good results. MATERIAL AND METHODS: The subjects were five patients with severe jaw deformities due to bilateral cleft lip and palate. They were treated with maxillary Le Fort I osteotomy and subsequent distraction performed separately for each bone segment using a halo device. In three of five patients, premaxillary osteotomy was not performed, and osteotomy and distraction were performed only for the right and left lateral segments with severe hypoplasia. RESULTS: All patients achieved distraction close to the desired amount. The widths of the alveolar clefts were narrowed, and satisfactory occlusion and maxillary arch form were achieved. After the surgery, three of five patients underwent bone grafting for bilateral alveolar cleft defects and the bone graft survival was satisfactory. CONCLUSIONS: This method had many benefits, including narrowing of alveolar clefts, improvement of maxillary hypoplasia, and achievement of a good maxillary arch form. In addition, subsequent bone grafting for alveolar cleft defects was beneficial, dental prostheses were unnecessary, and frequency of surgery and surgical invasiveness were reduced. This method is a good surgical procedure that should be considered for patients with bilateral cleft lips and palates who have premaxillary protrusion and hypoplasia of the right and left lateral segments.

Clinical application of distraction osteogenesis for traumatic maxillofacial deformities.

Treatment including occlusal function is important for maxillofacial fractures. Because distraction osteogenesis is now used frequently for the treatment of various types of congenital maxillary hypoplasia, the authors applied this technique to six cases of traumatic maxillofacial deformity caused by old maxillofacial fractures. Several kinds of devices were used for the various deformities associated with severe malocclusion, resulting in extension of both bone and soft tissues. In each case, favorable facial aesthetics and occlusion were achieved. Distraction osteogenesis also appears to be an extremely effective method for repair of traumatic maxillofacial deformities.

Evaluation of physiological tooth movements within clinically normal periodontal tissues by means of periodontal pulsation measurements.

The three-dimensional physiological tooth movement synchronized with heartbeat, periodontal pulsation, has already been demonstrated by previous researchers. The purpose of this study was to estimate physiological tooth movements within clinically normal periodontal tissues, thus establishing periodontal pulsation standards. The measuring device consisted of an amorphous sensor and a small magnet, which could detect tooth displacement without coming into contact with the tooth surface. The samples consisted of the left maxillary central incisors of 23 adult subjects. Three points on the labial surface were used as reference points to measure the labial-lingual movement range, and a point on the incisal edge was used for measuring the incisal-apical movement range. Periodontal pulsation and electrocardiogram measurements were recorded simultaneously for six minutes. The results showed that the mean amplitude of periodontal pulsation concerning the labial-lingual movement range was equal at the three labial points (0.59 microm) and tended to be smaller than that of the incisal-apical movement range (0.87 microm). There were no significant differences in the mean amplitude of periodontal pulsation at any of the four reference points. Significant correlations of the amplitude of periodontal pulsation existed among the three labial points. Though most teeth pulsated uniformly to the labial direction, some teeth moved in a complex manner including rotation with an axis among the three labial points. These results suggest that the values of periodontal pulsation amplitude which were recorded employing the amorphous sensor device are reliable standards of periodontal pulsation within clinically normal periodontal tissues.