Binder Syndrome Research Articles

Common craniofacial anomalies: the facial dysostoses.

After studying this article, the participant should be able to: 1. Understand the etiology and pathogenesis of facial dysostosis syndromes. 2. Recognize and classify common facial dysostoses. 3. Understand the different management plans for the reconstruction of facial dysostoses. The wide spectrum of craniofacial malformations makes classification difficult. A simple classification system allows an overview of the current understanding of the etiology, assessment, and treatment of the most frequently encountered craniofacial anomalies. Facial dysostoses are reviewed on the basis of their diverse etiology, pathogenesis, anatomy, and treatment. Conditions discussed include craniofacial microsomia, Goldenhar syndrome, Treacher Collins syndrome, Nager syndrome, Binder syndrome, and Pierre Robin sequence. Approaches to the surgical management of these conditions are reviewed.

Maxillonasal dysplasia (Binder syndrome): a lateral cephalometric assessment

Abstract Binder syndrome or maxillonasal dysplasia was first described by Binder in 1962, and is a disorder characterised by nasomaxillary hypoplasia. The records of 33 patients who had been diagnosed clinically with Binder syndrome at the Royal Children’s Hospital of Melbourne were examined. Of these 33 patients, 14 were selected because they met the inclusion criteria: that they had not had prior surgical and/or orthodontic treatment, and that high-quality lateral cephalometric radiographs were available. The craniofacial morphology of these patients was determined on lateral cephalometric radiographs and compared with published age- and sex-matched norms. In agreement with published studies, the anteroposterior lengths of the anterior cranial base and maxilla were reduced, and the majority of patients had a Class III skeletal relationship. Although the lower incisors tended to be prominent, both overjet and overbite fell within the ranges for the normal population. Despite the fact that the orthodontic and surgical treatment for patients with Binder syndrome is normally carried out within specialised units, clinicians should be aware of the variety of ways in which this condition may present.

Bone grafts to the nose in Binder's syndrome (maxillonasal dysplasia): a follow-up of eleven patients with the use of profile roentgenograms.

Bone grafts are used to lengthen the dorsum and elevate the tip of the nose in patients with Binder's syndrome. Disappointing long-term results in some patients generally have been assumed to be a result of resorption and/or displacement of the grafts. Treatment outcome was studied in 11 patients with the use of serial profile roentgenograms. At 40 months, the mean values after surgery were reduction of the initial transplant length by 28 percent and reduction of the transplant angle by 4 degrees. The initial nose length was increased by 1 mm, and nose tip projection was increased by 2 mm. Although the mean changes of nose length and nose tip projection seemed small, treatment outcome varied considerably between patients. In contrast with earlier assumptions, no correlation could be found between the degree of transplant resorption and/or displacement and the effect of surgery on nose length and nose tip projection.

Binder syndrome: staging of reconstruction and skeletal stability and relapse patterns after LeFort I osteotomy using miniplate fixation.

The present study prospectively assesses the skeletal stability in a consecutive series of Binder syndrome patients (n = 7), aged 16 to 20 years, who underwent LeFort I osteotomy fixed with miniplates and the associated morbidity. All patients underwent a one-piece LeFort I osteotomy fixed with four miniplates in conjunction with orthodontic treatment during the period of 1986-1992. Five of seven patients underwent iliac grafting to their deficient premaxilla and interpositionally at their osteotomy sites. Six of seven patients underwent bone graft augmentation of their deficient nose (four costochondral, one cranial, and one iliac). Serial cephalometric radiographs were taken at standard intervals after surgery (1 week, 6-8 weeks, 1 year). Horizontal, anterior vertical, and posterior vertical directional changes were then measured at each interval. With the radiographs superimposed, the amount of change was measured by the method of anatomic best fit. The 1-year postoperative cephalograms also were assessed for overjet and overbite. The medical records were reviewed for morbidity. Each patient had a complete set of longitudinal records. Follow-up ranged from 1.5 to 5.5 years at the close of the study. Perioperative morbidity was unremarkable, other than one patient whose cranial bone graft dehiscence through the nasal skin required regrafting. The mean (effective) maxillary advancement for the group was 6.0 mm, with 5.9 mm maintained 1 year later. The mean anterior vertical change of the maxilla was 4.2 mm, with 3.1 mm maintained; whereas the mean posterior vertical change was 2.8 mm, with 2.2 mm maintained. All patients maintained a positive overjet and overbite at 1 year. In our series, a staged reconstructive approach for Binder syndrome was carried out in the teenage years and included orthodontic treatment, orthognathic surgery, and nasal augmentation. The extent of skeletal relapse of the LeFort I osteotomy fell within a range that could be managed effectively to maintain a long-term positive overjet and overbite.

Lengthening the nose: a lesson from craniofacial surgery applied to posttraumatic and congenital deformities.

Various methods of nasal lengthening presented in the literature are discussed, including extensive skin undermining, provision of extra skin by forehead flaps, bone grafts, cartilage grafts to the nasal tip, extensive dissection of nasal lining, and buccal flaps to provide additional nasal lining. This paper describes a method that entails a nasofrontal osteotomy and mobilization of previous fractures in posttraumatic foreshortened noses and a perinasal osteotomy (Le Fort II minus Le Fort I) in congenitally short noses such as seen in Binder's syndrome. Nine posttraumatic and two congenital short noses have been treated in this manner without complication. The procedure draws on experience in craniofacial surgery, where there is a nasofrontal osteotomy and division of lining in extracranial Le Fort II and III osteotomies with insertion of an interpositional bone graft. This method is recommended for markedly foreshortened noses of either traumatic or congenital origin.

The Mask Rhinoplasty

Congenital and acquired facial deformities in which the nose is small relative to the rest of the face present a problem in reconstruction. Binder's syndrome is the most common of such deformities, where the noise is both short and lacking tip projection. A method of reconstructing such deformities is described. By means of a coronal skin incision and an approach through the upper buccal sulcus, the nasal soft tissues including the alar cartilages are mobilized. Nasal lengthening and improved tip projection are achieved and supported by a cantilevered graft of lyocartilage. The defect created within the nasal vestibule is repaired by means of mucosal flaps raised from the upper buccal sulcus. Ten cases have so far been treated successfully using this technique we call mask rhinoplasty.

Simultaneous reconstruction of maxillary and nasal deformity in a patient with Binder's syndrome (maxillonasal dysplasia)

Maxillonasal dysplasia, or Binder’s syndrome, a malformation characterized by midface hypoplasia and a flattened nasal profile, is one of the many facial deformities the oral and maxillofacial surgeon may encounter. Orthognathic surgical evaluation integrated with nasal reconstruction can enhance function and esthetics in such patients. Maxillonasal dysplasia was first described by Binder in 1962’ and further detailed by Gorlin2 and Holmstrom.3 The syndrome’s etiology, although unknown, has been postulated to be a congenital malformation3 or occur secondary to midface trauma.4 A hereditary association has been reported, but remains undefined.3 Hypoplastic nasomaxillary growth with degrees of severity may occur. The extent of reconstruction is tailored to the individual degree of deformity. The syndrome results in hypoplasia of the maxilla and nose while sparing the malar region. The flattened nasal profile is characteristic of the deformity. Associated with the nasal hypoplasia is an excessively obtuse or absent nasofrontal angle and a very acute nasolabial angle. The nasal alae are flattened, the nostrils are crescent shaped, and the columella is shortened. The upper lip is convex with a poorly developed, shallow philtrum. The anterior nasal spine (ANS) is hypoplastic or absent and the nostril sills are hypoplastic. The associated maxillary retrusion results in a class III deformity with relative mandibular prognathism. The sense of olfaction remains normal.2.3 A surgical-orthodontic coordinated treatment plan must assess the specific needs and extent of the defor

Binderʼs Syndrome: Peculiarities in Japanese Patients

We have seen 4 Japanese patients with Binder's syndrome or maxillonasal dysostosis. The diagnosis of Binder's syndrome was very difficult, based on facial appearances alone. Although the flattened nasal tip was quite unusual in Japanese individuals, the midface retrusion and flattened nasal bridge would be considered within normal limits. Consequently, the diagnosis of Binder's syndrome is only rarely made in Japanese persons. The peculiarities of this syndrome in Japanese patients are discussed and our surgical methods are shown.

Nasal Base, Maxillary, and Infraorbital Implants—Alloplastic

The aesthetic surgery of the facial skeletal contour requires either the performance of ostectomies of excessively prominent segments or the augmentation of retruded segments with organic or synthetic material, in order to achieve balanced tridimensional relations of each segment with regard to the total facial unit. Craniomaxillofacial surgeries are necessary in major malformations or in those combined with malocclusion. In the nasal dorsum or tip, the author prefers the use of cartilage, because synthetic materials need adequate soft-tissue bulk for cover to be inserted without tension and absence of passive mobility of the reception site. For malar augmentation, first proposed by the author and independently by Spadafora in 1971, for chin augmentation up to 8 mm, and for augmentation of the mandibular angle, the author prefers silicone implants because they do not change in shape or volume, may be premanufactured or custom-made, have a similar consistency to that of bone, and do not support bacterial growth. On the other hand, autologous bone grafts adapt less to curved bony surfaces, have an erratic rate of resorption, and need an additional surgical step for removal with the corresponding morbidity and scar. Subperiosteal insertion is preferred because it confers greater stability and the cavity is easier to dissect without soft-tissue damage. Although bone erosion may occur, with over 1200 implants clinically no major change in the soft-tissue contour has been observed, nor has the author been consulted for late complication. In the malar region this may be due to the large surface of the implant and absence of muscular pressure. In the chin, an insertion over the site of the dental roots is avoided. For midface augmentation the following implants are used: (1) The premaxillary lower nasal base implant, proposed in 1971, is indicated to correct a concave midfacial profile, frequent in Asian, black, and Mestizo patients from Latin America and in Caucasian patients with maxillonasal dysplasia or Binder's syndrome, after trauma, with excessive septum and nasal spine resections, and in nasal-maxillary sequels in cleft patients. In case of dental malocclusion, orthognathic surgery is the technique of choice. A prototype implant is available in two sizes, to be inserted through a lateral incision at the base of the columella. In 108 patients two implants have been partially removed. After the first month the patient is usually well adapted to the foreign body.(ABSTRACT TRUNCATED AT 400 WORDS)

Craniofacial Anomalies in Twins

Studies of twins provide insight into the relative contribution of genetic and environmental factors in the causality of structural anomalies. Thirty-five affected twin pairs were identified from a group of 1114 patients with congenital craniofacial deformities evaluated from 1972 to 1989. Forty-three of these 70 twins exhibited one or more craniofacial anomalies; these were analyzed for dysmorphic characteristics, zygosity, concordance, and family history. The anomalies were categorized into two groups: malformations and deformations. The malformations (n = 36) included hemifacial microsomia (n = 10), cleft lip and palate (n = 8), cleft palate (n = 4), rare facial cleft (n = 2), craniosynostosis (n = 2), Binder syndrome (n = 2), Treacher Collins syndrome (n = 2), craniopagus (n = 2), CHARGE association (n = 1), frontonasal dysplasia (n = 2), and constricted ears (n = 1). The deformations (n = 7) included plagiocephaly (n = 5), hemifacial hypoplasia (n = 1), and micrognathia (n = 1). Twenty-one monozygotic and 14 dizygotic twin pairs were identified. The concordance rate was 33 percent for monozygotic twins and 7 percent for dizygotic twins.(ABSTRACT TRUNCATED AT 250 WORDS)

Maxillonasal dysplasia (Binder's syndrome)

Maxillonasal dysplasia (Binder's syndrome)

The craniofacial morphology in persons with maxillonasal dysplasia (binder syndrome): A longitudinal cephalometric study of orthodontically treated children

The aim of this investigation was to study the influence of orthodontic treatment on facial growth in subjects with maxillonasal dysplasia. The subjects were followed longitudinally from early childhood until growth was completed or almost completed. Fifteen patients, 9 boys and 6 girls, were orthodontically treated by conventional methods. They were compared with (1) another longitudinal group consisting of 13 orthodontically untreated children, 7 boys and 6 girls with the same syndrome, and (2) with a control material taken from computer-filed data on Swedish children and young adults with orthognathic profiles and normal occlusion. The orthodontic treatment led to acceptable dental conditions in 10 of the patients. No influence on craniofacial growth could be demonstrated. However, the mean facial polygons of the children showed that certain differences already existed at early ages between the treated subjects with a generally more severe form of the syndrome and the untreated children. The growth pattern seemed, however, to follow about the same rate in both groups. In six patients the orthodontic treatment was followed by surgical correction at adult age to solve their craniofacial problems. The optimum care of these patients requires an interdisciplinary treatment approach with thorough treatment planning by orthodontists in collaboration with oral and plastic surgeons.

An etiologic study of maxillonasal dysplasia--Binder's syndrome.

As the etiology of maxillonasal dysplasia (Binder's syndrome) is unclear, an attempt has been made in this study to check the presence of hereditary factors. Pedigrees have been established for 50 patients with the syndrome who had actively requested orthodontic treatment and/or plastic surgery and for whom hereditary connections had been found in 36%. In some of the subjects the propositi volunteered further family data, which were included in the study. The total number thereby became 60 families. The results did not disprove the possibility of a genetic etiology although the suspicion of an autosomal recessive inheritance may not be the full explanation for the syndrome. If the syndrome is in fact of a genetic origin, one possibility is that the syndrome is indeed inherited as an autosomal recessive trait. In that case, the hypothesis of an incomplete penetrance must be added. Another possibility is that the syndrome is a threshold character with a genetically multifactorial background. Since no frequency counts among populations are available, further studies are required to collect families with maxillonasal dysplasia and to obtain population frequency data for the syndrome.

The craniofacial morphology in individuals with maxillonasal dysplasia (Binder's syndrome). A longitudinal cephalometric study of orthodontically untreated children.

Thirteen children with maxillonasal dysplasia, 7 boys and 6 girls, not orthodontically treated, were studied longitudinally. Three boys had rejected all kinds of treatment in spite of an objective need for correction. The other 10 children had no or little need for orthodontic treatment. The aim of the investigation was to study individual growth in this untreated group of subjects with Binder's syndrome. Thirty-five variables were measured on lateral skull radiographs, taken at intervals from early childhood until growth was completed, or in a few cases almost completed. The results are presented in diagrams and compared with Swedish children and young adults with orthognathic profiles and normal occlusion, matched for sex and age. Some of the variables showed large individual variations and some were similar to the reference values, but those variables responsible for the characteristic appearance of individuals with maxillonasal dysplasia were all divergent from normal standards. The graphical presentation showed, however, that during growth most variables in the Binder subjects changed in parallel with those in normal children and mainly during puberty.

Clinical and pathologic features of maxillonasal dysplasia (Binder's syndrome): Significance of the prenasal fossa on etiology

Clinical and pathologic features of maxillonasal dysplasia (Binder's syndrome): Significance of the prenasal fossa on etiology

The cranlofacial morphology in individuals with maxillonasal dysplasia (Binder's syndrome). A longitudinal cephalometric study of orthodontically untreated children

Thirteen children with maxillonasal dysplasia, 7 boys and 6 girls, not orthodontically treated, were studied longitudinally. Three boys had rejected all kinds of treatment in spite of an objective need for correction. The other 10 children had no or little need for orthodontic treatment. The aim of the investigation was to study individual growth in this untreated group of subjects with Binder's syndrome. Thirty-five variables were measured on lateral skull radiographs, taken at intervals from early childhood until growth was completed, or in a few cases almost completed. The results are presented in diagrams and compared with Swedish children and young adults with orthognathic profiles and normal occlusion, matched for sex and age. Some of the variables showed large individual variations and some were similar to the reference values, but those variables responsible for the characteristic appearance of individuals with maxillonasal dysplasia were all divergent from normal standards. The graphical presentation showed, however, that during growth most variables in the Binder subjects changed in parallel with those in normal children and mainly during puberty.

Clinical and pathologic features of maxillonasal dysplasia (Binder's syndrome): significance of the prenasal fossa on etiology.

Clinical and pathologic anatomic parameters were studied in 50 patients with maxillonasal dysplasia (Binder's syndrome). The skeletal deformity causing the flat and low-set nose was in typical patients a palpable depression in the anterior nasal floor (fossa prenasalis) and a localized maxillary hypoplasia in the alar base region. Class III malocclusion was found in 54 percent. In 6 percent of the patients a slope (sulcus prenasalis) was found instead of a fossa in the anterior nasal floor, and in one patient a rudimentary fossa was found. Concomitant malformations were noted in 18 percent, and a hereditary connection was seen in 16 percent. The etiology is discussed in relation to the development of the premaxilla and the appearance of a secondary external trabecular network of bone in the canine region. An inhibition of the latter ossification center would explain the localized hypoplasia in the floor and walls of the piriform aperture in maxillonasal dysplasia.

The craniofacial morphology in individuals with maxillonasal dysplasia (Binder's syndrome): a cross-sectional cephalometric study.

Ninety-seven Swedish children from three different geographical areas, 51 boys and 46 girls, with maxillonasal dysplasia were cephalometrically examined. The children were divided into four age groups and their ages ranged from 6 to 17 years. None of them had received orthodontic treatment prior to the registration. Their craniofacial morphology was studied and compared with that of children with normal occlusion and orthognathic profile, matched for sex and age. Thirty-six variables were measured. For several of these, highly significant differences were obtained, referable to the very special characteristics in persons with maxillonasal dysplasia. The major part of the significant differences were found in the younger age groups, indicating a severe form of the syndrome, with greater need for orthodontic treatment.

Nasomaxillary hypoplasia--Binder's syndrome. Morphology and treatment of two separate varieties.

Two varieties of nasomaxillary dysplasia are presented in 13 patients; one with a nose of normal length and another with a short nose. Several cephalometric and soft tissue differences and similarities are found between these groups. Orthodontic treatment of the malocclusion and nasal reconstruction with an L-shaped iliac bone graft composed of two pegs is the treatment of choice. In the short nose variety, the tight nasal mucosa has to be lengthened with an auricular composite graft.

Chondrodysplasia punctata (mild type) presenting as binder's syndrome

Chondrodysplasia punctata (mild type) presenting as binder's syndrome