CASO CLÍNICO

Orthognathic surgery and rhinoplasty in Binder syndrome

Cirugía ortognática y rinoplastia en el síndrome de Binder

M. Tito, C. Piero

Department of Maxillo-Facial Surgery. University of Rome "La Sapienza", Roma, Italia

Correspondence

ABSTRACT

Binder syndrome is a disorder characterized by nasomaxillary hypoplasia that results in a short nose, a frontonasal angle of almost 180 degrees, hypoplasia of the frontal sinuses, an absent anterior nasal spine, a short columella and an acute nasolabial angle. The patient can be treated orthodontically or surgically depending on the seriousness of the malformation. We review the literature on the etiology, differential diagnosis and treatment of Binder syndrome. We present the case of a boy with this syndrome surgically treated with orthognatic surgery and rhinoplasty with an L-shaped rib cartilage graft.

Key words: Maxillonasal dysplasia; Binder syndrome; Rib cartilage graft.

RESUMEN

El síndrome de Binder es una patología caracterizada por hipoplasia nariz-maxilar, ángulo naso-frontal plano, senos frontales hipoplasicos, ausencia de la espina nasal anterior, columela corta y ángulo nasolabial agudo. El tratamiento del los pacientes con síndome de Binder puede ser ortodóntico o quirúrgico según la gravedad de la malformación. En este trabajo hemos realizado una revision bibliográfica sobre la etiología, el diagnóstico diferential y el tratamiento de la sindrome de Binder y presentamos un caso clínico de un paciente binderiano sometido a intervención de cirugía ortognática y rinoplastia con injerto de cartílago costal para recostruir el dorso y la punta nasal.

Palabras clave: Displasia maxilo-nasal; Síndrome de Binder; Injerto de cartílago costal.

Introduction

Binder syndrome is a congenital pathology characterized by a failure in the development of the premaxilla.¹ In 1962, Binder described three children with the following characteristics: maxillonasal hypoplasia, convex upper lip, vertical nose, flat nasofrontal angle, absence of an anterior nasal spine, atrophy of the nasal mucosa, and hypoplasia of the frontal sinuses. Before 1962, the few cases described of this malformation were identified in scientific publications with other names such as "facies scaphoidea", "flat face" or "congenitally flat nose syndrome"

Noyes² described the clinical features of maxillonasal dysplasia before Binder, but he did not recognize it as a syndrome. The first description of maxillonasal dysplasia goes back to 1882 when Zuckerkandl³ described an anomaly of the nasal fossa floor in which the normal crest that separates the nasal floor from the anterior surface of the maxillary bone was absent. In its place there was a small depression, a prenasal fossa in the lower margin of the nasal vestibule.⁴ Recently in Quarai, New Mexico, a prehistoric skeleton of an Amerindian female aged 16-17 was found with typical dysmorphogenetic characteristics of Binder syndrome. ⁵ Although this syndrome is uncommon, studies have been published with a relatively high number of patients by Delaire et al.⁶ (37 patients), Olow-Nordenram and Radberg⁷ (43 patients), Jackson et al.⁸ (26 patients), Horswell et al.⁹ (19 patients). Binder syndrome has a different degree of severity in different patients. In certain cases orthodontic therapy will be sufficient while, in the more serious cases, combined orthodontic-surgical multi-step treatment will be necessary. This is orientated towards the correction of the deformity of the nasal pyramid and the re-establishment of a correct dentoskeletal maxillo-mandibular relationship.

Case report

The patient DG, was presented at the age of 14. His parents went back to the beginning of his medical history when, during his infancy, they had observed his flattened nasal pyramid in conjunction with Angle class III malocclusion. Orthodontic therapy was then started with palatal expansion and vestiboloversion of the upper incisors in an attempt at compensation of the class III malocclusion, with little aestheticfunctional results. Once under our observation, the patient’s frontal, side and axial views showed eurhythmia of the face that was characterized by: a flattened, high forehead with a nasofrontal angle of approximately 180º, a flattened nasal pyramid and a slight dorsal hump, accentuation of the nasolabial fold, retraction of the columella with an acute nasolabial angle, crescent-shaped nostrils, mandibular protrusion together with retrusion of the upper jaw (Fig. 1A). In the objective endoral examination, a severe class III malocclusion of the dentoskeleton was detected with a 3 mm overjet, open bite in the posterolateral sections, bilateral crossbite, lingual inclination of all dental elements of the lower dental arch and contraction of the upper jaw (Fig. 1B). Ricketts cephalometric analysis showed the absence of the anterior nasal spine, reduction of the anterior cranial base (SN = 73 mm), considerable reduction in the maxillary depth angle (NA^FH = 82º), with increased mandibular depth (NB^FH = 87º), (Fig. 2). The radiograms of the cervical spine excluded any malformation of this segment of the skeleton. Based on clinical examination and radiographic findings, the diagnosis of Binder syndrome was made and presurgical orthodontic treatment was indicated. At the age of 18 the patient underwent surgery, which consisted in a Le Fort I osteotomy of the upper jaw with symmetric anterior lifting of 1 mm and posterior lifting of 3 mm and advancement of 3 mm. A bilateral sagittal osteotomy of the mandible was performed, Obwegeser-Dal Pont technique, which was repositioned in occlusion with the upper jaw. A year after this intervention rhinoplasty surgery was carried out, which consisted in lengthening the columella through v-y plasty and rib cartilage grafting in order to reconstruct the nasal dorsum and the anterior nasal spine (Fig. 3). The follow-up after the final intervention was at 13 months and the aesthetic and functional results were excellent (Fig. 4).

Discussion

The diagnosis of Binder syndrome is by clinical and radiological examination. The most important clinical characteristics of the syndrome concern the nasal pyramid and dental occlusion. The individual with Binder syndrome has a flattened, depressed tip because of the skeletal defect of the premaxilla and because of the reduced horizontal dimensions of the nasal septum. The nostrils appear triangular, the columella is short and the nasolabial angle is acute. As a result of the contraction of the upper jaw due to atrophy involving the premaxilla, the skeletodental relationship always results in class III occlusion. In the more serious cases, the reduction in the diameter of the nasal cavity in relationship with the nostrils, as a result of contraction of the upper jaw, can cause neonatal respiratory distress. Radiologically and in the cephalometric analysis (9) retraction of the glabella can be observed, together with hypoplasia of the frontal sinuses, a shortened anterior skull base, a reduction of the horizontal dimensions of the jaw, an obtuse nasofrontal angle and atrophy of the premaxilla. There is in addition a sign which, if found, is pathognomonic of Binder syndrome: the absence of the anterior nasal spine.

Not all authors believe that a radiologically demonstrated absence of the anterior nasal spine is necessary for a diagnosis of Binder syndrome. According to Holmstrom, author of a study⁴ that was aimed at describing precisely the anatomy of the nasal region of Binder patients, the characteristic pathognomonic feature of Binder syndrome would be the small cavity by the most anterior portion of the nasal vestibule that corresponds with Zuckerkandl’s "fossa prenasalis". In his study Holmstrom located this feature in all his patients (50). The anterior nasal spine was often hypoplastic, but sometimes it had normal proportions. While recognizing a typical Binder syndrome patient is therefore easy, if the clinical phenotype and radiographic images of the patient are ambiguous, the individual sensitivity of the specialist will have to be exercised.

Distinguishing Binder syndrome from other pathologic conditions or syndromes with common characteristics is necessary. The differential diagnosis should be made with acrodysostosis, warfarin embryopathy, Stickler syndrome, Robinow syndrome, Marshall syndrome, fetal alcohol syndrome and especially with Chondrodysplasia Punctata.¹⁰ Acrodystosis can be distinguished from Binder syndrome because, in addition to midfacial hypoplasia, there is brachidactyly of fingers and toes. Skeletal anomalies that are distinctive of Stickler syndrome consist of a cleft palate and arthropathy. Chondrodysplasia Punctata is a pathologic condition and it is the hardest to differentiate from Binder syndrome because of the same typically shaped nose. However, it can be differentiated because of calcifications by the epiphysis, the malformations of the palate and anterior dental area, and by the fact that children with Chondrodysplasia Punctata often have perinatal problems.¹⁰ These characteristics tend to disappear with growth, and for this reason carrying out a differential diagnosis between both syndromes in an adult is sometimes impossible. For this reason some authors believe that Binder syndrome and Chondrodysplasia Punctata represent the extremes of a more ample spectrum of chondrodysplasias.¹¹ The analogies with warfarin embryopathy and with fetal alcohol syndrome have led to a belief that these diseases have a common origin. In all these syndromes there is an intrauterine deficit of Vitamin K, as with the use of phenytoin during pregnancy.^12,13

Many authors have given attention to the similarities between the facial and skeletal anomalies induced by warfarin embryopathies and those produced by phenytoin. The similarities suggest that these anomalies are due to a common vitamin K deficiency in the fetus. Experimental studies on rats have demonstrated that warfarin-induced maxillonasal hypoplasia is linked with abnormalities of the nasal septum, which appears less developed and with less calcification than normal. A vitamin K-dependant protein has been found within the cartilage, matrix GLA protein. Although the exact function of this protein is not known, its presence in growth cartilage suggests that its normal function is to prevent calcification. In fetuses with vitamin K deficiency, the matrix GLA protein should be present in an inactive form and therefore it would not be able to prevent uncontrolled calcification. In Binder syndrome, ectopic calcification of the suture between the vomer and premaxilla, between the sixth and twelfth week of embryonic life, could be responsible for maxillonasal hypoplasia. However, Howe’s hypothesis^{12, 13} is not applicable to all individuals with Binder syndrome, as not all have experienced a prenatal deficit of Vitamin K, but it does helps to clarify the etiopathogenesis of Binder syndrome, which is still very confusing. Various theories as to its pathogenesis have been put forward by many authors. Binder¹ believed that the syndrome with his name derived from a failed induction attempt of the prosencephalon on the growth of the premaxilla. Resche et al.¹⁴ suggested that the pathogenic noxa responsible for Binder syndrome had to act at the same time as the cervical vertebrae were developing, as the malformation of the latter were present in more than 50% of Binder syndrome cases. Converse et al.¹⁵ believed that the reduction in the growth of the premaxilla was due to disturbance to the cartilaginous structure, while Noyes² believed that the syndrome was caused by trauma during birth. While its etiopathogenesis is greatly debated, together with the definition of Binder syndrome itself, there is even less consensus in the literature with regard to therapy. This is probably due to the rarity of the syndrome that does not permit carrying out studies with a sufficient number of cases, and which does not permit treatment comparisons based on solid statistics. It is obvious that Binder patients should be treated specifically according to their age and the seriousness of the pathology. We may in fact come across patients with an Angle Class III molar relationship and a moderately flattened nose, and we may also find individuals with advanced Class III, with an anterior open bite, a nasofrontal angle of 180º, with associated cervico-spinal pathologies and mental retardation. Orthodontia plays a primary role in the milder cases, as well as in the more serious cases. In the former cases orthodontic therapy may be sufficient for reestablishing an acceptable aesthetic appearance together with acceptable stomatognathic function. The objectives of orthodontia are to:

1)Antagonize or correct the contraction of the upper jaw; 2) achieve occlusal adjustment; 3) stimulate the sagittal development of the maxillary bone (Delaire mask); 4) prepare the patient for orthognathic surgery when this is necessary.

Following a radiological study on an 11 year old boy, Rune et al.¹⁶ maintained that the Delaire mask was less effective in advancing the maxilla in Binder patients compared to those patients without the syndrome. According to the authors, this was as a result of the reduced growth capacity of the circum-maxillary sutures. In addition, when the Delaire mask was removed, there was near complete relapse.

The more serious cases require surgical therapy. The objectives of surgery are: to ensure the base of the nose is correctly fixed to the tip and dorsum; to improve the patient’s aesthetic appearance; to solve any accompanying psychosocial problems; to re-establish correct occlusion.

There is no consensus among the different authors as to the most appropriate period for starting surgical treatment of the nose. Authors such as Ortiz Monasterio¹⁷ propose starting surgical treatment promptly because the child will have a psychological advantage as his body image will improve, and because there will be a gradual increase in structural support together with progressive soft tissue expansion at a time when these are more elastic. However, authors such as Posnick¹⁸ warn of the possible risks of this type of early surgery, especially the risk of scar tissue formation that make further interventions more difficult, and which may represent an obstacle to the horizontal and vertical growth of the upper jaw.

To ensure proper support for the nasal base, many authors have proposed using bone or cartilage grafts.^18,19 The main grafts used are iliac crest, cranial bone and rib cartilage. Bone or cartilage grafts are also used for the reconstruction of the nasal dorsum. Neu²⁰ recommends the use of cranial bone for the reconstruction of the upper two thirds of the nasal dorsum, and cartilage from the nasal septum, or auricular cartilage for the lower third and tip. The tip of the nose will not therefore have the rigidity of a reconstruction carried out of the dorsum, tip and columella with a single L-shaped bone graft. We believe that rhinoplasty surgery should be carried out once growth is complete and after orthognathic surgery. The rib cartilage graft used for providing support for the nasal base and for reconstructing the nasal tip and dorsum, once shaped into an L, has allowed us to achieve optimum aesthetic-functional results, with good stability and with no rigidity of the nasal tip. In addition, with regard to the correction of occlusal alteration, the orthognathic surgical procedure most used is the Le Fort I osteotomy, in conjunction with a bilateral sagittal osteotomy of the mandible and/or median disjunction of the upper jaw in order to achieve expansion. This intervention, according to our school, should be carried out at the end of the growth period (15-16 years). Posnick¹⁸ demonstrated with seven Binder syndrome patients who underwent Le Fort I osteotomies with rigid internal fixation, that relapse in the vertical dimension was minimal in the maxilla, while in the horizontal dimension relapse was practically nil. In addition, our experience with a follow-up of four years, shows that internal rigid fixation guarantees total skeletal stability.

Conclusions

The diagnosis of Binder syndrome is through clinical and radiological examination. Clinically, premaxillary hypoplasia together with class III dentoskeletal features and deformity of the nasal pyramid are the distinctive characteristics Radiologically, the absence of the anterior nasal spine is the pathognomonic feature for diagnosing this syndrome. The therapy for more serious forms should therefore be surgical and based on orthognathic surgery, which should be carried out at the end of the growth period after adequate orthodontic pre-surgical treatment, and this should be followed by rhinoplasty surgery. This should envisage an autologous tissue graft for reconstructing the nasal dorsum and tip, and for supporting the nasal base. In our experience, rib cartilage has shown itself to be ideal, given the possibility of guaranteeing adequate support while maintaining the natural movement of the structures in the lower portion of the nasal dorsum, tip and columella.

Correspondence:
Marianetti Tito Matteo
Viale delle provincie, 55 Int. 18
00162 Roma, Italia
E-mail: titomatteo.marianetti@tin.it

Recibido:27.12.05
Aceptado: 06.10.06

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