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Department of Otorhinolaryngology, Head and Neck and Facial Plastic Surgery, Klinikum Fulda, Teaching Hospital of the Philipps-University Marburg, Fulda, Germany
Department of Otorhinolaryngology, Head and Neck and Facial Plastic Surgery, Klinikum Fulda, Teaching Hospital of the Philipps-University Marburg, Fulda, Germany
Department of Otorhinolaryngology, Head and Neck and Facial Plastic Surgery, Klinikum Fulda, Teaching Hospital of the Philipps-University Marburg, Fulda, Germany
Maxillonasal dysplasia or Binder's syndrome is an uncommon though easily recognizable congenital condition characterized by a retruded mid-face with an extremely flat nose. The facial deficiencies lead to functional as well as psychological problems. We report on 10 patients with maxillonasal dysplasia whose noses were corrected with onlay costal cartilage grafts using a combined oral vestibular and external rhinoplasty approach. The technique has been used in children as well as adults with promising results. Since the degree of malformation in Binder's syndrome varies significantly surgical correction needs to be tailored individually based on the principles demonstrated. In all patients, minor malocclusion was first treated by orthodontists. Over a follow up period up to 14 years the advancement of the nose was found to be stable, even in lateral cephalograms.
The treatment of these patients is a challenge for every surgeon and needs interdisciplinary cooperation.
Maxillonasal dysplasia (Binder's syndrome) is a congenital malformation characterized by nasomaxillary hypoplasia due to an underdevelopment of the midfacial skeleton. In 1962, Binder was the first to give a detailed description of the defect, although reports of this malformation had previously been published under other designations, such as facies scaphoidea or dish face and congenital flat nose syndrome.
He reported on three cases and recorded six special characteristics: (1) arhinoid face; (2) abnormal position of the nasal bones; (3) intermaxillary hypoplasia with consecutive malocclusion; (4) reduced or absent anterior nasal spine; (5) atrophy of the nasal mucosa and (6) absence of the frontal sinus (not obligatory). The result is a typical facial appearance including a midfacial hypoplasia with a flat nose, flattened tip and alar wings, half-moon shaped nostrils, short columella, acute nasolabial angle, frontonasal angle of almost 180° eventually resulting in a concave midfacial profile.
All Binder's patients have some degree of these characteristics, which may range from mild to severe.
Since Binder recognized the syndrome in 1962 well over 250 cases have been reported. Males and females are affected equally. The etiology of maxillonasal dysplasia is not known. Although the majority of these cases are sporadic, hereditary factors may be operating, since the frequency of disease in relatives is about 15%.
While Binder believed this condition to be a form of an arhinocephalic malformation, Holström proposed that there is inhibition of the ossification center that would normally have formed the lateral and inferior borders of the piriform aperture during the fifth and sixth gestational week.
Surgical treatment of patients with maxillonasal dysplasia is still controversial, especially regarding the questions of the optimal age for performing surgery and of the appropriate surgery to be done improving the facial contour as well as the functional conditions.
In the present study we report our experience on the correction of the nasal and paranasal areas in 10 patients with Binder's Syndrome. Analyzing the long term results, we describe our treatment strategy using costal cartilage grafts for augmentation of the midface and advancing the nose which we undertake depending on the functional and psychological problems of the patients. Interdisciplinary treatment planning is by ENT and plastic surgeons, maxillofacial surgeons in collaboration with orthodontists.
2. Patients and surgical technique
Since 1985 we have treated 10 patients with maxillonasal dysplasia. Physical exam findings included midfacial hypoplasia, flattened nose, short columella with acute nasolabial angle, and retrusion of the anterior nasal spine. None of the patients presented with class III malocclusion (according to Angle
) requiring maxillary advancement by Le Fort osteotomies, but orthodontist had treated all of them before. Patients' ages at the time of initial consultation ranged from 10 to 28 years. Surgical treatment was started between age 11 and 16 in three patients and completed in seven patients after facial growth. All patients were evaluated by photography pre- and postoperatively. In addition, some underwent postoperative lateral cephalographic radiograms. Postoperative follow-up ranged from 1 to 14 years.
Our surgical treatment plan consists of nasal advancement and premaxillary augmentation using costal cartilage grafts. The grafts were harvested through a small submammary incision in females and a lower oblique incision in males. To achieve anterior projection of nose and midface usually three cartilaginous strips were implanted through a combined external rhinoplasty and oral vestibular approach. One graft was placed to the dorsum, one to the columella and one below the piriform aperture onto the superior alveolar process, respectively (Fig. 1(A) and (B)) . The latter was fixed at the maxilla by screws also to serve as a hypomochlion buttress for the columellar strut. Screws should be avoided in children before second dentition to protect their tooth roots. Instead, in early childhood we prefer wire fixation of the graft at the anterior nasal spine and a premaxillary subperiostal pocket appropriate to the graft size. The dorsal and columella grafts each were carved from the central section of the costal cartilage to prevent warping.
Additionally, the dorsal cartilaginous splinter was stabilized by placing the upper end into a hole drilled in the glabellar region. In cases where a soft tissue lengthening of the columella was needed the external approach was combined with a V-Y fashioned incision of the philtrum (Figure 2, Figure 4) .
Figure 1(A, B) Schematic drawing of cartilage grafting procedure in maxillonasal dysplasia. Placement of the three cartilaginous splinters, one to the dorsum, one into the columella and one onto the maxilla fixed by screws avoiding dental roots. The arrows point to the fixation at the glabellar region as well as to the interlocking of the grafts.
Figure 2Surgical procedure: implantation of the three cartilaginous grafts (two are marked red and green) through a combined external rhinoplasty and oral vestibular approach.
Figure 4Twenty eight year old male with maxillonasal dysplasia. (A, D, G) Preoperative photographs. (B, E, H) Result one year after surgery. (C, F, I) Stable postoperative result fourteen years after surgery.
One surgeon operated on seven patients and another one on three. Both surgeons used the technique described.
3. Results
All patients were operated on only once. Costal cartilaginous grafts maintained their volume in all areas of the nose regardless of the patient's age. The same was true for the cartilages augmented to the premaxillary area. In all cases clinical follow up to 14 years postoperatively showed the advancement to be permanent. It must be stressed that by our technique the midface with the nasal base as well as the nose itself were advanced, not just the tip of the nose or only the maxillary bone, respectively.
Two of our patients are presented in Figure 3, Figure 4. In Figure 3(A–F) the digital superposition demonstrates the degree of maxillary and nasal augmentation achieved with three autogenic rib grafts. Note also the significant improvement to the length of columella and the position of nasal openings. The long term follow up of the patient demonstrated in Figure 4 shows only minimal cartilaginous resorption at the nasal dorsum and the improvement of the V-Y scar at the upper lip over the years.
Figure 3(A) Seventeen year old female with Binder's nasal and maxillary hypoplasia. Preoperative planning: digital superposition of the cartilaginous grafts to be implanted. (B) Comparison of preoperative state and postoperative result by digital superposition of the patient's photographs. (C, E) Preoperative frontal and mental views. (D, F) Result two years after surgery.
The orientated profile roentgenograms of our patients showed reorganization of the transplanted cartilage grafts in six of the ten patients at 12 month (mean) after surgery, whereas it could not be shown satisfactorily in four patients at the last examination more than five years after surgery.
The result of a questionnaire showed that on the whole the patients' feelings toward their operations were positive. Only one patient was dissatisfied because of a minor shift of the cartilage grafts, but he did not want another operation.
No signs of infection, necrosis, or other complications were observed. At the columella these scars were acceptable in all patients.
4. Discussion
In Binder's syndrome the hypoplasia of the nasal floor and adjacent part of the maxilla mainly due to a deficient horizontal growth of the maxilla produces the characteristic dish face anomaly and a flat nose.
Since these deformities are evident at a very young age and often lead to severe psychological problems besides the functional restrictions surgical correction is demanded. Planning the treatment strategy, two questions have to be taken into special consideration: (1) What is the appropriate surgery, and (2) Which is the optimal age for performing surgery?
Bone and cartilage grafts have been traditionally used to straighten out the maxillonasal hypoplasia. Ragnell described the application of iliac cancellous onlay bone chips to the anterior surface of the maxilla through an median incision at the columellar base.
However, the results of bone grafts remain unpredictable. Resorption often occurs especially if the soft tissue cover is very tight, and displacement of the bone strut has been described to lead to disappointing long term results.
The patients are very often disturbed by the stiffness of the tip of the nose, and the rigidity of the bone implant poses a problem as it may be easily fractured.
Costal cartilage grafts, on the other hand, maintain its volume over the years and produce a more natural feeling of the nose making it the ideal material for augmentation. To prevent warping of the large grafts required for the dorsum they must be carved from the central part of the rib and placed into 0.9% sodium chloride solution for about 30 min. Although, the balance of the surface tension forces is not maintained, thereby, the residual stress of the cartilage splinter comes to the fore and can be taken into account when implanting. Another solution to this fact is the insertion of a thin K wire inside the graft as proposed by Gunter et al.
also described convincing results in augmenting the midfacial deficiencies by using cartilaginous onlay grafts to the pyriform area, as L-strut dorsal grafts and columellar strut grafts.
The flat nose in Binder's syndrome has also been considered to be a problem of soft-tissue deficiency in the columella. Lengthening of it has been achieved by the use of a free auricular graft, small flaps from the upper lip, bilateral flaps from the nasal floor, and VY-plasty of the columella.
Our concept is to lengthen the columella by a VY-plasty if there is a real shortage of skin, but if there is just a retraction into the hypoplastic nasal floor the skin advancement can be achieved by undermining the skin at the lip-columellar junction and by the help of the nasal cartilage grafts. If necessary the cartilaginous septum is rotated forward additionally supporting the nasal dorsum.
A limitation to the achievement of an optimal result is presented by the constriction of the soft tissue covering the nose and of the lining of the nasal cavities which were not expanded progressively as occurs in normal patients. As Ortiz Monasterio et al.
have described this problem can be prevented if surgical treatment starts early because the corrected facial conditions follow a pattern similar to normal growth. At least equally important is the advantage of improving the self-image of the patients during their growth period when performing surgery early in life. Therefore, we cannot agree with Tessier et al.
that for surgery in Binder's syndrome the ideal age is 16 years when growth of the maxilla is completed, at least one should use an onlay graft technique without osteotomies, exclusively.
In our series all patients have had mild malocclusion which has been treated satisfactorily by orthodontics until teenage. In cases with severe malocclusion, particularly Type III, maxillary retrognathia should be corrected by a Le Fort I maxillary advancement. But, even if the septum and the nasal bones are included in the advanced segment, as in a Le Fort II osteotomy, the flat nose and the depressed alar base remain and with it the facial characteristics of Binder's syndrome.
Furthermore, a Le Fort II osteotomy lessens the normal glabellar depression and this may be a limiting factor as a nasal dorsum coming straight off the lower forehead is aesthetically not ideal.
These facts point up to the major importance of nasal correction in patients with Binder's syndrome. In severe cases of the syndrome Holmström and Kahnberg
recommend a two stage surgical procedure, firstly maxillary osteotomy and followed by the nasal improvement secondarily, both independently of the patients age.
Since the degree of malformation in Binder's syndrome varies significantly surgical correction needs to be tailored individually based on the principles demonstrated. Before any treatment is instituted, patients with maxillonasal dysplasia should be evaluated by all specialists involved to establish a sufficient plan for correction. The proper way to handle these cases, in our opinion, is to treat the malocclusion orthodontically or by orthognathic surgery followed by surgery of nose and midface. For best possible social integration we recommend surgery to be performed before starting school. The onlay grafting technique seems to influence facial growth positively. Minor secondary corrections are options at any time needed.
References
Holmstrom H
Clinical and pathologic features of maxillonasal dysplasia (Binder's syndrome): significance of the prenasal fossa on etiology.
Wolfgang Draf Prof. Dr Med., FRCS Ed, Head of the Department of Otorhinolaryngology, Head and Neck and Facial Plastic Surgery
Ulrike Bockmühl Priv.-Doz. Dr med., Senior Surgeon
Bernard Hoffmann, Trainee
Department of Otorhinolaryngology, Head and Neck and Facial Plastic Surgery, Klinikum Fulda, Teaching Hospital of the Philipps-University Marburg, Pacelliallee 4, D-36043 Fulda, Germany
Article info
Publication history
Accepted:
March 27,
2003
Received:
July 23,
2002
Footnotes
☆Partly presented at the International Meeting of the European Academy of Facial Plastic Surgery, 16–18.9.2001, London
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