Binder syndrome, also known as congenitalmidfaceretrusion and maxillonasalDysplasia, is characterized by midfacial and nasal dysplasia. In 1962, Binder established the syndrome as a class of syndromes, naming it after himself. The prevalence of Binder’s syndrome is not low in the Oriental population and is often undiagnosed due to lack of awareness of the disorder. Most patients are treated as saddle nose deformity and undergo simple rhinoplasty, but the results are often unsatisfactory.
I. Clinical presentation
Binder syndrome is a common disease in craniomaxillofacial surgery, and its pathogenesis is not well understood. The clinical manifestations vary greatly. In mild cases, there may be a simple saddle nose deformity and mild changes in the soft tissues of the lower part of the nose. In severe cases, all tissue deformities involving the midface and changes in the occlusal and jaw relationships may be present.
Typical changes include the following: short nose; collapsed nasal dorsum, some of which may be accompanied by a hump-like nasal dorsum; short, deviated nasal column; nostrils with a special “half-moon” shape; sharp nasolabial angle; flattening around the nasal wings; deviated nasal septum or hypoplasia of the lower nasal septum. The upper lip is convex and the midpersonal ridge is wide. The occlusal relationship can be normal or show different degrees of Angle III misalignment, which is often called “geodesic” facial shape.
Diagnosis
The diagnosis of Binder’s syndrome is not difficult. The position of the nasal root, the maxilla and the mandible and the angle in relation to the skull base can be measured by cephalometric film. In more severe cases, a 3D CT scan of the skull is required. In order to fully and truly reflect the details of the skeletal features of the skull and provide a reliable basis for a comprehensive treatment plan.
III. Treatment
1.Treatment timing: There is no definite conclusion. Some scholars believe that the correction of Binder syndrome should be operated early (Phase I surgery should be performed at the age of 7-10 years old, and Phase II surgery should be chosen after puberty), while another part of physicians point out that the treatment should be chosen after the basic end of facial development (i.e., 15 or 16 years old).
2.Treatment: The main treatment is symptomatic correction of the existing deformity, and the treatment for this type of disease has a sequential nature, but because our patients are not fully aware of this type of disease, they often come to the clinic as adult patients. For this group of patients, surgical treatment is the best approach.
Since the clinical manifestations of the syndrome vary widely, the surgical plan should be tailored to the degree of deformity of each patient. Mild patients may undergo orthodontic treatment only to mask the lack of midface depression, but with less improvement in facial contour. Moderate patients with only nasal and periapical depressions without anterior dentition can have bone or cartilage grafts applied to reshape the nose, while severe patients require orthognathic surgery along with rhinoplasty to correct receding maxillary and malocclusion deformities.
Given that the syndrome has both soft tissue deformities and bone tissue defects, the surgery is also divided into two aspects: bone tissue correction and soft tissue correction, and the main principles of the surgery are as follows.
(1) Correction of anticlinal malocclusion.
(2) Correction of flattening around the nose caused by dysplasia around the pear-shaped foramen.
(3) soft tissue deformities of the nasal dorsum, nasal columella and nasal wing.
3.Bone tissue surgery: including both maxillary osteotomy and bone grafting, which need to be applied jointly in most cases. Osteotomy refers to maxillary LeFortI, LeFortII osteotomy and various procedures with appropriate improvements to the standard procedure. Bone grafting mainly refers to the use of autologous bone free graft around the pyriform foramen to increase its prominence, with the rib and iliac bone often used as the donor area.
(1) LeFortI type osteotomy: the anterior nasal crest point is moved forward to reach the preoperative design position, and autologous ribs and iliac bone are grafted around the pyriform foramen. This method is simple, less traumatic for the patient, and not very demanding for the surgeon. This method is suitable for patients with maxillary recess and anterior malocclusion, but not with medial orbital rim dysplasia.
(2) LeFort II type osteotomy with anterior displacement: The nasal root, bilateral anterior wall of maxillary sinus and the whole superior alveolus are included in the anterior displacement bone flap, and the area around the pyriform foramen can also be adequately displaced anteriorly, and the bone graft is mainly in the gap after the anterior displacement to prevent its recurrence. This method is mainly applied to patients with severe Binder syndrome, and it is more adequate for the correction of deformity, which can increase the length of the anterior skull base and the length of the maxilla, so it can be the most causal treatment, but the surgery is more invasive and requires a higher surgeon.
(3) Osteotomy around the pyriform foramen: the bone block is moved forward after truncation, the surgical method is simple and less traumatic, and it is suitable for patients who cannot perform the above two procedures because of poor soft tissue development, and it needs to be combined with bone graft at the bone gap and at the dorsum of the nose.
(4) Maxillary sinus anterior wall bone graft: it can increase the midface prominence and significantly improve the appearance of the lateral part, and the surgery is simple and less traumatic, and the filler is mostly autologous iliac bone or rib bone.
4.Soft tissue surgery: The reconstruction of nasal dorsum and nasal tip and the lengthening of nasal columella can improve the appearance of patients to a greater extent.
(1) Reconstruction of nasal scaffold: including L-shaped cartilage graft and L-shaped bone graft. If bone grafting is performed, the amount of bone grafted should be relatively large to offset the postoperative bone resorption. Cartilage grafts are derived from autologous rib cartilage; autologous bone grafts are taken at the iliac bone or ribs.
(2) Correction of nasal base direction: for patients with nasal dorsal collapse, bone grafting can be used to reconstruct the nasal scaffold, and the nasal base will be corrected to a normal horizontal or slightly downward sloping direction at the same time; for patients with nasal dorsal convexity but lack of length, the rotational advancement method of nasal septal cartilage flap can be used.
(3) Correction of short nasal column: if it is not serious, V-Y molding can be performed at the base of nasal dwelling and upper lip; if the amount of tissue loss is too much, ear cartilage composite tissue flap free transplantation or bilateral nasal base flap filling can be used.
(4) Trimming of the nasal tip and nose wing: local cartilage forming is mostly used; if the amount of cartilage deficiency is more, ear cartilage or rib cartilage transplantation can be adopted.
In conclusion, there are many correction methods for Binder’s syndrome, which should be designed according to the severity of each patient’s deformity, local soft tissue conditions, as well as the patient’s economic situation and surgical tolerance, to develop a personalized plan for each patient. For patients with severe deformity, multiple surgeries are required, which requires close cooperation and careful follow-up.