Aesthetic Plastic Surgery

, Volume 33, Issue 4, pp 661–665

Correction of Septal and Midface Hypoplasia in Maxillonasal Dysplasia (Binder’s Syndrome) Using High-Density Porous Polyethylene

Authors

    • Medical FacultyBaskent University
  • Beyza Hancioglu Kircelli
    • Department of Orthodontics, Faculty of DentistryBaskent University
  • Baris Caglar
    • Department of Plastic and Reconstructive SurgeryBaskent University
Case Report

DOI: 10.1007/s00266-009-9312-5

Cite this article as:
Seyhan, T., Kircelli, B.H. & Caglar, B. Aesth Plast Surg (2009) 33: 661. doi:10.1007/s00266-009-9312-5

Abstract

Background

Maxillonasal dysplasia (Binder’s syndrome) is a congenital malformation characterized by an extremely flat and retruded nose.

Methods

This report describes an 18-year-old woman with maxillonasal dysplasia (Binder’s syndrome). The septal deficiencies and maxillary retrusion of the patient were corrected by using an L-shaped implant and a crescent-shaped high-density porous polyethylene implant through the oral vestibular sulcus via an external rhinoplasty approach.

Results

After the operation, the tip of the nose had moved 5.5 mm anteriorly in the sagittal plane and 11.9 mm coronally in the vertical plane. Also, the nasolabial angle had increased after the surgery. Follow-up evaluation at 24 months showed good correction of the nasal and midface projection.

Conclusions

High-density porous polyethylene implants are a good alternative for patients with Binder’s syndrome, especially those who will not accept costal cartilage grafts or orthognathic surgery and who have tendency for hypertrophic scarring.

Keywords

Binder’s syndromeMidface retrusionPorous polyethylene

Binder’s syndrome (maxillonasal dysplasia) is an easily recognizable congenital condition featuring nasomaxillary hypoplasia that produces a characteristic “dish-face” appearance and a retruded flat nose. Binder [2] first described the specific characteristics of the syndrome. Its major characteristics are midface hypoplasia, a retruded nose, a convex upper lip with a broad philtrum, a short columella, and a reduced or absent anterior nasal spine. Pseudomandibular prognathism or true mandibular prognathism may be associated with the syndrome [2, 9, 10, 16].

Before the description of Binder’s syndrome in 1962, shaped or chipped bone grafts from the iliac crest had been used to correct midface hypoplasia [3, 17]. L-shaped bone grafts were used to reconstruct the dorsum, and the shortened columella in patients with Binder’s syndrome [4, 8, 19]. Autogenous costal cartilage has been used successfully to augment the midface, columellar, and septal deficiencies [5, 12, 15]. We successfully used a high-density porous polyethylene sheet and carved crescent-shaped high-density porous polyethylene implants to correct the septal and midface deficiencies of Binder’s syndrome in a patient who refused harvesting of autologous costal cartilage grafts.

Case Report

An 18-year-old woman was admitted to our clinic with a nasofacial deformity that caused her considerable psychological strain. She had no other anomaly of the craniofacial region. The woman was of normal intelligence. She had three siblings, all of whom were reported as having normal appearances. None had a complaint similar to that of the patient.

At physical examination, the woman had maxillary hypoplasia with an extremely flat nose, low nasal tip, and a wide philtrum. She had poor oral hygiene and an angle class 1 malocclusion with anterior crowding. Her lips were competent at rest. She had some hypertrophied scars on her deltoid regions resulting from vaccination and minor traumas and reported a tendency to hypertrophic scarring. Three-dimensional computed tomography imaging showed a depression in the anterior nasal floor and the anterior wall of the maxillary sinuses as well as hypoplasia of the anterior nasal spine (Fig. 1a–d).
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Fig. 1

a, b Preoperative views of the patient. c Occlusal view of the patient. d Three-dimensional tomographic view of the patient’s skull

To correct the deformities, combined orthodontic and surgical treatment were suggested. However, the patient refused multibracketed orthodontic and surgical treatment because it would have taken a relatively long time. We then advised reconstructing the defects with a costal cartilage graft, but again, the patient and her family did not want us to harvest the costal cartilage graft due to the patient’s tendency of hypertrophic scarring. We therefore agreed to do the reconstructions with high-density porous polyethylene implants.

Informed consent for the operation was obtained from the patient. A standard open rhinoplasty technique was used during the surgery. The alar cartilages and nasal dorsum were skeletonized. Dissection was extended in the subperiosteal plane over the bony dorsum. Exploration showed the lateral crura of the lower lateral cartilages to be broad and the medial crura to be short. The upper lateral cartilages and septum were hyoplastic and deficient both anteriorly and dorsally.

The upper lateral cartilages were carefully dissected from the dorsal septum, and the septal mucosa was dissected in the subperichondrial plane bilaterally. The bony dorsum was rasped to open the roof of the bony vault. Excess cranial alar cartilages were excised, leaving approximately an 8-mm strip of the cartilage. To narrow the nasal base, low-to-low lateral osteotomies were performed through the bilateral intraoral incisions.

A 1.5-mm-thick L-shaped high-density porous polyethylene graft was cut from the high-density porous polyethylene sheet (Medpor; Porex Surgical, Newman, GA, USA). The L-shaped high-density porous polyethylene graft was inserted proximally between the nasal bones and settled dorsally and anteriorly to the existing deficient septum. The high-density porous polyethylene graft was sutured to the septum with 5-0 polypropylene suture and inserted between the medial crura of the alar cartilage to provide columellar support (Fig. 2a). The tip of the L-shaped graft was not fixed to the maxillary bone but just inserted into a slit made with an osteotome in the intermaxillary fissure above the hypoplastic anterior nasal spine. The sharp edges on the margin of the graft were rounded off with a bone rasp. Interdomal 4-0 polypropylene sutures were used to narrow the lateral crura of the nasal tip. The excised alar cartilages were trimmed and used as a dorsal onlay graft on the dorsal part of the L-shaped high-density porous polyethylene graft (Fig. 2b).
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Fig. 2

a Intraoperative view of the L-shaped porous polyethylene implant. b Schematic view of the porous polyethylene implants

The subperiosteal pockets were prepared near the pyriform apertura through the intraoral incisions. Crescent-shaped high-density porous polyethylene implants (30 × 28 × 7 mm, catalog no. 9525, 9526-Porex; Newnan, USA) were carved and placed in these pockets bilaterally and fixed to the underlying bone with 11-mm microscrews as onlay grafts (Fig. 2b). The pockets were irrigated, and the implants were impregnated with a rifampicin solution. Implant contouring was done after the implants had been fixed in place.

The intraoral incisions were sutured with 4-0 chromic catgut sutures. The open rhinoplasty incision was sutured with 6-0 polypropylene. Antibiotic impregnated nasal packings were placed after surgery and removed 1 day later. A nasal splint was kept in place for 7 days.

The woman’s postoperative course was uneventful. Follow-up evaluation at 24 months showed acceptable correction of the nasal and midface projection and noticeable psychological improvement of the patient (Fig. 3a–c).
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Fig. 3

a–c Postoperative treatment records. d Superimposition of the cephalogram before and after surgery

Cephalometric Analysis Results

We obtained preoperative and postoperative cephalometric radiographs of the patient. The scanned images were digitized and processed with Dolphin Imaging software version 10.0 (Dolphin Imaging & Management Solutions, Chatsworth, CA, USA). A Björk analysis was used to assess the cranial and dental features. Tracings were superimposed on the Sellanasion line at Sella.

After the operation, the tip of the nose had moved 5.5 mm anteriorly in the sagittal plane and 11.9 mm coronally in the vertical plane. Also, the nasolabial angle (Columellasubnasale-upper lip), which was 93.5º preoperatively, had increased to 114.0º after surgery (Fig. 3d). A lateral cephalometric analysis of the patient indicated a short anterior cranial base (N-S, 65.9 mm; normal, 75.3 mm) and a retrusive maxilla (SNA angle, 76.9º; normal, 82º) and mandible (SNB angle, 73º; normal, 80.9º) relative to the cranial base. Maxillary length (Ans-Pns, 41.5; normal, 51.6) had been reduced due to the hypoplasia of the anterior nasal spine. The patient had an increased lower anterior face height (Ans-gn, 76.1º; normal, 65º), a decreased ramus length (Go-ar, 44.2 mm; normal, 48.5 mm), and an increased craniomandibular angle (MP-SN, 47º; normal, 32.4º).

Discussion

Autologous tissues are criterion-standard graft materials for reconstructive nasal surgery because they have many qualities far superior to those of allografts. With regard to autogenous graft materials, cartilage has been the mainstay for augmentation in rhinoplasty. Septal cartilage is most commonly used for this, but in cases of maxillonasal dysplasia, septal cartilage often is unavailable in adequate quantities. Costal cartilage grafts are safe, readily available, pliable, stable, cost effective, and available in adequate quantities. Costal cartilage grafting has some disadvantages including an additional incision, visible scarring, postoperative pain, and a tendency of the graft to buckle.

Autogenous bone grafts have been suggested for correcting the deformities of Binder’s syndrome [3, 4, 8, 17, 19]. However, because of the unpredictable resorption pattern, the stiffness of the nose, and the tendency to fracture [8, 19], bone grafts have not been used as often as cartilage grafts.

High-density porous polyethylene implants have been used safely for nasal dorsal augmentation, for reconstruction of major saddle nose deformities, and as a spreader graft in nasoseptal surgery [1, 7, 11, 14, 18]. These implants permit ingrowth of connective tissue with related vascularization. Furthermore, they pose no problems to the donor area and do not require extra surgery. To the best of our knowledge, ours is the first report of high-density porous polyethylene implants used to correct dorsal and anterior septal deficits and midface hypoplasia in a patient with Binder’s syndrome.

High-density porous polyethylene implants require additional costs and are associated with some complications involving infection and extrusion. To reduce the risk of infection, we advise using antibiotics, irrigating the operation area with an antibiotic solution, keeping the insertion incision far away from the implant, and impregnating the implant with an antibiotic solution. Especially during correction of septal deficiencies with a high-density porous polyethylene sheet, the clinician must ensure that no mucoperichondrial tears are present during septal dissection because such tears could contaminate the implant and require its removal. They are the greatest drawback to this technique.

Extrusion of high-density porous polyethylene can be prevented by avoiding tension around the implant and using thicker soft tissue coverage on it. We placed a dorsal onlay cartilage graft between the implant and the nasal skin to thicken the tissue coverage. Also, rounding off the sharp edges of the implant prevents extrusion. When the decision is made to remove a high-density porous polyethylene implant for any reason, it is a difficult process because the implant is securely adhered to the surrounding tissue. If partial extrusion of a polyethylene implant occurs after weeks or months, partial resection of the implant can be done, and the remainder of the implant can be left in situ. However, it may cause a noticeable and unpleasant external scar.

The following cephalometric characteristics of patients with Binder’s syndrome have been reported: short anterior cranial base, a small cranial base angle, reduced sagittal development of the nose, a straight profile, and a retrognathic and short maxilla [6]. Pseudomandibular prognathism or true mandibular prognathism combined with a hypoplastic maxilla also have been reported with Binder’s syndrome [9].

The reported patient showed no mandibular prognathism. She did, however, have an increased vertical facial pattern with an increased lower face height and a steep mandibular plane. This vertical growth pattern of the mandible compensated the retrognathic maxilla so that she appeared with a convex profile rather than a straight or concave profile.

In his original description, Binder [2] attributed the flattened nasal tip and dorsum of the nose to the midfacial hypoplasia and the partial or total absence of the anterior nasal spine. In the reported patient, we saw that the anterior and dorsal cartilaginous septum were deficient, which led to retraction of the columella and to hypoplastic and collapsed upper lateral cartilages. The height of the proximal bony vault was normal in our patient. We believe patients with Binder’s syndrome should undergo correction of the anterior and dorsal septal deficits because both nasal length and nasal tip projection are inadequate in these patients.

The size of the L-shaped graft can be adjusted according to the deficit of the dorsal and anterior septum. In this study, the infracture of the nasal bones after low-to-low lateral osteotomies provided enough bony vault height in our patient. Draf et al. [5] used a V-Y–fashioned incision of the philtrum (including the columella) to lengthen the columella.

The retracted columella tolerated elongation of the septum in our patient. Therefore, we needed no extra maneuvers to lengthen the columella. Hölmstrom [8] stated that the columella had no real shortage of skin, only a retraction into the hypoplastic nasal floor of patients with maxillonasal dysplasia. Tessier et al. [20] reported that nasal skin can be lengthened to almost any extent in a short nose without the need for skin grafts or flaps.

In the reported patient, the nasal tip was advanced 5.5 mm anteriorly in the sagittal plane and 11.9 mm coronally in the vertical plane using high-density porous polyethylene implants. Hölmstrom [8] reported that the nasal tip was moved forward 6 to 10 mm in patients using an L-shaped bone graft from the iliac crest. Munro et al. [13] reported that the nose could be advanced as much as 15 mm and lengthened 10 mm with a combined Le Fort I and Le Fort II osteotomy in their patients with maxillonasal dysplasia. These authors advised such types of osteotomies for patients with severe malocclusion [13].

For the reported patient, the reconstruction was done in one stage. We therefore agree with Tessier et al. [20] that when surgery is contemplated for patients with Binder’s syndrome, they should be 16 years of age or older because at this age the maxilla is fully grown. In severe instances of the syndrome, Holmström and Kahnberg [9] recommend a two-stage surgical procedure: A maxillary osteotomy is performed first followed by nasal improvement.

We reemphasize that autogenous grafts should be considered as the first option for corrective rhinoplasty and midface hypoplasia. However, high-density porous polyethylene implants are a good alternative for patients with Binder’s syndrome, especially those who will not accept costal cartilage grafts or orthognathic surgery and who have a tendency to hypertrophied scarring. However, some of the aforementioned precautions should be taken to reduce the risk of high-density porous polyethylene implant complications.

Copyright information

© Springer Science+Business Media, LLC and International Society of Aesthetic Plastic Surgery 2009