Our Strategy in Management of Maxillonasal Dysplasia in Pediatric Patients

Abstract

Maxillonasal dysplasia or Binder syndrome is an uncommon condition. It is a congenital disease characterized by undergrowth of the central face and may include elements of the nose and upper jaw. The hallmark of the deformity is a retruded mid-face and an extremely flat nose. The timing and the surgical approaches for management of such deformity are still controversial. In this paper, we are going to present our experience in management of Binder syndrome in children. Seven children ranging in age from 6 to 13 years with classic features of Binder syndrome were operated upon in this study. The surgical approach was done at an early age and included 3 main components; nasal dorsum augmentation by costal cartilage graft, maxillary augmentation by rib grafts and columellar reconstruction by VY-plasty and strut grafts. Patients were followed for up to 6 years. Excellent results were obtained in all patients with this surgical procedure. Half of our patients required more than 1 surgery but none of them required any secondary orthognathic surgery. Therefore, we recommend that children with Binder syndrome should be managed at an early age at least for correction of their nasal deformities. Maxillary augmentation can also be done simultaneously or delayed for a second stage. During the second stage, further nasal augmentation can be accomplished. Definitive orthognathic surgeries have to be postponed to adolescence. This strategy can dramatically improve the patient aesthetic and alleviate the psychological upset without much disturbance of the facial growth.

Binder's syndrome: A narrative review

“…However, the timing of surgery has been a point of contention for many years. Many surgeons advocate early corrections due to the considerable psychological effect on the growing child 59 . It is also hypothesised that early augmentation gradually expands the soft tissue covering the nose and the lining of the nasal cavities, as it does in normal patients 60 .…”

“…The ideal approach would be to correct skeletal malocclusion orthodontically or through orthognathic surgery, followed by correction of nasal deformities 63,68 . However, few authors recommend undergoing an early nasal correction before starting school for psychological well‐being and good social integration with peers 59 . But parents should be informed about the need for definitive rhinoplasty after growth completion.…”

“…63,68 However, few authors recommend undergoing an early nasal correction before starting school for psychological well-being and good social integration with peers. 59 But parents should be informed about the need for definitive rhinoplasty after growth completion. The combination of orthodontic camouflage and augmentation of the paranasal maxillary region with cartilage grafts alone can improve the midfacial profile while avoiding the potential complications of maxillary osteotomies.…”

“…Many surgeons advocate early corrections due to the considerable psychological effect on the growing child. 59 It is also hypothesised that early augmentation gradually expands the soft tissue covering the nose and the lining of the nasal cavities, as it does in normal patients. 60 The main drawback is the need for secondary rhinoplasty during adulthood.…”