A Periodic Case of Maxillo-Nasal Dysplasia or Binder Syndrome Successfully Operated With Bilateral Le Fort II Osteotomy With Distraction Osteogenesis

Abstract

Binder syndrome (BS) is an uncommon congenital disorder affecting the face. The condition, which also goes by the names naso-maxillary hypoplasia (NMH) and maxilla-facial dysplasia (MFD), causes the central face to develop inward and may also affect the upper jaw and the nose. A 19-year-old male with a known case of BS presented with a complaint of poor esthetics since birth. Previously, the patient was admitted to a private hospital where he was operated on for cleft lip and palate in the years 2003 and 2005. In 2017, he visited the dental clinic where the orthodontic treatment started for poor esthetics, and then he was referred to the oral surgery ward for surgical intervention. For about five years, he has been undergoing orthodontic treatment. A physical examination of the oral cavity was done and the physician suggested a CT scan of the brain. Recently, the patient underwent bilateral Le Fort II osteotomy with distraction osteogenesis under general anesthesia which repaired the patient's esthetics.

Progressive Strategy for Congenitally Missing Anteriors in Binder Syndrome: A Case Report

“…Jawade et al [8], in their study, mentioned distinctive facial features associated with this syndrome, including maxillary hypoplasia resulting in a reduced midface profile, severe malocclusion, a diminished nasal spine, atrophy of the nasal mucosa, and abnormal positioning of the nasal bones. While the absence of frontal sinuses can sometimes be present, it is not always the case [8]. Additionally, Binder's syndrome often presents with a concave facial profile, a reduced naso-frontal angle, and the possibility of hypertelorism [9].…”

“…Binder's syndrome primarily presents as an arhinoid face, characterized by a flat and vertically oriented nose due to the underdevelopment of the nasomaxillary structures [8]. Jawade et al [8], in their study, mentioned distinctive facial features associated with this syndrome, including maxillary hypoplasia resulting in a reduced midface profile, severe malocclusion, a diminished nasal spine, atrophy of the nasal mucosa, and abnormal positioning of the nasal bones. While the absence of frontal sinuses can sometimes be present, it is not always the case [8].…”

“…The psychological stress can be alleviated by carefully developing orthodontic treatment plans that are compatible with prosthetic rehabilitation [7]. It is possible to address the unattractive feature of missing anterior teeth temporarily during orthodontic therapy by providing a temporary prosthesis that will not interfere with orthodontic treatment mechanics [8]. Several methods are proposed to address this issue.…”

Craniofacial disorders and dysplasias: Molecular, clinical, and management perspectives

“…There are different approaches to the management of Binder's syndrome depending on the patient's age. Early orthodontic treatment is indicated to correct occlusal and skeletal anomalies ( Heo and Jin, 2018 ; Jawade et al, 2022 ). Furthermore, bone augmentation of the hypoplastic premaxilla is crucial before the patient reaches adulthood.…”

“…Multidisciplinary approach involving orthognathic, otorhinolaryngologic and reconstructive surgery are all possible treatments for Binder's syndrome. Osteotomies and cartilage or bone grafts are usually required to augment the hypoplastic skeleton in this condition ( Jawade et al, 2022 ).…”