The maxillonasal dysplasia

Maxillonasal dysplasia also called Binder’s syndrome is a congenital deformity characterized by nasomaxillary hypoplasia that is attributed to underdeveloped mid-facial skeleton (1). They have a characteristic appearance that is effortlessly identifiable (2). Binder, who first defined this syndrome as a distinct clinical entity in 1962, attributed the cause of this syndrome to the disturbance of the prosencephalic induction center during embryonic life (3). Noyes in 1939 described the salient features of Binder’s syndrome (4). Binder reported 3 cases and presented 6 characteristic features for this syndrome (5). Arhinoid face, abnormal position of the nasal bones, intermaxillary hypoplasia with consecutive malocclusion, reduced or absent anterior nasal spine, atrophy of the nasal mucosa and absence of the frontal sinus.


Dear Editor,
Maxillonasal dysplasia also called Binder's syndrome is a congenital deformity characterized by nasomaxillary hypoplasia that is attributed to underdeveloped mid-facial skeleton (1). They have a characteristic appearance that is effortlessly identifiable (2). Binder, who first defined this syndrome as a distinct clinical entity in 1962, attributed the cause of this syndrome to the disturbance of the prosencephalic induction center during embryonic life (3). Noyes in 1939 described the salient features of Binder's syndrome (4). Binder reported 3 cases and presented 6 characteristic features for this syndrome (5). Arhinoid face, abnormal position of the nasal bones, intermaxillary hypoplasia with consecutive malocclusion, reduced or absent anterior nasal spine, atrophy of the nasal mucosa and absence of the frontal sinus.
The third month of pregnancy marks the period in which the process of nose formation generally takes place (6). There is equal predilection for males and females. This syndrome is rare, which may be a reason for the dearth of information regarding its true cause, patterns of inheritance and actual prevalence (7).
A 19 year old female patient visited the Department of Oral Medicine and Radiology with a chief complaint of defective nose since birth. Patient complained of associated nasal twang in the tone of her voice. Patient gave a history of irregularly placed upper and lower front teeth since the time of its eruption. Prenatal, natal, post-natal history and family history was non-contributory.
On extraoral examination patient was found to have concave facial profile, absent nasofrontal angle, hypoplastic nose with flattened alae and nasal tip, flattening of right and left cheek. There is a palpable depression in the anterior nasal floor and localized maxillary hypoplasia in the alar base region. Nose was hypoplastic, with flattened alae and short columella. The nostrils are half-moon shaped when viewed from below, increased mandible gonial angle,   Based on clinical and radiographic features, a diagnosis of Binder's syndrome was arrived at. Warfarin embryopathy, Down syndrome, Apert syndrome, Stickler syndrome, Keutel syndrome and acrodysostosis were considered as differential diagnosis.
Majority of cases of Maxillonasal dysplasia are isolated. Few cases have been reported where positive family history was stated in 36% of the subjects Therefore the inheritance pattern may be either autosomal recessive with reduced penetrance or multifactorial in nature. There are reports that Binder's syndrome has a direct relationship with vitamin K deficiency (8).
Arhinoid face with a vertical and flattened nose, mid-face hypoplasia, reduced nasal spine, atrophied nasal mucosa, malocclusion, lack of frontal sinus are the most common features. There may be concave facial profile, reduced naso-frontal angle, hypertelorism and short columella. When observed from below, the nostrils, have a semilunar or a half moon or crescent shape. In severe cases, it may be triangular in shape. According to Holmstroem et al in normal human beings naso-labial angle is 103-117°, while in Binder's syndrome it has a value of 76-88° (6,7).
The anterior crest that separates the floor of nasal cavity is absent, and there is a hypoplastic or absent anterior nasal spine resulting in a flat facial profile without a nasal prominence. Short anterior cranial base, posteriorly positioned maxilla, which leads to Class III malocclusion and relative prognathism is seen. Some patients may present a cleft palate (7).
Binder's syndrome can be easily diagnosed by using 2D and 3D ultrasonography, from the commencement of the 21st week of pregnancy. Several syndromes are considered among the differential diagnosis of Binder's syndrome (Table 1) (9). On the basis of the degree of facial deformity, orthodontic procedures and surgical treatments can be planned and is tailor made for the particular individual. Augmentation of the premaxilla is essential. Nasal augmentation and lengthening of the columella with autogenous costal cartilage grafts must be done for effective treatment. Grafts must be carved from a central segment and dipped in 0.9% of NaCl solution to prevent warping and to reduce rate of resorption. Cartilage grafts with slots helps in better fixation of the grafts.
Augmentation is enough to give an aesthetically appealing and acceptable facial profile in mild to moderate cases (10).