CHRISMED Journal of Health and Research

CASE REPORT
Year
: 2016  |  Volume : 3  |  Issue : 3  |  Page : 232--235

Binder's syndrome: Presentation of a rare case


Kumuda Rao, Subhas G Babu, Renita Lorina Castelino, Supriya Bhat 
 Department of Oral Medicine and Radiology, A. B. Shetty Memorial Institute of Dental Sciences, Nitte University, Mangaluru, Karnataka, India

Correspondence Address:
Kumuda Rao
Department of Oral Medicine and Radiology, A. B. Shetty Memorial Institute of Dental Sciences, Nitte University, Mangaluru, Karnataka
India

Abstract

Binder«SQ»s syndrome (BS) or Maxillo-nasal dysplasia is a congenital malformation described first by Noyes. The syndrome was not recognized until Binder«SQ»s comprehensive report of three unrelated children in 1962. In patients with BS, the mid-face appears flattened, the columella is short and the upper lip slants backward. We hereby present a case with these typical findings of BS in a male patient.



How to cite this article:
Rao K, Babu SG, Castelino RL, Bhat S. Binder's syndrome: Presentation of a rare case.CHRISMED J Health Res 2016;3:232-235


How to cite this URL:
Rao K, Babu SG, Castelino RL, Bhat S. Binder's syndrome: Presentation of a rare case. CHRISMED J Health Res [serial online] 2016 [cited 2020 Jul 6 ];3:232-235
Available from: http://www.cjhr.org/text.asp?2016/3/3/232/183755


Full Text

 INTRODUCTION



Binder's syndrome (BS) individuals have a characteristic appearance that is easily recognizable. [1] Maxillo-nasal dysplasia (MND) or BS is an uncommon congenital condition characterized by a retruded midface with an extremely flat nose. [2] The mid-face profile is hypoplastic, the nose is flattened, the upper lip is convex with broad philtrum, nostrils are typically crescent or semi lunar-shaped giving a half-moon appearance, columella is short with deep fossa or folds between the upper lip and the nose, and resulting in an acute nasolabial angle. The frontonasal angle is almost 180°, resulting in a concave mid-face profile. [2],[3] If the columellar hypoplasia is severe, the nostrils may have a triangular shape. [4] Binder has also suggested that there was a disturbance of the prosencephalic induction center during embryonic growth. [5] Gorlin et al. suggest that MND is a nonspecific abnormality of the nasomaxillary complex and believed that familiar examples are a result of complex genetic factors, similar to those involved in producing a malocclusion. [6] Our patient reported to the department for esthetic concern secondary to midfacial deficiency and malar hypoplasia.

 CASE REPORT



A 23-year-old male patient reported to the department of oral medicine and radiology with a chief complaint of poor esthetics due to mid-facial deficiency and malar hypoplasia. There was no relevant prenatal, natal history, or history of long-term maternal drug intake. The patient's family history was also non-contributory. The patient gave no history of any previous orthodontic treatment.

On extra oral examination, no gross facial asymmetry was detected. Flattening of the right and left cheek was noticed [Figure 1]. The concave profile was seen due to midface deficiency causing MND. Localized hypoplasia was noticed in the alar basal region [Figure 2]. Premaxilla was hypoplastic with flattening of maxillary base and sagittal shortening of the maxillary arch. A palpable depression was present in the ala-nasal floor and maxillary sinus region. The nose was hypoplastic, with flattened alae and the columella was short. Nasal bridge was flattened [Figure 3]. Philtral crests were poorly developed having a bow-shaped appearance and rose vertically without convergence. Nostrils were compressed giving it a semi-lunar shape when viewed from below [Figure 4]. The sense of smell was normal. Intraorally, the palate was high arched; the tongue showed multiple fissures on the lateral borders [Figure 5]a and b. Following clinical examination, a lateral cephalogram was made [Figure 6] which revealed that the maxillary sinuses were hypoplastic. The frontonasal angle was absent. Mandible had relative prognathism with increased gonial angle. The patient had concave facial profile. Relative mandibular prognathism was seen due to hypoplastic maxilla.{Figure 1}{Figure 2}{Figure 3}{Figure 4}{Figure 5}{Figure 6}

The patient was surgically treated, and the depression over the sinus was filled with silicone implants. Surgical posterior repositioning of the chin was done [Figure 7]. The results were esthetically pleasing with the patient showing straight profile following surgery [Figure 8]a. Postsurgical lateral cephalogram was made [Figure 8]b. Regular follow-up was done for over a year, and the healing was uneventful.{Figure 7}{Figure 8}

 DISCUSSION



Binder in 1926 reported three cases and six characteristic features: [7] (1) Arhinoid face; (2) abnormal position of the nasal bones; (3) inter-maxillary hypoplasia with consecutive malocclusion; (4) reduced or absent anterior nasal spine; (5) atrophy of the nasal mucosa, and (6) absence of the frontal sinus. Our patient reported with esthetic displeasure due to malar hypoplasia. The patient presented with a concave profile. The orthodontists and surgeons are more closely associated with these patient's due to the malocclusion and facial abnormalities, and the information regarding BS is very sparse in the literature. [8] Although most cases involve only the nasomaxillary complex, a variety of other anomalies has been recorded, especially anomalies of cervical vertebrae, also various other skeletal defects, cardiac anomalies, orofacial clefting, strabismus, mental retardation and other abnormalities. [4],[9] However, our patient did not present with any of these abnormalities. Maxillary hypoplasia leads to relative prognathism and Angle's Class III malocclusion. In some cases, the mandibular length may be greater than normal, thus suggesting true prognathism. [10],[11],[12] Our patient presented with concave profile, maxillary hypoplasia, Class I molar relation which was associated with skeletal prognathism of the mandible. Cases such as these require combined surgical and orthodontic team effort. Our patient was treated with surgical graft placement for maxillary hypoplasia in the malar region, surgical posterior repositioning of the chin.

 CONCLUSION



The characteristics of BS usually present since young age. It can also be present in combination with other deformities. The treatment of BS depends on the degree and the severity involving the deformity. In severe cases, the syndrome requires combined orthodontic and surgical treatment to achieve a satisfactory facial profile. Patient satisfaction with respect to esthetics and function following treatment holds a very important key in the management of BS patients.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/ their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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