|
OJHAS Vol. 10, Issue 2:
(Apr-Jun 2011) |
|
|
| Binder’s Syndrome |
|
Subhas
Babu G, Prof. and
Head, Department of Oral Medicine and Maxillofacial Radiology, AB Shetty
Memorial Institute of Dental Sciences, Mangalore,
Kumuda Arvind
Rao HT, AB Shetty
Memorial Institute of Dental Sciences, Mangalore,
Shishir Ram Shetty, Assistant
Professor, Department
of Oral Medicine and Maxillofacial Radiology, AB Shetty
Memorial Institute of Dental Sciences, Mangalore,
Renita
Lorina Castelino, AB Shetty
Memorial Institute of Dental Sciences, Mangalore |
|
|
| |
|
|
|
|
|
|
Address for Correspondence |
AB Shetty
Memorial Institute of Dental Sciences,
Mangalore, India.
E-mail:
kumuda_arht@rediffmail.com |
|
|
|
|
|
Babu SG, Rao KAHT, Shetty SR, Castelino RL.
Binder’s Syndrome. Online J Health Allied Scs.
2011;10(2):20 |
|
|
|
|
Submitted: Apr 15,
2011; Accepted: Jul 15, 2011; Published: Jul 30, 2011 |
|
|
|
|
|
|
|
| |
|
| Abstract: |
|
Binder's Syndrome also known as Maxillo-Nasal Dysplasia is a developmental
disorder primarily affecting the anterior part of the maxilla and nasal
complex (nose and jaw). It is a rare disorder and the causes are unclear.
It is an uncommon condition characterized by a retruded mid-face with
an extremely flat nose. Hereditary factors and vitamin D deficiency
during embryonic growth have been researched as possible causes. Morphological
characteristics of the syndrome are of fundamental importance for the
correct diagnosis and treatment planning of these patients. We hereby
report to you a rare case of Binder's syndrome with clinical, radiographic
features and discussed the treatment options.
Key Words:
Maxillo-Nasal Dysplasia; High arch palate; Hypertelorism
|
|
Binder's syndrome (BS) is a congenital malformation characterized by nasomaxillary hypoplasia due to an underdevelopment of the mid-facial
skeleton. Binder type maxillonasal dysplasia(MND) is a rare condition
characterized by abnormal development (dysplasia) of the nasal and upper
jaw (nasomaxillary) regions. Many researchers suggest that Binder type
maxillonasal dysplasia does not represent a distinct disease entity
or syndrome, but, rather, is a nonspecific abnormality of the nasomaxillary
regions. In most cases, the condition appears to occur randomly for
unknown reasons (sporadically); rare familial cases have also been reported.
A 10-year old
female patient reported with a chief complaint of mal-aligned teeth since 3-4
years and was dissatisfied by poor esthetics.
There was no relevant history of pre-natal, natal disorders or long
term maternal drug intake. The patient’s family history was also non-contributory.
On extra oral examination, no gross facial asymmetry was detected. Concave
profile was seen due to midface deficiency. Flattening of right and
left cheek was noticed. Localised hypoplasia was noticed in the
alar basal region. Premaxilla was hypoplastic with flattening of maxillary
base and sagittal shortening of the maxillary arch. Nose was hypoplastic,
with flattened alae and the columella was short. Nasal bridge was flattened. Fronto nasal angle was absent. Nostrils were compressed giving it a
triangular shape when viewed from below. Sense of smell was normal.
Hypertelorism was noticed. Philtral crests were poorly developed, bow
shaped and rose vertically without convergence. Mandible showed normal
width and increased gonial angle. Relative mandibular prognathism was
seen due to maxillary shortening. Palpable depression
was present in the ala-nasal floor and maxillary sinus region. Macrostomia
with everted lower lip and hypoplastic upper lip and sparse hair in the
eyebrow region were also noticed.
 |
|
Figure
1: A- Showing malar hypoplasia and macrostomia; B- Showing
mid-face hypoplasia, depressed nasal bridge, relative mandibular prognathism;
C- showing triangular shaped nostrils. |
On intra-oral examination narrow, high arched, V-shaped palate was observed. Macroglossia was present. Patient presented with a class I malocclusion.
Spacing was seen in relation to upper anteriors. The tooth eruption
pattern appeared normal.
Lateral cephalogram revealed hypoplastic anterior nasal spine
and thinning
of labial plate of the alveolar bone over upper incisors. The maxilla
was retrognathic. The lower third face height was increased. The naso
maxillary angle was increased. Cephalometric studies revealed increased
gonial angle and proclination of incisors. Decreased anterior cranial
base measurements, smaller maxilla vertically and antero-posteriorly were also
noted. Based on clinical and radiographic features, a diagnosis of Binder’s
syndrome was arrived at.
 |
 |
|
Figure
2: Showing macroglossia and high arch palate. |
Figure
3: Lateral cephalogram showing hypoplastic anterior nasal spine.
|
Patient was
referred for orthodontic and surgical evaluation for effective management.
The essential feature of MND was initially described by Noyes in 1939,
although it was Binder who first defined it as a distinct clinical entity
in 1962.1 He reported on three cases and recorded six specific
characteristics: Arhinoid face, abnormal position of nasal bones,
inter-maxillary hypoplasia with associated malocclusion, reduced
or absent anterior nasal spine, atrophy of nasal mucosa, absence of
frontal sinus (not obligatory).2
The orthodontists and surgeons are more closely associated with these
patient’s due to the malocclusions and facial abnormalities, and the
information regarding BS is very sparse in the literature.
MND is a nonspecific abnormality of
the nasomaxillary complex. The familial examples are a result of complex
genetic factors, similar to those involved in producing a malocclusion.3
Although most cases involve only the nasomaxillary complex, a variety
of other anomalies have been recorded including especially cervical
vertebrae anomalies, but also various other skeletal defects, cardiac
anomalies, orofacial clefting, strabismus, mental retardation, and other
abnormalities.4,5 However these features were not observed
in our case. The affected patients show mid-face profile which is hypoplastic, flattened nose, convex upper lip with a broad philtrum,
typically crescent or semi-lunar in shaped nostrils due to the short
collumela, and a deep fold or fossa occurring between the upper lip and
the nose, resulting in an acute nasolabial angle.6
All but the last features were observed in our case.
The maxilla was hypoplastic in both antero-posterior and vertical directions
in early childhood and the latter showing some improvement with age.7
This causes the feature of relative mandibular prognathism although
mandibular length may be greater than normal, suggesting true prognathism
in some cases.8,9
Binder believed that his patients had a mild form of arhinencephaly
but there have been no reports of difficulties with the sense of smell
to support this hypothesis.2
Similar facial features may be seen in other well defined conditions,
including warfarin embryopathy, acrodysostosis, and Stickler's syndrome
which may be included under differential diagnosis. It should be possible
to distinguish each of these syndromes on the basis of additional historical
and clinical features.10
After assessment of the degree of facial bone abnormality, orthodontic
and surgical procedures can be planned.11 More severe cases
require a Le Fort I or II osteotomy with nasal grafting.12
As the degree of malformation in BS varies significantly, surgical correction
needs to be individually tailored.13
The characteristics of the Binder’s syndrome are typically visible
since a young age. It can also be present in combination with other
malformations. In such severe cases, the syndrome requires combined
orthodontic and surgical treatment to achieve an adequate facial profile.
- Noyes FB. Case report.
Angle Orthod 1939;9:160–165
- Binder KH. Dysostosis
maxillo-nasalis, ein archinencephaler Missbildungskomplex. Deutsche Zahnarztuche Zeitschift 1962;17:438–444.
- Gorlin R, Pindborg
JJ, Cohen M Jr. Maxillonasal dysplasia (Binder syndrome). Syndromes
of the head and neck. 2nd ed. New York: McGraw-Hill; 1976.
- Delaire J et al.
clinical and radiographic aspects of maxillonasal dysostosis (Binder’s
syndrome). Head Neck Surg 1980;3:105-122.
- Olow-Nordenram MA,
Radberg CT. Maxillonasal dysplasia (Binder’s syndrome) and associated
malformations of the cervical spine. Acta
Radiol (Diagn) 1984;25:353-360.
- Dyer FMV, Willmot
DR. Maxillo-nasal dysplasia, Binder's syndrome: review of the literature
and case report. Journal of Orthodontics. March 2002;29(1):15-21,
- Horswell BB, Holmes
AD, Levant BA, Barnett JS. Cephalometric and anthropomorphic observations
ofBinder's syndrome: A study of 19 patients. Plast Reconstr Surg 1988;83:325-335.
- Olow-Nordenram M.
Masillonasal dysplasia (Binder's syndrome). A study of craniofacial
morphology, associated malformations and family relations. Swted Dent
J [Suppl] 1987;47:1-38.
- Munro IR, Sinclair
WJ, Rudd NL. Maxillonasal dysplasia (Binder's syndrome). Plast Reconstr
Surg 1979;63:657-663.
- Quarrell OWJ, Koch
M, Hughes HE. Maxillonasal dysplasia (Binder's syndrome)
Med Genet 1990;27:384-387
- Rintala A, Ranta
R. Nasomaxillary hypoplasia-Binder's syndrome. Scand J Plast Reconstr
Swrg 1984;19:127-134.
- Holstrom H. Surgical
correction of the nose and midface in maxillonasal dysplasia (Binder's
syndrome). Plast Reconstr Surg 1986;78:568-580.
- Bhatt YC, Vyas
KA, Tandale MS, Panse NS, Bakshi HS, Srivastava RK. Maxillonasal dysplasia
(Binder's syndrome) and its treatment with costal cartilage graft: A
follow-up study. Indian
J Plast Surg. 2008 Jul;41(2):151-159
|
