Nasal Correction in Binder`s Syndrome: The Evolution of a

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Nasal Correction in Binder`s Syndrome: The Evolution of a
Aesth. Plast. Surg. 21:299–308, 1997
© 1997 Springer-Verlag New York Inc.
Nasal Correction in Binder’s Syndrome: The Evolution of a Treatment Plan
Fernando Ortiz Monasterio, Fernando Molina, and John Scott McClintock
Hospital General ‘‘Manuel Gea Gonzalez,’’ Mexico
Abstract. Maxillofacial dysplasia, or Binder’s Syndrome is a
challenge for the surgeon. The evolution of a surgical treatment
plan has led to improved facial contour and patient self-image.
We studied 27 patients with maxillonasal dysplasia of variable
degrees, both on a short- and long-term basis. In some patients,
surgical treatment began as early as 3 years of age, while others
were treated as teenagers or young adults. Surgical options
included cartilaginous onlay grafts to the pyriform area, nasal
dorsal grafts (linear or L-strut in design), and columellar strut
grafts. Le Fort osteotomies were reserved for those patients
with Class III malocclusion (15% in this series). The overall
goals were to augment skeletal deficiencies of the midface and
begin the soft tissue expansion process as early as possible.
From our long-term follow-ups (up to 15 years) it has become
apparent that surgical treatment should begin early. This leads
to improved self-image by the child’s preschool years, taking
advantage from their youthful skin elasticity. In the young patient, sequential lengthening procedures of the dorsum and
columella are beneficial. Paranasal and midfacial augmentation
is reserved until midfacial growth is near complete when the
patient is in their midteenage years.
Key words: Maxillonasal dysplasia—Binder’s Syndrome—
Tissue expansion
Maxillofacial dysplasia, better known as Binder’s Syndrome, has been a well-recognized facial deformity even
before the classical description by K.H. Binder in 1962
[2]. In 1952 Ragnell described a similar condition in one
of his patients with maxillary hypoplasia calling it dishface deformity [21]. Through the years Binder’s syndrome has also been called several other names: fossae
praenasalis [24], dysostosis maxillo-nasalis [15], archinencephalic malformation [2], and nasomaxillary hypoplasia [10].
Correspondence to Dr. Fernando Ortiz Monasterio, Calzada
Tlalpan 4800, Mexico 14000, D. F. Mexico
Maxillonasal dysplasia is a condition with unique
characteristics. Binder’s original description included
dysostosis maxillo-nasalis, midfacial hypoplasia with a
flat nose, flattened tip and alar wings, half-moon shaped
nostrils, short columella, acute nasolabial angle, absent
nasofrontal (glabellar) angle, and a concave midfacial
profile [2]. Holmstrom described further that these patients also typically have a palpable depression of the
anterior nasal floor, concavity of the inferior border of
the pyriform apperture centrally, hypoplastic posterior
nasal spine, and recession of the anterior nasal spine. He
also found that 54% of the Binder’s patient had a class III
malocclusion [11]. All Binder’s patients will have some
degree of these characteristics and they may range from
mild to severe forms.
The etiology of maxillonasal dysplasia is not completely known. Binder believed this condition was a form
of arhinencephalic malformation [2]. The problem with
this theory regarding these patients is that they rarely
have anomalies with their brain or upper third of their
face. Holmstrom proposed that there is inhibition of the
ossification center that would normally have formed the
lateral and inferior borders of the pyriform apperture
during the fifth and sixth gestational week [5]. Ossification of the maxillary external trabecular network is seen
around the eigth and ninth gestational week [11].
Surgical treatment for patients with maxillonasal dysplasia typically began no earlier than when they were 15
years of age. Ragnell applied iliac cancellous onlay bone
chips to the anterior surface of the maxilla through an
intraoral approach in a 16-year-old patient [21]. Converse described placing shell-like segments of bone
grafts to improve the contour of the midface. He also
preferred a vestibular approach for access around the
pyriform apperture [3]. Later, he proposed using an Lstrut bone graft to reconstruct the dorsum and shortened
columella [4]. Obwegeser also placed onlay bone and
cartilage grafts around the pyriform apperture and performed a LeFort I maxillary advancement in patients
with Class III malocclusion [16]. Henderson and Jackson
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advocated performing a LeFort II osteotomy and advancement [10]. Jackson later proposed, in addition to a
LeFort II advancement, placing bone grafts on the nasal
dorsum and paranasalis areas while performing V-Y
columellar lengthening [14]. Others favored performing
either LeFort I or LeFort II osteotomies but preferred
using costochondral grafts for nasal dorsal augmentation
to create a softer nasal tip. Several other techniques of
columellar lengthening have been applied to the treatment plans of the Binder’s patient [6,13,15]. All these
techniques leave a visible, unsightly columellar scar.
Tessier proposed in 1981 that columellar scarring techniques were not necessary in order to achieve adequate
columellar lengthening. He further stated that composite
grafts and flaps in the columellar area should not be used
because soft tissue expansion can be achieved alternatively. He stated that nasal skin has the remarkable ability to stretch to almost any extent as long as adequate
undermining at the lip-columellar junction is performed.
He preferred autogenous bone grafting to create soft tissue expansion of the columella. Approximately 1 year
later, he replaced the rigid bone graft with cartilage graft
to soften the nose [23].
Holmstrom in 1986 proposed a complete treatment
plan for the teenage Binder patient using bone grafts
around the perimeter of the pyriform apperture. He also
employed either L-shaped strut grafts for the dorsal support or septal advancement for those patients who had
adequate height of the nasal dorsum. He rarely had to
perform a LeFort I advancement. Orthodontics were required in only half of the patients in his series [12].
In this article we are reporting our experience on the
correction of the nasal and the paranasal areas in a series
of patients with Binder’s Syndrome treated at different
ages.
Materials and Methods
We have treated a series of 27 patients with maxillonasal
dysplasia since 1972. Degree of severity of maxillonasal
dysplasia ranged from mild to severe based on their
physical examinations. Physical exam findings include
midfacial hypoplasia, flattened nose, short columella
with an acute nasolabial angle, and retrusion or absence
of the anterior nasal spine. Class III malocclusion was
present in only four of the 27 patients. Patients’ ages at
the time of initial consultation ranged from 3 months to
25 years. Surgical treatment was started at age 4 in two
patients, between 8 and 11 in three, and after facial
growth was completed in 20. Postoperative follow-up
ranged from 1 to 15 years.
Nasal correction in the adult group was accomplished
by chondrocostal bone grafts to the dorsum and costal
cartilage grafts to the columella, to the nasal tip, to the
pyriform areas and in front of the nasal spine. In two
cases a costal cartilage graft was used to augment the
dorsum instead of costal bone. Preoperatively some columellar elongation and widening of the nostrils was obtained by nasal conformers.
The same treatment plan was followed in the two pa-
Nasal Correction in Binder’s Syndrome
tients treated between 8 and 11 years of age except for
the paranasal graft.
In the two young children, columellar elongation by
a modified Cronin technique was done at age 4 in combination with a chondrocostal dorsal graft. Costal cartilage grafts to the dorsum and to the columella were repeated every few years to keep up with facial growth
until age 14.
Midface advancement by a LeFort I osteotomy was
done in four patients after age 14.
Surgical Techniques
Through small bilateral intercartilaginous incisions, the
nasal dorsum is extensively undermined from the tip to
the glabela. Subperiosteal undermining is extended laterally to the nasal cheek junction to allow for maximum
expansion. Through a small unilateral intranasal incision,
the columella is undermined using Fomon curved scissors. A pocket is made between the two medial crurae
and extended inferiorly to the nasal spine and superiorly
to nasal tip.
A small infracartilaginous incision is made near the
alar dome on one side, and the nasal tip is undermined
with curved blunt Iris scissors. The dissection plane is
maintained between the dermis and the superficial fascia
of the SMAS.
Chondrocostal grafts are harvested through a small
submammary incision in females and a lower oblique
incision in males. Chondrocostal grafts were used for the
dorsum in the early series. The chondrocostal junction is
preserved with a portion of cartilage about 5–10 mm.
long. The costal bone is split manually with an osteotome
preserving the cancellous portion and the cortex on one
side as well as the periosteum. Shaping on the ‘‘new’’
dorsum is done with a Tessier crusher. The graft is made
of a long bony section and a short cartilaginous section
joined at the original chondrocostal junction. The cartilaginous end of the graft can be placed at the cephalic or
at the caudal end of the dorsum at the area where more
volume is needed. Obviously it should be carved to fit
the recipient area (Figure 1). In two patients, late in the
series the dorsal graft was carved entirely out of costal
cartilage. The graft was modeled to the optimal shape
and a thin K wire was introduced along the length of the
graft to prevent warping [9], (Figure 2).
The dorsal graft is introduced through the intercartilaginous incision. If necessary, it can be fixed to the nose
with a percutaneous K wire to prevent lateral displacement. Displacement in superior direction is prevented by
the limits of the subperiosteal pocket.
The columellar graft is then carved from the central
section of the costal graft to prevent warping. The shape
of the graft varies according to the retrusion of the columella and the position of the nasal spine. It may be
triangular in shape about 15–20 mm long and 5–8 mm.
wide at its base. It is inserted in the pocket previously
dissected between the medial crurae. The base of the
triangle is located inferiorly to achieve anterior projec-
F. Ortiz Monasterio et al.
301
Fig. 1. Diagram showing the modeling of
a chondrocostal graft for the nasal
dorsum. A small portion of cartilage is
left at one end and carved to fill the area
of maximum deficiency which may be
located at the tip or at the nasion.
Fig. 2. A costal cartilage graft taken from
the central core of the rib is carved to
provide support and to achieve caudal
protrusion of the columella.
Fig. 3. It is better to use a cartilage graft
when major augmentation of the dorsum
is required. A thin K-wire inside the graft
prevents warping. Columellar and tip
cartilage grafts are introduced through
small separate incisions.
Fig. 4. To maintain the position of the
cartilages when multiple cartilage grafts
are used, it is convenient to use a
hypodermic needle.
Fig. 5. A small incision is made at the
upper buccal sulcus to dissect the
paranasal area.
Fig. 6. A semilunar costal graft is
introduced into the pocket.
Fig. 7. A thin cartilage is inserted
transversally in front of the nasal spine.
Fig. 8. Diagram showing the areas
augmented with cartilage grafts: dorsum,
tip, columella, pyriform fossae, and
premaxilla.
302
tion of the columellar base (Figure 3). A triangular extension of the graft may be carved when possible at the
vertex of the triangle in order to achieve tip projection. In
most cases it is easier to carve a separate triangular or
shield-shaped graft, which is introduced through the
small infracartilaginous incision [17–19,22], (Figure 4).
Stabilization of the tip graft and maintenance of its relation with the dorsal graft is obtained by the percutaneous
insertion of a hypodermic needle which is removed at the
third postoperative day (Figure 4).
A 1-cm incision is then made on each side on the
upper buccal sulcus at the level of canines. A subperiosteal pocket is dissected around the pyriform apperture
under the alar cheek junction. The dissection is extended
medially under the sill of the nostrils and in front of the
nasal spine forming a tunnel communicating with the
subperiosteal pocket of the opposite side (Figure 5).
Two triangular pieces are carved from the costal cartilage. Its final shape is half a cone 13 × 15 mm. and
about 6–8 mm. thick at the base of the cone. The graft is
introduced into the pocket on the pyriform area where it
Nasal Correction in Binder’s Syndrome
should fit snugly to prevent displacement. Finally, a cartilage barr about 4 mm in diameter is inserted in the
transverse tunnel in front of the nasal spine [19]. The
nasal and oral wounds are closed with absorbable material (Figures 6, 7 and 8).
A standard LeFort I osteotomy was performed in four
patients to correct the maxillary hypoplasia.
Columellar lengthenings were performed in two patients early in our series [5]. Alternatively, in five patients, nostril conformers were used preoperatively for
6–12 months to allow soft tissue expansion of the columella area and the nostrils.
Results
The number of operations ranged from 1 to 6 (average
2.5) for each patient. Costal cartilage grafts maintained
their volume in all areas of the nose regardless of the age
of the patient. The same is true for the cartilages implanted in the pyriform area.
F. Ortiz Monasterio et al.
303
Fig. 9. (A,B) two-year-old girl with
nasomaxillary hypoplasia. (C,D) After
the first chondrocostal graft at age 5.
(E,F) Second cartilage grafting at age 9.
(G,H) Final grafting at age 13. (I,J)
Results at age 15 showing normal facial
contour and convexity with adequate
nasal projection
Bone grafts in children maintained their volume on the
cephalic portion of the dorsum corresponding to the nasal bones, but major resorption was observed in other
areas like the distal dorsum, the tip and the columella
(Figure 9). Similar results were obtained in five patients
operated between 8 and 10 years of age (Figure 10). In
these two groups, repeated dorsal augmentations were
necessary. From the 20 patients operated after 13 years
of age, 13 presented minimal bone graft resorption not
requiring further dorsal augmentation. In seven patients,
a secondary dorsal graft was necessary. Two patients
underwent dorsal augmentation with a cartilage graft reinforced with a thin K wire. No loss of volume has been
observed, and the shape of the dorsum remained unchanged after 1 and 2 years, respectively (Figures 11 and
14).
Minimal complications were observed in this series.
No skin necrosis occurred in spite of the extensive undermining and the insertion of relatively large grafts under moderate tension. One patient presented early post-
operative infection followed by extrusion of the bone
graft.
In all four patients who underwent LeFort I osteotomy
with maxillary advancement, normal dental occlusion
was achieved. There were no relapses but they clearly
had paranasal deficiency when seen on profile requiring
cartilage grafts (Figures 12 and 13).
Discussion
The lack of nasal and paranasal growth in patients with
Binder’s Syndrome is clearly related to a deficiency of
the osteocartilaginous framework formed by the septum,
the nasal bones, the upper and lower lateral cartilages,
and the maxilla.
Bone and cartilage grafts have been traditionally used
to correct this deformity. The major limitation to achieve
an optimal result is presented by the constriction of the
304
Nasal Correction in Binder’s Syndrome
Fig. 10. (A,B,C). A 12-year-old boy with typical Binder’s nasal and paranasal deformity. (D,E,F). Result after nasal and paranasal
cartilage grafting.
soft tissues covering the nose and of the lining of the
nasal cavities which were not expanded progressively as
it occurs in a normal child by the growth of the osteocartilaginous framework. If surgical treatment is started
early, then this problem can be prevented. In our two
patients in whom nasal augmentation procedures were
repeated every few years, normal nasal growth was
achieved avoiding the notching of the alar rims and the
depression of the nostril floor typical of this syndrome
(Figure 9).
In some older patients, the constriction of the soft
tissues limited the size of the pocket for the grafts resulting in suboptimal results requiring secondary procedures. The dorsal skin can be readily expanded but would
not yield as much if the pocket is dissected subperiostially. The periosteal layer must be released laterally to
allow for a good expansion. The tip presents a different
problem. In spite of extensive dissection all the way to
the nasolabial angle, the expansion is limited by the
shortness of the nasal lining preventing a good caudal
displacement of the columella (Figures 9–12). The same
phenomenon occurs at the nostrils firmly attached to the
underlying hypoplastic alar cartilages and to the constricted mucosal lining resulting in suboptimal results.
For these reasons, we prefer to use nasal conformers
preoperatively to expand the vestibular lining, the nostrils, and the columella. Obviously staged surgery started
early in life produces better final results because it follows a pattern similar to normal growth. The advantage
of improving the self-image of the patient during the
growing years is, in our opinion, very important.
The results of bone grafts remain unpredictable. Resorption can occur especially when the soft tissue cover
is very tight. Costal cartilage grafts, on the other hand,
maintain its volume over the years making it the ideal
material for augmentation. The large grafts required for
the dorsum must be carved to adapt to the bed and produce optimal shape. This may result in warping of the
F. Ortiz Monasterio et al.
305
Fig. 11. (A,B) Preoperative photos of
Binder’s patient with maxillo-mandibular
disharmony. (C,D) Postoperative results
after LeFort I advancement and
chondrocostal graft to the nose. Notice
the tightness of the columellar skin.
graft because the balance of the surface tension forces is
not maintained [1, 7, 8]. The insertion of a thin K wire
inside the graft as originally proposed by Gunter [9]
appears to be the solution to this problem.
Columellar elongation and other procedures designed
to increase the size of the nostrils leave permanent scars.
We feel this can be avoided by the prolonged use of nasal
conformers. Sequential treatment started early in life
seems indicated because increased structural support and
progressive soft tissue expansion are obtained simultaneously.
Early maxillary distraction (which was not used in this
series) is also indicated to correct maxillary hypoplasia.
This procedure has also the advantage of achieving simultaneous bone advancement and soft tissue expansion
[20].
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Nasal Correction in Binder’s Syndrome
Fig. 12. (A,B,C) Female adult with Binder’s showing nasal and maxillary hypoplasia. (D,E,F) Results after LeFort I advancement
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307
Fig. 13. (A,B,C) Preoperative condition
of 17-year-old female with Binder’s
without maxillo-mandibular disharmony.
(C,D) Results 2 years after paranasal,
dorsal tip, and columellar grafting. A K
wire was introduced into the dorsal
cartilage to prevent warping.
Fig. 14. (A,B,C,D) An 18-year-old female with Binder’s nasal deformity. (E,F,G,H) Results 1 year after augmentation of dorsum,
tip, columella, and paranasal area with costal cartilage grafts. (I) X-Ray showing the K wire inside the dorsal cartilage graft.
308
Fig. 14. Continued.
Nasal Correction in Binder’s Syndrome