TECHNICAL STRATEGY

Nasal Reconstruction for Maxillonasal Dysplasia

Anthony D. Holmes, FRACS,* Shu Jin Lee, MRCS, MD,* Andrew Greensmith, FRACS,*
Andrew Heggie, FRACDS (OMS),* and John G. Meara, MBA, FRACS*

Background: Maxillonasal dysplasia, Binder type (Binder syndrome and nasomaxillary hypoplasia), is a spectrum of decient
nasomaxillary osteocartilaginous framework, decient nasal soft
tissues, and a short columella. The correction of these deformities is
challenging, and results are often disappointing. Tissue expansion
with multiple bone grafts for nasal augmentation from childhood has
been advocated as a means to address the constricted soft tissues.
However, bone grafts in children have been associated with unpredictable growth and resorption. Agreeing with the principle of serial
nasal augmentation that commences in childhood, we used alloplastic material for tissue expansion followed by denitive reconstructive rhinoplasty at the completion of growth and orthognathic
surgery as required. Denitive rhinoplasty mainly used a 1-piece
costochondral graft cantilevered to the frontal bone.
Materials and Methods: Thirty-one patients over a period of
27 years were reviewed. The patients were divided into 2 groups
based on the age of presentation, namely, prepubertal and postpubertal. The prepubertal group underwent serial tissue expansion
of the constricted nasal envelope with customized silicone implants
and nal reconstruction by costochondral rhinoplasty at the end
of puberty. The postpubertal group underwent 1-stage costochondral rhinoplasty. The denitive rhinoplasty used a cantilevered 1piece costochondral graft retaining the dorsal periosteum that was
dowelled into the frontal sinus wall.
Results: In the prepubertal group (n = 20), 41 silicone implants were
placed in the childhood years for tissue expansion of the nasal envelope. One patient developed implant infection, and another required
replacement after extrusion. Long-term follow-up showed minimal
resorption of the costochondral graft in the pre-expanded prepubertal
group and minimal to moderate graft resorption in the postpubertal
group.
Conclusions: Successful treatment of maxillonasal dysplasia is dependent on the following: an understanding of the underlying pathologic anatomy, namely, that of the constricted nasal tissues, serial tissue
expansion of the nasal envelope in childhood, and denitive costochondral rhinoplasty at the end of growth. Early tissue expansion
with the placement of alloplastic silicone implants effectively stretches
the constricted nasal soft tissues in Binder syndrome to limit graft
From the *Department of Plastic and Maxillofacial Surgery, Royal Childrens
Hospital, Melbourne, Victoria, Australia; and Department of Plastic Surgery, Childrens Hospital Boston, Boston, Massachusetts.
Received October 23, 2009.
Accepted for publication November 15, 2009.
Address correspondence and reprint requests to John G. Meara, MBA,
FRACS, Childrens Hospital Boston, Boston, MA 02115;
E-mail: john.meara@childrens.harvard.edu
Copyright * 2010 by Mutaz B. Habal, MD
ISSN: 1049-2275
DOI: 10.1097/SCS.0b013e3181d024b0

The Journal of Craniofacial Surgery

resorption after denitive nasal reconstruction with costochondral rib

grafts. There is a possible role for similar tissue expansion in the
postpubertal patient with alloplastic material before costochondral
grafting if the soft tissues are inadequate. Long-term resorption of
cantilevered, 1-piece, periosteum-covered costochondral grafts was
minimal.
Key Words: Maxillonasal dysplasia, maxillonasal dysostosis,
nasomaxillary hypoplasia, Binder syndrome, Binder association
(J Craniofac Surg 2010;21: 543Y551)

axillonasal dysplasia results from hypoplasia of the osteocartilaginous framework of the nasomaxillary region1Y11 and
deciency in anteroposterior growth of the nasomaxillary complex.12,13 This results in signicant shortage of both the bony
framework and the soft tissues of the nose. The osseous deciency
is evident in the decient length and projection of the caudal portion of the nose with the pathognomonic feature being absence
of the anterior nasal spine.2,4,5,7,10,11 There is deciency of the
nasal soft tissue envelope and the nasal lining and, in particular, a distinctive short columella (Fig. 1). The nasomaxillary hypoplasia may be associated with a normal or class 3 dental occlusal
relationship.3,7,11,14
Correction of the nasal deformity in maxillonasal dysplasia
is challenging, and results are often disappointing.3,15,16 Many surgeons approach the correction of the nasal bony and soft tissue
deciencies separately. Attempts to correct the foreshortened nose
have been unsatisfactory with obvious scarring resulting from V-Y
or similar procedures to lengthen the nose,17Y19 forehead aps to
provide additional skin covering,17 and skin grafts for nasal dorsal inlays.20 Composite conchal grafts for nasal lengthening require multiple operations and provide only small amounts of nasal
lengthening.3,21 The problem of decient nasal lining has been
previously addressed with local turnover hinge aps, nasolabial
aps,17 and intranasal epithelial inlays that require permanent supporting prostheses3,22,23 to overcome scar contraction.
Correction of the bony deformity was rst attempted using
the LeFort II osteotomy that was championed to provide simultaneous correction of both the nasomaxillary hypoplasia and the foreshortened nose.3,4,24Y27 The LeFort II osteotomy does not adequately
address the retruded midface, and the nose was insufciently
lengthened.12,27 Others used additional bone and cartilage grafts inserted via visible external incisions for paranasal augmentation and
nasal lengthening, and local aps are used for columella elongation.27
These procedures are often associated with visible scarring16 and unpredictable growth28 and resorption of bone grafts.16 Obvious scarring
also followed the use of the trapdoor incision over the nasal dorsum
for nasal lengthening29 and the paranasal incision for LeFort II osteotomies.27 The LeFort II advancement can lead to the conversion of
normal or mild class 3 occlusion to severe class 2 occlusion.4,27 The
LeFort I osteotomy alone was unsatisfactory because it accentuates
the nasal retrusion.30

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Holmes et al

FIGURE 1. Classic Binder prole showing the classic short

nose with short columella.
The approach to the Binder syndrome patient then became
2 staged, with the correction of the maxillary deformity with orthognathic treatment of the malocclusion rst and, later, the
correction of the nasal deformity with bone graft nasal augmentation.11,30,31 As treatment commences after puberty, the limitations of
this approach include school-age psychologic problems and that the
constricted nasal envelope remains unaddressed throughout childhood. The decient nasal envelope not only limits the size of the
bone graft but has also been proposed to be constrictive and cause
bony resorption that is especially noticeable in the region of the
foreshortened nasal tip.16
To overcome this limitation, Tessier32 proposed tissue expansion of the decient nasal envelope and columella with serial bone
grafting, and Ortiz-Monasterio et al15 showed satisfactory correction
of the nasal deformity using multiple costochondral rib grafts from
early childhood32 to achieve serial expansion before denitive costochondral rhinoplasty.
We present 1 surgeons (A.D.H.) experience with the correction of the nasal deformity in maxillonasal dysplasia in 31
consecutive cases. This approach rst addresses the problem of soft
tissue deciency with serial alloplastic augmentation of the nasal
envelope from early childhood. Final nasal correction is performed
with costochondral grafts at the completion of growth. Orthognathic
surgery is performed at the time of nal nasal correction or thereafter, if indicated.

PATIENTS AND METHODS

The records of 31 consecutive cases of maxillonasal dysplasia treated in our institution since January 1, 1980, were reviewed. There were 2 age group distributions: prepubertal and
postpubertal. The male to female ratio was 1:1. The anterior nasal

& Volume 21, Number 2, March 2010

spine was hypoplastic or absent in all the patients. The age at the
rst nasal surgery ranged from 5 to 24 years, with a mean of
12.3 years. In the prepubertal group (n = 20), the age range was from
5 to 12 years, and in the postpubertal group (n = 11), the age range
was from 14 to 24 years.
In the prepubertal group, all the patients underwent silicone
nasal augmentation. Between 1 and 3 silicone nasal implants were
inserted while the patients passed in to their teenage years. Most
patients required 2 implants (n = 15/20) to expand in tandem with
facial growth. Two patients had 1 implant placed. Three patients
required 3 implants. In this group, 1 patient had commenced treatment early at the age of 5 years, 1 patient had extrusion of the
implant after an altercation, and another had an infection that
required removal of the spacer, debridement, and intravenous antibiotics (n = 2/18). The spacer was replaced after 6 months with no
further infections.
In the prepubertal group, denitive costochondral rhinoplasty
was performed from the ages of 19 to 22 years, with the mean age of
19 years (n = 17). Two patients are still in their childhood years and
awaiting denitive rhinoplasty. One patient refused denitive rhinoplasty, being content with the silicone implant. The postpubertal
group of patients had a mean age of 16.2 years at the time of nasal
reconstruction (n = 11). In this group of patients with delayed
presentation, nasal reconstruction followed orthognathic surgery
with LeFort I maxillary advancement. Seventy percent of the
patients had class 3 dental occlusion (n = 21), and 30% of the
patients had class 1 dental occlusion (n = 10). Ninety percent
(n = 17) of the patients underwent orthodontics, and 60% (n = 11)
underwent orthognathic surgery to correct class 3 malocclusion via
LeFort I maxillary osteotomy. Follow-up periods after completion
of the denitive nasal reconstruction ranged from 4 to 22 years,
with a mean of 15 years.

RESULTS
In the prepubertal group of 19 patients, 41 implants were
placed in total. There was 1 case of implant infection that required
removal and antibiotics treatment. The patient underwent the placement of another implant 6 months later with no further problems.
Another patient had exposure of the implant after a schoolyard
altercation requiring implant replacement.
Resorption of the bone graft was assessed from 3 years
postoperatively. Those who had serial tissue expansion and subsequent adult or postpubertal costochondral grafting did best
with minimal resorption noted. Prepubertal bone grafting was
noted to be associated with overgrowth in 1 case. Those who presented postpubertal costochondral and had a 1-stage costochondral reconstruction had minimal to moderate resorption (n = 3)

FIGURE 2. Transbuccal insertion of customized nasal implant with the short limb of the L-shaped implant fashioned
to be stouter than the long limb and allowing for extension from the pyriform margin to the proposed nasal tip.

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Binder Syndrome

FIGURE 3. In the harvesting of the costochondral graft, it is important to choose a straight segment of rib and to maintain
lateral periosteal aps that are folded over the lateral aspects of the graft.

in the region of the nasal tip ranging from 3 to 5 mm. Those

patients (n = 3) who had other bone grafts (iliac) had a large resorption rate as compared with patients with costochondral grafts. In the
initial experience with the postpubertal group, 1 patient had a rhinoplasty performed with an iliac bone graft that on follow-up, was
noted to have complete resorption of the caudal end of the iliac bone
graft, requiring correction with a costochondral rib graft.

Treatment Protocol
Serial Silicone Nasal Spacers for
Tissue Expansion
The placement of silicone nasal spacers was performed in the
prepubertal group. An L-shaped silicone implant is customized to
the dimensions required and carved from a block of medical-grade
silicone (Fig. 2).

The vertical limb is more substantial than the conventional

silicone nasal implant and is carved to have a stout rectangular
cross section. The vertical limb extends from the superior edge of
the pyriform margin to project 3 to 5 mm higher and more anterior
to the nasal tip to stretch the columella vertically and anteriorly.
The dimensions of the vertical limb are adjusted to provide the
maximal length able to be accommodated within the soft tissue
nasal pocket without blanching of the overlying skin. The
horizontal dorsal limb extends to the nasofrontal junction but is
tapered cranially where the bony deciency is less marked than in
the caudal portion of the nose.
Earlier cases in our chart review revealed that the silicone
implants were inserted via an intra-oral superior alveolar incision,
dissecting the nose via a membranous septal tunnel to the dorsum.
Later, an open rhinoplasty technique was used to ensure that the alar
domes were joined to cover the prosthesis in the region of the nasal

FIGURE 4. Lateral periosteal aps are sutured to the upper lateral cartilages to maintain the integrity of the internal nasal
valves, and at the region of the nasal tip, the lower lateral cartilages are sutured above the costochondral graft.
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Binder Syndrome

tip and reduce the risk of implant exposure. There was no signicant
difference in the results, and the open rhinoplasty is now preferred
because of improved visibility and access to upper and lower lateral
cartilages.

Denitive Nasal Reconstruction With

Costochondral Graft
At the completion of growth, patients underwent denitive
nasal reconstruction. The technique involves a fully cantilevered
1-piece costochondral graft with the dorsal periosteum retained.
The senior author has used this technique successfully for 29
years, particularly for nasal reconstruction cases where strong
nasal support is required and the dorsum is decient. In maxillonasal dysplasia, the distal part of the nose has inadequate
support and requires more buttressing than can be offered by
septal grafts or similar techniques. Preoperatively, skull x-rays
are taken to conrm the presence of a pneumatized frontal sinus
and to avoid dural penetration during preparation for stabilization of the graft.
Via a stepped columella incision and infracartilaginous rim
incisions (open rhinoplasty), the nasal envelope is elevated from
the nasal cartilages under the submuscular aponeurotic system
plane of the nose and then subperiosteally up to the level of the
frontal sinus. The medial crura are separated to approach the caudal
septum. The medial attachments of the upper lateral cartilages are
carefully dissected from the septum without breaching the nasal
mucosa.
The nasal dorsum is rasped or burred to create a level surface
for the placement of the graft, and a medium pineapple burr is then
used to create a 5- to 8-mm aperture through the anterior wall of the
frontal sinus. The desired length of graft is measured from the frontal
bone to the proposed projection of the nasal tip to create a nasolabial
angle of 90 degrees in the male and 110 degrees in the female. Extra
length is harvested to allow for nal trimming of the caudal end
when the graft is xed.
A straight segment of the costochondral cartilage is required
and usually corresponds to the 9th or 10th rib (Fig. 3). The graft is
harvested via a small Kocher incision, and the donor site is closed.
The grafts are harvested with a sleeve of the periosteum and the
perichondrium if possible (especially on the dorsal surface). Typically, the grafts are two-thirds osseous and one-third cartilaginous.
Care is taken not to disrupt the osseochondral junction.
Shaping of the graft was performed by drawing a pattern on
the straightest part of the rib, and then the periosteum was elevated
from the sides before burring to achieve the nal size and shape. The
periosteum is then apped over the contoured edges at the side. The
periosteum at the edges is then sutured to medial dorsal edges of
the upper lateral cartilages, hence acting as a Bspreader[ graft by
opening the internal nasal valves. The entire subcutaneous surface of
the graft is therefore covered in periosteum.
The costochondral graft was then oriented with the bony end
cephalad and the cartilaginous portion caudal (Fig. 4). In most
cases reviewed, the uppermost 10 mm of the grafts were trimmed to
create a tapered cortical peg for stable insertion of the grafts into
the trephined basal bone of the frontal sinus. This technique was
ascertained by the authors from Paul Tessier in 1977. Additional
xation is usually not required. Occasionally, to avoid lateral

FIGURE 6. Patient 2. The patient presented as a child.

Upper row, the patient as a child with features of Binder
syndrome. Middle row, improvement of the nasal form
after the silicone nasal implant. Lower row, 1 year after
the costochondral graft for nasal reconstruction. She also
underwent orthognathic surgery to correct a class 3
malocclusion at the completion of growth.
instability, the addition of percutaneous Kirschner wires is required
and inserted at a right angle to the graft through the dorsal skin
proximally and through to the nasal bone. In the absence of a

FIGURE 5. Patient 1. The patient presented as a child. First row, preoperative pictures. Second row, insertion of silicone nasal
prosthesis. Third row, postoperative pictures after insertion. Fourth row, the patient 4 years after the silicone nasal implant.
Fifth row, preoperative photos before the costochondral graft for nasal reconstruction at age 16 years. Last row, postoperative
photos after the nal nasal reconstruction.
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Holmes et al

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& Volume 21, Number 2, March 2010

of the medial canthii. A thermoplastic external dorsal splint is

applied and maintained for 10 to 14 days.

Patients
Patient 1 (Silicone Nasal Implant Placement
in a Prepubertal Patient)
A 7-year-old girl presented with the classic nasal deformity for which she requested treatment (Fig. 5). She underwent
the insertion of a customized silicone nasal implant placed via
the transoral insertion at age 8 years (intraoperative pictures
are those of a similar patient). The implant was placed under
the alar cartilages. There was satisfactory increase in tip projection, nasal length, and columella length. Postoperative pictures
are shown at ages 8, 12, and 16 years. At 17 years of age, a
maxillary advancement followed by a costochondral rhinoplasty
was performed.

Patient 2 (Prepubescent Presentation)

FIGURE 7. Patient 3. The patient presented in adulthood.
Upper row, preoperative pictures. Lower row, 2-year
postoperative pictures showing satisfactory nasal projection.

frontal sinus, a bony ledge is burred in the cephalad end of the

nasal dorsum and the graft is secured by 2 percutaneous Kirschner
wires left in situ for 2 weeks. Lateral nasal bone in-fractures are
performed where necessary to narrow the nasal width. The
cartilaginous portion of the costochondral graft is trimmed to
length and to a relatively thin width at the region of the nasal tip.
The upper lateral cartilages are then connected to the periosteum of
the lateral aspects of the graft with 4-0 Vicryl sutures to enhance
the opening of the internal nasal valves. The lower lateral cartilages
are then mobilized to allow elevation and attachment of the medial
crura and domes to the cartilaginous pole of the graft to provide for
adequate projection and shape. In some cases, Sheen-type dome
augmentation grafts were added in thick-skinned individuals to
get more tip denition. Extra cartilage was sometimes used to
augment the decient caudal septum region to help with an aesthetic columella nasolabial angle.
The columella skin incision is then closed with a 6-0 nylon;
and the mucosa, with a 5-0 catgut. If necessary, alar base reductions
are performed to ensure that the interalar width is equal or less to that

A 12-month-old girl presented with a nasal deformity

(Fig. 6). She underwent silicone nasal augmentation at the age
of 8 years, placed via a transcolumellar incision. Correction of
the class 3 dental malocclusion was performed with combined
orthodontic treatment and orthognathic surgery and consisted of
a LeFort I maxillary advancement (5 mm, anterior; 5 mm, anterior
impaction; and 2 mm, rotation to the left), asymmetric mandibular
reduction with bilateral sagittal section osteotomies, and an
advancement genioplasty (6 mm) at the age of 17 years. A denitive costochondral rhinoplasty, with the graft secured into the
base of the frontal sinus bone together with Sheen tip grafts, was
performed at age 19 years. Minimal graft resorption was noted at
10 years follow-up.

Patient 3 (Postpubescent Presentation)

A 24-year-old woman presented without a diagnosis,
requesting correction of her poor nasal projection. She underwent
a 1-stage costochondral reconstruction secured via a frontal bone
trephine. There was no obvious graft resorption on 8 years followup (Fig. 7).

Patient 4 (Complication of Graft Overgrowth)

A 7-year-old boy presented having had a previous procedure
with another surgeon who performed a costochondral graft

FIGURE 8. Upper row, lateral cephalogram showing overgrowth of the costochondral graft in patient 4. Lower row,
postoperative lateral cephalogram showing improvement of facial proportions and improved nasal form.

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cantilevered to the frontal bone via a bicoronal approach. At the

completion of puberty, he presented with graft overgrowth as
evidenced by a long, curved graft; increased nasal length; and an
acute nasolabial angle (Fig. 8). He had a class 3 malocclusion that
required orthognathic surgery, and the previous graft had to be
removed to facilitate surgical access for maxillary mobilization and
advancement. At the age of 19 years, orthognathic surgery was
performed simultaneously with removal and replacement with a new
costochondral nasal graft (Fig. 9). At follow-up at 10 years, there
was minimal graft resorption.

Binder Syndrome

nasal tip. This allows the vertical limb of the implant to expand the
short columella and avoids columellar lengthening aps and
consequent scarring. The standard commercial nasal prosthesis is
inadequate. In the evolution of our treatment plan, we previously
tried the transoral approach for the placement of the silicone
implants. We now prefer an open rhinoplasty approach because it
allows direct access to the lower lateral cartilage.
Carved silicone nasal implants are well suited to the Binder
population who may require multiple-staged procedures. In our early
experience, we used costochondral graft for nasal augmentation in
childhood, but growth was unpredictable because some resorbed and

Patient 5 (Postpubescent Presentation)

This 14-year-old girl presented concerned about her
nasal deciency. She had a mild maxillary deciency and a class
3malocclusion. A costochondral nasal reconstruction was performed primarily followed by maxillary advancement when she
was more mature. Postoperative pictures were taken at age 19 years
(Fig. 10).

DISCUSSION
The 3 main deformities in maxillonasal dysplasia are the
hypoplastic osteocartilaginous nasomaxillary region, constricted distal nasal soft tissues, and a short columella. Some surgeons tend
to commence treatment after puberty and to address the problems
separately. Maxillary advancement via the LeFort II osteotomy does
not adequately correct the foreshortened nose.11,27 Nasal soft tissue
lengthening procedures often result in conservative nasal elongation
and undesirable scarring,1,17Y21 and local columella-lengthening
aps result in visible scarring.16,27 As the 3 main deformities are interrelated, it seems reasonable to address them with a staged protocol. After our experience, treatment should ideally commence in
childhood with the key introduction of tissue expansion of the nasal
envelope in early childhood.15,26
The commencement of treatment in childhood also confers psychologic benet through the alleviation of the stigmata
of the syndrome and avoids teasing by peers. The timing of the
initial surgery is usually dictated by the patient. Nearly all of our
prepubertal patients requested surgery between the ages of 7 and
9 years.
Our introduction of alloplastic implants for tissue expansion in Binder syndrome has been proven successful and well
tolerated. The use of alloplastic material in childhood limits
donor site morbidity and preserves the costal graft for nal
reconstruction. In addition, costal osseochondral grafts in childhood are more difcult because the donor site is not fully developed and there is a higher risk of resorption or, occasionally,
increased growth.
The results of early tissue expansion in our prepubertal group
conrm the advantages of commencement of tissue expansion in
childhood. Early tissue expansion of the nasal envelope lining
capitalizes on the tissue elasticity of youth, allowing for expansion
of the nasal envelope and lining, and nal accommodation of a larger
denitive costochondral graft at the completion of growth.15,16,32
The expanded nasal envelope may limit graft resorption. The
minimal graft resorption in this group contrasts favorably with
reports of signicant graft resorption in the absence of tissue
expansion.16 The nding of slightly more graft resorption in our
patients presenting in the postpubertal group without tissue
expansion is consistent with previous reports.16 There may also be
a role for tissue expansion in this postpubertal group with silicone
implant placement before denitive costochondral grafting to
attempt to limit graft resorption.
In the design of the silicone implant, it is important to create a
sizable columellar strut that extends from the pyriform margin to the

FIGURE 9. Patient 4. Upper row, the patient with features

of Binder syndrome. Second row, the child after the
costochondral graft in childhood. Third row, showing
features of overgrowth of the costochondral graft. Fourth
row, after simultaneous orthognathic surgery and repeated
costochondral graft to the nose.

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FIGURE 10. Patient 5. Upper row, the patient with mild maxillary deciency, class 3 malocclusion. Lower row, 5-year
postoperative photos after the costochondral nasal reconstruction and the maxillary advancement.

1 overgrew. Similar experience has been reported by other authors.28

There is a long history of use of silicone for nasal augmentation in
the Asian population.33Y40 Silicone implants have been reported to
be well tolerated in up to 84% of patients.34,35,40 The key to avoiding
extrusion and infection is aseptic technique,33,34 minimal tension,33,40 and adequate soft tissue coverage especially in the region
of the nasal tip where the implant should ideally be placed deep to
the local lateral cartilages.38
For denitive reconstruction, our strong preference is for
cantilevered costochondral grafts because of the ease of harvesting,
the ability to obtain the correct 1-piece full-thickness shape, and the
maintenance of a periosteal covering. In addition, the distal chondral
segment is easy to shape and connect with the lower lateral
cartilages. The rigid peg and hole xations seldom require screws or
pins and, along with retaining the periosteal cover, probably account
for the lack of resorption. The 9th or 10th rib are preferred because
of their straighter shape.
Sixty percent of the patients in our series were noted to have
malocclusion. Orthognathic surgery was an important component of
management to address the fundamental maxillary deciency in
maxillonasal dysplasia. The denitive rhinoplasty was performed
concurrently with a small number of maxillary advancements
and was possible because the cantilevered graft does not rely on
maxillary platform support.

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