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Craniofacial microsomia (CFM) is the second most common craniofacial anomaly treated surgically in craniofacial centers worldwide. This craniofacial condition is variably associated with anomalies of the ears, jaws, orbits, soft tissue of face and function of the facial nerve. It can also be associated with extra- cranial deformities like cervical and rib anomalies. Largely, the etiology of CFM is unknown, but prenatal exposures of some drugs and genetic abnormalities may be associated with the condition. Diagnosis and treatment of CFM is challenging due to a wide spectrum of deformities (both osseous and soft tissue). Depending upon the severity of the deformity a wide variety of surgical treatment plans exist. After treating forty cases of CFM, we experienced that, though the treatment of severe form of CFM is diffi cult, but a coordinated multi-specialty team approach, especially of Reconstructive plastic surgery, orthognathic surgery, ear, nose and throat specialists leads to a successful and rewarding outcome.
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January-June 2015 / Vol 2 / Issue 1
Journal of Cleft Lip Palate and Craniofacial Anomalies
Craniofacial microsomia
R. K. Mishra, Surajit Bhattachrya1
terms describing this malformation complex can be
found in the literature including first and second
branchial arch syndrome,[1] hemifacial microsomia,[4]
dysostosis otomandibularis,[2] Congenital oto-cephalic
syndrome,[2] auriculo-branciogenic dysplasia,[2] oto-
cranio cephalic syndrome,[2] and Goldenhar-Gorlin
syndrome.[5] Goldenhar syndrome is a variant of CFM
and has cervical (neck) and rib anomalies and epibulbar
dermoids [Figure 2].[6]
Paramount to the understanding of CFM is an
appreciation of head and neck embryology.
Development of the branchial arches, facial growth,
and differential craniofacial growth contribute to
the formation of the facial structures. All structures
derived from the first and second branchial arches —
bones, muscles, cranial nerves, organs, and glands
can be involved.[7,8] The collected group of deformities
that make up this syndrome may vary greatly in extent
and degree covering a wide spectrum ranging from
mild underdevelopment of the lower jaw to severe
deformity of the skull and face [Figure 3a and b].
Characteristically this deformity, as the name implies
(hemifacial), involves one side of the face, however
involvement of both sides to some degree can be
observed in as high as 15% of all cases [Figure 4].[2,7]
The most obvious deformity involves the lower
jaw and ear, but soft tissue deficiency, maxillary
hypoplasia and even orbit and skull anomalies may
also be present.[3] Lack of development of the external
ear is a common feature with the severity of the ear
deformity proportional to the jaw deformity.[9] The
parotid can be malformed or missing, auricular and
facial nerve abnormalities have been reported in up
to 50% of affected children. The presence of hearing
loss of varying degrees necessitates otolaryngologic
evaluation.[9] Isolated microtia is now considered
a microform of CFM and should prompt thorough
evaluation before undertaking cosmetic repairs.[3]
In addition, when there is a significant facial bone
hypoplasia, patients can develop airway obstruction
Departments of Plastic and Reconstructive Surgery, Sushrut Institute of
Plastic Surgery, 1Sahara Hospital, Lucknow, Uttar Pradesh, India
Address for correspondence:
Dr. R. K. Mishra,
Sushrut Institute of Plastic Surgery, 29, Shahmeena Road,
Lucknow - 226 003, Uttar Pradesh, India.
E-mail: drrkmishra@hotmail.com
ABSTRACT
Craniofacial microsomia (CFM) is the second most
common craniofacial anomaly treated surgically in
craniofacial centers worldwide. This craniofacial
condition is variably associated with anomalies of the
ears, jaws, orbits, soft tissue of face and function of
the facial nerve. It can also be associated with extra-
cranial deformities like cervical and rib anomalies.
Largely, the etiology of CFM is unknown, but prenatal
exposures of some drugs and genetic abnormalities
may be associated with the condition. Diagnosis
and treatment of CFM is challenging due to a wide
spectrum of deformities (both osseous and soft tissue).
Depending upon the severity of the deformity a wide
variety of surgical treatment plans exist. After treating
forty cases of CFM, we experienced that, though the
treatment of severe form of CFM is dif cult, but a
coordinated multi-specialty team approach, especially
of Reconstructive plastic surgery, orthognathic
surgery, ear, nose and throat specialists leads to a
successful and rewarding outcome.
Key words: Craniofacial microsomia, dysostosis
otomandibularis, rst and second branchial arch
syndrome, goldenhar syndrome, hemifacial
microsomia
Review Article
INTRODUCTION
Craniofacial microsomia (CFM) is a spectrum of
morphogenetic abnormalities involving structures
derived from the first and second branchial arches[1,2]
[Figure 1]. It is the second most common facial
birth defect after cleft lip and palate.[3,4] Fourteen
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Mishra and Bhattachrya: Craniofacial microsomia
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called sleep apnea. Untreated, this can lead to poor
weight gain, small stature, cerebral hypoxia, and
even death.
Most patients with CFM have some degree of
mandibular hypoplasia that is seen clinically as a
deviated chin [Figure 3a], occlusal cant [Figure 5]
and an asymmetry in the position of the corners of
the mouth[9] [Figure 1]. Patients with mandibular
growth disturbances can present at any age. The
challenge in treating many of these patients lies in the
variability of age and associated pathology of other
facial structures such as the maxilla, the muscles of
mastication, and the zygoma. All these elements have
a well-orchestrated interplay with one another and,
therefore, the type of treatment chosen to address the
individual deformity must be specific to the patient’s
needs.
CLASSIFICATION
David et al.[10] propounded the most comprehensive
classification from the Australian Cranio-Facial Unit
(ACFU) of Adelaide, Australia. Three categories were
sought in each patient — skeletal, auricular, and soft
tissue.
The five skeletal categories are [Figure 6a-e]:
S1 — Small mandible of normal shape
S2 — Condyle, ramus, and the sigmoid notch
identifiable but grossly distorted. Mandible
strikingly different in size and shape from normal
S3 — Mandible severely malformed, ranging from
poorly identifiable ramal component to complete
agenesis of ramus
S4 — S3 mandible + orbital involvement — gross
posterior recession of lateral and posterior orbital
rims
S5 — S4 defect + orbital dystopia, hypoplasia and
asymmetrical neuroranium and a flat temporal
fossa.
Figure 1: A case of right sided craniofacial microsomia. The visible
deformities are: Microtia and cleft lip/palate, occlusal cant, elevated angle
of mouth and deviated chin
Figure 4: Bilateral craniofacial microsomia
Figure 2: Epibulbar dermoid in a case of craniofacial microsomia
Figure 3: Varying degree of deformities in craniofacial microsomia may
vary from mildly hypoplastic mandible and microtia (a), to very severe
deformity with cleft lip and palate, absent ramus and condyle of mandible,
orbital dystopia (b)
b
a
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The four auricular categories are [Figure 7a-c]:
A0 — Normal
A1 — Small malformed auricle, retaining all
features
A2 — Rudimentary auricle, with hook at cranial
end corresponding to the helix
A3 — Malformed lobule + absent rest of pinna.
And the three soft tissue categories are [Figure 8a-c]:
T1 — Minimal contour defect with no cranial nerve
involvement
T2 — Moderate defect
T3 — Major defect, obvious facial scoliosis, severe
hypoplasia of cranial nerves, parotid, muscles of
mastication, eye involvement + facial clefts.
The classification system of mandibular hypoplasia
most frequently used is that of Pruzansky:[11] Grade-1
mandibles are normal in configuration, but reduced
in size [Figure 3a]. Grade-2 mandibles demonstrate
hypoplasia plus mal-development of the associated
condyle and coronoid processes. Kaban et al.[12] later
sub-classified the latter group as either 2a or 2b.
Grade-2a mandibles have hypoplastic and malformed
condyles, but the condylar head/glenoid fossa spatial
relationship is spatially maintained in the sagittal
dimension similar to that of the contralateral side. In
these patients, the misshapen condyle is functional
and can be used in the mandibular reconstruction.
Grade-2b mandibles have a severely hypoplastic and
malformed condyle, which is displaced outside of the
sagittal plane of the contralateral temporomandibular
joint (TMJ). These patients frequently have restricted
TMJ function. Grade-3 mandibles are severely
hypoplastic and lack a condyle, coronoid process
and glenoid fossa [Figure 3b]. This classification
system can be applied to patients with unilateral or
bilateral mandibular hypoplasia. Any treatment plan
constructed for these patients must factor in the age
of the patient and the degree of skeletal hypoplasia in
order to optimize long-term results [Table 1].
Meurman[13] proposed the first classification scheme for
CFM based on auricular findings. Murray et al.[14] has
proposed a new scheme based on skeletal abnormalities,
which has proven useful in planning surgical
management in this diverse group of patients. Type
one patients have small mandibles with normal shape,
a normal glenoid fossa and a short mandibular ramus.
Type two findings include anteriorly and medially
displaced TMJs, a short and abnormally shaped ramus,
and an abnormally contoured TMJ cavity. Complete
absence of the mandibular ramus and glenoid fossa, no
Figure 5: Clinical method to examine occlusal cant
Figure 6: Various skeletal (S) categories in David et al.[10] classi cation. S1
- Small mandible of normal shape (a). S2 - Condyle, ramus, and sigmoid
notch identi able but grossly distorted. Mandible strikingly different in
size and shape from normal (b). S3 - Mandible severely malformed, ranging
from poorly identi able ramal component to complete agenesis of ramus
(c). S4 - S3 mandible + orbital involvement — gross posterior recession
of lateral and posterior orbital rims (d). S5 - S4 defect + orbital dystopia,
hypoplasia and asymmetrical neurocranium and a at temporal fossa (e)
a
c
e
b
d
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TMJ and the body of the mandible ending at the molar
region classifies type three patients.
The other widely used system is the OMENS (O for
orbital distortion; M for mandibular hypoplasia; E
for ear anomaly; N for nerve involvement; and S for
soft tissue deficiency) classification scheme[15] later
modified to the OMENS + to include extracranial
manifestations.[16] The acronym stands for orbit,
mandible, ear, nerve, and soft tissue; each feature is
assigned a severity score. More recently, a pictorial
representation of the OMENS system was introduced[17]
and later modified[18] to facilitate ease of use.
INCIDENCE
Craniofacial microsomia has an incidence reported
between 1/3500 and 1/26,550 live births. The male to
female and right to left sided ratios are both 3:2. Bilateral
involvement occurs in roughly 10% of cases. The majority
of cases are sporadic with no definite inheritance being
proven in the literature. Recurrence risk is consistently
reported at 2-3% for subsequent pregnancies.
AETIOPATHOLOGY
In a paper published, Poswillo[8] attributed the
development of facial deformities consistent with CFM
to disruption of the stapedial artery. The stapedial artery
functions as a stopgap vascular channel during days 33-
45 of embryologic development. Poswillo fed pregnant
rats triazene and pregnant monkeys thalidomide and
showed the consistent mal-development of first and
second branchial arch structures. Robinson[19] supported
Poswillo’s theory by demonstrating carotid flow
abnormalities in two and defects-related to vascular
disruption in a third child with CFM.
PREOPERATIVE EVALUATION
The evaluation of CFM includes a thorough history and
physical examination, photographic and cephalometric
analysis, and three-dimensional computed tomographic
study. Family history of consanguinity, intrauterine
exposure to infection and toxins, and problems with
delivery should be explored. Physical exam should focus on
facial asymmetry as well as on isolated findings consistent
with this syndrome. Photographs and cephalometry allow
for monitoring of facial symmetry over time and aid in
planning surgical approaches to individual patients.
Three-dimensional computed tomography provides
accurate reconstruction of patients’ craniofacial skeletons
and alleviates the need for constructing physical models.
This allows for faster and more accurate surgical planning.
Figure 7: Various Auricular categories in David et al.[10] classi cation.
A-1 category (a), A-2 category (b) and A-3 category (c)
a b
c
Figure 8: Various soft tissue categories in David
et al
.[10] classi cation.
T1 - Minimal contour defect with no cranial nerve involvement (a), T2 -
Moderate defect (b), T3 - Major defect, obvious facial scoliosis, severe
hypoplasia of cranial nerves, parotid, muscles of mastication, eye
involvement + facial clefts (c)
ab
c
Table 1: Surgical treatment plan of craniofacial
microsomia based on David et al. classi cation system
T3 Osteotomies/Distraction
and
Micro-vascular Soft
Tissue Transfer
Micro-vascular
Composite Tissue
Transfer
Trans-cranial
Orbital shift
+
Free Tissue
Transfer
T2
T1 Genioplasty Osteotomies
and
Bone Grafting
S1 S2 S3 S4 S5
S1: Small mandible of normal shape, S2: Condyle, ramus, and sigmoid notch
identifiable but grossly distorted. Mandible strikingly different in size and shape from
normal, S3: Mandible severely malformed, ranging from poorly identifiable ramal
component to complete agenesis of ramus, S4: S3 mandible + orbital involvement —
gross posterior recession of lateral and posterior orbital rims, S5: S4 defect + orbital
dystopia, hypoplasia and asymmetrical neuroranium and a flat temporal fossa, T1:
Minimal contour defect with no cranial nerve involvement, T2: Moderate defect, T3:
Major defect, obvious facial scoliosis, severe hypoplasia of cranial nerves, parotid,
muscles of mastication, eye involvement + facial clefts
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TREATMENT
Treatment of CFM is individualized. Principles basic
to all cases include treating bony tissue deficits first,
followed by soft tissue augmentation. The mandible
is addressed initially since correction of mandibular
malformations often stimulates maxillary growth.
Maxillary growth is further enhanced with the use of
maxillary activators. Costochondral grafts[20,21] must be
used in TMJ reconstructions. Soft tissue deficits are
corrected with local and microvascular free flaps.[22] Facial
nerve defects usually are permanent and can be managed
accordingly by tendon or muscle transfer.[14] Hearing must
be assessed early to allow for hearing augmentation.
Reconstruction of middle ear structures is often delayed
until craniofacial reconstruction is complete.[9]
The UCLA Craniofacial Clinic Protocol and the ACFU
Protocol[10] are aimed at maximizing results and
minimizing the number of procedures. The timing and
types of procedures may vary depending on the severity
of the deformity and the individual patient. In general,
corrections include many of the following:
1. Preauricular skin tags (age under 1 year): Excision;
2. Macrostomia or wide mouth (age under 1 year):
Commisuroplasty;
3. Mandibular hypoplasia (5-8 years of age): Internal
distraction osteogenesis is used to lengthen
the lower jaw (for severe cases with absence of
mandibular condyle and ramus, a rib graft may be
necessary)[23,24]
4. External ear deformity or absence (6-8 years of
age): Staged ear reconstruction [Figure 9a-d]
with a rib graft framework, elevation, lobule (ear
lobe) and tragus (front of ear) reconstruction are
performed[25-27]
5. Orbital dystopia (asymmetric eyes) (6-11 years of
age): Although rarely required, repositioning of the
orbit and/or advancement of the forehead and brow
(fronto-orbital advancement) may be performed.
6. Jaw asymmetry (15-18 years or age of skeletal
maturity): Preoperative orthodontics, followed by
jaw (orthognathic) surgery with Le Fort I (upper
jaw) and mandibular sagittal-split (lower jaw)
osteotomies are often necessary.[28]
7. Soft tissue asymmetry (after jaw surgery): Final
facial contouring with autogenous fat grafting,
dermal fat grafts or even a fascial-fat free flap from
the upper back are often necessary after other
corrections.
Mandibular distraction
Distraction has the advantage over other techniques in
that it requires minimal operative time, carries little
risk, minimizes hospitalization time, obviates the need
for blood transfusion, bone graft and intermaxillary
fixation, and has minimal relapse rates. Patients who
require only unidirectional lengthening and have
adequate mandibular bone stock are ideal candidates
for intraoral distraction [Figure 10a]. Patients with
severe mandibular deficiencies and require distraction
in multiple dimensions are best treated with a multi-
planer extraoral device. In addition, patients who
have previous external scars from other procedures
are treated with an extraoral device. Since multi-
planer extra oral devices are costlier and not easily
available, double osteotomy along with two uni-planer
simple distractors can be used simultaneously with a
central common pin to gain distraction in two planes
[Figure 10b and c]. With an extraoral approach, care
is taken not to damage soft tissue that may be needed
Figure 9: Staged ear reconstruction of microtia (a) using costo-chondral
graft framework (b). Final result after lobule transposition and elevation
(c and d)
a
c
b
dFigure 10: Variety of intraoral mandibular distractor devices available in the
market (a). Extraoral bi-planer mandibular distraction achieved by using
two uni-planer distractors to distract ramus in vertical plane and body in
horizontal plane. Frontal (b) and lateral (c) view
a
b c
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for future surgeries, such as external ear remnants or
microvascular soft tissue augmentation.
The site and direction of the osteotomy is based solely
on the bony pathology as well as the position of tooth
follicles. The vector of the distraction is also a variable.
Distraction can occur in the vertical, horizontal, or
oblique vectors (based on the relationship of the vector
to the long axis of the mandibular body).[29] A vertical
vector of distraction is preferred for lengthening
a deficient ramus in a vertical dimension or for
transporting the condyle up into the glenoid fossa. The
horizontal vector along the long axis of the mandible is
chosen in order to lengthen the mandible in a purely
horizontal plane, as in bilateral micrognathias whose
deficiency is predominately in the mandibular body. If
an oblique vector (a direction between the vertical and
horizontal vectors) is chosen, the osteotomy is placed
anterior to the coronoid in order to prevent impingement
of the coronoid on the zygomaticomaxillary buttress
during distraction. An oblique distraction vector
not only lengthens, but also vertically elongates the
mandible. Before converting the corticotomy into an
osteotomy, the screws or pins are placed.
After a delay of 5-7 days (termed the latency period),
distraction commences at a rate of 0.5 mm twice daily
(termed the activation phase). This rate is continued
until the mandibular length is overcorrected by several
millimeters. During distraction, the vertical or oblique
vector will typically become more horizontal, due to the
counterclockwise pull of the muscles of mastication. At
this time orthodontic intermaxillary elastics may be used
to mold the regenerating bone and optimize the occlusion
(termed molding the regenerate). The device is left in
place to serve as an external fixator for 8 or more weeks,
until there is radiographic evidence of mineralization.
This stage is known as the consolidation phase.
In patients with unilateral CFM undergoing distraction,
it is important that a dental impression be taken and
a bite block placed in the surgically created posterior
open bite when the device is removed. This will allow
the orthodontist to level the maxillary occlusal plane
by allowing for eruption of the ipsilateral maxillary
dento-alveolar complex. Distraction will also affect the
entire facial milieu: The soft tissue envelope bulk will
increase due to a combination of soft tissue expansion
and muscle hypertrophy and leveling of the oral
commissure are usually noted.
Age is also a factor in developing a treatment plan.
Under 2 years of age, mandibular distraction is not
usually performed because it is difficult to identify
tooth buds at this age, and therefore permanent dental
injury is a likely occurrence. Second, distraction at this
age can be a daunting experience for the patient and
the parents. The exception to this would be when early
mandibular distraction is used to prevent tracheotomy
in a newborn with micrognathia that is causing severe
airway obstruction.
Huisinga-Fischer et al.[30] observed for longitudinal
results of mandibular distraction and found that in
around 50% of the cases, there seems to be a relapse
occurring 1-year after distraction osteogenesis, and this
relapse has a progressive character.
Meazzini et al.,[31] Suh et al.,[32] and Sakamoto et al.[33]
reported improvement of symmetry, occlusal cant
and mandibular length and height resulting into
satisfied patients after distraction osteogenesis of
mandible. However, they also mention that distraction
osteogenesis starting after 5-years of age did not
influence the maxillary skeletal base. They all reported
that mandibular vertical changes showed a gradual
return of the asymmetry with growth in all patients
probably due to genetically determined craniofacial
growth patterns.
Final orthognathic surgery and free composite
tissue transfer
Children with S3, S4, and S5 (absent ramus, condyle,
and/or glenoid fossa), are initially treated with an
autogenous costochondral rib graft reconstruction
at approximately 3-4 years of age (first stage). The
costochondral graft will increase mandibular length,
reconstruct the condyle, and form a pseudoarthrosis
with the glenoid fossa. In a second stage, at least
6 months after removal of the fixation, distraction
of the rib graft can be performed.[34] At the time of
skeletal maturity however microvascular free tissue
transfer is offered to create absent parts of the mandible
[Figure 11a-c] as well as correction of orbital rim
deformities using addition of rib grafts.[35]
Tanna et al.[36] compared the effectiveness of free
tissue transfer and staged fat grafting in case of CFM.
He reported that the mean number of procedures was
less for the free tissue transfer cohort without any
significant complication. Rahpeyma et al.[37] suggested
preoperative evaluation of facial artery and anterior
belly of the digastric muscle are essential steps for
success in free tissue transfer. Furthermore, bone suture
technique, especially hitching the soft tissue with fixed
bony points, helps achieve more predictable results
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and reduces the need for postoperative bulky dressing.
Based on the composition of the flap, it can be used as
a myocutaneous, faciocutaneous, and osteomuscular
flap. Free flaps are difficult procedures and can
have considerable complications. The complications
of free tissue transfer may be immediate (vascular
compromise — venous/arterial, hematoma, bleeding),
delayed (facial asymmetry, malocclusion, implant
failure and malunion, donor site morbidities) and
very late (sagging of the flap, color mismatch, altered
sensation, bulky flap during weight gain periods).
During free tissue transfer, avoid muscle (as de-
neurotized muscle atrophies to a thin sheet) and prefer
dermis and fat with as small as skin pedicle possible,
preferably as pre-auricular skin strip. The side burn
should be taken into consideration.
When the glenoid fossa is absent, a new one is
constructed with rib grafts fixated to the zygomatic arch.
From age 6 to the teen years, during the period of mixed
dentition, orthodontic treatment is needed to promote
growth of the affected dentoalveolus and to aid in the
proper eruption of the permanent teeth. Indications for
surgery in the teen years include:
1. Residual postsurgical skeletal deficiency due to
surgical relapse or abnormal growth,
2. Unsatisfactory bone contour,
3. Malocclusion, or
4. Absence of previous treatment.
Any appropriately chosen maxillofacial surgical
procedure could be performed during this time ranging
from sagittal split osteotomies, to bone grafting, to
distraction. In patients with minimal mandibular
deformities, classic orthognathic procedures are
indicated. Mandibular distraction should be considered
in patients with moderate to severe skeletal deficiency,
or bilateral disease, in whom pressure from the
soft tissues would significantly increase the risk of
postoperative graft resorption or skeletal relapse.
Restricted mandibular growth is frequently associated
with abnormal maxillary development. The ipsilateral
maxilla and dento-alveolar processes are often
deficient in the vertical dimension. In mild cases
this can be treated with a bite block and orthodontic
therapy as described above; however, in more severe
circumstances a maxillary (Le Fort I) leveling procedure
may be considered. Traditionally, this has involved a
Le Fort I osteotomy followed by ipsilateral lengthening
of the mandible with bone grafts and a contralateral
impaction. The deficient maxilla can be distracted in
conjunction with the mandible. In this technique, a Le
Fort I corticotomy is made at the time of the mandibular
osteotomy and placement of the distraction device. The
upper and lower jaws are wired into intermaxillary
fixation. After a 5-day latency period, distraction is
commenced at the rate of 1 mm/day. At the conclusion
of maxillary/mandibular distraction, the device is left
in place for 8 weeks to allow for bone consolidation.
Using this technique, we have had excellent soft tissue
and bony results with complete leveling of the dental
occlusion. Intermaxillary fixation is not employed
during the latency period; instead heavy guiding
elastics are placed at the time of distraction. The bands
are modified throughout the process to obtain optimal
dental alignment.
Ancillary procedures to achieve facial symmetry
Nonvascularized dermis fat graft and lipo-filling are
the simplest methods mentioned in literature for the
treatment of soft tissue deformity or asymmetry in
CFM.[36,37] Obviously, lipo-filling is much simpler but
with great resorption rates and the need for procedure
repetition. To achieve facial symmetry in unilateral
cases some ancillary procedure is usually required in
the form of sliding genioplasty and/or autologous fat
grafting. Autologous fat grafting is usually needed in
the cheek and mandibular margin area to correct minor
discrepancies. Tanna et al.[38] reported that serial fat
grafting provided a useful alternative to microvascular
free tissue transfer after skeletal reconstruction.
Although the mean number of procedures was less
for the microvascular free flap group versus the fat
grafting group (2.2 vs. 4.3), the combined surgical time
was greater for the microvascular free flap group. The
Figure 11: Reconstruction of right mandible (S3 T3) in a severe case of
craniofacial microsomia using microvascular free bula ap in a teenager.
Preoperative clinical picture and three-dimensional scan showing severe
deformity of mandible with absent ramus (a). Intraoperative picture (b),
and postoperative picture showing good facial symmetry and survival of
reconstructed mandible (c)
a
c
b
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Mishra and Bhattachrya: Craniofacial microsomia
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volume of soft tissue implanted and symmetry rating
was 20-25% higher in microvascular group than fat
grafting group, but the complication rate was also higher
for the microvascular free flap group than that for the fat
grafting group. No statistically significant difference in
patient or physician satisfaction was noted. We usually
harvest fat from lower abdomen or medial thigh, allow
it to sediment for 30-40 min. The fat is mixed with
autologous platelet rich plasma activated with calcium
just before injection into the required and marked area
to achieve better take of fat and good results [Figure 12].
SUMMARY
Craniofacial microsomia is a syndrome with diverse
presentation. The majority of cases are sporadic with
stapedial artery disruption being considered the most
likely etiology. The ACFU classification based on
skeletal, external ear, and soft tissue abnormalities is
most comprehensive and treatment specific. Treatment
schemes are adapted to the specific dysmorphology
of individual patients. Distraction histiogenesis,
orthognathic surgeries, composite free tissue transfer
along with good preoperative and postoperative
orthodontics and timely reconstruction of external
ear and other facial clefts and soft tissue blemishes
completes the treatment.
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1. Converse JM, Coccaro PJ, Becker M, Wood-Smith D. On hemifacial
microsomia. The rst and second branchial arch syndrome. Plast
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Figure 12: A case of left sided craniofacial microsomia with facial
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rich plasma over the left cheek, zygomatic area and mandibular margin to
improve facial asymmetry
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Mishra and Bhattachrya: Craniofacial microsomia
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January-June 2015 / Vol 2 / Issue 1
Journal of Cleft Lip Palate and Craniofacial Anomalies
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Cite this article as: Mishra RK, Bhattachrya S. Craniofacial microsomia. J Cleft
Lip Palate Craniofac Anomal 2015;2:11-9.
Source of Support: Nil. Con ict of Interest: None declared.
[Downloaded free from http://www.jclpca.org on Friday, January 15, 2016, IP: 1.22.138.21]
... Craniofacial microsomia presents with a wide phenotypic spectrum of cranial and extracranial anomalies. [1,2] Because of the variable presentation, planning individualized reconstruction is based on the severity of the defect, availability of treatment options, and patient needs. [3] Mandibular and maxillary skeletal corrections with distractions, osteotomies, cartilage or bone grafting, and vascularized soft tissue transfers are all technically complex, expensive surgeries associated with high surgical risk and unaesthetic scars. ...
... Craniofacial microsomia is the second most common craniofacial anomaly in India. [2] There is virtually no "catch up" growth on the affected side and the facial deformity progressively worsens with age. [5] As the associated psychological problems also increase with the facial deformity, early intervention is necessary to correct the growth vectors. ...
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The purpose of this study was to analyze the prevalence, diagnosis, and management of velopharyngeal insufficiency (VPI) in patients with craniofacial microsomia (CFM).Craniofacial microsomia patients 13 years of age and above treated at 2 centers from 1997 to 2019 were reviewed retrospectively for demographics, prevalence of VPI, and management of VPI. Patients with isolated microtia were excluded. Comparisons were made between patients with and without VPI using chi-square and independent samples t tests.Among 68 patients with CFM (63.2% male, mean 20.7 years of age), VPI was diagnosed in 19 patients (27.9%) at an average age of 7.2 years old. Among the total cohort, 61 patients had isolated CFM, of which 12 (19.6%) were diagnosed with VPI. Of the patients with isolated CFM and VPI, 8 patients (66.7%) were recommended for nasoendoscopy, of which only 2 patients completed. Seven isolated CFM patients (58.3%) underwent speech therapy, whereas none received VPI surgery. In contrast, 7 patients were diagnosed with both CFM and cleft lip and/or palate (CL/P), all of whom had VPI and were recommended for nasoendoscopy, with 5 (71.4%) completing nasoendoscopy, 6 (85.7%) undergoing speech therapy, and 6 (85.7%) undergoing corrective VPI surgery. Overall, we demonstrated that VPI was present in 27.9% of all CFM patients. On subset analysis, VPI was diagnosed in 20% of patients with isolated CFM and 100% of patients with CFM and CL/P. In addition, despite clinical diagnosis of VPI, a sizeable proportion of isolated CFM patients did not undergo therapy or surgical interventions.
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The goals of treatment for hemifacial microsomia include horizontalization of occlusal plane and acquisition of facial symmetry. Although horizontalization of occlusal plane can be easily achieved, facial symmetry, particularly in relation to mandibular contour, can be difficult to attain. Soft tissue is generally reconstructed to correct facial asymmetry, and no studies have described correction of facial asymmetry through skeletal reconstruction. A 12-year-old girl presented with grade IIb right-sided hemifacial microsomia. She was treated using Nakajima's angle-variable internal distraction (NAVID) system for mandibular body distraction. Following treatment, appropriate facial symmetry was achieved, and the patient was extremely satisfied with the results. Thus, we successfully treated the present patient by our novel method involving distraction osteogenesis. This method was effective and useful for several reasons including; the changes were not accompanied by postoperative tissue absorption, donor sites were not involved, and the treatment outcome could be reevaluated by adjusting distraction while the patient's appearance was being remodeled.
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Soft tissue augmentation in hemifacial microsomia patients is a challenging procedure. Free microvascular flap transfer is considered usually as the most accepted choice. On the other hand, bone grafting, simultaneous with facial soft tissue augmentation using de-epithelialized orthograde submental flap, is a suggested procedure. Moreover, preoperative evaluation of facial artery and anterior belly of the digastric muscle are essential steps for success in such flaps. Furthermore, bone suture technique helps achieve more predictable results and reduces the need for postoperative bulky dressing.
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The purpose of this study was to evaluate the results of long-term follow-up in patients with relatively severe unilateral craniofacial microsomia after mandibular distraction. The sample consisted of 26 patients with an average age of 6.08 years at the time of distraction. All patients had nonsyndromic unilateral craniofacial microsomia (Pruzansky-Kaban types IIA, IIB, and III). Follow-up for all patients continued until the completion of growth. The 26 clinical records and posteroanterior cephalograms of the patients, taken before distraction (time 0) and approximately 1, 4, and 11 years (time 1, time 2, and time 3, respectively) after distraction, were used. Four items (i.e., supraorbital tilting angle and occlusal tilting angle to the horizontal reference line, and maxillary and mandibular tilting angles to the vertical reference line) were analyzed at each of the four time intervals. The overall mean distraction amount was 23 mm vertically and 21 mm horizontally. The average cephalometric follow-up was 131.7 months. In this study, the mandibular horizontal and vertical changes after distraction showed a gradual return of the asymmetry with growth in all patients. There were no statistically significant differences in any of the analyzed angles between the preoperative and final records, except for the supraorbital tilting angle. The longitudinal results achieved by distraction osteogenesis were unstable and generally led to relapse, although they were very good in the early postoperative period. Therefore, further efforts are suggested to find a solution that prevents relapse. Therapeutic, IV.
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This report is based on an analysis of 100 patients with the unilateral form of HFM, in which the patients presented no other known craniofacial or extracranial malformations. The analysis was based on a three level gradation of severity of the external ear and the mandible, resulting in nine different combinations. This analysis of selected components of the syndrome clearly illustrates the phenotypic heterogeneity within the sample. It also follows that treatment should be guided by the number and severity of the affected components.
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The authors investigated the use of serial autologous fat grafting to restore soft-tissue contour in craniofacial microsomia patients. Patients with moderate to severe craniofacial microsomia were divided into two groups. Microvascular free flap patients had reconstruction with inframammary extended circumflex scapular flaps at skeletal maturity (n = 10). Alternatively, patients had fat grafting during multiple staged operations for mandible and ear reconstruction (n = 21). Sex, age, severity of deformity [determined by OMENS (orbital deformity, mandibular hypoplasia, ear deformity, nerve involvement, and soft-tissue deficiency) classification], number of procedures, operative times, and augmentation volumes were recorded. A digital three-dimensional photogrammetry system was used to determine "final fat take" and symmetry (affected side versus unaffected side). Physician and patient satisfaction were elicited. Microvascular free flap and fat grafting groups had similar OMENS scores, 2.4 and 2.3, and similar mean prereconstruction symmetry scores, 74 percent and 75 percent, respectively. Although the mean number of procedures was less for the microvascular free flap group versus the fat grafting group (2.2 versus 4.3), the combined surgical time was greater for the microvascular free flap group. The complication rate for the microvascular free flap group was 12 percent and that for the fat grafting group was 5 percent. The mean microvascular free flap volume implanted was 131 cc, with a final measured volume of 106 cc. Mean fat grafting volume injected per case was 33 cc, with total fat injections of 146 cc and a final measured volume of 121 cc. There was a mean fat loss of 25 cc and 83 percent fat take. Symmetry score was 121 percent for the microvascular free flap group and 99 percent for the fat grafting group. No statistically significant difference in patient or physician satisfaction was noted. Serial fat grafting provided a useful alternative to microvascular free tissue transfer after skeletal reconstruction.
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Craniofacial microsomia is one of the most common conditions treated by craniofacial teams. However, research regarding the cause of this condition or the surgical outcomes of treatment is scant. This is attributable to the lack of diagnostic criteria and the wide phenotypic spectrum. Standardized description of the craniofacial malformations associated with craniofacial microsomia is a necessary first step for multicenter, interdisciplinary research into this complex condition. The authors used the previously published pictorial Orbit, Mandible, Ear, Nerve, and Soft tissue-Plus classification scheme to assign a phenotypic severity score to patients with craniofacial microsomia treated at the Craniofacial Center at Seattle Children's Hospital. The authors modified the tool based on feedback from multidisciplinary focus groups. The authors also developed a standardized photographic protocol to facilitate assessment of patients using two-dimensional images. Feedback from focus groups was synthesized to create a phenotypic assessment tool for craniofacial microsomia based on the pictorial Orbit, Mandible, Ear, Nerve, and Soft tissue-Plus classification system. This tool allows for more comprehensive description of the phenotype of craniofacial microsomia and is found to be effective for clinical use within a multidisciplinary craniofacial team. In addition, the photographic protocol for patients with craniofacial microsomia allows for classification from a two-dimensional photographic database, thereby facilitating research using archived photographs. The phenotypic assessment tool for craniofacial microsomia protocol provides a simple and standardized method for practitioners and researchers to classify patients with craniofacial microsomia. We anticipate that this tool can be used in multicenter investigational studies to evaluate the cause of this condition, its natural history, and comparative effectiveness research.
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Single-vector distraction devices have been criticized for creating a malocclusion in an attempt to correct a three-dimensional mandibular deficiency, resulting in the evolution of a multiplanar device. Although there are indications for the use of a multiplanar device, a vast number of patients with mandibular hypoplasia can be effectively treated with a single-vector device, producing a normal occlusion and an aesthetic result while minimizing facial scarring and simplifying postoperative care. The purpose of this review was to describe surgical techniques whereby a single-vector device is effectively used in treating a multivector mandibular deficiency.A retrospective analysis of all patients who underwent mandibular distraction at the Children's Hospital of Philadelphia between 1996 and 2005 with a semiburied, uniplanar device was conducted. Charts, photographs, graphs, operative reports, computed tomography scans, and cephalometrograms were reviewed for those patients undergoing uniplanar mandibular distraction.Ten unilateral and 4 bilateral distractions were performed. In these 14 patients, causes included hemifacial microsomia, Treacher Collins syndrome, posttraumatic hypoplasia, and temporomandibular joint ankylosis with hypoplasia. The average device distraction was 29 mm (range, 18-34 mm). The average age at distraction was 8.4 years (range, 4-15 years). Surgical techniques for these patients will be described in detail. The single-vector, semiburied device can be effectively used to aesthetically correct a three-dimensional problem and to produce or maintain a class I occlusion while simplifying postoperative management and minimizing facial scarring.
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Although hemifacial microsomia is a relatively common craniofacial malformation, there is some debate regarding the ideal treatment of severe mandibular hypoplasia. Traditionally, patients with severe mandibular deficits have been treated with iliac or costochondral bone grafts followed by distraction osteogenesis, with mixed results. The authors present their experience with the use of the fibula osteocutaneous free flap for mandibular reconstruction in severe hemifacial microsomia patients. From 1999 to 2006, 10 patients aged 3 to 10 years (mean, 7.2 years) underwent 10 free flap reconstructions. Of the 10 patients, six were girls and four were boys. Data were collected retrospectively from the patients' records, photographs, and radiographs. The authors report the surgical technique used, complications, and long-term outcome. Nine of 10 flaps were successful, for a flap survival rate of 90 percent. Donor bone length was 5 to 10 cm, with a mean of 6.3 cm. Skin paddles ranged from 8 to 36 cm, with a mean size of 18.7 cm. Mean operation time was 412 minutes and mean follow-up was 45.4 months (range, 12 to 94 months). Two patients underwent successful distraction osteogenesis subsequent to their free flap mandible reconstruction. All patients demonstrated stable bony union of the free flap by physical and radiographic examination. One major complication (a failed free flap) and two minor complications were observed. The free flap is safe and effective, and should be considered as a first choice in mandibular reconstruction in severe cases of hemifacial microsomia where distraction osteogenesis is not possible.