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Craniofacial microsomia: Goals of treatment, staged reconstruction and long-term outcome

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Craniofacial microsomia (CFM) is a complex congenital condition and includes a spectrum of malformations primarily involving structures derived from the first and second branchial arches. to present a review of the clinical presentations, assessment, treatment and outcome of the patients with CFM who were treated by the authors in Srinagarind hospital, Khon Kaen University. Medical records were reviewed of patients with CFM, seen and managed by the authors at Srinagarind Hospital between 1993 and 2011; for an analysis of the clinical and radiologic assessments, treatments and outcomes. There were 23 patients (14 males and 9 females), 4 of whom had bilateral deformities, 19 unilateral deformities, and 1 syndromic CFM. The clinical presentation ranged from microtia to more severe deformities involving the mandible, zygoma and maxilla. Surgical treatments included: correction of macrostomia, distraction osteogenesis, staged ear reconstruction, orbit-maxillary-mandibular surgery, soft tissue reconstruction with dermis fat graft, vascularized free tissue transfer, cleft lip-cleft lip nose repair and palatoplasty. Most of the patients were satisfied with the medical treatment and many patients were still in the growing skeletal age and had staged reconstruction planned. The goals of treatment for CFM are to (a) manage respiratory insufficiency and feeding problems (b) maximize hearing and communication (c) improve facial symmetry and proper facial growth and (d) optimize dental occlusion. These patients require staged reconstruction and coordinated care by an experienced multidisciplinary craniofacial team in order to ensure a thorough, comprehensive assessment of the nature and extent of the problems and to provide better treatment planning and long-term outcome adapted to the respective anatomic and functional deformities of each patient, and the needs of their family and other involved stakeholders.
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S100 J Med Assoc Thai Vol. 94 Suppl. 6 2011
Craniofacial Microsomia: Goals of Treatment, Staged
Reconstruction and Long-Term Outcome
Bowornsilp Chowchuen MD, MBA*, Poonsak Pisek DDS**,
Prathana Chowchuen MD***, Sanguansak Thanaviratananich MD****
* Division of Plastic Surgery, Department of Surgery, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand
** Department of Orthodontics, Faculty of Dentistry, Khon Kaen University, Khon Kaen, Thailand
*** Department of Radiology, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand
**** Department of Otorhinolaryngology, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand
Background: Craniofacial microsomia (CFM) is a complex congenital condition and includes a spectrum of malformations
primarily involving structures derived from the first and second branchial arches.
Objective: to present a review of the clinical presentations, assessment, treatment and outcome of the patients with CFM who
were treated by the authors in Srinagarind hospital, Khon Kaen University.
Material and Method: Medical records were reviewed of patients with CFM, seen and managed by the authors at Srinagarind
Hospital between 1993 and 2011; for an analysis of the clinical and radiologic assessments, treatments and outcomes.
Results: There were 23 patients (14 males and 9 females), 4 of whom had bilateral deformities, 19 unilateral deformities,
and 1 syndromic CFM. The clinical presentation ranged from microtia to more severe deformities involving the mandible,
zygoma and maxilla. Surgical treatments included: correction of macrostomia, distraction osteogenesis, staged ear
reconstruction, orbit-maxillary-mandibular surgery, soft tissue reconstruction with dermis fat graft, vascularized free tissue
transfer, cleft lip-cleft lip nose repair and palatoplasty. Most of the patients were satisfied with the medical treatment and many
patients were still in the growing skeletal age and had staged reconstruction planned.
Conclusion: The goals of treatment for CFM are to (a) manage respiratory insufficiency and feeding problems (b) maximize
hearing and communication (c) improve facial symmetry and proper facial growth and (d) optimize dental occlusion. These
patients require staged reconstruction and coordinated care by an experienced multidisciplinary craniofacial team in order
to ensure a thorough, comprehensive assessment of the nature and extent of the problems and to provide better treatment
planning and long-term outcome adapted to the respective anatomic and functional deformities of each patient, and the needs
of their family and other involved stakeholders.
Keywords: Craniofacial microsomia, Goals of treatment, Staged reconstruction, Long-term outcome
Correspondence to:
Chowchuen B, Division of Plastic Surgery, Department of
Surgery, Faculty of Medicine, Khon Kaen University, Khon
Kaen, 40002, Thailand.
Phone: 043-363-123
E-mail: bowcho@kku.ac.th, bchowchuen@gmail.com
J Med Assoc Thai 2011; 94 (Suppl. 6): S100-S108
Full text. e-Journal: http://www.jmat.mat.or.th
Craniofacial microsomia (CFM) is a complex
congenital condition and includes a spectrum of
malformations primarily involving structures derived
from the first and second branchial arches. The
phenotype of CFM is highly variable in severity,
ranging from almost unnoticeable to severely
disfiguring. Bilateral involvement, usually asymmetrical,
may be found in 15-30% of patients. Associated
craniofacial anomalies may include macrostomia,
micrognathia, cleft lip with or without cleft palate, pre-
auricular skin tags or pits, ear deformities (varying from
microtia, anotia to aural atresia), hearing loss and
mandibular deformity. Non-craniofacial anomalies may
include the cardiac system, the vertebral or central
nervous system and/or the limbs(1,2).
A diagnosis of CFM is based on clinical
findings and the proposed minimal diagnostic criteria
is asymmetric hypoplasia of the facial structures with
pre-auricular tags or microtia(3,4). Coursley(5) discussed
research findings which affect all aspects of CFM, with
an emphasis on facial anomalies which are important
for diagnosis, classification and treatment. Heike and
Hing(2) used the term CFM to include hemifacial
microsomia, first and second branchial arch syndrome,
otomandibular dysostosis, oculo-auriculo-vertebral
J Med Assoc Thai Vol. 94 Suppl. 6 2011 S101
spectrum, facio-auriculo-vertebral syndrome,
Goldenhar syndrome and lateral facial dysplasia.
The incidence of CFM is not known but it
represents the second most common group of birth
defects after cleft lip and palate(5,6). Kay et al advocated
that microtia should be considered a microform of
CFM(7). About 45% of patients have affected relatives,
and 5-10% have affected siblings(8,9).
The objectives of the study are to review the
clinical presentations, assessment, treatment and
outcome of the patients with CFM who were treated by
the authors in Srinagarind Hospital, Khon Kaen
University between 1993-2011. The results of the
analysis may be useful for setting appropriate
guidelines for diagnosis and treatment of these patients,
goals of treatment and long-term outcome.
Patients and Method
Setting Srinagarind Hospital is the only university
hospital and the main tertiary referral center for
northeastern Thailand. The geopolitical region has a
population of about 22 million and is the poorest region
and one which has numerous endemic and genetic
diseases.
Material and Method
Study design
The medical records of the patients with CFM
seen and managed by the authors in Srinagarind
Hospital between 1993 and 2011 were reviewed. The
diagnosis of CFM was accomplished using clinical
assessments and radiology reports. The details of
these, the treatment and outcomes were reviewed.
The protocol of this study was reviewed and
approved by the Ethics Committee of Khon Kaen
University, using the standards set out in the
Declaration of Helsinki. Additionally, written informed
consent was obtained from each patient or from the
parents (in which case assent was sought from the
pediatric patients).
Results Table 1 shows detail of the 23 patients, which
included 14 males (M) and 9 females (F). Four patients
had bilateral deformities and 19 unilateral deformities.
The clinical presentation ranged from microtia to more
severe deformities involving the mandible, zygoma and
maxilla. Syndromic CFM presented in only one patient.
Fig. 1 shows the geographic distribution of the 23
patients (using Geographic Information System-GIS).
Khon Kaen, Maha Sarakham and Sakon Nakhon were
the provinces in which the highest number of patients
was found.
The surgical treatments included (a)
correction of macrostomia in 3 patients (b) distraction
osteogenesis in 1 (c) staged ear reconstruction in 10
(d) orbit-maxillary-mandibular surgery in 6 (e) soft tissue
reconstruction with dermis fat graft in 1 (f) vascularized
free tissue transfer in 2 (g) cleft lip-cleft lip nose repair
in 1 and (h) palatoplasty in 1. Almost all of the patients
were satisfied with treatment and many of them were
still in the growing skeletal age (at the time of this
writing) and had plan for further staged reconstruction.
Patient reports
Patient No. 6
A female patient, born in 1973 in Sakon Nakhon
province, presented with hypoplasia of the maxilla and
mandible with left microtia. The surgical reconstruction
included LeFort I osteotomy with bilateral sagittal split,
sliding genioplasty and soft tissue reconstruction with
a parascapular free flap. At 27 years of age, she was
satisfied with her facial appearance and no longer came
in for follow-up.
Patient No. 10
A male patient, born in 1997 in Khon Kaen
province, presented with macrostomia, hypoplasia of
the right maxilla and eight mandibles, right microtia and
hand anomalies. Aural atresia with right conductive
hearing loss was noted. Correction of macrostomia,
hand deformities, and staged ear reconstruction were
performed. At the time of the last follow-up at the age
of 14, his parents and he were satisfied with his
appearance. Additional maxillary and mandibular
surgeries and soft tissue reconstruction were, However,
planned once he reached the age of facial skeletal
Fig. 1Geographic distribution of our 23 patients with
CFM, treated at Srinagarind Hospital between 1993
and 2011
S102 J Med Assoc Thai Vol. 94 Suppl. 6 2011
Patient Sex Birth Province Clinical Treatment (s)
No. year finding (s)
1 M 1987 Maha Sarakham Right microtia -Staged ear reconstruction
2 F 1989 Khon Kaen Left lateral facial cleft -Correction of macrostomia
3 M 1981 Khon Kaen Bilateral CFM -Left ear reconstruction
4 M 1978 Khon Kaen Left microtia -Left ear reconstruction,
prefabricated technique
5 M 1985 Si Sa Ket Bilateral CFM, -Mandibular onlay bone graft
asymmetry of
mandible
6 F 1973 Sakon Nakhon Right CFM -LeFort I osteotomy+
sagittal split-parascapular free flap
-dermis fat graft-sliding genioplasty
7 M 1985 Kalasin Right microtia -Staged ear reconstruction
8 F 1973 Sakon Nakhon Right CFM -LeFort I osteotomy + sagittal split
9 F 1995 Chaiyaphum Right CFM -Correction of macrostomia
10 M 1997 Khon Kaen Right CFM -Staged ear reconstruction
+ polydactyly
+ hand anomalies
11 M 1978 Nong Bua Right microtia -Staged ear reconstruction
Lam Phu
12 M 1994 Maha Sarakham Right microtia -Staged ear reconstruction
13 M 1998 Khon Kaen Left CFM -Distraction osteogenesis
14 M 1983 Maha Sarakham Left CFM and Left Cleft lip-nose revision
unilateral cleft lip + alveolar bone graft
-calvarial bone graft
+ genioplasty
-dermis fat graft to upper lip
15 M 1981 Khon Kaen Bilateral CFM -LeFort I osteotomy
+ bilateral sagittal split
-Parascapular flap free flap for soft
tissue augmentation
16 F 1989 Khon Kaen Left microtia -Staged ear reconstruction
17 M 1990 Roi Et Right microtia -Staged ear reconstruction
18 M 1985 Khon Kaen Left CFM -
+ left microtia
19 M 2005 Kalasin Bilateral CFM -
20 F 2000 Sakon Nakhon Left CFM -Correction of macrostomia
21 F 2004 Nong Khai Bilateral microtia, -Palatoplasty
bilateral hearing loss
and cleft palate
22 F 1996 Khon Kaen Syndromic right CFM -Orbital, zygomatic, and temporo-
with torticollis, mandibular joint reconstruction
scoliosis and right upper
extremity anomalies.
23 F 1991 Nakhon Left microtia -Staged ear reconstruction
Ratchasima
Table 1. Details of 23 patients with craniofacial microsomia (CFM) treated at Srinagarind Hospital, Khon Kaen University
between 1993 and 2011
J Med Assoc Thai Vol. 94 Suppl. 6 2011 S103
maturity (Fig. 4).
Patient No. 12
A male patient, born in Maha Sarakham
province in 1994, presented with right microtia. Staged
ear reconstruction was performed with a costochondral
cartilage graft (at the age of 6). He was lost to follow-
up but revisited the hospital in 2011, at the age of 17.
His parents and he were satisfied with the results,
however, further revision was planned.
Patient No. 13
A male patient born in Khon Kaen in 1998,
Fig. 2Patient No. 6 presented with hypoplasia of the
maxilla and mandible with left microtia. She was
treated by LeFort I osteotomy, bilateral sagittal
split, sliding genioplasty, and soft tissue recon-
struction with a parascapular free flap. Intra-op-
erative photos (taken in 1996) show the LeFort I
osteotomy with bilateral sagittal split and soft tis-
sue reconstruction with a parascapular free flap.
Follow-up photos, taken at 27 years of age, show
the satisfactory facial appearance
Fig. 3 Patient No. 10 presented with right macrostomia,
hypoplasia of maxilla and anomalies of the man-
dible, microtia and hand. He was treated to correct
the macrostomia and hand deformities, and was
slated for staged ear reconstruction. At the last
follow-up, at the age of 14, the better facial ap-
pearance is evident. Skull radiolographs reveal hy-
poplasia of the zygoma, orbit and maxilla
Fig. 4Patient No. 12 presented with right microtia, treated
by staged ear reconstruction with a costochondral
cartilage graft. The follow-up photos were taken
at the age of 17 amd these show satisfactory re-
sults
S104 J Med Assoc Thai Vol. 94 Suppl. 6 2011
presented with hypoplasia of the left zygoma, orbit
and mandible. Distraction osteogenesis of the left
mandible was performed when the patient was 2 years
old. At the time of last follow-up in 2011 (when the
patient was 13), he was satisfied with his facial
appearance.
Patient No. 20
A female patient, born in 2000 in Sakon Nakhon
province, presented with left macrostomia, hypoplasia
of the maxilla and mandible and left microtia. A CT scan
was performed which revealed the hypoplasia. Surgical
correction of the macrostomia was performed when the
child was 6 years of age (Fig. 6). She was lost to follow-
up after correction of the macrostomia.
Patient No. 22
A female patient, born in 1996 in Khon Kaen
province, presented with syndromic CFM with clinical
features of hypoplasia of the orbit, maxilla and mandible,
lagophthalmos, right microtia, bilateral hearing loss,
torticollis, scoliosis, and right upper extremity
anomalies. A CT scan revealed hypoplasia of the
zygoma, orbit, maxilla and mandible. Diagnosis of
syndromic CFM was made. When the patient was 11
years of age, orbital reconstruction was performed with
a calvarial bone graft and TMJ reconstruction with a
costochondral graft. At the time of follow-up in 2011 at
the age 15, the patient was feeling more satisfied with
her facial appearance. Facial analysis and planning for
further bony surgery have to wait until after the age of
facial skeletal maturity (Fig. 7-9).
Discussion
Craniofacial microsomia (CFM) is
characterized by facial asymmetry resulting from a
unilateral or bilateral congenital deficiency of the
affected skeletal and soft tissue structures, derived from
the first and second branchial arches. The etiology can
be divided into environmental, heritable, multifactorial
and unknown. It is considered the second most
common craniofacial anomaly after cleft lip and palate(10)
and has an estimated prevalence between 1:5,600 and
1:26,550 live births. The male to female ratio is 3:24.
The diagnosis of CFM can be based on
clinical examination and the common findings are:
macrostomia, cleft lip and/or palate, ankylosis,
asymmetric mandible, mid-face hypoplasia, maloc-
clusion, epibulbar dermoid, asymmetric shortening of
the palpebral fissure, microphthalmia or anophthalmia,
coloboma of the upper eye lid, vertical displacement of
the orbit and vertebral anomalies. The clinical findings
in individuals with craniofacial microsomia can be
associated with: syndromes, developmental anomalies,
Fig. 5Patient No. 13 presented with hypoplasia of the
left zygoma, orbit and mandible, and was treated
by distraction osteogenesis of left mandible (at the
age of 2 years). The follow-up photos were taken
at 5 and 13 years of age and these show a better
facial appearance
Fig. 6Patient No. 20 presented with left macrostomia
and hypoplasia of the maxilla and mandible (first
row). A 3-D CT reconstruction with SSD demon-
strates hypoplasia of left maxilla and mandible,
and asymmetrical and hypoplastic soft tissue of
the face and cheek (second row). Surgical correc-
tion of macrostomia was performed (third row)
J Med Assoc Thai Vol. 94 Suppl. 6 2011 S105
and/or sequences such as VATER, CHARGE, MUECS,
and OEIS; which led to the hypothesis that these
conditions may represent developmental abnormalities
resulting in anomalies that may be a part of a broad
spectrum(11).
Classification of patients with CFM is difficult
and the most effective and universally-accepted and
used in treatment plans are (a) the earlier classification
by Pruzanski(12) and (b) Kaban’s modification(13). Later,
two more comprehensive and modified classifications
were introduced, including (c) the Orbital Mandible
Ear Nerve Soft tissue (OMENS) classification(14), and
(d) the OMENS-Plus which included associated
extracranial manifestations(15). Birgfeld et al developed
(e) a modified pictorial OMENS-Plus and (f) a
standardized photographic protocol for a more
comprehensive and structured phenotypic assessment
tool for CFM(16).
A multidisciplinary craniofacial team
comprising a medical geneticist or physician who
specializes in craniofacial disorders, a plastic and
craniofacial surgeon, a pediatric dentist, an
orthodontist, an audiologist/otolaryngologist, a speech
pathologist, a social worker and a nurse coordinator
is needed to ensure a more thorough comprehensive
assessment of the problems and provide a better
treatment planning(17). The analysis and record of a
patient’s anatomic and functional deficiencies should
include facial asymmetry, severity of soft tissue and
skeletal deformities, ear findings and hearing
evaluation, ophthalmologic examination, as well as the
functional requirement, and the subsequent growth and
Fig. 8Intra-operative photos of patient no. 12 show or-
bital reconstruction with a calvarial bone graft and
TMJ reconstruction with a costochondral graft
Fig. 9Early post-operative photos and the most recent
(last) follow-up photos of patient No. 12, at the
age of 15, show the satisfactory facial appearance
Fig. 7Patient No. 12 had syndromic CFM and presented
with hypoplasia of the orbit, maxilla and man-
dible, lagophthalmos, right microtia, bilateral hear-
ing loss and right upper extremity anomalies (first
and second row). A plain film of right upper ex-
tremity and lateral view show radial aplasia, and
hypoplastic thumb with absent metcarpal bone
(third row, left). Film skull, AP view (third row,
right), coronal (fourth row, left) and axial (fourth
row, middle and right) CT images of skull and fa-
cial bones demonstrate hypoplasia of right max-
illa, mandible, zygomatic arch, maxillary antrum,
petrous and mastoid bones
S106 J Med Assoc Thai Vol. 94 Suppl. 6 2011
development(18). Additionally, other non-craniofacial
associated anomalies such as the neck, heart, spine,
limbs, and kidneys should be evaluated.
Though radiologic imaging is not necessary
for making a diagnosis of the patient with CFM, a 3-D
CT scan is helpful for refining the anatomy and
morphology for pre- and post-surgical treatment
planning of both bony and soft tissue deficiency and
outcome evaluation of these patients. Radiographic
evaluation of patients with CFM may reveal asymmetric
hypoplasia of the zygoma, maxilla and mandible.
Additionally, other imaging studies may be indicated,
including x-rays of the cervical spine, echocardiogram,
renal ultrasound examination, and CT scan of the
temporal bone in patients with significant hearing
impairment.
Airway and feeding are among the first
priorities during early management of these patients
and perinatal endotracheal intubation, tracheostomy,
mandibular distraction, and gastrostomy may be
indicated. The options for surgical techniques for the
correction of the skeletal and soft tissue deformities of
the patient with CFM include (a) correction of
macrostomia (patient No. 2, 9 and 20) (b) bone
reconstructions (patient No. 5, 6, 8, 14, 15 and 22) (c)
distraction osteogenesis (patient No. 13) (d) staged
ear reconstruction (patient No. 1, 3, 4, 7, 10, 11, 12, 16,
17 and 23) (e) dermis-fat grafts (patient No. 14) and (f)
microvascular free tissue transfers (patient No. 6, and
15). Macrostomia and pre-auricular skin
appendages are the most obvious deformities that may
need to be corrected at an early age, especially since
early repair of macrostomia can significantly improve
feeding. The surgical technique should include (a) the
correct position of the oral commissure for measuring
the distance from the midline to the oral commissure on
the non-cleft side (b) the repair of the underlying muscle
and (c) the use of a Z-plasty to reduce scarring (patient
No. 20, Fig. 7). For removal of a pre-auricular skin tag, it
is important that the cartilage remnants be completely
removed to avoid a depression deformity of the scar.
Assessment of hearing by an audiologist
should be done as early as possible- that is, in the first
two weeks of life- using a brain stem auditory response
test. Parents of patients with CFM who have hearing
impairment should receive guidance regarding
recommendations for hearing aids, prevention of further
hearing loss, and close monitoring for speech and
language development.
Prior to planning surgical correction of
microtia, it is recommended that a CT scan of the
temporal bone be done between 5 and 6 years of age to
assess the external ear canal, middle- and inner-ear
structures in order to identify candidates for whom
surgical correction would improve hearing.
For skeletal reconstruction, most patients with
CFM with mandibular hypoplasia can be managed
conservatively during the early years, except for the
patients who are functionally affected by the
mandibular hypoplasia asymmetry or absence of ramus,
condyle and temporomandibular joint fossa(17) who may
require a bone graft and/or mandibular distraction
osteogenesis to lengthen the mandible and/or create a
functional TMJ. If indicated, the reconstruction of ramus
can be performed by the use of a costochodral graft
reconstruction between the ages of 3 to 4 years. Rib or
iliac bone grafting has been used for mandibular
reconstruction (Pruzansky type III-absence of the
ramus, condyle and temporomandibualr joint)(18). The
reconstructed ramus can later be distracted to provide
more length of ramus. Correction of the hypoplastic
orbit (or distropia) should be delayed and may be
considered when the patient is between ages of 5 and
7 years. A costochondral graft is used for ear cartilage
framework reconstruction of microtia and may be
performed around the age of 8 years or older which
allows for better positioning, particularly in patients
with a more severe deformity.
Mandibular distraction osteogenesis may be
indicated in patients with grade I, IIA and IIB
mandibular hypoplasia, and can be applied at any age
from neonates to adults(19). In the patient with unilateral
craniofacial microsomia, the surgical goal is to increase
the vertical or superoinferior dimension of the ramus
and movement of the chin point to the midline(17).
Any definitive skeletal reconstructive
procedure should be delayed until growth of the facial
skeleton is complete; that is, after the age of 18 years,
depending on orthodontic and orthognathic
assessment of the deformities.
Minimal or moderate soft tissue deficiencies
may not require any treatment. Consideration of any
soft tissue augmentation may be performed after the
age of 18 years; that is, at the time of, or during,
orthognathic surgery. Treatment by fat injections has
produced significant post-treatment atrophy which
may be from insufficient skin expansion(20). Dermis fat
graft from the lower abdomen or thighs may be used.
However, in large soft tissue deficiencies, free
vascularized tissue transfers are recommended for soft
J Med Assoc Thai Vol. 94 Suppl. 6 2011 S107
tissue augmentation(21,22). Objective assessment of the
extent and magnitude of soft tissue and bony
deficiencies is important for better surgical planning;
such as soft tissue expansion and free flap transfer.
The combination of distraction osteogenesis and
microvascular free tissue transfer has been reported to
provide more functional and esthetic outcomes(23).
Conclusion
The goals of treatment for CFM are to (a)
manage respiratory insufficiency and feeding problems
(b) maximize hearing and ability to communicate (c)
improve facial symmetry and proper facial growth and
(d) optimize dental occlusion. These patients require
staged reconstruction and coordinated care by an
experienced multidisciplinary craniofacial team for (a)
comprehensive assessment (b) optimum treatment with
planning, execution and long-term outcome adapted to
the anatomical and functional deformities (c) and
consideration of the needs of patients and their family
and other involved stakeholders.
Acknowledgement
The present study was supported by the
Tawanchai Foundation for Cleft Lip-Palate and
Craniofacial Deformities and the Center of Cleft Lip-
Cleft palate and Craniofacial Deformities, Khon Kaen
University, in Association with the Tawanchai Project.
The authors thank (a) all the patients with their families
and the staff of the Foundation (b) the Cleft Center and
the Audio-Visual Unit of Faculty of Medicine, Khon
Kaen University, for their supportive participation and
(c) Mr. Bryan Roderick Hamman and Mrs. Janice
Loewen-Hamman for their assistance with the English-
language presentation of the manuscript.
Potential conflicts of interest
None.
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20. Inigo F, Jimenez-Murat Y, Arroyo O, Fernandez M,
Ysunza A. Restoration of facial contour in
Romberg’s disease and hemifacial microsomia: ex-
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21. Siebert JW, Anson G, Longaker MT. Microsurgical
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ครานิโอเฟเชียลไมโครโซเมีย: เป้าประสงค์ของการรักษา การเสริมสร้างแบบเป็นระยะและผล
การรกษาในระยะยาว
บวรศลป เชาวน์ชื่น, พูนศกดิ์ ภิเศก, ปรารถนา เชาวน์ชื่น, สงวนศกดิ์ ธนาวิรัตนานิจ
ภูมิหลัง: ครานิโอเฟเชียลไมโครโซเมียเป็นภาวะความพิการแต่กำเนิดที่มีความซับซ้อน และมีความเกี่ยวข้องปฐมภูมิ
กับอวัยวะที่เจริญมาจากแนวโคงแบรนเคียลที่หนึ่งและสอง
วัตถุประสงค: เพื่อนำผลการทบทวนลักษณะการตรวจพบทางคลินิก การประเมินการรักษาและผลลัพธ์ของผู้ป่วย
ครานิโอเฟเชียลไมโครโซเมียที่ได้รับการรักษาโดยผู้นิพนธ์ในโรงพยาบาลศรีนครินทร์ มหาวิทยาลัยขอนแก่น
วัสดุและวิธีการ: การศึกษาเป็นการทบทวนบันทึกทางการแพทย์รวมถึงการวเคราะห์การประเมินทางคลินิก
และรังสีวิทยา การรักษา และผลลัพธ์ของการรักษาของผู้ป่วยครานิโอเฟเชียลไมโครโซเมีย ที่ได้รับการรักษา
โดยผู้นิพนธ์ในโรงพยาบาลศรีนครินทร์ ในระหว่างปี .. 2536-2554.
ผลการศกษา: มีผู้ป่วยทั้งหมด 23 ราย เปนเพศชาย 14 ราย และเพศหญิง 9 ราย 4 รายเปนความพการสองขาง 19
ราย เป็นความพิการข้างเดียว และ 1 ราย เป็นกลุ่มอาการ การตรวจพบทางคลินิกพบได้ตั้งแต่การเจริญเติบโต
น้อยของห ไปจนถงความพการรนแรงของกระดกขากรรไกรลาง กระดกโหนกแก้ม และกระดกขากรรไกรบน การรกษา
ทางการผ่าตัดประกอบด้วย การแก้ไขภาวะมุมปากกว้าง การยืดถ่างขยายกระดูก การเสริมสร้างใบหูแบบเป็นระยะ
การผ่าตัดกระดูกกระบอกตา-กระดูกขากรรไกรบน-กระดูกขากรรไกรล่าง การเสริมสร้างเนื้อเยื่ออ่อนโดยใช
การปลูกถ่ายไขมันและชั้นหนังแท้ การซ่อมแซมปากแหว่งและการแหว่งของจมูก ผู้ป่วยส่วนใหญ่พอใจผลการรักษา
และมีหลายรายที่อยู่ในช่วงอายุกระดูกใบหน้ายังเจริญไมเต็มที่และอยู่ในระหว่างแผนการเสริมสร้างแบบเป็นระยะ
สรุป: เป้าประสงค์ของการรักษาผู้ป่วยครานิโอเฟเชียลไมโครโซเมีย คือ การดูแลภาวะการอุดกั้นทางเดินหายใจ
ปัญหาการให้อาหาร การได้ยินและสื่อสาร ความสมมาตรของใบหน้า การเจริญของใบหน และการสบฟันที่ดี
ผู้ป่วยต้องการการเสริมสร้างแบบเป็นระยะ และการประสานงานกันเป็นอย่างดีของทีมการดูแลแบบสหวิทยาการ
ของศรษะและใบหน้า เพื่อการประเมินโดยละเอียด การวางแผนการรักษาที่เหมาะสม และผลลัพธ์ระยะยาวของ
ความพิการทั้งทางกายวิภาคและหน้าที่การทำงาน รวมถึงความต้องการของผู้ป่วย ครอบครัว และผู้มีส่วนได้ส่วนเสีย
ที่เกี่ยวข้อง
... CFM, we reviewed the clinical presentations, assessment, and long-term management and outcome of a patient with CFM, treated by early surgical reconstruction, distraction osteogenesis and comprehensive care per the protocol of the Tawanchai Center (3) seen and managed at Srinagarind Hospital between 1993 and 2011. The patient was treated by mandibular distraction osteogenesis with long-term follow-up. ...
... A 3-D CT scan is helpful for pre-and postsurgical treatment planning of bony and soft tissue and outcome evaluation, focus on asymmetric hypoplasia of facial skeleton, and temporal bone. Other images may include x-rays of the cervical spine, echocardiogram, and renal ultrasound examination (3) . A CT scan of the temporal bone to assess the external, middle and inner ear structures at 5 and 6 years before microtia reconstruction is helpful to identify patient who need surgical correction to improve hearing. ...
... Long-term protocol of Craniofacial Center is essential (3) . The treatment algorithm is divided into the neonatal period and infancy, early childhood (18 months to 3 years), childhood (4 to 13 years), and adolescence and adulthood (M >16 and F >17). ...
Article
Background: Craniofacial microsomia (CFM) is a complex, congenital, malformation, primarily involving structures derived from the first and second branchial arches. There is limited information on its long-term management and outcomes. Objective: To present the long-term management and outcome of a patient with CFM treated by early distraction osteogenesis and a protocol of comprehensive care at the Tawanchai Center, Srinagarind Hospital, Khon Kaen University. Material and Method: After reviewing the medical records for the clinical presentations, assessments, and long-term managements and outcomes of patients with CFM at Srinagarind Hospital, we focused on one patient, treated by early surgical reconstruction, mandibular distraction osteogenesis (DO), and comprehensive care according to the protocol developed at the Tawanchai Center. Results: The patient presented normal speech, mouth breathing, normal swallowing, and normal temporomandibular joint function. He had an antimongoloid slant, left malar hypoplasia, a cross bite, occlusal plane canting and a slightly deviated chin to the right, a good mouth opening, and a normal bite pattern. The patient was completely satisfied according to overall satisfaction, nose, and upper lip; and moderately satisfied according to overall face, head shape, and occlusion. Conclusion: Our study suggests that the use of DO in young children with CFM provides good long-term distraction on the growth of the mandible and greater facial symmetry. The study addresses the comprehensive evaluation of the long-term, interdisciplinary, comprehensive care of a patient with CMS. Consideration of the needs and expectations of the patient and his/her family and other involved stakeholders is essential. Keywords: Craniofacial microsomia, Early mandibular distraction osteogenesis, Comprehensive management, Long-term outcome
... The previous burn room at 3C ward (1999-2003) and the present Burn Center (2003-present) in the Srinagarind Hospital. Many aspects of plastic surgery were treated in the unit, including basic principles of plastic surgery, wound care and scar management (7)(8)(9) (Fig. 8), burns (10,11) (Fig. 9), maxillofacial injuries (12,13) craniofacial and cleft surgery (14)(15)(16)(17)(18)(19)(20)(21)(22) (Fig. 10, 11), head and neck cancer and reconstruction (23)(24)(25)(26)(27)(28) , microvascular surgery and free tissue transfer (including multiple free flaps) (29,30) (Fig. 12-17), breast reconstruction (31) (Fig. 18), hand surgery (Fig. 19), lower extremity reconstruction (Fig. 20) and reconstruction of genitalia (Fig. 21). ...
Article
Full-text available
Plastic surgery consists of two major fields: reconstructive surgery and cosmetic surgery with its roots lie in the reconstruction aspect. In Thailand, the plastic surgery procedures, performed during the initial period, included cleft lip and cleft palate repairs and skin graftings. In 1987, the Plastic Surgery Unit was established in the Department of Surgery, Srinagarind Hospital in Khon Kaen, which is the city in the Center of the northeast Thailand. In 1991, the partnership training of resident in plastic surgery with Siriraj Hospital was established and continued until the present time. All fields of plastic surgery were managed and educated in the Plastic Surgery Unit. Since the first batch of plastic surgery training program in 2009, the unit has many advanced in interdisciplinary management, the management of cleft center, burn center, postgraduate training program, research, community services, and national, regional and international coloration. The future perspectives involve education for 21st century skills, integration of teaching, research and community services, and a focus on evidence based medicine and benchmarked outcomes are the future perspectives.
... Several authors have stated that patients with CFM should be screened routinely for OSA. 20,23,24 Nevertheless, the exact prevalence of OSA in CFM and the severity of the pathology on which these statements are based are not mentioned in these expert opinions. ...
Article
Children with craniofacial microsomia (CFM) are at risk of obstructive sleep apnoea (OSA). This systematic review provides an overview of the literature on the prevalence of OSA in children with CFM. A search was performed in PubMed, Embase, Cochrane Library, and Web of Science for articles on CFM and OSA. The following data were extracted from the articles: number of patients, patient characteristics, presence of OSA, polysomnography outcomes, and the treatments and outcomes of OSA. We included 16 articles on CFM and OSA, four of which reported the prevalence of OSA (range 7-67%). Surgical treatment was more often described in these patients than conservative treatment. According to the literature, OSA is related to CFM. However, as there have been no prospective studies and few studies have presented objective measurements, no definitive conclusions can be drawn. Prospective studies are needed to determine the prevalence of OSA in patients with CFM. Copyright © 2015 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
Article
The aim of this systematic review was to review the literature on hearing impairment and ear anomalies in patients with craniofacial microsomia and to determine their prevalence. Sixty-two records including 5122 patients were included. Ear anomalies were present in 52–100% of patients. The most reported external ear malformations were microtia, pre-auricular tags, and atresia of the external auditory canal. Ossicular anomalies were the most reported middle ear malformations, whereas the most reported inner ear malformations included oval window anomalies, cochlear anomalies, and anomalies of the semicircular canals. Hearing loss in general was reported in 29–100% of patients, which comprised conductive hearing loss, mixed hearing loss, and sensorineural hearing loss. Between 21% and 51% of patients used hearing aids, and 58% underwent a surgical intervention to improve hearing. The relationship between different phenotypes of craniofacial microsomia and the type and severity of hearing loss is mostly unclear. In conclusion, the high prevalence of ear and hearing anomalies in patients with craniofacial microsomia underlines the importance of audiological screening in order to facilitate individual treatment.
Article
Full-text available
The main objective of the present study was to apply the Baby-ROMA index in a Portuguese preschool population of the District of Lisbon. The application of this index allows the evaluation of the need for orthodontic treatment and the risk of malocclusion. Another objective was to analyze the relationship between the Baby-ROMA Index and sociodemographic variables. A cross-sectional study was carried out with a non-probabilistic convenience sample, consisting of 89 children. The data collection was performed through a questionnaire and an intraoral examination. Observations and application of the index were performed by a calibrated observer. The questionnaire collected information on sociodemographic variables and some of the Baby-ROMA Index factors. The intraoral examination was carried out in schools, collecting information about the other factors of the index. A descriptive analysis of the variables was performed and the chi-square test (α = 0.05) was used. After analyzing the results, it was verified that more than a quarter of the population (26.1%) needed immediate orthodontic treatment. About half of the children (51.1%) had a high risk of malocclusion. The results emphasize the importance of early detection of malocclusion risk factors, allowing the monitoring of the harmful factors present in the deciduous dentition in order to intervene early through the application of preventive and / or interceptive methods and the implementation of effective preventive strategies to reduce the high prevalence of malocclusion.
Article
Background: The challenge for anesthetic management for children with craniofacial deformities is to develop comprehensive anesthetic care. The purposes of this study were to review the authors' experience with airway management and ventilator support and evaluate outcome during the perioperative period among these patients at the Tawanchai Center, Srinagarind Hospital. Methods: A retrospective (2005-2009), descriptive study was conducted to analyze the anesthetic management and perioperative outcomes among children with craniofacial deformities at the Tawanchai Center. Techniques for airway management included direct laryngoscope (DL), DL with stylet, fiber-optic bronchoscope (FOB), laryngeal mask airway, retrograde intubation, a combination of these techniques and more than one anesthesiologist. Results: Forty-five children with craniofacial deformities were classified as having the following: craniosynostosis; craniofacial microsomia; Treacher-Collins syndrome (TCS); coloboma of the eye, heart defects, atresia choanae, retarded growth and/or development, genital and/or urinary abnormalities, ear abnormalities and deafness (CHARGE) syndrome; frontoethmoidal encephalomeningocele; Pierre Robin sequence; and craniofacial cleft. Most of the patients underwent induction of anesthesia by inhalation of an anesthetic agent. The children with TCS were the most difficult group with regard to airway management with DL view (13%), and multiple airway accessories and intubation techniques were needed. The two most common methods for management of difficult airway obstructions were the use of stylet and FOB. No complications occurred during intubation and minor complications were observed during the perioperative period. Conclusion: A successful outcome depended on the provision of interdisciplinary craniofacial team management, comprehensive anesthetic evaluation and management, well-informed and cooperative children and supportive family members.
Article
Hemifacial microsomia (HFM) is a complex congenital condition. This review discusses recent research findings which affect all aspects of HFM, and addresses some prevailing misconceptions. Firstly, the broad phenotype is outlined, with an emphasis on the facial anomalies which are important for diagnosis, classification and treatment. The range of HFM anomalies and their possible embryology also account for the varied terms used in the literature. In addition, consideration of causation helps to shape our understanding of HFM as a clinical entity. Aetiology is described with particular emphasis on the involvement of genetic factors, although at present this is largely hypothetical. Finally, the principles of HFM management are reviewed. Attention is given to the integrated planning and team approach necessary to treat such patients. In addition, the possible attributes of new treatments, such as distraction osteogenesis, are highlighted.
Article
Hemifacial microsomia (HFM) is a complex congenital condition. This review discusses recent research findings which affect all aspects of HFM, and addresses some prevailing misconceptions. Firstly, the broad phenotype is outlined, with an emphasis on the facial anomalies which are important for diagnosis, classification and treatment. The range of HFM anomalies and their possible embryology also account for the varied terms used in the literature. In addition, consideration of causation helps to shape our understanding of HFM as a clinical entity. Aetiology is described with particular emphasis on the involvement of genetic factors, although at present this is largely hypothetical. Finally, the principles of HFM management are reviewed. Attention is given to the integrated planning and team approach necessary to treat such patients. In addition, the possible attributes of new treatments, such as distraction osteogenesis, are highlighted.
Chapter
Craniofacial microsomia (CFM) includes a spectrum of malformations primarily involving structures derived from the first and second branchial arches. Findings include facial asymmetry resulting from maxillary and/or mandibular hypoplasia; preauricular or facial tags; ear malformations that can include microtia (hypoplasia of the external ear), anotia (absence of the external ear), or aural atresia (absence of the external ear canal); and hearing loss. Severity can range from subtle facial asymmetry with a small skin tag in front of an otherwise normal-appearing ear to bilateral involvement (typically asymmetric), microtia/anotia with atresia of the ear canals, microphthalmia, and respiratory compromise from severe mandibular hypoplasia. Other craniofacial malformations including cleft lip and/or palate can be seen. Non-craniofacial malformations, especially vertebral, cardiac, and limb, can be seen. The diagnosis of CFM is based on clinical findings. CFM most frequently occurs as a simplex case (i.e., occurrence in a single individual in a family) with unknown etiology; recurrence risks are empiric. If an individual with CFM is found to have an inherited or de novo chromosome abnormality, genetic counseling for that condition is indicated. Occasional autosomal dominant or autosomal recessive inheritance is observed. If a proband has CFM and no reported family history of CFM, the risk to sibs is two to three percent; this may be an underestimate because of the difficulty of obtaining an accurate family history for some of the subtle features of CFM. Treatment of manifestations: For optimal outcome children with CFM require timely and coordinated assessments and interventions. Ideally, children should be managed by an experienced multidisciplinary craniofacial team. The goals of treatment for CFM are to assure adequate respiratory support and feeding in infants with severe facial malformations, maximize hearing and communication, improve facial symmetry, and optimize dental occlusion. Treatment is age-dependent, with time-sensitive interventions at appropriate stages of craniofacial growth and development.
Article
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.
Article
Lengthening of the mandible by gradual distraction was performed on four young patients (average age 78 months). The amount of mandibular bone lengthening ranged from 18 to 24 mm; one patient with Nager's syndrome underwent bilateral mandibular expansion. Following the period of expansion, the patients were maintained in external fixation for an average of 9 weeks to allow ossification. The patients were followed for a minimum of 11 months to a maximum of 20 months with clinical and dental examinations as well as photographic and radiographic documentation. The technique holds promise for early reconstruction of craniofacial skeletal defects without the need for bone grafts, blood transfusion, or intermaxillary fixation.
Article
The wide spectrum of anomalies associated with hemifacial microsomia (HFM) has made systematic and inclusive classification difficult. We propose a nosologic system in which each letter of the acronym O.M.E.N.S. indicates one of the five major manifestations of HFM. O for orbital distortion; M for mandibular hypoplasia; E for ear anomaly; N for nerve involvement; and S for soft tissue deficiency. The O.M.E.N.S. system is easily adapted for data storage, retrieval, and statistical analysis. A retrospective study of 154 patients with HFM classified according to the O.M.E.N.S. system confirmed the concept that the mandibular deformity is the cornerstone of the anomaly. Statistical analysis demonstrated a positive association between mandibular hypoplasia and the severity of orbital, auricular, neural, and soft tissue involvement. This study did not confirm a previously reported predominance of gender or sidedness. Analysis of statistical correlations failed to substantiate a Goldenhar variant as a syndromic entity. Our analysis showed that palatal deviation is probably caused by muscular hypoplasia and not by weakness of a particular cranial nerve.
Article
Terms such as oculoauriculovertebral dysplasia, Goldenhar syndrome, and hemifacial microsomia have been used to describe microtia with specific combinations of other craniofacial anomalies. Microtia is also observed with anomalies of postcranial structures. Statistical studies were performed on 297 patients with microtia and other anomalies to identify subgroups of patients representing previously described or new associations. Analysis identified 15 subgroups of patients with specific patterns of anomalies. Log-linear analyses of cranial and postcranial variables demonstrated a positive association between mandibular hypoplasia and cervical spine fusion, which was, in turn, positively associated with other spine anomalies (P less than .02) and other skeletal anomalies (P less than .001). Although unilateral microtia was commonly observed with mandibular hypoplasia, mandibular hypoplasia was negatively associated with bilateral microtia. Many of the associated anomalies were of structures not derived from the 1st and 2nd branchial arch neural crest. However, most associated anomalies were of structures derived from migratory cell populations or populations undergoing differentiation prior to migration between the 19th and 24th day post-fertilization (neural crest, ectodermal placode, mesoderm, surface ectoderm). These findings suggest that many different cell populations may be disturbed in the pathogenesis of microtia in association with other anomalies. The timing of the pathogenetic event may determine the specific pattern of associated anomalies.
Article
This is a follow-up study of 20 children who had surgical correction of hemifacial microsomia in an effort to improve facial growth and minimize secondary distortion. In group 1 (skeletal types I and IIA), 10 children underwent elongation and lengthening of the mandible. In group 2 (skeletal types IIB and III), 10 children had total construction of a new temporomandibular joint and mandibular ramus with rib grafts and costochondral junction. In both groups, an open bite was created on the affected side to provide space for tooth eruption and downward growth of the middle face. The mean follow-up was 50.9 months (18 to 117 months) in group 1 and 45 months (18 to 50 months) in group 2. Analysis of our early results showed that all patients have had downward growth of the midface on the affected side. All five patients in group 1, followed to complete closure of the surgically created open bite, have maintained a level occlusal plane. In group 2, the one patient followed to completion of facial growth continues to have a level occlusal plane and facial symmetry. Appropriately timed mandibular construction and/or elongation in children with hemifacial microsomia is safe and effective. There is a decrease in secondary deformity on the affected side, and the eventual overall facial growth is optimized. Some group 2 children will need secondary elongation and augmentation of the mandible. Nevertheless, this study demonstrates that early mandibular correction may obviate the need for maxillary and orbital procedures in adulthood.