Facial Cleft #2-12
ARTIGO ORIGINAL
Series of facial cleft number 2-12: strategies for treatment
Séries de fissuras faciais número 2-12: estratégias para tratamento
Renato da Silva Freitas1, Nivaldo Alonso2, Luciano Busato3, Alessandra dos Santos Bitencourt4, Alessandro Zorzi4, Brunno Gomes Rocha4,
Larissa Lopes Rocha4, Thais de Freitas Azzolini5
RESUMO
abstract
Introdução: A fissura facial número 2 é uma malformação
extremamente rara, de etiologia desconhecida. O acometimento
de tecidos moles começa com uma fissura comum de lábio superior e de terço medial de borda alar. A cartilagem alar pode ser
hipoplásica e deslocada superiormente na sua porção média. Sua
correspondente craniana é a fissura 12. Objetivo: O objetivo
deste estudo foi revisar os resultados funcionais e estéticos de
diferentes técnicas aplicadas a cada caso. Método: O presente
estudo foi baseado na observação de 10 pacientes com fissura
número 2-12 de Tessier, atendidos no CAIF- Centro de Atendimento Integral ao Fissurado Lábio Palatal, durante o período
de 1996 e 2008. Foram também revisados os dados clínicos e
radiológicos de cada caso. Resultados: O tratamento iniciouse no primeiro ano de vida da maioria de nossos pacientes. Sete
deles foram submetidos a reconstrução labial, sendo quatro pela
técnica de Millard, e três pacientes à adesão labial. Três pacientes
não apresentavam acometimento labial. O reposicionamento da
cartilagem alar foi realizado em três casos, a correção do coloboma alar usando retalhos locais em um, reconstrução de ausência
de heminariz com retalho frontal em dois, e uso de retalho em V-Y
em 2 pacientes. A sobrancelha foi corrigida pelo reposicionamento
dos cabelos, quando estes estavam deslocados, ou utilizando o
microimplante de cabelo associado à tatuagem, em casos sem
sobrancelha remanescente. Conclusão: O tratamento da fissura
2-12 necessita de abordagem planejada, estagiada e sequencial,
para produzir um resultado final adequado. Pele, tecidos moles e
osso devem ser abordados para conseguir um bom seguimento. O
acometimento do lábio, nariz, canto palpebral medial e craniano
foram tratados no mesmo ato operatório, e utilizamos retalhos
locais sempre que possível. Pacientes com hiperteleorbitismo
deveriam ser tratados após oito anos de idade.
Introduction: The facial cleft number 2 is an extremely rare
malformation of unknown etiology. The soft tissue defect begins
with a common cleft of the upper lip and an involvement of the
middle third of the alar rim. The nasal alar cartilage may be hypoplasic and drown upward in the midportion. Its northbound is the
cranial cleft number 12. Objective: The aim was to review the
functional outcome and aesthetic results of the different techniques
applied for each case. Methods: The present study is based on
observations of 10 patients with Tessier number 2 cleft seen at
Assistance Center for Cleft Lip and Palate during the period from
1996 to 2008. We reviewed the clinical databases and radiologic
studies. Results: The treatment was begun in the first year of life
in majority of our patients. Seven of them were submitted to lip
repair using Millard technique (4 cases) and simple lip adhesion (3
cases), and three patients did not have any surgery over the upper
lip. Nasal cartilage reposition was performed in three cases, repair
of the alar coloboma using local flaps in one case, two half-nose
reconstructions using a forehead flap, and two local V-Y flaps. The
eyebrow was corrected repositioning the hair when it was dislocated or using a micrograft associated to tattooing in cases without
hairs. Conclusion: The treatment of the cleft number 2 demands
a planned, staged, sequential approach to produce the ideal endresult. Skin, soft tissue, and bone must be addressed to achieve an
ideal result with optimize outcome. Lip, nose, medial canthus and
cranial involvement were treated in the same stage, and we have
utilized local flaps in all possible situation. Hypertelorism correction
should be delayed to eight years of age.
Descritores: Fissura facial. Fissura labial. Malformação
facial. Fissura Nasal.
Key words: Facial cleft. Cleft lip. Facial malformation.
Cleft nose.
1. Associate Professor, Section of Plastic and Reconstructive Surgery, Federal
University of Paraná; and Plastic Surgeon of the Assistance Center for Cleft
Lip and Palate (CAIF).
2. Chief of the Craniofacial Surgery Unit, Section of Plastic Surgery, Sao
Paulo University.
3. Plastic Surgeon of the CAIF.
4. Medical Student, School of Medicine, Federal University of Paraná.
5. Medical Student, School of Medicine, Faculdade Evangélica do Paraná.
Correspondence: Renato da Silva Freitas
Rua General Carneiro, 181, 9º andar – Curitiba, PR, Brazil – Zip Code:
8000-000
E-mail: [email protected]
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225
da Silva Freitas et al.
Five of these patients were male and five were female. The
average patient age at time of surgery was 7,9 years. The clinical
findings associated with lip malformation were: alveolar cleft,
cleft palate, nose and eyelid involvement, lacrimal obstruction,
dislocation of medial canthus, eyebrow involvement including
cleft and dislocation, hypertelorism and encephalocele. The
surgical procedures and tomographic studies were evaluated.
We have discussed our protocol of the treatment.
INTRODUction
The Tessier classification system is one of the most useful
in facial cleft because it makes the correlation between clinical
appearance and surgical anatomy on craniofacial clefts. Also, it
integrates topographic clinical observations with the underlying
skeletal disturbance. The facial cleft number 2 as described by
Tessier is an extremely rare malformation of unknown etiology
with distinct soft tissue and bone characteristics1.
At the facial cleft number 2, the soft tissue defect begins
with a common cleft of the upper lip and an involvement of
the middle third of the alar rim. The nasal alar cartilage may be
hypoplasic and drown upward in the mid portion. The dome is
intact. The lateral aspect of the nose is flattened and the dorsum
is broad. A shallow soft tissue groove extends superiorly to the
asymmetrically widened nasal root2,3.
The skeletal defect begins on the maxilla. The cleft crosses
the alveolus in the region of the lateral incisor, and extends into
the pyriform aperture. A hard and soft palate cleft may occur. The
nasal septum is intact but deviated to the opposite side. A notch
is frequently present near the junction of the nasal bone and the
frontal process of the maxilla, which is widened. Related to cleft
number 12, the northbound of facial cleft number 2, above this
nasomaxillary notching, the ethmoid sinus is undeveloped and
there is not pneumatization of the frontal sinus of this side. The
lacrimal system, palpebral fissures and eyebrow may be intact1,4.
Few articles have been published about the cleft number
25-12. These articles have often described the challenge of the
treatment. We conducted a retrospective analysis of our series
consisting of 10 cases of Tessier number 2 cleft. The objective
was to review the functional outcome and aesthetic results of
the different techniques applied for each case.
RESULTS
At Assistance Center for Cleft Lip and Palate (CAIF),
7900 patients with facial malformation have been treated in
18 years of activity. Around 300 patients have had rare facial
clefts, the majority of them with craniofacial microsomia. From
this group, 10 patients with a number 2 cleft were reviewed.
Four patients were evaluated in the first year of life, with an
average age of presentation of three years. Five were female
and five male. One patient had amniotic bands in limbs with
amputations of some fingers.
Related to cleft lip, three patients were found to have bilateral cleft lip and four patients unilateral cleft lip (Table 1).
Three patients did not have any involvement of the upper lip.
All patients with normal upper lip did not have alveolar clefts
either. The alveolar bridge was compromised in six patients,
with varying degree. One patient had a cleft lip without an
alveolar cleft. Four patients had cleft palate.
Two patients presented normal shape and position of the
nose. However, eight patients had some nasal malformation,
including three cases with half-nose, two cases with alar base
upward deviated, two cases with a “regular” unilateral cleft
nose, and two alar coloboma.
In the orbitopalpebral area, four cases of lacrimal duct
obstruction were identified (Table 2). Two patients had medial
eyelid coloboma and the medial canthus was dislocated
downward in four cases. Seven cases of cleft number 12 were
observed. Encephalocele was present in three patients of these
patients. The eyebrow was clefted in four cases and superiorly
METHODS
Ten patients with Tessier number 2 cleft were admitted to
Assistance Center for Cleft Lip and Palate (CAIF) since 1996.
The age at treatment ranged from newborn to 30 years-old.
Table 1 - Tessier number 2 cleft. Clinical findings.
1
Gender
Age
Lip
Alveolar
female
2
BCL
clefted
2
male
0
lUCL
clefted
3
4
5
6
male
female
male
male
18
13
0
0
BCL
BCL
rUCL
normal
clefted
normal
clefted
normal
7
female
3
normal
normal
8
9
10
female
male
female
13
30
0
7,9
normal
rUCL
rUCL
normal
clefted
clefted
Nose
upperward +
cleft
R upperward +
cleft
normal
normal
UCN
halfnose
halfnose +
proboscis
halfnose
UCN
UCN
Palate
Associated
clefted
12 + encephalocele
clefted
in limbs
no
clefted
normal
normal
normal
mentosum
no
microtia
12 + encephalocele
12
normal
no
no
normal
normal
clefted
12
12
12 + encephalocele
no
no
no
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12 + xeroderma pig-
Others
amniotic bands
no
no
no
no
Facial Cleft #2-12
nose. All the cleft palate was closed using a von Langenback
technique and intravelar veloplasty. Only two patients were
necessary to perform the reposition of medial canthus using a
flap from the upper eyelid with medial pedicle. The eyebrow
was corrected repositioning the hair when it was dislocated
or using hair micrografts and tattoing in cases with absence
of eyebrow (Figure 2). Four cranioplasties were necessary,
three using bone grafting and one using acrylic to reshape the
forehead. Only one case of hypertelorism was submitted to
orbital advancement with Tessier technique.
dislocated in two cases. Six cases of hypertelorism, one case of
iris coloboma and one of keratitis were observed.
The treatment was begun in the first year in majority of
our patients (Table 3). Seven of our patients were submitted
to lip repair, using Millard technique (four cases) (Figure 1),
or lip adhesion (three cases), and three patients did not have
any surgery over the upper lip. Nasal cartilage reposition was
performed in three cases, repair of the alar coloboma using
local flaps in one case, two half-nose reconstructions using a
forehead flap, and two local V-Y flaps. Two patients had normal
Table 2 - Tessier number 2 cleft. Orbitopalpebral findings.
Lacrimal
Eyelid
Medial Canthus
Eyebrow
Hypertelorism
Eye Globus
1
normal
normal
normal
normal
yes
normal
2
normal
normal
normal
R clefted
no
iris coloboma
3
normal
normal
normal
normal
yes
normal
4
normal
normal
normal
normal
no
normal
5
normal
normal
dislocated
clefted
yes
normal
6
obstruction
normal
normal
dislocated
no
normal
7
obstruction
clefted
normal
normal
no
normal
8
obstruction
clefted
dislocated
dislocated
yes
keratitis
9
normal
normal
dislocated
clefted
yes
normal
10
obstruction
normal
dislocated
clefted
yes
normal
Table 3 - Surgical procedures.
#
Surgeries
Lip Repair
Nose
Palate
Orbitopalpebral
Eyebrow
Others
hypertelorism
1
5
adhesion
V-Y flap
von Langhenbeck
no
no
2
4
Millard
local flap
von Langhenbeck
no
no
3
3
Millard
skoog
no
no
4
1
PR*
PR*
PR*
5
1
Lip review
no
no
6
5
Millard
McComb
7
2
no
8
3
Lip review
9
0
no
no
10
4
Millard
McComb
half-nose
reconstruction
half-nose
reconstruction
von
Langhenbeck
no
cranioplasty with
hairs
bone graft
no
no
maxilla osteotomy
no
no
no
micrograft and
cranioplasty with
tattoo
bone graft
no
no
no
medial
canthopexy
no
no
von
medial
Langhenbeck
canthopexy
* PR: Previous repair
Rev Bras Cir Craniomaxilofac 2010; 13(4): 225-9
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acrylic
local flap with
no
no
cranioplasty with
no
no
micrograft
cartilage graft to
nose
no
cranioplasty with
bone graft
da Silva Freitas et al.
DISCUSSION
Figure 1 - 30 years-old man, with right cleft lip,
associated to hypertelorism, absence of mid-eyebrow and
frontal bone (cleft #12). A: Pre-operative photograph
(frontal view); B and C: Tomographic view; D: Postoperative of lip repair (Millard technique) and frontal bone
grafting. E: Follow-up of 8 years.
A
B
The treatment of these rare facial clefts is usually not
directed at a single-stage surgical correction, but demands a
planned, staged, sequential approach to produce the ideal endresult in the end of facial growth. Therefore, our cases were
submitted to three surgeries in average. Reconstruction in these
cases must consider the restoration of all elements – skin, soft
tissue and skeleton, in a complex interrelated fashion at an
age-appropriate time to optimize outcome.
The result, both functional and aesthetic, is however principally determined by the quantity, quality and redraping of
the soft tissues. Reconstruction with soft tissues comparable
in color and consistency, and free from distorting traditional
forces, idealizes restoration of facial features and permits
tension-free sitting of vital soft tissue landmarks. The tissue
expansion is a useful adjunct to provide a greater amount of
better vascularized local tissue for reconstruction of the soft
tissue, by cheek rotation and advancement flaps. We did not
have any case that was necessary to indicate tissue expander.
In general, cleft number 2 presenting only alar coloboma
can be corrected by full-thickness rotation flaps taken from
the lateral aspect of the nose. Satisfactory rim contour is generally obtained, especially in minor alar clefts, no associated
to hypertelorism or encephalocele. However, sometimes the
asymmetrical nostrils may require secondary correction. More
severe clefts extending along the whole length of the nose can
be corrected by rotation of longer nasal flaps alone or combined
with forehead flaps. Cases associated with orbital hypertelorism, the nasal correction may be carried out simultaneously
with orbital mobilization. One of our cases was submitted to
advancement of two V-Y flaps to create the nostrils. Specifically
to this patient, she had hypertelorism and forehead encephalocele, which was partially corrected at eight years of age. In
the minor case of alar coloboma, a subcutaneous pedicled V-Y
flaps from nasojugal area achieved great contour of the nostril
and good quality of the scar. Other 3 patients had nasal distortion similar to unilateral cleft nose, and they were submitted
to primary rhinoplasty using McComb (2 cases) and Skoog (1
case) techniques. Cases of half-nose from out unit were already
published and discussed13. In CAIF we had 3 cases of halfnose,
one of them refused the treatment.
Few papers were published describing the malformation1-4
and others few demonstrating the treatment5-12. Ozek published
different surgical approaches to be utilized9. Facial bipartition
may be indicated to treat orbital hypertelorism, as suggested by
van der Meulen14. When the nasolacrimal duct was involved,
treatment includes total excision, since repeated infection tends
to develop in the distorted ductal system. In patients with alar
coloboma, the authors corrected by full-thickness rotation flaps
taken from the lateral aspect of the nose and the complete cleft
lip can be repaired using technique to repair regular cleft lip,
as Millard. In our series, we have used the same approach for
cleft lip.
Absence of eyebrow was present is some patients, and
distortion of its position in others. Some of patients were
managed using hair micrografts and tattooing15, others only
micrografts, and a third group rotation of residual hair to
eyebrow line. All of these techniques demonstrated to be good
options to treat this abnormality. Dystopia of medial canthus
C
D
E
Figure 2 - Patient with 5 months-old, with right cleft lip and palate,
absence of mid-eyebrow. A: Pre-operative photograph; B: Tomographic view, demonstrating alveolar cleft and frontal defect. C:
Final result after lip repair (Millard), Palatoplasty (veloplasty), frontal
bone grafting, eyebrow reconstruction with micrograft and tattoing.
A
B
C
Rev Bras Cir Craniomaxilofac 2010; 13(4): 225-9
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Facial Cleft #2-12
was identified in four cases, and indicated the transposition
of a flap from upper eyelid with medial pedicle associated to
medial canthopexy.
Osteotomy and bone grafting of the craniofacial skeleton
with stable internal fixation can be performed with an acceptably predictable result, even in clefts. The timing of intervention and pattern of osteotomy is individualized for each
of the rare clefts deformities, but involves orbital osteotomy
early before 5 years, with delay of midfacial and mandibular
osteotomy until the completion of facial growth16. One of our
cases was submitted to osteotomy of maxilla and advancement
of 5mm, after orthodontia.
Surgical results are not as good in asymmetrical clefts such
as 2-12, associated with orbital hypertelorism. The alterations of
the orbital and nasal skeleton, as well as the soft-tissue alterations on the cleft side, represent a hard problem to reconstruct.
In terms of symmetry, the center of the face is extremely critical
and, unfortunately, it can rarely be achieved. With improved
techniques it is now possible to obtain excellent results in many
nasal clefts, but challenging problems are still present.
REFERENCES
1.Tessier P. Anatomical classification facial, cranio-facial, and latero-facial
clefts. J Maxillofac Surg. 1976;4(2):69-92.
2.Kawamoto HK Jr. The kaleidoscopic world of rare craniofacial clefts: order
out of chaos (Tessier classification). Clin Plast Surg. 1976;3(4):529-72.
3.Thorne CH. Craniofacial clefts. Clin Plast Surg. 1993;20(4):803-14.
4.Alonso N, da Silva Freitas R. Craniofacial clefts and other related deformities. In: Guyuron B, Ericksson E, Persing JA, eds. Plastic surgery:
indications and practice. vol. I. Philadelphia:Saunders Elsevier;2009.
p.455-71.
5.Ortiz-Monasterio F, Fuente del Campo A, Dimopulos A. Nasal clefts. Ann
Plast Surg. 1987;18(5):377-97.
6.van der Meulen JC, Vaandrager JM. Facial clefts. World J Surg.
1989;13(4):373-83.
7.Tiwari P, Bhatnagar SK, Kalra GS. Tessier number 2 cleft, a variation. Case
report. J Craniomaxilofac Surg. 1991;19(8):346-7.
8.Shewmake KB, Kawamoto HK Jr. Congenital clefts of the nose: principles
of surgical management. Cleft Palate Craniofac J. 1992;29(6):531-9.
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craniofacial anomaly: Tessier No. 2 cleft. J Craniofac Surg. 2001;12(4):35561.
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3 clefts accompanied by a single median lip pit. Cleft Palate Craniofac J.
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11.Losee JE, Kirschner RE, Whitaker LA, Bartlett SP. Congenital nasal anomalies: a classification scheme. Plast Reconstr Surg. 2004;113(2):676-89.
12.Ortiz-Monasterio F, Taylor JA. Major craniofacial clefts: case series and
treatment philosophy. Plast Reconstr Surg. 2008;122(2):534-43.
13.da Silva Freitas R, Alonso N, de Freitas Azzolini T, Busato L, Dall’Oglio
Tolazzi AR, Azor de Oliveira E Cruz G, et al. The surgical repair of halfnose. J Plast Reconstr Aesthet Surg. 2010;63(1):15-21.
14.van der Meulen JC. Medial faciotomy. Br J Plast Surg. 1979;32(4):339-42.
15.da Silva Freitas R, Bertolotte W, Shin J, Busato L, Alonso N, Grande CV,
et al. Combination micrografting and tattooing in the reconstruction of
eyebrows of patients with craniofacial clefts. Ophthal Plast Reconstr Surg.
2008;24(4):340-2.
16.Tessier P, Rougier J, Hervouet F, Woilllez M, Lekieffre M, Demore P. Plastic
surgery of the orbits and eyelids. Chicago:Masson Publishing USA;1981.
CONCLUSION
The treatment of the cleft number 2 demands a planned,
staged, sequential approach to produce the ideal end-result in
the end of facial growth. In general, our patients were submitted
to three surgical procedures. Skin, soft tissue, and bone must
be addressed to achieve an ideal result with optimize outcome.
Therefore, color and consistency of soft tissue are important
during the decision about the surgical options. Lip, nose, medial
canthus and cranial involvement were treated in the same stage,
and we have utilized local flaps in all possible situation. Hypertelorism correction should be delayed to eight years of age.
Paper performed at Assistance Center for Cleft Lip and Palate (CAIF), Curitiba, PR, Brazil; Section of Plastic Surgery, Federal University of Paraná,
Curitiba, PR, Brazil; Craniomaxillofacial Surgery Unit, Section of Plastic Surgery, Sao Paulo University, São Paulo, SP, Brazil.
Article received: 12/6/2010
Article approved: 8/10/2010
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