Phẫu Thuật Tạo Hình Grabb & Smith’s (Bản Cập Nhật – Kevin C. Chung)

Two-Jaw Surgery
Moving the maxilla and the mandible in a single surgery is more involved than simply combining
individual Le Fort and SSO techniques. Two-jaw surgery requires precision at every step, from
orthodontic preparation to presurgical planning to performing the osteotomies to rigid fixation to the use
of postoperative guiding elastics. 17-20
The maxillary and mandibular movements are performed in series. Traditionally, the Le Fort I osteotomy
is performed first, followed by the mandibular movement. More recently, mandible-first surgery has been
widely adopted with results that are equal to the more time-honored maxilla-first approach. Currently,
the choice of technique is often an arbitrary one based on surgeon preference. 21
Regardless of the order of osteotomy, as described earlier, an intermediate splint is generated during the
presurgical planning process that indexes the position of the jaws following the first movement.
Interdental fixation is obtained and the intermediate splint holds the jaws in the desired position while
rigid fixation is achieved. MMF is then released, bone cuts for the second jaw movement are made, and
the final splint is used to stabilize the mandible and maxilla in the final desired occlusion while rigid
fixation is obtained.
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Perioperative Care and Management of Complications
Postoperative management focuses on patient comfort and the prevention of complications. Cold packs
or compresses and head elevation can significantly decrease edema. In addition, a short course of oral
steroid therapy may help minimize swelling, as well as the risk of postanesthesia nausea and vomiting.
Antiemetics are routinely provided to the patient. Chlorhexidine oral rinses after and between meals help
maintain an antiseptic environment in the mouth and mitigate against oral wound infection. Oral
antibiotic administration following surgery has been shown to reduce the risk of infection.
A liquid diet is instituted immediately after surgery, progressing to a mechanical soft diet at
postoperative follow-up. Patients should be counseled that they may experience significant weight loss
because of limited caloric intake in the immediate postoperative period, and the importance of adequate
nutritional intake should be emphasized. Guiding elastics are used to help maintain the stability of the
new occlusion and begin musculoskeletal training, so patients may get accustomed to their new jaw and
dental relationships.
Every orthognathic surgery patient will require some degree of postsurgical orthodontic therapy to
complete their rehabilitation. The length of orthodontic care following orthognathic surgery varies greatly
and is largely dependent upon the presurgical plan, the extent of presurgical orthodontic preparation,
and the specific endpoints set forth by the orthodontist.
Postoperative bleeding is rare, as most bleeding is encountered and controlled during surgery. In unusual
circumstances, injury to the internal maxillary artery or the plexus of vessels at the skull base behind the
maxilla may manifest following completion of the procedure. In these cases, evaluation and intervention
must be immediate and aggressive, potentially involving interventional radiology services or surgical
exploration. 22
After mandibular and two-jaw surgery, most patients will experience transient decrease in sensation in
the distribution of the inferior alveolar nerve. It is important during the SSO to identify and preserve the
nerve as it enters the mandible proximally, throughout its course within its bony canal, and as it exits the
mental foramen. If a transection is identified, neurorrhaphy should be performed immediately.
Postoperative malocclusion is a rare but serious complication of orthognathic surgery. Technical errors
during surgery may predispose a patient to this adverse outcome. The final occlusion should be
thoroughly checked with the condyles seated in the glenoid fossae after rigid fixation is achieved. If the
occlusion does not exactly correlate with the presurgical plan, rigid fixation should be removed, and the
position of one or both jaws must be adjusted. Bony nonunion and malunion are also rare outcomes
following orthognathic surgery. Both require reoperation. Repeat osteotomy and bone grafting may be
necessary in these situations.
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Summary
With appropriate planning and preparation, orthognathic surgery is associated with excellent outcomes,
low-risk profiles, and very satisfied patients. A wide range of versatile techniques exist that can be
implemented in the treatment of malocclusion, and new technologies allow for detailed VSP and other
streamlined perioperative processes that contribute to surgical success. Careful consideration of the
impact of planned jaw movements on the position of both skeletal and soft tissue components of the face
allows surgeons to achieve desired functional outcomes, optimize facial aesthetics, and meet patient
expectations.
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Questions
1. A 30-year-old man presents to your office because he is bothered by his prominent chin. On
physical examination, he has class III malocclusion. Cephalometric evaluation shows an SNA of 81°
(normal 80–84) and an SNB angle of 83° (normal 78–80). There are no other abnormalities. Which of
the following procedures is most appropriate in this patient?
a. Setback genioplasty
b. SSO and setback
c. Le Fort I maxillary advancement
d. Two-jaw surgery
2. A skeletally mature patient presents to your office after referral from her orthodontist. She has
transverse maxillary deficiency with bilateral posterior crossbite. What is the most appropriate
intervention?
a. Orthodontic palatal expansion
b. SARPE
c. Le Fort I advancement
d. Bilateral SSO mandibular setback
3. Which of the following orthognathic movements is likely to cause the appearance of facial aging?
a. Le Fort I advancement
b. Le Fort I with downward repositioning
c. Le Fort I with upward repositioning
d. Bilateral sagittal split and advancement
e. Advancement genioplasty
4. A 22-year-old woman comes to your office with concerns about her lower-third facial appearance,
specifically her weak chin. She has no concerns about her midfacial appearance. She has had
previous orthodontia and is in class I malocclusion. Her overall facial height is proportional in all
views, but on profile, her chin appears weak in an anterior-posterior direction. Cephalometric
analysis demonstrates that her SNA angle is 82° (normal 78–82) and her SNB angle is 80° (normal 78–
80). What is the most appropriate surgical management?
a. Bilateral sagittal split and advancement
b. Two-jaw surgery with maxillary and mandibular advancement
c. Vertical lengthening genioplasty and bone grafting
d. Horizontal sliding genioplasty and advancement
e. Le Fort I setback
5. A 25-year-old man is referred to your office for concerns about lower facial asymmetry. On
examination, his maxillary dental midline is in line with his overall facial midline, but his
mandibular dental midline is shifted 5 mm to the right. His chin point is in line with his mandibular
dental midline. With appropriate orthodontics, which orthognathic procedure would best treat this
patient’s lower facial asymmetry?
a. SSO with leftward shift alone
b. SSO with rightward shift alone
c. SSO with leftward shift and sliding genioplasty to the right
d. SSO with rightward shift and sliding genioplasty to the left
e. Le Fort I osteotomy with rightward shift
1. Answer: b. The patient has prognathism with an abnormal SNB angle. Mandibular osteotomies
and setback will address both the patient’s prominent chin and his malocclusion. Given that his SNA
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angle is within the normal range, Le Fort I maxillary advancement is not the ideal choice in this
scenario. Likewise, two-jaw surgery is unnecessary with a normal SNA angle and a relatively small
overall movement. Setback genioplasty may address the patient’s concerns about his prominent chin,
but will not address his malocclusion.
2. Answer: b. The key to this question is that the patient is skeletally mature. This means that the
palatal suture is likely to be fused and orthodontic palatal expansion alone is unlikely to correct her
transverse maxillary deficiency. Both Le Fort I and bilateral SSOs are incorrect because both
primarily address anterior-posterior discrepancies. SARPE involves surgically splitting the palatal
suture followed by orthodontic expansion. This will allow transverse expansion of the maxilla
without changes in the anterior-posterior direction.
3. Answer: c. All of the movements described expand the facial skeleton except for Le Fort I with
upward repositioning (impaction). Le Fort I impaction reduces facial height, but because the existing
soft tissue envelope is draped over a smaller skeletal frame, this may result in deeper rhytids and the
appearance of facial aging. In contrast, movements that expand the facial skeleton cause the existing
soft tissue envelope to be draped over a larger frame, softening and flattening rhytids.
4. Answer: d. The patient described has microgenia, but normal facial height and would benefit from
augmenting her chin by advancing it horizontally (a chin implant, although not one of the answer
choices, would also be a reasonable option). This patient has normal SNA and SNB angles on
cephalometric analysis and is in class I malocclusion; she also has no concerns about her midfacial
appearance. These factors all argue against two-jaw surgery with advancements; additionally, this
would not change her relative microgenia. Vertical lengthening genioplasty and bone grafting is not
indicated because her overall facial height is proportionate (vertical thirds are similar). Le Fort I
setback and bilateral sagittal split with advancement would both create malocclusion and neither
would address the patient’s weak chin.
5. Answer: a. This patient’s maxillary midline is coincident with his overall facial midline; only the
mandibular midline is malpositioned. Although Le Fort I osteotomy and rightward shift could align
the dental midlines, this would actually make the patient appear more asymmetric and is, therefore,
incorrect. Additionally, the patient’s chin point is coincident with his mandibular dental midline,
meaning that moving the mandibular dental midline into the correct position will move the chin
point at the same time, rendering genioplasty unnecessary. If the chin point was coincident with the
facial midline rather than the mandibular dental midline, a compensatory genioplasty in the opposite
direction of the mandibular movement would be needed. This leaves sagittal split alone as the only
option, and in this case the patient is already off to the right, so his midline needs to be shifted to the
left.
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References
Reyneke, JP. Surgical cephalometric prediction tracing for the alteration of the occlusal plane by means of rotation of the maxillomandibular
complex. Int J Adult Orthodon Orthognath Surg. 1999;14(1): 55-64.
Ricketts, RM. Cephalometric analysis and synthesis. Angle Orthod. 1961;31(3):141-156.This article describes the cephalometric tracings and analysis
that have come to be known as a Ricketts analysis for the diagnosis of dentofacial deformities. Ricketts also describes how cephalometric analysis relates
to, and is distinct from, treatment planning for orthognathic surgery.
Schendel, SA, Eisenfeld, J, Bell, WH, et al. The long face syndrome: vertical maxillary excess. Am J Orthod. 1976;70(4):398-408.
Reyneke, JP, Masureik, CJ. Treatment of maxillary deficiency by Le Fort I downsliding technique. J Oral Maxillofac Surg. 1985;43(11):914-916.
Wagner, S, Reyneke, JP. The Le Fort I downsliding osteotomy: a study of long-term hard tissue stability. Int J Adult Orthodon Orthognath Surg.
2000;15(1):37-49.
Selber, JC, Rosen, HM. Aesthetics of facial skeletal surgery. Clin Plast Surg. 2007;34(3):437-445.
Rosen, HM. Facial skeletal expansion: treatment strategies and rationale. Plast Reconstr Surg. 1992;89(5):798-808.This article describes Rosen’s
philosophical and clinical preference for skeletal expansion rather than contraction in orthognathic surgery. It emphasizes the paramount importance of
aesthetics over orthognathic perfection and illustrates this approach using case examples in which facial disproportion was created or maintained to
optimize the aesthetic outcome.
Bell, WH. Le Forte I osteotomy for correction of maxillary deformities. J Oral Surg. 1975;33(6):412-426.
Trauner, R, Obwegeser, H. The surgical correction of mandibular prognathism and retrognathia with consideration of genioplasty. Part I.
Surgical procedures to correct mandibular prognathism and reshaping of the chin. Oral Surg Oral Med Oral Pathol. 1957;10(7):677-689.In
the first of a series of classic articles, the authors describe various mandibular osteotomies that are still in use today, including the sagittal split and
inverted L sliding genioplasty technique that are also described.
Bell, WH. Surgical correction of mandibular retrognathism. Am J Orthod. 1966;52(7):518-528.
Bell, WH. Correction of mandibular prognathism by mandibular setback and advancement genioplasty. Int J Oral Surg. 1981;10(4):221-229.
Bell, WH, Jacobs, JD, Legan, HL. Treatment of the class II deep bite by orthodontic and surgical means. Am J Orthod. 1984;85(1):1-20.
Al-Moraissi, EA, Ellis, E III. Is there a difference in stability or neurosensory function between bilateral sagittal split osteotomy and intraoral
vertical ramus osteotomy for mandibular setback? J Oral Maxillofac Surg. 2015;73(7):1360-1371.
Converse, JM, Wood-Smith, D. Horizontal osteotomy of the mandible. Plast Reconstr Surg. 1964;34(5):464-471.
Posnick, JC, Choi, E, Chang, RP. Osseous genioplasty in conjunction with bimaxillary orthognathic surgery: a review of 262 consecutive
cases. Int J Oral Maxillofac Surg. 2016;45(7):904-913.
Trauner, R, Obwegeser, H. The surgical correction of mandibular prognathism and retrognathia with consideration of genioplasty. Part II.
Operating methods for microgenia and distoclusion. Oral Surg Oral Med Oral Pathol. 1957;10(8):
 787-792.
Bell, WH, Jacobs, JD, Quejada, JG. Simultaneous repositioning of the maxilla, mandible, and chin. Treatment planning and analysis of soft
tissues. Am J Orthod. 1986;89(1):28-50.Cephalometric and aesthetic analysis as well as indications for two-jaw orthognathic surgery are described.
Case examples illustrate the diagnostic, analytic, and orthodontic and surgical treatment processes.
Bell, WH, Sinn, DP, Finn, RA. Cephalometric treatment planning for superior repositioning of the maxilla and concomitant mandibular
advancement. J Maxillofac Surg. 1982;10(1):42-49.
Brammer, J, Finn, R, Bell, WH, et al. Stability after bimaxillary surgery to correct vertical maxillary excess and mandibular deficiency. J Oral
Surg. 1980;38(9):664-670.
Park, N, Posnick, JC. Accuracy of analytic model planning in bimaxillary surgery. Int J Oral Maxillofac Surg. 2013;42(7):807-813.
Perez, D, Ellis, E III. Sequencing bimaxillary surgery: mandible first. J Oral Maxillofac Surg. 2011;69(8):2217-2224.The article is a commentary on
mandible-first two-jaw orthognathic surgery and its advantages over more traditional maxilla-first approaches. Requirements for a mandible-first
approach as well as circumstances favoring mandible-first, unique aspects of planning, and disadvantages are also described.
Bradley, JP, Elahi, M, Kawamoto, HK. Delayed presentation of pseudoaneurysm after Le Fort I osteotomy. J Craniofac Surg. 2002;13(6):746-
750.
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C H A P T E R 3 2
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Craniofacial Clefts and Orbital Hypertelorism
Jesse A. Goldstein and Joseph E. Losee
Key Points
Treat each with a craniofacial cleft and/or hypertelorism individually
Soft tissue contours follow the underlying bony abnormality
Establishing the brain/nose barrier is critical early in life
Definitive surgical intervention for orbital hypertelorism should wait until the orbit has reached
maturity (at least 5 to 7 years of age)
Maintain medial canthal attachments if possible; if not, resuspend with transnasal wiring
No. 7 craniofacial clefts are the most prevalent
Orbital hypertelorism is a physical finding, not a syndrome
Craniofacial clefts and orbital hypertelorism (OHT) represent two distinct entities that have in common
an extreme variation in presentation. They span severity from subtle anomalies of little clinical
consequence to the most profound forms of craniofacial disfigurement encountered in plastic surgery.
With few exceptions, these entities are rare and result from uncharacterized disruptions during
embryogenesis; however, several well-defined syndromes and diagnoses exist whose primary
manifestation is hypertelorism or craniofacial clefting. As a whole, these entities should be thought of as
part of a phenotypical spectrum and not as those that share a clearly delineated etiopathogenesis. Even
so, developing a comprehensive framework for understanding, evaluating, and treating patients with
these complex anomalies is crucial to success. This chapter is meant to provide the framework for
assessing and categorizing these complex anomalies as well as provide some basic principles of treatment
that may be broadly applied.
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Tessier Classification
Craniofacial clefts are often referred to as Tessier clefts, a reference to Paul Tessier (1917–2008) who
founded the field of craniofacial surgery and developed a system for understanding these diverse entities.
1 The Tessier classification 2 is the most complete and commonly utilized framework today, describing
both the soft tissue anomalies and the underlying bony abnormalities. Tessier’s classification was a
synthesis of anatomic and clinical study of patients and was developed before the advent of computed
tomography (CT). 3 It has withstood the transition into the modern era of imaging including 3D CT and
magnetic resonance imaging technologies.
The Tessier classification of rare craniofacial clefts centers around the orbits and eyelids, which represent
the region of interface between the cranium and the facial structures (Figure 32.1). Therefore, the clefts
that occur cephalad to the orbits are defined as cranial clefts, and those occurring caudal to the orbit
represent the facial clefts. Tessier’s numbering system helps reinforce this point: facial clefts are
numbered 0 to 7, whereas cranial clefts are numbered 8 to 14. Furthermore, cranial and facial clefts often
occur in distinct patterns. When Tessier clefts occur in combination, they often include a facial cleft and a
cranial cleft such that the sum of the cleft numbers is 14. For example, a midline cleft may begin at the lip
and nose (no. 0 cleft) but extend up through the forehead (no. 14 cleft). Other common combinations of
craniofacial clefts include 1 and 13, 2 and 12, 3 and 11, etc. (Table 32.1) Craniofacial clefts rarely exhibit
the same extent of soft tissue and skeletal involvement. A diagnosis of a craniofacial cleft can be made
more reliably based on the skeletal manifestations which are more consistent than the soft tissue
manifestations.
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FIGURE 32.1 Tessier classification of craniofacial clefts. Soft tissue clefts are demonstrated on the left. The skeletal clefts are
depicted on the right. The facial clefts are numbered 0 through 7 and the cranial clefts are numbered 8 to 14.
TABLE 32.1
Comprehensive List of Craniofacial (Tessier) Clefts With Associated Bony and Soft Tissue Landmarks
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Features of Craniofacial Clefts
Median Clefts: No. 0 and No. 14 Clefts
Median craniofacial clefts represent a unique form of facial clefting which can be associated with both
hypoplasia of midline structures and tissue hyperplasia. The no. 0 clefts define the facial component of
the spectrum of median clefts, whereas the no. 14 clefts define the cranial, or frontoethmoidal, component
of median clefts. Median clefts can be further divided into hypoplasia or agenesis, dysraphia, and
hyperplasia. 4
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Median Craniofacial Hypoplasia
Hypoplasia associated with no. 0 clefts can range from small but normal to complete agenesis of the
midline facial structures, whereas their extension into a no. 14 cleft can present with mild or severe
hypotelorism and central nervous system (CNS) anomalies. Severe forms of median craniofacial
hypoplasia fall under the diagnosis of holoprosencephaly and include cyclopia, ethmocephaly with
midline proboscis, and cebocephaly, and there is a positive correlation between the severity of the facial
findings and degree of CNS anomalies resulting in significantly limited life expectancy or intrauterine
demise. In patients with milder forms, assessing CNS anatomy is crucial with CT or magnetic resonance
imaging to differentiate between lobar and alobar holoprosencephaly and to determine the integrity of
the hypothalamic-pituitary axis.
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Soft Tissue Deficiencies
No. 0 clefts can affect the soft tissues of the upper lip and nose and can range from agenesis to subtle
hypoplasia. A false median cleft lip may be present with absence of the philtrum and central tubercle. The
columella may be narrowed, shortened, or absent, and the nasal tip may lack projection because of a
hypoplastic or absent caudal septum. Hypotelorism and midline fusion of the eyebrows can occur in no.
14 clefts.
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Skeletal Deficiencies
As with all aspects of craniofacial clefts, the skeletal involvement in no. 0/14 clefts can range from subtle
separation between the maxillary central incisors to complete absence of the premaxilla and primary
palate resulting in a large central palatal cleft. Hypoplastic nasal structures can also be present including
septal, ethmoidal, and nasal bone deficiencies resulting in a flat nasal bridge, hypotelorism, and
microcephaly (Figure 32.2). Additionally, intrinsic dental anomalies may also be present including absent
or hypoplastic maxillary central incisors or a midline central incisor.
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FIGURE 32.2 No. 0–14 cleft. A. Skeletal involvement demonstrating midline palatal hypoplasia and hypotelorism. B. Patient
with midline craniofacial hypoplasia associated with a false medial cleft lip, absent columella, flat nasal bridge, and
hypotelorism. C. Skeletal involvement demonstrating midline craniofacial hypertrophy and hypertelorism. D. Patient with midline
craniofacial hyperplasia associated with hypertelorism and excess and disorganized nasal cartilage.
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Midline Craniofacial Dysraphia
Dysraphia represents a failure of fusion, or cleft, of midline structures without deficiency or excess. The
two diagnoses associated with a no. 0/14 cleft are a median cleft lip and an encephalocele.
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Soft Tissue Involvement
This includes a true median cleft lip where all central structures are present. These clefts represent an
embryological failure of fusion of the paired medial nasal processes and results in a narrow median cleft
with present philtral columns. An encephalocele is a herniation of CNS structures including brain and
cerebrospinal fluid through a weakened midline space and can result in a mass effect and further
separation of normal facial structures.
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Skeletal Involvement
True median clefts can result in a midline alveolar cleft and a midline primary palatal cleft, as well as
hypertelorism if the cleft extends cephalad toward the orbits. Anterior encephaloceles can be divided into
basal encephaloceles, where the CNS herniates through a defect in the cribriform plate or sphenoid bone
into the ethmoidal space and frontoethmoidal encephaloceles, where the herniation occurs anterior to the
crista galli at the junction of the frontal and ethmoidal bones.
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Midline Craniofacial Hyperplasia
The spectrum of midline craniofacial hyperplasia ranges from clinically minor duplication of the nasal
septum and subclinical hypertelorism to severe forms of frontonasal dysplasia together with severe
hypertelorism and nasal duplication. 5–7
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Soft Tissue Involvement
Soft tissue excess may manifest as a widened philtrum, a bifid nasal tip or duplication, and abnormal
nasal cartilage as well as widely spaced nasal soft tissue structures (Figure 32.2).
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Skeletal Involvement
Skeletal excess in a hyperplastic no. 0/14 cleft can range from a widening of the maxillary alveolar process
resulting in a central incisor diastema to a widened and shortened central dental arch resulting in an
anterior open bite. A duplicated nasal spine may be present, and the nasal bones and maxillary processes
of the maxilla may be flat, broad, and laterally displaced. Hypertelorism and frontal bossing/diastasis
may be present because of varying degrees due to widening of the ethmoid sinuses and anterior cranial
fossa, while the cribiform plate is low.
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No. 1 Cleft
Soft Tissue Involvement
No. 1 Tessier clefts can often be confused with common cleft lips, as the cleft passes through Cupid’s bow
to the nasal base. The distinguishing feature of a no. 1 cleft, however, is the involvement of the medial
nose including notched soft triangle and alar dome. This cleft can extend up the nose, lateral to midline,
and be associated with soft tissue furrows and medial canthal displacement resulting in telecanthus
(Figure 32.3). The eyelids are unaffected. No. 1 clefts can continue cephalad as no. 13 clefts, which can
result in orbital dystopia.
FIGURE 32.3 No. 1 cleft. A. Skeletal involvement is through the piriform aperture just lateral to the nasal spine and septum.
The orbit is displaced laterally. B. Patient with a notched alar dome and mild orbital dystopia.
(Reproduced from Losee, JE, Kirschner, RE. Rare craniofacial defects. In: Comprehensive Cleft Care. Vol 2. 2nd ed. New York, NY: Thieme Medical
Publishing; 2016
.)
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Skeletal Involvement
An anterior open bite and keel-shaped maxilla are present, whereas a paramedian cleft may extend from
between the central and lateral incisor to the nasal floor lateral to the nasal spine. A primary and
secondary palatal cleft may be present. The cephalic extension of the cleft occurs between the nasal bone
and the frontal process of the maxilla and can result in flattening and widening of the nasal dorsum.
Widened ethmoids can result in hypertelorism.
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No. 2 Cleft
Soft Tissue Involvement
Similar to no. 1 clefts, no. 2 Tessier clefts can also mimic common cleft lips. They are also paramedian,
originating at the peak of Cupid’s bow before extending up to the nasal sill. Unlike no. 1 clefts, however,
no. 2 clefts pass through the middle third of the ala, lateral to the soft triangle, resulting in a hypoplastic
ala rather than a notch. The nose can be broad and flat, and the medial canthus can be displaced. The
lacrimal system and eyelids are unaffected (Figure 32.4). No. 2 clefts can continue cephalad as no. 12
clefts, which can lead to orbital dystopia and medial brow distortion.
FIGURE 32.4 No. 2 cleft. Patient with a lateral alar deficiency and mild orbital dystopia.
(Reproduced from Losee, JE, Kirschner, RE. Rare craniofacial defects. In: Comprehensive Cleft Care. Vol 2. 2nd ed. New York, NY: Thieme Medical
Publishing; 2016
.)
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Skeletal Involvement
This cleft originates lateral to the lateral maxillary incisor and extends up to the piriform aperture and
primary/secondary palate similar to a common cleft lip/palate. Similar to a no. 1 cleft, the cephalic
extension of the no. 2 cleft occurs between the nasal bone and the frontal process of the maxilla and can
result in flattening and widening of the nasal dorsum as well as ethmoidal involvement and
hypertelorism.
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No. 3 Cleft
The no. 3 cleft is a common Tessier cleft and is often referred to as a Tessier oro-naso-ocular cleft (Figure
32.5).
FIGURE 32.5 Right no. 3 cleft; left no. 4 cleft. A. Skeletal involvement demonstrates deformity of right medial maxilla and
inferior orbital rim/floor with orbital dystopia and left-sided involvement lateral to the piriform extending to the inferior orbital
rim/floor with orbital dystopia. B. Patient with deficiency extending from the alveolus into the piriform with microphthalmia and
orbital dystopia. C. Patient with mild no. 4 cleft demonstrating cleft lip lateral to the nasal sill and subtle inferior displacement of
the medial canthus.
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Soft Tissue Involvement
When isolated to the lip, no. 3 clefts manifest as a common cleft lip, starting at Cupid’s bow peak and
extending to the lateral nasal sill. The cleft extends through the lateral ala, which can be notched or
clefted, to the lower eyelid between the medial canthus and the inferior punctum. There can be a
significant soft tissue deficit in the midface with inferior displacement of the lower lid and globe, leading
to a risk of exposure keratopathy. The globe may also be microphthalmic in some cases.
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Skeletal Involvement
Skeletal involvement of the no. 3 cleft begins at the alveolar process, between the lateral incisor and
canine, extending up to the lateral piriform aperture and into the medial orbit. The medial wall of the
maxillary sinus is absent. The frontal process of the maxilla is disrupted as the bony component of the
cleft terminates in the lacrimal groove in the inferior-medial orbit.
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No. 4 Cleft
Soft Tissue Involvement
As with other craniofacial clefts, soft tissue involvement in no. 4 clefts presents as a wide spectrum of
deformities. 8 The cleft begins lateral to the peak of Cupid’s bow, midway between the commissure and
the philtrum. This is the first of the clefts to travel lateral to the nose, although nasal anatomy can still be
indirectly displaced superiorly. The cleft extends to the lower lid lateral to the medial canthus, which is
displaced inferior-laterally, and can involve the lacrimal system at the level of the punctum and the
lacrimal sac. The distance between the lower eyelid and the upper lip is foreshortened and can result in
significant corneal exposure (Figure 32.5).
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Skeletal Involvement
The skeletal involvement affects the inferior maxilla similarly to a no. 3 cleft; however, the no. 4 cleft
courses lateral to the piriform region through the maxillary sinus, including the palate and choanae,
superiorly to the infraorbital rim between the frontal process of the maxilla and the infraorbital foramen.
The lacrimal system is usually spared. Variable orbital floor defects can be present.
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No. 5 Cleft
No. 5 clefts are the least common of the oblique facial clefts.
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Soft Tissue Involvement
Soft tissue involvement begins medial to the lateral oral commissure and proceeds cephalad toward the
lower eyelid in the middle third. Similar to no. 4 clefts, there is often a soft tissue deficiency between the
mouth and the lower lid, resulting in higher risk of corneal exposure (Figure 32.6).
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FIGURE 32.6 Right no. 5 cleft; left no. 6 cleft. The right no. 5 cleft is demonstrated by a subtle soft tissue furrow that begins
medial to the oral commissure and extends toward the middle lower lid. The no. 6 cleft begins at the commissure and extends
toward the lateral lower lid.
(Reproduced from Losee, JE, Kirschner, RE. Rare craniofacial defects. In: Comprehensive Cleft Care. Vol 2. 2nd ed. New York, NY: Thieme Medical
Publishing; 2016
.)
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Skeletal Involvement
The no. 5 cleft affects the alveolus lateral to the canine and extends through the maxillary sinus by
encountering the inferolateral orbit lateral to the infraorbital foramen.
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No. 6 Cleft
The no. 6 cleft is associated with mild forms of Treacher Collins syndrome and Nager syndrome, both of
which present with zygomaticomaxillary dysplasia.
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Soft Tissue Involvement
The soft tissue involvement often presents as a vertical cleft or furrow originating in the oral commissure
and extending cephalad to the lateral lower eyelid. This can result in inferior displacement of the lateral
canthus, ectropion, and an oblique orientation of the palpebral axis, oftentimes referred to as an
antimongoloid slant. Colobomas can be present at the inferior lateral eyelid (Figure 32.6).
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Skeletal Involvement
Skeletal involvement tracks along the zygomaticomaxillary suture and excludes the alveolar process. The
posterior maxillary buttress (pterygomaxillary buttress) can be foreshortened resulting in a steep
maxillary occlusal plane and choanal atresia. The cephalad extend of the no. 6 cleft is the lateral orbital
floor with a communication with the inferior orbital fissure.
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No. 7 Cleft
No. 7 clefts are by far the most common of the craniofacial clefts. When they occur in isolation, they result
in isolated macrostomia (Figure 32.7) often associated with a preauricular remnant. Conversely, they can
occur as part of a known syndrome such as hemifacial macrosomia or Goldenhar syndrome which are
associated with isolated no. 7 clefts, 9–12 or Treacher Collins and Nager syndromes which are associated
with no. 6 to 8 craniofacial clefts.
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FIGURE 32.7 Left no. 7; right no. 8 cleft. A. Skeletal involvement demonstrates deformity of the lateral alveolus, extending
through the pterygomaxillary junction to the zygomatic arch and involvement of the lateral orbital rim centered around the
zygomaticofrontal suture. B. The right soft tissue no. 7 cleft is shown with obliteration of the lateral commissure and a
preauricular skin tag. C. Left no. 8 cleft is shown with subtle changes at the lateral canthal region including brow malposition.
(B,C. Reproduced from Losee, JE, Kirschner, RE. Rare craniofacial defects. In: Comprehensive Cleft Care. Vol 2. 2nd ed. New York, NY: Thieme Medical
Publishing; 2016
.)
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Soft Tissue Involvement
The no. 7 cleft begins at the lateral commissure and extends to a variable extent posteriorly to the
preauricular region. Most commonly, the cleft terminates medial to the masseter. A preauricular remnant
may be present as well as varying degrees of microtia. Cranial nerve V may be involved as can the tongue
and palate on the affected side.
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Skeletal Involvement
The skeletal cleft passes through the pterygomaxillary junction and may affect the ipsilateral posterior
maxillary and mandibular ramus, which can be vertically foreshortened contributing to a steel occlusal
plane. The zygomatic body is variably dysplastic with an incomplete or absent arch. The cranial base can
also be affected leading to asymmetry of the glenoid fossa and sphenoid bone.
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No. 8 Cleft
The no. 8 cleft divides the facial clefts from the cranial clefts and is centered around the frontozygomatic
suture. It is the lateral most of the cranial clefts. It is associated with several syndromes including
Treacher Collins and Goldenhar syndromes.
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Soft Tissue Involvement
The no. 8 cleft extends from the lateral canthus laterally to the temporalis region and can be associated
with a coloboma of the iris or lateral canthal region. 13 In Goldenhar syndrome, an epibulbar dermoid cyst
is present on the globe (Figure 32.7).
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Skeletal Involvement
The no. 8 cleft is a cleft in the frontozygomatic suture.
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Treacher Collins and Nager Syndromes
Treacher Collins and Nager syndromes are discussed elsewhere in this text. However, it is important to
present these anomalies here to demonstrate how this specific syndrome relates to the Tessier
classification of craniofacial clefts. It was Tessier who first attributed the no. 6, 7, and 8 cleft to Treacher
Collins syndrome. 14,15 The classic findings of Treacher Collins syndrome include zygomatic hypoplasia
(no. 6 cleft), vertical pterygomaxillary deficiency with a steep occlusal plane (no. 7 cleft), inferior-lateral
decent of the lateral canthus (no. 7 cleft), and absence of the inferior and lateral orbit as zygomatic arch
(no. 7 and 8 clefts). Nager syndrome has a similar phenotype to Treacher Collins syndrome but also
includes limb anomalies. 16
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No. 9 Cleft
The no. 9 cleft is the rarest of the isolated cranial clefts and begins the medial movement of the cranial
clefts. This cleft has been termed fronto-sphenoid dysplasia and is the cranial extension of the no. 5 cleft.
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Soft Tissue Involvement
Soft tissue involvement includes abnormalities in the lateral third of the upper eyelid and brow. It
extends up to the temporoparietal hairline which can be anteriorly displaced with a projection of hair in
the temporal region. The orbit may be displaced laterally and microphthalmia may also be present. The
frontotemporal branch of the facial nerve can be affected (Figure 32.8).
FIGURE 32.8 Left no. 9; right no. 10 cleft. A. Skeletal involvement of the no. 9 cleft demonstrates disruption of the
superolateral orbit and frontotemporal region. Skeletal involvement of the no. 10 cleft originates with the superior orbital fissure
and extends through the central supraorbital rim into the frontal bone. B. Patient with no. 9 cleft demonstrating orbital rim, brow,
and forehead irregularities. C. Patient with bilateral no. 10 clefts demonstrating soft tissue colobomas of the medial third of the
upper eyelid and brown and forehead involvement.
(B. Reproduced from Losee, JE, Kirschner, RE. Rare craniofacial defects. In: Comprehensive Cleft Care. Vol 2. 2nd ed. New York, NY: Thieme Medical
Publishing; 2016
.)
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Skeletal Involvement
The no. 9 cleft extends superiorly through the superior-lateral orbit displacing the greater wing of the
sphenoid and the squamosal temporal bone. This can lead to posterior rotation of the lateral orbital wall.
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