Textbook of Plastic, Reconstructive,

Agrawal
and Aesthetic Surgery
Volume III

Head and Neck

Textbook of Plastic, Reconstructive, and Aesthetic Surgery is a comprehensive and illustrated work for students,

Reconstruction
Head and Neck
teachers, and practicing surgeons. It is a six-volume set with the topics of the volumes based on the clinical needs
of plastic surgeons in developing countries. Along with latest developments, these volumes incorporate landmark

Reconstruction
contributions, innovations, and techniques used by Indian clinicians who regularly deal with unique and complicated
conditions. While a majority of authors have been chosen from the Indian subcontinent, a few international authors
whose work is considered indispensable for understanding the subject have also been included.

The six volumes seek to incorporate in one work issues relevant to the developing world as well as insights from
national and international plastic surgery practices which highlight newer management techniques along with
traditional methods. This makes these volumes a “must-have” resource for students and practitioners of plastic
surgery across the globe.

This ‘Head and Neck Reconstruction’ volume covers a wide spectrum of topics. It covers surgery techniques
for reconstructions of clefts, treatment of craniofacial anomalies, onco-reconstruction, occuloplasty, and
reconstruction of ear and nose. The team of authors has been carefully selected and they are highly experienced
in their respective fields. The book is divided into three sections. The cleft section covers the entire gamut of clefts
in detail, including operative steps and clinical photographs. The second and the third sections cover the recent
advances in craniofacial anomalies and reconstruction techniques of head and neck section, respectively. Each topic
of the book is a must read for any plastic surgeon. Editor-in-Chief
Salient features: Karoon Agrawal
• Includes important chapters on topics like “approach to the patients of cleft” and “aesthetic onco-
reconstruction”.
• Provides historical background of each topic along with recent advances and clinically relevant information.
• Untangles the mysteries of decision making in many areas of head and neck reconstruction.
Volume Editor

Volume III
• Adequate emphasis is given to the basic sciences relevant to each chapter.

Surajit Bhattacharya
Karoon Agrawal is currently Director Professor of Burns, Plastic, and Maxillofacial Surgery at Safdarjung Hospital
and Vardhman Mahavir Medical College, New Delhi, India. He was the President of the Association of Plastic
Surgeons of India (2016); President of the National Academy of Burns-India (2010); and President of the Indian
Society of Cleft Lip Palate and Craniofacial Anomalies (2009). Section Editors
Surajit Bhattacharya is currently the President of Association of Plastic Surgeons of India and Senior Consultant in
Plastic, Reconstructive, and Aesthetic Surgery at Sahara Hospital, Lucknow, Uttar Pradesh, India. He is trained at
Rajesh Powar
King George Medical University, Lucknow and has undergone fellowship in Micro-, Hand, and Craniofacial Surgeries
from Australia.
Alok Sharma
Rajesh Powar is currently working as Professor and Head of Plastic Surgery and Project Director of KLES Smile Train Gautam Biswas
Project at Jawaharlal Nehru Medical College, Belgaum, Karnataka, India.

Alok Sharma retired as commandant of a multi-speciality Military Hospital at Jabalpur, Madhya Pradesh, India.

Gautam Biswas is presently working as a Consultant at Tata Medical Centre, Kolkata, West Bengal, India, a tertiary
care oncology centre, established in 2012.

ISBN 978-93-88257-15-2

9 789388 257152 www.thieme.in

Agrawal_Textbook Plastic Surgery_Vol III_k6_spine42.indd 1 30.10.2018 09:43:51

21
Hypertelorism
Ramesh K. Sharma

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¾¾ Introduction •• Nasal Augmentation
¾¾ Some Definitions •• Management of Excessive Skin
¾¾ Normal Measurements •• Canthopexy
¾¾ Classification of Hypertelorism

m
¾¾ Pathology in Orbital Hypertelorism

¾¾ Relevant Embryology

¾¾ Clinical Variants of Hypertelorism

¾¾ Indication of Surgery in Orbital Hypertelorism

¾¾ Essentials of Hypertelorism Surgery

¾¾ Illustrative Case Examples

•• Symmetrical Hypertelorism

•• Asymmetrical Hypertelorism

•• Facial Bipartition

¾¾ Pseudo-hypertelorism (Telecanthus)

•• Naso-orbito-ethmoid Fracture
ie
¾¾ Timing of Surgery
•• Nasofrontal Meningoencephalocele
¾¾ Selection of Surgical Procedure

•• Orbital Box Osteotomy •• Craniofacial Fibrous Dysplasia

•• Facial Bipartition •• Ethmoid Tumors

•• Subcarinal U-Shaped Osteotomy ¾¾ Complications

◊ Prevention of Ascending Infection ¾¾ Summary and Conclusion

Th
458 Craniofacial Surgery
Introduction
Hypertelorism is a Greek word that means “widely apart,”
and the term can be applied to any paired bodily parts, such
as the eyes, breasts, kidneys, etc.1 In fact, Greig used the term
“ocular hypertelorism” in 1924 to denote increased distance
between the eyes.2 He used the distance between the two
pupils to qualify this condition. However, it was soon realized
that the interpupillary distance (IPD) can be increased even
in the absence of any bony socket abnormality. Therefore,
Tessier suggested term “orbital hypertelorism” (ORH) that
denotes “a true lateralization of the whole of the bony orbit
in a way that the distance between the lateral canthus and
auditory meatus is shortened.”3 Another acceptable term for
this condition is “teleorbitism.”
Some Definitions
m
The term hypertelorism should not be used loosely and
should strictly be used in situations in which the whole of
orbits have moved laterally resulting in increased inter­
canthal distance (ICD) between the two medial canthi and
two lateral canthi.
Fig. 21.1 graphically explains the various terminologies
used in description of hypertelorism. The distance between
two medial canthi is known as (medial) ICD. In normal
individuals, the distance of medial canthus from midline
ie
on either side is equal. However, it can increase on one or
OCD
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ICD
IPD
PFL
Fig. 21.1 Various measurements for hypertelorism. ICD,
(medial) intercanthal distance; IPD, interpupillary distance;
OCD, outer canthal distance; PFL, palpebral fissure length.
both sides following trauma to the naso-orbito-ethmoid
(NOE) region or in cases of NOE encephaloceles. The isolated
increased medial ICD without any change in other values
is called as “telecanthus” or “pseudo-hypertelorism.” The
palpebral fissure length (PFL) on both sides is similar on both
sides in normal individuals. However, it may be decreased in
cases of telecanthus. The outer canthal distance is designated
as OCD. The distance between the two pupils in front gaze
is called as IPD. The OCD and IPD remain normal in all cases
of telecanthus. However, in cases of true hypertelorism (also
known as ORH or teleorbitism), all the ICD, OCD, and IPD are
increased. In other words, there is true lateralization of the
orbit in such cases. This can be uni- or bilateral.
The normal intercanthal space is maintained by the
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ethmoid sinuses on both sides. Fig. 21.2 graphically shows
the anatomy of the intercanthal space—the ethmoids, crista
galli, and nasal septum.
Fig. 21.3 is a diagrammatic representation of the normal
orbit, the orbit with telecanthus and the orbit with hyper­
telorism. The IPD remains normal in telecanthus whereas
the IPD increases in hypertelorism. In telecanthus, only the
medial ICD increases whereas in ORH, all the distances such
as medial ICD, IPD, and OCD increase. The PFL would remain
normal in a classic case of hypertelorism.
Normal Measurements
Excessive (medial) ICD or hypertelorism may be mild
(30–34 mm), moderate (35–39 mm), or severe (> 40 mm).
In the growing child, anything more than 25 mm may be
considered as hypertelorism. More specific information
on bony interorbital distance by age and sex in growing
children can be found in normative data tables such as that
provided by Waitzman et al.4
Fig. 21.2 Diagrammatic representation of the normal
interorbital space. Ethmoid sinuses are present in this
space. Please note that the medial walls are almost parallel
throughout the anteroposterior extent.
 Hypertelorism 459

Normal ICD
Normal OCD
Normal orbit
Normal IPD
Normal PFL

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Normal medial canthus left side
Laterally pushed medial canthus right side
Telecanthus right Normal IPD
Normal OCD
Small PFL right side
Normal PFL left side

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Telecanthus bilateral
Laterally pushed medial canthus right side
Laterally pushed medial canthus left side
Normal IPD
Normal OCD
Small PFL both right and left side
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Increased medial ICD right side
Hypertelorism right side Normal medial ICD left side
Th

Increased OCD
Increased IPD
Normal PFL

Increased medial ICD both side

Increased OCD
Increased IPD
Normal PFL
Hypertelorism bilateral

Fig. 21.3 A diagrammatic representation of the normal orbit, orbit with telecanthus, and orbit with hypertelorism. In
telecanthus only the medial intercanthal distance (ICD) increases whereas in hyper­telorism ICD, outer canthal distance (OCD),
and intercanthal distance (IPD) increase. PFL, palpebral fissure length.
460 Craniofacial Surgery
Classification of Hypertelorism
The hypertelorism may be classified in a variety of ways.
Tessier5 classified it on the basis of ICD as follows:
••Type I: 30–34 mm
••Type II: 35–39 mm
••Type III: > 39 mm
Munro and Das have classified the hypertelorism on the basis
of the medial wall deformities.6 Type I has parallel medial
walls. In type II, the medial wall is wedge shaped posteriorly.
It is oval shaped in type III and is wedged anteriorly in type
IV (Fig. 21.4). Types I and II are the most common varieties
seen in almost in all cases. Another descriptive classi­fication
is by axial rotation.6 Orbital displacement may be only uni­
axial in the lateral plane. More frequently, there is polyaxial
displacement with the lateral orbital wall rotated posteriorly.
There may also be vertical rotation or displacement, causing
three-dimensional malposition of the orbit.
m embryo (Fig. 21.5). The angle between the optic nerves
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Fig. 21.4 Munro classification based on the shape of medial
wall on CT scans. Upper row left type I, upper row right type
II, lower row left type III, and lower row right type IV.
Th
Fig. 21.5 Embryology of hypertelorism. Orbits are laterally placed in the early embryo (upper row). These shift medially
around 28 mm embryo stage (lower row).
Pathology in Orbital Hypertelorism
The orbits become lateralized in ORH because of the
increased size of the ethmoid sinuses, and this increase is
confined to the anterior half of the ethmoids; the size of
posterior half is normal in almost all situations with ORH.
The size of the cribriform plate and the distance between the
optic foramina are also placed normally in almost all of the
cases.7 It means that one needs to address only the anterior
part of the interorbital space to correct the deformity in
hypertelorism. Hwang et al have reported high incidence
of epiblepharon, astigmatism, and large-angle extrophia in
children with hypertelorism.8
e
Relevant Embryology
The orbits in the early embryonic stage are laterally placed,
and these start moving toward midline around 28-mm-sized
at the chiasma in the first week is about 180 degrees, and
this then gradually decreases to about 70 degrees.9 This
arrested development can happen because of a variety
of reasons. The NOE complex fails to move toward the
midline probably because of defective development of
the nasal capsule.10 It is also possible to have inherent
deficient movement of the NOE complex per se.11 Premature
ossification of the lesser wings of the sphenoid wings could
also be a reason for continuation of the fetal position of the
orbits in the hyperteloric position.12 Persistent embryonic
position of the frontonasal process can also prevent the
lateromedial movement of the optic placodes resulting in
hypertelorism.13 In cases of Apert’s and Crouzon’s syndrome,
the disturbed development of the anterior skull base can
Normal embryo around 28 mm size
Eye placodes placed laterally
Medial movements of the eye placodes as the
developing embryo grows
 Hypertelorism 461

lead to craniosynostosis and hypertelorism. The widow’s
peak deformity of the forehead hairline results as the fields
of “hair suppression” are kept apart because of ORH.14
Clinical Variants of Hypertelorism
Hypertelorism is not a specific disease but is a clinical
manifestation that can be seen in a variety of conditions
such as craniofacial clefts, craniofacial dysplasias, and
craniosynostosis syndromes such as Apert’s and Crouzon’s
syndromes (Fig. 21.6). It can be bilateral or may be present
only on one side. In addition to the lateralization of the
orbit, there can be associated orbital dystopias as well.
Fig. 21.7 shows examples of the ORH with craniosynostosis
and orbital dystopia. The increased medial ICD alone
without any change in OCD or IPD may be seen in cases
of meningoencephaloceles, following trauma or tumors in
the NOE region. In such situations, the telecanthus can give
appearance of wide-set eyes but is in reality a “pseudo-
hypertelorism.” Fig. 21.8 shows various examples of
telecanthus.

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Fig. 21.6 Various conditions in which types of hypertelorism can be present. Upper row shows
a case of hypertrophic craniofacial dysplasia with bilateral hypertelorism. Middle row shows a
craniofacial cleft with hypertelorism. Lower row shows a unilateral hypertelorism.
462 Craniofacial Surgery

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Fig. 21.7 Examples of the ORH with craniosynostosis and orbital dystopia .Upper row shows marked orbital
dystopia in a case of hypertelorism. Middle row shows a case with plagiocephaly and hypertelorism. Lower row
shows a case with plagiocephaly, hypertelorism, and orbital dystopia.
 Hypertelorism 463

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Fig. 21.8 Examples of pseudo-hypertelorism or telecanthus. Upper row shows medial intercanthal widening in a case of
fibrous dysplasia. Middle row shows example of an encephalocele resulting in lateral movement of medial orbital walls on both
sides leading to telecanthus. Lower row shows telecanthus on right side in a case of naso-orbito-ethmoid fracture.
464 Craniofacial Surgery
Table 21.1 Syndromes associated with hypertelorism
Syndrome General clinical features
Apert’s syndrome Craniosynostosis
Syndactyly
Crouzon’s syndrome Craniosynostosis
Limb anomalies
Jackson-Weis syndrome (JWS), Craniosynostosis, ear abnormalities
ptosis, and hypertelorism
Saethre-Chotzen syndrome Craniosynostosis, ear abnormalities
Waardenburg’s syndrome Iris abnormality
Deafness
Coffin-Lowry syndrome X-linked mental retardation
Stickler’s syndrome Midface hypoplasia, cleft palate
hearing loss
Source: Modified from Dollfus and Verloes.15
m
Syndromes associated with hypertelorism are listed in
Table 21.1.
Indication of Surgery in Orbital
Hypertelorism
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In most patients, mental and physical development is
normal. The patients may have issues with the body image.
The corrective surgery is indicated primarily for cosmetic
reasons. The surgical correction can help the patients get
integrated into the society.
Till recently, surgical treatment was not possible for the
correction of the ORH. It was the pioneering work of Paul
Tessier that laid the foundations of the modern treatment of
Th
hypertelorism in early sixties. He developed the combined
intra- and extracranial technique for the correction of the
deformity.16,17 Dr. Tessier demonstrated that it was possible
to move the orbits in three dimensions without causing
any deleterious effects on the vision. He also showed that
inherent laxity of optic nerve permits a movement of the
orbit. He proved that extensive areas of the craniofacial
skeleton can be de-vascularized, can be manipulated on a side
table, and then be put back into the body. He demonstrated
that all these reassembled bones would survive completely
if a healthy vascularized lining and cover are present. Later
Tessier,3,18–21 Converse et al,22 van der Mulen23,24 Ortiz-
Monasterio et al,25 and Marchac et al26 further refined the
technique of correcting the hypertelorism. These pioneers
visualized the bony orbit consisting of two parts: the outer
square-shaped box containing the globe and inner cone-
shaped part housing the optic nerve. If these parts can be
separated from each other by making bony cuts, the outer
box can be moved in three dimensions without adversely
affecting the vision (Fig. 21.9).
Orbital manifestations
Hypertelorism, proptosis, orbital asymmetry,
strabismus
Hypertelorism, proptosis, orbital asymmetry
Ptosis, hypertelorism
Dystopia canthorum
Hypertelorism, downslanting palpebral fissures,
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eye protrusion
Hypertelorism, downslanting palpebral fissures,
eye protrusion
Essentials of Hypertelorism Surgery
The main aim is to bring a better aesthetic appearance to
the face. This can be achieved by carrying out the following
steps during the surgery:
••Bring the lateralized bony orbits closer to midline.
••Remove excess tissue in the midline. This includes
both the soft tissue and bones.
••Correction of orbital dystopias, if any.
••Create a nose that is having adequate projection and a
pleasant nasofrontal takeoff angle.
••Correction of any abnormally located tuft of hair. In
many situations, the eyebrows may also be abnormally
located. These would also need to be addressed.
The surgery needs to be performed both extra- and
intracranially. A coronal incision is used in all the cases.
The periorbital soft tissue is degloved circumferentially
exposing the roof, medial wall, and floor. The zygomatic
arch is also exposed. Addition of either a transconjunctival
or a subciliary incision along with the coronal incision gives
access to the aforementioned areas.
Timing of Surgery
The anxious parents may insist for a surgical correction
at the earliest. However, because the operation involves
placing of bony cuts in the teeth-bearing segments of the
maxilla, it may be advisable to delay the surgery until the
unerupted maxillary teeth have descended enough to
permit such a maneuver without causing any damage to
the teeth elements. It may therefore be advisable to delay
the surgery to about 6 to 7 years of age. However, an ugly
soft tissue excess can be managed by an early excision
(Fig. 21.10). It may also be sometimes advisable to augment
 Hypertelorism 465

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m
Fig. 21.9 The orbit can be visualized as consisting of a square-shaped outer bony box that houses a cone containing
optic nerve and the globe. The square can be moved in three dimensions independent of the cone without having any
deleterious effect on the contents of the cone.
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Fig. 21.10 Excess ugly looking skin can be

excised even at a young age of a few months
if the parents are worried. The nasal dorsum
has also been augmented with a split cranial
bone graft. The definitive surgery would be
undertaken at the age of about 6 years or so.
466 Craniofacial Surgery
the nasal dorsum with a bone graft on the nose to have some
semblance of nasal projection. The definitive surgery can
then be performed at an appropriate age.
Selection of Surgical Procedure
The orbital movements can be accomplished by either an
“orbital box osteotomy” or a “facial bipartition” technique.
Three specific indications for these procedures are described
as follows:
Orbital Box Osteotomy
It is performed when the occlusion is normal. This procedure
does not result in any change in the volume of the orbital
sockets. This osteotomy involves en bloc movement of
the orbits medially into the space created by resection of
abnormally wide nasal bone and ethmoid sinuses. Tessier
m
advocated keeping a supraorbital bar to guide the medial
movement of the mobilized orbits.3,16–19 The circumferential
box osteotomy permits translational movement along either
horizontal or vertical axes as per requirement. The cuts in
the infraorbital margin are made below the infraorbital
foramen. This surgery is usually undertaken at the age of
around 6 to 7 years so that all the unerupted maxillary teeth
have descended. A bilateral frontal craniotomy is performed,
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Th
and the frontal lobes are retracted gently, thereby providing
access to the orbital roof and cribriform region. Either a
transconjunctival or subciliary incision is given to gain
access to the floor and infraorbital margin. All the soft tissue
is separated from the orbital roof, lateral wall, medial wall,
and floor. The proposed markings for the orbital osteotomy
are shown in Fig. 21.11. The orbital roof is cut about 2 cm
from the supraorbital margin. Medially this cut stops short
of the cribriform region to save olfactory nerves. Another cut
is given in the medial wall staying posterior to the lacrimal
crest to avoid any damage to the nasolacrimal duct and
lacrimal sac. Laterally, the lateral orbital margin is split in
the sagittal plane. Many surgeons, however, prefer to go
through the greater wing of sphenoid as it is technically
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much easier than sagittal split of the lateral orbital wall.
The cut in the infraorbital region is below the infraorbital
nerve exiting from the infraorbital foramen. This cut in
the infraorbital margin extends on to the floor of the orbit.
The desired bony resection is marked on the dorsum of the
nose. The excess nasal bones, frontal process of maxilla,
nasal septum, and enlarged ethmoid sinuses are removed.
After completion of these steps, the bony orbits can now
be moved medially. The mobilized orbits are held in place
either with plates and screws or by wires. The medial shift of
the orbits results in bony gaps in the lateral orbital wall, and
these gaps are bone grafted. The split cranial bone is a good
option for this purpose.
Fig. 21.11 Box osteotomy. Upper
row left shows symmetrical hyper­
telorism with normal occlusion and
level maxillary arch. Upper row right
shows the planned resection in the
midline region. Lower row left shows
the markings for the box osteotomy.
The dotted lines depict the cuts in the
roof, medial wall, and floor and lateral
walls. Lower row right shows the final
appearance.

Facial Bipartition
The facial bipartition, proposed by van der Meulen,23,24 is
indicated in situations in which the occlusion is angulated
such as in cases of Apert’s syndrome or some craniofacial
clefts. These cases with an inverted V-shape occlusion have
a shortened midface. The facial bipartition procedure would
level the occlusion and lengthen the midface. The medial
resection of the nasal dorsum is in a V-shaped form, and
the palatal bone is split in the midline. The lateral cuts go
through the zygomatic arch and pterygomaxillary junction
(Fig. 21.12). The ensuing rotation allows correction of the
narrow maxillary arch and also widens the nasal fossae and
improves breathing. It would also result in change of the
orbital axis. Facial bipartition would also increase the orbital
volume and may improve the proptotic eyes seen in many of
these cases. This procedure can be performed at an early age.
In facial bipartition cases, no frontal bar is preserved so
that unhindered rotation of the facial halves is possible.
m
However, in box osteotomy the frontal bar is preserved to
guide the medial movement of the orbit. This would prevent
any inadvertent rotational deformity.
Subcarinal U-Shaped Osteotomy
This was described by Raveh and Vuillemin.27,28 In milder
varieties of hypertelorism or when the cribriform plate is
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Th
Hypertelorism 467
in high position, the subcranial U-shaped osteotomy can be
used for achieving either orbital mobilization or hemifacial
rotation. The central excess bone excision including the
ethmoids is done; no cut is made in the orbital roof. A
horizontal cut is made at the level of central bony resection,
and this extends onto the lateral orbital rim and then to the
lateral wall and the malar area. It then extends to the floor
and meets the cut in the medial wall. The cut in the medial
wall is made posterior to the lacrimal crest. The orbits are
then mobilized medially into the defect created by resection.
To perform the facial bipartition by subcranial route, the
cut in the lateral wall is extended across the malar bone to
the maxillary tuberosity and a pterygomaxillary disjunction
is performed. The maxilla is cut in midline, and the two
e
facial halves can be rotated (Fig. 21.13).
The subcranial U-shaped osteotomy is not very popular
as there is real risk of injuring the dura as the horizontal
cuts at the level of resection are made blindly. However, the
advantages are that it remains an extracranial procedure
and that no retraction of the frontal lobes is done.
Prevention of Ascending Infection
In the surgery for correction of hypertelorism, whether by
“box osteotomy” or “facial bipartition,” the extradural space
is exposed to the nasopharyngeal cavity. This can result in
ascending infection and can cause meningitis. It is therefore
desirable to separate these two by soft tissue interposition.
Fig. 21.12 Facial bipartition. Upper
row left shows sym­metrical hyper­
telorism with normal occlusion and a
V-shaped maxillary arch. A V-shaped
excision is marked in the midline (upper
right). Lower left row shows markings
for the cuts in the lateral orbital wall,
pterygomaxillary disjunction, and zygo­
matic arch. A cut is also shown in the
midline of maxilla. The dotted lines
depict the cuts in the roof, medial wall,
and floor and lateral walls. Lower row
right shows the final appearance.
468 Craniofacial Surgery
m telorism with low ethmoid
ie
Jackson et al29 showed that introduction of a vascularized
tissue such as axial front galeal flap can effectively prevent
this dreaded complication.
Nasal Augmentation
A dorsal nasal augmentation is required in all the cases after
Th
the hypertelorism surgery. A split cranial bone graft can be
used as a cantilever graft for this purpose. These autogenous
bone grafts would increase in size proportionately with the
growth of the child in future.
Management of Excessive Skin
The excess skin present in these patients would re-drape
itself to the newly created nasal dorsum after the correction.
However, in certain situations, the unaesthetic excess kin
with folds may require excision (Fig. 21.14).
Canthopexy
This is an important part of the operation. The medial
canthus tendons need to be identified and fixed at the
level of the lacrimal crest. The literature describes use of
transnasal wires to fix the canthal tendons; however, this
can be quite frustrating at times. The author has devised
a technique wherein a two-hole titanium plate is used for
e
Fig. 21.13 Subcranial osteot­
omy. Upper row shows hyper­
sinuses. The bony resection has
been shown on the right side.
Lower row shows the markings
for the subcranial osteotomy
and final appearance after
completion of the procedure.
this purpose .30 The plate is secured to the thick nasal bone,
and the lower hole is kept at the level of lacrimal crest. The
ipsilateral canthus is securely fixed in this hole using steel
wires (Fig. 21.15).
Illustrative Case Examples
Symmetrical Hypertelorism
The patient shown in Fig. 21.16 has bilateral symmetrical
hypertelorism. The dental arches and occlusion were
normal. A “box osteotomy” was performed, and the orbits
were medialized after removing the excess bone from the
midline nasal area. The defects in the lateral side were bone
grafted. The supraorbital bar was kept intact for guiding
the movement of the orbital box medially. A split cranial
bone craft was cantilevered to augment the projection of the
nose. The postoperative pictures in the lower row after 13
years show stable result.
Asymmetrical Hypertelorism
Fig. 21.17 shows a patient of craniofacial cleft who has
hypertelorism only on his left side. The other side bony orbit
has normal position. It was therefore decided to shift the
bony orbit medially only on the affected side. The middle
 Hypertelorism 469

e
m Fig. 21.14 The excess skin may need to be excised
when it is, thin, wrinkled, and unhealthy as is seen in
this case of nasofrontal encephalocele (upper row).
Immediate postoperative appearance is shown in lower
row left. The skin quality and color match improve with
time as seen in the 5-year postoperative picture (lower
row right).
ie
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Fig. 21.15 Author’s technique of medial canthopexy. Upper row left shows telecanthus in a child after trauma. Middle
picture shows the position of lacrimal crest in a skull. A two-hole plate is placed with the lower hole at the level of posterior
lacrimal crest. Upper row right shows the medial canthal tendon that has been isolated. Lower row left shows the bone where
the plate will be fixed. Middle picture shows threading of the canthal wire into the lower hole of the plate prior to fixing the
plate at the upper hole with a screw to the solid nasal bone. Picture on right shows the canthopexy appearance on table.
470 Craniofacial Surgery

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Fig. 21.16 The upper row shows a symmetrical bilateral orbital hypertelorism in a 7-year-old girl. Middle row on left shows
the appearance after completion of the box osteotomy cuts and removal of the bone from the midline. The picture on right
shows medially mobilized orbits. Lower row shows picture 15 years postoperatively.
 Hypertelorism 471

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Fig. 21.17 The upper row shows an adult with unilateral hypertelorism on the left side. Middle row shows close-up of
the deformity. Note a contour deformity on the ipsilateral frontal bone. The picture on right shows completion of the
unilateral box osteotomy cuts. Lower row shows result in the 3-week postoperative period.
472 Craniofacial Surgery
row shows the unilateral orbital box osteotomy cuts on
the operating table. The patient also had nasal augmentation
with split cranial bone graft. The 3 weeks postoperative
pictures show correction of the unilateral deformity.
Facial Bipartition
This 16-year-old girl presented with hypertelorism and
broad nasal dorsum with ill-defined tip. She also had
an angulated maxillary arch with dental malocclusion
(Fig. 21.18). She underwent a facial bipartition procedure.
She also had augmentation of the nasal dorsum with cranial
bone graft. The 2 years postoperative picture in the lower
row shows stable hypertelorism correction and a well-
maintained nasal tip projection.
Pseudo-hypertelorism (Telecanthus)
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Naso-orbito-ethmoid Fracture
This 20-year-old girl had NOE fractures. The left eye ball had
been eviscerated following globe rupture. There was also a
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Fig. 21.18 A 16-year-old girl with hypertelorism. She also has occlusal cant and slight dental arch deformity (upper
row). Lower row shows 2-year postoperative picture after facial bipartition procedure. She also had dorsal nasal
augmentation with split cranial bone graft.
bony defect in the glabellar region. The bony defects were
corrected, and dorsal nasal augmentation was performed
using split cranial bone grafts. The postoperative appearance
after 2 years is shown in the lower row (Fig. 21.19).
Nasofrontal Meningoencephalocele
This 9-month-old child has a nasofrontal meningoencepha­
locele that gives the child an appearance of widely set eyes
(Fig. 21.20). However, the position of the bony socket has
not become lateralized. There is lateral displacement of the
medial orbital walls only. The defect in the frontal bone and
anterior skull base was reconstructed with split cranial bone
graft. The nasal dorsum was also augmented with the bone
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graft. The medial canthopexy was performed on both sides.
The lower row shows result 6½ years after the surgery.
Craniofacial Fibrous Dysplasia
Fig. 21.21 shows a case of craniofacial fibrous dysplasia that
has resulted in pseudo-hypertelorism in a 15-year-old girl.
The lesion was removed using both intra- and extracranial
approaches. The lower row shows appearance 2 years
postoperatively.
 Hypertelorism 473

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Fig. 21.19 Upper row shows telecanthus (pseudo-hypertelorism), saddle nose deformity,
bone loss in glabellar region, and loss of eye globe on left side following a panfacial fractures
including naso-orbito-ethmoid injuries. Lower row shows postoperative results following repair
with split cranial bone graft, medial canthopexy, and insertion of eye prosthesis.
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Fig. 21.20 Upper row shows a 9-month-old child with nasofrontal meningoencephalocele.
The picture on right shows the appearance after repair using Chula technique. Lower row shows
postoperative result after 6 years.
474 Craniofacial Surgery
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Fig. 21.21 A case of craniofacial fibrous dysplasia producing telecanthus (upper row). Lower row
shows appearance 2 years postoperative after removal of the fibrous dysplasia by excision and
contouring.
Ethmoid Tumors
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This young girl had proptosis and telecanthus because of
osteoma in the ethmoid sinuses. The lower row shows
appearance after removal of the tumor 5 years post­
operatively (Fig. 21.22).
Complications
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Correction of hypertelorism would necessitate a combined
intra and extracranial approach. The ethmoid sinuses are
opened up, and there is a danger of ascending infection
from the nasal passages. The use of vascularized tissue such
as frontogaleal flap can effectively seal this passage and
prevent infection.29 There is a real danger of vision loss, but
with a careful approach these problems can be avoided.
In the immediate postoperative period, there is always a
possibility of some cerebrospinal fluid (CSF) leak. This can
be avoided if meticulous care is taken to repair any obvious
dural rents. Small leaks tend to settle down on conservative
treatment.
Palpable hardware in later life can be quite disturbing.
However, this morbidity is not seen where absorbable plates
are being used.
These cases may need small touchup procedures in the
form of soft tissue corrections. Many of these cases would
also benefit with revision of augmentation rhinoplasty and
management of excessive skin.
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Summary and Conclusion
As can be seen from the preceding illustrative cases,
hyper­telorism could be present in a variety of situations.
It is important to differentiate between a true ORH and a
pseudo-hypertelorism. The former necessitates a far more
extensive procedure including a combination of intra- and
extracranial approaches. The orbits can be medialized
either by box osteotomy technique or by facial bipartition
technique. The facial bipartition is chosen when there are
associated dental arch deformities that are seen in cases of
Apert’s syndrome or other craniofacial cleft cases. The box
osteotomy can not only help one medialize the orbits, but
it also permits correction of vertical orbital dystopia. This
technique can be easily adapted for correction of unilateral
hypertelorism. The medial canthopexy is an important part
of the procedure and can bring in dramatic improvement
in the result. The author prefers use of a two-hole plate for
securing the canthal tendon at the level of the posterior
lacrimal crest.
The surgery has become very safe these days as we
are able to separate the nasal cavity from the extracranial
space by interposition of vascularized tissue such as
frontogaleal flaps.
Telecanthus is also known as pseudo-hypertelorism and
can be present in cases of nasofrontal meningoencephaloceles,
after trauma or tumors in the NOE region. The surgeon needs
to address only the medial orbital wall in these cases, and
there is no need for any orbital relocation in these cases.

m Fig. 21.22 Upper row shows osteoma in the ethmoid sinuses producing telecanthus and
proptosis. Lower row shows postoperative pictures 5 years after removal of the lesion.
The morbidity and mortality in these cases are negligible
if the ORH correction is carried out in dedicated large-
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volume craniofacial centers.
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