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HYPERTELORISM
INTRODUCTION
Telecanthus: increased distance between the medial canthi. The inter-orbital distance
is normal.
Hypertelorism: increased distance between the orbits.
Orbital hypertelorism signifies an increased distance between both medial sides and
lateral sides of the orbits.
Interorbital hypertelorism(bony telecanthus) denotes increased distance only
between the inner orbital walls.
Interorbital hypertelorism - interorbital distance more than 2 standard deviations from
normal mean, age and gender matched, with a normal outer orbital distance.
Orbital hypertelorism - both interorbital and outer orbital distances of more than 2
standard deviations from the normal mean
Embryology
Orbital hypertelorism occurs during the period prior to the 28-mm C-RL
stage(approximately 50 days) of embryonic development.
The anomaly is not lateralization but failure of medialization of the entire orbits.
In contrast, interorbital hypertelorism more likely occurs during the period following
medialization of the orbits.
A localized(field) defect(s) or sequence leading to abnormal development of the
frontonasoethmoid complex could prevent primary medialization or cause excessive
secondary lateralization of the medial orbital walls.
CLASSIFICATION
Tessier
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At birth, IOD = 16mm; at 12 years, IOD = 25mm; in adults, the normal inter-orbital
distance is 25 mm in women and 28 mm in men.
1) 1st degree hypertelorism: IOD = 30-34 mm
2) 2nd degree hypertelorism: IOD = 35-40 mm
3) 3rd degree hypertelorism: IOD > 40 mm
Can also be classified according to the configuration of the medial orbital wall: parallel,
divergent, bulging or convergent. The last 2 are the rarest and most difficult to Rx.
CAUSES
Traumatic.
Congenital
1) frontonasal malformation
a. most common cause of hypertelorism
b. also known as Median Cleft Face Syndrome, Frontonasal Syndrome,
Frontonasal Dysplasia
c. symmetrical, orbital type hypertelorism
d. median cleft or bifid nose, median cleft upper lip and premaxilla, median
cleft palate, widow's peak, anterior cranium bifidum occultum
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(Above, left) Facies A: frontonasal malformation in a 4-year-old girl. Note the symmetrical second-degree
interorbital hypertelorism, prominent widow's peak, and broad bifid nasal tip. She had resection of a nasal
dermoid that extended intracranially.(Above, center) Facies B: frontonasal malformation (minor) with
bipartate nasal tip and second-degree interorbital hypertelorism.(Above, right) Facies B: frontonasal
malformation (major) in a 3-month-old girl. Note the symmetrical third-degree orbital hypertelorism,
disjoined nares, median cleft lip/palate, and basal encephalocele. (Below, left) Facies D: frontonasal
malformation in an 11-month-old girl with third-degree orbital hypertelorism, neurogenic blepharoptosis,
alar colobomas with widely separated nasal openings, choanal atresia, and midfacial hypoplasia. She had
an interfrontal encephalocele and mental retardation. Facies D is a combination of facies B(major) and
facies C. (Below, center) Rugose frontonasal malformation in a 9-month-old girl. Note the thickened
corrugated skin, short columella, poorly defined alar-cheek junctions, and symmetrical second-
degreeinterorbital hypertelorism. (Below, right) Six-year-old girl with rugose frontonasal malformation
and first-degreeinterorbital hypertelorism.
3) craniofacial cleft(s)
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Asymmetrical orbital hypertelorism with right-sided Tessier no. 2-12 cleft in a 10-year-old
girl
TREATMENT
patient with orbital hypertelorism requires transcranial translocation of the entire
orbital complex, whereas translocation of the medial orbital walls can suffice for
interorbital hypertelorism, depending on the position of the cribriform plate
Correction is subcranial or combined intracranial/extracranial.
Subcranial may be either
1) Medial orbital wall migration: maximum correction 10 mm.
a. Complete ethmoidectomy followed by bilateral medial canthopexy
2) Four wall (classic Tessier)
a. Best for adults
b. Inferior orbital nerve is sacrificed
c. Modification by Converse preserves the cribriform plate
3) Three wall (Inverted U) osteotomy
a. mobilising the medial, lateral and superior orbital walls en block
b. maximum correction 15 mm.
c. best for children during primary or mixed dentition, or for asymmetric
orbital shapes
d. inferior orbital nerve is preserved
4) facial bipartition
5) Lammelar split orbital osteotomy
a. Outer table split from inner table
b. Does not correct true hypotelorism, useful as an adjunct to refine the
orbital contour post 3-4 wall osteotomies
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4 wall
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COMPLICATIONS
Early:
Infection
Haemorrhage (including retrobulbar haematoma)
Sensory disturbances (eg SON, ION)
Anosmia
Wound dehiscence
Late:
Canthal drift
Enophthalmos.exopthalmos
Lacrimal duct damage
Ptosis
Temporal depression
Abnormal orbital configuration – over-correction / under-correction
Blindness