Craniofacial Clefts and Orbital Hypertelorism Flashcards
Definitive surgical intervention for orbital hypertelorism
at which age?
Definitive surgical intervention for orbital hypertelorism
should wait until the orbit has reached maturity at least 5
to 7 years of age.
Orbital hypertelorism is Type of syndrome
Orbital hypertelorism is a physical finding, not a syndrome
facial clefts
are numbered O to 7, whereas cranial clefts are numbered 8 to 14
T
A diagnosis of a craniofacial cleft can be made more reliably based on the s soft tissue manifestations
F A diagnosis of a craniofacial
a cleft can be made more reliably based on the skeletal manifestations
which are more consistent than the soft tissue manifestations
Median craniofacial clefts represent a unique form of facial clefting
which can be associated with both hyperplasia of midline structures
and tissue hypoplasia
F Median craniofacial clefts represent a unique form of facial clefting
which can be associated with both hypoplasia of midline structures
and tissue hyperplasia
there is a positive correlation
between the severity of the facial findings and degree of CNS anomalies
T
What in investigation that we need to do in a mild form of median craniofacial hypoplasia ?
In patients with milder forms, assessing CNS anatomy in Median Craniofacial Hypoplasia 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
A false median cleft lip
maybe present with the absence of the philtrum and central tubercle in cleft number 0
T
The columella may be narrowed, shortened, or absent, and the nasal
tip may lack projection because of a hypoplastic or absent caudal septum IN no. 14 clefts
F In number 0 cleft
IN 0/14 cleft secondary palate can be present
F. complete absence of the premaxilla and
primary palate resulting in a large central palatal cleft
Meaning of Craniofacial Dysraphia
Dysraphia represents a failure offusion, or cleft, ofmidline structures
without deficiency or excess.
a true median cleft lip where all central structures are
apsent
F a true median cleft lip where all central structures are
present
median cleft with present philtral columns
T
An encephalocele meaning
is a herniation
of CNS structures including brain and cerebrospinal fluid through a
weakened midline space
Type of Anterior encephaloceles
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.
No. 1 Tessier clefts can often be confused with common cleft lips
T
No 1 cleft passes through Cupid’s bow to the nasal base
T
The eyelids are affected. with No. 1 clefts
F The eyelids are unaffected. No. 1 clefts
no. 1 cleft, however, is the involvement
of the medial nose including a notched soft triangle and alar dome
T
medial canthal displacement occurs in cleft number 1
T. medial canthal displacement resulting
in telecanthus
A primary and
secondary palatal cleft may be present in cleft N .1
T
Widened ethmoids can result in hypertelorism.
T
NO.2 originating at the peak of Cupid’s bow before extending up to the lateral nasal spine
F originating at the peak ofCupid’s
bow before extending up to the nasal sill
Difference bet no. 1 clefts, no. 2 clefts
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 lacrimal system and eyelids are affected by No.2 cleft
F The lacrimal system and eyelids are unaffected
NO.1 can cause medial brow distortion
F NO.2
primary/secondary palate similar to
a common cleft lip/palate in NO.2
T
Tessier oro-naso-ocular cleft
No. 3 Cleft
The cephalic extension of NO. 3
to the lower eyelid between the medial canthus and the inferior punctum.
NO. 3 pass to the middle third of the ala
F lateral ala
The Risk of keratopathy is more with no2 than 3
F more common with no.3
The medial wall of the
maxillary sinus is absent in no 3
t
bony component of the no.3 cleft terminates before the lacrimal
groove .
bony component of the cleft terminates in the lacrimal
groove in the inferior-medial orbit
the first of the clefts to travel lateral to the nose?
No.4
Lacrimal system involved with cleft no.4 ?
can involve the lacrimal system at
the level of the punctum and the lacrimal sac
Bone extension of cleft no.4
between the frontal process of the
maxilla and the infraorbital foramen.
The lacrimal system is usually
spared in no.4
T
the least common of the oblique facial clefts
N.5
The bony extension of cleft no.5 lateral to the infraorbital foramen
T
Cleft occurs with mild forms of Treacher-Collins syndrome and Nager syndrome, ?
no. 6 cleft both of which present with zygomaticomaxillary dysplasia.
The no. 6 cleft begins at the commissure and extends toward the lateral lower lid
T
oblique orientation of the palpebral axis,
oftentimes referred to as an antimongoloid slant with no.6
T
Colobomas can be
present at the inferior lateral eyelid with no.5
F NO.6
Causes of choanal atresia in N .6 cleft
The posterior maxillary buttress
(pterygomaxillary buttress) can be foreshortened resulting in a steep
maxillary occlusal plane and choanal atresia
wich cleft that continue with inferior orbital fissure
NO.6
No. 7 clefts are by far the most common of the craniofacial clefts
T
The presentation of the isolated form of cleft no.7
They result in isolated macrostomia
Treacher Collins and Nager
syndromes which are associated with no. 7 craniofacial clefts
F Treacher Collins and Nager
syndromes which are associated with no. 6 to 8 craniofacial clefts
Cleft no 7 end at lateral edge of masseter
F Most commonly,
the cleft terminates medial to the masseter
Cranial nerve no.7 involved in cleft n.7
F Cranial nerve
V may be involved as can the tongue and palate on the affected side
The cranial base can also be
affected leading to asymmetry of the glenoid fossa and sphenoid
bone. in no. 7
T
steel occlusal plane occurs with no.7 cleft
T 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 no. 7 cleft divides the facial clefts from the cranial clefts and is
centered around the zygomaticomaxillary suture.
F The No. 8 cleft divides the facial clefts from the cranial clefts and is
centered around the frontozygomatic suture.
Soft Tissue Involvement in no 8
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
The no. 8 cleft is a cleft in the frontozygomatic suture
T
Treacher Collins Characteristics feature?
The classic findings of teacher 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)
- absence of the inferior and lateral orbit as
zygomatic arch (no. 7 and 8 clefts)
Nager syndrome vs Treacher Collins syndrome
Nager syndrome has a similar
phenotype to Treacher Collins syndrome but also includes limb
anomalies
The no. 5 cleft is the rarest of the isolated cranial clefts
F The no. 9 cleft is the rarest of the isolated cranial clefts termed
fronto-sphenoid dysplasia
microphthalmia may present in cleft number 8
F NO .9
The frontotemporal branch of the facial
nerve can be affected with which cleft?
N.9
Skeletal Involvement in cleft no.9
extends superiorly through the superior-lateral orbit
displacing the greater wing of the sphenoid and the squamosal temporal bone.
Soft tissue involvement includes the middle third of the upper eyelid
and central eyebrow. in cleft no 9
F no. 10
Eyelid deficiencies are common, ranging from
colobomas to complete ablepharon with number 10
T
Skeletal involvement IN NO 10 begins at the superior orbit, medial to the
supraorbital foramen
F Lateral to supraorbital foramen
hypertelorism can occur with no 10
T
The cranial base and anterior cranial fossa are involved in no.11 cleft
F The cranial base and anterior cranial fossa are uninvolved
In No 12 cleft the soft tissue cleft lies medial to the medial canthus,
T
The ethmoid sinuses are widened which contributes to hypertelorism and telecanthus
T
encephaloceles are common IN NO 12
encephaloceles are uncommon.
The cribriform plate and olfactory groove are
unaffected with no 12
T
The eyelid and eyebrow are affected with cleft no 13 clefts.
The eyelid and eyebrow are intact in this cleft
the medial
brow may be inferiorly displaced. in no 13
T
A classic finding
of the no. 13 cleft is the involvement of the cribriform plate, leading
to widening of the olfactory groove, cribriform plate, and ethmoid
sinuses
T
severe forms of hypertelorism occures with no. 13 cleft
T Especially when bilateral, this involvement can produce
some of the most severe forms of hypertelorism encountered
ln microforms/mild forms, a lower lip notch may be the only sign ofa
no. 0 cleft
F ln microforms/mild forms, a lower lip notch may be the only sign ofa
no. 30 cleft
The hyoid bone
may be clefted, or absent, as well as the thyroid cartilage and rarely
the sternum.IN NO 30
T
The no. 13 cleft represents the median cranial extension of the
no. 1 facial cleft
F The no. 13 cleft represents the paramedian cranial extension of the
no. 1 facial cleft
distinguish hypertelorism from telecanthus (or pseudohypertelorism
Telecanthus is the widening of the intercanthal distance often a result of medial canthal tendon avulsion or nasoorbitalethmoid fracture. In telecanthus, the bony orbits are in a normal position.
the hyperteloric orbit is entirely
displaced and the bony distance between the orbits (interdacryon
distance) is increased. In addition to the horizontal vector, orbits can
be displaced vertically (orbital dystopia), anteriorly or posteriorly,
and rotationally.
measurement of intradcrynon distance is best made radiographically or intraoperatively;
T
Grading of hypertelorism
In an adult, the average interdacryon distance is 25 mm in a
female and 28 mm in a male. First-degree hypertelorism is 30 to 34 mm, second degree 34 to 40 mm,
and third degree is defined as an interdacryon distance ofgreater than 40 mm
In children the measurement is different, how?
first-degree hypertelorism is defined as an interdacryon
distance 2 to 4 mm greater than normal, second degree is 4 to 8 mm
greater than normal, and third degree is more than 8 mm greater than
normal
hypertelorism can be inherited?
yes Craniofrontonasal dysplasia is a unique inherited form of hypertelorism It occurs in both
inherited and sporadic forms
The typical manifestations of an inherited form of hypertelorism ?
Include uni coronal or bi-coronal craniosynostosis, hypertelorism, bifid or absent nasal tip, widow’s peak, and wiry hair. Additionally, patients may
have cleft lip and/or palate and limb anomalies including syndactyly
medial cranial clefts (nos. I 1-14), early
intervention is recommended.
T
frontonasal dysplasia, frontoethmoidal
encephaloceles, or any of the medial cranial clefts (nos. I 1-14) why we should do early intervention ?
to reduce and separate the intracranial compartment from the paranasal sinuses. To avoid the dangerous
sequelae of an ascending CNS infection such as meningitis
patients with significant
ocular involvement including eyelid deficiency or displacement may
require earlier intervention if eye lubrication is insufficient to prevent
corneal damage
t
at which age the early intervention is considered?
as soon as the patient is old enough to tolerate
the procedure (>3 months)
isolated perioral clefts (nos. 0-7) can
be performed at 3 to 6 months of age
T
Nasal anomaly in peroral cleft should be treated in adulthood
F Nasal anomalies can be treated at the time of perioral
cleft repair however, patients with frontonasal dysplasia and disorganized accessory nasal cartilage may benefit from surgery at an older
age to minimize growth restriction
dacryocystorhinostomyis indicated to which type of facial cleft?
Medial orbital (nos. 3/11 and
4/10) clefts may require dacryocystorhinostomy ifsymptomatic
Bony anomalies should be reconstructed in a delayed manner
T Bony anomalies should be reconstructed in a delayed manner
unless causing functional impairment. In general, cranial bone grafts
are necessary to reconstruct the orbits, facial buttresses, and nose,
whereas cancellous iliac crest bone grafts can be used to treat persistent alveolar clefts.
Repair of orbital dystopia and hypertelorism
should be done after 18 years
F. Repair of orbital dystopia and hypertelorism
should be avoided before school age and preferably performed at
orbital maturity (6-9 years ofage)
The different types of surgical approaches in hypertelorism t treatmenmt?
When hypertelorism occurs, treatment should focus on safely
decreasing the interorbital distance to less than 17 mm by performing a box osteotomy or a facial bipartition
What box osteotomy?
box osteotomy is limited to repositioning the orbits and the zygomas
and can effectively narrow the interorbital distance while preserving
midfacial and occlusal relationships
What facial bipartition
a facial bipartition procedure is ideal for patients who, in addition to OHT, also have maxillary hypoplasia, a narrow maxillary arch, and malocclusion
What is the meloschisis ?
Oral-ocular clefts do not disrupt the integrity of the nose. They occur lateral
to Cupid’s bow and extend through the cheek and maxillary process. This is called meloschisis
Cleft number 4 can disrupt the nose.
F Cleft 4 is one of the oral-ocular clefts (4–6) which disrupt the mouth and eye but avoid disruption of the nose
Cleft numbers 0 to 3, termed oral-nasal clefts, occur between the midline and Cupid’s bow, disrupting both the
lip and nose
T
What is Treacher Collins syndrome ?
Bilateral oral ocular and lateral facial clefts are typically seen, and the airway is a major priority in these patients,
many of whom will require long-term tracheostomy placement. This is secondary to mandibular hypoplasia in
combination with a narrow pharynx
In Treacher Collins Syndrom patient can have ptosis
F Upper eyelid surgery
may be required as patients have an excess of upper eyelid skin laterally giving a false impression of ptosis
In Treacher Collins Syndrom patient may need rhinoplasty
F. No need
Cleft No.7 effect male and female equally
f m > f
cleft no. 7 bilateral in 10% of cases
T
hypoplasia of the greater wing of the sphenoid most commonly occures with cleft no.9
T
Clefts 3, 4, and 5 is oral-ocular clefts
T
A Harlequin deformity in which type of cleft present?
Cleft no.14 A Harlequin deformity may be seen on
radiographs due to upslanting of the anterior cranial fossa
In cleft number 30 The thyroid gland itself is not involved,
T
