Craniofacial Tumors and Conditions Flashcards
Fibrous dysplasia is a benign anomaly of the bone
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What is the
most common craniofacial tumor encountered by plastic surgeons
FIBROUS DYSPLASIA
the percentage of skull involvement in fibrous dysplasia
Skull involvement occurs in
27% of monostotic patients and up to 50% of polyostotic patients
Craniofacial fibrous dysplasia
most commonly begins
after childhood
F Craniofacial fibrous dysplasia
most commonly begins in childhood then progresses through adolescence. It was previously thought that the disease progression halted after childhood
the disease frequently
continues well into adulthood.
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The most common bone involved in Fibrous dysplasia
in the craniofacial skeleton most often affects the frontal and
sphenoid bones and the maxilla
((monostotic disease ))
what is the most common sign of the fibrous dysplasia
Signs and symptoms
of craniofacial disease are diverse and may include swelling of the
affected side (most commonly).
Causes of Fibrous dysplsia
bone maturation in the woven
bone stage
Gene responsible for the fibrous dysplasia mutation
GNAS gene
A subset of patients with dysplasia mutation have an associated endocrinopathy what it call
McCune-Albright syndrome. This condition includes
precocious puberty, cafe au lait spots, and other endocrinopathies
due to the hyperactivity of various endocrine glands
What is cherubism?
Cherubism
or familial fibrous dysplasia is a self-limited disease of the maxilla
and mandible in children
Giant cell disorder or cherubism after regression leave som deformity
F regresses without operation and leaves no deformity
malignant transformation is rarely seen in fibrous dysplasia
malignant transformation may be seen
in 3.2% of patients especially in polyostotic
fibrous dysplasia most commonly transforms to
chondrosarcoma
F fibrous dysplasia most commonly transforms to osteosarcoma, and occasionally to
chondrosarcoma or fibrosarcoma
exclusive ofMcCune-Albright
syndrome can diagnosed clinically
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Radiological finding in fibrous dysplasia
The typical plain radiographic appearance is of radiolucent lytic lesions with a homogenous ground-glass appearance and
ill-defined borders.
Differential diagnosis for Fibrous dysplasia
ossifying
fibroma and Paget disease
CT) provides
substantially better radiographic information
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Fibrous dysplasia has a
ground-glass pattern only on CT
F Fibrous dysplasia has a ground-glass pattern (56%),
homogeneously dense pattern (23%), or
a cystic variety (21%)
CT can give diagnostic information only.
F CT also improves operative planning by evaluating the extent
of disease, particularly in evaluating the extent of the optic nerve canal
involvement
What is the Chen and Nordhoff approach?
based on their treatment approach to the occurrence
in four designated zones:
■ Total excision for zone 1 (fronto-orbital, zygomatic, and upper
maxillary regions).
■ Conservative excision (contouring) for
□ Zone 2 (hair-bearing cranium),
□ Zone 3 (the central cranial base), and
□ Zone 4 (the tooth-bearing regions of the maxillary alveolus
and mandible)
patients undergoing resection had a lower recurrence
rate and required fewer total operations but had a slightly increased postoperative complication
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Radiotherapy has no role in treatment of
fibrous dysplasia and should be avoided
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Optic nerve decompression for optic canal involvement is controversial
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What si the outcome of optic nerve decompression?
the outcome of optic nerve decompression ranges from
(1) halting vision loss, (2) improvement in vision, (3) worsening
in vision
Clinical signs of PARRY-ROMBERG SYNDROME
characterized as a progressive hemifacial
hypoplasia with regional fat atrophy, medial canthal malpositioning,
enophthalmos, dystopia, and skin hyperpigmentation
Romberg disease occurs more on the R side than L
both sides of the face are equally effected
Some (but not all) cases of Romberg disease are believed to undergo remission or burnout after puberty
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in more severe cases, bony, ophthalmological, and/or neurological
involvement occurs
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Burnout of disease may not occur for 10 years
or more
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The take of fat grafting in the diseased Romberg region is less
than fat graft take in normal regions
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What is the option of treatment in skeletally mature patients?
Dermal fat grafts or adipofascial free flaps (such as a parascapular or inframammary extended circumflex scapular [IMECS] flaps
The back region provides
better adipose-fascia! fill for facial contouring than that of other free
flaps
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Treacher Collins syndrome (TCS) featurees
Hypoplasia of the zygomatic complex and the mandible, antimongoloid slanting of palpebral fissures, coloboma of the lower eyelids,
complete or partial cleft palate, and atresia of external ear canals with
abnormalities of the external ears accompanied by conductive hearing loss
The causes of TREACHER COLLINS SYNDROME
93% ofTCS is caused by loss-of-function
mutations in the TCOFI gene
TCOFl gene
is inherited in an autosomal dominant fashion and all patient inherited the mutated from of the gene
F TCOFl gene
is inherited in an autosomal dominant fashion, with only 40% of
patients inheriting a mutated copy of the gene and 60% of cases arising from de novo mutagenesis
If obstruction is limited to the tongue
base and epiglottis, mandibular distraction can be effective in the
newborn age or at a later age if sleep apnea develops.
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tracheostomy considred the standared fro airway obstraction in TC SYNDROM
T In the newborn, severe airway obstruction may require a
tracheostomy (the standard)
Surgery that needs to be done for patients with teacher choline syndrome at early age?
- tracheostomy (the standard in severe cases
– Mandibular distraction can be effective in sleep apnea develops. - Choanal atresia, obstructing the posterior nasopharyngeal airway, may also need to be treated with dilatation or bony removal.
cleft lip repair, cleft palate repair, macrostomia (Tessier no. 7 cleft), or removal of preauricular skin tags. In addition, optimizing hearing with BAHA
Surgery in 6-9 years of age
■ mandibular distraction lengthening
■ malar, zygomatic, and eyelid reconstruction
■ total external ear reconstruction
Relapse of this abnormal mandibular morphologic shape has been noted in long-term
follow-up from the strong genioglossal muscle pull
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For total ear reconstruction, the two-stage Firmin technique for all type
F preferred unless there is a paucity of skin as in a very low lobule or
anotia. 16 For these patients, the four-stage Brent technique
At which age the Malar/orbital reconstruction should be attempted
At age 8 to 12 years, malar/
orbital reconstruction may be undertaken with full-thickness parietal
skull bone grafts for the zygomatic arch and lateral orbit, with split
thickness grafts for the orbital floor
A common surgical procedure to treat the open bite in Treacher choline syndrome without airway compromise is Lefort 1
F for the Treacher Collins patient, this will likely worsen the airway and aesthetics because of the short cranial base and abnormal mandible
What is the alternative surgery that peseve the airway ?
Tessier described l’integrale
procedure combining a Le Fort II osteotomy, bilateral mandibular
advancement, orbital bone grafting, and genioplasty for the severe,
uncorrected form of TCS with airway compromise
when we can detect the Clinical manifestations of neurofibromatosis?
Clinical manifestations of neurofibromatosis are detected at birth
in about 40% of patients, over 60% have manifestations by the
the second year of life starting with cafe au lait spots
Cafe au lait spot seldamly present at birth
They are frequently present at birth and increase in
size in childhood
Approximately one-third of patients
develop plexiform neurofibromas
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about 6% may develop malignancy
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Features of neurofibrimatosis?
In young children, macrocephaly without hydrocephalus, short stature, hypertelorism, and thoracic abnormalities are frequently found
The lifetime risk of developing neurosarcoma or neurofibrosarcoma?
The lifetime risk of development is 5% to 10% with the mean age of diagnosis at 41 years of age.
Pain is the most reliable sign of malignant transformation
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Neurofibromatosis, type I (NFl) features
NF! accounts for 90% of all
cases. Clinical diagnosis is suspected when six or more cafe au lait
spots, cutaneous neurofibromas, and melanocytic hamartomas
projecting from the surface of the iris (Lisch nodules) are found
Neurofibromatosis, type II (NFII) features
Neurofibromatosis, type II (NF2): NF2 is characterized by bilateral acoustic neuromas with hearing loss usually present as a
teenager or young adult . Cafe au lait spots and cutaneous neurofibromas are present but usually less common than NFL Plexiform
neurofibromas and central nervous system tumors may also be
present occasionally
What are the two type of Craniofacial plexiform neurofibromas?
□ Orbitotemporal neurofibromas
□ Plexiform neurofibromas resulting in hemifacial hypertrophy
In the most severe of cases,
involvement of the globe leads to blindness in Orbitotemporal neurofibromas
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A second type of craniofacial involvement by plexiform neurofibromas occurs in which nerve?
This type typically forms from the trigeminal, facial, or glossopharyngeal nerves
In case of Orbitotemporal Plexiform Neurofibroma what are the best surgical tech?
resection of the neurofibroma through a supratarsal incision
Bone reconstruction with either bone graft or titanium or methylmethacrylate
shortening of the levator aponeurosis may be necessary
orbital box repositioning may be necessary to treat vertical orbital dystopia .
A bony defect of the orbit may should be reconstructed at the same time
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the best choice for reconstruction of most osseous
defects
bone grafts, resorption of the graft is common
because of the pulsatile intracranial contents. Thus, in this particular
location, alloplastic (titanium or methylmethacrylate) should be used
n the treatment of
hemifacial hypertrophy, it is necessary to sacrifice certain amount of
normal soft tissue structures,
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In Pierre Robin Sequence cleft palate deformity is an associated abnormality but
not a requirement for the sequence
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micrognathia ensues and causes the retropositioning of the
tongue. If this process occurs before the 5th weekof gestation, closure
of the palatal shelves is prevented
F 8th week
Patients with PRS may improve after delivery T F
T becoz postnatal mandibular catch-up growth
Management of PRS
Pron position
Intubation (nasopharyngeal tube or an endotracheal tube )
glossopexy, subperiosteal mandibular stripping, mandibular
distraction, and tracheostomy
glossopexy does not
address the core anatomical disturbance
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Contraindication for
mandibular distraction in PRS
as central apnea, multilevel airway obstruction, neurologic
compromise, or severe airway edema from reflux
Mandibular distraction and laryngotracheal reconstruction may be needed after
tracheostomy removal
F Mandibular distraction and laryngotracheal reconstruction may be needed before
tracheostomy removal
n syndromic PRS
patients, there is a higher rate of temporomandibular joint ankylosis after distraction
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What is MOEBIUS SYNDROME
combined palsies
of the abducens (CN VI) and facial (CN VII) nerves
limb abnormalities, chest wall defects, and developmental
delay.
Which nerve is involoved in moebius syndrome?
6,12,9,3,5
What is the first sign of the Moebius syndrome?
Clinically, paralysis of the orbicularis oculi is the first sign of Moebius syndrome detected in the neonate
Feeding difficulties may resolve over time in Moebius syndrome
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Absence or hypoplasia of the sternal head
of the pectoralis major is a common finding in moebius
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How many the percent of patients whom had moebias and poland syndrom
15-20 %
How many the percent of Scoliosis in Moebius
Scoliosis is also found in approximately 14% of cases
Limb abnormalities occur in over half of all patients diagnosed with
Moebius syndrome T F
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What is the most common abnormality of the lower limp in Moebius syndrom
The most common abnormality is clubfoot in
40% to 50% of patients followed by limb reduction abnormalities
in approximately 20% of patients
Intellectual developmental delay
occurs in 10% to 30% of patients with Moebius syndrome
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Autism has been associated with Moebius syndrome.
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Pathogenesis of moebius syndrome
misoprostol used in first trimester
genetic inherited an autosomal dominant, autosomal recessive, or
X-linked pattern.
Male are affected more than females in Mobius syndrome
F EQUALLY
functional muscle transfers frequently can be done with facial nerves successfully
F functional muscle transfers frequently require innervation by nonfacial nerves such as the
masseteric nerve
In the absence of suitable facial nerves as the donor innervation,
How we can echieve the re animation of the facial muscle
The most commonly used one-stage procedure is
the innervated, partial gracilis free muscle flap
Microsurgical anastomoses of the vascular supply of the gracilis muscle are performed last, typically to the facial vessels.
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If CN V is functional how do you constract the facial animation in Moebius?
lengthening temporalis myoplasty or Labbe procedure may be performed
Cause of lagophthalmos in Moebius
The mechanism of lagophthalmos in Moebius syndrome is due to the lack of opposition of the
levator palpebrae muscle by the paralyzed orbicularis oculi muscle
For Lagophthamus positions have been described for the weight placement
(1) pretarsal and (2) postseptal.
the weight
chosen should be 0.2 g heavier than the preoperatively estimated
weight.
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Quantifiable complications such as hematoma and flap failure in
facial reanimation are rare
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Dermoid
cysts are differentiated from the typical epidermoid cysts by the presence of mature adnexal structures such as hair and glands
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dermoid cysts are rarely
associated with some type of skeletal abnormality
F frequently, if not always,
associated with some type of skeletal abnormality
Dermoid cysts are
more common in females than in males
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the most common site
of craniofacial dermoids
the frontotemporal region are the most common type
Dermoids the frontotemporal (lateral eye brow ) do not warrant imaging before surgical resection without
additional clinical suspicion
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the second most common area for dermoid cysts
of the face
The orbit is the second anatomical subdivision of lateral cysts
Extraocular mobility is typically affected by orbital
cysts
F Extraocular mobility is typically not affected by orbital
cysts
orbital dermoid causes simple depression o depressions within
the bone
F orbital dermoids frequently traverse sutures, such
as the frontozygomatic suture ). In such cases,
the dermoid has an intraorbital component and an extra orbital
component.
Unlike lateral orbital cysts, the midline dermoid cysts may have
more extensive and potentially intracranial involvement
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The estimated incidence of intracranial involvement ranges around 19% of
all nasal dermoids
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Midline dermoids may be found in the upper,
middle face , only
F Midline dermoids may be found in the upper,
middle, or lower face
nasoethmoid region, the external
clinical manifestations will be sever
F nasoethmoid region, the external
clinical manifestations may be particularly mild
mild clinical presentations may portend extensive
involvement of internal structures and intracranial involvement.
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midline dermoids have a
higher propensity to display puncta and sinus ostia
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Dermuid will not decrease or increase in it size through adulthood
F Owing to the
presence of adnexal structures, dermoid cysts have the capability of
enlarging over time
Calvarial osteomyelitis can occur with dermoid T F
T. Although rare, complications of dermoid cysts
include infection, with several instances of calvarial osteomyelitis
reported in the literature
Dermuid start to appear at 8 week of gestation
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The sign on the CT scan for intracranial extension of dermoid
The two signs on the CT scan are suggestive of intracranial
extension are a patent foramen cecum and a bifid crista galli.
In nasal dermoids
positioned more inferiorly, an open rhinoplasty approach should be
considered.
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Resection
of dermoids with intracranial extension may be complicated with
osteomyelitis, incomplete resection, or cerebrospinal fluid leak
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Underlying bony abnormalities are
also found, thus frequently requiring bone grafting for reconstruction of the mandible, maxilla, or zygoma as necessary in Facial Plexiform Neurofibromas With Hemifacial
Hypertrophy
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PRS has been associated with other anomalies, it is often divided into
■ PRS: isolated (Figure 35.9)
■ PRS plus: with comorbidities, Stickler, 22q deletion
■ Syndromic PRS: Treacher Collins syndrome, Nager syndrome
U-shaped cleft palate occures with PRS
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Skeletally mature, untreated PRS patients
may require mandibular advancement with a sagittal split osteotomy
for class II malocclusion after orthodontic preparation.
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The reported complications of mandibular distraction are respiratory failure, relapse, nerve injury, tooth injury, infection, incorrect distraction vector, and device failure
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Ptosis, nystagmus, strabismus, epicanthal folds,
hypertelorbitism, and lagophthalmos are all frequently manifested with Mobius
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The main procedures performed by the latter two specialties are strabismus correction and clubfoot correction In moebius
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In the postseptal position, the septum is
incised and the weight is placed immediately superior to the tarsus for lagophthalmos in moebius
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A small punctum, bulge of the glabella, or widening of the nasal dorsum may be
seen. However, mild clinical presentations may portend extensive
involvement of internal structures and intracranial involvement
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unlike the lateral dermoids, midline dermoids have a
higher propensity to display puncta and sinus ostia
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Dermoid cysts are most often found as isolated entities and very
rarely found in families
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dissection of the cyst should be through the soft tissues to
the periosteum; in dermiod
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In nasal dermoids, diagnosis requires imaging with an initial
fine-cut CT scan If normal and there is suspicious an MRI
should be performed next
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The coup de
sabre sign may just involve skin and fat hypoplasia but not necessarily bone
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Alloplastic implants are not recommended for
Treacher Collins reconstruction in children
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For severe cases, face transplantation has
been performed but carries long-term risks associated with
immunosuppression in hemifacial hypertrophy
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Cutaneous neurofibromas appear around puberty
and increase throughout life.
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