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➔Examination ticket 1

1. Goldenhaar syndrome. Clinical representation, pathogenesis, surgical


approach

is a rare disorder that’s present at birth and it affects the development of your
face and skull
- It is a variant of hemifacial macrosomia.

- Causes:
Experts don’t know exactly what causes Goldenhar syndrome. It occurs because of a
change in a chromosome

- clinically:
Consist of: microtia (small ears) , macrostomia (big mouth) , agenesis of mandibular
ramus and condyle, vertebral abnormalities , cardiac , renal , or skeletal abnormalities.
- Diagnosis:
CT scan , Ultrasound

- TREATMENT
(SURGERY)

1- Growth centre transplantation:


Insertion of a cartilaginous graft, typically an autograft, in the osteotomy site is the most
common method used to accomplish the desired result. Costochondral rib grafts
(costochondral junction of the 5th, 6th or 7th rib) are most suitable for this purpose as
they can be transplanted as growth centre replacements to achieve mandibular growth.

2- Orthognathic surgery and orthodontics:


A combination of maxillary and mandibular osteotomies can be performed to correct the
skeletal deformity.

2. Congenital upper lip cleft classification


- Clefts can be unilateral or bilateral; microform, incomplete, or complete; and may
involve the lip, nose, primary palate, and/or secondary palates
- ETIOLOGY:
By factors including: chemical exposures , radiation , maternal hypoxia , teratogenic
drugs , nutrtional deficiency ,

- TREATMENT PLANNING AND TIMING:

- Cleft palate repair is usually performed at approximately 9 to 18 months of age.


- Most children will require an intact palate to produce certain speech sounds by 18
months of age

- approximately 20% of children will have inadequate closure of the velopharyngeal


mechanism (velopharyngeal insufficiency or VPI), and this may produce hypernasal
speech

- A pharyngeal flap or sphincter pharyngoplasty may be used to treat VPI


-“Reconstruction of Cleft Lip and Palate: Secondary Procedures.” Approximately 75% of
patients with any type of cleft will present with clefting of the maxilla and alveolus.24–26
Bone graft reconstruction of this site is performed during the mixed dentition prior to the
eruption of the permanent canine and/or the permanent lateral incisor

- Orthognathic reconstruction of maxillary and mandibular discrepancies is


performed at 14 to 18 years of age

3. Haemangioma in children and adolescents (clinical


representation, diagnosis, treatment)
- Hemangiomas are the most common tumors in infancy, especially in premature babies
weighing less than 1000 g, with girls affected three to five times more often than boys.

- Hemangiomas: are proliferative lesions characterized by increased turnover of


endothelial cells. They appear as solitary lesions in 80% of children.

- Hemangiomas may be:


1- superficial: Hemangiomas typically appear during the first 2 years of life, if they
are not present or evident at birth, as an erythematous patch.

2- deep: Deep lesions may appear bluish, or a deep purple color.

3- visceral: The visceral ones will generally not be evident on clinical examination.

- Clinically:
- First; proliferative phase of rapid enlargement 4-8 months of age, bright color of
the lesion.
- By age 12 months, the involution phase starts and usually continues for the next
5 to 7 years.
- Various tumor factors and markers of angiogenesis (e.g., fibroblastic growth
factor, vascular endothelial cell growth factor, and proliferating cell nuclear
antigen) are increased during the rapid proliferative phase and begin to decrease
with the initiation of involution.

- Diagnosis:
is by clinical examination 95% of cases, sometimes MRI.
On MRI the lesion will appear as the same intensity of the adjacent muscle tissue.

- TREATMENT:
Completely involuted lesions does not need treatment but those with partial involution or
those located infraorbital will need surgical intervention.
Intralesional steroids or surgical resection has been the proposed treatment modalities
4. Patient, 6 months, has skin redness on the right buccal area. The parent notes
that the child has this spot of redness from the birth, that has been growing in
size. She also has added that the size of the spot increases daily during
emotional distress and crying. The doctor sees that this spot has a positive filling
symptom. Diagnosis and treatment method?

hemangioma

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➔Examination ticket 2
1. Osteoblastoma and Osteoid Osteoma. Clinical representation,
pathogenesis, surgical approach
● Osteoblastomas:
They are uncommon lesions of the bone that occur in the posterior mandible or in the maxilla
during the patient’s twenties. Other common sites include the vertebrae, skull, sinonasal tract,
and temporal bone.

- etiology:
remains unknown, but osteoblastomas are considered benign neoplasms that demonstrate a
2:1 male-to-female predilection.

- Clinically:
- include localized swelling, bony expansion, and pain.

- Radiographically, a mixed lucent and opaque pattern exists that may be predominantly
lucent or predominantly opaque (sclerotic) and, occasionally, may appear as a sun-ray
pattern.

- Treatment:
These lesions are treated with enucleation and curettage or complete local excision when found
to infiltrate surrounding tissues.

● The osteoid osteoma:


- It is considered a smaller version of an osteoblastoma although certain differences make
these lesions clinically distinct.

- Osteoblastomas are larger lesions, typically over 1.5 cm, and the pain experienced does
not follow a circadian rhythm nor is the pain relieved with nonsteroidal anti-inflammatory
medications or aspirin.

- Treatment:
is the same as for osteoblastoma and includes enucleation and curettage.

2. Van der Woude syndrome. Clinical representation, pathogenesis,


surgical approach
- Autosomal dominant inherited syndrome. Present in 2% of cleft lip patients
- causes:
by genatices

- Symptoms:
● Cleft lip
● Cleft palate
● Lip pits (depressions) or mounds (raised bumps) on one or both sides of the
lower lip
● Lip pits are small sinuses to minor salivary glands.
● Hypernasal voice.
● cleft or bifid uvula may be present
● Missing teeth (hypodontia) or missing teeth enamel (dental hypoplasia)

- TREATMENT:
The same for cleft lip and palate

3. Parents of a 9-year-old boy appealed to a dentist with complaints of


deformation of the mandible on the left. On radiographs of the mandible
bone defect with sharp edges 2-3 cm containing 44 tooth follicle is
observed. Define a diagnosis

Odontogenic bone cyst

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➔Examination ticket 3
1. Synovial chondromatosis and osteochondroma. Clinical representation,
pathogenesis, surgical approach
- Both synovial chondromatosis and osteochondroma are conditions that occur in the
temporomandibular joints and may be considered variants of the chondroma and
osteoma.

● synovial chondromatosis:
- In this case there is a proliferation of small particulate, generally unattached,
chondromas within the confines of the joint capsule.

- Occurs in the superior compartment of TMJs.

- Etiology:
- is unknown, but it is thought to be trauma.
- When these lesions become symptomatic, they should be removed via a standard
preauricular approach.

- Also endoscopic approach and perforation into the middle cranial fossa has been
described.

- Symptoms:
- normally consisting of pain and swelling but most often with deviation of the mandible toward
the unaffected side.

● The osteochondroma:
- It is believed to be a benign lesion that arises predominantly in long bones from a
herniation of cartilage through the epiphyseal plate.

- It tends to present with a predominantly osseous core with a cartilaginous cap.


The lesion becomes symptomatic when function is affected, for example, a malocclusion or
mandibular asymmetry develops.

- Diagnosis:
- On MRI it appears as an extraneous appendage to the temporomandibular joint and is
usually brighter than the surrounding mandible.

- Treatment:
is symptomatic; when symptoms occur, localized excision is recommended via the normal
temporomandibular approach.

2. A 13-year-old child appealed toa dentist with complaints of discoloration of the


11 tooth From history we know that 4 years ago the injury of the front section of
the upper jaw occurred. Objectively: the 11 tooth intact, percussion is painless.
ON The X-ray: the bone is thin at the top of the root of tooth 11 with clear oval
edges. Your diagnosis and treatment plan.

ANS/ CLEAR OVAL EDGES (CYST Y3NII)

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➔ Examination ticket 4
1. Dates, periods and possible complications during and after cleft lip and
cleft palate operations. Secondary correctional operation date periods

possible complications during and after cleft lip and cleft palate operations:

- during:
- Allergies to tape, suture materials and glues, topical preparations or injected
agents
- Bleeding
- Damage to deeper structures – such as nerves, blood vessels, muscles and auditory
canal can occur and may be temporary or permanent

- after :
- Irregular healing of scars including shortening, thickening or overgrowth
- Infection
- Respiratory problems after surgery

2. Pierre-robin sequence. Clinical representation, pathogenesis,


surgical approach
a rare congenital birth defect characterized by an underdeveloped jaw, backward
displacement of the tongue and upper airway obstruction.

- Cause:
The exact cause of Pierre Robin syndrome is unknown.

- Clinical representation
- trouble breathing
- feeding early on, resulting from the tongue's position, smaller jaw size and
the cleft palate formation.
- Repeated ear infections.
- Natal teeth.

- surgical approach
enucleation and curettage has been the preferred treatment method, whereas resection is
reserved for more aggressive, or recurrent, lesions.

3. Central Giant Cell Granuloma. Clinical representation, pathogenesis,


surgical approach
Central giant cell granulomas (CGCG) are localized and benign but locally aggressive
tumors occurring exclusively in the jaws.
- Clinical Features:
- GCG can occur at any age, but the majority present before the age of 30 years.

- The anterior part of the mandible is a favorable site, and lesions commonly cross
the midline.
- The majority of the GCGs of the jaw present as a painless expansion of bone or
are detected on routine radiographic examination.

- The lesions appear as a well-defined radiolucency that may be either unilocular


or multilocular.

- Rapid enlargement of GCGs during pregnancy was reported by several authors.

- pathogenesis:
The etiology is unknown, but is thought to be a reactive process, possibly secondary to
trauma or inflammation; however, some believe it is a benign neoplasm.

- treatment
Traditionally, CGCG was treated surgically with aggressive curettage and peripheral
ostectomy. The more aggressive type or recurrent lesions require wide resection, that
lead to major defects in the jaws. This form of surgical treatment could be particularly
disfiguring for a child or young adult.

4. A 9 year-old girl during treatment of the 75 tooth the homogeneous rounded thinning
of bone was found. There is the temporary tooth roots and permanent tooth in follicle.
What diagnosis can be assumed by X-ray?

ANS/ CLEAR OVAL EDGES (CYST Y3NII)

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➔Examination ticket 5
1. Cruzon syndrome. Clinical representation, pathogenesis, surgical
approach

Crouzon syndrome is a genetic disorder characterized by the premature fusion of


certain skull bones, leading to craniosynostosis. This condition affects the shape of the
head and face and may impact various systems in the body.

- Clinical Representation:

- Craniosynostosis: Premature fusion of skull bones can result in an abnormal


head shape, a condition known as craniosynostosis. The head may appear short
and broad with a flattened forehead.
- Midface Hypoplasia: The midface may be underdeveloped, leading to shallow
eye sockets and a protruding or beaked nose.
- Hypertelorism: Increased distance between the eyes, giving them a wide-set
appearance.
Proptosis: Bulging of the eyes.

- High-arched Palate.

- Hearing Loss.

- Caused:
by mutations in the FGFR2 gene, which plays a role in the development of bones and tissues.
The mutation leads to premature fusion of certain skull bones, affecting the normal growth and
development of the head and face.

- Surgical Approach:
Orthognathic Surgery:
Jaw surgery may be considered to address malocclusion and other dental issues.

3. A 16-year-old patient complains of the slight swelling in the right lower jaw
area. Objectively: there is a slight asymmetry of the face in right lower jaw area.
The 45 tooth is absent. The X-ray of the right half of the lower jaw in a lateral
projection revealed the cell of bone tissues of oval form (2*3 cm), with clear
contours. The reverse crown part of horizontally placed the 45 tooth is localized
in the cavity. What is the most credible diagnosis?

ANS/ CLEAR OVAL EDGES (CYST Y3NII)

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➔ Examination ticket 6

1. Neck congenital cysts and hole-channels. Clinical representation,


pathogenesis, surgical approach

3. Cherubism. Clinical representation, pathogenesis, surgical approach

Cherubism is a rare genetic disorder characterized by excessive bone development in


the jaws, leading to a characteristic appearance of facial swelling and a cherubic
(angelic) appearance.

- Clinical Representation:
● Facial Swelling: Cherubism is characterized by bilateral and symmetrical
swelling of the cheeks, giving affected individuals a cherubic or
"chipmunk" appearance.
● Eyebrow Elevation: The upward displacement of the eyes due to the jaw
enlargement can cause an elevation of the eyebrows.
● Dental Issues: Cherubism can lead to dental abnormalities, including
displacement and malformation of teeth.

​ - Pathogenesis:
● Cherubism is caused by mutations in the SH3BP2 gene, which plays a role
in the regulation of bone cell function.
● The genetic mutation leads to an overactive stimulation of osteoclasts,
the cells responsible for bone resorption.

​ - Surgical Approach:
- is involves regular monitoring to assess the progression of the disease.
- Surgical Intervention: If the facial deformity is severe and affects the individual's
quality of life, surgical intervention may be considered.
- Orthognathic Surgery: In some cases, orthognathic surgery may be performed to
correct the jaw alignment and improve facial aesthetics.

4. Patiant, 4 months of age, got new formation on the cheek. as parents observes
formation progresses over time and increases in size daily during emotional
distress and crying, becomes more pronounced, color darkens rom pink to red
and size of formation increases. formation was first observed after 20 days from
birth as a little lump. Giveprimary diagnosis, adittional examination plan,
formulate treatment approach.
ANS/ HEMANGIOMA
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➔Examination ticket 7
3. Nonodontogenic cyst of the the jaws. Clinical representation,
pathogenesis, surgical approach

● Benign Mesenchymal Tumors:


most common in pediatrics:
giant cell lesions,fibro-osseous lesions, and the desmoplastic fibroma.

In the giant cell lesion group, three entities are usually included:
1. the central giant cell granuloma (CGCG)
2. the brown tumor of hyperparathyroidism
3. the giant cell tumor

in the fibro-osseous lesions: fibrous dysplasia, cherubism, ossifying fibroma juvenile


(aggressive), and osteoblastoma.

● Giant Cell Lesions:


- central giant cell granuloma (CGCG):
occurs almost exclusively in the jaws, predominantly affects children and young adults, and
female more often than male patients.

- Clinically: there is a painless expansion involving the anterior or premolar regions


mainly in the mandible

Treatment: enucleation and curettage has been the preferred treatment method, whereas
resection is reserved for more aggressive, or recurrent, lesions.

● Brown tumors:
may represent a clinical manifestation of secondary, or hereditary, hyperparathyroidism in
children.
- Clinically: In the jaws, these appear as multiple radiolucent lesions that histologically
are identical to CGCG lesions. Jaw lesions that show typical

- Management:
process should result in resolution of jaw lesions, but occasionally enucleation and curettage,
similar to the CGCG, may be required.

● Fibro-osseous Lesions:
It is a disease of bone, is a condition most commonly encountered up to and through the teen
years with a tendency for stabilization at puberty and slow growth,or regression.
Fibrous dysplasia results from the replacement of normal medullary bone by abnormal fibrous
connective tissue and new bone formation.

- clinical forms:
- include monostotic, polyostotic,McCune-Albright syndrome, and Jaffe-Lichenstein
syndrome (with no bone involvement).
- The maxilla is most commonly involved, and in the polyostotic form, the ribs, femur and
tibia may also be affected.
- There is a slow painless unilateral expansion tha

- Treatment:
of fibrous dysplasia may include surgical recontouring of the involved bones in the facial
skeleton for cosmetic reasons (It is best to delay this surgery until after cessation of the growth
spurt, or after puberty, unless the condition causes such a severe deformity).
● Osteoblastomas:
They are uncommon lesions of the bone that occur in the posterior mandible or in the maxilla
during the patient’s twenties. Other common sites include the vertebra, skull, sinonasal tract,
and temporal bone.

- Etiology:
- remains unknown, but osteoblastomas are considered benign neoplasms that
demonstrate a 2:1 male-to-female predilection.

- Clinical characteristics:
include localized swelling, bony expansion, and pain.

- Treatment:
is the same as for osteoblastoma and includes enucleation and curettage.

● Desmoplastic fibroma:
- It is a rare benign, but locally aggressive, lesion of bone that involves the long bones and
pelvis and may occasionally be found in the mandibular ramus.
- The origin of these tumors is not clear.

- Progressive, asymptomatic growth eventually results in swelling of the jaw.

- Treatment:
is embolic resection, and teeth in the area must be sacrificed in order for the lesion to be
completely eradicated.

High recurrence rates, up to 25%,

4.Patient, 6 months, has skin redness on the right buccal area. The parent notes
that the child has this spot of redness from the birth, that has been growing in
size. She also has added that the size of the spot increases daily during
emotional distress and crying. The doctor sees that this spot has a positive filling
symptom. Diagnosis and treatment method?
ANS/ HEMANGIOMA

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● Examination ticket 8
1. Paget's disease. Clinical representation, pathogenesis, surgical
approach

Definition:
It is a slowly progressive bone condition of unknown etiology, predominantly affecting males
over the age of 50 years.

Clinically:
- there is hyperactive bone turnover with alternate resorption of bone, a vascular phase,
and finally, a sclerosing phase.

- Most bones of the body are involved.

- In the facial region, the maxilla is affected more often than the mandible.

- Initial presentation is usually related to bone deformity or pain. In the head and neck,
headaches and symptoms owing to vascular and nerve compression have been noted.

Treatment:
is both systemic and local. Systemic treatment currently consists of the use of salmon calcitonin
or non–nitrogen-containing bisphosphonates to inhibit bone resorption.

4. Parents of a 9-year-old boy appealed to a dentist with complaints of


deformation of the mandible on the left. On radiographs of the mandible bone
defect with sharp edges 2-3 cm containing 44 tooth follicle is observed. Define a
diagnosis

ANS/ CYST

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● Examination ticket 9
1. Fibrous Dysplasia. Clinical representation, pathogenesis, surgical
approach

- Definition:
It is a disease of bone, is a condition most commonly encountered up to and through the teen
years with a tendency for stabilization at puberty and slow growth,or regression.

- Etiology:
Fibrous dysplasia results from the replacement of normal medullary bone by abnormal fibrous
connective tissue and new bone formation.

- clinical forms:
- The clinical forms of fibrous dysplasia include monostotic, polyostotic,McCune-Albright
syndrome, and Jaffe-Lichenstein syndrome (with no bone involvement).
- The maxilla is most commonly involved, and in the polyostotic form, the ribs, femur and
tibia may also be affected.

- Treatment:
of fibrous dysplasia may include surgical recontouring of the involved bones in the facial
skeleton for cosmetic reasons (It is best to delay this surgery until after cessation of the growth
spurt, or after puberty, unless the condition causes such a severe deformity).

Low-dose radiation has been used in the past for treatment of polyostotic disease
McCune-Albright syndrome(It is a less common variant of polyostotic fibrous dysplasia):

management: bisphosphonate medications specifically pamidronate

4. A 13-year-old child appealed toa dentist with complaints of discoloration of the


11 tooth From history we know that 4 years ago the injury of the front section of
the upper jaw occurred. Objectively: the 11 tooth intact, percussion is painless.
ON The X-ray: the bone is thin at the top of the root of tooth 11 with clear oval
edges. Your diagnosis and treatment plan.
ANS/ CYST

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● Examination ticket 10
4. A 9 year-old girl during treatment of the 75 tooth the homogeneous rounded
thinning of bone was found. There is the temporary tooth roots and permanent
tooth in follicle. What diagnosis can be assumed by X-ray?
ANS/ CYST

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