The Indian Dental Academy is the Leader in continuing dental education , training dentists in all aspects of dentistry and
offering a wide range of dental certified courses in different formats.for more details please visit
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Crouzon Syndrome.
Primarily characterized by premature closure of the fibrous joints (cranial sutures) between certain bones in the skull (craniosynostosis) and distinctive facial abnormalities
Crouzon Syndrome.
Primarily characterized by premature closure of the fibrous joints (cranial sutures) between certain bones in the skull (craniosynostosis) and distinctive facial abnormalities
Fibro-osseous lesions of the jaws
Fibrous dysplasia
Cemento-osseous dysplasia
Focal cemento-osseous dysplasia
Periapical cemento-osseous dysplasia
Florid cemento-osseous dysplasia
Ossifying fibroma
Juvenile aggressive ossifying fibroma
Cherubism
Fibro-osseous lesions (FOL) are characterized by replacement of normal bone architecture by collagen fibers and fibroblasts containing calcified tissue.
They include a wide variety of lesions of developmental, dysplastic and neoplastic origins with clinical and radiographic presentation and behavior.
Because of the histological similarities between diverse diseases, proper diagnosis requires correlation of history, clinical and radiographic findings.Fibrous Dysplasia
2. Reactive (dysplastic lesions arising in the tooth-bearing area (presumably of periodontal origin).
a. Periapical cemento-osseous dysplasia
b. Focal cemento-osseous dysplasia
c. Florid cemento-osseous dysplasia
3. Fibro-osseous neoplasms (widely designated as cementifying fibroma, ossifying fibroma or cemento-ossifying fibroma.Bone dysplasias
a. Fibrous dyspla i. Monostoticii. Polyostotic
iii. Polyostotic with endocrinopathy (McCune-Albright)
iv Osteofibrous dysplasia
b. Osteitis deformansc. Pagetoid heritable bone dysplasias of childhood
d. Segmental odontomaxillary dysplasia
2. Cemento-osseous dysplasias
a. Focal cemento-osseous dysplasia b. Florid cemento-osseous dysplasia
3.Inflammatory/reactive processes
a. Focal sclerosing osteomyelitisb. Diffuse sclerosing osteomyelitis
c. Proliferative periostitis
4. Metabolic Disease: hyperparathyroidism
5. Neoplastic lesions (Ossifying fibromas)
a. Ossifying fibromab. Hyperparathyroidism jaw lesion syndrome
c. Juvenile ossifying fibroma i. Trabecular typeii. Psammomatoid type
d. Gigantiform cementomas
Craniofacial syndromes /certified fixed orthodontic courses by Indian dental ...Indian dental academy
Indian Dental Academy is the Leader in continuing dental education , training dentists in all aspects of dentistry and offering a wide range of dental certified courses in different formats.
Fibro-osseous lesions of the jaws
Fibrous dysplasia
Cemento-osseous dysplasia
Focal cemento-osseous dysplasia
Periapical cemento-osseous dysplasia
Florid cemento-osseous dysplasia
Ossifying fibroma
Juvenile aggressive ossifying fibroma
Cherubism
Fibro-osseous lesions (FOL) are characterized by replacement of normal bone architecture by collagen fibers and fibroblasts containing calcified tissue.
They include a wide variety of lesions of developmental, dysplastic and neoplastic origins with clinical and radiographic presentation and behavior.
Because of the histological similarities between diverse diseases, proper diagnosis requires correlation of history, clinical and radiographic findings.Fibrous Dysplasia
2. Reactive (dysplastic lesions arising in the tooth-bearing area (presumably of periodontal origin).
a. Periapical cemento-osseous dysplasia
b. Focal cemento-osseous dysplasia
c. Florid cemento-osseous dysplasia
3. Fibro-osseous neoplasms (widely designated as cementifying fibroma, ossifying fibroma or cemento-ossifying fibroma.Bone dysplasias
a. Fibrous dyspla i. Monostoticii. Polyostotic
iii. Polyostotic with endocrinopathy (McCune-Albright)
iv Osteofibrous dysplasia
b. Osteitis deformansc. Pagetoid heritable bone dysplasias of childhood
d. Segmental odontomaxillary dysplasia
2. Cemento-osseous dysplasias
a. Focal cemento-osseous dysplasia b. Florid cemento-osseous dysplasia
3.Inflammatory/reactive processes
a. Focal sclerosing osteomyelitisb. Diffuse sclerosing osteomyelitis
c. Proliferative periostitis
4. Metabolic Disease: hyperparathyroidism
5. Neoplastic lesions (Ossifying fibromas)
a. Ossifying fibromab. Hyperparathyroidism jaw lesion syndrome
c. Juvenile ossifying fibroma i. Trabecular typeii. Psammomatoid type
d. Gigantiform cementomas
Craniofacial syndromes /certified fixed orthodontic courses by Indian dental ...Indian dental academy
Indian Dental Academy is the Leader in continuing dental education , training dentists in all aspects of dentistry and offering a wide range of dental certified courses in different formats.
A dysmorphic feature is a difference of body structure. It can be an isolated finding in an otherwise normal individual, or it can be related to a congenital disorder, genetic syndrome, or birth defect. Dysmorphology is the study of dysmorphic features, their origins and proper nomenclature.
Inheritance and malocclusion / /certified fixed orthodontic courses by India...Indian dental academy
The Indian Dental Academy is the Leader in continuing dental education , training dentists in all aspects of dentistry and offering a wide range of dental certified courses in different formats.
Indian dental academy provides dental crown & Bridge,rotary endodontics,fixed orthodontics,
Dental implants courses.for details pls visit www.indiandentalacademy.com ,or call
00919248678078
The Indian Dental Academy is the Leader in continuing dental education , training dentists in all aspects of dentistry and
offering a wide range of dental certified courses in different formats.for more details please visit
www.indiandentalacademy.com
The Indian Dental Academy is the Leader in continuing dental education , training dentists in all aspects of dentistry and
offering a wide range of dental certified courses in different formats.for more details please visit
www.indiandentalacademy.com
Opportunity for Dentists (BDS/MDS )to relocate to United kingdom -Register as a DENTAL HYGIENIST/ DENTAL THERAPIST without Board exams and after approval you can register in GDC as a DH/DT and start working as a DH/DT Immediately and get paid.
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Eligibility / requirements-
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at the appropriate level.(Within 2 yrs of application date )
2: A recent primary dental qualification that has been taught and examined in English..(Within 2 yrs of application date )
3: A recent pass in a language test for registration with a regulatory authority in a country where the first language is English.
If you are interested Please contact us for more details.
1ST, 2ND AND 3RD ORDER BENDS IN STANDARD EDGEWISE APPLIANCE SYSTEM /Fixed ort...Indian dental academy
Indian Dental Academy: will be one of the most relevant and exciting training center with best faculty and flexible training programs for dental professionals
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Dear Doctor,
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Course includes:
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Thanks & Regards
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--
Indian Dental Academy
Leader in continuing dental education
www.indiandentalacademy.com
skype:indiandentalacademy
+919248678078
The Indian Dental Academy is the Leader in continuing dental education , training dentists in all aspects of dentistry and
offering a wide range of dental certified courses in different formats.for more details please visit
www.indiandentalacademy.com
The Indian Dental Academy is the Leader in continuing dental education , training dentists in all aspects of dentistry and
offering a wide range of dental certified courses in different formats.for more details please visit
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Cytotoxicity of silicone materials used in maxillofacial prosthesis / dental ...Indian dental academy
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Diagnosis and treatment planning in completely endntulous arches/dental coursesIndian dental academy
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The Indian Dental Academy is the Leader in continuing dental education , training dentists in all aspects of dentistry and
offering a wide range of dental certified courses in different formats.for more details please visit
www.indiandentalacademy.com
The Indian Dental Academy is the Leader in continuing dental education , training dentists in all aspects of dentistry and
offering a wide range of dental certified courses in different formats.for more details please visit
www.indiandentalacademy.com
The Indian Dental Academy is the Leader in continuing dental education , training dentists in all aspects of dentistry and
offering a wide range of dental certified courses in different formats.for more details please visit
www.indiandentalacademy.com
The Indian Dental Academy is the Leader in continuing dental education , training dentists in all aspects of dentistry and
offering a wide range of dental certified courses in different formats.for more details please visit
www.indiandentalacademy.com
The Indian Dental Academy is the Leader in continuing dental education , training dentists in all aspects of dentistry and
offering a wide range of dental certified courses in different formats.for more details please visit
www.indiandentalacademy.com
The Indian Dental Academy is the Leader in continuing dental education , training dentists in all aspects of dentistry and
offering a wide range of dental certified courses in different formats.for more details please visit
www.indiandentalacademy.com
The Indian Dental Academy is the Leader in continuing dental education , training dentists in all aspects of dentistry and
offering a wide range of dental certified courses in different formats.for more details please visit
www.indiandentalacademy.com
The Indian Dental Academy is the Leader in continuing dental education , training dentists in all aspects of dentistry and
offering a wide range of dental certified courses in different formats.for more details please visit
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Macroeconomics- Movie Location
This will be used as part of your Personal Professional Portfolio once graded.
Objective:
Prepare a presentation or a paper using research, basic comparative analysis, data organization and application of economic information. You will make an informed assessment of an economic climate outside of the United States to accomplish an entertainment industry objective.
Normal Labour/ Stages of Labour/ Mechanism of LabourWasim Ak
Normal labor is also termed spontaneous labor, defined as the natural physiological process through which the fetus, placenta, and membranes are expelled from the uterus through the birth canal at term (37 to 42 weeks
Embracing GenAI - A Strategic ImperativePeter Windle
Artificial Intelligence (AI) technologies such as Generative AI, Image Generators and Large Language Models have had a dramatic impact on teaching, learning and assessment over the past 18 months. The most immediate threat AI posed was to Academic Integrity with Higher Education Institutes (HEIs) focusing their efforts on combating the use of GenAI in assessment. Guidelines were developed for staff and students, policies put in place too. Innovative educators have forged paths in the use of Generative AI for teaching, learning and assessments leading to pockets of transformation springing up across HEIs, often with little or no top-down guidance, support or direction.
This Gasta posits a strategic approach to integrating AI into HEIs to prepare staff, students and the curriculum for an evolving world and workplace. We will highlight the advantages of working with these technologies beyond the realm of teaching, learning and assessment by considering prompt engineering skills, industry impact, curriculum changes, and the need for staff upskilling. In contrast, not engaging strategically with Generative AI poses risks, including falling behind peers, missed opportunities and failing to ensure our graduates remain employable. The rapid evolution of AI technologies necessitates a proactive and strategic approach if we are to remain relevant.
The French Revolution, which began in 1789, was a period of radical social and political upheaval in France. It marked the decline of absolute monarchies, the rise of secular and democratic republics, and the eventual rise of Napoleon Bonaparte. This revolutionary period is crucial in understanding the transition from feudalism to modernity in Europe.
For more information, visit-www.vavaclasses.com
Biological screening of herbal drugs: Introduction and Need for
Phyto-Pharmacological Screening, New Strategies for evaluating
Natural Products, In vitro evaluation techniques for Antioxidants, Antimicrobial and Anticancer drugs. In vivo evaluation techniques
for Anti-inflammatory, Antiulcer, Anticancer, Wound healing, Antidiabetic, Hepatoprotective, Cardio protective, Diuretics and
Antifertility, Toxicity studies as per OECD guidelines
Model Attribute Check Company Auto PropertyCeline George
In Odoo, the multi-company feature allows you to manage multiple companies within a single Odoo database instance. Each company can have its own configurations while still sharing common resources such as products, customers, and suppliers.
June 3, 2024 Anti-Semitism Letter Sent to MIT President Kornbluth and MIT Cor...Levi Shapiro
Letter from the Congress of the United States regarding Anti-Semitism sent June 3rd to MIT President Sally Kornbluth, MIT Corp Chair, Mark Gorenberg
Dear Dr. Kornbluth and Mr. Gorenberg,
The US House of Representatives is deeply concerned by ongoing and pervasive acts of antisemitic
harassment and intimidation at the Massachusetts Institute of Technology (MIT). Failing to act decisively to ensure a safe learning environment for all students would be a grave dereliction of your responsibilities as President of MIT and Chair of the MIT Corporation.
This Congress will not stand idly by and allow an environment hostile to Jewish students to persist. The House believes that your institution is in violation of Title VI of the Civil Rights Act, and the inability or
unwillingness to rectify this violation through action requires accountability.
Postsecondary education is a unique opportunity for students to learn and have their ideas and beliefs challenged. However, universities receiving hundreds of millions of federal funds annually have denied
students that opportunity and have been hijacked to become venues for the promotion of terrorism, antisemitic harassment and intimidation, unlawful encampments, and in some cases, assaults and riots.
The House of Representatives will not countenance the use of federal funds to indoctrinate students into hateful, antisemitic, anti-American supporters of terrorism. Investigations into campus antisemitism by the Committee on Education and the Workforce and the Committee on Ways and Means have been expanded into a Congress-wide probe across all relevant jurisdictions to address this national crisis. The undersigned Committees will conduct oversight into the use of federal funds at MIT and its learning environment under authorities granted to each Committee.
• The Committee on Education and the Workforce has been investigating your institution since December 7, 2023. The Committee has broad jurisdiction over postsecondary education, including its compliance with Title VI of the Civil Rights Act, campus safety concerns over disruptions to the learning environment, and the awarding of federal student aid under the Higher Education Act.
• The Committee on Oversight and Accountability is investigating the sources of funding and other support flowing to groups espousing pro-Hamas propaganda and engaged in antisemitic harassment and intimidation of students. The Committee on Oversight and Accountability is the principal oversight committee of the US House of Representatives and has broad authority to investigate “any matter” at “any time” under House Rule X.
• The Committee on Ways and Means has been investigating several universities since November 15, 2023, when the Committee held a hearing entitled From Ivory Towers to Dark Corners: Investigating the Nexus Between Antisemitism, Tax-Exempt Universities, and Terror Financing. The Committee followed the hearing with letters to those institutions on January 10, 202
2. It is small wonder that anomalies of the craniofacial
complex are of major significance not only to dental
health but also to mental health and social well-
being.
The anomalies of development of the craniofacial
complex are extremely vast and varied. For most
patients, it is difficult or impossible to describe a
specific etiologic cause.
In an adult, the etiologic factors that caused the
problem to develop during growth may no longer
have an effect even if they are still present.
And yet, some thought must be given to etiology
when treatment is planned because this is a major
factor in estimating the stability of results,
particularly in children but also in adults.
INTRODUCTION
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4. •SYNDROME: syn= together, dromos= a running
The aggregate of signs & symptoms associated with any morbid
process & constituting together the picture of the disease & related
to each other anatomically, biochemically or physiologically.
•An anomaly is a deviation from the average or norm, anything that
is structurally unusual or irregular or contrary to a general rule.
•A sequence is a pattern of multiple anomalies derived from a
single known or presumed structural defect or mechanical factor.
•An association is a nonrandom occurrence in two or more
individuals of multiple anomalies not known to be a polytopic field
defect, sequence, or syndrome
•Dysmorphology is an area of clinical genetics that is concerned
with the diagnosis and interpretation of patterns of structural
defects. Recurrent patterns of birth defects are the hallmarks of
syndrome recognition. www.indiandentalacademy.comwww.indiandentalacademy.com
5. There are four clinically significant types of congenital anomaly
Malformation
Disruption
Deformation and
Dysplasia
MALFORMATION :- A morphological defect of an
organ, part of an organ, or larger region of the body that
results from an intrinsically abnormal developmental process
Intrinsic implies that the developmental potential of the
primordium is abnormal from the beginning, such as a
chromosomal abnormality of a gamete at fertilization.
Most malformations are considered to be a defect of a
morphogenetic or developmental field "which responds as a
coordinated unit to embryonic interaction and results in
complex or multiple malformations
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6. DISRUPTION
A morphological defect of an organ, part of an
organ, or a larger region of the body that results
from the extrinsic breakdown of, or an interference
with, an originally normal developmental process.
Thus, morphological alterations following
exposure to teratogens —agents such as drugs and
viruses — should be considered as disruptions.
A disruption cannot be inherited, but "inherited
factors can predispose to and influence the
development of a disruption."
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7. DEFORMATION
An abnormal form, shape, or position of a part of the body
that results from mechanical forces.
Intrauterine compression that results from oligo-
hydramnios — insufficient amount of amniotic fluid —
produces a clubfoot an example of a deformation
produced by extrinsic forces.
Some central nervous system defects, such as
meningomyelocele — a severe type of spina bifida produce
intrinsic functional disturbances that also cause fetal
deformation.
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8. DYSPLASIA
An abnormal organization of cells into tissue (s)
and its morphological result (s).
Dysplasia is the process and the consequence of
dyshistogenesis (abnormal tissue formation).
All abnormalities relating to histogenesis are
therefore classified as dysplasias, e.g., congenital
ectodermal dysplasia
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9. TYPES OF PROBLEMS
IN MORPHOGENESIS
DISRUPTION DYSPLASIADEFORMATIONMALFORMATION
ABNORMAL
ORGNZN OF CELLS
IN TISSUE
BREAKDOWN OF
NORMAL TISSUE
UNUSUAL FORCES
ON NORMAL TISSUE
POOR TISSUE
FORMATION
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10. ANOMALAD:
Term coined by Dr. F. Clarke Frase
To denote a pattern of morphologic defects stemming from a
single localized structural anomaly, which result in a cascade of
consequent defects.
Ending “ad” is meant to imply more than one defect (as in diad,
triad)
So anomalad connotes the initiating anomaly and its derived
secondary defects.
The cause for initiating structural defect may be heterogeneous,
so anomalad does not connote a particular mode of etiology.
As severity of initial morphologic anomaly may vary, so the
range of severity & extent of anomalad may also be variable.
Eq. Robin Anomalad
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12. 1)1) Richard & Goodman classified multiple
malformation disorders as :
i) Syndrome
a) Chromosomal abnormality
b) Gene abnormality
c) Environmental factor
ii) Anomalad
iii) Association
iv) Combination of malformations not
assigned to any of above categories..
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13. 2) Embryonic or histologic classification:
i) Single germ layer involved
ii) All the three germ layers involved
ex. Gardner syndrome
3) Monothetic syndrome classification:
Syndromes that share single common feature are grouped
together.
ex. Syndromes with cleft palate
Syndromes with polydactyly
This aids in differential diagnosis
4) Polythetic classification:
Syndromes sharing a large proportion of their principal
anomalies are grouped together.They are even better aid in
differential diagnosis.
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14. 5) Syndrome prototype: By Herrmann & Opitz 1974 & Cohen 1982
i) Dysmetabolic syndrome (Metabolism)
• Normal at birth,
• Uniform clinical features,
• No congenital malformations,
• Biochemically defined,
• Recessive mode of inheritance
• eg. Hurler Syndrome
Tay- Sachs Disease
ii) Deformation syndrome (Region)
• Changes in shape of previously normal
structures
• Lack of movement- mechanical ,
functional
• Commonly affects musculoskeletal
system
iii) Malformation syndrome (Organ or Feild)
• Noncontiguous Malformation
• Embryonic Pleiotropy
• Lack of biochemical definition
• eg. Trisomy 13 syndrome
Rubenstein Tyabi Syndrome
iv) Dyshistogenetic syndrome (Tissue)
1) Simple
• Involvement of only 1 germ layer
• Inheritance- dominant or recessive
• eg. Achondroplasia
2) Hamartoneoplastic Sydrome
• Hamartomas or neoplasias
• More than 1 germ layer
• Commonly dominant
• eg. Peutz Jeghers Syndrome
Gardners Syndromewww.indiandentalacademy.comwww.indiandentalacademy.com
15. Graphic illustration of the causes
of human birth defects
Unknown Etiology
Multifactorial
Inheritance
Chromosomal
Abnormalities
Mutant Gene
Environmental
Agents
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16. 1. Syndrome Associated With Midfacial Deficiency
2. Syndromes Associated With Midfacial Excess
3. Syndromes Associated With Mandibular Deficiency
4. Syndromes Associated With Mandibular Prognathism
5. Syndromes Associated With Problems Of Facial Height
6. Syndromes Associated With Facial Asymmetry
7. Syndromes Associated With Ocular Hypertelorism
8. Syndromes Associated With Chromosomal Disorders
9. Environmental Induced Syndromes
10. Other Common Syndromes
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18. ACHONDROPLASIA
The term Achondroplasia was first used by Parrot in 1978 to describe short
limbed dwarfism associated with enlarged head.
AETIOLOGY:-
•Autosomal dominant, about 90 percent of the cases
represent a fresh gene mutation.
•Older paternal age has been a contributing factor in
these cases.
• It is a disturbance of Endochondral bone formation.
•The most common chondrodysplasia, true
achondroplasia, occurs with a frequency of
about 1 in 15,000.
CLINICAL FINDINGS:-
•Small stature. Mean adult height in males is 131 ± 5.6
cm and in females is 124± 5.9 cm.
•Megalocephaly
•Small foramen magnum
•Short cranial base with early spheno occipital closure.
•Low nasal bridge with prominent forehead.
•Mild midfacial hypoplasia with narrow nasal passages
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19. •Short Limbed dwarfism
•Waddling gait with exaggerated lordotic
lumbar spine
• Prominent Buttocks
• Protuberant abdomen
•Short trident hand, fingers being similar in
length, with short proximal and mid phalanges.
•Short femoral neck; incomplete extension of
elbow.
•The metacarpals and phalanges are
shortened
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20. LeFort III osteotomy
Pre- treatment
Post- treatment
•Correction of class III
malocclusion and midface
deficiency in achondroplasia
ideally is accomplished by a
LeFort III osteotomy to move
the entire midface forward.
•Unfortunately, no analogous
surgical procedure exists to
deal with the limb deficiencies
although progress has been
made recently toward
lengthening the short limbs
TREATMENT
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21. CRANIOSYNOSTOSIS SYNDROMES
These constitute a group of conditions each characterized by premature fusion
of the cranial sutures resulting in disproportionate growth of the cranial bones
and as a sequence the growth of the facial bones.
TYPES OF
CRANIOSYNOSTOSIS
•Scaphocephaly (Sagital Suture)
•Trigonocephaly (Metopic suture)
•Turricephaly (Bilateral Coronal
•Suture)
•Plagiocephaly
•Frontal Plagiocephaly ( Unilateral Coronal Suture)
•Occipital Plagiocephaly (Unilateral Lambdoid Suture)
•Parallelism ( Frontal Bone/Contralateral Occipital Lobe)www.indiandentalacademy.comwww.indiandentalacademy.com
22. APERT SYNDROME
•The condition was reported by Wheaton in 1894.
• In 1906, Apert summarized nine cases, and in 1920,
Park and Powers published an exceptional essay on this
entity. Numerous cases have been reported.
AETIOLOGY:-
•Autosomal dominant
•Most cases sporadic
•Estimated in 1 in 160,000 live births
•Apert FGFR2 mutation lead to an increase in the
number of precursor cells that enter the osteogenic
pathway,leading ultimately to increased subperiosteal
bone matrix formation and premature calvaria
ossification during fetal development
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23. Clinical FeaturesClinical Features
Craniofacial.Craniofacial.
Short anteroposterior diameterShort anteroposterior diameter
with high, full forehead and flatwith high, full forehead and flat
occiput.occiput.
Irregular craniosynostosis,Irregular craniosynostosis,
especially of coronal suture.especially of coronal suture.
Fontanels may be large and lateFontanels may be large and late
in closure.in closure.
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24. Flat facies,Flat facies,
Supraorbital horizontalSupraorbital horizontal
groove,groove,
Shallow orbits,Shallow orbits,
Hypertelorism,Hypertelorism,
Strabismus,Strabismus,
Down-slanting of palpebralDown-slanting of palpebral
fissures,fissures,
Small nose,Small nose, ..
Lips frequently assume aLips frequently assume a
trapezoidal cofiguration in thetrapezoidal cofiguration in the
relaxed state.relaxed state. www.indiandentalacademy.comwww.indiandentalacademy.com
25. Maxillary hypoplasiaMaxillary hypoplasia
The maxillary dental arch may beThe maxillary dental arch may be
V-shaped with severely crowded teeth andV-shaped with severely crowded teeth and
bulging alveolar ridges..bulging alveolar ridges..
Narrow palate with median groove, with orNarrow palate with median groove, with or
without cleft palate or bifid uvula.without cleft palate or bifid uvula.
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26. Delayed and/or ectopic eruption and shovel-Delayed and/or ectopic eruption and shovel-
shaped incisors.shaped incisors. ..
Supernumerary teeth have been observed.Supernumerary teeth have been observed.
Class III malocclusion is usually present, withClass III malocclusion is usually present, with
anterior open bite or cross bite and unilateral oranterior open bite or cross bite and unilateral or
bilateral posterior crossbite.bilateral posterior crossbite.
DENTAL ANOMALIESDENTAL ANOMALIES
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27. OTHER CLINICAL
FINDINGS
Syndactyly in varying degrees generally affecting second to
fourth fingers and usually all toes.
Considerable range of mental deficiency noted although
normal intelligence occasionally found.
Numerous skeletal abnormalities of elbow, shoulder, lip,
and cervical spine.
Various cardiac and gastrointestinal malformations are
noted.
Acne vulgaris about forearms common
Hearing loss.
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28. Goals of surgery
-Increase intracranial space in the
anterior cranial vault for the brain.
-To increase the orbital volume.
-To improve the morphology of the
forehead and upper orbits.
Thus it requires
-Bicoronal suture release with
decompression
-simultaneous cranial vault and
upper orbital Osteotomies with
reshaping and advancement.
Usually done at 9 to 11 months
of age unless signs of increased
intracranial pressure are identified
earlier in life.
PRE POST
TREATMENT
TREATMENT
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29. CROUZON DISEASECROUZON DISEASE
InIn 1912 Crouzon1912 Crouzon first described a woman and her sonfirst described a woman and her son
with this disorder.with this disorder.
InIn 19151915 he reported a family in which seven of twenty onehe reported a family in which seven of twenty one
members affected and thus stressed themembers affected and thus stressed the genetic aspectsgenetic aspects
of the syndromeof the syndrome
ETIOLOGYETIOLOGY
Autosomal dominantAutosomal dominant
Wide range of expressivityWide range of expressivity
About one third cases arise as a new mutationAbout one third cases arise as a new mutation
CLINICAL FEATURES
•Premature closure, especially of coronal suture,
occasionally lambdoidal.
•Variable cranial form depending on order and rate of
progression of suture closure
• Optic nerve damagewww.indiandentalacademy.comwww.indiandentalacademy.com
30. FACIAL FEATURE
Exophthalmos
Divergent strabismus
Hypertelorism
Short upper lip
Hypoplasia of maxilla with
relative mandibular prognathism
Nose may appear beaked because
of midfacial hypoplasia
High arched, short palate
V-shaped maxillary dental arch.
Crowding of the upper teeth.
Class III Malocclusion
Bilateral Atresia of Auditory
meatus noted occasionally
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31. PFEIFFER SYNDROME
• Described by Pfeiffer in 1963 in eight members of one family
•Autosomal dominant inheritance pattern.
ETIOLOGY:
• It is heterogeneous because it is caused by a single recurring
mutation(Pro 252 Arg) of the FGFR1 gene and by several different
mutations affecting FGFR2.
•Type I
•Type II
•Type III
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32. ·Generally compatible with life
• Tower Skull
· Normal or near normal intelligence
· Bilateral coronal synostosis
· Midface growth often normal.
· Minimal Ocular proptosis..
· Hydrocephalus is absent.
· Supraorbital ridge recessed.
· Anterior cranial base short and wide
• Orbits are shallow,eyes are proptopic
with a degree of hypertelorism
•Syndactyly
• Great toe deviated medially
PFEIFFER SYNDROME
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33. TREATMENT
• Primary cranio-orbital decompression and reshaping in
infancy at 9-11 months of age.
•Management of total midface deformity.
•A frontofacial(monobloc) osteotomy with horizontal
advancement and need for additional segmentation of the orbits
to normalize the dysmorphology is generally required.
•Le Fort III osteotomy.
•Age of treatment:5 to 7 years of age.
•Management of orthognathic deformity in adolescents.
•Mandible has a normal basic growth pattern, as the growth of
maxilla may be limited, Angles class III malocclusion with
anterior open bite often results
•A Le Fort I Osteotomy with horizontal advancement
Segmentation in combination with an oblique osteotomy of the
chin(genioplasty) to correct lower face deformity.
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34. •Autosomal dominant, high degree of
penetrance
•Flattening of midface and nasal bridge
•Facial Asymmetry
•Micrognathia
•Submucous palatal cleft
•Epicanthal folds
•Myopia
•Hearing Loss
•Bony enlargement of joints
•Joint hypermobility
•Hip joint Deformity
•Occasional Mental Retardation
STICKLER SYNDROME (1965)
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35. CLEIDOCRANIAL DYSPLASIA
Syn. : Marie Sainton Disease, Cleidocranial dysostosis
In 1765 Martin first reported the clavicular findings
In 1897 Marie and Sainton noted the genetic
transmission and coined the name cleidocranial
dysostosis
In 1975 Goodman and co-workers described an
autosomal recessive form of the disease
Autosomal dominant with wide variability in expression,
but usually showing penetrance.
Autosomal Recessive disease is usually more severe
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36. CLINICAL FEATURES:
Growth.
Slight to moderate shortness of stature.
Craniofacial.
Brachycephaly with bossing of frontal,
parietal, and occipital bones;
Late closure of fontanels and
mineralization of sutures;
Late or incomplete development of
accessory sinuses and mastoid air cells;
Wormian bones
Small sphenoid bones.
Calvarial thickening.
Midfacial hypoplasia with low nasal
bridge, narrow high-arched palate.
Hypertelorism.
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37. Late eruption,
Malformed roots,
Retention cysts,
Enamel Hypoplasia,
Caries,
Supernumerary teeth.
•Partial to complete
aplasia of clavicle with
associated muscle
defects,
•small thorax with short
oblique ribs.
DENTITION
CLAVICLE AND CHEST
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38. MARFAN SYNDROME
A hereditary disease transmitted as an autosomal
dominant trait.
It is basically a disease of connective tissue related to a
defective organization of collagen. Here collagen is
abnormally soluble.
An outstanding feature is excessive length of tubular bones
resulting in :
Dolichostenomelia disproportionately long thin
extremities
Arachnodactyly spidery fingers.
Shape of face and skull is long and narrow
Hyperextensibility of joints, habitual dislocations,
Kyphosis or scoliosis, flat foot.
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39. Myopia is usually present
Cardiovascular
complications – aortic
aneurysm and aortic
regurgitation, valvular
defects, enlargement of the
heart.
High arched palatal vault
is very prevalent and may
be a constant finding.
Odontogenic cysts of
maxilla and mandible
TMJ dysarthrosis
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40. TREACHER COLLINS SYNDROME
• Autosomal dominant condition with variable expressivity.
• Bilateraly symmetrical abnormalities of structures within
the first and second branchial arches.
• Incidence:1 in 25,000 to 1 in 50,000 live births.
• Pathogenesis:- Theories include failure of differenciation
of the branchial arch mesoderm, defective facial bone
ossification, and tissue ischemia resulting from stapedial
artery hypoplasia.
• Behrents, Mcnamara, and Avery pointed out that all major
aspects of MFD are fully expressed by the 15th week of
embryonic development.
• Current research suggest that the abnormality may occur
early as developmental defects of the neural crest cells
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42. · MANDIBLE
• Mandibular defects
• Micrognathia
• Open bite
OTHER FEATURES
• Fish like facial Appearance
• Deafness frequent
• Mental retardation
Infrequent
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43. TREATMENT
• Zygomatic and orbital reconstruction.
• Maxillomandibular reconstruction.
• Nasal reconstruction.
• Soft tissue reconstruction
• External ear reconstruction.
• External auditory canal and middle ear reconstruction
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44. HEMIFACIAL MICROSOMIA
Syn.:- Goldenhar syndrome, Occulo Vertebral Dysplasia
The term Hemifacial microsomia was issued by Gorlin to describe
patients with unilateral microtia , macrostomia and failure of formation of
the mandibular ramus and condyle.
•Gorlin suggested that the Goldenhar syndrome was a variant of this
complex characterized in addition by vertebral anomalies and epibular
dermoids
AETIOLOGY :-
•The mode of inheritance is not established with rare familial aggregation
•Multifactorial, Drugs- Thalidomide, Triazene
•Poswillo, using animal model of hemifacial microsomia, was able to
show that destruction of differentiating tissues in the region of the ear
and jaw by an expanding hematoma produced a branchial arch dysplasia.
•Thus a vascular abnormality can disrupt the normal embryologic
development of first and second branchial archeswww.indiandentalacademy.comwww.indiandentalacademy.com
45. CLINICAL FEATURES
•Prominent fore head
•Midfacial Asymmetry
•Varying malformation of the mandible
•Flattened gonial angle and narrowed maxilla
on the involved side
•Maxillary, temporal and malar bones on the
involved side are reduced in size
•Muscles of palate, mastication, tongue, face
may be hypoplastic or paralyzed
•Malformation of the external ear may vary
from displaced pinna to complete aplasia
•Macrostomia of mild degree and crowding of
teeth
•Palpebral fissures slant downwards
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46. OTHER CLINICAL FEATURES
•Mild mental retardation
occasionally
•Bony anomalies, especially of the
vertebral column
•Congenital heart diseases like
VSD and hypoplasia of the lung
TREATMENT
•Mandibular osteotomies to reposition the mandible.
-Unilateral mandibular osteotomy.
-Bilateral mandibular osteotomy.
-Le fort I osteotomy to correct the maxilla.
•Functional orthopedic appliance therapy.
•Orthodontic therapy after combined surgical correctionwww.indiandentalacademy.comwww.indiandentalacademy.com
47. FRONTONASAL DYSPLASIA
Syn.:- Median Cleft Face Syndrome
AETIOLOGY :-
•The basic defect is unknown but thought to result from failure of flow of
ectomesenchyme allowing brain vesicle to fill space normally occupied
by the nasal capsule
CLINICAL FEATURES:-
•Facial alteration graded from mild to severe
•Anterior encephalocoele in some
•Occular hypertelorism
•Microopthalmia
•Nose often flattened with widely spaced nostrils
•Cleft lip
•Mental Retardation
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49. PEIRRE ROBIN ANOMALAD
In 1923 Robin reported this condition, In 1975 the term
anomalad was joined
AETIOLOGY
•May be isolated or in association with other syndromes eg.
Stickler syndrome, Fetal Alcohol Syndrome, drug induced
syndromes.
•The primary defect lies in arrested development and
ensuing hypoplasia of the mandible, ultimately producing
the characteristic “bird facies”.
•This in turn prevents the normal descent of the tongue
between the palatal shelves, resulting in cleft palate.
Because of this mechanism a cleft lip is not found in
conjunction with the cleft palate.
•Incidence is 1 in 39,000 live births
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50. CLINICAL FEATURES
•The primary components are cleft palate
micrognathia and glossoptosis.
•Occasional hydrocephaly or
microcephaly
•U shaped cleft palate
• Mandibular hypoplasia
•Eye anomalies such as congenital
cataract
•Deafness
•Mental retardation
•Sternal Anomalies
•Congenital heart defects
•Jaw malformation may result in
respiratory difficulty as the tongue may
fall back and obstruct the epiglottis.
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51. NAGER ACROFACIAL DYSOSTOSIS
•Possibly autosomal Recessive inheritance
CLINICAL FEATURES
•Malar Hypoplasia
•Down slanting palpebral fissures
•Absent Eyelashes
•Cup shaped low set ears
•Cleft palate
•Micrognathia
•Missing or hypoplastic thumbs
•Syndactyly
•Abnormal Radius or Ulna
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52. MOEBIUS SYNDROME
Syn.:- Congenital Facial Diplegia
•Basic defect is unknown but nuclear hypoplasia accounting for
cranial nerve paralysis has been documented
CLINICAL FEATURES
•Both 6th
and 7th
nerve palsy.
•Bilateral facial paralysis
•Convergent Strabismus
•Protruding malformed ears
•Micrognathia, high broad nasal bridge
•Atrophy of tongue due to lack of hypoglossal innervation
•Drooping angles of the mouth
•Mental retardation
•Limb reduction defects www.indiandentalacademy.comwww.indiandentalacademy.com
54. KLINEFELTER SYNDROME
• Due to non disjunction of the X chromosome during meiosis
• Commonly affects males and the have an extra X chromosome
XXY, XXXY
CLINICAL FEATURES
• Skeletal disproportion
• Mandibular prognathism
• Hypertelorism, Strabismus
• Upward slant of the Palpebral fissure
• Depressed Nasal bridge
• Cleft Palate
• Taurodontism
• Mental Deficiency www.indiandentalacademy.comwww.indiandentalacademy.com
55. Syn.:- Brittle bones disease
•Van der Hoeve in 1918
•Disturbance of mesodermal tissues especially calcified tissues
CLINICAL FEATURES
•Extreme fragility and porosity of bones with increased
proneness to fracture
•Blue Sclera, D/D Ehler Danlos syndrome, Marfan syndrome
•Dentinogenesis Imperfecta
•Triangular facies with craniofacial disproportion
•Ears displaced/ Deafness
•Mandibular prognathism
•Short stature due to repeated fractures
•Loose ligaments- dislocation of the TMJ
OSTEOGENESIS IMPERFECTA
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56. GORLIN AND GOLTZ SYNDROME
(Jaw cyst basal cell nevus bifid rib syndrome)
1st described by Binkley and Johnson in 1951, thoroughly
reviewed by Gorlin et al.
It is transmitted as an autosomal dominant trait
CLINICAL FEATURES
•Cutaneous anomalies : Basal
cell carcinoma, other benign
dermal cysts and tumors.
•Palmar pitting, palmar and
plantar keratosis and dermal
calcinosis.
• Dental and osseous lesions :
Odontogenic keratocyst (often
multiple), mandibular
prognathism.
•Bifid ribs, vertebral anomalies,
Basal Cell Carcinoma
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57. Eyes Hypertelorism with wide nasal
bridge,congenital blindness
Neurologic anomalies Mental
retardation, dural calcification,
agenesis of corpus callosum,
congenital hydrocephalus
Sexual abnormality Hypogonadism
in males, ovarian tumors
Oral findings odontogenic
keratocysts
As they often develop early in life,
deformity and displacement of
developing teeth may occur.
TREATMENT
•Several cases of ameloblastoma have developed in
cysts of this syndrome
•This emphasizing the importance of surgery of
normal cysts and and their histologic examination
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58. Syndromes Associated With Problems
Of Facial Height
•Amelogenesis Imperfecta
•Beckwith – Weidmann Syndrome
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59. BECKWITH WIEDMANN SYNDROME
•Uncertain mode of inheritance although familial occurrence has
been observed
CLINICAL FEATURES
•Macroglossia
•Anterior open bite
•Mandibular prognathism
•Earlobe grooves
•Depression on posterior rim of ear
•Exopthalmos
•Infra orbital hypoplasia
•Midfacial recession
•Mild mental retardation
•Neonatal hypoglycemia www.indiandentalacademy.comwww.indiandentalacademy.com
61. FACIAL HEMIHYPERTROPHY
AETIOLOGY
•Condition has been ascribed to hormonal imbalance, chromosomal
abnormalities, Localized alteration on intrauterine development,
lymphatic abnormalities vascular abnormalities
CLINICAL FEATURES
•Enlargement of one half of the head
•Dentition on the affected side of abnormal
shape and size
•Both bone and soft tissue is involved
•Tongue involvement is common
•Mandibular hemihypertrophy
•May be associated with Wilm’s tumor of
the kidney and adrenal cortical carcinoma
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62. FACIAL HEMEATROPHY
Syn.:- Parry Romberg Syndrome
AETIOLOGY
• Exact cause is unknown but suggested factors are
• Onset is noticed in the 1st
or 2nd
decade of life as a white line,
furrow on one side of the face or brow near the midline
• This initial lesion extends to include atrophy of the skin,
subcutaneous tissue muscle and bone depending on the severity
of the atrophy
1. Trophic malformation of the
cervical sympathetic system
2. Trauma
3. Infection
4. Heredity
5. Peripheral Trigeminal Neuritis
6. Form of localized Scleroderma
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63. CLINICAL FEATURES
•Unilateral facial involvement
•Trigeminal neuralgia or parasthesia
•Vitiligo
•Atrophy of half of tongue
•Delay in dental eruption on the affected
side
•Eye involvement
•Migraine headaches or epilepsy
•Affected skin becomes darkly pigmented
•Loss of facial hair
•Predilection for involvement of left side
of face
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64. NEUROFIBROMATOSIS
Syn.:- Von Recklinghausen’s disease
•Simple autosomal dominant inheritance with variable penetrance
•Incidence of 1 in 3000 live births
•Two types- superficial nodules, deeper diffuse lesions
CLINICAL FEATURES
•Café- au-lait spots
•Neurofibromas of tongue skin
•Skeletal defectswhen tumor is located within bone
•Endocrinedisturbances
•Macrocephaly
•Mandibular asymmetry
•Malignant transformation of lesions in 15%
•Associated congenital defect mental retardation and ocularwww.indiandentalacademy.comwww.indiandentalacademy.com
65. Syndromes Associated With Ocular
Hypertelorism
•Apert Syndrome
•Basal Cell Nevus Syndrome
•Crouzon Syndrome
•Frontonasal dysplasia
•Fetal Face Syndrome
•Hypertelorism- Hypospadias Syndrome
•Noonan Syndrome
•Oro facial Digital Syndrome
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66. FETAL FACE SYNDROME
•Syn.:- Robinow Syndrome
•Both autosomal dominant and recessive inheritance
CLINICAL FEATURES
•Facial resemblance to a fetus of 8 weeks
•Frontal bossing
•Hypertelorism
•Short upturned nose
•Macrocephaly
•Triangular mouth with downturned corners
•Dental malalignment
•Vertebral anomalies www.indiandentalacademy.comwww.indiandentalacademy.com
68. ORO FACIAL DIGITAL SYNDROME
In 1954 Papillon described a syndrome characterized by
congenital anomalies of the face, oral cavity, and digits
X-linked dominant
Possibly 1 in 250,000
CLINICAL FEATURES
Frontal bossing
Alopecia or dryness of scalp
Pseudocleft of mid-upper lip
Cleft or defect of hard palate
Malposition of teeth
Supernumerary teeth
Hypoplasia of malar bones
Broad nasal root
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69. Cleft tongue
Ankyloglossia
Absence of lower lateral incisors
Thick fibrous bands (frenula) in
upper and lower mucobuccal
fold
mental retardation
Abnormalities of hair and
sebaceous glands
Digital anomalies include
syndactyly, clinodactyly,
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70. Syndromes Associated With Chromosomal
Disorders
• Down Syndrome
•Trisomy 18
•Trisomy 13
•Turner Syndrome
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71. DOWN SYNDROME
In 1866 Langdon Down first adequately described this
syndrome.
This disease is the most common chromosomal abnormality
to occur in man.
3 forms of down syndrome:
a) Typical trisomy 21 with 47 chromosomes (95% of cases)
b) Translocation type- Here the extra chromosome material of
number 21 is translocated to another chromosomed of G or
D group (3%)
c) Chromosomal mosaicism (about 2% of cases)
The risk of having an affected child of the typical trisomy 21
type is one is 2000 live births in women under 30 years of age
Rises dramatically to one is 50 live births is women over 45
years of age.
Overall incidence is 1 in 600 live births
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72. CLINICAL FEATURES
Brachycephalic skull
Open metopic suture, late closure of
fontanels
Hypoplastic maxilla and nasal bones
Flattening of nasal bridge, orbital ridges,
and maxilla
Ocular hypotelorism
Short hard palate
Cleft lip/palate about 3 times frequency in
normal population
Protruding fissured tongue with hypertrophy
of papillae
Dentition delayed, increased periodontal
disease, and reduced dental caries
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73. Oblique palpebral fissuresOblique palpebral fissures
Epicanthal foldsEpicanthal folds
Spotting of irisSpotting of iris
CataractsCataracts
StrabismusStrabismus
Small earSmall ear
Excessive skin on napeExcessive skin on nape
Reduced HeightReduced Height
Delay in skeletal maturationDelay in skeletal maturation
Short Broad NeckShort Broad Neck
Hypermobility of JointsHypermobility of Joints..
Cardiac abnormalitiesCardiac abnormalities
IQ range from 25 to 70 and decreases with age.IQ range from 25 to 70 and decreases with age.
Simian crease and clinodactylySimian crease and clinodactylywww.indiandentalacademy.comwww.indiandentalacademy.com
74. TRISOMY 18 – EDWARDS SYNDROME
•Triplication of chromosome 18, most common cause is non disjunction.
CLINICAL FEATURES
•Prominent occiput
•Micrognathia
•Small oral opening
•Short upper lip
•Cleft lip and palate
• narrow palatal arch
•Short palpebral fissures
•Corneal opacities
•Microopthalmos
•Low set malformed ears
•Short neck www.indiandentalacademy.comwww.indiandentalacademy.com
75. TRISOMY 13 – PATAU SYNDROME
•Trisomy 13 stated account for 15 % of the spontaneous abortions and
there is a substantial tendency for recurrence
CLINICAL FEATURES
•Microcephaly
•Port wine stain in glabellar area
•Scalp defects in parietoccipital area
•Absent eye brows
•Shallow supraorbital ridges
•Micropthalmos with multiple eye defects
•Deafness
•Cleft lip and palate
•Micrognathia
•Congenital abnormalities involving heart, kidney, brain and GITwww.indiandentalacademy.comwww.indiandentalacademy.com
76. TURNER SYNDROME
About 1% of monosomy X female embryos
survive.
The incidence of 45, X or Turner syndrome in
newborn females is approximately 1 in 8000 live
births.
It accounts for about 18% of all abortions
caused by chromosome abnormalities.
Half the affected individuals have 45, X; the
other half have a variety of abnormalities of a
sex chromosome
The phenotype of Turner syndrome is female.
Secondary sexual characteristics do not
develop in 90% of affected girls and hormonal
replacement www.indiandentalacademy.comwww.indiandentalacademy.com
77. CLINICAL FEATURES
1. Facies -shows premature
aging
2. Face frequently heart shaped
3. Multiple eye findings
including cataract,
strabismus, blue sclera.
4. Color Blindness
5. Depressed Corners of mouth
6. High arched palate
7. Dental malocclusion
8. Micrognathia
9. Prominent ears
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78. OTHER CLINICAL FINDINGSOTHER CLINICAL FINDINGS
Short stature, average adult height 140 cmShort stature, average adult height 140 cm
Webbed neckWebbed neck
Shield-like chestShield-like chest
Undeveloped breastsUndeveloped breasts
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79. Low hairline on posterior neck
Infant often presents with
lymphedema of hands and feet
Short fourth metacarpal bone
in some patients
Hypoplastic toenails
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80. Environmental Induced Syndromes
An environmental agent which is demonstrated to have a
statistically significant, nonrandom association with a
particular congenital malformation or set of malformation
is termed as environmental teratogenic agent or
teratogen.
•Potter Syndrome
•Fetal Alcohol Syndrome
•Fetal Hydantoin Syndroem
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81. POTTER SYNDROME
AETIOLOGY :- Oligohydraminos- reduced amniotic fluid
CLINICAL FEATURES
•Occular hypertelorism
•Prominent skin fold from the inner canthus to the cheek
•Flattened nose with downward turned nasal tip
•Large flattened ears
•Micrognathia
•Mandibular asymmetry
•Congenital sternomastoid torticollis
•Clefts of lip and palate
•Micropthalmia
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82. FETAL ALCOHOL SYNDROME
Infants born to chronic alcoholic mothers
exhibit a specific pattern of defects, including
prenatal and postnatal growth deficiency,
mental retardation, and other anomalies.
Exposure to high level of ethanol at an early
stage of fetal development produces fetal
alcohol syndrome [FAS]
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83. Deficiencies of midline tissue
of the neural plate very early in
embryonic development give
rise to a sires of malformation
collectively known as the
holoprosencephalies which
are characterized by a failure of
the first three ventricles of the
brain to separate
The olfactory placodes which
are partly derived from the
anterior neural plates, are too
close together, and this causes
deficient development of the
median and nasal
prominences.
The result is a spectrum of
facial deformities ranging from
total absence of the nose and
related structures to an intact
but moderately
underdeveloped midface.
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84. FETAL HYDANT01N SYNDROME
The fetal hydantoin syndrome occurs in 5 to 10% of
children born to mothers treated with phenytoins or
hydantoin anticonvulsants.
GENERAL CLINICAL FEATURES
Short neck,
Rib anomalies,
Umbilical and inguinal hernias,
Coarse profuse scalp hair, hirsutism,
Low-set hairline,
Abnormal palmar crease.
Strabismus.
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85. CLINICAL FEATURES
Microcephaly
Wide anterior fontanel
Metopic ridging
Ocular hypertelorism
Broad, depressed nasal bridge
Short nose with bowed upper lip
Broad alveolar ridge
Cleft lip and palate
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86. Limbs
Hypoplasia of distal phalanges with
small nails, especially postaxial digits;
Low arch dermal ridge patterning of
hypoplastic fingertips;
Digitalized thumb;
Dislocation of hip.
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87. Other Common Syndromes
• Binder Syndrome
• Ehlers Danlos Syndrome
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88. BINDER SYNDROME
Syn. :- Maxillonasal Dysostosis
•Affects primarily the anterior part of the maxilla and nasal complex
•Initially described by Noyes in 1939, but clinically defined as a syndrome
by Binder in 1962
CLINICAL FEATURES
•Failure of development in the premaxillary
area, with associated deformities of the nasal
skeleton
•Rudimentary nasal spine
•Mandibular prognathism
•Increased gonial angle
•Typical Class III occlusion with marked
proclination of the upper incisors
•Poorly developed philtrum
•Half moon appearance of external nares
inferiorly
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89. EHLERS – DANLOS SYNDROME
(CUTIS HYPERELASTICA)
Originally described by Van Meekeren in 1682
Further classified by Ehler in 1901 & Danlos in
1908
Ten distinct forms of syndrome have now been
delineated
EDS I, EDS II, EDS III are more common.
Inherited as autosomal dominant
EDS V has X-linked inheritance.
It is a hereditary disorder of connective tissue with
abnormal collagen
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90. Auricles are hyper mobile with
tendency towards “lop ears”.
Mitral valve prolapse with or
without tricuspid valve prolapse.
Oral mucosa is of normal color
but excessively fragile and
bruised easily.
Healing of mucosa is slightly
retarded and no remarkable hyper
extensibility of mucosa can be
demonstrated.
Gingival bleeding after brushing
Hyper mobility of TMJ, repeated
dislocations
•CLINICAL FEATURES
•Hyperelasticity of skin, hyperextensibility of joints and fragility of skin
and blood vessels resulting in excessive bruising as well as defective
healing of skin wounds.
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91. Accurate diagnosis of a specific syndromes among the 0.7% of
babies born with multiple malformations is a necessary pre
requisite of providing a prognosis and plan of management for
the affected infant, as well as genetic counseling for the
parents.
Until recent years, treatment of dentofacial deformity
concentrated on the dentition, orthodontic treatment was the
accepted approach to these problems. However the situation
has changed with explosive rapidity and new treatment options
involving comprehensive intervention have enhanced the
management of craniofacial syndromes.
When dealing with craniofacial syndromes and the individuals
affected by them it is worthwhile to keep in mind the words of
James Paget
“We ought not to set them aside with idle thoughts or idle
words about "curiosities" or "chances." Not one of them is
without meaning; there is not one that might not become thewww.indiandentalacademy.comwww.indiandentalacademy.com
92. REFERENCES
• Oral & maxillofacial pathology, 2nd edi, Neville.
• Text book of oral pathology, 4th edi, Shafer.
• Atlas of “The face in genetic disorder” Richard &
Goodman 2nd edi.
• Syndromology : an updated conceptual overview Int. J.
Oral Maxillofacial
Surg. 1989
• Surgical Correction of Dentofacial Deformities, Bell
Proffit and White
• J.W. Fergnson and RPJ Thomson EJO 7:145
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