The document summarizes the development of the face from the 4th week of embryonic development. It discusses how the frontonasal prominence, maxillary prominences, and mandibular prominences form the basic structures of the face. It describes the development of specific structures like the nose, palate, ears, eyes, lips, and tongue from these prominences. It also discusses the theories of craniofacial growth and the role of the pharyngeal arches, pouches, grooves, and clefts in facial development.

1. Guided by :
Dr S. Prakasam Presented by
Dr K.C. Ponnappa Dr.Supratim tripathi

2. Development of face
We are the lucky ones amongst the millions of
those who could not make it.

3. Contents
• Introduction
• Theories of development
• Pharyngeal arches , pouches, grooves , clefts.
• Development of face
• Clinical aspects
• Conclusion
• References

4. Introduction
• The embryonic period extends from the
beginning of the fourth week till the end of the
eighth week.
• The trilaminar embryonic area differentiates as
follows:
1] ectoderm providing protection,
2] endoderm providing nutrition ,
3] mesoderm forming skeletal tissue ,muscle and
blood vascular system
• The fourth week is characterized by the
differentiation of the three germ layers and the
folding of the embryo
• In the second month the organs and tissues are
laid down and the embryo has a distinct human
appearance

5. Phases of development
1)Growth-this is the increase in the size by cell division
2)Morphogenesis-this is the development of form
3)Differentiation-includes the maturation of physiological processes
 Folding of the embryo:-
The trilaminar germ disc is flattened and pear-shaped .The cells in
the central part grow more rapidly than those at the
periphery.Because of the lack of space ,the embryo undergoes head
fold ,tail fold and lateral folds by the end of the third week .This
converts the flattened germ disc into a cylindrical embryo.

6. Theories of development
• The growth is strongly influenced by the genetic factors ,but it also can be
significantly affected by the environment in the form of nutritional status, degree
of physiological activity ,health or illness and a number of similar factors.
• Three main theories in the recent years have attempted to explain the
determinants of craniofacial growth:
(1)Bone ,like other tissues ,is the primary determinant of its own growth,
(2)Cartilage is the primary determinant of skeletal growth , while the bone responds
secondarily and passively
(3)The soft tissue matrix in which the skeletal elements are embedded as the primary
determinant of growth

7. Functional matrix theory of growth
• This theory was given by MOSS , in 1960.
• He theorizes that growth of the face occurs as the response to functional
needs and neurotrophic influences and is mediated by the soft tissue in
which the jaws are embedded.
• In this view the soft tissues grows and the bone and cartilage react.
• This phenomenon can be seen readily in humans in three experiments of
nature:
a)Microcephaly ,
b)Hydrocephaly,
c)Size of the orbit.

9. Development of face
• The most typical feature in the development of the head and neck
is formed by the pharyngeal or branchial arches.
• These arches appear in the 4th and 5th weeks of development and
contribute to the characteristic external appearance of the embryo.
• Initially there are bars of mesenchymal tissue separated by deep
clefts known as pharyngeal or branchial clefts .

10. • With the development of the arches and clefts ,a number of
outpocketings arise ,the pharyngeal pouches .
• Although the development of pharyngeal arches ,clefts ,and
pouches resembles formation of gills in fish and amphibia,in
the human embryo; real gills (branchia)are never formed.
• Therefore the term pharyngeal has been adapted.

11. • When the embryo is 4 ½ weeks old five mesenchymal prominenses can be
recognised
-the frontonasal prominence , a slightly rounded elevation cranial to the
stomodeum
-the maxillary prominences (dorsal portion of the 1st arch),lateral to the
stomodeum ;
-the mandibular prominences ,caudal to the stomodeum;
-Development of face is later complemented by the formation of the nasal
prominences .
1 Frontonasal
prominence
2 Maxillary
prominences
2 Mandibular prominences
Stomodeum

12. Pharyngeal arches
• Each pharyngeal arch is charrecterized by its
own muscular components ,their nerve
component and it’s own arterial component.

13. First pharyngeal arch
• Consists of two portions:
 Dorsal:-also known as the maxillary process , giving rise to premaxilla ,maxilla,
zygomatic bone and part of the temporal bone.
 Ventral:- also known as the mandibular process , which contains the MECKEL’S
CARTILAGE. During further development , Meckel’s cartilage disappears except for
two small portions at it’s dorsal end that persist and form the Incus and Malleus.
Musculature of the 1st pharyngeal arch includes the muscle of mastication
(temporalis,masseter,lateral pterygoid and medial pterygoid),anterior belly of the
digastric,mylohyoid,tensor tympani and tensor palatini.

14. Nerve supply to the muscles of the 1st arch is provided by Mandibular
branch of Trigeminal nerve.
Sensory supply of the skin of the face is provided by Ophthalmic , Maxillary
and Mandibular branches of the Trigeminal nerve.
 Muscles of the different arches do not always attach to the bony or
cartilagenous components of their own arch but sometimes migrate into
surrounding regions. The origin of these muscles can be traced ,since their
nerve supply is derived from the arch of origin.

15. Second pharyngeal arch
• The cartilage of the second arch (hyoid arch)is
called as REICHERT’S CARTILAGE which gives rise
to the stapes ,styloid process of the temporal
bone , stylohyoid ligament, the lesser horn and
upper part of the body of the hyoid bone .

16. • Muscles of the hyoid arch are the stapedius ,
stylohyoid,posterior belly of digastric ,auricular
and mucles of facial expressions.
• Facial nerve-the nerve of the 2nd arch supplies
all these muscles.

17. Third pharyngeal arch
• The cartilage of this arch produces the lower part of
the body and the greater horn of the hyoid bone.
• Muscle of this arch is the Stylopharyngeus muscle
which is innervated by the Glossopharyngeal nerve.

18. Fourth and Sixth pharyngeal arches
• Cartilagenuos components of the 4th and 6th pharyngeal arches fuse
to form the thyroid, cricoid , arytenoid , corniculate and cuneiform
cartilages of the larynx.
• Muscles of the fourth arch are supplied by the Superior laryngeal
branch of the Vagus nerve and the intrinsic muscles of the larynx
are supplied by Recurrent laryngeal branch of Vagus nerve

19. Pharyngeal pouches
• The human embryo has five pairs of pharyngeal pouches.
• The last one of these is atypical and is considered as a part of the 4th
• First pharyngeal pouch:
It forms a stalk like diverticulum –the tubotympanic recess;
 one side of this comes in contact with the external auditary meatus
 The distal aspect of this widens in a sac like structure ,the primitive
tympanic or middle ear cavity
 The proximal part remains narrow forming auditary (eustacian )tube.
 The lining of the tympanic cavity forms the tympanic membrane or the
eardrum

20. • Second pharyngeal pouch:
The epithelial lining forms the primordium of the palatine tonsil.
• Third pharyngeal pouch:
It is characterized by a dorsal and a ventral wing .The dorsal wing
proliferates to form the inferior parathyroid gland and the ventral
wing forms the thymus

21. • Fourth pharyngeal pouch:
Epithelium of the dorsal wing of this pouch forms the superior
parathyroid gland.
• Fifth pharyngeal pouch :
The 5th pouch is the last one to develop and is considered to be a part
of the 4th pouch.This pouch leads to the development of
ultimobranchial body which in future leads to the development of
thyroid gland.

22. Pharyngeal grooves
• The 2nd ,3rd & 4th pharyngeal grooves merge together to form the
cervical sinus which with further development disappears .
• The 1st pharyngeal groove forms the external auditory meatus
which is the only invagination on the side of the head.

23. Pharyngeal cleft
• The 5 week embryo is characterized by the presence of 4
pharyngeal clefts of which only one contribute to the development
of the definitive structure of the embryo.
• The dorsal part of the 1st cleft gives rise to the external auditory
meatus

24. Clinical correlates
• Branchial fistulas :
When the 2nd pharyngeal arch fails to grow caudally over the 3rd and
the 4th arches , leaving remnants of the 2nd 3rd &4th clefts in contact
with the surface by a narrow canal.
• Internal branchial fistulas :
It is very rare and in this the cervical sinus is connected to the lumen of
the pharynx by a small canal which usually opens in the tonsillar
region.

26. Development of face
• DEVELOPMENT OF MAXILLA
• DEVELOPMENT OF MANDIBLE
• DEVELOPMENT OF NOSE
• DEVELOPMENT OF PALATE
• DEVELOPMENT OF EAR
• DEVELOPMENTOF EYE
• DEVELOPMENT OF LIP
• DEVELOPMENTOF TONGUE

27. Derivatives of Facial Components
The frontonasal
prominence forms the:
 Forehead and the bridge
of the nose
 Frontal and nasal bones
The maxillary prominences form the:
 Upper cheek regions and most of the upper lip
 Maxilla, zygomatic bone, secondary palate

28. The mandibular
processes fuse and form
the:
 Chin, lower lip, and lower
cheek regions
 Mandible
The lateral nasal prominences form the ala of the
nose
The medial nasal prominences fuse and form the
intermaxillary segment

29. Chronological development of
different parts of the head
• The development of the face occurs mainly between 5 – 8 weeks
• The lower jaw (mandible) is the first to form (4th week)
• The facial proportions develop during the fetal period (9th week to
birth)
• During infancy & childhood, following the development of teeth
and paranasal sinuses, the facial skeleton increases in size and
contribute to the definitive shape of the face

30. stomodeum
THE FACE and ORAL REGION
maxillary
process
a. Face consists of a few primordial tissue
masses partially surrounding the future mandibular
oral region. arch 5 weeks
4 weeks
b. The oral cavity (stomodeum) is an
ectodermal depression separated from the
foregut by the Oral plate formed of ectoderm
on one side and endoderm on the other.
c. The face is dominated by the frontal 5.5 weeks 6 weeks
prominence of the overhanging forebrain.
d. Laterally, the maxillary processes of the
1st branchial arch are visible.
7 weeks 8 weeks

31. • 1. On either side of the frontal prominence are stomodeum
horseshoe-shaped elevations are found maxillary
around the nasal placodes. process
• a. Medial limbs are nasomedial processes
• b. Lateral limbs are nasolateral processes
mandibular
arch 5 weeks
• 2. Growing toward midline are the maxillary 4 weeks
processes, approaching mandibular arches and nasomedial
process
merging with them at the angle of the mouth.
• a. Maxillary processes grow to crowd the nasal
processes closer together.
• b. Nasomedial processes grow quickly, pushing
5.5 weeks 6 weeks
the frontal prominence , then fuse with
the
maxillary processes to complete the arch
philtrum
of the
upper jaw.
• c. Nasomedial tissues give rise to philtrum of
lip 7 weeks 8 weeks

33. • The single frontonasal
prominence ventral to the
forebrain
• The paired maxillary
prominences develop
from the cranial part of
first branchial arch
• The paired mandibular
prominences develop
from the caudal part of
first branchial arch
Lateral view

34. DEVELOPMENT OF MAXILLA
During the initial 2 weeks
the maxillary prominences
continue to increase in size .
Simultaneously they grow in a
medial direction thereby
compressing the medial nasal
prominences toward the midline.
The cleft between the medial nasal prominences and the
maxillary prominence is lost.

35. • Initially the maxillary and the lateral nasal prominences are
separated by a deep furrow ,the nasolacrimal groove.
• The nasolacrimal duct then runs from the medial corner of the eye
to the inferior meatus of the nasal cavity.
• The maxillary prominence then enlarge to form the cheek and
maxillae .

36. Development of nasolacrimal duct
NASOLACRIMAL DUCT
- Develops as solid cord
– connects anterior from medial angle of
eye to nasal cavity eye to nasal cavity
- becomes canalized.
Obstructed Duct - failure of duct to canalize;
is opened surgically for tears to drain to nasal
cavity

37. DEVELOPMENT OF MANDIBLE
• “The Human Mandible Has No One Design For Life . Rather It
Remodels Through The Stages Of Life ,From The Slim Arbiter
Of Things To Come In The Infants ,Through The Powerful
Dentate Machine And Even Weapon In The Full Flesh Of
Maturity ,To The Pencil-thin Parcelain Like Problem That We
Struggle To Repair In The Adversity Of Old Age.”
- E Poswillo,1988
• The first structure to develop in the region of the lower jaw is the mandibular
division of the trigeminal nerve.
• The prior presence of the nerve has been postulated as requisite for inducing
osteogenesis by the production of neurotropic hormones.
• MECKEL’S CARTILAGE ,of the first branchial arch is responsible for the formation of
mandible.

38. • Single ossification centre for each half of the mandible arises in the 6th
week i.u.
• Ossification begins below and around the Inferior Alveolar Nerve and its
Incisive branch and upwards to form a trough for the developing teeth.
• Spread of intramembranous ossification dorsally and ventrally forms the
body and the Ramus of the mandible .
• The shape and size of he fetal mandible undergo considerable
transformation during it’s growth and development .

39. • The ascending ramus of the neonatal mandible is low and wide ;the
coronoid process is relatively large and projects well above the
condyle ;the body is merely an open shell containing the buds of the
deciduous teeth.
• The initial separation of the right and left bodies of the mandible at
the midline SYMPHYSIS MENTI is gradually eliminated between the
4th and 12th months postnatally.
• The main sites of postnatal mandibular growth are at the condylar
cartilages ,the posterior borders of the ramii and the alveolar ridges

40. Development of nose
• By the end of 4th week, Nasal
bilateral oval-shaped placode
ectodermal thickenings called Nasal
‘nasal placodes’ appear on placode
each side of the lower part of
the frontonasal prominence Frontonasal
prominence
• Nasal placodes are primordia
of the nose and nasal cavities.

41. • Mesenchymal cells proliferate
at the margin of the placodes
and produce horse-shoe
shaped swellings around
these.
• The sides of these swellings
are called ‘medial’ and ‘lateral’
nasal prominences
• The placodes now lie in the
floor of a depression called
‘nasal pits’
Each lateral nasal prominence is separated from the
maxillary swelling by nasolacrimal groove

42. Embryo: 6 weeks

43. • With the formation of the medial
and lateral nasal prominences,
the nasal placodes lie in the floor
of depressions called the nasal
pits
• By the end of 6th week, nasal pits
deepen and form nasal sacs

44. • Initially the nasal sacs are
separated from the oral
cavity by oronasal
membrane.
• The oronasal membrane
ruptures by the 6th week,
communicating the
primitive nasal cavities with
the oral cavity

45. • These communications are called the
primitive choana and are located
posterior to the primary palate
• After the development of the
secondary palate, the choana change
their position and become located at
the junction of nasal cavity and the
pharynx

46. • The nasal septum develops
as a downgrowth from the
internal parts of merged
medial nasal prominences
• Fuses with the palatine
process in 9-12 weeks,
superior to the hard palate

47. • The superior, middle and
inferior conchae develop on
the lateral wall of each nasal
cavity
• The ectodermal epithelium
in the roof of each nasal
cavity becomes specialized
as the olfactory epithelium

48. • The olfactory cells of the
olfactory epithelium give
origin to olfactory nerve
fibers that grow into the
olfactory bulb

49. 1.Eye placode
2.Nasal placode
3.Oronasal membrane
4.Nasal cavity
5.Tongue
1 3 6.Primary palate
4
7.Secondary palate
2
7
6 5

50. • Intermaxillary Segment: As a result of medial growth of the maxillary prominences ,the
two medial nasal prominences merge not only at the surface but also at a deeper level.
• The structure formed by the two merged prominences is called as the intermaxillary
segment.
• It is composed of :
 A)Labial component –which forms the philtrum of
the upper lip.
 B)Upper jaw component –which carries the four incisor
teeth.
 C)Palatal component-which forms the triangular primary
palate

51. The medial nasal swellings
enlarge, grow medially and
merge with each other in the
midline to form the
intermaxillary segment
Human embryo: 7 weeks

52. Development of palate
(Palatogenesis)
• Begins at the end of the 5th week
• Gets completed by the end of the
12th week
• The most critical period for the
development of palate is from the
end of 6th week to the beginning
of 9th week
The palate develops from
two primordia:
- The Primary palate
-The Secondary palate

53. The Primary Palate
• Begins to develop:
 Early in the 6th week
 From the deep part of the
intermaxillary segment, as
median palatine process
• Lies behind the premaxillary
part of the maxilla
• Fuses with the developing
secondary palate

54. The primary palate represents only a small
part lying anterior to the incisive fossa, of the
adult hard palate
Primary
palate
Hard palate
Secondary palate
Soft palate

55. The Secondary Palate
• Is the primordia of hard
and soft palate posterior
to the incisive fossa
• Begins to develop:
 Early in the 6th week
 From the internal aspect of
the maxillary processes, as
lateral palatine process

56. • In the beginning, the lateral
palatine processes project
inferomedially on each side of
the tongue
• With the development of the
jaws, the tongue moves
inferiorly.
• During 7th & 8th weeks, the
lateral palatine processes
elongate and ascend to a
horizontal position above the
tongue
Tongue

57. • Gradually the lateral
palatine processes:
 Grow medially and fuse
in the median plane
 Also fuse with the:
• Posterior part of the
primary palate
&
• The nasal septum

58. • Fusion with the nasal
septum begins
anteriorly during 9th
week, extends
posteriorly and is
completed by 12th week
Bone develops in the
anterior part to form the
hard palate. The posterior
part develops as muscular
soft palate

60. Changes in Face during Fetal period
• Mainly result from changes in the proportion
& relative positioning of facial structures
• In early fetal period the nose is flat and
mandible underdeveloped. They attain their
characteristic form during fetal period
• The enlargement of brain results in the
formation of a prominent forehead
• Eyes initially appear on each side of
frontonasal prominence ;move medially
• Ears first appear on lower portion of lower jaw,
grow in upper direction to the level of the
eyes

61. Development of ear
• The three parts of the ear-
-External
-Middle and
-Internal ,arise from separate ,diverse
embryonic origins .

62. • The external ear forms around the first branchial groove which deepens to
become the external acuostic meatus .
• Malleus and Incus are derived from the dorsal end of the first branchial
arch cartilage –MECKEL’S CARTILAGE.
• Stapes arise from the dorsal end of the second branchial arch cartilage-
REICHERT’S CARTILAGE .
• This is the first sensory organ to begin development .

63. Development of eye
• The light sensitive portion of the eye retina ,is the outgrowth from the
forebrain ,projecting bilaterally as the optic vesicles which are
connected to the brain by the optic stalks, this results in a thickening
called as lens placodes.
• These placodes invaginates in it’s centre by the development of
peripheral folds .
• The optic vesicles invaginate partly to form the double layered optic
cusps and the optic stalk becomes the optic nerve .
• The outer layer of the optic cup acquires pigmentation to become the
pigmented layer of the retina.

64. Development of lips
• Lower lip :The mandibular processes of the two sides grow towards
each other and fuse in the midline ,they form the lower margin of
the stomatodeum .
• Upper lip :Each maxillary process now grows medially and fuses,
first with the lateral nasal process and the with the medial nasal
process.

65. • The mesodermal basis of the lateral part of the lip is formed from the
maxillary process.
• The overlying skin is derived from the ectoderm covering this process.
• The mesodermal basis of the medial part of the lip(PHILTRUM)is formed from
the frontonasal process .
• The muscles of the face (including those of the lips)are derived from the 2nd
branchial arch and are supplied by the facial nerve.

66. Development of tongue
• The tongue appears in the embryo of approximately 4 weeks in the form
of two lateral lingual swelling and one medial swelling –TUBERCULUM
IMPAR .
• The three swellings orginate from the 1st pharyngeal arch.
• As the 3 swellings increase in size ,they overgrow the tuberculum impar
and merge with each other thus forming the anterior two thirds or the
body of the tongue.

67. • At the junction of the anterior 2/3rd and the posterior 1/3rd is a groove
called as the SULCUS TERMINALIS.
• Sensory innervation-2/3rd - Mandibular branch of
Trigeminal nerve
• Sensory innervation-1/3rd - Glossopharyngeal nerve .
• Motor innervation Hypoglossal nerve
• Special sensory innervation - Chorda tympani branch of Facial nerve

69. •CLINICAL ASPECTS

70. Cleft lip and palate
Classification systems
Numerous classification systems:
• Veau
• American Cleft Palate-Craniofacial Assn (ACPA)
• Kernahan and Stark
Veau’s system:
I Cleft of soft palate only
II Cleft of hard and soft palate to incisive foramen
III Complete unilateral cleft of soft and hard palate and lip
and alveolar ridge on one side
IV Complete bilateral cleft of soft and hard palate and lip
and alveolar ridge on both sides

72. Cleft lip coupled with clefts of the anterior palate or entire
palate.

73. Causes of cleft lip
and palate
• Genetic disorders
• Chromosomal abnormalities
• Environmental factors
(teratogenic agents)
• Mechanical factors

74. Chromosomal abnormalities
• Trisomy 13
– Rare chromosomal abnormality
– The baby is born with 47
chromosomes instead of 46 due
to an extra copy of
chromosome 13
– Multiple congenital
malformations-
• severe brain bnormalities,
• Congenital heart defects,
• Polydactyly,
• Spina bifida,
• Severe eye defects.
– Mean life expectancy is 130
days

75. Environmental factors
• Recognized teratogenic agent:
– Alcohol overdose

76. Mechanical factors
• Intrauterine
crowding :
-tumors,
-multiple births,
- Placenta
abnormalities

77. Incidence of cleft lip and palate
• 1:750 live births
• Clefts of lip is more frequent and is more
severe in males
• Clefts of palate is more frequent in females

78. Facial clefting. A, Absence of the intermaxillary segment with hypotelorism. The maxillary
processes form the normal lateral thirds of the upper lips. The midline rectangular defect indicates the site of the
deficient intermaxillary segment with absent prolabium, incisors, and primary palate. There was consequent clefting
of the secondary palate. Absent intermaxillary segment with hypotelorism signifies a high likelihood of
holoprosencephaly. B, True midline cleft of the upper lip and philtrum with hypertelorism. The nose is normal. A
7-month-old girl with transethmoidal cephalocele and left optic nerve dysplasia (morning glory syndrome). True
midline cleft lip signifies the high likelikhood of midline craniofaciocerebral and optic dysraphysm. C and D,
Midline cleft lip is also found in association with Mohr syndrome (orofacial digital syndrome II [OFD II]). The
presence of reduplicated great toes bilaterally helps to identify OFD II and to distinguish it from OFD I.

79. Facial clefting. A, Right unilateral common cleft lip and palate in a 4-day-old girl. The cleft
extends into the base of a widened nostril. The intermaxillary segment is distorted. B, Bilateral common cleft lip and
cleft palate with discordant forward growth of the intermaxillary segment in a 4-year-old boy. The normal canthi,
alae nasi, and lateral thirds of the lip and jaw indicate normal formation and merging of the maxillary and nasolateral
processes. The abortive prolabium, premaxillary segment, and central incisors attach to the vomer and project well
anterior to their expected position, because failure to merge the facial processes led to discordant growth of the
maxillary and intermaxillary segments. C and D, Bilateral common cleft lip and palate prior to (C) and following
(D) surgical repair. There is near-symmetric restoration of the nose and upper lip, with some residual distortion
caused by scar.

80. Facial clefting. Bilateral oblique oroocular clefts with bilateral common cleft
lip. A, Frontal view. B, Lateral view

81. Facial clefting. Unilateral transverse facial cleft and macrostomia in an infant girl.
(From Bauer BS, Wilkes GH, Kernahan DA. Incorporation of the W-plasty in repair of
macrostomia. Plast Reconstr Surg 1982;70:752–757.)

82. Facial clefting. Nonanatomic clefts in a 12-year-old mentally retarded girl with the syndrome of amnionic bands.
Lateral view. A long, thin band-like scar extends across the scalp and face from the temporoparietal region through
the cheek and the corner of the mouth to the lower lip. The large posterior zone of atrophic skin, absent hair, tissue
bulging, and inferior displacement of the ear indicate the site of an associated temporoparietal encephalocele.
Imaging studies showed notching and separation of teeth where the band crossed the alveolar ridge.

83. Median cleft face syndrome, typical facies. A, Sedano facies type A in 3-month-old boy. B,
Sedano facies type B in 4-day-old boy. C, Sedano facies type C in a young boy after repair of concurrent bilateral
common cleft lip and palate. D, Sedano facies type D in a 31⁄2- year-old boy. (A, B, and D from Naidich TP, Osborn
RE, Bauer B, et al. Median cleft face syndrome: MR and CT data from 11 children. J Comput Assist Tomogr
1988;12:57.)

84. Frontonasal form of frontoethmoidal cephalocele. A, Three-
dimensional CT of the skin surface.The asymmetric glabellar
mass obscures the left orbit and projects over the bony and
cartilaginous nose. B and C, T2-weighted MR images in the
axial (B) and sagittal © planes demonstrate hypertelorism,
anterior herniation of both frontal lobes into the cephalocele
through a defect between the frontal bones and the nasal
bones, and asymmetric distention of the sac by CSF. The
medial frontal anatomy is distorted by the herniation.

85. Typical facies associated with holoprosencephaly. Five types. A, Facies 1: cyclopia. (Courtesy of
Dr. Fred Epstein, New York.) The complete upper lip, with a hint of a labial tubercle in the midline, could represent
either fusion of the nasomedial processes independent of the frontonasal process or fusion of the two maxillary
processes across the midline. B, Facies 2: ethmocephaly. (Courtesy of Dr. Michael Cohen, Halifax, Nova Scotia,
Canada.) C, Facies 3: cebocephaly with synophrys (fusion of the two eyebrows across the midline). D, Facies 4:
absent intermaxillary segment, flat nasal bridge, and rudimentary alae nasi (cf. Fig. 12A). Imaging disclosed alobar
holoprosencephaly with dorsal cyst. E, Facies 5: hypotelorism with an intermaxillary rudiment (white arrowhead ).
Imaging disclosed lobar holoprosencephaly. (From Smith MM, Thompson JE, Naidich TP, Castillo M, Thomas D,
Mukherji SK. Facies to remember. Cebocephaly with single midline proboscis. Alobar prosencephaly. Int J
Neuroradiol 1996;2:251–263.)

86. Microtia and hemifacial microsomia in two patients. A, Microtia. The pinna is deformed. The face appears
normal. B, Hemifacial microsomia. The line formed by the two palpebral fissures and the line formed by the
mouth converge to the region of the deformed, hypoplastic pinna. The right orbit, right eye, and entire right side
of the face are asymmetrically smaller. The skin tag falls along the line between the pinna and the mouth.

87. Hemifacial microsomia. Goldenhar syndrome.
•This 4-month-old girl shows a large coloboma of the medial portion of the left upper lid (between the curved white
arrows) and a whitish choristoma (straight white arrow) that straddles the corneoscleral limbus inferotemporally.
• There is a second, small coloboma of the lower lid medial to the choristoma. The caruncle is unusually prominent.
(Case courtesy of Dr. Myron Tannenbaum, Miami, Florida.) (From Naidich TP, Smith MS, Castillo M, Thompson JE,
Sloan GM, Jayakar P, Mukherji SK. Facies to remember. Number 7. Hemifacial microsomia. Goldenhar syndrome. OAV
complex. Int J Neuroradiol 1996;2:437–449.)

88. •Treacher Collins syndrome in an 8-year-old boy.
• Three-dimensional CT of the skin surface. A to D, Malformed pinnae bilaterally, an
antimongoloid slant of the transverse orbital axis, malar hypoplasia with deficient
lateral orbital walls bilaterally, hypoplastic mandible with prominent antegonial notch,
narrow anterior vault, and overprojection of the central face.

89. •Pierre Robin sequence in a 21⁄2-year-old boy with no catch-up growth of the
mandible. A and B, Lateral 3D CT of the skin surface (A) and facial skeleton (B) show
severe retrognathia and micrognathia. C, Coronalbone CT shows marked buttressing
of the mandibular condyle. D, Axial CT section shows a vertical orientation of the
maxillary incisors but a horizontal course of the mandibular dentition.A

90. • Fetal alcohol syndrome:Alcohol exposure in i.u, life . Most sensitive period of exposure is the first trimester of
pregnancy.Women having 2 – 4 drinks per day are at a risk of having smaller birth size.
• Clinical features:Microcephaly,Short palpebral fissures ,Short nose,Flat philtrum,Thin upper lip.

92. Management of clefts
• Surgical :
It consists of two stages:
a)1st stage-Lip closure within 12 weeks of age.
b)2nd stage-Palate closure within between 9 to 12 months of age.
• Dental:
• Missing teeth ;
• Rotated teeth ;
• Supernumeraray teeth;
• Audiological

93. Speech
• Speech:
– Articulation
• Continuous
Positive
Airway
Pressure
(CPAP)

94. – Voice:
Whistling-Blowing Auditory Visual Tactile Nosepinch/
therapy feedback feedback feedback (cul-de-sac)
technique

95. Conclusion
What matters most is how you see yourself …

96. References
-Color atlas of embryology –Ulrich drews
-Langman’s medical embryology –Sadler
-Netter’s atlas of human embryology –Cochard
-Human embryology –Inderbeer singh
-Craniofacial embryology –Sperber
-Contemprary orthodontics –William profit
-Cleft lip and craniofacial anomalies –Ann kummer
-World wide web