Embryology & Surgical Anatomy of Cleft Lip.pptx

1. Embryology & Surgical
Anatomy of Cleft Lip
By
Ahmed El-Sayed Sharaf
Demonstrator of Plastic Surgery

2. Contents
• Definition
• Embryology
• Anatomy (Normal & Abnormal)
• Associated Nasal Deformities
• Epidemiology
• Pathogenesis

3. Definition
• cleft is an abnormal opening or a fissure in an
anatomical structure that is normally closed.
• Cheiloschisis/ Cleft Lip A cleft of the lip is a congenital malformation
that occurs in utero during the first trimester of pregnancy.

4. Embryology
• Development of the face begins by the end of the fourth week of
gestation, concurrent with the limbs and the heart.
• The face is assembled from fusion of the five facial prominences.

5. First Pharyngeal Arch
• Cartilaginous components
1- Maxillary process: the premaxilla,
maxilla, zygoma and part of the
temporal bone.
2- Mandibular process: Meckel’s
cartilage >> the incus and malleus,
anterior ligament of malleus,
sphenomandibular ligament and the
mandible
• Muscles: muscles of mastication, tensor
palati, tensor tympani, anterior belly of
digastric & mylohyoid.
• Artery: maxillary artery, external
carotid
• nerve: is the trigeminal n.

6. Frontonasal Prominence:
Lateral Nasal Prominence:
Maxillary Prominences:
Philtrum
Mid-Upper lip
Primary palate
Forehead
Midline of the nose
Nasal septum
Alae of the nose
lateral upper lip
secondary palate.
upper cheek
the maxilla
secondary palate
Facial Prominences

7. Anatomy of The Lip
Layers:
• Skin
• Subcutaneous fat
• Muscle
• Mucosa
External landmarks
• Commissure
• Cupid’s bow
• Philtral columns
• Tubercle
• White roll
• Wet-dry vermilion line

8. Periorbital Muscles

9. Orbicularis Oris
• Origin and Insertion: This muscle encircles the mouth
between the skin and labial mucosa of the lips, with no bony
attachment. It then inserts in the skin of the lips at the labial
commissures. In the upper lip, fibers also insert on the ridges
of the philtrum.
• Action: This muscle has four relatively distinct movements:
pressing together (closing lips), tightening and thinning
(pursing lips), rolling inward between the teeth (grimacing),
and thrusting outward (pouting and kissing).
• Nerve Supply: buccal and mandibular branches of the facial
nerve.
• Divided into Pars Superficialis & Pars Marginalis

10. Arterial Supply
Inferior Labial a.
Superior Labial a.
The facial artery passes forward in the cheek, lying
superficial to the buccinator muscle and deep to the
zygomaticus major muscle. It branches to:
• Superior Labial a. : runs between the labial mucosa
and orbicularis oris muscle at the level of the upper
margin of the red lip. It runs medially, giving
ascending branches at both sides of the labial mucosa
and skin, and then it anastomoses with the contralateral
artery.
• Inferior Labial a.: runs between the orbicularis oris
and buccinator muscles and then reaches the lower lip.
It passes transversely between the orbicularis oris
muscle and labial mucosa at the level of the boundary
of the red and white parts of the lips .

11. Sensory Nerve Supply
• Infraorbital N. (Maxillary Dev./V2 of Trigeminal):
The infraorbital nerve is the direct continuation of the
maxillary nerve and passes to the cheek through
Infraorbital foramen 1 cm below the infraorbital rim
lying along in the same vertical plane with the SON
and the mental nerves.
• Mental N. (Mandibular Dev./V3 of Trigeminal):
It is the continuation of the inferior alveolar nerve,
exiting the mandible through the mental foramen.

12. Lymphatic Drainage
• Upper lip:
-Primarily drains to ipsilateral submandibular nodes
-Occasionally drains to ipsilateral preauricular,
infraauricular parotid, or submental nodes
• Lower lip:
-Lateral thirds drain to ipsilateral submandibular nodes.
-Medial third drains to submental and submandibular
nodes.

13. Anatomy of Cleft Lip

14. Complete Unilateral Cleft Lip
• Discontinuity in the skin and soft tissue of the
upper lip.
• Vertical and transverse soft tissue deficiency on
the cleft side.
• Abnormal attachment of lip muscles into the alar
base and nasal spine.
• Outwardly rotated and prominent premaxilla.
• Retro-positioned and hypoplastic lateral maxillary
element.
• Cleft in the alveolus, usually found at the site of
future canine tooth eruption.
• Nasal deformity.

15. • The nostril sill will therefore be intact.
• Nasal sill contains some soft-tissue elements
“Simonart’s band”
• Microform Cleft:
∘ A notch in the vermilion
∘ A vertical fibrous band from the wet–dry
vermilion border (the red line) to the nostril
floor
∘ A kink in the nasal ala on the same side.
Incomplete Unilateral Cleft Lip

16. • Orbicularis Oris abnormally inserts onto
the ala bilaterally
• Prolabium has no Cupid’s bow, no
philtrum or Philtral columns, and no
orbicularis. And is often protruded
forward.
• Severe deficiency of columellar height
• Wide alar bases
Bilateral Cleft Lip

17. • Deviation of the nasal spine, columella and caudal septum away
from the cleft side.
• Dislocation of the inferior edge of the septum out of the vomer
groove.
• Separation of the domes of the alar cartilages at the nasal tip.
• Dislocation of the upper lateral nasal cartilage from the lower
lateral cartilage on the cleft side.
• Sagging of the lateral crus of the lower lateral cartilage on the
cleft side.
• Alar base displaced laterally, posteriorly, and sometimes inferiorly
• Deficient vestibular lining on the cleft side.
• Flattening and displacement of the nasal bone on the cleft side.
• columella is shorter & deviated to the noncleft side
• The lower turbinate on the cleft side is hypertrophic.
unilateral CL nasal deformity

18. • Bilateral displacement and distortion
of osseocartilaginous skeleton of
nose.
• Shortened columella
Bilateral CL nasal deformity

19. Epidemiology
• prevalence of CL/P 0.2 to 2.3 per 1000 births.
• Sex ratio (male/female) for CL/P is 2:1.
• Combined CL&P is most common, seen in 50% of cases.
∘ Isolated CP occurs in 30% of cases.
∘ Isolated CL occurs in 20% of cases.
• The ratio of left: right: bilateral CL is 6:3:1.

20. Pathogenesis
• Clefts can occur due to disruptions or delays in cell
migration.
• CL/P results from failure of fusion of the medial nasal and
maxillary processes
• There are several basic causes of clefts These include
chromosomal disorders, genetic disorders, environmental
teratogens or by mechanical factors in utero.

21. Family History
• The risk of CL/P in the 1st degree relatives of individuals with is approximately
3%

22. Maternal Medications
• Retinoids: used for ttt of acne and sever psoriasis. Associated with 26
fold increase in possibility of having a child with cong. Malformation.
• Anticonvulsants: Orofacial clefts have been reported in the offspring of
women taking phenytoin, phenobarbital, valproic acid, topiramate,
lamotrigine, or carbamazepine alone or in combination.
• Folate Antagonists: drugs with antifolate properties, including
dihydrofolate reductase inhibitors (trimethoprim, triamterene, and
sulfasalazine) & anticonvulsants, have also been associated with an
increased risk of congenital malformations, including orofacial clefts

23. • Benzodiazepines: used to treat anxiety, insomnia, and epilepsy, have
been associated with an increased risk of malformations.
• Corticosteroids: maternal systemic corticosteroid use associated with an
increased risk of orofacial clefts in off springs.
• Other Medications: beta-blockers, NSAIDs, some antibiotics (such as
nitrofurantoins) and bronchodilators have been suggested to increase
risk of malformations but with little evidence.
Maternal Medications

24. • Diabetes: Women with non-gestational diabetes are at increased
risk of having a child with a range of congenital malformations
(known as diabetic embryopathy), including orofacial clefts.
• Hyperthermia: may increase the risk for a range of birth defects.
• Smoking: the risks of CL/P and CP are increased in the off spring of
women who smoke.
• Alcohol Consumption: The off spring of women who consume
excessive amounts of alcohol during pregnancy are at increased
risk for a range of malformations (fetal alcohol syndrome).
Maternal Diseases & Behavior

25. • Obesity: the risk of orofacial clefts is increased by at least 1.3-
fold in maternal obesity
• Stress: The occurrence of at least one stressful maternal life
event is associated with the risk of orofacial clefts in off spring.
• Folic Acid Deficiency: maternal peri-conceptional folic acid
supplementation can reduce the risk of orofacial clefting.
• Vitamin A Overdosing: Maternal intake of very high doses of
vitamin A from supplements (25,000 IU or more per day) has
been associated with a pattern of malformations, including
orofacial clefts.
Maternal Diseases & Behavior

26. • Contaminants in Drinking Water: disinfection by-products, mothers exposed to
chlorate were four times more likely to have a child with CL/P.
• Hazardous Waste Sites: Residential proximity to hazardous waste sites has been
associated with orofacial cleftings.
• Pesticides: maternal occupational exposure to pesticides is associated with
orofacial clefts .
• Occupation: Occupational exposures to toxicants other than pesticides have also
been evaluated as potential risk factors for orofacial clefts.
Environmental Exposures

27. Van der Woude Syndrome
 CHARGE Syndrome
FAV/OAV Spectrum
Down Syndrome
22q11.2 deletion syndrome
Deletion 4p Syndrome
Fetal Alcohol Syndrome
Syndromic CL/P
• Autosomal
dominant
• Mutated
IRF6 gene
• Lower lip pits
• Hypodontia
• Autosomal
dominant
• Mutated CDH7
gene
• Coloboma,
• Heart defect,
• Atresia choanae
• Retarded
growth
• Genital
anomalies
• Ear anomalies
-Sporadic
-Microtia/Ear tags
-Hypoplasia of the
malar, maxillary,
and mandibular
regions
-Macrosomia
-Vertebral
anomalies
-Trisomy 21
-intellectual
disability
-upper airway
obstruction
-Autosomal
dominant
-More isolated CP
-Short palpebral
fissures
-Hooded eyes
-Prominent nose
-Square nasal root
-Hypoplastic alae
nasi
-Cardiac defects
-Long tapered
fingers
-Posterior midline
scalp defects
-Ocular
hypertelorism
-Iris anomalies
-Arched eyebrows
-Short upper lip &
philtrum
-Preauricular tags or
pits
-Hypospadias
-Cardiac defects.
-profound prenatal
and postnatal
growth deficiency
-microcephaly
-Short palpebral
fissures
-Smooth philtrum
-Thin vermilion
-Joint contractures
-Cardiac defects.

28. Trisomy 13 Syndrome
Trisomy 18 Syndrome
EEC syndrome
Opitz Syndrome
Waardenburg Syndrome
Gorlin Syndrome
Syndromic CL/P
-Holoprosencephaly
–Microphthalmia
-Scalp defects
-Cardiac defects
-Polydactyly
-bulbous nose
-Prenatal growth
deficiency
-Prominent occiput
-Hypoplastic
supraorbital ridges
-Small nose
-Small mouth
Short sternum
-Camptodactyly
-Nail hypoplasia
-Short dorsiflexed
hallux
-Autosomal
dominant
-Split hand or foot
-Light, thin scalp
hair
-Hyperkeratosis
-Partial Anodontia
-Malformed teeth
-Lacrimal defects
-Prominent forehead
-Ocular
hypertelorism
-Broad nasal bridge
-Anteverted nares
-Posteriorly rotated
ears
-Hypospadias
-Cryptorchidism
-Bifid scrotum
-Laryngotracheal
malformations
-Autosomal
dominant
-Congenital deafness
-Partial albinism
-Hypopigmentation
of the iris, retina,
skin, and hair
-Broad, high nasal
bridge
-Rounded nasal tip
-Hypoplastic alae
Nasi
-Medial flare of
bushy eyebrows
-Autosomal
dominant
-Macrocephaly
-Frontal bossing
-Ocular
hypertelorism
-Prominent
supraorbital ridges
-Heavy eyebrows
-Short metacarpals
-Scoliosis
-Nevoid BCC

29. Classification

30. Davis and Ritchie’s classification (1922)
Group I: Prealveolar process cleft (clefts affecting the lip)
• 1. Unilateral (right/left: complete/incomplete)
• 2. Bilateral (right: complete/incomplete; left:
complete/incomplete)
• 3. Median (complete/incomplete)
Group II: Postalveolar process cleft (clefts affecting the
palate)
• 1. Soft palate
• 2. Hard palate
Group 3: All complete clefts of the lip and palate (unilateral
or bilateral)

31. Cleft lip
Class I : U/L notching of vermillion border, not extending into
the lip.
Class II : cleft extending into the lip, but not including the floor
of the nose.
Class III: extending into the floor of the nose.
Class IV: any b/l cleft of the lip, whether incomplete or
complete.
Victor Veau’s classification (1931)

32. Modified Kernahan and Stark’s stripped-Y

33. Thank You