CLEFT LIP

Dr Gautam Kalra
SR, Department of Plastic & Reconstructive Surgery
CH (SC), Pune
CONTENTS
◦ INTRODUCTION
◦ INCIDENCE
◦ EMBRYOLOGY
◦ AETIOLOGY
◦ PREDISPOSING FACTORE
◦ ASSOCIATED PROBLEMS
◦ SYNDROMES ASSOCIATED
◦ CLASSIFICATION SYSTEM
INTRODUCTION
◦ SECOND MOST COMMON CONGENITAL ANOMALY (1st – Hearing
defects)
(source: WHO data 2000-2016)
◦ Hippocrates(400BC) and Galen (150 AD), mentioned cleft lip.
 INCIDENCE
◦ Worldwide  1 in 600 live births
INDIA
◦ Cleft lip (with or without the cleft palate) 9.92/10,000
◦ Cleft lip alone  3.28/10,000
◦ Cleft lip and palate together  6.64/10,000
◦ Birth prevalence of cleft 27,000 to 33,000 per year
◦ IN India meta-analysis of 25 studies from 1960-1979  incidence of CLP 1.08
and CL 0.23 in 100 live births.

SOURCE: Reddy S et al, Incidence of cleft lip and palate in state of Andhra Pradesh. Indian J Plast Surgery. 2010 Jul-Dec
 Demographics
◦ Male-to-female ratio of 2:1
◦ Males predominant in CLP; Females predominant in Isolated CP
◦ Unilateral clefts are nine times more common than bilateral
◦ The ratio of left (L) to right (R) to bilateral (B) clefts (L:R:B): 6:3:1.
◦ The ratio of CLP to CL is 2:1.
◦ Risk for the next child having deformity based on current family members.
 ◦ Most common cleft lips are-
◦ Unilateral
◦ Males female ratio is 2:1
◦ Left-sided
Males are more affected by cleft lip and females by cleft palate

SOURCE: Reddy S et al, Incidence of cleft lip and palate in state of Andhra Pradesh. Indian J Plast Surgery. 2010 Jul-Dec
 Etiology
◦ It is a multifactorial disorder influenced by both genetic and environmental
factors.
• Genetic
Genetic abnormalities can result in syndromes that include clefts of the
primary or secondary palates.

More than 40% of isolated cleft palates are part of malformation

syndromes, compared with
less than 15% of cleft lip and palate cases.
 Environmental factors

• Maternal smoking or tobacco exposure

• Viral infections: Rubella virus
• Poor nutrition-Deficiency of vit A, riboflavin, folic acid, pantothenic acid,
nicotinic acid
• Certain Medicinal drugs: Anti seizure-Phentoin, Retinoic acid, Steroids
• Teratogens like: Alcohol,Cortisone/ steroids, Mercaptopurine, Methotrexate,
Valium
PREDISPOSING FACTORS
 High maternal age
 Diabetes
 Toxemia (pre-eclampsia)
 Racial – mongoloids
 Radiations

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SYNDROMES ASSOCIATED
WITH CLEFT LIP & PALATE
 SYNDROMES
 Around 400 syndromes

 Non-inherited syndrome
• Pierre Robin Syndrome – Triad of Cleft palate, Glossoptosis (asso. with airway
obstruction), Micro or Retrognathia

•
Pierre Robin Syndrome – Triad of Cleft palate,
Glossoptosis (asso. with airway obstruction), Micro or Retrognathia

•
Hemifacial Microsomia facial deformity involving the structures derived from first and
second pharyngeal arches.
 Inherited syndrome
• Van der Woude (AD) characterized by a cleft lip or cleft palate, distinctive pits of
the lower lips, or both.

• Sticklers (AD)

• Mandibulofacial Dysostosis
-Treacher Collins (AD)
•
- Digeorge syndrome
• Aperts syndrome (AD) Treacher Collins

• Crouzon Syndrome (Craniofacial Dysostosis)

Aperts
 Chromosomal anomalies
•
Trisomy 13 (Patau)
•
Trisomy 18 (Edward)
•
Trisomy 21 (Down’s)
•
Shprintzen Syndrome
 Teratogenic
•
Fetal alcohol syndrome
•
Fetal phenytoin syndrome
•
Fetal valproate syndrome

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EMBRYOLOGY
◦ Development of Facial structures start by the end of 4 th week.

◦ Need to know five facial prominence around the Stomatodeum

◦ Unpaired frontonasal process
◦ Paired maxillary process
◦ Paired mandibular process
Anatomy of the lip (normal)
◦ Muscles of the lip-
◦ orbicularis oris
◦ levator labii superioris
◦ nasalis
◦ Orbicularis oris-
◦ it passes around the oral fissure.
◦ It is in intimate contact with the skin and the mucous membrane.
◦ It consists of anatomically and functionally two parts- superficial and
deep part.

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◦ The superficial portion brings the lips together and its fibers
contract independently to provide fine shades of expression.

◦ The deep layers

◦ encircle the orifice of the mouth and function solely as constrictors.

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◦ The muscles on each side decussate in the midline and form part of
the
◦ bulk of the philtral columns.

◦ The lower portion of this bulk consists of

◦ levator labii superioris,
◦ the upper portion is the
◦ orbicularis and nasalis.

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 Nasalis
◦ These muscle insert into the foot plates of the medial crura.
◦ This muscle on contracting depresses the tip of the nose
through its insertion

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NASALIS
LEVATOR LABII
SUPERIORIS
◦ The labial musculature inserts thickly into the skin at the base of
the columella and on the nostril floor,
◦ attaching this skin to the underlying bone. (philtrocolumellar angle)
◦ Columella is the
◦ fleshy external termination of the nasal septum
◦ supported by the medial crura of the alar cartilages
◦ covered by skin.

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Complete Cleft lip (muscles)

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◦ Unilateral cleft lip-
complete unilateral cleft lip- fibers of the cleft lip
proceeding
◦ horizontally from the commisure towards the midline,
◦ turn upwards along the margin of the cleft.
They terminate
◦ laterally beneath the ala of the nose and
◦ medially beneath the base of the columella,
◦ where most of them attach to the periosteum of the maxilla.

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35
Incomplete Cleft Lip (muscles)
> 2/3rd Lip Height Involved in which only narrow
bridges are formed, the muscle has similar
characteristic.
< 2/3rd Lip Height Involved the muscle fibers reach
over the tip of the cleft and pass from the lateral to the
medial lip segments.
◦ The muscle within the cleft, however is interspread by the
trabeculae of the collagenous tissue.

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 Incomplete Cleft Lip (muscles)
◦ A protrusion of excess muscle may be seen and palpated
◦ on the lateral aspect of the complete cleft as well as incomplete
clefts.
◦ This is caused by contraction or heaping up of the muscle which
was prevented from developing to its normal length.
◦ The musculature on the medial side,
◦ is under developed and
◦ does not extend as far forward to the edge of the cleft, as it does on
the lateral side.

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Incomplete Cleft Lip (muscles)
◦ In the lesser form of the incomplete cleft lip, characterized
by
◦ a small coloboma in the lower part of the lip
◦ a groove on the skin passes upward to the threshold of the
nostril.
◦ The external finding is manifest on the muscle ring,
◦ which is depressed beneath the cutaneous groove.

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Incomplete Cleft Lip (muscles)
◦ Minimal of the clefts of the lip have a deformity that is
more extensive. –
◦ skin overlying this area is devoid not only of hair but also of
sweat glands.
◦ Beneath any lip groove that might be present,
◦ there is a break in the continuity of the lip musculature that can
be confirmed histologically.

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◦ Superior labial artery on the lateral side of the cleft follows the
course of the orbicularis muscle bundles and the edge of the cleft
upward to the nasal ala
◦ where it anastomoses with the lateral nasal or angular artery.
◦ In incomplete clefts,
◦ the artery in the form of thin branch passes through the bridge.
◦ The artery on the medial side behaves in the similar way, but
◦ its diameter is visibly smaller and
◦ its branches are fewer than on the lateral side.

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◦ The lesser degree of arterial development
◦ corresponds to the lesser degree of musculature development on the
medial aspect of the cleft.
◦ Its terminal branches extend into the columella,
◦ where they mainly anastomose with the posterior septal arteries.

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Unilateral cleft- lip and columella

◦ Columellar skin is more developed than the b/l cleft, but

◦ the deviated nasal septum and asymmetric alar cartilages
◦ jeopradize the prospects of normal development of a symmetric
columella and adequate support for the nose.

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Associated cleft lip nasal abnormalities
◦ NASAL TIP U/L- Deviated to the non cleft side
B/L- Broad depressed and flat, asymmetric
◦ INFERIOR EDGE OF THE NASAL SEPTUM AND ANTERIOR NASAL SPINE U/L-
Dislocated from the vomerine groove anteriorly and the nasal spine
B/L- Displaced inferiorly relative to the alar base
◦ COLUMELLA U/L- Vertical height on the cleft side is reduced, slanted obliquely
B/L- Markedly Shortened

◦ ALAR CARTILAGE U/L- Dislocated from the tip, flattened and attenuated
B/L- Deformed with short medial crura, widely separated at the tip, flat elongated lateral crura and
obtuse dome

◦ ALAR BASE U/L-Inferior, posterior, and lateral malposition

B/L- Lateral displacement, sometimes inferior and posterior

◦ NOSTRIL- U/L- Dorsally positioned with a transverse axis and circumference more than the normal nostril
B/L- Horizontal orientation and asymmetric
Soft Tissue And Skeletal Deformities Associated With Cleft

◦ Displacement of Cupid’s bow

◦ with deficient vermilion
◦ Shortened lip on the cleft side
◦ Shortened columella on the cleft side
◦ Aberrant insertion of the orbicularis
in the nasal base
◦ Malformed lower lateral cartilage with
a depressed dome and flattened lateral segment
◦ Lateral and outward rotation of the alar base
Soft Tissue And Skeletal Deformities Associated With Cleft

◦ Webbing of the alar base.

◦ Deficient vestibular lining
◦ Dislocation of the septum out of the vomer
◦ Outwardly rotated premaxilla and
◦ retropositioned lateral maxilla
TYPES OF CLEFT LIP
Microform Cleft Lip
◦ The microform cleft is characterized by
◦ a furrow or scar transgressing the vertical
length of the lip,
◦ a vermilion notch,
◦ imperfections in the white roll, and
◦ varying degrees of vertical lip shortness.
◦ Nasal deformity may be present and
◦ is sometimes more extensive than the associated lip
problem.
Microform Cleft Lip
◦ Surgery is generally indicated
◦ but is approached cautiously to avoid a surgical deformity
worse than the congenital defect.
◦ If there is isolated disruption of the orbicularis oris
sphincter,
◦ it can be repaired through an intraoral approach.
Microform Cleft Lip
 Unilateral Incomplete Cleft Lip
◦ Unilateral incomplete clefts are characterized by
◦ varying degrees of vertical separation of the lip,
◦ but they all have in common an
◦ intact nasal sill, or
◦ Simonart band.
◦ They require the same surgical technique as a complete cleft lip in
order to restore normal nasal and lip anatomy,
Unilateral Incomplete Cleft Lip
 Unilateral complete clefts
◦ are characterized by
◦ disruption of the lip,
◦ nostril sil, and
◦ Unlike the incomplete cleft lip
◦ there is no Simonart band connecting the alar base to the
footplates of the lower lateral cartilages of the nose
◦ consequently, the abnormal attachments of the
orbicularis oris muscles on either side of the cleft
cause
◦ a variable degree of collapse of the lower lateral cartilage
framework and
◦ an associated increased nasal deformity.
 CLASSIFICATION OF CLEFTS
◦ It enables uniform description and diagnosis in order to facilitate
intra and interdisciplinary communication, plan treatment, prognosticate outcome, compare results,
maintain records and promote research

◦ Ideal classification system should have

-Embryo and anatomic basis, be concise, clearly defined and comprehensive, user friendly, easily
reproducible, universally acceptable and enable data entry for analysis and research.
 Classification based on-
◦ Anatomy or Morphology
Davis and Ritchie (1922); Veau (1931)
◦ Embryologic Perspective
Kernahan and Stark (1958); ACPA (1962); International (1969)
◦ Symbols and Diagrams
Kernahan Striped Y (1971)
◦ Coding System
Otto Kriens LAHSHAL (1989); Allori CLAP (2016)
◦ Indian Classification system
C. Balakrishnan (1975) Based on Kernajan and Stark’s classification with addition of coding and abbrivations;
Agarwal 2014 Modified by adding additional abbreviations
 Anatomy or Morphology
◦ John Davis and Harry Ritchie Classification 1922
Based on proposition that alveolar process forms the basis of surgical grouping
◦ 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 III: Alveolar process cleft (any cleft involving the alveolar process)
1. Unilateral (right/left: complete/incomplete)
2. Bilateral (right: complete/incomplete; left: complete/incomplete)
3. Median (complete/incomplete)
◦ Veau Classification 1931
Grp I Cleft of SP only
Grp II Cleft of SP + HP upto incisive foramina
Grp III Complete uni cleft of SP,HP, lip and alveolar ridge
Grp IV Complete bilateral cleft of SP,HP, lip and alveolar ridge
(did not include midline clefts and cases of primary palate)
 Embryologic Perspective
◦ Kernahan And Stark 1958
Incisive foramen the reference point in dividing Palate. Pri and sec
Grp I Cleft of structures anterior to the incisive foramen
Grp II Cleft of structures posterior to the incisive foramen
Grp III Clefts of structures andterior and posterior to the incisive froamen
◦ ACPA American Association for Cleft Palate Rehabilitation 1962
Grp I Cleft of Prepalate- Lip, alveolar process and primary palate
Grp II Cleft of hard palate from incisive foramen and/or soft palate
Grp III Cleft of prepalate and palate
Grp IV Complex facial clefts
 Based on Coding System
◦ Otto Kriens 1989 LAHSHAL
-RIGHT TO LEFT
-L:LIP, A:ALVEOLUS, H:HARD, S:SOFT PALATE
Lower case letters depict incomplete clefts and period indicating no clefts
 Indian System of Classification
◦ 1975 C. Balakrishnan
Based on Kernahan and Stark’s
classification with the addition of coding and abbreviations
◦ Grp 1 Primary clefts anterior to Incisive formen
◦ Grp 1A with Cleft Alveolus
◦ Group 2 (Secondary) behind Incisive foramen
◦ Group 3 Clefts including incisive foramen
◦ 2014 Agarwal, Modified Indian
classification as some deformity
classifications could not be
represented in the original
classsification
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