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CONGENITAL CLEFT LIP AND PALATE

  • DR GARGADI,S.I
  • PLASTIC AND RECONSTRUCTIVE SURGEON (FWACS)

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INTRODUCTION

  • CLEFT LIP AND PALATE ARE THE MOST COMMON CONGENITAL ABNORMALITY OF THE OROFACIAL STRUCTURES.
  • INCIDNCE:

1:2000 LIVE BIRTH IN AFRICA

1:1000 IN CAUCASIANS

1:500 IN ORIENTAL GROUP

1:276 IN INDIAN TRIBES OF MONTANA

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SURFACE LANDMARK OF THE LIP

AA

A.ALAR CARTILAGE PROMINENCE

B.COLUMELLA

C.NASAL SILL

D.ALAR BASE

E.PHILTRAL DIMPLE

F.PHILTRAL COLUMN

G.WHITE ROLL

H.PHILTRAL TUBERCLE

I.CUPID BOW

J.COMMISSURE

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INTRODUCTION

  • CLEFT LIP AND PALATE IS COMMONER IN MALES (MORE COMMON OF THE CLASSES OF CLEFT LIP AND PALATE)

  • ISOLATED CLEFT PALATE IS COMMONER IN FEMALES AND IS OFTEN SYNDROMIC

  • CLEFT LIP ALONE IS COMMONER IN MALES (15%)

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SYNDROMES ASSOCIATED WITH CLEFT LIP AND PALATE

  1. DOWN SYNDROME
  2. APERT SYNDROME
  3. TREACHER COLLINS SYNDROME

OTHERS:

  1. CROUZONS SYNROME
  2. STICKLER’S SYNDROME
  3. VANDER WOUDE’S SYNDROME

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EMBRYOLOGY

  • THE FACE AND THE JAWS ARE FORMED BY FUSION OF THE FIVE FACIAL ELEMENTS: FRONTONASAL, TWO LATERAL MAXILLARY AND TWO MANDIBULAR SEGMENTS BY MESENCHYMAL MIGRATION BETWEEN 6-12WKS OF INTRAUTERINE LIFE.
  • THE UPPER LIP IS FORMED BY FUSION OF THE MEDIAL NASAL PROCESSES AND THE MAXILLARY PROCESS.
  • PALATE IS FORMED BY FUSION OF THE PALATINE SHELVES OF THE MAXILLARY PROCESS.
  • THE TWO MANDIBULAR PROCESSES FORMS THE LOWER JAW.

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EMBRYOLOGY OF CRANIOFACIAL CLEFT

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EMBRYOLOGY OF CRANIOFACIAL CLEFTS

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ANATOMY OF CLEFT LIP

  • CLEFT LIP RESULTS IN DISRUPTION OF MUSCLES OF UPPER LIP AND NASOLABIAL REGION.

  • NASOLABIAL MUSCLE:

  1. TRANSVERSE NASALIS
  2. LEVATOR LABII SUPERIORIS ALAEQUE NASI
  3. LEVATOR LABII SUPERIORIS

. ORAL SPHINCTER:

ORBICULARIS ORIS

. LOWER JAW MUSCLES:

  1. DEPRESSOR ANGULI ORIS
  2. DEPRESSOR LABII INFERIORIS
  3. MENTALIS

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DEFORMITIES IN CLEFT LIP

  1. DEFECT IN THE SKIN AND SOFT TISSUE OF THE UPPER LIP

  • VERTICAL SOFT TISSUE DEFICIENCY ON THE CLEFT SIDE.

3. ABNORMAL ATTACHMENTS OF THE LIP MUSCULATURE INTO THE ALAR BASE AND NASAL SPINE.

4. A CLEFT IN THE ALVEOLUS IS LOCATED AT THE SITE OF ERUPTION OF SUBSEQUENT CANINE TOOTH.

5. A DEFECT IN THE HARD PALATE ANTERIOR TO THE INCISIVE FORAMEN

6. NASAL DEFORMITY :FLATTENED ALAR OR REDUCTION IN HEIGHT OF THE DOME.

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AETIOPATHOGENESIS

THE AETIOLOGICAL FACTORS ARE BOTH ENVIRONMENTAL AND GENETIC:

1.HEREDITRY FACTOR: CLEFT LIP AND PALATE IS FAMILIAL TRANSMITTED IN MALE SEX RECESSIVE GENE.

2.NUTRITIONAL DEFICIENCY:DEFICIENCIES OF VITAMIN A,B AND FOLIC ACID.

3.ENDOCRINE DISORDERS:E.G DIABETES MELLITUS.

4.MATERNAL INFECTION:E.G TOXOPLASMOSIS,RUBELLA,CYTOMEGALOVIRUS,HISTOPLASMOSIS AND SYPHILIS (T.O.R.C.H.S SYNDROME)

5.DRUGS :E.G CORTICOSTEROIDS,ANTICONVULSANTS.

6.ANOXIA:E.G RESULTING FROM THREATENED ABORTION.

7.EXPOSURE TO RADIATION:WHICH IS A POTENT TERATOGENIC AGENT.

8.MATERNAL AGE:CHILDREN DELIVERED BY MOTHERS AT LATE AGE(>35YEARS) TEND TO HAVE CONGENITAL ANORMALIES.

9.COSANGUINEOUS MARRIAGE: TENDS TO PROPERGATE ABNORMAL GENE WHICH LEADS TO CONGENITAL ANOMALIES.

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CLEFT LIP

DEFINITION

A CONGENITAL DEFECT OF THE PRIMARY PALATE

(THE LIP ,THE ALVEOLUS AND THE HARD PALATE ANTERIOR TO THE INCISIVE FORAMEN)

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CLASSIFICATION OF THE CLEFT LIP

ANATOMICAL CLASSIFICATI0N

(a)LATERAL CLEFT LIPS: OCCURS DUE TO FAILURE OF FUSION BETWEEN NASAL PROCCESS AND THE MAXILLARY PROCESS

(I)UNILATERAL OR BILATERAL

(II)COMPLETE (CLEFT EXTENDS TO THE NOSTRILS) OR INCOMPLETE (DOES NOT EXTEND TO THE NOSTRIL)

(III)SIMPLE OR COMPOUND(ASSOCIATED WITH CLEFT OF THE ALVEOLUS)

(IV)COMPLICATED(CLEFT LIP ASSOCIATED WITH CLEFT PALATE) OR UNCOMPLICATED.

(b)CENTRAL CLEFT :IT IS RARE

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LEFT UNILATERAL INCOMPLETE CLEFT LIP

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RIGHT UNILATERAL COMPLETE CLEFT LIP

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COMPLETE LEFT UNILATERAL CLEFT LIP

BEFORE SURGERY

AFTER SURGERY

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BILATERAL CLEFT LIP AND PALATE

BEFORE SURGERY

AFTER SURGERY

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INCOMPLETE BILATERAL CLEFT LIP

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COMPLETE BILATERAL CLEFT LIP

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PREOPERATIVE CRITERIA FOR CLEFT LIP REPAIR

MILLARD’S RULE OF 10

1. THE CHILD SHOULD BE 10 WEEKS OLD OR MORE.

2.THE CHILD SHOULD WEIGH 10 POUNDS OR 4.5KG.

3.THE CHILD’S HAEMATOCRIT SHOULD BE 10GM/%.

REPAIR IS DONE AT THE AGE OF 3 TO 6 MONTHS

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MILLARD’S REPAIR (ROTATION ADVANCEMENT REPAIR)

BEFORE

AFTER

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MILLARD’S REPAIR

BEFORE

AFTER

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CLINICAL PRESENTATION\PROBLEMS ASSOCIATED WITH CLEFT LIP AND PALATE

  1. COSMESIS:CLEFT LIP AND PALATE LOOKS UGLY AND IS THE COMMONEST REASON FOR PRESENTATION.
  2. DEFECTIVE SUCTION:NEGATIVE PRESSURE DURING SUCTIONING OF MILK FROM THE BREAST REQUIRE INTACT ORBICULARIS ORIS AND INTACT PALATE.AIR ESCAPES THROUGH THE DEFECT IN THE NASOPARYNX.
  3. ABNORMAL DENTITION:TEETH PROTRUDE THROUGH THE DEFECT IN THE ALVEOLUS (DENTAL ANARCHY).
  4. DEFECTIVE SPEECH:

(a)PROBLEM PRONOUNCING CONSONANTS(EXPLOSIVE WORDS) E.G ‘G’ ,’D’ ,’M’ ,’N’ INSTEAD OF PRONOUNCING DADDY THEY WILL SAY ‘aaey’.

(b)PROBLEM PRONOUNCING SIBILANTS E.G ‘S’

(c)PROBLEM PRONOUNCING FRICATIVES E.G ‘Z’.

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CLINICAL PRESENTATION/PROBLEMS ASSOCIATED WITH CLEFT LIP AND PALATE

5.DEFECTIVE SMELL: CONSTANT IRRITATION OF NASAL MUCOSA BY FOOD FROM THE MOUTH IMPAIRES SMELL.

6.CHEST INFECTION:CHEST INFECTION MAY OCCUR DUE TO PASSAGE OF FOOD INTO THE AIR WAY DURING SWALLOWING (ASPIRATION).

7.CONJUNCTIVITIS:DUE TO CONTAMINATION OF THE CONJUNCTIVA WITH BUCCAL FLORA VIA THE LACRIMAL DUCT WHICH DRAINS INTO THE NASAL CAVITY BELOW THE INFERIOR TURBINATE.

8.EAR INFECTION:DUE TO REGURGITATION OF BUCCAL CONTENT VIA THE EUSTACIAN TUBE IN THE NASOPHARYNX.

9.PROBLEMS AT CHILD BIRTH:BABIES WITH ISOLATED CLEFT LIP HAVE LESS PROBLEM WITH SUCKING BREAST WHILE BABIES WITH CLEFT PALATE WILL REQUIRE SOME ASSISTANCE WITH SUCTIONING AND RESPIRATION.

BABIES WITH PIERRE’S ROBIN SEQUENCE WILL EXPERIENCE BREATHING PROBLEM REQUIRING ASSISTED VENTILLATION.

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ANATOMY OF THE PALATE

  • THE SECONDARY PALATE LIES POSTERIOR TO THE INCISIVE FORAMEN.
  • HARD PALATE : IS COMPOST OF-:
  • PALATAL PROCESSES OF THE MAXILLA ANTERIORLY.
  • THE PALATINE BONES POSTERIORLY

. SOFT PALATE: IS MADE OF-:

  1. BUCAL MUCOSA
  2. NASAL MUCOSA
  3. MUSCLES : TENSOR VELI PALATINI,LEVATOR VELI PALATINI, MUSCULARIS UVULAE

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PREOPERATIVE CRITERIA FOR REPAIR OF CLEFT PALATE

REPAIR IS DONE AT THE AGE OF

6-18 MONTHS

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SURGICAL REPAIR OF CLEFT PALATE

VON LANGENBECK REPAIR

A BIPEDICLE MUCOPERIOSTEAL FLAPS RAISED BY LONGITUDINAL INCISION ALONG INNER ASPECT OF EACH ALVEOLAR ARCH,BASED ON GREATER PALATINE VESSELS SUPERIOLY.

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VON LANGENBECK

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PRIVATE PLASTIC SURGERY PRACTICE

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PRIVATE PLASTIC SURGERY PRACTICE

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PLASTIC SURGERY PRACTICE

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PLASTIC SURGERY PRIVATE PRACTICE

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NATURE IS A CHARM

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MY CLEFT (SPACE) OF LEISURE( AT RAPH LAUREN SHOP : BAL HARBOUR SHOPS FLORIDA U.S.A)

FIND PEACE IN WHAT YOU DO

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WHO WE ARE DETERMINES HOW WE SEE OTHERS