بسم الله الرحمن الرحيم ”وقل رب زدنى علما “. dr: imrana zulfikar...

الرحمالرحماللهاللهبسمبسمالرحيمالرحيمنن

زدنى” رب زدنى” وقل رب وقلعلما “علما “

Dr: IMRANA ZULFIKARDr: IMRANA ZULFIKARASSISSTANT PROFESSORASSISSTANT PROFESSOR

Surgical dept: Surgical dept:

Cleft:Clefting is deformity, in fusion of

mid face skeleton with soft tissue, occurs in second month of pregnancy.

Cleft lip and palate(1:600 live birth).

Cleft palate alone (1:1000 live birth)

• Incidence:• Cleft lip and palate------45%, F > M

• Cleft lip alone-------------15%

• Cleft palate alone-------40% M>F

• Cleft lip and palate is more common on the left side

• Inc: in oriental groups (1:500)

• Dec: in negro population (1:2000)

• ETIOLOGY:Genetic : • Family history: 1st degree affacted have risk

of(1:25 live birth).• Isolated: • Syndrome: over 150 syndroms are associated Pierre robin syndrome, Syickler syndrome Shprintzen, Down, apert, treacher collins.

Environment:• Maternal epilepsy• Drug ( steroid, phenytion, diazepam)

Genetic factorsGenetic factors• Syndrome vs. sequence• Pierre Robin sequence

– Small mandible (micrognathia) causes failure of tongue to descend

– Failure of the tongue to descend causes cleft– Heart problems, conductive hearing loss, developmental deficits– about 40% of infants with Pierre Robin have Stickler Syndrome

and about 15% have Velocardiofacial Syndrome • Treacher Collins syndrome

– Under-developed cheekbones with scalp hair– Malformed ears– Conductive hearing loss– Normal cognitive functioning

Chromosomal abnormalitiesChromosomal abnormalities• Trisomy 13

– Rare chromosomal abnormality

– Multiple congenital malformations

– Mean life expectancy is 130 days

Environmental factorsEnvironmental factors

• Recognized teratogenic agents– Drugs – Aspirin overdose– Acne medications– Alcohol overdose– EPILEPTIC DRUGS– PHENYTOIN

Mechanical factorsMechanical factors

• Intrauterine crowding (tumors, multiple births, placenta abnormalities)

• AMNIOTIC BAND SYDROME

EMBRYOLOGYEMBRYOLOGY

• When the fetus is developing, the face forms by the fusion of five different facial processes, that must fit together perfectly to create a complete face.

• Frontonasal process.• Maxillary process• Mandibular process.• Medial nasal process.• Lateral nasal process. When two of these processes fail to come

together, for whatever reason, a cleft is formed

Physiology of palate:• Separate the nasal cavity from oral cavity.• Soft palate cuts off oropharynx from

nasopharynx during feeding. • Palate generating suction with their

mouths, during feeding the infant. • During speech, closure of soft palate is

essential.• an intact palate with normal muscle

function is necessary for proper drainage of the middle ear.

classificationclassification

• Cleft lip and palate • Cleft lip alone This separation can vary from a subtle notch or groove to a wide gap. • Cleft palate alone Cleft palate occur in midline (isolated) extend to uvula, soft palate, hard palate, incisive foramen

LAHSHAL lahsl

• L CLEFT LIP

• A ALVEOLUS

• H HARD PALATE

• S SOFT PALATE

Cleft lip and palateCleft lip and palateClassification systemsClassification systems

Numerous classification systems:• Veau• American Cleft Palate-Craniofacial Assn (ACPA)• Kernahan and StarkVeau’s system:I Cleft of soft palate onlyII Cleft of hard and soft palate to incisive foramenIII Complete unilateral cleft of soft and hard palate and lip

and alveolar ridge on one sideIV Complete bilateral cleft of soft and hard palate and lip

and alveolar ridge on both sides

• Anatomical defects:

Cleft lip: Nasolabial and bilabial muscle rings

disruption results deformities involving

mucocutanous tissue, muscular and

bony deformity.

Unilateral: Muscle rings disrupt on one side results

asymmetrical deformity.

Bilateral : Bilateral disruption produce symmetrical

deformity.

Complete CL:

A gap goes all the way from the lip, mouth into nose, called Complete cleft.

Incomplete CL:

A gap does not extend from mouth all the way into nose, called an Incomplete cleft.

Microform CL:

Incomplete cleft is very minor (a groove or a notch), called a Microform cleft.

Cleft palate:

• Failure of fusion of two palatine process.it may involve:

• Soft palate• Hard and soft palate.

Hard palate may be:Complete: nasal septum and vomer bone completely

separated from palatine process.

Incomplete: cleft of hard palate remains attached to nasal

septum and vomer.

Antenatal diagnosis:

Only possible for cleft lip by US after 18wk of gestation.

Postnatal diagnosis:• Immediate problems: • feeding problem

• breathing problem

• Associated anamolies.

• Late problems: Recurrent serous otitis media Hearing language delays speech problem (hypernasality, articulation error ) Orthodentic complication (delayed development. Delayed eruption, morphological abnormalities, hypodontia, hyperdontia) Cosmetic disfigurement. psychological problems.

Primary managementPrimary management

Feeding problem manage :Feeding problem manage :

Breathing problems managBreathing problems manag

Primary surgical procedurePrimary surgical procedure

• Feeding problem management:Mother advised:

• Breastfeeding is unlikely to be unsuccessful.

• Bottle feeding with soft plastic bottle (squeezed rhythmically to squirt milk into oral cavity).

• Position of baby should upright, prevent nasal regurgitation.

• Breathing problems management:

• Nursing the in prone position.

• Persistent airway compromise managed by “retained nasopharyngeal intubation”.

• Labioglossopexy in first few days is alternative.

Primary surgical Primary surgical procedure procedure

Goals of surgery:• Normal appearance of lip, nose, face.

• Normal speech

• Dentition and facial growth within normal range of development.

Principals of surgery:• To restore the normal anatomy by

muscular reconstruction.

Timing of primary Timing of primary surgical proceduresurgical procedure

CL repair is performed B/W 3-6 CL repair is performed B/W 3-6 month of age.month of age.

CP repair is performed B/W 6-CP repair is performed B/W 6-18month of age. 18month of age.

• Cleft lip alone: one operation• Unilateral: 5-6 month.• Bilateral: 4-5 month.

• Cleft palate alone:• Soft palate only: 6month• Soft and hard palate: Two operation Soft palate at 6month Hard palate at 15-18 month.

Cleft lip and palate: Unilateral and Bilateral: Two operation - Cleft lip and Soft palate at 5-6 month. - Hard palate and gum pad with or - without lip revision at 15- 18 month

Secondary Secondary managementmanagement

Hearing problems Hearing problems Speech problems Speech problems

Dental development Dental development Facial growthFacial growth

Hearing management:Sensoneuranal hearing loss:

with hearing aid

Conductive loss due to serious otitis media Prophylactic myringotomy grommet insertion. Regular autological testing.

• Speech problem management:• Speech and language therapy

• Secondary palatal surgery

• Intralveolar veloplasty

• Pharyngoplasty

• Speech training devices.

• Orthodontic management: • In two phases

1. mixed dentition (8-10 yrs) to expend maxillary arches by alveolar bone graft.

2. Parmanent dentition (14-18 yrs) to aligndentition by maxillar osteotomy.

• Cleft lip revision surgery:• Indication:• Lip deformity: -Misaligned vermilion -Asymmetrical or muscle discontinuity.• Nasal deformity: -Lateral drift of alar base -Poor nasal tip projection -Deviation of cartilaginous nasal septum.