BY DR MAHESH KUMAR FCPS HOD AND CHAIRMAN PLASTIC & RECONSTRUCTIVE SURGERY DEPARTMENT L.U.M.H.S.

JAMSHORO

OBJECTIVES :

      

The development of CLP The etiology of Cleft CLP The normal anatomy of CLP The anatomy of Cleft LP The classification of Cleft LP The key features of the Perioperative care of the child with Cleft lip & Palate The associated complications of Cleft lip & their management

DEVELOPMENT OF FACE :



After 8 weeks Embryo is called ‘Fetus’ due to the fusion of prominences.



Development of face



Appearance of ‘5’ prominences

 

Growth of these prominences Fusion of the prominences

EMBRYOLOGY OF LIP:

EMBROLOGY

Palatal development

to 10 th  7 th weeks 

Palatal shelves

are initially oriented vertically 

Head

straightens , allowing the grows & the

tongue neck

falls away

palatal shelves

to rotate upward into normal horizontal position.

 Growth factors & hyalronic acid 

Frontonasal Forehead, bridge of nose, medial & lateral nasal prominences, nasal septum Maxillary Cheeks, lateral portion of upper lip Medial nasal Philtrum of upper lip, crest & tip of nose Lateral nasal Alae of nose Mandibular Lower lip

THE INCIDENCDE:

       

The isolated cleft palate is 0.5:1000 Cleft lip with or without palate is 1:1000 CL/P predominates in males Isolated CP predominates in females Majority of bilateral CL(86%) & unilateral CL(68%) are associated with a CP Unilateral CL : Left side affected in 60% Unilateral CL are 9 times common than Bilateral Typical distribution of cleft type is:

  

Cleft lip alone: 21% Cleft lip & palate: 46% Isolated cleft palate: 33%

   

ETIOLOGY Idiopathic Genetic defect Environmental Teratogens



Phenytoin , Other Anticonvulsants, steroids



Maternal Smoking , old age marraiges



Alcohol & Retinoic Acid Familial-

  

1 Affected Child Or Parent, Risk Of Child Of The Next Pregnancy Having CLP Is 4% If 2 Previous Children Have CLP Risk Increase To 9% If 1 Parent & 1 Child Were Previously Affected, Risk Is 17



Not associated with solar or moon eclipse



LIP ANATOMY



, White Skin Roll & Vermillion.

, Symmetrical Alar Arches & Equal Alar Base.



MusclLip – Consists Of Symmetrical Cupids Bow & Philtral Colum – Orbicularis Oris .



Nose – Straight Columella & Septum

CLASSIFICATION



CLEFT LIP



UNILATERAL



BILATERAL



MEDDIAN

○

COMPLETE/ SEVERE

○

INCOMPLETE/ MODERATE

○

MICROFORM/ MILD

CLEFT ALVEOLUS

1.

NARROW- NON COLLAPSE 2.

NARROW- COLLAPSE 3.

WIDE- NON COLLAPSE 4.

WIDE- COLLAPSE

NASAL DEFORMITY

           

Mild Lateral Displacement Of The Alar Base, called flaring of ala of nose Normal Alar Contour & Dome Projection Moderate – flaring of Alar Base, Columella Deficiency A Depressed Dome.

Severe Flaring of ala nose deprressed Alar Dome Complete Collapse Of Lower Lateral Cartilage Severe Deficiency Of Columella Height.

Hypoplasia of alar cartilage

MICROFORM CLEFT LIP



Furrow Or Scar .

   

A Vermilion Notch.

Imperfection In White Roll. Varying Degree Of Vertical Lip Shortness.

Nasal Deformity – May Be Present

UNILATERAL INCOMPLETE CLEFT LIP

 

Varying Degree Of Vertical Seperation Of Lip An Intact Nasal Sill/ Simonart Band

UNILATERAL COMPLETE CLEFT LIP

 

Disruption Of The Lip, Nostril Sill & Alveolus(complete Primary Palate) No Simonart Band

INCOMPLETE BILATERAL CLEFT LIP

  

INCOMPLETE WITH A NEAR NORMAL NOSE.

NORMALLY POSITIONED PREMAXILLA.

SIMONART BANDS & CLEFT INVOLVING ONLY LIP.

COMPLETE BILATERAL CLEFT LIP Protuded Premaxilla Poorly Formed Or Absents Anterior Nasal Spine Severe Nasal Deformity, Flat Nasal Tip The Portion Of LIP between Philtral Columns Form Wide, Short Disk Called Prolabium.

classification of Cleft Palate

EMRYOGENIC  Cleft of primary palate Cleft of secondary palate ANATOMIC  Ceftt of uvula  Cleft of soft palate Cleft of hard palate Cleft of alveolus    SIMPLE Anterior and posterior

Classification

Kernehans striped Y Classification Veau Classification 1931  Veau Class I: isolated soft palate cleft Veau Class II: isolated hard and soft palate Veau Class III: unilateral CLAP Veau Class IV: bilateral CLAP    

Symptoms

Separation of the lip  Separation of the palate (roof of the mouth) Nasal distortion Misaligned teeth     Recurring ear infections 

Symptoms (cont.)

Failure to gain weight Nasal regurgitation when bottle feeding Poor speech    Growth retardation 

PROBLEMS (PATHOPHYSIOLOGY)

 

LIP

 

Inability to have a tight seal Malocclusion,



alveolar defect & teeth deformities PALATE

 

Inability to separate nasal from oral Feeding difficulty

  

Regurgitation Middle ear disease Speech problem

PROBLEMS

Upper airway  Speech  Feeding difficulty  Ear infection 

Airway Problems

Cleft Palate patients e.g. Pierre-Robin Sequence Micrognathia , Cleft Palate, Glossoptosis    CYANOSIS  develop airway distress from tongue fall and touch pharanyx lodged in palatal defect  

FEEDING PROBLEM

FEEDING IN HEAD ELEVATED POSITION  FEEDING WITH SPCIAL CP BOTTLES OR D/SYRINGE OR DROPPER  AFTER FEED LAY BABY ON SHOULDER AND SLAB ON BACK TILL RETCHING 

Hearing problem

ETD- Due to abnormal insertion of levator veli palatini and salpingo pharyngeus muscle into hard palate  milk enter into eustachian tube and lead to serous otitis media and infective oscicles  otitis media and finally ankylosis of 30% develop permanent deafness 

Speech Disorders

Errors in Articulation: Fricatives, Affricates Velopharyngeal Competence competence after initial palate surgery Incompetence- nasal emission or snort Evaluation- Direct exam , Fiberoptic Exam    

TIMING OF SURGERY



RULE OF TENS FOR CL:



1O POUNDS

  

10 gm OF Hb 10 WEEKS OF AGE 10,000 TLC

FOR PALATE 10 KG 10 GM Hb 10 MONTH 10000 Tlc     

Treatment

Treatment involves many things which include plastic surgery, orthodontics, and speech therapy 

PRIMARY MANAGEMENT

   

Antenatal Diagnosis

 

Diagnosed By US 3D After 18 Weeks’ Gestation Parents Need Counseling Reassure The Parents Explain Functional Problems Advise On



Feeding



Timing Of Surgery

○

Ideally, The Newborn Infant With A Cleft Is Evaluated By Cleft Team In 1 st Weeks Of Life

PRESURGICAL MANAGEMENT

1:Presurgical infant orthopedics:

 Appliances latham appliance for collapsed alveolar arch

2:Presurgical nasoalveolar molding : objective of NAM :

  To align & approximate the alveolar segment To correct the malposition of the nasal cartilage & alar base on affected side  To idealize the position of philtrum & columella

Naso alveolar mold

Surgical techniques:



For unilateral cleft lip:

Modern accepted technique is the modified Millards rotation & advancement repair 

For microform cleft lip:

Straight line repair Modified Millard rotation – advancement repair 

For bilateral cleft lip:

1,

Manchester repair 2. bilateral millard repair

MANCHESTER REPAIR

Surgical Repair- Cleft Palate

Several Techniques less scarring and less tension on palate   Scarring of palate may cause impaired mid-facial growth(alveolar arch collapse, midface retrusion, malocclusion) Facial growth may be less affected if surgery is delayed until 18 months, but feeding, speech, socialization may suffer.

Surgical techniques

Von langenback operation  Veau, Wardill, Kilner push back palatoplasty  Intravelar veloplasty  Furlow z – plasty  Bordeck palatoplasty 

Complications of lip repair

Unilateral cleft lip:

  Deficient tubercle Vermilion deficiency & irregularity  Short upper lip or Long upper lip  Tight upper lip  Unfavorable scar

Bilateral cleft lip:

 Whistle deformity  Nostril stenosis

complications

Immediate Bleeding  Delayed Fistula formation Failure of repair Speech problem     

Bilateral incomplete cleft lip bilateral millard procedure

bilateral millard repair Bilateral complete cleft lip

unilateral Complete cleft lip

Incomplete cleft lip



POSTOPERATIVE CARE

Soft arm restrain for 2 weeks  Analgesics  Feeding  Suture line care  Stitch removal  Avoid oral suction

POSTOPERATIVE CARE

Fluids for one week Water after every feed Semi solids for next two weeks and water after every diet Solids are allowed after three weeks No need to remove stiches (vicryl)     

Cleft palate

Lower lip cleft (cleft 30)

CONFIDENCE LIKE ART NEVER COMES FROM HAVING ALL THE ANWERS, BUT IT COMES FROM BEING OPEN TO ALL QUESTIONS !