Congenital abnormal space or gap in the upper lip, alveolus and palate

More common in south far Asians: 1 in 500

Less frequent in Africans: 1 in 2000

Prevalence in Europeans and Americans: 1 in 750

Prevalence in Pakistan1: 1 in 523

1. Elahi MM, Jackson IT, Elahi O, Khan AH, Mubarak F, Tariq GB, Mitra A. Epidemiology of cleft lip and cleft palate in Pakistan. Otolaryngol Clin North Am. 2007 Feb; 40(1):27-60.

Male to female ratio – 3 : 2

Cleft Lip and Palate are twice as common in

boys as in girls

Isolated Clefts of Palate are more common in

girls

75% of Clefts are Unilateral, rest are Bilateral

Left side is more frequently involved

The medial nasal swellings enlarge, grow medially and merge with each other in the midline to form the intermaxillary segment

Human embryo: 7 weeks

Kernahan “Striped Y”

Millard Modification of Kernahan “Striped Y”

Kriens “LAHSHAL”

Kernahan “Striped Y”

Lip

Alveolus

Primary Palate

Hard Palate

Hard Palate

Soft Palate

Millard Modification of Kernahan “Striped Y”

Kriens “LAHSHAL” Capital letter = complete cleft Lowercase letter = incomplete cleft “.” or “-” = normal

Example: LA- - - - l = complete right cleft lip and alveolus,

incomplete left cleft lip

Microform Cleft: May look like

a little dent in the red part of lip a scar from the lip up to nostril

Muscle tissue underneath cleft can be affected and may require surgery

Submucous Cleft Palate: Midline deficiency or lack of muscular tissue Often a submucous cleft palate is associated

with a bifid or cleft uvulaPosterior nasal spine is almost always missingSpeech problems are common

Transabdominal Ultrasonography :Reliable after 4th monthClefts of lipClefts of palate:

Sagittal View Axial View Coronal View

Transvaginal UltrasonographyThree-dimensional ultrasonography

Advantages of Prenatal Diagnosis:

1. Time for parental education2. Time for parental psychological

preparation3. Preparation for neonatal care

and feeding4. Opportunity to investigate other

associated anomalies5. Gives parents the choice of

continuing the pregnancy6. Opportunity for fetal surgery

8-9 Year: Initial interventional

Orthodontics Preparation for

alveolar bone grafting10 Year:

Alveolar Bone Grafts12-14 Year:

Definite Orthodontics16 Year:

Nasal Revision Surgery17-20 Year:

Orthognathic Surgery

Birth: Initial Assessment Pre-surgical assessment

3 Month: Primary Lip repair

9-18 month: Palate Repair

2 Year: Speech assessment

3-5 Year: Lip Revision Surgery

Genetic Scientist Pediatrician Pedodontist Orthodontist Oral and Maxillofacial Surgeon Prosthodontist ENT Surgeon Plastic Surgeon Psychiatrist Speech Therapist Social Worker

Feeding

Dental problems

Nasal Deformity and Esthetic Problems

Ear Problems

Speech Difficulties

Cleft lip Makes it more difficult for an infant to suck

Cleft Palate May cause milk to be accidently taken up into

nasal cavity Inability to create negative pressure inside oral

cavity Frequent regurgitations Upper respiratory tract infections

In Patient with Cleft lip Special nipples to allow baby to latch properly

Mead Johnson/Enfamil Cleft Feeder

Pigeon Feeder Dr. Brown’s Natural Flow

Special Needs Feeder / Haberman Feeder

In patients with cleft palate

Avoid feeding without palatal obturator

Feeding in an upright position

Local Dental Problems: Congenitally Missing teeth (Hypodontia, Oligodontia) Hyperdontia Presence of natal and neonatal teeth Abnormal tooth morphology (microdontia, macrodontia ) Fused teeth Enamel Hypoplasia Gemination, Dilacerations Poor periodontal support

Orthodontics Problems: Class III tendency Anterior and Posterior Cross bite Spacing and crowding

Facial Disfigurements

Poor nasal shape

Scar marks of surgeries

Poor lip function during

speech

Poor dental alignment and

smile

Middle ear disease - 22% to 88% Conductive hearing loss and chronic

suppurative otitis mediaRepeated tympanostomy tube placement

Abnormal curvature of eustachian tube lumen

Altered width and angulation of skull base

Abnormal insertions of the tensor and levator veli

palatini muscles into cartilages and skull base

96% of children require tympanostomy tube placement1

50% of these children required repeat tympanostomy tube placement 1

Frequency of otitis media decreases as the child with CP ages

Audiology and tympanometry as well as exams / clinical history

1Muntz HR, An overview of middle ear disease in cleft palate children, Facial Plast Surg. 9 (1993) 177-180.

Hearing loss hampers proper development of speech

Velopharyngeal Insufficiency (VPI)Poor pronunciation of Bilabial, Labiodental,

Linguoalveolar sounds

Maxillary StrappingNasoalveolar Moulding Appliances (NAM)

Advantages:1. Reduces the size of cleft; aids in surgery2. Partial obturation aids in feeding3. Parental reassurance at a crucial time

Indications of NAMs:1. Large lip defect requiring presurgical

approximatiom2. Severe deviations in nasal cartilages columella,

nasal tip, and lateral wall3. Post surgical nasal molding and tension

reduction across suture line

Premaxilla is extremely protrusive

Premaxilla and prolabium can be of variable size

Columella is deficient/almost nonexistent

Palatal shelves are collapsed

Bilateral Cleft Lip

Bilateral Cleft Lip Repositioning of protrusive

maxilla Support from intraoral

component Retention with

Denture adhesive Elastic strap

• Time of surgery: approximately at10 weeks “Rule of Ten”

Child weighs 10 pounds Child has a hemoglobin of at least 10 grams/dl Child has a WBC count < 10 thousand Child is at least 10 weeks of age

Cleft Lip Repair Unilateral

Millards’s rotation-advancement flap

Cleft Lip Repair Bilateral

bilateral rotation advancement with attachment to premaxilla mucosa

Dorf and Curtin1

10% occurrence of articulation errors when palatoplasty was completed by 1 year

86% incidence of articulation errors when repair was complete after 1 year

Haapanen and Rantala2

Significantly fewer children in the groups repaired before 18 months had hypernasal speech, articulation errors, or required secondary surgery to correct speech

1. Dorf DS, JW Curtin: Early cleft palate repair and speech outcome: A ten year experience. J Bardach HL Morris Multidisciplinary Management of Cleft Lip and Palate. 1990 WB Saunders Philadelphia 341-348.

2. Haapanen ML, Rantala SL. Correlation between the age at repair and speech outcome in patients with isolated cleft palate. Scand J Plast Reconstr Surg Hand Surg. 1992;26(1):71-8.

1. Schweckendick’s Primary Veloplasty

2. Von Langenbeck Palatal Repair

3. V-Y Pushback

4. Furlow Palatoplasty

1. Schweckendick’s Primary Veloplasty Incisions made in soft palate Muscle bundles released from posterior hard

palate and rotated Reconstruction of levator sling Closure of mucosal layers separately

2. Von Langenbeck Operation

V-Y Pushback (WARDILL OPERATION) Two uni-pedicled flaps (greater palatine artery)

raised Posterior flaps rotated in a V-Y advancement Improved speech results Indicated for incomplete clefts

V-Y Pushback (WARDILL OPERATION)

Furlow Palatoplasty Lengthens soft palate Reconstructs the muscle sling Used to correct velopharyngeal insufficiency Can be used for treatment of submucous clefts

Aim: Prepare the dentition adjacent to cleft for the secondary alveolar bone graft

Avarge Duration: 6-12 months Appliances:

1. Bonded edgewise appliance2. Supported with a maxillary expander quad

helix expander

Use of Quadhelix to expand maxillary arch

Use of Spider appliance to expand maxillary arch

Use of bonded edgewise appliance

Primary Bone Grafting

1. Bone graft done at the time of primary cheiloplasty

2. Bone graft done during the first 2 years of life

3. Bone graft done prior to the eruption of the primary canine

Secondary Bone GraftingDone before eruption of the permanent canine

In CLP dental age lags behind chronological age

Early bone graft: 2-5 years of age Intermediate bone graff: 6-15 years of age Late bone graph: 16 year and older

Advantages:1. Provides bone for eruption and orthodontic

repositioning of teeth2. Closure of oro-nasal fistulas3. Support and elevation of alar base4. Stabilization of pre-maxilla in bilateral cases5. Establishing continuity of alveolar ridge

Autogenous Cancellous- iliac crest Cortical- calvarium, mandible Cortico-cancellous- iliac, rib, tibia, mandible

Allogeneic1

Graft resorbs, remodels, may contribute to osteoinduction and osteoconduction

Alloplast2

Bone grows into, around alloplast No active osteoinduction but some osteoconduction Teeth do not erupt through alloplast

1. Nique T, Fonseca RJ, et al: Particulate allogeneic bone grafts into maxillary alveolar clefts in humans- A preliminary report. J Oral Maxillofac Surg 45: 386-392, 1987.2. Horswell BB, El Deeb M: Nonporous HA in the repair of alveolar cleft defect in a primate model. J Oral Maxiilofac Surg 47:946-952, 1989.

Preoperative Post Operative

Preoperative Cleft Defect

Postoperative Bone Graft

• Correction of anterior crossbite

• Arch expansion• Stability of results is

questionable• Slower results as

compared to normal subjects

Face mask therapy:

Late mandibular growth may aggravate problems

Hypernasality may increaseDefinitive dental prosthesis may be plannedTreatment planning

Mild skeletal discrepancy: Dental compensations Moderate skeletal discrepancy: Orthognathic surgery Severe skeletal discrepancy: Distraction osteogenesis

Lefort I maxillary advancement Relapse after rigid fixation1

Mean maxillary horizontal relapse of 20.5% Mean vertical relapse of 22.2% within first year

Velopharyngeal insufficiency

1. Heliövaara A, Ranta R, Hukki J, Rintala A. Skeletal stability of Le fort I osteotomy in patients with unilateral cleft lip and palate. Scand J Plast Reconstr Surg Hand Surg 2001;35:43-49.

Maxillary advancement with distraction Osteogenesis

Midfacial Advancement Pretreatment Decompensation

Maxillary advancement with intraoral distractor

Pretreatment Postreatment

Maxillary advancement with Extraoral distractor

Patients with a cleft lip and palate require Patients with a cleft lip and palate require highest standard of highest standard of multidisciplinary care multidisciplinary care

Management starts pre-natally after initial Management starts pre-natally after initial diagnosis, followed by a long treatment aiming diagnosis, followed by a long treatment aiming at restoration of oral health and function while at restoration of oral health and function while achieving optimum estheticsachieving optimum esthetics

Right intervention at right time Right intervention at right time can significantly can significantly improve the quality of lifeimprove the quality of life

Timing: 17-20 years of age

Standard techniques Tip projection Alar rotation Columellar lengthening

Goals of Surgery

1. Reducing the size of opening between oral and nasal cavities

2. Reconnecting palatal muscles to restore function

3. Restore the anatomical length of soft palate

Dorrance and Brown’s – U shaped push back palatoplasty

Use of Pharyngeal flap

Posterior pharyngeal wall augmentation with implants and injections