In 4-week old 5 mesenchymalprominence can be recognized :1. Unpaired FrontonasalProminence2. Medial and Lateral Nasalprominences3. 2 maxillary prominences4. 2 mandibular prominencesEMBRYOLOGY
EMBRYOLOGYDuring the fifth week, two fast growing ridges, thelateral and medial nasal swellings, surround the nasalvestige.The lateral swellings will form the alae of the nose, themedial swellings will give rise to four areas :(1) the middle portion of the nose(2) the middle portion of the upper lip(3) the middle portion of the maxilla(4) the entire primary palateSimultaneously the maxillary swellings will approach themedial and lateral nasal swellings but remainseparated from them by well marked grooves.
During the next 2 weeks, the appearance of theface changes considerably.The maxillary swellings continue to grow in a medialdirection and compress the medial nasal swellingstoward the midline.Subsequently these swellings simultaneously mergewith each other and with the maxillary swellingslaterally.Hence the upper lip is formed by the two medialnasal swellings and the two maxillary swellings.The two medial swellings merge not only at thesurface but also at the deeper level.
The structures formed by the two merged swellings areknown together as the intermaxillary segament whichis comprised ofthree components(1) a labial component, which forms the philtrum ofthe upper lip(2) an upper jaw component, which carries the fourincisor teeth(3) a palatal component, which forms the triangularprimary palate.
Two shelflike outgrowths from the maxillaryswellings form the secondary palate.These palatine shelves appear in the sixth week ofdevelopment and are directed obliquelydownward on either side of the tongue.In the seventh week, however, the palatineshelves ascend to attain a horizontal positionabove the tongue and fuse with each other,thereby forming the secondary palate.Anteriorly the shelves fuse with the triangularprimary palate, and the incisive foramen isformed at this junction.
Cleft lip : is a birth defect that results in aunilateral or bilateral opening in the upper lipbetween the mouth and nose (interruption ofdevelopment before 7th week lead to lipdeformity).Cleft palate : is a birth defectcharacterized by an opening in the roof of themouth caused by a lack of tissuedevelopment( interruption of development.before 12th week lead to palate deformity
Development Of cleft :1.Cleft Lip :Various theories have been given for itsdevelopment1.Failure of fusion between median nasal process andmaxillary process2.Failure of mesodermal migration between the twolayeredepithelial membrane .This leads tobreakdown and cleft formation.3.Rupture of cyst formed at the site of fusion.
2.Cleft palateVarious theories have been given1.Alteration in intrinsic palatal shelf force2.Failure of tongue to dropdown3.Non fusion of shelves4.Failure of mesodermal migration5.Rupture of cyst formed at the siteof fusion
Etiology1.GENETIC FACTOR AND ASSOCIATED WITH SOME SYNDROMELIKE PIERRE ROBIN SYNDROME.VIT. A,B DEFICIENCY) 2.NUTRITIONAL DISTURBANCES DURINGDEVELOPMENT3.PHYSIOLOGIC ,EMOTIONAL OR TRAUMATIC STRESSESDURING DEVELOPMENT4. DEFECTIVE VASCULAR SUPPLY TO AREA INVOLVED5.MECHANICAL DISTURBANCES WHERE THE SIZE OF THETONGUE MAY PREVENT UNION PARTS6.VARIOUS ENVIRONMENTAL FACTOR LIKE :INFECTION (E.G. RUBELLA)EXPOSURE TO RADIATIONDRUGS (E.G. THALIDOMIDE , ANTIEPILEPTIC DRUGS , HORMONAL PILLS)6. MATERNAL CONSUMPTION OF ALCOHOL AND SMOKING
General Classification1- Veau classification2- Kernahan and Stark classification3- Kernahan classification4- Harkins classification5- Spina classification6- Tessiers classification
-Tessier described a classification scheme that isuniversally utilised,in a landmark article of 1976.Oro-facial clefts can manifest as: Unilateral or bilateral Complete, incomplete, or microform (e.g., sub-mucous cleft palate) Clefting of the lip with or without the palate, or of thepalate in isolation Atypical cranio-facial clefts
complete : cleft involves the entire primary and secondarypalates. It extends from the uvula all the way into thealveolar ridge. It involves both the primary palate andsecondary palate.Types-Unilateral-Bilateral
incomplete: cleft starts at the back of the palate with the uvulaand extends forward. It may or may not reach the incisiveforamen. In simpler terms, it only involves the secondary palate asit does not extend all the way forward to include the alveolarridge.Bifid Uvula: The least severe of theincomplete clefts in appearance, a bifiduvula is the most common palatal cleft. It isalso referred to as a “cleft uvula.” A bifiduvula appears as a splitting or forking ofthe uvula. It may be very subtle, evidencedonly by a small notch, or the uvula mayappear as two distinct entities. A bifid uvulain and of itself is not problematic. Thisoccurs in about 2 percent of the population.However, usually a bifid uvula is indicativeof a submucosal cleft.
Submucosal Cleft: A submucosal cleft is a cleft that isunder the mucosa that lines the roof of the mouth --hence the term “sub.” Because a submucosal cleft isunder the mucosa, the only physical indicator of itspresence may be a bifid uvula. Even though not seenfrom the surface, the muscles of the palate are notjoined at the midline in a submucosal cleft. This createsan inability to move the palate for some speech sounds.Hence, a submucosal cleft is usually diagnosed when achild has abnormal speech development and a bifiduvula is present.Soft Palate Cleft: A cleft of the soft palate runs fromthe tip of the uvula and stops before or at the junctionof the soft and hard palate. Not only is it more obviousin its appearance than a submucosal cleft, it createsthe same speech problems as a submucosal cleft. Themore severe (longer) soft palate clefts are detected atbirth due to feeding difficulties. The cleft of the palatemakes it difficult for the infant to create a tight oral sealaround the nipple. As a result, the infant may not beable to suckle. A partial or shorter soft palate cleft maynot show symptoms at birth or may reveal itself asnasal reflux of liquids or foods.
Soft and Hard Palate Cleft: A cleft thatinvolves both the hard and soft palate willinclude the entire soft palate and any part ofthe hard palate up to the incisive foramen.The most severe form involves the entiresecondary palate, seen as a gap in the palatefrom the tip of the uvula to the incisiveforamen. This is the most overt of theincomplete palate clefts. Similar to theisolated soft palate clefts, the combined softand hard palate cleft is usually detected atbirth because of feeding problems. Speechdevelopment will be impaired.
complete: cleft extends all the way from the lip to the nose, The orbicularisoris muscle is not in continuity, Instead of encircling the mouth, the muscleinserts into the base of the nose on both sides of the cleft. This disruptioncreates difficulty speaking, eating and drinking. The nose is distorted with acleft lip deformity. The nostril is widened and the floor of the nostril is missingUnilateral: only affects one side of theupper lip, Two thirds of the Cupid’sbow, one philtral column and thephiltral dimple are preserved on thenoncleft/normal sidBilateral: A bilateral complete cleft lip affects both the right andleft sides of the lip. There are no philtral columns, no philtraldimple, and there is no orbicularis muscle in the centralsegment. On both sides, the cleft extends from the vermillion tothe nostril. The lack of continuity in the muscle makes clearspeech difficult for these individuals. Feeding can be moredifficult.There is nasal distortion with a bilateral complete cleft lip. Bothalar rims are widened, the nasal sills are missing and the nosetakes on a flattened appearance.
Incomplete can take on a variety of appearances. There may be just a smallgap or cleft in the vermillion or it may slightly extend into the skin above the lipor extend almost to the nostril. As with a complete cleft lip deformity, the nosehas some distortion. The distortion is usually to a lesser degree. The nostrilmay be widened, but the floor of the nostril is intact. Additionally, there is abridge of tissue at the base of the nostril on the cleft side. This is known as aSimonart’s bandUnilateral: has clefting of the lip on one sideonly. There is a normal philtral column,Cupids bow and philtral dimple on the sidewithout a cleft. What makes it different froma complete cleft lip is that some orbicularismuscle fibers may cross the cleftBilateral: A bilateral cleft lip has a gap on both sides of the upperlip. Just like with a bilateral complete cleft lip, the philtral columnsare affected. To what degree depends on how far up the cleftextends from the vermillion. There may be some orbicularis muscleintact. The nostril sills are intact. The philtral dimple is intact.In a bilateral incomplete cleft lip deformity, the nose is affected,although not to the same degree as with a bilateral complete cleftlip deformity. In those individuals with a bilateral cleft lip deformity,one side may be an incomplete deformity and the other side acomplete deformity.
Mixed Bilateral Incomplete and CompleteCleft LipIn those who have a bilateral cleft lip, one sidemay be an incomplete cleft lip and the otherside a complete cleft lip deformity. Anincomplete cleft lip may be barely imperceptible.A microform cleft lip(also known as a "forme fruste") is the mildest versionof a cleft lip, and is categorized as an incomplete cleft.A microform cleft lip does not have the obvious cleftappearance of the other clefts. It may simply appearas a vertical scar from the lip to the nose. There maybe a notch in the vermillion border and the nose maybe affected. While a microform cleft may not beobvious, there may still be a problem with functionality.There is some disruption of the continuity of theorbicularis oris muscle. This may cause eating anddrinking issues as well as speech issues.The nose is usually normal with a microform cleftdeformity
Diagnosis of cleft lip and palate during pregnancy (antenataldiagnosis)•cleft lip and cleft palate are being diagnosed by ultrasound before the baby isborn. An ultrasound is a test that uses sound waves to create pictures of thedeveloping fetus. When analyzing the pictures, a doctor may detect anabnormality in the facial structures.A vertical hypo-echoic region through the fetal upper lip usually represents thedefect in cleft lipCleft lip may be detected with ultrasound beginning around the 18th-20 week ofpregnancy. As the fetus continues developing, it may be easier to accuratelydiagnose a cleft lip. Cleft palate that occurs on its own is more difficult todiagnose using ultrasound, since it can be difficult to see inside the fetus mouth.Occasionally a cleft is not picked up on the scan because the face is not visibleon the scan. Cleft palate without cleft lip is difficult to detect by antenatal scans
Advantages of PrenatalDiagnosisTime for parental educationTime for parental psychological preparationOpportunity to investigate other associatedanomaliesGives parents the choice of continuing thepregnancyOpportunity for fetal
Diagnosis of cleft lip and palate after birthMost cases of cleft lip and cleft palate are immediately apparent at birthand dont require special tests for diagnosis.Diagnosis may be made at birth using complete physical examination bythe physician. Since many of the associated syndromes show defects inthe genes and chromosomes, a chromosomal analysis is suggested forthe baby.Diagnosis of associated problems like feeding problems, hearing loss,ear infections, speech defects and teething problems is also important intreatment of cleft lip and/or palate
Cleft lip and palateteamPediatrician :provide medical care and refer toplastic surgeonplastic surgeon : initial lip repair and palatal surgery, pharyngoplasty and nose surgeryMaxillofacial surgery :bone grafting andorthopedic surgery in later stage.
Neurosurgery :for any craniofacial syndromePedodontic: the key member to asses patientand his parents , monitor growth ,guideocclusion and facial growthOrthodontic: definitive orthodontic treatmentonce full permanent arch eruptSpeech pathologist: monitor speechdevelopment to normal ,test for adequatepalate and pharyngeal closure
Audiologist :test hearing in infant andchildhoodPsychologist: play important role whenchild and family under stress
Problems Associated With Cleft Lipand PalateFeedingDental problemsNasal Deformity and Esthetic ProblemsEar ProblemsSpeech Difficulties
Feeding Difficulties Cleft lip= makes it more difficult for an infant tosuck on a nipple Cleft Palate= may cause formula or breast milkto be accidently taken up into the nasal cavity Inability to create negative pressure inside oralcavity Frequent regurgitations Upper respiratory tract infections
Management of feeding problems :i) For cleft lip patient1- Breast feeding: mother can close cleft by theareola or her hand to provide a seal.2- Bottle feeding : a broad based nipple on a regularbottle to provide the seal.ii) For cleft palate :Depending on severity of the cleft a variety offeeding devices are available :a) Soft premature nipples: conforms better to thepalatal defect and thus improves sucking.b) Cross cut nipples: allow for easier flow of formula,and thus decreasing strain on the child.c) Long nipples: can be positioned posterior to thedefect.
Dental Problems Local Dental Problems: Congenitally Missing teeth, Hypodontia, Hyperdontia,Oligodontia Presence of natal and neonatal teeth Anamalies of tooth morphology like microdontia,macrodontia etc Fused teeth Enamel Hypoplasia Poor periodontal support, early loss of teeth Gemination, Dilacerations Orthodontics Problems: Class III tendency Anterior and Posterior Cross bite Spacing and crowding
Nasal Deformity and EstheticProblemsFacial DisfigurementsPoor nasal shapeScar marks of surgeriesPoor lip function duringspeechPoor dental alignmentand smile
Ear Problems Middle ear disease - 22% to 88% Conductive hearing loss and chronic suppurative otitismedia may result Repeated tympanostomy tube placement
Causes of ear problem Mechanical Infection Dynamic
Speech Problems: Hearing loss hampers proper development ofspeech Velopharyngeal Insufficiency (VPI) Abnormal air Poor pronunciation of Bilabial, Labiodental,Linguoalveolar sounds
General Management Protocol for theCleft PatientImmediately after the birth –pediatric consultation,feeding instructions,evaluation by geneticistdiagnosis of life expectancy of a child
At 10 to 12 weeks - surgical repair of the lipCleft Lip Repair Goals Approximate cleft edges Maintain Cupids bow and philtrum Align vermillion border Create an intact nasal floor Reconstituting the circumferentialintegrity of the orbicularis oris muscle
From age 1 year to 18 months –team evaluation and surgical repair of cleft palateadvantages for early closure of palatal defects:-(1) better palatal and pharyngeal muscle development(2) ease of feeding(3) better development of phonation skills(4) better auditory tube function(6) improved psychologic state for parents and baby.
If the cleft is very wide, it is better to delay closure.- Delayed palatal surgery will limit the inhibition of themaxillary growth and minimizing the need for surgery towiden the maxilla.But, will lead to development of compensatory speechand swallowing pattern, which are not easily corrected.During this period we useNon-Surgical methodsDental Obturator. a plastic/acrylic, removable roof of themouth, which aids in speech, eating, and proper air flow.For high-risk patients or those that refuse surgery.Advantage- High rate of closureDisadvantage- Need to wear a prosthesis, and need tomodify prosthesis as child grows.Three months after palate repair- teamevaluation for speech and language assessment.
Three to six years – team evaluation behavioral intervention asneeded. Speech therapy treatment for middle yearinfection, fistula repair, psychological evaluation.
At seven years - Orthodontic treatment phase I.Nine to eleven years - alveolar bone grafting to Provide bone for the eruption and/or orthodonticrepositioning of teeth Closure of oro-nasal fistulas Stabilization of the pre-maxilla in bilateral casesTwelve years or later - Full orthodontic treatment phase II.Fifteen to eighteen years - at the end of orthodontictreatment, placement of implants, fixed bridge, etc. formissing teeth.
Eighteen to twenty-one years – when most of growth is completed,Surgical advancement of maxilla, ifrequired. Final nose and lip revision -rhinoplasty, 16-18 years.
In conclusion Family support is critical for yourchild. Love and understanding willhelp him or her grow up with a senseof self-esteem that extends beyond thephysical defect
Langman Essential medical embryology forT.W.salderConlemporary oral and maxillofacialsurgeryHand book of Pediatric Dentistry ByRichard P widmer