Clefts of the Lip and Palate

Clefts of the Lip and Palate

©1997 Erlanger Health System Tennessee Craniofacial Center 1(800) 418-3223

Each year approximately 227,500 or 7percent of births in the United States areaffected by birth defects of the head andface. The most common of these areclefts of the lip and palate which occuronce in every 700 births. Clefts occur ininfants of all races with a 2:1 male tofemale ratio. The incidence of clefts ishighest in the Asian population andlowest in African Americans. Of all oro-facial clefts, 21 percent present as cleftlip only (unilateral and bilateral), 46 per-cent present as cleft lip and palate, whilethe remaining 33 percent have cleftpalate alone.

What is a cleft? A cleft is a divisionor separation of parts of the lip or roof ofthe mouth that is formed during theearly months of development of theunborn child. All of the parts of the lipor roof of the mouth are present; theysimply failed to fuse in a normal way.Surgical intervention is necessary toalign the parts and join them. Often thebones of upper jaw (maxilla) and/or theupper gum are affected. A cleft lip can beincomplete with a variable degree ofnotching of the lip, or complete, extend-ing through the lip and into the nose.

Preoperative Postoperative

CLEFTS OF THE LIP AND PALATE

Variations in clefts of the palate.

A). Cleft of soft palate only. B). Incomplete cleftof soft and hard palate. C). Complete unilateralcleft palate. D). Complete bilateral cleft palate.


FEEDING AN INFANT WITH A CLEFT

Feeding an infant is important not only inproviding nourishment, it also provides anintimacy and closeness for both the parentand the child. Infants with a cleft of the lip orof the soft palate seldom have problems withfeeding either by bottle or breast.

In babies with clefts of the hard palate, theopening in the roof of the mouth often causesdifficulty in creating adequate pressure onthe nipple, thus creating an inability to suckwell enough to get adequate nourishment.Feeding the infant takes patience and prac-tice. At our center we recommend the use of asoft squeezable plastic bottle like MeadJohnson with an orthodontic nipple such asNuk. You can increase the flow by gentlysqueezing or putting pressure on the bottle. Itis important to feed the infant before he/shebecomes too hungry. Position the infant in anupright position with the head tilted backslightly. This position allows the milk to flowdown into the throat and less into the nose.Infants with clefts do swallow more air andneed to be burped more frequently. At first, itmay take extra time, but this will steadilydecrease . Feeding time of the newborn variesfrom 20-30 minutes. When feeding takeslonger than 45 minutes, the infant may beburning up calories necessary to gain weight.If this occurs the feeding consultant should becontacted to help with the feeding technique.

Breast feeding the newborn with a cleft ofthe hard palate is often unsuccessful.Generally the infant cannot produce enoughnegative pressure to obtain ample breast milkto provide adequate nourishment. Using abreast pump to extract the milk and feedingthe infant breast milk from a squeezable bottle is recommended.

Clefts of the palate can vary in severity.Some may involve just the uvula and the softpalate. These are incomplete clefts of thepalate. Others extend the length of the palateand are complete clefts. They may involveone side of the palate (unilateral) or bothsides (bilateral).

Etiology: The exact cause of lip andpalatal clefting is not known, but mostexperts feel that it is due to both genetic andenvironmental factors. Clefts are associatedwith abnormalities in the genes which maybe a result of inheritance or from a sponta-neous mutation during fetal development.We recommend genetics counseling to dis-cuss causes of the cleft and the recurrencerisk factors.

Team Assessment: Children born withclefts should be carefully assessed by thecraniofacial team in order to detect potential-ly serious abnormalities that can be associat-ed with clefting. There are over 150syndromes that include cleft lip or palate intheir differential diagnosis. Generally, cleft-ing is the only congenital abnormality thatthe child has, but nearly 15 percent of all cleftlip or palate patients present clinically withmultiple problems.

The team concept allows a systematic,comprehensive treatment plan to be devel-oped and allows the team members to worktogether to identify problems before theybecome significant. The most common spe-cialities involved in the care of a child with acleft are: plastic surgery, otolaryngology, den-tistry, audiology, speech pathology, geneticsand pediatrics. Once a complete assessmentof the child with a cleft has been performed,a plan for treatment can be outlined.


SURGERYCleft Lip Repair - The objec-

tive in repairing the lip is toclose the cleft to create a pleas-ing face that will develop nor-mally with minimal scarring.Closure of the lip is performedby the plastic surgeon when thebaby is approximately 3 monthsof age and weighs at least 10pounds. When there is involve-ment of the alveolus and palate,an orthodontic appliance maybe placed in the maxillary seg-ments as the first procedure.This is performed by the teamdentist as an outpatient surgicalprocedure. The appliance isused to align the alveolus so thatit can be repaired (gingivope-riosteoplasty) at the time of thelip repair or lip adhesion. Thisimproves nasal support on thecleft side and creates a tunnelthat should develop bone, clos-ing the cleft. If the alveolus is notclosed in infancy, then the alveo-lar ridges will be orthodontical-ly aligned and a bone graftperformed to stabilize the maxil-la (5-10 years of age). Correctionof the nasal deformity is usuallyperformed at the time of liprepair. Additional proceduresmay be necessary to enhance theappearance of either the lip ornose.

Unilateral Cleft Lip with Custom Latham

Photos to the right:

A unilateral cleft lip is shown. A customfitted Latham appliance has been fabricatedand placed to rotate alveolar segmentstogether. Postoperative result is shown afterrepair of alveolar cleft and lip.


UNILATERALCLEFT LIP REPAIR

A unilateral cleft lip results fromfailure of the union of the maxillaryand median nasal processes, thuscreating a split or cleft in the lip oneither the left or right side. It may bejust a notching of the lip or extendcompletely through the lip into thenose and palate. A number of proce-dures have been described to repairthe unilateral cleft lip. The proce-dure used at our Center is theMillard rotation advancement tech-nique. The procedure is designed toreconstruct the lip, muscle, oralmucosa, and to reposition the nose.It is performed under general anes-thesia with surgery lasting 2-3 hoursand a hospital stay of 2-4 days.Special considerations are necessaryfor feeding and positioning theinfant postoperatively. The baby’selbows are restrained from bendingto prevent him/her from disruptingthe nose or lip. Positioning the childin an infant seat keeps him/her fromrolling over and injuring the lip ornose. Pacifiers and nipples are notallowed. The baby is fed with a spe-cial syringe feeder with a soft tube. Ittakes approximately 3 weeks for thewound to gain enough strength todiscontinue the above precautions.The lip scar is initially red andswollen, but it begins to mature andimprove in appearance in six-twelvemonths.

Unilateral Cleft Lip




Photos of infants who underwent repair ofunilateral cleft lip with rotation advance-ment technique.



BILATERALCLEFT LIP

The bilateral cleft lip involvesseparation of the lip alongphiltral lines, isolating the centralsegment (prolabium). Fifteen per-cent of children born with cleftlips have bilateral clefts. Theassociated nasal deformity is usu-ally more severe than the unilat-eral cleft due to a very shortcolumella and flaring of both nos-trils. Surgical correction of thebilateral cleft lip is usually per-formed in one procedure at threemonths of age; however, the pro-cedure may be staged, closingone cleft at a time. Rotation of thenostrils to a more normal positionis performed in the first proce-dure. A second procedure is per-formed by 2-3 years of age tolengthen the columella. Patientswith complete bilateral cleft lipsfrequently require additional pro-cedures to enhance the appear-ance of the lip and nose.Performed under general anes-thesia, the operation generallyrequires 2-3 hours. A hospitalstay of 2-4 days should be expect-ed. Feeding, positioning andelbow restraints are the same asthose for repair of the unilateralcleft lip.

Bilateral Cleft Lip





Photos of infants who underwent onestage repair of bilateral cleft lip.


CLEFT PALATE REPAIRThe objective of cleft palate

surgery is to close the palate torestore normal function to eatingand drinking and to enhance thedevelopment of normal speech.

Clefts of the palate can occur asisolated deformities or in combina-tion with a cleft of the lip. Cleftpalates result from failure of fusionof the embryonic facial processesresulting in a fissure through thepalate. This may be complete(extending through the hard andsoft palates) or any degree ofincomplete (partial cleft). Thepalate forms the roof of the oralcavity and the floor of the nose;thus, a cleft causes a free communi-cation between these two cavities.As a result, treatment of palatalclefts is complex because of poten-tial problems with feeding, speech,middle ear infections, occlusionand jaw alignment.

Surgical treatment of the cleftpalate is best accomplished in onesurgical procedure before the childreaches 12-14 months of age. Thecleft palate is surgically closed byelevating two muscoperiostealflaps. The levator muscles are ele-vated, redirected and repaired; anda three layer closure of nasalmucosa, muscle and oral mucosaaccomplished. Surgery under gen-eral anesthesia usually lasts about2 hours. Special precautions asthose after the repair of the cleft lipare necessary for 2-3 weeks. Weprefer that the child be weanedfrom the bottle and pacifier prior tothe palatal repair. No hard orcrunchy foods are allowed for 3weeks post operatively.

Cleft Palate Repair:

Closure of cleft palate with pushback palatoplasty. A). Twomucoperiosteal flaps are outlined. B). Flaps are elevated off thehard palate. C,D). The abnormal levator muscle insertion to thehard palate is identified and cut free. E). The nasal lining isclosed as a separate layer and the levator muscle reapproximated.F). The palatal mucoperiosteal flaps are closed in a V-Y fashion.


PHARYNGEAL FLAPApproximately 70-80 percent of

all cleft palate patients will developvelopharyngeal competence afterpalate closure and thus the potentialfor normal speech. The remaining20-30 percent will require speechtherapy and/or an additional surgi-cal procedure called a pharyngealflap. To correct persistent hypernasalspeech, this procedure involves rais-ing a flap of tissue from the posteriorpharynx and inserting it into the softpalate. This flap is indicated whenthe repaired palate is too short or themuscles do not function properly,causing a persistent hypernasalspeech. The procedure is performedusually after the age of 4-5 whenspeech and velopharyngeal compe-tence can be thoroughly assessedand before the child begins school.

LATE CLEFT TREATMENT

The Craniofacial Center can alsohelp those individuals that havegrown up without access to a com-prehensive, coordinated teamapproach. For adults with speechproblems, the previously mentionedpharyngeal flap, combined with anintensive regimen of speech therapy,can produce significant improve-ments. Orthognatic surgery is avail-able to patients with deformities ofthe jaws to improve their appearanceas well as to correct dental occlusion.For soft tissue revision of a severelytightened or notched upper lip, anAbbe flap is the surgical option. Thisprocedure is usually indicated inbilateral cleft patients who have ashort or deficient columella and atightened upper lip. This operation

Pharyngeal Flap:

The pharyngeal flap procedure for hypernasal speech. A superior-ly based flap of tissue is raised from the posterior pharynx andsutured to the soft palate thereby decreasing the amount of airthrough the nose. Lateral ports or holes are left so that the nosewill not be obstructed.

Inadequate Closure

Adequate Closure

Corrected Closure

patients will have velopharyngeal incompe-tence or hypernasal speech after surgery, andmay require a pharyngeal flap to correct itaround the age of 4-5 years.

DENTALClefts of the palate generally have an effect

on dental development. In the area of the cleft,teeth often erupt in a crooked position withextra teeth or missing teeth being common inthe cleft area. Radiographs are often taken todetermine the exact position of the teeth.Dental problems have an effect on speech,chewing, appearance and frequently requireorthodontic treatment. Early orthodontic inter-vention may require a palatal expansion devicewith further alignment of the dental arches.Later treatment after the primary teeth haveerupted can begin at 10-12 years of age.Orthognathic surgery may be indicated if amalocclusion develops due to abnormalgrowth of the maxilla.

PIERRE ROBIN SYNDROME

This syndrome was described in 1923 byPierre Robin in which he described airwayobstruction associated with glossoptosis andhypoplasia of the mandible. Today this syn-drome is characterized by retrognathia ormicrognathia, glossoptosis, and airway obstruc-tion. An incomplete cleft of the palate is associ-ated with the syndrome in approximately 50%of these patients.

In patients with micrognathia (small jaw) orretrognathia, the chin is posteriorly displacedcausing the tongue to fall backward toward theposterior pharyngeal wall. This results inobstruction of the airway on inspiration. Cryingor straining by these children can often keepthe airway open. However, when the childrelaxes or falls asleep, airway obstructionoccurs. Due to these respiratory problems, feed-ing may become very difficult. This can lead to


can add fullness to the upper lip as well aslengthen the columella. A number of addi-tional surgical therapies, similar to the onesdescribed, are available to patients whodesire further improvements.

HEARINGChildren with cleft palate have a higher

incidence of hearing problems. TheEustachian tube connects the middle earspace to the back of the throat. It normallyopens and shuts to relieve pressure thatbuilds up behind the ear drum. If theEustachian tube does not open, then the pres-sure increases until mucus or “fluid” accu-mulates behind the eardrum. The musclesresponsible for opening the Eustachian tubedo not function as well in children with cleftpalates resulting in more frequent problemswith fluid, otitis media and ear infectionswhich can be very painful. Because of thisproblem, it is important to have the infant’shearing tested during the first few months. Ifhearing is impaired by fluid buildup orunequal pressure, it may be necessary for theotolaryngologist to place pressure equalizing(PE) tubes. Tubes are often placed at the timeof the lip or palate surgery. It is crucial thatchildren with cleft palates have regular hear-ing tests to monitor middle ear problems thatcould alter the development of normal hear-ing as well as speech. As the child grows, thefrequency of ear infections and fluid in theears seem to decrease.

SPEECHSpeech development in children with cleft

lip only should be normal. The unrepairedcleft palate causes speech to sound hyper-nasal because air passes through the nosewhile talking. Most speech sounds requirethe nose to be closed off from the mouth. Cleftpalate surgery usually remedies the problem,but speech therapy is still recommended.Approximately 20-30 percent of cleft palate


a sequence of events: glossoptosis,airway obstruction, crying or strain-ing with increased energy expendi-ture and decreased oral intake. Thisvicious cycle of events if untreatedcan led to exhaustion, cardiac fail-ure, and ultimately death.

Treatment of this syndrome canbe divided into conservative therapyversus surgical intervention. Themajority of these patients can bemanaged by placing the infant in theprone position until adequategrowth of the jaw occurs. This caus-es the jaw and the tongue to fall for-ward opening the airway. If this typeof treatment fails the infant shouldthen be considered for a tongue-lipadhesion (a procedure to pull thetongue forward) or a tracheostomy.

In children with severe underde-velopment of the lower jaw, a newtechnique called mandibular boneexpansion is now available. Thistechnique also called distractionosteogenesis involves placement ofan expansion device that is turneddaily to slowly lengthen the jaw. Anexternal incision is required to makea surgical cut through the jaw bonewith placement of pins that aresecured to the expansion device.Once the amount of expansion of thebone has been obtained (4-5 weeks)the device is then kept in place untilthe bone gap heals with new boneformation (8 weeks). This techniquecan be performed at a very early agewhich is a significant advantageover the traditional technique oflower jaw lengthening.

Results after removal of lengthening devices. Patient now readyfor removal/closure of tracheostomy.

Bilateral mandibular lengthening devices surgically placed withlengthening in progress.

Two year old with Pierre Robin and severe airway obstruc-tion requiring tracheostomy.

Pierre Robin

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Clefts of the Lip and Palate

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