Cleft Lip Palate Pic 2013 10

8/12/2019 Cleft Lip Palate Pic 2013 10

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Outline

Introduction

Prevalence

Etiology

Embryology

Classification

Management

Defects and Reconstruction


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Introduction Approximately 15 types of orofacial clefting with cleft lip and palate being the

most common

Most common congenital malformations of the head and neck

1 in 700 live births (1/1000 in the US)

In the US, approximately 7500 babies are born with orofacial clefts per yearwith an average lifetime medical cost of $100,000 (750 million per year)

Associated problems include otological disease, speech and language

problems, dental deformities, and psychosocial issues

Best managed with a multidisciplinary approach (medical and surgical)

NOTES: There are approximately 15 types of orofacial clefting with cleft lip and palate being the most common. Orofacial clefts are the second most

common congenital malformation of the body, and the most common congenital malformation of the head and neck(Cummings). They occur in

approximately 1/700 live births (Ballengher) with a rate of about 1/1000 live births in the US. There are approximately 7500 babies born in the US with

orofacial clefts per year with an average lifetime medical cost of $100,000 per baby or 750 miilion dollar lifetime cost for all children per year. Cleft lip and

palate have many associated medical problems which include otological disease, speech problems, dental deformities and psychosocial issues. These

problems are best managed with a multidisciplinary approach with a coordinated medical and surgical care plan providing the best outcomes.


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Prevalence

Ethnic groups(CL+/-P)

Highest rate

Native American and Asians ( 2/1000 live births)

Intermediate rate

European descendants (1/1000 live births)

Lowest rate

African populations (1/2500 live births)

No difference between ethnic groups for cleft palate only (1/2000 live birth)

Gender

2:1 M:F ratio cleft lip +/- palate

1:2 M:F ratio cleft palate only (late closure of palatine shelves)

NOTES: here are both ethnic and gender differences in birth prevalence of cleft lip and palate. Among ethnic groups, thehighest rate occurs among Native American and Asian populations with approximately 2/1000 live births. There is anintermediate rate among those of European descent with approximately 1/1000 live births. The lowest rate of cleft lip andpalate occurs among African populations with 1/2500 live births. There is no difference between ethnic groups for cleft palateonly, with approximately 1/2000 liver births. There is a gender difference when it comes to cleft lip and palate formation.There is a 2:1 male to female ratio when comes to clefts involving the lip with or without cleft palate, and there 1:2 male to

female ratio for clefts involving the palate only. It is thought that the reason the ratio changes for cleft palate only is due tothe fact that the palatine shelves close 1 week later in girls than boys


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Causes

Folklore explanations Aztecs eclipses occurred because a bite had been taken out of the

moon

Prevented with an obsidian knife above the pregnant abdomen

Modern Mexico prevented with keys and safety pins

NOTES: There have been many speculations to the causes of cleft lip and palatethroughout human history with many folklore explanations. The aztecs believed

eclipses occurred because a bite had been taken out of the moon and exposureto an eclipse during pregnancy could lead to a bite out of the babys mouth. Toprevent clefts, pregnant women were required to place obsidian knives overthere abdomen before going out at night. This belief has now extended tomodern day Mexico and during eclipses a metal key or safety pin is placed overthe abdomen for protection.


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Early Chinese

Eating rabbit hare lip

Bad karma or wrongdoings

Philippines

Force to the fetal face

Familial or In the blood

NOTES: An early Chinese belief was that eating rabbit during pregnancy could lead to ahare lip, and others believed that bad karma or wrongdoings were the cause. Inmodern day Filipinos, a common belief is that force to the fetal face when fingers are inthe mouth is the cause of clefts. Many other cultures believed that clefts were familialor in the blood. Most of the early thoughts on the causes of cleft have been disprovedof course but the belief that there was familial component still stands today.


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Familial 2 unaffected parents with 1 child

affected

Risk for future children:

4.4% for CL+/- palate

2.5% for CP only

1 parent affected

Risk for future children


6.8% for CP only

1 parent affected with 1 child affected

Risk for future children


14.9% for CP only

NOTES: It has long been known thatfamilial inheritance plays a role in cleftformation. Here is some of the data thatsupports familial inheritance. In 2unaffected parents with 1 child affected,the risk for future children is 4.4% forcleft lip with or without cleft palate and

2.5% for cleft palate only. If only 1parent is affected the risk for having achild with cleft lip with or without palateis 3.2% and the risk for CP only is 6.8%. Ifone parent and one child is affected therisk for future children increases to 15.8%for cleft lip with or without cleft palate

and 14.9% for cleft palate only. Theseexact percentages vary from study tostudy, but the common theme amongstudies is that as the number of familymembers with cleft deformities increase,the risk for future children increase aswell.NOTES


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Etiology Majority of orofacial clefts are

nonsyndromic

70% of CL +/- palate

50% of CP only

Nonsyndromic clefts

multifactorial

Clusters in families but not mendelian

Palate development complex process

with several proteins, growth factors, and

transcription factors involved

IRF-6, TGF B2, TGF-alpha

Any disturbance in the process can result

in clefting

NOTES: The majority of clefts are

considered nonsyndromic or

isolated anomalies. Approximately

70% of cleft lip with or without

palate and 50% of cleft palate only

are nonsyndromic. There is no exact

mechanism that has been

discovered for the cause ofnonsyndromic cleft, and the cause is

believed to be multifactorial. As I

showed in the previous slide clefts

tend to cluster in families but are

not exactly mendelian in

inheritance. We do know that

normal palate development is acomplex process with several

proteins, growth factors, and

transcription factors involved. Any

disturbance in the developmental

sequence can result in cleft

formation.


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Etiology

Syndromic clefts:

Associated with over 300

syndromes

Van der Woude syndrome the most

common

Autosomal dominant

Lower lip pits

Teratogen exposure

Ethanol, thalidomide, phenytoin

Environmental factors

Amniotic banding, maternal

diabetes, maternal folate deficiency

NOTES: Syndromic clefts are less

common than nonsyndromic clefts

and by definition are associated

with other malformations orsyndromes. Over 300 syndromes

have been identified that are

associated with orofacial clefting.

The most common syndrome

associated with cleft lip and palate

is van der woude syndrome. This

syndrome is autosomal dominantand has characteristic lower lip pits

with orofacial clefts. Teratogens

exposure and environmental

factors have also been linked to

cleft formation.


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Anatomy

Orbicularis oris Vermillion (wet/dry border)

Cupids bow

Along the upper vermillion

cutaneous border (white

roll), two midline

elevations form the bow

Philtrum Philtral columns and

dimple

Tubercle

I will now be covering some of the normal anatomy.The obuclaris oris is the muscle that surrounds the lipsand acts as a sphincter for the mouth. The vermillionis the red portion of the lips and important landmark iswet/dry border. Along the upper vermilion-skin

border or white roll, two midline elevations form theCupids bow. Two raised vertical columns of tissue

extending from the apices of the bow are known asthe philtral columns. The area of depression betweenthe columns is known as the philtral dimple. Thephiltrum extends from the cupids bow to thecolumella which are both important structures whenreconstructing the lip. The tubercle which is notlabeled is the midline protuberance of the upper lip

which is also important in reconstruction.


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Normal Anatomy Hard Palate

Palatine processes of the maxilla

Horizontal plates of the palatine bone

Incisive foramen, greater and lesser palatine foramen


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Normal Anatomy The hard palate is made up of the palatine processes of the maxilla

and the horizontal plates of the palatine bone. It is bound anteriorly

and laterally by the alveolar arches and is continuous with soft palate

posteriorly. An important structure here is the incisive foramen which

marks the division between the primary and secondary palate during

development and is the foramen through which the nasopalatine

nerve enters the palate. Also important is the greater palatine and

lesser palatine foramen from which the greater palatine and lesser

palatine nerve/artery enters the palate. This is important because theflaps for 2 flap palatoplasty receive its blood supply from the greater

palatine artery.


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Normal Anatomy

Soft palate Palatine aponeurosis tendon of tensor veli palatini

Muscular portion consists of the tensor veli palatini (CN V), levator veli

palatini, palatoglossus, palatopharyngeus, and musculus uvulae (CN

X)


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Normal Anatomy

The soft palate consist of 2 parts. The anterior portion is the

palatine aponeurosis which attaches to the posterior margin of the

hard palate and is formed by the expanded tendon of the tensor

veli palatini. The muscles of the soft palate attach to the

aponeurosis. The muscular posterior portion of the soft palate

consist of 6 muscles: tensolr veli palatini, levator veli palatini,

palatoglossus, palatopharyngeus, superior pharyngeal constrictor,and the uvular muscle.


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Normal Anatomy Vasculature

Greater palatine, lesser palatine , and

ascending palatine artery

Nerve supply

Hard palate

Greater palatine nerve supplies the gingivae,

mucous membranes, and glands of most of

the hard palate

Nasopalatine nerve supplies the mucosa of

the anterior portion of the hard palate

Soft palate

Sensory lesser palatine nerves

Motor tensor veli palatini is supplied by CN

V and the other muscles are supplied by CN X

NOTES: The blood supply to thepalate is mainly from the greaterpalatine artery which arises from themaxillary artery, but it also receivessome supply from the lesser palatineand ascending palatine arteries. Thehard palate is innervated by the

greater palatine and the nasopalatinenerves. The greater palatine nervesupplies the gingivae, mucousmembranes, and glands of most ofthe hard palate and the nasopalatinenerve supplies the mucosa of theanterior portion of the palate. The

soft palate receives sensoryinnervation from the lesser palatinenerves and receives its motorinnervation from 2 cranial nerves.The tensor veli palatini is supplied bycranial nerve V and the 4 othermuscles are supplied by cranial nerveX.


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Embryology

Development of the lip and palate

begins around the 4thweek of

embryological development

Completed by the end of the 12th

week

By the end of the 4thweek

5 facial prominences have formed

frontonasal process

paired maxillary processes

paired mandibular processes


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Embryology

During the 5thweek

Nasal placodes invaginate to form the nasal pits

Lateral and medial nasal prominences


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Embryology By the end of the 6thweek

Paired maxillary processes have grown medially and pushed the paired medial nasal prominencestogether

Fusion of the paired medial nasal prominences form:

Philtrum

Middle upper lip

Nasal tip Columella

Fusion of the paired maxillary prominences with the paired medial nasal prominences forms the

complete upper lip (maxillary prominences form lateral lip)

The lateral nasal prominences form the bilateral nasal ala


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Embryology Notes

Normal embryological of the lip and palate is essential in understanding how clefts form. The development

of the lip and palate begins around the 4thweek of embryological development and is completed by the end

of the 12thweek. By the end of the 4thweek, the five facial prominences have formed which include the

frontonasal processes, the paired maxillary processes, and the paired mandibular processes. These 5 facial

prominences surround the developing oral cavity.

During the 5thweek of development the nasal placodes form from the the frontonasal process. The nasal

placodes invaginate to form the nasal pits, along with the lateral and medial nasal prominences or swellings

By the end of the 6thweek, the paired maxillary processes have grown medially and pushed the medial nasal

prominences together. Fusion of the medial nasal prominences form the plitrum, middle upper lip, nasal tip,

and collumella. Fusion of the paired maxillary prominences with the paired medial nasal prominences forms

the complete upper lip with the maxillary prominences forming the lateral lip. The lateral nasal prominences

form the bilateral nasal ala.


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Palate Formation

Palate formation begins at the end of the 5thweek of

development and is completed by the 12thweek

The completed palate is formed by the primary palate

and the secondary palate which are separated by the

incisive foramen


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Primary Palate

Medial nasal prominences

fuse to form the primary

palate

Consists of maxillary

alveolar arch with 4

incisors and the hard

palate anterior to the

incisive foramen

Primary palate forms

before the secondary

palate begins formation


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Secondary Palate

During the 6th

week Shelf-like outgrowths from the bilateral maxillary processes, grow vertically

down on both sides of the tongue

During the 7thweek

The tongue moves inferiorly and the palatal shelves migrate to a horizontalposition above the tongue

Palatal fusion occurs in an anterior to posterior direction and completes

with uvular fusion (1 week later in females)


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Cleft Lip and Palate Formation

Disruptions at any stage of the developmental process can result in

clefts

Timing

Positioning

Pierre Robin Sequence micrognathia

Wide U shaped cleft palate


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Fusion

Failure of fusion of the maxillary and medial nasal prominences

unilaterally or bilaterally result in unilateral or bilateral cleft lip

with/without primary palate

Failure of fusion of the palatal shelves result in clefts of the secondary

palate

Cleft Lip and Palate Formation


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Classification

Clefts

Unilateral or bilateral

Complete or incomplete

Veau classification

Class I incomplete cleft involving only the soft palate

Class II cleft involving the hard and soft palate

Class III complete unilateral cleft involving the lip and palate

Class IV complete bilateral cleft

Modified versions


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Unilateral Cleft Lip

Incomplete Muscle fibers of the orbicularis oris are often

intact but hypoplastic

Varying degrees of clefting

Complete Orbicularis oris inserts at the columella medially

and ala laterally on the cleft side

Columella is displaced to the normal side

Nasal ala on the side of the cleft is displacedlaterally, inferiorly, and posteriorly

Nasal tip is deflected towards the noncleft side

Alveolus may or may not be involved


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Bilateral Cleft Lip

Orbicularis oris attaches at the lateral cleft marginsbilaterally at the nasal ala

Premaxilla protrusion

Symmetrical nasal deformities

Laterally displaced ala widely flared

Extremely short columella


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Cleft Palate

Primary palate

Clefts anterior to the incisive

foramen

Secondary palate

Posterior to the incisive foramen

Develops due to failure of the

palatal shelves to fuse

Abnormal insertion of themuscles into the medial edges

of the cleft

Vomer attachment is variable


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Cleft Lip/Palate Management

Multidisciplinary approach

Cleft care team

Plastic surgery

Audiology Speech pathology

Otolaryngology

Orthodontist

Oral maxillofacial surgery Psychologist

Geneticist

Pediatrician


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Management

Birth

Airway concerns

Feeding problems

Otological disease

Speech and language problems

Surgical Repair


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Airway Management Isolated cleft palate rarely results in airway compromise

Airway issues are usually associated with coexisting

structural abnormalities

Pierre Robin sequence most documented

Micrognathia, glossoptosis, and cleft palate

Associated with several syndromes (stickler, velocardiofacial syndrome, etc.)

Management prone positioning (severe cases sometimes require

tracheostomy

Mandibular distraction

Isolated cleft palate rarely results in airway compromise. Airway issues are usually associated with

coexisting structural abnormalities. Pierre Robin sequence is the most documented cause of airway

compromise in children born with cleft palate. Pierre Robin sequence refers to the grouping of

micrognathia, glossoptosis, and U shaped cleft palate. Glossoptosis can lead to airway obstruction.

Several syndromes have been associated with the development of pierre robin sequence with stickler

and velocardiofacial syndrome being the most common. The initial management of newborns with

pierre robin sequence is sometimes as simple as placing the newborn in prone position with severecases sometimes requiring tracheostomy. Mandibular distraction can also be used for correction.


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Feeding Difficulties

Critical aspect in management

Nutrition and feeding

Cleft palate limits the ability to suck due to the common cavity

Cleft lip alone

Special bottles

Premaxillary orthopedics

One of the most critical aspects in management of a newborn withcleft lip and palate other than airway concerns is nutrition and feeding.Before the patient leaves the hospital feeding techniques must beestablished. Cleft palate limits the ability to suck due to the commoncavity between the mouth and the nose. Cleft lip alone usually doesnot cause feeding problems. Special bottles have been developed toimprove the feeding in patients with cleft palate. Some patients

require premaxiilary orthopedics to feed appropriately.


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Otological Manifestations

Abnormal insertion of tensor veli palitini

Persistent OME has been estimated to be between 80-95% in children

with cleft palate

Paradise et al. in 1969 devised the term universality of otitis media in

cleft palate children after demonstrating that 96% of cleft patients had

middle ear effusion in a study of cleft patients at his institution

Most children with cleft palate have some form of ear disease. Theabnormal insertion of the tensor veli palitinini is believed to be thecause or contribute to the development of middle ear disease.Persistent OME has been estimated to be between 80-95% inchildren with cleft palate. Paradise et al. in 1969 devised the termuniversality of otitis media in cleft palate children afterdemonstrating that 96% of cleft patients had middle ear effusion in

a study of cleft patients at his institution.


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Otological Manifestations

Szabo et al. 2009

Retrospective study looking at the presence of MEE and treatment outcomes in

cleft palate patients from Connecticut Children's Medical Center between 2000-

2005

61 0f 74 (82%) of cleft palate patients passed their newborn hearing screen

98% of the patients in the study developed middle ear fluid requiring at least 1 set

of tubes

Approximately 74% of the patients in this study only required 1-2 sets of tubes

before resolving the ETD sufficiently that MEE did not reaccumulate

Sazabo et al. in 2009, did a retrospective study looking at the presence of MEE and treatment outcomes incleft palate patients from Connecticut Childrens Medical Center between 2000-2005. They found that 61 of74 or 82% of patients passed there newborn hearing screen. 98% of the patients in the study developedmiddle ear fluid requiring at least 1 set of tubes. Interestingly, 74% of the patients in this study only required1or 2 set of tubes before resolving the Eustachian tube dysfunction sufficiently the MEE did notreacummulate.


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Speech Development

Unrepaired cleft palate speech abnormality

Primary goal of palate repair is to restore function of the velopharyngeal valve

normal speech

10-20% will manifest VPI following surgical closure of the palate Hypernasality and articulation errors (glottal stops and pharyngeal fricatives)

Speech pathologist important role

Managed surgically (pharyngoplasty or pharyngeal flap) or with dental

prosthesisPractically, all patients with unrepaired cleft plate will develop some form of speech abnormality.The primary goal of palate repair is to restore function of the velopharyngeal valve which shouldrestore normal speech. Even with surgical closure of the palate 10-20% of patients have beenreported to have velopharyngeal insufficiency. These patients will have varying degrees ofhypernasality and articulation errors such as glottal stops and pharyngeal fricatives. The VPIfollowing palate surgery is first managed by speech pathology. If speech pathology alone isinsufficient, it can be managed surgically with pahryngoplasty or palatoplasty. Dental prosthesis

have also been used.


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Surgical Correction

Age 1-3 months Lip taping andnasoalveolar molding

Age 3 months - Repair of cleft lip (andplacement of ventilation tubes)

Age 9-12 months - Repair of cleft palate

Age 1-7 years - Orthodontic treatment

Age 7-8 years - Alveolar bone graft

18 years old or skeletal maturityMidfaceadvancement and continued orthodontic

treatmentTiming of surgical correction varies from institution to institution.Typically, presurgical techniques are used from 1-3 months. Cleft lip isrepaired at 3 months of age and myringotomy tubes are placed at the timeof repair. The palate is usually repaired from 9-12 months of age. Initialorthodontic treatment begins at 1 year of age. Alveolar bone grafting isperformed from 7-8 years of age before permanent teeth have erupted.Around 18 years of age or skeletal maturity midface advancement is

performed, if necessary.


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Presurgical Wide cleft lip or premaxilla protrusion

Advantageous to narrow the cleft and mold thepremaxilla before proceeding with surgery

Taping

Effective in reducing the width of the cleft in a

nonsurgical manner Strip of hypoallergenic tape is placed with

tension across the cleft and secured to thepatients cheek

Molds bony tissues by applying pressure to

protruding portions of the maxilla Must be worn 24 hours per day

Sometimes presurgical steps must be taken to improve surgical outcomes. When a wide cleft orpremaxilla protrusion is present, it is advantageous to narrow the cleft and/or mold the premaxilla beforedefinitive surgery is performed. There are several techniques that facilitate this correction. Taping hasbeen used effectively to reduce the width of a cleft and reduce premaxilla protrusion in a nonsurgicalmanner. With taping, strips of hypoallergenic tape is placed with tesnion across the cleft and sceured tothe patients cheek. This molds bony tissue by applying gentle pressure to protruding portions of the

maxilla. The only disadvantage to taping is that it must be worn 24 hrs per day.


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Presurgical Nasoalveolar molding devices

Custom made devices which utilize

wiring and nasal stenting to mold

the nasal cartilage, premaxilla, and

alveolar ridge Nasal stenting can be elongated and

adjusted to lengthen the columella

and mold the nasal cartilage

Takes advantage of the malleability

of nasal cartilage

Another form of nonsurgical correction which is being used to narrow clefts is the use oforthopedic devices. Nasoalveolar molding devices are custom made orthopedics whichutilize wiring and nasal stenting to mold the nasal cartilage, premaxilla, and alveolarridge. The nasal stenting can be elongated and adjusted to lengthen the columella andmold the the nasal cartilage. This method takes advantage of the malleability ofimmature nasal cartilage and its ability to maintain form once corrected. Here is a

picture of the correction that can be obtained by using nasoalveolar molding.


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Presurgical

Lip adhesion Surgically convert a complete cleft to an

incomplete cleft

Performed at 2-4 weeks with definitive repair at5-6 months

Indications Wide unilateral cleft where conventional repair

might produce excessive tension

Bilateral cleft premaxilla protrusion

Disadvantages scar tissue

When taping and nasoalveolar modling is not effective, lip adhesion can beconsidered. Lip adhesion is when a complete cleft is surgically converted toan incomplete cleft. Lip adhesion is performed at 2-4 weeks of age andextends definitive repair to 4-6 months to allow proper healing. It has thesame indications as the nonsurgical forms of correction which include wideunilateral clefts or premaxilla protrusion. The disadvantage to performinglip adhesion is the development of scar tissue which could interfere withdefinitive repair. Most surgeons do not perform lip adhesion because of thepossibility of extensive scar tissue formation.


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Cleft Lip Repair

Typically performed at 3 months of age

Rule of Tens

10 weeks old, 10 lbs, and hemoglobin of 10

Wide clefts or clefts with premaxilla protrusion that requirelip adhesions will have definitive lip repair at 5-6 months of

age

Definitive cleft lip repair is performed near 3 months of age. Many surgeonsfollow the rule of tens when deciding the proper timing for lip repair. The ruleof tens states that a patient must be at least 10 weeks old, weigh at least 10lbs,and have a hemoglobin of 10 or higher. Definitive lip repair must be delayed to5-6 months of age if lip adhesion is performed to allow for proper healing andscar maturity.


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Cleft Lip Repair

Milliard rotation-advancement technique Introduced in 1957

Most widely used procedure for unilateral cleft lip repair

46% of North American surgeons, 38% modified versions

Advantages

Places scar along philtral borders

Allows complete muscular repair and primary cleft rhinoplasty

Minimizes wasting of normal tissue

Disadvantage

Extensive undermining

The milliard rotation-advacnement technique was first introduced in 1957. It is the most widely used procedure for cleft lip repair.In North America, 46% of physicians report using the milliard technique and 38% report using a modified version of the technique.The advantages of this technique include placing of the scar along the philtral columns, allows complete muscular repair andprimary cleft rhinoplasty, and minimizes wasting of normal tissue. Disadvantages include the requirement for extensiveundermining.


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Milliard Rotation-Advancement

9 landmarks are marked using bluedye

Rotation flaps cut first(medially),followed by advancementflaps(laterally)

Downward and lateral rotation ofmedial segment and medialadvancement of the lateral segment

Closed in 3 layers

9 landmarks are marked using blue dye to create thecuts necessary to get proper symmetry with closure.The rotation flaps are first cut on the medial edge ofthe the cleft followed by the advancement cuts onthe lateral portion of the cleft. Downward and lateralrotation of the medial segment and medialadvancement of the lateral segment into the cleftallows for closure. The closure consist of 3 layers:mucosal, muscular, and cutaneous.


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Goals

Tensionless closure

Reapproximation of orbicularis oris

Formation of Cupids bow

Creation of a philtrum

Repositioning of nasal ala

Complications

Hypertrophic scarring

Aggressive massage

Nostril stenosis on cleft side

Milliard Rotation-Advancement

The ultimate goals of Milliard technique consist of tensionlessclosure, formation of the cupids bow, creation of the phitrum,and repositioning of the nasal ala to a more symmetrical position.There are not many complications but the most common ishypertrophic scarring and scar contracture which can be partiallyprevented with early aggressive massage. Nostril stenosis canoccur when repairing wide clefts.


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Bilateral Cleft Lip Repair

Technically challenging Goals

Symmetry

Orbicularis oris closure Proper philtral size and shape

Tubercle formation

Positioning of alar cartilages to construct the nasal tipand columella

Bilateral cleft lip repair is a challenging and technical procedure. There are many surgical techniques that are currentlyused for repair of bilateral cleft lips, but there are common goals for the different techniques which include symmetry,orbicularis oris closure, philtrum formation, formation of the median tubercle, and positioning of the alar cartilages toconstruct the nasal tip and columella.


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Cleft Palate Repair

Primary goals Separate the nasal cavity from the oral cavity

Creation of velopharyngeal valve for swallowing and speech

Preservation of midface growth

Timing

Controversial

Speech outcomes improved with early closure

Midface growth maybe hindered by early closure (2 stage palaterepair)

Most repaired between 8-12 months of age to minimize

speech abnormalities

The primary goals of cleft palate repair is to restore function by separating the nasal and oralcavity, creating a velopharyngeal valve for swalllowiing and speech, and preservation of midfacegrowth. The timing of closure has been somewhat controversial. Speech outcomes are betterwith early closure but midfacial growth may be hindered. Some surgeons have advocated for twostage palate closure with soft palate early and hard palate late. This eliminate the issues withfacial growth at the expense of speech abnormalities which can be difficult to correct later in life.Most surgeons repair cleft palate between 8-12 months of age to minimize speech abnormalities.


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Cleft Palate Repair

Surgical techniques

Bardach two flap palatoplasty

Furlow double opposing z-plasty

The badarch two flap palatoplasty and the furlow palatoplasty or some modifiedversion of the two are the most common surgical techniques used in the US forrepair of cleft palate


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Bardach Palatoplasty

Margins of the cleft are cut separatingthe nasal and oral mucosa

Lateral incisions are made to the boneand carried posteriorly

Mucoperiosteal flaps are elevated Care must be taken to avoid damage to

the neurovascular bundle

The abnormal muscular attachmentsare raised from the hard palate

Margins of the cleft are cut to separate the nasal and oral mucosa.Lateral incisions are made to the bone, medial to the alveolus andcarried posteriorly to the retromolar area. Mucoperiostal flaps areelevated bilaterally and the neuromuscluar bundle is isolated. Caremust be taken to avoid damage to the neurovascular bundle arisingfrom the greater palatine foramen. Damage to this vessel wouldcompromise blood supply to the flap. The abnormal velarattachments are raised from the hard palate.


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Bardach Palatoplasty

Closure occurs in 3 layers

Nasal mucosal

Muscular layer

Oral Mucosal

Closure occurs in 3 layers. Closure of the nasal

mucoperisoteum proceeds from anterior toposterior. The velar musculature isreapproximated, and the oral mucoperiostium isclosed from posterior to anterior along the cleftmargin. The lateral oral mucoperiosteum is thenreapproximated to the medial alveolus.


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Bardach Palatoplasty Keys to optimizing outcomes

Aggressive repositioning of the soft palate muscles

Adequate mobilization of flaps to create a tensionless closure

Complete palatal closure in one setting

Complications

Fistula 3.9 29%

Tensionless closure and careful tissue handling

Postoperative arm splints

Primary sites of fistula formation are the anterior hard palate and the soft/hard palate junction

Maxillary growth disturbance

VPI

Keys to optimizing surgical outcomes with two flap palatoplasty include aggressive repositioning of the soft palate muscles, adequatemobilization to create a tensionless closure, and complete palatal closure in one setting. Complications include oronasal fistulas whichhave been reported to have an incidence anywhere from 3.4% to 29%. It is important to make a tensionless closure and handle thetissue with care to help prevent fistula formation. The use of postoperative arm splints decrease the risk for wound dehiscence bypatient manipulation but they are controversial. Other complications include maxillary growth disturbance and VPI


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Furlow Double Opposing Z-plasty

Closure of soft palate and reconstruction of levator sling

Advantages

Repositions musculature into a more anatomical location

Lengthens the palate with potential for improved speech outcomes(controversial)

Disadvantages

Postoperative airway obstruction

Fistula formation wide clefts

Technically challenging

Furlow double opposing z-plasty is a surgical technique for closure of the soft palate and recontruction of the levator sling. It has theadvantage of repositioning the musculature into a more anatomical location, lengthens the palate, and improves speech outcomes.The increased length of the the palate is an advantage and disadvantage because it can result in postop airway obstruction. As withother types of palatoplasty fistulas can form especially in the presence of wide clefts. The last disadvantage is that the surgicalprocedure is technically challenging.


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Furlow Double Opposing Z-plasty Oral mucosa with muscle is raised on the

left from the nasal mucosa and elevatedto the posterior

Oral mucosal flap is raised on the right(oral mucosa and submucosa is raisedfrom the muscle) and elevated anteriorly

Nasal mucosa/muscle flap is raised onthe right and elevated posteriorly

Nasal mucosal flap is raised on the leftand raised anteriorly

4 flaps (2 anterior mucosal flaps and 2posterior mucomuscular flaps)

The first flap is from the left which consist of oral mucosa and muscle, and is raised posteriorly. Then an oral mucosa andsubmucosal flap is raised on the right and elevated anteriorly. Then a nasal mucosa and muscle flap is raised on the rightand elevated posteriorly followed by a nasal mucosa flap on the the left which is raised anteriorly. This creates 4 flaps withtwo anterior mucosal flaps and two posteriorly situated mucomusclar flaps.


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Lower flaps with nasal mucosa are transposed Upper flaps with oral mucosa are transposed

Modified for clefts involving the hard palate

Furlow Double Opposing Z-plasty

The lower flaps with nasal mucosa are transposed and sutured together, followed by the upper

flaps with oral mucosa. This recreates the muscular sling and lengthens the palate. This

surgical technique can be modified for clefts involving the hard palate. It combines two flap

palatoplasty with z-plasty.


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Conclusion

Common head and neck congenital

malformations

Multidisciplinary approach Medical and surgical

Otolaryngologist

Cleft lip and palate are common head and neck congenital malformations thatrequire a multidisciplinary approach to management of the many medical andsurgical needs of the patient. Otolaryngologist play a pivotal role in cleft care teamand are capable of managing many of the problems these patients face.


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3. Dixon, Michael J., et al. "Cleft lip and palate: understanding genetic and environmental influences." Nature Reviews Genetics12.3

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neck surgeryotolaryngology, 4th edn. Lippincott Williams & Wilkins, Philadelphia(2006): 1317-1335.

5. Grayson, Barry H., and Deirdre Maull. "Nasoalveolar Molding for Infants Born with Clefts of the Lip, Alveolus, and Palate." Seminars inPlastic Surgery. Vol. 19. No. 4. Thieme Medical Publishers, 2005.

6. Liau, James Y., A. Michael Sadove, and John A. van Aalst. "An evidence-based approach to cleft palate repair." Plastic and

reconstructive surgery126.6 (2010): 2216-2221.

7. Marazita, Mary L. "The evolution of human genetic studies of cleft lip and cleft palate."Annual Review of Genomics and Human

Genetics13 (2012): 263-283.

8. Moore, Keith L., and A. M. R. Agur. Essential Clinical Anatomy. Philadelpia, PA: Lippincott Williams & Wilkins, US, 2006. Print.

9. Robin, Nathaniel H., et al. "The multidisciplinary evaluation and management of cleft lip and palate." Southern medical journal99.10

(2006): 1111-1120.

10. Szabo, C., et al. "Treatment of persistent middle ear effusion in cleft palate patients." International journal of pediatric

otorhinolaryngology74.8 (2010): 874-877.

11. van Aalst, John A., Kamal Kumar Kolappa, and Michael Sadove. "MOC-PS (SM) CME Article: Nonsyndromic Cleft Palate." Plastic and

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References

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