Cleft Lip and Palate Steven Smith, MD

Mentors: Harold Pine, MD and Eric Cole, MD

The University of Texas Medical Branch (UTMB Health)

Department of Otolaryngology – Head & Neck Surgery

Grand Rounds Presentation

October 30, 2013

Outline

• Introduction

• Prevalence

• Etiology

• Embryology

• Classification

• Management

• Defects and Reconstruction

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 year

with 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.

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, the highest rate occurs among Native American and Asian populations with approximately 2/1000 live births. There is an intermediate rate among those of European descent with approximately 1/1000 live births. The lowest rate of cleft lip and palate occurs among African populations with 1/2500 live births. There is no difference between ethnic groups for cleft palate only, 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 to the fact that the palatine shelves close 1 week later in girls than boys

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 palate throughout human history with many folklore explanations. The aztecs believed eclipses occurred because a bite had been taken out of the moon and exposure to an eclipse during pregnancy could lead to a bite out of the baby’s mouth. To prevent clefts, pregnant women were required to place obsidian knives over there abdomen before going out at night. This belief has now extended to modern day Mexico and during eclipses a metal key or safety pin is placed over the abdomen for protection.

• 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 a “hare lip,” and others believed that bad karma or wrongdoings were the cause. In modern day Filipinos, a common belief is that force to the fetal face when fingers are in the mouth is the cause of clefts. Many other cultures believed that clefts were familial or in the blood. Most of the early thoughts on the causes of cleft have been disproved of course but the belief that there was familial component still stands today.

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

• 3.2% for CL+/- palate

• 6.8% for CP only

• 1 parent affected with 1 child affected

– Risk for future children

• 15.8% for CL+/- palate

• 14.9% for CP only

NOTES: It has long been known that familial inheritance plays a role in cleft formation. Here is some of the data that supports familial inheritance. In 2 unaffected parents with 1 child affected, the risk for future children is 4.4% for cleft lip with or without cleft palate and 2.5% for cleft palate only. If only 1 parent is affected the risk for having a child with cleft lip with or without palate is 3.2% and the risk for CP only is 6.8%. If one parent and one child is affected the risk for future children increases to 15.8% for cleft lip with or without cleft palate and 14.9% for cleft palate only. These exact percentages vary from study to study, but the common theme among studies is that as the number of family members with cleft deformities increase, the risk for future children increase as well.NOTES

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 of nonsyndromic 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 a complex process with several proteins, growth factors, and transcription factors involved. Any disturbance in the developmental sequence can result in cleft formation.

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 or syndromes. 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 dominant and has characteristic lower lip pits with orofacial clefts. Teratogens exposure and environmental factors have also been linked to cleft formation.

Anatomy • Orbicularis oris

• Vermillion (wet/dry border)

• Cupid’s 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 lips and acts as a sphincter for the mouth. The vermillion is the red portion of the lips and important landmark is wet/dry border. Along the upper vermilion-skin border or white roll, two midline elevations form the Cupid’s bow. Two raised vertical columns of tissue extending from the apices of the bow are known as the philtral columns. The area of depression between the columns is known as the philtral dimple. The philtrum extends from the cupid’s bow to the columella which are both important structures when reconstructing the lip. The tubercle which is not labeled is the midline protuberance of the upper lip which is also important in reconstruction.

Normal Anatomy • Hard Palate

– Palatine processes of the maxilla

– Horizontal plates of the palatine bone

– Incisive foramen, greater and lesser palatine foramen

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 the

flaps for 2 flap palatoplasty receive its blood supply from the greater

palatine artery.

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)

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.

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 the palate is mainly from the greater palatine artery which arises from the maxillary artery, but it also receives some supply from the lesser palatine and ascending palatine arteries. The hard palate is innervated by the greater palatine and the nasopalatine nerves. The greater palatine nerve supplies the gingivae, mucous membranes, and glands of most of the hard palate and the nasopalatine nerve supplies the mucosa of the anterior portion of the palate. The soft palate receives sensory innervation from the lesser palatine nerves and receives its motor innervation from 2 cranial nerves. The tensor veli palatini is supplied by cranial nerve V and the 4 other muscles are supplied by cranial nerve X.

Embryology

• Development of the lip and palate

begins around the 4th week of

embryological development

• Completed by the end of the 12th

week

• By the end of the 4th week

– 5 facial prominences have formed

• frontonasal process

• paired maxillary processes

• paired mandibular processes

Embryology

• During the 5th week

– Nasal placodes invaginate to form the nasal pits

• Lateral and medial nasal prominences

Embryology • By the end of the 6th week

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

together

– 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

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 4th week of embryological development and is completed by the end

of the 12th week. By the end of the 4th week, 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 5th week 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 6th week, 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.

Palate Formation

• Palate formation begins at the end of the 5th week of

development and is completed by the 12th week

• The completed palate is formed by the primary palate

and the secondary palate which are separated by the

incisive foramen

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

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 7th week

– The tongue moves inferiorly and the palatal shelves migrate to a horizontal

position above the tongue

• Palatal fusion occurs in an anterior to posterior direction and completes

with uvular fusion (1 week later in females)

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

• 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

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

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 displaced

laterally, inferiorly, and posteriorly

– Nasal tip is deflected towards the noncleft side

• Alveolus may or may not be involved

Bilateral Cleft Lip • Orbicularis oris attaches at the lateral cleft margins

bilaterally at the nasal ala

• Premaxilla protrusion

• Symmetrical nasal deformities

– Laterally displaced ala – widely flared

– Extremely short columella

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 the

muscles into the medial edges

of the cleft

– Vomer attachment is variable

Cleft Lip/Palate Management

• Multidisciplinary approach

– Cleft care team

• Plastic surgery

• Audiology

• Speech pathology

• Otolaryngology

• Orthodontist

• Oral maxillofacial surgery

• Psychologist

• Geneticist

• Pediatrician

Management

• Birth

– Airway concerns

– Feeding problems

• Otological disease

• Speech and language problems

• Surgical Repair

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 severe cases sometimes requiring tracheostomy. Mandibular distraction can also be used for correction.

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 with cleft lip and palate other than airway concerns is nutrition and feeding. Before the patient leaves the hospital feeding techniques must be established. Cleft palate limits the ability to suck due to the common cavity between the mouth and the nose. Cleft lip alone usually does not cause feeding problems. Special bottles have been developed to improve the feeding in patients with cleft palate. Some patients require premaxiilary orthopedics to feed appropriately.

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. The abnormal insertion of the tensor veli palitinini is believed to be the cause or contribute to the development of middle ear disease. 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.

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 in cleft palate patients from Connecticut Children’s Medical Center between 2000-2005. They found that 61 of 74 or 82% of patients passed there newborn hearing screen. 98% of the patients in the study developed middle ear fluid requiring at least 1 set of tubes. Interestingly, 74% of the patients in this study only required 1or 2 set of tubes before resolving the Eustachian tube dysfunction sufficiently the MEE did not reacummulate.

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

prosthesis

Practically, 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 should restore normal speech. Even with surgical closure of the palate 10-20% of patients have been reported to have velopharyngeal insufficiency. These patients will have varying degrees of hypernasality and articulation errors such as glottal stops and pharyngeal fricatives. The VPI following palate surgery is first managed by speech pathology. If speech pathology alone is insufficient, it can be managed surgically with pahryngoplasty or palatoplasty. Dental prosthesis have also been used.

Surgical Correction • Age 1-3 months – Lip taping and

nasoalveolar molding

• Age 3 months - Repair of cleft lip (and placement 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 maturity– Midface advancement and continued orthodontic treatment

Timing of surgical correction varies from institution to institution. Typically, presurgical techniques are used from 1-3 months. Cleft lip is repaired at 3 months of age and myringotomy tubes are placed at the time of repair. The palate is usually repaired from 9-12 months of age. Initial orthodontic treatment begins at 1 year of age. Alveolar bone grafting is performed 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.

Presurgical • Wide cleft lip or premaxilla protrusion

– Advantageous to narrow the cleft and mold the premaxilla 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 the patient’s 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 or premaxilla protrusion is present, it is advantageous to narrow the cleft and/or mold the premaxilla before definitive surgery is performed. There are several techniques that facilitate this correction. Taping has been used effectively to reduce the width of a cleft and reduce premaxilla protrusion in a nonsurgical manner. With taping, strips of hypoallergenic tape is placed with tesnion across the cleft and sceured to the patient’s 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.

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 of orthopedic devices. Nasoalveolar molding devices are custom made orthopedics which utilize wiring and nasal stenting to mold the nasal cartilage, premaxilla, and alveolar ridge. The nasal stenting can be elongated and adjusted to lengthen the columella and mold the the nasal cartilage. This method takes advantage of the malleability of immature 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.

Presurgical • Lip adhesion

– Surgically convert a complete cleft to an incomplete cleft

– Performed at 2-4 weeks with definitive repair at 5-6 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 be considered. Lip adhesion is when a complete cleft is surgically converted to an incomplete cleft. Lip adhesion is performed at 2-4 weeks of age and extends definitive repair to 4-6 months to allow proper healing. It has the same indications as the nonsurgical forms of correction which include wide unilateral clefts or premaxilla protrusion. The disadvantage to performing lip adhesion is the development of scar tissue which could interfere with definitive repair. Most surgeons do not perform lip adhesion because of the possibility of extensive scar tissue formation.

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 require lip adhesions will have definitive lip repair at 5-6 months of age

Definitive cleft lip repair is performed near 3 months of age. Many surgeons follow the “rule of tens” when deciding the proper timing for lip repair. The “rule of 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 to 5-6 months of age if lip adhesion is performed to allow for proper healing and scar maturity.

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 and primary cleft rhinoplasty, and minimizes wasting of normal tissue. Disadvantages include the requirement for extensive undermining.

Milliard Rotation-Advancement

• 9 landmarks are marked using blue dye

• Rotation flaps cut first(medially), followed by advancement flaps(laterally)

• Downward and lateral rotation of medial segment and medial advancement of the lateral segment

• Closed in 3 layers

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

• Goals

– Tensionless closure

– Reapproximation of orbicularis oris

– Formation of Cupid’s 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 tensionless closure, formation of the cupid’s 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 is hypertrophic scarring and scar contracture which can be partially prevented with early aggressive massage. Nostril stenosis can occur when repairing wide clefts.

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 tip and columella

Bilateral cleft lip repair is a challenging and technical procedure. There are many surgical techniques that are currently used 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 to construct the nasal tip and columella.

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 palate

repair)

• 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 oral cavity, creating a velopharyngeal valve for swalllowiing and speech, and preservation of midface growth. The timing of closure has been somewhat controversial. Speech outcomes are better with early closure but midfacial growth may be hindered. Some surgeons have advocated for two stage palate closure with soft palate early and hard palate late. This eliminate the issues with facial 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.

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 modified version of the two are the most common surgical techniques used in the US for repair of cleft palate

Bardach Palatoplasty

• Margins of the cleft are cut separating the nasal and oral mucosa

• Lateral incisions are made to the bone and carried posteriorly

• Mucoperiosteal flaps are elevated

– Care must be taken to avoid damage to the neurovascular bundle

• The abnormal muscular attachments are 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 and carried posteriorly to the retromolar area. Mucoperiostal flaps are elevated bilaterally and the neuromuscluar bundle is isolated. Care must be taken to avoid damage to the neurovascular bundle arising from the greater palatine foramen. Damage to this vessel would compromise blood supply to the flap. The abnormal velar attachments are raised from the hard palate.

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 to posterior. The velar musculature is reapproximated, and the oral mucoperiostium is closed from posterior to anterior along the cleft margin. The lateral oral mucoperiosteum is then reapproximated to the medial alveolus.

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, adequate mobilization to create a tensionless closure, and complete palatal closure in one setting. Complications include oronasal fistulas which have been reported to have an incidence anywhere from 3.4% to 29%. It is important to make a tensionless closure and handle the tissue with care to help prevent fistula formation. The use of postoperative arm splints decrease the risk for wound dehiscence by patient manipulation but they are controversial. Other complications include maxillary growth disturbance and VPI

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 the advantage 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 with other types of palatoplasty fistulas can form especially in the presence of wide clefts. The last disadvantage is that the surgical procedure is technically challenging.

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

left from the nasal mucosa and elevated to the posterior

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

• Nasal mucosa/muscle flap is raised on the right and elevated posteriorly

• Nasal mucosal flap is raised on the left and raised anteriorly

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

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

• 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.

Conclusion

• Common head and neck congenital

malformations

• Multidisciplinary approach

– Medical and surgical

• Otolaryngologist

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

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