Download Cleft Lip and Palate

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
Cleft Lip and Palate Formation
• 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
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 20002005
•
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.
Milliard Rotation-Advancement
• 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
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.
Furlow Double Opposing Z-plasty
• Lower flaps with nasal mucosa are transposed
• Upper flaps with oral mucosa are transposed
• Modified for clefts involving the hard palate
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.
References
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