CHAPTER 28: MANAGEMENT OF PX WITH OROFACIAL CLEFT

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cleft

colloquial term is ”harelip”

occurs in 1 in 700 births

a congenital abnormal space or gap in the upper lip, alveolus or palate

the most common serious congenital anomalies to affect the orofacial region

• cleft lip is more common in boys

• cleft palate is more common in girls

• ¾ of the clefts are unilateral, ¼ bilateral

4 weeks after conception

development of the face:

• frontonasal process

• paired mx-mn processes

5th-6th weeks

maxillary processes and the medial nasal process merge to form the upper lip, alveolus, primary palate

lateral nasal process → ala of the nose

mandibular processes → lower lip and jaw

6th-8th weeks

palatal shelves fuse to form the secondary palate

primary palate

structures anterior to the incisive foramen (lip and alveolus)

secondary palate

structures posterior to the incisive foramen (hard and soft palates)

maxillary processes and medial nasal process

give rise to: upper lip, alveolus, primary palate

lateral nasal process

give rise to: ala of the nose

mandibular processes

give rise to: lower lip and jaw

palatal shelves

give rise to: secondary palate

cleft lip

aka: cheiloschisis

4-6 weeks in utero

incomplete fusion of maxillary process and medial nasal process

cleft palate

6-8 weeks in utero

palatal shelves

if soft palate = staphyloschisis

if hard palate = uranoschisis

class 1 cleft palate

cleft of the soft palate

class 2 cleft palate

cleft of the hard and soft palate

Class 3 cleft palate

complete unilateral cleft lip and palate

class 4 cleft palate

complete bilateral cleft lip and palate

causative factors of cleft

multifactorial; exact cause is UNKNOWN

nonsyndromic

• isolated or no other health problem

• genetic predisposition

syndromic

• due to birth disorders or syndromes, such as:

• van der woude, treacher-collins, stickler’s

• pierre-robin’s syndrome, down syndrome

environmental factors

nutritional deficiencies vitamin excess or deficiencies

radiation, drugs, hypoxia, viruses

van der woude syndrome

around 3% of patients with syndromic cleft lip and palate

problems with IRF6 gene (interferon regulating factor 6)

a genetic autosomal dominant condition that affects embryologic facial development

pathognomonic:

• bilateral pitting

• paramedial pits of the lower lip

treacher collins syndrome

aka: mandibulofacial dysostosis

an autosomal dominant

clinical findings:

• malformed middle ear ossicles

• eye abnormalities (coloboma of the lower eyelids)

• hypoplasia of the mandible and zygomatic complex

pierre robin syndrome

congenital birth defects that occur during fetal developments

clinical findings:

• airway obstruction

• glossoptosis → backward positioned tongue

• micrognathia → underdevelopment of lower jaw

causes:

• not fully understood

• isolated genetic mutation

• syndrome (stickler syndrome)

stickler syndrome

a genetic condition affecting connective tissues leading to problems with vision, hearing and movement

facial abnormalities like a cleft palate

clinical findings:

• abnormal functioning & physiology of the eyes

• recessed small lower jaw, flat face, small nose

common symptoms:

• bone and joint

• ear and hearing problems

• eye and sight problems

down syndrome

aka: trisomy 21

more of periodontal problems than dental caries

clinical findings:

• low-set small folded ears

• upslanting palpebral fissures

• brushfield spots, epicathal fold

• short neck, flattened nasal bridge

problems of individuals with clefts

nasal deformity esp if cleft extends to the nasal floor

associated anomalies 20x to have another congenital anomalies

speech difficulties retardation of consonant sounds (P,B,T,D,K,G)

ear problems predisposition to middle ear infections (otitis media)

pseudoprognathism → malocclusion class III MO (retrusion of the maxilla not protrusion of the mandible)

congenital absence of teeth (lateral and canine) or supernumerary teeth

feeding underdeveloped musculature → no negative pressure to allow sucking breast milk or bottle milk

types of cleft

cleft lip

alveolar cleft

submucosal cleft

cleft of the soft palate

cleft of the hard palate

cheilorrhaphy

surgical correction of the cleft lip

usually the earliest surgical procedure for cleft deformities.

• derived from:

  • cheilo = lip

  • rhaphy = junction by a seam or suture

• considers “rule of 10”

  • 10 weeks old

  • 10 lb body weight

  • 10 g/dL hemoglobin

  • 10k WBC

• benefits:

  • restoring the orbicularis oris muscle:

    • improves muscular function

    • favorable molding effect on the developing alveolar segments

• limitations:

  • ideal results are difficult to achieve despite surgical skill.

  • main problems include:

    • poor quality of tissues at the cleft margins

    • distortion of normal structures before surgery

• surgical technique

  • unilateral cleft

    • the unaffected side serves as a guide for: lip length, symmetry

  • scar design

    • the scar lines should be broken up rather than forming one straight line, why?

      • fibrosis and scar contracture can distort the lip

      • linear closure may result in characteristic notching of the upper lip

techniques for cheilorrhaphy

tennison operation

wynn operation

LeMesurier technique for incomplete unilateral cleft

millard operation (rotation advancement technique) → most common technique

2 main objectives of cheilorrhaphy

• functional objective

  • reestablish normal upper lip function

  • restore the normal arrangement and continuity of the orbicularis oris musculature

    • if muscle continuity is not restored

      • an aesthetically undesirable depression can appear when the lip is functioning.

• aesthetic objective

  • create a lip with normal anatomic features, including:

    • vermilion tubercle

    • cupid's bow

    • philtrum

    • the repaired lip should be:

      • symmetric, soft, supple

      • well contoured

      • have inconspicuous scars

  • nasal deformity

    • another aesthetic goal is to partially correct the associated nasal deformity

palatorrhaphy

surgical repair of the cleft palate, usually completed in one operation.

• sometimes performed in 2 operations:

  1. soft palate closure → staphylorrhaphy (8-18 months of age)

  2. hard palate closure → uranorrhaphy (4-5 y/o)

• objectives:

  • create a palate that allows:

    • normal speech, deglutition

    • minimal interference with future maxillary growth

• 2 important requirements

  • competent velopharyngeal mechanism

  • complete separation of the nasal and oral cavities

• surgical technique:

  • techniques vary greatly because each cleft is unique.

  • clefts differ in:

    • width, completeness, palatal length

    • amount of hard and soft tissue available

  • therefore, the technique may differ:

    • between surgeons, from one patient to another

techniques for palatorrhaphy

vomer flap technique

von langenbeck operation

von langenbeck operation

this technique is a one-layer closure

for closure of the hard palate using lateral releasing incision

nasal aspect of palatal flaps will epithelialize, as will denuded areas of the palatal bone

vomer flap technique

a one-layer closure

• indication:

  • the vomer is long

  • attached to the palatal shelf opposite the cleft

  • cleft is not too wide

• technique:

  • raise a mucosal flap from the vomer.

  • suture it to the palatal tissues on the cleft side.

• advantages:

  • requires little stripping of palatal mucoperiosteum

  • produces minimal scar contraction

  • therefore, less potential restriction of maxillary growth

• healing:

  • denuded areas of the vomer and flap reepithelialize.

advantages of early closure

easier feeding

better auditory tube function

better development of phonation/speech skills

better palatal and pharyngeal muscle development

improved psychological state of the parents and baby

better oral hygiene because the oral and nasal cavities are separated

2 major disadvantages of early closure

surgical scarring may cause restriction of maxillary growth.

surgery is more difficult in younger children because the structures are smaller.

advantages of delaying hard palate closure

allows more maxillary growth

reduces the effect of surgical scarring on growth

makes the use of orthodontic appliances easier

soft palate

usually closed between 8–18 months

functions as an important valve to control the distribution of escaping air between the oropharynx and nasopharynx

a muscular "valve" that separates the oral and nasal cavities during speech and swallowing

hard palate

may be left open as long as possible to allow unrestricted maxillary growth

may not be closed at the same time as the soft palate, esp when the cleft is wide

removable palatal obturators → may be used during the waiting period (it separates the oral cavity and nasal cavity)

• how long can it be delayed?

→ closure may be postponed until at least all deciduous teeth have erupted

→ since a significant amount of maxillary growth has already occurred by 4–5 years, hard palate closure is usually performed around this time (completed before the child enters school)

compensatory velopharyngeal mechanism

passavant's ridge a temporary bulge on the posterior pharyngeal wall that forms during speech and swallowing

pharyngeal walls narrow the passageway between the oropharynx and the nasopharynx

tongue postures to help valve the air

superficial muscles around the nose limit the amount of air escaping from the nasal cavity

submucosal cleft

aka: occult cleft

a defect due to lack of continuity in the musculature of the soft palate

not readily seen on clinical exam since oronasal mucosa covers the muscular defect

how to diagnose?

• let the patient say “ah”

• this action lifts the soft palate → a furrow in the midline is seen

• hypernasal speech without obvious soft palate cleft

alveolar cleft

a cleft of the alveolus

usually not repaired during the initial cleft lip or palate surgery

residual oronasal fistulas may also remain; maxillary alveolus remains discontinuous.

• problems caused if unrepaired:

  • oral fluids can enter the nasal cavity.

  • nasal secretions can enter the oral cavity.

  • teeth may erupt into the cleft.

  • alveolar segments may collapse.

  • a large cleft may negatively affect speech.

• benefits if repaired:

  • unites the alveolar segments

    • helps prevent collapse, constriction of the dental arch

  • provides bone support for teeth

  • closes oronasal fistula

    • separates: oral cavity, nasal cavity

    • prevents fluid from passing between them.

  • augments the alveolar ridge

    • provides a better supporting base for dental prostheses.

  • supports the lip and nasal alar base

    • creates a solid foundation for: lip, alar base of the nose

  • improves nasal structure because the tissues receive osseous support.

timing of alveolar bone grafting

• performed between 6-10 y.o, why?

  • much of maxillary growth has already occurred

  • the graft is less likely to interfere with future maxillary growth.

  • ideally, the graft should be in place before permanent canine eruption.

• ideal timing relative to canine

  • when ½ -⅔ of the unerupted canine root has formed.

  • some surgeons prefer grafting closer to the time when the maxillary central incisors are erupting.

[alveolar bone grafting should be performed before nasal revisions]

surgical procedure for alveolar cleft graft

• soft tissue coverage

  • bone grafts must be covered by intact mucoperiosteal flaps

  • flaps are developed from:

    • nasal, palatal, labial mucosa

• closure

  • sutured → prevents infection of the bone graft.

    • tension-free, watertight

bone graft material

ilium, cranium

• other materials:

  • allogeneic bone → bone from another individual

  • bone morphogenetic proteins

• graft preparation

  • graft is made into a particulate form:

  • packed into the defect after:

    • nasal / palatal mucosa closure

  • labial mucosa is then closed over the graft

orthodontic expansion

• maxillary arch expansion can be done:

  • before / after grafting

• both can be effective.

[some surgeons prefer expansion before bone grafting because it provides better surgical access to the cleft]

palatal obturator

latin word 'obturare' which means to close or shut off

a prosthesis that can be used to close defects such as an opening in the roof of the mouth

staphylorrhaphy

soft palate closure

considered the most technically difficult procedure

always closed in 3 layers and in this order: nasal, muscular, oral

• why is it difficult?

  • it is located deep in the back of the mouth.

  • problems include:

    • limited access

    • difficulty with lighting & retraction

    • working from the oral side while repairing both oral and nasal surfaces

    • thin or atrophic tissues → closure must remain intact during function and healing

uranorrhaphy (hard palate)

it is closed using soft tissue only

usually, an osseous partition is not created between the oral and nasal cavities.

• two-layer closure: nasal layer, oral/palatal layer

  • nasal mucosa is mobilized from:

    • nasal floor, septal region

    • lateral nasal wall

  • the nasal mucosa is sutured together before the oral closure

• basic technique

  • incise the soft tissues along the cleft margins.

  • dissect the tissues away from the palatal shelves.

  • bring the tissues together over the cleft.

  • lateral relaxing incisions near the teeth are often necessary

• closure

  • soft tissues are sutured watertight over the cleft.

  • the exposed bone from the lateral relaxing incisions heals by secondary intention.

  • the superior surface of the palatal flaps becomes the nasal floor and develops respiratory epithelium.

common procedures for palatal lengthening

W-Y push-back procedure (Wardill)

U-shaped push-back procedure (Dorrance and Brown)

common skeletal problems of cleft px

maxillary retrusion

transverse maxillary constriction

pharyngeal flap procedure

most commonly used secondary procedure for improving velopharyngeal competence

• a wide vertical strip is raised from the posterior pharyngeal wall of:

  • pharyngeal mucosa, pharyngeal muscle
    

• the flap is inserted into the superior aspect of the soft palate

• result:

  • the flap connects the pharynx and soft palate.

  • this leaves two lateral openings/ports between the:

    • oropharynx, nasopharynx

  • this reduces airflow between the two spaces.