Cleft Lip & Palate Chapter 9

Cleft Lip & Palate Chapter 9

What is a cleft? An opening in an anatomical region that is not normally open

·  Clefting can occur anywhere a structure is formed by fusion of two halves at midline.

·  Cleft lip/palate are congenital defects, which means that they occur in utero

Incidence: 1/750 live births

Embryology – science of the origin and development of the organism from:

Cleft lip/palate occurs very early in development.

·  When infant is four weeks old and about the size of a pea, the facial area begins to form

·  Around week 5, the lip and palatal shelves ______begin to form

·  The halves of the lips fuse at the midline between 5-6 weeks of life ______

·  Between weeks 7-8, the palatal shelves and the nasal septum fuse at the midline to form the secondary palate

·  palatal growth is complete by 9 weeks

Cleft Classification:

See Figure 9.7 + Table 9.1 in your text.

Clefting can occur:

• In the lip and/or palate (either the soft palate, hard palate, or both)

• Either unilaterally (to one side of nasal septum) or bilaterally (on both sides)

o

o

• In the palatal muscles and bone of the hard palate (creates a notch in the bone) while leaving the skin and mucous membranes intact.

o

o

o

Etiologies

·  Many underlying causes can lead to cleft lip/palate

·  CL/P is a symptom and is often a part of larger syndromes. It is associated with over 400 syndromes including Apert’s syndrome, fragile X syndrome, and velocardiofacial syndrome.

Categories of possible etiologies:

1. Chromosomal abnormalities:

•

• Clefting could be caused by

• Could also cause heart and limb defects, mental retardation.

2. Genetic abnormalities:

• This is where the syndromes mentioned earlier come into play.

Two recessive genes from each parent are inherited containing information leading to a syndrome that involves clefting ______

or

one dominant gene is inherited from one parent ______

3. Teratogenic agents:

• Environmentally induced.

• External agents that pass from mother to child via placenta; for example:

• Few teratogens lead to clefting so this is not a frequent cause.

4. Mechanical influences:

·  abnormal jaw development in the child hindering development of the palate,

·  uterine abnormality in the mother during pregnancy, crowding in the uterus

5. Unknown

·  No inheritance pattern; totally random.

Complications That May Occur with Cleft Lip/Palate

·  Abnormal mid-face development – usually severe deficiency of tissue in midline of the face, central cartilaginous structure of nose (columella) is often absent.

·  Speech/Language impairment – will discuss later

·  Feeding Impairment – will discuss later

·  Abnormal Eustachian tube function – children with cleft are particularly susceptible to otitis media due to the shape of their Eustachian tubes (recall that the Eustachian tube ventilates the middle ear and equalizes pressure)

·  Dental Problems – related to misalignment of the teeth and of the mandible.

Multidisciplinary Team Approach

Treatment is ideally provided by a variety of dental, medical, and behavioral specialists. These specialists will all see the patient and jointly decide on a treatment plan.

• Reconstructive surgeon – performs surgical repair of the cleft lip and palate.

• Oral surgeon – performs surgery involving the teeth and jaw.

• Otolaryngologist (ENT) – performs ear, nose, and throat evaluation and treatment.

• Orthodontist – aligns teeth and jaw.

• Prosthodontist - constructs prosthetic devices to replace missing teeth and palate (palatal lift or obdurator for example)

• Pediatric dentist and dental hygienist - ensure appropriate dental and oral health care.

• SLP - assists family with training in feeding techniques at birth and management of dysphagia, provides language, voice, resonance, and articulation therapy, monitors for nasality, may also aid in oral aversion therapy and train parents how to stimulate speech/language development

• Audiologist - identifies hearing loss as early as possible (nobody is too young to have their hearing tested), aids in management/decisions of otitis media and whether or not to place PE tubes

• Social worker/psychologist - provides support and guidance for child and family members, helps with adjustment issues, often mediates between busy physicians and parents, may act as a case coordinator, can aid in obtaining financial resources during all stages of a child’s treatment program

• Geneticist – diagnoses and counsels for genetic disorders and assesses likelihood of a syndrome

• Parents/caregiver – play a crucial role in intervention planning and progression