Cleft Lip and Cleft Palate

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Cleft Lip and Cleft Palate

• Pam Kraus, Janelle OConnell, Jessica Daiker
• Amy Barron, Laura Underwood, Jaci Gauna

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Overview

• A cleft lip or palate is an oral-facial birth
  defects in which the tissues of the mouth or lip
  don't form properly during fetal development.
•  
• In the United States, clefts occur in 1 in 700 to
  1,000 births, making it one of the most common
  major birth defects.
•  
• A cleft lip may be just a small notch in the lip.
  It may also be a complete split in the lip that
  goes all the way to the base of the nose.
•  
• A cleft palate can be on one or both sides of the
  roof of the mouth. It may go the full length of
  the palate.
•  
• The incidence of cleft palate alone is one in 500
  live births
• Considering the cleft deformities of all races
  grouped together, 50 are cleft lip and palate,
  30-35 are palate only, and 15-20 are cleft lip
  only.

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• The cleft palate was not recognized as a
  congenital disorder until 1556.
• For centuries, perforations of the palate were
  considered to be secondary to syphilis.
• The first successful closure of a soft palate
  defect was reported in 1764 by LeMonnier, a
  French dentist.
• The first closure of the hard palate was
  performed in 1834.
• Clefts occur more often in kids of Asian, Latino,
  or Native American descent.
• More boys than girls have a cleft lip, while more
  girls have cleft palate without a cleft lip.
• A child may have one or more of these conditions
  at birth.

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Problems that may be present because of a cleft
lip or palate are

• Failure to gain weight
• Feeding problems
• Flow of milk through nasal passages during
  feeding
• Misaligned teeth
• Poor growth
• Recurrent ear infections
• Speech difficulties

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Pathophysiology

• Cleft lip
• Caused by incomplete fusion of the nasomedial or
  intermaxillary process during the 2nd month of
  embryonic development
• Cleft causes structures of mouth and face to
  develop without the normal restraints of
  encircling lip muscles
• May affect external nose, nasal cartilages, nasal
  septum, and alveolar processes also
• Usually just beneath the center of one nostril
• Can occur bilaterally, symmetrically or
  asymmetrically
• More complete the cleft lip, the greater the
  chance that teeth in the line of the cleft will
  be missing or malformed
• Complete cleft entire thickness of the lip
• Incomplete cleft only a portion of the lip is
  involved

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Pathophysiology

• Cleft Palate
• Often associated with cleft lip, but may occur
  without it
• Fissure may affect only the uvula and soft palate
  (secondary palate, formed 9 weeks), or may
  extend forward to the nostril and involve the
  hard palate and the maxillary alveolar ridge
  (primary palate, formed 4-5 weeks )
• Complete involves the primary and secondary
  palate
• Incomplete involves the secondary palate only
• Unilateral on one side the palatal process of
  the maxilla is fused with the nasal septum
• Bilateral not attached to the nasal septum, and
  the septum is visible through the cleft
• Cleft occupies the midline posteriorly and can go
  as far forward as the alveolar process. Clefts
  involving the palate only are usually midline
• Some cases, the vomer and nasal septum are partly
  or completely undeveloped
• When these facial bones are involved, the nasal
  cavity and the oral cavity are open to each other

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Cultural Beliefs Regarding Cleft Lip and Cleft
Palate

• South Africa
• 2006 study participants included 35 cleft
  patients and their families
• Beliefs about the cause
• God
• Witchcraft
• Ancestors punishing the mother
• Fate
• Genetics and family history of the condition
• 2007 study looked at the beliefs and practices
  of religious healers
• Most common belief for both Muslim and Hindu
  healers it was Gods will or because of an
  eclipse
• Both also identified evil spirits, witchcraft and
  genetics as causes
• Hindu group identified karma as being a possible
  cause
• Both groups reported that their patients came to
  traditional healers because they were looking for
  comfort, or to be relieved of guilt because of
  their culture and what their ancestors did
• Beliefs of African Americans in US are similar
  (punishment for something the mother has done,
  evil spirits, or displeasing a god)

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Cultural Beliefs Regarding Cleft Lip and Cleft
Palate

• Nigeria
• 2007 study regarding the beliefs of mothers
  in Nigeria who had a child with a cleft lip or
  palate
• Identified as Hausu/Fulani (Muslim) believed
  cleft was an act of God
• Other beliefs included the mother going outside
  during an eclipse while pregnant or laughing at
  someone with a cleft lip
•  Because of their belief that the cleft is an act
  of God, may feel less shame related to the
  condition and also less likely to have it
  repaired
• Identified as Yoruba (Christian) believed cleft
  was due to evil spirits or punishment from
  ancestors
• Other beliefs included curses, not having enough
  food during pregnancy or hereditary/environment
  factors

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Cultural Beliefs Regarding Cleft Lip and Cleft
Palate

• Asia
• 1990 paper discussing the beliefs of people of
  Asian/Pacific Island descent living in the United
  States
• Some view a cleft as a gift from God, while
  others view it as a curse
• Many beliefs exist about the role different foods
  play in causing or healing physical problems
• Ex/ eating hare while pregnant could cause a
  cleft lip
• Members of some religions, such as Buddhism, may
  view a cleft as part of a person's karma and not
  treat it at all
• Beliefs regarding the cause include spirits,
  negative forces, punishment for the actions of
  ancestors and eating certain foods
• Depending on the belief regarding the cause of
  the cleft, patient may feel shame

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Cultural Beliefs Regarding Cleft Lip and Cleft
Palate

• Latino
• Article focused on Latino people (from Mexico,
  Central and South America, and the Caribbean) who
  are living in the United States
• Eiology of a cleft lip or palate may be accounted
  for by folklore or mythology
• Some beliefs include that cleft is due to natural
  events (eclipse), actions of the parent (drinking
  alcohol or having a STD), or emotional factors
  (stress during pregnancy)
• Witchcraft or evil spirits also believed to be a
  cause
• Health care underutilized due to lack of
  insurance and/or fear of not being a legal
  citizen of the US

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Impact on Neonatal Nursing

• Nurses need to remember
• to be sensitive when dealing with the family of
  a baby with cleft lip and palate
• Try to remember this may be quite a shock and
  they may be grieving the healthy normal baby
  they wanted
• Encourage parents to do cares for the baby as
  you normally would
• Sometimes listening is the best way to show
  empathy, just let them know youre there and
  youre someone they can confide in

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Impact on Neonatal Nursing

• Beyond the cosmetic abnormality, there are other
  possible complications that may be associated
  with cleft lip and cleft palate, including the
  following
• feeding difficultiesFeeding difficulties occur
  more with cleft palate abnormalities. The infant
  may be unable to suck properly because the roof
  of the mouth is not formed completely.
• ear infections and hearing lossEar infections
  are often due to a dysfunction of the tube that
  connects the middle ear and the throat. Recurrent
  infections can then lead to hearing loss.
• speech and language delayDue to the opening of
  the roof of the mouth and the lip, muscle
  function may be decreased, which can lead to a
  delay in speech or abnormal speech. Referral to a
  speech therapist should be discussed with your
  child's physician.
• dental problemsAs a result of the abnormalities,
  teeth may not erupt normally and orthodontic
  treatment is usually required.

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• Infant with a Cleft Lip and Palate using a Pigeon
  system bottle, which encourages active feeding.
  
• The baby still has to suck, but not as hard and
  there is a one way valve that doesnt allow milk
  to go back into the bottle after it has been
  sucked through.
• This helps the child later on with feeding after
  surgery has been complete since the baby has
  already taken an active roll in eating.
• Corpaks are also utilized to provide the baby
  nutrition without burning so many calories.
• Most NICU protocols will not allow the nurse to
  try to feed for more than 20 minutes.
• The remainder of the bottle is then corpaked.
• Breastfeeding can still be accomplished but it is
  recommended to consult a lactation educator
  before attempting.

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Impact on Neonatal Nursing

• There may be many people involved in management
  of a cleft abnormality, because the skills of
  many different areas are needed to help with the
  problems that can occur with cleft abnormalities.
  The following are some of the members of the
  team
• Plastic/craniofacial surgeon- a surgeon with
  specialized training in the diagnosis and
  treatment of skeletal abnormalities of the skull,
  facial bones, and soft tissue will work closely
  with the orthodontists and other specialists to
  coordinate a surgical plan.
• Pediatrician- a physician who will follow the
  child as he/she grows and help coordinate the
  multiple specialists involved.
• Orthodontist - a dentist who evaluates the
  position and alignment of the child's teeth and
  coordinates a treatment plan with the surgeon and
  other specialists.
• Pediatric Dentist - a dentist who evaluates and
  cares for the child's teeth.
• Speech and language specialist- a professional
  who will perform a comprehensive speech
  evaluation to assess communicative abilities and
  who will closely monitor the child throughout all
  developmental stages.

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• Otolaryngologist (Ear Nose Throat specialist) - a
  physician who will assist in the evaluation and
  management of ear infections and hearing loss
  that may be side effects of the child's cleft
  abnormality.
• Audiologist (hearing specialist)- a professional
  who will assist in the evaluation and management
  of hearing difficulties the child may have.
• Genetic counselor - a professional who reviews
  the medical and family history, as well as
  examines the child to help in diagnosis. A
  genetic counselor also counsels the family
  regarding risk for recurrence in future
  pregnancies.
• Nurse team coordinator - a registered nurse who
  combines experience in pediatric nursing with
  specialization in the care of the child and acts
  as liaison between the family and the cleft team.
• Social Worker- a professional who provides
  guidance and counseling for the child and family
  in dealing with the social and emotional aspects
  of a cleft abnormality and assists the family
  with community resources and referrals (i.e.,
  support groups).

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Signs and Symptoms

• Split in lip or palate
• Symptoms related to location of cleft
• Hard soft palate Facial defect
• Soft palate leaking of ingested liquids from the
  nose
• Hard palate weak, dysfunctional suck, trouble
  swallowing, gagging, choking
• Submucous Cleft- only in muscles of soft palate
  and hidden by lining of mouth
• May not be diagnosed until later in life
• Indicated by trouble feeding, nasal drainage,
  excessive gas, nasal sounding voice, increased
  nasal discharge

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Risk Factors

• Family History Cleft lip more likely to be
  inherited than cleft palate
• Race More common in Native American, Hispanic
  Asian patients
• Sex Males 2x as likely to have cleft lip
  Females 2x as likely to have cleft palate
• Environmental factors exposure of fetus to
  alcohol, cigarette smoke, or drugs
• Maternal Nutrition Deficienciesespecially lack
  of folate
• Encourage use of prenatal vitamins.

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Diagnosis

• Physical examination at birth
• Ultrasound not always visible
• Genetic testing for parents to determine risk of
  having additional children with cleft lip/palate

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Treatment and Interventions

• Treatment of the child with CL is surgical and
  usually involves no long-term interventions other
  than possible scar revision
• However, management of CP involves the care of a
  multidisciplinary health care team to provide
  optimum results
• includes pediatrics, plastic surgery,
  orthodontics, otolaryngology, speech pathology,
  audiology, nursing and social work
• Management of both is directed towards closure of
  the cleft, prevention of complications and
  facilitation of normal growth and development of
  the child.

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Treatment and Interventions

• Surgery
• Can make a big difference to the child's ability
  to grow and develop normally
• The baby will usually have an operation to close
  his or her cleft lip before the age of three
  months old.
• An operation to repair a cleft palate usually
  happens a bit later, but usually before the age
  of one year.
• Under general anesthesia, the surgeon will trim
  the tissues and sew the lip together. The
  stitches will be very small so that the scar is
  as small as possible. Most of the stitches will
  absorb into the tissue as the scar heals, so they
  will not have to be removed later.
• Speech and Hearing
• A tympanostomy tube is often inserted into the
  eardrum to aerate the middle ear
• Speech problems are usually treated by a
  speech-language pathologist
• Encourage the child's early attempts to make
  sounds, even before the cleft is repaired

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Treatment and Interventions

• Prevention
• If you have had a child with a cleft lip or
  palate, the chance of any more children you have
  being affected may be slightly increased
• Genetic counseling or testing may provide answers
• Daily folic acid supplements in the first month
  before conception and in the first two months of
  pregnancy
• Avoid alcohol and drug consumption during
  pregnancy
• Nutrition
• Assess the infant's nutritional intake, adequate
  amount of nutrients is necessary to maintain
  growth
• Feeding is best accomplished in an upright
  position
• Special nipples or other feeding devices may be
  needed
• Breastfeeding is usually a viable option and
  sometimes more successful than bottle-feeding
• Avoid the use of suction or objects in the mouth
  such as thermometers, spoons or straws
• Coping 
• Address parent's emotional needs, answer and
  acknowledge questions and concerns
• Provide support to the child and the family
  members
• Promote attachment/bonding
• Emphasize positive aspects of baby's appearance
• Express optimism

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Implications for Nursing Practice

• The exact cause of cleft lip and palate is
  unknown but it is believed to be a combination of
  genetic (inherited) and environmental factors.
• Certain Drugs, illnesses, and the use of alcohol
  or tobacco while the woman is pregnant are
  thought to be contributing factors.
• restrict alcohol and drug consumption while
  pregnant

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Implications for Nursing Practice

• FEEDING
• Cleft lip makes it more difficult for an infant
  to suck on a nipple
• use special nipples to allow the baby to latch
  properly (either pump or use formula)
• Cleft Palate may cause formula or breast milk to
  be accidently taken up into the nasal cavity
• dont feed baby without palatal obturator
  (prosthetic palate)
• feed in an upright position to keep milk from
  coming out of the nose
• Babies are more susceptible to altered nutrition
  (inadequate) related to difficulty eating due to
  physical defect
• SPEECH
• Muscle function is decreased and cleft lip or
  palate makes it more difficult for the child to
  form words properly
• need to see a speech therapist at 18 24 months

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Mead Johnson/Enfamil Cleft Feeder
Special Needs Feeder / Haberman Feeder

Pigeon Feeder
Dr.
Browns Natural Flow to relieve gas

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References

• Author Unknown. (n.d.) Lucile Packard Childrens
  Hospital at Stanford. Retrieved January 28,
  2010, from http//www.lpch.org/diseaseHealthInfo/
  healthLibrary/craniofacial/cleft.html
• Author Unknown (2009, June). Cleft Lip and
  Palate. Retrieved January 30, 2010, from
  http//hcd2.bupa.co.uk/fact_sheets/html/cleft_lip
  .html
• Bartoshesky, L.E. (2010). Kids Health Cleft Lip
  and Palate. Retrieved January 28, 2010, from
  http//kidshealth.org/parent/medical/ears/cleft_l
  ip_palate.html
• Cute, S. (2009, July). Cultural Considerations in
  Cleft Lip and Palate Improving Relationships
  and Health Care Delivery Between Patients and
  Professionals. Knol, 25. Retrieved February 1,
  2010, from http//knol.google.com/k/cultural-consi
  derations-in-cleft-lip- and-palate
• Hockenberry, M. J. Wilson, D. (2007). Wong's
  Nursing Care of Infants and Children, 8458- 465.
  St Louis Elsevier.
• Huether, S.E. McCane, K.L. (2008).
  Understanding Pathophysiology, 4985-986. St.
  Louis, MO Mosby, Inc.
• Khwab, E.K. (2009, August). After birth cleft lip
  and palate NAM. Retrieved January 28, 2010, from
  http//ekkhwab.com/2009/08/11/feeding-bottles-for-
  clefts/
• Rush, M. (2008, January).Children Born with Cleft
  Lip or Cleft Palate. Retrieved January 28, 2010,
  from http//hubpages.com/hub/Children-born-with-Cl
  eft-Lip-or-Cleft-Palate
• Stewart, M.G. (1991, June). Introduction to Cleft
  Lip and Palate. Retrieved February 1, 2010, from
  http//www.bcm.edu/oto/grand/6191.html
• Tolarova, M.M. (2009, March). Cleft Lip and
  Palate. Retrieved January 23, 2010, from
  http//emedicine.medscape.com/article/995535-over
  view
• Wiki (2009, September). Palatal Obturator.
  Retrieved January 28, 2010, from
  http//en.wikipedia.org/wiki/Palatal_obturator