q1879 cleft lip ploutz - cdn.ymaws.com · •babies born with cleft lip and/or palate benefit from...

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Caring for Cleft Lip and Palate:Optimizing Outcomes from Birth to Maturity

Presented by: Melisande J Ploutz, CPNP-PC

Learning Objectives

•List causes of cleft lip and palate

• Identify feeding challenges and specialized cleft palate bottles

•State timeline for surgery and other key interventions

•Describe interdisciplinary team care

•Explain roles of different specialists on the Cleft and Craniofacial Team

What is a cleft lip and/or cleft palate?

•Among the most common of all birth defects

• 1 in 700 NBs in U.S.

•Occurs early in fetal development

• Isolated cleft lip

• Isolated cleft palate

•Unilateral cleft lip and palate

•Bilateral cleft lip and palate3

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Causes•Spontaneous (60%)

•Genetics

•Syndromes

• Van der Woude syndrome

• 22q11.2 deletion syndrome

• Stickler syndrome

• Pierre Robin sequence

•Environment

• Anti-seizure medication (Dilantin)

• Smoking

• Alcohol 4

Diagnosis

•5-6 weeks gestation: lip

•8-12 weeks gestation: palate

•Cleft lip can be detected on anatomy ultrasound

•Can occur as part of a syndrome• Amniocentesis• Genetic counseling

•10-15% with CL and/or CLP have related syndrome

•40-50% with CP alone have related syndrome

Goals of treatment

•Repair the birth defect

•Achieve normal speech, language, and hearing

•Achieve functional dental occlusion and good dental health

•Optimize psychosocial and developmental outcomes

•Minimize costs of treatment

•Facilitate ethically sound, family-centered, culturally sensitive care

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How Do We Achieve These Goals?

1. Early assessment and intervention

2. Interdisciplinary cleft lip/palate team

3. Providers with training and expertise

4. Continuity of care

5. Proper timing of interventions

6. Coordination of care

7. Better early management

It Takes A Team!

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Cleft and Craniofacial Team

• Team Coordinator• Surgeons (Plastics / Craniofacial, ENT, OMFS)• Pediatrician• Sleep Medicine Specialist• Speech Language Pathologist• Audiologist• Psychologist• Social Worker• Pediatric Dentist• Orthodontist• Genetic Counselor

Age Range Intervention

Prenatal Refer to cleft lip/palate team; medical diagnosis and genetic counseling; address psychosocial issues; provide feeding instructions; make feeding plan

Birth-1 month Monitor feeding and growth; begin NAM if indicated

4-6 months Repair cleft lip; monitor ears and hearing

10-12 months Repair cleft palate; +/- tympanostomy tubes; instruct parents in oral hygiene

16-24 months Assess ears and hearing; assess speech-language

2-5 years Manage VPI; consider lip/nose revision before school; assess development and psychosocial adjustment

6-11 years Orthodontics; alveolar bone graft; assess school adjustment

12-21 years Jaw surgery, rhinoplasty (as needed); orthodontics-bridges, implants as needed; genetic counseling

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Prenatal Diagnosis

Counseling• Unknown severity• Unknown number of interventions• Establish trust and availability• Reassurance of institutional ability and outcome

Prenatal Diagnosis

FAQ’s• Will I need to deliver at a certain hospital?• Will my baby be able to eat?• How many DAYS old will they be when the

first surgery is done?• My OB said it’s “just 2 or 3 stitches”, right?• Is this cosmetic surgery?










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Birth and Newborn Period

•A cleft lip and/or palate does not make mom’s pregnancy or delivery high risk.

•Babies born with cleft lip and/or palate benefit from remaining with their parents on the birth center and being discharged home as a unit.

•These babies do not routinely need to be assessed by the neonatal team or admitted to the NICU.

•Babies born with cleft lip and/or palate benefit from the support of Lactation, Occupational Therapy, and the Craniofacial Team.

•Feeding tubes and IV fluids are not usually necessary for full-term babies with cleft lip

and/or palate.

•The cleft does not make these babies prone to aspirating during feeds.

•Babies do not need a feeding evaluation before RNs/parents can feed them.

Birth and Newborn Period

Newborn Feeding

•Newborns with cleft lip only should not have difficulty with breast or bottle feeding.

• “Incomplete” cleft lip

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•Newborns with cleft lip and palate, or cleft palate only, have an opening in the roof of their mouths.

•This prevents them from making adequate suction to pull milk from the nipple.

•These babies will have normal sucking and swallowing reflexes, but because of the cleft palate, they will need a special bottle to allow

the milk to flow without the help of suction.

Newborn Feeding

•Dr. Brown Specialty Feeding System–• For newborns with cleft palate or cleft lip and palate• Infant-Paced Feeding Valve• Valve creates a “compression” nipple

Newborn Feeding

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Other Cleft Palate Bottles

•Mead Johnson Cleft Palate Nurser

•Medela Special Needs Feeder – “Haberman”

Feeding Strategies

•Keep feedings to 30 minutes or less

•Upright positioning

•Frequent burping

•Nasal regurgitation is normal and expected. It is not dangerous and does not mean the baby is choking.

•Back to birth weight by 2 weeks old

What About Breastfeeding?

•Support and educate parents on the benefits of breastmilk, pumping, and breast experiences.

•Encourage pumping: double hospital grade electrical pump

•Once the baby has become successful feeding with the Dr. Brown Specialty bottle, he/she may be put to breast for non-nutritive sucking.

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What Happens After Discharge?

•Continued / frequent monitoring of growth and weight gain with PCP

•Appointment with Cleft Team within a week

•Home nursing visits not usually necessary

Birth-1 Month: Begin Nasal Alveolar Molding

•Orthodontics for babies!

•Non-surgical, passive method

•Starts in first 2 weeks of baby’s life, can last up to 6 months

•Optimizes surgical result

Pre-NAM

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Pre-NAM

Pre-NAM

Progress NAM: 1 month later

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Progress NAM: 1 month later

Nasal Stent Addition

Progress NAM: 2 months later

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NAM Final: 4 months later

NAM Final: 4 months later

Before NAM After NAM

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Primary Cleft Lip-Nasal Repair

•FAQ’s• Why can’t you just go ahead and fix the palate?• Will my baby be able to eat after surgery?• Will he/she still look like my baby?

• Average age for repair is 4 months

• General anesthetic

• 2-3 hour surgery

• Overnight stay

Post-op

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Post-op

Post-op Considerations

•Feeding

•Activity

•Pain

• Incision care

•Follow-up

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•Hard palate• Close oronasal fistula

• Soft palate• Close oronasal fistula• Muscular reconstruction• Lengthen palate• Uvular reconstruction• Good speech, swallow

Cleft Palate Repair: Goals of Surgery

Cleft Palate Repair

• Average age for repair is 10-12 months

(before meaningful speech develops)

• General anesthetic

• 2-3 hour surgery

• Overnight stay

Cleft Palate Repair

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Cleft Palate Repair

Cleft Palate Repair

Post-op Considerations

•Feeding

•Activity

•Pain

• Incision care

•Follow-up

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16-24 Months: Speech and Hearing

•Chronic middle ear effusions and infections can lead to hearing loss (conductive)

•Adversely influences speech and language, with consequences for cognitive development and psychological adjustment

•Monitor middle ear status and hearing every 6 months

•Replace ear tubes as needed

•Speech-language evaluation

•Early Intervention/Head Start










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2-5 Years

•Manage VPI (Velopharyngeal Insufficiency)• Structural problem that occurs when the throat and roof of the

mouth do not work right during speech• 10-20% of children with cleft palate will have VPI• Air escapes through their nose during speech causing

“hypernasality”• Can usually be eliminated with surgical or prosthetic treatment to

close the site of air leakage

•Work-up involves coordination between Speech-Language Pathologist, Surgeons, and Sleep Medicine doctor










6-11 Years

•Orthodontics

•Alveolar bone graft surgery

•Monitor school performance and psychological adjustment

• Involve child in medical decision-making process

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Alveolar Bone Graft

•Bony cleft in the maxilla

•When teeth erupt into this cleft, they are unsupported by bone and will be lost

•Bone grafting to the cleft is essential• Provides bony base for erupting adult dentition• Constructs floor of nose• Provides support for nasal alar base

•Cancellous bone is best- usually taken from child’s own iliac crest

•Timing is critical! Involves coordination with orthodontist and dentist

Alveolar Bone Graft

Alveolar Bone Graft

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Before and After: Alveolar Bone Graft

Before and After: Orthodontics










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12-21 Years

•Orthognathic jaw surgery

•Rhinoplasty if needed

•Final orthodontics / tooth implant

•Genetic counseling

•Assess overall psychological adjustment

•Review school issues/future plans

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Final Thoughts

• Interdisciplinary team care is imperative

•Every child is different

•Always involve the child in decision making

•Advocate, advocate, advocate!

• It takes a TEAM!!!

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References• Goodacre, T. & Swan, M. C. (2012). Cleft lip and palate: current management. Pediatrics and Child Health, 22(4), 160-168.

• Ha, S., Koh, K. S., Moon, H., Jung, S., & Oh, T. S. (2015). Clinical outcomes of primary palatal surgery in children with nonsyndromic cleft palate with and without lip. BioMed Research International, doi: 10.1155/2015/185459

• Miller, C. K. (2011). Feeding issues and interventions in infants and children with clefts and craniofacial syndromes. Seminars in Speech and Language, 32(2), 115-127.

• Mossey, P. A., Little, J., Minger, R. G., Dixon, M. J., & Shaw, W. C. (2009). Cleft lip and palate. The Lancet, 374, 1773-1785.

• The Center for Children with Special Needs, Seattle Children’s Hospital. (2010). Cleft lip and palate: Critical elements of care. https://cshcn.org/pdf/critical-elements-of-care-cleft-lip-and-palate.pdf

• Tibesar, R. J., Black, A., & Sidman, J. D. (2009). Surgical repair of cleft lip and cleft palate. Operative Techniques in Otolaryngology, 20(4), 245-255.

q1879 cleft lip ploutz - cdn.ymaws.com · •babies born with cleft lip and/or palate benefit from...

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