comprehensive nursing management of the infant with cleft lip and palate

Patricia Chibbaro, NPPediatric Nurse Practitioner

Institute of Reconstructive Plastic Surgery

NYU Langone Medical Center

Incidence 1:350 - 700 Unilateral/Bilateral Incomplete/Complete +/- Alveolus (primary palate) +/- Hard (secondary) palate +/- Soft (secondary) palate Isolated hard and/or soft palate Submucous cleft palate Pierre Robin Sequence

Mandibular Micrognathia (Mild – Severe) Retropositioning of the Tongue (Glossoptosis) +/- Cleft Palate May Require Intubation,Tracheostomy May Require Gastrostomy

Plastic Surgeon Orthodontist Prosthodontist Pedodontist Otolaryngologist Psychologist Geneticist Primary Care

Provider

Nurse/Practitioner Speech/Language

Pathologist Team Coordinator Social Worker Audiologist

Prenatal Counseling Consult to birth

hospital Feeding instruction Pre/post-op teaching Pre-op H/P, Consent Post-op inpatient

management Resource to pediatric

nursing/housestaff

Cleft Palate Team Member

Patient Resource, Advocate, Case Manager

Community Outreach/Education

Liaison with Community Health Care Providers

• 1981- 1st reported ultrasound cleft detection

• Routine or high resolution sonogram

• Transvaginal: early as week 12

• Week 14 – facial contour almost complete

• Transabdominal: 16-22 weeks (time of 1st routine ob sonogram)

• Ideal detection is at 20-22 weeks

• MRI (usually done to look for other abn)• Incomplete clefts often not seen until 3rd

trimester us• Studies report associated birth incidence: 4.3 -

63.4%• Mild (skin tags)- lethal deformities (trisomy)• 350 reported associated syndromes• Most common – CNS, skeletal, urogenital, CV• Critical to refer couple for prenatal consult!

3-D Sonogram

Complete Unilateral CleftComplete Unilateral Cleft

Obtain pregnancy history, delivery plans and info re: family structure/resources

Clarify info from prenatal meetings w/genetics, surgeon/other cleft team members

Clarify internet information! Review pre/postop photos Discuss/demonstrate feeding

options/provide samples and ordering info

Offer advice re: explaining diagnosis to family, friends, siblings

• Network to other parents• Preparation for NAM therapy• Briefly explain expected

hospital/postop/home care following initial cl/p surgeries

• Refer to Cleft Palate Foundation-Cleftline, Website, Feeding Video

• Provide team literature/website• Encourage parents to

communicate with birth hospital pre-delivery – optimize experience/prevent overtreatment

• Provide cleft team contact info for family/staff to call after birth

Prenatal Consultation Nursing Care During Labor and

Delivery Newborn Nursery/NICU Care Postpartum Nursing Care Pediatric Nursing Care of the Surgical

Patient Pediatric nurses in the community The Cleft Team Nurse Specialist/Nurse

Coordinator/Nurse Practitioner

Goal is to optimize the labor/delivery experience – parents will remember their nursing care

Parents may know that the baby will be born with a cleft

If cleft not prenatally diagnosed, important to support parents immediately/provide information

Be aware of your reaction to the infant Unless infant is premature, has cardiac or

airway problem, allow parents to hold/bond

Try to place infant in newborn nursery unless premature or with cardiac or airway problems

If rooming-in is available and infant is a candidate, it should be offered

If most experienced RN feeders are in the NICU – that would be an indication to place infant there

Do not place infant in the back of the nursery to avoid “the cleft” being seen

If baby is in NICU, do not overtreat (i.e. IV, feeding tube) just on the basis of the cleft

Sensitivity to Parental Response to Baby (especially if cleft not detected prenatally)

Parents will take cues from nurse Feeding – assess their knowledge, provide

cleft bottles, breast pump, lactation consultant, CPF feeding video

Feeding – if cleft palate, unlikely to be able to directly, exclusively nurse – do not pressure them to do so

Referral to a team, especially if cleft not detected prenatally

Discharge planning – feeding supplies, home nursing visit, follow-up appt. with team and primary care provider

The normal process of feeding involves an intact and coordinated sequence

Sucking/swallowing/breathing

Poor Oral Suction Poor Intake Lengthy Feedings Nasal Regurgitation Choking Gagging Excessive Air Intake Poor Weight Gain Excessive Energy Output Stressful Feeding Intake

A cleft makes it difficult for infant to form the seal necessary to create negative intraoral pressure/suction

Cleft Lip – prevents formation of anterior seal on nipple

Cleft Palate – prevents formation of seal within oral cavity needed to create suction

Even small clefts of soft palate/submucous clefts (often missed) can cause feeding problems