8/3/2020

1

Presented by: Tina McDermott, BSN, RN, Boys Town Pediatrics

Cindy Green, RN, Boys Town Pediatrics

The Journey of a Child with a Cleft Lip and/or Palate

•July is designated as National Cleft/Craniofacial Awareness and

Prevention Month

8/3/2020

2

Dear Parent,Congratulations on the birth of your child. Your child wasted no time giving you your first surprise: your child has been born with a cleft lip and/or palate. As you and your family welcome your new bundle of joy into the world, we encourage you to celebrate the things that make your child unique and special, including strengths, weaknesses, beauty and imperfections.

Having a child born with a cleft can seem like a major crisis. Most parents go through pregnancy looking forward to the delivery of the “perfect child.” When this is not the case, it can at first feel like a major loss, bringing with it many emotions: fear, guilt, happiness, sadness, anxiety, uncertainty and hope. These feelings are normal. In fact, you share the feelings of many new parents – of not knowing enough, of doing the wrong thing, of making mistakes. You can’t know everything, and no one expects you to.

Letter to the parents of a cleft child written by the Children’s Healthcare of Atlanta:

•Remember Baby First

•And

• Cleft condition Second

Objectives

• To define cleft lip and cleft palate including types

• To recognize the prevalence of clefting in the population

• Identify the Craniofacial Team members and function within the team

• Describe parental expectations of the Craniofacial Team to include frequency of visits and timing of surgeries

• Discuss post operative care of these patients

8/3/2020

3

Definition and Occurrence

• A cleft is defined as a gap in a body structure that results from incomplete closing of a specific structure during development.

• Clefting occurs in the first 6-12 weeks of gestation

Incidence• 1:650-700 live births (US)

• Highest incidence in Asians and Native Americans (1:500)

• African Americans lowest incidence(1:2000)

• Most common congenital birth defect after club foot

• Isolated defect or part of syndromes (40% cleft palate; 15% cleft lip)

Incidence

• It is estimated that, worldwide, a child is born every 3 minutes with a cleft

• The CDC estimates 7000 babies will be born in the US with a cleft this year

8/3/2020

4

Commonalities• Cleft lip alone (15%)

• Cleft lip and palate (45%)

• Isolated cleft palate (40%)

• Cleft Lip Left > Right 2:1

• Cleft Lip & Palate Males> Females 2:1

• Cleft Palate Females>Males 2:1

Causes

• No single cause

• Genetics

• Having one child with a cleft increases the risk of having other children with clefts

Cleft and/or Palate

• 55% isolated nonsyndromic clefts• Familial clefts – clefts in families

• Multifactorial -- sporadic ~ 80%

• Isolated -- no close family members

• 45% other anomalies

• Heart defects are the most common with cleft palate as a midline defect

• ~25% have associated birth defects/chromosomal abnormality or genetic syndrome

8/3/2020

5

Associated Syndromes

• There are >400 syndromes reported in association with cleft lip and/or palate

• Pierre Robin Sequence

• Van der Woude Syndrome

• Stickler Syndrome

• 22q11.2 Deletion

• Velo-Cardio-Facial Syndrome

• DiGeorge Syndrome

• Shprintzen Syndrome

Nurse’s Role at Time of Delivery

• Be sure to congratulate the family on their new addition

• Reassure parents there is nothing they did to cause the clefting

• This birth defect does not prevent the child from becoming a healthy, productive adult.

8/3/2020

6

• Be sure to examine the mouth closely, as often times a cleft palate goes undiagnosed for several days

• Most people born with a cleft lip and or palate lead productive lives often becoming actors, news casters, football players, politicians, nurses, SLP’s, physicians.

Famous people with Clefting

• Jessie Jackson King Tut

• Peyton Manning Tad Lincoln

• Tom Brokaw Cameron Richard

• Doc Holiday

• Stacy Keach

• King Tut

Cleft Lip

8/3/2020

7

Types of Cleft Lip

• Occult cleft

• Unilateral incomplete cleft

• Unilateral complete cleft

• Bilateral complete cleft

• Bilateral incomplete cleft

Incomplete Bilateral Cleft Lip

Complete Bilateral Cleft Lip

8/3/2020

8

INCOMPLETE CLEFT LIP

UNILATERAL

COMPLETE CLEFT LIP

Cleft Lip Repair

8/3/2020

9

BILATERAL CLEFT LIP

AND PALATE

CLEFT PALATE

8/3/2020

10

Complete Left Cleft Lip and Palate

Cleft Lip Repair Animation

Cleft Palate

8/3/2020

11

Anatomy of the Roof of the Mouth

• Hard Palate

• Soft Palate

8/3/2020

12

Types of Cleft Palate

• Cleft of the soft palate

• Bilateral complete cleft palate

• Unilateral complete cleft lip and palate

• Bilateral complete cleft lip and palate

• Submucous cleft palate

Types of Palatal Clefts

• Submucous

• Soft

• Unilateral

• Bilateral

8/3/2020

13

8/3/2020

14

Cleft Palate Repair

Cleft Palate Repair Animation

Purpose of the Craniofacial Team

• Members are experienced in treating children with clefts

• Road map for the future

• Evaluation and consultation

• Coordination and services

8/3/2020

15

Team Members

• Plastic Surgeon

• Audiology

• ENT

• Geneticist

• Pediatrician

Team Members

• Nurse (feeding)

• Speech/Language Pathologist

• Dentist

• Orthodontist

• Prosthodontist

Team Members

• Team Coordinator

• Oral Maxillofacial Surgeon

• Dietitian

• Social Worker

8/3/2020

16

Goals of Treatment for the Child with Cleft Lip/Cleft Palate

• Repair the birth defect• Lip, palate, nose

• Achieve normal speech, language and hearing

• Achieve functional dental occlusion and good dental health

• Optimize psychosocial and developmental outcomes

• Minimize costs of treatment

Team Visits• Initial visit

• One year of age

• Three – six months after palate repair

• Usually yearly until age five – six

• Age seven – ten either yearly or every other year

• This depends on orthodontics or speech, usually

Surgical Sequence•Lip repair 2-4 mos

•Palate closure 9-12(18) mos

•Pharyngoplasty 4-6 years

•Minor lip/nose revision preK

•Alveolar cleft grafting 7-12 years

•Orthognathic surgery 15-18 years

•Final lip and nose surgery 16-19 years

8/3/2020

17

Nasoalveolar Molding (NAM)• Helps reduce the size if

the cleft

• Uses forces to push segments together

Associated Problems• Feeding

• Ear Problems

• Speech Difficulties

• Dental Problems

• Malocclusion

• Nasal Deformity

Feeding

8/3/2020

18

Feeding Goals

• Maximize oral stimulation to facilitate oral-motor development

• Conserve infant and parent time

• Close to normal feeding as possible

• Conducive to parent-infant bonding

Structures Involved in Feeding• Tongueo In breast feeding the tongue draws out the nipple in

the mouth

o In bottle or breast feeding it seals the oral cavity

o Serves as a compression force to express milk either from the breast or the nipple

o Forms a central groove, the tongue cups the nipple and channels liquid to the pharynx

o Forms a bolus in preparation for swallowing

• Jaw

o Provides stability for coordinated movements of the tongue, lips and cheek

o Moves downward during the sucking to expand the size of the oral cavity, this in turn creates a negative intra-oral pressure

o Jaw movements should be smooth and rhythmical with graded jaw excursions

Structures Involved in Feeding

8/3/2020

19

• Lips and Cheeks

o Stabilize position of nipple within the mouth

o Lips help form a seal around the nipple

o Cheeks provide stability and help maintain the shape of the mouth

o Lip seal is not important for suction; however, it is needed to position the nipple in the mouth

Structures Involved in Feeding

• Palateo Hard palate provides compression surface for the

tongue

o Soft palate along with the tongue seals posterior oral cavity to help create negative intra-oral pressure or suction

o Soft palate elevates during swallowing to occlude nasal cavity in order to prevent nasal-pharyngeal escape (also known as nasal regurgitation)

Structures Involved in Feeding

Anatomy

o Clefts of the hard palate, soft palate, submucousclefts, high arched palates, abnormal palate function affect the degree of suction

o The effectiveness of compression depends on the degree of clefting

8/3/2020

20

Mead Johnson Cleft Palate Nurser

o Helps baby get food in mouth without tiring

o Soft-sided bottle that is squeezed in coordination with infant’s sucking efforts

o Squeeze and release

o Crosscut nipple for faster flow

o Watch facial expressions (eyebrows will often raise if going too fast)

o Cheap ~ $2/bottle

Pigeon

o Special nipple that maximizes amount of milk babe gets from compressions

o One-way valve inside nipple to prevent milk from flowing back into the bottle

o Vertical slit so hole opens when babe compresses the nipple

o Allows the babe to control flow of milk with normal rhythm of sucking, swallowing and prevents choking

o Cost ~ $15-25/bottle with 2 nipples and 2 valves

o Available on Amazon

Dr. Brown Specialty Bottle

• Functions like the Pigeon Bottle

• One way valve inside the nipple to prevent milk from flowing back into the bottle

• Allows babe to control flow of milk with normal rhythm of sucking, swallowing and prevents choking

• Cost ~ $20 for 2 bottles with 4 nipples and 4 valves

8/3/2020

21

Habermano Best for a weak suck

o Has a large squeezable nipple with a slit rather than a crosscut

o One-way valve at its base that keeps the nipple full of milk

o Infant’s effort to compress the soft nipple is often sufficient to dispense the milk into the infant’s mouth

o Feeding can be assisted by squeezing the nipple Markings around the base of the nipple indicate position of slit relative to the infant’s mouth

o These markings allow the feeder to control how quickly the milk flows

o Nipple comes in 2 sizes – regular and mini

o Cost is ~ $30 per bottle

Medicaid and insurance does not

cover the cost of bottles

Successful Feeding

o Feedings should be less than 30 minutes

o Burp often

o Feed infant in an upright position

o Keep bulb suction nearby

o Minimum intake for successful weight gain is 2 oz per pound in 24 hrs

o Recommend infant be taken in for weight checks two – three times a week until adequate weight gain has been established

o Ideally infant to be back to birth weight by two weeks of age

8/3/2020

22

Ear Problems

• Children with a cleft palate often need ear tubes to equalize the pressure in the middle ear

• Often times the babe will pass the Newborn Hearing Screen but by the time babe comes to the team, there is fluid in the middle ear which impacts hearing and ultimately speech

• Not unusual for these children to need several sets of tubes and often times until they are in the primary grades

Velopharyngeal Competence

• The ability of the speech mechanism to create a seal between the nasal and oral cavities during speech

• Soft palate

• Pharyngeal walls

• Associated musculature

8/3/2020

23

Velopharyngeal Insufficiency

• Hypernasal speech secondary to air escape

• Secondary to scarring, short palate length, abnormal muscle function

• Age 5-7

• Speech therapy

Pharyngoplasty

• Pharyngeal flap or sphincter pharyngoplasty procedures

• Creates side ports and blocks excessive airflow

VPI Animation

8/3/2020

24

Post Operative

• Soft diet

• No PE or Sports 1 month

• No speech for 1 month

• Return to clinic in 3-6 months

• Out of school usually 1 week

Alveolar Bone Graft

• Provides support for teeth

• Consolidate the maxillary arch

• Closure of the oronasal communication

• Support for alar base

• Orthodontic expansion to correct crossbite and open the cleft

Palatal Expansion

8/3/2020

25

Repair nasal floor

Incisions

Bone packed into defect

Flaps for oral

mucosal

closure

Bone Graft Animation

8/3/2020

26

Post Surgery

• Soft diet 4 weeks

• Avoid nose blowing

• Oral swabs with peridex; avoid direct brushing for hip graft…

• No swimming or tub bath for 7-10 days

• No strenuous activity/contact sports for 6-8 weeks

• No sports or PE for 1 month

• Soft diet for 1 month

• Avoid biting into food

• Treat your jaw like a china cup

Orthognathic Surgery

• Maxillary retrusion

• Multiple surgeries cause scarring and restricted upper jaw growth

• Very difficult to surgically move the maxilla without distraction

8/3/2020

27

LeFort I Osteotomy

Figueroa and Polley. External Distraction J Oral Maxillofac Surg 2008.

Internal Distraction Devices

• No external frame

• More difficult to control vertical vector

• Requires second surgery

8/3/2020

28

PREOP POSTOP

8/3/2020

29

Basic Steps in Distraction• Osteotomy – LeFort

• Application of device

• Latency period – wait 3 to 4 days

• Application of expansion force – begin activiation 1 mm per day

• Consolidation period – 3 months

• Removal of distraction device

Post-Surgery…

• Swelling peaks 2-3 days

• Usually do better around POD 5

• Problems if inadequate oral intake

• Significant infections are rare (2%)

• Very rare significant post-op bleeding

8/3/2020

30

Distraction Surgery

• Soft foods 4 weeks

• No contact sports for 6 weeks

Remember:

Beyond the Face

is a Heart

Resources for Professionals and Parents

8/3/2020

31

Best Resource on the Internet

Sponsored by the American Cleft Palate Association and is the most reliable resource on the internet

Cleftline.org

8/3/2020

32

We all smile the same language

QUESTIONS?

Professional Resources

• Photos used in this presentation are property of Boystown National Research Hospital

• Cleft Palate-Craniofacial Journal

• Comprehensive Cleft Care – Losee & Kirschner

• Comprehensive Cleft Care Family Edition –Losee & Kirschner

• Cleft Lip and Palate – Wyszynski

8/3/2020

33

Craniofacial BVIS

• Website for Animations

• University of Illinois at Chicago graduate students who specialize in medical animation spent over 300 hours to create each of these animations. Their hope is to educate patients, families and professionals caring for these children