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Cleft Lip and Palate

CRITICAL ELEMENTS

OF CARE

Produced by

The Center for Children with Special Health NeedsChildrens Hospital and Regional Medical Center, Seattle, WA

First Edition, Revised 5/2003

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Childrens Health Care System 1997, 2003

The Critical Elements of Care(CEC) consider care issues throughout the life span of the child. The

intent of this document is to educate and support those caring for a child with cleft lip and palate. The CEC is

intended to assist the primary care provider in the recognition of symptoms, diagnosis and care management

related to a specific diagnosis. It provides a framework for a consistent approach to management of these

children.

These guidelines were developed through a consensus process. The design team was multidisciplinary

with state-wide representation involving primary and tertiary care providers, family members and a represen-tative from a health plan.

Original Consensus Team Members:

Barbara Anderson, RN, BSN Wendy E. Mouradian, MD

Cynthia K. Anonsen, MD Kathy Mullin, RN

Cassandra Aspinall, MSW Bruce W. Novark, MD, DDS

Mary Bray, MS, CCC-A Roger Oakes, MD

Mindy Bunch, patient Ute Ochs, MD

Rebecca Bunch, parent Marsha Ose, RN, BSN, MSEd Denning Jean Popalisky, RN, MN

Kenneth Gass, MD Sharon Reinsvold, RN, BSN

Charles A. Gilmore, DDS, MSD Kathy Sie, MD

Peter Holden, MD Joni Strong, RNC, BSN

Linda Linneweh, BS Laurel Vessey, RN, BSN

Carl O. McGrath, Ph.D., CCC-S Barbara Wolters-Johnson

Content reviewed and updated 5/03:

Marsha Ose, RN, BSN, MS

This document is also available on the Center for Children with Special Needs website at:

http://www.cshcn.org

DISCLAIMER: Individual variations in the condition of the patient, status of patient and family,

and the response to treatment, as well as other circumstances, mean that the optimal treatment outcome for

some patients may be obtained from practices other than those recommended in this document. This consen-

sus-based document is not intended to replace sound clinical judgement or individualized consultation with

the responsible provider regarding patient care needs.

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Standards of Care for Cleft Lip and Palate

Prenatal Diagnosis

Nursing, Coordination of Care and Feeding Issues

Pediatrics and Primary Care

Psychosocial and Developmental Issues in Cleft Care

Genetics/Dysmorphology

Plastic Surgery

Otolaryngology/Audiology

Speech Problems in Children with Clefts

Orthodontics/Dental Medicine

CLEFT LIP AND PALATECRITICAL ELEMENTS OF CARE

INTRODUCTION 1

I. KEY INTERVENTIONS FOR CLEFT LIP/PALATE

Overview: Summary of Key Interventions by Age 3

Prenatal 4

Birth through 1 Month 5

1 through 4 Months 6



2 through 5 Years 9

6 through 11 Years 10

12 through 21 Years 11

Overview: Summary of Key Interventions by Specialty 12

II. APPENDICES

TABLE OF CONTENTS

I.

II.

III.

IV.

V.

VI.

VII.

VIII.

IX.

X.

13

15

16

18

20

23

25

27

30

33

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Oral and Maxillofacial Surgery

Ethics and Children with Cleft Lip/Palate

Types of Cleft Lip/Palate

Glossary

Washington State Cleft Lip/Palate Teams

References and Resources

XI.

XII.

XIII.

XIV.

XV.

XVI.

CLEFT LIP AND PALATECRITICAL ELEMENTS OF CARE

II. APPENDICES (cont.)

TABLE OF CONTENTS (cont.)

35

37

39

41

43

44

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INTRODUCTION

The Washington State Department of Health,Division of Family and Community Services, Childrenwith Special Health Care Needs Program has fundedinterdisciplinary work groups to identify Critical

Elements of Care (CEC)for children with special healthproblems, including cleft lip and palate. This documentwas created by the cleft lip/palate consensus team,made up of primary care physicians, specialty provid-ers, regional cleft lip/palate team coordinators, parentsand third-party payers. The CEC draws upon a number

of sources, especially the American Cleft Palate-Craniofacial Association (ACPA) Parameters of Care(1993) and Team Standards (1995) documents.1, 2, 3, 4

Central to these documents, which are summarized inthe appendix, is the principle that patients with cleftlip/palate are best cared for by an interdisciplinaryteam of specialists with experience in this field. TheCEC also draws on the literature of cleft lip and palateoutcomes,5as well as the experience of the CECteam

members.

The goals of treatment for the child with a cleftlip/palate are:

Repair the birth defect (lip, palate, nose)

Achieve normal speech, language and hearing

Achieve functional dental occlusion and gooddental health

Optimize psychosocial and developmentaloutcomes

Minimize costs of treatment

Facilitate ethically sound, family-centered,culturally sensitive care

Seven key themes are important for achievingthese goals:

Early assessmentand interventionisimperative and should begin in the newbornperiod with referral to a Cleft Lip/PalateTeam.

Aninterdisciplinary cleft lip/palate teamisneeded because cleft lip/palate outcomes arein surgical, speech, hearing, dental, psycho-social and cognitive domains.

Providerswith training and expertise incleft lip/palate careare needed because ofthe complexity of treatment interventions.

Continuity of careis essential becauseoutcomes are measured throughout the

childs life. Proper timing of interventionsis critical

because of the interaction of facial growth,dental occlusion and speech.

Coordination of care is necessary becauseof the complexity of the medical and socialfactors that must be considered in treatmentdecisions.

Better early management leads to betteroutcomes, fewer surgeries and lower costs.

Organization of this Document

This document elaborates on the above goalsand themes of treatment. Overviews on pages 3 and12highlight key interventions by age group anddiscipline, respectively. These are explained more fullyin the body of the document and in the appendices thatfollow. A glossary of terms, description of cleft types,and resource guide with a listing of cleft lip/palateteams in Washington state are also included.

The following pages list problems and inter-ventions for the child with a cleft lip/palate. Most ofthe interventions listed are provided by specialists on

the cleft lip/palate teams. Others become the responsi-bility of the primary care provider (PCP). The divisionof these tasks will vary depending upon geographiclocation and the expertise and interest of the PCP. Theservices that result must be closely coordinated withthe treatment plans of the patients cleft lip/palateteam. In addition, cleft lip/palate teams vary in both thedisciplines participating and the interventions provided.For these reasons, specific providers are often notmentioned.

1Critical Elements of Care: Cleft Lip and Palate

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Critical Elements of Care: Cleft Lip and Palate

In many cases, the PCP will need to initiate areferral to the cleft lip/palate team and preauthorize

visits with different specialists. It is the intent of thisdocument to assist the PCP caring for these childrenby summarizing interventions for each age group. Asthe interventions listed are necessarily brief, appendi-ces have been included to provide additional informa-

tion in many of the key areas.

It is important to remember that children withcleft lip/palate may be eligible for Birth-To-Threeservices as mandated by the Individuals With Disabili-ties Education Act (IDEA). Referrals to these ser-

vices can be facilitated by any Children with SpecialHealth Care Needs Coordinator at local public healthdepartments or by the cleft lip/palate team coordinator.

No further mention of the IDEA or Birth-To-Threeservices will be made elsewhere in this documentbecause other materials exist which describe these indetail.

NOTE: The interventions listed in this docu-ment are to be considered asguidelinesonly. Allinterventions may not be needed by every patient.Conversely, some patients may require interventionsnotmentioned in these recommendations. Eachpatients care plan should be individualized consideringmedical needs, psychosocial and cultural variables, andresources available in each community. Communica-tion between the community provider and the cleft lip/palate team members is essential for developing andimplementing these care plans.

Acknowledgments

We gratefully acknowledge the Mead JohnsonCompany for permission to use several illustrations andwording from their book,Looking Forward; SamuelBerkowitz and the Quintessence Publishing Companyfor permission to adapt the glossary from The Cleft

Palate Story; and Daryl Tong for the medical illustra-tions on pages 39 and 40.

1Surgeon Generals Report: Children with Special HealthCare Needs. Office of Maternal and Child Health. U.S.Department of Health and Human Services, June 1987.

2Parameters for the Evaluation and Treatment of Patientswith Cleft Lip/Palate or Other Craniofacial Anomalies.

Cleft Palate-Craniofacial Journal 1993; 30: (Suppl. 1).3Team Standards Self-Assessment Instrument. American Cleft

Palate-Craniofacial Association, 1996.

4Standards of Care for Cleft Lip and Palate . AmericanAssociation of Oral and Maxillofacial Surgeons, 1996.

5Shaw, William C., et al. A Six-Center International Study ofTreatment Outcome in Patients with Clefts of the Lipand Palate: Parts 1-5. Cleft Palate-Craniofacial Journal1992; 29(2): 393-418.

2

Introduction

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Prenatal

Birth-1 month

1-4 months

5-15 months

16-24 months

2-5 years

6-11 years

12-21 years

KEYINTERVENTIONSFORCLEFT/LIPPALATE

*VPI = velopharyngeal insufficiency. See Appendix IX, pg. 30.

I.

3

OVERVIEW: SUMMARY OF KEY INTERVENTIONS BY AGE

Note: This table is only a summaryand does not contain every intervention that could be needed by aparticular child at a certain age. For more details see pages referenced.

INTERVENTION REFER TO PAGE

4

5

6

7

8

9

10

11

AGE RANGE

Refer to cleft lip/palate teamMedical diagnosis and genetic counselingAddress psychosocial issuesProvide feeding instructionsMake feeding plan

Refer to cleft lip/palate teamMedical diagnosis and genetic counselingAddress psychosocial issuesProvide feeding instructions and monitor growth

Monitor feeding and growthRepair cleft lipMonitor ears and hearing

Monitor feeding, growth, developmentMonitor ears and hearing; consider ear tubesRepair cleft palateInstruct parents in oral hygiene

Assess ears and hearingAssess speech-languageMonitor development

Assess speech-language; manage VPI*Monitor ears and hearingConsider lip/nose revision before schoolAssess development and psychosocial adjustment

Assess speech-language; manage VPIOrthodontic interventionsAlveolar bone graftAssess school/psychosocial adjustment

Jaw surgery, rhinoplasty (as needed)Orthodontics; bridges, implants as neededGenetic counselingAssess school/psychosocial adjustment

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PRENATAL

Summary of critical interventions at the time of prenatal diagnosis:Medical diagnosis and genetic counselingRefer to cleft lip/palate team

Make feeding planAddress psychosocial crisis

6 Previously, when the antenatal diagnosis of a cleft was made, it was often in the context of other anomalies andmedical problems. However, with improvements in ultrasonography, isolated clefts now can be diagnosed more

readily. Nonetheless, it is prudent to caution families that definite plans will be formulated after the baby is born and has

been carefully examined. (See Appendix II, pg. 15.)

PROBLEM INTERVENTION

Family need for information on

care of cleft lip/palate

Psychosocial crisis in family

Anticipated closure of cleft lip/palate

Need to anticipate feedingplan

Team/family need for accu-rate medical/diagnosticinformation on cleft (and anyother anticipated medicalproblems) 6

Refer family to cleft lip/palate team for information on cleftcare, including feeding, speech, ear and dental problems,surgical management, etc.

Team nurse/psychosocial worker meet with familyRefer to community resources as needed

Team plastic surgeon meets with family if possible todiscuss plans for closure

Team nurse coordinator or public health nurse counselsand provides feeding instructions and specialized bottles(see Appendix III, pg. 16)

Team reviews ultrasound results and information fromperinatologist, geneticist, primary care physician, etc.If relevant, discusses implications for cleft care with familyFamily discusses other issues with appropriate care providers

(primary physician, OB, perinatologist, geneticist, radiologist,etc.)

4

I. Key Interventions for Cleft Lip/Palate

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BIRTH THROUGH 1 MONTH

Summary of critical interventions for ages birth through 1 month:

Referral to cleft lip/palate teamMedical diagnosis and genetic counselingFeeding and growth interventionsAddress psychosocial issues


Need for interdisciplinary care ofthe cleft

Refer to cleft lip/palate team

Cleft lip/palate Team specialists assess the cleft and examine infant fordysmorphic features and other anomalies

Discuss diagnosis and treatment plan with the familyAddress etiology and recurrence risksConsider presurgical orthopedics, depending on the

structure of the cleft (see Appendix X, pg. 33)

Feeding difficulty and high riskfor poor weight gain

Team nurse or public health nurse counsels family onappropriate feeding technique (see Appendix III, pg. 16)

Assess weight weekly for first month to verifyadequate gain: should regain birth weight by two weeks,

and 5-7 oz/week thereafterAdditional consultation with cleft feeding specialist or

dietitian if neededProvide psychological support if feeding plan involves

loss of ability to breast-feed

Middle ear status, hearing,airway

Assess middle ear statusAssess hearing (BAER and evoked otoacoustic

emissions)Rule out airway problems, especially if diagnosis is

Robin Sequence or if the cleft is part of a syndrome(see Appendix VIII, pg. 27)

Familys need for information andpsychosocial support

Help family deal with guilt, loss and adjustment issuesIdentify community resources and support groupsAddress barriers to care: insurance issues, transporta-

tion needs, absence from work, language and culturaldifferences

Provide psychosocial support and assessment to opti-mize child and family adjustment

5


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1 THROUGH 4 MONTHS

Summary of critical interventions for ages 1 through 4 months:Monitor feeding and growthRepair cleft lipConsider ear tubes

6



Need for interdisciplinary care of thecleft

Cleft lip/palate

Feeding and growth problems

Middle ear status, hearing, airway

Cleft lip and nasal deformity

Familys need for specific pre- and

post-operative lip repair information

Familys ongoing need for informationand psychosocial support

Refer to cleft lip/palate team (if not already done)Team coordinates care and needed surgeries

Team specialists assess the cleft and examine infantfor dysmorphic features and other anomalies (if not alreadydone)

Discuss diagnosis and treatment plan with the familyAddress etiology and recurrence risks (if not already done)Monitor presurgical orthopedics (if being used)

Monitor feeding; provide instructions as neededVerify adequate weight gain by plotting on growth gridRefer to cleft feeding specialist/dietitian, as needed

Monitor middle ear status (consider ear tubes with liprepair if middle ear effusions persistent or recurrent)

BAER and evoked otoacoustic emissions (if not alreadydone)

Monitor for airway problems if diagnosis is Robin Sequenceor other syndrome (see Appendix VIII, pg. 27)

Repair cleft lip, usually at 3-4 months (cheiloplasty, seeAppendix VII, pg. 25)

May include primary nasal reconstruction (rhinoplasty)Dental models (at or before lip repair)

Teach pre- and post-operative care requirements

(e.g. feeding plan, use of arm splints, pain management)Assess familys understanding of these instructions and

ability to follow throughHelp family make the necessary medical and social

arrangements

Help family deal with guilt, loss, and adjustment issuesIdentify community resources and support groupsAddress barriers to care: insurance issues, transportation

needs, absence from work, language and culturaldifferences

Provide psychological support to optimize child andfamily adjustment

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5 THROUGH 15 MONTHS

Summary of critical interventions for ages 5 through 15 monthsMonitor feeding, growth and developmentConsider ear tubes/assess hearingRepair cleft palate

7



Need for continued interdisciplinarycare of the cleft


Cleft palate

Familys need for accurate geneticinformation

Familys need for specific pre- andpost-operative palate repair information

Feeding, growth and developmentissues

Abnormal dental development

Speech-language production


Cleft lip/palate team coordinates cleft care includingsurgeries

Place/replace ear tubes if persistent or recurrentmiddle ear effusions >three months (coordinate withpalate repair if possible)

Assess hearing at six months of age and at six monthintervals thereafter

Monitor for airway problems if diagnosis is RobinSequence or other syndrome (see Appendix VIII, pg. 27)

Repair cleft palate, usually at 9-15 months (palato-plasty, see Appendix VII, pg. 25); obtain dental models

Provide genetic counseling (if not already done)(see Appendix VI, pg. 23)

Teach pre- and post-operative care requirements (e.g.feeding plan, arm splints, pain management)

Assess the familys understanding of these instructionsand ability to follow through

Help family make the necessary medical and socialarrangements

Advance to solid foodsAddress feeding difficultiesProvide regular growth and development screening

Instruct parents in oral hygiene; monitor tootheruption

Provide parents with information about expected dentaldevelopment

Provide speech-language counseling to parents beforethe palate repair

Assess childs speech-language 3-6 months afterpalate repair

Continue to review family/child adjustment issuesIdentify community resources and support groups

Address barriers to care: insurance issues,transportation needs, absence from work, language andcultural differences


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16 THROUGH 24 MONTHS

Summary of critical interventions for ages 16 through 24 monthsMonitor ear tubes and hearingAssess speech-language and developmentMonitor development

8






Feeding, growth and developmentissues

Repaired cleft lip/palate



Cleft lip/palate team provides cleft care andcoordination

Monitor middle ear status every six monthsPlace/replace tubes if middle ear effusions persist

>three monthsAssess hearing every six months

Assess speech-language, especially velopharyngealmechanism (see Appendix IX, pg. 30)

Address feeding difficultiesProvide regular growth and development screening

Monitor integrity of the surgical repairsAssess the palate for fistula(e)

Monitor tooth eruption and oral hygieneReassure parents regarding expected dental

development

Continue to review family and child adjustmentissues

Identify community resources and support groupsAddress barriers to care: insurance issues,

transportation needs, absence from work, languageand cultural differences


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2 THROUGH 5 YEARS

9






Abnormal dental development andalveolar defect


Childs overall developmental andbehavioral adjustment


Cleft lip/palate team coordinates cleft care andcoordination

Monitor middle ear status every six monthsPlace/replace ear tubes if middle ear effusions

persist for >three monthsAssess hearing at six months intervals until age three,

then every 6-12 months as indicatedAssess airway, sleep disturbances (workup if

suspect obstructive sleep apnea that could be dueto small jaw, large tonsils/adenoids, pharyngeal flapor sphincter pharyngoplasty)

Caution needed when considering adenoidectomy(see Appendix VIII, footnote 12, pg. 28)

Assess speech-language, monitor for velopharyngealinsufficiency (VPI)

Formal VPI workup, as indicated (see Appendix IX,pg. 30)

Consider treatment options (speech therapy, surgeryor speech prosthesis)

Orthodontic exam and dental records at age 4 or 5for bone graft timing and management of abnormaldentition

Dental extractions if needed

Consider lip/nose revision before school entryClose palatal fistula(e) if indicated

Monitor for developmental/behavioral problemsProvide counseling or make referrals as needed

Continue to review family adjustment issuesIdentify community resources and support groupsAddress barriers to care: insurance issues, transpor-

tation needs, absence from work, language andcultural differences

Provide psychological support to optimize child andfamily adjustment (school entry and peer comments

may be sources of stress)

Summary of critical interventions for ages 2 through 5 years:Assess speech for VPI; consider interventionsMonitor ear tubes and hearingRevise lip/nose before school if neededAssess childs development, including language and psychological adjustment

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6 THROUGH 11 YEARS

10


Summary of critical interventions for ages 6 through 11 years:Assess speech for VPI; consider interventionsOrthodontic interventions and alveolar bone graftingMonitor school performance and psychological adjustmentInvolve child in medical decision-making process




Abnormal jaw growth and dentaldevelopment

Persistent bony cleft of alveolus andoro-nasal fistula(e)


Childs overall developmental andbehavioral adjustment

Childs and familys ongoing needfor information and psychosocialsupport

Cleft lip/palate team provides cleft care and coordination

Monitor middle ear status every 6-12 monthsPlace/replace ear tubes if middle ear effusions persist >three

monthsAssess hearing every 6-12 months as indicatedAssess airway, sleep disturbances (workup if suspect

obstructive sleep apnea that could be due to small jaw,large tonsils/adenoids, pharyngeal flap or sphincterpharyngoplasty)

Caution needed when considering adenoidectomy (seeAppendix VIII, footnote 12, pg. 28)

Assess speech-language; monitor for velopharyngeal

insufficiency (VPI)Formal VPI workup as indicated (see Appendix IX, pg. 30)Consider treatment options (speech therapy, surgery,

obturation)Communicate with school or outside clinician if the child is

receiving speech therapy

Regular orthodontic exams and records to monitor jawgrowth and readiness for alveolar bone graft

Orthodontics often needed before and after the bone graftDental extractions as needed; monitor dental hygiene

Bone graft to the alveolar cleft(s) and closure of the oro-nasalfistula(e) (timing is critical)

Close palatal fistula(e) if indicatedConsider lip/nose revision as needed

Monitor school performance, emotional and behavioral issuesMake referrals as necessary (see Appendix V, pg. 20)

Continue to review family adjustment issuesIdentify community resources and support groupsAddress barriers to care: insurance issues, transportation needs,

absence from work or school, language and cultural differences

Provide psychological support to child and familyInvolve child in decision-making process as age/abilities allow


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12 THROUGH 21 YEARS

11


Summary of critical interventions for ages 12 through 21 years:Jaw surgery, rhinoplasty if neededFinal orthodonticsGenetic counselingAssess overall psychological adjustmentReview school issues/vocational plans

Need for continuedinterdisciplinary care of the cleft





Maxillary/mid-face hypoplasiawith malocclusion

Adolescents and familysongoing need for information andsupport

Adolescents overalldevelopmental adjustment

Cleft lip/palate team provides cleft care and coordination

Monitor middle ear status every 6-12 monthsPlace/replace ear tubes if middle ear effusions persist for >three

monthsAssess hearing every 6-12 months until ears are clear and hearing

normal for two yearsAssess airway, sleep disturbances (workup if suspect obstructive

sleep apnea that could be due to small jaw, large tonsils/adenoids,pharyngeal flap or sphincteroplasty)

Caution needed when considering adenoidectomy (see Appendix VIII,footnote 12, pg. 28)

Assess speech, rule out velopharyngeal insufficiency (VPI)Workup VPI if indicated (see Appendix IX, pg. 30)Consider treatment options (therapy, surgery, obturation)Communicate with school or outside speech clinician

Regular orthodontic exams and dental records to monitor bone graftand jaw growth

Final orthodontics when facial growth completeProvide bridges or implants as needed

Consider lip/nose revisionMonitor palate for fistula(e)

Orthodontic treatment and/or jaw surgery

Continue to review adolescent and family adjustment issuesIdentify community resources and support groupsAddress barriers to care: insurance issues, transportation needs,

absence from work or school, language and cultural differencesProvide psychological support to optimize adolescent and family

adjustment (peer teasing, adolescent self-esteem and schooltransitions are areas of focus)

Provide adolescent/family with appropriate genetic information,including risks for recurrence

Involve adolescent in medical decisions; respect preferences on

elective procedures (see Appendix V, pg. 20)

Review school performance, academic/vocational plansScreen for behavioral/emotional problems; refer as needed


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Critical Elements of Care: Cleft Lip and Palate 12

OVERVIEW: SUMMARY OF KEY INTERVENTIONS BY SPECIALTY

Note: This table is only a summaryand may not include all disciplines needed for a particular child.Team participants may also vary depending upon community and location.

INTERVENTION APPENDIX

Cleft lip/palateteam

Nursing (teamcoordinator, publichealth nurse,feeding therapist)

Pediatrics/primarycare/genetics

Social work andpsychology

Surgery (plasticsurgery, otolaryn-gology, oral andmaxillofacial

surgery)

Audiology

Speech andlanguage

Orthodontics anddentistry

Coordinate careProvide experienced specialistsMonitor medical and social issues

Coordinate careFeeding counselingMonitor psychosocial issuesPre- and post-operative teaching

Monitor general medical issues

Assist with coordination of care and referralsMonitor developmental and behavioral issuesGenetics/dysmorphology assessment

Monitor psychosocial issuesDevelopmental/behavioral problemsRefer to community resourcesAssist with coordination of care

Lip and palate repair; lip scar revisionVelopharyngeal surgery for VPIEar tubesRhinoplasty

Alveolar bone graftJaw surgery; dental extractions

Monitor hearingRecommend preferential seating and

amplification when appropriate

Monitor speech-language developmentAssist with VPI evaluationCommunicate with school or outside therapistsProvide speech-language therapy; provide speech

prosthesis therapy

Presurgical orthopedics as neededFollow dental eruption, hygieneMonitor facial and jaw growthMove dental arches/teethProvide speech prosthesis, bridges, implants as

needed

SPECIALTY


I, pg. 13

III, pg. 16V, pg. 20

II, pg. 15IV, pg. 18VI, pg. 23

V, pg. 20

VII, pg. 25VIII, pg. 27

XI, pg. 35

VIII, pg. 27

IX, pg. 30

X, pg. 33

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II. Appendices

II. APPENDICES

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General standards of care for children withcleft lip and palate and other craniofacial anomalieshave been created by the American Cleft Palate-

Craniofacial Association (ACPA). These standardsare contained in two documents summarized below.Central to these documents is the principle thatmanagement of patients with cleft lip/palate is bestprovided by an interdisciplinary team of specialistswith experience in this field. Both documents areavailable from the ACPA national office (seeAppendix XVI, pg. 44).

1. ACPA Parameters of Care (1993)

This document is based on a national consen-sus conference funded by the Bureau of Maternal and

Child Health, in conjunction with the ACPA. It drawson the 1987 Surgeon GeneralsReport on children withspecial health care needs.

Fundamentals of care for children withcleft lip/palate (and other craniofacialanomalies):

1. Requires an interdisciplinary team ofspecialists with experience in cleft lip/palate

2. Team must see sufficient numbers tomaintain expertise

3. Optimal time for team evaluation is in first fewdays or weeks of life

4. Team should assist families in adjustment tothe birth defect

5. Team should adhere to principles of informedconsent, form partnership with parents, andallow participation of the child in decision-making

6. Care is coordinated by the team, and isprovided locally if possible and appropriate

7. Team should be sensitive to cultural,psychosocial and other contextual factors

8. Team is responsible for monitoring short- andlong-term outcomes, including qualitymanagement and revision of clinical practices,when appropriate

9. Treatment outcomes include psychosocialwell-being, and effects on growth, functionand appearance

10. Long-term care includes evaluation and

treatment in the areas of audiology, dentistry/orthodontics, genetics/dysmorphology, nursing,oral and maxillofacial surgery, otolaryngology,

pediatrics, plastic surgery, psychosocialservices and speech-language pathology

2. Summary of ACPA Team Standards Self-Assessment Instrument (1996)

This document sets standards for cleft lip/palate (and craniofacial) teams. Teams are to beevaluated by self-assessment, and then listed by theACPA as Cleft Lip/Palate Teamsif they meet thecriteria outlined below. In addition, other teams may belisted which do not meet all these criteria, but areeither new, provide only evaluation and treatmentreview, or serve low-population areas. In reportingprofessional services to the ACPA, teams may notinclude patients treated on overseas missions to meetrequired standards.

Basic Criteria : Cleft lip/palate teammust meet alleight:

1. Team meets face-to-face at least six times/year, with at least four disciplines present

2. Team evaluates at least 50 new/return patientsa year

3. Team has central and shared files on eachpatient

4. The team has at least an actively involvedsurgeon, orthodontist and speech-languagepathologist. All patients are evaluated bythese specialists and one other specialist.

5. Team assures that all children are evaluatedby a primary care physician (pediatrician,family physician or general internist) on or offteam

6. Evaluations of patients by this team include ascreening hearing test and tympanogram (allpatients with clefts are referred to anotolaryngologist for examination, consultationor treatment)

7. At least one surgeon on the team has operatedon 10 or more patients for primary repairs ofa cleft lip and/or palate in the past year

8. Team refers patients requiring facialskeletal surgery (bone grafts, orthognathicsurgery) to a surgeon with education,training experience preparing him/her forthis surgery, and who also has performed 10osteotomies or more in the past year

13

I. Appendix

STANDARDSOFCAREFORCLEFTLIPANDPALATE

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In the past, prenatal diagnosis of a cleft lipwas almost always made in association with otherabnormalities in the fetus. With improvements inultrasound technology, the prenatal diagnosis ofisolated cleft lipis increasingly common. However, itis easy to miss cleft lip on diagnostic ultrasounds,particularly those performed for routine indications inthe physicians office7 (The American Institute ofUltrasound in Medicine does not even require views ofthe lips on screening ultrasounds8 ). If there is a familyhistory of clefting, or if there is a concern about apossible cleft for other reasons, a referral should bemade for a complete diagnostic ultrasound and geneticcounseling. The most accurate ultrasound examinationis obtained at about 20 weeks gestation or possiblylater. However, many patients are scanned earlier,

especially if amniocentesis is being considered (typi-cally performed at 15-17 weeks). As it is more difficultto detect cleft lips in these earlier ultrasounds, a laterscan can be performed if concerns about a possiblecleft remain (e.g. positive family history). Ultrasoundcan often establish whether a cleft lip is unilateral orbilateral. It is still very difficult to make the diagnosisof acleft palateantenatally, unless it is detected inassociation with a large cleft lip.

Once a cleft lip/palate is identified, the familyshould be referred for genetic counseling to discussother testing, including amniocentesis. During the

genetic counseling session, a complete pregnancy andfamily history should be performed. This should includeinformation on any teratogenic exposures, and thepresence of family members with clefts or other birthdefects, developmental problems and genetic syn-dromes. Even if genetic tests are negative, parentsshould be informed that an accurate diagnosis andcomplete discussion of prognosis and recurrence riskscan only take place after the baby is born.

When a cleft lip/palate is detected prenatally,the family should be referred to a cleft lip/palate teamto learn about the care and management of childrenwith clefts. The diagnosis of this birth defect creates acrisis for a family, so attention to psychosocial andemotional issues is essential at this time. Most familiesexperience a grief reaction, although many feel angerand/or guilt as well. Relationships may be strained andthere may be blaming of various family members.Supportive counseling and referral to communityresources may be needed. If appropriate resources areprovided, most families can adjust adequately to thisunexpected news.

At the familys first visit with the cleft lip/palate team, feeding instructions should be provided,and a clear plan for the newborn period should beformulated. Additional medical information provided atthis visit should include a general description of thetypes of problems the baby may encounter. Thisopportunity to formulate a feeding plan, learn about thefuture care their child will receive, and meet theproviders involved in this care can greatly increase aparents sense of control and preparedness in the faceof this unanticipated diagnosis.

15

7 Sniders, R.J.M., Nicholaides, K.H.Ultrasound

Markers for Fetal Chromosomal Defects. New York:

The Parthenon Publishing Group, 1996: 101.

Studies with a total N = 24,802 found that only

23 percent of facial clefts were detected by

ultrasonography. 8 J Ultrasound Medicine 1991; 10: 577.

II. Appendix

PRENATALDIAGNOSIS

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Specialty nurses, including cleft lip/palate teamnurses and public health nurses associated with theChildren with Special Health Care Needs Program(Department of Health), play an important role in thecare of patients with cleft lip/palate. They assist withcoordination of care, provide peri-operative counselingand help monitor psychosocial issues. When appropri-ate, they may also provide community outreach andrefer to community resources. In addition, because oftheir knowledge of the medical issues at stake in thecare of these children, they may be called upon to helpwith referrals.

One of the most important functions of nursesand other knowledgeable specialists is offering detailedfeeding instructions and support for new parents ofbabies with cleft lip/palate. The importance of feedingissues in the care of these infants prompts this sum-mary of feeding issues:

Feeding the Infant with a Cleft Lip/Palate

I. For those infants with a cleft lip only:

Infants that have only a cleft lip can be fed byeither breast or bottle. Some problem-solvingmay be needed to ensure that the infant canget a tight seal around the breast or nipple.Early referral to the infant-feeding specialists

or nurses associated with cleft lip/palate teamscan facilitate this problem-solving.

II. For those infants with a cleft palate, with or withouta cleft lip:

The infant with a cleft palate will requirespecific bottles and a special feedingtechnique. Breast-feeding and use of a regularbottle are rarely possible. Lack of knowledgeof this important fact can lead to failure tothrive.

A. Why the infant with a cleft palate cannotbreast-feed or use a regular bottle:

The purpose of the palate is to separate themouth from the nose. Normally the soft palateat the back of the mouth moves up to close offthe passage to the nose during feeding. Thiscreates a closed system, and the suckingmotions create negative pressure which pullsthe milk out of the breast or bottle. A cleft

palate prevents the infant from creating a closedsystem in his/her mouth, and makes it impos-sible for the milk to be pulled out. The infantwill look like he/she is sucking, but he/she willbe using up precious calories in a futile attemptto gain adequate nutrition.

B. How to feed the infant with a cleft palate:

The proper bottle is the key to a successfulfeeding plan. There are three options currentlywidely used. The first is the Cleft Palate

Nurser made by the Mead Johnson Company.It is a soft-sided bottle that is squeezed incoordination with the infants sucking efforts,and thus milk is delivered into the mouth. The

second is the Haberman feeder availablefrom the Medela Company. This feederconsists of a large, compressible nipple with aone-way valve at its base that keeps the nipplefull of milk. The infants effort to compress thesoft nipple is often sufficient to dispense themilk into the infants mouth, but this can alsobe assisted by squeezing the nipple to increasethe flow. The third option is the Pigeon CleftPalate Nurser distributed by Childrens MedicalVentures. This system also makes use of a one-way valve at the base of the nipple. In addi-tion, the nipple is constructed with a thinner,

more compressible side so that the infantstongue is effective in compressing the nipple toproduce the flow. None of these bottles areavailable in stores, but all cleft teams canprovide them to families or provide phonenumbers for ordering. All three of these bottleswork without the infant needing to create intra-oral suction in order to pull milk out of thenipple. They all require parent training forproper use. For training, contact the cleft lip/palate team for referral to the infant feedingtherapist or nurse experienced in feedinginfants with clefts.

Mothers and families need adequatepsychosocial support to process the loss of theability to breast-feed their infant. Pumpingbreast milk for use in the specialized bottleallows the mother who wishes to breast-feedthe ability to give her baby her own milk.However, long-term pumping requires aconsiderable commitment of time and effort tomaintain an adequate milk supply in the

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Critical Elements of Care: Cleft Lip and Palate 17

III. Appendix

absence of normal infant sucking. The needfor supplemental feedings with formula mustbe closely monitored.

C. Establishing feeding goals and monitoringweight gain for the infant with a cleft palate:

Even with a specialized bottle, closeattention to weight gain is mandatory for theinfant with a cleft palate. If the infant does notmaintain an upward climb on the growthcurve, feeding re-evaluation and changes intechnique may be needed. The nurse ortherapist experienced in feeding an infant witha cleft can evaluate the feeding process andmake changes. Sometimes a consultation witha dietitian is needed to establish calorie goalsand to provide recipes to increase the caloriesin the breast milk or formula. There should belittle tolerance for any failure to follow anormal growth curve in the first months of life.

These two feeding parameters must beobserved to promote adequate weight gain:

1. The infants intake over 24 hoursshould be 2.5 ounces of milk for each poundthat he/she weighs.

2. No feeding session should take longer than35 minutes. If it takes longer than this,the infant is working too hard and burningcalories needed for growth.

The measure of success of the feeding plan isadequate weight gain. Weekly weights andplotting the data on the growth curve are theproper way to evaluate this.

D. The introduction of solid foods:

The timing and strategy of introducing solidfoods should be the same for the baby with acleft palate as for any other child. Experimentwith the consistency of the food to minimizeregurgitation out of the nose while still allowinga smooth swallow. Some sneezing may occurbecause the exposed nasal passages can beirritated by food. Following each meal withswallows of milk or water is all that is neededto remove any remaining food in the mouth.

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The pediatrician or other primary care pro-vider (PCP) is indispensable in the care of the patientwith cleft lip and palate. Ideally, the PCP becomes anextended member of the cleft lip/palate team, followingmany of the same medical issues as the team special-ists (such as recurrent otitis media, airway concerns,growth failure and developmental progress). Inaddition, the PCP may have the special task of advo-cating for the child in a particular health care system,and preauthorizing visits to the cleft lip/palate teamproviders. For these reasons it is essential that thePCP be familiar with the special aspects of cleft care.The following are particularly important issues for thePCP:

1. Feeding. Although many newborns havefeeding problems, babies born with cleft palatesare particularly at risk for significant failure tothrive. One reason for this is the difficulty theyhave creating suction with the cleft palate, leadingto inefficient, calorie-wasting attempts to suck,resulting in inadequate nutritional intake. Inaddition, some babies (e.g. with Robin Sequence,discussed below) have difficulty coordinatingbreathing, sucking and swallowing, which furtherimpedes adequate intake. Thus growthparameters must be monitored very closelyin thefirst few weeks of life. Adequate feeding ispossible with special bottles and techniques, both

available from cleft feeding specialists(usuallynurses or feeding therapists) associated with cleftlip/palate teams. The knowledgeable nurse in thenewborn nursery can initiate proper feeding, but itis essential that these babies be monitored overthe long-term (see Appendix III, pg. 16).

2. Robin Sequence. Babies born with RobinSequence typically have a large U-shaped palatalcleft and a small lower jaw (retro/micrognathia),which results in a posterior tongue position thatcan interfere with breathing. If the baby appearsto have this condition and is having difficultybreathing due to obstruction by the tongue(glossoptosis), the baby should be placed in theprone position immediately. If this does not relievethe infants distress and allow for normaloxygenation (as monitored by an oximeter), thenplacement of a nasopharyngeal (NP) tube ortemporary oropharyngeal tube is indicated. It isdesirable to involve an experiencedotolaryngologist if any of these interventions areneeded. Some of these babies may require

prolonged use of the NP tube or a tracheotomy.

Even when the baby with Robin Sequenceappears to be comfortable at rest, he/she maybe so stressed during feeding that adequateweight gain does not take place. Therefore closemonitoring is essential. Many factors maycontribute to failure to thrive in these babies:difficulty coordinating suck/swallow; inefficiencyof feeding with the cleft palate; glossoptosis withincreased work of breathing; and caloricconsumption. These issues can be difficult toresolve, and generally require the coordinatedefforts of pediatrics/primary care, otolaryngology,nursing, occupational therapy and respiratorytherapy. Occasionally, a polysomnogram (sleepstudy) with CO

2monitoring may be necessary to

determine if ventilation is adequate. These issues

are best addressed with a cleft lip/palate teamand in a hospital where there is access topediatric anesthesia. In the event of a respiratoryemergency, these babies can be very difficult tointubate because of their abnormal anatomy.

3. Middle Ear Effusions. Infants with cleft palateare at high risk for recurrent and chronic middle-ear disease (90-95 percent). Many of theseinfants will require ear tube placement. Thehearing loss which may result from these effu-sions can be significant, and may interfere withspeech and language development. Due to the

difficulty of reliably diagnosing middle eareffusions in infants, it is recommended that anotolaryngologist periodically evaluate thesechildren (see Appendix VIII, pg. 27).

4.Genetics/Dysmorphology. Genetic counselingis necessary to provide patients and families withinformation on recurrence risks, and should beoffered after the child is born, at adolescence orwhenever family questions about etiology andrecurrence risks. Because a significant number ofchildren with cleft lip/palate have geneticsyndromes (especially those with a cleft palate),this possibility should be considered if a patienthas atypical facial features, developmental delays,learning problems or other anomalies. If thepatient is followed by a cleft lip/palate team thatis without a dysmorphologist or a geneticist,consider referral to an outside specialist. Recentstudies suggest that pre-conceptual and prenataldietary supplementation with folic acid maydecrease the risk of cleft lip/palate, although theoptimum dosing is not known. In any case,prospective mothers should take a minimum of

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0.4mg of folic acidthe amount in mostprenatal vitaminsstarting three months prior toconception. This is the general recommendeddose for prevention of neural tube defects. Thedose may be higher to prevent recurrences ofcleft lip and palate (or neural tube defects) infamilies with a positive history. (See Appendix

VI, pg. 23.)

5. Dental Issues. Dental issues are of paramountimportance in the management of patients withcleft lip/palate. Unfortunately, dental care is often

viewed as optional and not included in typicaldefinitions of medical necessity, which isparticularly deleterious for patients with cleft lip/palate. The PCP is often asked to authorize visitsto orthodontists and oral and maxillofacialsurgeons, and must appreciate the integralmedical role of these specialists in the care of thechild with cleft lip/palate.

First, if presurgical orthopedics are needed(e.g. tape or an internal appliance to bring thelip/jaw segments closer together beforesurgery), an appropriate dental specialist(pediatric dentist, prosthodontist ororthodontist) must be involved in the initialassessment during the first few weeks of life.

Second,good oralhygieneis essential forsuccessful cleft habilitation. Thus once theteeth have erupted, preventive counselingshould take place regarding baby bottle caries,proper tooth brushing, etc. The PCP has an

important role to play reinforcing proper dentalcare and hygiene.

Third, correctplacementof the teeth anddental arches is necessary before alveolarbone grafting can take place. Alveolar bonegrafting is usually needed when clefts extendthrough the upper gum (alveolus). Thisprocedure is generally performed between theages of 6-12 years, depending upon dentaldevelopment. The alveolar bone graft providesthe foundation for the erupting teeth andsupport for the nasal base. Orthodontic

interventions are necessary before and afterthis bone graft.

Orthodontic interventions are also needed inadolescence to bring teeth into final alignmentand address malocclusion resulting fromdeficiencies in upper/lower jaw growthacommon problem in patients with cleft lip/palate. A certain number of these patients willalso need jaw surgery because the deficiencyis too great for orthodontic compensationalone.

Orthodontists, oral and maxillofacialsurgeons, and craniofacial surgeons affiliatedwith the cleft lip/palate teams generallymonitor these issues. (See Appendix X, pg.

33; Appendix XI, pg. 35.)

6. Development.Development should be

monitored in all children. However, children withcleft lip/palate are at increased risk for develop-mental and behavioral problems. First, speechproduction problems can result from theanatomical differences associated with the cleftpalate. Second, speech and language delays canresult from intermittent hearing lossaccompanying recurrent or persistent middle eareffusions. Third, a significant number of patientswith cleft lip/palate (especially those with isolatedcleft palate) will have a syndrome withdevelopmental implications. Finally, psychosocialissues stemming from the cleft can affect thechilds emotional well-being, school performanceand overall developmental adjustment. Specialtyhelp is available in these areas to assist childrenand families with these issues. Both PCPs andcleft lip/palate teams should monitordevelopmental, behavioral and psychosocialissues. Interventions and resources should berecommended as appropriate (see Appendix V,pg. 20).

7. General Medical Care. These children, like allothers, require ongoing well-child care. It may be

difficult to accomplish this when the focus of thefirst months is on cleft-related issues. However,it is important that the PCP continue regularhealth maintenance, including administration ofimmunizations, attention to any other healthproblems and provision of anticipatory guidance inother areas of health and development. Duringadolescence, health issues should be monitoredappropriately, and the PCP should includescreening for issues related to sexual activity,substance abuse, depression and other healthproblems. Adolescence is a difficult time for mostpeople, but can be especially difficult for those

who look or sound different from their peers.

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Psychosocial issues are a critical part of theassessment and management of the child with cleft lip/palate, and must be addressed from the onset of care.The birth of a child is always a time of great familyadjustment, and it is especially stressful when the childis born with a birth defect such as cleft lip/palate.Parents often experience feelings of sadness, guilt,anger and fear for their childs future social accep-tance. Some parents feel the extent of their emotionalturmoil is unwarranted with such a repairable birthdefect, and experience guilt that a facial deformity isso disturbing to them. In addition, the feeding difficul-ties these infants experience can be threatening to newparents, who may doubt their own ability to feed andnurture an infant with such differences. The loss of theability to breast-feed is especially traumatic for some

mothers. In part, through good psychosocial supportand proper instructions, most families are able to workthrough their own emotional turmoil and effectivelymaster the skills needed to feed and nurture thesebabies. Other issues of concern for new parents relateto accessing professional and community services,securing adequate financial resources and coping withthe stress of sending a child to surgery.

As the child grows, the family will have otherconcerns, often relating to teasing, peer acceptance,speech difficulties, learning and behavior problems. Formany families, securing appropriate community and

financial resources remain important issues. Childrenshould have their evolving decision-making roleacknowledged, and should be personally addressedduring appointments. During adolescence there arenew challenges, as the maturing teen strives forindependence and copes with being different in ahighly appearance-conscious culture. Adolescents andpre-teens should be given the opportunity to confiden-tially share feelings and concerns with a qualifiedprofessional. Older children and teens often requireconsiderable support in preparing for major proceduressuch as alveolar bone grafting (usually performedbetween the ages of 6-12 years) and jaw surgery

(performed when growth is complete, in the mid- tolate-teens). Psychosocial assessment and support mayalso become necessary when a high level of patientcompliance and family commitment are required forcertain interventions, such as obturator therapy. Whenconsidering elective procedures such as lip scarrevision or rhinoplasty to correct facial disfigurement,the maturing childs preferences should be respected.

Other important circumstances that are oftenaddressed by a psychosocial professional include childabuse/neglect, substance abuse, domestic violence andother family dysfunction. It is not uncommon to see achild in a dysfunctional family become overly focusedon fixing my face as a way for them to fix thedysfunction in the family. There is research to suggestthat unless such emotional issues are addressed priorto surgery, such interventions alone are less likely tochange self-image and improve quality of life.

A detailed and specific psychosocial assess-ment is appropriate for all families presenting to a cleftpalate team, regardless of socioeconomic status andperceived stability. In assessing children and families,their unique cultural and social characteristics must betaken into account, with a clear understanding of anyimplications for providing health care. Cultural differ-ences as well as other unexplored parental worries andconcerns often contribute to behavior perceived andlabeled by health care providers as noncompliant.Understanding cultural and psychosocial issues isessential for the delivery of good health care.

Learning Disorders and Behavioral Problems

Children with cleft lip/palate may be atincreased risk for learning disorders. Fluctuatinghearing loss associated with middle ear disease mayimpair speech and language development. Some

children with clefts may have learning difficultiesassociated with a syndromic diagnosis (e.g.

velocardiofacial syndrome, Opitz-Frias syndrome, fetalalcohol syndrome). However, children with isolatedclefts (especially cleft palate), also appear to be atincreased risk for learning problems.

Children with cleft lip/palate may be atincreased risk for behavioral disorders as well. Again,these disorders may be associated with a syndromicdiagnosis (velocardiofacial syndrome, fetal alcoholsyndrome), but can occur in children with isolatedclefts as well. Symptoms may include social with-

drawal, depression, conduct problems or school failure.Furthermore, social and educational circumstances,peer dynamics, problems in the child-parent relation-ship, and intrinsic characteristics of the child (includingtemperament and underlying cognitive problems) cancombine to create a complex clinical picture. For allthese reasons, children with cleft lip/palate should bemonitored regularly for psychosocial, learning and

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behavioral problems. When such problems arise,relevant areas should be assessed, and the interactionof these variables recognized. Specialties suited toscreen for these disorders include psychology, socialwork, nursing, developmental pediatrics, primary care,and speech-language pathology. However, all teammembers and primary care providers should be alerted

to the potential for difficulties in these areas, so whenproblems arise, appropriate referrals can be made.

Table 1 on the following page lists key psychosocialand developmental interventions by age.

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AGE INTERVENTION

Prenatal

Birth to1 month

2-15 months

16-24 months

2-5 years

6-11 years

12-21 years

Genetics consultation if ultrasound is abnormal, or parents have questions aboutrecurrence risks

Complete medical and family historyDysmorphology/genetics assessmentDiscuss prognosis and implications for treatmentAddress etiologyOffer family additional counseling and resources when appropriate

Discuss recurrence risks, prenatal diagnosis for clefts (ultrasound)

Consider genetic syndrome if developmental delays or other atypical features arepresent

Additional genetics workup as indicated

Consider genetic syndrome if developmental delays are present

Additional genetics workup as indicated

Consider genetic syndrome, especially if learning problems presentAdditional genetics workup as indicated

Revisit recurrence risk issues and offer formal genetics consultation

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Implicit in the choice of a surgeon for the childborn with cleft lip/palate is the understanding that thefirst surgeon to operate has the best opportunity for a

good outcome. Once crucial tissues are surgicallymanipulated or lost, it may be difficult to achieveoptimal results. With this information in mind, it is clearthat qualifications and expertise are of paramountimportance. They should include:

Board certification or board eligibility in plasticsurgery, otolaryngology, or oral and maxillo-facial surgery with explicit documentation oftraining in cleft care 9

A surgical caseload that ensures regularexperience in cleft lip/palate care

Affiliation with a cleft lip/palate teamrecognized by the State of Washington

Commitment to attend cleft lip/palate teammeetings and discuss surgical planning andoutcomes

Ongoing continuing medical education andexpertise in cleft lip/palate care

Table 3 lists key plastic surgery interventions byage.

25

AGE INTERVENTION

Prenatal

Birth to1 month

1-3 months

3-15 months

16 months-5 years

6-11 years

12-21 years

Meet parents and child, outlineplan

Meet parents and child, outlineplan

Consider presurgical orthopedicsin consultation with theappropriate dental specialist a

Monitor progress of presurgicalorthopedics with orthodontist

Repair cleft lip (and possiblenose), usually at 3-4 months b

Repair cleft palate, usually at9-15 months c

Monitor speech-languagedevelopment with speech-language pathologist (refer forspeech-language therapy asneeded)

Monitor for symptomaticfistulae

Consider prosthetic or surgical

management as needed forVPI d

Lip/nasal surgery as needed forresidual deformity

If VPI, consider sphincter,pharyngeal flap surgery orspeech prosthesis

Bone graft to alveolar cleft withclosure of oro-nasal fistulae e


Rhinoplasty as needed (nasalrevision)


Orthognathic surgery (seeAppendix XI, pg 35, superscript b)

PLASTICSURGERY

VII. Appendix

9Depending upon locale, surgeons from these

subspecialties may perform plastic surgery procedureson children with cleft lip/palate. In any case, the

particular education, training and experience of the

surgeon which qualifies him/her to perform these repairs

must be established. This should include documented

evidence of residency training (as an operating surgeon,

not as an assistant) in lip, palate and nasal procedures.

This cannot include patients treated on overseas

missions or treated for craniofacial trauma. (ACPA Team

Standards Self-Assessment Instrument, 1996)

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aAbout presurgical orthopedics

It is difficult to obtain a good lip repair if thecleft in the lip and alveolus is very wide, or if there is aprotruding premaxilla as in bilateral clefts. The lip andalveolar segments can be brought closer together orthe premaxilla moved to a more normal position

through an intervention calledpresurgical maxillaryorthopedics. This can involve the application ofexternal taping across the cleft, a surgically appliedinternal device, or a plastic molding device taped inplace. The specifics regarding the timing and nature ofthe orthopedic device vary from center to center.Potential advantages and disadvantages for a givenchild should be discussed with the cleft lip/palate teamat the time treatment is recommended.

bAbout cleft lip repair

If other medical factors are stable, cleft lip

repair is usually done when the child is between 3-4months old. Closure involves meticulous repair of theskin, muscle and mucosa of the lip. Correction of thecleft lip nasal deformity is usually done at the sametime, especially in the unilateral cleft. In wide clefts, apreliminary lip adhesion procedure is occasionally donefirst to mold alveolar ridges; the definitive repair isdone several months later.

cAbout cleft palate repair

The ideal time for palatoplasty is less clear.Theoretically, optimal speech is best served by earlierrepair, and optimal facial growth by later repair. Todaythe usual age for cleft palate repair is 9-15 months,which roughly corresponds to the emergence of earlyinfant speech. Closure of the palate (palatoplasty) iscomplex and often involves reorientation and closureof the layers of the soft palate, as well as tissues ofthe hard palate. This helps to minimize nasal airleakage and velopharyngeal insufficiency. Occasion-ally, the palate is closed in two stages, however, theremay be a higher risk of fistulae and speech problemswith this approach. The usual practice is to repair thepalate completely the first time. Pictures of typical

clefts are provided in Appendix XIII, pg. 39 and pg.40.

Some patients may have a submucous cleftpalate, which is more difficult to diagnose. In a submu-cous cleft of the soft palate, there is continuity of themucosa, but not of the underlying muscle. A submu-cous cleft palate is classically diagnosed by thepresence of a bifid (split) uvula, a tented central areain the soft palate, and a palpable notch at the back of

the hard palate. Since most individuals with submucouscleft palate are asymptomatic, this type of palatal cleftis repaired only when there are significant symptoms(speech and feeding problems, and ear infections).

dAbout treatments for VPI

Surgical intervention offers the possibility forlong-term improvement in speech for the child with

velopharyngeal insufficiency (VPI). Disadvantagesinclude: a significant risk for over-correction of the airleak leading to post-operative obstructive sleep apnea(OSA) and hyponasality. When these occur, additional

surgical modifications may be needed. Tailoring thesurgical intervention to match the size and characteris-tics of the velar gap as determined by the VPIworkup can lessen the likelihood of OSA. Speechprostheses (lifts or obturators) provide a non-surgicaloption for some patients, and may improve oralfunction enough to minimize the need for futuresurgical intervention. However, they are labor-inten-sive and require family commitment and child coopera-tion. (For a more complete discussion of VPI andobturators, see Appendix IX, pg. 30.)

eAbout alveolar bone grafting

Alveolar bone grafting is usually necessary toclose the residual bony cleft in the maxilla. Theseprocedures are performed by an oral and maxillofacialsurgeon or a plastic surgeon with special training/expertise in this area. See Appendix XI, pg. 35.

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Due to the abnormal anatomy of the palateand oropharynx, the incidence of recurrent middle eardisease in children with cleft lip/palate is very high (90-95 percent). While middle ear status is frequentlymonitored by the primary care physician, it is essentialthat a qualified otolaryngologist also be involvedbecause of the difficulty in diagnosing middle ear fluidreliably in infants and young children. Many of thesechildren will require one or more sets of ear tubes.

Some infants also have airway compromise(such as infants with Robin Sequence10). If placingthese infants in prone position is not sufficient toalleviate the respiratory distress, the placement of anasopharyngeal tube or a tracheotomy may be neces-sary. Still other children have obstructed breathingfollowing pharyngeal flap or sphincter pharyngoplasty.In such cases, additional studies such as apolysomnography (sleep study) may be needed.

Otolaryngologists also take part in the assess-ment and surgical management of velopharyngealinsufficiency. They perform the nasopharyngoscopywith the speech-language pathologist to assess the

velopharyngeal gap, and together they recommend tothe team the appropriate surgical or prosthetic inter-

vention. Depending on the otolaryngologists surgicalexpertise, they may then perform theintravelarveloplasty, sphincter pharyngoplasty, orpharyngeal flap to manage the VPI.

Participation of an experienced otolaryngolo-gist is essential for good team care. The otolaryngolo-gist must be familiar with the chronicity of the prob-lems associated with clefts, the unique aspects of cleftcare (such as the need to be cautious about adenoidec-tomy in patients with cleft palate), and the need forcoordination with other surgical procedures. As with allcleft lip/palate team specialists, qualifications andexperience of the otolaryngologist are important andshould include:

Board certification or board eligibility in

otolaryngology A surgical caseload that ensures regular

experience in cleft lip/palate care


Commitment to attend cleft lip/palate teammeetings, and to discuss surgical planning andoutcomes

Ongoing continuing medical education andexpertise in cleft lip/palate care

AUDIOLOGY

The chronic middle ear effusions and infec-tions experienced by the child with a cleft palate areoften associated with hearing loss. For this reason, it isessential that hearing be monitored regularly by aqualified audiologist. Hearing loss secondary to middleear disease is called a conductive hearing loss; senso-rineural hearing loss occurs in a very small number ofchildren with cleft lip/palate. Conductive hearing losssecondary to middle ear disease can vary in terms ofdegree and configuration. A persistent conductivehearing loss can adversely influence speech andlanguage, with consequences for cognitive develop-ment and psychological adjustment. Because of theunpredictable course of middle ear disease in youngchildren, the early and routine audiologic monitoring ofchildren with cleft palate is mandatory, and should

include the use of impedance audiometry(tympanograms). In addition, the American Academyof Pediatrics now recommends screening all newbornsfor hearing loss (with otoacoustic emissions/BAERs).

Table 4 on the following page lists key otolaryngol-ogy and audiology interventions by age.

27

10 Note: Robin Sequence: A large U-shaped palatal cleft

and a small lower jaw, which keeps the tongue in the

back of the mouth where it can interfere with breathing

and swallowing.

OTOLARYNGOLOGY

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11 Cleft lip/palate repairs are usually performed by plastic surgeons, but in some centers otolaryngologists or oral and

maxillofacial surgeons with special training perform these surgeries.

12 Adenoidectomy should be approached cautiously in the patient with a cleft palate as adenoids often play a role in

velopharyngeal competency, particularly the posterior and inferior portions of the adenoids. The cleft lip/palate team shouldreview these issues before making a final decision on adenoidectomy.

AGE INTERVENTION

Birth-1 month

1-4 months

5-15 months

16-24 months

2-5 years

6-21 years

If Robin Sequence or other syndrome, assess for airway problems and usepositioning, nasopharyngeal tube, or tracheotomy as needed

Assess middle ear status (fluid or infection)

Assess hearing (BAER/otoacoustic emissions)

Monitor airway status, intervene as neededAssess middle ear status: place ear tubes with lip repair if fluid persistentAssess hearing if not done already

Monitor airway after palate closure in Robin Sequence/other syndromesPlace ear tubes with palate repair11 if middle ear fluid present >three monthsMedical management for ear fluid or infections if tubes already presentAssess hearing with behavioral and impedance audiometry at 6-7 months of age, and

monitor at six month intervalsConsider amplification when indicated

Assess airway statusMonitor middle ear status at least every six months; place/replace ear tubes as

neededAssess hearing every six months

Assess airway status (rule out obstructive sleep apnea if small jaw, flat mid-face,large tonsils/adenoids, sphincter pharyngoplasty or pharyngeal flap present)

Deviated septum may require repairUse caution with adenoidectomy12

Monitor middle ear status every six monthsIf ears clear >18 months, consider removing ear tubesMonitor hearing every six months to age three years, then every 6-12 months as

neededNasal endoscopy with speech therapist to evaluate VPI (see Appendix 9, pg. 30)Consider surgical interventions for VPI (intravelarveloplasty, sphincter

pharyngoplasty or pharyngeal flap) usually by plastic surgeon or otolaryngologist

Assess airway status (rule out obstructive sleep apnea if small jaw, flat mid-face,large tonsils/adenoids, sphincter pharyngoplasty or pharyngeal flap present)

Deviated septum may require repairUse caution with adenoidectomy12

Monitor middle ear status every six months; chronic problems may necessitaterepeated ear tubes, tympanoplasty, mastoidectomy

Ear tube removal when appropriateNasal endoscopy with speech therapist to evaluate VPI

Consider surgical interventions to improve velopharyngeal function(intravelarveloplasty, sphincter pharyngoplasty or pharyngeal flap)Monitor hearing every 6-12 months until normal for two consecutive years

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VIII. Appendix

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In children with cleft palate, the eustachiantube often does not function properly, in part, becausemuscles responsible for opening the eustachian tubeare involved in the cleft. If the eustachian tube doesnot open effectively, then pressure changes occur andfluid may fill the middle ear space, which often leadsto an ear infection. With fluid or infection, the eardrumand the ossicle bones of the middle ear no longer

vibrate properly, hindering the transfer of sound fromthe air through these structures to the nerves of theinner ear. If the eustachian tube remains blocked or ifthe fluid persists long enough, the eardrum or theossicles may be permanently damaged. The functionof myringotomy tubes is to keep the middle ear aeratedand to prevent fluid from accumulating.

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VIII. Appendix

Anatomy of the Middle Ear

FIGURE1: THEANATOMYOFTHEEAR

Outer Ear

Middle Ear

MalleusIncus

Stapes Inner Ear

Ear Drum

Middle Ear Space

Eustachian Tube

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A cleft palate poses serious threats to speechdevelopment. In spite of surgical closure of the palate,many children remain unable to create adequate intra-oral pressure for normal speech. This is called

velopharyngeal insufficiency (VPI), and can usually beeliminated with prosthetic and/or surgical treatment toobturate (close) the site of air leakage. Speech andlanguage assessments should take place regularly asoutlined below by a certified speech-language patholo-gist with expertise in cleft lip/palate care. The teamspeech-language pathologist should also communicatewith school and outside clinicians, and monitor therapytaking place with professionals who are not part of theteam.

When VPI is significant and interferes with achilds communication, plans for a full VPI workupcan be made. This can take place when the child is oldenough to cooperate (usually 2-5 years old). Thisworkup should include an oral exam, analysis ofspeech articulation, videofluoroscopic speech studyand naso-endoscopy, and is generally conducted by ateam speech-language pathologist, otolaryngologist andradiologist. Occasionally, nasometric studies andaerodynamic measures are also used. When theresults of the VPI workup are available, recommenda-tions for surgery, speech prosthesis or speech therapycan be made by the team.

Some children with cleft lip/palate also have

an articulation and/or language disorder with or withoutVPI. Regular monitoring by a speech-languagepathologist will ensure that other speech and languagedisorders receive timely assessment and intervention.

Table 5 lists key speech-language interventions byage.

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TABLE 5: Key Speech-LanguageInterventions

IX. Appendix

SPEECHPROBLEMSINCHILDRENWITHCLEFTS

6-9 months

9-18 months

18-24 months

2-5 years

6-11 years

12-21 years

AGE INTERVENTION

Speech-language consultation toaddress abnormal speech patterns

Speech-language evaluation(>three months after palate repair)

Speech-language evaluation

Annual speech-languageevaluation

VPI workup if neededProceed with prosthetic or surgical

management (if child cannotcooperate, interventions may berecommended in the absence of afull workup)

If indicated, provide or refer forarticulation and/or languagetherapy

Communicate with school and/oroutside speech-language pathologist

Annual speech-language evaluationuntil involution of adenoids

VPI workup and interventions ifneeded

Provide or refer for speech-language therapy if needed

Communicate with school and/oroutside speech-language pathologist,and monitor progress of therapy

Speech-language evaluation every2-3 years or as needed

Speech-language therapy ifneeded

Communicate with school and/oroutside speech-language pathologistInterventions for VPI if needed

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About speech prostheses to treat VPI

A speech prosthesis is a removable appliancethat is attached to the teeth with wire clasps, and isfabricated by a dental specialist in consultation withthe speech-language pathologist. There are two typesof speech prostheses: lifts and obturators. A palatal lift

appliance lifts the palate in order to close thevelopharyngeal gap. An obturator closes thevelopharyngeal gap with a plastic bulb that matchesthe gaps size and shape. The design of the prosthesisis determined by the pattern of air leakage shown inthe VPI workup, and its fit is modified and fine-tunedas needed.

Advantages:

May decrease the development of VPI-relatedmisarticulations in young children, therebyallowing for normal speech development and

decreasing the need for long-term speechtherapy

In the young child, it allows the developmentof correct speech sounds, improves intelligibil-ity and may diminish behavior problems due tocommunication difficulties

The speech bulb is infinitely adjustable in sizeand shape, and requires minimal movement ofthe velopharyngeal structures to work well

It is removed at night so it does not causeobstructive sleep apnea

Prosthetic treatment provides a reversible trialfor controlling VPI before committing the childto a surgical procedure

A speech bulb may make the child a bettersurgical candidate by decreasing the variabilityand/or size of the VP gap

Some patients will not need permanentmanagement of VPI (either prosthetic orsurgical) following a course of prostheticmanagement

Prosthetic treatment makes control of VPIavailable to patients for whom surgery is notadvised

Disadvantages:

Requires parent commitment, patientcompliance and a professional skilled atworking with children

Initial desensitization needed to reduce gagreflex

Requires modification as the child grows

Requires many office visits for developmentand adjustment

Long-term presence of any removable dentalprosthesis makes oral hygiene difficult andincreases risk of periodontal problems

Can be lost or broken

About surgery to treat VPI

Refer to Appendix VII, pg. 25, for a discussionof the surgical management of VPI. Surgery providesa permanent treatment for VPI by reconfiguring thetissues of the velopharyngeal space so that the VP gapcan be reliably closed for speech sounds requiringclosure, but remain open for other speech sounds andfor breathing during sleep. The advantage of surgery isthe potential permanent resolution of VPI symptoms.

The disadvantage is the potential for over-correction,creating post-operative obstructive sleep apnea and/orhyponasal speech (i.e. not enough air flow through thenose during speech).

About the decision to treat VPI

The decision to treat a childs VPI should bemade through consultation among the child andparents, and the relevant members of the cleft lip/palate team. Recommendations for surgery, speechtherapy, a speech appliance, or some combination ofthese should be based on the needs of the individualchild.

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IX. Appendix

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Hard Palate

The hard palate is the bony roof of the mouth.

Soft Palate

The soft palate is the muscular extension of the hardpalate located at the back of the mouth. Movement ofthe muscles of the soft palate is essential for normalspeech and eustachian tube function. In infancy,closure of the velopharyngeal space by palatal muscu-lature is necessary for generating suction duringfeeding. Without adequate closure of this space, airand food escape through the nose.

Adapted with permission from Looking Forward: A Guide for Parents of the Child with CleftLip and Palate, (Mead Johnson, 1995).

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IX. Appendix

FIGURE2: ANATOMYOFTHEPALATE

Anatomy of the Roof of the Mouth

The hard and soft palates separate the nasal cavity from the mouth.

Soft palate open:Muscles relax for breathing andmaking certain sounds.

Soft palate closed:Muscles in the soft palate and throat sealoff the nasal cavity for swallowing foods

and liquids, and making certain speechsounds.

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Orthodontics (also known as dentofacialorthopedics) and dental care are integral parts of thehabilitation of the child with a cleft lip/palate. Theinterventions of the orthodontist are particularlycritical, and it is important that he/she have experiencein cleft lip/palate care. Qualifications of the orthodon-tist include:

Board certification or board eligibility inorthodontics

An orthodontic caseload that ensures regularexperience in cleft lip/palate care


Commitment to attend cleft lip/palate teammeetings and to discuss treatment plans and

outcomes Ongoing continuing education in cleft lip/palate

care

About orthodontics and dental care

Children with cleft lip/palate have both theusual childhood dental needs and special problemsarising from the clefts. Good dental care is essential.These children have an increased need for preventiveand restorative dental care due to underlying dentalanomalies and the use of braces and other orthodontic

appliances. Dental hygiene must be closely monitored.Unhealthy teeth and gums compromise laterorthodontic and surgical interventions, and maycontribute to low self-esteem.

Early involvement of the orthodontist isnecessary in the newborn period if presurgical ortho-pedics (external taping, internal appliance, or moldingdevice) are used. Later, the orthodontist monitors thedevelopment and eruption of teeth. There may bemissing, rotated, incorrectly shaped, extra or displacedteeth. Occasionally, extractions are needed.

The orthodontist also monitors facial growth

and obtains important dental records (x-rays, modelsand photographs) needed for planning and timinginterventions. Much of what the orthodontist does isorthopedicpositioning bony segments to provide theunderlying framework for the soft tissue. This isespecially true before important surgical proceduressuch as the alveolar bone graft or jaw advancement(see Appendix XI, pg. 35). Cleft repair without orth-odontic intervention at the proper stages will produceunstable, inferior results, with subsequent

tooth loss and inadequate chewing ability/jaw function.Proper orthodontic care enhances soft tissue repair,speech production, oral function and self-image.

Orthodontic treatment should be performed indiscreet phases with specific, limited objectives.Continuous active treatment from early yearsthrough permanent dentition should be avoided.

Neither the teeth nor the child can tolerate suchlengthy treatment. Sometimes orthodontic interventionsare carried out by community orthodontists in consulta-tion with the cleft lip/palate team orthodontist. Thisfacilitates community-based care while ensuringcoordination with surgical, speech and other treatmentsplanned by the team.

Table 6 on the following page lists key orthodontic

and dental interventions by age.

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ORTHODONTICS/DENTALMEDICINE

X. Appendix

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Birth-

1 month

1-4 months

5-24 months

2-5 years

6-11 years

12-21 years

TABLE 6: Key Orthodontic/DentalInterventions for the Childwith Cleft Lip/Palate

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Presurgical orthopedics a

Obtain dental models at lip repair

Parent teaching regardingoral hygiene, dental developmentand future treatment plans

Monitor eruption of teeth anddental hygiene

Orthodontic dental records (x-rays,photos) at 4-5 years of age inpreparation for evaluation of teethand cleft size

Monitor dental hygiene; provideappropriate preventive andrestorative care

Orthodontic records as needed todetermine timing of bone graft

Assist with speech prosthesis b, asneeded

Positioning of maxillary segmentsin preparation for alveolar bone graft

Recommend extractions as needed

Monitor dental hygiene; provideappropriate preventive andrestorative care

Assist with speech prosthesis asneeded

Monitor growth; maxillaryprotraction as needed

Dental records to monitor jawgrowth, dental development andbone graft

Braces for dental alignment asneeded

If retrusion is severe, combination ofjaw surgery and braces is neededIf not done earlier, alveolar bone

grafting in conjunction with jawsurgery 13

Prosthetic replacement of missingteeth as needed

Monitor dental hygiene; provideappropriate preventive and restorativecare

AGE INTERVENTION

aAbout presurgical orthopedics

If the cleft lip is very wide, or there is aprotruding or prominent premaxilla (as in bilateralclefts), the segments can be brought closer togetherand the premaxilla moved to a more normal position byuse of external taping across the cleft, an intra-oral

appliance placed anesthesia, or an extra molding devicetaped into place. To be effective, these interventions,calledpresurgical orthopedics,must occur in the firstweeks of

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