cleft lip and palate.pdf

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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

Fourth Edition, Revised 6/2006


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1997, 2003, 2006 Childrens Hospital and Regional Medical Center, Seattle, Washington. All rights reserved.

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 6/06:

Anne Hing, MD

Marsha Ose, RN, BSN, MS

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

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

26

28

31

34


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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.)

36

38

40

42

44

45


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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 preauthorizevisits 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 servicescan be facilitated by any Children with Special HealthCare Needs Coordinator at local public health depart-ments or by the cleft lip/palate team coordinator. Nofurther 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 as guidelinesonly. 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 CleftPalate Story; and Daryl Tong for the medical illustra-tions on pages 40 and 41.

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.

American Cleft Palate-Craniofacial Association, 20043Team Standards Self-Assessment Instrument. American Cleft

Palate-Craniofacial Association, 1996.

4Standards of Care for Cleft Lip and Palate. AmericanAssociation of Oral and Maxillofacial Surgeons, 1995.

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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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 growthBegin presurgical orthopedics if indicated

Monitor feeding and growthRepair cleft lipMonitor ears and hearingBegin/continue presurgical orthopedics if indicated

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

Prenatal

Birth-1 month

1-5 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. 31.

I.

3

OVERVIEW: SUMMARY OF KEY INTERVENTIONS BY AGENote: 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


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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, 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 issuesBegin presurgical orthopedics if indicated


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. 34)

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. 28)

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 5 months:Monitor feeding and growthRepair cleft lipConsider ear tubesBegin/continue presurgical orthopedics if indicated

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 (ear tubes may be placed with liprepair if indicated)

BAER and evoked otoacoustic emissions (if not alreadydone)

Monitor for airway problems if diagnosis is Robin Sequenceor other syndrome (see Appendix VIII, pg. 28)

Repair cleft lip, usually at 3-5 months (cheiloplasty, seeAppendix VII, pg. 26)

May include primary nasal reconstruction (rhinoplasty)

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 month

intervals thereafterMonitor for airway problems if diagnosis is Robin

Sequence or other syndrome (see Appendix VIII, pg. 28)

Repair cleft palate, usually at 9-15 months (palato-plasty, see Appendix VII, pg. 26)

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. 31)

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 effusionspersist 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. 29)

Assess speech-language, monitor for velopharyngealinsufficiency (VPI)

Formal VPI workup, as indicated (see Appendix IX,pg. 31)Consider treatment options (speech therapy, surgery

or 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 (see

Appendix VIII, footnote 12, pg. 29)

Assess speech-language; monitor for velopharyngeal

insufficiency (VPI)Formal VPI workup as indicated (see Appendix IX, pg. 31)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. 29)

Assess speech, rule out velopharyngeal insufficiency (VPI)

Workup VPI if indicated (see Appendix IX, pg. 31)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 speechprosthesis 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. 26VIII, pg. 28

XI, pg. 36

VIII, pg. 28

IX, pg. 31

X, pg. 34


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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 principlethatmanagement 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. 45).

1. ACPA Parameters of Care (2004)

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 few

days 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 Teams if 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 maynotinclude 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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Additional Criteria:Cleft lip/palate team must meet30 of the following:

1. The team has a speech-language pathologistwith education, training and experience intreatment of cleft lip/palate who attends teammeetings.

2. The team has at least one speech-languagepathologist who evaluates at least 10patients/year with cleft lip/palate.

3. The team speech-language pathologistperforms structured speech assessment duringteam evaluations.

4. Clinical speech instrumentation (e.g.videofluoroscopy, endoscopy, etc.) is used toassess velopharyngeal dysfunction (VPI).

5. The team has an orthodontist with education,training and experience in treatment of cleftlip/palate who attends team meetings.

6. The team has at least one orthodontist whoprovides care for at least 10patients a yearwith cleft lip/palate.

7. Patients requiring orthognathic treatment (jawsurgery) are referred to an orthodontist withthe education, training and experience forprovision of orthodontic care as a part oforthognathic treatment.

8. Orthognathic surgical treatments are ad-equately documented with intra-oral dentalcasts, facial and intra-oral photographs, andappropriate radiographs.

9. Orthognathic surgical planning and outcomesare discussed at team meetings.

10. The team has or refers to a pediatric/generaldentist/prosthodontist with education, trainingand experience in dental management of cleftlip/palate.

11. The team has a surgeon who attends meetingswith education, training and experience intreatment of cleft lip and palate.

12. The team has a psychologist, social worker orother mental health professional who evaluates

all patients on a regular basis.13. The team routinely tests or screens patients

for learning disabilities, and developmental,psychological and language skills.

14. When indicated, the team collects schoolreports and other learning information.

15. The team has a nurse or other professional toprovide supportive counseling and feedinginformation.

16. When requested by the family, the team refersto parent support groups in the community.

17. The team provides pre- and post-operativesupportive counseling and instruction toparents and patients.

18. The team provides formal genetic counseling

or clinical genetic evaluation.19. Hearing is tested by an audiologist before the

child is one year of age.

20. The team has an otolaryngologist witheducation, training and experience in treatmentof cleft lip/palate.

21. The team evaluation includes an ear exam byan otolaryngologist on a routine basis beginningbefore one year of age.

22. After team evaluation, the patient and familyhave an opportunity to ask questions anddiscuss the treatment plan.

23. The team routinely prepares summary lettersor reports containing the treatment plan to besent to the family in a timely fashion.

24. Treatment plan reports are sent to the patientscare providers in the community in a timelyfashion (with parental permission).

25. The team records include diagnosis.

26. The team records include complete medicalhistory.

27. The team records include plan or treatmentgoals, which are reviewed regularly.

28. The team records include a social andpsychological history.

29. The team records include dental andorthodontic findings and history.

30. When indicated, the team makes intra-oraldental casts on patients.

31. The team takes facial photographs on patientsin treatment or evaluation.

32. When indicated, the team takes appropriateradiographs including lateral cephalograms.

33. The team has an office and a coordinator.

34. The team supports, encourages or offerscontinuing medical education in cleftlip/palate care to members.

35. The team provides case management (follow-up, referral, coordination of care) and providesadvocacy and assistance, as needed.

14

I. Appendix


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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 lip is increasingly common. However, itis easy to miss cleft lip on diagnostic ultrasounds,particularly those performed for routine indications inthe physicians office as the American Institute ofUltrasound in Medicine does not even require views ofthe lip on screening ultrasounds.7 A wide range offetal cleft lip detection rates (between 13-63%) havebeen reported with rountine antenatal 2D ultrasounds.8

In the United Kingdom, routine views of the face andlips were added to antenatal ultrasound guidelines in2000 and detection rates of cleft lip in low risk popula-tions increased from 16-33% to 75% with 2D ultra-

sound between week 18-23 gestation.9

The use of 3Dultrasound of the face improves detection rate signifi-cantly.10,11 Thus, if there is a family history of cleftingor if there is a concern about a possible cleft for otherreasons, a referral should be made for a completediagnostic ultrasound (including 3D images if possible)and genetic counseling. Ultrasounds obtained during18-24 weeks gestation have been most accurate.However, if patients are scanned earlier, especially ifamniocentesis is being considered (typically performedat 15-17 weeks), a later scan can be performed ifthere are concerns about a possible cleft. Ultrasoundcan often establish whether a cleft lip is unilateral or

bilateral. It is still very difficult to make the diagnosisof a cleft palate antenatally, unless it is detected inassociation with a large cleft lip. Reported detectionrates for cleft palate only vary from 0-22%.12,13

Recently, fetal MRI has been used to detect fetalabnormalities including cleft palate. Experience andavailability of fetal MRI, however, is extremely limitedat this time.14,15

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

family 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 be

formulated. 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 American Institute of Ultrasound in Medicine Guidelines, 2003.

8 Johnson, N. & Sandy, J. (2003). Prenatal diagnosis of cleft lip

and palate. Cleft Palate-Craniofacial Journal, 40, 186-189.

9 Wayne, C., Cook, K., Sairam, S., Hollis, B., Thilaganathan, B.

(2002). Sensitivity and accuracy of routine antenatal

ultrasound screening for isolated facial clefts. British Journal

of Radiology, 75, 584-589.

10 Chmait, R., Pretorius, D., Jones, M., Hull, A. James, G.,

Nelson, T., Moore, T. (2002), Prenatal evaluation of facial

clefts with two-dimensional and adjunctive three-dimensional

ultrasonography: a prospective trial. American Journal of

Obstetrics & Gynecology, 187, 946-949. 11 Tonni, G. Centini, G., Rosignoli, L. (2005). Prenatal

screening for fetal face and clefting in a prospective study on

low-risk population: can 3- and 4- dimensional ultrasound

enhance visualization and detection rate? Oral Surgery Oral

Medicine Oral Pathology Oral Radiology Endodontics, 100,

420-426.

12 Cash, C., Set, P., Coleman, N. (2001). The accuracy of

antenatal ultrasound in the detection of facial clefts in a low-

risk screening population. Ultrasound in Obstetrics and

Gynecology, 18, 432-436.

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 thissummary:

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

16

III. Appendix

NURSING, COORDINATIONOFCAREANDFEEDINGISSUES

13 Hanikeri, M., Savundra, J., Gillett, D., Walters, M., McBain, W.

(2006). Antenatal transabdominal ultrasound detection of cleft lip

and palate in Western Australia from 1996 to 2003. Cleft Palate-

Craniofacial Journal, 43, 61-65.

14 Ghi, T., Tani, G., Savelli, L., Colleoni, G., Pilu, G., Bovicelli, L.

(2003). Prenatal imaging of facial clefts by magnetic resonance

imaging with emphasis on the posterior palate. Prenatal

Diagnostics, 23, 970-975.

15 Kazan-Tannus, J., Levine, D., McKenzie, C., Lim, K.-H., Cohen,

B., Farrar, N., Busse, R., Mulliken, J. (2005). Real-time magnetic

resonance imaging aids prenatal diagnosis of isolated cleft palate.

Journal of Ultrasound Medicine, 24, 1533-1540.


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III. Appendix

for 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, close

attention 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 is

adequate 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.

palate prevents the infant from creating aclosed system in his/her mouth, and makes itimpossible for the milk to be pulled out. Theinfant will look like he/she is sucking, but he/she will be using up precious calories in afutile attempt to 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 PalateNurser 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. Thesecond 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 are

available 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 theabsence of normal infant sucking. The need


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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. Robin Sequence consists ofmandibular hypoplasia (micrognathia),glossoptosis, and a posterior U-shaped cleftpalate which results in a posterior tongue positionthat can interfere with breathing. This constella-tion of findings was first reported by the French

stomatologist, Pierre Robin. 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 experienced otolaryngolo-

gist if any of these interventions are needed.

Some of these babies may require prolonged useof the NP tube, a tracheotomy, or early mandibu-lar distraction.

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. Consideration of supple-mental nasogastric tube feedings or gastrostomytube feedings may be necessary for some infants.Many factors may contribute to failure to thrivein these babies: difficulty coordinating suck/swallow; inefficiency of feeding with the cleftpalate; glossoptosis with increased work ofbreathing; and caloric consumption. These issuescan be difficult to resolve, and generally require

the coordinated efforts of pediatrics/primary care,otolaryngology, nursing, occupational therapy andrespiratory therapy. Occasionally, apolysomnogram (sleep study) with CO

2monitor-

ing may be necessary to determine if ventilation isadequate. These issues are best addressed with acleft lip/palate team and in a hospital where thereis access to pediatric anesthesia. In the event of arespiratory emergency, these babies can be verydifficult to intubate because of their abnormalanatomy.

3. Middle Ear Effusions. Infants with cleft palate

are 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 thedifficulty of reliably diagnosing middle eareffusions in infants, it is recommended that anotolaryngologist periodically evaluate thesechildren (see Appendix VIII, pg. 28).

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,

18

PEDIATRICSANDPRIMARYCARE

IV. Appendix


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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 of0.4mg of folic acidthe amount in most

prenatal 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 AppendixVI, pg. 23.)

5. Dental Issues. Dental issues are of paramountimportance in the management of patients withcleft lip/palate. Unfortunately, dental care is oftenviewed as optional and not included in typicaldefinitions of medical necessity, which is

particularly 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 initial

assessment during the first few weeks of life. Second, good oralhygieneis essential for

successful cleft habilitation. Thus once theteeth have erupted, preventive counselingshould take place regarding baby bottle caries,proper tooth brushing, etc. The PCP has animportant 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 extend

through 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. Orthodonticinterventions 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.

34; Appendix XI, pg. 35.)

6. Development.Development should bemonitored 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 children

and 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 bedifficult 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 of

immunizations, 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 thosewho look or sound different from their peers.

19

IV. Appendix


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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

20

PSYCHOSOCIALANDDEVELOPMENTALISSUESINCLEFTCARE

V. Appendix


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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.

21

V. Appendix


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Birth to1 month

1-15 months

16-24 months

2-5 years

6-11 years

12-21 years

AGE INTERVENTION

Assessment of grief and loss issuesIdentify and validate other concernsAssess family functioning: recognize strengths, weaknesses, cultural differencesAssess familys understanding of medical information

Help incorporate family needs into treatment planMake appropriate community referrals

Follow-up on psychosocial needs of familyCheck family arrangements for surgical stays (lip and palate repairs)Address family stresses surrounding surgeryEnsure family understands post-op care needsReview financial issues

Review familys experiences with hospital and surgeryExplore how parents believe child is perceived by others because of appearance/

speech differencesScreen for developmental problems; make referrals if appropriate

Review family functioningReview issues surrounding future pregnancies, including the availability of genetic

counseling and prenatal ultrasound, and pre-conceptual folic acid supplementationAt school entry, review concerns related to speech, appearance differences andpeer acceptanceScreen for developmental/behavioral problems; refer if appropriateAssess familys understanding of team treatment plan including management of

speech problemsTalk directly with child to assess his/her concerns

Review family function and new stressesAssess family need for community resources and help getting to medical

appointmentsAssess childs fears and concerns before surgeries and hospital stays, especiallybefore bone graftAssess childs concerns related to peer acceptance, speech and facial differencesModel/refer for social skills training, if neededScreen for learning/behavioral disorders; refer as appropriateAcknowledge childs evolving role in the decision-making processReview plans requiring high patient/family compliance (e.g. orthodontic interventions,

obturation), including financial issues and family and childs ability to follow throughwith treatment

Acknowledge teens evolving role in the decision-making process

Assess teens fears and concerns before surgeries/hospital staysCheck for unrealistic expectations of surgeryAssess teens concerns related to peer acceptance, speech and facial differencesModel/refer for social skills training if neededScreen for school problems; review academic/vocational plansAssess psychosocial adjustment of teen and possibility of depression, substance

abuse, etc.; make referrals as needed.Assess teen and family understanding of recurrence risks, need for additional genetic

counseling

22

V. Appendix


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A dysmorphology or genetics assessment ispart of the complete evaluation of every child with acleft. Cleft lip and palate affects approximately 1:1000

Caucasian, 1:500 Asians, and 1:2000 African Ameri-cans in this country. Although the majority of patientswith cleft lip and palate are otherwise healthy, approxi-mately 25% have associated birth defects/chromo-somal abnormality, or a genetic syndrom.16Althoughthere are more than 400 syndromes reported inassociation with cleft lip or cleft palate17 the followingthree syndromes should receive special consideration.Velocardiofacial syndrome, due to a deletion ofchromosome 22q11.2, should be considered in childrenwith velopharyngeal insufficiency, submucous cleftpalate, or cleft palate. Van der Woude syndrome, anautosomal dominant condition, should be considered in

a child with either cleft lip/palate or cleft palate whohas a family history of mixed clefting in which eitherthe child or another family member has lower lip pits.Stickler syndrome, an autosomal dominant disorder ofcollagen with variable congenital myopia, clefting, andarthropathy, should be considered in all infants withRobin sequence.

A complete medical history should be obtainedon every child with a cleft, including a detailed prenatalhistory, teratogenic exposures, and a three-generationfamily history. This family history should includeoccurrences of clefting (and lower lip pits), hypodontia,

other birth defects, developmental disabilities or knowngenetic syndromes. A complete physical examinationby clinical geneticist or dysmorphologist should bepursued to identify dysmorphic features and/or associ-ated birth defects or medical concerns. Children whohave a family history of mixed cleft types (both cleftlip and cleft palate in different family members) shouldbe evaluated for hypodontia, lip pits, and ansomia.Additional studies, including opthalmologic consultation,echocardiography, or other radiographic studies, andlaboratory studies (such as chromosome analysis)should be directed by the examination and familyhistory to facilitate syndrome/chromosomal diagnoses.

These conditions may have prognostic implications thatmust be taken into account to help guide medicaldecisions.

Parents typically have many questions aboutthe etiology of clefts to be addressed by the cleft lip/palate team. There is considerable cultural and socialvariability in family attitudes towards birth defects andtheir causation. These issues should be explored and,when appropriate, correct information supplied,

recognizing that western medical information will notnecessarily supplant other cultural and ethnic beliefs.Since genetic factors play a role in clefting conditions

even in the nonsyndromic child, information on causa-tion and empirical recurrence risks should be providedto all families with clefts based upon the family history.For parents with one affected child, the recurrencerisk for future pregnancies is 2-5%. This risk in-creases if there are additional family members withclefts. Condition-specific recurrence risks and prena-tal testing options should be provided to families of achild with syndromic clefting condition.

Parents should be informed of the option ofultrasonography for future pregnancies. A discussionregarding the sensitivity of prenatal ultrasound to

detect clefts should be considered given that only 22%of cleft palates, 67% of cleft lip without cleft palate,and 93% of cleft lip with cleft palate can be detectedon antenatal ultrasound studies between 18-24 weeksgestation if appropriate facial views are obtained.18

Similarly, a discussion regarding the potential preventa-tive role of preconception/prenatal folate supplementa-tion and avoidance of environmental risk factors(tobacco smoke, alcohol, and isotretinion) should beconsidered.

Ideally, a genetics evaluation should beconsidered at several points. After a prenatal diagnosis

of cleft lip/palate, the family should be referred for agenetics evaluation and a complete diagnostic ultra-sound. If appropriate, amniocentesis or other tests maybe ordered. Preliminary genetics counseling shouldstress that diagnosis and risks of recurrence cannot beaccurately discussed until after the baby is born andexamined. At this time, families should also be referredto a cleft lip/palate team for discussion of managementissues and formulation of a feeding plan.

If the diagnosis of a cleft lip/palate is made inthe newborn period, a prenatal and family historyshould be taken, the infant examined for dysmorphicfeatures and genetic counseling offered. When theinitial crisis has subsided (generally after six months), itis appropriate to bring up risks of recurrence again.Parents also can be informed of the possibility ofultrasonography for future pregnancies. If a formalgenetics evaluation has not previously taken place, itshould be offered now.

23

VI. Appendix

GENETICS/DYSMORPHOLOGY


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VI. Appendix

The possibility of a genetic condition should also beconsidered as the child matures, because facialmorphology changes with growth. In addition, develop-mental problems and learning disorders may notsurface until later. At adolescence, risks of recurrenceshould be revisited with both the patient and family.Because of the rapid change in genetic informationand technology, all families with adolescents should beoffered the opportunity to have their concerns ad-dressed in a formal genetics consultation. If adysmorphologist or geneticist is not a member of thecleft lip/palate team, the possibility of an outsideconsultation should be discussed, and a referraloffered. Additional psychosocial support also may beneeded at these times, as parents may have difficultycoping with the provided information.

Table 2 on the following page is a summary ofgenetic and dysmorphology interventions by age.

16 Wyszynski, D. (2002). Cleft Lip and Palate: From Origin to

Treatment. New York: Oxford University Press.

17 Online Mendelian Inheritance in Man (OMIM)- database of

syndromes associated with cleft palate.

www.ncbi.nlm.nih.gov/entrez/query.fcgi?CMD=search&DB=omim

(Search syndromes with cleft palate or cleft lip)

18 Cash, C., Set, P., Coleman, N. (2001) The accuracy of antenatal

ultrasound in the detection of facial clefts in a low-risk screening

population. Ultrasound in Obestetrics and Gynecology, 18, 432-

436.


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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 etiology

Offer 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 arepresentAdditional genetics workup as indicated

Consider genetic syndrome if developmental delays are presentAdditional 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

VI. Appendix

25


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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 19

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.

26

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, outlineplanConsider presurgical orthopedics

in consultation with theappropriate dental specialist a

Monitor progress of presurgicalorthopedics with orthodontist

Repair cleft lip (and possiblenose), usually at 3-5 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

Consider prosthetic or surgicalmanagement as needed for VPId

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


Rhinoplasty as needed (nasalrevision)


Orthognathic surgery (seeAppendix XI, pg 36, superscript b)

PLASTICSURGERY

VII. Appendix

19Depending 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(Latham device)20, or a plastic moldingdevice taped in place (Nasoalveolar molding de-vice)21,22. The specifics regarding the timing and natureof the 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 liprepair is usually done when the child is between 3-5months 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. In wide clefts, some surgeons first do a prelimi-nary lip adhesion procedure to mold alveolar ridges,and the definitive repair is done several months later.

For the child who has had nasoalveolarmolding, there is the additional possibility of primaryclosure of the alveolar cleft using the technique ofgingivoperiosteoplasty (GPP). In a certain percentageof children undergoing this procedure, later alveolarbone grafting may not be needed.

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 of

the 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 typicalclefts are provided in Appendix XIII, pg. 40 and pg.41.

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 submucous cleft palate is classically diagnosed by thepresence of a bifid (split) uvula, a tented central area inthe soft palate, and a palpable notch at the back of thehard palate. Since most individuals with submucous cleftpalate are asymptomatic, this type of palatal cleft isrepaired only when there are significant symptoms(feeding problems, speech difficulties, and ear infec-tions).

dAbout treatments for VPI

Surgical intervention offers the possibility forlong-term improvement in speech for the child withvelopharyngeal insufficiency (VPI). Surgical optionsinclude palatal lengthening to achieve VP closure. If thisis not sufficient, a sphincter pharyngoplasty or pharyn-geal flap may be considered. Disadvantages include: asignificant risk for over-correction of the air leak leadingto post-operative obstructive sleep apnea (OSA) andhyponasality. When these occur, additional surgical

modifications may be needed. Tailoring the surgicalintervention to match the size and characteristics of thevelar 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 oral functionenough to minimize the need for future surgical interven-tion. However, they are labor-intensive and requirefamily commitment and child cooperation. (For a morecomplete discussion of VPI and obturators, see Appen-dix IX, pg. 31.)

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. 36.

fAbout jaw surgery

Plastic-craniofacial surgeons as well as oral-maxillofacial surgeons may do orthognathic surgery.Refer to Appendix XI, pg. 36, for discussion of jawsurgery.

20 Millard, D. & Latham, R. (1990). Improved primary surgical and

dental treatment of clefts. Plastic and Reconstructive Surgery, 86,

856-871.21 Grayson, B. & Cutting, C. (2001). Presurgical nasoalveolar

orthopedic molding in primary correction of the nose, lip, and alveolus

of infants born with unilateral and bilateral clefts. Cleft Palate-

Craniofacial Journal, 37, 193-198.22 Santiago, P., Grayson, B., Cutting, C. (1998). Reduced need for

alveolar bone grafting by presurgical orthopedics and primary

gingivoperiosteoplasty. Cleft Palate-Craniofacial Journal, 35, 77-80.

27

VII. Appendix


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Infants with Robin sequence (cleft palate,micrognathia, and glossoptosis) frequently have upperairway compromise. If placing these infants in a proneposition is not sufficient to alleviate the respiratory

distress, the placement of a nasopharyngeal tube ortracheotomy may be necessary. Mandibular distrac-tion is an emerging treatment option for infants withsignificant airway compromise to avoid tracheotomy.In such cases, additional studies including: 1)nasendoscopy/laryngoscopy to assess airway, or 2)polysomnography (sleep study) to determine severitymay be indicated.

On some teams, otolaryngologists repair thecleft palate (Appendix VII, pg. 27). Otolaryngologistsalso take part in the assessment and surgical manage-ment of velopharyngeal insufficiency. They performthe nasopharyngoscopy with the speech-languagepathologist to assess the velopharyngeal gap, andtogether they recommend to the team the appropriatesurgical or prosthetic intervention. Depending on theotolaryngologists surgical expertise, they may performa palate lengthening procedure, pharyngeal flap, orsphincter pharyngoplasty to manage the VPI (Appen-dix VII, pg. 27). Some children develop obstructedbreathing following pharyngeal flap or sphincterpharyngoplasty. In such cases, polysomnography maybe indicated.

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.

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 all cleft lip/palate team specialists, qualifica-tions and experience of the otolaryngologist areimportant and should include:

Board certification or board eligibility inotolaryngology

A surgical caseload that ensures regular

experience in cleft lip/palate care Affiliation with a cleft lip/palate team

recognized by the State of Washington

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 shouldinclude the use of impedance audiometry(tympanograms). In addition, the American Academyof Pediatrics now recommends screening all newbornsfor hearing loss (with otoacoustic emissions/BAERs),and ongoing hearing assessments (as described above)in high risk children including those with a cleft.23,24

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

28

OTOLARYNGOLOGY

VIII. Appendix

23 Cunningham, M. & Cox, E. (2003). Hearing assessment in

infants and children: recommendations beyond neonatal screening.

Pediatrics, 111, 436-440.24 Joint Committee on Infant Hearing. (2000). Year 2000 position

statement: principles and guidelines for early hearing detection and

interventi on. Pediatric s, 106, 798-817.


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

maxillofacial surgeons with special training perform these surgeries.26 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

1-5 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, tracheotomy, or mandibular distraction as neededAssess middle ear status (fluid or infection)

Assess hearing (BAER/otoacoustic emissions)

Monitor airway status, intervene as neededAssess middle ear status: ear tubes may be placed with lip repair if indicatedAssess hearing if not done already

Monitor airway after palate closure in Robin Sequence/other syndromesPlace ear tubes with palate repair25 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

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