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## Complete Summary

#### GUIDELINE TITLE

Parameters for evaluation and treatment of patients with cleft
lip/palate or other craniofacial anomalies.

#### BIBLIOGRAPHIC SOURCE(S)

American Cleft Palate-Craniofacial Association. Parameters for
evaluation and treatment of patients with cleft lip/palate or other
craniofacial anomalies. Chapel Hill (NC): American Cleft
Palate-Craniofacial Association; 2000 Apr. 30 p. \[4 references\]

#### GUIDELINE STATUS

This is the current release of the guideline.

According to the guideline developer, this guideline has been reviewed
against current literature and is still considered to be current as of
2005.

### COMPLETE SUMMARY CONTENT

 SCOPE\
 METHODOLOGY - including Rating Scheme and Cost Analysis\
 RECOMMENDATIONS\
 EVIDENCE SUPPORTING THE RECOMMENDATIONS\
 BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS\
 QUALIFYING STATEMENTS\
 IMPLEMENTATION OF THE GUIDELINE\
 INSTITUTE OF MEDICINE (IOM) NATIONAL HEALTHCARE QUALITY REPORT
CATEGORIES\
 IDENTIFYING INFORMATION AND AVAILABILITY\
 DISCLAIMER

### SCOPE

#### DISEASE/CONDITION(S)

Craniofacial anomalies including:

-   Cleft lip and/or palate

-   Apert syndrome

-   Crouzon disease

-   Mandibulofacial dysostosis

-   Hemifacial microsomia

-   Other associated malformations

#### GUIDELINE CATEGORY

Counseling\
Evaluation\
Management\
Rehabilitation\
Treatment

#### CLINICAL SPECIALTY

Dentistry\
Family Practice\
Medical Genetics\
Nursing\
Otolaryngology\
Pediatrics\
Plastic Surgery\
Psychology\
Speech-Language Pathology\
Surgery

#### INTENDED USERS

Advanced Practice Nurses\
Allied Health Personnel\
Dentists\
Nurses\
Physicians\
Psychologists/Non-physician Behavioral Health Clinicians\
Public Health Departments\
Social Workers\
Speech-Language Pathologists

#### GUIDELINE OBJECTIVE(S)

-   To recommend practices in the care of patients with craniofacial
    anomalies

-   To update the October 1992 recommendations by the American Cleft
    Palate-Craniofacial Association

#### TARGET POPULATION

-   Neonates with craniofacial abnormalities

-   Patients of any age with craniofacial abnormalities

-   Family members and/or caregivers of patients with craniofacial
    abnormalities

#### INTERVENTIONS AND PRACTICES CONSIDERED

**Management by Interdisciplinary Teams**

1.  Determining composition of interdisciplinary team

2.  Ensuring qualifications of team member

3.  Assigning team responsibilities to ensure quality and continuity of
    patient care

4.  Developing treatment plans

5.  Establishing communication lines with patient and patient´s family

6.  Providing patient/family education

**Team Practices During Neonatal Period and Infancy**

1.  Performing first evaluation of child with congenital anomalies
    within first few day of life

2.  Monitoring child´s development through first year of life

**Longitudinal Evaluation and Treatment**

1.  Audiologic care

2.  Cleft lip/palate surgery (primary and secondary)

3.  Craniofacial surgery and maxillofacial surgery

4.  Dental care

5.  Genetic/dysmorphology services

6.  Nursing care and education of nurses in care of children with
    craniofacial anomalies

7.  Otolaryngologic care

8.  Pediatric care

9.  Psychological and social services

10. Speech-language pathology services

#### MAJOR OUTCOMES CONSIDERED

Not stated

### METHODOLOGY

#### METHODS USED TO COLLECT/SELECT EVIDENCE

Hand-searches of Published Literature (Primary Sources)\
Hand-searches of Published Literature (Secondary Sources)\
Searches of Electronic Databases

#### DESCRIPTION OF METHODS USED TO COLLECT/SELECT THE EVIDENCE

Not stated

#### NUMBER OF SOURCE DOCUMENTS

Not stated

#### METHODS USED TO ASSESS THE QUALITY AND STRENGTH OF THE EVIDENCE

Expert Consensus (Committee)

#### RATING SCHEME FOR THE STRENGTH OF THE EVIDENCE

Not applicable

#### METHODS USED TO ANALYZE THE EVIDENCE

Review

#### DESCRIPTION OF THE METHODS USED TO ANALYZE THE EVIDENCE

Not stated

#### METHODS USED TO FORMULATE THE RECOMMENDATIONS

Expert Consensus (Consensus Development Conference)

#### DESCRIPTION OF METHODS USED TO FORMULATE THE RECOMMENDATIONS

**Original Guideline**

The work to develop the original guideline began in May 1991 with a
consensus conference in which 71 individuals participated. The majority
of the participants were professionals experienced in the diagnosis and
treatment of craniofacial anomalies and related disorders. The remainder
of the participants were selected to represent patients and their
families, multilingual multicultural interests, and government agencies
involved in the funding of care for such patients. Following four days
of presentations by experts and discussion by participants, the
attendees voted by ballot on each of 386 resolutions that the grant
project committee had distilled from the written records of the
proceedings. The recommendations on which at least 75% of the conference
attendees concurred\* were included in a draft of a parameters of
practice document. Copies of the document were then distributed for both
select and wide spread peer reviews; subsequent revisions were made by
the committee in response to reviewers\' comments.

\*In a few instances in which the recommendation(s) involved
specialty-specific issues and individuals outside that specialty were
neutral in their responses, the recommendation(s) were based on at least
a 75% concurrence of individuals representing that specialty.

**Revision**

Suggestions for changes to the guideline document were reported to the
chair along with supporting documentation. The suggested changes were
then discussed among all appropriate committee members. If 75% or more
of the committee members agreed that the change(s) was indicated, the
proposed change was presented to the American Cleft Palate-Craniofacial
Association (ACPA) council. If the Council approves the change, it is
offered in the Spring issue of the ACPA/Cleft Palate Foundation (CPF)
Newsletter and presented at the next business meeting of the ACPA for a
vote by the members present. If 75% of those voting approve of the
change, it is incorporated in the next edition of the Parameters
document.

#### RATING SCHEME FOR THE STRENGTH OF THE RECOMMENDATIONS

Not applicable

#### COST ANALYSIS

A formal cost analysis was not performed and published cost analyses
were not reviewed.

#### METHOD OF GUIDELINE VALIDATION

External Peer Review\
Internal Peer Review

#### DESCRIPTION OF METHOD OF GUIDELINE VALIDATION

Copies of the parameters of practice document were distributed for both
select and wide spread peer reviews; subsequent revisions were made by
the committee in response to reviewers\' comments. The revised document
was returned to the participants in the consensus conference for
ratification and was subsequently approved as American Cleft Palate
Craniofacial Association policy by the Executive Council.

### RECOMMENDATIONS

#### MAJOR RECOMMENDATIONS

Several fundamental principles underlie the recommendations regarding
the optimal care of patients with craniofacial anomalies, regardless of
the specific type of disorder:

I.  Management of patients with craniofacial anomalies is best provided
    by an interdisciplinary team of specialists.

II. Optimal care for patients with craniofacial anomalies is provided by
    teams that see sufficient numbers of patients each year to maintain
    clinical expertise in diagnosis and treatment.

III. The optimal time for the first evaluation is within the first few
     weeks of life and, whenever possible, within the first few days.
     However, referral for team evaluation and management is appropriate
     for patients at any age.

IV. From the time of the first contact with the child and family, every
    effort must be made to assist the family in adjusting to the birth
    of a child with a craniofacial anomaly and to the consequent demands
    and stress placed upon the family.

V.  Parents/caregivers must be given information about recommended
    treatment procedures, options, risk factors, benefits, and costs to
    assist them in (a) making informed decisions on the child\'s behalf,
    and (b) preparing the child and themselves for all recommended
    procedures. The team should actively solicit family participation
    and collaboration in treatment planning. When the child is mature
    enough to do so, he or she should participate in treatment
    decisions.

VI. Treatment plans should be developed and implemented on the basis of
    team recommendations.

VII. Care should be coordinated by the team but should be provided at
     the local level whenever possible; however, complex diagnostic and
     surgical procedures should be restricted to major centers with the
     appropriate facilities and experienced care providers.

VIII. It is the responsibility of each team to be sensitive to
      linguistic, cultural, ethnic, psychosocial, economic, and physical
      factors that affect the dynamic relationship between the team and
      the patient and family.

IX. It is the responsibility of each team to monitor both short-term and
    long-term outcomes. Thus, longitudinal follow-up of patients,
    including appropriate documentation and record-keeping, is
    essential.

X.  Evaluation of treatment outcomes must take into account the
    satisfaction and psychosocial well-being of the patient as well as
    effects on growth, function, and appearance.

**Interdisciplinary Teams**

**Composition**

The staff of the interdisciplinary team may include individuals from the
following areas of professional practice: anesthesiology, audiology,
diagnostic medical imaging/radiology, genetic counseling,
genetics/dysmorphology, neurology, neurosurgery, nursing, ophthalmology,
oral and maxillofacial surgery, orthodontics, otolaryngology,
pediatrics, pediatric dentistry, physical anthropology, plastic surgery,
prosthodontics, psychiatry, psychology, social work, and speech-language
pathology. Consultation with other professionals may also be
appropriate. The specific staff will be determined by the availability
of qualified personnel and by the types of patients served by the team.
When the team cannot provide all of the types of examinations or other
services required by its patients, team members are responsible for
making appropriate referrals and for communicating with those to whom
patients are referred to facilitate the implementation of appropriate
and coordinated treatment plans.

**Qualifications of Team Members**

The paramount interest of both the Bureau of Maternal and Child Health
and the American Cleft Palate-Craniofacial Association is the quality of
care for patients. It is thus essential that all team members be trained
and experienced in the care of patients with craniofacial anomalies.
However, this document does not address the scope of practice of
individual professional specialties. The educational and experiential
requirements for the specialties represented on teams are variously
determined by their own specialty boards, professional associations,
state licensing boards, etc. These requirements are continually subject
to change. Each team must take responsibility for assuring that team
members not only possess appropriate and current credentials but also
have requisite experience in evaluation and treatment of patients with
craniofacial anomalies. Teams should assist members in keeping current
with their specialties by supporting and encouraging their participation
in continuing education activities and attendance at professional
meetings.

**Team Responsibilities**

The principal role of the interdisciplinary team is to provide
integrated case management to assure quality and continuity of patient
care and longitudinal follow-up. Each patient seen by the team requires
comprehensive, interdisciplinary treatment planning to achieve maximum
habilitation with efficient use of parent and patient time and
resources. Each interdisciplinary team should do the following:

-   Maintain an office with a secretary and/or coordinator and a listed
    telephone number.

-   Maintain centralized and comprehensive records on each patient,
    including histories, diagnoses, reports of evaluations, treatment
    plans, reports of treatment, and supporting documentation such as
    photographs, radiographs, dental models, and audiotaped speech
    recordings.

-   Designate a coordinator who facilitates the function and efficiency
    of the team, ensures the provision of coordinated care for patients
    and families, and assists patients and families in understanding,
    coordinating, and implementing treatment plans.

-   Designate a member(s) to make initial contact with the patient
    and/or family and also with direct care providers, as appropriate.

-   Evaluate patients at regularly scheduled intervals, the frequency
    and specific content of those evaluations being determined by the
    condition and needs of the patient and family.

-   Hold regularly scheduled face-to-face meetings for discussion of
    findings, treatment planning, and recommendations for each patient.

-   Develop a longitudinal treatment plan for each patient that is
    modified as necessitated by craniofacial growth and development,
    treatment outcomes, and therapeutic advances.

-   Weigh all treatment decisions against the expected outcomes and
    related factors such as facial growth, hearing, speech, dentition,
    and psychosocial impact on patient and family.

-   Communicate the treatment recommendations to each patient and family
    in written form as well as in face-to-face discussion.

-   Provide updated information to families as the treatment plan
    unfolds, and repeat information frequently enough to assure its
    assimilation.

-   Demonstrate sensitivity and flexibility in provision of care to
    accommodate linguistic, cultural, and ethnic diversity among
    patients and their families, ensuring that appropriate interpreters
    are available to assist in both verbal and written communication.

-   Assist families in locating resources for financial assistance
    necessary to meet the needs of each patient.

-   Communicate on a routine and ongoing basis with direct care
    providers in the home community, and invite these care providers to
    participate in team meetings involving their patients.

-   Perform regular, formal assessments of the quality of patient care
    with participation by each member of the team and, when appropriate,
    utilize external peer review. Teams should also conduct periodic
    surveys of patient satisfaction.

-   Maintain a reliable list of sources for any services that are either
    not provided by the team itself or are better provided at the
    community level.

-   Assist families in planning for treatment in a new geographic
    location by referring them to an interdisciplinary team in that
    area, and facilitate contact with the new team.

-   Provide assistance to adolescents and their families in planning for
    the termination of active treatment and offer information regarding
    the services that, if needed, will be available to them as adults.

-   Attempt to inform adult patients who may have completed treatment of
    new developments in diagnosis and treatment as they become
    available.

-   Promote early identification of children with craniofacial anomalies
    through programs designed to inform delivery room personnel,
    neonatal care personnel, and primary care providers in the community
    about these birth defects.

-   Provide educational programs for hospital personnel and primary care
    providers addressing feeding and other critical aspects of early
    health care for children with craniofacial anomalies.

-   Promote understanding of, and sensitivity to, the needs of patients
    by providing educational information about craniofacial anomalies
    and related disorders to parents and patients, to other professional
    people, and to the general public.

-   Promote understanding of, and sensitivity to, the concerns of both
    parents, recognizing that each parent may have separate concerns.

-   Aid in the formation and encouragement of parent-run support groups,
    and encourage cooperation with hospital visitation programs by
    trained volunteers.

**Neonatal Period and Infancy**

The optimal time for the first evaluations of a child with congenital
anomalies is within the first few days of life. Subsequent evaluations
should be scheduled at regular intervals, the frequency and specific
content of each of those evaluations being determined by the condition
and needs of the individual infant and family.

**Team Practices**

One or more members of the interdisciplinary team---typically the team
nurse---should assume responsibility for providing the following
services based on the needs of the infant:

-   Contacting the family immediately after referral to provide
    assistance with feeding and to offer other basic information, as
    needed, in conjunction with the primary care provider (i.e., the
    pediatrician or family physician)

-   Ensuring weekly assessment of nutritional intake and weight gain
    during the first month of life

-   Maintaining support for the family through home visits, telephone
    calls, or a visiting nurse program

-   Instructing caretakers of children with compromised airways in
    techniques of airway maintenance including positioning, nasogastric
    tube insertion, care of a tracheostomy, and apnea-bradycardia
    monitoring

-   Recommending or arranging for hearing assessment of the neonate as
    soon as possible

At the time of the infant\'s first visits, and based on the individual
needs of each infant, the team should:

-   Obtain a history of pre- and postnatal development.

-   Provide a full pediatric evaluation including nutritional and
    feeding assessments

-   Provide instruction and educational materials on feeding, and offer
    other information such as the availability of special feeding
    devices. (Note: the mother\'s choice of feeding method should be
    supported as long as that method sustains a normal suckle-swallow
    reflex and adequate nutrition is achieved.)

-   Perform a genetic/dysmorphology screening with subsequent provision
    of, or referral for, a complete genetic evaluation as necessary. If
    a recommendation is to be made for a full genetic evaluation and
    counseling, consideration should be given to the family\'s emotional
    status. If the family declines the recommendation of the team for
    genetic evaluation and/or counseling, this fact should be documented
    in the patient\'s record.

-   Arrange for any evaluations pertinent to the genetic/dysmorphologic
    diagnosis, including imaging studies, chromosome analysis,
    ophthalmological evaluation, etc.

-   Perform or arrange for an otolaryngologic evaluation.

-   Perform or arrange for an audiologic evaluation.

-   Instruct the caretakers regarding symptoms of otologic disease,
    hearing loss, and sleep apnea.

-   Arrange for cardiopulmonary assessments if the infant is at risk for
    respiratory obstruction or sleep apnea.

-   Offer information to parents, as appropriate, about normal speech
    and language development, speech and language impairments for which
    the child may be at risk, and ways in which they may facilitate
    speech and language development.

-   Conduct an assessment of prelinguistic speech-language development.

-   Conduct or arrange for a psychosocial interview of the family to
    obtain information regarding the family history and adjustment of
    the family to the child; to assess cultural and linguistic
    influences affecting the family; to identify sources of family
    support; and to identify financial needs and resources.

-   Provide examination by a dental specialist to evaluate factors that
    may influence surgical management, to assist in treatment planning,
    and to obtain baseline diagnostic records.

-   Provide counseling on care of the oral cavity and prevention of
    dental caries, especially nursing bottle caries.

-   Provide examination by a surgeon and consultation among team members
    and family members regarding management procedures and options.

-   Provide families with contacts to appropriate parent-run support
    groups.

The team should monitor the child\'s development throughout the first
year of life, and provide or refer for any required interventions. Areas
of specific concern include (a) height, weight gain, nutrition, feeding
disorders, and growth; (b) continuity of routine pediatric care,
including immunizations; (c) motor, cognitive, and social development;
(d) speech and language development; (e) otologic health; (f) hearing
status; (g) parent-child adaptation, parental skills, behavior
management, and nurturance; (h) the condition of the developing
dentition and supporting tissues, with counseling regarding early oral
hygiene and prevention of nursing caries; (i) the physical status of the
child as it pertains to readiness for surgical management; and (j)
modifications of the treatment plan as necessitated by any additional
information such as more complete genetic/dysmorphologic diagnoses or
new developments in family status.

**Longitudinal Evaluation and Treatment**

**Audiologic Care**

Individuals with craniofacial anomalies may have congenital
abnormalities of the auditory structures and are also subject to an
increased incidence of ear disease. These children are at high risk for
hearing disorders that may occur intermittently or become permanent, and
that vary from mild to severe. Hearing loss can have a significantly
adverse influence on speech and language development, educational and
psychological status, and eventually on social and vocational status.
For these reasons, children with craniofacial anomalies require ongoing
audiologic surveillance.

-   Each child should have an appropriate assessment of hearing
    sensitivity for each ear within the first three months of age.

-   The timing of audiological follow-up examinations should be
    determined on the basis of the child\'s history of ear disease or
    hearing loss. Audiological follow-up examinations should continue
    through adolescence.

-   Acoustic-immittance (tympanometric) measures should be obtained as a
    part of each audiological evaluation to monitor middle ear status.

-   All children undergoing myringotomies and placement of ventilating
    tubes should be seen pre- and postoperatively for audiologic
    assessment.

-   When a persistent hearing loss is identified, amplification (hearing
    aids, auditory training systems) should be considered.

-   When hearing loss occurs in the presence of microtia or atresia,
    whether unilateral or bilateral, bone conduction amplification
    should be considered depending upon degree of loss; an implantable
    bone conduction aid may be a treatment option.

-   Once amplification has been provided, a regular follow-up schedule
    is needed to monitor hearing thresholds and the function of the
    amplification system.

-   For any child with a documented hearing loss, referral should be
    made to the child\'s school district for appropriate educational
    services as soon as the hearing loss is identified.

-   In the absence of a positive history of otologic disease or hearing
    loss, audiologic examination or screening should still be carried
    out at least yearly through the age of six years to assure adequate
    monitoring of hearing.

**Cleft Lip/Palate Surgery**

In addition to primary surgical closure of the lip and palate, many
patients will require secondary surgical procedures involving the lip,
nose, palate, and jaws. These procedures usually are staged over a
period of several years. In all cases, surgical techniques should be
individualized according to the needs and condition of the patient.
Surgical procedures should be coordinated to minimize the number of
anesthetic exposures and hospitalizations. Evaluation of complications
(morbidity and mortality) of cleft lip and palate repairs should be
completed on an annual basis and subjected to peer review. The major
factor in the quality of surgical outcome is the skill, training, and
experience of the cleft care team.

*Primary Cleft/Lip Palate Surgery*

-   An anesthesiologist knowledgeable and experienced in pediatric
    anesthesia must be present for all surgical procedures involving
    children.

-   Surgical repair of the cleft lip is usually undertaken in the first
    six months of life and may be performed as early as is considered
    safe for the infant.

-   Presurgical maxillary orthopedics to improve the position of the
    maxillary alveolar segments prior to surgical closure of the lip may
    be indicated for some infants.

-   The nasal deformity is an integral part of the cleft lip. Depending
    on the severity, primary nasoplasty may be done at the time of the
    primary lip repair.

-   A preliminary lip adhesion is a procedure that may be used in
    selected patients preceding definitive lip repair.

-   The goal of lip repair is to restore the normal functional and
    anatomic features.

-   In the normally developing child, the palate should be closed by the
    age of 18 months and preferably earlier when possible.

-   The goal of palate surgery is to achieve normal function. Repair of
    the soft palate may include muscle reconstruction.

-   Patients with submucous clefts should be monitored closely and their
    palates repaired only if there is evidence of feeding, otologic, or
    speech problems.

*Secondary Cleft/Lip Palate Surgery*

-   Although rhinoplasty and nasal septal surgery are usually advocated
    only after completion of nasal growth, earlier intervention for
    reasons of airway problems or nasal tip deformity may be indicated.

-   Repair of the cleft lip nasal deformity can be accomplished with
    limited external incisions on the nose.

-   The timing of nasal surgery should be discussed with the patient and
    parents so that the goals are understood and expectations are
    realistic.

-   The patency of the nasal airway should be considered when planning
    either nasal reconstructive procedures or secondary velopharyngeal
    operations such as a pharyngeal flap or other type of
    pharyngoplasty.

-   Secondary palatal and pharyngeal surgery for velopharyngeal
    inadequacy should be performed only after evaluation of the
    velopharyngeal mechanism and review by the team.

-   Surgical or prosthetic closure of palatal fistulae may be needed if
    the fistulae are symptomatic.

-   The timing of bone grafting of the alveolar cleft should be
    determined by the stage of dental development. The graft should be
    placed before the eruption of the permanent maxillary teeth in the
    region of the cleft, although in late-presenting patients bone
    grafting may necessarily take place after full eruption of the
    permanent teeth. The timing of the operative procedure should be
    determined in collaboration with the orthodontist. Autogenous bone
    should be used when tooth movement through the graft is anticipated.
    The occlusion should be stabilized before materials that have
    potential to impede tooth movement are used.

-   In some instances, enlarged tonsils may interfere with
    velopharyngeal function, and a tonsillectomy may be indicated.

-   Tonsillectomy and/or adenoidectomy may be indicated to permit safe
    performance of a pharyngeal flap or other type of pharyngoplasty.

**Craniofacial Surgery and Maxillofacial Surgery**

The complex nature of many types of craniofacial anomalies often
necessitates multiple operative procedures at different stages of
development. Reduction of morbidity and mortality from craniofacial
operations requires establishment of a dedicated surgical team, frequent
performance of operative procedures, and adequate support facilities.
Longitudinal follow-up is necessary for these patients even when the
intervention has been successful with respect to the anatomical defect.

-   Initial evaluation of cranial vault anomalies should include a
    pertinent history and physical examination by team members who are
    specialists in genetics/dysmorphology, neurosurgery, ophthalmology,
    and craniofacial surgery, among others.

-   For patients with orbitocranial anomalies, including abnormalities
    of orbital position and shape and globe position, the initial
    evaluation and longitudinal follow-up is the same as that for those
    with isolated cranial vault defects.

-   The specific components of the pre-, peri-, and postoperative
    evaluations of craniofacial surgery should be based upon the type of
    anomaly and the craniofacial zone(s) affected.

-   Conventional skull radiographs may be used for screening. Computed
    tomography (CT) scanning and, when indicated, magnetic resonance
    imaging should be obtained. Such imaging techniques are to be used
    in preoperative planning and postoperative evaluations.

-   The timing of surgery, including distraction osteogenesis, for
    orbital craniofacial anomalies depends on associated soft tissue
    defects, functional impairments, and psychosocial concerns.

-   An anesthesiologist knowledgeable and experienced in pediatric
    anesthesia must be present for all surgical procedures involving
    children.

-   Appropriate intensive care facilities must be available for all
    patients undergoing craniotomy or any procedure that might
    compromise the airway.

-   Patients should be followed at regular intervals at least into
    adolescence to monitor cranial and facial growth, overall
    development, neurological status, ocular function, speech and
    hearing, and psychosocial adjustment.

-   For patients with microtia, surgical reconstruction of the external
    ear, auditory canal and middle ear are treatment options. The use of
    ear prostheses which may be attached with adhesive or
    osseointegrated implants is an alternative. The choice depends on
    the medical condition and patient preference. Use of osseointegrated
    implants, however, may compromise future surgical external ear
    reconstruction.

-   Secondary procedures are often necessary to correct residual
    deformities of the mandible, maxilla, orbits, zygoma, forehead, and
    nose.

-   Orthognathic surgery (and/or distraction osteogenesis) is indicated
    when orthodontic treatment cannot achieve functional and/or
    acceptable aesthetic occlusion and facial harmony. Such surgery
    should be timed to minimize any adverse effect on possible
    subsequent growth, and the timing should be determined in
    consultation with the team. Whenever possible, orthognathic surgery
    should be delayed until physical maturation is essentially
    completed. Earlier surgery may be indicated when there is
    compromised airway, jaw function, speech, or psychosocial
    adjustment. However, the patient and family must understand that
    additional procedures may be required to optimize the outcome.

-   If mandibular ankylosis is present, surgical release should be
    considered as soon as the condition is diagnosed. Both surgical
    intervention and subsequent physical therapy may be necessary to
    facilitate mandibular development, to improve the airway, or to
    ameliorate feeding difficulties.

-   In the case of surgical procedures that may alter dental occlusion,
    model surgery and prediction tracings should be completed in the
    treatment planning process.

**Dental Care**

Patients with craniofacial anomalies will require dental specialty
services as a direct result of the medical condition, and the dental
treatment is an integral part of the habilitative process. Provision of
dental services for these patients includes not only primary care but
routine maintenance throughout life. The necessary procedures focus on
monitoring craniofacial growth and development, on maintaining a healthy
dentition and periodontium, and on correcting jaw relationships and
dental occlusion when necessary in order to achieve optimal function and
appearance. In the interest of patient comfort, and thus in the interest
of future patient compliance, all efforts should be made to ensure that
dental and orthodontic procedures are as nontraumatic as possible.

-   Dental films, cephalometric radiographs, photographs, and computer
    imaging as indicated should be utilized to evaluate and monitor
    dental and facial growth and development.

-   For patients at risk for developing malocclusion or
    maxillary-mandibular discrepancy, diagnostic records including
    properly occluded dental study models should be collected at
    appropriate intervals.

-   As the primary dentition erupts, each team evaluation should include
    a dental examination and referral to appropriate providers for
    caries control, preventive measures and restorative dental
    treatment, and space management if such services are not being
    provided.

-   Before the primary dentition is completed, the skeletal and dental
    components should be evaluated to determine if a malocclusion is
    present or developing.

-   Depending upon the goals to be accomplished, and also upon the age
    at which the patient is first seen, orthodontic management of
    malocclusion may be performed in the primary, mixed, or permanent
    dentition. In some cases, orthodontic treatment may be necessary in
    all three stages.

-   While continuous active orthodontic treatment from early mixed
    dentition to permanent dentition should be avoided, each stage of
    therapy may be followed by orthodontic retention and regular
    observation; the orthodontic retention period for the permanent
    dentition may extend into adulthood.

-   For some patients with craniofacial anomalies, functional
    orthodontic appliance may be indicated.

-   For patients with craniofacial anomalies, orthodontic treatment may
    be needed in conjunction with surgical correction of the facial
    deformity.

-   Congenitally missing teeth may be replaced with a removable
    appliance, fixed restorative bridgework, or osseointegrated
    implants.

-   Patients should be closely monitored for periodontal disease.

-   Prosthetic obturation of palatal fistulae may be necessary in some
    patients.

-   A prosthetic speech appliance may be used to treat velopharyngeal
    inadequacy in some patients.

**Genetic/Dysmorphology Services**

A comprehensive clinical genetic evaluation is a key component in the
management of patients with congenital craniofacial anomalies and should
include (1) diagnosis, (2) recurrence risk counseling, and (3)
counseling regarding prognosis. Complex syndromes involving craniofacial
anomalies may not fully express clinical manifestations that can be
recognized in the first year of life. Thus, genetic screening and
follow-up evaluations will be necessary for some patients until puberty.
Patients who are first seen by the team at later ages should also be
evaluated.

-   Indications for referral for a complete genetic evaluation include,
    but are not limited to, (a) positive family history; (b) prenatal
    growth deficiency; (c) unexplained postnatal growth deficiency; (d)
    developmental delay or mental retardation; (e) associated major
    malformations and/or disorders; (f) associated minor malformations
    and/or disorders inconsistent with the genetic background; (g)
    family request; and (h) recognized genetic diagnosis.

-   For families who live in areas which are geographically remote, the
    team should arrange for referral to an accessible source where
    genetic services can be provided, and should encourage the family to
    follow through on the referral.

-   Personal genetic counseling should be offered to the maturing
    adolescent.

**Nursing Care**

Complex interdisciplinary management for individuals with congenital
craniofacial anomalies requires a high level of ongoing coordination of
services. The patient and family need appropriate information to
understand the rationale for, and implications of, such management.
Nursing assessment, interventions, and ongoing follow-up evaluations are
integral to the long term care needs of the child or individual with
congenital craniofacial anomalies and family. Services for the patient
and family include:

-   Feeding assessments, interventional teaching, and follow-up of
    nutritional and growth assessments

-   Serving as role models of acceptance and nurturance for the patient
    and family

-   Preparation of patients and families for what to expect when either
    in-patient or out-patient surgical procedures are scheduled.
    Information should be provided on pre- and postoperative feeding,
    the rationale for the use of restraints, special positioning and
    equipment, wound care, skin management, activity restriction,
    anticipated outcome or general status immediately following surgery,
    and the necessity for, and timing of, postoperative examinations.
    Instructions must be given in a manner that assures understanding on
    the part of the family and other caregivers.

In addition to the direct nursing services provided to patients and
families, members of the craniofacial team bear the responsibility for
education of hospital and community nurses in feeding and other aspects
of the special care required by children with craniofacial anomalies.

-   Ongoing educational programs for, and consistent liaison with, the
    nursing staff of hospitals in the community as well as in remote
    locations improve patient care and facilitate timely referrals for
    interdisciplinary patient management.

-   In addition to emphasizing procedures necessary for providing
    special care, instructional programs for nurses should stress the
    role of nurses as models of acceptance and nurturance in their
    interaction with patients and families.

-   Hospital nurses should be instructed in postoperative care (e.g.,
    arm restraints, feeding and hydration, airway maintenance, pain
    management, surgical site protection, patient and family adjustment,
    and instructions to be given to the family for home care).

**Otolaryngologic Care**

Comprehensive care of children with craniofacial anomalies typically
requires long-term monitoring of the ears, nose, and throat due to the
prevalence of ear disease, ear malformations, and upper airway problems.

-   Physical examination of the ears should be provided on a regular
    basis, beginning within the first six months of life and continue
    through adolescence.

-   Treatment of middle ear disease may include use of antibiotics,
    myringotomies with insertion of tympanotomy tubes, tympanoplasties,
    gel foam patches, removal of cholesteatomas, mastoidectomies, and
    middle ear reconstruction.

-   Any child with middle ear disease should be carefully followed
    during and after treatment to ensure a successful outcome and to be
    sure that tympanotomy tubes are removed if necessary.

-   Although some otolaryngologic operative procedures such as
    myringotomies may be classified as minor procedures, the potential
    psychological impact on the child and family attendant upon any
    surgical procedure must be taken into consideration, particularly
    when the child is subjected to repeated procedures. All procedures
    should be preceded by appropriate exchange of information with the
    patient and family, and every attempt made to allay fears regarding
    effects of anesthesia, the immediate post-operative course,
    post-operative precautions, etc.

-   Sequential airway assessments may be required to differentiate
    anatomical causes of airway difficulties from other causes (e.g.,
    allergies). Such assessments may require endoscopy, radiologic
    studies, airflow studies, computed tomography (CT) scans, magnetic
    resonance imagings (MRIs), and polysomnography.

-   Structural and functional laryngeal problems may exist in these
    patients and may require medical as well as surgical treatment.

-   An adenoidectomy and/or tonsillectomy as well as other oropharyngeal
    (tongue reductions, mandibular distraction, etc.) and
    laryngotracheal procedures may be indicated if the child is
    experiencing sleep apnea or other signs of airway obstruction.
    However, the team should provide a speech evaluation and assessment
    of the velopharyngeal mechanism prior to any decision to perform
    such a procedure.

**Pediatric Care**

Pediatric care provided within the context of the team is fundamental in
assuring that the health needs of the child with craniofacial anomalies
are fully identified and appropriately treated.

-   A primary care physician should be identified for each child, and
    should become an extended member of the team.

-   Physical examinations should be provided on a regular basis within
    the context of the team.

-   Parental questions regarding health issues should be addressed.

-   Parental understanding of the child\'s health needs and of the team
    treatment plan should be monitored.

-   When special needs are identified, referral to appropriate
    specialists should be made in cooperation with the primary
    physician. Family follow-through on these referrals should be
    monitored.

-   Frequent monitoring is required for children who may be at risk for
    growth failure, delayed development, abuse and neglect, or any other
    significant problem.

-   The child\'s health status should be evaluated prior to any surgical
    procedures that are planned.

**Psychological and Social Services**

The accomplishment of the goals of treatment of the patient with
craniofacial anomalies requires periodic assessment of the psychosocial
needs of both the patient and the family. The psychosocial interviewer
may come from the ranks of professionals such as those in social work,
psychology, pediatrics, nursing, and psychiatry. However, psychological
tests must be administered and interpreted under the supervision of a
licensed psychologist, preferably a person familiar with craniofacial
anomalies and related speech and hearing disorders.

-   Psychosocial screening interviews should be conducted periodically
    to assess parental competence and nurturance, child management
    skills, parent-child relationships, and the emotional and behavioral
    adjustment of the child. The high rate of learning disorders in
    children with craniofacial conditions requires that each child be
    screened for potential learning disorders beginning in infancy until
    cognitive competence has been established. Each family should
    receive or be referred for evaluation, guidance, and counseling when
    possible problems are identified through the screening process.

-   Parents should receive guidance regarding such problems as behavior
    management, teasing, rejection by other family members, public
    attitudes, fear of and expectations from surgical procedures, and
    emotional adaptation to treatment.

-   Screening evaluations should be conducted to identify possible
    problems in the child\'s cognitive development, behavior, self
    concept, educational progress, and psychosocial development. The
    screening evaluations should be conducted periodically, beginning in
    infancy and continuing through adolescence. When problems are
    suspected or identified in these areas, the child should receive or
    be referred for formal developmental/cognitive evaluations,
    guidance, counseling, or other assistance as needed.

-   When there is concern for possible developmental delay, repeated
    psychological evaluations are necessary to facilitate appropriate
    program planning and prevent inappropriate labeling of the child.

-   Information about learning performance, through liaison with
    schools, should be obtained periodically. When problems are
    suspected or identified, arrangements should be made for cognitive
    and educational assessments.

-   Children who have craniofacial anomalies may benefit from contacts
    with other children who have similar conditions. The team should
    facilitate contact with other children and their families through
    support groups and networking.

-   Social skills training programs should be available to help children
    and adolescents learn how to handle stressful social situations.

-   As they mature, children should be given information about their
    craniofacial anomaly and should be permitted and encouraged to
    become active participants in treatment planning. All care providers
    should be sensitive to how treatment discussions can be perceived by
    children, and should do everything possible to ensure that the child
    understands the treatment plan as much as possible. Towards this
    end, it is helpful for the team to ensure that each child has
    someone who will listen to his/her fears, concerns, and opinions
    regarding treatment.

-   Referrals for vocational training and guidance should be provided
    for adolescents, as appropriate.

**Speech-Language Pathology Services**

Children who have craniofacial anomalies are at high risk for
speech-language disorders. Evaluation of speech and language development
provides information that is needed by the team in planning of
treatment, including surgical, dental and speech management, and in the
assessment of treatment outcomes. Further, information about the
patient´s speech and language is important in the assessment of the
outcome of treatment. Speech-language evaluations should occur often
enough to assure adequate documentation of each child\'s progress and to
develop recommendations for intervention.

-   With the possible exception of children with cleft lip only,
    speech-language evaluations with appropriate documentation should be
    conducted for each child at least annually until the age of four
    years.

-   After the age of four years, even if speech-language development has
    been appropriate and no problems are noted, screenings should take
    place on a regular basis (preferably annually) until after adenoid
    involution, and at least every three years thereafter until dental
    and skeletal maturity are reached.

-   For children with problems in speech and language development,
    reevaluations should take place as often as deemed necessary by
    members of the interdisciplinary team in consultation with local
    care providers.

-   Evaluations should include perceptual assessment of laryngeal
    function.

-   When the development of speech and language skills is not at an
    age-appropriate level or when speech patterns are deviant,
    arrangements should be made for an early speech-language stimulation
    program or for speech-language remedial services.

-   For patients with craniofacial anomalies who are candidates for
    orthognathic surgery, pre- and postoperative perceptual speech
    evaluations are necessary.

-   Pre- and post-treatment speech assessments are necessary in
    determining candidacy for, and outcomes of, surgical, behavioral
    and/or prosthetic management of the velopharyngeal system.

-   Careful evaluation of velopharyngeal function is required for many
    patients. Procedures include in-depth analysis of articulatory
    performance, aerodynamic measures, videofluoroscopy,
    nasopharyngoscopy, and nasometric studies, all of which should be
    conducted with the participation of the team speech-language
    pathologist.

-   Remedial speech-language services will be needed for some patients
    with craniofacial anomalies.

-   Biofeedback therapy may be useful in treating some patients with
    velopharyngeal dysfunction.

#### CLINICAL ALGORITHM(S)

None provided

### EVIDENCE SUPPORTING THE RECOMMENDATIONS

#### TYPE OF EVIDENCE SUPPORTING THE RECOMMENDATIONS

The type of evidence supporting the recommendations is not specifically
stated.

### BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS

#### POTENTIAL BENEFITS

These recommendations may help improve care of patients with
craniofacial anomalies by:

-   Reducing diagnostic errors

-   Increasing recognition and treatment of the full spectrum of health
    problems associated with these anomalies

-   Reducing the incidence of unnecessary and poorly timed treatment

-   Reducing inappropriate or poorly performed procedures

#### POTENTIAL HARMS

Complications of surgical procedures for repair of craniofacial
anomalies

### QUALIFYING STATEMENTS

#### QUALIFYING STATEMENTS

-   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.

-   The clinical practices presented in this document reflect current
    knowledge and experience. Changes in these practices will be guided
    by advances in technology and research, including data on clinical
    outcome. Thus, timely review and revision of this document will be
    necessary.

### IMPLEMENTATION OF THE GUIDELINE

#### DESCRIPTION OF IMPLEMENTATION STRATEGY

**Quality Management**

The quality of care for patients with craniofacial anomalies and related
disorders must be carefully monitored by the team providing diagnosis,
treatment planning, and treatment services. This requires (1)
longitudinal assessments of the outcomes of treatment, (2) periodic team
review of the clinical outcome data, and (3) team adaptation of
treatment procedures when clinical outcome assessments do not reach
referenced criteria.

#### IMPLEMENTATION TOOLS

Patient Resources

For information about
[availability](#identifying-information-and-availability), see the
\"Availability of Companion Documents\" and \"Patient Resources\" fields
below.

### INSTITUTE OF MEDICINE (IOM) NATIONAL HEALTHCARE QUALITY REPORT CATEGORIES

#### IOM CARE NEED

Getting Better\
Living with Illness

#### IOM DOMAIN

Effectiveness\
Patient-centeredness

### IDENTIFYING INFORMATION AND AVAILABILITY

#### BIBLIOGRAPHIC SOURCE(S)

American Cleft Palate-Craniofacial Association. Parameters for
evaluation and treatment of patients with cleft lip/palate or other
craniofacial anomalies. Chapel Hill (NC): American Cleft
Palate-Craniofacial Association; 2000 Apr. 30 p. \[4 references\]

#### ADAPTATION

Not applicable: The guideline was not adapted from another source.

#### DATE RELEASED

2000 Apr (reviewed 2005)

#### GUIDELINE DEVELOPER(S)

American Cleft Palate-Craniofacial Association - Professional
Association

#### SOURCE(S) OF FUNDING

The Maternal and Child Health Bureau, Health Resources and Services
Administration, U.S. Public Health Service, Department of Health and
Human Services, Grant #MCJ-425074, \"Development of Standards for Health
Care of Infants, Children and Adolescents with Craniofacial Anomalies\"
awarded to the American Cleft Palate-Craniofacial Association.

#### GUIDELINE COMMITTEE

Not stated

#### COMPOSITION OF GROUP THAT AUTHORED THE GUIDELINE

*Grant Project Committee*: Betty Jane Philips, EdD, *Co-chair*,
Speech-Language Pathology, Cleft Palate-Craniofacial Program, Boys Town
National Research Hospital, Omaha, Nebraska; Donald W. Warren, DDS, PhD,
*Co-chair*, Prosthodontics/Prosthetics, Craniofacial Center, University
of North Carolina, Chapel Hill, North Carolina; Sally Peterson-Falzone,
PhD, *Document Editor*, Speech-Language Pathology, Center for
Craniofacial Anomalies, University of California, San Francisco,
California; Howard Aduss, DDS, MS, Orthodontics, Center for Craniofacial
Anomalies, University of Illinois, Chicago, Illinois; Alphonse R. Burdi,
PhD, Anatomy, Craniofacial Anomalies Program, University of Michigan,
Ann Arbor, Michigan; Albert H. Guay, DMD, Orthodontics, American Dental
Association, Chicago, Illinois; James A. Lehman, Jr., MD, Plastic
Surgery, Akron Craniofacial Center, Akron, Ohio; Don LaRossa, MD,
Plastic Surgery, Facial Reconstruction Center, Hospital of the
University of Pennsylvania, Philadelphia, Pennsylvania; Michael Lewis,
MD, Plastic Surgery, Cleft Palate Clinic, New England Medical Center,
Boston, Massachusetts; Nancy C. Smythe (ex-officio), Executive Director,
American Cleft Palate-Craniofacial Association, Pittsburgh, Pennsylvania

#### FINANCIAL DISCLOSURES/CONFLICTS OF INTEREST

Not stated

#### GUIDELINE STATUS

This is the current release of the guideline.

According to the guideline developer, this guideline has been reviewed
against current literature and is still considered to be current as of
2005.

#### GUIDELINE AVAILABILITY

Electronic copies: Not available at this time.

Print copies: Available from the American Cleft Palate-Craniofacial
Association, 1504 East Franklin Street, Suite 102, Chapel Hill, NC
27514-2820, Phone: (919)933-9044, Fax: (919)933-9604

#### AVAILABILITY OF COMPANION DOCUMENTS

None available

#### PATIENT RESOURCES

Cleft Palate Foundation Patient Publications can be accessed at:
[www.cleftline.org](http://www.cleftline.org/).

#### NGC STATUS

This NGC summary was completed by ECRI on September 24, 2004. The
information was verified by the guideline developer on October 12, 2004.

#### COPYRIGHT STATEMENT

This NGC summary is based on the original guideline, which is subject to
the guideline developer\'s copyright restrictions.

### DISCLAIMER

#### NGC DISCLAIMER

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Date Modified: 11/10/2008