INTRODUCTION

In 1991, the American Cleft Palate-Craniofacial Association (ACPA) received funding from the Maternal and Child Health Bureau' to develop standards for the special needs of children born with cleft lip/palate and other craniofacial anomalies. Following a consensus conference and widespread peer review process, the Parameters for Evaluation and Treatment of Patients with Cleft Lip/Palate or Other Craniofacial Anomalies was developed and published. A summary of the recommendations of this report follows.

SUMMARY

PARAMETERS FOR EVALUATION AND TREATMENT OF PATIENTS WITH CLEFT LIP/PALATE OR OTHER CRANIOFACIAL ANOMALIES

The complex needs of a child with a cleft lip/palate or any other craniofacial anomaly are best met by a dedicated cleft or craniofacial team of health care providers with special expertise and experience in the care of such disorders. The professionals on these teams provide care regularly for a reasonable number of patients in a facility with the resources necessary for such care.

Audiologic Care

Individuals with craniofacial anomalies may have congenital abnormalities of the auditory structures and 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, these children require ongoing audiologic surveillance beginning at one month of age and continuing through adolescence. Tympanometric and audiometric measures should be obtained. Treatment may involve myringotomies and placement of ventilation tubes, hearing aids, auditory training systems, bone conduction amplification, and external, middle, and inner ear surgery.

Cleft Lip/Palate Surgery

In addition to primary surgical closure of the cleft lip and cleft palate which is initiated within the first 12 months of age, many patients will require secondary surgical procedures involving the lip, nose, palate, and jaws that usually are staged from infancy through adulthood. 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 subject to peer review. The major factor in the quality of surgical outcome is the skill, training, and experience of the cleft care team. An anesthesiologist knowledgeable and experienced in pediatric anesthesia must be present for all surgical procedures. Additional procedures for cleft lip/palate may include pre-surgical maxillary orthopedics, lip adhesion, pharyngoplasty, tonsillectomy/adenoidectomy, orthodontic maxillary expansion, alveolar bone grafting, nasal reconstruction including nasal septal surgery, and jaw surgery in conjunction with preand postoperative orthodontics.

Craniofacial Surgery and Maxillofacial Surgery

The complex nature of many types of craniofacial anomalies often necessitates multiple operative procedures at different stages of growth and 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, at least into adolescence, even when the intervention has been successful with respect to the anatomic defect. 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. Treatment involves reconstructive surgery to the craniofacial region, and may include hard and soft tissue remodeling, reconstruction, grafting, distraction, and implantation. Secondary procedures are often necessary due to the effects of growth, attainment of maturity or persistent residual deformities. An anesthesiologist knowledgeable and experienced in pediatric anesthesia must be present for all surgical procedures.

Dental Care

Patients with craniofacial anomalies require dental services as a direct result of the medical condition, and 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 dental 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. Dental care begins once the primary dentition erupts and continues into adolescence. Treatment requires diagnostic records (study models, cephalometric radiographs, photographs, and, at times, computer imaging) to monitor dentofacial growth and dental development, preventive and restorative dental treatment, and, when indicated, orthodontic treatment, alveolar bone grafting, orthognathic (jaw) surgery, and prosthetic and periodontal interventions.

Genetic/Dysmorphology Services

A comprehensive clinical genetic evaluation is a key component in the management of patients with congenital craniofacial anomalies and should include diagnosis, recurrence risk counseling, and 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.

Nursing Care

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 the family. Assessments of feeding, interventional teaching, and follow-up of nutritional and growth assessments are examples of the nursing services provided. In addition to these direct nursing services, members of the craniofacial team bear the responsibility for education of hospital and community nurses in feeding and other aspects of the special care that may be required.

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 examinations and airway assessments begin perinatally for patients with airway compromise or within the first 6 months of life for those with stable airways and continue through adolescence. Such assessments may require endoscopy, radiologic studies, airflow studies, CT scans, MRI's, and polysomnography. Structural and functional laryngeal problems may exist in these patients, and may require medical as well as surgical treatment. Adenoidectomy and/or tonsillectomy may be indicated for airway obstruction but only after careful velopharyngeal functional assessment. Other oropharyngeal and laryngotracheal procedures may be necessary as well.

Pediatric Care

Pediatric care provided within the context of the team, along with a primary care physician, is fundamental in assuring that the health needs of the child with craniofacial anomalies are fully identified and appropriately treated. Physical examinations should be provided on a regular basis, parental questions regarding health issues should be addressed, and referrals to appropriate specialists should be made in cooperation with the primary physician.

Psychological and Social Services

Accomplishing the goals for treatment of the patient with craniofacial anomalies requires periodic assessment of psychological needs of both the parent and family. 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 be referred for psychological evaluation and, as appropriate, intervention when problems are identified through the screening procedures.

Speech-Language 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 the planning of treatment, including surgical, dental, and speech management, and in the assessment of treatment outcome. Speech-language evaluations should occur within the first year of life, and then often enough to assure adequate documentation of each child's progress and to develop appropriate recommendations for intervention. Evaluation of velopharyngeal function, conducted with the participation of the team speech-language pathologist, is required for many patients and may include articulatory performance, aerodynamic measures, videofluoroscopy, nasopharyngoscopy, and nasometric studies.

¹Maternal and Child Health Bureau, Title V, Social Security Act, Health

Resources and Services Administration, U.S. Public Health Service,

Department of Health and Human Services, Grant #MCJ-425074.

Copies of the entire document, Parameters for Evaluation and Treatment of Patients with Cleft Lip/Palate or Other Craniofacial Anomalies may be obtained by contacting:

American Cleft Palate-Craniofacial Association
1829 East Franklin Street, Suite 1022
Chapel Hill, North Carolina 27514
(919)933-9044
Fax (919)933-9604

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