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Cleft Lip and Palate - The Team Approach

 

 

 

Congenital malformations, including clefting, are abnormalities of prenatal origin that are present at birth. The causes of these problems fall into five major categories:

  • Chromosomal

  • Single gene disorders

  • Environmental factors (teratogens)

  • Multifactorial

  • Unknown

 

About one in every 700 – 1000 infants will have clefting of the lip or palate. This ratio can vary based on race and family. Predictions about recurrence risks for families can be made based on the condition and its etiology. Genetic counseling is highly recommended for parents of new cleft lip and palate infants.

 

Clefts of the lip and palate are classified into four major groups:

  • Cleft lip

  • Cleft palate

  • Unilateral cleft lip and palate

  • Bilateral cleft lip and palate

 

Cleft lip generally occurs at about the sixth to seventh week in utero. It is a result of the failure of the epithelial groove between the medial and the lateral nasal processes to be penetrated by the mesodermal cells.

 

Cleft palate is a result of epithelial breakdown at about the eighth week of embryonic development, with in growth failure of mesodermal tissue and lack of lateral palatal segment fusion.

 

Varying degrees of clefting can occur ranging from notching of the vermilion border, bifid uvula, soft palate clefting, alveolar clefting and bilateral complete clefts of the lip, alveolus and palate.

 

Corrective Surgery

Parents are often eager to have corrective surgery done. Most operations are performed during infancy and early childhood. Generally cleft lip repair is performed when the child is stable around 10 pounds, has hemoglobin of 10 mg per dl and/or is 10 weeks of age. Cleft palate closure can occur between 1 to 2 years of age and is often dependent on the severity of the cleft and surgeon’s preference. During early childhood, children will often require revision of the lip and possible pharyngeal flap surgery to assist with speech development.

 

Preventive dental services should begin early and a dental visit is recommended at the site of the first tooth or the infant becoming 1 year old. Scarring after lip and palatal closure procedures can inhibit growth of the dental arches. Orthodontic evaluation is recommended during the primary or mixed dentition. Orthodontic treatment can be intermittent for several years with the goal of establishing normal arch form. Orthograthic surgery is often recommended after growth has ceased for proper arch alignment, dental function and optimal facial aesthetics.

 

Early audiologic and speech evaluation is highly recommended. Chronic otitis media and low-frequency hearing loss are results of improper orientation of the Eustachian tubes and inserting muscles resulting in middle ear fluid stasis and retrograde infection.

 

Plastic surgical procedures to correct aesthetics and function of the vermillion border, lip, philtrum and nose are often recommended. It is important to remember that every child’s treatment plan is unique. Surgeons differ in their preferences for surgical techniques and sequence of procedures.

 

Feeding the Cleft lip/palate infant.

Feeding the cleft lip and palate infant poses challenges to the parents. These infants often have difficulty closing their mouth around the nipple of the mother or the bottle to make a seal. In addition these infants may have excessive air intake, nasal regurgitation and choking. Shorter and more frequent feedings may be necessary in the beginning because the baby tires easily. Special nipples and bottles are available that create an easy flow. A soft bottle can be squeezed gently during feeding to let the baby get enough formula.

Feeding position is important. The infant should be in a sitting or upright position tilted back just slightly to make it easier for the liquid to flow down into the throat. The baby’s head should be lined up with the body and not tilted forward or backward.

A feeding appliance may be a favorable option for babies that are having feeding problems. The feeding appliance functions as an obturator in the cleft area. Several benefits are improved sucking, less nasal regurgitation, elimination of naso-gastric tube feedings, and the guiding of dentoalveolar segments into normal anatomic position thus facilitating lip and palate surgical closure.

 

Velopharyngeal Incompetence

Clefts of the soft palate, including submucosal clefts and severe palatal clefting, can involve velopharyngeal incompetence and Eustachian tube dysfunction. During speech, the soft palate moves up meeting the back wall of the throat (posterior pharyngeal wall) and at the same time the muscles of the back and sides of the throat (the posterior and lateral pharyngeal walls) contract moving inward and upward to meet the soft palate. This action is called velopharyngeal closure. Velopharyngeal closure closes off the nose from the mouth (the nasal cavity from the mouth) during speech. When velopharyngeal closure is incomplete, the speech mechanism does not work properly. Air may escape through the nose and cause speech that is hypernasal.

 

Pharyngeal flap surgery may be recommended to correct hypernasal speech. A pharyngeal flap is a wide bridge of tissue that is constructed by the surgeon to separate the nose from the mouth. This helps reduce hypernasal speech by preventing most of the air used during speech from flowing out of the nose.

 

Dental Abnormalities

The prevalence of dental abnormalities associated with cleft lip and palate is considerable. Both the primary and permanent dentitions can be affected and abnormalities of tooth number, size, morphology, eruption and calcification have been described. The lateral incisor on the side of the cleft is often affected in some way and 50% of the time is congenitally missing. The prevalence of hypodontia in cleft lip and palate patients increases directly with the severity of the cleft. Supernumerary teeth are also common in the site of the cleft.

 

Cleft Palate Teams

An advantage of the team approach is that team members cooperate to develop a systematic, comprehensive treatment plan for the child during the course of their lives. Most major medical centers have cleft palate teams or a parent/physician can contact the Cleft Palate Foundation for information regarding the nearest cleft palate team.

Craniofacial and Cleft Lip/Palate Team Members - A multidisciplinary team approach: coordinator, parents, pediatrician, otolaryngologist (ear, nose and throat), audiologist, plastic surgeon, psychologist, social worker, speech pathologist, maxillofacial prosthodontist, oral and maxillofacial surgeon, orthodontist, pediatric dentist, geneticist, and dietician.

 

 

Reference

Regezi and Scuibba, WB Saunders, Second Edition, 1989.

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