Cleft lip and cleft palate
Cleft lip is one of the commonest congenital abnormalities and is also associated with cleft palate. A single factor in their development has not been established but in some there appears to be a genetic factor. In others there seems to be an association with excess alcohol consumption, smoking, drugs such as phenytoin, retinoids and cocaine, and low folic acid. As palate formation begins at the end of the fifth week of gestation, it is recommended that these risks be avoided and that folic acid (5mg) be taken daily prior to, and during, the first trimester of pregnancy.
Van der Woude syndrome
Varying degrees of cleft lip and palate occur. They have been classified into clefts of the soft palate alone, clefts of the soft and hard palate, complete unilateral clefts of the lip and palate and complete bilateral clefts of the lip and palate. The variation of Van der Woude syndrome is associated with submucous cleft palate and bilateral lower lip pits. In the case of this child, there were problems of nasal regurgitation of food while possetting (not feeding) and there were problems with the development of speech. Surgery was planned to correct the submucous cleft palate and revise the lower lip pits.
Surgical repair of cleft lip
Cleft lip and palate may cause problems with feeding. A specialist in feeding and swallowing disorders can advise on positioning the child and any equipment that may be required as an aid to feeding, such as special bottles and nipples. Most babies with this problem are bottle-fed but breastfeeding is not necessarily impossible. Generally it is better to feed the child in an upright position. Frequent burping is also recommended as babies with clefts tend to take in a lot of air when sucking. Early repair of the lip deformity is recommended, as in this child who underwent surgery at four months.
Complications of cleft lip and palate
Complications following cleft lip repair are unusual unless the wound is infected. In this case, scar revision was undertaken to improve the appearance of the scar. Cleft palate is more of a problem and may cause airway obstruction associated with posterior positioning of the tongue. Otitis media is a common problem in children with cleft palate. Following repair there is a risk of post-operative palatal fistula. It is recommended that major clefts of the palate are repaired before the child is a year old. About two in 10 children will have speech problems after palate repair.
Dental problems commonly occur in those with oral clefts. Teeth may be missing, malformed or there may be extra teeth, and the care of the dentist and orthodontist is paramount. Orthodontic treatment starts with the arrival of the second dentition, with oropalatal expansion to increase the width of the upper jaw. Later, extra teeth may require extraction, dental implants may be used to replace missing teeth and/or braces to straighten teeth. In some cases, orthognathic surgery is necessary to align teeth and help the upper jaw to develop.
TeamWork and emotional support
Most families faced with a child with cleft lip/palate will require support to be able to cope with anxieties and social embarrassment. Teamwork is essential from the beginning in order to plan the approach, prepare for surgery, achieve the best results and also to offer genetic counselling if the family are worried about the risk to further children. The plastic surgeon, ENT specialist, dentist, speech therapist, audiologist, social worker and geneticist must all be involved and a co-ordinator appointed to ensure continuity of care.
The family of this child stated that dental problems ran in the family. Both the mother and brother had the same problem of misplacement of the lower incisors. In most cases this is related to an inherited underdevelopment of the facial bones. Problems may arise as the result of malocclusion, and in some cases, speech problems. Referral to a dentist, orthodontist and faciomaxillary surgeon is important and may lead to the use of braces to straighten teeth or removal of teeth if overcrowding is a problem. Occasionally surgery may be necessary to reshape the jaw.
Hypodontia is the failure to develop teeth. It is a common problem, with up to a third of the population lacking wisdom teeth. In this case, the girl lacked upper lateral incisors. The cause is unknown but it is thought that genetic and environmental factors may be involved, as well as occurring in patients with ectodermal dysplasia and Down's syndrome. It is possible that hypodontia may be more common in those who develop epithelial ovarian carcinoma. Treatment of hypodontia is unnecessary unless appearance causes embarrassment. If necessary, false teeth can be fitted.