- Commonest congenital abnormalities of the orofacial structure
- Cleft lip / palate occurs in 1:600 live births
- Isolated cleft palate occurs in 1:1000 live births
- Often occur as isolated deformities
- Can be associated with other anomalies (e.g. congenital heart disease)
- Cleft lips and palates are a diverse and variable congenital abnormality
- Cleft lip / palate predominates in males
- Isolated cleft palate is more common in female
Aetiology
- Cleft lip and palate is believed to have both a genetic and environmental component
- Cleft palate may be inherited as an autosomal dominant condition with variable penetrance
- Family history in a first-degree relative increases the risk by a factor of 20
- Environmental factors include:
- Maternal epilepsy
- Drugs - steroids, diazepam, phenytoin
- ? Folic acid deficiency
- Cleft lip and palate also occurs as part of over 100 syndromes
- Pierre Robin Syndrome - cleft palate, retrognathia, posteriorly displaced tongue
- Stickler Syndrome
- Down's Syndrome
- Treacher Collins' Syndrome
Embryology
- Cleft lip deformity is established in first 6 weeks of life
- Possibly due to failure of fusion of maxillary and medial nasal processes
- May be due to incomplete mesodermal ingrowth into the processes
- Extent of deficiency determines the extent of the cleft
- Palatal clefts result from failure of fusion of the palatal shelves of the maxillary processes
Clinical features
- Typical distribution of cleft types is :
- Cleft lip alone (15%)
- Cleft lip and palate (45%)
- Isolated cleft palate (40%)
- Cleft lips are more common on the left
Picture provided by Farhad Saleem, Azadi Hospital, Dubok, Iraq
Primary management of cleft lip and palate
- Antenatal diagnosis of cleft lip may be possible
- Feeding is rarely a difficulty
- Breast feeding may be achieved or modified teats for bottle feeding may be required
- Major respiratory obstruction is uncommon
- The aims of surgery are:
- To achieve a normal appearance of the lip, nose and face
- To allow normal facial growth
- To allow normal speech
Surgery
- Many different techniques have been advocated
- Cleft lip repair is usually performed between 3 and 6 months of age
- Cleft palate repair is usually performed between 6 and 18 months
- Two or more operations may be required
Secondary management of cleft lip and palate
- A multidisciplinary team approach is essential
- Other aspects that need to be addressed included
- Hearing
- Speech therapy
- Dental
- Orthodontics
Bibliography
Habel A, Sell D, Mars M. Management of cleft lip and palate. Arch Dis Child
1996; 74: 360-366.
Kirschner R E, LaRossa D. Cleft lip and palate. Otolaryngol Clin North Am 2000;
33: 1191-1215. |