Amniotic band syndrome and facial malformations

Amniotic band syndrome (ABS) is a collection of fetal congenital malformations affecting mainly the limbs, but also the craniofacial area. The pathogenic mechanism is still unclear. The facial malformations in ABS are multiple, polymorphous, and asymmetric. The type and extent of facial defects depend on band location and time of onset. Defects may include a combination of facial cleft, cleft palate and lip, ocular lesions, and cranial malformations. The management of this disease must be multidisciplinary and the outcome depends on the gravity of malformations.     

Velopharyngeal sequels in labial-alveolar-velopalatine clefts. Veloplasty and pharyngoplasty using an inferior or superior pedicle pharyngeal flap

Velopharyngeal insufficiency remains a sequel of labial-alveolar-velopalatine clefts. It may occur despite a good quality primary repair. A surgical management must be considered as soon as speech therapy is no longer efficient or before any irreversible compensatory speech pattern appears. Thus, surgery should be decided on after consultation between the surgeon and the speech pathologist or speech therapist, when considering that speech therapy has failed. Several surgical techniques are discussed: intravelar veloplasty, Furlow double-opposing Z-plasty, pharyngoplasty using an inferior or superior pedicle flap. Superior pedicle flap surgery is currently the most commonly used technique. For the past twenty years we have used this technique as described by Petit and modified by Malek, because of the excellent speech results. The drawbacks are known and can be contained by a preventive management.     

Velopharyngeal sequels in labial-alveolar-velopalatine clefts. Analysis, conclusions and speech therapy management

The association of voice and speech defines phonation. Phonation depends on various organs: the lungs for air, the larynx as a vibration generator, and cavities of resonance modeling articulation and tone. The role of the soft palate is important in French and for everyday conversation. Velopharyngeal incompetence is defined as a voice and articulation disruption by anatomic and/or functional deficiency between the soft palate and pharynx. Hypernasality is studied on an acoustic, phonetic, and vocal level and graded in various degrees of hypernasality. The various clinical and perceptive tests of velopharyngeal incompetence are described according to age. The difficulty to modelize hypernasality is discussed based on a recent study published in 2007. The differential diagnosis with hyper-closed nasality is discussed. The treatment for this condition is essentially speech therapy associated with pharyngoplasty in some cases. The assessment of children born with a labial-alveolar-velopalatine cleft is mainly clinical and perceptive. Therapeutic management is necessary for the child and his family. The follow-up requires a multidisciplinary approach.