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Cleft palate incomplete closure of the roof of the mouth is present in the majority of patients. Hearing loss and speech difficulty are often associated with PRS. Mechanical basis[ edit ] The physical craniofacial deformities of PRS may be the result of a mechanical problem in which intrauterine growth of certain facial structures are restricted, or mandibular positioning is altered. This compression of the chin interferes with development of the body of the mandible, resulting in micrognathia.
The concave space formed by the body of the hypoplastic mandible is too small to accommodate the tongue, which continues to grow unimpeded. With nowhere else to go, the base of the tongue is downwardly displaced, which causes the tip of the tongue to be interposed between the left and right palatal shelves. This in turn may result in failure of the left and right palatal shelves to fuse in the midline to form the hard palate.
Later in gestation at around 12 to 14 weeks , extension of the neck of the fetus releases the pressure on the mandible, allowing it to grow normally from this point forward. At birth, however, the mandible is still much smaller hypoplastic than it would have been with normal development. After the child is born, the mandible continues to grow until the child reaches maturity. Genetic basis[ edit ] Alternatively, PRS may also be caused by a genetic disorder.
In the case of PRS which is due to a genetic disorder, a hereditary basis has been postulated, but it usually occurs due to a de-novo mutation. The infant usually has respiratory difficulty, especially when supine. The palatal cleft is often U-shaped and wider than that observed in other people with cleft palate. Management[ edit ] The goals of treatment in infants with PRS focus upon breathing and feeding, and optimizing growth and nutrition despite the predisposition for breathing difficulties.
If there is evidence of airway obstruction snorty breathing, apnea, difficulty taking a breath, or drops in oxygen , then the infant should be placed in the sidelying or prone position, which helps bring the tongue base forward in many children.
In nasopharyngeal cannulation or placement of the nasopharyngeal airway or tube , the infant is fitted with a blunt-tipped length of surgical tubing or an endotracheal tube fitted to the child , which is placed under direct visualization with a laryngoscope, being inserted into the nose and down the pharynx or throat , ending just above the vocal cords.
This tube or cannula, which itself acts as an airway, primarily acts as a sort of "splint" which maintains patency of the airway by keeping the tongue form falling back on the posterior pharyngeal wall and occluding the airway, therefore preventing airway obstruction, hypoxia and asphyxia. Nasopharyngeal airways are not available at every center; however, when available, nasopharyngeal cannulation should be favored over the other treatments mentioned in this article, as it is far less invasive; it allows the infant to feed without the further placement of a nasogastric tube.
This treatment may be utilized for multiple months, until the jaw has grown enough so that the tongue assumes a more normal position in the mouth and airway at birth, the jaws of some infants are so underdeveloped that only the tip of the tongue can be seen when viewed in the throat. Some institutions discharge the infant home with a nasopharyngeal tube in place. Enlargement of the lower jaw brings the tongue forward, preventing it from obstructing the upper airway.
The process of DO begins with preoperative assessment. They may then select the most appropriate distraction device or sometimes have custom devises fabricated. When possible, intraoral devices are used. DO surgery starts with an osteotomy surgical division or sectioning of bone followed by the distraction device being placed under the skin and across the osteotomy. A few days later, the two ends of the bone are very gradually pulled apart through continual adjustments that are made to the device by the parents at home.
This gradual distraction leads to formation of new bone between the two ends. After the process is complete, the osteotomy is allowed to heal over a period of six to eight weeks. A small second surgery is then performed to remove the device. In many centres there is now a cleft lip and palate team comprising these specialties, as well as a coordinator, a speech and language therapist, an orthodontist, sometimes a psychologist or other mental health specialist, an audiologist, and nursing staff.
The glossoptosis and micrognathism generally do not require surgery, as they improve to some extent unaided, though the mandibular arch remains significantly smaller than average. In some cases jaw distraction is needed to aid in breathing and feeding. Lip-tongue attachment is performed in some centres, though its efficacy has been recently questioned.
A cleft palate PRS or not makes it difficult for individuals to articulate speech sounds, which may be due to the physical nature of cleft palate or the hearing loss that is associated with the condition. Hearing should be checked by an audiologist regularly and can be treated with hearing amplification such as hearing aids. Because middle ear effusion is found in many patients with PRS, tympanostomy ventilation tubes are often a treatment option.
However, it has been found internationally that children with PRS are often slightly below average size, raising concerns of incomplete development due to chronic hypoxia related to upper airway obstruction as well as lack of nutrition due to early feeding difficulties or the development of an oral aversion.
However, the general prognosis is quite good once the initial breathing and feeding difficulties are overcome in infancy. Most PRS babies grow to lead a healthy and normal adult life. The most important medical problems are difficulties in breathing and feeding. Affected infants very often need assistance with feeding, for example needing to stay in a lateral on the side or prone on the tummy position which helps bring the tongue forward and opens up the airway.
Babies with a cleft palate will need a special cleft feeding device such as the Haberman Feeder. Infants who are unable to take in enough calories by mouth to ensure growth may need supplementation with a nasogastric tube. This is related to the difficulty in forming a vacuum in the oral cavity related to the cleft palate , as well as to breathing difficulty related to the posterior position of the tongue.
Given the breathing difficulties that some babies with PRS face, they may require more calories to grow as working of breathing is somewhat like exercising for an infant. Infants, when moderately to severely affected, may occasionally need nasopharyngeal cannulation , or placement of a nasopharyngeal tube to bypass the airway obstruction at the base of the tongue. Sometimes endotracheal intubation or tracheostomy may be indicated to overcome upper respiratory obstruction.
In some centers, a tongue lip adhesion is performed to bring the tongue forward, effectively opening up the airway. Mandibular distraction can be effective by moving the jaw forward to overcome the upper airway obstruction caused by the posterior positioning of the tongue. Given that some children with PRS will have Stickler syndrome , it is important that children with PRS be evaluated by an optometrist or ophthalmologist.
Because the retinal detachment that sometimes accompanies Stickler syndrome is a leading cause of blindness in children, it is very important to recognize this diagnosis.
Epidemiology[ edit ] The prevalence of PRS is estimated to be 1 in 8, to 14, people.
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Cleft palate incomplete closure of the roof of the mouth is present in the majority of patients. Hearing loss and speech difficulty are often associated with PRS. Mechanical basis[ edit ] The physical craniofacial deformities of PRS may be the result of a mechanical problem in which intrauterine growth of certain facial structures are restricted, or mandibular positioning is altered. This compression of the chin interferes with development of the body of the mandible, resulting in micrognathia. The concave space formed by the body of the hypoplastic mandible is too small to accommodate the tongue, which continues to grow unimpeded. With nowhere else to go, the base of the tongue is downwardly displaced, which causes the tip of the tongue to be interposed between the left and right palatal shelves. This in turn may result in failure of the left and right palatal shelves to fuse in the midline to form the hard palate.
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