Lingual and Labial Movement of Pierre-Robin Syndrome Patients Treated by Beverly Douglas Procedure

Abstract

In 1923 Pierre Robin (6) described a condition in young children in which the tongue assumed a posterior position in the oral cavity because of hypoplasia of the mandible. Robin described associated difficulties of feeding and respiration. Since his writings, other information has been made available about the syndrome which bears his name. The Pierre-Robin Syndrome is usually thought to consist of a small jaw (micrognathia) , a falling backward and downward on the tongue (glossoptosis); and often an isolated cleft palate. In normals, according to Douglas (2) and Steigrad (9), the attachment of the genioglossus muscle and the frenulum of the tongue to the mandibu— lar symphysis is the primary means by which the tongue is held forward. Goldberg (3) states that the genioglossi pull the tongue forward, thus overcoming the backward and downward pull of the hyoglossal muscle and the upward and backward pull of the styloglossal muscle. In micro— gnathia the tongue receives little, if any, support from the genioglossi attachments since these attachments are much more posteriorly placed. The tongue, consequently, acts as a ball valve and presses on the epiglottis, allowing expiration but preventing inspiration of air (2, 3, 7, .9, I2). Ac— cording to Steigrad (9), and others (2, 3, 5, 6, 10), respiratory and feeding difficulties such as cyanosis, malnutrition, sternal retraction, pneumonia, or even death from suffocation may therefore occur. A number of procedures have been utilized in treating glossoptosis of the tongue. Robin (6) utilized an intraoral supporting device called a monobloc. Goldberg (3) has described the Eley and Farber extraoral brace technique as well as the traction method of Callister. Davis and Dunn (1) have reviewed the bottle guard which is used as an attachment on the ordinary nursing bottle.