Pierre Robin Syndrome

CHAPTERS

Introduction

Clefts of the Lip and Palate

Ear Reconstruction

Craniosynostosis

Orbital Reconstruction

Treacher Collins Syndrome

Nasal Reconstruction

Orthognathic Surgery

Trauma Reconstruction

Hemifacial Microsomia

Summary

This syndrome was described in 1923 by Pierre Robin in which he described airway obstruction associated with glossoptosis and hypoplasia of the mandible. Today this syndrome is characterized by retrognathia or micrognathia, glossoptosis, and airway obstruction. An incomplete cleft of the palate is associated with the syndrome in approximately 50% of these patients.

In patients with micrognathia (small jaw) or retrognathia, the chin is posteriorly displaced causing the tongue to fall backward toward the posterior pharyngeal wall. This results in obstruction of the airway on inspiration. Crying or straining by these children can often keep the airway open. However, when the child relaxes or falls asleep, airway obstruction occurs. Due to these respiratory problems, feeding may become very difficult. This can lead to a sequence of events: glossoptosis, airway obstruction, crying or straining with increased energy expenditure and decreased oral intake. This vicious cycle of events if untreated can led to exhaustion, cardiac failure, and ultimately death.

Treatment of this syndrome can be divided into conservative therapy versus surgical intervention. The majority of these patients can be managed by placing the infant in the prone position until adequate growth of the jaw occurs. This causes the jaw and the tongue to fall forward opening the airway. If this type of treatment fails the infant should then be considered for a tongue-lip adhesion (a procedure to pull the tongue forward) or a tracheostomy.

In children with severe underdevelopment of the lower jaw, a new technique called mandibular bone expansion is now available. This technique also called distraction osteogenesis involves placement of an expansion device that is turned daily to slowly lengthen the jaw. An external incision is required to make a surgical cut through the jaw bone with placement of pins that are secured to the expansion device. Once the amount of expansion of the bone has been obtained (4-5 weeks) the device is then kept in place until the bone gap heals with new bone formation
(8 weeks). This technique can be performed at a very early age which is a significant advantage over the traditional technique of lower jaw lengthening.

Pierre Robin:
Two year old with Pierre Robin and severe airway obstruction requiring tracheostomy.

Bilateral mandibular lengthening devices surgically placed with lengthening in progress.

Results after removal of lengthening devices. Patient now ready for removal/closure of trachiostomy.

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The Tennessee Craniofacial Center, part of the Erlanger Health System, is located in Chattanooga, Tennessee. The Center, led Larry A. Sargent, M.D., specializes in the evaluation and treatment of patients of all ages with craniofacial deformities. Location: 975 East Third Street. Chattanooga, Tennessee 37403
Phone: 423-778-9192 or 800-418-3223 Fax: 423-778-8172
Internet: www.craniofacialcenter.com Copyright ©1997, 2000, Erlanger Health Systems