Pierre Robin Syndrome
Pierre Robin Syndrome is characterized by micrognathia (small lower jaw), cleft palate, and glossoptosis (the tongue tends to fall back and downwards). As a result of the physical characteristics of Pierre Robin Syndrome, both feeding and breathing difficulties are often present in varying degrees of severity. The causes of Pierre Robin Syndrome are unknown, however Robin sequence is seen in a number of genetic syndromes. Children born with a cleft palate must be seen by an Ear, Nose and Throat specialist who will carefully monitor fluid buildup behind the ear. Initially when airway obstruction occurs, the primary goal is to create an adequate airway by using distractor techniques that prevent the need for a tracheostomy. If left untreated, patients have problems with feeding, obstructive sleep apnea, ear infections, and reduced hearing.Treatment of Pierre Robin Syndrome using Distraction Osteogenesis
The first step to correction is most dependent on the patients with Pierre Robin Syndrome airway stability. If the airway is extremely compromised, we will perform bilateral mandibular osteotomies with distraction osteogenesis, which slowly brings the mandible (lower jaw) forward and creates a more normal airway. This procedure takes approximately 3-4 hours in surgery and the patient remains in the hospital 2-3 days after surgery. The parents are instructed on how to turn the distraction osteogenesis device and we typically see patients back in the office on a weekly basis. Once the mandible is in a more favorable position, we will lock down the distraction osteogenesis device and it remains on the patient for 3-4 months to allow the bone to heal. The distraction osteogenesis units are then removed under anesthesia, which is considered an outpatient procedure. Newer dissolvable distraction osteogenesis devices may be used that do not require in hospital removal.For patients with Pierre Robin Syndrome that have this deformity typically the first step in correction would be to close the palate around 6-9 months of age. This procedure performed early helps with speech and feeding issues. The two-palatoplasty is the most common technique we use in closing the palate and has yielded excellent surgical and speech outcomes for patients with Pierre Robin Syndrome. In this two-hour surgical procedure, the muscles of the palate are surgically dissected and brought together in a more normal position. The incision is closed with dissolvable sutures. Most patients remain in the hospital 1-2 days after surgery and require a 2-week postoperative appointment in our office to check with healing.
Pierre Robin Syndrome patients continue to be monitored throughout growth for any changes in breathing patterns. Occasionally, future surgery on these Pierre Robin Syndrome patients may be required to obtain normal speech and dental alignment later in life.
Case Study
Pierre Robin Syndrome