Pierre Robin sequence (PRS) is a condition that presents with symptoms such as microgenia, glossoptosis, and respiratory disorder caused by upper airway obstruction, and is often associated with cleft palate.
Respiratory and feeding management is necessary immediately after birth, and there is often distress during perioperative management, speech and swallowing training.
We retrospectively examined 26 cases of PRS who attended our center from 2006 through 2010. The PRS cases were classified into three groups: non syndromic (ns) PRS or syndromic (s) PRS in relation to the presence of determined syndromes, and unique (u) PRS, with unidentified congenital anomalies. The following results were obtained.
1. There were 10 males and 16 females: 14 cases of ns PRS, 10 cases of s PRS, and 2 cases of u PRS.
2. The age at first visit varied from 1 to 291 days. The weights at birth were 2,932±447g in ns PRS, 2,850±571g in s PRS, 1,607±804g in u PRS. There was no significant difference between ns PRS and s PRS.
3. As for the management of respiratory and feeding disability, in stage I (from neonates to six months) , tracheal intubation was needed in 1 case and tube feeding in 10 cases for ns PRS, tracheostomy was needed in 3 cases and tube feeding in 8 cases for s PRS, and tracheal intubation and tube feeding was needed in 1 case for u PRS.
In stage II (from 1 year to 18 months; period considered the appropriate time for surgery) , no respiratory or feeding management was needed for ns PRS and u PRS. In contrast, for s PRS, tracheostomy was maintained in 3 cases and tube feeding was continued in 6 cases.
4. As for the palatoplasty, for ns PRS and u PRS, palatoplasty was performed at the age of 24.9±7.0 months and 40.5±4.9 months respectively, in all cases. On the other hand, in s PRS, palatoplasty was not necessary in 1 case, was performed in 1 case, was planned in 2 cases, and has not been performed in 6 cases.
Post-operative hemorrhage was encountered in an ns PRS case as a perioperative complication.
View full abstract