Pierre Robin Sequence!?
A pierre-robin-sequence syndrome affected baby – Before, during and after distraction images.
1 in every 8000 to 1 in every 30000 live births are estimated to have Pierre Robin syndrome. It affects babies all over the world. So…
What is Pierre Robin Sequence syndrome all about?
This syndrome was described by a French doctor in 1932, it is a congenital disorder, affecting babies of both genders equally. The child has a smaller than normal lower jaw, a tongue that obstructs the airway and a cleft palate. These children will have difficulty in breathing due to airway obstruction and thus making the condition fatal & requiring urgent care.
One such patient has been operated in our hospital around four years back & has been in regular follow-ups. She was born with Pierre Robins syndrome. She had a cleft palate, glossoptosis and a smaller than usual or hypoplastic mandible.
The child had severe breathing issues which needed to be addressed and hence we decided to do a lower jaw distraction procedure for her. The lower jaw had to be brought forward in order for her to breathe properly. The patient after the initial pre-surgical checkups was cleared for surgery under general anesthesia.
The surgery starts with an osteotomy or sectioning of bone then the distraction device being placed under the skin across the bony cut. After, a few days the two ends of the bone are very gradually pulled apart by means of a screw of a key present on the exterior surface at a rate of 1 mm per day. This gradual distraction leads to the formation of new bone between the two ends. After the process is complete, the osteotomy or bony cuts are allowed to heal over a period of six to eight weeks. A small second surgery is then performed to remove the device.
The same was done in our case, we achieved a distraction of approximately 22 ms of new bone in the lower jaw and thus bringing the lower jaw forward and relieving the breathing problems. The cleft palate was also surgically repaired.
The patient has remained in our follow up for more than 4 years now, her mandibular position has improved and she speaks very well too.
Pierre Robin syndrome through a rare disease, Requires surgical treatment as these children are more prone to airway collapse and thus leading to the condition being fatal.
Here at Richardson hospital, we treat many babies affected with this disorder and these babies are in our long-term follow-up as well. These children require a long-term therapy as they grow up, some may require a second osteotomy procedure too.