Surgical treatment of @entity1635 in XXXX : a case report.

@entity2122 @entity1 @entity1635 @entity1514 @entity5837 @entity615 @entity358 @entity74 @entity158 @entity16546 @entity85 @entity1516
INTRODUCTION: @entity16546 is an @entity74 resulting in @entity2122 , and divided into types 1 and 2. Scoliosis has not been reported as one of the extra-cranial manifestations of @entity16546 . CASE PRESENTATION: We present a 14-year-old British Caucasian @entity1 with @entity16546 who developed a severe double thoracic @entity1635 measuring 39   and 68   respectively. Her @entity1635 was associated with @entity1514 , causing a marked reduction in the anteroposterior diameter of her chest and consequent severe @entity358 . The @entity85 was noted by her local pediatrician as part of a @entity5837 assessment when she was aged 13 years, and gradually progressed as the result of spinal growth. Our @entity1 underwent a posterior spinal arthrodesis using a single concave pedicle hook and screw rod construct and locally harvested autologous graft supplemented by allograft bone. This spinal fixation technique was selected because of our @entity1 's low body weight to avoid prominence of the instrumentation causing skin healing problems and @entity158 . Her @entity1635 was corrected to 18   and 30   and we achieved a balanced spine in the coronal and sagittal planes. An underarm spinal jacket was provided for six months after surgery. During her latest follow-up at skeletal maturity, our @entity1 had an excellent cosmetic outcome with no @entity85 correction or detected @entity1516 and a normal level of activities. CONCLUSION: Scoliosis can develop in young @entity1 with @entity16546 , with the @entity85 deteriorating around the pubertal growth spurt. Surgical treatment can correct the @entity85 , balance the spine and prevent @entity615 . It can also stabilize the chest area and avoid respiratory complications developing as the @entity1635 progresses, which can result in severe @entity358 . The use of single concave instrumentation is indicated in very slim @entity1 with poor muscle bulk; in our @entity1 , this produced satisfactory @entity85 correction and a favorable outcome at completion of growth. Peri-operative care in this group of @entity1 can be very challenging because of associated co-morbidities as well as the presence of severe behavioral issues that result in poor @entity1 compliance.
