%0 Journal Article
%T Rubinstein-Taybi syndrome with scoliosis
%A Yasunori Tatara
%A Noriaki Kawakami
%A Taichi Tsuji
%A Kazuyoshi Miyasaka
%A Tetsuya Ohara
%A Ayato Nohara
%J Scoliosis
%D 2011
%I BioMed Central
%R 10.1186/1748-7161-6-21
%X The authors present the case of a 14-year-old boy with Rubinstein-Taybi syndrome (RSTS) presenting scoliosis.There have been no reports on surgery for RSTS presenting scoliosis.The patient was referred to our hospital for evaluation of a progressive spinal curvature. A standing anteroposterior spine radiograph at presentation to our hospital revealed an 84-degree right thoracic curve from T6 to T12, along with a 63-degree left lumbar compensatory curve from T12 to L4. We planned a two-staged surgery and decided to fuse from T4 to L4. The first operation was front-back surgery because of the rigidity of the right thoracic curve. The second operation of lumbar anterior discectomy and fusion was arranged 9 months after the first surgery to prevent the crankshaft phenomenon due to his natural course of adolescent growth. To avoid respiratory complications, the patient was put on a respirator in the ICU for several days after both surgeries.Full-length spine radiographs after the first surgery revealed no instrumentation failure and showed that the right thoracic curve was corrected to 31 degrees and the left lumbar curve was corrected to 34 degrees. No postoperative complications occurred after both surgeries.We succeeded in treating the patient without complications. Full-length spine standing radiographs at one year after the second operation demonstrated a stable bony arthrodesis with no loss of initial correction.Rubinstein-Taybi syndrome (RSTS) is characterized by mental retardation and physical abnormalities such as broad thumbs and halluces, short stature, and a peculiar facial expression - 'comical face' which is characterized by a beaked nose, down-slanting palpebral fissures and hypoplastic maxilla [1-5]. There have been several reports [6-15] on surgical intervention to treat congenital dislocation of the patella or thumb deformity in patients with RSTS, but we have yet to see case reports on surgical procedures to correct scoliosis. In this report, we presen
%K Rubinstein-Taybi syndrome
%K scoliosis
%K surgery
%U http://www.scoliosisjournal.com/content/6/1/21