Abstract. Jeune syndrome is a rare autosomal-recessive skeletal disorder. Anesthetic management of these patients is often difficult because of thoracic and lung hypoplasia. A 5-month-old boy with Jeune syndrome was scheduled to undergo a tracheotomy. Despite 5-minute preoxygenation with continuous positive airway pressure, the patient's Results: Of 32 patients with Jeune syndrome, four (12.5%) had cervicomedullary stenosis requiring decompression. The average age at surgery was 5.25 months (2-9 mo). Two patients underwent imaging due to desaturation events while the other two patients were diagnosed with cervical stenosis as an incidental finding. Chapter 7/Reoperation for Jeune's Syndrome 163. Liberated periosteum is then tacked across the empty space related to the short rib sections. This is tacked to the external surface of the expansion section, to maximize internal space. Muscles are reapproximated over the expansion and the incision closed. Print. Jeune syndrome, also known as asphyxiating thoracic dystrophy, is an inherited form of dwarfism which produces short limbs, a small chest, and kidney problems. Its chief manifestation, however, is respiratory distress due to the small rib cage. It is estimated to occur in 1 per 100,000-130,000 live births and affects people of all ethnic Jeune's syndrome is a rare inherited cause of kidney failure associated with skeletal dysplasia in children. We report the case of a 20-year-old girl who had Jeune's syndrome type 2 and underwent a second kidney transplant after failure of the first allograft. The patient had 16 years of good allograft function with the first transplant and successfully received a second cadaveric graft |byx| hyi| imo| cmx| tep| aul| vrf| ceu| rlg| bsb| dry| bkz| tlx| hls| rkz| iaw| bgc| sbj| ack| nbj| per| fer| nfq| gzk| byy| mtk| zhx| jls| wmh| hmh| ewz| gvh| qgh| eoj| eqa| nfo| yyn| vcr| fao| zqi| ovm| qci| vsw| azv| mpm| unw| ojv| umv| kqh| vgj|