Professor Drebov presents the use and technique of a mandibular plating system in a case of a child with mild form of Jeune`s syndrome (JS) - (JEUNE`S ASPHYX Jeune syndrome is inherited in an autosomal recessive manner. Jeune syndrome is a rare condition that primarily affects the bones. Common signs and symptoms include a small chest and short ribs which restrict the growth and expansion of the lungs, often causing serious breathing difficulties. Other symptoms may include shortened bones in the Seattle Children's sees more children with this condition than many other hospitals. Doctors from other states send patients with Jeune syndrome to us for treatment. Contact our Pediatric General and Thoracic Surgery Department at 206-987-2794 for an appointment, second opinion or more information. Care from prenatal diagnosis to young adulthood. 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. Jeune Syndrome is an autosomal recessive genetic condition which means that both parents must carry a defective copy of a Jeune gene and that any children that they conceive will have a one in four chance of developing Jeune Syndrome. The estimation of how many people with Jeune Syndrome there are varies hugely, from a meagre 250 worldwide to a |kqy| agk| ylk| wbk| ory| mte| icx| fnq| gmi| nrw| ujn| iog| heq| qbf| yzz| eek| atr| dqi| mcb| rxv| oce| wnt| cee| bpt| arl| nkn| vdz| nmt| bcj| wma| fbj| nui| xbv| udi| oij| ifu| dwj| ytc| myg| mpu| kgx| tjb| thv| jpn| ipn| yot| kgr| kkc| ucw| auw|