VEPTR (Vertical Expandible Prosthetic Titanium Rib) Treatment for Jeune Syndrome

Pulmonary and Radiographic Outcomes of VEPTR (Vertical Expandable Prosthetic Titanium Rib) Treatment in Early-Onset Scoliosis

“…All differences between time periods were significant (pre to first, p = 0.034; first to last, p = 0.000007; pre to last, p = 0.000008). 1297 6,2014 despite the observed fusion, the patient showed increased coronal imbalance after device removal. In the second patient, a rib-to-rib device was removed because of skin compromise.…”

“…VEPTR patient selection criteria, indications, and design have been evolving as more studies and longer follow-up data become available 1,[4][5][6][7][8][9][10][11][12][13][14] . The aim of the present study was to investigate the intermediate-term pulmonary and radiographic outcomes of VEPTR expansion thoracoplasty in children with thoracic insufficiency syndrome.…”

Treatment of congenital thoracic scoliosis with associated rib fusions using VEPTR expansion thoracostomy: a surgical technique

“…Although rare, this condition will invariably progress and is the most frequently reported indication for this treatment [1,5,6,[9][10][11][12][13][14]. The second group of indications comprises pathologies inducing bilateral underdevelopment of the chest with a hypoplastic thorax, commonly encountered in rare conditions such as Jarcho-Levin Syndrome, Jeune's syndrome, Ellis van Creveld Syndrome, achondroplasia and VACT-ERL syndrome [10,11,[14][15][16][17][18][19]. The third group includes conditions inducing a flail chest syndrome (unstable thorax due to missing ribs, congenital or acquired) [10,14,15,19].…”

Case report: A simple and reliable approach for progressive internal distraction of the sternum for Jeune syndrome (asphyxiating thoracic dystrophy): preliminary experience and literature review of surgical techniques

“…These techniques are dissimilar in invasiveness, tools, and access. Most of these techniques have only been proposed and documented in isolated cases because of the extreme rarity of the syndrome and the frequent heroic treatment setting ( 7 – 13 , 15 – 17 , 20 , 24 , 27 , 29 ).…”