Introduction Jeune's syndrome, or asphyxiating thoracic dystrophy (ATD), is a rare autosomal recessive disorder characterized by skeletal dysplasia. Ribs are typically short and horizontal resulting—in lethal variant—in severe lung hypoplasia, progressive respiratory failure, and death. Lateral thoracic expansion (LTE) consists in staggered bilateral ribs osteotomy leading to chest expansion and lung development. Studies on LTE in ATD patients report encouraging data, but the rarity of ATD implies the lack of a standardized surgical path. The aim of this report is to present our experience with LTE, the technical modification we adopted, and patients' clinical outcome.
Materials and Methods We retrospectively reviewed data of 11 LTE performed in 7 ATD patients with lethal variant. Information regarding pre- and postoperative clinical conditions and surgical details was collected. We adopted a single-stage or a two-stage approach based on patient clinical condition. Computed tomography (CT) scan was performed before and after surgery and lung volume was calculated.
Results Five patients are alive, while two died in intensive care unit for other than respiratory cause (sepsis). Most patients experienced clinical improvement in terms of decreased respiratory infections rate, need for ventilation, and improved exercise tolerance. Postoperative CT scan demonstrated a median lung volume increase of 88%.
Conclusion Mortality in ADT patients is high. However, LTE is a feasible and safe surgical approach, which could improve clinical conditions and survival rate. Survived patients showed postoperatively less oxygen requirement and improved clinical conditions.
Purpose Posterior tracheopexy has been proposed as a novel approach to treat tracheobronchomalacia, in addition or alternative to more traditional treatments as aortopexy. Reports on this technique are still very sporadic. Our aim is to discuss technical surgical details based on our preliminary experience of thoracoscopic and robotic posterior tracheopexy. Methods Technical details on 6 patients (from 8 months to 13 years of age) operated for posterior tracheopexy with a thoracoscopic approach are presented and discussed. In two cases robotic posterior tracheopexy was performed. Results Two patients presented multiple comorbidities and had a tracheostomy previously performed. Airway obstruction evaluated at pre-operative bronchoscopy was between 70 and 100%, with posterior intrusion of pars membranacea. Operative time ranged from 110 to 320 min. In three cases aortopexy was associated, one at the same time, in the other two before or after tracheopexy. No specific complications of posterior tracheopexy were observed. Robotic approach made the esophageal dissection and the tracheobronchopexy technically easier. Conclusion Thoracoscopic approach is feasible, robotic assistance is helpful for improving visualization, esophageal dissection, and making easier and more precise the pexy of the trachea and bronchi on all its length. Both bronchi can be approached and pexied if necessary, and this is a specific advantage of tracheopexy with respect to aortopexy. Intraoperative bronchoscopy and strict collaboration between surgeons and anesthesiologists are essential.
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