Abstract

Background: This report describes a case of thoracic insufficiency/short rib syndrome treated with sagittal distraction of the sternum in a juvenile male.

Methods: An acute increase in the thoracic cage volume was achieved in an 8-year-old male with thoracic insufficiency syndrome by sternum splitting and distraction of 3 centimeters. He had previously been treated with bilateral opening wedge and thoracic vertical expandable prosthetic titanium rib instruments. At 8 years, his respiration was rapidly deteriorating, and posterior revision surgery with resection of the posterior wall at this stage had no further positive influence on his respiratory function. Two years later, a second distraction of the sternum and acute distraction were performed as part of the thoracic expansion procedure.

Results:The procedure resulted in an increase in his respiratory functions, and his pulmonary artery parameters decreased to normal values. His clinical condition improved, and after surgery, he could ride a bicycle to school and even participate in sports, such as cross-country skiing and soccer.

Conclusion:Short rib syndrome presenting with respiratory failure in a neonate is potentially fatal without surgical intervention to expand the thoracic cage. Our patient had severe thoracic insufficiency syndromeand experienced clinical improvement from the procedure. Sternum distraction has been previously used in neonates, but to the best of our knowledge, this is the first distraction of the sternum addressing short rib syndrome in a juvenile. 

Keywords

Short rib syndrome, Early onset scoliosis, Sternal distraction, Scoliosis, Thoracic insufficiency syndrome, Forced vital capacity, Forced expiratory volume, Sagittal distraction, Osteotomy, Respiratory function, Clinical improvement, Thoracic dystrophy, Costo-chondral junction, Diaphragm, Pulmonary hypertension