Zur Klinik und Problematik der Thoraxdystrophie

Abstract

We report about a child with Jeune-syndrome in whom surgical enlargement of the thorax at the age of 4 months was performed. In the beginning there was a significant improvement of the respiratory situation. Due to resorption of the interposed ribs there was again a shrinking of the thorax and a deterioration in the child's condition. A reoperation at the age of 8 months couldn't improve the situation anymore, because at that time the child had already additional problems such as pulmonary hypertension and broncho-pulmonary dysplasia. We overview the literature and line out our opinion about the indication for surgical enlargement of the thorax in children with Jeune syndrome.

Jeune syndrome: Case report

“…Kids who survive to infancy may benefit from surgical treatment. Hoffman et al reported a total of 5 children with Jeune syndrome underwent surgical chest-expanding correction and concluded that indication for an operation is already given in newborns if failure of the right side of the heart and/or bronchopulmonary dysplasia do not allow fast improvement of their respiratory failure [10].…”

Thoracoplasty for treatment of asphyxiating thoracic dysplasia in a newborn

“…According to the literature review of Hoffmann and coauthors [10], until 1988, a total of 5 children with Jeune syndrome underwent surgical chest-expanding correction. Main purpose of the operation was bridging the defects by longitudinal splitting of neighboring ribs or by transplanting them after harvesting from the opposite side.…”

“…Main purpose of the operation was bridging the defects by longitudinal splitting of neighboring ribs or by transplanting them after harvesting from the opposite side. For stabilization, metal struts and wires have been used, connective tissue has been replaced by tendon strips, patches, or tissue engineering [1,3,10]. For a thoracic expansion-plasty, rib pieces were interpositioned into the sternal gap.…”

“…The 4 -month-old baby showed initially a clear improvement in ventilation parameters, but after implant absorption, rapid deterioration occurred. Because of ongoing bronchopulmonary dysplasia and hypertonia, no further improvement could be achieved till the 16th month of life [10].…”

“…In both cases, their lungs showed no hypoplastic changes. At that time, Hoffmann et al [10] concluded that indication for an operation is already given in newborns if failure of the right side of the heart and/or bronchopulmonary dysplasia do not allow fast improvement of their respiratory failure. Thus, our case report represents the first one of an expandable thoracoplasty with successful postoperative outcome according to literature.…”