In 1995, Haller reported on a peculiar group of adolescents suffering from a condition coined “acquired Jeune’s syndrome,” later also known as “Acquired restrictive thoracic dystrophy” (ARTD). All these patients underwent pectus excavatum repair at an early age, after which their chest failed to grow adequately and became rigid with severely restricted respiratory function.
To shed light on the cause of this heretofore unknown disease, we have studied clinically five patients with ARTD and coordinated our findings with the scarce literary data available on the subject.
All the patients whose data were available presented with severe respiratory impairment. Evidently, in the course of their pectus repair their costal cartilages from the second rib down were radically extirpated, including their medial and lateral centers of growth. Some of the operative reports and all of our imaging studies also indicated that the perichondrial strips were sewn together behind the sternum to maintain its corrected position (Fig. 1). As a result, the patient developed a rib cage that remained infantile in size, odd in appearance and failed to rise in inspiration or fall when the patient exhales.
Performing repair of pectus excavatum the way described above is a grave mistake and should be avoided. We also disagree with those who suggest that the best way to avoid ARTD is to delay pectus repair to the age when the chest is already fully developed. As our experience of 300 patients operated at a very early childhood shows, the operation may be safely performed at any age, given appropriate operative plan and technique.
The sparing of the growth plates and synovial joints of the ribs during cartilage resection preserves growth potential of the thoracic cage, prevents the development of acquired restrictive thoracic dystrophy after pectus excavatum repair and allows surgical correction of chest wall deformities to be done at early age.
Alexander Fokin, None.