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This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Eric W. Fonkalsrud Permission Requests Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Fonkalsrud, E. W. Search for Related Content PubMed Articles by Fonkalsrud, E. W. Related Collections Chest wall Related Article

Ann Thorac Surg 2001;72:878
© 2001 The Society of Thoracic Surgeons

Invited commentary

^a Department of Surgery, UCLA Medical Center, 10833 LeConte Ave, Room 72-126, Los Angeles, CA 90095-1741, USA

e-mail: efonkalsrud{at}mednet.ucla.edu

The authors have compiled a remarkably large series of 10 children with the rare Jeune’s syndrome on whom they have performed a very creative operative technique to enlarge the volume of the thoracic cavity. Surgical attempts to enlarge the thoracic cavity have most often consisted of vertical splitting of the sternum with widening of the chest using prostheses or autologous bone, which has resulted in prolonged hospitalization and ultimate respiratory failure and death for most. Lateral extension of two ribs on each side of the chest, first described by the authors in 1995 [1] has provided an additional centimeter or more of rib length. The new configuration appears somewhat unstable from the torque caused by the realignment of adjacent ribs, as evidenced by the frequent fracture of titanium support plates. An intramedullary support across the bony junction combined with external support might provide greater stability. Placement of autologous or allograft bone, bone morphogenic protein, or prostheses to bridge the gaps between the adjacent shortened ribs might provide even more stability. Combining the lateral thoracic expansion together with the sternal widening technique might provide greater chest expansion and less torque and stress on the chest wall than either procedure alone. The lack of supporting tissue between the lungs and a portion of the thoracic skeleton may be problematic and one wonders if the periosteal sheaths might be reconstructed to provide broader coverage of the chest wall. Biologic membranes such as dura, SIS (oasis wound dressing), or others, which might incorporate the patient’s, own body tissues might be helpful in preventing the lungs from adhering to the restructured ribs. Although the authors have shown an increase in total lung volume in four of five patients, it is unclear whether the tidal volume and vital capacity are increased, or if the reconfigured chest is held in a semi-rigid adynamic position, analogous to a severe pectus carinatum. It is unclear if the division of several intercostal nerves and muscles during the operation will effect spontaneous respiratory excursions. Whether extensive osseous and cartilaginous rib reconstruction during the first few years of life will disturb subsequent growth centers of the bony thorax remains to be determined. Regardless of these concerns, the authors have introduced a new concept in thoracic expansion for these almost hopeless children with remarkably good success.

References

1. Davis J.T., Ruberg R.L., Leppink D.M., et al. Lateral thoracic expansion for Jeune’s asphyxiating dystrophy: a new approach. Ann Thorac Surg 1995;60:694-696.[Abstract/Free Full Text]

This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Eric W. Fonkalsrud Permission Requests Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Fonkalsrud, E. W. Search for Related Content PubMed Articles by Fonkalsrud, E. W. Related Collections Chest wall Related Article