Neues therapiekonzept für kinder mit thoraxinsuffizienz-syndrom aufgrund von wirbelsäulenfehlbildungen

Anna K. Hell, R. M. Campbell, F. Hefti

Resultado de la investigación: Articlerevisión exhaustiva

10 Citas (Scopus)

Resumen

Background: Children with congenital thoracic scoliosis associated with fused ribs and unilateral unsegmented bars adjacent to convex hemivertebrae will inevitably develop thoracic insufficiency syndrome and curve progression with hemithorax compression without treatment. It is assumed that the concave side of such curves and their unilateral unsegmented bars do not grow. In the past early spinal fusion was performed with consecutive short thoracic spines and loss of lung volume. Little attention has been paid to lung function. These patients often suffered from lung failure and early death due to a small thorax. Method: A new surgical technique is based on an indirect deformity correction and enlargement of the thorax due to a longitudinal implant, the vertical expandable prosthetic titanium rib (VEPTR). The spine is not fused, thus promoting growth of the spine, the thorax and the lungs. Elongation of the implant is done every six months. Since 2002 this method has been performed on fifteen children in Basel as the first European center. Results: Patients (mean age 6 years; 11 months to 12 years) were suffering from thoracic insufficiency syndrome due to unilateral unsegmented bars with fused ribs (n = 4), absent ribs (n = 2), bilaterally fused ribs (n = 2), hemivertebrae (n = 3) or neuromuscular scoliosis (n = 6). Doing fifteen primarily implantations and thirteen elongations there were three complications (two hook dislocations, one skin breakage). All patients improved cosmetically, functionally and radiologically which was shown on X-rays as a reduction of the Cobb angle from an average of 76° (40-110°) to 55° (30-67°). Conclusions: Expansion thoracoplasty and VEPTR implantation is a new treatment concept for children with thoracic insufficiency syndrome due to spinal deformities, which is based on distraction and expansion of the thorax thus allowing growth of the spine, the thorax and probably lungs. Presently it seems to be superior to any other method for the treatment of small children with progressive scoliosis and thoracic insufficiency syndrome.

Título traducido de la contribuciónNew treatment concept for children with thoracic insufficiency syndrome due to congenital spine deformity
Idioma originalGerman
Páginas (desde-hasta)268-273
Número de páginas6
PublicaciónKlinische Padiatrie
Volumen217
N.º5
DOI
EstadoPublished - sept. 2005

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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