Abstract
Our aim is to present the treatment of one of the skeletal manifestations of Jeune's
syndrome (JS), the hypoplastic chest, which can result in thoracic insufficiency syndrome
and present “on-demand” stage surgical technique using mandible locking plate system
for the fixation of ribs. The diagnosis “Jeune's syndrome” was presented clinically
in a 3-month-old girl from a family in which the first child died of JS at the age
of 18 months. After close follow-up for several months and preoperative planning,
we decided to make reconstructive chest operation with atypical use of a double-angled
mandible locking plate for fixation. The plate was shaped as a “crown” to ensure the
three dimension stability, from the dorsal part of the most curved ribs (paravertebrally)
to the sternum after the resection of this area. Operation was done at the period
of worsened breathing. For nearly 1 year, the rib cage preserved its stability and
the child was in good condition. During the next 3 months, the upper part of the deformation
started to grow inward fast. Second operation was “on demand,” and the implants used
were mandible locking plates curved anterolaterally to effectuate extension of the
rib cage and the sternum. In both the reconstructive operations, we spared the rectus
and pectoral muscles and achieved good enlargement of the thoracic volume. The postoperative
period is smooth and the child is active, without complications. We believe that in
the future, the treatment should be “on demand” according to the course of the illness
and the results of the follow-up examinations and adequate to the progress of chest
wall deformity.
Keywords
Jeune's syndrome - asphyxiating thoracic dystrophy - mandible locking plate - thoracic
insufficiency syndrome
