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Scoliosis  2006 

Painful rib hump: a new clinical sign for detecting intraspinal rib displacement in scoliosis due to neurofibromatosis

DOI: 10.1186/1748-7161-1-10

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Abstract:

A 13-year-old girl was evaluated for progressive left thoracic kyphoscoliotic curve due to a type I neurofibromatosis. Clinical examination revealed multiple large thoracic and abdominal "cafe-au-lait" spots, neurological impairment of the lower limbs and the presence of a thoracic gibbous that was painful to pressure at the level of the left eighth rib (Painful Rib Hump). CT-scan showed detachment and translocation of the cephalic end of the left eighth rib into the adjacent enlarged neural foramen. The M.R.I. examination of the spine showed neither cord abnormality nor neurogenic tumor.The patient underwent resection of the intraspinal mobile eighth rib head and posterior spinal instrumentation and was neurologically fully recovered six months postoperatively.Spine surgeons should be aware of intraspinal rib displacement in scoliotic curves in neurofibromatosis. Painful rib hump is a valuable diagnostic tool for this rare clinical entity.Neurofibromatosis (NF) is an autosomal dominant hereditary disorder that was first described by Frederick Daniel von Recklinghausen and is associated with skeletal, skin and soft tissue abnormalities. The most common skeletal manifestations in NF are scoliosis and kyphoscoliosis due to dystrophic osseous changes [1].The typical spinal deformity in NF has been described as a progressive short, sharp, angular kyphoscoliotic curve [2]. Right thoracic curves predominate in scoliosis in NF but there is an increasing incidence of left thoracic curves compared to idiopathic scoliosis [3].Rib dislocation into the spinal canal that causes spinal cord compression in patients with NF and scoliosis has previously been described [4-10]. The present report introduces a new clinical sign for detection of this rare clinical entity.A 13-year-old girl was referred for evaluation of a progressive left thoracic kyphoscoliotic curve (Figure 1) due to a type I NF. Clinical examination revealed multiple large thoracic and abdominal "cafe-au-lait" spots

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