Literature DB >> 29299101

Rib head dislocation causing spinal canal stenosis in a child with neurofibromatosis, type 1.

Alan M Chen1, Jeffrey B Neustadt2, Jennifer Neville Kucera1,3.   

Abstract

A 10-year-old female with neurofibromatosis type 1 and severe dystrophic scoliosis presented with a two-month history of difficulty ambulating due to low back pain. The patient did not have any neurological symptoms. MRI of the thoracolumbar spine demonstrated subluxation of the right posterior tenth and eleventh ribs through their respective neural foramina, with mild mass effect on the thecal sac without abnormal cord signal or cord compression. Groups of neurofibromas were present along the right ribs and paravertebral soft tissues around these levels. CT evaluation was completed for greater osseous definition. Cases of rib head dislocation into the central canal in the setting of dystrophic scoliosis were documented in only a limited sample of case reports. The angulated short-segment curvature in dystrophic scoliosis causes vertebral body rotation, foraminal enlargement, spindling of transverse processes, and penciling of the apical ribs. These changes can alter the articulation of the rib along the transverse process. The enlarged foramina can also create a larger space into which a rib may displace. As a result, in most reported cases, the subluxed ribs were on the convex apex of the curve in the mid-to-lower thoracic region. The risk of cord injury from rib head dislocation makes the complete depiction of the anatomy essential for proper surgical management.

Entities:  

Keywords:  Dystrophic scoliosis; Neurofibromatosis type 1; Rib head dislocation; Spinal canal stenosis; Thoracic spine

Mesh:

Year:  2017        PMID: 29299101      PMCID: PMC5743134          DOI: 10.3941/jrcr.v11i8.3113

Source DB:  PubMed          Journal:  J Radiol Case Rep        ISSN: 1943-0922


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  3 in total

Review 1.  Towards a neurobiological understanding of pain in neurofibromatosis type 1: mechanisms and implications for treatment.

Authors:  Shreya S Bellampalli; Rajesh Khanna
Journal:  Pain       Date:  2019-05       Impact factor: 6.961

2.  Management of NF-1 dystrophic scoliosis associated with rib heads dislocation into the spinal canal in neurological intact patients: a systematic literature review.

Authors:  Martin M Estefan; Gaston Camino-Willhuber; Santiago T Bosio; Miguel Puigdevall; Ruben A Maenza
Journal:  Spine Deform       Date:  2021-10-27

3.  Surgical Treatment of the Intraspinal Rib Head Dislocation in Children With Dystrophic Scoliosis Secondary to Type 1 Neurofibromatosis.

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Journal:  J Pediatr Orthop       Date:  2022-03-01       Impact factor: 2.324

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