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

• Published in: Spine deformity Vol. 10; no. 2; p. 285
• Main Authors: Estefan, Martin M, Camino-Willhuber, Gaston, Bosio, Santiago T, Puigdevall, Miguel, Maenza, Ruben A
• Format: Journal Article
• Language: English
• Published: England 01-03-2022
• Subjects: Humans; Joint Dislocations; Kyphosis - complications; Kyphosis - surgery; Ribs - surgery; Scoliosis - complications; Scoliosis - surgery; Spinal Canal - surgery
• ISSN: 2212-1358
• DOI: 10.1007/s43390-021-00422-3

The management of scoliosis and kyphoscoliosis in patients with Type 1 Neurofibromatosis (NF-1) among spinal surgeons is still challenging due to the severity of the deformity especially in dystrophic deformity types. This rapid and progressive condition is likely to be associated with dislocated rib heads into the spinal canal, hence representing a real dilemma on the decision making between its resection versus not resection during the corrective surgery, especially in patients with normal neurological status. The objective of this publication is to discuss the management options in this patient population through a literature review.
A comprehensive systematic literature search was performed for relevant studies using PubMed, Web of Science, and Scopus databases. Previous publications depicting neurologically intact patients with NF-1 and rib dislocation into the canal were reviewed. Articles reporting individual cases or case series/cohorts with patient-discriminated findings were included.
The data collection retrieved a total of 55 neurologically intact patients with NF-1 dystrophic scoliosis and rib penetration into the canal who underwent spinal surgery. Among them, 37 patients underwent surgery without head rib resection and 18 patients with rib excision. No patient presented postoperative neurological deficit except for one case of late postoperative neurological deterioration reported in a patient within situ fusion in which the surgeons ignored the presence of previous spinal cord compression.
Corrective surgery for patients with NF-1 and rib penetration into the canal in neurologically intact patients can be safely performed without the resection of the dislocated rib heads without a higher risk of neurological compromise.