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Neurofibromatosis type I with severe dystrophic kyphoscoliosis and its operative management via a simultaneous anterior-posterior approach: a case report and review of the literature.
Spine J. 2005 Jul-Aug; 5(4):461-6.SJ

Abstract

BACKGROUND CONTEXT

Neurofibromatosis is an autosomal-dominant hereditary disorder with two subtypes: NF-1 (type I) and NF-2 (type II). NF-1 is a complex disorder with a constellation of manifestations that can also entail skeletal abnormalities, including spinal deformity of a noncongenital nature with early age onset. The short, sharp, angular curve usually present in the thoracic region, as exhibited in NF-1, presents a quandary in its surgical management. Various studies have reported on the efficacy of anterior correction as opposed to posterior alone, whereas others have advocated a sequential, combined approach to diminish the degree of deformity and achieve solid arthrodesis. However, despite solid arthrodesis, curve progression may still ensue. Nonetheless, a simultaneous anterior-posterior approach to treat such a condition of NF-1 with severe dystrophic kyphoscoliosis is a rare occurrence.

PURPOSE

To describe the presentation and operative management of a patient with NF-1 and severe dystrophic kyphoscoliosis.

STUDY DESIGN

A case report and review of the literature.

METHODS

A clinical and radiographic review of a 51-year-old male patient who presented with NF-1, a 165-degree thoracic kyphotic deformity, associated scoliosis, varied degree of vertebral destruction of T9-T11, and paraparesis below T10.

RESULTS

Operative intervention of the deformity consisted of a simultaneous anterior-posterior approach and entailed posterior cord exposure, anterior vertebrectomy of T9-T11, cord decompression, posterior osteotomy (posterior elements were auto-fused), anterior distraction and kyphosis correction, anterior strut grafting, anterior rod instrumentation, and posterior compression instrumentation and fusion from T6-L2. The deformity was reduced, sold fusion was noted, and the patient was asymptomatic.

CONCLUSIONS

A simultaneous anterior-posterior approach for the surgical treatment of severe dystrophic kyphoscoliosis in neurofibromatosis type I is an avenue to properly visualize the spinal cord, achieve solid arthrodesis, and to minimize as well as prevent the progression of deformity.

Authors+Show Affiliations

Department of Orthopaedic Surgery, Rush-Presbyterian-St. Luke's Medical Center, 1725 W. Harrison Street, POB 1063, Chicago, IL 60612, USA.No affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Review

Language

eng

PubMed ID

15996618

Citation

Singh, Kern, et al. "Neurofibromatosis Type I With Severe Dystrophic Kyphoscoliosis and Its Operative Management Via a Simultaneous Anterior-posterior Approach: a Case Report and Review of the Literature." The Spine Journal : Official Journal of the North American Spine Society, vol. 5, no. 4, 2005, pp. 461-6.
Singh K, Samartzis D, An HS. Neurofibromatosis type I with severe dystrophic kyphoscoliosis and its operative management via a simultaneous anterior-posterior approach: a case report and review of the literature. Spine J. 2005;5(4):461-6.
Singh, K., Samartzis, D., & An, H. S. (2005). Neurofibromatosis type I with severe dystrophic kyphoscoliosis and its operative management via a simultaneous anterior-posterior approach: a case report and review of the literature. The Spine Journal : Official Journal of the North American Spine Society, 5(4), 461-6.
Singh K, Samartzis D, An HS. Neurofibromatosis Type I With Severe Dystrophic Kyphoscoliosis and Its Operative Management Via a Simultaneous Anterior-posterior Approach: a Case Report and Review of the Literature. Spine J. 2005 Jul-Aug;5(4):461-6. PubMed PMID: 15996618.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Neurofibromatosis type I with severe dystrophic kyphoscoliosis and its operative management via a simultaneous anterior-posterior approach: a case report and review of the literature. AU - Singh,Kern, AU - Samartzis,Dino, AU - An,Howard S, PY - 2004/03/03/received PY - 2004/09/28/accepted PY - 2005/7/6/pubmed PY - 2005/12/13/medline PY - 2005/7/6/entrez SP - 461 EP - 6 JF - The spine journal : official journal of the North American Spine Society JO - Spine J VL - 5 IS - 4 N2 - BACKGROUND CONTEXT: Neurofibromatosis is an autosomal-dominant hereditary disorder with two subtypes: NF-1 (type I) and NF-2 (type II). NF-1 is a complex disorder with a constellation of manifestations that can also entail skeletal abnormalities, including spinal deformity of a noncongenital nature with early age onset. The short, sharp, angular curve usually present in the thoracic region, as exhibited in NF-1, presents a quandary in its surgical management. Various studies have reported on the efficacy of anterior correction as opposed to posterior alone, whereas others have advocated a sequential, combined approach to diminish the degree of deformity and achieve solid arthrodesis. However, despite solid arthrodesis, curve progression may still ensue. Nonetheless, a simultaneous anterior-posterior approach to treat such a condition of NF-1 with severe dystrophic kyphoscoliosis is a rare occurrence. PURPOSE: To describe the presentation and operative management of a patient with NF-1 and severe dystrophic kyphoscoliosis. STUDY DESIGN: A case report and review of the literature. METHODS: A clinical and radiographic review of a 51-year-old male patient who presented with NF-1, a 165-degree thoracic kyphotic deformity, associated scoliosis, varied degree of vertebral destruction of T9-T11, and paraparesis below T10. RESULTS: Operative intervention of the deformity consisted of a simultaneous anterior-posterior approach and entailed posterior cord exposure, anterior vertebrectomy of T9-T11, cord decompression, posterior osteotomy (posterior elements were auto-fused), anterior distraction and kyphosis correction, anterior strut grafting, anterior rod instrumentation, and posterior compression instrumentation and fusion from T6-L2. The deformity was reduced, sold fusion was noted, and the patient was asymptomatic. CONCLUSIONS: A simultaneous anterior-posterior approach for the surgical treatment of severe dystrophic kyphoscoliosis in neurofibromatosis type I is an avenue to properly visualize the spinal cord, achieve solid arthrodesis, and to minimize as well as prevent the progression of deformity. SN - 1529-9430 UR - https://www.unboundmedicine.com/medline/citation/15996618/Neurofibromatosis_type_I_with_severe_dystrophic_kyphoscoliosis_and_its_operative_management_via_a_simultaneous_anterior_posterior_approach:_a_case_report_and_review_of_the_literature_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1529-9430(04)00996-9 DB - PRIME DP - Unbound Medicine ER -