Dystrophic Lumbar Kyphoscoliosis Associated with Giant Dural Ectasia in a 19-Year-Old Patient with Neurofibromatosis Typ
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SURGERY
Dystrophic Lumbar Kyphoscoliosis Associated with Giant Dural Ectasia in a 19-Year-Old Patient with Neurofibromatosis Type 1. Case Report Nicolas Plais 1
&
Peter H. Connolly 2 & Renaud Lafage 3 & Debra Jacobs 3 & Virginie Lafage 3 & Frank Schwab 3
Accepted: 13 August 2020 # Springer Nature Switzerland AG 2020
Abstract Dystrophic curves in neurofibromatosis type I (NF-1) are refractory to conservative treatment. The association with dural ectasia is a rare entity with a high risk of spine dislocation that often requires complex surgical treatment. We present the case of a 19-year-old woman diagnosed with NF-1 and a lumbar scoliosis associated with giant dural ectasia. She was complaining of pain and progressive collapse of her spine. Complementary exams revealed an extended lumbar dural ectasia with scalloping erosion of the anterior and posterior column of the spine as well as missing pedicles at L3, L4, and L5. Due to the severity of the spinal deformity and progressive collapse, surgery was undertaken. The patient underwent a two-stage surgery with an anterior and a posterior approach. Five interbody titanium mesh cages were inserted at the lumbar disc levels from S1 to L1 and a posterior instrumentation was placed to reduce the kyphosis and stabilize the spine. Iliac bone graft, allograft, BMP (bone morphogenetic protein), and two fibular grafts were placed to ensure fusion. The lumbar kyphosis was reduced and the spine was stabilized. The patient had excellent outcome at 28 months follow-up with complete resolution of symptoms. Dystrophic deformities with kyphoscoliosis and giant dural ectasia in NF-1 require reduction of the kyphosis, stabilization of the spine with and anterior and posterior approach, and the use of both autologous and allogenic bone graft to achieve a solid fusion. It’s necessary to avoid any injury of the dural sac during the procedure. Keywords Dural ectasia . Tarlov cyst . Neurofibromatosis type 1 . Kyphoscoliosis . Dystrophic spinal deformity . Anterior and posterior approach Level of evidence: 5 Key Points • Dystrophic curves in Neurofibromatosis type 1 are refractory to conservative treatment. • The association with dural ectasia is a rare entity with a high risk of spine dislocation that often requires complex surgical treatment. • These complex deformities require the reduction of the kyphosis, the stabilization of the spine with an anterior and posterior approach and the use of both autologous and allogenic bone graft to achieve a solid fusion. This article is part of the Topical Collection Surgery * Nicolas Plais [email protected] 1
Departement of Orthopaedic Surgery, Hospital Universitario San Cecilio, Paseo del Agua, 15, Parque del Cubillas, 18220 Albolote, Granada, Spain
2
Vascular Surgery Service, New York-Presbyterian Hospital/Weill Cornell Medical Center, New York, NY, USA
3
Department of Orthopedic Surgery, Hospital for Special Surgery, New York, NY, USA
SN Compr. Clin. Med.
Orthopedic disorders and more specifically, spinal abnormalities are the most
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