Recent Developments in Surgical Treatment of Spinal Deformity in Pediatric Patients: Experience from a Single-Center Series of 42 Neurofibromatosis Type 1 Patients
1
Pediatric Orthopedic Department, Altona Children’s Hospital, Bleickenallee 38, D-22763 Hamburg, Germany
2
Department of Orthopedics, University Medical Center Hamburg-Eppendorf, D-20246 Hamburg, Germany
*
Author to whom correspondence should be addressed.
Cancers 2024, 16(23), 4079; https://doi.org/10.3390/cancers16234079
Submission received: 28 September 2024
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Revised: 24 November 2024
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Accepted: 26 November 2024
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Published: 5 December 2024
(This article belongs to the Special Issue Neurofibromatosis)
Simple Summary
Approximately 60% of the patients with neurofibromatosis type 1 (NF-1) demonstrate involvement of the skeletal system, with the spine being the most commonly affected element. In the vast majority of the cases with an NF-1-associated spinal deformity, the natural history is unfavorable and worsening is observed in cases that proceed without treatment. Non-operative management is rarely successful and surgery is necessary in most of the cases, especially in the presence of the characteristic osseous dystrophic changes. Since the disease is rare and features a heterogeneous presentation, evidence-based treatment protocols are not available. The aim of this retrospective study is to share the experience and to report the outcomes after treatment of spinal deformities in pediatric patients with NF-1. The results show that good and sustainable correction of spinal deformities can be achieved by means of early surgical treatment even in immature patients. However, surgery remains challenging, and the complication rate is higher than in patients with spinal deformities of other etiologies.
Abstract
Background: The management of spinal deformities in patients with NF-1 is challenging. The study aimed to assess the outcomes of the surgical treatment of spine deformities in children with neurofibromatosis type 1 with our treatment approach. Methods: A retrospective single-center study on pediatric patients with spinal deformities associated with NF-1 who received surgical treatment between 2006 and 2024. Results: The study group comprised 42 patients with a mean age at surgery of 9.8 years. Twenty-five patients (60%) were treated by means of growth-preserving techniques and 17 patients (40%) by means of definitive fusion. Preoperative halo-gravity traction was used in 14 (33%) cases. In the group treated with a growth-preserving technique, a 54.1% mean curve correction was observed at the latest follow-up, and growth of the thoracic spine was maintained at a physiological rate; however, 25 unplanned revision surgeries (mostly due to mechanical complications) were necessary. In the group treated by definitive fusion, a 66% curve correction was achieved at initial surgery, which remained unchanged at latest follow-up, and revision surgery was performed in three cases for augmentation of the fusion mass. There was one neurological complication (2%). Another patient developed a deep wound infection (2%). Conclusions: Good and sustainable surgical correction of spinal deformities can be achieved in pediatric patients with NF-1. Due to the bony dystrophic changes, surgical treatment is challenging and the complication rate is higher than in spinal deformities of other etiologies.
Keywords:
spinal deformity; neurofibromatosis type 1
