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Surgical management of omega deformity in a patient with neurofibromatosis type 1: a case report

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Abstract

Purpose

To describe the surgical treatment in a patient with a partial omega deformity in the thoracic spine with neurofibromatosis type 1.

Methods

The patient was a 55-year-old man with an omega deformity, which is defined as a curvature in which the end vertebra is positioned at the level of, above, or below the apical vertebra (i.e., a horizontal line bisecting it). We performed halo gravity traction (HGT) for 7 weeks, followed by posterior spinal instrumented nearly equal in situ fusion from T2–L5 with three femoral head allografts and a local bone autograft. We avoided reconstruction of the thoracic anterior spine because of his severe pulmonary dysfunction.

Results

HGT improved the % vital capacity from 32.5 to 43.5%, and improved the Cobb angle of the kyphosis from > 180° before traction to 144° after traction.

The Cobb angle of kyphosis and scoliosis changed from > 180° preoperatively to 155° and 146°, respectively, postoperatively, and 167° and 156°, respectively, at final follow-up. His postoperative respiratory function deteriorated transiently due to bilateral pleural effusions and compressive atelectasis, which was successfully treated with a frequent change of position and nasal high flow for 1 week. At final follow-up, his pulmonary function improved from 0.86 to 1.04 L in VC, and from 32.5 to 37.9% in %VC. However, there was no overall improvement in preoperative distress following surgery, although his modified Borg scale improved from 3 preoperatively to 0.5 postoperatively. One month after discharge, he felt worsening respiratory distress (SpO2:75%) and was readmitted for pulmonary hypertension for 2 months. He was improved by non-invasive positive pressure ventilation (biphasic positive airway pressure) for 1 week, medication and daily lung physiotherapy. Thereafter, he has been receiving permanent daytime (0.5 L/min) and nighttime (2 L/min) oxygen therapy at home. A solid arthrodesis through the fusion area was confirmed on computed tomography. However, the kyphosis correction loss was 12° (i.e., 155°–167°), while the scoliosis correction loss was 10° (i.e., 146°–156°) at 2 years of recovery.

Conclusions

We suggest that nearly equal in situ fusion is a valid option for preventing further deformity deterioration and avoiding fatal complications.

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Acknowledgements

We thank Masaya Hiramatsu1, Yuki Hori1, and Tomoyuki Horikawa1 who contributed to the study but do not fulfill all the criteria for authorship, and Edanz (https://jp.edanz.com/ac) for editing a draft of this manuscript.

Funding

The authors received no financial and material support associated with this manuscript.

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Authors and Affiliations

  1. Department of Orthopaedic Surgery, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu, 501-1194, Japan

    Chizuo Iwai, Satoshi Nozawa, Kazunari Yamada & Haruhiko Akiyama

  2. Department of Orthopaedic Surgery, Gifu Prefectural General Medical Center, Gifu, Japan

    Kazunari Fushimi

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  1. Chizuo Iwai
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  2. Satoshi Nozawa
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  3. Kazunari Fushimi
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Correspondence to Satoshi Nozawa.

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Iwai, C., Nozawa, S., Fushimi, K. et al. Surgical management of omega deformity in a patient with neurofibromatosis type 1: a case report. Eur Spine J (2024). https://doi.org/10.1007/s00586-024-08288-5

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