Surgical Management of Atypical Scheuermann's Disease with Spinal Cord Compression: Reporting a Rare Case and Literature Review

Abstract

Atypical Scheuermann's disease is a rare spinal disorder characterized by progressive kyphotic deformity and, in rare instances, spinal cord compression. Unlike classic Scheuermann's disease, which affects the thoracic spine, atypical variants may involve the thoracolumbar or lumbar regions, leading to neurological compromise. This report presents a rare case of atypical Scheuermann's disease with spinal cord compression, its surgical management, and a review of relevant literature. A 24-year-old male presented with progressive lower limb weakness, gait instability, and back pain for 6 months. Neurological examination revealed spastic paraparesis, hyperreflexia, and sensory deficits below the affected spinal level. Imaging studies, including magnetic resonance imaging and computed tomography, demonstrated a severe thoracolumbar kyphotic deformity with vertebral wedging, disc degeneration, and posterior ligamentous hypertrophy leading to significant spinal cord compression. Considering the progressive neurological deficits, the patient underwent posterior decompression, deformity correction, and instrumented fusion. A pedicle subtraction osteotomy was performed to restore sagittal alignment, followed by posterior spinal stabilization using pedicle screw fixation. Postoperatively, the patient showed gradual neurological improvement with enhanced motor function and pain relief. At the 6-month follow-up, the patient exhibited significant recovery in motor function and ambulation. Radiological evaluation confirmed adequate correction of kyphosis and stable spinal instrumentation. Rehabilitation continued with physiotherapy to improve strength and balance. Atypical Scheuermann's disease with spinal cord compression is exceedingly rare, necessitating early diagnosis and timely surgical intervention to prevent irreversible neurological deficits. This case highlights the role of posterior decompression, osteotomy, and fusion in achieving favorable outcomes. A literature review underscores the importance of individualized surgical planning based on severity, neurological status, and spinal alignment. Early recognition and surgical intervention are crucial in managing atypical Scheuermann's disease with spinal cord compression. Posterior decompression and instrumented fusion effectively restore function and prevent long-term disability.

Keywords

atypical Scheuermann's disease - spinal cord compression - posterior decompression - thoracic kyphosis

Introduction

Scheuermann's disease is a structural kyphosis characterized by vertebral body wedging, irregular endplates, and disc space narrowing.[1] It predominantly affects adolescents and may result in long-term complications such as chronic low back pain, progressive deformity, and, in rare cases, spinal cord compression.[2] The exact cause of Scheuermann's disease remains unknown, though it is believed to be due to genetic, hormonal, and mechanical factors, characterized by[3] [4] [5]:

1. Vertebral body wedging: anteriorly by at least 5 degrees in three consecutive vertebrae

2. Irregular endplates: Schmorl's nodules indenting the endplates

3. Disc space narrowing: resulting in loss of disc height, progressively leading to kyphotic deformity

The kyphotic deformity progressively leads to significant biomechanical stress on the spine, accompanied by compensatory hyperlordosis of the lumbar region, severe chronic back pain, and functional disability. In adults, intervertebral discs degenerate, exacerbating these deformities and sometimes causing spinal cord compression.

Based on the location, Scheuermann's disease can be classified into two subtypes[6] [7]:

1. Typical: thoracic kyphosis

2. Atypical: lumbar/thoracolumbar region with radiological evidence like Schmorl node, limbus vertebra, > 5 degrees anterior wedging in 1 to 2 vertebral bodies, changes in endplates, narrowed disk space

In this report, we present a rare case of atypical Scheuermann's disease diagnosed in a 24-year-old male patient.

Case Presentation

A 24-year-old male patient was admitted to our neurosurgery department with complaints of gradually worsening lower back pain, weakness of both lower limbs, and gait instability with numbness and tingling in both legs without any history of previous trauma or spinal surgery. Neurological examination revealed hyperreflexia and decreased motor strength (⅗) in both lower limbs, sensory deficits below L2 level, and marked thoracic kyphosis. Subsequent magnetic resonance imaging of thoracolumbar spine revealed marked kyphosis consistent with Scheuermann's disease, prolapsed intervertebral disc, and hypertrophied ligamentum flavum, resulting in significant spinal cord compression at the L1-L3 levels[8] ([Fig. 1A]). Surgical intervention was planned to decompress the spinal cord and stabilize the spine. Preoperative lumbar spine X-ray (anteroposterior and lateral views) further demonstrated kyphotic deformity with anterior wedging of multiple lumbar vertebrae, irregular endplates, and possible Schmorl's nodules, consistent with Scheuermann's disease with atypical lumbar involvement ([Fig. 1B]).

Fig. 1 (A) Preoperative T2-weighted sagittal magnetic resonance imaging (MRI) of the thoracolumbar spine revealed marked kyphosis, consistent with Scheuermann's disease. Prolapsed intervertebral discs and hypertrophied ligamentum flavum resulted in significant spinal cord compression between the levels of L1-L3. (B) Preoperative X-ray of the lumbar spine (anteroposterior [AP] and lateral views, left and right, respectively) demonstrates kyphotic deformity with anterior wedging of multiple lumbar vertebrae, irregular endplates, and possible Schmorl's nodules, consistent with Scheuermann's disease with atypical lumbar involvement. The radiographic findings correlate with the patient's clinical presentation of lumbar myelopathy, likely due to spinal cord compression secondary to progressive kyphosis.

A midline posterior approach was used to expose the affected lumbar vertebrae. Laminectomy was performed from L1 to L3 to decompress the spinal cord with careful resection of hypertrophied ligamentum flavum. Bilateral transpedicular screws were placed from L1 to L3 under intraoperative fluoroscopic guidance. Rods were fixed after contouring to restore normal sagittal alignment and stabilization of the spine. Autologous bone grafts were used along decorticated transverse processes and laminae for fusion. Throughout the surgery, somatosensory evoked potentials and motor evoked potentials were used to monitor the neurological function. Postoperative imaging demonstrated adequate decompression of the spinal cord and satisfactory postoperative alignment of the spine ([Fig. 2A] and [B]). The patient was subsequently transferred to the intensive care unit. The patient was discharged on the 7th postoperative day with ⅘ motor strength in both lower limbs and mild sensory deficits below the level of L2, and referred to physical therapy and rehabilitation for muscle strengthening exercises. At the sixth-month follow-up, the patient showed significant improvement in motor strength (4 +/5) and ambulation. Sensory deficits markedly improved, and back pain was controlled with a conservative approach.

Fig. 2 (A) Postoperative radiograph displays the successful instrumented correction of Scheuermann's kyphosis using bilateral pedicle screw-rod fixation (L1-L3). The anteroposterior view (left) demonstrates well-positioned pedicle screws spanning the affected vertebral levels, ensuring adequate stabilization. The lateral view confirms effective kyphotic deformity correction, restoring sagittal balance and improved lumbar alignment. No signs of hardware misplacement, screw loosening, or immediate complications such as implant failure are evident. Clinically, the procedure aims to relieve spinal cord compression, improve neurological function, and enhance overall spinal mechanics. (B) Postoperative noncontrast computed tomography (NCCT) of the thoracolumbar spine demonstrates optimal pedicle screw trajectory at L1-L3, with screws positioned within the vertebral pedicles bilaterally. The medial-lateral angulation aligns with anatomical landmarks, confirming safe placement without cortical breach or encroachment on neurovascular structures. Sagittal reformats reveal restoration of normal spinal alignment. Laminectomy defects at L1-L3 are well visualized, confirming adequate posterior decompression with no residual osseous compression of the spinal canal. Screw–bone interfaces appear stable, with no evidence of loosening or hardware failure.

Discussion

Scheuermann's disease, aka juvenile kyphosis or Scheuermann's kyphosis (SK), is a structural spinal disorder, characterized by wedging of the vertebral body, endplate irregularities, and narrowing of the disc space, commonly presents during adolescence; however, its implications can extend into adulthood, necessitating surgical intervention in severe cases.[8] The current literature provides a comprehensive understanding of the disease's pathology, indications for surgery, and outcomes following surgical management. Early recognition and timely surgical intervention are essential in managing adult patients with Scheuermann's disease and preventing irreversible neurological damage.[9] Long-term follow-up and rehabilitation are critical to ensure the patient's recovery and quality of postoperative life.

Scheuermann's disease is diagnosed by anterior wedging of at least three consecutive vertebrae by 5 degrees or more, often accompanied by Schmorl's nodules and endplate irregularities. This kyphotic deformity is most commonly observed in the thoracic spine but can also affect the lumbar region. Patients typically present with poor posture and back pain during adolescence, which may progress to significant pain and disability in adulthood. The etiology of Scheuermann's disease remains uncertain, but it is believed to involve genetic, hormonal, and mechanical factors. As the disease progresses, it can lead to compensatory lumbar hyperlordosis, chronic pain, and in severe cases, neurological deficits due to spinal cord compression.

O'Donnell et al provided a comprehensive review of SK, focusing on its pathophysiology, diagnosis, and nonoperative and operative treatment strategies.[10] Although the exact etiology of SK remains unclear, it is thought to involve genetic, hormonal, and mechanical factors. Clinical presentation included poor posture, back pain, and, in severe cases, cardiopulmonary compromise due to restrictive lung disease. Nonoperative management is generally the first line of treatment, including physical therapy and bracing. The Milwaukee brace has historically been used to manage SK, effectively halting progression in growing children. Exercise programs were also recommended to strengthen the paraspinal muscles and improve posture. Nonoperative approaches were preferred for patients with mild to moderate deformity and those who are still growing. Surgical intervention was considered for patients with severe, progressive kyphosis or those who experience significant pain and functional limitations. The article discussed the evolution of surgical techniques, highlighting a trend toward posterior-only approaches due to their lower complication rates and effective correction.[10] Techniques such as posterior spinal fusion with instrumentation are commonly employed. When deciding whether to perform surgery, factors such as curve magnitude, the patient's symptoms, and other patient-specific considerations should be taken into account. Recent studies have focused on refining surgical techniques and improving patient outcomes. The study also noted advances in minimally invasive procedures and the importance of careful preoperative planning to avoid complications such as junctional kyphosis.[10] It also emphasized the role of patient-reported outcomes in assessing treatment success. They concluded that the management of SK required a nuanced approach tailored to the patient's needs.

While nonsurgical treatments are preferred for managing Scheuermann's disease, surgery is indicated in cases with severe kyphotic deformity (typically over 75 degrees), persistent pain unresponsive to conservative measures, and neurological deficits due to spinal cord compression. Betz and Samdani reviewed surgical strategies for Scheuermann's disease, emphasizing three core goals: decompression of neural structures, spinal stabilization, and deformity correction.[6] They identified surgical candidacy based on severe kyphosis (> 75 degrees), refractory pain, or neurological compromise. The authors highlighted posterior spinal fusion with modern instrumentation as the gold standard, achieving reliable correction while minimizing morbidity. They noted osteotomies (e.g., Ponte or pedicle subtraction) as critical for sagittal realignment and stressed the importance of preoperative planning to address rigid deformities. Their work underscored surgery's role in improving quality of life through pain reduction and functional restoration, while cautioning about potential complications like junctional kyphosis.

Regarding the different surgical treatment options, posterior-only approaches have gained popularity in recent years due to their effectiveness in correcting deformity and providing stabilization. Ouellet and Mac-Thiong also reported successful outcomes in their multicenter series, noting that posterior surgery for SK resulted in substantial deformity correction and pain relief.[11] They emphasized the importance of careful patient selection and preoperative planning to minimize complications and optimize outcomes. In cases with severe kyphosis, a combined anterior-posterior approach may be necessary. This involves an anterior release and discectomy to address the rigid deformity, followed by posterior instrumentation and fusion.

The study by Lee et al reviewed the clinical outcomes of treating SK using a posterior-only surgical approach with pedicle screw fixation.[8] The research aimed to evaluate the effectiveness and safety of this method in correcting kyphotic deformities. The procedure involved the removal of the posterior elements (laminae and ligamentum flavum) to decompress the spinal cord, followed by the placement of pedicle screws and rods to stabilize the spine and restore sagittal alignment. This approach aimed to avoid anterior surgery, thereby reducing surgical risks and recovery time. Outcomes were assessed using radiographic measurements to determine the degree of kyphosis correction and clinical evaluations to gauge pain relief and functional improvements. The Scoliosis Research Society (SRS) outcomes questionnaire was used to measure patient satisfaction and quality of life. Significant improvements in spinal alignment were observed postsurgery. The mean preoperative kyphosis angle was notably reduced, demonstrating the effectiveness of the posterior-only approach in correcting the deformity. Patients reported substantial pain relief and improvements in physical function following the surgery. The SRS outcomes scores indicated high levels of patient satisfaction. The study documented a low rate of complications, with no significant neurological deficits reported. Minor complications included superficial wound infections, which were effectively managed. The authors discussed the advantages of the posterior-only approach, including reduced surgical time, less blood loss, and a lower risk of complications compared with combined anterior-posterior approaches. They emphasized the importance of proper patient selection and surgical planning to maximize outcomes. They concluded that the posterior-only approach using pedicle screw fixation is a safe and effective method for treating SK. The technique provided significant deformity correction and pain relief, with a low complication rate and high patient satisfaction.

Winter et al outlined a structured approach for adult SK management, emphasizing surgical intervention for severe pain, neurological decline, or deformities exceeding 75-degree thoracic kyphosis.[12] The authors advocated posterior-only approaches with modern pedicle screw instrumentation for flexible deformities, achieving approximately 50% correction, while reserving combined anterior-posterior techniques (e.g., discectomy/corpectomy with posterior fusion) for rigid cases requiring anterior release. Osteotomies, such as pedicle subtraction osteotomy, were pivotal for sagittal realignment. Although combined approaches yielded greater correction, they posed a higher risk of pseudoarthrosis and hardware failure than posterior-only strategies. The study underscored the value of intraoperative neuromonitoring to mitigate neurological complications, reinforcing the need for individualized planning based on deformity rigidity and patient factors. These principles aligned with our posterior-only approach for atypical Scheuermann's disease, balancing correction efficacy with reduced morbidity.

Surgical management of Scheuermann's disease is not without risks. Potential complications include infection, hardware failure, pseudoarthrosis, and neurological injury. However, with advancements in surgical techniques and intraoperative monitoring, the incidence of these complications has significantly decreased. Cheng et al examined the surgical treatment of severe adult SK through a detailed case series.[13] The study provides long-term follow-up data, focusing on surgical outcomes, complications, and patient satisfaction. They reported a low complication rate in their series, attributing this to meticulous surgical technique and the use of intraoperative neuromonitoring to ensure spinal cord integrity. The study reported significant correction of kyphotic deformity postsurgery, with substantial improvements in the sagittal profile of the spine. Average kyphosis angles were reduced to within the normal range. Patients experienced considerable pain relief and reported high satisfaction levels with the surgical outcomes. Improvements in physical function and quality of life were also noted. The study identified some complications associated with the surgeries, including hardware-related issues and wound infections. However, these were managed effectively, and no long-term adverse effects were reported. Follow-up data over several years indicated that the surgical corrections were maintained, and the benefits in pain relief and functional improvement were sustained.

Long-term follow-up studies have shown that surgical correction of SK can improve spinal alignment, pain relief, and overall quality of life. Vera et al demonstrated that patients undergoing surgical correction of SK experienced significant improvements in cardiorespiratory function, further highlighting the multifaceted benefits of surgical intervention.[14] In their study, postoperative assessments showed substantial improvements in pulmonary function. The values of forced vital capacity and forced expiratory volume in 1 second increased, indicating enhanced lung capacity and airflow. The results demonstrated an increase in peak oxygen uptake (VO₂ max), reflecting improved cardiovascular efficiency and exercise tolerance. Radiographic evaluations confirmed substantial correction of the kyphotic deformity, contributing to the enhanced thoracic cavity volume and respiratory mechanics. Patients reported reduced pain and improved quality of life, correlating with the objective improvements in cardiorespiratory function.

Sebaaly et al comprehensively reviewed the current understanding of SK and its management.[4] The article covered the pathophysiology, clinical presentation, diagnostic criteria, and evolving surgical treatment strategies for SK. The severity of the kyphosis was assessed using the Cobb angle on lateral spine radiographs. Nonsurgical treatment was primarily reserved for patients with mild deformities and those who were skeletally immature. This approach included physical therapy, bracing, and pain management. The goal was to halt the progression of the kyphosis and alleviate symptoms. Surgery was indicated for patients with severe deformities, significant pain, or neurological deficits that do not respond to conservative treatment. Historically, a combined anterior and posterior approach was the gold standard. However, there had been a shift toward posterior-only approaches with segmental pedicle screw fixation, which had shown favorable outcomes with fewer complications. Advancements in surgical techniques and instrumentation drove this shift. The authors proposed a treatment algorithm that considered the severity of the deformity, the presence of symptoms, and the patient's skeletal maturity. For skeletally immature patients with mild deformities, nonsurgical management was recommended. For those with severe deformities or intractable symptoms, surgical intervention was advised. The posterior-only approach was preferred for its effectiveness and reduced morbidity. In conclusion, this article provided an up-to-date overview of SK, emphasizing the importance of individualized treatment plans based on the severity of the deformity and patient-specific factors.

Conclusion

The surgical management of Scheuermann's disease, particularly in cases with significant kyphotic deformity and neurological deficits, has evolved significantly over the past decade. Advances in surgical techniques, instrumentation, and intraoperative monitoring have improved the safety and efficacy of these procedures, leading to better patient outcomes. While nonsurgical treatments remain the first line of management, timely surgical intervention is crucial for patients with severe deformities and neurological compromise. Future research should focus on long-term outcomes of various surgical approaches, the role of minimally invasive techniques, and the development of guidelines for optimal patient selection and management. As our understanding of Scheuermann's disease continues to grow, it is essential to integrate new insights into clinical practice to provide the best possible care for patients suffering from this challenging condition.

Conflict of Interest

None declared.

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Publication History

Article published online:
19 November 2025