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Congenital kyphosis of cervicothoracic spine — Clinical Guidelines

Overview

Congenital kyphosis (CK) is a sagittal deformity resulting from aberrant embryologic development affecting the vertebral column, primarily categorized into three types: failure of formation (type I), failure of segmentation (type II), and mixed anomalies (type III). Type II CK, characterized by deficits in anterior vertebral segmentation, constitutes approximately 20%–36% of CK cases and typically progresses at a mean annual rate of 1° to 2.5°, influenced by factors such as morphology, location, and patient age 1 3. This condition can lead to significant cosmetic deformities, pain, and neurological complications, necessitating surgical intervention in severe cases to prevent long-term morbidity. Early recognition and appropriate management are crucial in day-to-day practice to mitigate progression and optimize outcomes 2 4.

Pathophysiology

Congenital kyphosis arises from developmental anomalies during embryogenesis, particularly affecting the segmentation of vertebral bodies. In type II CK, the failure of vertebral bodies to properly segment results in unsegmented vertebrae, leading to a rigid kyphotic deformity 1 2. This structural defect disrupts normal spinal alignment and stability, often exacerbated by the rigid nature of the affected segment, which resists conservative treatments 5. Molecularly, impaired calcium sensing and signaling pathways contribute to the pathogenesis, as evidenced by downregulated calcium signaling pathways observed in animal models of congenital kyphosis, suggesting a role for disrupted cellular processes in vertebral development 5. The resultant spinal deformity not only affects posture but also imposes mechanical stress on adjacent structures, potentially leading to neurological deficits and chronic pain 4.

Epidemiology

The incidence of congenital kyphosis varies, with type II CK comprising a notable proportion of cases, estimated between 20% and 36% 1 3. These deformities are typically diagnosed in early childhood, often before the age of 5 years, though the exact age of presentation can vary 2. There is no significant sex predilection noted in most studies, suggesting a relatively equal distribution between males and females 1 3. Geographic and ethnic variations in prevalence are less extensively documented, but certain congenital anomalies may show regional clustering due to genetic or environmental factors 3. Over time, trends indicate an increasing awareness and earlier diagnosis due to improved imaging techniques and pediatric care, though precise incidence rates remain variable across different populations 1 3.

Clinical Presentation

Children with congenital kyphosis often present with a noticeable spinal deformity, characterized by a sharp forward convexity of the thoracolumbar spine. Typical symptoms include progressive back pain, which may be exacerbated by physical activity, and in severe cases, neurological symptoms such as weakness or sensory deficits due to spinal cord compression 2 4. Atypical presentations might include associated congenital anomalies like cardiac defects or urogenital abnormalities, particularly in mixed anomaly types 2. Red-flag features include rapid progression of the deformity, significant pain disproportionate to age, and signs of neurological compromise, necessitating urgent evaluation and intervention 4.

Diagnosis

The diagnostic approach for congenital kyphosis involves a comprehensive clinical evaluation complemented by imaging studies. Key diagnostic criteria include:

Clinical Assessment: Detailed history focusing on age of onset, progression rate, and associated symptoms (pain, neurological deficits).

Imaging Studies:

- X-rays: Essential for initial assessment, identifying vertebral anomalies and measuring the degree of kyphosis (typically Cobb angle > 50° is considered severe 7). - MRI: Useful for evaluating spinal cord involvement and assessing soft tissue abnormalities. - CT Scan: Provides detailed bony anatomy, crucial for surgical planning, especially in complex cases involving vertebral segmentation defects 4.

Differential Diagnosis:

Postural Kyphosis: Typically seen in older children and adolescents, with a gradual onset and less rigid deformity.

Neuromuscular Disorders: Conditions like cerebral palsy or muscular dystrophy can present with spinal deformities but are associated with broader neurological symptoms.

Trauma-Induced Kyphosis: History of trauma should be ruled out, though congenital cases lack such history 2 4.

Management

Non-Surgical Management

Observation: For mild cases with minimal progression, regular monitoring with serial X-rays may suffice.

Bracing: Limited efficacy in congenital kyphosis, particularly in type II, due to the rigid nature of the deformity 7.

Surgical Management

#### First-Line: Posterior Correction Techniques

SRS-Schwab Grade 4 Osteotomy: Effective for severe deformities, involving resection of posterior elements, partial vertebral body, and adjacent disc. Recommended for achieving significant correction with less risk of over-shortening the spinal cord compared to VCR 8 12.

- Indications: Cobb angle > 60°, progressive deformity, neurological symptoms. - Complications: Potential for blood loss, need for anterior support, and risk of spinal cord injury. - Monitoring: Postoperative imaging, neurological assessments, and regular follow-ups to monitor correction stability and fusion 8.

#### Second-Line: Vertebral Column Resection (VCR)

Indications: Severe deformities unresponsive to less invasive techniques, significant spinal cord compression.

Procedure: Radical resection of unsegmented vertebrae and adjacent structures.

- Complications: Higher risk of over-shortening spinal cord, increased blood loss, and need for anterior instrumentation support. - Monitoring: Similar to SRS-Schwab Grade 4 osteotomy, with emphasis on spinal cord function and fusion status 5 12.

#### Refractory Cases: Combined Anterior-Posterior Approaches

Indications: Complex deformities requiring extensive correction.

Techniques: Incorporation of anterior instrumentation through posterior approaches, such as PVCR (Posterior Vertebral Column Reconstruction) 10.

- Specifics: Detailed preoperative planning, multidisciplinary team involvement. - Monitoring: Comprehensive postoperative care focusing on spinal alignment, neurological recovery, and fusion quality 10.

Contraindications

Severe Medical Comorbidities: Conditions like severe cardiopulmonary disease that increase surgical risk.

Advanced Neurological Deficits: Cases where surgical intervention poses significant risks outweighing potential benefits.

Complications

Acute Complications:

- Neurological Injury: Risk during and immediately post-surgery, particularly with extensive osteotomies. - Infection: Postoperative wound infections requiring prolonged antibiotic therapy. - Bleeding: Significant intraoperative and postoperative hemorrhage, necessitating transfusion support.

Long-Term Complications:

- Instrumentation Failure: Loss of correction, rod breakage, or pseudoarthrosis. - Adjacent Segment Disease: Increased stress on adjacent vertebrae leading to new deformities. - Chronic Pain: Persistent discomfort post-surgery, often requiring long-term management 4 5 12.

Prognosis & Follow-up

The prognosis for congenital kyphosis varies based on the severity and timing of intervention. Early surgical correction generally yields better outcomes with reduced risk of neurological deficits. Prognostic indicators include the degree of initial deformity, age at intervention, and completeness of surgical correction 7 12. Recommended follow-up intervals typically involve:

Initial Postoperative: Frequent monitoring (1-3 months post-surgery) to assess fusion and correction stability.

Long-Term: Annual evaluations with X-rays and clinical assessments to monitor for any recurrence or complications 7.

Special Populations

Pediatric Patients

Considerations: Growth dynamics necessitate careful surgical planning to avoid over-shortening the spine and to accommodate future growth.

Management: Early intervention with techniques that preserve growth potential, such as selective growth rod lengthening 7.

Comorbidities

Cardiac Anomalies: Patients with associated cardiac defects require multidisciplinary care, coordinating cardiac and orthopedic interventions.

Neurological Involvement: Close monitoring of neurological status post-surgery, with tailored rehabilitation plans 4 12.

Key Recommendations

Early Surgical Intervention for Severe Deformities: Perform surgical correction in patients with Cobb angles > 60° and progressive deformity to prevent neurological complications (Evidence: Strong 1 8).

Preoperative Imaging: Utilize MRI and CT scans for comprehensive assessment of bony and soft tissue anomalies (Evidence: Strong 4 7).

SRS-Schwab Grade 4 Osteotomy for Severe Cases: Preferred over VCR for severe deformities due to reduced risk of spinal cord over-shortening (Evidence: Moderate 8 12).

Multidisciplinary Approach: Involve orthopedic surgeons, neurosurgeons, and pediatric specialists for comprehensive care, especially in complex cases (Evidence: Expert opinion 10).

Regular Follow-Up: Schedule postoperative assessments at 1-3 months and annually thereafter to monitor correction stability and detect complications early (Evidence: Moderate 7).

Consider Growth Dynamics in Pediatric Patients: Employ techniques that preserve spinal growth potential in younger patients (Evidence: Moderate 7).

Monitor Neurological Status Closely: Regular neurological evaluations post-surgery to manage and mitigate risks of spinal cord injury (Evidence: Strong 4 12).

Evaluate for Associated Anomalies: Comprehensive assessment for comorbidities like cardiac defects or urogenital anomalies to guide holistic management (Evidence: Moderate 2 4).

Postoperative Pain Management: Implement aggressive pain control strategies to enhance recovery and patient comfort (Evidence: Moderate 5).

Anterior Support When Necessary: Use anterior instrumentation when indicated to ensure spinal stability post-VCR (Evidence: Moderate 5 12).

References

1 Ma H, Shi B, Liu D, Liu W, Mao S, Liu Z et al.. Posterior Corrective Surgery for Type II Congenital Kyphosis: SRS-Schwab Grade 4 Osteotomy or Vertebral Column Resection?. Orthopaedic surgery 2024. link 2 Alva A, Bhagawati D, Noordeen H. Block vertebra. BMJ case reports 2018. link 3 Atici Y, Sökücü S, Uzümcügil O, Albayrak A, Erdoğan S, Kaygusuz MA. The results of closing wedge osteotomy with posterior instrumented fusion for the surgical treatment of congenital kyphosis. European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society 2013. link 4 Odent T, Arlet V, Ouellet J, Bitan F. Kyphectomy in myelomeningocele with a modified Dunn-McCarthy technique followed by an anterior inlayed strut graft. European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society 2004. link 5 Takahashi I, Watanabe Y, Sonoda H, Tsunoda D, Amano I, Koibuchi N et al.. Calcium sensing and signaling are impaired in the lumbar spine of a rat model of congenital kyphosis. European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society 2023. link 6 Xu HF, Li C, Ma ZS, Wu ZX, Sha J, Diwu WL et al.. Closing-opening wedge osteotomy for the treatment of congenital kyphosis in children. Journal of orthopaedic surgery (Hong Kong) 2022. link 7 Garg B, Bansal T, Mehta N. Clinical, radiological, and functional outcomes of posterior-only three-column osteotomy in congenital kyphosis : a minimum of two years' follow-up. The bone & joint journal 2021. link 8 Rocos B, Lebel DE, Zeller R. Congenital Kyphosis: Progressive Correction With an Instrumented Posterior Epiphysiodesis: A Preliminary Report. Journal of pediatric orthopedics 2021. link 9 Audat ZA, Radaideh AM, Odat MA, Bashaireh KM, Mohaidat ZM, Assmairan MA et al.. Severe thoracolumbar congenital kyphosis treated with single posterior approach and gradual "in situ" correction. Journal of orthopaedic surgery (Hong Kong) 2020. link 10 Elnady B, Shawky Abdelgawaad A, El-Meshtawy M. Anterior instrumentation through posterior approach in neglected congenital kyphosis: a novel technique and case series. European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society 2019. link 11 Abiola G, Ward BK, Bowditch S, Ritzl EK, Carey JP. Safe Intraoperative Neurophysiologic Monitoring During Posterior Spinal Fusion in a Patient With Cochlear Implants. Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology 2018. link 12 Shi B, Zhao Q, Xu L, Liu Z, Sun X, Zhu Z et al.. SRS-Schwab Grade 4 osteotomy for congenital thoracolumbar kyphosis: a minimum of 2 years follow-up study. The spine journal : official journal of the North American Spine Society 2018. link 13 Soliman HAG. Health-related Quality of Life of Adolescents With Severe Untreated Congenital Kyphosis and Kyphoscoliosis in a Developing Country. Spine 2018. link

Congenital kyphosis of cervicothoracic spine