Suspected Spinal Cord Compression in Children and Young People with Malignancy - Management of

Publication: 20/02/2015  
Next review: 07/08/2023  
Clinical Guideline
CURRENT 
ID: 4114 
Approved By: Trust Clinical Guidelines Group 
Copyright© Leeds Teaching Hospitals NHS Trust 2020  

 

This Clinical Guideline is intended for use by healthcare professionals within Leeds unless otherwise stated.
For healthcare professionals in other trusts, please ensure that you consult relevant local and national guidance.

Management of Suspected Spinal Cord Compression in Children and Young People with Malignancy

Background

Compression of the spinal cord and/or cauda equina is an oncological emergency that may lead to permanent, avoidable paralysis if not treated effectively. Treatment guidelines are available for patients of 18 years and older following NICE guidelines.

Spinal cord compression can occur acutely in children with cancer. Rapid recognition of early signs and early, effective treatment maximise the chances of full recovery.

Cord compression can occur by a number of mechanisms including:

  • Direct spread of tumour
  • Extension of tumour through vertebral foramina into epidural space
  • Bony disease within vertebrae with secondary cord compression

Sarcoma is the underlying diagnosis in the majority of cases, with neuroblastoma, lymphoma and leukaemia making up the bulk of the rest of cases. Occasionally leptomeningeal disease (e.g. metastatic medulloblastoma) can cause diffuse cord compression with less reliable localising signs. Primary spinal tumours and intraspinal metastases can also cause acute cord neurological dysfunction.

Cord compression may be the presenting feature of a new malignancy, or more frequently occurs as a consequence of metastatic disease progression or relapse.

Back pain occurs in up to 80% of children, and abnormal bladder or bowel function is common. Any patient with this combination of symptoms needs urgent investigation to rule out cord compression. Sensory abnormalities are less common, but need to be taken seriously when they occur. Patients (and their carers) who have a risk of spinal cord compression secondary to the site of the primary tumour should be made aware of such symptoms, and advised to seek urgent review if any develop.

A child with cancer who develops back pain should be considered to have spinal cord compromise until proved otherwise. The absence of weakness or sensory abnormalities does not reliably exclude the diagnosis.

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Diagnosis

The signs and symptoms of spinal cord compression vary with age, site of the lesion and nature of the disease process. A focused history and examination should be undertaken prior to investigations, addressing:

History
Take detailed history paying particular attention to the location and nature of pain, if present. Pain when straining to open bowels and pain at night are particularly concerning.

Ask about weakness, gait, bladder and bowel function (constipation, retention or incontinence) and any sensory abnormality (including perineal).

Establish the time since onset of symptoms and document the speed of progression.

Examination
Detailed neurological examination is mandatory

Most patients with cord compression have weakness; the degree and location depend on the site of compression.

Sensory abnormalities and sensory levels are often more difficult to identify

The bony spine should be palpated for tenderness, which is a reliable localising sign if present.

Clinical examination often allows compressive lesions to be localised (See Table 1)

TABLE 1 : CLINICAL LOCALISATION OF EPIDURAL CORD COMPRESSION

SIGN

SPINAL CORD

CONUS

CAUDA EQUINA

WEAKNESS

Symmetric, profound

Symmetric, variable

Asymmetric
May be mild

TENDON REFLEXES

Increased or absent

Increased knee,
decreased ankle

Decreased, asymmetric

babinski

Extensor

Extensor

Plantar

sensory

Symmetrical
Sensory level

Symmetrical
Saddle anaesthesia

Asymmetric
Radicular

sphincter
abnormality

Spared until late

Early involvement

May be spared

progression

Rapid

Variable, may be rapid

Variable, may be rapid

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Investigation

The immediate management of spinal cord compression is aimed at confirming the diagnosis to institute measures to prevent permanent nerve damage.

Pre and Post Contrast MRI spine is the investigation of choice, and should be performed urgently, absolutely before 24 hours have elapsed.  For children presenting in District General Hospitals with a strong suspicion of spinal cord compression, urgent transfer to a principal treatment centre is recommended to undertake further investigation and management. In some instances (for example, patients with advanced disease in the terminal phase of their illness) this approach may not be warranted and discussion of all cases with the Consultant Paediatric Haematologist/Oncologist is advised.

In most instances the whole spine is imaged. This is mandatory if there is any doubt about the level involved or there is a likelihood of multiple lesions (e.g. medulloblastoma). The conus should be included as this is a common site for metastatic central nervous system tumours, but may be difficult to image fully with MRI.

The case should be discussed with the Consultant Neuroradiologist on-call to arrange imaging. In addition, an anaesthetist will frequently be required in the case of young children.

Spine radiographs are not diagnostic in suspected spinal cord compression, nor can they rule out a problem. They may have a limited role in assessment of the vertebral bodies, although 50% of children will have normal x-rays; if vertebral body stability is questioned, close consultation should be undertaken to decide if CT rather than plain x-rays

Routine investigation should also include haematological and biochemical assessment (FBC, U&E, calcium, LFTs) to assess potential other disease processes and further complications.

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Treatment / Management

The immediate management of spinal cord compression is aimed at preventing further injury whilst the underlying cause is identified and definitive management instituted.

All patients must be discussed with Oncology/Haematology Consultant. The neurosurgical/spinal surgical team and Clinical Oncologists (Paediatric Radiotherapist) should also be included in these discussions.

Definitive Management
Management is complex and requires the input of a Consultant Haematologist/Oncologist in consultation with Neurosurgery/Spinal Surgery and Clinical Oncology. The notes below explain some of the issues involved, but should not be seen as an alternative to a Consultant-led decision. Depending on the urgency and management route decided upon, it may be reasonable to wait until working hours before contacting the Clinical Oncology team.

Definitive management requires cord decompression which can be achieved in a number of ways; the most appropriate course of action depends on a number of factors including diagnosis (or lack of), speed of symptom progression and phase of illness e.g. presentation vs. terminal phase.

Dexamethasone can be given to reduce cord oedema associated with the compressive lesion. At best it can buy some time for imaging and allow definitive management to be arranged. The evidence underpinning dosing schedules is weak. If cord compression is suspected Dexamethasone (0.25mg/kg, max 16mg/day) may be given IV prior to emergency MRI scan. Dexamethasone should be continued onwards on the advice of the neurosurgical/clinical or paediatric oncology teams, usually as a once daily dose (although split doses may be considered), for two weeks, tapering after 3 days or as soon as clinically indicated (see BNFc). Note steroids are relatively contraindicated in suspected fungal infection.

If an epidural mass is demonstrated the spinal cord should be urgently decompressed if appropriate. Although corticosteroids reduce oedema rapidly and result in neurological improvement they are not an alternative to cord decompression.

Decompression may be achieved by surgery, local radiotherapy, or chemotherapy (depending on the underlying condition). In some children e.g. infants with neuroblastoma, or children with lymphoma, chemotherapy may be rapidly effective with fewer long-term side effects than other modalities.

When the diagnosis is uncertain, surgery provides an opportunity to biopsy the lesion in addition to treating the mass and may be the treatment of choice in good prognosis patients with surgically operable masses.

In children with a radiosensitive but not rapidly chemosensitive tumour, urgent radiotherapy is often preferable to other approaches.  Dexamethasone is continued during radiotherapy.

Please note: management is complex and requires the input of a Consultant Haematologist/Oncologist. The notes above explain some of the issues involved, but are not an alternative to a Consultant-led decision.

Supportive Management
Children and young adults with suspected spinal cord compression require close and attentive supportive care. The following issues may need to be addressed:

  • Spinal stability.
    Lesions infiltrating bone may significantly weaken the spine and until proven otherwise the child should be nursed flat with a neutral spine. Care of pressure areas is important
  • Pain.
    Significant pain may require opiates or analgesia for neuropathic pain such as gabapentin
  • Constipation.
    Efforts should be made to maintain soft and regular bowel motions
  • Urinary retention.
    Catheterisation may be required
  • Side effects of dexamethasone
    Hyperglycaemia, hypertension and gastric erosion may occur. Consider monitoring +/- gastric acid suppression
  • Biochemical derangement.
    Although uncommon, children and young people may suffer hypercalcaemia as part of an infiltrative process. They may also undergo tumour lysis and renal dysfunction secondary to dexamethasone therapy (in leukaemia/lymphoma)
  • Thromboprophylaxis.
    Consideration should be given to use of stockings +/- LMWH in those at very high risk of thrombosis as per local guidelines

Following treatment of spinal cord compression, discussion with physiotherapy should be undertaken to assess the need for, and manage, a rehabilitation programme.

Provenance

Record: 4114
Objective:

Aims

To improve the diagnosis and management of spinal cord compression in children and young people with malignant disease.

Objectives

To provide recommendations for appropriate diagnosis, investigation and management of spinal cord compression in children and young people (of less than 18 years) with malignant disease.

Clinical condition:

Spinal cord compression

Target patient group: Children and Teenagers under management of paediatric/TYA oncology service with suspected spinal cord compression
Target professional group(s): Secondary Care Doctors
Secondary Care Nurses
Adapted from:

Evidence base

References  and Evidence levels:
A. Meta-analyses, randomised controlled trials/systematic reviews of RCTs
B. Robust experimental or observational studies
C. Expert consensus.
D. Leeds consensus. (where no national guidance exists or there is wide disagreement with a level C recommendation or where national guidance documents contradict each other)

Further Information and References

Based on: Neurological Emergencies: Spinal Cord Compression, Sheffield Children’s Hospital Clinical Guidelines. D Yeomanson and M Gerrard, May 2008

Oncologic Emergencies (chapter 39) in Principles and Practice of Pediatric Oncology. Eds Pizzo & Poplack, 5th edition (2006)

NICE. Metastatic spinal cord compression. Diagnosis and management of adults at risk of and with metastatic spinal cord compression. CG 75 (2008)

Neurological and Neuromuscular Symptoms (chapter 26) in Oxford Textbook of Palliative Care for Children. Eds Goldman, Hain & Liben, 1st Edition (2006)

Updated Systematic Review and Clinical Practice Guideline for the Management of Malignant Extradural Spinal Cord Compression . D. Andrew Loblaw, Gunita Mitera, Michael Ford, Normand J. Laperriere, A International Journal of Radiation Oncology-Biology-Physics, 2012: 84(2): 312-317 http://dx.doi.org/10.1016/j.ijrobp.2012.01.014.

Initial bolus of conventional versus high-dose dexamethasone in metastatic spinal cord compression. Vecht CJ, Haaxma-Reiche H, van Putten WL, et al: Neurology 39:1255-1257 (1989)

De Martino, L., Spennato, P., Vetrella, S. et al. Symptomatic malignant spinal cord compression in children: a single-center experience. Ital J Pediatr 45, 80 (2019). https://doi.org/10.1186/s13052-019-0671-5

Approved By

Trust Clinical Guidelines Group

Document history

LHP version 2.0

Related information

Audit and Monitoring Compliance

This guideline reflects a rare, emergency presentation.

Each child who requires treatment under this guideline will have their case reviewed by a departmental morbidity meeting, and this guideline will be used to compare the care they received against this current statement of best practice.

The results will be presented to the paediatric haematology/oncology audit meeting, which will agree actions arising from the recommendations, and monitor the progress of the actions.

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