SPINAL CORD COMPRESSION

A CASE DISCUSSION

INTP - PPO, PHO, IAP. P7 22 slides

J, 12 YEAR OLD MALE CHILD

PRESENTED WITH
PROGRESSIVE NECK SWELLING LOSS OF APPETITE AND WEIGHT VAGUE ACHES AND PAINS SKIN RASH 2MO

1WK

INTP - PPO, PHO, IAP. P7 2/22

 INVESTIGATED 3 MONTHS EARLIER FOR THROMBOCYTOPENIA BONE MARROW EXAMINATION THEN REVEALED
 NO ABNORMAL CELLS
 INCREASED MEGAKARYOCYTES

TREATED AS A CASE OF ITP WITH ORAL PREDNISONE FOR 4 WEEKS

INTP - PPO, PHO, IAP. P7 3/22

PHYSICAL EXAMINATION
SICK CHILD PALLOR GENERALISED LYMPHADENOPATHY PURPURIC SPOTS HEPATOSPLENOMEGALY NORMAL CNS EXAMINATION OTHER SYSTEMS: WNL

INTP - PPO, PHO, IAP. P7 4/22

INVESTIGATIONS
CHEST X RAY MEDIASTINAL MASS LN BIOPSYLYMPHOBLASTIC LYMPHOMA BONE MARROW STUDY INFILTRATION WITH ABNORMAL CELLS RESEMBLING LYMPHOBLASTS 65% CONSISTENT WITH LYMPHOBLASTIC LYMPHOMA WITH MARROW INFILTRATION
INTP - PPO, PHO, IAP. P7 5/22

TREATMENT
TREATED AS PER PROTOCOL FOR ACUTE LYMPHOBLASTIC LEUKEMIA REMISSION ACHIEVED CHILD STARTED ON MAINTENANCE CHEMOTHERAPY

INTP - PPO, PHO, IAP. P7 6/22

3 MONTHS LATER PRESENTED WITH:

GEN. LYMPHADENOPATHY SEVERE BACK PAIN
- NOT RELIEVED IN SUPINE POSITION - AGGRAVATED BY COUGHING - RADIATION OF PAIN TO ANTERIOR THIGH - NUMBNESS OF LOWER LIMBS - NO H/O BOWEL/BLADDER INVOLVEMENT

2 WEEKS -- 1 WEEK

EXAMINATION FINDINGS? WHAT WOULD YOU LOOK FOR?

INTP - PPO, PHO, IAP. P7 7/22

CLINICAL EXAMINATION
NORMAL SENSORIUM NO CRANIAL NERVE PALSIES UNSTEADY GAIT TAKES FEW STEPS WITH SUPPORT TENDERNESS TO PERCUSSION OVER 1ST AND 2nd LUMBAR VERTEBRAE EXAGGERATED DTRS PLANTAR REFLEX B/L UPGOING LOSS OF CREMASTERIC REFLEX

POSSIBLE DIAGNOSIS?
INTP - PPO, PHO, IAP. P7 8/22

CLINICAL POSSIBILITIES
 RELAPSE OF LYMPHOBLASTIC LYMPHOMA WITH SPINAL CORD METASTASIS RESULTING IN SPINAL CORD COMPRESSION  STEROID TOXICITY OSTEOPOROSIS AND VERTEBRAL COLLAPSE WITH SPINAL CORD COMPRESSION

INTP - PPO, PHO, IAP. P7 9/22

PLAN OF MANAGEMENT
EMERGENCY ADMINISTRATION OF STEROIDS
 FIRST AND MOST IMPORTANT STEP  PRECEDES INVESTIGATIONS  ORAL/IV DEXAMETHASONE IN PROPER DOSES

INVESTIGATE TO ESTABLISH DIAGNOSIS AND ASSESS SEVERITY AND EXTENT OF LESION TISSUE DIAGNOSIS OF RELAPSE INVOLVE NEUROSURGEON, RADIOTHERAPIST AND RADIOLOGIST IN MANAGEMENT CLOSELY MONITOR FOR PROGRESSION OF NEUROLOGICAL SIGNS AND SYMPTOMS

INTP - PPO, PHO, IAP. P7 10/22

INITIAL TREATMENT USED IN THIS CHILD

CHILD GIVEN IV DEXAMETHASONE, LOADING DOSE FOLLOWED BY 6TH HRLY MAINTENANCE DOSES AND FOLLOWED UP CLOSELY

AFTER STEROID ADMINISTRATION PLAIN XRAY AND MRI SCAN OF SPINE WAS DONE

LIKELY MRI FINDINGS?

INTP - PPO, PHO, IAP. P7 11/22

X-RAY THORACO LUMBAR SPINE SHOWING NO EVIDENCE OF OSTEOPOROSIS / COLLAPSE

INTP - PPO, PHO, IAP. P7 12/22

SAGITTAL SECTION MRI SHOWING

HYPERINTENSE PARAVERTEBRAL MASS (LARGE ARROW) (L1 LEVEL)

NOTE DURAL DISPLACEMENT (SMALL ARROW) CAUSED BY EPIDURAL MASS EFFECT

INTP - PPO, PHO, IAP. P7 13/22

PRE (L) AND POST (R) CONTRAST SCAN SHOWS VERTEBRAL BODY ENHANCES STRONGLY. NOTE CORD COMPRESSION.

INTP - PPO, PHO, IAP. P7 14/22

AXIAL T-1 WEIGHTED MRI SCAN AT L-1 LEVEL SHOWS EPIDURAL NHL (LARGE ARROWS) WITH THECAL SAC COMPRESSION (SMALL ARROWS)

INTP - PPO, PHO, IAP. P7 15/22

 BY 48 HOURS
 BACK PAIN REDUCED  SHOOTING PAINS REDUCED  MARKED IMPROVEMENT IN MUSCLE POWER. CHILD ABLE TO WALK WITH MINIMAL SUPPORT  DTRS BRISK AND UPGOING PLANTAR

CLINICAL FINDINGS SUGGEST IMPROVEMENT IN NEUROLOGICAL STATUS

INTP - PPO, PHO, IAP. P7 16/22

 CT GUIDED BIOPSY FROM THE MASS REVEALED LYMPHOBLASTIC LYMPHOMA DIAGNOSIS OF SPINAL CORD COMPRESSION DUE TO LYMPHOBLASTIC LYMPHOMA (RELAPSE) CONFIRMED (MRI SCAN + BIOPSY)
NEXT STEP IN THERAPY?
INTP - PPO, PHO, IAP. P7 17/22

 CHEMOTHERAPY? RADIOTHERAPY? SURGERY?

WHAT MODALITY OF TREATMENT BEST FOR THIS CHILD?

INTP - PPO, PHO, IAP. P7 18/22

 CHEMOTHERAPY FOR RELAPSED LYMPHOBLASTIC LYMPHOMA WITH 2ND LINE AGENTS STARTED SINCE:
 CHILD IMPROVED MARKEDLY WITH STEROIDS AND NO NEUROLOGICAL PROGRESSION PRESENT  LYMPHOBLASTIC LYMPHOMAS ARE CHEMOSENSITIVE TUMOURS

RADIO THERAPY USUALLY NOT FIRST LINE THERAPY MAY BE USED IF THERE IS NEUROLOGICAL PROGRESSION OR RADIO SENSITIVE TUMOUR RADIOTHERAPY CAN CAUSE IATROGENIC SPINAL DEFORMITIES AND SECOND CANCERS HENCE AVOID IF POSSIBLE
INTP - PPO, PHO, IAP. P7 19/22

CHILDS PROGRESS
RESPONDED WELL TO CHEMOTHERAPY

NEUROLOGICALLY STABLE BY 2 WEEKS

AT 4 WEEKS - NO SIGNIFICANT NEUROLOGICAL DEFICIT EXCEPT FOR MILD WEAKNESS OF LOWER LIMBS
INTP - PPO, PHO, IAP. P7 20/22

KEY POINTS
SPINAL CORD COMPRESSION A TRUE MEDICAL EMERGENCY SUSPECT IN ANY CHILD WITH KNOWN MALIGNANCY AND A PERSISTENT, SEVERE AND PROGRESSIVE BACK PAIN MAY BE A PRESENTING SIGN OF A MALIGNANCY EARLY ADMINISTRATION OF STEROIDS WILL OPTIMISE OUTCOME AND REDUCE MORBIDITY AND MORTALITY MRI PREFERRED FOR DIAGNOSIS MULTI DISCIPLINARY APPROACH

INTP - PPO, PHO, IAP. P7 21/22

KEY POINTS
DEFINITIVE THERAPY MAY INVOLVE CHEMOTHERAPY AND/OR RADIOTHERAPY OR SURGERY

CHEMOTHERAPY PREFERRED OVER RADIOTHERAPY CHILDHOOD TUMOURS ARE USUALLY CHEMOSENSITIVE

PROGNOSIS DIRECTLY RELATED TO EXTENT OF INVOLVEMENT AND TIME ELAPSED BETWEEN ONSET OF SYMPTOMS AND INTERVENTION

INTP - PPO, PHO, IAP. P7 22/22