Case of Spinal TumorComplicating Pregnancy

Presentor : Dr. Deepmala

Designation : Fellow in HRP and Perinatology

Hospital : Fernandez Hospital, Hyderabad

Date of Presentation : 12.04.2011

Contents of this presentation

Approach to Paraplegia / Backache

Case Details

Discussion:

– Spinal Tumor

– Subacute combined degeneration of cord

– Muscular dystrophy

Approach To Paraplegia

Definition: Paralysis of lower part of the body,

commonly affecting both legs and often

internal organs below waist

Level of lesion

Upper Motor Neuron Lesion

Lower Motor Neuron Lesion

Spinal Cord

Spinal Reflex Arc

UMN -LMN

UMN Lesions Causing ParaplegiaSPINAL LESIONS (common)

SPINAL CORD COMPRESSION

– Pott’s disease

– Disc prolapse

– Fracture

– Tumors

– Epidural abscess,

– Cervical spondylosis

VASCULAR

– Hemorrhage

– Infarction

SPINAL LESIONS (common)

SYSTEMIC DEGENERATION OF TRACTS

– Multiple sclerosis

– Sub acute combined degeneration of cord

INFECTION

– Transverse myelitis

– Neurosyphilis

CEREBRAL LESIONS (uncommon)

– Thrombosis of superior sagital sinus

– Tumor of falx-cerebri

– Hydrocephalus

UMN Lesions Causing Paraplegia

LMN Lesions Causing Paraplegia

Anterior horn cells : Poliomyelitis, Motor

neuron disease

Peripheral nerve : Peripheral neuropathy

Neuromuscular junction : Myasthenia gravis

Muscles : Muscular dystrophies

UMN Vs LMN Lesion

UMN-Spinal Cord Lesions

Intramedullary

Extramedullary, intradural

How to Differentiate Clinically?

Extramedullary Intramedullary

Root pain common rare

UMN signs early late

Sensory deficit

Contralateral loss of pain/temp;

ipsilateral loss of proprioception

Dissociated sensory loss

Approach to Backache - (Causes)

Degenerative : Musculoligamentous, facet joint,

osteoporosis

Trauma

Tumour : Intra / extradural, pathological fracture

Infection : Pyogenic, TB, discitis, osteomyelitis

Inflammatory

Vascular : Aortic aneurysm

Diagnostic triage Includes

– History

– Examination

– Investigation

Aids differentiation between :

Simple backache

Nerve root pain

Serious pathology

Tumour/Infection/Central disc prolapse

Simple Backache

Presentation : 20 – 55 yr age

Lumbosacral region : Buttocks and thighs

Mechanical pain : Varies with physical activity

and time

Patient well

Prognosis : 90 % recover

Nerve Root (Radicular) Pain

Unilateral leg pain worse than LBP

Radiation to foot or toes

Radicular Sx or signs : Straight leg raising / Sensory

disturbance / Motor weakness / Reflex changes

Prognosis : 50% carries good prognosis

Warning Signs of Serious Pathology

Age of onset : < 20 and > 55 years

Constant unremitting progressive pain

Thoracic radicular pain

Systemically unwell, weight loss

Drug abuse, HIV

Structural deformity

Significant trauma

Backache - Inflammatory Disorders

Gradual onset before 40 years

Marked morning stiffness

Persisting limitation of spinal motion

Peripheral arthritis

Iritis, psoriasis, colitis, urethritis

Family history

Cauda Equina Syndrome : Central Disc Prolapse

Bilateral leg pain

Saddle anaesthesia

Bilateral foot

weakness

Bil. loss of ankle reflex

Loss of anal tone

Urinary incontinence

Case Details

Mrs X, 25 yrs

Married since FEB 2012

Nonconsanguinous marriage

G2A1,39 Wks; Referred in view of paraparesis (6th Nov)

Obstetric history:

G1- Missed miscarriage @ 8 wks,2012 spontaneous

expulsion

G2- Present pregnancy, Spontaneous conception

Case Details

First Trimester – Folic acid

Second Trimester – Iron, calcium supplements;

Fetal anomaly scan (TIFFA) – Normal

Third Trimester – Fetal wellbeing scan Normal

– Two doses Tetanus toxoid √, Hematinics√

Symptoms

Low backache since 1 year; gradual onset ; slowly

worsening

Difficulty in walking- fourth month of pregnancy

Difficulty in sitting ,getting up & turning in bed

Gradual onset, slowly progressing

Symptoms

Initially walking with support of wall / staircase;

Since 15 days restricted activity, almost

bedridden,(only going to washroom with walker

and support of an attendant)

No h/s of upperlimb weakness, cranial nerve

palsies or bowel-bladder involvement

Case Details

Past history: Nil

Family history:

Parents HTN and DM

Muscular Dystrophy-Father and Paternal

cousins

Two of her brother had tuberculosis

Clinical Findings

Obese lady BMI=39, comfortable in supine

Pedal oedema++, U/A-Trace

PR-90/min,BP-120/70 mm Hg

RS- Air entry bilaterally equal, CVS-S1,S2+

P/A –Uterus Term, relaxed,

– Presentation: Cephalic 4/5, Fetal Heart Sound+

Motor Examination of lower limbs

Exam RT LL LT LL

Bulk N N

Tone (Proximal/Distal) N/Flaccid N/Flaccid

Power 1/5 3/5

Knee jerk Brisk Brisk

Ankle jerk ++ ++

Plantar jerk Upgoing Upgoing

Other Neurological Findings

Patchy sensory loss in lower limbs- touch,pain,

position sense;?level

Motor and sensory: Both Upper limbs normal

Higher functions N, All Cranial Nerve N

No Cerebellar signs/meningeal signs

Spine normal

Differential Diagnosis

Myeloradiculopathy

Subacute combined degeneration of

spinal cord

Muscular dystrophy

Initial Investigations

Hb- 10 gm/dl

Blood group: O Positive

TSH- 1.26

HIV & HBsAg- Nonreactive

OGTT- 79/123/98 @ 20 wks

TIFFA,Fetal wellbeing scan- Normal

Lab Investigations

Lab Parameters 31/10 6/11

Hb / WBC 13.5/9800 13.5/11,100

PLT 2.14 1.63

LFT /RFT N N

CUE /U-C N N

Vit B12 - 201

CPK - 174

LDH - 353

ESR - 84

Multidisciplinary Care

Obstetrician, Physician, Anaesthetist, Neurophysician and

Neurosurgeon

MRI DORSAL SPINE with Screening of Cervical Spine

Well defined, regular, Space occupying lesion (2.2

x1.01 cm ) iso-intense(Gray matter ) all sequences

Intradural, extramedullary between D2/D3

Subtle cord edema

Possibly Schwannoma / Meningioma

MRI Images

Delivery Details

Elective LSCS with Thoracic( T1-3)

Laminectomy and Excision of intradural

Meningioma under GA on 9/11/13

Girl , 3.5 kg ,(8/9/9)

HPE- Meningioma

(Meningiotheliomatous/Transitional type)

Post Op Period

Inj Ceftriaxone, Amikacin, LMWH X 3 Days

Regular-Physiotherapy

Suture removal on 10th pod –Wound Healthy

Power in lower limbs improved by 20%

Discharged on 10th Day, Hb- 11.7

Summary

25 yr old primi presented with gradual onset, slowly

worsening symptoms of LBA and paraparesis with

patchy sensory involvement, few UMN signs, no

bowel/bladder or cranial nerve involvement; MRI

concluded extramedullary, intradural tumor,

resected at LSCS, HPE confirmed meningioma; post

op gradual recovery in progress

Spinal Cord Tumors

Extradural

Secondary carcinomaPrimary sarcomaReticulosisChordomaNeurinomaMeningioma

Extramedullary NeurinomaMeningiomaEpidermoid

Intramedullary EpendymomaAstrocytomaAngioma

Intradural

Meninges of the Spinal Cord

Meninges of the spinal cord

Spinal Meningioma

Peak age: 40- 70YR

Female:Male= 4: 1

Intradural- 90%

Extradural- 5%

Both- 5%

Less Frequent compared to intracranial(7-12% of all Meningioma)

Multiple meningioma-Rare

Thoracic 82 %

Cervical 15 %

Lumbar 3 %

Lateral 68%

Posterior 18%

Anterior 15%

Genetic Component

Complete / Partial Loss of Chr 22

Abnormality - Cancer related

gene1p,9q,10q,17q

? Dependence on Sex Hormone

M/C Symptoms

@compression

/onset@surgery

Local /Radicular Pain 42 % 53%

Motor deficit 33 % 92%

Sensory deficit 25 % 61%

Sphincter disturbances 50%

An intradural enhancing meningioma , the ventrally located tumor has produced cord compression and with displacement of the thoracic cord to the right side. Calcified nature of the tumor is identified on sagittal

T2 wtd. image as an area of dark signal intensity (yellow arrow in B) and confirmed by CT imaging as an area of high attenuation density.

Treatment

Rx: resection by safest route

Total resection including

dural attachment and/or

cauterization of the

adjacent dura

Subtotal resection has much

higher risk of recurrence

Deficits often dramatically

reversible

Meningioma

Nest of tumor cells

Abundant cytoplasm

Bland oval nuclei

Occasional

Psammoma bodies

Subacute Combined Degeneration of Spinal Cord

Causes of B12 deficiency

– GI malabsorption

• pernicious anemia (lack of intrinsic factor)

• post-gastrectomy

• Crohn disease

• Celiac disease

– Nutritional – Vegetarian

SACD : Pathophysiology

B12 Deficiency

– Accumulated methylmalonic acid cause

myelin toxicity

– Upregulation of tumor necrosis factor and

downregulation of epidermal growth factor

Nitrous oxide

Susceptible patients, B12 is inactivated

SACD: Pathology

Loss of myelin in the spinal cord white matter

tracts:

– Dorsal columns ( vibration,jt position,fine

touch)

– Lateral columns( pain,temperature)

– Ventral columns(pressure,crude touch)

SACD: Clinical Findings

Insidious subacute onset

– parasthesias, sensory ataxia, hyperreflexia,

and + Babinski

Severe disability in weeks to months

Symmetric;progresses distal to proximal

INV: Megaloblastic anemia; low B12

SACD : Imaging Findings

MRI: high T2 signal intensity involving the

cervical and/or thoracic spinal cord

– Dorsal, lateral and ventral white matter

Cord diameter-normal

No Gd enhancement

SACD: Imaging Findings

SACD: Treatment

B12 supplementation

Cannot recover lost axons

Avoid nitrous oxide anesthesia

Muscular Dystrophy

Group of genetic disorders

Characterized by progressive loss of muscle

integrity; wasting; weakness

Degeneration and regeneration of muscle fibers

(in contrast with static or structural

myopathies)

Muscular Dystrophy- Association

Covers all muscular dystrophies and

myopathies

Multisystem diseases : ALS or Friedreich’s

Ataxia

Dystrophinopathy

Disorders involving dystrophin

Dystrophin: large gene on the X-chromosome

– ubiquitous in the human body

Two most common and severe dystrophy

Duchenne MD and Becker MD

Duchenne’s Muscular Dystrophy

X-linked recessive disorder

Deficiency of protein dystrophin in muscles

Symptoms start in childhood

Become severe in adolescence

Death occurs by age 20 years

Becker Muscular Dystrophy

Same gene affected as Duchenne MD

Disorder of function or decreased amount of

dystrophin rather than absence of the protein

Slowly progressive form

Muscle biopsy, immunostaining for dystrophin:

patchy staining

Myotonic Muscular Dystrophy (Steinert’s disease)

Presentation: adult; multi-system involvt

Primarily distal and facial weakness

Facial features

– frontal balding in men, ptosis, low-set ears,

dysarthria, swan neck,

Myotonia: worse in cold weather, > 20 yr

Myotonic Muscular Dystrophy

Heart: conduction block : Evaluate syncope

Smooth muscle: Constipation, problems with childbirth,

BP lability

Brain: Learning disabilities

Ophthalmology: Cataracts

Endocrine: Insulin resistance, hypothyroidism, testicular

atrophy