LIMB GIRDLE DYSTROPHY AND CAESARIAN SECTION by Dr.Sravani Vishnubhatla

LIMB GIRDLE DYSTROPHY
AND CAESARIAN SECTION
SPEAKER: DR V.SRAVANI
MODERATOR: DR JAGADISH OMKAR

MUSCULAR DYSTROPHY
INTRODUCTION:
✓ Muscular dystrophies are a group of hereditary diseases
CHARACTERISTICS:
✓ Progressive symmetrical skeletal muscle weakness and
wasting
✓ No evidence of skeletal muscle denervation
✓ Sensation and reflexes are intact
Dr.Sravani Vishnubhatla

MUSCULAR DYSTROPHY
PATHOPHYSIOLOGY:
Breakdown of the dystrophin-glycoprotein complex
Painless degeneration of muscle fibers
Myonecrosis and fibrosis.

TYPES OF MUSCULAR DYSTROPHIES
✓ Duchenne muscular dystrophy
✓ Becker muscular dystrophy
✓ Limb-girdle muscular dystrophy
✓ Facioscapulohumeral muscular dystrophy
✓ Myotonic muscular dystrophy

LIMB-GIRDLE MUSCULAR DYSTROPHY
✓ Genetically inherited
✓ Autosomal Dominant (10%) or recessive(90%)
✓ Incidence:
▪ Rare <1:1,00,000
✓ Slowly progressive but relatively benign disorder

SUBTYPES

✓ Onset: second to the fifth decade.
✓ Symptoms: worsen with age
▪ Proximal muscle wasting and weakness
▪ Shoulder girdle or pelvic girdle muscles
▪ Within 20yrs walking becomes difficult
✓ Rate of progression: slow

✓ Complications:
▪ Cardiopulmonary complications occurring in later
stages of the disease
✓ Expected lifespan:
▪ Varies
▪ May survive upto middle age or late adulthood
▪ Death due to cardiac and pulmonary complications

✓ DIAGNOSIS:
▪ Careful medical history and a thorough physical
examination
▪ Family history
▪ Serum Creatinine phosphokinase levels
▪ EMG
▪ Muscle biopsies
▪ Gold standard DNA test :mutation of the dystrophin gene
(DNA blood test or SCAIP sequencing)

ANESTHETIC IMPLICATIONS OF MUSCULAR
DYSTROPHIES
✓ Orthopedic surgeries early in life
▪ Scoliosis correction
▪ Muscle biopsies
▪ Tendon release
▪ Tendon transfers.
✓ Dystrophies affecting the heart, the patients will require
anesthesia for implanting cardio defibrillators and pacemakers
✓ In advanced stages, tracheostomies and gastrostomies
✓ Therapeutic or diagnostic procedures

PRE ANESTHETIC EVALUATION
✓ Mainly difficult airway should be assessed
✓ Any pressure ulcers should be ruled out
✓ The forced vital capacity (FVC) and the forced expiratory volume in
both supine decubitus and seating position should be evaluated.
✓ FVC <50% of the expected level- high risk of requiring postop non-
invasive ventilation
✓ Occasionally, additional tests to assess the diaphragmatic function-
a dysfunctional diaphragmatic function requires non-invasive
mechanical ventilation support before surgery.

ANESTHETIC MANAGEMENT
✓ Particularly difficult because of the risk of complications
✓ Anesthetic risk depends on :
▪ The type of surgery
▪ The clinical condition of the individual patient,
▪ The progression of the disease

PREMEDICATION:
✓ Aspiration prophylaxis with H2 antagonists,
metoclopramide and sodium citrate
✓ For sedation and analgesia:
➢ Low-dose ketamine and midazolam
➢ Opioids such as remifentanil and fentanyl
INDUCTION:
✓ Propofol, thiopental or etomidate

❑ INHALATIONAL AGENTS:
✓ Volatile anesthetic agents are not used as these may
trigger myotony and crisis of rhabdomyolysis
✓ nitrous oxide and sevofluorane- used with caution
❑ MUSCLE RELAXANTS:
✓ DMR – succinylcholine avoided
✓ NDMR: Rocuronium, atracurium can be used
❑ REVERSAL AGENTS:
✓ Sugammadex can be used and neostigmine avoided

✓ High risk of apnea and death following extubation, over
the next 24 hours after surgery
✓ In case of respiratory depression following reversal of the
neuromuscular relaxation, consider deferring the
extubation 24 to 48hours, or consider the use of non-
invasive mechanical ventilation

✓ Regional caudal or epidural spinal anesthesia:
▪ Reliably and successfully used
▪ Challenging because of the spinal abnormalities
✓ Warming strategies with hot fluids and electric blankets
to prevent hypothermia
✓ Fluid therapy with potassium-free crystalloids

POSTOPERATIVE MANAGEMENT AND
PERIOPERATIVE COMPLICATIONS
✓ Good analgesia is a must
✓ More sensitive to the effects of opiates (systemic and
neuraxial)-higher risk of:
▪ Respiratory depression
▪ Exacerbated gastrointestinal paresis
▪ Increased risk of reflux, aspiration
▪ Ventilation dysfunction.

✓ Patients with dystrophies may be classified based on
the perioperative risk
✓ Intermediate risk - grade MIRS 3, and
✓ Very high risk - grades 4 and 5
MIRS
(MUSCULAR IMPAIRMENT RATING SCALE)

PERIOPERATIVE COMPLICATIONS
✓ Respiratory failure
✓ Rhabdomyolysis
✓ Arrhythmias
✓ Cardiac arrest
✓ Reactions similar to malignant hyperthermia
✓ Hyperkalemia

IN PREGNANCY
✓ There is progression of disease during pregnancy
✓ Obstetric complications occur if they develop severe
pelvic girdle weakness or respiratory insufficiency
✓ Multi-disciplinary approach essential

IN PREGNANCY
✓ PREOP ASSESSMENT:
▪ Detailed medical and family history
▪ Physical examination
▪ Systemic examination:
❑Mainly documentation of power of muscles both upper and
lower limbs
▪ Airway assessment

IN PREGNANCY
✓ INVESTIGATIONS:
▪ Complete blood picture
▪ Renal function tests
▪ Coagulation profile
▪ Thyroid profile
▪ Pulmonary function tests- advise incentive
spirometry and chest physiotherapy if restrictive
pattern present
▪ Echocardiography

INDICATIONS FOR CAESARIAN SECTION
✓ Abnormal blood gases
✓ Vital capacity below 1-1.5 L
✓ Presence of pulmonary hypertension
✓ Presence of right heart failure
✓ Weak diaphragm or abdominal muscles
✓ Presence of pelvic abnormalities

MANAGEMENT
✓ Explanation of procedure
✓ Written and informed consent
✓ NPO from midnight
✓ Aspiration prophylaxis
✓ Operative room kept ready including difficult
intubation cart and percutaneous tracheostomy set
✓ Iv line secured
✓ ASA standard monitors (NIBP, SPO2,ECG,temp)
✓ Capnography, ABG analysis, neuromuscular
monitoring if necessary

INTRAOP MANAGEMENT
✓ NEURAXIAL ANESTHESIA:
▪ Spinal or epidural anesthesia preferred to general
anesthesia unless contraindicated
▪ But it may affect respiratory function depending on
the extent of neuraxial blockade
▪ Less extensive motor blockade show minimal effects
on ventilatory function
▪ NPPV support can be given

INTRAOP MANAGEMENT
GENERAL ANESTHESIA:
✓ INDICATIONS:
▪ Pts unable to tolerate supine position despite
respiratory support
▪ Pts having bulbar muscle involvement
✓ INDUCTION: thiopentone
✓ MUSCLE RELAXANT: atracurium
✓ Suxamethonium and volatile anesthetics better
avoided
✓ Due to risk of life threatening complications like
rhabdomyolysis and malignant hyperthermia

POSTOPERATIVE CARE
✓ Thromboprophylaxis:
Enoxaparin 40mg/day
✓ Postop analgesia:
▪ Epidural infusion
0.0625% bupivacaine with fentanyl 2mcg/ml @6ml/hr
▪ Timing of epidural catheter removal:
After 36hr of surgery and 12 hr after previous dose of
enoxaparin
✓ Fluid therapy
✓ Urine output and other vitals monitoring
✓ Incentive spirometry, chest physiotherapy

THANK YOU!

LIMB GIRDLE DYSTROPHY AND CAESARIAN SECTION by Dr.Sravani Vishnubhatla

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LIMB GIRDLE DYSTROPHY AND CAESARIAN SECTION by Dr.Sravani Vishnubhatla