1-KYPHOSCOLIOSIS case examination preforms and approach Final (1).pptx

Kyphoscoliosis approach

1. HISTORY Ramesh 20 yrs male Resident of Delhi Vendor by occupation c/o progressive deformity of spine since 4 years l

2. HISTORY OF PRESENT ILLNESS Progressive deformity of spine X 4 years Insidious in onset, gradually progressive, not associated with pain, no aggravating and relieving factors Inability to lie supine since last 2 years No difficulty in sitting or standing Sleeps in lateral position

3. H/o breathing difficulty on exertion (climbing one flight of stairs), over last 6 months, insidious onset, progressive, relieved on taking rest, not associated with chest pain, palpitations and not aggravated on lying down. No H/o any trauma, previous surgery, radiation therapy No history s/o muscle weakness , neurological deficit, mental retardation No history of pain in back No h/o swelling in the feet, pain in abdomen No h/o difficulty swallowing/regurgitation

4. PERSONAL HISTORY Non vegetarian Non smoker, non alcoholic Normal bowel habits Sleep pattern normal PAST HISTORY NO H/o previous surgery or anesthesia exposure No H/o DM / HTN / TB / asthma No H/o blood transfusion

5. FAMILY HISTORY No history of similar disease in the family TREATMENT HISTORY No treatment received till now

6. GENERAL EXAMINATION Conscious, oriented to time, place and person. Average built Pallor(-), icterus (-), edema (-) , lymphadenopathy (-) , clubbing(-) Weight - 52 kg, height -146cm, BMI: 24.39 Pulse – 102/min, regular, good volume, no radio-radial/radio-femoral delay BP – 126/70 mmHg RUL / sitting ( unable to lie supine) RR – 16/min regular, no use of accessory muscles SpO2 – 94% on room air

7. GENERAL APPEARANCE Right shoulder level higher than the left Increased space between body and left sided elbow while standing in neutral position Left hip level slight higher than the right Prominence of chest ribs on the right side Leg length was equal on both sides Gait appears to be normal No visible pigmentation seen

8. SPINE EXAMINATION INSPECTION Visible kypho scoliotic deformity in thoraco-lumbar region Convexity to right side PALPATION Thoraco-lumbar kyphoscoliosis confirmed Inability to palpate intervertebral spaces in thoraco-lumbar region

9. RESPIRATORY EXAMINATION Inspection Bony deformity of thorax Trachea midline B/L chest expansion unequal (L>R) Respiratory rate-16/min regular, thoraco-abdominal, no accessory muscles use, nasal flaring or subcostal retractions Crowding of ribs on left side Palpation Confirmed inspectory findings Percussion Resonant on all lung fields Auscultation Reduced air entry on right side , normal vesicular breath sounds , no added sounds

10. CARDIOVASCULAR SYSTEM Inspection Chest shape – asymmetrical (R>L) Precordium normal in shape Apical impulse not appreciated No engorgement of superficial veins Carotid pulsations visible Palpation Apex beat present in 5 th intercostal space in mid clavicular line No parasternal heave No appreciable thrill or pulsation in any area Auscultation S1 S2 audible, no murmur Loud P2

11. ABDOMINAL EXAMINATION Inspection Scaphoid & uniformly moving with respiration Umbilicus inverted, midway between xiphisternum & pubis No visible scar, pigmentation, ulcer, venous prominence, swelling, pulsation or movement Genitals- Normal Palpation Normal temperature and soft on touch No muscle guarding, lump, pulsation, fluid thrill, rebound tenderness or organomegaly Scrotum & testicles- normal Percussion Normal tympanic note, no shifting dullness. no organomegaly Auscultation Normal intestinal peristaltic sounds audible; 5 per minute

12. CENTRAL NERVOUS SYSTEM Higher functions are intact Cranial nerves are all normally functioning Built, tone, power, co-ordination of all the motor units are normal All sensory functions and reflexes are intact Cerebellar and autonomic functions are normal Gait is normal No trophic changes and no tender peripheral nerves 12

13. AIRWAY EXAMINATION Bearded Upper incisor length- 1cm; No buck teeth; Inter-incisor gap - 4.5 cm Palate - Not arched or narrow Modified Mallampati class- 2 Upper lip bite test- Class I Thyromental distance - 7 cm Submandibular compliance- soft on palpation Neck circumference - 28 cm, Neck ROM > 90⁰ 13

14. PROVISIONAL DIAGNOSIS 20 year old male with right sided adolescent onset idiopathic thoraco-lumbar kyphoscoliosis

15. What is kyphoscoliosis?

16. How is this deformity classified - according to region involved, according to etiology?

17. Classification according to region involved Cervico thoracic Thoracic Thoracolumbar Lumbar Combined double primary

18. Etiological Classification of Scoliosis STRUCTURAL Structural defect in vertebral column. Does not correct on bending or lying supine. Assoc. with vertebral rotation & rib cage deformity. Significant decrease in Total Lung Volume. Surgery required. NONSTRUCTURAL No structural defect of spine. Corrects on lying supine. No assoc. deformity. No surgery required. RX underlying cause.

19. Structural Scoliosis 1 . Idiopathic (genetic) scoliosis Infantile (Early onset < 4 years) Juvenile (Early onset – age 4-9 years) Adolescent (Late onset – age 10 or over)   2. Congenital scoliosis Abnormality of development of vertebrae – - hemivertebrae - congenitally fused ribs

20. 3. Neuromuscular scoliosis   Neuropathic forms Lower motor neuron disease (e.g., poliomyelitis) Upper motor neuron disease (e.g., cerebral palsy) Others (e.g., syringomyelia)   Myopathic forms Progressive (e.g., muscular dystrophy) Static (e.g., amyotonia congenita) Others (e.g., Friedrich’s ataxia, unilateral amalia )   4. Neurofibromatosis (Von Recklinghausen’s disease)  

21. 5. Mesenchymal disorders Congenital (e.g., Marfan’s syndrome, Morquio’s disease, amyoplasia congenita, various types of dwarfism) Acquired (e.g., rheumatoid arthritis,Still’s disease) Others (e.g., Scheurmann’s disease, osteogenesis imperfecta)   6. Trauma Vertebral (e.g., fracture, irradiation, surgery) Extravertebral (e.g., burns, thoracic surgery )

22. How do you measure severity of kyphoscoliosis?  

23. What is the correlation of clinical manifestations with severity of kyphoscoliosis?

24. Correlation of clinical manifestations with severity of kyphoscoliosis < 10 ° : normal curvature > 25 ° : ECHO evidence of increased pulmonary artery pressure > 40 ° : surgical intervention required > 65 ° : significant restrictive lung disease >100 ° : dyspnoea on exertion >120 ° : alveolar hypoventilation, chronic respiratory failure

25. Name some conditions associated with kyphoscoliosis

26. Conditions associated with kyphoscoliosis Cerebral palsy Cerebral muscular dystrophy Spina bifida Duchenne’s muscular dystrophy Familial dysautonomia Friedreich’s ataxia Skeletal dysplasia Marfan’s syndrome Neurofibromatosis Connective tissue disorders Craniospinal axis disorders : syringomyelia

27. What are the changes in other organ systems?

28. Changes in Respiratory System Cobb’s Angle > 65 o  respiratory function compromised Restrictive lung disease (decreased vital capacity, functional residual capacity, and total lung volume) – maximum reduction in vital capacity Ventilation-perfusion maldistribution Work of breathing and alveolar hypoventilation increases hypoxemia hypercapnia secondary electrolyte aberrations pulmonary hypertension and respiratory failure

29. Changes in cardiovascular system Right ventricular hypertrophy Pulmonary hypertension

30. Neuromuscular system Duchenne’s muscular dystrophy [DMD] Cerebral palsy may involve the bulbar muscles  postoperative aspiration DMD patients - sensitive to nondepolarizing agents Succinylcholine  Hyperkalaemia

31. This patient is posted for Posterior correction & instrumentation. How will you investigate and prepare this patient for surgery?

32. Patient’s Investigations Hemoglobin: 13.5 gm % TLC: 5300/cu.mm (N65 L32 M1 E2) Platelets: 2.6 lacs/cu.mm Urea: 23 mg/dl; Creatinine: 0.5 mg/dl; Na + : 135 meq /l; K + : 3.7 meq /l; Blood sugar - 105 mg/dl (R) Total bilirubin: 0.5 mg/dl Total protein: 6.90 gm/dl; Albumin: 3.5 gm/dl SGOT/SGPT: 15/17 IU/L, ALP: 42 IU/L Urine (routine & microscopy)- Within normal limit

33. Patient’s Investigations … ECG : RVH ABG : pO2 - 82 mmHg, pCO2 - 36 mmHg rest WNL ECHO : EF 50% , suggestive of PAH (grade2) PFT : FEV1 76%, FEV1/FVC WNL IMAGING CXR : crowding of ribs on left, dorsolumbar scoliosis with convexity to right side, lung fields clear, cobb’s angle 60 o MRI : kyphoscoliotic deformity of dorsolumbar spine , rest normal

34. What will be the anaesthetic concerns in this case?

35. Anaesthetic concerns Pre-operative Concerns Resp involvement CVS involvement Assessment and documentation of neurologic status Airway Congenital anomalies

36. Anaesthetic concerns… Intraoperative Concerns Spinal Cord monitoring (EP/wake up) Blood loss – assessment & replacement Position related problems (prone) Hypothermia Venous air embolism  

37. Anaesthetic concerns…   Postoperative Concerns Pain Pulmonary function Postop mech ventilation Bleeding & coagulation abnormalities Hyponatremia – due to inappropriate ADH secretion

38. How would you anaesthetize and monitor this patient?  

39. What are various causes of intraoperative neurological injury? How is spinal cord function monitored intraoperatively?  

40. Which are the physiologic parameters that alter SSEP & MEP?

41. Physiological factors affecting SSEP Physiological Factors Amplitude latency Hypotension (below cerebral autoregulation) Decreases No change Hypothermia Decreases Increases Hyperthermia Decreases Hypoxia Decreases Isovolemic haemodilution Variable Increases

42. Which are the anaesthetic agents that alter SSEP & MEP?

43. Anaesthetic agents affecting SSEP & MEP Agent Amplitude Latency Halothane ↓ ↑ Desflurane ↓ ↑ Isoflurane ↓ ↑ Sevoflurane ↓ ↑ Nitrous oxide ↓ ↔ Barbiturates ↓ ↑ Etomidate ↑ ↔ Ketamine ↑ ↔ Midazolam ↓ ↔ Opioids ↔ ↔ Propofol ↔ ↔

44. What should be done if neurophysiologic signals change?  

45. What are the problems associated with prone position and what precautions should be taken to avoid these?      

46. How would you estimate allowable blood loss? What are various methods to manage blood loss?

47. What is massive transfusion? What could be the anticipated problems of massive transfusion?

48. What are various predictors for postoperative mechanical ventilation?

49. How would you provide postoperative pain relief?

50. What are the other situations where you could come across a kyphoscoliotic patient?

51. A patient with severe kyphoscoliosis has presented for caesarean section. Discuss the options for anaesthetic management.

52. Algorithm to guide neuraxial techniques in scoliotic patients Bowens et al. An approach to neuraxial anaesthesia in severely scoliotic spine. BJA 2013; 111:807-11.