Deformities in leprosy Dr Manasa Shettisara Janney

References
IAL textbook of leprosy
Hastings handbook of leprosy

1. Deformities in Leprosy and management Dr manasa s j, 2 nd yr pg student, Dept of DVL, chafb

2. Introduction Leprosy feared for its unsightly disabilities and deformities If leprosy had not caused deformities & disabilities- it would not have been a dreaded disease Socioeconomic dehabilitation in leprosy  deformities Nerve damage  deformities

3. Basics Impairment - Any loss or abnormality of psychological, physiological or anatomical structure or function Ex: loss of sensation due to nerve involvement Deformity- V isible alteration in the form, shape or appearance of the body due to impairment produced by disease process Ex: claw hand, loss of eyebrows etc. Disability - Any restriction or lack of ability (resulting from impairment) to perform an activity considered normal for a human being Ex: difficulty in walking due to foot-drop

4. Basics Handicap- D isadvantage resulting from an impairment or disability that limits or prevents fulfillment of a role that is normal depending on patient’s age and sex as well as relevant social and cultural factors Examples: inability to earn a living on account of disability or needing help in performing activities of daily life

5. DEFORMITIES

6. Causation 1. Direct infiltration of tissues 2. Nerve damage 3. Secondary to anesthesia

7. Causation 1. Direct infiltration of tissues 2. Nerve damage 3. Secondary to anesthesia

8. Nerve damage Only bacillus known to infect peripheral nerve s Peripheral nerves consist of sensory, motor and autonomic nerve fibres Nerve involvement may be partial or total , i.e., only sensory impairment or both sensory and motor impairment , autonomic fibres are generally involved in both cases

9. Stages of nerve involvement ( i )Stage of parasitization : Transition phase between "disease" and "non-disease“ States; M. leprae found inside Schwann cells,yet no host tissue response (ii) Stage of tissue response: Persistence and multiplication of the bacilli eventually evokes a tissue response which is initially non-specific or indeterminate but,becoming specified (iii) Stage of clinical infection: Nerve gets thickened and becomes clinically regonizable as such

10. Stages of nerve involvement (iv) Stage of nerve damage: Neural functional deficit becomes clinically demonstrable .Nerve damage is reversible at this stage (v) Stage of nerve destruction: Conducting elements are totallydestroyed and collagenized . Caseation and cold abscess formation may occur in tuberculoid leprosy

11. Various deformities in leprosy

12. Factors associated with deformities

13. Factors associated with deformities

14. Types 1. Specific deformities Local infection or infiltration with M.leprae Most often seen in face (Ex: Loss of eyebrows, nasal deformity), less often in hands (Ex: banana fingers, reaction hand deformities) and occasionally in feet 2.Paralytic deformities Damage to motor nerves Seen most often in hands (Ex: Claw fingers), less often in feet (Ex: Claw toes, drop foot) and occasionally in face (Ex: Lagopthalmos, facial palsy)

15. Types 3.Anaesthetic deformities Neglected injuries in parts rendered insensitive because of damage to sensory nerves Found most often on feet and hand (ex. Ulceration, shortening of digits , mutilation and skeletal disorganization of foot)

16. Grading

17. Deformities of hands

18. Ulnar nerve(C8,T1) Medial cord Posterior to medial epicondyle Pierces two heads of  FCU Muscular branches,Palmar & dorsal cutaneous branches At wrist, superficial to flexor retinaculum Enters the hand via  Guyon’s canal Terminates into supfl ( palmaris br.)& deep branches ( hypothenar , interossei , medial 2 lumbricals , Add. pollicis , Flexor pollicis brevis -deep head)

19. Median nerve(C5-T1) Medial and lateral cords Crosses brachial artery(lateral to medial) Enters anterior comptmnt of forearm  Muscular branches Travels between FDP and FDS In forearm, AIN , Palmar cutaneous nerve Enters hand via carpal tunnel Terminates into Recurrent branch ( thenar muscles) & Palmar digital branch (cut. & lateral 2 lumbricals )

20. Radial nerve(C5-T1) Posterior cord Posterior to   axillary artery Muscular branches-  triceps brachii Radial groove Anteriorly over lateral epicondyle  of humerus Terminates into deep branch  (posterior comptmnt of forearm) & superficial branch  (sensory)

21. Sensory supply

22. Motor paralytic deformities Common Destruction of motor fibres in the major nerve trunk MC nerve- Ulnar nerve Less commonly,combined paralysis of ulnar and median nerves Median nerve- affected at the level of forearm

23. Ulnar nerve paralysis Partial or ulnar claw hand High ulnar palsy : muscles of the forearm(FCU, FDP) Low ulnar palsy: small muscles of hand Extensors pull MP joints in extension bringing about compensatory flexion at PIP joints Only ulnar nerve  ulnar claw Ulnar and partial median nerve  subtotal claw Ulnar and median nerves  total claw

24. CLAW HAND

25. Ulnar nerve paralysis Deformities: Clawing: hyperextension –MCP; flexion-IPJ. Flattening of hypothenar eminence Depression over dorsum of thumb (wasting of dorsal interosseous muscle) Disability: Difficulty in typing, eating rice with hand & coin slips off the finger Fine work with delicate manipulation –difficult Weakening of power grip (paralysis of adductor pollicis )

26. Median nerve paralysis Simian hand Thumb does not lift off the palm to oppose other digits Paralysis of abductor pollicis brevis and opponens pollicis

27. Triple palsy Damage above the level of elbow Paralysis of all muscles in back of forearm Claw hand is abolished, because of paralysis of digital extensors Wrist drop - When pt is asked to keep wrist & fingers straight ,with forearm stretched & pronated –difficult to actively lift wrist 9/30/2016 28

28. WRIST DROP

29. Non specific deformities Banana finger - H eavy infiltration of skin followed by atrophy & deposition of fat Shortening of fingers - F ragmentation and resorption of terminal phalanx Reaction hand - Foci of acute inflammation occur and eventually resolve with dense fibrosis & crippling effects Twisted finger – Bizarre deformity in reactions, osteoporosis & pathological #

30. Deformities of feet

31. Sciatic nerve(L4-S3) Lumbosacral plexus Enters gluteal region via greater sciatic foramen It emerges inferior to piriformis Enters posterior thigh by passing deep to long head of  biceps femoris In thigh, muscular branches (hamstrings & adductor magnus ) At apex of popliteal fossa , bifurcates into tibial and common fibular nerves

32. Tibial nerve(L4-S3) Popliteal fossa Muscular branches Branches contributing towards   sural nerve Passes posteriorly and inferiorly to themedial malleolus , through  tarsal tunnel Cutaneous innervation to the heel Terminates into medial & lateral plantar nerves

33. Common peroneal nerve(L4-S3) Cutaneous branches Wraps around the neck of fibula Terminates into superficial fibular and deep fibular nerves

34. Sensory supply

35. Motor paralytic deformities 1.Claw toes-paralysis of intrinsic muscles , supplied by medial plantar nerve - ulceration of tip of toes , under the metatarsal head 2.Foot drop- Paralysis of common peroneal nerve at neck of fibula - Paralysis of dorsiflexor and evertors of foot - C/F high stepping gait - Ulcers over toe tips 9/30/2016 36

36. FOOT DROP

37. Anaesthetic deformities Neuropathic disorganisation of foot Disruption of the skeletal structure caused by neurological deficit Disruption of Forefoot : common ,neither progressive nor any complications Mid foot or Hind foot : progressive disorganisation leading to serious disability 9/30/2016 38

38. Causes: septic disorganisation : aseptic /traumatic disorganisation Prognosis poor when degenerative disorganisation coupled with extensive infection Amputation- best treatment 9/30/2016 39

39. Neuropathic plantar ulcer Insensitive sole injured from outside Dry anaesthetic skin develops fissure & cracks Stress & strain on forefoot Loss of arches of foot

40. Distribution of plantar ulcer Forefoot-79%, midfoot-7%, hind foot 14% 9/30/2016 41

41. Stages 1. Threatened Ulcer 2. Concealed ulcer- Necrosis blister 3. Overt ulcer

42. Deformities of face Non paralytic & paralytic

43. Madarosis -super ciliary & ciliary 9/30/2016 44

44. Corrugations-deepening of skin markings Leonine facies (sagging face) Premature senile appearance 9/30/2016 45

45. External ear infiltration megalobule (Buddha ear) 9/30/2016 46

46. Rat bitten appearance of ear : irregular & scalloped due to loss of skin & bits of cartilage 9/30/2016 47

47. Infiltration of the nasal structure- sunken nose deformity Negligence of nasal hygiene- myiasis Septal perforation 9/30/2016 48

48. Eyes Eyelids-infiltrated & thickened, nodular Conjuctivitis , scleritis , episcleritis , superficial punctate keratitis Iris pearls : deposits of tightly packed bacilli within the swollen macrophages (slit lamp) Secondary cataract , Glaucoma , Ciliary staphyloma Mouth Hard palate perforation Upper incisor teeth missing-part of skull changes ( Facies Leprosa – named by Moller Christensen ) 9/30/2016 49

49. Paralytic deformities Upper facial palsy - facial nerve (zygomatic branch) - Lagophthalmos -upper eyelid - Ectropion -lower eye lid -Exposure keratitis Lower facial palsy ( buccal & mandibular branch): -drooping of angle of mouth to affected side -obliteration of nasolabial fold -dribbling of saliva -unable to purse lips/whistle Trigeminal nerve - sensory loss 9/30/2016 50

50. Lagophthalmos with ectropion

51. Other deformities Larynx V ocal cords : fibrotic form ,ulcerative form leading to hoarseness glottis narrowed- stridor Genitalia testicular atrophy-altered sexual hair pattern/altered sexual functions Gynaecomastia

52. Management

53. Management Prevention of primary deformity Early detection of nerve damage & prompt Rx ( Rest, MDT, Steroids, Thalidomide, Splints) Surgical decompression of nerve indicated if Intractable pain , Nerve abscess, Entrapment of nerve Reablement - Splints, active and passive excercises , Reconstructive surgery Prevention of secondary deformity Skin care procedure Injury care procedure Joint care procedure Mx of ulcers 9/30/2016 54

54. Skin care Absence of sweating  dry, brittle, cracks Insensitive skin  ignorance  deep chronic infection Daily soaking of hands in water-15 min Rubbing palms vigorously to remove superficial keratin layer Smear with liquid paraffin, neem or castor oil 9/30/2016 55

55. Injury care Preventing or promptly attending to them if they do occur Injury consciousness & protective behaviour Protective covering of hand with thick towel/glove or using utensils with insulated handles Habit of inspecting limbs daily( cuts,blisters.hot spots) Cover with bulky bandage &rest for 24-72 hrs, consult medical advice if doesn’t subside 9/30/2016 56

56. Joint care Mobile, supple, free from contracture Daily oil massage Repeated passive stretching Serial splinting

57. Splints Circular splints Thumb web splints Gutter splint Functional splint Dynamic splint Walking plastic cast Namasivayan’s splint( Hand splint Galavanised iron wire inserted into rubber tubing for ‘Intrinsic muscle paralysis of the hand’)  

58. Adductor splint for abductor deformity of little finger 9/30/2016 59

59. Opponens splint for ape thumb deformity & first web space contracture 9/30/2016 60

60. Cock up splint

61. Finger loop splint

62. Knuckle bender splint

63. Immobilisation

64. Corrective surgeries Restoration of normal appearance Improvement in function of hand Preoperative preparation- Assessment of deformity, disability, integrity of extensor apparatus -Prevent & release contractures -Muscle training 9/30/2016 65

65. Claw hand Lasso insertion(independent flexor) Zancolli’s operation( Volar capsule shortening) augment flexion forces at MCP jt Srinivasan’s operation(extensor diversion) Bunnel’s operation(FDS) Brand operation(ECRL) augments extension forces at PIP jt Antia (PL)

66. Claw thumb: abductor- opponens replacement operation, Brand’s, Snow Fink Triple nerve palsy- multiple tendon transfer operation 9/30/2016 67

67. CLAW HAND CORRECTION BY TENDON TRANSFER 9/30/2016 68

68. Lower limb deformities Foot drop Early cases : spontaneous recovery-surgery deffered –1yr Established cases: tibialis posterior tendon transfer( circumtibial / interosseus route) Claw toe Flexor digitorum tendon transfer 9/30/2016 69

69. Facial deformities Lagophtholmos : lateral tarsorraphy temporalis transfer Very severe cases : lower lid shortened& retensioned using procedure of palpebroplasty Nose: first stage: postnasal inlay second stage : cantilever bonegraft for nasal support 9/30/2016 70

70. Lateral tarsorraphy

71. Rhinoplasty

72. Facial deformities Eyebrows:reconstruction - free graft of hair bearing skin,island scalp pedicle graft micro follicular hair grafting Ears: reduction auriculoplasty Face: rhytidectomy /face lift procedures Others : Gynaecomastia-mastectomy by websters technique Para phimosis - circumcision External meatal stenosis - periodic dilatation/ meatotomy 9/30/2016 73

73. Secondary deformities Education Prevention and treatment of injuries Use eyes in compensation to loss of sensation Grip aids, Protective footwear 9/30/2016 74

74. Plantar ulcer 1)STAGE OF THREATENED ULCERATION: -foot should be rested in a splint -no wt bearing on the affected foot 2)STAGE OF NECROSIS BLISTER: -blister is padded well -if danger of breaking open,it is snipped & sealed with adhesive plaster and a below knee POP -cast removed after 3 wks & asked to use protective footwear

75. ACUTE ULCER: -absolute bed rest -elevate the foot -Eusol bath,irrigation,dressing -limit surgery to drinage proced -antibiotic if needed -treat as chronic ulcer after acute phase subsides

76. CHRONIC SIMPLE : -Scraping floor of the ulcer -sticking plaster or vaseline gauze dressing -below knee POP cast or bulky dressing -protective footwear+foot care training 2) CHRONIC COMPLICATED: -Ulcer debridement -physiological rest by below knee POP cast -protective footwear on POP removal -corrective deformity,if necessary -identify other complication & treat accordingly -skin graft of large ulcer

77. RECURRENT: - improve quality of scar(scar revision using exision and suture local flap,distant flap,free flap) - reduce load on scar by footwear modification or corrective surg -eradicate infection

78. Protective footwear should have a tough outer sole that will resist penetration by thorn,nails,glass itself doesnt have any nails upper/straps and buckle should not rub against the toes or cause undue pressure MCR(microcellular rubber ) m/c used for reducing the stress generated during walking

79. MCR Footwear

80. Foot care practice 1.Infected ulcer/Cracks 2.Wounds/injury 3.Weakness/paralysis Clean with soap & water Rest & apply antiseptic dressing Apply cooking oil/Vaseline Soak in water Clean and apply clean bandage Protect when working/cooking Oil massage Exercises

81. TROPHIC ULCER 9/30/2016 82

82. Grip Aids Indications –Grossly deformed hand with loss of fingers, fixed contractures, loss of sensory input, total fixed claw hand Made up of – epoxy resin putty grip aids Fitted to any tool / utensils Adheres to any surface Washable and autoclaved Improve grip & protect skin from abrasion and ulcer Improves quality of life Disadvantage – not suitable for heating 9/30/2016 83

83. Rehabilitation Physical & mental restoration Able to resume their place in the home, society & industry Education of the patient, his family & the public 9/30/2016 85

84. References IAL textbook of leprosy Hastings textbook of leprosy Handbook of leprosy- Jopling BD Chaurasia textbook of anatomy 9/30/2016 86

85. “Leprosy work is not merely medical relief; it is transforming frustration of life into joy of dedication, personal ambition into selfless service...” Mahatma Gandhi 9/30/2016 87 THANK YOU