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Neurosurgery in Hansen’s
Disease
Dr Amit Agrawal, MCh
India
∗ To share difficulties
∗ Answer to queries
∗ Available evidence about surgery
Purpose
∗ 25–30 per cent
∗ Reactions or nerve damage at one time or another
∗ The longer the delay between the appearance of the first
symptoms of Hansen’s Disease and the start of treatment,
the more likely it is for nerve damage to occur
Hansen’s Disease and nerve
damage
Risk of new nerve damage developing in new
cases of Hansen’s Disease
∗ Croft RP et al, A clinical prediction rule for nerve-function
impairment in leprosy patients. Lancet 2000;355: 1603-6.
PB MB
Nerve function at diagnosis 1% 16%
Impaired nerve function at diagnosis 16% 65%
∗ Sudden increase in neural pathology
∗ Extension of MDT programmes
∗ Without adequate infrastructure to detect and treat early
neuritis
∗ Salafia A, Chauhan G. Nerve abscess in children and
adults leprosy patients: analysis of 145 cases and review
of the literature. Acta Leprol. 1996;10(1):45-50.
∗ Primary impairment
∗ Muscle weakness and/or sensory loss
∗ Loss of sweating makes the skin more vulnerable to injury
∗ Secondary impairment
∗ Wounds, Ulcers, Osteomyelitis
∗ Loss of tissue (fingers/toes)
∗ Fixed contractures/deformities of hand or foot
∗ Corneal damage and its sequel
Nerve damage
∗ Without obvious symptoms
∗ Can be presenting symptom
∗ New nerve damage during treatment
∗ Worsening in nerve damage on treatment
Manifestations
∗ Late nerve damage (more than a year after completing
MDT)
∗ Nerve damage more than three years after completing MDT
(true relapse or reaction)
∗ Part of reaction
Manifestations
∗ Cranial and peripheral nerves
∗ Neuritis
∗ Thickened, tender or painful nerves
∗ Abscess
∗ Fibrosis
Nerve lesions
∗ Screening for loss of protective sensation
∗ Nerve conduction studies
∗ Functional status of the muscles
∗ Electromyography
∗ MRI and MRN
∗ USG/Color Doppler
∗ Nerve biopsy/Skin biopsy
Investigations
∗ Maas M, Slim EJ, Akkerman EM, Faber WR. MRI in clinically
asymptomatic neuropathic leprosy feet: a baseline study.
Int J Lepr Other Mycobact Dis. 2001 ;69(3):219-24.
∗ Department of Radiology, Suite G1-231, Academic Medical
Center, Meibergdreef 9, 1105 AZ Amsterdam Zuidoost, The
Netherlands.
MRI
∗ Mainly in trauma
∗ MRN can add clinically useful diagnostic
information in many situations in which physical
examinations, electrodiagnostic tests, and existing
image techniques are inconclusive.
∗ Filler AG, Kliot M, Howe FA, Hayes CE, Saunders DE,
Goodkin R, Bell BA, Winn HR, Griffiths JR, Tsuruda JS.
Application of magnetic resonance neurography in the
evaluation of patients with peripheral nerve pathology. J
Neurosurg. 1996 ;85(2):299-309.
Magnetic resonance neurography (MRN)
∗ US and MR imaging are able to detect nerves abnormalities
in leprosy. Active reversal reactions are indicated by
endoneural color flow signals as well as by an increased T2
signal and Gd enhancement. These signs would suggest
rapid progression of nerve damage and a poor prognosis
unless antireactional treatment is started.
Martinoli C, Derchi LE, Bertolotto M, Gandolfo N, Bianchi S, Fiallo P,
Nunzi E. US and MR imaging of peripheral nerves in leprosy. Skeletal
Radiol. 2000 ;29(3):142-50.
∗ Thickened nerve and abscess
∗ Hari S, Subramanian S, Sharma R. Magnetic resonance imaging
of ulnar nerve abscess in leprosy: a case report. Lepr Rev. 2006
;77(4):381-5.
MRN
∗ Skin lesions may not reflect the extent of nerve
involvement
∗ Clinical features may not reflect the extent of nerve
involvement
∗ Role of investigations to be defined
Difficulties
∗ To stop permanent nerve damage
∗ Reverse the nerve damage
∗ Preventing mutilating damage
∗ Management of co-morbid conditions
∗ Rehabilitation
Management: Aim
∗ Anti-inflammatory drugs and chemotherapy
∗ Early detection of leprosy and treatment with MDT remains
the best way to prevent disability
∗ Operative intervention
∗ Both
Management: Options
∗ Specialists
∗ Multidisciplinary approach
∗ A motivated patient
∗ An effective medical care system
Pre-requisites
∗ Pain relief
∗ Prevention or reduction in motor and sensory deficits
∗ Substantial recovery
Surgery: Aim
∗ Painful leprous nerve
∗ Non-functional nerve
∗ Heavily infected nerve
∗ Nerve swelling during management
∗ Reaction
Surgery: Assessment
∗ Number
∗ Extent
∗ Slowly progressive, rapid or fulminant
∗ Reversible or irreversible
∗ Extent of disability
∗ Status of immunity
Surgery: Assessment
∗ Neurolysis
∗ External
∗ Extraneural
∗ Funicular
∗ Transposition
∗ Pandya SS. Surgery on the peripheral nerves in leprosy.
Neurosurg Rev. 1983;6(3):153-4.
Surgery: options
∗ Treatment of nerve abscess
∗ Pandya SS. Surgery on the peripheral nerves in leprosy.
Neurosurg Rev. 1983;6(3):153-4.
∗ Agrawal A, Dalal M, Makkannavar JH, Shetty JP. Ulnar
nerve abscess and relapse in a patient with
indeterminate leprosy 1 year after completion of
multidrug therapy. Pediatr Neurosurg. 2005 ;41(3):162-4.
Surgery: options
∗ Excision of the granulomatous lesions
∗ Bridging with nerve autografts
∗ Sunderland S. The internal anatomy of nerve trunks in
relation to the neural lesions of leprosy. Observations
on pathology, symptomatology and treatment. Brain
1973 ; 96 :865-88.
Surgery: options
∗ Major nerve trunks
∗ Need of long cable graft
∗ Limited donor material
∗ Sensory donor nerve-indispensable
∗ Diseased donor nerve
∗ Pereira JH, Cowley SA, Gschmeissner SE, Bowden REM,
Turk JL. Denatured muscle grafts for nerve repair an
experimental model of nerve damage in leprosy. J Bone
Joint Surg [Br J] 1990; 72-B:874-80.
Unsuccessful
∗Free gracilis-muscle transfer
∗ Richard BM. Location of the extracranial extent of leprous
facial nerve pathology may allow leprous facial palsy to be
reanimated by free muscle transfer. Br J Plast Surg. 2003 ;
56(1):14-9.
Substitutes for nerves
∗Autogenous muscle graft
∗ Pereira JH, Cowley SA, Gschmeissner SE, Bowden REM,
Turk JL. Denatured muscle grafts for nerve repair an
experimental model of nerve damage in leprosy. J Bone
Joint Surg [Br J] 1990; 72-B:874-80.
∗ Keynes RJ, Hopkins WC, Huang L-H. Regeneration of
mouse peripheral nerves in degenerating skeletal
muscle. Brain 1984; 29ı :275-8 1.
∗Needs further clinical trials
∗ Role of surgery is still not well defined
∗ Malaviya GN. Shall we continue with nerve trunk
decompression in leprosy? Indian J Lepr. 2004;76(4):331-
42.
Challenges
∗ Choice of a nerve
∗ Can we use infected nerve
∗ Healing of nerves
∗ Autologus graft
∗ Will it act as a source of bacilli
Challenges
∗ Determining nerve damage
∗ Anticipating nerve damage
∗ Response to medical treatment
∗ Safety of medical treatment
∗ Wait for recovery
∗ When to intervene
Challenge
∗ Mainly seen by neurologists and specialists
∗ Few experts
∗ Very few specialists in surgery
∗ Even fewer expert
∗ Reconstructive surgeons
∗ Hand surgeons
∗ Orthopedic surgery
Challenges
∗ Patient is always on risk of nerve damage
∗ Best
∗ Surgery or medicine
∗ Surgery on nerves or reconstructive
∗ Nerve repair or graft or replacement
∗ Less consensus
∗ More controversies
Conclusion
∗ Non-controversial management guidelines
Conclusion
Neurosurgery in Hansen’s Disease

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Neurosurgery in Hansen’s Disease

  • 1. Neurosurgery in Hansen’s Disease Dr Amit Agrawal, MCh India
  • 2. ∗ To share difficulties ∗ Answer to queries ∗ Available evidence about surgery Purpose
  • 3. ∗ 25–30 per cent ∗ Reactions or nerve damage at one time or another ∗ The longer the delay between the appearance of the first symptoms of Hansen’s Disease and the start of treatment, the more likely it is for nerve damage to occur Hansen’s Disease and nerve damage
  • 4. Risk of new nerve damage developing in new cases of Hansen’s Disease ∗ Croft RP et al, A clinical prediction rule for nerve-function impairment in leprosy patients. Lancet 2000;355: 1603-6. PB MB Nerve function at diagnosis 1% 16% Impaired nerve function at diagnosis 16% 65%
  • 5. ∗ Sudden increase in neural pathology ∗ Extension of MDT programmes ∗ Without adequate infrastructure to detect and treat early neuritis ∗ Salafia A, Chauhan G. Nerve abscess in children and adults leprosy patients: analysis of 145 cases and review of the literature. Acta Leprol. 1996;10(1):45-50.
  • 6. ∗ Primary impairment ∗ Muscle weakness and/or sensory loss ∗ Loss of sweating makes the skin more vulnerable to injury ∗ Secondary impairment ∗ Wounds, Ulcers, Osteomyelitis ∗ Loss of tissue (fingers/toes) ∗ Fixed contractures/deformities of hand or foot ∗ Corneal damage and its sequel Nerve damage
  • 7. ∗ Without obvious symptoms ∗ Can be presenting symptom ∗ New nerve damage during treatment ∗ Worsening in nerve damage on treatment Manifestations
  • 8. ∗ Late nerve damage (more than a year after completing MDT) ∗ Nerve damage more than three years after completing MDT (true relapse or reaction) ∗ Part of reaction Manifestations
  • 9.
  • 10. ∗ Cranial and peripheral nerves ∗ Neuritis ∗ Thickened, tender or painful nerves ∗ Abscess ∗ Fibrosis Nerve lesions
  • 11. ∗ Screening for loss of protective sensation ∗ Nerve conduction studies ∗ Functional status of the muscles ∗ Electromyography ∗ MRI and MRN ∗ USG/Color Doppler ∗ Nerve biopsy/Skin biopsy Investigations
  • 12. ∗ Maas M, Slim EJ, Akkerman EM, Faber WR. MRI in clinically asymptomatic neuropathic leprosy feet: a baseline study. Int J Lepr Other Mycobact Dis. 2001 ;69(3):219-24. ∗ Department of Radiology, Suite G1-231, Academic Medical Center, Meibergdreef 9, 1105 AZ Amsterdam Zuidoost, The Netherlands. MRI
  • 13. ∗ Mainly in trauma ∗ MRN can add clinically useful diagnostic information in many situations in which physical examinations, electrodiagnostic tests, and existing image techniques are inconclusive. ∗ Filler AG, Kliot M, Howe FA, Hayes CE, Saunders DE, Goodkin R, Bell BA, Winn HR, Griffiths JR, Tsuruda JS. Application of magnetic resonance neurography in the evaluation of patients with peripheral nerve pathology. J Neurosurg. 1996 ;85(2):299-309. Magnetic resonance neurography (MRN)
  • 14. ∗ US and MR imaging are able to detect nerves abnormalities in leprosy. Active reversal reactions are indicated by endoneural color flow signals as well as by an increased T2 signal and Gd enhancement. These signs would suggest rapid progression of nerve damage and a poor prognosis unless antireactional treatment is started. Martinoli C, Derchi LE, Bertolotto M, Gandolfo N, Bianchi S, Fiallo P, Nunzi E. US and MR imaging of peripheral nerves in leprosy. Skeletal Radiol. 2000 ;29(3):142-50.
  • 15. ∗ Thickened nerve and abscess ∗ Hari S, Subramanian S, Sharma R. Magnetic resonance imaging of ulnar nerve abscess in leprosy: a case report. Lepr Rev. 2006 ;77(4):381-5. MRN
  • 16. ∗ Skin lesions may not reflect the extent of nerve involvement ∗ Clinical features may not reflect the extent of nerve involvement ∗ Role of investigations to be defined Difficulties
  • 17. ∗ To stop permanent nerve damage ∗ Reverse the nerve damage ∗ Preventing mutilating damage ∗ Management of co-morbid conditions ∗ Rehabilitation Management: Aim
  • 18. ∗ Anti-inflammatory drugs and chemotherapy ∗ Early detection of leprosy and treatment with MDT remains the best way to prevent disability ∗ Operative intervention ∗ Both Management: Options
  • 19. ∗ Specialists ∗ Multidisciplinary approach ∗ A motivated patient ∗ An effective medical care system Pre-requisites
  • 20. ∗ Pain relief ∗ Prevention or reduction in motor and sensory deficits ∗ Substantial recovery Surgery: Aim
  • 21. ∗ Painful leprous nerve ∗ Non-functional nerve ∗ Heavily infected nerve ∗ Nerve swelling during management ∗ Reaction Surgery: Assessment
  • 22. ∗ Number ∗ Extent ∗ Slowly progressive, rapid or fulminant ∗ Reversible or irreversible ∗ Extent of disability ∗ Status of immunity Surgery: Assessment
  • 23. ∗ Neurolysis ∗ External ∗ Extraneural ∗ Funicular ∗ Transposition ∗ Pandya SS. Surgery on the peripheral nerves in leprosy. Neurosurg Rev. 1983;6(3):153-4. Surgery: options
  • 24. ∗ Treatment of nerve abscess ∗ Pandya SS. Surgery on the peripheral nerves in leprosy. Neurosurg Rev. 1983;6(3):153-4. ∗ Agrawal A, Dalal M, Makkannavar JH, Shetty JP. Ulnar nerve abscess and relapse in a patient with indeterminate leprosy 1 year after completion of multidrug therapy. Pediatr Neurosurg. 2005 ;41(3):162-4. Surgery: options
  • 25.
  • 26. ∗ Excision of the granulomatous lesions ∗ Bridging with nerve autografts ∗ Sunderland S. The internal anatomy of nerve trunks in relation to the neural lesions of leprosy. Observations on pathology, symptomatology and treatment. Brain 1973 ; 96 :865-88. Surgery: options
  • 27. ∗ Major nerve trunks ∗ Need of long cable graft ∗ Limited donor material ∗ Sensory donor nerve-indispensable ∗ Diseased donor nerve ∗ Pereira JH, Cowley SA, Gschmeissner SE, Bowden REM, Turk JL. Denatured muscle grafts for nerve repair an experimental model of nerve damage in leprosy. J Bone Joint Surg [Br J] 1990; 72-B:874-80. Unsuccessful
  • 28. ∗Free gracilis-muscle transfer ∗ Richard BM. Location of the extracranial extent of leprous facial nerve pathology may allow leprous facial palsy to be reanimated by free muscle transfer. Br J Plast Surg. 2003 ; 56(1):14-9. Substitutes for nerves
  • 29. ∗Autogenous muscle graft ∗ Pereira JH, Cowley SA, Gschmeissner SE, Bowden REM, Turk JL. Denatured muscle grafts for nerve repair an experimental model of nerve damage in leprosy. J Bone Joint Surg [Br J] 1990; 72-B:874-80. ∗ Keynes RJ, Hopkins WC, Huang L-H. Regeneration of mouse peripheral nerves in degenerating skeletal muscle. Brain 1984; 29ı :275-8 1.
  • 31. ∗ Role of surgery is still not well defined ∗ Malaviya GN. Shall we continue with nerve trunk decompression in leprosy? Indian J Lepr. 2004;76(4):331- 42. Challenges
  • 32. ∗ Choice of a nerve ∗ Can we use infected nerve ∗ Healing of nerves ∗ Autologus graft ∗ Will it act as a source of bacilli Challenges
  • 33. ∗ Determining nerve damage ∗ Anticipating nerve damage ∗ Response to medical treatment ∗ Safety of medical treatment ∗ Wait for recovery ∗ When to intervene Challenge
  • 34. ∗ Mainly seen by neurologists and specialists ∗ Few experts ∗ Very few specialists in surgery ∗ Even fewer expert ∗ Reconstructive surgeons ∗ Hand surgeons ∗ Orthopedic surgery Challenges
  • 35. ∗ Patient is always on risk of nerve damage ∗ Best ∗ Surgery or medicine ∗ Surgery on nerves or reconstructive ∗ Nerve repair or graft or replacement ∗ Less consensus ∗ More controversies Conclusion
  • 36. ∗ Non-controversial management guidelines Conclusion

Editor's Notes

  1. Aim of my presentation will be to find out the answer to some queries and clarification of about available evidence
  2. Probably 25–30 per cent of all people with leprosy experience reactions or nerve damage at one time or another The longer the delay between the appearance of the first symptoms of leprosy and the start of treatment, the more likely it is for nerve damage to occur
  3. Can be attributed, in part, to the extension of MDT programmes without adequate infrastructure to detect and treat early neuritis
  4. Has a wide spectrum to damage New nerve damage may occur without obvious symptoms Nerve lesions can be the presenting symptom New nerve damage during treatment Worsening in nerve damage on treatment
  5. Can involve both cranial and peripheral nerves
  6. Is there any way to investigate neural fibrosis like MRI and MRN If yes will it be able to predict the type of intervention
  7. Whether skin lesions reflect the extent of nerve involvement? No Clinical features reflect the extent of nerve involvement? No Before clinical presentation a significant amount of damage has happened Than how to assess
  8. Reverse the nerve damage to the extent of full recovery Management of co-morbid conditions (AIDS, diabetes, eye involvement, ulcers etc.) Rehabilitation- functional and occupational Preventing foot complications Identifying those at risk Emotional stress Physical disability Productivity and financial losses Management of ulcers (particularly foot)
  9. Early detection of leprosy and treatment with MDT remains the best way to prevent disability
  10. Specialist and Multidisciplinary approach to deal and detect nerve injury in leprosy
  11. Whether these are the problems Whether is it a part of reaction Whether this damage is happening in spite of treatment Nerve swelling during management including MDT and steroids Reaction
  12. Is it possible to determine the extent of nerve damage? Whether experts are available or not, medications, operative and intervention facilities, investigations How much of the length of nerve is involved
  13. Excision of the granulomatous lesions and bridging with nerve autografts
  14. Major nerve trunks are involved Needing long cable graft repairs with multiple cutaneous nerves Limited donor material means that large lesions cannot be repaired The loss even of one sensory nerve is detrimental to a patient already suffering from some anaesthesia Finally the donor nerve may itself be diseased
  15. Every nerve is indispensable particulary when these are compromised When choosing a nerve is it normal or affected Whether infected nerve can be used for graft Healing of that nerve? Whether infected graft will act as a source of bacilli as it may be difficult to eradicate the infection
  16. Is it possible to determine and anticipate nerve damage Who will respond to medical treatment Medical treatment will not aggravate the nerve damage Whether to wait for recovery in nerve function Why these questions because there is very effective medical treatment is available When to intervene and when to do the reconstruction
  17. This issue is further complicated by the following facts Mainly seen by neurologist and specialists in the fields Lack of awareness and experts Very few specialists in surgery Even fewer surgeons with knowledge in reconstructive, hand, and orthopedic surgery
  18. Need to define present consensus and controversies Need to develop well defined non-controversial and management guidelines It is important to realize that patient is always on risk of nerve damage even he is on treatment including steroids One must search carefully for nerve damage in a leprosy patient