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Reconstructive surgery in children to correct ulnar claw hand deformity due to leprosy.

Introduction

The National Leprosy Elimination Programme (NLEP) 2012-2013 report indicates that 134,752 new cases of leprosy were detected in India during the year. This gives an Annual New Case Detection Rate (ANCDR) of 10 78 per 100,000 population. The NLEP report also shows that a total of 13,387 of these were new child cases, giving a Child Case rate of 1-07/100,000 population. The number of new child cases with Grade II disability were 4,650, which shows the new child disability rate of 3-72/million population. (1)

Ulnar nerve paralysis is the most common impairment in leprosy causing deformities and deficiencies which can be identified by specific tests and signs. (2,3,4) The pattern observed on children was similar to the findings of the 2005 study done by Kar et al. in SLR&TC Karigiri. (5) The muscle imbalance that results from ulnar nerve paralysis creates the characteristic claw hand, due to hyperextension at the metacarpophalangeal (MCP) joints and flexion at the proximal interphalangeal (PIP) joints. (6) As a result, hand function is compromised and impedes the execution of daily activities. (7) The consequence of this easily visible disability on children would be manifold as it hinders the physical, social and academic development of the child. The aim of reconstructive surgery on the hand, as in adults, (8,9) is to increase the capabilities of the child for activities of daily living (ADL), academic development and to restore normality adequately to accelerate the child's integration into the school and society.

Lasso surgery for intrinsic replacement, using the Flexor Digitorum Sublimus (FDS) of the middle or ring finger, the palmaris longus or extensor carpi radialis longus with fascia lata graft, has been a standard procedure for correcting clawed fingers in leprosy. (10,11) To the best of our knowledge, the benefit of reconstructive surgery on children with deformities of the hand was not reported earlier. This paper aims to study the impact of tendon transfer to correct the ulnar claw deformity of leprosy in children.

Methodology

The Leprosy Mission Community Hospital at Naini, Allahabad district in Uttar Pradesh, India is a Referral Centre for leprosy, recognised by the Government of India and has been managing leprosy complications since 1876. About 300 tendon transfers for correcting deformities are done at the hospital each year. The operations are done for established deformities of longer than 6 months duration. The centre has an excellent medical records system and each patient has a unique registration number, facilitating the recording of every detail at baseline and during follow-up. All medical records were computerised in 2007.

All patients less than 14 years of age who underwent claw hand correction during the period 2007 to 2012 have been included in this study. Patients who had an associated median nerve deficit were excluded. During this period, a total of 82 claw hand corrections were done on 79 patients of this group. Apart from basic demographic details and clinical data on leprosy, the ulnar claw hand was assessed in terms of unassisted angles, grip contact, pinch contact, functional activities like opening and closing a jar, buttoning big and small sized buttons, fastening a zip, and simulated counting of money. The unassisted angles were measured using a standard procedure with a goniometer.

Grip contact measures the number of phalanges in contact with a standard wooden cylinder; Pinch contact measures the pulp contact of thumb, index and middle finger during tripod pinch. Functional activities are assessed by measuring the frequency of performing a particular task within a fixed time. The results of these assessments are available for preoperative status, after completion of post-op physiotherapy, as well as at a later follow-up. Associated problems and post-operative complications are also recorded. All the assessments were done by an Occupational therapist, Physiotherapist or physio-technician, each with experience in therapy for reconstructive surgery, and were recorded on a surgical audit form. All surgical procedures were done by the same surgeon. The pre- and post- operative physiotherapy programs were the same for all patients.

SURGICAL PROCEDURE

The insertion of the tendon slips for lasso is through the a1 and proximal part of a2 (a2A) pulleys of each of the four digits. This slip is then looped over the pulley and tied to itself with nylon and sutured with cotton. The motor tendon is usually the flexor digitorum superficialis (FDS) of the ring finger or middle finger. Less frequently, the palmaris longus (PL) or extensor carpi radialis longus (ECRL) tendon are used with a facia lata graft.

PRE AND POST-OPERATIVE PHYSIOTHERAPY MANAGEMENT

Pre-operative therapy focuses on teaching isolated contraction of the tendon to be transferred. The patient is taught active and passive extension exercises of the proximal interphalangeal joint to make the fingers mobile. Serial cylindrical finger cast, gutter splint, outrigger splints were applied when required to correct soft tissue contractures.

Post-operatively, a below elbow Plaster of Paris cast is applied which incorporates a volar slab. This cast is to immobilise the wrist in the neutral position, metacarpophalangeal joints at 90 degrees and interphalangeal joints straight (lumbrical position). The patient is discharged on the 3rd post-operative day.

The plaster is removed on the first day of the 4th week. Post-operative re-education is provided in a staged manner for the next 3 weeks.

During the 4th week, the patient practices the isolated meta-carpophalangeal joint flexion and extension exercises. The volar slab is retained for night use for the initial 3 days. The MCP joint full extension is achieved at the end of the 4th week. During the 5th week, flexion and extension exercises for the proximal and distal interphalangeal joints and grasp activities are introduced. During the 6th week, the patient has functional training like writing, fine manipulation, activities of daily living (ADL) and then discharged from therapy.

Results

The age of the children ranged from 7 to 14 years with a mean (SD) age of 12 (2) years and 41% of them were female. The mean (SD) duration of deformity was 17 (10) months. 55 (67%) of them had lasso procedure using the ring finger FDS tendon, 13 (16%) with Middle finger FDS and 14 (17%) with PL or ECRL tendon with a facia lata graft. Sixty children had multibacillary leprosy and 22 had paucibacillary leprosy. The mean (SD) follow up was at 2 (1) months after discharge for the 1st follow up and at 10 (7) months for the final follow up.

The Mean (SD) unassisted angle is shown in Table 1. This shows the correction of the deformity at the completion of post operative therapy which is maintained at the follow up assessments.

The impact of duration of deformity on Reconstructive surgery (RCS) outcome is shown in Table 2. The results of surgery in terms of unassisted angle are better when the duration of deformity is shorter. In three patients, surgical soft tissue release with full thickness skin graft of the proximal inter-phalangeal joints for the little finger had to be done. Other secondary deformities such as hooding (six hands) and long flexor contractures (three hands) were also prevalent in patients of longer duration of deformity.

The impact of duration of follow-up on outcome of reconstructive surgery is shown in Table 3. There was no statistical significance of follow-up duration on the unassisted angles.

Table 4 compares the functional improvement of hand before and after surgery. There is an improvement in performing specific tasks after correction of deformity which improves further with subsequent follow-ups.

Table 5 shows the impact of age on outcome in terms of unassisted angles. There is no statistically significant difference between the groups.

Discussion

Development of visible deformity in children due to leprosy is unfortunate, especially considering that timely intervention can prevent disability. (5,12) Children with leprosy often report late due to a lack of awareness and by then they may have established deformities. Additionally, children with leprosy are prone to a high risk of developing deformities as compared to the rest of the population. (5,13) Although nerve function impairment can be treated conservatively if detected within 6 months, delay necessitates surgical intervention.

Reconstructive surgery (RCS) in leprosy has a significant role in restoring normal appearance and function of the hand in adults, (8,10) and can have the same impact on children by enabling them to live normal lives in the family, school and society. In this study we have highlighted the effect of RCS on children with established visible deformities in terms of correction of deformity and improvement of hand function.

In this case series 84% of hands had lasso surgery using FDS of either middle or ring fingers. The remaining hands had hypermobile proximal inter-phalangeal joints in which removal of FDS was not favoured as it may cause intrinsic plus deformity. (14) Hence, for the motor tendon, Palmaris Longus (PL) or Extensor Carpi Radialis Longus (ECRL) were used with a fascia lata graft. The unassisted angle, which indicates inadequate functioning of lumbricals and interossei supplied by the ulnar nerve, was drastically reduced after post operative physiotherapy. This enables the patients to straighten their fingers, thus improving appearance and removing a major contributory factor for stigma.

The assessment includes functional ability of the hand based on sample activities which incorporates the components of hand functions like grasp, pinch and in-hand manipulation. (15) The improvement in these activities can be generalized into daily routine activities. Post operatively there was improvement in almost all the components evaluated in this study. This demonstrates that the child can complete daily routine and academic activities without difficulty after surgery and physiotherapy. Also, it is important that during the third week of post-operative physiotherapy protocol, therapy related to activities of daily living and writing tasks be included in occupational therapy to maximise the functional rehabilitation of the hand.

Pre-operative physiotherapy and splints are essential (6) to reduce all the secondary impairments before being selected for surgery. Among the 82 patients, for three patients the contracture was not released with pre-operative physiotherapy and a surgical release of proximal inter-phalangeal joint of little finger with skin graft was done along with the lasso procedure.

There is a general concern that the longer duration of deformity could compromise the outcome of the results. Therefore, in this study we have divided duration of deformity into three groups as less than 1 year, 1-2 years and more than 2 years and compared the unassisted angle at discharge following post-operative physiotherapy. The results of surgery were better when the children were operated on earlier, suggesting that awareness needs to be improved and intervention done at the earliest.

Patients were grouped into three groups of less than 6 months, 6-12 months and more than 12 months based on their duration of final follow-up. There was no statistical significance between the groups in unassisted angle measurements, suggesting that the correction of claw was maintained during follow-up.

Analysis was done to determine the impact of age on outcome in terms of unassisted angle at discharge. They were divided into those more than and less than 10 years of age. There was no statistical significance between the groups suggesting that the outcome is not influenced by age. There was a drop in number of patients during follow-up. The main reason is that the patients come from hundreds of kilometres away; once the deformity is corrected they do not come back unless there are other leprosy complications.

Conclusions

The deformity in hands due to leprosy in children is a tragedy as it hampers the use of hands in daily routine activities, school attendance and other social interactions. Tendon transfer surgery should be done on children to correct established clawing of fingers as it yields good results and helps in facilitating hand functions to complete daily routine and academic activities. Delay in having surgery should be avoided as this compromises the results leading to a life time of residual visible deformity.

Acknowledgements

We thank the staff in the physiotherapy department, medical records department and operation theatre as well as the ward staff for their co-operation and compassionate services. Thank you to the patients for their co-operation during the procedure and physiotherapy as well as travelling long distances for follow-up.

References

(1) National Leprosy Eradication Programme 2012-2013 report, http://nlep.nic.in/data.html

(2) Saunderson PR, Gebre S, Dasta K et al. The pattern of leprosy related neuropathy in the AMFES patients in Ethiopia, definitions, incidence, risk factors and outcome. Lepr Rev, 2000; 71: 285-308.

(3) Saha SP, Das KK. Disability pattern amongst leprosy cases in an urban area (Calcutta). Indian J Lepr, 1993; 65: 305-314.

(4) Sharma P, Kar HK, Beena KR et al. Disabilities in multibacillary leprosy patients before, during and after multidrug therapy. Ind J Lepr, 1996; 68: 127-136.

(5) Kar BR, Job CK. Visible deformity in childhood leprosy--A 10-year study. IntJLep Other Mycobact Dis, 2005; 73: 243-248.

(6) Fritchi EP. Surgical reconstruction and rehabilitation in leprosy. The Leprosy Mission, New Delhi, 1984.

(7) Rajkumar P, Premkumar R, Rajkumar P, Richard J. The impact of hand dominance and ulnar and median nerve impairment on strength and basic daily activities. J Hand Ther, 2005; 18: 40-45.

(8) Anderson GA. The surgical management of deformities of the hand in leprosy. J Bone Joint Surg (Br), 2006; 88: 290-294.

(9) Srinivasan H. Palande. Essential Surgery in Leprosy Techniques for District Hospitals: Surgery for correction of paralytic deformities of the hand. WHO, Geneva, 1997; pp. 81-116.

(10) Malaviya GN. Comparative evaluation of effectiveness of different motor muscles in modified lasso procedure for correction of finger clawing. J Hand Surg[Eur], 2003; 28: 597-601.

(11) Narayanakumar TS. Claw-finger correction in leprosy using half of the flexor digitorum superficialis. J Hand Surg[Eur], 2008; 33: 494-500.

(12) Selvasekar A, Joseph G, Kurian N et al. Childhood leprosy in an endemic area. Lepr Rev, 1999; 70: 21-27.

(13) Chaitra P, Bhat RM. Post elimination status of childhood leprosy: Report from a tertiary-care hospital in south India. Biomed Res Int, 2013; 2013. http://dx.doi.org/10.1155/2013/328673

(14) Malaviya GN. Unfavourable results after surgical correction of claw fingers in leprosy. Ind J Lepr, 1997; 69: 43-52.

(15) Cynthia Cooper. Evaluation of the Hand and Upper extremity. 2nd ed. Fundamentals of Hand Therapy, chapter 5, Elsevier; pp. 80-83.

G MANIVANNAN *, PREMAL DAS **, G KARTHIKEYAN * & ANNAMMA S JOHN ***

* Occupational therapist, TLM Community Hospital Naini, Allahabad, Uttar Pradesh, India

** Surgeon & Superintendent, TLM Community Hospital Naini, Allahabad, Uttar Pradesh, India

*** Research Co-ordinator, The Leprosy Mission Trust India, CNI Bhavan, New Delhi, India

Accepted for publication 11 May 2014

Correspondence to: G Manivannan, Occupational Therapist, The Leprosy Mission Hospital, Naini, Allahabad--211 008, Uttar Pradesh, India (e-mail: manibot_2001@yahoo.com, tlmnaini@tlmindia.org)
Table 1. Comparison of the Mean (standard deviation) of unassisted
angle at different stages of assessment

                                           1st follow-   Final follow-
Angles in degrees   Pre (82)   Post (82)     up (52)        up (40)

Index Finger        34 (21)      2 (5)        2 (4)          2 (4)
Middle Finger       37 (27)      2 (6)        1 (4)          2 (4)
Ring Finger         66 (29)      2 (5)        1 (3)          1 (3)
Little Finger       69 (23)      4 (7)        4 (7)          4 (7)

Table 2. Impact of duration of deformity on unassisted angles measured
at discharge from post-operative physiotherapy

                                 Duration of deformity

Angles in degrees   < 1 year (49)   1-2 years (22)   > 2 years (11)

Index finger             2 (5)           2 (5)            2 (5)
Middle finger            2 (6)           1 (2)             4(9)
Ring finger              1 (3)           2 (3)            6(12)
Little finger            2 (4)           6 (8)           10 (14)
Average                  1 (4)           3 (3)            5 (9)

Angles in degrees   P value

Index finger         1.000
Middle finger        0.374
Ring finger          0.016
Little finger        0.002
Average              0.026

Table 3. Impact of duration of follow-up on un-assisted angles

                          Duration of follow-up

Angles in degrees   < 6 months (20)   6-12 months (10)

Index Finger             1 (3)             3 (5)
Middle Finger            1 (4)             1 (3)
Ring Finger              1 (3)             2 (3)
Little Finger            6 (9)             3 (5)
Average                  2 (4)             2 (3)

                       Duration of
                        follow-up

Angles in degrees   > 12 months (10)   P value

Index Finger             2 (4)          0.398
Middle Finger            3 (5)          0.411
Ring Finger              1 (3)          0.662
Little Finger            3 (4)          0.426
Average                  2 (3)          1.000

Table 4. Comparison of the Mean (standard deviation) of functional
activities at various stages of assessment

                                     Pre Op    Post Op
                                      (82)      (82)

Grasp contact (Number of phalanges   10 (2)    13 (1)
  touching the object)
Pinch contact (Number of phalanges    3 (1)     3 (0.2)
  in contact)
Opening and closing jar               4 (1)     4 (1)
  (Number of times)
Buttoning-small (Number of times)     4 (2)     5 (2)
Buttoning-big (Number of times)       3 (2)     4 (2)
Fastening Zip (Number of times)       8 (3)     9 (3)
Counting money (Total counted)       21 (10)   22 (9)

                                          First     Final
                                     follow-up   follow
                                        (52)      (40)

Grasp contact (Number of phalanges    13 (1)     13 (1)
  touching the object)
Pinch contact (Number of phalanges     3 (0.2)    3 (0.2)
  in contact)
Opening and closing jar                5 (1)      6 (2)
  (Number of times)
Buttoning-small (Number of times)      6 (2)      7 (2)
Buttoning-big (Number of times)        4 (2)      5 (2)
Fastening Zip (Number of times)       10 (4)     10 (4)
Counting money (Total counted)        24 (10)    28 (17)

Table 5. Comparison of unassisted angle at discharge versus age group

                   Age group in years

          Less than 10   More than 10
             N = 20         N = 62      P value

Index        1 (2)          2 (6)        0.141
Middle       1 (2)          2 (7)        0.212
Ring         2 (7)          2 (5)        0.878
Little       4 (7)          4 (8)        0.813
Average      2 (3)          3 (5)        0.346
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Author:Manivannan, G.; Das, Premal; Karthikeyan, G.; John, Annamma S.
Publication:Leprosy Review
Article Type:Report
Date:Jun 1, 2014
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