Disabilities in leprosy: an open, retrospective analyses of institutional records

Abstract Background and objectives: Leprosy remains a leading cause of peripheral neuropathy and disability in the world. Primary objective of the study was to determine the incidence of deformities present at a time of diagnosis and new deformities that patients develop over follow up period. Material and methods: An open, retrospective cohort study was performed at a tertiary medical center in western India. Recruitment phase of the study was of 2 years (2009-2010) followed by observation/follow up phase of 7 years till 31st December 2017. New patients with leprosy and released from treatment cases who presented with deformity as defined by WHO disability grade (1998) and subsequently developing new deformities during the follow up period of up to 7 years were included in the study. Results: The study included 200 leprosy patients. Of the total 254 deformities, 168 (66.14%) deformities were noticed at the moment of diagnosis, 20 (7.87%) deformities occurred during the follow up phase. Of all patients, 21.25% had Grade 1 deformity and 6.31% had Grade 2 or more severe deformity. Deformities of hand were most common in 44.48%, followed by feet 39.76%, and face 15.74% respectively. Limitation of study: Mode of inclusion of patient was self-reporting during follow up phase so there is possible under reporting of the disabilities. Conclusion: New deformities continue to develop in certain forms of leprosy even after release from treatment. Long-term & regular follow up of patients who have been released from treatment is required.

Disabilities in leprosy: an open, retrospective analyses of institutional records.

BACKGROUND AND OBJECTIVES: Leprosy remains a leading cause of peripheral neuropathy and disability in the world. Primary objective of the study was to determine the incidence of deformities present at a time of diagnosis and new deformities that patients develop over follow up period. MATERIAL AND METHODS: An open, retrospective cohort study was performed at a tertiary medical center in western India. Recruitment phase of the study was of 2 years (2009-2010) followed by observation/follow up phase of 7 years till 31st December 2017. New patients with leprosy and released from treatment cases who presented with deformity as defined by WHO disability grade (1998) and subsequently developing new deformities during the follow up period of up to 7 years were included in the study. RESULTS: The study included 200 leprosy patients. Of the total 254 deformities, 168 (66.14%) deformities were noticed at the moment of diagnosis, 20 (7.87%) deformities occurred during the follow up phase. Of all patients, 21.25% had Grade 1 deformity and 6.31% had Grade 2 or more severe deformity. Deformities of hand were most common in 44.48%, followed by feet 39.76%, and face 15.74% respectively. LIMITATION OF STUDY: Mode of inclusion of patient was self-reporting during follow up phase so there is possible under reporting of the disabilities. CONCLUSION: New deformities continue to develop in certain forms of leprosy even after release from treatment. Long-term & regular follow up of patients who have been released from treatment is required.

Lessons From Leprosy: Peripheral Neuropathies and Deformities in Chronic Demyelinating Diseases.

In light of the World Health Organization's push to accelerate progress toward a leprosy-free world by 2020, it is fitting to look back on the evolution of progress in treating lepromatous neuropathy and limb deformities. To date, no surgeon has had as great an impact on the understanding and treatment of this disease as Dr Paul Brand. Before Dr Brand's accomplishments, few surgeons participated in the management of the deformed leprous patient. By challenging conventional beliefs, Dr Brand revealed that many of the deformities associated with leprosy were in fact caused by nerve damage and subsequent limb anesthesia. His pioneering work centered on tendon transfers to provide hand and foot mobility to leprous patients, revolutionizing the surgical management of this patient population and restoring functionality to the lives of otherwise stigmatized and functionally handicapped individuals. In the process, he provided us with the surgical principles and techniques that we still apply today. Because of its predilection for the peripheral nervous system, leprosy also provides an excellent opportunity to investigate mechanisms of demyelination and chronic nerve degeneration in nonacute peripheral neuropathies. Processes underlying demyelination of infectious, traumatic, and genetic etiologies overlap and precede the onset of acute neuronal derangement. Glial pathology has been shown to be a common pathological element in leprosy, Charcot-Marie-Tooth type I, multiple sclerosis, and chronic nerve compression injury. The aim of this article is to provide an overview of lepromatous neuropathy with its subsequent deformities as it relates to the pathophysiology, surgical management, and potential therapeutic targets of other modern peripheral neuropathies.

Desempenho funcional de pacientes com deformidades visiveis na hanseniase / Functional performance of patients with visible deformities in leprosy

Esse estudo tem por objetivo avaliar a capacidade funcional da mão das pessoas atingidas pela hanseníase na realização das atividades básicas e instrumentais da vida diária. A amostra foi constituída por 50 pacientes, maiores de 18 anos, que estavam sendo acompanhados no ambulatório do Instituto Lauro de Souza Lima. Para avaliação das características sociodemográficas e clínicas foi elaborado um questionário próprio. As dificuldades manuais para realizar atividades nas áreas de vestuário, alimentação, higiene pessoal, cuidados com a casa, escrita e outros foram avaliados por meio da Avaliação Funcional das Mãos em Hanseníase (AFMH). Os resultados demonstram que em todas as atividades houve algum grau de dificuldade, porém, a maioria dos pacientes as realiza com independência. As atividades consideradas mais difíceis de serem executadas pelos pacientes com garra ulnar foram: pegar objetos pequenos em superfície plana, abrir/fechar fecho de corrente e cortar unhas. Na população de pacientes com garra ulnar/mediano foram: pegar objetos pequenos em superfície plana, abotoar/desabotoar, dar laço/amarrar cadarço e abrir/fechar fecho de corrente. As atividades são realizadas com algum grau de dificuldade, porém, com independência pela maioria dos indivíduos que tem ou tiveram hanseníase e apresentam deformidades visíveis. Muitas dessas dificuldades podem ser minimizadas por meio de transferências tendinosas ou pela indicação, confecção e uso de tecnologia assistiva.

This study aims to evaluate the functional capacity of the hand of people affected by leprosy in performing basic and instrumental activities of daily living. The sample consisted in 50 patients, older than 18 years, who were being treated at the Lauro de Souza Lima Institute’s ambulatory. A questionnaire was developed to assess sociodemographic and clinical characteristics. The manual difficulties to perform activities in the areas of clothing, food, personal hygiene, home care, writing and others were evaluated according to the Functional Assessment of Leprosy Hands (AFMH).The results shows that in all activities there were some degrees of difficulty, however, most patients perform them independently. The activities considered more difficult to perform by ulnar claw patients were: to pick up small objects on a flat surface, to open / close chain closure and to cut nails. In the population of patients with ulnar / median claws were: pick up small objects on a flat surface, button / unbutton, tie / tie shoelace and open / close chain closure. The activities are performed with some degree of difficulty, however, independently by most individuals who have or have had leprosy and have visible deformities. Many of these difficulties can be minimized by means of tendinous transfers or the indication, confection and use of assistive technology.

Clinico-radiological Correlation of Bone Changes in Leprosy Patients Presenting with Disabilities/Deformities.

Leprosy is a medical - social disease, it is associated with stigma in the society due to the resulting deformities in some persons. Although stigma has decreased after the widespread use of MDT, some disabilities do occur which are mostly due to late initiation of treatment and inappropriate care. Besides the nerve and skin involvement bone changes have been reported to be common in leprosy. These bony changes need to be understood in the present MDT era specially in the context of clinical spectrum and duration of disease/ deformities. Fifty clinically diagnosed and histologically classified leprosy patients with deformities/ disabilities of either hands/feet/face who attended the OPD of Department of Dermatology, Venereology and Leprosy, Government Medical College, Amritsar were examined and evaluated in the study. Radiological examination of hands, feet and skull was done in each case and the bone changes in hands and feet; and skull and paranasal sinus changes were correlated with clinical parameters. Bone changes were observed in 90% of cases radiologically. Specific bone changes in hands and feet, non-specific bone changes in hands, feet, skull and paranasal sinuses were seen in 66%, 82% and 32% of cases respectively. Common specific bone changes in hands and feet observed were primary periostitis (14%), honey combing (46%), bone cyst (36%), thinning and irregularity of cortex (28%) and area of bone destruction (20%); Among the non-specific bone changes observed were contracted fingers/claw hands/claw toes (64%) and absorption of terminal phalanges (40%). The maxillary sinus, and paranasal sinus changes were the most common radiological findings observed in skull. The study of the radiological changes may help the clinicians to understand the gravity of the situation and undertake steps for timely prevention of permanent loss of function and the occurrence of deformities and disabilities.

Adaptive shortening of long flexor in patients with claw hand: A short report.

Adaptive shortening of long flexors is the common secondary impairment which can occur in patients with long standing claw hand, particularly in those who do not undergo any supervised physiotherapy programme.¹ The main purpose of this short report is to describe the current physiotherapeutic means which are commonly employed for the management of long flexor tightness. This report further gives an insight into the consequences of the delay in corrective surgery and ultimately describes the dire need for further research on this deformity.² Adaptive shortening of the muscles is a phenomenon which can occur for many reasons, like prolonged immobilisation of the of the body segment, postural imbalance, muscle imbalance, impaired muscle performance due to neuromuscular problems or may be due congenital and acquired deformity.Tightness of the muscles leads to limitation of joint range of motion.³ In leprosy the cause of adaptive shortening of the long flexors is muscle imbalance which occurs due to paralysis of the ulnar nerve at the elbow joint. Adaptive shortening of the muscles is a secondary impairment.4 Secondary impairments have multidimensional effects on the management of the deformity, including delay in surgery for the correction of deformity which in turn is responsible for the unemployment, social stigma and problems in social integration.5 Secondary impairment in some way or other is responsible for the above stated problems, but adaptive shortening of the long flexor (long flexor tightness) is important because sometimes months of therapy are required to attain full muscle length.

Zancolli lasso procedure for correction of paralytic claw hands.

PURPOSE: To evaluate the outcome of 20 claw hands corrected with the Zancolli lasso procedure. METHODS: 20 patients aged 7 to 48 (mean, 28) years with claw hand deformity for a mean of 33.3 months secondary to leprosy (n=17), traumatic ulnar nerve injury (n=2), or tardy ulnar nerve palsy (n=1) underwent the Zancolli lasso procedure, in which the flexor digitorum superficialis (FDS) of the middle finger was divided in to 4 slips (one for each finger) and reattached to itself after passing through the proximal pulley. Deformity, power, movement, and function of the hands were evaluated before and after surgery. RESULTS: The mean duration of surgery was 58.4 (range, 50-107) minutes. The mean duration of follow-up was 18 (range, 12-24) months. Deformity correction was good in 15 patients, fair in 3, and poor in 2. The mean angle improvement was maximum in the little finger, followed by the ring, index, and middle fingers. The transverse metacarpal arch (compared with the normal hand) was increased/reversed in 7 patients and decreased in 13 patients. The grip and pinch strength increased in 13 patients, remained unchanged in 6, and decreased in one. Motion and function of the hand was good in 12 patients, fair in 6, and poor in 2. One patient developed swan neck deformity in the little finger owing to suture tension of the transferred slip, and another had paraesthesia over the index finger. They underwent reoperation using the FDS of the ring finger; no sensory or motor recovery was achieved. CONCLUSION: The Zancolli lasso procedure is easy to perform and does not require extensive postoperative training. It restores synchronous finger flexion and spares other superficialis tendons, thus avoiding swan neck deformity of the fingers.

Split flexor pollicis longus tendon transfer to A1 pulley for correction of paralytic Z deformity of the thumb.

PURPOSE: To test the hypothesis that split flexor pollicis longus (FPL) transfer to the A1 pulley will correct a thumb paralytic Z deformity and that the transfer can be subjected to early postoperative active mobilization protocol. METHODS: In a prospective trial, 19 consecutive thumbs with ulnar or combined ulnar and median nerve paralysis received split FPL transfer to the thumb A1 pulley and active mobilization of transfer after 48 hours. Outcomes were assessed by correction of Z deformity during pinch, tendon transfer insertion pullout during early active mobilization, range of motion at the thumb metacarpophalangeal and interphalangeal joints, and postoperative treatment time. Data from historical records of 20 thumbs with split FPL to extensor pollicis longus (EPL) and 3 weeks' immobilization, treated before the prospective trial in the same institution, were used for comparison. RESULTS: All 19 thumbs with split FPL to A1 pulley achieved Z deformity correction at discharge from rehabilitation. There was no incidence of transfer insertion pullout during active mobilization, and patients were discharged 22 days earlier than the controls who received transfer of FPL to EPL insertion. Seventeen thumbs were available for follow-up more than 1 year after the index procedure. Fifteen thumbs retained deformity correction, and 2 had recurrence of Z deformity. The interphalangeal joint had considerably greater active motion following split FPL to A1 pulley compared with transfer of split FPL to EPL insertion. CONCLUSIONS: This study supports the hypothesis. Split FPL tendon transfer to thumb A1 pulley can correct paralytic thumb Z deformities and be mobilized early for transfer re-education. Improved interphalangeal joint active motion and reduced treatment time are added advantages over FPL transfer to the EPL insertion. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic III.

Factors affecting functional outcome of surgical correction of claw hand in leprosy.

The objective of this paper is to study the results and the factors that impact the results of claw hand surgery in leprosy. 110 patients who underwent claw hand correction between 2002 and 2006 were followed up and studied. Brand's criteria for objective assessment and a visual analog scale for subjective assessment were used. The factors studied were age, sex, clinical, duration of paralysis, long flexor tightness, degree of contracture and type of surgery. Objective assessment showed Excellent or Good results in 76.6%, Fair in 20% and Poor in 3.4%. Subjective assessment showed that 80.8% were fully satisfied or satisfied. Regression analysis showed that age, degree of contracture,duration of paralysis and long flexor contracture were seen as significant factors impacting results of claw hand correction.

Correction of the claw hand.

Intrinsic paralysis can be the manifestation of a variety of pathologic entities (stroke, cerebral palsy, Charcot-Marie-Tooth, muscular dystrophy, leprosy, trauma, cervical disease, and compressive and metabolic neuropathies). Patients present with a spectrum of clinical findings dependent on the cause and severity of the disease. The 3 main problems caused by intrinsic weakness of the fingers are clawing with loss of synchronistic finger flexion, inability to abduct/adduct the digits, and weakness of grip. Clawing is defined as hyperextension of the metacarpophalangeal joints and flexion of the interphalangeal joints. This article describes the clinical evaluation and surgical treatment options for claw hand.

Disability profile in leprosy patients' diagnoses in a rural reference leprosy centre in Ethiopia during 1999-2009.

We assessed the epidemiology of disabilities in leprosy cases treated in a rural hospital over a 10-year period. This is a retrospective data collection using leprosy registers and treatment cards in a rural private mission hospital. Over the 10-year period, 210 patients with leprosy were registered for treatment. One hundred and twenty-eight (61.5%) had disabilities (26.0% grade 1 and 35.6% grade 2): 13.5% ocular disabilities, 44.5% disabilities in hands and 44.7% foot impairment. Patients >19 years had more disabilities (66.7% versus 50.7%) (P = 0.03), especially ocular disabilities (16.7% in >20 versus 6.0% in <20 years) (P = 0.03). This study detected a high prevalence of disabilities.

Multiple grade II deformities in a child: tragic effect of leprosy.

Stigma is almost synonymous with visible deformity and this is what makes leprosy a dreaded disease for the common man. Deformities in children are more distressing, due to the limitation of their physical, academic and social life. An 11-year-old female child was presented with trophic ulceration of right foot and triple nerve paralysis (i.e. total claw hand with wrist drop) of the left hand of 2 years duration. In addition, she had multiple hypopigmented hypoaesthetic patches on the extremities and buttocks. She was diagnosed as a case of borderline tuberculoid leprosy and was started on WHO MB MDT therapy. Leprosy in children is an indicator for disease prevalence in general population and occurrence of deformities points towards the delay in making the diagnosis and the stigma associated with the disease.

Salvage surgery for severely deformed hands in leprosy.

Results of surgery of nine cases of severely deformed hands (considered "useless/hopeless") and the techniques used in each of them to achieve a 'functional hand' are described. The techniques used were: Pollicization of Prof G Brunelli, Cocked-hat technique of Gillies, JESS distractor of BB Joshi. All patients had good results after surgery. However, two patients developed some deformity because of insensitive hands'; This type of surgery is not done routinely but authors would like to encourage other surgeons to try these techniques as hands of a number of patients can be made 'functional' by any of these procedures.