Epithelioid malignant peripheral nerve sheath tumour of the ulnar nerve around the elbow: a diagnostic and therapeutic challenge.

Epithelioid malignant peripheral nerve sheath tumour (EMPNST) is a rare histological subtype of malignant peripheral nerve sheath tumour (MPNST), accounting for 5% to 17% of MPNSTs. The clinical and MRI findings of EMPNST mimic those of nerve abscesses, similar to the presentation in Hansen's disease. We present one such case with this kind of diagnostic dilemma. Intraoperative findings suggest a tumour changed the course of management subsequently. The development of neurological deficits postoperatively after tumour resection was a reconstructive challenge. To provide motor power and sensation through a procedure that provides a complete functional outcome for a young patient, distal nerve transfers were chosen. This provided an improvement in the quality of life and hastened the neurological recovery of the involved limb. Level of evidence: V.

Epineurotomy for leprous, high ulnar neuropathy: defining a safe corridor based on the epineural vascular anatomy.

BACKGROUND: Leprous neuropathy is treatable but still a source of disability worldwide. Multidrug therapy (MDT) and oral steroids are the main stay of treatment. Ulnar nerve, at the elbow, is commonly involved. Nerve decompression may be required in selected cases by an epineurotomy (internal neurolysis). The preferred surface of ulnar nerve for performing this procedure to minimize iatrogenic vascular compromise is a matter of debate. QUESTIONS/PURPOSES: We describe the epineural vessel arrangement on the medial and lateral surface of ulnar nerve around the medial epicondyle while performing epineurotomy for leprous neuropathy. METHODS: We enrolled patients of symptomatic leprous ulnar neuropathy of less than one year duration on MDT that did not respond to steroids, for surgical decompression. Ten patients underwent epineurotomy of ulnar nerves (N = 11) around medial epicondyle. The epineural vessels were classified as per Sunderland's classification of arteriae nervorum. The number of epineural vessels was assessed on the medial and lateral surface of the ulnar nerve adjoining the medial epicondyle. The epineurotomy incision was placed over the surface of ulnar nerve having relatively less vessels. RESULTS: The mean number of epineural vessels on the medial surface was 9.72 (range; 7-14) and on the lateral surface were 4.72 (range; 3-6). The average number of vessels per cm2 of the medial and lateral surface of the nerve was 0.94 and 0.48, respectively. The most common type of epineural vessel was type 3 on both medial and lateral surface of the nerve. Lateral epineurotomy was performed in all 11 cases. All the patients had relief from neuropathic pain. The mean VAS score improved from 3.20 ± 0.89 to 0.50 ± 0.34 at 2 years follow-up (p = 0.02). The mean motor score improved from 9.31 ± 4.12 to 15.42 ± 3.10 and sensory score improved from 40.0 ± 30.70 to 85 ± 9.90 at two years follow-up (p < 0.01). CONCLUSION: Lateral surface (facing the medial epicondyle) of ulnar nerve has a lesser density of epineural vessels in comparison to its medial (subcutaneous) surface. CLINICAL RELEVANCE: This anatomical understanding may be helpful in minimizing the iatrogenic vascular compromise of ulnar nerve while performing its epineurotomy around the medial epicondyle for leprous neuropathy. The findings may be extrapolated to other clinical indications of epineurotomy of ulnar nerve, for example, in cubital tunnel syndrome, traumatic ulnar neuroma in continuity, and benign ulnar nerve tumors.

Ulnar nerve sonography in leprosy neuropathy.

A 23-year-old woman presented with a half-year history of right forearm sensory and motor dysfunction. Ultrasound imaging revealed definite thickening of the right ulnar nerve trunk and inner epineurium, along with heterogeneous hypoechogenicity and unclear nerve fiber bundle. Color Doppler exhibited a rich blood supply, which was clearly different from the normal ulnar nerve presentation with a scarce blood supply. The patient subsequently underwent needle aspiration of the right ulnar nerve, and histopathological examination confirmed that granulomatous nodules had formed with a large number of infiltrating lymphocytes and a plurality of epithelioid cells in the fibrous connective tissues, with visible atypical foam cells and proliferous vascularization, consistent with leprosy. Our report will familiarize readers with the characteristic sonographic features of the ulnar nerve in leprosy, particularly because of the decreasing incidence of leprosy in recent years.

Decompression of peripheral nerve trunks in leprosy prevents the development and progression of deformities?

Peripheral nerve involvement results in deformities formation in leprosy. High doses of (40-60 mg) steroids along with the anti-leprosy drugs is preferred even though the 70-75% cases develop deformity with the above treatment. 772 ulnar nerves, 120 median nerves and 108 posterior tibial nerves not responding to above medical treatment in 12 weeks, were undertaken for external and internal nerve trunk decompression. These cases were followed-up for 5-20 years at various intervals. The pain in nerves (neuritis) recovered in all cases of ulnar, median and posterior tibial nerves. Full sensory recovery with pin prick/feather or cotton wool touch was seen in 50% cases of all the three nerves. 20% cases maintain the pre-operative levels of sensory status. Plantar ulcers healed within 6 months after decompression of posterior tibial nerve. Only 6 cases showed reoccurrence. Overall motor recovery in ulnar nerve was seen 89% and 70% in median nerve. The sensory recovery restores protective sensation which prevents secondary injuries. The improvement of motor power gave better functional hands and improved the appearance which in absence of surgical intervention was not possible.

Resultados da descompressão e anteriorização do nervo ulnar no cotovelo / Results of ulnar nerve descompression and anterior transposition at the elbow

INTRODUÇÃO: A compressão do nervo ulnar no cotovelo é a segunda causa mais frequente de neuropatia compressiva no membro superior. Na maioria dos casos, a compressão ocorre no canal cubital, vulnerável à compressão extrínseca, intrínseca ou idiopática. O tratamento cirúrgico é controverso. MÉTODO: Os autores descrevem os resultados da descompressão e transposição anterior do nervo ulnar realizadas em 58 pacientes. RESULTADOS: Identificou-se como causa principal a fratura de cotovelo e a hanseníase. Na Rede Sarah, entretanto, têm sido frequente (15 por cento) também as indicações por compressão causada por ossificação heterotópica em casos de lesados medulares e/ou cerebrais. Realizamos 57 por cento de procedimentos associados à retinaculotomia dos flexores, descompressão no canal de Guyon e transferência tendínea para músculos intrínsecos. Obteve-se 80 por cento de bons e excelentes resultados, 20 por cento de complicações, dor neuropática, distrofia simpática reflexa e manutenção dos sintomas, observadas em pacientes diabéticos e portadores de hanseníase.

INTRODUCTION: Ulnar nerve compression is the second most frequent entrapment neuropathy of the upper extremity. The most cases to occur at the elbow, with an extrinsic, intrinsic or idiopathic compression. The surgical technique varies. METHODS: This study assesses the results of a series of subcutaneous transpositions of the ulnar nerve. RESULTS: The most frequent cause is elbow fracture, and Hansen disease. In the Sarah Network is frequent heterotopic ossification entrapment because spinal cord injury and stroke. We performed 57 percent associated surgical procedures for Guyon, carpal decompression and tendinous transference. This study shows 80 percent cases were good and excellent results and 20 percent of complications, neuropathy pain, complex regional pain and persistent symptoms in diabetes and Hansen disease.

Digital neuropathy of the median and ulnar nerves caused by Dupuytren's contracture: Case report.

INTRODUCTION: Digital neuropathy is a pure sensory neuropathy of a digital nerve. It may be caused by acute or chronic local trauma or pressure, or accompany systemic illnesses such as rheumatoid disease, leprosy, Raynaud disease, dysproteinemia, or diabetes mellitus. We describe an extraordinary case of digital neuropathy of the median and ulnar nerves caused by Dupuytren contracture. CASE REPORT: A 56-year-old right-handed man was presented with numbness and tingling of the little finger of the right and ring finger of the left hand. The clinical and EMG findings in this patient were consistent with a lesion of the median and ulnar palmar digital nerves of the right and left ring and little fingers. CONCLUSION: Dupuytren tissue usually affects the palmar fascia, superficial to the digital nerves, and it may rarely affect the spiral cord in the digits. A spiral cord may cause sensory loss due to impingement of digital nerves or Dupuytren tissue may have been compressing the palmar digital nerves against the relatively inelastic deep transverse metacarpal ligament. As a result, digital neuropathy can develop in those with Dupuytren's contracture, and nerve conduction studies should also be performed to determine the condition. New studies are needed to provide better diagnostic criteria for the condition.

Ulnar nerve abscess and relapse in a patient with indeterminate leprosy 1 year after completion of multidrug therapy.

The introduction of multidrug therapy has efficiently controlled leprosy in developing countries. However, Mycobacterium laprae may survive and cause relapse despite adequate treatment with antileprosy drugs. Relapse may be characterized by a combination of new signs or symptoms and presence of acid-fast bacilli on skin or nerve biopsy samples. We report a case of a child in whom ulnar abscess developed 12 months after successful completion of multidrug therapy with clinical and histopathological evidence of relapse.

Ulnar and median nerves in paucibacillary leprosy--a follow-up study of electrophysiological functions in patients before and after nerve trunk decompression.

Electrophysiological functions of ulnar and median nerves in paucibacillary leprosy patients were studied. Patients who showed deterioration of sensory motor functions in spite of steroid therapy were offered nerve decompression together with oral steroids. On periodic follow-up of those who opted for surgery, it was observed, in general, that NCV and amplitude remained reduced even though clinical recovery occurred. Only 80% recovery of electrophysiological functions was seen (as compared to control levels), even in cases that showed good results. Motor function recovered better than sensory function. Complete electrophysiological recovery, if it occurs at all, takes much longer than clinical recovery.

Restoration of sensibility in irreparable ulnar and median nerve lesions with use of sensory nerve transfer: long-term follow-up of 20 cases.

A prospective study was conducted to evaluate patient outcomes following sensory nerve transfer. Twenty patients with irreparable ulnar or median nerve lesions underwent the procedure. Nerve involvement was bilateral in 5 cases. The mean age of the patients at the time of surgery was 29 years. The mean paralysis time and the average length of follow-up were 59 and 78 months, respectively. Eighteen of 20 patients attended a sensory re-education program after surgery. Outcome was assessed objectively by functional sensory recovery testing and by the British Medical Research Council standards. Subjective outcome was assessed by a questionnaire. Two-point discrimination of less than 10 mm was achieved in 15 of 25 hands. The mean functional sensory recovery score was 83. Eighteen of 20 patients reported that the function of their hands improved after the procedure. Good or excellent results were associated with immediate transfer of the nerve, young age, and patients' attendance to the sensory re-education program after surgery. No differences were found between the recovery of ulnar and median nerves. Based on these results we suggest that sensory nerve transfer is a simple and reliable way of restoring sensibility to the hand with favorably comparable results over conventional nerve grafting in selected cases.

Ramo cutaneo dorsal do nervo ulnar (RCDU): alternativa para biopsia diagnostica em neuropatias perifericas / ?

Co o objetivo de avaliar a importancia e viabilidade da biopsia do ramo cutaneo dorsal do nervo ulnar (RCDU), foram estudadas 14 amostras de nervo retiradas de doadores com morte encefalica e 17 biopsiadas, atraves de incisao transversal, em pacientes com idades de 5 a 73 anos, em investigacao por neuropatia periferica. Em dez pacientes foi verificada a area de hipo/anestesia resultante da biopsia. Os nervos foram fixados em glutaraldeido a 2,5 por cento em tampao cacodilato de sodio, pos-fixados em OsO4, desitratados em etanol e incluidos em resina Epon. Amostras de cada nervo tambem foram incluidas em parafina e coradas pelos metodos de Faraco, tricromico de Gomori, vermelho congo e hematoxilina-eosina. As secoes transversais semifinas foram coradas com azul de toluidina.

Surgery for neuritis in leprosy: indications for and results of different types of procedures.

From December 1988 to December 1992, 129 surgical procedures were performed on the peripheral nerves of 64 leprosy patients at the Hospital Cardinal Léger de l'Institut Fame Pereo for leprosy control in Haiti. Sixty-four patients totalizing 129 nerves with sufficient clinical data form the basis of this study. Based on the retrospective analysis of the operated cases, a new classification built on macroscopic findings of the involved nerves is presented. Five grades, according to the presenting aspects of these nerves, are set up as guides for different surgical procedures to be performed on the nerves: external decompression for the lesser grades I and II, intraneural neurolysis, interfascicular neurolysis for the higher grades III and IV, cleaning, and debridement for grade V. The final results are discussed. This new macroscopic grading done at surgery helps to minimize the aggressive procedures performed on nerve trunks, decrease the morbidity of surgical action on the nerve vascular structures, and consequently, preserves all possible sensory and motor functions of a nerve.

Resultados de neurolise de nervo ulnar em neurite hansênica / Results of neurolysis of the ulnar nerve in leprosy neuritis

Um grupo de 33 pacientes de hanseníase apresentando neurite do nervo ulnar foram submetidos a neurolise com transposiçao anterior sub-cutânea deste nervo. A cirurgia foi indicada devido à progressiva piora da funçao neural mesmo com tratamento clínico. O teste com monofilamentos de Semme-Weinstein e avaliaçao de força motora foram utilizados para avaliar os pacientes antes e 12 meses após a cirurgia, 39,3 por cento apresentaram melhora, 39,3 por cento permaneceram estáveis e 21,4 por cento apresentaram piora da funçao neural.

Intraoperative spinal root stimulation to detect most proximal site of leprous ulnar neuritis.

In 10 patients with leprous ulnar neuritis, we investigated the most proximal site of lesion in the affected nerves. Spinal roots C8 and T1 were stimulated intraoperatively to evoke efferent mixed compound nerve action potentials which were recorded from the exposed ulnar nerves. The site at which amplitudes reached a maximum was considered the most proximal site of lesion. Nerve damage was found far proximally from the thickened segments in otherwise inconspicuous sections. Epineuriotomy within these apparently unaffected segments revealed fibrosis of the interfascicular epineurium in 9 patients, which is an indication for microsurgical interfascicular neurolysis.

Treatment of leprous neuritis by neurolysis combined with perineural corticosteroid injection.

A study on leprous neuritis, involving the ulnar nerve, was carried out on 39 patients. The evaluation of nerve function was done before and after treatment by a score chart. Patients were divided into two groups. Group A (21 patients) was subjected to neurolysis only, and group B (18 patients) were given the combined treatment of neurolysis and perineural corticosteroid injection at the same time as neurolysis and subsequently at the end of the second and third weeks. In group B, 83.3% of patients showed 10% or more increase in the posttreatment score in comparison with 57.1% in group A. Improvement was more marked in paucibacillary cases and when the duration of nerve involvement was less than 3 months. Patients with short segments of nerve involvement with minimal thickening had better recovery. This procedure was observed to be simple, easy and well accepted by the patients, with a marked beneficial effect.