LOCALIZED CONJUNCTIVAL AL-AMYLOID DEPOSITS SECONDARY TO A RETINAL DETACHMENT SURGERY RADIAL EXPLANT.

PURPOSE: To describe a hitherto unreported late ocular surface complication of retinal detachment surgery around a radial segment explant. METHODS: A single case report of a 72-year-old white man, with a previous history of right scleral buckling surgery for retinal detachment surgery 25 years ago, presented with right-sided ptosis of 6 months duration. RESULTS: Ocular surface examination showed a prominent right supero-nasal quadrant radial segment explant, around which there was bulky pink conjunctival mass, extending from the supero-medial fornix down to the medial canthal area and inferior medial fornix with similar changes seen on the upper medial tarsal conjunctiva. The clinical differential diagnosis was either inflammation from an exposed radial explant or lymphoma. Biopsies of the conjunctival mass showed perivascular and interstitial solid eosinophilic deposits of amyloid, with scattered giant cells; the amyloid was of AL type. There was no morphological or immunohistochemical evidence of lymphoma or a plasma cell neoplasm in the specimen. CONCLUSION: This is the first report of localized conjunctival amyloid deposition, secondary to a retinal detachment radial explant. It is proposed that the localized amyloid deposit arose from the ocular surface irritative effects of the radial explant.

RE-INTERVENTION IN DE NOVO VITREOUS OPACITIES AFTER PARS PLANA VITRECTOMY IN FAMILIAL AMYLOIDOTIC POLYNEUROPATHY TTR VAL30METPORTUGUESE PATIENTS.

PURPOSE: To report management of de novo vitreous amyloid opacities after previous pars plana vitrectomy in familial amyloidotic polyneuropathy transthyretin Val30Met. METHODS: This work is a retrospective observational consecutive case series of five eyes of four patients. Demographic data, transthyretin mutation involved, age at the beginning of disease, duration of disease, treatment (liver transplant or tafamidis), time between vitrectomy and re-intervention, and ophthalmologic changes were evaluated. Surgical re-intervention included phacoemulsification with intraocular lens implantation in phakic eyes, re-vitrectomy as complete as possible with posterior capsulectomy, and internal limiting membrane peeling if wrinkling of internal retinal surface was present. RESULTS: All patients had transthyretin Val30Met mutation, and three were women. Mean age of onset of the disease was 52 ± 11.0 years, and average evolution time of the disease was 8 years. Three patients had been submitted to liver transplant 4, 9, and 15 years before. Time between first vitrectomy and surgical re-intervention was longer than 2 years in all cases. Two eyes had amyloid deposits on anterior lens surface and pupillary border with scalloped pupil. Two eyes were phakic. Glaucoma was present in two eyes; one of them had previous trabeculectomy. All cases had vitreous opacities behind posterior lens capsule and at vitreous base area. After re-intervention, no further recurrence was observed (average follow-up of 10 months). CONCLUSION: De novo vitreous amyloid opacities may occur several years after pars plana vitrectomy. Amyloid deposition in vitreous cavity was observed only in strong vitreous adherence locations (behind posterior lens capsule and at vitreous base area). The authors expect that this procedure, an extensive re-vitrectomy associated with posterior capsulectomy, will prevent de novo vitreous amyloid opacities.

A Median Nerve Anterior Transposition Procedure for Multi-Recurrent Hemodialysis-Related Carpal Tunnel Syndrome.

BACKGROUND: Some long-term hemodialysis patients suffer from multi-recurrent carpal tunnel syndrome because amyloid originating from ß2-microglobulin continues to be deposited mainly in the flexor tendons, tendon sheaths and flexor retinaculum during maintenance hemodialysis. These amyloid deposits inside carpal canal (tunnel) tissues increase carpal canal pressure and this leads to compression of the median nerve. When multi-recurrent carpal tunnel syndrome occurs, previous operative scarring of soft tissue may prohibit further enlargement of the carpal canal even if any carpal canal decompression procedure is used. For this reason, we developed a median nerve anterior transposition procedure, as a new approach in the treatment of multi-recurrent hemodialysis-related carpal tunnel syndrome. METHODS: Median nerve anterior transposition procedures were performed on seven hands in six patients with multi-recurrent carpal tunnel syndrome. The mean age of the patients was 68.3 years and the mean hemodialysis duration was 35.3 years. Mean follow-up period was 9.9 months. The median nerve is transposed from inside to outside of the carpal canal under local and infiltration anesthesia without a pneumatic tourniquet on an outpatient basis. This procedure is based on the same principles applied in ulnar nerve anterior transposition procedures for cubital tunnel syndrome. RESULTS: Main preoperative patient complaints were intolerable tingling and/or pain in the diseased hands throughout the day. Following the surgeries, preoperative clinical symptoms began to subside and eventually improved in all hands. Postoperative abductor pollicis brevis muscle power using manual muscle testing improved except in one hand. Abnormal preoperative distal motor and sensory latency were improved except in two hands following the surgeries. CONCLUSIONS: The median nerve anterior transposition procedure is a beneficial treatment for patients suffering from hemodialysis-related multi-recurrent carpal tunnel syndrome.

Synovial deposition of wild-type transthyretin-derived amyloid in knee joint osteoarthritis patients.

OBJECTIVE: To investigate histological features of deposited amyloid in the synovial tissue and its clinical significance in knee joint osteoarthritis (OA) patients. METHODS: We prospectively enrolled 232 consecutive patients who underwent arthroplasty or total replacement of the knee joint for treatment of OA. Congo red staining and immunohistochemistry were performed in the synovial tissue obtained at surgery. When transthyretin (TTR)-derived amyloid was positive, we analyzed all 4 exons of the TTR gene using the direct DNA sequencing method in order to detect mutations. RESULTS: We analyzed 322 specimens in this study. Twenty-six specimens (8.1%) obtained from 21 patients (5 men and 16 women; mean, 79.0 ± 4.6 years) showed deposition of amyloid, which was positively stained with the anti-TTR antibody. Eighteen patients showed inhomogeneous accumulations of amyloid in the loose connective tissue under the synovial epithelia sometimes with nodule formation, while in the remaining three, small vessels in the adipose tissue were involved. Medical records of these patients revealed nothing remarkable in the clinical course, laboratory data or macroscopic intraarticular findings at surgery. No mutations were detectable in the TTR gene analysis. CONCLUSION: Wild-type TTR-derived amyloid may affect the synovial tissue as a result of long-term mechanical stress or as a part of senile systemic amyloidosis in approximately 8% of knee joint OA patients. No obvious clinical significance was found in synovial deposition of amyloid.

Hemodialysis-related upper cervical extradural amyloidoma presenting with intractable radiculopathy.

INTRODUCTION: Destructive spondyloarthropathy may occur in long-term hemodialysis patients, but focal amyloid deposits in the spine are rare. We present a case of upper cervical extradural amyloidoma with a history of long-term hemodialysis presenting with progressive and intractable radiculopathy. CLINICAL PRESENTATION: We describe a 51-year-old female with a long-term history of hemodialysis treatment. She suffered progressive and intolerable right occipital headache. Neurological examination revealed right C2 radiculopathy. Magnetic resonance imaging (MRI) of the cervical spine showed a solid focal extradural mass lesion at the C2 level. She underwent subtotal resection of the extradural mass lesion and decompression of the right C2 nerve root by a posterior approach. Histological examination revealed amyloid deposits. The occipital headache immediately disappeared after surgery. Follow-up MRI 10 months after surgery demonstrated no recurrence of the extradural amyloidoma. CONCLUSION: Development of an upper cervical extradural amyloidoma after long-term hemodialysis is extremely rare. Prompt evaluation of long-term hemodialysis patients suffering from progressive cervical pain should be recommended, and treatment is required if there are signs or symptoms of compression of a nerve root or the spinal cord.