Genetic Alterations in Melanocytoma Associated with Oculodermal Melanocytosis: Molecular Characteristics.

Melanocytoma is a benign tumor with histologic similarity to oculodermal melanocytosis, which can undergo malignant transformation in rare cases. Melanocytoma more commonly involves the optic disc, and few cases of orbital melanocytoma have been reported. Primary orbital melanoma is a rare malignancy known to arise from pigmentary conditions, although there is little information on this tumor arising from melanocytoma. The authors present a case of malignant transformation of orbital melanocytoma in the setting of ipsilateral oculodermal melanocytosis. This case illustrates histopathologic features associated with malignant transformation and highlights the significance of GNAQ, BAP1, and specific intrachromosomal alterations occurring in oculodermal melanocytosis and melanocytoma. The molecular markers observed in this case are of interest as they have overlap with those present in uveal melanoma. This case demonstrates a timeline of genetic and molecular alterations occurring in the malignant transformation of orbital melanocytoma.

Metacarpophalangeal Joint Pyrocarbon Arthroplasty for Osteoarthritis: An Analysis of 44 Arthroplasties.

PURPOSE: The objective of this study was to analyze postoperative outcomes in a group of patients who underwent metacarpophalangeal (MCP) arthroplasty using a pyrocarbon prosthesis for noninflammatory arthritis. METHODS: An analysis of 44 MCP joint arthroplasties in 30 patients with >2 years of follow-up over a 12-year period was reviewed. The mean age was 63 years. The primary operative indication was pain and stiffness from osteoarthritis refractory to nonsurgical management. RESULTS: At a mean follow-up of 6 ± 3 years, 8 (18%) joints underwent reoperation, including 5 (11%) that underwent revision arthroplasty. The 2- and 5-year rates for survival free of revision arthroplasty were 95% and 93%, respectively. One (2%) operation was complicated by intraoperative fracture. Postoperative complications occurred in 8 (18%) fingers and included ligament/tendon rupture (n = 3) and instability (n = 2). There was significant postoperative improvement in pain levels, MCP arc of motion, pinch strength, and grip strength. At a mean 5 years of radiographic follow-up, 7% had progressive implant instability because of grade 3 or greater loosening. No joints experienced implant instability from progressive subsidence. CONCLUSIONS: Metacarpophalangeal arthroplasty using a pyrocarbon implant for osteoarthritis demonstrates an 7% revision rate at 5 years after surgery. Complications lead to reoperation in 1 of 5 arthroplasties. Radiographic evidence of implant instability was uncommon. Overall, patients experienced predictable pain relief and improvements in their range of motion and pinch strength. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Contemporary Outcomes of Transfemoral Amputation After Total Knee Arthroplasty.

BACKGROUND: Transfemoral amputation (TFA) is a salvage procedure for unreconstructable failed total knee arthroplasty (TKA). Prior studies have reported poor outcomes, patient survival, and prosthetic use. The purpose of this study was to analyze patient outcomes and prosthetic utilization in a contemporary group of patients undergoing TFA in the setting of a TKA. METHODS: We reviewed 112 patients undergoing TFA with a prior TKA. Indications for amputation and postoperative functional measures were captured through chart review. Patients were contacted by survey to assess the quality of life. The mean follow-up after TFA was 4 years. RESULTS: Amputations were performed for a chronically infected TKA (n = 87, 78%) and an ischemic limb without signs of an infected TKA (n = 22, 20%). The 10-year survival after TFA was 21%. Of the patients not lost to follow-up, 53 (47%) patients were fitted for a prosthesis. Patients who underwent a TFA after the year 2000 were more likely to be fit for a prosthesis (odds ratio 7.27, P < .01); however, patients were likely to be ambulatory before TFA than after TFA (odds ratio 3.68, P < .01). After TFA, the mean 12-Item Short Form Survey scores for the mental and physical components were 54 ± 13 and 34 ± 7, with no difference in scores between patients fitted for a prosthesis and those who were not (P > .05). CONCLUSION: Patients undergoing a TFA after TKA due to failure of the TKA are more likely to be fit for a prosthesis; however, they reported no better quality of life and satisfaction compared with patients not fit for a prosthesis. LEVELS OF EVIDENCE: Level III, Therapeutic.

Humeral amputation following total elbow arthroplasty.

PURPOSE: Total elbow arthroplasty (TEA) is associated with a relatively high complication rate, and exceptionally catastrophic complications might lead to amputation. The purpose of this study was to determine the incidence and aetiology of amputation performed at our institution in upper extremity limbs with a prior TEA. METHODS: Between 1973 and 2018, 1906 consecutive TEAs were performed at our institution. Upper extremity amputation was performed in seven (0.36%) elbows with five transhumeral amputations and two shoulder disarticulations. The group consisted of five females and two males with a mean age of 64 years (range, 37-80). The index TEA had been performed for rheumatoid arthritis (n = 2), rheumatoid arthritis with acute fracture (n = 2), radiation associated nonunion (n = 2), and metastatic cancer (n = 1). Mean follow-up after amputation was three years (range, 3 months-5 years). RESULTS: Mean time between amputation and TEA was 5 years (range, 2 months-13 years). The indications for amputation included uncontrolled deep infection in six (86%) elbows and tumor recurrence in one (14%) elbow. Only one elbow (14%) was fitted with a prosthesis. Six (86%) patients died at a mean of three years (range, 3 months-5 years) after amputation. CONCLUSION: The results of this study highlight a low incidence of amputation after TEA. Most amputations were the direct result of TEA complications, with infection being the most common cause of amputation. Outcomes after amputation are concerning, with poor overall survival and few patients being fit for a prosthesis.

Extraskeletal myxoid chondrosarcoma: A high incidence of metastatic disease to lymph nodes.

BACKGROUND: Extraskeletal myxoid chondrosarcoma (ESMC) is a rare type of soft-tissue sarcoma with limited series reporting outcome of treatment. Currently there is limited data on the incidence and impact on patient outcome in those with metastatic disease to lymph nodes in ESMC. METHODS: Thirty (21 males, 9 females) patients, mean age 50 ± 16 years, with ESMC were reviewed. The tumors were most commonly located in the lower extremity (n = 23, 77%) and the mean tumor size and volume were 9 ± 5 cm and 490 ± 833 cm3 . Mean follow up was 7 ± 4 years. RESULTS: Six (20%) patients either presented (n = 3, 10%) or developed (n = 3, 10%) lymph node metastatic disease. When comparing patients without, with lymph node metastasis and metastasis elsewhere, patients with lymph nodes metastasis had worse survival than those without metastasis, however better 10-year disease specific survival than those with metastasis elsewhere (100% vs 62% vs 0%; P < .001). CONCLUSION: There is a high incidence of lymph node metastatic disease in patients with ESMC. Although survival in these patients is worse compared to those without metastasis, their survival is better than those with metastasis elsewhere. Due to the high incidence of lymph node metastatic disease, preoperative staging of the lymph node should be considered.

Outcomes of Surface Replacement Arthroplasty in Metacarpophalangeal Joints Affected by Noninflammatory Arthritis.

BACKGROUND: The purpose of this study was to investigate the outcomes of surface replacement arthroplasty (SRA) for noninflammatory arthritis of the metacarpophalangeal (MCP) joint. METHODS: Records from 20 SRAs performed between 1995 and 2017 in 17 patients with noninflammatory arthritis affecting the MCP joint were retrospectively reviewed. The mean follow up was 6.6 years. RESULTS: Three arthroplasties (15%) underwent 4 revision operations. The 2-, 5-, 10-, and 15-year rates of survival from surface replacement implant revision were 90%, 90%, 79%, and 79%, respectively. Major complications occurring in revised joints included arthrodesis (n = 1) and amputation (n = 1). The overall reoperation rate was 35%. The 2-, 5-, 10-, and 15-year rates of overall reoperation-free survival were 75%, 69%, 60%, and 60%, respectively. CONCLUSIONS: Pain ratings and MCP arc of motion significantly improved following arthroplasty. Metacarpophalangeal SRA for noninflammatory arthritis can improve arc of motion and pain. Revision is uncommon; however, 1 in 3 joints requires reoperation.

Outcomes of Pyrocarbon Arthroplasty in Metacarpophalangeal Joints Affected by Rheumatoid Arthritis.

BACKGROUND: Rheumatoid arthritis (RA) affecting the metacarpophalangeal (MCP) joint may warrant arthroplasty. The purpose of this study was to investigate implant survivorship, complications, radiographic outcomes, and clinical outcomes in patients undergoing MCP arthroplasty with a pyrocarbon implant to treat RA. METHODS: In all, 124 MCP joint pyrocarbon arthroplasties in 40 patients performed to treat RA were reviewed. Operations were at the index (n = 43, 35%), middle (n = 33, 27%), ring (n = 27, 22%), and small (n = 21, 17%) fingers from 1998 to 2009 in 105 (85%) female and 19 (15%) male joints with a mean age of 54 ± 11 years. Mean postoperative follow-up was 6 ± 3 years. All patients achieved at least 2 years of follow-up. RESULTS: Rates of implant survivorship at 1, 2, 5, and 10 years were 98%, 98%, 90%, and 81%, respectively. Fifteen percent (n = 18) of arthroplasties underwent revision at a mean 5 ± 3 years postoperatively. The overall reoperation rate was 29% (n = 36). Rates of survival from reoperation at 1, 2, 5, and 10 years were 85%, 84%, 76%, and 68%, respectively. Complications occurred in 32% (n = 40). Pain ratings improved postoperatively (P < .01). Arc of motion improved from 37 ± 21 to 43 ± 19 (P = .03). Both appositional and oppositional strength improved after surgery; however, there was no improvement in grip strength (P < .01). CONCLUSIONS: Metacarpophalangeal arthroplasty with a pyrocarbon implant demonstrated reliable improvement in pain and arc of motion in patients with RA. Complication and overall reoperation rates were high, while 1 in 10 undergo revision within 5 years postoperatively.

Incidence and clinical features of pediatric ocular trauma in a population-based cohort.

PURPOSE: To report the incidence and clinical characteristics of pediatric ocular and adnexal injuries diagnosed over a 10-year period in Olmsted County, Minnesota. METHODS: This multicenter retrospective, population-based cohort study included all patients <19 years of age in Olmsted County diagnosed with ocular or adnexal injuries from January 1, 2000, through December 31, 2009. RESULTS: A total of 740 ocular or adnexal injuries occurred during the study period, yielding an incidence of 203 (95% CI, 189-218) per 100,000 children. Median age at diagnosis was 10.0 years, and 462 (62.4%) were males. Injuries presented to the emergency department or urgent care setting most frequently (69.6%) and often occurred while outdoors (31.6%) during summer months (29.7%). Common injury mechanisms included blunt force (21.5%), foreign bodies (13.8%), and sports activities (13.0%). Isolated anterior segment injuries occurred in 63.5% of injuries. Ninety-nine patients (13.8%) had visual acuity of 20/40 or worse at initial examination, and 55 patients (7.7%) had visual acuity of 20/40 or worse at final examination. Twenty-nine injuries (3.9%) required surgical intervention. Significant risk factors for reduced visual acuity and/or the development of long-term complications include male sex, age ≥12 years, outdoor injuries, sport and firearm/projectile injury mechanism, and hyphema or posterior segment injury (P < 0.05). CONCLUSIONS: Most pediatric eye injuries are minor anterior segment injuries with infrequent long-lasting effects on visual development.

Long-term Outcomes of MCP Surface Replacement Arthroplasty in Patients With Rheumatoid Arthritis.

Background: Surface replacement arthroplasty (SRA) can be used in the treatment of rheumatoid arthritis (RA) affecting the metacarpophalangeal (MCP) joint. The authors of this study sought to investigate the outcomes of MCP SRA in patients with RA. Methods: Retrospective review of medical records and an institutional joint registry were used to gather data on 80 MCP SRAs performed in 27 patients with RA. Data collected included demographics, SRA revisions, reoperations, complications, pain, and MCP arc of motion. Results: The mean postoperative follow-up was 9.5 years (range, 2.1-20.5 years), with all SRAs achieving at least 2 years of follow-up. Thirteen digits (16%) underwent revision arthroplasty, and 29 (36%) required reoperation. The 5-, 10-, 15-, and 20-year rates of survival from implant revision were 95%, 85%, 80%, and 69%, respectively. The 5-, 10-, 15-, and 20-year rates of survival from overall reoperation were 80%, 65%, 55%, and 46%, respectively. Metacarpophalangeal joint arc of motion, grip strength, and pain levels significantly improved following surgery. Conclusions: Metacarpophalangeal SRA can offer benefit to patients with RA for improvement in function and pain. High overall reoperation rates remain concerning; however, most do not involve arthroplasty revision.

Prevalence and clinical characteristics of age-related distance esotropia.

Age-related distance esotropia (ARDET) is characterized by an esodeviation greater at distance than near in older aged patients and generally managed with prism spectacles or surgery. The purpose of this study is to describe the prevalence, clinical characteristics, and natural history of age-related distance esotropia in a defined population. The medical records of all adult (≥19 years of age) residents of Olmsted County, Minnesota, diagnosed with an esodeviation at least 2 prism diopters (PD) greater at distance than near, from 1 January 1985, through 31 December 2004, were retrospectively reviewed. Seventy-three (9.7%) of 751 new cases of adult-onset strabismus were diagnosed with age-related distance esotropia during the 20-year period. The mean age of onset was 70 years (range, 19 to 93 years) and 48 (65.8%) were female (p = .007). The mean angle of esodeviation was 7.6 (range, 2 to 20) prism diopters (PD) at distance and 0.4 (range, 10 PD of XT to 12 PD of ET) PD at near. The Kaplan-Meier rate of progression, as defined by a ≥ 6 prism diopter (PD) increase in esotropia, was 50% by 15 years after diagnosis. Half of the patients had hypertension, while one-third had coronary artery disease or other cardiac comorbidities. Age-related distance esotropia comprised 1 in 10 adults with new-onset strabismus in this population and was significantly more common among women. Hypertension and cardiovascular disease may be risk factors for this form of strabismus, and approximately half of the patients worsened over a 15-year period.

Utility of the Free Vascularized Fibula Flap to Reconstruct Oncologic Defects in the Upper Extremity.

BACKGROUND/AIM: Non-vascularized methods to reconstruct bone defects following tumor resection are associated with complications. As a result, the free vascularized fibular flap (FVF) has become a mainstay for reconstruction. The purpose of this study was to analyze FVF reconstruction in the upper extremity. PATIENTS AND METHODS: We reviewed 28 (14 female and 14 male, mean age of 29±20 years) patients reconstructed with an FVF in the upper extremity following tumor resection. Reconstruction most commonly involved the humerus (n=17, 61%) and a malignant (n=23, 82%) tumor. RESULTS: The limb salvage rate was 93% (n=26), with primary union occurring in 71% (n=20) of patients. Following bone grafting the overall union was 96% (n=27) at a mean 13±11 months. At most recent follow-up the mean MSTS93 rating was 82±22%. CONCLUSION: FVF effectively provided a functional reconstruction following tumor resection. Although, one in four patients will need additional bone grafting, the overall union rate is high.

Utility of free vascularized fibular flaps to treat radiation-associated nonunions in the upper extremity.

BACKGROUND: Nonunion is a common complication following a fracture in the setting of previous radiotherapy; however, currently there is a paucity of data describing treatment for these nonunions in the upper extremity. Free vascularized fibular (FVF) flaps successfully treat bone defects; however, their efficacy with respect to treatment of radiated nonunions is limited. The purpose of the study was to assess the outcome following FVF for radiation-associated upper extremity nonunions. METHODS: Seven patients underwent FVF for the treatment of radiation-associated upper extremity nonunion between 1998 and 2016. RESULTS: There were 5 male and 2 female patients, with a mean age and follow-up of 44 years and 4 years, respectively. Mean total radiation dose was 41.3 Gy, given at a mean of 11 years prior to FVF. The average FVF length was 15 cm. First time union rate was 71%, however, following repeat bone grafting all patients healed. The median time to union was 10 months. Musculoskeletal Tumor Society scores improved from 57% preoperatively to 89% at latest follow-up (p < 0.0001). CONCLUSIONS: FVF is a reliable treatment option for radiation-associated nonunions of the upper extremity, providing an overall union rate of 100% and an improvement in functional outcome.

Hip Arthroplasty Following Subtotal Sacrectomy for Chordoma.

BACKGROUND/AIM: Chordomas often affect the sacrum with a high predilection for local-regional recurrence. Patients typically retain their ability to ambulate, and the development of metastatic disease in the periacetabular region can have significant morbidity and pain with ambulation. The purpose of the study was to describe the outcome of patients undergoing a hip arthroplasty following resection of a sacral chordoma. PATIENTS AND METHODS: From 1990 to 2015, 84 patients underwent sacrectomy for chordoma, while four of these (5%) patients underwent hip arthroplasty. The most common level of nerve root sacrifice was S2-5 (n=2). The mean time between sacrectomy and hip arthroplasty was 7 years. Indications for arthroplasty included metastatic disease (n=3) and coxarthrosis (n=1). RESULTS: Postoperatively two patients ambulated with a gait aid, and no patient had a Trendelenburg gait. The mean Harris Hip Score significantly improved from 49 to 80 postoperatively (p=0.02). CONCLUSION: The results of this study indicate that hip arthroplasty is a durable treatment option for patients with metastatic disease or coxarthrosis following subtotal sacrectomy for chordoma.