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Chiari malformation (CM) is a common neurological disorder with foramen magnum decompression (FMD) as a commonly accepted treatment. The authors present a case of CM-1 wherein there was no radiological instability preoperatively and FMD was done as a treatment, after which the patient improved transiently only to deteriorate further. Atlantoaxial fixation was done as a second-stage procedure, after which the patient improved clinically and radiologically. The knowledge of this case and surgical entity should be borne in mind before the formulation of a treatment plan. It is important that the solution is to identify and treat the underlying pathology rather than to decompress and directly manipulate the tonsils.
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Endometriosis is a chronic inflammatory condition affecting nearly 10-15% of women of reproductive age and most frequently occurs in the pelvis. Extrapelvic endometriosis leading to sacral neuropathy is rare. A 29-year old woman, para 1 (vaginal delivery 8 years previously), presented with worsening right hip and buttock pain radiating to the right leg, resulting in limping and other gait changes due to delayed diagnosis and treatment. A diagnosis of sciatic nerve endometriosis was made based on both clinical and imaging findings. The final diagnosis was confirmed on histopathological examination. Transgluteal excision of the sciatic nerve with concomitant laparoscopy for exploration of the pelvic part of the sciatic nerve and excision of the pelvic endometriosis were undertaken. The operating team included a gynaecologist, an orthopaedic surgeon and a neurosurgeon. Postoperative recovery was uneventful and physiotherapy was started. Pain and gait improved. Endometriosis of the sciatic nerve should be suspected if a patient complains of cyclical sciatica. Magnetic resonance remains the imaging modality of choice. This case report illustrates the benefit of a multidisciplinary approach.
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PURPOSE: To study etiology, clinical presentation, anatomical, and functional outcomes of patients with giant retinal tear detachment at a tertiary eye institute. METHODS: Retrospective, consecutive case series of 396 patients (396 eyes) who underwent surgery were reviewed. Factors affecting the final anatomical and functional success were determined. RESULTS: Mean age was 37 years (range; 1-79 years), and 86% (n = 339) of the subjects were men. Trauma (21%) and high myopia (11%) were predisposing risk factors. Two hundred and seventy-seven eyes (70%) had giant retinal tear configuration of >180° and <270°, associated with partial retinal detachment in 282 (71%) eyes and macular detachment in 262 (66%) eyes. Primary surgery included pars plana vitrectomy (n = 240, 61%), pars plana vitrectomy with encirclage band (n = 152, 38%), or scleral buckle (n = 4, 1%). The mean follow-up duration was 15 months (median, 8.4 months; range, 3-83 months). Anatomical success after initial surgery was 64% (255 eyes), which improved to 78% (308 eyes) after undergoing a second vitreoretinal procedure for recurrent retinal detachment (53 eyes). Median visual acuity improved from 20/1,500 preoperatively to 20/400 at final follow-up ( P = 0.01), and 15% of eyes achieved postoperative visual acuity of 20/60 or better. Factors associated with poor anatomical success included age <16 years ( P = 0.005) and presenting visual acuity 20/400 or less ( P = 0.001). CONCLUSION: Trauma and myopia constituted the major risk factors for giant retinal tear detachment in our series. Surgery for giant retinal tear detachment managed with pars plana vitrectomy with or without encirclage band and silicone oil tamponade had good anatomical and favorable visual outcomes at last follow-up.
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Miopía , Desprendimiento de Retina , Perforaciones de la Retina , Masculino , Humanos , Adulto , Adolescente , Femenino , Perforaciones de la Retina/diagnóstico , Perforaciones de la Retina/etiología , Perforaciones de la Retina/cirugía , Desprendimiento de Retina/diagnóstico , Desprendimiento de Retina/etiología , Desprendimiento de Retina/cirugía , Estudios Retrospectivos , Curvatura de la Esclerótica/efectos adversos , Resultado del Tratamiento , Vitrectomía/métodos , Miopía/complicacionesRESUMEN
OBJECTIVE: Treatment of chondrosarcomas is grade based; intralesional curettage for grade 1 and resection for grade 2 or more. Currently used methods to determine grades before surgery are not highly accurate and create a dilemma for the surgeon. We have used a PET-CT combined with imaging to answer the following study questions: (1) Does SUVmax value from an 18F-FDG PET/CT correlate with the grade of chondrosarcoma? (2) Can a cutoff SUVmax value be used to differentiate between various grades of chondroid neoplasms with sufficient sensitivity and specificity? (3) Does SUVmax guide the clinician and add value to radiology in offering histologic grade-dependent management? METHODS: SUVmax values of patients with suspected chondrosarcoma were retrospectively correlated with the final histology grade for the operated patients. Radiologic parameters and radiology aggressiveness scores (RAS) were reevaluated and tabulated. RESULTS: Totally 104 patients with chondroid tumors underwent 18F-FDG PET/CT assessment. In total 73 had tissue diagnosis available as a pretreatment investigation. Spearman correlation indicated that there was a significant positive association between SUVmax and the final histology grading of chondroid tumors (correlation coefficient = 0.743; P < 0.01). SUVmax cutoff of 13.3 was 88.9% sensitive and 100% specific for diagnosing dedifferentiated chondrosarcomas. An RAS cutoff value of 3 or more could diagnose IHGCS with a sensitivity of 80.7% and specificity of 93.75%. Adding an SUVmax cutoff of 3.6 improves the sensitivity to 89.5%. CONCLUSION: SUVmax value can reliably help diagnose dedifferentiated chondrosarcoma and when added to the radiology score can improve the accuracy of grading chondrosarcoma.
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Tomografía Computarizada por Tomografía de Emisión de PositronesRESUMEN
Pseudomyogenic hemangioendothelioma (PHE) is a soft-tissue tumor of intermediate malignant potential, recognized as a separate entity in the WHO (World Health Organization) classification of tumors of soft tissue and bone, in 2013. This is a case report of a 33-year-old man with intraosseous scapular PHE reported on small biopsy and immunohistochemistry. The patient presented with local recurrence and metastases even after wide local excision and adjuvant radiotherapy after 14 months. The rarity of this lesion at this site and morphologic clues to diagnosis are important for optimizing the treatment protocol.
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Hemangioendotelioma/diagnóstico , Escápula/patología , Adulto , Biopsia , Humanos , MasculinoRESUMEN
INTRODUCTION: Traditionally, centralization of the fibula with fusion across the tibiotalar joint has been used to reconstruct distal tibial defects. Although effective, it requires long periods of protected weight-bearing. The fibula or the fixation often fails before fibular hypertrophy necessitating multiple additional surgeries. A method of using ECRT with the available ipsilateral fibula (nonvascularized) to reconstruct the distal tibia defect with the aim of early return to weight-bearing was evolved. This paper documents our early experience. Patients and Methods. Four patients; with the diagnosis of osteosarcoma in 3 patients and recurrent giant cell tumor of the bone in 1 patient, underwent resection of the distal tibia for tumors between 2017 and 2019. Extracorporeally irradiated (50 Gy) distal tibia along with ipsilateral nonvascularized fibula was used to bridge the defect and fuse the tibiotalar joint. A plate was used to rigidly hold the construct. The final outcome was compared to the historical control group that underwent only pedicled ipsilateral fibula transposition and ankle arthrodesis without recycled autograft or allograft between 2009 and 2017. Oncological reconstruction and functional outcomes were compared for each group. Patient reported outcomes on the acceptability of ankle fusion; cosmesis and function were analyzed and compared between the two groups. RESULTS: The mean resection length in the study group (4 patients) was 7.75 cm (7 to 8.5 cm). As compared to the historical cohort of 7 patients, the study population showed statistically superior results in all reconstruction, functional, and patient-reported outcomes except time to proximal junction union (p=0.068). There were no reconstruction failures, infection, or nonunions in the study group, whereas the control comparative group had 2 proximal junction nonunions and a mean time to fibular hypertrophy of 143 weeks (82 to 430 weeks) with fibula centralization. Earlier weight-bearing was allowed (mean 26.75 weeks; median 27 weeks) compared to (mean 80.75 weeks; median 80 weeks) in the control group. CONCLUSION: We think that ECRT with ipsilateral vascularized fibula is a promising method of reconstructing the distal tibia. The recycled autograft tibia added strength to the distal tibia construct in our study and aided the anatomical reconstruction of the distal tibia. The patient-reported outcomes for cosmesis and acceptability add to the benefits of performing this procedure. Consistent early union across the proximal junction and earlier weight-bearing were clear advantages of this method.
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PURPOSE: The aim of this study was to report on the impact of COVID-19 "Unlock-I" on Network of Eye Centers in Southern India. METHODS: Our eye health pyramid model has a network of eye care centers in four Indian states. The network constitutes a center of excellence (CoE) at the apex followed by tertiary care centers (TC) located in urban areas, secondary care centers (SC), and primary care vision centers (VC) at the base located in rural areas. We collected data on patients seen between June 2019 and June 2020, which included age, gender, total patients seen (new or follow-up), and socioeconomic status (paying and nonpaying). A comparative study was done between the data for outpatients and surgeries performed pre-COVID-19 and during Unlock-I in COVID-19 period. RESULTS: There was a 36.71% reduction in the overall outpatients seen in June 2020 (n = 83,161) compared to June 2019 (n = 131,395). The reduction was variable across different levels of the pyramid with the highest reduction in CoE (54.18%), followed by TCs (40.37%), SCs (30.49%) and VCs (18.85%). Similar pattern was seen for new paying patients with the highest reduction in CoE (54.22%), followed by TCs (25.86%) and SCs (4.9%). A 43.67% reduction was noted in the surgeries performed in June 2020 (n = 6,168), compared to June 2019 (n = 10,950). Reduction in paying services was highest in CoE (47.52%), followed by TCs (15.17%) and SCs (4.87%). There was no significant change in the uptake of services by gender in the network. CONCLUSION: Highest reduction in patient footfalls during "Unlock-1" was noted in urban centers. Going forward, there is a need to develop strategies to provide eye care closer to the doorsteps.
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COVID-19/epidemiología , Transmisión de Enfermedad Infecciosa/prevención & control , Pandemias , SARS-CoV-2 , Centros de Atención Terciaria/estadística & datos numéricos , Adulto , COVID-19/transmisión , Femenino , Humanos , India/epidemiología , Masculino , Cuarentena/organización & administraciónRESUMEN
BACKGROUND: Ten years ago, we reported the results of a procedure in which we translocated the ipsilateral ulna as a vascularized autograft to reconstruct defects of the distal radius after tumor resection, with excellent functional results. At that time, wrist arthrodesis was achieved by aligning the translocated ulna with the scapholunate area of the carpus and usually the third metacarpal. This resulted in wrist narrowing. We then wondered if aligning the translocated ulna with the scaphoid and the second metacarpal would result in ulnar deviation and thereby improve grip strength. We believed lateralization would reduce the wrist narrowing that occurs with fusion to the third metacarpal and would make the cosmesis more acceptable. We also modified the incision to dororadial to make the scar less visible and thus improve the cosmesis. QUESTIONS/PURPOSES: (1) Is there an objective improvement in grip strength and functional scores (Musculoskeletal Tumor Society [MSTS] and Mayo wrist) when the translocated ulna is lateralized and the wrist is fused with the translocated ulna and aligned with the second metacarpal versus when the translocated ulna is aligned with the third metacarpal? (2) Did lateralization caused by the wrist fusion aligned with the second metacarpal minimize wrist narrowing as measured by the circumference compared with the fusion aligned with the third metacarpal? METHODS: From 2010 and 2018, we treated 40 patients with distal radius tumors at our institution, 30 of whom had a distal radius enbloc resection. Twenty-eight patients had an ipsilateral ulna translocation and wrist arthrodesis in which the radius and translocated ulna were aligned with either the second (n = 15) or the third (n = 13) metacarpals. Two patients in the second metacarpal group and three patients in the third metacarpal group were lost to follow-up before 24 months after surgery and were excluded. A retrospective analysis of 23 patients (20 with giant cell tumors and three with malignant bone tumors) included a review of radiographs and institutional tumor database for surgical and follow-up records to study oncologic (local disease recurrence), reconstruction (union of osteotomy junctions, implant breakage or graft fracture, and wrist circumference), and functional outcomes (MSTS and Mayo wrist scores and objective grip strength assessment compared with the contralateral side). The results were compared for each study group (second metacarpal versus third metacarpal). There was no difference in the incidence of local recurrence or the time to union between the two groups. There were no implant breakages or graft fractures noted in either group. RESULTS: Patients in the second metacarpal group lost less grip strength compared with the unoperated side in the third metacarpal group (median 12% [range -30% to 35%] versus median 28% [15% to 42%], difference of medians 16%; p = 0.006). There were no between-group differences in terms of MSTS (median 30 [24 to 30] versus median 26.5 [22 to 30], difference of medians 3.5; p = 0.21) or Mayo wrist scores (median 83 [65 to 100] versus median 72 [50 to 90], difference of medians 11; p = 0.10). The second metacarpal group also had less wrist narrowing as seen from the median difference in circumference between the operated and unoperated wrists (median narrowing 10 mm [3 to 35 mm] in the second metacarpal group versus median 30 mm [15 to 35 mm] in the third metacarpal group, difference of medians 20 mm; p = 0.04). CONCLUSION: Wrist arthrodesis after ulna translocation with alignment of the translocated ulna and the second metacarpal provides a functional position with ulnar deviation that offers some improvement in grip strength but no improvement in the MSTS or Mayo scores. Radialization/lateralization of the translocated ulna achieved from the alignment with the second metacarpal decreases the reduction in the wrist circumference and therefore reduces wrist narrowing. LEVEL OF EVIDENCE: Level III, therapeutic study.
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Artrodesis/métodos , Neoplasias Óseas/cirugía , Tumor Óseo de Células Gigantes/cirugía , Osteotomía/métodos , Cúbito/trasplante , Muñeca/cirugía , Neoplasias Óseas/fisiopatología , Trasplante Óseo , Femenino , Tumor Óseo de Células Gigantes/fisiopatología , Fuerza de la Mano , Humanos , Masculino , Radio (Anatomía)/cirugía , Rango del Movimiento Articular , Estudios Retrospectivos , Resultado del Tratamiento , Muñeca/fisiopatologíaRESUMEN
Purpose: The objective of this study was to identify the diagnostic features of optic nerve head melanocytoma (ONH-MCT) on spectral domain optical coherence tomography (SD OCT) and OCT angiography (OCT-A). Methods: Retrospective study of 11 patients for their demographic, clinical features and imaging including SD OCT (tumour location, extent and interface) and OCT-A (surface and intrinsic vascularity) were reviewed. Flow rate percentage (FR %) was calculated over the lesion and compared to fellow eye and similar pigmented lesions. Results: The average age was 52.8 ± 10.9 years. ONH-MCT tumors occupied 3-tissue spaces- optic disc (n = 2), retinal layer (n = 5) and retina-choroidal layers (n = 4). SD OCT (11 eyes) showed elevated hyper reflective disorganized retinal layers with posterior shadowing (9 eyes) and hyper reflective dots within the tumor (all eyes). Microvascular features on OCT-A (8 eyes) in radial peripapillary capillary slab showed surface vascularization (7 eyes) and intrinsic vascularity in choroidal slab (8 eyes) with surrounding hypo reflective boundary. The mean FR % was higher at 65.1 ± 3.77% (CI: 61.9-68.2) compared to mean FR at 60.4 ± 1.06% (CI: 59.5-61.2) in the fellow eye (p = 0.01). Comparison with nevus and melanoma SD OCT showed a high reflective choroidal layer with normal or irregular outer retinal layers respectively; OCT-A showed hypo reflective area at the center with hyper reflective boundary and iso reflective area at center with hyper reflective boundary respectively. Conclusion: SD OCT and OCT-A features may help to differentiate ONH-MCT from clinically similar looking pigmented lesions like nevus and melanoma.
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Disco Óptico , Neoplasias Cutáneas , Adulto , Coroides , Humanos , Persona de Mediana Edad , Estudios Retrospectivos , Tomografía de Coherencia ÓpticaRESUMEN
BACKGROUND: Megaprosthetic infections continue to be a leading mode of failure after limb salvage surgery. Though challenging, amputations can be avoided with proper management in majority of the cases. This study aims to describe the spectrum of mega-endoprosthetic infections at our institute and assess the treatment efficacy in these patients. MATERIALS AND METHODS: All patients treated for endoprosthetic infection at our institute between 2010 and 2018 were retrospectively analyzed for overall survival of reconstruction method, site and type of megaprosthesis, adjuvant therapy, microbial isolates, surgical and medical management and outcomes. RESULTS: Thirty-five patients (22 males: 13 females) were analyzed following treatment for endoprosthetic infection. Majority were around the knee joint [most commonly with proximal tibia (n = 14) followed by distal femur (n = 12) megaprosthesis]. Ten patients had undergone primary surgical procedure at our institute, while 25 patients presented with infection after megaprosthesis implantation. In the 28 culture-positive infections, the most common micro-organism was Staphylococcus spp. (18 patients: methicillin-sensitive Staphylococcus aureus = 9, coagulase-negative Staphylococcus = 5, methicillin-resistant Staphylococcus aureus = 1, Staphylococcus epidermidis = 3) and poly-microbial infection was present in three patients. Nine patients underwent successful debridement and wound wash with insertion of antibiotic impregnated cement beads in 5/9 cases. Twenty-one patients required a two-stage revision. Of these 30 patients, all but one has completely resolved infections. One patient with resurfaced late infection after re-implantation is on chronic suppressive antimicrobial therapy and close follow-up. Amputation because of uncontrolled infection was performed in three patients (one death post-operatively due to systemic complications of septicemia), while two patients opted for amputation as opposed to stage revisions. Median antimicrobial therapy duration was 6 weeks (1-12 weeks). Reconstructive surgery for soft tissue cover was required in seven patients. CONCLUSIONS: In patients with early or acute presentation without frank granulation or pus around the implant, debridement and insertion of antibiotic cement beads was adequate. Two-stage revisions with complete removal of the megaprosthesis showed best results in infections that could be controlled with antimicrobial therapy. More than one exchange of cement spacer was required for uncontrolled infections. Multidisciplinary approach in consultation with the infectious disease team is essential to determine choice of antibiotic cement for beads/spacer as well as appropriate adjuvant antimicrobial therapy to solve the challenging problem of endoprosthetic infections following bone tumor surgery. Adequate and healthy soft tissue cover of the implant should be achieved wherever indicated.
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AIM: To highlight radiological features and emphasize the need for tissue diagnoses to confirm bone pathology. Tuberculosis is known to present without constitutional symptoms and with unconventional imaging features mimicking sarcomas as shown in our series of 25 patients; where the imaging and biopsy protocols at our institute helped to solve these diagnostic dilemmas. MATERIAL AND METHODS: We retrospectively analyzed clinical and radiological features and tissue diagnoses in 25 patients referred to the department of orthopedic oncology with radiological suspicion of tumor. RESULTS: Only 7 patients had cultures positive for Mycobacterium Tuberculosis T.B. Radiological features suggestive of infection were Metaphyseal and joint involvement, permeative lesions, absence of Codman's triangle, and soft tissue mass suggestive of a cold abscess. The predictive accuracy of the orthopedic oncologist was 60% and musculoskeletal radiologist was 72% (based on radiology). Final diagnosis is 100% confirmed on histopathology. CONCLUSION: Diagnosis based primarily upon imaging is a wrong approach. A multimodal approach to differentiating tuberculous bone infections from sarcomas is essential.
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Purpose: To study optical coherence tomography (OCT) and optical coherence tomography angiography (OCT-A) features of circumscribed choroidal hemangioma (CCH) following treatment with photodynamic therapy (PDT) and transpupillary thermotherapy (TTT). Methods: A retrospective chart review of consecutive patients treated for CCH over 2 years (May 2016-April 2018). The investigations, in addition to comprehensive eye examination, included color fundus photography, B-scan ultrasonography, OCT, and OCT-A. Results: The study included 16 eyes of 16 patients (9 males and 7 females). The mean age at presentation was 43.5 ± 9 years (range 33-62 years). Macula (n = 6) and superior arcade (n = 5) were the common tumor locations. Twelve eyes received multiple treatment sessions: TTT (seven eyes; mean 2.4 sessions) and PDT (five eyes; mean 2 sessions). Four eyes were observed because vision was not threatened. Pretreatment OCT features were Bruch's membrane atrophy (15 eyes), retinal pigment epithelial atrophy (13 eyes), outer retinal abnormalities (12 eyes), and macular subretinal fluid (12 eyes). Pretreatment OCT-A features were complete loss of choriocapillaris (16 eyes), irregularly arranged fine arborizing vessels (11 eyes), and more than 50% signal void hyporeflective areas (12 eyes). Posttreatment OCT-A showed persistence of choriocapillaris loss, flat scar with fibrosis and thinning of choroid in all eyes treated with TTT, and persistence of deeper choroidal vessels and no loss of choriocapillaris in eyes treated with PDT. Conclusion: OCT and OCT-A help understand the structural outcome following PDT and TTT in circumscribed choroidal hemangioma.
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Neoplasias de la Coroides/diagnóstico , Angiografía con Fluoresceína , Hemangioma/diagnóstico , Hipertermia Inducida , Fotoquimioterapia , Tomografía de Coherencia Óptica , Adulto , Neoplasias de la Coroides/terapia , Técnicas de Diagnóstico Oftalmológico , Femenino , Hemangioma/terapia , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Agudeza VisualRESUMEN
Primary tumors of the sacrum are difficult to manage, as they often require morbid resections and complex reconstructions. In the case of tumors such as chordoma or chondrosarcoma, aggressive resections are often required to achieve appropriate margins (extending disease-free survival), followed by complex reconstructions. These reconstructions are aimed at restoring the pelvic ring and have traditionally resulted in a lumbosacral construct that utilizes structural allograft/autograft bone (fibula most commonly used) and more recently, reconstruction with 3D-printed custom sacral prostheses. While there are no reports of anatomical reconstruction using sacral allografts, extracorporeal radiation therapy (ECRT) and reimplantation provides a size and shape-matched irradiated autograft which avoids the cultural stigma, structural strength and graft-host concerns associated with allografts, as well as the high costs and time to production associated with custom 3D-printed implants. Here we present an illustrative case with technical notes, outlining the steps used at our center for ECRT. While early results with ECRT in the sacrum are promising, future larger studies should be carried out to help detect differences that may exist in long-term complications.
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INTRODUCTION: Limb salvage surgery following proximal ulna resection poses a challenge in reconstruction of the complex elbow anatomy. Various reconstruction methods described offer inadequate restoration of function and stability. Following resection of proximal ulna tumors, we aimed to restore the joint using the resected osteochondral segment of proximal ulna treated with extracorporeal irradiation and reimplantation. QUESTIONS/PURPOSES: (1) Does irradiated osteoarticular autograft reconstruction for proximal ulna allow anatomical joint restoration and what are the oncological and functional outcomes? (2) Is there evidence of graft-related complications or osteoarthritis at a minimum of 2 years follow-up with irradiated osteoarticular autografts for the proximal ulna? (3) How does our method of reconstruction fare as compared to reported reconstruction options in the literature? Materials and Methods. 3 patients with primary bone tumors involving the proximal ulna underwent limb salvage surgery with en bloc resection and reconstruction using the resected bone after treating it with extracorporeal irradiation of 50 Gy. Minimum follow-up of 2 years was considered for assessment of final outcomes. Radiographs were assessed for bony recurrence, union across osteotomy junction, and signs of joint arthritis. Functional outcome measures included range of movement, muscle power testing, and functional and disability scores. RESULTS: 2 complete and 1 partial olecranon involving proximal ulnar resections were performed for three cases of Ewing's sarcoma in 2 males and 1 female. Follow-up ranged from 28 to 42 months, and all patients continue to remain disease free. All 3 patients have achieved full range of flexion-extension and pronosupination movement in comparison to the contralateral side. Muscle power for flexion and extension was 5/5. MSTS score: 100% (30/30); MEPS score: 100; and DASH score: zero were achieved for all patients. Union across osteotomy junctions at median follow-up of 8 months without need for intervention/bone grafting was achieved. No complications related to joint laxity/subluxation, infection, graft fracture, or implant failure was noted. None of the patients have clinical or radiological signs of joint arthritis across the irradiated articulation. CONCLUSION: Biological restoration of elbow anatomy using osteoarticular irradiated graft for proximal ulna reconstruction offers great joint stability and functional outcomes. Although the potential risks of infection and graft failure need to be considered, reconstruction with the size-matched radiated autograft eliminates donor site morbidity, offers a low-cost alternative to endoprosthesis, and provides outcomes superior to any other methods of reconstruction as analyzed from the literature.
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INTRODUCTION: The major neoplastic and proliferative component of GCTB is the stromal tumor cells; that they have shown no evidence of bone destruction, instead the massive tissue destruction appears to be a result of tumor induced osteoclastogenesis. The discovery of receptor activator of nuclear factor kB (RANK) and RANK binding ligand (RANKL) uncovered the bone homeostasis and molecular mechanism by which multiple compounds (including vitamin D) regulated osteoclast differentiation; a function mediated by osteoblastic cells and osteoclast-precursor cells. HYPOTHESIS: In a country burdened by vitamin D deficiency, causal relation between hypovitaminosis D and GCTB was hypothesized based on the vitamin D mediated RANKL expression and osteoclastogenesis, as India is also a population with higher incidence of GCTB as compared to Western populations described in the literature. The possibility of vitamin D regulated osteoclastogenesis in GCTB is postulated on the evidence from molecular research linking it to the RANK/RANKL/OPG pathway. The aim of this study was to analyse the prevalence of Vitamin D deficiency in patients with primary GCTB and to elucidate any difference in serum Vitamin 25(OD)D3 levels amongst the matched control population data. MATERIALS AND RESULTS: 130 patients of primary GCTBs were matched to 310 controls from the general health check population and serum levels of 25(OH)D3 were analyzed. Statistical analysis performed on the non-parametric data and Mann Whitney U Test used to derive inference with significance set at pâ¯<â¯0.05. 56 females and 76 males with median Vitamin D level in the GCTB group was 15.9â¯ng/ml (Mean 19.41; Range 1.03 to 92) as compared to the control population with median level of 22.2â¯ng/ml (Mean 25.1; Range 2.6 to 87.9). The results were significant (p valueâ¯<â¯0.05) as compared to the control population in all decades except the third decade (p value 0.0548). DISCUSSION: The differential expression of RANKL and OPG in response to levels of vitamin D has been established. The stromal cells of osteolytic GCTB express high levels of RANKL, which is a key signal regulator in development of this disease and bone destruction typical of GCTBs. This has resulted in research targeting this pathway for therapeutic approach in GCTBs. As vitamin D supplementation is simple and safe, increased awareness to assess and if necessary correct vitamin D status of patients is warranted, however the question as to whether patients with low vitamin D levels are more prone to develop GCTB and thus would profit from vitamin D supplementation remains unanswered. To conclude, it is essential to assess vitamin D levels in patients with GCTB as deficiency is pronounced. Future research on this hypothesis might lead to an association between Vitamin D deficiency and the onset/natural history of GCTB that may in the future help us cure or prevent GCTBs.
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Tumores de Células Gigantes/etnología , Tumores de Células Gigantes/etiología , Ligando RANK/metabolismo , Deficiencia de Vitamina D/sangre , Deficiencia de Vitamina D/complicaciones , Adolescente , Adulto , Anciano , Femenino , Homeostasis , Humanos , Incidencia , India , Masculino , Persona de Mediana Edad , Modelos Teóricos , Osteoclastos/metabolismo , Receptor Activador del Factor Nuclear kappa-B/metabolismo , Vitamina D/metabolismo , Adulto JovenRESUMEN
INTRODUCTION: Bizarre parosteal osteochondromatous proliferationis a distinct entity which requires proper radiological and pathological distinction for correct surgical management. CASE REPORT: We present a series of four cases which were misdiagnosed by the treating surgeons, reporting radiologists and pathologists due to clinical and investigatory consistencies with other benign or malignant orthopedic tumors. Due to common diagnostic errors of these uncommon tumors, the patients had recurrence and required multiple invasive procedures which could have been avoided with high index of suspicion. CONCLUSION: Dueto high local recurrence rates and a lack of adjuvant therapy options, this lesion will continue to pose a challenge for orthopedic surgeons and more awareness ofthis lesion will help identify and understand that a wide excision, with no compromise related to the margins, is required for this benign-appearing lesion.
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BACKGROUND: Pelvic resections are challenging, and reconstruction of the resected acetabulum to restore mobility and stability is even more difficult. Extracorporeal radiation therapy (ECRT or extracorporeal irradiation) of autograft bone and reimplantation allows for a perfect size match and has been used with some success in the extremities. Although the risk of wound complications in pelvic surgery has discouraged surgeons from using ECRT of autografts in that anatomic site, we believe it may be a reasonable option. QUESTIONS/PURPOSES: In a small series, we asked: (1) What was the median surgical time and blood loss for these procedures, and what early complications were observed? (2) Is there evidence of osteonecrosis or cartilage loss at a minimum of 2 years after ECRT of acetabular autografts, and what functional scores were achieved? (3) What were the oncologic outcomes after ECRT? METHODS: Between March 2007 and September 2016, one surgeon performed 12 ECRT acetabular autografts and reimplantations after resections of pelvic or acetabular tumors. Of those, 10 with minimum 2-year followup are reported on here with respect to oncologic, functional, and radiographic assessment; all 12 are reported on for purposes of surgical parameters and early complications. During that period, we generally performed this approach when we judged it possible to achieve a tumor-free margin, adequate bone stock, and sufficient remaining hip musculature to allow use of the bone as an autograft with restoration of hip mobility. We generally did not use this approach when we anticipated a difficult resection with uncertain margins or where remaining bone was judged of poor strength for use as a graft or if both iliopsoas and abductors were sacrificed. Since 2010, this series represents seven of the 21 pelvic resections with reconstruction that we performed (five patients in this series had the procedure performed before 2010). Followup was at a median of 65 months (range, 33-114 months) for nine patients whose functional outcomes were evaluated. The median patient age was 30 years (range, 10-64 years). Clinical parameters were recorded from chart review; radiographic analysis for assessment of cartilage was performed by looking for any obvious loss of joint space when compared with the opposite side. Functional scoring was done using the Musculoskeletal Tumor Society score, which was obtained from chart review. Oncologic assessment was determined for local recurrence as well as metastases. RESULTS: Median surgical time was 8.6 hours and median blood loss was 2250 mL. There were no perioperative wound-related complications. Two patients underwent a second surgical procedure during the postoperative period, one for a femoral artery thrombus and another for a complete sciatic nerve deficit. No patients developed avascular necrosis of the femoral head. None of the patients who underwent osteoarticular grafting showed radiographic evidence of joint space narrowing. The median Musculoskeletal Tumor Society score was 28 (range, 17-30). No fractures in the radiated segment of reimplanted bone were seen in this small series. CONCLUSIONS: Results from this small series suggest that ECRT is a potential option in selected patients who have good bone stock and adequate soft tissue coverage. Although technically challenging, ECRT is a low-cost alternative to prostheses in providing a mobile and stable hip. Although we did not observe cartilage wear on plain radiographs, followup here was short term; it may appear as we continue to follow these patients. Future studies from retrieval specimens may shed light on the actual status of cartilage on the acetabulum. LEVEL OF EVIDENCE: Level IV, therapeutic study.
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Acetábulo/efectos de la radiación , Acetábulo/cirugía , Trasplante Óseo/métodos , Articulación de la Cadera/efectos de la radiación , Articulación de la Cadera/cirugía , Osteotomía , Neoplasias Pélvicas/terapia , Reimplantación , Acetábulo/diagnóstico por imagen , Acetábulo/fisiopatología , Adolescente , Adulto , Fenómenos Biomecánicos , Pérdida de Sangre Quirúrgica , Trasplante Óseo/efectos adversos , Niño , Femenino , Articulación de la Cadera/diagnóstico por imagen , Articulación de la Cadera/fisiopatología , Humanos , Masculino , Persona de Mediana Edad , Tempo Operativo , Osteotomía/efectos adversos , Neoplasias Pélvicas/diagnóstico por imagen , Neoplasias Pélvicas/fisiopatología , Complicaciones Posoperatorias/etiología , Datos Preliminares , Radioterapia Adyuvante , Rango del Movimiento Articular , Recuperación de la Función , Reimplantación/efectos adversos , Estudios Retrospectivos , Factores de Riesgo , Factores de Tiempo , Tomografía Computarizada por Rayos X , Trasplante Autólogo , Resultado del TratamientoRESUMEN
BACKGROUND: Although giant cell tumors (GCTs) are benign, their aggressiveness and tendency to recur locally challenge the orthopaedic surgeon's ability to perform joint-preserving intralesional surgery with an acceptably low risk of local recurrence. Denosumab has emerged as a possible medical treatment of GCT because it seems to halt the progression of GCT, alleviate pain, and increase perilesional bone formation, but its exact role has been questioned, and specifically its efficacy and associated complications are not well characterized. QUESTIONS/PURPOSES: (1) Does denosumab reduce the risk of recurrence after resection or intralesional surgery? (2) What are the complications associated with the use of denosumab? METHODS: Fifty-four patients with 30 primary and 25 recurrent tumors between November 2013 and July 2016 were treated with denosumab after a confirmed histopathologic diagnosis of GCT. Another 17 patients in the same period were treated without denosumab. During the study period, we encouraged the use of denosumab in all patients except those who refused, could not afford it, or where it was contraindicated (eg, in pregnancy). In all patients undergoing intralesional surgery, we arbitrarily planned six doses before surgery. Variations in total doses before surgery were dependent on patient-related factors; in some, we gave less doses because patients expressed the inability to afford any more doses, whereas in some patients, extra doses were added when the patient wished to delay surgery as well as the because of surgeon judgment wherein in some patients, we stopped before six doses when we thought adequate bone had formed for intralesional curettage. The mean number of doses was 6.8 per patient (median, 6; range, 3-17) preoperatively. The minimum followup was 12 months (median, 27 months; range, 12-42 months). Every patient showed improvement clinically in terms of pain and halting of tumor progression within three to four doses. This was seen radiologically as a sharply defined soft tissue mass as well as hazy ossification within the tumor. For a case-matched comparison study, we identified controls as 34 patients undergoing curettage from the retrospective analysis of 68 patients curetted without denosumab between February 2010 and July 2016 matched to 25 denosumab-treated patients in terms of site, size, Campanacci grade, and recurrent versus primary status, and with a minimum 2 years followup for the control group. Fisher's exact test was used for statistical study. Patients undergoing resection were planned for surgery after three doses of denosumab to allow the tumor to solidify and potentially decrease tumor spillage at the time of surgery. The resections could not be case-matched for comparison owing to the smaller numbers. RESULTS: We observed 14 recurrences out of the 37 curetted tumors (38%). In the case-matched analysis, 11 of 25 patients in the denosumab-treated curettage group had recurrences (44%) compared with seven of 34 (21%) in the nondenosumab-treated control group. The risk of denosumab-treated patients experiencing local recurrence as compared with the nondenosumab-treated patients was nonsignificant with a two-tailed p value of 0.085 (significance at p < 0.05) as derived from Fisher's exact test (odds ratio, 3.03; 95% confidence interval, 0.96-9.54). There was no recurrence in the resection group. Because we do not have a control group for resection, we are unable to comment on the importance of this finding. One major complication that we observed was a recurrence with malignant transformation in a patient with a proximal humeral GCT. We did not observe any other complications related to the denosumab therapy. CONCLUSIONS: Although we could not demonstrate a higher risk of local recurrence with preoperative denosumab for intralesional surgery in the dose and frequency we administered, we advise caution in its routine use for intralesional procedures because it may be important to curette up to margins on pretreatment imaging owing to the potential residual tumor within the denosumab-mediated thick bony shell, which may result in local recurrence. We believe that denosumab treatment before resection of a large tumor aids resection without tumor spillage, particularly where important structures like the neurovascular bundle are dissected away from the tumor margin, although we cannot confirm that it helps lower the incidence of recurrence. We are concerned regarding the malignancy-causing potential from our observation in one patient as well as reports of this by others and recommend judicious use of this drug in patients with GCT. LEVEL OF EVIDENCE: Level III, therapeutic study.
Asunto(s)
Antineoplásicos/administración & dosificación , Conservadores de la Densidad Ósea/administración & dosificación , Neoplasias Óseas/terapia , Denosumab/administración & dosificación , Tumor Óseo de Células Gigantes/terapia , Osteotomía , Adolescente , Adulto , Anciano , Antineoplásicos/efectos adversos , Conservadores de la Densidad Ósea/efectos adversos , Neoplasias Óseas/diagnóstico por imagen , Neoplasias Óseas/patología , Quimioterapia Adyuvante , Denosumab/efectos adversos , Femenino , Tumor Óseo de Células Gigantes/diagnóstico por imagen , Tumor Óseo de Células Gigantes/patología , Humanos , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Osteotomía/efectos adversos , Estudios Retrospectivos , Factores de Riesgo , Factores de Tiempo , Resultado del Tratamiento , Adulto JovenRESUMEN
Primary malignant lymphomas of the salivary glands are rare, accounting for 2-5% of salivary gland tumors and 5% of extranodal lymphomas, frequently seen in the parotid gland. There are single case reports mentioned in the literature. Clinical presentation is not characteristic and the disease is often overlooked with delay in diagnosis and treatment. We are reporting a case of bilateral parotid gland lymphoma in a 55-year-old male, presented with bilateral enlarged parotids. Magnetic resonance imaging (MRI) showed bilateral enlarged parotid glands with multiple well-defined intraparotid lesions. Fine Needle Aspiration Cytology (FNAC) of both showed mixed population of lymphoid cells with large monocytoid cells with scant cytoplasm, anisonucleosis with prominent nucleoli, and numerous mitoses suggestive of non-Hodgkin's lymphoma (NHL). Histopathology showed sheets of large lymphoma cells destructing the salivary acini and infiltrating the periparotid fat. Immunohistochemistry (IHC) showed diffuse CD20 positivity, B-cell lymphoma 6 protein (Bcl-6) was focally positive and negative for cluster of differentiation (CD) 3, CD5, CD10, and Multiple myeloma oncogene-1 (MUM1) which led to the diagnosis of NHL-Diffuse large B cell type.
RESUMEN
Despite the eradication of smallpox from the world in 1980 the osteo-articular sequelae of smallpox are still occasionally noticed in previously endemic areas. The sequelae of osteomyelitis variolosa may raise a diagnostic challenge to the untrained eyes of the surgeon. We present a case of "osteomyelitis variolosa" in a 70 years old patient. The patient had bilateral dislocation of the elbow joint with multidirectional instability. There was distortion of the articular surfaces and ankylosis of the bilateral proximal radio-ulnar joint. Hypoplasia of the right ulna with short fourth and fifth metacarpals of the left hand and hypoplasia of right fourth metacarpal with cortical thickening was noted radiologically. The patient had minimal disability of his elbows despite the striking radiological abnormality and was functionally independent.