Outcomes following Total Hip and Knee Arthroplasty in Patients Who Have Paget's Disease of Bone: A Systematic Review.

BACKGROUND: Patients who have Paget's Disease more frequently require total hip arthroplasty (THA) and total knee arthroplasty (TKA) than matched controls. However, controversy remains regarding their outcome. We aimed to evaluate the literature regarding outcomes following THA and TKA in patients who have Paget's Disease. METHODS: MEDLINE, EMBASE and Cochrane databases were searched for all articles evaluating outcomes following THA and TKA in patients who have Paget's Disease. Quality of included studies was assessed using the Newcastle-Ottawa Scale. RESULTS: A total of 19 articles (published between 1976 and 2022) were included, comprising 58,695 patients (48,766 controls and 10,018 patients who have Pagets Disease), from 209 potentially relevant titles. Patients with Paget's Disease have a pooled mortality of 32.5% at a mean of 7.8 years (range, 0.1 to 20) following THA and 31.0% at a mean of 8.5 years (range, 2 to 20) following TKA, with a pooled revision rate of 4.4% at 7.2 years (range, 0 to 20) following THA and 2.2% at 7.4 years (range, 2 to 20) following TKA. Renal and respiratory complications, as well as heterotopic ossification and surgical-site infection were the most common post-operative complications. CONCLUSION: There is marked heterogeneity in outcome reporting of studies assessing arthroplasty in patients who have Paget's Disease, with studies of low to moderate quality. Patients with Paget's Disease undergoing THA and TKA appear to have similar implant longevity as their unaffected counterparts. However, they appear to have an increased risk of medical and surgical complications and may have a higher mortality risk from their procedure.

Clinical and radiological outcomes of total hip arthroplasty in patients affected by Paget's disease: a combined registry and single-institution retrospective observational study.

BACKGROUND: Total hip arthroplasty (THA) in patients with Paget's disease can be associated with technical difficulties related to deformities and altered mechanical bone properties, and hypervascularity leads to significative intra-operative bleeding. The purpose of this registry and single-institution study was to investigate overall survival and causes of failure of THA in pagetic patients, together with an analysis of the clinical and radiological complications. MATERIAL AND METHODS: Registry-based survival and complication analysis, type of fixation, intra- and post-operative complications, clinical (pharmacological history, blood transfusions, Harris hip score [HHS]) and radiographic (cup orientation, stem axial alignment, osteolysis around the cup and the stem and heterotopic ossification [HO]) data were reviewed. RESULTS: In total, 66 patients (27 males and 39 females, mean age at surgery 71.1 years for males and 74.8 years for female) from the registry study presented a 10-year survival of 89.5%. In the institutional study, involving 26 patients (14 males and 12 females, 69 years average) and 29 THAs, hip function improved significantly. Average cup orientation was 40.5°, while varus stem alignment was 13.8%. In total, 52% of hips had heterotopic ossifications. Peri-acetabular osteolysis was in 13.8% of implants and in 45% of hips was found around the stem. Allogenic and autologous blood transfusion rate were 68.2% and 31.8%, respectively, with an average transfusion of 2 units of blood (range 1-6 units). HHS improved by an average of 34 points, with excellent result in 64.3% of patients. Two implants failed, one due to traumatic ceramic head fracture 64 months after surgery, and one due to mobilization of the cup on the second post-operative day. CONCLUSION: THA surgery in Paget's patients is a safe procedure, and implant survival is only partly affected by bone remodelling and choice of fixation. The post-operative functional outcome is largely similar to that of other patients. Bleeding-related complications are the main complications; a careful pharmacological strategy should be recommended to decrease the risk of transfusions and of HO development. LEVEL OF EVIDENCE: Level III.

Long-term outcomes following total hip and total knee arthroplasty in patients with Paget's disease of bone (PDB) - A national study.

BACKGROUND: The incidence of total hip and total knee arthroplasty (THA/TKA) is 3.1- and 1.7-fold higher in patients with Paget's disease of bone (PDB) compared to age-matched controls. No large studies or joint registry reports exist describing outcomes following THA or TKA in PDB patients. METHODS: The study objectives were to investigate the outcomes following THA or TKA in PDB patients using national registry data. Data were requested from the Scottish Arthroplasty Project for all PDB patients undergoing THA or TKA in Scotland from 1996 to 2013. RESULTS: Between 1996 - 2013, 144 patients underwent primary THA and 43 patients underwent primary TKA for PDB in Scotland. Following primary THA, the most common surgical complications within one year were haematoma (1.4%), and surgical-site infection (1.4%). The overall incidence of dislocation was 2.8%. Revision THA was performed in 2.8% of patients. THA implant survival was 96.3% (CI:92.8-99.8) at 10-years, and patient survival was 50.0% (CI:39.6-60.4) at 10-years. Following TKA, only one revision surgery occurred within one year (2.3%). Revision TKA was performed in 4.7% of patients, across the whole study period. TKA implant survival was 94.5% (CI:87.1-100) at 10-years; patient survival was 38.3% (CI:16.7-59.9) at 10-years. Compared with published literature and registry data, implant longevity and patient survival are comparable between PDB patients and the general population. CONCLUSION: This is the largest reported series of outcomes following primary THA/TKA in PDB patients. PDB patients are not at increased risk of surgical complications following primary THA or TKA compared with the general population.

Skeletal dissemination in Paget's disease of the spine.

PURPOSE: Paget's disease of bone (PDB) is a common skeletal disorder that is associated with locally increased bone turnover, skeletal deformity and pain. We report a case of skeletal dissemination in PDB of the spine. METHODS: Case report. RESULTS: A 46-year-old former professional athlete suffered from disseminated PDB throughout the spine and hips after various surgical interventions including spondylodesis, bone grafting and bone morphogenetic protein (rhBMP-2) administration. Only intravenous zoledronic acid prevented the further progression of skeletal dissemination, which was expressed by a normalization of (bone-specific) alkaline phosphatase levels. The biopsy obtained from the lumbar spine confirmed the diagnosis of PDB in the absence of malignant transformation. CONCLUSIONS: We outline skeletal dissemination as a possibly surgery-related complication in a patient with PDB in the lumbar spine. Bisphosphonates remain the treatment of first choice in PDB and surgical interventions should be considered very carefully.

Paget's disease of the lumbar spine: decompressive surgery following 17 years of bisphosphonate treatment.

BACKGROUND: We present a rare case of Paget's disease (PD) with involvement of the lumbar spine over a period of 19 years. We discuss the diagnostic process to rule out alternative diagnoses and medical and surgical treatment strategies. CASE DESCRIPTION: A 58-year-old man first diagnosed with PD in 1998 with solid involvement of the 4th lumbar vertebra has been undergoing periodic examinations over a period of 18 years. Since then, the patient has been treated conservatively with bisphosphonates. When conservative treatment options have been exhausted, surgery was indicated due to a progressively reduced ability to walk. Surgery with undercutting decompression via laminotomy was performed. PD was confirmed by biopsy. Bisphosphonate treatment was continued pre- and postoperatively. Follow-up examinations showed an improvement in clinical outcome measures. CONCLUSIONS: Conservative treatment remains the gold standard for PD with spinal involvement. This patient had been asymptomatic on bisphosphonate therapy for almost 17 years, but presented with new onset back pain. In such cases, fracture and rare conversion into sarcoma must be ruled out, and biopsy should be performed even in the absence of signs of malignancy. Currently, there are no clear treatment recommendations available in the literature regarding cases of PD with expansive growth and involvement of the spinal canal causing neurologic deficits. Furthermore, laminectomy has been shown to cause complications in up to 27% of cases with the risk of early postoperative death. In contrast, extended laminotomy and undercutting decompression should be considered.

Surgical treatment in spine Paget's disease: a systematic review.

Paget's disease of bone (PDB) is a disease characterized by a disorder in the bone metabolism. The spine is the second region affected after the pelvis. Surgical treatment is reserved for cases refractory to medical treatment. We performed a systematic review of patients with Paget disease of bone affecting the spine, treated surgically in the last 30 years. The main objective of the review is to find out indications for surgery, outcomes of these patients and also the standard perioperative management.

Vulvar Reconstruction Following Surgery for Vulvar Cancer Using a Stepladder V-Y Advancement Medial Thigh Flap.

OBJECTIVE: The aim of this study was to identify the risks and benefits of the stepladder V-Y advancement medial thigh flap for vulvovaginal reconstruction in comparison to direct skin closure. METHODS: Fifty-four patients with vulvar cancer treated in our hospital between 1992 and 2013 were enrolled in this study. The cohort group (group A) consisted of 25 patients who underwent surgery from August 2006 until April 2013. During this period, we changed our surgical paradigm to incorporate reconstructive plastic surgery immediately following surgery for vulvar cancer performed by gynecologic oncologists. The control group (group B) consisted of 29 patients treated between 1992 and August 2006. During this period, our surgical approach was limited to direct skin closure with no reconstructive plastic surgery. Perioperative findings and clinical outcomes were compared retrospectively. RESULTS: Patient characteristics and surgical procedures, other than the reconstructive surgery, were the same for the 2 groups. The mean blood loss and operative times were similar, and there were no major complications in either group; however, the average length of hospital stay was significantly shorter in group A (P = 0.04). CONCLUSIONS: Stepladder V-Y advancement medial thigh flap lowers posttreatment morbidity and improves quality of life for patients with vulvar cancer. Rapid recovery from surgery is reflected in the short hospital stay, and it enables immediate induction of adjuvant therapy. It may possibly contribute to improved treatment outcome.

Cementless hip arthroplasty in Paget's disease at long-term follow-up (average of 12.3 years).

We present a long-term follow-up report of 33 cementless total hip arthroplasties in 27 patients who have an established diagnosis of Paget's disease. The medium term results of this series were reported in 2007 (Lusty et al. Journal of Arthroplasty. 2007;22:692). Fourteen cases were available for follow-up at an average of 12.3 years (range 10-17). Harris Hip scores improved from 56/100 preoperatively (16-98/100) to 83/100 post operatively (72-90/100). All surviving components were radiographically ingrown. Based on these findings, cementless total hip arthroplasty has a good long-term outcome in Paget's disease.

Total hip arthroplasty and Paget's disease: case report and literature review.

Paget's disease (PDB) is a chronic osteopathy more common in male, Caucasic, European population, after the age of 50 years, that can lead to bone deformities. A challenging surgical solution for affected hip with severe hip osteoarthritis is total hip replacement (THA). We describe a case of THA in a 71-year-old patient with PDB and we present a literature review. In particular we find out that more studies comparing cementless THA with cemented one are necessary, in order to understand if one implant is better than the other.

Vertebroplasty for symptomatic monostotic Paget disease.

Paget disease (PD) is a chronic metabolically active bone disorder. The spine is the second most commonly involved site; the pathologic changes can cause back pain, myeloradiculopathy, and vertebral fracture. Symptomatic patients are treated medically, and surgery is required when certain complications occur. A case is presented of monostotic vertebral PD treated by percutaneous vertebroplasty (PV) with successful outcome characterized by pain relief and improved disability at 6-month follow-up. PV is proposed as a primary treatment for back pain secondary to PD when unresponsive to conservative therapy and when not associated with other complications.

Shoulder Arthroplasty for Patients With Proximal Humeral Paget's Disease.

Poorly controlled Paget's disease leads to excessive blood loss following total hip arthroplasty. The effect in shoulder arthroplasty is unknown. The authors reviewed 3 patients with Paget's disease involving the proximal humerus, comparing them with 17 patients with Paget's disease but no humeral involvement. The 3 patients had an estimated blood loss of 1400 mL, 1100 mL, and 350 mL, compared with an average of 280 mL in the control group. The first 2 cases required 4 units of packed red blood cells intraoperatively, and both were not managed with bisphosphonates. Paget's disease of the humerus leads to more intraoperative blood loss and higher blood transfusion requirements, particularly in cases not managed with bisphosphonates. [Orthopedics. 2021;44(4):e614-e619.].

Cementless total hip arthroplasty in Paget's disease of bone: a retrospective review.

Paget's disease of bone (PDB) is a localised chronic osteopathy leading to bone deformities, bone hypervascularity, structural weakness and altered joint biomechanics. The pelvis and upper femur are frequently involved, resulting in disabling hip disease, and total hip arthroplasty (THA) may be required. We performed a retrospective study on the management and the outcome of 39 uncemented hydroxyapatite fully-coated THA in patients with PDB of the hip. The follow-up averaged 79.4 months (range 24-194). Functional scores improved significantly and, using the Harris hip score, 84% of patients had an excellent clinical outcome at the latest follow-up. Despite one case of an uncemented acetabular component with probable loosening, no implant revision had been required at our latest follow-up. Signs of implant loosening were found to be significantly more frequent in patients with active disease. For this reason, we advocate the use of pre-operative medication with bisphosphonates to reduce disease activity. Another benefit of this treatment is the significant decrease of intra-operative blood loss. Provided the control of disease activity in the pre-operative period with bisphosphonates is achieved, good outcome of cementless THAs can be expected. Bisphosphonates reduced the risk of implant loosening and excessive intra-operative blood loss.

Solitary orbital paget disease: a case report.

PURPOSE: To describe the clinical findings in a patient who had developed painless supraorbital prominence. METHODS: Clinical and histopathological findings of the case are reviewed and presented. RESULTS: The patient had an isolated histologically confirmed Paget disease of the orbit. Local excision of the lesion was performed. CONCLUSION: Paget disease can present not only as a diffuse process but also as a painless, solitary lesion of the orbit.

Monostotic Vertebral Paget's Disease of the Lumbar Spine.

Paget's disease in the Far East is quite rare. Age at diagnosis is usually greater than 50 years, and the disease typically affects the spine. Most patients are usually diagnosed with Paget's disease following radiographic examination for other purposes. It usually occurs at multiple vertebral levels, with only 10-25% of vertebral Paget's disease being monostotic. The disease rarely causes neurologic complications resulting from compression of intraspinal nerve tissue. Here, we present 2 cases of monostotic vertebral Paget's disease of the third lumbar vertebra. The first patient, who may be the first documented case of Paget's disease in the lumbar spine with progressive neurologic deficiency in an Asian population, received decompressive laminectomy due to marked spinal stenosis with neurologic deficits. The symptoms were greatly relieved following surgery, and ambulatory ability was restored. The second patient was diagnosed with Paget's disease following surgical biopsy. He remained asymptomatic at the most recent follow-up.

Paget's disease 2: exploring diagnosis, management and support strategies.

This is the second of a two-part unit on Paget's disease. Part 1 outlined the epidemiology, pathophysiology, causes and clinical features of the condition. This part outlines advice on diagnosis, drug therapy, surgery, other management options and emotional support.

A rare emergency condition in neurosurgery: foot drop due to Paget's disease.

Paget's disease is a chronic, focal skeletal disorder that usually affects the pelvis and spine. Spinal cases are generally asymptomatic; in the symptomatic cases, the neurological dysfunctions are related to non-compressive vascular defects, hemorrhage, sarcomatoid degeneration, spinal stenosis, or pathological fractures, primarily in the lumbar region. The Neurosurgeon should have a fundamental understanding of the complications of Paget's disease and should be familiar with the indications for treatment, as well as available medical and surgical therapies. In the present paper, we report a case of Paget's disease that presented with an isolated foot drop due to a pathological fracture of L5 vertebra, and then discuss the therapeutic strategies presented in the literature.

Degeneration of Paget's disease into sarcoma: clinical and therapeutic influencing factors.

We analysed a series of 119 patients presenting with Paget's bone disease treated at the Istituto Ortopedico Rizzoli (Bologna Italy) over 35 years (from 1970 to 2006). Among these, we found 18 cases that had degenerated into sarcoma. The first goal of this study was to underline the potential factors of Paget's disease transformation into sarcoma. In detail we considered: age, gender, duration of disease, clinical expression, levels of total alkaline phosphatase and type of therapy. The results highlighted an interesting correlation between therapy and degeneration into sarcoma; more specifically, malignant transformation occurred both in patients who had not received any therapy and those who had received regular calcitonin treatment; otherwise, no sarcoma degeneration occurred in the patients treated with bisphosphonates. These data seem to indicate a preventing effect of bisphosphonates towards the degeneration of Paget's disease into sarcoma.

Surgical management of Paget's disease of bone.

Paget's disease is a chronic nonmetabolic bone disorder that is characterized by increased bone resorption, bone formation, and remodeling. This unbalanced process may lead to osseous deformities, structural weakness, and altered joint biomechanics, all of which can make surgical reconstruction difficult. Although few patients with Paget's disease ever need surgical treatment, successful surgical management of orthopedic manifestations of the disease has improved the quality of life for these patients. Surgical options include corrective osteotomy for long bone deformity, fracture fixation, joint arthroplasty, spinal decompression, and tumor resection. Patients are at increased risk for surgical complications such as blood loss and heterotopic bone formation. Issues relating to the surgical management of patients with Paget's disease such as appropriate preoperative diagnosis, technical challenges of surgery, and strategies to improve the long-term outcome of surgical intervention are discussed.

Monostotic Paget's disease of the patella.

Paget's disease is a common skeletal disorder in the elderly; however, monostotic Paget's disease of the patella is very rare and only a few cases have been reported. We present the clinical, radiographic, surgical and histological findings in a patient with monostotic Paget's disease of the patella, presenting with knee pain.