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.

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.

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.

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.].

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.

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.

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.

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.

Varus deformity of the left lower extremity causing degenerative lesion of the posterior horn of the left medial meniscus in a patient with Paget's disease of bone.

We report on a 42-year-old woman who presented with persistent pain in her left knee with no history of trauma. Sagittal T1-weighted MRI of the left knee showed discontinuity between the anterior and posterior horns of the left medial meniscus, causing effectively the development of degenerative lesion of the posterior horn. The latter was correlated to varus deformity of the left lower extremity associated with subsequent narrowing of the medial knee joint. The unusual craniofacial contour of the patient, the skeletal survey and the elevated serum alkaline phosphatase were compatible with the diagnosis of Paget's disease of the bone. To alleviate the adverse effect of the mal-alignment of the left femur onto the left knee, corrective osteotomy of the left femoral diaphysis by means of fixators was performed. To the best of our knowledge this is the first clinical report describing the management and the pathological correlation of a unilateral varus deformity of the femoral shaft and degenerative lesions of the left knee in a patient with Paget's disease of the bone.

Five polyostotic conditions that general orthopedic surgeons should recognize (or should not miss).

General orthopedic surgeons frequently encounter patients with conditions affecting multiple bones. It is important to recognize common polyostotic diseases. This article describes five polyostotic conditions: Multipe Enchondromatosis (Ollier Disease and Maffucci syndrome), Multiple Hereditary Exostosis (Diaphyseal Aclasis), Fibrous Dysplasia (McCune-Albright syndrome and Mazabraud syndrome), Paget's Disease of bone (Osteitis Deformans), and Skeletal Metastases. This is a survey of the clinical, pathologic and radiographic features that assist in diagnosing these conditions. Also, an overview of the laboratory findings, treatment, follow-up, and prognosis is presented. Recognizing these diseases will aid in prompt and accurate diagnosis and appropriate referral and therapy.

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.

Syringomyelia associated with Paget disease of the skull.

A 38-year-old man presented with a rare case of syringomyelia associated with Paget disease of the skull. Syringosubarachnoid (SS) shunting was performed. We speculate that deformation of the skull secondary to Paget disease caused narrowing of the foramen magnum with progressive impairment of the cerebrospinal fluid circulation, which led to syringomyelia and neurological symptoms. SS shunting is safe, effective, and technically simple, so may be a useful initial treatment for syringomyelia associated with Paget disease of the skull.

The transconjunctival transorbital approach: a keyhole approach to the midline anterior skull base.

OBJECTIVE: To report an initial experience with a medial transorbital approach to the midline skull base performed via a transconjunctival incision. METHODS: The authors retrospectively reviewed their clinical experience with this approach in the management of benign cranial base pathology. Preoperative imaging, intraoperative records, hospitalization charts, and postoperative records were reviewed for relevant data. RESULTS: During the period 2009-2011, six patients underwent a transconjunctival craniotomy performed by a neurosurgeon and otolaryngologist-head and neck surgeon working together. The indications for surgery were esthesioneuroblastoma in one patient, juvenile angiofibroma in one patient, Paget disease in one patient, and recalcitrant cerebrospinal fluid leaks in three patients. Three patients had prior cranial base surgery (either open craniotomy or an endonasal approach) done at another institution. The mean length of stay was 3.8 days; mean follow-up was 6 months. Surgery was considered successful in all cases (negative margins or no leak recurrence); diplopia was noted in one patient postoperatively. CONCLUSIONS: The transconjunctival medial orbital craniectomy provides a minimally invasive keyhole approach to lesions located anteriorly along the anterior cranial fossa that are in the midline with lateral extension over the orbital roof. Based on our initial experience with this technique, the working space afforded limits complex surgical dissection; this approach is primarily well suited for less extensive pathology.

Treatment of Paget's disease of bone.

Paget's disease of bone is the paradigm of bone focal distortion with accelerated bone turnover. Over the years, a number of different drugs have been used to control its activity but, since biphosphonates were introduced for the treatment of the disease, they have become the preferred treatment. This review will update the therapeutic indications, available drugs and therapeutic response monitoring.

Extirpación de la primera costilla por videotoracoscopia para el tratamiento del síndrome del estrecho torácico / First Rib Resection Using Videothorascopy for the Treatment of Thoracic Outlet Syndrome

Introducción: El síndrome del estrecho torácico es una patología provocada por la compresión de la arteriasubclavia, vena subclavia y/o el plexo braquial. Su tratamiento más efectivo es la sección de la musculaturaescalena y la extirpación de la primera costilla. El objetivo de este trabajo es mostrar la técnica de laextirpación de la primera costilla por videotoracoscopia.Técnica: Describimos la técnica de extirpación costal por videotoracoscopia a través de tres puertas deentrada de 12mm (si bien una se amplía 3 cm para la introducción de periostotomos y costotomos).Hemos realizado esta intervención a tres casos de síndrome del estrecho torácico con resultados muybuenos.Conclusiones: La extirpación de la primera costilla por videotoracoscopia es una técnica factible, muyestética y de escasa morbilidad. Es una opción muy buena para la mayoría de pacientes afectos de estesíndrome, especialmente en pacientes obesos o con síndrome de Paget-Schroetter(AU)

Objective: Thoracic outlet syndrom is a condition caused by compression of the subclavian artery, subclavianvein and/or the brachial plexus. Scalene muscle section and first rib removal is the most effectivetreatment. The objective of this article is to demonsrate first rib resection using videothoracoscopy.Material and method: We describe first rib removal by videothoracoscopy using three 12mm entranceports (although one is widened to 3 cm to introduce periosteotomy cutters and rib shears). We haveperformed this operation on three cases of thoracic outlet syndrome with very good results.Conclusions: Removal of the first rib by videothoracoscopy is a viable and very aesthetic technique with alow morbidity. It is a very good option for the majority of patients affected by this syndrome, particularlyin obese patients or those with Pager-Schroetter syndrome(AU)

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.