Do's and Don'ts in Primary Aneurysmal Bone Cysts of the Proximal Femur in Children and Adolescents: Retrospective Multicenter EPOS Study of 79 Patients.

BACKGROUND: Aneurysmal bone cysts (ABC) are rare benign cystic bone tumors, generally diagnosed in children and adolescents. Proximal femoral ABCs may require specific treatment strategies because of an increased pathologic fracture risk. As few reports are published on ABCs, specifically for this localization, consensus regarding optimal treatment is lacking. We present a large retrospective study on the treatment of pediatric proximal femoral ABCs. METHODS: All eligible pediatric patients with proximal femoral ABC were included, from 11 tertiary referral centers for musculo-skeletal oncology (2000-2021). Patient demographics, diagnostics, treatments, and complications were evaluated. Index procedures were categorized as percutaneous/open procedures and osteosynthesis alone. Primary outcomes were: time until full weight-bearing and failure-free survival. Failure was defined as open procedure after primary surgery, >3 percutaneous procedures, recurrence, and/or fracture. Risk factors for failure were evaluated. RESULTS: Seventy-nine patients with ABC were included [mean age, 10.2 (±SD4.0) y, n=56 male]. The median follow-up was 5.1 years (interquartile ranges=2.5 to 8.8).Index procedure was percutaneous procedure (n=22), open procedure (n=35), or osteosynthesis alone (n=22). The median time until full weight-bearing was 13 weeks [95% confidence interval (CI)=7.9-18.1] for open procedures, 9 weeks (95% CI=1.4-16.6) for percutaneous, and 6 weeks (95% CI=4.3-7.7) for osteosynthesis alone ( P =0.1). Failure rates were 41%, 43%, and 36%, respectively. Overall, 2 and 5-year failure-free survival was 69.6% (95% CI=59.2-80.0) and 54.5% (95% CI=41.6-67.4), respectively. Risk factors associated with failure were age younger than 10 years [hazard ratios (HR)=2.9, 95% CI=1.4-5.8], cyst volume >55 cm 3 (HR=1.7, 95% CI=0.8-2.5), and fracture at diagnosis (HR=1.4, 95% CI=0.7-3.3). CONCLUSIONS: As both open and percutaneous procedures along with osteosynthesis alone seem viable treatment options in this weight-bearing location, optimal treatment for proximal femoral ABCs remains unclear. The aim of the treatment was to achieve local cyst control while minimizing complications and ensuring that children can continue their normal activities as soon as possible. A personalized balance should be maintained between undertreatment, with potentially higher risks of pathologic fractures, prolonged periods of partial weight-bearing, or recurrences, versus overtreatment with large surgical procedures, and associated risks. LEVEL OF EVIDENCE: Level IV, therapeutic study.

An Audit of the effect of Bone Morphogenetic Protein-2 Treatment in patients with non-union of long bone fractures.

OBJECTIVE: To evaluate the outcomes, clinical and radiological application of Bone Morphogenetic Protein-2 alone versus Bone Morphogenetic Protein-2 with autograft in long bone non-unions. METHODS: A prospective review of patients was done with fracture non-union admitted to Aga Khan University Hospital, Karachi, from January 2016 to January 2019. The patients were divided into two groups; those exposed to Bone Morphogenetic Protein-2 alone in group 1, and those exposed to Bone Morphogenetic Protein-2 plus autologous graft in group 2. RESULTS: Background characteristics of both the groups were analysed. Patients were followed up at 6, 12 and 24 weeks through their medical records. The primary outcome was postoperative union at 6, 12 and 24. Union was defined by having the clinical union as well as the radiological union at the same time of assessment. Of the 80 patients enrolled, 13(16.25%) were excluded, and 5(6.25%) were lost to follow-up. The final sample had 62(77.5%) patients; 35(56.5%) in group 1, and 27(43.5%) in group 2. Union at 6 weeks was observed in 13(21%) patients; 8(62%) in group 1, and 5(38%) in group 2. Union at 12 weeks was observed in 38(61%) patients; 20(53%) in group 1, and 18(47%) in group 2. CONCLUSIONS: Results showed that using the adjuvant treatment alone was not worse than using it along with bone autologous graft.

Our experience of Mega-prosthesis in bone tumours: A retrospective cross-sectional study in a tertiary care hospital.

OBJECTIVE: To evaluate the oncological and functional outcomes of bone tumour patients who underwent reconstruction with mega prosthesis. METHODOLOGY: A retrospective study was conducted in the department of Orthopaedics Aga Khan University Hospital, Karachi. All the paediatric and adult age group patients diagnosed with malignant, benign and metastatic bone tumours and meeting the inclusion criteria were selected and analysed. Retrospective data was collected from January 2008-January 2018. RESULTS: Sixty-two patients, 30 (48.4%) females and 32 (51.6%) males. were included in the study. Of these 57 (92%) cases had involvement of the lower limb. The mean age was 36.95±19.1 years with a range of 9-81 years. The duration of patients follow up was from 1-124 months (mean 32.7±36.43 months). There were 29 (47%) malignant cases. The most commonly occurring tumour site was distal femur and proximal femur. There were 53 (85%) primary surgeries (first time conducted surgeries) while 9(15%) revision surgeries were done. Major complications were encountered in 19 (30.6%) patients and 13 (20.9%) had minor complications. Post-surgery local recurrence occurred in 2 (3.2%) patients while 7 (11.2%) had distant metastasis. In functional outcomes the mean MSTS score of our patients was 72.09±26.43. The survival rate was 69.8% with 45 patients recovered. CONCLUSIONS: With a good patient selection, adherence to the principles of tumour surgery and an adequate applied knowledge of mega prosthesis insertion, a good functional outcome was achieved.

Strengthening Orthopaedic care at national level: Output of a structured residency programme at Aga Khan University.

This descriptive review of the output of the orthopaedic residency programme of Aga Khan University, Karachi, comprised information regarding the number of graduated residents and their educational background which was retrieved from departmental records. Information about their work location, subspecialty, current working status, participation in medical camps and national disaster relief efforts were obtained from various sources, including fellow surgeons, and social media profiles. From 1989 to 2017, a total of 48 residents graduated from the programme, with only 2(4.2%) of them being females. Overall, 19(39.6%) residents hailed from areas outside Karachi; 28(58.3%) belonged to Karachi; 1(2%) came from Kenya; 41(85.4%) remained to serve in Pakistan working mostly in tertiary healthcare centres; and 7(14.6%) moved abroad on consultancy and teaching assignments. Subspecialty training had a general trend towards general orthopaedics and trauma 21(43.7%), followed by arthroplasty surgery 13(27%).

Treatment and outcomes of soft tissue sarcoma of groin, hip and thigh: a retrospective review from a tertiary care hospital.

OBJECTIVE: To study the frequency of the thigh, hip and groin soft tissue sarcomas and retrospectively analyse the management, treatment results, and outcomes of these uncommon malignant tumours, in a tertiary care hospital of the city of Karachi. METHODOLOGY: Data of soft tissue tumours registered from 2017-2018 was retrieved during January 2019 to March 2019 from Aga Khan University Hospital, Karachi bone and soft tissue tumour registry. A retrospective review was performed and all soft tissue tumour cases treated with surgical intervention (with adjuvant /neoadjuvant therapy) or palliative intention were included. RESULTS: Total 119 cases of soft tissue tumours (STS) were identified out of which 85 were malignant cases (sarcomas) while 30 were benign. On presentation 84 (70.6%) were primary cases. On topographical distribution, there were 25 patients who had hip, groin and thigh sarcoma. Of these, 15 were males and 10 were females. As treatment, neo-adjuvant radiation was done in 4 (16%) patients and adjuvant chemo/radio therapy was given to 13 (52%) patients. Wide margin excision was performed in 19 (76%) patients and 4 (16%) had amputation. Reconstruction was offered to 3 (12%) patients. In post-surgical complications, 1 (4%) patient had wound infection. On final surgical histopathology, majority of the sarcomas were liposarcomas, myxofibrosarcoma, synovial sarcoma and Leiomyosarcoma. Post-surgery recurrence occurred in 7 (28%) patients. Overall survival was 76%. CONCLUSIONS: In treatment of soft tissue sarcoma, limb salvage is an achievable option and survival results are also good.

Our experience of treating adult bone lymphoma, A retrospective cross-sectional study in a tertiary care center, Aga Khan University Hospital, Karachi.

OBJECTIVE: To determine the experience at the Aga Khan University Hospital in diagnosing and treating adult patients with primary lymphoma of bone. METHODOLOGY: All patients with Primary lymphoma of bone (PLB) that were diagnosed and/or treated at Aga Khan University Hospital, Karachi from 2005 to 2019 were included as part of this study. RESULTS: There were 17 patients with PLB including 13 (76.5%) males and 4 (23.5%) females with a mean age of 44 ± 16.5 years. Nine patients were between 30-59 years of age at diagnosis. The mean follow-up time of patients was 80±46.7 months. Six patients had tumours of pelvic bone followed by tibia (5) and femur (4). Four patients had a pathological fracture at the time of presentation whereas 2 (11.8%) required surgical fixation of the pathological fracture. The stage of the tumour was based on Ann Arbor classification. Nine (52.9%) cases had Stage 1 disease, 7 (41.2%) had stage IV disease with metastasis in extra nodal tissues. As for treatment, every patient received chemotherapy whereas 5 (29.4%) received adjuvant radiotherapy. Complete remission in the size of the tumour was seen in 11 (64.7%) patients while 6 (35.3%) had partial remission. Post-treatment, 4 (23.5%) patients expired. The mean Overall Survival (OS) time was 80.18 ± 46.71months with a survival rate of 76.5. CONCLUSIONS: Primary lymphoma of the bone can be treated with medical regime and good prophylactic surgeries to avoid pathological fracture such as intramedullary nailing.

Functional outcomes and complications of total hip arthroplasty with dual mobility cup : an audit.

OBJECTIVE: To determine the functional outcomes in total hip arthroplasty with a dual mobility cup, performed in our hospital. METHODS: After receiving an exemption from the Ethics review committee of the hospital, data collection for audit was started in January 2019. Records from July 2016 to June 2018 were included. All patients who underwent total hip arthroplasty with dual mobility prosthesis without any age limit were included. A proforma was prepared to collect the required information. Data was entered and analyzed on SPSS v. 21. RESULTS: Two hundred and ten patients were included, 114 females and 96 males. Of the total, 188 patients underwent unilateral surgery while 22 had bilateral hip arthroplasty. The mean postoperative hospital stay was 5.91±3.9 days. . Mean pre-op Harris score was 33.7±7.6 and the post-op mean score was 75.9± 5.34. Eighty-three (39.5 %) patients had the neck of femur fracture, 31(14.8%) had osteoarthritis while 28(13.3%) had avascular necrosis. Post-surgery complications included, wound infection, surgical site haematoma, NSTEMI, and only one patient reported dislocation after use of dual mobility cup. CONCLUSIONS: The dislocation rate which was the prime concern, has been reduced with the use of dual mobility implant in total hip arthroplasty patients.

Long-term functional outcomes after total scapulectomy with dual suspension reconstruction in children - A case series.

Implant reconstruction following scapulectomy in children is a challenging task. Dual suspension reconstruction may offer an alternative but there is a dearth of literature on functional outcomes following this procedure for malignant tumours in children. A retrospective study was conducted at the Aga Khan University Hospital, a tertiary care centre in Karachi, Pakistan. Children with malignant tumours of the scapula who underwent total scapulectomy with dual suspension reconstruction (n=5) between Jan 2009 and June 2015 were included. Mean follow up was 50±13.39 months. There were four boys and one girl having mean age of 11±3.57 years. All patients were Enneking Stage IIB with 4 patients diagnosed as Ewing's Sarcoma and 1 as osteosarcoma. The MSTS scores ranged from 20-25 points, with a median of 23. One patient developed postoperative surgical site infection requiring surgical debridement whereas all patients remained disease-free till last follow up. Our findings suggest that scapulectomy with dual suspension reconstruction achieves satisfactory functional results with low rate of complications.

Outcome of percutaneous screw fixation of posterior pelvic ring injuries.

OBJECTIVE: To evaluate the clinical outcome of percutaneous fixation for unstable pelvic ring injury. METHODS: This retrospective study was conducted at orthopaedics section of Aga Khan University Hospital Karachi, Pakistan from July 2015 to December 2018. Data was retrieved from trauma registry from July 2015 till December 2018, including all patients who underwent percutaneous fixation for pelvic ring injury. Majeed pelvic score was used to determine the functional outcome. RESULTS: A total number of 30 patients were included, 27(90.0%) adults, and 3(10.0%) paediatrics patients. There were 21(70.0%) males and 9(30.0%)females. . Mean age of patients was 37.1±16.1 years. Post op mean Majeed functional pelvic Score was 85.8. Of the 30 patients, 18 (60.0%) had Excellent, 10 (33.3%) good and 02(6.7%) fair scoring. CONCLUSIONS: Percutaneous fixation of posterior ring injuries has excellent functional outcome, with minimal blood loss and no soft tissue striping..

Spectrum of histological features of Denosumab treated Giant Cell Tumor of Bone; potential pitfalls and diagnostic challenges for pathologists.

OBJECTIVES: Denosumab is Receptor Activator of Nuclear factor Kappa-B Ligand (RANKL) inhibitor which is being used in the treatment of locally advanced, recurrent and metastatic Giant Cell Tumor of Bone (GCTB). It causes reduction in monocyte recruitment and Osteoclast-Like Giant Cell (OLGC) formation which limits bone destruction. After Denosumab treatment, GCTB exhibit diverse morphological features which can pose diagnostic challenge. Our aim was to study the spectrum of histologic features seen in Denosumab treated GCTB which could be helpful in establishing correct diagnosis. METHODS: We retrieved and reviewed H&E stained microscopic glass slides of 38 GCTB cases who received Denosumab as neoadjuvant treatment. These cases were treated at different institutes and diagnosed at our institute between January 2017 and October 2019. Morphologic features such as presence of residual OLGC, appearances of mononuclear stromal and bony components were assessed along with other non-specific features. RESULTS: Patients' median age was 29 years. Male to female ratio was 1.53:1. Femur was the most commonly involved bone. Microscopically, peripheral shell of reactive bone was observed in all cases. In 20 (52.6%) cases, there was complete elimination of OLGC. Mononuclear stromal cells were predominantly bland spindle shaped and arranged in fascicular and storiform patterns. Focal atypia was noted in 3 cases. Bony component manifested as trabeculae of woven bone with osteoblastic rimming and immature trabeculae of unmineralized osteoid with haphazardly present osteoblasts. Spectrum of stromal changes included cystic spaces, foamy macrophages, inflammatory infiltrate, hemangiopericytoma-like (HPC-like) vessels, hyalinization, edematous areas and hemosiderin pigment. The tumors showed areas which resembled other bony and soft tissue lesions such non-ossifying fibroma, fibrous dysplasia, osteoblastoma, sclerosing epithelioid fibrosarcoma and osteosarcoma. CONCLUSION: Denosumab treatment induces a variety of changes in GCTB. Clinical history and knowledge of these features are necessary for excluding differential diagnoses and avoiding misdiagnosis.

Musculoskeletal tumours throughout history and beyond: Clinical features, imaging, staging and biopsy.

BACKGROUND: Over the last century, there has been a remarkable development in the study of bone and soft tissue sarcomas. This is primarily due to the improved knowledge of the nature of these lesions and the improved imaging technology. In literature there are many protocols that are being used and all of them have reported various advantages and disadvantages of each technique used. However, there is no set guideline and whatever has been proposed has been developed on the basis of the experience of different centres and different surgeons. OBJECTIVE: The current systematic review was planned to thoroughly evaluate the levels of evidence on which we base decisions for surgical management of lower extremity bone tumours. METHODS: The review included descriptive studies published in the English language. Studies included case reports, case series and experiences of different centres for the surgical management of lower extremity bone tumours. Articles reporting all levels of evidence - Level I to V - were included. PubMed, ERIC, MEDLINE, EMBASE and Cochrane Reviews databases from 2002 to 2012 were searched. RESULTS: Information was gathered and thoroughly studied from 63 articles. There were no Level I studies, 2(3.2%) Level II studies, 47(74.6%) Level III, and the remaining 14(22.2%) studies were Level IV and Level V. CONCLUSIONS: Sarcomas are rarely occurring neoplastic conditions which are present in all age groups but commonly affect young age population. Most are asymptomatic but can present with pain or pathological fracture. These lesions are commonly diagnosed with plain radiographs. CT scan and MRI may be used to delineate anatomy and to quantify the extent of soft tissue involvement. Various advantages and disadvantages associated with each aspect in the management of patients starting from the basic history-taking, physical examination, imaging, biopsy principles, peri-operative laboratory work-up and staging of the cancer were studied. Treatment ranges from conservative to en-block resection including extended curettage. Aggressive tumours should be closely followed up for recurrence and metastasis.

A comprehensive basic understanding of pelvis and acetabular fractures after high-energy trauma with associated injuries: Narrative review of targeted literature.

Acetabular fractures are caused by energy trauma which is high enough to cause such a fracture with incidence of 3 patients / 100000. In older individuals, most commonmechanism of injury is fall and, in younger individuals, road traffic accidents. Acetabular fractures are usually associated with visceral injuries and other musculoskeletal injuries (about 50% of patient). In this narrative review of targeted English literature from all level of evidences, which is written and supervised by experienced specialized orthopedic and trauma surgeons who were among the pioneers of conducting pelvis fracture management workshops in the country, we aim to describe the mechanism of injury, assessment principles and associated injuries, decision-making and preoperative planning and indications of non-operative managements.

Sexual dysfunction in men after high energy pelvic fractures: Narrative review of targeted literature.

Pelvic fractures represents high energy trauma with associated other organ injuries including intra-abdominal injuries, haemorrhage and extremity injuries. Anatomical location of genitourinary structures makes them vulnerable to injury with pelvic fracture. Incidence of sexual dysfunction varies in literature with 5% incidence of dysfunction in patients without urethral injury and 42% with urethral injuries. Hence in pelvic fracture, erectile dysfunction may be due to neurogenic, vascular, corporal and psychogenic injury. In this narrative review of targeted English literature from all level of evidences, which is written and supervised by experienced specialized orthopaedic, trauma and urology surgeons who were among the pioneers of conducting pelvis fracture management workshops in the country, we aim to describe the mechanism that can lead to erectile dysfunction after pelvic fracture, assessment principles, decision-making and preoperative planning and indications of operative managements.

Low cost reconstructive options after musculoskeletal tumour resection in developing countries. Affordable, effective and durable alternatives.

Over the last two, three decades, the overall survival rates for non-metastatic malignant tumours of the bone have dramatically improved. This has become possible due to the recent advances and multidisciplinary approach towards these diseases, specifically the advent of multi-agent chemotherapy and radiotherapy. Limb salvage has now become the norm in the treatment of musculoskeletal tumours without compromising on the overall survival and recurrence of the disease. In the era of metal, prosthetic reconstruction has become the standard procedure specifically in the large tumours which involve the joints as this method of reconstruction helps in joint mobility and early weight-bearing. Considering the costs and resource constraints, multiple cost-effective, stable, durable reconstruction options have evolved over the last decade and these have also shown favourable func tional outcomes without compromising on the amount of resection and risk of local recurrence. The current literature review was planned to discuss various cost-effective, durable reconstructive options and their advantages and disadvantages. These include Van ness rotationplasty, allograft, autograft, devitalised tumour bone and Masqueletor induced membrane technique . .

Management of stress fractures of the proximal tibia in patients with advance knee osteoarthritis. A case series.

To evaluate the outcome of one stage long stem total knee arthroplasty (TKA) of patients with stress fracture of the proximal tibia of the knee joint. Record of 15 patients, 14 females and one male who underwent one stage long stem TKAfrom the year January 2008 till December 2014 were reviewed retrospectively. Outcome variable was fracture healing which was seen clinically (pain free and postop ambulation) as well as radiologically (union of three out of four cortices). Mean age of the patients were 65 years and the mean BMI was 31. Of the 15 knees 13 had varus malalignment and 2 had valgus malalignment. The mean duration of fracture healing was four and a half months and the mean duration of follow-up was 26 months. All the patients were ambulated full weight bearing with walker. One patient had non-union at fracture site which required bone grafting. Long stem TKA is an effective method of treating tibial stress fractures associated with advance osteoarthritis as it not only restores the normal mechanical alignment but also facilitates fracture healing.

Benign tumours and tumour like lesions of bone.

Over the last century, there has been a remarkable development in the study of benign bone tumours. This is primarily due to the improved knowledge of the nature of these lesions and improved imaging technology. They present as a diverse group of clinical and pathological entities, which vary in their clinical behaviour and aggressiveness and, hence, multidisciplinary approach is necessary in their management. Combined opinion from an orthopaedic surgeon, radiologist and a pathologist is therefore required. Incidence of these tumours is debatable because they are often asymptomatic. Many protocols have been reported in studies with respect to the management of these tumours based on the experience of different centres and different surgeons with no set guidelines. English-language studies, including case reports, case series and systemic reviews, from PubMed, ERIC, MEDLINE, EMBASE and Cochrane Reviews databases from 2002 to 2016 were included in the current. Articles reporting all levels of evidence - Level I to V - were included.

Varied percutaneous and endovascular management of large iatrogenic Profunda Femoris artery pseudoaneurysm.

The case of an elderly male diagnosed with a large iatrogenic pseudoaneurysm arising from the profunda femoris artery on ultrasound examination is presented. The immediate postoperative period was uneventful; however later he developed leg swelling and mild oozing at the incision site. In view of deranged renal function CT angiogram was not done and decision was made to treat the pseudoaneurysm with percutaneous thrombin injection. Almost complete thrombosis was achieved however a small residual portion remained patent near the neck. Later limited contrast angiography was done which re-demonstrated a small anneurysm. Instead of embolizing the vessel completely repeat prolonged balloon inflation was done which later showed no further filling of the pseudoaneurysm.

Clinic-based biopsy with a small curette: is it a reliable and safe option in extremity tumours?

OBJECTIVE: To establish the diagnostic accuracy and safety of clinic-based biopsy done with a small curette. METHODS: The retrospective study was conducted at Aga Khan University Hospital Karachi and comprised data of patients who underwent biopsy procedure of extremity tumours in clinic under local anaesthesia from July 2009 to June 2012. Patients who underwent the procedure in operating room under general anaesthesia were excluded and so were those with insufficient or missing information, or those who had the final procedure done elsewhere. Clinical parameters were evaluated and histo-pathology was compared with the final resected specimen. RESULTS: The mean age of the 51 patients in the study was 32±19.6 years. Lower extremity was involved in 37(73%) cases, and the most common tumour was osteosarcoma in 9(17%).Biopsy was inconclusive in 2(4%) patients, leaving the clinical accuracy to be 94%. The cost of clinic-based biopsy was at least seven times less than those done in the operating room under general anaesthesia. CONCLUSIONS: Biopsy done in a clinic with a small curette is accurate, safe and cost-effective method.