Aggressive fibromatosis in pediatric population: A case series

Introduction: Aggressive fibromatosis or Desmoid fibromatosis is a benign tumor which originated form mesenchymal tissues of the body. This tumor has strong potential of recurrence and infiltration but it does not metastasize to other organs of the body. This case series is focused to determine the treatment outcomes for pediatric patients of aggressive fibromatosis. Methodology: It is a retrospective case series conducted on 7 patients presented to section of Orthopedics, department of surgery of our institute in Karachi. We included all the cases of pediatric patients from 1 to 16 years, with biopsy proven fibromatosis from January 2000 to December 2015. Presentation of Cases: Out of 7 pediatric patients, there were 6 (85.7%) males and 1 (14.3%) female patient. The median age was 6 years IQR (5-11) years. Gluteal region was the most common site of disease. Four patients (57.1%) had positive tumor margins while three (42.9%) had negative margins. Out of 7 patients, 4 patients (57.1%) had recurrent disease and they had positive margins. The median Disease Free survival time was 14 months and there was no expiry of patients. Conclusion & Recommendation: The conclusion of our study was that aggressive fibromatosis is more prevalent in children below 15 years of age and disease burden is higher in male gender. Positive margins after surgery indicate a high risk for disease recurrence therefore; primary surgery with negative margins is the treatment of choice for children with AF. However, we recommend that multicenter trials should be conducted in the future to clarify the role of adjuvant treatment for patients with pediatric AF

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

Malignant distal femur tumors management in children, low cost innovations with affordable care in a tertiary care hospital, a cross sectional study

Introduction: Malignant musculoskeletal tumors in children are mostly comprised of Osteosarcoma and Ewing\u27s sarcoma and distal femur is the most common site for primary bone tumors. This study examined the surgical management outcomes of pediatric patients (\u3c18 years) presented in our setup with malignant distal femur tumors.Methodology: We retrospectively reviewed the medical records of patients diagnosed with malignant sarcomas of distal femur and younger than 18 years of age who underwent limb salvage surgery during June 2009-June2017.Results: There were 31 pediatric patients who had distal femur malignant tumors and out of them 20 patients were selected who underwent limb salvage as a primary surgery. The mean age (range) of patients was 11.90 (6-17) years. Seventeen had osteosarcoma and three had Ewing\u27s sarcoma. In surgery all patients were offered reconstruction. Post-operative complications were observed in (50%) patients while other 50% patients had no complications. Wound infection, flap necrosis, broken plates with non-union and recurrence of disease were the reported complications. Revision surgery was performed in 10 patients out of 20 patients.Conclusion: Salvage was the primary option in our surgery and revision surgery was performed in the cases due to complications. Small sample size was the limitation of the study

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

Quantitative gait analysis as a method to assess mechanical hyperalgesia modulated by disease-modifying antirheumatoid drugs in the adjuvant-induced arthritic rat

In the present study, azothioprine, chloroquine, D-penicillamine, methotrexate and sodium aurothiomalate (gold salt) were evaluated for possible disease-modifying effects in the adjuvant-induced arthritis model of human rheumatoid arthritis in rats. Gait analysis was used to examine the role of disease-modifying antirheumatic drugs in the development of pain. Body weights were also measured to monitor the progression of disease and the systemic antiarthritic effects of the test compounds used in this study, as well as their systemic toxicity. Our results showed that azothioprine (5 mg/kg/day), chloroquine (12.5 mg/kg/day), sodium aurothiomalate (2.5 mg/kg/day) and methotrexate (1 mg/kg/week) not only inhibited the macroscopic changes such as erythema and swelling of limbs, but also exhibited significant reversal of gait deficits seen in the untreated or saline-treated arthritic rats. No reduction in the body weights were observed in the arthritic rats treated with azothioprine, chloroquine, sodium aurothiomalate and methotrexate. D-Penicillamine (12.5 mg/kg/day), however, showed a significant reduction (P < 0.03) in the body weights of the arthritic rats over a period of 22 days; furthermore, it was unable to show any reduction in arthritic score (P < 0.1). In earlier experiments, chloroquine and methotrexate failed to suppress carageenan-induced edema, suggesting that the mode of antiarthritic action may be different from those of nonsteroidal anti-inflammatory agents. Since these disease-modifying antirheumatic drugs are reported to have an immunomodulatory role, especially the gold salt, which influences the monocyte–macrophage system, it is suggested that the observed antiarthritic effects of disease-modifying antirheumatic drugs may be partly attributed to their immunomodulatory activity

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

Hip replacement for femur neck fracture in the elderly, effects of delayed surgical intervention on morbidity and mortality, a retrospective comparative study

Hip fracture is one of the most common injuries in the elderly population. Delay in operating on patients with hip fracture is associated with greater mortality and morbidity. A retrospective review of medical charts of patients who underwent primary total hip replacement (THR) for neck of femur fractures at our tertiary care level 1 trauma was carried out. Data was collected from the patients\u27 charts and analysed for 30-day mortality and morbidity. A total of 96 patients were included in the study. Out of the 36 patients in the delayed THR group, mortality within 30 days was observed in 4 (11.1%) patients while none was noted in the early THR group. The difference was statistically significant with a P-value of 0.008. With regards to post-operative complications, significantly higher percentage of patients were noted to have developed electrolyte imbalances (P = 0.003), postoperative psychosis (P = 0.02), and acute kidney injury (AKI) (P = 0.02) in the delayed THR group compared to the early THR group. Delay in surgery for neck of femur fracture is associated with increased 30-day mortality and postoperative complications

The frequency of vitamin B12 derangements in females presenting for workup of iron deficiency anemia

Objective: Iron deficiency anemia (IDA) is the most prevalent hematological disorder in women of child-bearing age. IDA poses a threat to feto-maternal wellbeing. It is the leading cause of morbidity and mortality in pregnancy. IDA in young females can coexist with other nutritional deficiencies such as vitamin B12 (Vit B12). An undetected Vit B12 deficiency carries a greater likelihood of development of maternal neuropsychiatric disorders and neural tube defects in the fetus. Materials and methods: Venous blood samples of non-pregnant female population (N=133) from Fauji Foundation Hospital, Rawalpindi were collected and analyzed for complete blood count (CBC), Vit B12 and serum Ferritin. Population was further subdivided into two groups. Group A, included females with low serum Ferritin and low MCV, group B restricted to normal serum Ferritin and low MCV, whereas control group had females with normal serum Ferritin and MCV levels. Results: Demographic data suggested that only height was slightly lower in IDA patients other than reduced serum Ferritin levels. Group A also showed reduced levels of Vit B12 as compared to group B and control group. CBC analysis indicated marked reduction in all hematological parameters except platelet count in group A. Conclusion: Our findings suggest that IDA and Vit B12 deficiency may coexist in young females. Monitoring Vit B12 along with iron deficiency parameters is essential to treat anemia and prevent gynecological complications. Paucity of information on their coexistence in females of childbearing age require further investigation. Keywords: Vitamin B12, Iron deficiency anemia, Microcytosis, Serum Ferritin

Clinicopathological prognostic factors of oral squamous cell carcinoma: An experience of a tertiary care hospital

Locoregional recurrence accounts for majority of the treatment failures in oral cancer patients. Current study aimed to determine the predictors of recurrence and survival in patients with biopsy proven Squamous Cell Carcinoma (SCC) of the oral cavity. This study included 88 patients of squamous cell carcinoma treated at our institution from 2007 till 2013. Primary intervention was surgery in all patients with radiation and chemotherapy in selected patients. Primary end point was locoregional recurrence, distant metastasis and death. Out of 88 patients, 23 (26.1%) patients developed locoregional recurrence and 6 (6.8%) patients developed distant metastasis. Overall survival rate was 77.3%. Follow up ranged from 1 month to 63 months with mean of 17.8±16.2. On multivariate analysis, lymph node involvement and loco-regional recurrence were independent parameters related to decrease overall survival. Lymphovascular invasion, perineural spread, TNM stage and lymph node involvement had significant impact on recurrence