P085 Chronic recurrent multifocal osteomyelitis: about 2 cases

Background Chronic recurrent multifocal osteomyelitis (CRMO) also known as aseptic osteomyelitis is a rare auto-inflammatory disease with an incidence estimated at 4/100 000 population [1]. The aim of our work was to report two cases of CRMO that illustrate challenges in the diagnosis of this rare disease. Method We report the case of two patients diagnosed with CRMO. Clinical, biological and radiological data as well as disease outcomes were described. We also collected data about treatment modalities. Results Two patients aged of 7 and 10 years respectively, without any notable pathological history, presented recurrent episodes multifocal painful swelling of limbs. In the first case, the symptoms concerned the left ankle and knee as well as the left hip, all associated with lameness and an altered general condition, with neither fever nor skin manifestations. In the second case, the swelling involved the right shoulder, right hip and the left ankle. There was no elevated CRP or ESR in any of patients. Immunological status (RF, anti-CCP, AAN) as well as the HLA-B27 antigen test were negative. In the first patient, standard radiographs showed lytic lesions of the proximal metaphysis of the tibia, the greater trochanter and the left lateral malleolus. MRI of the pelvis, knee, and sternum of the first patient revealed edematous involvement of the left greater trochanter, the right ilium, the proximal metaphyseal region of the tibia and the right edge of the sternum, whereas in the second patient, a whole-body MRI showed inflammatory signs over the left greater trochanter, the insertion of the gluteus medius and obturator externus, right trochanteric bursitis and oedema of the entire right ilium. In the first patient, bone scintigraphy showed intense uptake of radioisotopes in the left ilium, the 7 th right costo-vertebral junction, the trochanteric mass, the upper end of the tibia and the lower end of the left fibula. Bone biopsy showed bone remodeling in both cases without evidence of infection or tumor. The diagnosis of CRMO was retained, supported by the prompt response to NSAIDs and short-term corticosteroid therapy. However, the second patient presented, 8 years later, pain in the sterno-clavicular joint as well as the right hip. A relapse of the disease was confirmed by MRI. Therapeutic escalation with zoledronic acid 0.025 mg/kg intravenous infusion every six months allowed the resolution of the symptoms. Conclusion These observations illustrated a rare disorder in children, characterized by lytic lesions predominantly in the metaphysis of long bones. Bone scintigraphy allowed an early assessment of disease extension and histological examination ruled out a malignant tumor and an infection. The first-line treatment is anti-inflammatory drugs. In case of failure, bisphosphonates seem to be effective.

Maffucci Syndrome Complicated by Giant Chondrosarcoma in the Left Ankle with an IDH1 R132C Mutation: Case Report

Background: Maffucci syndrome is a rare, nonhereditary congenital mesodermal dysplasia characterized by multiple enchondromas and hemangiomas. It is associated with an increased risk of the development of malignant tumors. We present a case of 45-year-old man with Maffucci syndrome to supplement the clinical manifestations and explore the molecular mechanism of Maffucci syndrome.Results: The patient was underwent amputation surgery to inhibit tumor development and diagnosed as Maffucci syndrome with 1-2 grade giant chondrosarcoma in the left ankle. In addition, the whole exon analysis by Next Generation Sequencing revealed isocitrate dehydrogenase 1 R132C mutation in chondrosarcoma lesions but not in blood DNA. Conclusions: This case report presents the genetic evidence for the inclusion of chondrosarcoma among tumors characterizing Maffucci syndrome. Consequently, it is suggested that patients with Maffucci syndrome should be followed up more actively to exclude neoplasms due to IDH1 R132C somatic mutation.

Reliability of the weight-bearing ankle dorsiflexion range of motion measurement using a smartphone goniometer application

Background Weight-bearing ankle dorsiflexion range of motion measurement (weight-bearing lunge test) is gaining in popularity because it mimics lower extremity function in daily physical activities. The purpose of the study is to assess the intra-rater and the inter-rater reliability of the weight-bearing ankle dorsiflexion range of motion measurement with a flexed knee using a smartphone application Spirit Level Plus installed on an Android smartphone. Methods Thirty-two young, healthy subjects participated in the study and were measured in four sessions by two examiners. One measurement was taken on each ankle in every session. Eight measurements were taken from each participant. A total of 256 were taken from all the participants. The measurements for the individual subject were repeated no sooner than 24 hours after the first session. In order to assess the reliability, intraclass correlation coefficients (ICC), standard error measurements (SEM) and minimal detectable change (MDC) at the 95% confidence interval were calculated. Results Statistical data analysis revealed moderate intra-rater reliability for the right ankle (ICC = 0.72, 95% CI [0.49–0.85]) and good intra-rater reliability for the left ankle (ICC = 0.82, 95% CI [0.66–0.91]). Inter-rater reliability is moderate for the right (ICC = 0.73, 95% CI [0.52–0.86]) and the left ankle (ICC = 0.65, 95% CI [0.39–0.81]). Conclusion The observed method is moderately reliable and appropriate when the main objective is to assess ankle dorsiflexion mobility in weight-bearing when weight-bearing is not contraindicated. The concurrent validity of the Spirit Level Plus application is excellent.

Management of Neglected Peroneal Tendon Dislocation of Iatrogenic Etiology: A Case Report

Introduction:Neglected peroneal tendon dislocation with iatrogenic etiology has been rarely reported in the literature and its management has not been fully understood to date. Case Report:We present a case of a 25-year-old male who presented with pain over the posterolateral aspect of his left ankle which was diagnosed to be a case of neglected peroneal tendon dislocation of iatrogenic etiology. Peroneal groove deepening with superior retinaculum repair was done in the patient along with loose body removal and osteophyte excision. Subsequent fibrosis augmented with the deepening of the groove maintained peroneal tendon position in the retromalleolar groove. On post-operative follow-up, the patient was completely satisfied with relief of pain and no complications. He also regained full range of motion and could walk without support. Conclusion:Surgical intervention of fibular groove deepening with superior peroneal retinaculum reconstruction results in an excellent outcome for neglected peroneal tendon dislocation Keywords:Peroneal tendon, Superior peroneal retinaculum, Fibular groove.

Tissue Is the Issue - A Case of Malignant Insulinoma

Introduction: Insulinomas are rare, functioning pancreatic neuroendocrine tumors, predominantly benign (90%). Most are sporadic, but they can occur as part of inherited disorders. Large size insulinomas are more likely to be malignant. 68Gallium (Ga)-DOTATATE scan is an essential tool in diagnosis and treatment of pancreatic neuroendocrine tumors. However, tissue diagnosis is the ultimate prognosis predictor and treatment guide. Clinical Case: An 80-year-old man presented with left ankle fracture after a syncopal episode. In the past several months he reported multiple episodes of lightheadedness. He denied history of diabetes. Past medical and surgical history included prostate cancer, gastroesophageal reflux disease and bilateral orchiectomy for undescended testes. His left ankle appeared swollen, otherwise the exam was unremarkable: he was well-nourished, the abdomen was soft and nontender, there were no palpable masses. He was noted to have several episodes of hypoglycemia. Hypoglycemia work up included negative sulfonylurea screen, plasma glucose 51 mg/dl (60-99 mg/dl), insulin of 31 uIU/ml (3-25 uIU/ml), proinsulin 85.4 pmol/L (<8 pmol/L), C peptide 3.4 ng/ml (1.1-4.4 ng/ml). Abdominal Computed Tomography with contrast showed a 4.7 x 3.1 cm exophytic mass in the body of pancreas, a 5.3 x 5 cm mass in the head of pancreas and multiple ill-defined hepatic lesions concerning for metastatic disease. 68Ga-DOTATATE scan revealed an avid pancreatic mass and multiple avid foci throughout the liver suspicious for metastatic disease. Octreotide and intravenous dextrose were started for the treatment of persistent hypoglycemia. He underwent Whipple procedure and resection of the metastatic liver lesions. Postoperatively hypoglycemia resolved. The pathology revealed large cell pancreatic neuroendocrine carcinoma with areas of well differentiated tumor and multiple liver metastatic neuroendocrine carcinoma, Ki-67 of 50-80% was reported in the primary tumor and metastatic lesions. Discussion: Metastatic insulinomas carry a significant morbidity and mortality risk. Surgical resection to decrease tumor burden can reduce the risk of hypoglycemia. Histopathology is essential in treatment decision making. Surprisingly our patient’s pathology revealed poorly differentiated neuroendocrine carcinoma, changing the prognosis and treatment. Treatment with platinum-based chemotherapy and etoposide is the standard of care for aggressive neuroendocrine neoplasms. Despite hypoglycemia resolution following surgery and good DOTATATE uptake by the well differentiated neuroendocrine tumor portion, our patient’s prognosis remained poor in view of the high-grade carcinoma. Due to complicated hospital course, and decreased functional status patient was not a candidate for chemotherapy immediately post hospital discharge.

Extra-articular distal tibia fibula fracture managed with single posterolateral approach: a case report

<p class="abstract">Extra-articular distal tibia fractures involve distal tibia approximately 4 cm within tibia plafond with no articular extension. The proper preoperative care, planning and selection of surgical approach is very essential to prevent postoperative wound-related complications. We present a case of a 29 year female patient, presented with left ankle pain and swelling with a wound over the medial aspect of the ankle. X-ray of the left ankle showed extra-articular distal tibia fibula fracture with no neurovascular deficit. We managed both the fractures with open reduction and internal fixation using a single posterolateral approach. At present 1 year follow-up, the patient is having a good range of ankle motion with radiological union with no implant failure and wound-related complications. Extra-articular distal tibia fibula fracture fixation using single posterolateral approach is a viable alternative approach to medial or anterolateral approach in cases of medial or anterior soft tissue problems. It helps in getting a better functional outcome, early mobilisation with less wound-related complications.</p>

644 Use of Plantar Foot Plate Splints and High-Profile Leg Net Devices for Prevention of Plantarflexion Contracture following Placement of Cultured Epidermal Autograft to Lower Extremities

Introduction Burn Therapists strive to prevent burn scar contracture through positioning strategies beginning in the acute phase of burn injury. This task is even more challenging when paired with posterior offloading and joint immobilization required for the viability of cultured epidermal autograft (CEA). High profile leg net devices are the standard for posterior offloading after application of CEA circumferentially to lower extremities but can result in poor positioning of the ankle. Custom foot plate splints were designed and fabricated to preserve ankle dorsiflexion during the initial stages of CEA healing. Methods The high-profile leg net devices were assembled using 3/4 inch PVC piping and PVC fittings (45 degrees, 90 degrees, and tees) with double layered elastic tubular netting to allow proper wound ventilation while supporting the lower extremity with the patient in supine. The plantar foot plates were custom molded to the patient’s foot using thermoplastic material and lined with medium density temper foam for pressure relief. The foot plate was attached to the frame using Velcro and straps. Instructions with photographs were posted in the patient’s room for nursing staff to reference. Netting was exchanged daily and frames were disinfected using standard techniques. Results Goniometric measures were taken for ankle dorsiflexion were taken on day of CEA application with lower extremities positioned on high profile nets (in alignment with cutaneous functional unit modified position): -6 degrees right ankle, -2 degrees left ankle. Repeat measures were taken after one week period of bilateral lower extremity immobilization per CEA protocol: -1 degree right ankle, 2 degrees left ankle. One month follow-up at the discontinuance of leg net devices showed bilateral ankle dorsiflexion preserved with 3-degree right ankle dorsiflexion and 5 degrees on the left. Conclusions The use of custom foot plates on high profile leg net devices appears to improve ankle dorsiflexion range of motion while maintaining adequate posterior offloading required for CEA precautions for a burn survivor with extensive lower extremity burn wounds.

Selective inhibition of adenylyl cyclase subtype 1 reduces inflammatory pain in chicken of gouty arthritis

Lack of uricase leads to the high incidence of gout in humans and poultry, which is different from rodents. Therefore, chicken is considered to be one of the ideal animal models for the study of gout. Gout-related pain caused by the accumulation of urate in joints is one type of inflammatory pain, which causes damage to joint function. Our previous studies have demonstrated the crucial role of calcium-stimulated adenylyl cyclase subtype 1 (AC1) in inflammatory pain in rodents; however, there is no study in poultry. In the present study, we injected mono-sodium urate (MSU) into the left ankle joint of the chicken to establish a gouty arthritis model, and tested the effect of AC1 inhibitor NB001 on gouty arthritis in chickens. We found that MSU successfully induced spontaneous pain behaviors including sitting, standing on one leg, and limping after 1–3 h of injection into the left ankle of chickens. In addition, edema and mechanical pain hypersensitivity also occurred in the left ankle of chickens with gouty arthritis. After peroral administration of NB001 on chickens with gouty arthritis, both the spontaneous pain behaviors and the mechanical pain hypersensitivity were effectively relieved. The MSU-induced edema in the left ankle of chickens was not affected by NB001, suggesting a central effect of NB001. Our results provide a strong evidence that AC1 is involved in the regulation of inflammatory pain in poultry. A selective AC1 inhibitor NB001 produces an analgesic effect (not anti-inflammatory effect) on gouty pain and may be used for future treatment of gouty pain in both humans and poultry.