An Unusual Presentation of Kaposiform Hemangioendothelioma in the Mandibular Bone

Introduction A 4-year-old child presented with neck pain for several months which progressed to jaw pain and limitation in opening of the jaw. Eventual diagnosis of Kaposiform hemangioendothelioma is notable for the later age of presentation, the involvement of bone and soft tissue and the response to therapy. Case In March of 2020 child first developed sore throat and pain in his neck which led to limitation in neck movements. An enlarged tonsil was noted and treated. Symptoms improved only to recur and worsen by August 2020. Child was waking up with pain at night. Tonsils were removed in September to address this. Shortly thereafter, his mouth wouldn't open well. They saw several physicians without a definitive diagnosis for this including pediatrician, ENT and audiology. In late February 2021 ENT was re-consulted. A lytic lesion was discovered on X-ray. MRI further elucidated this as a destructive mass within the right mandibular ramus with soft tissue extension. A biopsy then characterized it as a Kaposiform Hemangioendothelioma. The biopsy showed a cellular vascular neoplasm forming variable small lobules with intervening fibrous tissue. The cells were monotonous and flat to spindled. Slit-like spaces and glomeruloid structures were seen. CD31 and D2-40 stains highlighted the endothelial cells and SMA stain pericytes. HHV8 stain was negative. No laboratory evidence of consumptive coagulopathy was noted. there was no evidence of Kasabach Merritt syndrome (KMP) Management Given the location of tumor and risks and morbidity of surgery, child was initiated on oral sirolimus aiming for a trough between 10 and 15 ng/ml and prednisone therapy with rapid taper in 4 weeks. Significant reduction in size of tumor was noted on serial scans. It measured 3.9 cm in craniocaudal, 2.8 cm in transverse, and 3.5 cm in AP dimension at diagnosis and reduced to 3.5 x 1.9 x 2.8 cm within 6 weeks. Child had improved mobility of jaw and improved speech. Nocturnal pain resolved on steroids but recurred off steroids. Aspirin was added to the treatment when steroids were weaned and helped resolve the pain. Hypercholesterolemia was noted on sirolimus, but otherwise very well tolerated at this dose. Clinical exam and serial MRIs have shown continued response to therapy. Summary We present this case as a rare presentation in an older child with the mandibular bone as the primary site. Kaposiform hemangioendotheliomas are very rare, and usually seen in the newborn or early infancy. The occurrence later in life with bone and adjacent soft tissue primary and cervicofacial location is more rare. Although 70% of KHE are complicated by Kassabach Merritt phenomenon, the older age and location in bone are features that have been associated with lack of KMP in larger series. In many cases surgical management would be preferred, but given the location of this tumor, we opted for medical therapy with sirolimus and prednisone. The impressive response to therapy is encouraging and makes us hopeful that we may decrease size such that surgery is less morbid or avoid surgery entirely. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

Translation, adaptation and validation of the Bulgarian version of the King’s Parkinson’s Disease Pain Scale

Background The purpose of the present study was to translate and cross-culturally adapt the King’s Parkinson’s Disease Pain Scale (KPPS) into Bulgarian and to investigate its psychometric properties in order to provide a validated Parkinson’s disease-specific pain instrument in Bulgarian language (KPPS-BG). Methods Translation into Bulgarian and a cultural adaptation were performed to obtain KPPS-BG. A total of 162 patients with idiopathic Parkinson’s disease were screened for pain using the complementary to the KPPS questionnaire – King’s Parkinson’s Disease Pain Questionnaire (KPPQ). KPPS-BG domain and total scores were calculated and internal consistency, construct validity and test-retest reliability were examined for 129 patients having one or more positive items in the KPPQ-BG. Results 79.6 % of the patients reported one or more types of pain. The most common type was musculoskeletal pain (83.7 %), followed by nocturnal pain (55.0 %), fluctuation-related pain (50.1 %), radicular pain (43.4 %), chronic pain (31.0 %), discoloration, edema/swelling (27.1 %) and, oro-facial pain (14.3 %). Mean KPPS-BG total score was 21.1 ± 17.3 SD. KPPS-BG showed a good reliability (Cronbach’s alpha 0.75). The test-retest reliability of the KPPS-BG was high and the intraclass correlation coefficient was 0.92, demonstrating а good repeatability. KPPS-BG total score was higher in patients with postural instability gait difficulty motor subtype, compared to tremor-dominant or indeterminate subtype. Significant positive correlations were found between KPPS-BG total score and modified H&Y, Movement Disorders Society Unified Parkinson’s Disease Rating Scale part III. Conclusions The KPPS-BG constitutes a reliable, comprehensive and useful tool for pain assessment in native Bulgarian patients with Parkinson’s disease.

Osteoid Osteoma: A Unique Presentation in a Child’s Lesser Toe

Introduction. Osteoid osteoma is an uncommon, small, benign, self-limiting, and usually painful tumor of the skeleton. Diagnosis can be straightforward if seen in the usual locations as the femur and the tibia in young adults, who present with nocturnal pain, alleviated by salicylates. The diagnosis can be more challenging in the spine, pelvis, hand, or feet. Case Report. We report the case of an 11-year-old boy who was treated symptomatically for a painful toe since 10 months, without a definitive diagnosis. X-ray, MRI, and scintigraphy, along with the typical nocturnal pain and swelling of the toe, suggested an osteoid osteoma, confirmed by histology after excisional biopsy of the lesion. Conclusion. Osteoid osteoma should always be included in the differential diagnosis when it comes to nocturnal pain without systemic signs, even in unusual places in children. The awareness should lead to a prompt diagnosis and treatment.

Radiofrequency Ablation of Osteoid Osteoma of Neck of Femur: A Case Report

Osteoid osteoma is the most common benign bone forming tumor characterized by a nidus surrounded by reactive sclerotic bone typically associated with nocturnal pain and most common in males less than 30 years. Diagnosis may be difficult in cases of atypical presentation, intraarticular localizations or very small size. Computed tomography guided radiofrequency ablation is one of the promising treatment methods being used with an advantage of minimal invasion, faster recovery and shorter hospitalization. We present a case of an 8-year-old boy with osteoid osteoma of neck of femur managed successfully with radiofrequency ablation. To our knowledge, this is the first case reported on radiofrequency ablation in Nepal.

Translation, adaptation and validation of the Bulgarian version of the King’s Parkinson’s Disease Pain Scale

Introduction:The purpose of the present study was to translate and cross-culturally adapt the King's Parkinson's Disease Pain Scale (KPPS) into Bulgarian and to investigate its psychometric properties in order to provide a validated Parkinson’s disease-specific pain instrument in Bulgarian language (KPPS-BG).Methods:Translation into Bulgarian and a cultural adaptation were performed to obtain KPPS-BG. A total of 162 patients with idiopathic Parkinson’s disease were screened for pain using the complementary to the KPPS questionnaire-King’s Parkinson's Disease Pain Questionnaire (KPPQ). KPPS-BG domain and total scores were calculated and internal consistency, construct validity and test-retest reliability were examined for 129 patients having one or more positive items in the KPPQ-BG.Results:79.6% of the patients reported one or more types of pain. The most common type was musculoskeletal pain (83.7%), followed by nocturnal pain (55.0%), fluctuation-related pain (50.1%), radicular pain (43.4%), chronic pain (31.0%), discoloration, edema/swelling (27.1%) and, oro-facial pain (14.3%). Mean KPPS-BG total score was 21.1±17.3 SD. KPPS-BG showed a good reliability (Cronbach's alpha 0.75). The test-retest reliability of the KPPS-BG was high and the intraclass correlation coefficient was 0.92, demonstrating а good repeatability. KPPS-BG total score was higher in patients with postural instability gait difficulty motor subtype, compared to tremor-dominant or indeterminate subtype. Significant positive correlations were found between KPPS-BG total score and modified H&Y, Movement Disorders Society Unified Parkinson’s Disease Rating Scale part III.Conclusion:The KPPS-BG constitutes a reliable, comprehensive and useful tool for pain assessment in native Bulgarian patients with Parkinson’s disease.

Neuropathic pain: a principal component of pain that should not be neglected in ankylosing spondylitis

Background Pain in AS is currently considered an inflammatory pain (IP). However, it was found that some patients still had the sensation of pain even without inflammation. Our study was to investigate whether ankylosing spondylitis (AS) pain has a neuropathic pain (NeP) component. Methods The study consisted of three parts. The first included 182 AS patients to assess neuropathic pain in AS patients. The second included63 patients to evaluate pain improvement after etanercept therapy. The third included 20 AS patients and 10 healthy controls (HCs) to detect serum neurotransmitters. Results We found out that 55 paitents (30.21%) had possible NeP, and 14 (7.70%) had definite NeP. There were significant differences between the groups with respect to nocturnal pain (NP), peripheral pain (PP), total back pain (TBP), BASDAI, ASDAS-CRP, HAD-A, HAD-D and BASDAI-fatigue but not CRP concentrations. Principal component analysis (PCA) of AS pain revealed that the weight of NeP was greater than PP in the first principal component (0.703 vs 0.639). Structural equation modelling (SEM) revealed that NeP altered disease activity (β = 0.62, P < 0.001), which influenced psychological status (β = 0.42, P < 0.001). Of 63 patients who used etanercept for 3 months, significant improvement was found in NP, TBP and PP (all P < 0.0001) but not in PDQ (10.60 ± 6.85vs 9.98 ± 6.40, P = 0.067). Serum norepinephrine concentrations in patients with positive PDQ were greater than those in patients with negative PDQ and HC. Conclusions We conclude that NeP contributes to pain in AS patients.

Triquetral Osteoid Osteoma: A Diagnostic Dilemma

Background: Osteoid osteoma (OO) is a benign osteoblastic neoplasm, mostly occurring in long bones of lower extremity. Its manifestation in unusual locations can be a diagnostic challenge and distressing for patients. OO in carpal bones is uncommon; its occurrence in triquetrum is even more so. Case Presentation: This study reports on a nineteen-year-old female with history of wrist pain for 4 years. She had sustained trauma to the same wrist before commencing of pain. Patient's pain was unresponsive to conservative treatments. Her routine x-rays were mostly interpreted as normal and obtaining further imaging helped us in diagnosis of OO of triquetrum. Surgical resection of the nidus was performed. Conclusions: Manifestation of OO in small carpal bones may not be typical; identifying the lesion or nidus could be challenging. Whatever unusual the location or presentation of such lesions may be, obtaining advanced imaging can be of significant help; therefore, we suggest clinicians to have low threshold in ordering advanced imaging specifically in patients suffering from chronic pain. Also, persistent history of nocturnal pain is a sign which is worth attention.

THU0453 THIRD OCCIPITAL NERVE RADIO-FREQUENCY UNDER FLUOROSCOPIC GUIDANCE IN MANAGEMENT OF CERVICOGENIC HEADACHE IN RHEUMATOID ARTHRITIS

Background:Rheumatoid arthritis is a common type of autoimmune arthritis characterized by chronic inflammation. Cervical spine is often affected specially in long lasting diseaseObjectives:Evaluate efficacy of Third occipital nerve Radiofrequency under fluoroscopic guidance to treat refractory cervicogenic headache in RA patients.Methods:The current study was revised and approved from the local ethical committee of Faculty of Medicine; Assiut University, then registered in the clinical trials under the number ofNCT03852355. Inclusion criteria included, Patients who fulfilled the American College of Rheumatology (ACR) (2010) criteria for RA and suffering from upper neck pain and/or headache due to bilateral 3rd occipital nerve involvement, excluding other local cervical spine pathologies was confirmed by MRI and previously failed conservative treatment for at least three months prior to enrollment. Sixty adult patients were randomly assigned to one of the two studied groups Group 1 (RF, n = 30), received bilateral Third occipital nerve Radiofrequency under fluoroscopic guidance or Group 2 (control group, n = 30), received oral prednisolone 10 mg/day. The two groups were then followed-up with neck disability index (NDI), nocturnal neck pain VAS score and headache score every two weeks for three months. Sleep disturbance, sleep disability index were reassessed six months post intervention. Post interventional assessment was done by pain physician who were kept blind to the grouping process.Results:Neck disability index (1ry outcome), Nocturnal pain VAS, and severity of headache showed significant differences during the whole post-interventional study period. The patients in RF group demonstrated significant improvement of pain in comparison to baseline value over the whole six months with p-value < 0.001 as regard to the fore-mentioned three parameters. On the other aspect, the control group patients showed significant improvement in comparison to its baseline value after the 2nd, 12th and 24th weeks only as follows: (0.001,0.003, 0.003 for the NDI) (p values of 0.02,0.01, 0.01 for the nocturnal pain VAS), (0.001 0.009, 0.005 for the headache VAS severity.Conclusion:Radiofrequency of 3rd occipital nerve is effective in treatment of refractory cervicogenic headache in RA.Disclosure of Interests: :None declared