Various Surgical Treatment of Hemophilic Pseudotumor : A Case Series.

BACKGROUND: Bleeding episodes in severe hemophilia may occur more frequently and spontaneously after mild trauma or daily activities. An inadequate treatment of that bleeding in hemophilia may result in pseudotumor, usually in the muscle adjacent to the bone. We reported haemophilic pseudotumor treated with various surgical interventions. METHODS: This study was conducted inthe Department of Orthopaedic and Traumatology at a government hospital over a period of 7 years(2010 -2017). Patients Perioperative management was done in accordance with the Integrated Hemophilia Team of our institution protocol.Diagnosis and management planning of hemophilic pseudotumor was confirmed via Integrated HemophiliaTeam meeting. After the surgery, all patients were asked to come for routine follow up. RESULTS: We reported six Haemophilia-A patients with pseudotumor in the pelvis, proximal femur and lower leg. One case in pelvic bone underwent hematoma evacuation, acetabular reconstruction using the Harrington procedure, and total hip arthroplasty.Two cases, a case in the proximal femur and another case in the distal fibula, were treated with amputation, other two cases, one was soft tissue psedotumor in the pelvic region and was treated by hematoma evacuation, and the remaining casewas managed with wide excision and followed by defect closure. CONCLUSION: Surgery is a preferable treatment for pseudotumors that have been present for years.It's associated with the best outcomes especially when selected as the primary line ofwith preventable and manageable bleeding complication. As previously published by many authors, this paper confirms that surgical excision is the treatment of choice but should only be carried out in major hemophilia centers by a multidisciplinary surgical team.

Current status of cancer care for young patients with nasopharyngeal carcinoma in Jakarta, Indonesia.

BACKGROUND: Nasopharyngeal carcinoma (NPC) is endemic in Indonesia and 20% of the patients are diagnosed before the age of 31. This study evaluates presentation and treatment outcome of young patients in Jakarta, in a tertiary referral centre. METHODS: Forty-nine patients under the age of 31, diagnosed with NPC between July 2004 and January 2007, were evaluated. Baseline data included histological type, stage of disease and presenting symptoms. We intended to follow all patients after diagnosis to reveal treatment outcome and overall survival (OS). RESULTS: All but two patients had advanced stage disease (94%), 7 (14%) had distant metastasis. The median interval between start of complaints and diagnosis was 9 months. Forty-two patients were planned for curative intent treatment. Eleven patients (26%) never started treatment, 2 patients did not complete treatment and 3 patients did not return after finishing treatment. Four patients died before radiation could start. Three patients died within 4 months after treatment. Nine patients (21%) had a complete response. Due to the high number of patients who were lost to follow-up (LFU), OS was analyzed as follows: a best-case (patients censored at last contact) and a worst-case scenario (assuming that patients who did not finish treatment or had disease at last contact would have died). The 2-year OS for patients without distant metastases was 39-71%. CONCLUSION: Treatment outcome for young patients with NPC in this institute was poor. Improvement can be achieved when NPC is diagnosed at an earlier stage and when there is better treatment compliance.

Computed tomography findings of retinoblastoma patients at Cipto Mangunkusumo Hospital Jakarta.

Aim As data of CT findings for retinoblatoma in Indonesia is not yet available, this study aimed to determine patient distribution and to describe CT fi ndings of RB patients at Cipto Mangunkusumo Hospital. Methods From April 2004 to August 2007, CT scans were performed on 64 new patients of RB with clinical fi ndings: leukocoria (19 patients), leukocoria with proptosis (41 patients), buphthalmus (2 patients) and red eyes (2 patients). The CT scan was performed using single slice CT scan and multislice CT scan with axial and coronal section. The cases were categorized into 3 groups: grade I: tumor confi ned to the globe, grade II: tumor extended to orbital soft tissue or involved the optic nerve, and grade III: tumor extended beyond the orbit or intracranial space. The CT features were evaluated in each group for the presence of calcifi cation, contrast enhancement and lateralization. Results Sixty four patients (30 females and 34 males) were evaluated. Age range: 3 months up to 9 years old; bilateral 20 patients, unilateral 44 patients. Six patients of 19 patients with clinical fi nding leukocoria were included in group I, and the remaining were included in group II. Twenty seven patients of 41 patients with proptotic eyes were included in group II, and the remaining were included in group III. Patients with clinical fi nding buphthalmus and red eyes were included in group II. All of the group I tumors (6 patients) showed calcification and enhancement. In group II (44 patients), calcification was detected in 41 patients, and there was no evidence of contrast enhancement in 6 patients. Group III (14 patients): no calcifi cation in 2 patients, all of the tumors showed enhancement. Conclusion Our study showed calcification in 92% of RB. Calcification was not a favorable prognostic sign, because calcification was detected in almost all of the extraocular and intracranial tumors. Tumor enhancement was not correlated with extra ocular tumor extension. The fi nding of leukocoria without proptosis could not exclude the presence of extraocular tumor extension. Suspicious intracranial invasion should be considered in proptotic RB patient. Most retinoblastoma cases in Indonesia are diagnosed at advanced stage, so that the objective of the therapy is life saving rather than visual salvation.