Impact of the HEAD-US Scoring System for Observing the Protective Effect of Prophylaxis in Hemophilia Patients: A Prospective, Multicenter, Observational Study

Objective: This study aimed to observe the preventive effect of prophylactic treatment on joint health in people with hemophilia (PwH) and to investigate the importance of integration of ultrasonographic examination into clinical and radiological evaluation of the joints. Materials and Methods: This national, multicenter, prospective, observational study included male patients aged ≥6 years with the diagnosis of moderate or severe hemophilia A or B from 8 centers across Turkey between January 2017 and March 2019. Patients were followed for 1 year with 5 visits (baseline and 3th, 6th, 9th, and 12th month visits). The Hemophilia Joint Health Score (HJHS) was used for physical examination of joints, the Pettersson scoring system was used for radiological assessment, point-of-care (POC) ultrasonography was used for bilateral examinations of joints, and the Hemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) score was used for evaluation of ultrasonography results. Results: Seventy-three PwH, of whom 62 had hemophilia A and 11 had hemophilia B, were included and 24.7% had target joints at baseline. The HJHS and HEAD-US scores were significantly increased at the 12th month in all patients. These scores were also higher in the hemophilia A subgroup than the hemophilia B subgroup. However, in the childhood group, the increment of scores was not significant. The HEAD-US total score was significantly correlated with both the HJHS total score and Pettersson total score at baseline and at the 12th month. Conclusion: The HEAD-US and HJHS scoring systems are valuable tools during follow-up examinations of PwH and they complement each other. We suggest that POC ultrasonographic evaluation and the HEAD-US scoring system may be integrated into differential diagnosis of bleeding and long-term monitoring for joint health as a routine procedure.

Early Detection of Central Nervous System Relapse of Pediatric Leukemia with Measurement of Optic Nerve Sheath Diameter on MRI.

BACKGROUND: Leukemia is the most common pediatric malignancy. Central Nervous System (CNS) is the most frequently involved extramedullary location at diagnosis and at relapse. OBJECTIVE: To determine if Magnetic Resonance Imaging (MRI) findings of optic nerves should contribute to early detection of CNS relapse in pediatric leukemia. METHODS: Twenty patients (10 boys, 10 girls; mean age 8,3 years, range 4-16 years) with proven CNS relapse of leukemia followed up between 2009 and 2017 in our institution were included. Orbital MRI exams performed before and during CNS relapse were reviewed retrospectively. Forty optic nerves with Optic Nerve Sheaths (ONS) and Optic Nerve Heads (ONH) were evaluated on fat-suppressed T2-weighted TSE axial MR images. ONS diameter was measured from the point 10 mm posterior to the globe. ONS distension and ONH configuration were graded as 0, 1 and 2. RESULTS: Before CNS relapse, right mean ONS diameter was 4.52 mm and left was 4.61 mm which were 5.68 mm and 5.66 mm respectively during CNS relapse showing a mean increase of 25% on right and 22% on left. During CNS relapse, ONS showed grade 0 distension in 15%, grade 1 in 60%, grade 2 in 25% and ONH demonstrated grade 0 configuration in 70%, grade 1 in 25% and grade 2 in 5% of the patients. CONCLUSION: MRI findings of optic nerves may contribute to diagnose CNS relapse by demonstrating elevated intracranial pressure in children with leukemia.

Recurrent Chronic Subdural Hematoma: Report of 13 Cases.

Chronic subdural hematoma is a frequent type of hemorrhage, which terminates with mortality if not diagnosed and treated early. The aim of this clinical study is to evaluate the patients with unilateral and bilateral recurrent chronic subdural hematoma. The study group consisted of 13 cases with unilateral and bilateral recurrent chronic subdural hematomas who underwent aggressive wide craniotomy, duraectomy, inner and outer membranectomy, dural border coagulation, incision through cortical vein trace and hang up of dural edge, between 2009 - 2016. All of our patients were diagnosed by preoperative Magnetic Resonance Imaging. We evaluated the age, gender, complaints and neurologic signs, localization and thickness of the hematoma. We can estimate that wide craniotomy, duraectomy and membranectomy is a good option in preventing recurrent chronic subdural hematoma and complications.

Computed tomography imaging of acute gastrointestinal graft-versus-host disease after haematopoietic stem cell transplantation in children.

AIM OF THE STUDY: To evaluate computed tomography (CT) findings of gastrointestinal graft-versus-host disease (GI-GVHD) occurring in children after haematopoietic stem-cell transplantation (HSCT). MATERIAL AND METHODS: From February 2013 to May 2018, 225 paediatric patients underwent HSCT. Sixty-eight patients (30%) presented with clinical diagnosis of acute GI-GVHD in the first 100 days after HSCT. Thirty-five (18 girls, 17 boys; age range, 2-18 years; mean age, 10.3 years) of 68 patients had abdominopelvic CT and included in study. RESULTS: Intestinal CT abnormalities were present in 33 (94%) and extra-intestinal CT findings were in 30 (86%) patients. Thickening of the bowel wall was the most common finding (31 patients, 89%), which involved the small bowel in 29 patients (83%), colon in 16 patients (46%), and both in 15 patients (43%). Oesophageal wall thickening was present in three patients (9%), and gastric wall thickening was in eight patients (23%). Bowel dilatation was detected in 13 patients (37%). Mucosal enhancement of the bowel wall was observed in 28 patients (80%). The prevalence of the extra-intestinal CT findings were: periportal oedema in nine (26%), ascites in 15 (43%), wall thickening and enhancement of gall bladder in 13 (37%), pericholecystic fluid in six (17%), hepatomegaly in 13 (37%), and splenomegaly in nine (26%) patients. One patient (3%) demonstrated free intraperitoneal air due to intestinal perforation. CONCLUSIONS: CT is useful to support the clinical diagnosis of acute GVHD in children with GI symptoms after HSCT. Radiological evaluation is important because early diagnosis and treatment affect the prognosis of GI-GVHD.

Rebound thymic hyperplasia after bone marrow transplantation in children with haemato-oncological diseases.

AIM OF THE STUDY: To evaluate prevalence of rebound thymic hyperplasia (RTH) after bone marrow transplantation (BMT) in paediatric patients with haemato-oncological diseases. MATERIAL AND METHODS: Between February 2013 and December 2017, BMT was performed in 189 paediatric patients with haemato-oncological diseases in our institution. Fifty-six patients who underwent at least two chest computed tomography (CT) exams performed before and after BMT were included in the study. Maximum transverse and anterior-posterior (AP) diameters and CT attenuation of the thymus were measured on axial images. Thymic enlargement was considered when both transverse and AP diameters increased. RTH was defined as the presence of thymic enlargement on CT after BMT relative to the CT taken before. RESULTS: Twenty of 56 patients (36%) demonstrated RTH (12 boys, 8 girls; age range = 4-18 years; median age = 9.8 years). In 20 patients with RTH, seven patients (35%) were diagnosed with ALL, five patients (25%) with thalassemia, two patients (10%) with AML, and one patient (5%) with various diseases. Mean follow-up period between pre-BMT CT and BMT was 46 days, which was 239 days between BMT and post-BMT CT. Mean thymic transverse and AP diameters were 9 mm and 16 mm, respectively, before BMT, which were 17 mm and 33 mm after BMT. Mean HU was 57 on contrast enhanced and 35 on unenhanced images before BMT, which were 59 and 36, respectively, after BMT. CONCLUSIONS: RTH is common finding after BMT in children with various haemato-oncological diseases and should be taken under consideration in paediatric patients after BMT.

Cecum perforation induced by mycophenolate mofetil after hematopoietic stem cell transplantation: A case report and review of literature.

GI perforation after stem cell transplantation is extremely rare and is associated with poor prognosis. In addition, the clinical limitations of MMF are associated with GI intolerance and hematologic suppression. However, the exact mechanism whereby MMF induces changes in GI mucosa is unknown. Currently, there is no definite method to distinguish between GI toxicity associated with MMF and GVHD. It is important to recognize association between MMF and the histologic changes mimicking GVHD, given that GVHD is a significant differential diagnosis in stem cell transplant patients. MMF-induced colitis and GI perforation are extremely rare but should be considered in patients presenting with diarrhea and abdominal pain. Histology and clinical features are helpful to distinguish this condition from ischemic colitis. Early recognition of GI perforation is necessary for proper diagnosis and subsequent intervention. Emergency medical treatment and laparotomy have been shown to reduce the risk of fatal complications in patients presenting with GI symptoms suspected of GI perforation.

Arrested pneumatization of the sphenoid sinus mimicking skull base tumours: MRI prevalence in children with haematologic diseases.

Objective: Arrested pneumatization of the sphenoid sinus is a developmental anatomic variation but may be confused with serious diseases of the skull base. The purpose of this study was to investigate the prevalence of arrested pneumatization of the sphenoid sinus in paediatric patients with haemotologic diseases like sickle cell anaemia, thalassemia and leukemia. Materials and Methods: One hundred and eight paediatric patients (43 girls, 65 boys; age range: 4-18 years; median age: 13 years) with haemotologic diseases who underwent at least one magnetic resonance imaging of the head and neck between 2010 and 2017 in a single institution were included. Magnetic resonance imaging studies were retrospectively reviewed. Well-defined and fat-containing non-expansile lesions located at the sphenoid sinus region were diagnosed as arrested pneumatization of the sphenoid sinus. Medical diagnoses, lesion sizes, age and sex of the patients were recorded. Results: Five (4.6%) of 108 patients demonstrated arrested pneumatization of the sphenoid sinus (3 girls, 2 boys; age range: 9-18 years; median age: 14 years). One (1.2%) of 83 patients with acute leukemia, 2 (15%) of 13 patients with sickle cell anaemia and 2 of 12 (17%) patients with thalassemia demonstrated arrested pneumatization of the sphenoid sinus. No statistical correlation was found between arrested pneumatization of the sphenoid sinus and possible risk factors like patient age and sex. Conclusions: Patients with haemoglobinopathies like sickle cell anaemia and thalassemia involving red-blood-cells had higher prevalence of arrested pneumatization of the sphenoid sinus than patients with leukemia which involves the white-blood-cells. This supported the suggestion that regional blood-flow disorders induce arrested pneumatization of the sphenoid sinus. Correct diagnosis prevents invasive procedures like biopsy and surgery.

Intraoperative sonographic guidance for intracavitary brachytherapy of cervical cancer.

BACKGROUND: To describe the role and benefits of intraoperative sonographic (US) guidance in intracavitary brachytherapy of cervical cancer. METHODS: The data of 142 patients who received tandem-based intracavitary brachytherapy for cervical cancer between January 2010 and June 2015 were retrospectively reviewed. US guidance was carried out for tandem selection and appropriate application. The complications and applicator conformity were assessed with planning CT. RESULTS: Intracavitary brachytherapy was performed under US guidance for 412 insertions in 113 consecutive patients with cervical cancer. Before we started to use US guidance, applications were done in 29 patients: uterine perforation occurred in two patients (6.9%), the tandem length was short in two patients (6.9%), the tandem length was long in four patients (13.8%), and tandem was in myometrium in three patients (10.3%). We then decided to use US guidance routinely. With US guidance, only 1 of 113 patients had uterine perforation (0.9%), tandem length was short in only one patient (0.9%), and tandem was in myometrium in one patient (0.9%). CONCLUSIONS: Real-time US provided safe and effective guidance for intracavitary brachytherapy of cervical cancer resulting in decreased rates of perforations and misplacement of applicators. © 2017 Wiley Periodicals, Inc. J Clin Ultrasound 46:8-13, 2018.

Eye Movement Disorders Following Allogeneic Bone Marrow Transplantation on FK506 (Tacrolimus) and Ganciclovir.

FK506 (tacrolimus) is an immunosuppressive drug and more potent than cyclosporine. FK506 is widely used for immunosuppression in the prevention and treatment of graft-versus-host disease after allogeneic bone marrow transplantation and solid organ transplantation. Neurotoxicity is a recognized complication of FK506 therapy, but ptosis and weakness of eye abduction unilaterally has not been reported in association with FK506 administration to date. We discuss a 13-year-old male patient who developed ptosis and weakness of eye abduction unilaterally 90 days after transplantation with bone marrow from an unrelated donor, for acute lymphoblastic leukemia in this case report. FK506 therapy was administered for graft-versus-host disease prophylaxis and CMV infection was treated with ganciclovir. The physical examination findings completely resolved 72 to 96 hours after concomitant FK506 and ganciclovir treatment were terminated.

Computed tomography based evaluation of prostatic fiducial marker migration between the periods of insertion and simulation.

OBJECTIVE: The aim of this study was to determine whether significant fiducial marker migration occurs between the periods of prostatic marker insertion and computed tomography (CT) performed for radiotherapy planning and if a waiting period is necessary. MATERIAL AND METHODS: Thirty-nine patients with prostate adenocarcinoma underwent fiducial marker insertion before radiotherapy between June 2013 and December 2015. Three markers were inserted by one radiologist under the guidance of transrectal ultrasonography. All patients underwent CT three hours after insertion to confirm the number and position of fiducial markers. Radiotherapy planning CT was performed on an average of 11 days (range 7-20) after insertion. CT images were imported into treatment planning system to analyze the position of fiducial markers. Point- based marker match algorithm was used to find the distance of marker migration. The mean and maximum distances between each fiducial markers were calculated. RESULTS: The mean distance of migration was 1.029±0.42 mm (range 0.23-1.93 mm) and the maximum distance was 1.361±0.59 mm (range 0.25-2.74 mm). The distance of marker migration was not statistically significant for the groups organized according to the timing of marker insertion, prostate volume, patient age, prostate specific antigen level and Gleason score. CONCLUSION: According to our results significant fiducial marker migration did not occur during the interval between insertion and treatment planning CT. It should be taken into consideration that performing simulation on the same day as marker insertion might prevent increased cost and delayed radiation therapy by saving the patients from extra visits to the clinic.

Photoselective Vaporization of the Prostate Using 120 W High Performance System: A Prospective Evaluation of Results Over 2 Years.

PURPOSE: This study evaluated the safety and efficacy of photoselective vaporization of prostate using the 120 W High Performance System for management of symptomatic benign prostatic hyperplasia (BPH). METHODS: We prospectively obtained data from 229 patients between January 2009 and December 2012. We evaluated the serum prostate specific antigen level, International Prostate Symptom Score (IPSS), prostate volume; maximum urine flow rate (Qmax); and postvoid residual urine volume (PVR) in the patients at presentation and on follow-up at 1, 6, 12, and 24 months. The mean duration of the surgery, energy used, hospital stay, and intra- and postoperative complications were assessed. RESULTS: The mean age of the patients was 71.2 ± 9.6 years, and the mean preoperative size of the prostate was 59.41 ± 28.1 mL. The mean duration of the surgery was 47.35 ± 16.14 min, and the mean energy use was 184.39 ± 101.3 kJ. The mean time to removal of the urinary catheter was 21.45 ± 11.06 h, while the mean duration of hospital stay was 24.82 ± 11.5 h. The IPSS declined and mean Qmax increased by over twofold within the first month. The PVR also declined significantly in all groups up to 6 months after the surgery and increased slightly thereafter. Urinary urgency and incontinence occurred in two patients, while four patients developed urinary strictures. Between 12 and 24 months after the surgery, four patients underwent repeat surgery. CONCLUSIONS: Our findings show that photoselective vaporization is safe and effective for the management of BPH and resulted in few complications. It yielded improvements in all parameters that were sustained even up to 2 years after the surgery.

Bilateral recurrent external obturator muscle hematoma: An unusual cause of pelvic pain in hemophilia.

Following joint hemorrhages, intramuscular hemorrhages are the second most prevalent bleeding pattern in hemophiliac patients. Hematomas of the iliopsoas muscle are a well-known complication of hemophilia; however, obturator muscle hematomas are rare. We herein report a case of spontaneous bleeding of the bilateral external obturator muscles, which occured three times within a period of 9 months in a hemophilia patient with factor VIII inhibitors. To the best of our knowledge, this is the first published case of an obturator externus muscle hematoma in hemophilia. In addition to hip hemarthrosis, iliopsoas hematomas and acute appendicitis, obturator muscle hematoma should be considered as one of the diagnostic alternatives for pelvic pain in hemophiliaψ patients. Magnetic resonance imaging enables rapid diagnosis of obturator muscle hematoma.

Primary pericardial extragastrointestinal stromal tumor: A case report and literature review.

Gastrointestinal stromal tumors (GISTs) are the most prevalent mesenchymal tumors of the gastrointestinal tract. GISTs are considered to originate from the interstitial cells of Cajal, the pacemakers of the peristaltic activity of the gastrointestinal tract. More than 95% of GISTs express KIT protein and discovered on GIST-1. GISTs may also be encountered in locations outside the gastrointestinal tract, in which case they are referred to as extra-GISTs (EGISTs) and often behave more aggressively. This is the case report of a primary pericardial EGIST in a 53-year-old male patient, confirmed by immunohistochemistry. To the best of our knowledge, this is the third case of EGIST diagnosed above the diaphragm, without being associated with the esophageal wall. Two cases of primary EGIST arising from the pleura were reported previously. In addition, this is the first reported case of an EGIST originating from the pericardium.

Sinonasal inflammatory myofibroblastic pseudotumor (plasma cell granuloma).

Inflammatory myofibroblastic pseudotumor (plasma cell granuloma) is a soft tissue lesion consisting of myofibroblasts, mature lymphocytes, histiocytes, plasma cells, eosinophils, and extracellular collagen. Various sites in the body may harbor these lesions. Lungs, omentum, intestines, mesentery, and urinary system are the most susceptible areas. It is usually seen in children and young adults. The lesion is rarely detected in the head and neck region. The orbit and the upper respiratory system are the most common localizations in the head and neck region. Sinonasal tract is a rare site of involvement. The differential diagnosis includes squamous cell carcinoma (spindle cell variant), inflammatory fibrosarcoma, leiomyosarcoma, schwannoma, and nonspecific inflammation. Our patient who had a sinonasal mass showed a benign tumor consisting of spindle tumor cells and inflammatory cells histopathologically. This case was presented due to its rare existence to this site.

Safety, efficacy and outcomes of the new GreenLight XPS 180W laser system compared to the GreenLight HPS 120W system for the treatment of benign prostatic hyperplasia in a prospective nonrandomized single-centre study.

INTRODUCTION: We compare and evaluate the safety, efficacy, and short-term outcomes of the new GreenLight XPS 180W (GL-XPS) laser system with the former generation GreenLight HPS 120W (GL-HPS) system for the treatment of benign prostatic hyperplasia (BPH) in a prospective nonrandomized single-centre study. METHODS: From May 2012 to June 2013, 161 consecutive patients with lower urinary tract symptoms secondary to BPH were included: 88 patients were treated with the GL-HPS system and 73 were treated with the GL-XPS system. The perioperative variables International Prostate Symptom Score (IPSS), quality of life (QOL), and maximum flow rate (Qmax) were recorded at baseline, at one month and 6 months. Serum prostate-specific antigen (PSA) was assessed at baseline. RESULTS: The mean age was 70.2 years in the GL-HPS group and 68.6 years in the GL-XPS group. Prostate volumes were 62.3 mL and 61.3 mL, respectively. Both groups showed significant postoperative improvement in the IPSS, QOL, Qmax variables compared to baseline levels. There were no significant differences in improvement in IPSS and QOL between groups. However, both operating and catheterization times were shorter in patients in the GL-XPS group. The overall postoperative complication rate was similar in both groups. CONCLUSION: Both GreenLight systems provide safe, effective tissue vaporization with significant clinical relief of BPH obstruction. The GL-XPS system appears more favourable with regard to reduced operating and hospitalization time, suggesting more cost-effective and efficient tissue removal.

Spontaneous rupture of the ureter.

Spontaneous rupture of the ureter is a very rare condition and usually results from ureteral obstruction by a calculus. Only theoretical mechanisms have been proposed and no possible explanation has yet been reported in the literature. Intravenous contrast-enhanced computed tomography is the most informative study with high sensitivity. Treatment should be individualised, and depends on the state of the patient. Minimally invasive endourological procedures with double-J catheter placement and percutaneous drainage offer excellent results. Conservative management with analgesics and antibiotic coverage may be an alternative to surgery. Herein, we present a case of spontaneous rupture of the proximal ureter with no evidence of an underlying pathological condition.

Nonodontogenic mandibular lesions: differentiation based on CT attenuation.

Mandibular lesions are classified as odontogenic and nonodontogenic based on the cell of origin. Odontogenic lesions are frequently encountered at head and neck imaging. However, several nonodontogenic pathologies may also involve mandible and present further diagnostic dilemma. Awareness of the imaging features of nonodontogenic lesions is crucial in order to guide clinicians in proper patient management. Computed tomography (CT) may provide key information to narrow diagnostic considerations. Nonodontogenic mandibular lesions may have lytic, sclerotic, ground-glass, or mixed lytic and sclerotic appearances on CT. In this article, our aim is to present various nonodontogenic lesions of the mandible by categorizing them according to their attenuations on CT.

Isolated rupture of the superficial vein of the penis.

Penile emergencies are rare but when they do occur, prompt diagnosis and treatment are warranted. Emergent conditions of the male genitalia are mainly traumatic, vascular or infectious. Penile emergencies are usually caused by trauma to the penis, during sexual intercourse or manipulation of an erect penis during masturbation. One of the traumatic vascular penile emergencies is superficial penile dorsal vein rupture. This is a rare condition, with just a few reported cases. It is usually taken into differential diagnosis with the other acute penile injuries that present, such as acute penile edema or ecchymosis. We report a case of 59-year-old male with a superficial penile dorsal vein rupture which occurred during manipulation of the erect penis.

Bone complications after pelvic radiation therapy: evaluation with MRI.

INTRODUCTION: The purpose of this study was to assess the incidence, distribution and MRI characteristics of pelvic bone complications after radiation therapy. METHODS: The medical charts of 345 patients who received pelvic radiation therapy were retrospectively reviewed. A total of 122 patients, 99 women and 23 men, with a mean age of 57 (range 32-87 years) were included in this study. The MR images were retrospectively reviewed by two radiologists. RESULTS: Fatty replacement of bone marrow was seen in all patients. Pelvic bone complications and focal red marrow changes were identified in a total of 38 patients (31.1%). Pelvic insufficiency fracture was diagnosed in 17 patients (13.9%, with a total of 64 lesions) and radiation osteitis was diagnosed in 5 patients (4.1%, with a total of 13 lesions). Avascular necrosis of the femoral head was detected in one patient (0.8%). Focal red bone marrow changes were seen in 15 patients (12.3%). The median time from the end of radiotherapy to the diagnosis of pelvic bone complications or changes was 25 months (range 2-45 months). The 1-, 2-, and 3-year cumulative incidences were 22%, 41% and 49%, respectively. The distribution of insufficiency fractures was as follows: sacral ala, sacral body, ilium, acetabulum, pubis and lumbar spinal vertebra. The distribution of radiation osteitis was as follows: sacral ala, ilium and pubis. CONCLUSION: Radiation-induced pelvic bone complications are not uncommon, and knowledge of characteristic imaging patterns is essential in order to rule out bone metastases and to avoid inaccurate or excessive treatment.