Deferiprone Related Arthropathy in 11-Year-Old Boy with Hereditary Spherocytosis

Regular transfusion therapy in children with intractable hematologic disorder results in hemosiderosis. It causes several organ damages such as liver, heart, and endocrine system and increases risk of infection. Thus, iron-chelating therapy is essential to promote normal development, prevent excess iron overload and death in children. In abroad, deferiprone has been approved for use in thalassemic children with transfusion induced iron overload. Side effects associated with deferiprone include agranulocytosis, arthropathy, gastrointestinal symptoms, increased alanine aminotransferase levels, and zinc deficiency. Arthropathy has been reported to occur approximately 9%–38% of patients. We present an 11-year-old boy patient with hereditary spherocytosis, who experienced arthropathy while under deferiprone therapy.

Recurred Segmental Schwannomatosis Without Neurofibromatosis Type 2

Schwannomas are the most common type of benign peripheral nerve sheath tumors. They typically present as a solitary lesion, but multiple schwannomas rarely occur in patients with neurofibromatosis type 2 (NF2), or patients without the other hallmarks of NF2. The latter is termed schwannomatosis. They most commonly occur in the head and neck involving the brachial plexus and spinal nerves. Although rarely found in the extremities, when these masses occur peripherally, they most commonly affect the sciatic, ulnar, and tibial nerve. It is reported that 2.4% to 5% of all patients undergoing schwannoma excision present as schwannomatosis. One-third of patients with schwannomatosis show tumors limited to a single extremity or segment of the spine and it is referred to as segmental schwannomatosis. We report a case of recurred segmental schwannomatosis of the posterior tibial nerve without features of NF2 after schwannoma excision.

A Case of En Plaque Meningioma of Jugular Foramen with Image Findings of Differential Diagnosis

Primary meningioma of jugular foramen is extremely rare, while paraganglioma or nerve sheath tumor are relatively common in jugular foramen. We reported a case of primary meningioma of jugular foramen. A 79-year-old female who had left tinnitus and hearing loss for three month came to the department of otorhinolaryngology. Temporal bone computed tomography scan showed sclerotic change and slightly irregular margins of left jugular foramen with relatively preservation of bony architecture. Temporal bone magnetic resonance image showed well defined homogeneous enhancing mass in left jugular foramen with extension to carotid space on gadolinium enhanced T1 weighted image. Prominent dural tail was also noted. On T2 weighted image, this mass showed intermediated signal intensity with no vascular signal voids. Meningioma was confirmed by pathology. In this article, we describe a case of primary en plaque meningioma of jugular foramen and review image findings of differential diagnosis.

Volumetric Changes in the Bony External Auditory Canal in Unilateral Chronic Otitis Media

BACKGROUND AND OBJECTIVES: Pneumatization of air cells in the mastoid bone is decreased in chronic otitis media (COM). A decrease in the size of the external auditory canal (EAC) is also found frequently in patients with COM, but this has been little studied. We compared the size of affected bony EACs and the contralateral side in patients with single-side COM using high-resolution computed tomography. SUBJECTS AND METHODS: In total, 99 patients with single-side COM were included. Four indicators related to the size of the bony EAC and IAC were measured using high-resolution computed tomography: the axial and coronal lengths of the tympanic membrane, the length of the isthmus, and the area of the bony ear canal. We also compared both internal auditory canals as negative controls. These assessments were made by radiologists who were blinded to the objective of this study. RESULTS: In patients with single-side COM, the axial length of the tympanic membrane was significantly shorter than normal, and the volume of the EAC was also significantly smaller. The length of the isthmus of the EAC was shorter on the affected side, but the difference was not significant. The IAC volume showed no difference between the two sides. CONCLUSIONS: COM affects general temporal bony development, including the bony EAC and mastoid bone. Therefore, whether to correct this should be considered when preparing for COM surgery.

Imaging of Herniated Discs of the Cervical Spine: Inter-Modality Differences between 64-Slice Multidetector CT and 1.5-T MRI

OBJECTIVE: To assess inter-modality variability when evaluating cervical intervertebral disc herniation using 64-slice multidetector-row computed tomography (MDCT) and magnetic resonance imaging (MRI). MATERIALS AND METHODS: Three musculoskeletal radiologists independently reviewed cervical spine 1.5-T MRI and 64-slice MDCT data on C2-3 though C6-7 of 51 patients in the context of intervertebral disc herniation. Interobserver and inter-modality agreements were expressed as unweighted kappa values. Weighted kappa statistics were used to assess the extents of agreement in terms of the number of involved segments (NIS) in disc herniation and epicenter measurements collected using MDCT and MRI. RESULTS: The interobserver agreement rates upon evaluation of disc morphology by the three radiologists were in fair to moderate agreement (k = 0.39-0.53 for MDCT images; k = 0.45-0.56 for MRIs). When the disc morphology was categorized into two and four grades, the inter-modality agreement rates were moderate (k-value, 0.59) and substantial (k-value, 0.66), respectively. The inter-modality agreements for evaluations of the NIS (k-value, 0.78) and the epicenter (k-value, 0.79) were substantial. Also, the interobserver agreements for the NIS (CT; k-value, 0.85 and MRI; k-value, 0.88) and epicenter (CT; k-value, 0.74 and MRI; k-value, 0.70) evaluations by two readers were substantial. MDCT tended to underestimate the extent of herniated disc lesions compared with MRI. CONCLUSION: Multidetector-row computed tomography and MRI showed a moderate-to-substantial degree of inter-modality agreement for the assessment of herniated cervical discs. MDCT images have a tendency to underestimate the anterior/posterior extent of the herniated disc compared with MRI.

Comparison of MRI T2 Relaxation Changes of Knee Articular Cartilage before and after Running between Young and Old Amateur Athletes

OBJECTIVE: To compare changes in T2 relaxation on magnetic resonance (MR) images of knee articular cartilage in younger and older amateur athletes before and after running. MATERIALS AND METHODS: By using a 3.0-T MR imager, quantitative T2 maps of weight-bearing femoral and tibial articular cartilages in 10 younger and 10 older amateur athletes were acquired before, immediately after, and 2 hours after 30 minutes of running. Changes in global cartilage T2 signals of the medial and lateral condyles of the femur and tibia and regional cartilage T2 signals in the medial condyles of femoral and tibia in response to exercise were compared between the two age groups. RESULTS: Changes in global cartilage T2 values after running did not differ significantly between the age groups. In terms of the depth variation, relatively higher T2 values in the older group than in the younger group were observed mainly in the superficial layers of the femoral and tibial cartilage (p < 0.05). CONCLUSION: Age-related cartilage changes may occur mainly in the superficial layer of cartilage where collagen matrix degeneration is primarily initiated. However, no trend is observed regarding a global T2 changes between the younger and older age groups in response to exercise.

US and CT Findings of Splenic Hydatid Cyst: A Case Report

Hydatid disease is a parasitic infection caused by the larvae of the cestode worms Echinococcus. In humans, the most commonly affected organ is the liver, the next second common organ is the lung. The third common affected organ is the spleen. In the case of splenic hydatid cyst, most cysts remain clinically silent and are diagnosed incidentally or when complications occur. We experienced a case of splenic hydatid cyst in a 28-year-old man. The patient complained of abdominal pain for 1 month. Abdominal ultrasound revealed a cystic lesion with daughter cysts in the spleen. The CT imaging also showed a cystic lesion with daughter cysts. We diagnosed it as a splenic hydatid cyst which was confirmed by pathology after surgery.

Angiolipoma of the Breast: A Case Report / 한국유방암학회지

An angiolipoma is a common mixed connective tissue tumor that commonly occurs in the trunk and extremities, and has been rarely reported in the breast. It is a benign variant of a lipoma that shows various appearances on mammography. It is usually seen with a well circumscribed homogeneously hyperechoic pattern on ultrasound. We report a rare case of an angiolipoma of the breast with no definite abnormality on mammography, appearing as an irregular homogeneously hyperechoic lesion on ultrasound.

Calcifying Aponeurotic Fibroma of the Elbow: A Case Report

Calcifying aponeurotic fibroma is a rare soft tissue tumor that mostly occurs in the distal extremities of children and adolescents. We report here on a case of calcifying aponeurotic fibroma of the right elbow in an 8-year-old boy, and the tumor was diagnosed by surgical excision. The patient complained of painless swelling and mild limitation of the range of motion of the elbow joint. Radiologically, the mass was ill-defined and showed stippled calcification with shallow bony erosion. Microscopically, the tumor was composed of spindle cells with nodular deposits of hyalination and calcification, and these deposits were surrounded by palisading polygonal plump cells. Immunohistochemically, the tumor showed a diffuse positive expression for CD99 and negativity for smooth muscle actin, S-100 protein and CD34. The patient has been well with no signs of recurrence during the 42 months after surgery.

Imaging Findings of Wirsungocele: A Report of Two Cases

Wirsungocele is defined as a cystic dilatation of the terminal portion of the main pancreatic duct, and this is an extremely rare malady. The pathophysiology of Wirsungocele has been proposed that a congenital or acquired weakness in the distal duct that's associated with functional obstruction may lead to the formation of Wirsungocele. In addition, mechanical obstruction of the distal duct has been considered as another mechanism of Wirsungocele. We report here on the imaging findings of two cases of Wirsungocele that seemed to occur by different pathophysiologic mechanisms.

Acute Sialadenitis in Children and Adolescents: CT Findings and Clinical Manifestations according to Glandular Involvement

PURPOSE: This study was designed to compare the CT findings and clinical manifestations in children and adolescents with acute sialadenitis according to the involved salivary glands. MATERIALS AND METHODS: The study included fifty children and adolescents (34 boys, 16 girls) with acute sialadenitis that was diagnosed during the past five years. All of the subjects were divided into three groups: group I (parotid gland involvement, n = 16), group II (submandibular gland involvement, n = 20) and group III (involvement of both glands, n = 14). We analyzed the presence of an abscess, sialolith, bilaterality, cellulitis and lymphadenopathy on CT scans. The analyzed clinical data were age, sex, lymphadenopathy, pain, swelling, presence of a mass, tonsillitis, treatment period and surgical treatment if it was performed. RESULTS: The presence of an abscess, sialolith, cellulitis, swelling, age, presence of a palpable mass and treatment period were statistically significant factors for the patients in the three groups. An abscess was combined only in group I patients. There was a high rate of sialolith in group II patients and cellulitis in group III patients as seen on CT scans. Swelling in group II patients and group III patients and the presence of a palpable mass in group I patients were identified as clinical manifestations. Age was younger in group I patients (mean age, 5.3 years) than in group II patients (mean age, 12.9 years) and group III patients (mean age, 15.2 years). The treatment period was longer for group I patients. CONCLUSION: For acute sialadenitis in children and adolescents, age, presence of an abscess, sialolith, cellulitis, swelling, presence of a palpable mass and treatment period were different according to the involved salivary glands.

Nontraumatic Subperiosteal Orbital Hematoma Associated with Ethmoid Sinusitis: A Case Report

Subperiosteal hematoma of the orbit is a rare disease and most of the cases occur in young adult males as a result of direct facial or orbital trauma. In the absence of direct facial or orbital trauma, nontranmatic subperiosteal orbital hematoma has rarely been reported in association with a sudden elevation of cranial venous pressure or venous congestion, systemic diseases associated with a bleeding diathesis and paranasal sinusitis. We report here on a rare case of subperiosteal orbital hematoma associated with ethmoid sinusitis, as was seen on CT imaging.

Cervical Thoracic Duct Cyst: A Case Report

Thoracic duct cysts are uncommon lesions that most commonly occur in the abdominal and thoracic portion of the thoracic duct: the cervical portion is the rarest location. The main causes of thoracic duct cyst are surgical injuries such as neck dissection and blunt trauma. We report here on a rare case of spontaneous cervical thoracic duct cyst that was noted on ultrasonography and CT. The thoracic duct cyst was confirmed by fine needle aspiration and it was treated by sclerotherapy.

A Case of Hungry Bone Syndrome after Parathyroidectomy in Patient with Tertiary Hyperparathyroidism and Hemodialysis Patient / 대한내분비외과학회지

Hyperparathyroidism is a common finding in patients with chronic renal failure. Among the hyperparathyroidism, tertiary hyperparathyroidism, which is secreting parathyroid hormone autonomously in spite of hypercalcemia. Sometimes it requires surgical intervention due to not only symptomatic hypercalcemia, but also longstanding asymptomatic hypercalcemia. Hungry bone syndrome was known to be developed due to extensive re-mineralization of skeleton after parathyroidectomy. It is characteristic of persistent hypocalcemia, hypophosphatemia and often with tetany. The patient's condition improved without complication after the calcium chloride and calcium carbonate administration. We report a case of hungry bone syndrome developed after parathyroidectomy in patient with tertiary hyperparathyroidism and chronic renal failure.

Giant Cell Tumor of Short Tubular Bone: Case Report

Giant cell tumors of the bone commonly occur in the epiphyseal scar of a long tubular bone, representing about 5% of all primary bone tumors. A short tubular bone, such as one in the hand or foot, is the site of less than 5% of all giant cell bone tumors. The authors report two cases in which giant cell tumors arose in the short tubular bone, and describe the clinical manifestations and radiologic findings.