Superselective Transcatheter Arterial Embolization for Large Unruptured Renal Angiomyolipoma in Lymphangioleiomyomatosis

Purpose

To retrospectively evaluate therapeutic performance and complications of superselective transcatheter arterial embolization (TAE) for unruptured renal angiomyolipoma (AML) in patients with lymphangioleiomyomatosis (LAM) and to investigate the correlation between percentage reduction in tumor volume and intratumoral fat content.

Papillonodular type of cystic partially differentiated nephroblastoma: a case report

We report a case of the papillonodular type of cystic partially differentiated nephroblastoma (CPDN), an extremely rare renal neoplasm that occurs in newborns and infants. The papillonodular type of CPDN is a variant of the conventional form of CPDN. MRI clearly demonstrated the gross pathologic features, distinguishing it from other renal multilocular cystic tumors.     

A case of inflammatory breast cancer following augmentation mammoplasty with silicone gel implants

A 54-year-old-woman who underwent augmentation mammoplasty with silicone gel implants 30 years previously, visited our hospital with complaints of bloody nipple discharge, redness and itching of her right breast. Cancer of the right breast was diagnosed by dynamic magnetic resonance imaging (MRI) examination with Gadolinium (Gd)-DTPA enhancement. Radical mastectomy was subsequently performed. The histopathological findings demonstrated scirrhous and inflammatory breast cancer with invasion of dermal lymphatics.     

Spontaneous subacute intratumoral hemorrhage of hepatic cavernous hemangioma

We report a case of giant hepatic cavernous hemangioma associated with spontaneous subacute intratumoral hemorrhage. Magnetic resonance imaging showed an oval, homogeneous, high-intensity lesion with a low-intensity rim in the original high-intensity tumor on T2-weighted images. On T1-weighted images, this oval lesion showed heterogeneous high intensity with peripheral higher intensity. The histologic specimen confirmed hepatic hemangioma with intratumoral hemorrhage.     

Magnetic resonance angiography versus endoscopy for the assessment of gastroesophageal varices in biliary atresia

This study was undertaken to compare magnetic resonance angiography (MRA) and gastrointestinal tract endoscopy (ENDO) for detecting varices in postoperative biliary atresia (BA) patients. Thirty-four BA patients were divided into groups according to age and liver function; group 1: 1–4 years old; group 2: 5–9 years old; group 3: over 10 years old; group A: normal liver function; and group B: moderate liver dysfunction. All subjects had MRA and ENDO. ENDO showed esophageal or gastric varices in 16 of 34 subjects. MRA depicted gastroesophageal vessels in 12 of 16 cases with varices on ENDO (sensitivity: 75%). Two subjects appeared to have varices on MRA that could not be confirmed on ENDO. Sixteen subjects had no varices on ENDO and normal MRA (specificity: 88.9%). Sensitivities and specificities of MRA for detecting varices were 20 (1/5) and 100% (6/6) in group 1 (n = 11), 100 (6/6) and 66.7% (4/6) in group 2 (n = 12), 100 (5/5) and 100% (6/6) in group 3 (n = 11), 100 (8/8) and 100% (9/9) in group A (n = 17), 50 (4/8) and 77.8% (7/9) in group B (n = 17), respectively. From these findings, MRA was not accurate in BA patients less than 10 years old or with moderate liver dysfunction. Therefore, periodic endoscopic examination is recommended for the accurate assessment and follow-up of varices in postoperative BA patients.     

High-density CT of muscle and liver may allow early diagnosis of childhood-onset Pompe disease

Pompe disease is classified into infantile-, childhood- and adult-onset forms based on onset age and the degree of organ involvement. Differing from the infantile-onset form which is characterized by marked organ involvement, the childhood-onset form usually presents with muscle weakness and elevation of serum creatine kinase (CK), mimicking those of progressive muscular dystrophy. We report our successful early diagnosis and initiation of enzyme replacement therapy (ERT) in a young girl with childhood-onset Pompe disease before the development of skeletal muscle symptoms. She was referred to our hospital at the age of 2 years 4 months because of hyperCKemia detected incidentally. She was active and lacked developmental delay and muscle weakness; however, hepatomegaly was noted. The combination of high-density changes in the liver and skeletal muscle on computed tomography (CT) images was suggestive of glycogen storage disorder, especially childhood-onset Pompe disease. Low alpha-glucosidase (GAA) activity on dried blood spots facilitated the diagnostic process, and genetic analysis of GAA allowed a definitive diagnosis, without performing muscle biopsy. We promptly started ERT at the age of 2 years 6 months. After 1 year, she still had not developed any skeletal muscle symptoms, and serum CK level was almost normal. Since the efficacy of ERT is thought to depend on the extent of muscle damage at its commencement, we expect that ERT may have prevented the manifestation of skeletal muscle involvement in this patient.     

Objective assessment of mastication predominance in healthy dentate subjects and patients with unilateral posterior missing teeth

We aimed to investigate mastication predominance in healthy dentate individuals and patients with unilateral posterior missing teeth using objective and subjective methods. The sample comprised 50 healthy dentate individuals (healthy dentate group) and 30 patients with unilateral posterior missing teeth (partially edentulous group). Subjects were asked to freely chew three kinds of test foods (peanuts, beef jerky and chewing gum). Electromyographic activity of the bilateral masseter muscles was recorded. The chewing side (right side or left side) was judged by the level of root mean square electromyographic amplitude. Mastication predominance was then objectively assessed using the mastication predominant score and the mastication predominant index. Self‐awareness of mastication predominance was evaluated using a modified visual analogue scale. Mastication predominance scores of the healthy dentate and partially edentulous groups for each test food were analysed. There was a significant difference in the distribution of the mastication predominant index between the two groups (P < 0·05). The mastication predominant score was weakly correlated with self‐awareness of mastication predominance in the healthy dentate group, whereas strong correlation was observed in the partially edentulous group (P < 0·05). The results suggest that the individuals with missing unilateral posterior teeth exhibited greater mastication predominance and were more aware of mastication predominance than healthy dentate individuals. Our findings suggest that an objective evaluation of mastication predominance is more precise than a subjective method.     

2D-FASE MRCP for pediatrics with congenital biliary dilatation: usefulness of non-breath-hold one-shot MRCP

The usefulness of magnetic resonance cholangiopancreatography (MRCP) using the non-breath-hold one-shot technique was evaluated. Ten children suffering from congenital biliary dilatation (CBD) were included. Four of them were preoperative cases, and the remaining six postoperative. All MR images taken were compared with endoscopic retrograde cholangiopancreatography or intraoperative cholangiography. MR images using the non-breath-hold one-shot technique clearly showed the confluence of the common bile duct and the main pancreatic duct in seven of the cases. The confluence of the common bile duct and main pancreatic duct was obscure in the other three cases, mainly due to motion artifact. These results show that this non-breath-hold one-shot technique is useful for diagnosis and postoperative follow-up of congenital biliary dilatation in children.     

Bio-psycho-social assessment of occlusal dysaesthesia patients

Dentists occasionally experience occlusal dysaesthesia (OD) patients, who complain of bite discomfort without evident occlusal abnormalities. It is suggested that this condition is related to somatosensory abnormalities of the trigeminal system and/or psychological problems such as somatoform disorders. The aim of this study was to investigate the characteristics of OD with a bio-psycho-social approach. Twelve OD patients (10 women, two men; mean age 54.7 years) and twelve healthy volunteers (10 women, two men; mean age 54.8 years) were selected. They were assessed using (i) interdental thickness discrimination ability test using 2-, 5- and 10-mm-thick standard blocks and 12 test blocks that were thinner or thicker than the corresponding standard block and (ii) psychological tests: General Health Questionnaire (GHQ60) and Profile of Mood States (POMS) brief-form. There was no significant difference in the interdental thickness discrimination ability between OD patients and controls (mixed-model anova, P=1.000). Regarding psychological tests, there were no significant differences between OD patients and controls in the total scores for either GHQ60 (P=0.143) or POMS brief-form (P=0.319) (Wilcoxon's test). However, OD patients showed significant differences from controls in several subscales, that is, 'somatic symptoms' (P=0.039) and 'severe depression' (P=0.039) for GHQ60 and 'depression-dejection' (P=0.014) and 'vigour' (P=0.008) for POMS brief-form (Wilcoxon's test). These results suggest there is no difference in interdental thickness discrimination ability between OD patients and normal controls, but OD patients tend to score higher on psychosomatic distress.