Iranian Journal of Radiology
Volume:8 Issue: 2, Jun 2011

Neurofibromatosis (NF) is divided into two types, NF type 1 and NF type 2. Optic nerve gliomas have a high degree of association with NF type 1. NF 2, less commonly seen, is a complex of cutaneous and deep neural tumors. It is an autosomal dominant familial disorder in which CNS is affected in about 15% of the cases. Bilateral acoustic neuromas are pathognomonic of NF type 2 which may be associated with meningiomas or ependymomas.Typical clinical manifestations of neurofibromatosis are cafe-au-lait spots and multiple cutaneous tumors. There is bone involvement as scoliosis, pseudoarthrosis of long bones, scalloping of vertebral bodies, abnormal rib tubulation and defective ossification of the skull. Extraskeletal manifestations of neurofibromatosis include optic nerve gliomas, pheochromocytoma, aneurysms of cerebral and renal arteries, acoustic neurilemmoma and superficial skin nodular neurofibromas.Here, we intend to present images of several cases of neurofibromatosis with different patterns of body involvement.

Moyamoya disease is a rare, chronic cerebrovascular occlusive disease of unknown etiology. It is characterized by progressive stenosis of the arteries of the circle of Willis leading to ischemic strokes in young people and cerebral hemorrhage, which is more frequent in adults. Secondarily, an abnormal network of fine collateral vessels arises at the base of the brain. The term moyamoya refers to the angiographic appearance of the cerebral vasculature. We present such a disease in an 18-month-old Iranian girl with global developmental delay, which is a very rare presentation of moyamoya disease. She was diagnosed by magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA).

Background/ Central nervous system (CNS) anomalies are the most seriouscongenital abnormalities. Ultrasound examination is an effective and noninvasive modality for prenatal diagnosis of these anomalies. The purpose of the current study was to determine the frequency of CNS and associated abnormalities.Patients and A total of 22500 pregnant women who were referred by obstetricians/ gynecologists for routine work up of pregnancy were scanned over a period of 3 years by two expert sonologists in a referral center using high resolution ultrasound unit. After transabdominal sonographic examination of 22500 pregnant women, 112 (0.5% fetuses were detected with CNS anomalies, some of whom had more than one anomaly. Forty-one (37%) Chiari malformations, 26 (23%) monro and aqueductal stenosis cases, 18 (16%) anencephaly cases, nine (8%) encephaloceles, seven (6%) microcephalies, five (4%) Dandywalker syndromes, two (2%) arachnoid cysts, two (2%) agenesis of corpus callosum cases, one (1%) holoprosencephalyand one (1%) schizencephaly were reported in our study. According to our results, Chiari malformation and hydrocephalus were the mostprevalent anomalies of CNS congenital abnormalities in East Azarbaijan, Iran. An accuratediagnosis depends upon fetal age, amniotic fluid volume, fetal position, operator experience and careful evaluation of the associated malformations, which are often present.

Background/ Idiopathic intussusception is an important abdominal emergency ininfancy and childhood. Non operative management for treatment is firstly considered due to less invasiveness, less complications and cost effectiveness compared to surgical treatment. This study summarizes our experience in the management of intussusception in children who were referred to a children hospital.Patients and A total of 102 children who were diagnosed as having intussusception were referred to one children hospital in Tehran during a period of 10 years, from 1997 to 2007. Reductions were performed upon 57 cases by a radiologist or radiology residents, if there was no medical contraindication. We used chi-square test for analysis. The success rate of reduction was eight out of 13 (61.5%) with barium, nine out of 17 (53.5%) with air and 22 out of 27 (81.5%) with saline (p value=0.116). One patient had recurrence with air reduction. Another case was complicated by peritonitis using barium enema. There was no significant relationship between the success rate of reduction and the type of reduction.

Background/ The accurate anatomic mapping and determination of the severity of arterial disease, an important health problem of the elderly, is of great significance. We aimed to determine the diagnostic value of 64-multislice CT angiography (MSCTA) in run-off and cut-off sites of arterial disease.Patients and Throughout the study, MSCTA followed by an operative intervention was carried out on a total of 38 patients with clinical signs and symptoms suggestive of arterial disease (AD) all of whom had the indication for vascular surgery. The mean age of patients was 34±15.86 (range, 23 to 93) years. MSCTA was executed using a 64-slice CT scanner, during the arterial phase of injecting the nonionic, contrast medium with a power injector at the rate of 5 ml/sec into the antecubital vein and exploration and revascularization of peripheral arterial disease was performed intraoperatively. Atherosclerosis and arterial disease, the most common causes of vascular occlusion, were more common in the lower extremities. According to MSCTA findings, the most frequent site of stenosis was the superficial femoral artery. Spearman's correlation coefficient showed a high degree of agreement amongst the raters. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and the accuracy of MSCTA compared to surgery ere 83.8%, 96%, 96.8%, 81.3% and 89%, respectively. MSCTA findings were compared with surgery s a standard of reference, which showed concordance in the majority of cases (81.6%). Cut-off sites were correctly identified by MSCTA in 97.3% of the patients and the most common sites of discordance were the run-off sites (18.2%). MSCTA angiography as a novel diagnostic modality may be a suitable alternative and a viable choice for routine clinical diagnosis.

Background/ Panoramic radiography has a great place among imaging techniquesbecause of its enormous advantages. One of the characteristics of an ideal imaging technique is to supply precise measurement. The purpose of the current study was to evaluate the accuracy of linear measurements of the jaws on panoramic radiographs.Patients and In this study, the vertical distances between the metal markers were measured by panoramic radiography in seven sites of two skulls in various head positions. Then the radiographic measurements were compared with the actual values. Eighty three percent of the measurements were underestimated, 8.5% wereoverestimated on panoramic radiography and 8.5% of the measurements had no difference with the real measurements. Overestimation was not greater than 1 mm. The difference between actual and radiographic measurements was less in the posterior areas and in the mandible. In all head positions, the greatest difference between actual and radiographic measurements occurred in the anterior area. Based on the results of this study, linear measurements on panoramic radiography are more reliable in the posterior areas and may be used in early clinical measurements.

A previously healthy 52-year-old man had a chest radiograph for medical check-up and found to have a right-sided aortic arch. Computed tomography of the thorax revealed a right-sided aortic arch with aberrant left subclavian artery originated from Kommerell's diverticulum. Barium swallow examination showed compression of the posterior wall of the esophagus. He was asymptomatic and no surgical intervention was performed.

Mediastinal tuberculous lymphadenitis is a rare disease in adults. Dysphagia as the accompanying symptom is even a rarer manifestation. Cases with esophageal symptoms may present as esophageal ulceration, mucosal or submucosal mass, fistula or sinus formation, extrinsic compression or displacement of the esophagus. Extrinsic compression may radiologically or endoscopically present as a submucosal tumor. Our case is a 30-year-old woman with dysphagia for a month. Extrinsic compression was seen endoscopically on the mid-esophagus. On thoracic CT and MRI images, a multiloculated cystic/necrotic mass, 5.5×4.8×3.1 cm in size consisting of multiple septa was located subcarinally in the middle mediastinum. In Wang needle aspiration, a mucopurulaent liquid was aspirated from the subcarinal localization by bronchoscopy. Diagnostic thoracotomy was carried out because histological and bacteriological examinations were not diagnostic. It was reported as tuberculous lymphadenitis pathologically. The control thoracic CT performed after antituberculous treatment showed complete regression of the mass. We herein report a rare form of tuberculous lymphadenitis.

Fibrodysplasia ossificans progressiva (FOP) is a rare autosomal dominant disorder, characterized by painful swelling of muscles and connective tissue in the early years of life, consequently leading to ossification at a mean age of 4-5 years. We report FOP in a 2-year-old boy with palpable masses in the frontal and lower cervical paraspinal and left periscapular muscles.He was born with hallux valgus. Despite this hallmark, he was referred to the hospital withthe primary diagnosis of hematoma, but further investigation indicated FOP. The patient was discharged from the hospital with non steroidal anti-inflammatory drugs (NSAID) and education of the parents. The importance of this case was that in spite of the early occurrence of the typical presentation of FOP for more than one year and the fact that the patient's mother was a physician who had consulted with many specialists, the diagnosis had been missed.This indicates that the general physicians, radiologists and other specialist's awareness andknowledge of FOP is insufficient.

The purpose of this study was to review the imaging and anatomopathologic findings and to discuss the main differential diagnosis of bizarre parosteal osteochondromatous proliferation (BPOP) or Nora's lesion, a rare benign surface lesion of the bone. Histologically confirmed plain radiographs, ultrasound, CT and MRI images of four patients with BPOP were obtained and retrospectively reviewed. Three cases involving the hand and one involving the foot are reported.On plain radiographs, BPOP is a well-marginated, calcified or ossified mass arising directly from the cortical surface of the underlying bone. Ultrasound images show a low echoic peripheral cap around the lesion. CT images show the wide base of the lesion. On MRI, BPOP was of a low signal on T1, enhancing following gadolinium administration. Underlying bone and adjacent surrounding soft tissues were normal.

A 59-year-old woman was referred with nausea, vomiting and generalized abdominal pain after colonoscopy. She is a known case of amyloidosis for 10 years that resulted in renal failure.