Schatzki ring in children and young adults: clinical and radiologic findings

BACKGROUND: The Schatzki ring is a well-known clinical and radiologic entity in adults, but is thought to be rare in childhood. Objective. To review the clinical presentations and radiologic findings of children and young adults with Schatzki rings. MATERIALS AND METHODS: A retrospective review of all barium swallow examinations done between 1990 and 1996 revealed 20 patients with Schatzki rings. RESULTS: The most frequent presenting symptoms of these patients were progressive dysphagia with solid food and acute food impaction. Radiographic findings of Schatzki rings were typical in all cases. Twelve patients had endoscopy and all had evidence of esophagitis. CONCLUSION: Schatzki rings are not rare in childhood. The patients are symptomatic, presenting with either progressive dysphagia with solids or acute food impaction. A thorough evaluation of the distal esophagus should be performed in patients with a suggestive clinical history.     

Giant perimedullary arteriovenous fistulas of the spine: clinical and radiologic features and endovascular treatment

Little is known about the epoxygenase pathway of the arachidonic acid cascade in uterine tissues. In this paper, we describe the formation of epoxyeicosatrienoic acids (EETs) and dihydroxyeicosatrienoic acids (DHETs) in human term placenta after uncomplicated pregnancies. Metabolism of [3H]-arachidonic acid was analyzed in short term tissue cultures of placenta by reverse phase HPLC. Major metabolites coeluted with authentic EETs and DHETs. The formation of EETs was inhibited by carbon monoxide. In non-radioactive incubations with biopsies from seven different placentas, sufficient material for GC/MS analysis was obtained. The combined media were purified by solid phase extraction and reverse phase HPLC. The fraction coeluting with DHETs was derivatized with pentafluorobenzylbromide (PFB) and bis-(trimethylsilyl)-trifluoroacetylacetamide (BSTFA) and analyzed by GC/NICI/MS/MS. 11, 12-DHET and 14, 15-DHET were identified by their mass spectra displaying specific fragments at m/z 149 and m/z 189, respectively. Our results suggest that the epoxygenase pathway is active in human term placenta.     

Primary intraventricular hemorrhage in adults: clinical features, risk factors, and outcome

BACKGROUND AND PURPOSE: Nontraumatic primary intraventricular hemorrhage in adults is uncommon. The purpose of this study was to identify clinical features, risk factors, and outcome of primary intraventricular hemorrhage in adults. METHODS: We identified computed tomography scans for nontraumatic primary intraventricular hemorrhage performed between 1982 and 1993 at our institutions and reviewed medical records to determine clinical features, risk factors, and outcomes of these patients. RESULTS: Of 14 cases studied, the onset of symptoms was acute in all and the most common symptom was headache (78%), followed by nausea/vomiting (71%), and mental status change (71%). Seizures as well as focal neurologic deficits were uncommon. The associated risk factors were hypertension in 7 cases, aneurysm or arteriovenous malformation in 5, coagulopathy in 1, and fibromuscular dysplasia in 1 case. Five patients died acutely, and all of the survivors returned to functional independence. The patients with lethargy, pupillary and extraocular movement abnormalities, quadriparesis, hydrocephalus, and aneurysmal intraventricular hemorrhage had worse outcomes. CONCLUSION: Patients with nontraumatic primary intraventricular hemorrhage present with sudden onset of diffuse neurologic symptoms. The mortality in early phase is high (36%) and the prognosis for survivors is good. Factors correlating with the outcome are discussed.     

Hepatic trauma: pediatric features

Conservative management of hepatic trauma has been demonstrated to be effective in children. However, surveillance of these patients in a specialized paediatric surgery unit is essential because of the possibility of rare, but unavoidable complications. The authors review the 20-year experience of conservative management of hepatic trauma reported in the literature.     

Pressure measurements in herniated lumbar disks: association with radiologic and clinical data

Sympathetic neuronal activity is primarily responsible for the neurogenic control of cerebral autoregulation. The stimulation of sympathetic nerves causes both large arterial constriction and small vessel dilation in experimental animals. However, the role of the sympathetic nervous system in the control of cerebral hemodynamics has yet to be clarified in humans. In order to assess the effect of sympathetic activation on human cerebral hemodynamics, we performed a simultaneous transcranial Doppler (TCD) monitoring of bilateral middle cerebral arterial flow velocity in 16 healthy male volunteers (mean age 26) during well-known sympathetic activation measures such as isometric hand-grip exercise (IHE) and cold pressor test (CPT). Blood pressure was checked manually before and at each minute during tests. The mean arterial pressure (MAP) was calculated as (systolic pressure + 2 X diastolic pressure)/3. There was a significant increase in MCA flow velocities during both sympathetic activation tests. The percent increase of diastolic velocity (36% with IHE and 24% with CPT) was significantly higher than systolic velocity (21% with IHE and 9% with CPT). The pulsatility index was significantly decreased during the tests (from 0.75 to 0.58 with IHE and from 0.81 to 0.63 with CPT). These results suggest that sympathetic activation increases MCA flow velocities, related with a reduction in small vessel resistance and/or a constriction of large arteries.     

Atypical squamous cell carcinoma of the larynx and hypopharynx: radiologic features and pathologic correlation

The objective of this study was to analyze the radiologic features of atypical forms of squamous cell cancer and correlate them with clinical, endoscopic, and histopathologic findings. The CT and MRI images of 31 patients with atypical forms of squamous cell carcinoma were reviewed retrospectively and the radiologic findings were correlated with clinical, endoscopic, and histopathologic findings. Histopathologic diagnoses included undifferentiated carcinoma of nasopharyngeal type (n = 8), verrucous carcinoma (n = 18), spindle cell carcinoma (n = 3), and basaloid cell carcinoma (n = 2). Undifferentiated carcinoma of nasopharyngeal type was located in the supraglottis or piriform sinus beneath an intact mucosa and initial endoscopic biopsy was most often negative. The discrepancy between an intact mucosa at endoscopy and a solid mass with homogenous enhancement at CT or MRI was characteristic for these tumors and warranted further investigations to obtain the definitive histologic diagnosis. Verrucous carcinoma displayed characteristic clinical, radiologic, and pathologic features, namely, an exophytic tumor arising from the glottic level displaying a rugged surface with finger-like projections but with only minor submucosal infiltration. Spindle cell carcinoma appeared as a polypoid mass with a thin stalk arising from the supraglottis. Basaloid cell carcinoma displayed a distinct lobulated enhancement pattern which was observed on contrast-enhanced T1-weighted SE images. Although the MR and CT features of atypical forms of squamous cell carcinoma cannot be considered pathognomonic they should raise the differential diagnosis even if endoscopic biopsy has been negative. The radiologist's awareness of the appearance of these unusual tumors on CT and MR images may greatly facilitate the diagnostic work-up and helps to guide the endoscopist to the adequate biopsy site in order to establish the correct diagnosis.     

Distinguishing features of focal cemento-osseous dysplasia and cemento-ossifying fibromas. II. A clinical and radiologic spectrum of 316 cases

The distinguishing histopathologic features of focal cemento-osseous dysplasia (FCOD) (including lesions occurring in both anterior and posterior jaws) and cemento-ossifying fibroma (COF) (ossifying fibroma and cementifying fibroma) were demonstrated in our earlier work. The aim of the current study was to further refine their clinical and radiographic features. We have assessed 18 clinical and radiographic parameters by univariate comparisons (chi-squared and Student t tests), and a multivariate assessment (logistic regression) in 241 cases of FCOD and 75 of COF. These cases were diagnosed from a combination of clinical, radiographic, and histopathologic information. FCOD was seen predominantly in black women, with a peak incidence in the fourth and fifth decades, whereas COF showed no female predilection except in the fourth decade (p < 0.005). COF occurred in patients an average of 10 years younger than patients with FCOD (p < 0.0001). Most patients with FCOD were asymptomatic (62%); the average lesion size was 1.8 cm. More than half of patients with COF displayed jaw expansion and a considerably larger size lesion (mean 3.8 cm, p < 0.001). The mandible was the most frequent site for both FCOD (86%) and COF (70%). Radiographically, a well-defined border was observed in 53% of cases of FCOD and 85% of cases of COF (p < 0.01). Cases of FCOD mostly demonstrated an irregularly mixed radio-opacity (69%), whereas 53% of COFs presented as a radiolucency (p < 0.005). In FCOD, there was a close association with tooth apices (70.6%, p < 0.0001) or with previous extraction sites (21%, p < 0.05); however, the majority of COF cases (86%) showed no relationship with either. Combining the radiographic feature of a periapical location with the pathology of multiple curetted fragments and "ginger root" bony trabeculae, allowed 90% sensitivity and 89% specificity in a logistic regression model to predict the lesion to be an FCOD. These findings provide guidelines not only to distinguish these two entities clinically, but also aid in reaching an accurate diagnosis histopathologically.     

CADASIL in a North American family: clinical, pathologic, and radiologic findings

Spoiled fast three-dimensional gradient-echo magnetic resonance imaging of the aorta and iliac arteries was performed in 13 patients after administration of a single bolus of gadopentetate dimeglumine. The imaging table was moved manually 40-45 cm between two acquisitions. Arterial signal-to-noise ratio did not vary significantly between the thoracic (aortic) and pelvic stations, but venous signal-to-noise ratios increased and arterial contrast-to-noise ratios decreased on the pelvic studies. This method was feasible and increased anatomic coverage.     

Liver metastases from colon cancer with intra-bile duct tumor growth: radiologic features

PURPOSE: Our goal was to characterize the radiologic features of liver metastases from colon cancer with intrahepatic bile duct (IHBD) dilatation. METHOD: Radiologic findings of liver metastases from colon cancer with IHBD dilatation of four patients were compared with pathologic findings. RESULTS: The cause of bile duct dilatation in all cases was due to papillary tumor growth in the bile duct. In two patients, intra-bile duct tumor growth (IBDTG) was observed on imaging. In the other two patients, IBDTG was not observed, but a nontapered abrupt obstruction of a dilated bile duct was seen, corresponding to the microscopically proven papillary tumor growth in the ductal lumen. In three patients who underwent an extensive hepatic resection, there has been no recurrence. In one patient who had a nonanatomic limited resection, a recurrence was seen 1 year after surgery. CONCLUSION: When liver tumor with IBDTG is suspected on imaging, liver metastases should be considered in the differential diagnosis besides hepatocellular carcinoma or cholangiocellular carcinoma. Careful preoperative assessment for IBDTG by imaging is essential to determine the extent of surgical resection.     

Corticobasal degeneration: clinical features

BACKGROUND: There are little epidemiologic data on psychiatric disorders of women in jails. Accurate data on female jail detainees are critical because of their increasing numbers and their unique treatment needs. METHODS: Using the Diagnostic Interview Schedule, independent interviewers assessed a randomly selected, stratified sample of 1272 female jail detainees awaiting trial in Chicago, Ill. We tabulated lifetime and 6-month prevalence rates of disorders by race or ethnicity (African American, non-Hispanic white, Hispanic), age, and education and compared the jail rates with general population rates for women in the Epidemiologic Catchment Area program. We also examined whether or not psychiatric disorder was associated with the severity of the detainee's current arrest charges. RESULTS: Over 80% of the sample met criteria for one or more lifetime psychiatric disorders; 70% were symptomatic within 6 months of the interview. The most common disorders were drug abuse or dependence, alcohol abuse or dependence, and post-traumatic stress disorder. Major depressive episode was the most prevalent major mental disorder. Rates were generally highest among non-Hispanic whites and among older detainees. Rates for all disorders were significantly higher than general population rates, except for schizophrenia. Most detainees with psychiatric disorders were arrested for nonviolent crimes. CONCLUSION: These results suggest substantial psychiatric morbidity among female jail detainees.     

Important radiologic findings following blunt thoracic trauma (author's transl)

In patients suffering from traffic accidents traumatic lesions of the chest are of special importance since in 50% of deaths the thoracic trauma is responsible for the final outcome. The fatal course is, however, not always due to severe and irreparable dammage to thoracic organs, but very often due to under-estimation of initial symptoms and thus inadequate treatment. For the proper diagnosis of traumatic lesions of the thorax the plain film of the chest is, therefore, fundamentally important. In this paper, which is mainly dealing with lesions of the airways and lungs, the significance of radiological symptoms is stressed and a correlation with pathophysiologic as well as histologic data is attempted.     

Predicting clinical performance of radiologic technology students

The lack of scientific studies on predictive criteria for evaluating the clinical potential of students in radiologic technology led to the current study. Students during a ten-year period was subjected to the ACT examinations. These scores were subsequently compared with the students' scores on the examination of The American Registry of Radiologic Technologists, high school grade point average and earned high-school grades in pertinent subjects to see if any predictive factors were evidenced. The result showed correlation of some of these factors with the Registry score of sufficient statistical significance to warrant using them as part of the process for selecting students for radiologic technology programs.     

A clinical and radiologic study of autotransplanted impacted canines

The aim of the present study was to evaluate the clinical and radiologic results of 20 autotransplantations of impacted canines performed in the Orthodontic and Pedodontic Department of the University of Geneva between 1979 and 1988. The sample, divided into two different age groups (group A: 13-20 years; group B: 20-48 years), demonstrated persistence of pulp vitality in 80% of the cases in group A, whereas routine endodontic treatment was instituted in all cases of group B. Periodontal healing was noted in 90% of the cases in group A, and in 70% of the cases in group B. The present clinical and radiologic data indicate that impacted canines can be transplanted at any age with good prognosis and are an alternative to orthodontic repositioning in selected cases of canine impaction.     

Incontinentia pigmenti: clinical and neuroradiologic features

Developmental brain malformations and destructive processes of unknown etiology were described in incontinentia pigmenti (IP). Two patients, a male and a female, with characteristic skin lesions and central nervous system (CNS) involvement are reported. Neuroradiological examinations revealed hypoplasia of corpus callosum, neuronal heterotopias, and periventricular white matter damage. No specific infectious, inflammatory, or metabolic abnormalities were identified. These neuroradiographic findings may suggest that an ischemic pathogenetic mechanism occurred prenatally. We speculate that the brain damage in IP may occur during CNS development and in successive stages. Magnetic resonance imaging appears more useful to detect white matter lesions and brain malformations in patients with IP.     

Paroxysmal dyskinesias: clinical features and classification

We studied 46 patients with paroxysmal dyskinesia and classified them according to phenomenology, duration of attacks, and etiology. There were 13 patients, 7 females, who had paroxysmal kinesigenic dyskinesia (PKD), 10 with attacks lasting 5 minutes or less (short lasting) and 3 with attacks lasting longer than 5 minutes (long lasting). Twenty-six patients, 18 females, had paroxysmal nonkinesigenic dyskinesia (PNKD), 9 with short-lasting and 17 with long-lasting PNKD. Five patients, 3 females, had paroxysmal exertion-induced dyskinesia (PED), 3 with short-lasting PED and the other 2 with long-lasting PED. In addition, there was 1 patient with paroxysmal hypnogenic dyskinesia (PHD) and 1 with paroxysmal superior oblique myokymia. Only 2 patients, 1 with PKD and 1 with PHD, had family history of paroxysmal dyskinesias. No specific cause could be identified in 21 patients; in the other 23 patients the etiologies included the following: psychogenic (9 patients), cerebrovascular diseases (4), multiple sclerosis (2), encephalitis (2), cerebral trauma (2), peripheral trauma (2), migraine (1), and kernicterus (1). Nine of 10 (90%) patients with PKD improved with medications, mostly anticonvulsants, compared with only 7 of 19 (37%) with PNKD. This new classification, based chiefly on precipitating events, allowed appropriate categorization of the attacks in all our patients with paroxysmal dyskinesias.     

Acute brucella sacroiliitis: clinical features

Chronic fatigue disorders are characterized by a subjectively defined group of symptoms such as chronic fatigue, mental confusion, exertional malaise, weight changes, and/or diffuse multi-joint pains. Significant clinical overlap exists between chronic fatigue disorders and the syndrome of serum inappropriate anti-diuretic hormone (SIADH). Both chronic fatigue disorders and SIADH are characterized by lethargy and mental confusion. Both disorders can be induced or exacerbated by viral illnesses, physical exertion, emotional stress and/or hypotension. Both can be treated with salt loading and glucocorticoids. Therefore, altered water metabolism resulting from inappropriate release and/or response to arginine vasopressin (AVP) is proposed as a pathophysiological basis of certain chronic fatigue disorders. Moreover, these data suggest that salt loading and/or direct inhibition of AVP may be an effective therapeutic approach in individuals with chronic fatigue disorders.     

Gaucher's disease: clinical features and natural history

Gaucher's disease is an inherited disorder characterized by pathological storage of glycolipid in mononuclear phagocytes: it is a multi-system disease associated with striking variation in its clinical manifestations, severity and course. Although molecular analysis of the glucocerebrosidase gene in patients with Gaucher's disease has permitted broad correlations between genotype and phenotype to be made, with few exceptions genetic variation at this locus does not allow confident prediction of clinical phenotype or prognosis. Partial deficiency of glucocerebrosidase is associated principally with parenchymal disease of the liver, spleen, bone marrow and, in severe cases, the lung, in non-neuronopathic, Type 1, Gaucher's disease: here storage material in macrophages originates from turnover of exogenous glycolipids. Severe deficiency of glucocerebrosidase caused by disabling mutations is additionally associated with neurological manifestations that in part reflect a failure to degrade endogenous neuronal glycosphingolipids, the so-called neuronopathic, Type 2 and Type 3 disease categories. Here we describe the clinical features, complications and natural history principally of Type 1 Gaucher's disease: emphasis is placed on emerging pulmonary, osseous and other manifestations of obscure pathogenesis that respond poorly to enzyme-replacement therapy.     

Idiopathic granulomatous hypophysitis: clinical and imaging features

AIMS/BACKGROUND: The syndrome of ocular histoplasmosis is usually prefaced by "presumed' as the aetiology is not yet clear. The aim of this study was to evaluate the clinical features of a similar ocular syndrome in the Netherlands where the fungus Histoplasma capsulatum is not endemic. METHODS: A retrospective multicentre study in which all patients were included who were diagnosed with a syndrome similar to presumed ocular histoplasmosis and in whom both fluorescein angiogram and all complete patient data were available. Fluorescein angiograms were examined by three authors in a masked fashion. Eighty one patients were selected who fulfilled the ophthalmic criteria for presumed ocular histoplasmosis. Fifty one patients showed the classic clinical picture, while 30 patients had an incomplete form as they did not show numerous histospots. RESULTS: No major difference in clinical characteristics could be identified when comparing the group of patients with the classic syndrome with the one with the incomplete syndrome. Final visual outcome of patients with macular subretinal neovascularisation after laser treatment was better when compared with untreated patients (p < 0.01). CONCLUSIONS: Since the fungus Histoplasma capsulatum is absent in the Netherlands, other aetiological agents must have led to this clinical entity similar to the presumed ocular histoplasmosis syndrome seen in the USA.     

Gastric duplication: endoscopic appearance and clinical features

The case of a 13-year-old female presenting with a 6 week history of significant vomiting resulting from a gastric duplication is described. At endoscopy, an antral mass which appeared to change in size and site with peristalsis was evident. Although rare, these lesions need to be considered in the differential diagnosis of patients presenting with symptoms suggestive of gastric outlet obstruction. However, in the majority of cases, the diagnosis is rarely made prior to surgery.     

Surgical treatment of extratemporal epilepsy: clinical, radiologic, and histopathologic findings in 60 patients

PURPOSE AND METHODS: The aim of this study was to analyze clinical, radiologic, and histopathologic findings in 60 consecutive patients with medically intractable extratemporal epilepsy who were operated on between November 1987 and May 1993. RESULTS: Histologically, there were distinct structural abnormalities in 50 (83%) of the surgical specimens. Signal abnormalities on magnetic resonance imaging (MRI) were present in all patients with neoplastic lesions (n = 17) and in 94% of patients with nonneoplastic focal lesions (n = 32). Overall, structural abnormalities were detected by MRI in 47 (96%) of 49 patients with focal lesions. During a mean follow-up of 4 years, 30 (54%) patients remained completely seizure free, 11 (20%) had < or = 2 seizures per year, seven (12%) showed a seizure reduction of > or = 75%, and eight (14%) had < 75% reduction in seizure frequency. The fraction of seizure-free patients was 12 (80%) of 15 in patients with neoplastic lesions, 16 (52%) of 31 in patients with nonneoplastic focal lesions, and two (20%) of 10 for those without histopathologic abnormalities. The differences in seizure outcome between patients with and without focal lesions were statistically significant (p < 0.05), if seizure-free outcome was compared with persistent seizures. CONCLUSIONS: Focal lesions and particularly neoplasms are associated with improved postoperative seizure control compared with patients without histopathologic abnormalities. We advise caution in considering surgery to treat extratemporal epilepsy in patients who have normal MRI scans, because the outcome with the approach described in this study is poor in such cases.