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Spontaneously reduced midgut malrotation: CT diagnosis.

Midgut malrotation is a relatively rare congenital malformation that arises from an error of rotation and fixation of the midgut. We report a case of spontaneously reduced duodenal malrotation diagnosed by computed tomography and roentgenography after the ingestion of barium.

Adult↗

Which one is at risk in intraoperative fluoroscopy? Assistant surgeon or orthopaedic surgeon?

BACKGROUND: Orthopaedic surgeons use intraoperative portable fluoroscopy and roentgenography. The present study was an attempt to find out if there is a difference between the occupational radiation exposure to the orthopaedic surgeon and assistant surgeon in the operating room while using intraoperative fluoroscopy or radiographic control and to measure it. METHODS: During a 3-month period, 107 consecutive operations were monitored for radiation exposure. At monthly intervals, the radiation doses were measured in millirem and recorded. The distance of the orthopaedic surgeon and the assistant surgeon from the X-ray source were noted in every fluoroscopic check. The orthopaedic surgeon was always at a safe distance (more than 90 cm), but the assistant surgeon always stood nearby (10 cm) the X-ray source for positioning of the patient. RESULTS: The radiation exposure according to the badge on the shoulder was consecutively 3, 4, 3 mrem for the orthopaedic surgeon and 20, 19, 22 mrem for the assistant surgeon. The radiation exposure according to the badges on the anaesthetic machine, in the room and under the apron of the orthopaedic surgeon were all zero, whereas the readings of the badge under the apron of the assistant surgeon were 7, 6, 5 mrem consecutively. CONCLUSION: Our findings show that although the radiation exposure during orthopaedic operations is below the recommendations of the European Committee on Radiation Protection, there is a higher risk of exposure for the assistant surgeon. It has to be kept in mind that there could be morphological and functional damage in cells exposed to radiation. Therefore, we should continue to use appropriate shielding precautions in view of the unknown long-term risks.

Film Dosimetry↗

Rapidly destructive coxopathy after subchondral insufficiency fracture of the femoral head.

Rapidly destructive coxopathy (RDC) is a rare disorder, and its pathophysiology is unknown. Here, we report a case of RDC evaluated by roentgenography and magnetic resonance imaging, from the onset of hip pain without changes on roentgenograms, to the terminal stage with collapse of the femoral head. Our serial radiological findings clearly illustrate the entire process of RDC, especially the initial stage. Subchondral insufficiency fracture of the femoral head seems to be an important preliminary event in the development of RDC.

Aged↗

Laparoscopic Heller's myotomy or botulinum toxin injection for management of esophageal achalasia. Patient choice and treatment outcomes.

BACKGROUND: Esophageal achalasia is a rare disease in which degenerating parasympathetic innervation of the lower esophageal sphincter (LES) leads to unopposed sympathetic tone and failure to relax on deglutition, resulting in a range of symptoms for the patient, most notably dysphagia, chest pain, regurgitation, and weight loss. Laparoscopic Heller's esophagomyotomy (Lap-HM) and botulinum toxin (BoTox) injection into the LES are two recently described methods for treating achalasia. No comparison of laparoscopic Heller's myotomy and botulinum toxin has yet been presented. METHODS: A total of 22 patients (15 men, 7 women; mean age, 57.9 years) diagnosed with idiopathic esophageal achalasia were given a choice of treatment modality: Lap-HM or BoTox. Patients were prospectively assessed before and after treatment with esophageal manometry, barium swallow roentgenography, and dysphagia score. Patients' preferences and treatment efficacy were evaluated. RESULTS: Of the 22 patients, 18 (81.8%) chose BoTox and 4 (18.2%) chose Lap-HM. Five patients in the BoTox group opted for Lap-HM a mean of 565 +/- 212 days after the first injection, having received a median of four (range, 3-5) injections. Both BoTox and Lap-HM were effective in improving dysphagia score: before BoTox (median, 7; range, 0-10) and 2 months afterward (median, 2; range, 0-6) (p < 0. 01); before Lap-HM (median, 9; range, 7-10) and 6 months afterward (median, 1.5; range, 0-4) (p < 0.05). Both methods also effectively reduced lower esophageal sphincter (LES) nadir pressure: before BoTox (mean mmHg, 22 +/- 9) and 2 months afterward (mean mmHg, 15 +/- 7) (p < 0.05); before Lap-HM (mean mmHg, 24 +/- 10) and 6 months afterward (mean mmHg, 3 +/- 4) (p < 0.05). However, only Lap-HM produced a significant reduction in LES basal pressure before (mean mmHg, 34 +/- 7) and afterward (mean mmHg, 3 +/- 3) (p < 0.01) and increased esophageal barium clearance (mean percentage of barium retained in esophagus at 5 min before (97% +/- 6%) and afterward (23% +/- 45%) (p < 0.05). CONCLUSIONS: BoTox injection and Lap-HM both significantly reduce achalasia symptoms, but only Lap-HM improves esophageal clearance of barium. BoTox injection is the most popular treatment method from a patient perspective, although symptom recurrence or failure rates are high. Lap-HM is favored by younger patients and is equally effective after BoTox therapy failure.

Adult↗

Changes in the proximal neck of abdominal aortic aneurysms early after endovascular treatment.

Long-term success of endovascular treatment of infrarenal abdominal aortic aneurysms (AAA) depends on secure "attachment" of the stent to the proximal and distal necks of the aneurysm. The purpose of this retrospective study was to detect changes in the proximal neck early after endovascular treatment of infrarenal AAA. Between November 1997 and December 1998, we performed endovascular treatment of infrarenal AAA in 43 patients. A bifurcated modular stent (AneuRx by Medtronic) was used in all patients. An oversized stent with a diameter 10% greater than the preoperative diameter of the proximal neck of the AAA was used in all cases. Follow-up examinations were carried out at 1, 6, and 12 months using plain roentgenography, color Doppler ultrasound, and CT scan with contrast injection. Statistical analysis was performed using the Student's t-test. Deterioration of the aortic neck is the result of a variety of mechanisms. The natural history of the infrarenal neck of AAA following endovascular repair (as well as conventional open repair) is progressive enlargement. Stenting could be an added risk factor for expansion. Strict follow-up is essential for early detection of this complication.

Aorta, Abdominal↗

A novel compound heterozygous mutation in the thyroglobulin gene resulting in congenital goitrous hypothyroidism with high serum triiodothyronine levels.

Thyroglobulin abnormality is a rare cause of congenital hypothyroidism and only a limited number of mutations in the thyroglobulin gene have been reported. We analyzed the thyroglobulin gene in a patient with congenital goitrous hypothyroidism. This girl was identified with hyperthyrotropinemia in a neonatal mass-screening test. The patient had goiter, and her body weight gain was poor. Distal femoral epiphysis was absent on roentgenography. Her serum thyroxine level was low; however, her triiodothyronine level was high. Autoantibodies against triiodothyronine, thyroid peroxidase, and thyroglobulin were all negative. Her serum thyroglobulin level was undetectable. The thyroglobulin gene from the genomic DNA of the patient was analyzed by direct sequencing. Two novel heterozygous missense mutations, Cys1897Tyr (exon 31) and Arg2336Gln (exon 40), were found in the patient. The former mutation was derived from her mother, suggesting a compound heterozygous state. Normal triiodothyronine and low thyroxine concentrations are often observed in patients with thyroglobulin gene mutations. We considered that some patients with thyroglobulin abnormality might have high triiodothyronine levels. In cases of congenital goitrous hypothyroidism with normal-to-high triiodothyronine levels and low serum thyroglobulin levels, thyroglobulin abnormality should be considered.

Amino Acid Substitution↗

A long-surviving patient with unresectable advanced gastric cancer responding to S-1 after receiving improved gastrojejunostomy.

Conventional gastrojejunostomy has been employed for unresectable advanced gastric cancer with pyloric stenosis; however, it is often not fully effective. We report a patient with unresectable gastric cancer who was effectively treated with an anticancer drug, S-1, after receiving an improved gastrojejunostomy. The patient was a 55-year-old woman who was referred to our hospital for epigastric pain. Upper gastrointestinal endoscopy showed a Borrmann III tumor in the antrum of the stomach, and gastric roentgenography showed pyloric stenosis. Preoperative findings were T3N2H0P0, stage III b. At operation, the tumor was found to have invaded the duodenum and the head of the pancreas, and disseminated nodules were found in the mesenterium of the small intestine, the left diaphragm, and the round ligament of the liver. A curative operation was impossible for the advanced gastric cancer. Therefore, an improved gastrojejunostomy was performed to allow oral intake. Oral intake started 7 days after the operation, and she left our hospital 20 days after the operation. She started treatment with 80 mg/day of S-1, given orally, for 28 days, followed by 14 days' rest, as 1 course. During 16 courses of the treatment, she maintained a performance status of 0 to 1 and maintained quality of life. However, she died because of pelvic dissemination and genital bleeding (caused by tumor invasion into the uterus) 2 years and 4 months after the surgery. This case suggested that the improved gastrojejunostomy was a useful method for treating unresectable gastric cancer, allowing the possibility of oral intake, and the use of S-1.

Adenocarcinoma↗

Thymoma masquerading as congenital partial absence of the left pericardium.

Interpretation of irregularities of the left heart border on routine posterior-anterior and lateral roentgenograms may be quite challenging. Partial absence of the left pericardium provides a characteristic abnormality of the left heart border. Described is a case of a thymoma which mimicked partial absence of the left pericardium on routine roentgenography. The importance of including both partial absence of the left pericardium and thymoma in the differential diagnosis of irregularities of the left heart border is discussed.

Adult↗

Prediction of left ventricular ejection fraction using a unique method of chest x-ray and ECG analysis: a noninvasive index of cardiac performance based on the concept of heart volume and mass interrelationship.

A reasonably accurate, simple, inexpensive, noninvasive method of determining ejection fraction (EF) is necessary to evaluate left ventricular function in epidemiologic studies and individual patients. Using the concepts of left ventricular mass reflected by precordial R wave summation (M) and left ventricular volume (V) estimated by chest roentgenography in 114 patients with myocardial disease undergoing left ventriculography, EF was predicted with the formula: EF = 63.74 - (2.16.V) + (0.34.M); R2 = 0.69; standard error of the estimate (SEE) = 11. Because angiographic inferior wall motion (IWM) abnormalities significantly affected the results, but inferior Q waves were usually only present in patients having one infarct, a noninvasive technique to predict the presence of inferior wall motion abnormality (IMA) in patients having multiple infarcts was developed and based on the relationship of precordial R wave summation (M) and roentgenographic heart volumenometry (V). By combining V, M, and IMA (which predicted IWM) to determine EF, multiple linear regression analysis showed that EF = 67.30 - (1.56.V) + (0.23.M) - (14.18 IMA) (R2 = 0.77; SEE = 9). Prospective validation of the formula was then done in 139 consecutive individuals, with R2 = 0.49 and SEE = 9. This study demonstrates that routinely performed, simple, inexpensive clinical tests provide data that can be combined by multiple regression analysis to predict resting EF in patients with heart disease affecting the left ventricle. This unique method may allow inexpensive ventricular function screening in large population studies and in addition might provide an independent index of myocardial performance for clinical use, since it reflects the amount of contractile mass per unit of left ventricular volume.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Effect of the vasodilator trimazosin versus placebo on exercise performance in chronic left ventricular failure.

The effect of the vasodilator trimazosin versus placebo on exercise duration until marked dyspnea was evaluated in a double blind randomized study in 16 patients with chronic left ventricular failure despite digitalis and diuretic therapy. Trimazosin caused a reduction in resting systolic and diastolic blood pressures and in resting product of systolic blood pressure times heart rate. The improvement in exercise duration from the average of values during the baseline and single blind placebo periods was greater after 3 and 6 weeks of trimazosin therapy (300 and 450 mg daily) after 3 and 6 weeks of double blind placebo therapy (P is less than 0.025). Four of eight patients receiving trimazosin had disappearance of pulmonary venous congestion on chest roentgenography compared with none of eight patients receiving placebo. These preliminary data suggest that trimazosin may be effective in treating chronic left ventricular failure.

Adult↗

Univentricular heart of right ventricular type: clinical, angiographic and electocardiographic features.

The sequential chamber analysis and angiographic, hemodynamic and clinical features, including those seen on electrocardiography, are presented in 18 patients with a univentricular heart of right ventricular type. In all cases a main chamber with a right ventricular trabecular pattern received both (or the sole) atrioventricular (A-V) connections, and a rudimentary chamber with a left ventricular trabecular pattern receiving no A-V connection was also present. The 18 patients were seen between 1971 and 1979; 6 died and the diagnosis was confirmed at autopsy. Twelve patients had double inlet, four absent right and two absent left A-V connections. Electrocardiography revealed right ventricular hypertrophy in all, and 11 had a superior mean frontal QRS axis. Chest roentgenography disclosed dextrocardia in 7 and levocardia in 11. Of 12 infants presenting with a marked decrease in pulmonary flow, 10 presented as ill neonates with hypoxia and acidosis. Four infants with increased pulmonary flow presented before 8 weeks with heart failure and mild cyanosis. Two with moderate pulmonary stenosis had mild cyanosis, but were asymptomatic. All had associated cardiac anomalies, pulmonary stenosis and persistent ductus arteriosus being the most common. For accurate diagnosis, angiographic demonstration of a posterior rudimentary chamber in addition to the main chamber of right ventricular trabecular pattern is mandatory.

Abnormalities, Multiple↗

The natural history of idiopathic dilated cardiomyopathy.

Between 1960 and 1973, a total of 104 patients at the Mayo Clinic had a diagnosis of idiopathic dilated cardiomyopathy on the basis of clinical and angiographic criteria; these patients were followed up for 6 to 20 years. Twenty-one percent of the patients had a history of excessive consumption of alcohol, 20 percent had had a severe influenza-like syndrome within 60 days before the appearance of cardiac manifestations and 8 percent had had rheumatic fever without involvement of cardiac valves several years before; thus, possible etiologic risk factors of infectious-immunologic type may be important. Eighty patients (77 percent) had an accelerated course to death, with two thirds of the deaths occurring within the first 2 years. Twenty-four patients (23 percent) survived, and 18 of them had clinical improvement and a normal or reduced heart size. Univariate analysis at the time of diagnosis revealed three factors that were highly predictive (p less than 0.01) of the clinical course: age, cardiothoracic ratio on chest roentgenography and cardiac index. Systemic emboli occurred in 18 percent of the patients who did not receive anticoagulant therapy and in none of those who did; thus, anticoagulant agents should probably be prescribed unless their use is contraindicated.

Alcohol Drinking↗

Radiation exposure of pediatric patients and physicians during cardiac catheterization and balloon pulmonary valvuloplasty.

Thermoluminescent dosimeters were applied to various areas of 61 pediatric patients and physicians to measure radiation doses during routine cardiac catheterization and during 4 cases of balloon pulmonary valvuloplasty. Radiation doses were measured during chest roentgenography, fluoroscopy and cineangiography. Average skin dose to the chest was 121 microGy during chest x-ray, 5,182 microGy during catheterization and 641 mGy during valvuloplasty. For the eyes, thyroid and gonads of the patients, the exposure during routine catheterization was equal to 0.4, 6 and 0.2 chest x-rays, respectively. Radiation dose of the operator was 3 microGy for the eyes and 6 miCroGy in the thyroid. About 56% of the operator's dose could be reduced by thyroid shields, and 80% by lead aprons. The assistant received only 1 microGy outside the thyroid shield. Therefore, we have concluded that the patients' dose during routine catheterization is largely based on our experimental results, but the dose is acceptable based on the risk factor analysis. The skin dose to the right lateral chest of the patient during valvuloplasty is extremely high, perhaps as high as the equivalent of 1,000 chest x-rays. Besides the clinical benefits of valvuloplasty, the long-term radiation-related hazards to the patient should be carefully monitored.

Adolescent↗

Clinical implications of a "nonspecific" transbronchial biopsy.

The diagnostic accuracy of transbronchial biopsy via fiberoptic bronchoscope was reviewed in 127 noncritically ill patients. Biopsy results were analyzed according to whether a "specific" pathologic diagnosis of neoplasm, granuloma or pneumonia, or a "nonspecific" diagnosis of inflammation, fibrosis or normal lung was made. The clinical significance of a "nonspecific" biospy specimen was evaluated by clinical follow-up of at least 12 months (mean 15 months) and by grouping patients according to the type of abnormality found on chest roentgenography. Clinical follow-up was available in 119 of these patients. The over-all "specific" diagnostic yield for biopsy with secretions was 49 per cent, with transbronchial biopsy being the sole means of specific diagnosis in 14 per cent of the patients with a peripheral mass lesion, in 18 per cent of the patients with localized infiltrative processes and in 52 per cent of the patients with diffuse infiltrative processes. In 64 (52 per cent) patients both biopsy specimens and secretions were diagnostically nonspecific. In 16 (77 per cent) patients with peripheral mass lesions but nonspecific biopsy findings and secretions, neoplasm was diagnosed by more invasive procedures. However, 22 (91 per cent) patients with localized and 12 (75 per cent) patients with diffuse infiltrative processes had benign clinical follow-up suggesting that open lung biopsy in such patients should be reserved for patients with obvious clinical or roentgenographic evidence of deterioration.

Biopsy↗

Small cell lung cancer. Complete remission and improved survival.

Thirty-two patients with limited-stage small cell lung cancer were treated with combined supervoltage radiotherapy and a combination of cyclophosphamide, doxorubicin and vincristine chemotherapy. The goal of obtaining a complete remission, the necessary step which precedes improved survival, was successful in 26 of 27 of completely re-evaluated patients. Fiberoptic bronchoscopy was useful in substantiating complete remission status. Seventeen of the 32 patients remain alive from 8+ to 28+ months (median 16+ months). Ten patients are alive and relapse-free from 12+ to 28+ months (median 19+ months). Transient granulocytopenia (mean nadir 650 cells/mm3) during induction therapy and the associated risk of infection were the most serious toxicities encountered. This therapy produces complete remission on roentgenography and bronchoscopy, symtomatic improvement and improved survival in the majority of patients with limited-stage small cell lung cancer. A portion of these patients may have their disease eradicated.

Adult↗

The reflex sympathetic dystrophy syndrome (RSDS). III. Scintigraphic studies, further evidence for the therapeutic efficacy of systemic corticosteroids, and proposed diagnostic criteria.

Sixty-four patients were evaluated prospectively for a reflex sympathetic dystrophy syndrome (RSDS), using quantitative clinical measurements, high-resolution roentgenography and scintigraphy. Five separate groups were identified by their clinical features, allowing us to distinguish patients with definite or incomplete forms of the RSDS as well as 16 patients with other disorders. Scintigraphy was found to be a useful diagnostic study that may also provide a method of predicting therapeutic response. Systemic corticosteroid therapy proved to be a highly effective mode of treatment for up to 90 percent of the patients with the RSDS.

Diagnosis, Differential↗

Congestive heart failure: echocardiographic insights.

This study was designed to assess the role of echocardiography in the evaluation and management of patients with the congestive heart failure syndrome. Fifty consecutive patients with congestive heart failure referred for echocardiography were evaluated. Thirty patients (60 percent) had ejection fractions under 50 percent (mean +/- SD 30 +/- 9 percent), left ventricular dilatation (6.5 +/- 0.7 cm), and normal wall thicknesses (1.0 +/- 0.2 cm). The echocardiographic findings were predictable on clinical grounds in 18 of the 30 patients (60 percent) and worse than clinically expected in 12 patients (40 percent). Management changes after echocardiography were indicated in 11 of 30 patients (37 percent). The remaining 20 of the 50-patient cohort (40 percent) had ejection fractions above 50 percent (mean 70 +/- 9 percent, p less than 0.01), and, as a group, were characterized by normal left ventricular size (5.1 +/- 0.8 cm, p less than 0.01) and borderline wall thicknesses (1.1 +/- 0.2 cm, p less than 0.01). The largest subgroup of these 20 patients had hypertensive heart disease (seven patients, 35 percent) associated with the congestive heart failure syndrome presumably related to left ventricular diastolic (compliance) dysfunction. The normal ejection fraction was unexpected clinically in 18 of these 20 patients (90 percent). Recommended management after echocardiography changed in all 18 patients. Since standard clinical findings (history, physical examination, and chest roentgenography) failed to separate patients with normal and abnormal ejection fractions, or those in need of changes in management, echocardiography was a useful and, at times, essential part of the evaluation of these patients with the congestive heart failure syndrome.

Adult↗

Analysis of the differential diagnosis and assessment of pleuritic chest pain in young adults.

The most important problem in the approach to young patients with acute pleurisy is distinguishing those with idiopathic or viral pleurisy from patients with pulmonary embolism. Three clinical features are helpful in making this distinction: (1) pleural effusion(s) present on chest roentgenography, (2) history of predisposing factors for or past history of veno-occlusive disease, and (3) physical signs indicative of phlebitis. Lung scanning should be performed in patients with these findings. If results of scanning are highly characteristic of pulmonary embolism (segmental or lobar defect with ventilation/perfusion mismatch) in such a patient, anticoagulation may be considered immediately. Patients in whom scanning reveals indeterminate characteristics or abnormalities not characteristic of pulmonary embolism should undergo pulmonary angiography if other clinical features suggest that the probability of pulmonary embolism remains at least moderately high.

Adult↗