Eugene C. Klatte

The radiographic spectrum in neurofibromatosis

N EUROFIBROMATOSIS (von Recklinghausen disease) is a hereditary condition resulting from dysplasia of neuroectodermal and mesoderma1 tissues. Any organ or system of the body may be primarily or secondarily involved in this disease. It is estimated that the incidence of neurofibromatosis is one in 3000 births.g It is transmitted as an autosomal dominant, but 50% of cases arise as spontaneous mutants. There is no known sex or racial predominance. von Recklinghausen gave the first organized description of the disease in 1882,57 although other authors had previously described some of its manifestations. From that time forward, knowledge of the entity has been broadened by hundreds of reports and studies. The etiology is not known. There is marked variation of the histologic pattern of lesions in neurofibromatosis and considerable disagreement as to the cell or cells of origin. It is known that the serum of a high percentage of these patients contains increased nerve growth stimulating activitypg which may account for the apparent multiple cell origin. l6 Primary neuroectodermal and mesodermal dysplasia apparently are responsible for most of the manifestations of neurofibromatosis. Lesions may arise from the neuroectodermal sheath cells of Schwann and mesodermal connective tissue elements.47 The classical clinical signs include cutaneous pigmentation (cafe au lait spots), multiple soft cutaneous tumors, and palpable neurofibromas of peripheral nerves. To this can be added manifestations of dysfunction of every organ system. Some of us were taught early in our medical careers that syphilis is the great imitator of other diseases. This statement is also true of neurofibromatosis. The purpose of this paper is to describe and

Accuracy of Preoperative Staging in Stages A and B Nonseminomatous Germ Cell Testis Tumors

AbstractPrimary radical retroperitoneal lymphadenectomy currently is used for pathologic staging of nonseminomatous germ cell testis tumors unless advanced disease is present initially. Many attempts have been made with serum markers, lymphangiograms, excretory urograms, gallium and ultrasound scans, and computerized tomography for accurate staging of these tumors preoperatively. We reviewed the accuracy of alpha-fetoprotein and beta-human chorionic gonadotropin serum markers, and abdominal ultrasound and computerized tomography scans in preoperatively staging 64 patients with stage A or B nonseminomatous germ cell testis tumors seen at our university between 1977 and 1980. The results of the preoperative staging studies were correlated with the pathologic stages obtained by retroperitoneal lymphadenectomy.The combined results of serum markers, ultrasound and computerized tomography staged correctly 24 of 32 cases of pathologic stage A disease (75 per cent) and 21 of 32 of pathologic stage B disease (66 p...

Percutaneous Transluminal Dilatation in the Treatment of Renal Vascular Hypertension

Twenty-six patients with renal vascular hypertension were treated with percutaneous transluminal dilatation. Twenty-one were followed for more than 1 year; 18 underwent repeat angiography. Recurrence of stenosis was shown angiographically in one of six patients with fibromuscular disease and in 12 of 12 patients with atherosclerotic disease. The procedure was more likely to result in cure of hypertension (six of 10) in patients with fibromuscular disease than in patients with atherosclerotic disease (one of 16). Although percutaneous transluminal angioplasty provides the advantages of simplicity, reduced cost, and probably reduced risk, long-term utility of the procedure remains to be established. A prospective randomized trial, establishment of a registry, or both would serve this purpose.

Origin of the right pulmonary artery from the ascending aorta. Report of a surgically corrected case.

A case of anomalous origin of the right pulmonary artery from the ascending aorta with an associated patent ductus arteriosus has been described. Successful complete surgical correction was accomplished after physiologic and cineangiocardiographic studies had established the diagnosis. Postoperative evaluation demonstrated a return to nearly normal physiologic values. The unparalleled value of selective angiocardiography in the anatomic assessment of cardiovascular lesions is emphasized.

Residual Pulmonary Findings in Clinical Hyaline-Membrane Disease

Abstract Seventy infants who had clinical hyaline-membrane disease as neonates were followed from two to five years with repeated physical examinations and x-ray films of the chest. Nineteen of them had such severe symptoms that they required mechanical ventilatory assistance for periods ranging from 17 to 116 hours. During the follow-up period six of the 19 had radiologic evidence suggestive of pulmonary fibrosis or overexpansion or both. Only two had clinical evidence of frequent infections and expiratory wheezing that failed to respond to bronchodilator therapy; lung biopsies in these two indicated patchy areas of peribronchial and alveolar fibrosis. Subsequently, both showed clinical improvement. The clinical, radiologic and pathologic sequelae that occurred in these infants surviving clinical hyaline-membrane disease appear to result from the reparative process of the lung to the perinatal insult that produces the original disease.

Ultrasound and computed tomography of the abdomen in the staging and management of testicular carcinoma.

Ultrasound and computed tomography (CT) are ideal for imaging enlarged retroperitoneal lymph nodes. The authors evaluated 290 ultrasound and 188 CT examinations in 136 patients with testicular cancer. Although interpretation of CT was more reproducible than that of ultrasound, there was no significant difference in accuracy of enlarged retroperitoneal lymph node detection between the two modalities. Accuracy improved when both examinations were used and interpreted in combination by a single radiologist. Accuracy rates for ultrasound and CT were equal to those reported for lymphangiography. Lymphangiography and excretory urography are no longer routinely indicated in testicular cancer patients.

Diagnosis and Treatment of Pericardial Cysts

The authors present 2 cases of pericardial cyst accurately diagnosed by cyst puncture and radiographic studies following the injection of contrast material. Both cysts were completely aspirated at the completion of the examination and have not recurred during a three-year period. It is concluded that pericardial cyst injection is a simple technique for the unequivocal diagnosis of pericardial cysts.

Comparison of nmr and ct imaging in the evaluation of metastatic retroperitoneal lymphadenopathy from testicular carcinoma

Twenty-five patients with nonseminomatous germ cell carcinoma of the testis underwent CT and nuclear magnetic resonance (NMR) of the retro-peritoneum followed by radical retroperitoneal lymph node dissection for surgical proof of metastatic disease. Computed tomography correctly predicted the presence or absence of adenopathy in 88% and assigned the correct stage in 84%. Nuclear magnetic resonance had comparable figures of 84 and 8;0%. Computed tomography appeared superior to NMR in detecting other abdominal abnormalities, although these were few in number. Nuclear magnetic resonance is nearly equivalent to CT in staging retroperitoneal lymphadenopathy from testicular cancer and may surpass CT following technical advances and the introduction of oral contrast agents.

Percutaneous Transluminal Angioplasty for Renal Artery Stenosis in a Solitary Functioning Kidney: An Alternative to Surgery in the High-Risk Patient

In five patients with hypertension and marked impairment of renal function due to renal artery stenosis in a solitary functioning kidney, a nonsurgical technique, percutaneous transluminal angioplasty, was used to dilate the stenotic renal artery. Blood pressure improved in all five patients, and renal function improved in three. One patient subsequently died of multiple cardiovascular complications, and in one patient the procedure resulted in acute renal failure requiring hemodialysis. Although preliminary, this experience suggests that percutaneous transluminal angioplasty may provide an attractive alternative to surgery in severely ill patients at high surgical risk.

Collateral circulation to an ischemic kidney.

A review of 301 consecutive abdominal arteriograms given to patients suspected of having occlusive arterial diseases was conducted. In 99 patients, 35% or more stenosis of the renal artery was demonstrated; of these, 40 showed demonstrable collaterals to the ischemic kidney. The adrenal and lumbar arteries contributed four times more frequently than the periureteric arteries to the collateral circulation. Characteristic ureteral notching from the periureteric artery collateral was noted in only 50% of the cases. Intrarenal collaterals were observed and appeared to contribute to the preservation of the size and function of the affected kidney.