Bilateral Ulnar Agenesis: Case Report and Review of the Literature

Bilateral ulnar agenesis is a rare abnormality. A total of 36 cases are analyzed: 35 of these are documented in the literature and 1 stillborn male is presented in this study. Most patients had one of the three conditions: Al-Awadi/Raas-Rothschild syndrome, syndrome of ulnar aplasia with split hand/split foot deformity, or the Brachmann—de Lange syndrome. Fifty percent of all cases with bilateral ulnar agenesis were associated with lower limb defects and these cases, for the most part, also belonged to the aforementioned syndromes. Nonskeletal, internal organ malformations were identified in 34% of all patients. Nine patients presented with isolated bilateral ulnar agenesis. The Al-Awadi/Raas-Rothschild syndrome and the split hand/split foot deformity are heritable disorders. There was no evidence for genetic etiology in most of the other cases. Bilateral ulnar agenesis in our fetus was part of the Brachmann—de Lange syndrome with associated cardiac defect, diaphragmatic hernia, and umbilical artery agenesis.     

Myelolipoma of the thoracic spine.

We describe a myelolipoma of the thoracic spine in a patient with gradual and progressive myelopathy. MR imaging showed this predominately fatty lesion to be extradural in location.     

Malignant fibrous histiocytoma of the temporal bone with endocranial extension.

Cranial malignant fibrous histiocytomas are rare tumors. Most are hypervascular, destructive masses that are similar to other malignant lesions and to malignant fibrous histiocytomas found elsewhere in the body. We describe a myxoid malignant fibrous histiocytoma of the temporal bone, possibly of dural origin, with features that more closely resembled a meningioma at CT, MR imaging, and angiography.     

Magnetization transfer effects in MR-detected multiple sclerosis lesions: comparison with gadolinium-enhanced spin-echo images and nonenhanced T1-weighted images.

PURPOSETo define the relationship between magnetization transfer and blood-brain-barrier breakdown in multiple sclerosis lesions using gadolinium enhancement as an index of the latter.METHODSTwo hundred twenty lesions (high-signal abnormalities on T2-weighted images) in 35 multiple sclerosis patients were studied with gadolinium-enhanced spin-echo imaging and magnetization transfer. Lesions were divided into groups having nodular or uniform enhancement, ring enhancement, or no enhancement after gadolinium administration. For 133 lesions, T1-weighted images without contrast enhancement were also analyzed. These lesions were categorized as isointense or hypointense based on their appearance on the unenhanced T1-weighted images.RESULTSThere was no difference between the magnetization transfer ratio (MTR) of lesions as a function of enhancement. MTR of hypointense lesions on unenhanced T1-weighted images was, however, lower than the MTR of isointense lesions.CONCLUSIONWe speculate that diminished MTR may reflect diminished myelin content and that hypointensity on T1-weighted images corresponds to demyelination. Central regions of ring-enhancing lesions had a lower MTR than the periphery, suggesting that demyelination in multiple sclerosis lesions occurs centrifugally. In addition, the short-repetition-time pulse sequence seems useful in the evaluation of myelin loss in patients with multiple sclerosis.     

ROLE OF NITRIC OXIDE IN THE CONTROL OF THE RENAL MEDULLARY CIRCULATION

1. Nitric oxide (NO) has been implicated as an important controller in the short- and long-term regulation of arterial pressure. Studies performed in our laboratory have demonstrated that chronic intravenous administration of the NO synthase inhibitor N^G-nitro-L-arginine methyl ester (L-NAME) selectively decreases renal medullary blood flow, causes sodium and water retention and leads to hypertension. 2. To determine the importance of the renal medullary effects in this model of hypertension, further studies were conducted to examine the influence of selective stimulation or inhibition of renal medullary NO on whole kidney function and cardiovascular homeostasis. With the use of a unique catheter to directly infuse into the renal medullary interstitial space, stimulation (bradykinin or acetylcholine) or inhibition (L-NAME) of renal medullary NO selectively increased or decreased renal medullary blood flow. 3. The changes in medullary flow in these experiments were associated with parallel changes in sodium and water excretion independent of alterations in renal cortical blood flow or glomerular filtration rate. 4. Studies were then undertaken to examine the long-term effects of selective NO inhibition in the renal medulla on cardiovascular homeostasis. Chronic infusion of L-NAME directly into the renal medullary interstitial space of uninephrectomized Sprague-Dawley rats led to a selective decrease in renal medullary blood flow that was sustained throughout the 5 day L-NAME infusion period. The decrease in medullary blood flow was associated with retention of sodium and the development of hypertension and the effects were reversible. 5. The data reviewed indicate that NO in the renal medulla has a powerful influence on fluid and electrolyte homeostasis and the control of blood pressure.     

Rural Hospital Inpatient Surgical Volume: Cutting-edge Service or Operating on the Margin?

Surgical services are an important part of modern health care, but providing them to isolated rural citizens is especially difficult. Public policy initiatives could influence the supply, training, and distribution of surgeons, much as they have for rural primary care providers. However, so little is known about the proper distribution of surgeons, their contribution to rural health care, and the safety of rural surgery that policy cannot be shaped with confidence. This study examined the volume and complexity of inpatient surgery in rural Washington state as a first step toward a better understanding of the current status of rural surgical services. Information about rural surgical providers was obtained through telephone interviews with administrators at Washington's 42 rural hospitals. The Washington State Department of Health's Commission Hospital Abstract Recording System (CHARS) data provided a count of the annual surgical admissions at rural hospitals. Diagnosis-related group (DRG) weights were used to measure complexity of rural surgical cases. Surgical volume varied greatly among hospitals, even among those with a similar mix of surgical providers. Many hospitals provided a limited set of basic surgical services, while some performed more complex procedures. None of these rural hospitals could be considered high volume when compared to volumes at Seattle hospitals or to research reference criteria that have assessed volume-outcome relationships for surgical procedures. Several hospitals had very low volumes for some complex procedures, raising a question about the safety of performing them. The leaders of small rural hospitals must recognize not only the fiscal and service benefits of surgical services--and these are considerable--but also the potentially adverse effect of low surgical volume on patient outcomes. Policies that encourage the proper training and distribution of surgeons, the retention of basic rural surgical services, and the rational regionalization of complex surgery are likely to enhance the convenience and safety of surgery for rural citizens.     

Experience with the P.A.S.-PORT Venous Access Device in Patients with Gynecologic Malignancies

Experience with the P.A.S.-PORT, a peripherally implanted central venous access device, is evaluated in a retrospective review of 154 patients from July 1991 to June 1994. Blood could not be aspirated from six patients. Complications included temporary minor thrombophlebitis in seven patients (4.5%), symptomatic axillary or subclavian vein thrombosis in five patients (3.2%), clotted port in two patients (1.2%), port pocket cellulitis in two patients (1.2%), and fungal sepsis in two patients (1.2%). In six patients (3.8%) the P.A.S.-PORT had to be removed because of complications. The P.A.S.-PORT facilitated delivery of chemotherapy, parenteral nutrition, blood products, antibiotics, hydration, and blood sampling. It was demonstrated that the P.A.S.-PORT may be inserted and used with a low incidence of complications in gynecologic cancer patients.