Successful surgical management of neutropenic enterocolitis in two patients with severe aplastic anemia. Case reports and review of the literature.

We describe two patients with severe aplastic anemia in whom neutropenic enterocolitis developed while they were undergoing treatment at the National Institutes of Health. Both patients had progressive symptoms while receiving optimal medical management and both underwent and survived surgical intervention despite continued prolonged neutropenia in the perioperative period. This experience contrasts with six cases reported in the literature and suggests that surgery can be employed even in patients with profound neutropenia. Thus, in patients who remain persistently septic or who develop clinical deterioration despite medical management or who have other indications for surgical intervention, neutropenia should not be a contraindication to the appropriate or necessary procedure.

Thyrotropin-secreting pituitary carcinoma.

Pituitary tumors rarely metastasize outside the central nervous system. Of the more than 100 reported TSH-secreting adenomas, we now describe the first carcinoma. A 40-yr-old woman had transsphenoidal surgery for a large TSH-secreting pituitary adenoma in 1984. She had increased thyroid hormone levels with a TSH that varied from 16-31 microU/mL, and an unusually high alpha-subunit that ranged from 125-150 ng/mL. Because of residual tumor, she had a left craniotomy in 1985 followed by radiation. Despite these therapies, she had a residual tumor that remained stable until January 1989 when her tumor nearly doubled in size. She received radiation therapy and octreotide with marked diminution of the tumor and clinical improvement. In August 1989, she presented with leg weakness, and magnetic resonance imaging revealed a large sacral mass. A biopsy confirmed that the sacral mass was a metastasis from the pituitary tumor. Due to additional metastases in the lung, she received 5-fluorouracil, cytoxan, and adriamycin, with marked decrease in her lesions. Further substantiation of the metastatic pituitary tumor was made when the patient returned in December 1989 with a pleural effusion containing pituitary tumor cells. Of all the reported cases of TSH-secreting adenomas, this case had the highest alpha-subunit portending future metastases. Furthermore, the apparent response to octreotide and response to chemotherapy are encouraging and suggest that new therapies should be explored. Finally, since TSH-secreting adenomas tend to be more invasive than other pituitary tumors, this case underscores the need for early diagnosis and aggressive treatment of these tumors.

The causes of death in patients with human immunodeficiency virus infection: a clinical and pathologic study with emphasis on the role of pulmonary diseases.

The clinical records and autopsy data of 75 patients dying with AIDS were reviewed to determine the frequency of individual diseases diagnosed premortem and postmortem, the significance of pulmonary processes found in the lungs at autopsy, and the clinical and pathologic causes of death. Cytomegalovirus (CMV) infection was identified histologically either premortem or postmortem in 81% of patients. The lungs and adrenal glands were infected most commonly. Only one-half of CMV infections were recognized premortem. Pneumocystis pneumonia and Kaposi sarcoma occurred in 68% and 59% of patients, respectively, but were not unsuspected premortem in any patient. Visceral involvement with Kaposi sarcoma, however, was frequently recognized only at autopsy. While disseminated M. avium-intracellulare infection was common (31% of patients), histologically documented pulmonary disease was uncommon (3% of patients). Cryptococcal infection, diagnosed in 10 patients, was confined to the central nervous system in only 1 patient. Toxoplasma, in contrast, infected the brain of only 6 patients. All 75 patients had one or more disease processes identified in their lungs or pleurae at autopsy. These processes included opportunistic infections in 76% of patients, neoplasms in 37% (Kaposi sarcoma in 36% and lymphoma in 3%), and other processes in 60%. The most prevalent pathogen, CMV was found in pulmonary tissue from 44 patients and caused significant disease in 21 patients. Five patients died due to CMV pneumonia. Pneumocystis carinii was found at autopsy in 24 patients. In spite of treatment, pneumocystis pneumonia was fatal in 11 patients. One patient died with concomitant CMV and pneumocystis pneumonia. Kaposi sarcoma, identified in the lungs of 23 patients, led to death in 5 patients via upper airway obstruction, hemorrhage, or parenchymal destruction. Other fatal pulmonary processes included bacterial pneumonia in 9 patients, idiopathic diffuse alveolar damage in 5, cryptococcosis in 2, and pulmonary hemorrhage in 1. Specific clinical criteria were used to determine the cause of death due to organ system failure. Fifty-one percent of patients died due to respiratory failure; 16% from neurologic disease; 17% from hypotension that was not caused by respiratory, neurologic, or cardiac disease; and 3% from cardiac dysfunction. Thirteen percent of deaths did not meet the clinical criteria defining these 4 categories. This clinical assessment was combined with autopsy data to identify specific diseases as causes of death.(ABSTRACT TRUNCATED AT 400 WORDS)

Sinusitis in HIV-1 infection.

PURPOSE: To determine the clinical and radiographic characteristics of sinusitis in patients with human immunodeficiency virus type 1 (HIV-1) infection. PATIENTS AND METHODS: A retrospective study was performed that identified all HIV-1-infected patients with sinus radiographs, sinus computed tomograms, or magnetic resonance imaging of the head between 1982 and 1989 (n = 145). Medical record review detailed the clinical course and laboratory parameters in all patients. RESULTS: Eighty-nine patients had radiographic evidence of sinusitis; 75 patients had adequate clinical data and comprise the study group. Acute sinusitis was seen in 10 patients (13%), while all 75 patients had mucosal thickening indicative of chronic sinusitis. Fifty patients (67%) were symptomatic with fever, nasal congestion or discharge, and headache being the most common symptoms; nineteen patients (25%) were asymptomatic when their radiographs showed active disease. The mean CD4 count for the group was 276 cells/mm3; 32 (43%) had CD4 counts less than or equal to 100 cells/mm3. Twenty-three patients (31%) received antibiotics orally, parenterally, or both. CONCLUSIONS: Sinusitis appears to occur frequently in HIV-infected patients, is often asymptomatic, may be recurrent or refractory, and may be associated with declining immunocompetence in HIV-infected patients.

Aortic hypoplasia in homozygous familial hypercholesterolemia.

Diagnosis of hypoplastic aortic root with ultrafast computed tomography provides important clinical information in homozygous familial hypercholesterolemic patients with supravalvular aortic stenosis.

Relation of cholesterol-year score to severity of calcific atherosclerosis and tissue deposition in homozygous familial hypercholesterolemia.

The high concentrations of low-density lipoprotein cholesterol in plasma lead to accelerated atherosclerosis in patients homozygous for familial hypercholesterolemia (FH). We addressed the hypothesis that lipid deposition in the arterial vasculature and in nonvascular tissues in these patients correlates with both the duration and severity of their hypercholesterolemia. The severity of calcific atherosclerosis was defined by calcification scores and a calcified volume determined by electron beam tomography. The extent of tendinous xanthomatosis was quantitated by computed tomography. A cholesterol-year score was calculated based on the age and the yearly mean serum cholesterol concentration of each patient. Seventeen patients homozygous for FH were followed up. The average total cholesterol concentration in the study group was 780 +/- 231 mg/dl (20.2 mmol/L), and the cholesterol-year scores ranged from 2,172 mg-year/dl (56 mmol-year/L) to 32,260 mg-year/dl (834 mmol-year/L). Achilles tendon width (r=0.86) and cross-sectional area (r=0.81; both p <0.001) were best correlated with the cholesterol-year score. In addition, the coronary (r=0.61; p<0.05), ostial (r=0.45; p<0.05), and total (r= 0.77; p<0.001) calcification atherosclerosis scores all were best correlated with the cholesterol-year score. Calcific atherosclerosis was not observed in these patients until the cholesterol-year score exceeded 10,000 mg-year/dl (260 mmol-year/L). These findings establish a direct association of cholesterol-year with extravascular lipid deposition in tissues of patients with FH. The cholesterol-year score may be useful in defining the risk of atherosclerosis in patients with more common forms of hypercholesterolemia.

Hypoxia and cyanosis in alpha-1-antitrypsin deficiency. Cirrhosis as an unusual etiologic factor.

A patient with type PiZZ alpha-1-antitrypsin deficiency was found to have severe hypoxia despite normal pulmonary function testing and a normal chest radiograph. A nuclear medicine ventilation-perfusion study revealed a right-to-left shunt. Computed tomography showed minimal bleb formation, no diffuse changes, and hepatic changes of cirrhosis with portal hypertension. No nodular pulmonary masses or enlarged peripheral pulmonary vessels were found. The diagnosis of diffuse intrapulmonic arteriovenous shunts ("pulmonary spiders of cirrhosis") was suggested and then confirmed with a dynamic radionuclide flow study.

Computed tomography appearance of a right cervical aortic arch.

The clinical diagnosis of cervical aortic arch rests on the detection of a pulsatile mass in the supraclavicular fossa. Unfortunately, clinical differentiation of a cervical arch from an aneurysm of the great vessels can be difficult. Dynamic computed tomography (CT) can aid in this differentiation and avert the need for angiography.

Choice of contrast material for four-hour delayed contrast-enhanced computed tomography of the liver.

Four-hour delayed contrast-enhanced computed tomography (DCECT) shows promise for improved detection of focal liver lesions. No previous studies have examined the use of nonionic contrast materials for DCECT. The authors undertook a case-controlled study in monkeys to define the relative efficacies of iopamidol and diatrizoate for DCECT. In this model, diatrizoate made the liver significantly more opaque at four hours than did iopamidol (mean 126.5 vs 87.6 HU, P less than or equal to 0.05). Opacification of the gallbladder and biliary tract occurred more often and was denser with diatrizoate. The relative efficacies of biliary excretion correlate with similarities in molecular structure. While it has been shown that the increased density from DCECT with diatrizoate may allow detection of more metastatic lesions, the clinical utility of the inferior parenchymal density obtained with iopamidol, extrapolated to be approximately 11 to 14 HU in humans, remains to be-established.

Evaluation of ultrafast CT scanning of the adult abdomen.

Various measures of image quality were compared from adult abdomen scans obtained with a subsecond computed tomographic (CT) scanner (Imatron Ultrafast C-100) and a conventional third-generation whole-body scanner (GE9800). Forty images from 13 patients scanned within 2 hours of each other on both scanners were evaluated with techniques standardized as much as possible for CT exposure factors and contrast enhancement. Two observers in consensus evaluated matched anatomic levels using standard window width and level settings. Each image was graded on a scale of 1 (worst) to 5 (best) for spatial resolution, image noise, and presence and type of artifacts. Overall image quality also was graded. Averaged scores were compared between the two scanners. In all categories, scores were slightly higher for the GE9800. However, the differences in spatial resolution, presence of artifacts, overall image quality were not significant using the sign test. There was a significant difference, in favor of the GE9800, in image noise. The types of artifacts differed; the GE9800 produced more motion artifacts from bowel and surgical clips and the Imatron C-100 produced more rib shadow artifacts projecting on the liver and spleen. While the GE9800 produced abdominal images of slightly superior quality in adults, the Imatron Ultrafast C-100 was shown to produce images suitable for routine abdominal imaging in adults.

Evaluation of STIR imaging as a complement to spin-echo MR and CT of the porta hepatis/hepatoduodenal ligament.

Short TI inversion-recovery (STIR) imaging provides specific advantages over standard spin-echo (SE) MR sequences by producing additive effects of T1 and T2 brightening of pathology and suppression of the signal from surrounding fat. We retrospectively evaluated 12 patients with abnormalities, primarily neoplastic, of the porta hepatis/hepatoduodenal ligament (PH/HdL) with CT and MR imaging, including SE and STIR imaging. Masses on CT were of slightly decreased density compared to liver and seen in contrast to surrounding fat in the PH/HdL region. On MR, T1-weighted images provided comparable anatomic detail to CT, with masses clearly distinguished from surrounding fat due to the low signal intensity of masses as compared to fat. T2-weighted images clearly depicted intrahepatic lesions because of their high signal intensity relative to liver. Increased signal in extrahepatic lesions made them less distinctly seen from surrounding fat. STIR images best demonstrated tumor relative to fat. In six cases, CT was equivalent in demonstrating pathology to the best MR sequence. At least one MR sequence demonstrated pathology better than CT in 6 of 12 cases. In five of these six cases, the STIR sequence was better than CT. Thus, MR, particularly STIR imaging, provides a useful technique in imaging of PH/HdL pathology.

Focal cytomegaly of the adult adrenal cortex. A rare variant of black adenoma.

A pigmented adrenocortical nodule was found incidentally in an adrenal gland resected for endocrine manipulation of breast carcinoma. The sharply circumscribed, nonencapsulated nodule was composed of cells with diameters as large as 95 micron. The cells had ample eosinophilic cytoplasm and contained much lipofuscin pigment. Electron microscopy disclosed tubulovesicular mitochondria, lipofuscin pigment, cytoplasmic vacuolization, and lipid without degenerative changes or intranuclear inclusions. This unusual lesion of adult adrenal glands is best considered as a rare variant of adrenocortical black adenoma.

Multidisciplinary airway stent team: a comprehensive approach and protocol for tracheobronchial stent treatment.

Tracheobronchial stents are being used with increasing frequency to treat major airway obstruction from both malignant and benign processes. Traditionally, stents have been placed via rigid bronchoscopy, flexible bronchoscopy, or fluoroscopy by members of various individual disciplines. We describe a novel multidisciplinary airway stent team (MAST) protocol for tracheobronchial stent placement and endoscopic management of major airway obstruction. A patient with symptoms of airway obstruction is generally first evaluated with a computed tomography scan and a videotaped flexible bronchoscopy. These studies are reviewed by the team otolaryngologist, pulmonologist, and interventional radiologist. A treatment plan, including the type and location of stents and the need for adjuvant therapies, is formulated. Stent placement is performed in the operating room under general anesthesia. Rigid bronchoscopy, with flexible bronchoscopy and fluoroscopy as needed, allows precise stent placement and the best use of various therapeutic methods. The MAST protocol combines the skills, knowledge, and unique therapeutic options of specialists from otolaryngology, pulmonology, and interventional radiology. This approach allows optimal stent placement and the use of other endobronchial therapies, including laser ablation, balloon dilation, photodynamic therapy, cryotherapy, and brachytherapy. A protocol with representative case reports is presented, along with a review and comparison of several of our most commonly used stents. Otolaryngologists who practice bronchoesophagoscopy, by virtue of their operative skill and knowledge of airway management, are well equipped to become leaders of MASTs and are encouraged to initiate MASTs at their institutions.

Pneumocystis carinii in pleural fluid. The cytologic appearance.

We describe the cytologic appearance of Pneumocystis carinii in pleural fluid of a patient with acquired immunodeficiency syndrome and a rapidly accumulating pleural effusion. The diagnosis of P carinii infection was made by examination of air-dried, Diff-Quik-stained Cytospin preparations of the pleural fluid. The diagnostic appearances of P carinii organisms stained by this method and by the Papanicolaou stain are reviewed. The unusual predominance of the trophozoite forms of the organism in this case made Diff-Quik an ideal special stain for identifying the organisms. Furthermore, this case illustrates a novel presentation of P carinii infection and suggests that P carinii should be considered an etiologic agent in the differential diagnosis of pleural effusion in an immunocompromised host.

Hypertrophied thyroid tissue in a thyroglossal duct remnant.

A 37-year-old woman was evaluated for a neck mass. This was located in the midline below the hyoid bone. Thirty-three years had elapsed since she had undergone subtotal thyroidectomy for hyperthyroidism. Eight years prior to examination, the patient had discontinued her thyroid maintenance medications. She had discovered the mass eight months prior to examination. Thyroid scan (Fig. 1) showed intense uptake in the mass. At operation the mass was attached to the midportion of the hyoid bone by a small fibrous band, as is usual in a thyroglossal duct remnant. Biopsy results showed diffuse thyroid hyperplasia. Thyroglossal duct remnants are known to harbor cysts or thyroid carcinoma, but growths of compensatory hyperplastic tissue are unusual.

Electron beam computed tomography screening of the coronary arteries: experience with 3,263 patients at Walter Reed Army Medical Center.

The U.S. Department of Defense desires to reduce the impact of coronary atherosclerosis on its active duty, retired, and dependent populations. Electron beam computed tomography (EBCT) is currently the best way to noninvasively image the coronary arteries directly. Between August 1997 and February 1999, a total of 3,263 patients were scanned by EBCT in the Radiology Department at Walter Reed Army Medical Center. Scans were performed on 2,415 men (74%) and 848 women (26%). The most common age group was 50 to 54 years (25%). Coronary calcification was found in approximately half of the patients (46%), and the magnitude of the score was strongly associated with age and male gender. Average scores increased exponentially with age, doubling every 7 years. In men, average scores ranged from 17 units in those aged 35 to 39 years to 842 in those older than 70 years old. In women, average scores ranged from 1 in those 35 to 39 years to 162 in those older than 70 years. Significant numbers of patients fell into the high-risk categories, with 8% of men in their 40s, 20% of men in their 50s, 33% of men in their 60s, and 49% of men in their 70s with high scores. Scores of more than 400 units were seen in 368 patients (8%) overall. There is a large amount of coronary calcium present in military personnel and their dependents, in patterns that are consistent with previous studies. Many patients had very high scores that are consistent with advanced coronary artery disease. EBCT should play a central role in the identification of occult calcific atherosclerosis of the coronary arteries in military, retired, and dependent patients.

Semierect computed tomography of the abdomen using the Imatron ultrafast CT scanner.

Computed tomography of the abdomen was successfully performed in the semierect position on an Imatron C-100 ultrafast CT scanner. Investigations of gravitational effects on masses or air and/or fluid collections are other potential uses for semierect scanning.