Stephen I. Schabel

Evaluation and management of scleroderma lung disease using bronchoalveolar lavage

PURPOSE Bronchoalveolar lavage (BAL) was performed in 43 nonsmoking patients with scleroderma (systemic sclerosis) to determine the frequency of alveolitis, the status of BAL findings over time, and the relationship of such findings to pulmonary status initially and at follow-up. PATIENTS AND METHODS Forty-three nonsmoking patients with systemic sclerosis underwent extensive pulmonary evaluation including pulmonary function tests, chest radiographs, and BAL with analysis of cells, IgG, albumin, immune complexes, and fibronectin. RESULTS Alveolitis was detected on initial BAL evaluation in 21 patients (49%). Alveolitis was characterized by hypercellular lavage fluid, due to an absolute increase in alveolar macrophages and due to an increase in both the absolute number and percentage of granulocytes (neutrophils and eosinophils). Patients with systemic sclerosis had significantly higher levels of IgG and immune complexes in BAL fluid than did control subjects, and alveolar macrophages from patients with systemic sclerosis released higher amounts of fibronectin in vitro. In serial studies, alveolitis was found to persist. Patients with alveolitis had greater dyspnea than patients without alveolitis (p = 0.02), and they had greater reductions in lung volumes and carbon monoxide diffusing capacity (DLCO) (p = 0.004). Furthermore, patients with persistent alveolitis had significantly greater reductions in pulmonary function over time than patients without alveolitis (forced vital capacity [FVC]: -0.69 L versus -0.05 L, p less than 0.001; DLCO: -2.94 mL/minute/mm Hg versus +0.16 mL/minute/mm Hg, p = 0.03). BAL was used to select patients with alveolitis and at risk of pulmonary deterioration, and treatment was instituted with cyclophosphamide and prednisone, resulting in significant improvement in dyspnea (p less than 0.001) and the rate of change of FVC (p = 0.02) and DLCO (p less than 0.001). CONCLUSION We conclude that alveolitis occurs frequently in systemic sclerosis and that BAL is useful in identifying such patients who are at risk for a further decline in pulmonary status. Preliminary observations suggest that treatment of patients with active alveolitis may result in improvement in pulmonary status.

Pulmonary Complications of the Flow-Directed Balloon-Tipped Catheter

Abstract Retrospective analysis of the course of 125 patients in whom flow-directed balloon-tipped catheters were employed showed that in nine (7.2 per cent), pulmonary ischemic lesions appeared to have occurred as a direct result of the use of the catheter. Mechanisms of the pulmonary ischemia included persistent wedging of the catheter tip in a peripheral artery (six cases), obstruction of a more central pulmonary artery by an inflated balloon (one case), and pulmonary embolism from venous thrombosis developing around the catheter (two cases). Awareness of the tendency for spontaneous wedging of the catheter to occur and of the possibility of air remaining in the balloon after use should reduce the frequency of these complications. (N Engl J Med 290:927–931, 1974)

Complications of nasoenteric feeding tubes

Small-bore, silicone nasoenteric feeding tubes are increasingly utilized in the critically ill patient to provide nutritional support. The metallic-weighted tips and stiffening introducing stylets create the potential for misplacement with potentially serious consequences. We have reported our experience with 14 patients who had inadvertent tube misplacement, resulting in complications that included pneumothorax, hydrothorax, empyema, mediastinitis, pneumonia, and esophageal perforation. The incidence of radiographically detected abnormal tube position was 1.3 percent. The presence of cuffed tracheostomy or endotracheal tubes did not prevent this occurrence. The institution of enteral feedings should, therefore, be performed according to strict guidelines which include radiographic confirmation of desired position before feedings are initiated, limited and supervised use of stylets, and a need for special precautions in patients who are obtunded or receiving intubated respiratory assistance.

Progressive systemic sclerosis sine scleroderma presenting as pulmonary interstitial fibrosis

PURPOSE The purpose of this work was to report the clinical features of 10 patients with systemic sclerosis presenting with lung disease in the absence of cutaneous involvement (systemic sclerosis sine scleroderma). PATIENTS AND METHODS The study involved a retrospective review of patients with systemic sclerosis at a tertiary care facility presenting with clinical and/or radiographic evidence of interstitial lung disease in the absence of cutaneous manifestations of scleroderma, and a MEDLINE, computer search for patients who presented with lung disease and later developed taut skin (scleroderma). RESULTS Ten patients with systemic sclerosis sine scleroderma presenting with pulmonary signs or symptoms were discovered. A positive antinuclear antibody (seven of eight), esophageal dysmotility (eight of nine), restrictive lung disease (six of eight), Raynauds phenomenon (five of eight), and abnormal nailfold capillaries (six of six) were present in these patients. Six of these 10 patients developed typical scleroderma from four months to seven years after presentation. Lung pathology was examined in six patients and all had changes consistent with interstitial pneumonitis and fibrosis. Four of the 10 patients had occupations that could have exposed them to potential pulmonary toxins. In contrast to patients with typical scleroderma, all 10 patients we discovered were men. conclusions: Systemic sclerosis should be considered in patients with idiopathic interstitial lung disease even in the absence of classic sclerodermatous cutaneous involvement. An esophagram, antinuclear antibody, and widefield nailfold examination may facilitate the diagnosis.

Intracranial placement of a nasotracheal tube after facial fracture: A rare complication

Extensive facial trauma is often associated with fractures to the skull base, cribriform plate, and sphenoid sinus. Attempted intubation of patients with facial trauma may result in intracranial penetration and placement of nasogastric or nasotracheal tubes into the brain. Such a complication carries significant morbidity and mortality. Intracranial placement of nasogastric tubes has been reported multiple times in the literature. Intracranial placement of a nasotracheal tube, however, has been reported only twice. Such a case is presented, along with a review of the literature and a discussion.

Evaluation of biliary cystadenoma and cystadenocarcinoma

Biliary cystadenomas and cystadenocarcinomas are among the rare neoplasms of the biliary ductal system. This article presents 3 adult patients in whom such large multiloculated biliary tumors were diagnosed. The characteristic features noted on ultrasonography, computed tomography, and angiography of the liver are illustrated and the literature is reviewed.

Obstructive jaundice caused by cavernous transformation of the portal vein

Extrahepatic portal obstruction developing in the neonatal period usually presents with manifestations of portal hypertension. Obstructive jaundice has not previously been described as a complication of this condition. A 22 year-old white male presented with jaundice, decreased appetite and tiredness for six months. He was known to have portal hypertension which followed neonatal omphalitis and portal vein thrombosis. At age four, splenectomy and lieno-renal shunt was done because of bleeding from oesophageal varices. Two further, self-limiting episodes of gastrointestinal bleeding occurred in his teens. He was normally developed and showed no other abnormalities apart from jaundice. Total bilirubin was 10.4 mgm % with a direct fraction of 7.2 mgm%. Alkaline phosphatase was elevated to 344 K.A. units, but liver function tests were otherwise normal. Liver biopsy was unsuccessful, but was complicated by puncture of the gall-bladder with haemorrhage into it, necessitating laparotomy. The wall of the gallblad...

Autosomal Dominant Polycystic Kidney Disease With Liver and Pancreatic Involvement in Early Childhood

In a patient with autosomal dominant polycystic kidney disease, liver and kidney cysts were found by biopsy at the age of 8 months. Computed tomography at the age of 16 years confirmed the presence of liver and kidney cysts and also revealed pancreatic cysts. Such early onset of liver cysts in a patient with autosomal dominant polycystic kidney disease has not been reported previously.

Quantitative analysis of the cervical spinal canal by computed tomography

SummaryComputed tomography measurements of the AP diameter, width, and cross-sectional area of the bony cervical canal were derived from cervical spine examinations of fifty-two normal adults. These quantitative parameters were then used to evaluate 80 patients with various cervical abnormalities to determine the clinical usefulness of the measurements. With the exception of spinal stenosis, quantitative cervical canal analysis was found to be of limited usefulness since normal measurements frequently occurred in the presence of significant cervical pathology.

A Modern Series of Percutaneous Intracavitary Instillation of Amphotericin B for the Treatment of Severe Hemoptysis From Pulmonary Aspergilloma

BACKGROUND Pulmonary aspergillomas may cause life-threatening hemoptysis. The treatment of this condition is problematic because poor pulmonary function often precludes definitive surgical resection. METHODS We retrospectively reviewed all patients hospitalized at our institution for hemoptysis associated with an aspergilloma over an 8-year period and who underwent percutaneous intracavitary instillation of amphotericin B (ICAB). ICAB consisted of catheter placement into the aspergilloma cavity with subsequent instillation of 50 mg amphotericin B in 20 mL 5% dextrose solution daily for 10 days. RESULTS ICAB was attempted for 23 distinct episodes of severe hemoptysis in 20 individual patients. Catheter placement was successful in 21 of the 23 episodes (91%), and of these, ICAB instillation was successfully completed in 20 episodes (95%). In these 20 episodes, hemoptysis ceased by hospital discharge in 17 of 20 patients (85%) and in all 18 who survived until a follow-up visit 1-month after treatment. Pneumothorax occurred in six of 23 (26%) catheter placement attempts without long-term complications. Recurrence of serious hemoptysis occurred after six of 18 episodes for which follow-up was available. Potential risk factors associated with severe, recurrent hemoptysis were a size increase or reappearance of the aspergilloma on a chest CT scan (P = .001), bleeding diathesis (P = .08), and lack of bronchial artery embolization during index hospitalization (P = .07). CONCLUSIONS Our data suggest that ICAB is an effective short-term treatment to control severe hemoptysis caused by pulmonary aspergilloma. The long-term benefit of this procedure is unknown. We identified several potential risk factors for recurrent hemoptysis after ICAB that could be examined prospectively in future trials.