Nuchal-type fibroma in two related patients with Gardner's syndrome.

Nuchal-type fibroma is a distinct subcutaneous and dermal fibrous tissue proliferation that has been previously definitely identified in one patient with Gardner's syndrome and has been possibly present in two others. Gardner's syndrome is an autosomal-dominant condition with variable expressivity that comprises epidermoid cysts, fibrous tumors, osteomas, intestinal polyposis, as well as other findings. We report two cases of nuchal-type fibroma presenting in a 13-year-old boy in the right upper back and in his 60-year-old grandfather in the upper chest at the posterior axillary line. Both individuals carried a diagnosis of Gardner's syndrome and neither of them had diabetes. Although the boy has as of now only presented with cutaneous manifestations of Gardner's syndrome, his grandfather has exhibited both cutaneous and intestinal evidence of this syndrome. In addition, the boy's mother and her sister have documented Gardner's syndrome. Light microscopic findings of nuchal-type fibroma from both patients include paucicellular, haphazardly arranged collagen bundles with entrapped adipose tissue. A marked diminution of elastic fibers was noted with Van-Gieson stains. The lesions were diffusely positive for CD34 and contained a few factor XIIIa-positive cells. Electron microscopic analysis revealed no differences between the collagen comprising the nuchal-type fibroma as compared with control dermal collagen obtained from skin away from the tumor. These cases strengthen the view that there is an association between nuchal-type fibroma and Gardner's syndrome.

Maintaining a computer-based patient education library.

The demand for patient education, both from patients themselves and from accreditation agencies for health and managed care organizations, is becoming more insistent. This article outlines the components of a system that maintains a library of computer-based patient education handouts that can be quickly integrated into a physician's practice.

Laparoscopic appendectomy, is it worth it?

The recent experience with open appendectomy was compared to our initial experience with laparoscopic appendectomy. Thirty-eight patients had open appendectomy for acute appendicitis. Two major and four minor complications occurred. Concurrently, 39 patients had laparoscopic appendectomy. There was one major and one minor complication. Of the laparoscopic patients, 69% received less than 24 hours of parenteral postoperative analgesia, compared to 44% of the patients in the open group. Fifteen of 39 laparoscopic patients (38%) were discharged within 24 hours of operation versus 3 of 38 (8%) in the open group. Total mean hospital cost for the laparoscopic group, $7,500, was significantly greater than for the open group, $5,700, because of increased laparoscopic equipment charges. Both open and laparoscopic appendectomy procedures were performed with minimal morbidity. The benefits of laparoscopy were earlier hospital discharge and less parenteral analgesic use, but it was significantly more expensive.

Tumor necrosis factor-induced mortality is reversed with cyclooxygenase inhibition.

OBJECTIVE: The authors hypothesized that TNF would induce eicosanoid synthesis, and a cyclooxygenase inhibitor would attenuate both eicosanoid synthesis and improve survival in an LD90 TNF-induced (150 ng/kg/i.v./5 min) mortality model. SUMMARY BACKGROUND DATA: Tumor necrosis factor is a cardinal mediator in sepsis; however, little is known about its effects on arachidonate metabolism. METHODS: Conscious male rats with carotid arterial and jugular venous catheters were randomized for mortality: group I, TNF alone (150 kg/i.v./15 min, n = 30); group II, ibuprofen (30 mg/kg/i.v. at t = -20 and +240 min), plus TNF, (n = 28); and for hemodynamics, eicosanoid synthesis, blood gases: group III, TNF alone, (n = 8); group IV, ibuprofen + TNF (n = 8); group V, monoclonal antibody to TNF plus TNF (n = 8). Mortality was determined at 4-72 hr. Other parameters determined over 4 hours (0, 5, 60, 120, 240 min). RESULTS: TNF stimulated synthesis of (a) TXB2 (71 +/- 30 pg/ml, mean +/- SE at base vs. 117 +/- 18 at 4 hr, p < 0.02); (b) PGE2 (70 +/- 6 pg/ml at base vs. 231 +/- 68 at 4 hr, p < 0.02); (c) 6PGF (52 +/- 6 pg/ml at base vs. 250 +/- 80 at 4 hr, p < 0.02). Ibuprofen significantly (p < 0.05) inhibited eicosanoid synthesis from TNF. TNF-induced mortality (87%, 26/30) was dramatically decreased with ibuprofen (11%, 3/28), at 4, 24, and 72 hr (p < 0.01). Monoclonal antibody to TNF prevented all abnormalities and had 100% survival. Hemodynamic events were similar in both groups, but metabolic acidosis was attenuated with ibuprofen. CONCLUSIONS: TNF stimulates arachidonic acid metabolism in vivo. A cyclooxygenase inhibitor attenuates eicosanoid synthesis and dramatically improves survival. TNF appears to have different effect on tissues that synthesize certain eicosanoids. Hypotension from TNF is not mediated via the eicosanoids. TNF-induced mortality, like endotoxemia/sepsis may be mediated, in part, via arachidonic acid metabolites. These new findings support the notion that cyclooxygenase inhibitors may be used as adjunctive therapy in clinical sepsis.

Extracorporeal membrane oxygenation as treatment of severe meconium aspiration syndrome.

Meconium aspiration syndrome (MAS) is a common cause of morbidity and mortality in neonates. Chemical pneumonitis can lead to persistent pulmonary hypertension of the newborn (PPHN) with irreversible hypoxia and death. Extracorporeal membrane oxygenation (ECMO) for the treatment of severe PPHN became available at the Ochsner Foundation Hospital in September 1983. We reviewed the first 28 cases in which ECMO was used for the treatment of PPHN due to severe MAS; 26 of the 28 infants survived. During the three years preceding our development of ECMO capability, ten neonates had PPHN due to severe MAS and met the criteria for ECMO; only three survived. The difference in survival demonstrates the efficacy of ECMO for the treatment of severe MAS. We believe that when established criteria are met, ECMO should be instituted without delay.

Persistent pulmonary hypertension in the neonate.

Respiratory failure is the leading cause of death in the neonatal period. The anatomic and functional basis for this, particularly in full-term infants, most often is persistent pulmonary hypertension of the neonate (PPHN). This condition is reversible but can cause very severe and unrelenting respiratory failure and ultimate death when uncontrolled. Recent technologic advances have expanded the scope of therapy available for PPHN, resulting in increasing therapeutic success for these critically ill infants. This article reviews the anatomic and functional anomalies of PPHN, as well as the methods of diagnosis and discusses current treatment.

Extracorporeal membrane oxygenation for respiratory and cardiac failure in infants and children.

Fifty-three neonates and seven pediatric patients were treated with extracorporeal membrane oxygenation from September 1983 until April 1986. Venoarterial bypass was achieved by cannulating the right atrium via the right internal jugular vein and the aortic arch via the right common carotid artery. In the neonatal group, 40 infants with acute respiratory failure were treated, and 36 (90%) survived. Five infants with congenital heart disease were treated and three (60%) survived. Among the eight patients with congenital diaphragmatic hernia, there were three (38%) survivors. In the pediatric group, four patients were treated for ventricular failure after cardiac operations. Two were weaned from bypass, with one long-term survivor. Three patients with acute respiratory failure were treated, with one survivor. salvaging high-risk neonates with minimal morbidity and mortality. It has also been useful in the support of infants with congenital heart disease and congenital diaphragmatic hernia. In pediatric patients one cannot expect to get results that are comparable to those found in neonates. Still, this modality can be useful in salvaging some moribund patients with pulmonary or cardiac failure, or both.

Extracorporeal membrane oxygenation for newborn respiratory failure.

Respiratory failure is the leading cause of death in the newborn. Conventional therapy is very successful with 80% of infants weaned from ventilatory support. For neonates with severe respiratory failure, unresponsive to maximal medical therapy, extracorporeal membrane oxygenation (ECMO) offers an alternative means of management. Venoarterial bypass is achieved by cannulating the right atrium via the internal jugular vein and the aortic arch via the right common carotid artery. A 5-inch roller pump is used to circulate the blood through a 0.4 or 0.8 m2 silicone membrane lung. Management includes heparinization, intravenous alimentation, antibiotic coverage, and reduction of FiO2 and airway pressure. Thirty infants aged 12 to 186 hours were placed on ECMO. Each met strict criteria designed to predict greater than 90% mortality. Time on bypass ranged from 37 to 250 hours. Success, defined by weaning from ECMO and ventilatory support, was achieved in 23. Twenty-one remain alive; 18 have excellent outcome with normal growth and development although follow-up is short (1 to 19 mos). These results corroborate reports from the pioneers of the technique and further support the use of ECMO for neonates with respiratory failure unresponsive to conventional therapy.