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Fibromuscular dysplasia (FMD), is a rare and poorly understood disease that may be confused with atherosclerosis. It is characterized by a narrowing of the artery due to thickening and fibrous replacement of the medial layer, the thick intermediate arterial layer composed of muscle and elastic connective tissue. FMD usually affects the kidney, but may involve other arteries. FMD involving the external iliac artery, located in the groin area, is rare and is usually without symptoms. Eight cases are presented of patients with symptomatic external iliac FMD who were seen over a 15-year period. Three patients had 'blue-toe', or episodic focal digital ischemia (decreased blood supply), caused by microemboli (small clots lodged in the small blood vessels). When removal of the diseased areas of the iliac artery was performed, the source of embolization (clots) and the symptoms disappeared. Four patients had evidence of chronic ischemia with gradual onset of pain and weakness in the leg. This was treated by dilatation of the internal opening (lumen) of the artery or bypass surgery. One patient had acute limb ischemia due to acute dissection of the external iliac artery, separation of the outer and middle layers of the artery wall. When this patient was treated with antiplatelet medications and exercise, her symptoms disappeared completely and the abnormality of lumen of the artery returned to normal. Diagnosis of FMD can only be made by arteriography, and treatment should be according to the symptoms. (Consumer Summary produced by Reliance Medical Information, Inc.)

A significant risk of untreated infrarenal aortic occlusion (blockage occurring in the abdominal aorta below the level of the kidney) is thrombus (blood clot) formation at the stump of the aorta, causing impairment of blood flow to the kidneys. A study was undertaken to determine the incidence of retrograde propagation of thrombus from an aortic stump, and to identify any associated risk factors. The study consisted of 52 patients who survived one year or more following interruption of the infrarenal aorta, which occurred at the time of graft removal due to infection. Blood pressure, renal function, and renal artery anatomy were studied before and after aortic interruption. Patients were followed for an average of 39.2 months. Follow-up aortography was performed on 21 patients (40.4 percent) at an average of 27.7 months following surgery. The results showed no evidence of any suprarenal propagation of aortic thrombus. During follow-up, 41 patients (78 percent) had no change in either blood pressure or serum creatinine, an indicator of kidney function. No evidence of retrograde propagation of thrombus from the aortic stump was observed in any patient. Although the results obtained in this study may not be applicable to patients with spontaneous aortic thrombosis, the information will probably prove useful in their treatment. No factors were identified that placed patients at increased risk for suprarenal aortic thrombosis. (Consumer Summary produced by Reliance Medical Information, Inc.)

• We analyzed the course of 79 adult patients treated for achalasia between 1977 and 1988. Sixty-six patients (84%) had pneumatic dilatation as the primary therapy. Fifty-three patients (80%) had immediate improvement in swallowing. Three patients required immediate redilatation, 2 developed pulmonary aspiration, and 8 (12%) suffered esophageal perforation. Esophageal perforation was treated by closure plus Heller's myotomy in 3 patients, closure only in 3, chest tube in 1, and antibiotics and nasogastric suction in 1. At 4 years' follow-up, 50% of patients who had dilatation remained asymptomatic, 30% had symptoms of gastroesophageal reflux, and 20% had persistent dysphagia. Eight Heller myotomies were performed, with excellent results in 7 and 1 postoperative death from respiratory failure. Seven additional patients with disabling esophageal symptoms after multiple operations for achalasia were ultimately treated by esophagectomy (n 5), hemigastrectomy and Roux-en-Y gastrojejunostomy (n =1), and repeated myotomy (n= 1). All recovered and are able to eat solid food. Thus, our experience indicates that pneumatic dilatation remains unperfected (ie, the line between undertreatment and overtreatment is finer than generally recognized), and unless improvements can be made, the role for surgery may need to be reexpanded.(Arch Surg. 1989;124:929-932)

To investigate the long-term success of heart transplantation in newborn infants who have complex congenital heart disease, we have developed a model of heterotopic heart transplantation in immature pigs. We chose the heterotopic technique because it is simple, does not require cardiopulmonary bypass or heparin, allows for significant size disparity between the recipient and donor hearts, and allows for experimental comparisons between the two hearts. Small newborn piglet hearts are harvested, prepared, and then transplanted into the left chest of larger weanling pigs to augment or substitute for the native left ventricle. Preliminary data from transplants into 49 pigs suggest that the technique is technically possible, the pigs can be immunosuppressed over the long term, and the donor heart can contribute bemodynamically. Experimentally, the model is well designed for the investigation of issues critical for the long-term success of heart transplantation in infants and children, including growth and development, optimal long-term immunosuppression, differences in immunotolerance, and the study of coronary obliterative disease. Clinically, the model has potential applicability in congenital heart anomalies if one native functioning atrium and ventricle are present.