Congenital diaphragmatic defects that present late.

Twenty two patients (age range: 1 month to 11 years) were treated for congenital diaphragmatic defects (excluding hiatus hernia) in the six year period 1983-8. Presenting features were failure to thrive (n = 7), abdominal pain and vomiting (n = 4), chronic respiratory symptoms (n = 3), and inability to wean from ventilatory support (n = 3). The defect was an incidental finding in five patients. Operative repair was performed with no mortality or serious postoperative morbidity. Dramatic improvement occurred in 15 of the 17 symptomatic patients. Awareness of the differential diagnosis should avoid delay in diagnosis or inappropriate treatment. Surgical correction is strongly recommended in all cases.     

Early treatment of juvenile xanthogranuloma of the iris with subconjunctival steroids.

Five infants with biopsy proved juvenile xanthogranuloma of the iris were reviewed. Early treatment with subconjunctival injection of steroids and topical steroid drops resulted in regression of the lesion in four patients. One patient, reviewed at the age of 8 months after glaucoma which had developed secondary to the iris xanthogranuloma, had been treated by surgery alone: the visual outcome was very poor.     

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.     

Renal angiomyolipoma: 6 case reports and literature review

We report 6 cases of renal angiomyolipomas and review the pertinent literature concerning this disease. One case involved the rare association of tuberous sclerosis and pregnancy. Another patient without tuberous sclerosis had renal cell carcinoma and later suffered a contralateral angiomyolipoma. The pathological conditions of renal angiomyolipomas with and without tuberous sclerosis are discussed. Since angiomyolipomas present with multiple clinical similarities to renal cell carcinoma, the primary task for the clinician is to differentiate this hamartoma from carcinoma. The distinguishing characteristics and the clinical management of renal angiomyolipomas are discussed.