Pulmonary Arteriovenous Fistulas in Children

Abstract

ALTHOUGH large series of pulmonary arteriovenous fistulas have been reported in adults,1-5 only 32 cases surgically treated and 12 cases diagnosed postmortem have been reported in children.2,6-8 The paucity of pediatric cases could be due to either (a) lack of awareness of this entity in pediatric practice, or to (b) its latency in children with onset of symptoms in adults. Lack of recognition of this disease is probably the explanation, since in 25% to 50% of the adult cases signs and symptoms have been evident since childhood.2,5The first autopsy diagnosis of pulmonary arteriovenous fistula was made in 1897, in a 12-year-old boy, by Churton.9 Smith and Horton 10 are credited with the first clinical diagnosis in a 47-year-old patient who had been a "blue baby." The first successful surgical treatment was reported by Hepburn and Dauphiner11 and performed by Shenstone 12a and Janes 12b in 1942. Rodes 13 is, perhaps, the first to make a clear associa¬ tion of pulmonary arteriovenous fistula with hereditary hemorrhagic telangiectasia. Report of CasesCase 1.-A 7-year-old boy was admitted to Saint Francis Hospital in December 1959 because of symptoms highly suggestive of bronchial asthma. His exercise tolerance was described as poor. A cardiac murmur had been heard at S years of age. Family history was not contributory.He weighed 55 lb (25 kg) and he was 52 inches (132 cm) tall. Slight cyanosis and slight clubbing of the fingers were observed. A grade 2 short, late systolic murmur was heard over the midpre-cordium, apex, and axilla. No telangiectasia was observed.The electrocardiogram showed an incomplete right bundle branch block pattern and an axis of +30 degrees.The chest films (Fig 1) demonstrated linear densities extending inferiorly from the left hilus, and localized to the lingula on the lateral view. Films, taken a year and a half earlier, showed the same opacity.The hematocrit value was 45% and the hemo¬ globin 15.4 gm/100 cc.Cardiac catheterization showed mild arterial unsaturation (93.8%) and normal right heart pres¬ sures pulmonary pressure=17/6 with a mean of 10 mm Hg). The cardiac index was 3.95 liters/ min/sq m. Serial angiocardiography showed a fistula with its arterial and venous connections (Fig 2). Surgery was performed, one week later, through a left parascapular incision. Excision of the lingula was performed with removal of the fistula. At this time the anesthesiologist was having con¬ siderable difficulty in inflating the remaining seg¬ ments of the upper lobe ; they were then removed to avoid postoperative complications. The patho¬ logical diagnosis was confirmatory of an arterio¬ venous fistula in the lingula. The postoperative course was uneventful.In the intervening five years since surgery, the boy has done well ; he now has normal exercise tolerance and his development has been normal.For the first time, telangiectatic spots were recently observed on the right and left arm, and epistaxis has occurred about three times a year There was no clubbing or cyanosis. The hemo¬ globin was 13.6 gm/1...

Radiological Case of the Month

“…His mother had occasionally observed these signs since age 4 years, but had noted a marked worsening over the last 2 months. During the same period, a progressive decrease in energy and attention had been noted by the child's first-grade teacher.…”