Foreign-Body Pulmonary Embolism

The minimal incidence of pulmonary foreign-body embolism in a general pediatric pathology experience was ascertained by reviewing autopsy records, and the true incidence of pulmonary foreign-body embolism was determined by studying lung sections from 64 autopsies of patients who had undergone cardiac procedures. Seventeen cases of embolism were reported from 370 autopsies, an incidence of 4.6%. The true incidence of pulmonary foreign-body embolism was found to be 21.9% in the cardiac surgical autopsies and the minimal incidence was 5.1% in patients who had other types of surgery. No patients other than those having surgical procedures had embolism. Hair was found to be the embolic material in 35% of cases. Embolic lesions were characterized, staged, and correlated with clinical data. The pathogenesis of this condition is unclear, but it probably involves contamination of surgical materials with particulate matter.     

Pulmonary Hypertension with Coexisting Portal Hypertension

A 10-year-old boy who had portal hypertension secondary to portal fibrosis/intrahepatic biliary atresia developed syncopal episodes related to strenuous activity. A work-up excluded a metabolic or neurologic etiology and cardiac catheterization demonstrated significant pulmonary hypertension. Six months later he died and an autopsy revealed pulmonary plexogenic arteriopathy without microemboli. Previous reported cases of this symptom complex are reviewed. In addition, he had a history of nonspecific colitis, ulcerative stomatitis, and conjunctivitis responsive to steroid therapy. The possible relationship of these manifestations to the portal pulmonary pathology is discussed.     

Total Anomalous Pulmonary Venous Return with the Circular Pulmonary Venous Connection: Outcome of Common Pulmonary Venous Agenesis

A rare case of total anomalous pulmonary venous return, in which the right and left peripheral pulmonary veins connected circularly and there was no central part of the pulmonary vein or the common pulmonary vein, is presented. To our knowledge, total anomalous pulmonary venous return with circular pulmonary venous connection has not been reported previously in the literature. It is thought that the complex connection between peripheral pulmonary veins with the absence of the central part of the pulmonary vein as well as the common pulmonary vein results from common pulmonary venous agenesis.     

Childhood Pulmonary Blastoma: A Pleuropulmonary Variant of the Adult-Type Pulmonary Blastoma

Two fatal childhood cases of the rare pulmonary blastema are reported. One was associated with a congenital cystic adenomatoid malformation. Both neoplasms extended to involve visceral pleura and were entirely composed of blastemal and mesenchymal elements without recognizable neoplastic epithelial components. The mesenchymal component in both instances consisted of malignant rhabdomyoblasts, undifferentiated mesenchyme, and differentiated, apparently benign, cartilage. Review of the literature suggests that these features may be specific for the childhood forms of pulmonary blastoma. It is further suggested that pulmonary blastema, malignant mesenchymoma of the lung, and primary pulmonary rhabdomyosarcoma may have a common pathogenetic origin.     

Pulmonary Agenesis Association with Nonimmune Hydrops

Bilateral pulmonary agenesis is a rare malformation with 13 cases previously reported. To our knowledge, none of these cases have been associated with hydrops fetalis. We report a 36 weeks, hydropic female infant (46XX) with bilateral pulmonary agenesis, unilateral microophthalmia, and bilateral renal dysplasia. These eye and pulmonary findings have now been associated several times and may constitute a new syndrome. Ultrasound examination before delivery showed polyhydramnios and fetal hydrops. At autopsy the heart was structurally normal, aside from absent pulmonary connections. The ductus arteriosus was partially closed. This was felt to be etiologic in the hydrops. As the source of the amniotic fluid here could not be urine or pulmonary secretions, direct fluid transfer from fetal vasculature or skin was the likely origin.     

Pulmonary vein stenosis mimicking chronic lung disease

The presence of recurrent respiratory symptoms and right heart enlargement in an ex-premature infant is suggestive of chronic lung disease. Pulmonary vein stenosis is a rare, progressive disorder that produces similar symptoms and signs. A case is reported in which pulmonary vein stenosis was revealed by Doppler echocardiography, and this application is recommended in similar cases. Pulmonary vein stenosis is an extremely rare and progressive disorder, which, if left untreated, is usually fatal. The possibility of this diagnosis may not be considered during clinical examination and may be overlooked during routine echocardiography.

Conclusion: This report describes a patient with pulmonary vein stenosis in whom the diagnosis was delayed as she had symptoms and signs more commonly associated with chronic lung disease.     

Pulmonary toxicity from carmustine (BCNU): A case report

A patient who had a pneumonectomy for lung carcinoma was treated with carmustine when brain metastases developed. His pulmonary function was mildly compromised prior to the pneumonectomy by many years of smoking. After six months of carmustine therapy [total dose: 2,250 mg (1,200 mg/m²)] he developed interstitial pulmonary fibrosis with histologic changes consistent with drug toxicity. With seven previously reported cases of this drug effect and the addition of our case, carmustine must be added to the list of cancer chemotherapeutic agents that can cause pulmonary toxicity.     

Dysphonia: A Frequently Encountered Symptom in the Evaluation of Infants with Unobstructed Supracardiac Total Anomalous Pulmonary Venous Connection

Total anomalous pulmonary venous connection is a relatively uncommon congenital cardiac anomaly. When there is obstruction to pulmonary venous drainage or severe pulmonary hypertension due to increased pulmonary blood flow, the patient is usually critically ill and the diagnosis is unambiguous. However, in some patients the symptoms may be mild and often overlap with those of other noncardiac diseases. Tachypnea, failure to thrive, and mild cyanosis are the most frequently reported symptoms. In our patient population, we encountered a frequent observation by parents that the child had an alteration in voice which we elected to refer to as dysphonia for the purpose of this study. We retrospectively reviewed all patients with total anomalous pulmonary venous connection who were evaluated at our institution from January 1996 to January 1999 and found that 27% of patients had dysphonia as a presenting complaint to their primary care provider. We hypothesize that the etiology of this phenomenon lies in the possible compression of the left recurrent laryngeal nerve in the vicinity of the dilated pulmonary artery and the left vertical vein.     

Pulmonary Resection for Localized Bronchiectasis in Cystic Fibrosis

ABSTRACT. Three children with cystic fibrosis and localized bronchiectasis were treated by surgical removal of the affected lobe, after attempts to clear the obstructed bronchus by medical therapy and bronchoscopy with suction and lavage had been unsuccessful. Three to six years after surgery, the results are excellent. Pulmonary disease is mild in all patients, shown by chest radiographs and pulmonary function tests. A review of 102 cases reported in the literature demonstrates that pulmonary surgery is effective and can be performed safely in selected patients.     

Pulmonary Malacoplakia in a Child

Pulmonary malacoplakia is a rare condition with only five previously reported cases in the literature, all occurring in adults. We describe a case of malacoplakia of the lung in a 6-year-old female with acute lymphoblastic leukemia in remission and a Rhodococcus equi pneumonia and septicemia. The case demonstrates that pulmonary malacoplakia can occur in immunocompromised pediatric patients and that malacoplakia should, therefore, be added to the list of causes of nodular lung infiltrates in immunocompromised children.     

Persistent Pulmonary Hypertension of the Newborn due to Alveolar Capillary Dysplasia

Three unrelated female term infants died when less than 1 month old from intractable pulmonary hypertension associated with deficient capillaries in airspace walls, anomalous small pulmonary veins in bronchiolar-arterial rays, and medial thickening in small pulmonary arteries together with peripheral muscularization. This complex uascular abnormality in the lungs has been termed alveolar capillary dysplasia and/or misalignment of lung uessels in seven previously reported cases. Each infant also showed abnormally immature parenchymal development in the lungs, as was noted in four of the seven prior cases. One had phocomelia; four of the seven prior cases had a variety of congenital anomalies. The primary pulmonary uascular anomaly is likely to be a failure of fetal lung uascularization dating from the second trimester and to be due to action of an unknown teratogen. Centroacinar veins may represent bronchial veins that do not normally develop beyond the ends of cartilaginous bronchi. Pulmonary arterial occlusive changes are interpreted, as reactive to obstruction at the level of pulmonary arterioles.     

Morphology of Pulmonary Extralobar Sequestration in Neonatal Death by Hyaline Membrane Disease

We present an unusual case of extralobar pulmonary sequestration associated with hyaline membrane disease (HMD) that caused the death of a premature baby in the first day of life. The sequestered parenchyma was nourished by an aberrant aortic vessel. Notable was the presence of typical HMD in all the lung parenchyma perfused by the pulmonary artery; the sequestered lung tissue presented a dysplastic structure compatible with CCAM. A few similar cases have been found in the literature. In all of the reported cases there are morphologic aspects characteristic of HMD in the portions normally receiving blood from the pulmonary artery. These findings suggest the importance of the blood pulmonary circulation in the pathogenesis of HMD, whose exact causes are not fully known.     

Isolated Pulmonary Goodpasture Syndrome

The case of a 13-year-old girl with Goodpasture syndrome is reported. The presentation at this age with only pulmonary hemorrhage is unusual among Goodpasture syndrome patients. The case illustrates well the diagnostic difficulties in Goodpasture syndrome. The choices of treatment modalities available for this disease and the results and risks of such treatment options are reviewed. A discussion and classification of diffuse pulmonary hemorrhage is presented. The case emphasizes that Goodpasture syndrome should be considered in the differential diagnosis of diffuse pulmonary hemorrhage in spite of a lack of renal abnormalities and that serum anti-glomerular basement membrane antibody testing can be of great help in confirming the diagnosis.     

Aortic Valvar Atresia, Interrupted Aortic Arch, and Quadricuspid Pulmonary Valve: A Rare Combination

Aortic atresia and interrupted aortic arch is a rare cardiac combination. Review of the literature revealed nine cases. We present two patients with this combination and the additional finding of quadricuspid pulmonary valves, one of which was severely stenotic. In the latter patient, an aortopulmonary window was present. The other had a unique blood supply to the brachiocephalic arteries and ascending aorta from systemic collateral arteries. To the best of our knowledge, the association of a quadricuspid pulmonary valve with this combination has not been previously reported.     

Pulmonary Interstitial Glycogenosis Associated With Pulmonary Hypertension and Hypertrophic Cardiomyopathy

A neonate with pulmonary interstitial glycogenosis, pulmonary hypertension, and hypertrophic cardiomyopathy is described. The fatal outcome for this patient contrasts with the reported favorable prognosis associated with isolated pulmonary interstitial glycogenosis. To the authors’ knowledge, the association of pulmonary interstitial glycogenosis and hypertrophic cardiomyopathy has not been reported previously. The authors have broadened the phenotype of pulmonary interstitial glycogenosis and demonstrate the diagnostic value of lung biopsy in cases of unexplained neonatal pulmonary hypertension.     

Absent Pulmonary Valve with Tricuspid Atresia or Severe Tricuspid Stenosis: Report of Three Cases and Review of the Literature

Absence of the pulmonary valve occurs usually in association with tetralogy of Fallot and occasionally with an atrial septal defect or as an isolated lesion. Very rarely it occurs with tricuspid atresia, intact ventricular septum, and dysplasia of the right ventricular free wall and of the ventricular septum. We present the clinical, anatomic, and histologic findings of a new case, and for the first time, the data from two patients with absent pulmonary valve and severe tricuspid stenosis, who exhibited similar histologic findings. We also reviewed the clinical and anatomic data of 24 previously published cases and compared them with the new cases. In all three new cases, the myocardium of the right ventricle was very abnormal. In the two cases with tricuspid stenosis, large segments of myocardium were replaced with sinusoids and fibrous tissue. In the case with tricuspid atresia, the right ventricular free wall contained only fibroelastic tissue. The ventricular septum in all three patients showed asymmetric hypertrophy and in two of the three patients, multiple sinusoids had replaced large segments of myocardial cells. The left ventricular free wall myocardium and the walls of the great arteries were unremarkable. Our data indicate that myocardial depletion involving the right ventricular free wall and the ventricular septum and its replacement by sinusoids and fibroelastic tissue occur not only in cases of absent pulmonary valve with tricuspid atresia but also in cases of absent pulmonary valve with tricuspid stenosis. The degree of myocardial depletion varies and is more severe when the tricuspid valve is atretic. Received April 8, 1999; accepted July 12, 1999.     

Total Anomalous Pulmonary Venous Connection in Siblings

ABSTRACT. Three pairs of siblings with total anomalous pulmonary venous connection (TAPVC) are reported. The types of the anomalous venous return (supra- or infracardial connections) varied within the families indicating that genetic regulation deals with the left atrial connection to the intrapulmonary veins. Having failed to establish this connection the intrapulmonary veins can attach themselves to any adjacent venous structure; hence the variety of connections at birth. Our observations also indicate the possibility that specific genetic mechanisms operate in at least some families. Since signs, such as murmurs and cardiomegaly typical of a cardiac anomaly, are not always present in TAPVC, special attention should be paid to any neonatal abnormality in siblings of known cases with TAPVC.     

Primary Pulmonary Hypertension in Childhood: A Report of two Brothers

We report 2 cases of primary pulmonary hypertension in brothers, 6 and 3 years old. Their lungs contained numerous plexiform vascular lesions. The literature is reviewed involving familial cases, especially those in which siblings were affected. Several of these cases lack the pathological verification necessary for acceptance as examples of plexogenic pulmonary arteriopathy.     

Pulmonary thromboembolism and unexpected death in infancy

Review of the Department of Histopathology autopsy files over a 30 year period from 1962 to 1991 revealed only three cases in which unexpected death occurred in infants under 1 year of age due to massive pulmonary thromboemboli. Predisposing factors included necrotizing enterocolitis with gut perforation and sepsis, a ventriculoatrial shunt and idiopathic arterial calcification. Diagnosis of the latter autosomal recessive condition was only made at autopsy. These cases demonstrate that pulmonary thromboembolism is a possible, although exceedingly rare, cause of sudden infant death, that some predisposing factors are unique to infancy and that the source of the pulmonary thromboembolus may be difficult to determine at autopsy. Massive pulmonary thromboembolism in infancy may point to the presence of other significant clinically-unsuspected, disorders.     

Solitary Unilocular Cyst of the Lung with Features of Persistent Interstitial Pulmonary Emphysema: Report of Four Cases

Neonatal interstitial pulmonary emphysema (IPE) is a well-characterized lesion usually presenting in preterm newborns as a complication of respiratory distress syndrome and/or assisted ventilation. Occasionally, IPE may occur spontaneously in infants with no underlying pulmonary disease. Persistence of IPE (PIPE) may be diffuse or localized. Localized PIPE usually presents as multiple cysts 0.3 to 3 cm in one or more lobes of the lung. In this report, we describe four cases of unilocular large cysts (up to 5 cm in diameter) partially lined by uni- and multinucleated histiocytes in a foreign body type reaction and showing gas dissection of the surrounding parenchyma (present in case 1). These histological features favored the diagnosis of PIPE and the cases were interpreted as such, since no other clear-cut diagnosis could be defined. However, because the lesion was limited to one lobe, and the children were full term, asymptomatic at birth and without history of respiratory distress or assisted ventilation, differential diagnosis with other pulmonary cystic lesions of infancy is mandated. Received July 27, 1998; accepted December 21, 1998.