De novo Circumscribed Pulmonary Lob...

De novo Circumscribed Pulmonary Lobar Cystic Lymphatic Anomaly in a Young Boy

Diffusely anomalous pulmonary lymphatic drainage in children is seen in the neonatal period. It is referr to as lymphangiectasis and attends to be uniformly fatal shortly after birth. it may be isolated or associated with cardiovascular malformations and, in particular, an anomalous pulmonary venous recur Occasionally, it may be part of a generalized lymphatic dysplasia affecting many sites. [1-3]

Apparently primary nevertheless localized pulmonary lymphatic anomalies have been reported forward rare occasions in adults. [4-8] They have been called lymphangiomas or lymphangiectasis. of that kind lesions, to our knowledge, are exceptional in children, [910] which makes the lesions unlikely to be included in the differential diagnosis of circumscribed cystic pulmonary lesins. This report suggests to document such a case and discuss its pathogenesis.

CASE REPORT

A nine-year-old male child was admitted to St. Christopher's Hospital for Children with the chief complaint of heat and hemoptysis. Born 12 weeks prematurely, he had required mechanical ventilation for the first three month of life and had bring outed necrotizing enterocolitis, which was treated surgically. At eight years of age, he was incidentally fix to have a small infiltrate in his right lower lobe, however was lost to follow-up until he make knowned low-grade fever and hemoptysis a year later. At this time, his PPD proof and cultures of sputum were negative and his chet x-ray film and CT showed a rotund well-defined, large right lower lobe mass (Fig 1) Contrast medium was administered during the studious mood and failed to demonstrate any arteriovenous connection. A right lower lobectomy was performed.

Pathologic Study

At the posterior basal portion of the resected lobe, a spherical mass widened from the pleura into the pulmonary parenchyma (Fig 2) The visceral pleural surface covering the mass contained multiple dark r pouchs measuring 0.2 to 0.5 cm in diameter. The intersect surface of the mass showed it to be 45 cm in diameter, orbicular and well demarcated. It had a spongeous consistency and was made of numerous cystic spaces filled with blood-stained fluid.

Histologic examination revealed a network of channels, principally of which centered around the bronchioles. a certain quantity of were present in the septa and in the subpleural region. There was no capsule separating the lesion from the surrounding parenchyma. The channels varied in size, and their walls were of variable thickness. the inner surface of the channels was lined according to flattened endothelial-looking cells (Fig 3) The walls were compos of connective tissue in which, in more [i]or[/i] less areas, groups of smooth muscle fibers were at hand Focal aggregates of lymphocyte could be seen in the walls of the channels and in the parenchyma adjacent to the lesion. an channels were filled with r line cells; others contained eosinophilic fluid. The surrounding pulmonary tissue was the site of a mild peribronchiolar lymphocytic infiltration. Acute intra-alveoalar hemorrhage was prominent in a hardly any ares.

DISCUSSION

Diffuse or localized dysplastic unfolding and increased intralymphatic pressure are essentially the sum of two units pathogenetic mechanisms traditionally invoked to explian the mien of lymphatic anomalies in children.

In the lung neonatal lymphangiectasis is perceived as resulting from diffuse dysplasia of the lymphatic network. Lymphangiomas, despite their "tumor-sounding" name, are a scaled-down version resulting from a localized error in growth and organization of the lymphatic tissue indigenous to the area (hamaromas). Increase in intralymphatic urgency compression, and obstruction of the lymphatics are operative where lymphagiectasis is seen in association with anomalous pulmonary venous answer cardiovascular anomalies, adn lymphatic invasion from tumors, and when lymphangiectasis perform the operations indicated ins after significant scarring has taken place in the interstitium.

In the cse of this patient, it is not possible to categorically establish the pathogenetic mechanism involved in the disclosure of the pulmonary lesion. Its first detection at eight years of age and gradual enlargement upon sequential chest roentgenograms is not necessarily an indication in favor of or against the diagnosis of lymphatic hamartoma (lymphagioma). It is well established that vascular hamartomas may be inconspicuous at birth. They enlarge by the agency of opening of collapsed channels, in addition to their natural extension which is coordinated with that of the quiet of the body. Therefore, this lesion, in the absence of detectable morphologic cause for its unravelling could be construed to be a lymphagioma, similar to the lesions reported ten years ago in three lads aged 13,16, and 19 years. [10] In pair of those cases, there was associated mediastinal involvement, and sum of two units lobes were affected. forward the other hand, one cannot easily dismiss the possible part played by pulmonary scarring which may have perform the operations indicated ined following the stormy perinatal period this child went by means of and which required mechanical ventilation for the first three month of life. Lymphangiectasis does lay open in the course of bronchopulmonary dysplasia; and although at nine years of age, there is no evidence of residual pulmonary damage, it is not inconceivable that this might have played a part in the pathogenesis of the existing lesion. Unfortunately, at this stage, we know of no way to answer the question, although a significant coming increase in the number of cases of late-onset localized pulmonary lymphatic anomalies would definitely favor like an explanation.