Lymphangioma is a benign and common disorder in the pediatric age group. Cervical cystic hygroma was first described clinically in 1828 by Redenbacker and pathologically in 1843 by Wernher.1 They are frequently seen in the neck (75%) and axilla (20%).2 Most mediastinal cystic hygromas are extensions of cervical lesions, and cystic hygroma confined solely to the mediastinum is rarely encountered. 3,4 We report a case of a female child with a purely extensive mediastinal cystic hygroma, outlining aspects of its unusual clinical features and management.
Case ReportA three-year-old female was referred to our hospital because of chest pain and bulging of the right side of the chest of two weeks' duration. The parents gave a history of weight loss, but there was no fever. On examination, she was found to be pale, but not jaundiced or cyanotic. The right side of the chest was found to bulge, in comparison with the left. There was decreased air entry on the right side of the chest, with dullness on percussion. Chest x-ray revealed a right-sided pleural effusion. Thoracentesis yielded serosanguinous fluid, which contained gelatinous material. Biochemical and cytological analysis of the fluid gave the following results: glucose 90 mg/dL, total protein 4.6 g/dL, LDH 115 U/L and 8200 WBC U/L, predominantly lymphocytes and many RBCs. Cytology of the aspirated fluid revealed no malignant cells. Bacterial, fungal and mycobacterial cultures showed no growth. PPD was negative.A right-sided thoracostomy tube was inserted and postdrainage chest x-ray ( Figure 1) revealed a large rightsided mass. HCG was normal and serum BHCG was <5. Ultrasonography of the chest could not be done because of the lack of a proper transducer. CT scan of chest (Figure 2) showed a large right-sided lobulated mass occupying the entire anterior mediastinum. The left margins of the mass were inseparable from the pericardial surface of the heart, which was displaced to the left. The chest was opened via a right anterolateral thoracotomy through the bed of the 5th rib. There was a large tumor which was fixed and infiltrating into the anterior chest wall. The posterior part of the tumor was firm, but the anterior part was friable and soft. There was a possibility of teratoma or lymphoma, and a large biopsy was taken. Histology revealed a vascular tumor made up of various sizes of vascular channels lined by endothelium (Figure 3). The stroma was edematous and myxoid, containing spindle and stellate cells with mild nuclear pleomorphism and no mitosis (Figure 4). The stroma contained a diffuse infiltrate of lymphonuclear cells and neutrophils. A fibrous capsule was seen with a few thick fibrous septa. The diagnosis of cystic lymphangioma was made. The patient continued to be symptomatic and so the chest was re-explored and near complete excision of the mass was done, leaving only small fragments that were densely adherent to the hilum of the lung. The cysts near the hilum of the lung and adherent to the pulmonary vessels were deroofed and as much of the capsule ...