Congenital pulmonary lymphangiectasis. Report of 11 examples with special reference to cardiovascular findings.
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This report describes the course and prognosis of four patients with Noonan syndrome and associated pulmonary lymphangiectasia. All patients had (1) superior deviation of the QRS axis in the frontal plane of the electrocardiogram; (2) moderate to severe valvular pulmonary stenosis, with an additional atrial septal defect secundum type present in two and a small ventricular septal defect in one; and (3) bilateral prominence of the pulmonary interstitial markings (dilated lymphatics), with or without accompanying pleural effusions on the chest radiographs. Surgical repair of the cardiac lesion may cause undue morbidity because of the interruption of the engorged pleural or mediastinal lymphatics.
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We report the case of a 63-year-old male hospitalised for chronic diarrhoea and weight loss of 11 kg within 2 years. The symptoms began after a trip to Thailand. Various investigations were negative and led to the assumption of tropical sprue, which was treated with tetracycline. Within 4 months the malabsorption deteriorated and the patient was readmitted with severe electrolyte imbalance. CT-scan of the abdomen revealed a thickened intestinal wall in the jejunum. Diagnostic laparotomy was performed and, surprisingly, revealed chylascites. Histology in a segment of the jejunum demonstrated intestinal lymphangiectasias as the cause of the malabsorption. These intestinal lymphangiectasias were most probably the sequela of radiotherapy 30 years earlier for testicular teratocarcinoma. Symptomatic therapy with middle chain triglycerides brought about substantially improvement.
The authors report the anatomo-clinical description of a case of a complex malformation of the Ivemark type with asplenism. They draw attention to the presence of a left-sided unilateral congenital pulmonary lymphangectasia due to the absence of the left pulmonary venous trunk. This observation confirms the role of total blockage of pulmonary venous circulation in the aetiology of these very rare cases of congenital pulmonary lymphangectasia.
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