Anomalous Inferior Vena Cava with Azygos Continuation, Dysgenesis of Lung

13 May

Dysgenesis of Lung

Owing to recent developments in roentgenographic tech­nique, the number of reported cases of congenital anomalies such as anomalous inferior vena cava with azygos continuation, congenital deficiency of left pericardium, or dysgenesis of lung diagnosed individually has increased. Thus, such anomalies are now listed in the differential diagnosis of the abnormal lung shadow. Recently, we ob­served a patient who had the triad of all three anomalies mentioned previously. We herein report the case in detail, since, to our knowledge, no case presenting simulta­neously with two or three of these anomalies has been reported.

Case Report

This 43-year-old well-developed Japanese man was first seen in March 1983 as an outpatient complaining of dyspnea and cough with blood in the sputum. These symptoms occurred in the spring of 1981 for the first time and disappeared soon without any treatment; then they recurred in the summer of 1982 and in December 1982. This time, these symptoms continued longer, and dyspnea became more severe than ever, and the patient was admitted to our hospital in March 1983.

FIGURE 1. Chest roentgenogram on admission showing moderate cardiomegaly with deviation to left side of thorax. Second left arch protruded, and its margin was obscure.

Physical examination on admission revealed a temperature of 36.5°C (97.7°F) and a regular pulse rate of 112 beats per minute. The respirator)’ rate was 23/min and regular. The blood pressure was 145/100 mm Hg. The jugular veins were slightly dilated. Coarse crackles were audible over the right lower lung field. Levins grade 3/6 systolic murmur was heard at the sternal border in the left second intercostal space, and a short early and a middiastolic murmur at the apex. The liver was detectable 2 cm below the costal margin. Edema was recognized in both lower limbs.

No family history of congenital anomaly was elicited. Initial laboratory data showed the following: hemoglobin, 13.1 g/dl; hem­atocrit, 37.2 percent; erythrocytes, 4.7 x lOVcu mm; and leukocytes, 4,900/cu mm, with 11 percent band forms, 48 percent granulocytes, 3 percent basophils, 4 percent monocytes, and 34 percent lympho­cytes. The blood chemistry values were as follows: aspartate amino transferase, 16 IU; alkaline phosphatase, 126 IU; blood glucose, 110 mg/dl; albumin, 3.8 mg/dl; globulin, 2.3 mg/dl; and blood urea nitrogen, 16 mg/dl. The erythrocyte sedimentation rate was 7 mm/h.

FIGURE 2. Bronchoscopic view showing blind pouch of left upper bronchus and one of right lobar bronchi branched out from trachea.

The electrocardiogram showed counterclockwise rotation. The chest roentgenogram (Fig 1) revealed moderate cardiac enlarge­ment, with marked deviation to the left. The second left arch was protruded, and the left lower cardiac margin was obscure. Tumor­like shadow was present in the right mediastinum, and both upper lobe bronchi were not detectable. There were increased pulmonary vascular markings and a bilateral pleural effusion.

Symptoms disappeared soon after treatment with diuretics and bronchodilators. Further diagnostic procedures were undertaken to clarify the cause of heart failure, as well as the abnormal pulmonary shadow.

The chest tomogram showed hypoplasia of the left upper lobe bronchus and double branches of the right upper bronchus. The azygos knob showed tumor shadow in the right mediastinum, and no tumor could be seen in the lung field. Pulmonary vessels were dilated. Bronchoscopy (Fig 2) showed a blind pouch of the left upper bronchus and double branches of the right upper bronchus; one upper bronchus branched out from the trachea and the other from the right main bronchus. The view from the trachea appeared to be a trifurcation pattern.