Computed tomography

(CT) of the thorax showed total absence of the right lung with a blindly-ending right main bronchus (aplasia), complete shift of the mediastinum to the right with a hyperinflated left lung herniating to the right hemithorax, and a prominent left pulmonary vasculature (figure 2). Fibre optic bronchoscopy revealed a normal left bronchial tree and a right main bronchus ending in a blind pouch (figure 3). Culture of aspirate from rudimentary bronchus grew Pseudomonas. An electrocardiogram showed a normal sinus rhythm of 90 beats per min, with a rightward axis. Echocardiography Inhibitors,research,lifescience,medical revealed a dilated right heart, with a normal left and right ventricular function, and no valvular abnormality. Figure 1 Chest radiography, showing opaque right hemithorax with the crowding of the ribs and mediastinal shift Inhibitors,research,lifescience,medical to right. Figure 2 Computed tomography (CT) of the thorax, showing the total absence of the right lung with a blindly-ending right main bronchus, complete shift of the mediastinum to the right, and

left lung hyperinflated with herniation to the right hemithorax. Figure 3 Bronchoscopic view at carina, showing a rudimentary right main bronchus ending in a blind pouch. Gynecologic evaluation for primary amenorrhoea was done. The examination revealed well-developed secondary sexual characteristics. There was partial vaginal atresia and Inhibitors,research,lifescience,medical on rectal examination, the pelvis was noted to be Inhibitors,research,lifescience,medical free, suggesting a Müllerian abnormality. Transabdominal ultrasonography confirmed the absent uterus, and both kidneys were not visualised in the lumbar regions. A solitary left TSA HDAC in vitro ectopic kidney, measuring 9.1×6.2 cm, was seen superior to the bladder in the pelvis. CT of the abdomen and pelvis confirmed right renal agenesis with left ectopic (pelvic) kidney and absent uterus (figure Inhibitors,research,lifescience,medical 4). Karyotyping verified the 46 XX pattern, thus confirming

the MRKH syndrome as a cause of the primary amenorrhea. The final diagnosis was confirmed as congenital right lung aplasia with bronchial stump infection, MRKH syndrome, right renal agenesis, and left pelvic kidney. The patient’s respiratory symptoms responded well to a 10-day course of anti-pseudomonal antibiotics and other supportive treatment. She was counselled about the treatment options available to restore her sexual function when she is emotionally mature and ready to start sexual many activity. Figure 4 Contrast-enhanced computed tomography of the pelvis, showing left renal pelvicalyceal system superior to the bladder with a short ureter, suggestive of left ectopic (pelvic) kidney. Discussion Schneider classified pulmonary agenesis into three groups, which were later modified by Boyden.2 There is a complete absence of lung and bronchus and there is no vascular supply to the affected side in type 1 (agenesis).