Case presentation
A 40-year-old, gravida 4, para 2 woman was referred to our perinatal center at 34 weeks’ gestation because of suspected left congenital diaphragmatic hernia (CDH). No abnormal family history was recorded.
A female infant weighing 1,920 g was born via Caesarean section at 37 weeks and 2 days’ gestation. Immediately after delivery, she was intubated, and synchronous intermittent mandatory ventilation (SIMV) was started. Low-set ears and widely spaced eyes were shown at birth. Chest X-ray showed an elevated hemidiaphragm with the mediastinum shifted to the right side and gastric herniation into the thoracic cavity. An echocardiogram on the first day of life showed ventricular septal defect (VSD), coarctation of aorta (CoA), and a large patent ductus arteriosus (PDA) with bidirectional shunt and severe pulmonary hypertension. A genetic analysis was performed because there were multiple malformations, revealing trisomy 18.
The respiratory and circulatory dynamics gradually stabilized, and surgery was performed at three days of life. Laparotomy revealed left diaphragmatic hernia with a hernial sac containing the left lateral segment of the liver and stomach. The sac was removed, and patch closure was performed with a Gore-Tex sheet.
Mechanical ventilation was also required after surgery. Chest X-ray showed that the left pleural effusion had increased gradually after initiating breast milk feeding (Figure 1a,b), so thoracentesis was performed at 15 days of life, and chylothorax was confirmed by the appearance and analysis of pleural effusion (Figure 1c, chylous effusion and elevated triglyceride 358 mg/dL). Furthermore, bilateral pleural effusion was shown on ultrasonography and chest X-ray, so bilateral thoracic drainage was performed. Conservative therapy with fasting and octreotide and corticosteroids was initiated, but the pleural effusion did not decrease. Octreotide infusion was initiated at 0.5 μg/kg/h and increased to a maximum dose of 10 μg/kg/h.
High pulmonary blood flow due to VSD became apparent, so bilateral pulmonary artery banding was performed at 25 days of life. The chylous effusion further increased after cardiovascular surgery (Figure 2). The chylothorax was resistant to conservative therapy for more than 3 weeks, so pleurodesis using OK-432 was planned at 38 days after obtaining informed consent. First, 1 KE OK-432 (at a concentration of 1 KE in 10 ml normal saline) was injected into the right pleural cavity. The chest drain was clamped after injection of OK-432 for as long as possible. The patient’s posture was changed from the supine position to the right lateral decubitus position two hours later and to the left lateral decubitus position a further two hours later. In the first pleurodesis, the chest drain was clamped for only four hours because the breathing status deteriorated as the pleural effusion increased. Chylous effusion was not decreased after initial pleurodesis. In the second pleurodesis session at 45 days of life, the same method was performed and led to the same result. The concentration was therefore changed from 0.1 KE/ml to 0.2 KE/ml, and 1 KE of OK-432 was injected into the same side at 52 days, with the chest drain clamped for 9 h. The same method was again used at 58 and 67 days, with the chest drain clamped for 14 and 20 h, respectively. The chylous effusion decreased gradually after the third pleurodesis procedure (Figure 2). The left and right chest drains were able to be removed at 72 and 74 days, respectively. There was no recurrence of chylothorax after removing the drainage tube. Mechanical ventilation was able to be withdrawn at 143 days. At present, only oxygenation with a 0.5-L nasal cannula is required at 1 year old, with no recurrence of chylothorax or left CDH having been noted (Figure 3).