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).