Pleurodesis using OK-432 for persistent pleural effusion after cardiac surgery in the neonatal period or early infancy.

OBJECTIVE: To evaluate the efficacy of pleurodesis using OK-432 after cardiac surgery in the neonatal period or early infancy. METHODS: We retrospectively reviewed the data of 11 consecutive patients who underwent cardiac surgery in the neonatal period or early infancy and pleurodesis using OK-432 for persistent postoperative pleural effusion in two institutions. RESULTS: The median age at surgery was 8 days (interquartile range [IR], 2-18) with a body weight of 2.84 kg (IR, 2.30-3.07). The maximum amount of pleural drainage before pleurodesis was 94.7 (IR, 60.2-107.7) ml/kg/day. Pleurodesis was initiated at postoperative day 20 (IR, 17-22) and performed in bilateral pleural spaces in seven patients and unilateral in four. The median numbers of injection were 4 (IR, 3-6) times per patient and 3 (IR, 2-3) times per pleural space. In 10 patients, pleural effusion was decreased effectively, and drainage tubes were removed without reaccumulation within 15 (IR, 12-28) days after initial pleurodesis. However, in one patient, with severe lymphedema, pleural effusion was uncontrollable, resulting in death due to sepsis. Adverse events were observed in nine patients; temporal deterioration of lung compliance and arterial blood gas occurred in two, insufficient drainage requiring new chest tube(s) in five, temporal atrial tachyarrhythmia in one, and lymphedema in four. CONCLUSIONS: Pleurodesis using OK-432 is effective and reliable for persistent postoperative pleural effusion in neonates and early infants. Most of the complications, which derived from inflammatory reactions, were temporary and controllable. However, severe lymphedema is difficult to control.

Recurrent cellulitis and bacteremia in a patient with Noonan syndrome: A case report.

A 28-year old Japanese man with Noonan syndrome (NS) presented to our emergency department with painful erythema of the trunk and lower extremities since the previous day. He had been diagnosed with protein-losing enteropathy (PLE) with intestinal lymphangiectasia at age 25 years, and undergone lymphaticovenular anastomosis (LVA) twice. Three episodes of cellulitis of both lower extremities had occurred in the past 2 years. Extensive cellulitis with sepsis was diagnosed and piperacillin/tazobactam was started, which was de-escalated to ceftriaxone. He was discharged after 13 days of antibiotic therapy. After discharge, low-dose trimethoprim-sulfamethoxazole (SMZ-TMP) was started as the primary prophylaxis, but three episodes of cellulitis occurred in the next year and were treated with other antibiotics. NS, an autosomal dominant disease known as a RASopathy, is caused by germline mutations in RAS-MAPK pathway genes. Lymphedema resulting from lymphatic abnormalities is a concomitant manifestation in 20 % of patients with NS, and can be a risk factor for cellulitis. Hypoalbuminemia and hypoglobulinemia associated with PLE facilitate infections such as cellulitis. As a treatment for lymphedema in the extremities, LVA has shown objective and subjective improvements in most patients, and some studies have also reported its efficacy for lymphedema in patients with NS. Targeted molecular therapy with mitogen-activated protein kinase enzyme (MEK) inhibitor is used in treatment of cancers with activation of the RAS/MAPK pathway. MEK inhibitors have recently been tried in patients with RASopathies and severe lymphatic disorders, and can lead to rapid resolution of symptoms.

Primary sutureless repair concomitant with the Warden procedure.

A 2-month-old girl who had supracardiac total anomalous pulmonary venous connection (Darling classification type 1b) was referred to our institution. Computed tomography showed that multiple right upper pulmonary veins drained into the vertical vein, near the entry to the superior vena cava. The common pulmonary venous chamber was located lower right than usual, and right upper pulmonary veins were far from the common chamber. We successfully performed primary sutureless repair concomitant with the Warden procedure. Postoperative computed tomography showed unobstructed pulmonary veins and superior vena cava routes, and the vertical vein between right upper and lower pulmonary veins shrank slightly.

Damus-Kay-Stansel procedure with ventricular septal defect enlargement.

A full-term infant who had tricuspid atresia with transposed great arteries, a ventricular septal defect, subpulmonary stenosis with posterior malalignment of the conus septum, bicuspid pulmonary valve, and a high-takeoff left coronary artery was referred to our institution. The subpulmonary stenosis gradually progressed and cyanosis worsened. We successfully performed a Damus-Kay-Stansel procedure and a bidirectional Glenn shunt concomitant with ventricular septal defect enlargement. The conus septum was resected along with thick fibrous tissue through both semilunar valves (without ventriculotomy). Postoperative echocardiography demonstrated that both the ventricular septal defect and the subpulmonary space were enlarged effectively without semilunar valve regurgitation.