Twenty-year trends in neonatal surgery based on a nationwide Japanese surveillance program.

AIM: To discuss the chronological changes observed in a national survey of neonatal surgery in Japan performed every 5 years by the Committee in the Japanese Society of Pediatric Surgeons. METHODS: We analyzed the data obtained for 20 years from 1993 to 2013 and herein report the chronological changes. RESULTS: The number of summarized cases was least in 1993, with 2806 cases, and subsequently increased to 3753 cases in 2013. The mortality rate among the patients with maternal transport linearly decreased (p = 0.0386). Although the proportion of extremely low birth weight infants linearly increased (p = 0.0014), with an annual rate of +0.39 %, the mortality rate linearly decreased (p = 0.0010), with an annual rate of -1.68 %. Moreover, the overall mortality rate linearly decreased (p = 0.0002), with an annual rate of -0.26 %. Most diseases were observed to exhibit a decline in the mortality rate with the same trend as overall mortality. The decline in the mortality rate was most robust with respect to congenital diaphragmatic hernia (CDH). The mortality rates, except for that of CDH, omphalocele, esophageal atresia, and intestinal perforation, declined to 5 % or lower by 2013. CONCLUSIONS: The present findings may be the result of remarkable progress in perinatal management.

A new artificial placenta with a centrifugal pump: long-term total extrauterine support of goat fetuses.

PURPOSE: We tried long-term total extrauterine support of goat fetuses at high pump flow, which was pulsatile and synchronized with the cardiac cycle and at low oxygen tension in the umbilical artery and vein by use of the new artificial placenta. METHOD: This system consisted of an arteriovenous extracorporeal membrane oxygenation using umbilical artery and vein and a thermoregulated water bath. Five goat fetuses (125 +/- 0.7 days of gestation, 2.0 +/- 0.9 kg) were incubated in lactated Ringer's solution. Mean pump flow rate ranged from 113 +/- 16 to 193 +/- 13 ml/min/kg, and umbilical arterial oxygen tension was maintained at 20 +/- 3 to 23 +/- 5 mm Hg for five fetuses. RESULT: Blood gas analysis echocardiogram showed that fetal circulation and sufficient oxygen consumption could be maintained, and fetal extrauterine support conditions were made as similar as possible to physiologic circulatory conditions. We achieved long-term extrauterine support of goat fetuses up to 237 hours (mean 137 +/- 58 hours). CONCLUSION: We believe that this system can be used for experimental models of the fetus and will come into clinical application for fetal extrauterine support systems and backup systems for fetal operations.

Arterio-venous extracorporeal membrane oxygenation of fetal goat incubated in artificial amniotic fluid (artificial placenta): influence on lung growth and maturation.

PURPOSE: The authors attempted to achieve extrauterine support of goat fetuses using arteriovenous extracorporeal membrane oxygenation (A-V ECMO) via umbilical vessels, incubated in a warm bath. They hypothesized that this extrauterine support system affected both the lung growth and maturation of goat fetuses. METHODS: Four goat fetuses at about 125 days' gestation (term, 150 days) were placed in this fetal ECMO system. Their four twin fetuses with similar body weights were harvested and examined for baseline data before ECMO (pre-ECMO data). The mean duration of A-V ECMO was 138.8+/-66.9 hours (range, 87 to 237 hours). Tracheal ligation was performed on all four fetuses to prevent the efflux of fetal tracheal fluid and to collect a sample of tracheal fluid daily. The surfactant and electrolytes of the tracheal fluid were analyzed in pre-ECMO (twin fetuses) and during ECMO. The surfactant of lung tissue, and lung weight were analyzed in pre-ECMO (twin fetuses) and after ECMO. Plasma cortisol and T3 levels were also assayed as hormonal factors that affected lung maturation in pre-ECMO and during ECMO. RESULTS: The phospholipid, phosphatidylcholine, and lecithin-sphingomyelin ratio of the tracheal fluid on day 5 during ECMO (15.0+/-7.1 mg/dL, 53.4+/-26.5%, and 6.2+/-7.1) were elevated above pre-ECMO (10.0+/-4.6 mg/dL, 43.0+/-0.6%, and 4.0+/-2.9), but there were no significant differences. The phosphatidylcholine and disaturated phosphatidylcholine of the lung tissue after ECMO were 72.3+/-15.4 mg/g and 19.2+/-8.2 mg/g, which were significantly higher than pre-ECMO (53.7+/-13.9 mg/g and 11.6+/-4.1 mg/g). The Cl- and K+ of the tracheal fluid were 123.5+/-6.2 mEq/L and 3.7+/-0.7 mEq/L on day 1 during ECMO, which were significantly lower than pre-ECMO (141.8+/-2.9 mEq/L and 6.8+/-1.1 mEq/L), but they then recovered to pre-ECMO levels. The wet and dry lung weights after ECMO were 87.8+/-18.0 g and 6.3+/-1.9 g, which were significantly higher than pre-ECMO (49.3+/-5.9 g and 4.2+/-1.3 g). Plasma cortisol levels and T3 levels during ECMO were significantly higher than pre-ECMO. Electron microscopy demonstrated a higher increase of mature type 11 cells in the lungs after ECMO than pre-ECMO. CONCLUSION: This fetal A-V ECMO system in the bath showed a production of surfactant, the maintenance of ion transport by the pulmonary epithelium, an increase in lung weight, and an increase in mature type II cells, resulting in lung growth and maturation.

[Surgical treatment of primary pulmonary malignant lymphoma: a report of successful case].

A 73-year-old male was admitted to our hospital because of abnormal shadow in the lung field. The pathological report of the percutaneous needle lung biopsy specimen under the CT scan was suggested lymphoma (lymphomatoid granulomatosis suspected), and the right middle lobectomy was performed. Histological and immunohistochemical examinations of the operative specimen revealed B-cell type malignant lymphoma (diffuse pleomorphic lymphoma, large cell type, high grade lymphoma). Primary pulmonary malignant lymphoma is a rare disorder, and it is difficult utilized to distinguish between true malignant lymphoma and lymphomatoid granulomatosis, or pseudolymphoma. Anyway, the first choice of the treatment is surgical excision, because of its satisfactory prognosis.

[Hepatobronchial fistula caused by intrahepatic bile duct carcinoma].

A 49-year-old female was complaining of persistent cough. CT-scan revealed abscess in the right hepatic lobe. Diagnostic percutaneous trans-hepatic abscessography disclosed hepatobronchial fistula, and biopsy of the lesion revealed intrahepatic bile duct carcinoma. She underwent right hepatic lobectomy, right pulmonary lower lobectomy and partial resection of the diaphragm, with removal of the fistulous tract. After operation, cough disappeared, but she died of intraabdominal hemorrhage, 40 days after operation.

A new approach to the treatment of congenital tracheal stenosis: Balloon tracheoplasty and expandable metallic stenting.

BACKGROUND/PURPOSE: Many surgical procedures have been unsatisfactory for the treatment of congenital tracheal stenosis. The authors used intraluminal balloon dilatation and expandable metallic airway stent for this condition. METHODS: From 1997 to 2000, balloon expandable stents (Palmaz) were inserted into the trachea in 5 infants, aged 7 days to 12 months who had severe airway obstruction caused by tracheal stenosis. Tracheal stents were placed after intraluminal balloon dilatation of complete tracheal rings. The stents were placed on balloon catheters and were inserted into the desired position bronchoscopically using x-ray control. They were expanded and fixed in place by inflating the balloon to appropriate diameter. The stents were 10 to 40 mm long and 6 to 8 mm in diameter. RESULTS: Four patients have been relieved of airway obstruction after this procedure. One patient died after 9 months of palliation. In all patients, granulation tissue developed over the stents and could be managed by scraping or balloon compression. Stents have been in place for 9 to 36 months after insertion without any other complication. CONCLUSIONS: Airway stents after balloon tracheoplasty can be left for prolonged periods to relieve tracheal obstruction. This technique may provide an important remedy in infants and children with congenital tracheal stenosis.

Ligation of medically refracted patent ductus arteriosus (PDA) in an extremely low body weight premature infant.

Medically refracted patent ductus arteriosus (PDA) in an extremely low birth weight (ELBW) preterm (gestation 24 weeks 2 days) infant was successfully ligated under general anesthesia in the neonatal intensive care unit (NICU). Pharmacological agents are more effective to close PDA in preterm infants than in full-term infants, although within 48 h three doses of indomethacin were not sufficient to close PDA in this case. At the age of 69 h the infant developed severe symptoms including bradycardia, systemic hypotension, pulmonary hypertension, diastolic steal (reverse distal aorta flow velocity), and anuria. A PDA ligation was thus performed surgically at 72 h of age. General anesthesia and surgical stress were tolerated by this 531 g infant. Postoperatively all symptoms improved dramatically and the general conditions were stable. On the 38th day the endotracheal tube was extubated and on the 50th day nasogastric milk feeding was started. The oxygen supply was weaned on the 78th day. Growth and development until 6 months were within the normal range of very low birth weight infants. A surgical ligation as early as possible in medically refracted PDA in an ELBW infant is thus considered to be a safe and effective treatment. It prevents the development of further complications of cardiopulmonary vascular problems. Color Doppler echocardiography can reliably measure the PDA size, flow velocity, and hemodynamic changes of persistent PDA, even in tiny infants.

Treatment of post-transfusion graft-versus-host disease with nafmostat mesilate, a serine protease inhibitor.

BACKGROUND: Cytotoxic T lymphocytes from donors are thought to injure the target organs in post-transfusion graft-versus-host disease (PT-GVHD) through perforin-granzyme- and Fas-dependent cell killings. The protease involved is a serine protease, and nafmostat mesilate (NM), a serine protease inhibitor, has been found to inhibit the in vitro allocytotoxicity of the T cell clone established from a patient with PT-GVHD, thus suggesting the usefulness of NM for treatment of PT-GVHD. CASE REPORT: A 47-year-old male with esophageal cancer, who received 3 units of packed red cells and 20 units of platelet concentrates from 5 unrelated donors, was diagnosed as having PT-GVHD on the basis of typical clinical features, HLA typing of the patient and the responsible donor, and a mixed chimera of CD8+ lymphocytes on microsatellite DNA polymorphism analysis. NM was administered to inhibit the activity of the serine proteases, thought to be granzymes; a liver dysfunction and thrombocytopenia with leukocytopenia simultaneously improved. Subsequently, a high-dose methylprednisolone pulse therapy and monoclonal anti-CD3 were administered to reduce the donor's proliferating lymphocytes, which resulted in lymphopenia accompanied by elimination of the donor's lymphocytes and normalization of the CD4/CD8 ratio. However, recurrence of the proliferation of the responsible donor's lymphocytes developed after cessation of NM administration, probably because of excessive immunosuppression caused by steroids and the monoclonal anti-CD3. CONCLUSION: This case indicates that administration of a serine protease inhibitor may improve PT-GVHD symptoms by inhibiting cytotoxic T-cell-mediated killing of target cells in fatal PT-GVHD. Steroids and monoclonal anti-CD3 were probably responsible for the transient clinical improvements. More studies are required, however, on mechanisms to eliminate the donor's lymphocytes.