Outcomes of Alagille syndrome following the Kasai operation: a systematic review and meta-analysis.

PURPOSE: Infants with Alagille syndrome (AGS) frequently develop neonatal cholestasis, and some AGS infants who suspected of biliary atresia subsequently undergo the Kasai operation with the diagnosis of biliary atresia. The aim of this study was to investigate the effect of the Kasai operation on liver and patient outcomes among AGS patients, using a meta-analysis. METHODS: A systematic review and meta-analysis of studies describing the outcomes of AGS patients with/without the Kasai operation were conducted. The analyzed outcomes were liver transplantation, not living with the native liver, and mortality for any reason. RESULTS: We identified 6 studies (394 AGS patients). All studies were retrospective cohort or case-control studies. The incidences of liver transplantation, not living with the native liver, and mortality were significantly higher in AGS patients who underwent the Kasai operation than in those who did not undergo the Kasai operation (odds ratio: 6.46, 95% CI 3.23-12.89, p < 0.00001; odds ratio: 25.88, 95% CI 2.83-236.84, p < 0.004; odds ratio: 15.05, 95% CI 2.70-83.93, p = 0.002, respectively). CONCLUSION: The Kasai operation was associated with poor outcomes in AGS patients. It remains unclear if the Kasai operation directly deteriorates liver and patient outcomes in AGS patients.

Direct hyperbilirubinemia in infants with congenital heart disease.

BACKGROUND: The association between congenital heart disease (CHD) and infantile cholestasis, a key finding for the diagnosis of biliary atresia (BA), has not been previously investigated. The aim of this study was therefore to investigate the characteristics of direct hyperbilirubinemia (D-HB) in infants with CHD. METHODS: All neonates admitted to the present hospital and diagnosed with CHD in 2015 and 2016 were included. D-HB (direct bilirubin ≥ 2.0 mg/dL) at ≤60 days of age and other clinical parameters were retrospectively reviewed. Statistical analysis according to presence of D-HB was performed using chi-squared test or Wilcoxon rank sum test. RESULTS: Seventy-six patients (M:F, 36:40) were included in this study. CHD consisted of ventricular septal defect in 17, patent ductus arteriosus in 10, and other in 49. Thirteen patients (17.1%) had D-HB at ≤60 days of age. Resolution of D-HB (DB < 2.0 mg/dL) occurred in 10 of the 13 patients during the hospital stay, and this occurred in ≤7 days in eight of the 10 patients. Sex, gestational age, birthweight, chromosomal anomalies, need for Fontan operation for CHD repair, and/or cardiac operation were not associated with D-HB at ≤60 days of age. CONCLUSION: While D-HB was frequently observed in infants with CHD, the majority of D-HB cases resolved spontaneously in ≤1 week. Neonatal clinical parameters or CHD status was not predictive of D-HB. D-HB lasting >1 week in infants with CHD should be evaluated for the cause.

Impact of Scoliosis on Laparoscopic Nissen Fundoplication.

AIM: Scoliosis, which is often associated with neurological impairment in children, sometimes makes it difficult to perform laparoscopic procedures. This study assessed the impact of scoliosis on performing laparoscopic Nissen fundoplication. METHODS: Medical records and radiographic examinations of patients who underwent laparoscopic Nissen fundoplication at a single institution from 2006 to 2015 were reviewed retrospectively. Patients' data on age at surgery, height, weight, duration of pneumoperitoneum, and amount of bleeding were collected. The Cobb angle was measured using X-rays, and the direction (right or left) of the scoliotic curve was recorded. The chest compression ratio was calculated using computed tomography axial images. RESULTS: Eighty-five patients were included and analyzed in this study, of which 89% were neurologically impaired. Median age, height, and weight were 120 months, 110 cm, and 17 kg, respectively. A positive correlation between age and the Cobb angle (ρ = 0.64) and a negative correlation between age and the chest compression ratio (ρ = -0.56) were observed. The right-curved scoliotic group showed significantly more bleeding than the nonscoliotic (<10°) group (P = .01; nonscoliotic, 0 mL; right curved, 7.5 mL; left curved, 0 mL). The severe scoliotic group (≥45°) showed more bleeding than the nonscoliotic group (P = .02). Neither the direction of the scoliotic curve nor scoliotic severity showed a significant difference in the duration of pneumoperitoneum. CONCLUSIONS: The older the patient, the more severe their scoliosis and chest compression were. Right-curved or severe scoliosis could be risk factors for intraoperative bleeding in laparoscopic Nissen fundoplication.

Slow release of basic fibroblast growth factor (b-FGF) enhances mechanical properties of rat trachea.

AIM: Severe tracheomalacia is a life-threatening disease, but symptoms usually improve with growth. The aims of this study were to investigate how slow release basic-Fibroblast Growth Factor (b-FGF) acts on tracheal cartilage, and whether growth-promoted trachea is more resistant against an increase in externally-applied pressure. METHODS: Biodegradable gelatin hydrogel sheets soaked in 10 µl of distilled water (sham) or 0.5 or 5 µg/10 µl of b-FGF solution were inserted behind the cervical trachea of three-week-old male Wistar rats. The cervical trachea was harvested 4 weeks later. Extratracheal pressure was increased from 0 to 40 cmH2O in a chamber, while video-recording the internal lumen. The luminal area at each pressure was expressed as a proportion to that at 0 cmH2O. The amounts of collagen type II and glycosaminoglycan were measured by ELISA. RESULTS: The luminal areas at 40 cmH2O in the control (no intervention), sham, and each of the b-FGF groups were 0.65, 0.62, 0.72, and 0.73, respectively. The amounts of collagen type II and glycosaminoglycan in each group were 127, 136, 193, 249 µg/mg, respectively, and 15, 16, 19, 33 µg/mg, respectively. There were significant differences between the control group and the FGF 5 group (P=0.02, 0.01, 0.01, for luminal area, collagen, and glycosaminoglycan, respectively). CONCLUSION: 5 µg of slow-release b-FGF promotes matrix production (collagen type II and glycosaminoglycan). The growth-enhanced trachea was more resistant to collapse, suggesting that slowly released b-FGF might be useful in patients with severe tracheomalacia.

Thoracoscopic plication for diaphragmatic eventration after surgery for congenital heart disease in children.

OBJECTIVE: The aim of this study was to clarify the role of thoracoscopic plication for diaphragmatic eventration after surgery for congenital heart disease (CHD) in children. PATIENTS AND METHODS: We retrospectively reviewed the medical charts of pediatric patients who had undergone thoracoscopic plication of diaphragmatic eventration after surgery for CHD between 2008 and 2013 at our department. RESULTS: Five patients were identified during the study period. The median age and body weight of the patients were 7.6 months and 6.6 kg, respectively. The associated CHDs were pulmonary artery atresia in 3 patients, truncus arteriosus in 1 patient, and double-outlet right ventricle in 1 patient. Four patients needed preoperative mechanical respiratory support. At operation, all the patients received CO2 insufflation (4 mm Hg), and single-lung ventilation was attempted in 3 patients using a bronchial blocker. A sufficient operative field was maintained by CO2 insufflation in all the patients regardless of single-lung ventilation. The procedure was not converted to open operation in any patient. Postoperative extubation was performed in the operating room in 1 patient, on the day of operation in 2 patients, and on postoperative Days 1 and 2 in 2 patients. Air embolism was not observed in any of the patients. Diaphragmatic eventration did not recur in any of the patients after thoracoscopic plication. CONCLUSIONS: Thoracoscopic plication is a safe and effective procedure for pediatric diaphragmatic eventration after surgery for CHD. Considering the sufficient operative field maintained by CO2 insufflation, single-lung ventilation using a bronchial blocker would be unnecessary for this procedure. With its safety and good outcome, early thoracoscopic plication is a good treatment option for pediatric patients with symptomatic diaphragmatic eventration after surgery for CHD.

Cyclic fluctuation of blood pressure in neonatal neuroblastoma.

Herein is described a case of neonatal neuroblastoma with cyclic blood pressure fluctuation and elevated catecholamines. The fluctuations stabilized after treatment with α-adrenergic blocker and the perioperative course was uneventful. The possibility of catecholamine-related symptoms including hypertension, heart failure, and blood pressure fluctuations should be considered in the treatment for neuroblastoma; if they are present, treatment with α-blockers is effective.