Shigehiro Takahashi

Bifidobacterium septicemia associated with postoperative probiotic therapy in a neonate with omphalocele.

We report the one case of sepsis caused by Bifidobacterium breve administered as probiotic therapy. Probiotics can be a potential cause of an invasive disease and should be used with care in vulnerable patients.

The current profile and outcome of congenital diaphragmatic hernia: a nationwide survey in Japan.

BACKGROUND/PURPOSE Few nationwide surveys have been reported regarding the perinatal status, clinical course and postnatal outcome of cases with congenital diaphragmatic hernia (CDH). The aim of this study was to review the current profile and the outcomes of a large cohort of CDH cases in Japan. METHODS A nationwide retrospective cohort study was conducted on neonates diagnosed to have CDH between January 2006 and December 2010. The questionnaires were sent to 159 representative regional institutions and 109 (68.6%) institutions responded to the preliminary survey which had 674 cases. Eleven institutions which had 60 CDH neonates did not respond to the second questionnaire, and 26 institutions had no cases. Finally, 614 CDH neonates from 72 institutions had been collected and were used in the detailed survey. The perinatal status, clinical course and the postnatal outcome were reviewed. Survival was defined as infants alive at hospital discharge, at the time of transfer or still in the hospital at the time of questionnaire, which was confirmed during the period from July 2011 to November 2011 by the investigators. RESULTS Four hundred sixty-three (75.4%) of 614 CDH neonates survived in this study. The overall survival rate of neonates with isolated CDH was 84.0%. A total of 444 (72.0%) patients were prenatally diagnosed, and had a survival rate of 70.8%. Four hundred thirty-three (70.9%) patients were treated with high-frequency oscillatory ventilation (HFOV) as the initial ventilation, 344 (56.0%) patients received inhaled nitric oxide (iNO) and 43 (7.0%) required extracorporeal membrane oxygenation (ECMO). The overall survival rates of the CDH neonates who had been treated using HFOV, iNO and ECMO were 74.3%, 68.3% and 37.2%, respectively. CONCLUSIONS This study demonstrated that the current status for CDH treatment in Japan and the overall survival rate were comparable to those of recent reports from other countries.

Evaluating mortality and disease severity in congenital diaphragmatic hernia using the McGoon and pulmonary artery indices.

PURPOSE Lung hypoplasia is associated with mortality in congenital diaphragmatic hernia (CDH). However, the association between lung hypoplasia and disease severity is unclear. Early prediction of disease severity would provide parents with more precise information about the anticipated course of treatment, minimize treatment disruption, and maximize the efficient management of patients with CDH. We aimed at identifying the relationship between McGoon index (MGI) and pulmonary artery index (PAI) scores and disease severity among infants with CDH. METHODS We retrospectively reviewed the medical records of 19 high-risk patients with CDH born between January 2006 and December 2007. McGoon index and PAI scores were determined on admission. We evaluated statistically the relationship between these scores and variables representing severity as follows: number of vasodilators, use of inhaled nitric oxide (iNO), closed method of diaphragm, duration of intubation, duration of hospitalization, and use of home oxygen therapy. Statistical significance was P < .05. RESULTS Overall median MGI and PAI scores were 1.40 and 108, respectively; scores for nonsurvivors were significantly (P < .05 and P < .01, respectively) lower than those for survivors. Among survivors, PAI scores were significantly (P < .05) lower in infants requiring iNO than in infants not requiring iNO and patch repair. The PAI scores were significantly correlated with the number of vasodilators (r = -0.789; P < .01) and duration of intubation (r = -0.610; P < .05). CONCLUSIONS McGoon index (cutoff value, 1.31) and PAI (cutoff value, 90) are reliable indices for predicting mortality in CDH. Pulmonary artery index appears to be more useful than MGI for predicting disease severity among survivors.

Effect of hospital volume on the mortality of congenital diaphragmatic hernia in Japan

During the last decade, new supportive modalities and new therapeutic strategies to treat congenital diaphragmatic hernia (CDH) have been introduced. In Japan, the large number of hospitals prevents centralizing infants with CDH in tertiary centers. The aim of this study was to evaluate the correlations between the number of CDH patients, survival rates, and the current strategies employed to treat CDH at the individual hospitals.

Persistent pulmonary hypertension of the newborn in twin–twin transfusion syndrome following fetoscopic laser surgery

Objective: We investigated persistent pulmonary hypertension of the newborn (PPHN) among monochorionic-diamniotic (MD) twins. Methods: A retrospective cohort study examined MD twins from 195 deliveries and 373 live-born neonates at our center. Results: PPHN occurred in three cases (3/373: 0.8%), all of which were recipients of twin–twin transfusion syndrome (TTTS), after fetoscopic laser surgery (FLS) (3/117: 2.6%). Although the clinical course of the three cases differed, all cardiothoracic area ratios exceeded 40%, and other cardiac parameters also worsened after FLS. Conclusions: The occurrence of PPHN in TTTS recipients should be noted, particularly when fetal cardiac function declines following FLS.

Extracorporeal membrane oxygenation for congenital diaphragmatic hernia in Japan

The aim of this study was to clarify how extracorporeal membrane oxygenation (ECMO) is used to treat congenital diaphragmatic hernia (CDH) in Japan.

The lung to thorax transverse area ratio has a linear correlation with the observed to expected lung area to head circumference ratio in fetuses with congenital diaphragmatic hernias

BACKGROUND/PURPOSE The purpose of this study was to clarify the relationship between the lung to thorax transverse area ratio (L/T ratio) and the observed to expected lung area to head circumference ratio (O/E LHR), based on the results of a nationwide Japanese survey conducted in 2011, and to evaluate the compatibility of these prognostic predictors of fetal CDH. METHODS Two hundred and forty-two prenatally diagnosed isolated CDH patients born between 2006 and 2010 were included in the present analysis. A regression analysis was conducted to investigate the relationship between the L/T ratio and the O/E LHR based on 191 simultaneous measurements of these parameters in 120 patients. RESULTS The linear regression equation between the L/T ratio and the O/E LHR was: L/T ratio=0.0233+(0.00222×O/E LHR), (R=0.847, p<0.0001). According to this equation, 25% of the O/E LHR, the cut-off value used in the fetal intervention for CDH, was equivalent to an L/T ratio of 0.08, a commonly accepted cut-off value for identifying the most severe cases of fetal CDH. CONCLUSIONS As there is a positive correlation between the L/T ratio and the O/E LHR, these two parameters proved to be used interchangeably according to the linear regression equation.

Reversible Atrioventricular Block and Junctional Ectopic Tachycardia in Coxsackievirus B3-Induced Fetal–Neonatal Myocarditis without Left Ventricular Dysfunction

We present a case of fetal–neonatal acute myocarditis caused by coxsackievirus B3 infection in a term neonate. The condition manifested as high-grade atrioventricular (A-V) block prenatally. After delivery, various arrhythmias such as high-grade A-V block, ventricular tachycardia, and junctional ectopic tachycardia appeared, and we had difficulty managing these arrhythmias. This is the first report describing a case of acute myocarditis due to coxsackievirus infection presenting with fetal A-V block. This case is also unique in that it is extremely rare that various arrhythmias occur serially in one patient without left ventricular dysfunction.

Prognostic factors of congenital diaphragmatic hernia accompanied by cardiovascular malformation

Congenital diaphragmatic hernia is associated with cardiovascular malformation. Many prognostic factors have been identified for isolated congenital diaphragmatic hernia; however, reports of concurrent congenital diaphragmatic hernia and cardiovascular malformation in infants are limited. This study evaluated congenital diaphragmatic hernia associated with cardiovascular malformation in infants. Factors associated with prognosis for patients were also identified.

Congenital mesoblastic nephroma: Its diverse clinical features – A literature review with a case report

To characterise congenital mesoblastic nephroma (CMN), with special emphasis on polyhydramnios and the neonatal prognosis, we summarise 31 CMN patients (30 reported patients and the present patient). CMN was detected at a median of 30 weeks’ gestation, and infants were delivered at a median of 34 weeks’ gestation. Of 27 patients with available data, 19 (70%) had polyhydramnios, of which 8 required amnio- drainage. Women with amnio-drainage gave birth significantly earlier (30.4 weeks’ gestation) than those without polyhydramnios (36.7 weeks’ gestation). Thus, CMN was frequently associated with polyhydramnios and this polyhydramnios was associated with a significant increase in the risk of preterm birth. Of 20 patients with available data, the affected-side kidney was ‘compressed’ in 16 and ‘replaced’ in 4: polyhydramnios was present in a half vs 100%, respectively, suggesting that a ‘replaced’ kidney may suggest a more aggressive tumour and may be associated with a poorer prognosis. Univariate analysis showed that early gestational week at diagnosis was the only feature significantly associated with poor prognosis. Thus, polyhydramnios, ‘replaced’ kidney and early gestational week at diagnosis, may indicate poor prognosis, to which obstetricians should pay attention.