Nobuyuki Morikawa

National Center for Child Health and Development, Edo, Tōkyō, Japan

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Publications (27)38.41 Total impact

  • Pediatrics International 04/2012; 54(2):285-7. DOI:10.1111/j.1442-200X.2011.03414.x · 0.73 Impact Factor
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    ABSTRACT: The aim of this study was to establish a prenatal prognostic classification system for risk-stratified management in fetuses with isolated congenital diaphragmatic hernia (CDH). A multi-institutional retrospective cohort study of isolated CDH, diagnosed prenatally in fetuses delivered during the 2002 to 2007 period at 5 participating institutions in Japan, was conducted. The risk stratification system was formulated based on the odds ratios of prenatal parameters for mortality at 90 days. The clinical severity in CDH infants were compared among the stratified risk groups. Patients were classified into the 3 risk groups: group A (n = 48) consisted of infants showing liver-down with contralateral lung-to-thorax transverse area ratio (L/T) ratio ≥0.08; group B of infants showing liver-down with L/T ratio <0.08 or liver-up with L/T ratio ≥0.08 (n = 35), and group C of infants showing liver-up with L/T ratio <0.08 (n = 20). The mortality at 90 days in groups A, B, and C were 0.0%, 20.0%, and 65.0%, respectively. The intact discharge rates were 95.8%, 60.0%, and 5.0%, respectively. This system also accurately reflected the clinical severity in CDH infants. Our prenatal risk stratification system, which demonstrated a significant difference in postnatal status and final outcome, would allow for accurate estimation of the severity of disease in fetuses with isolated CDH, although it needs prospective validation in a different population.
    Journal of Pediatric Surgery 10/2011; 46(10):1873-80. DOI:10.1016/j.jpedsurg.2011.06.007 · 1.39 Impact Factor
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    ABSTRACT: To review the recent Japanese experience with prenatally diagnosed congenital diaphragmatic hernia (CDH) based on a multi-institutional survey. A multicenter, retrospective cohort study was conducted on 117 patients born between 2002 and 2007 with isolated prenatally diagnosed CDH. All patients were managed by maternal transport, planned delivery, immediate resuscitation and gentle ventilation. The primary outcome measurements were the 90-day survival and intact discharge. The examined prenatal factors included gestational age (GA) at diagnosis, lung-to-head ratio (LHR), lung-to-thorax transverse area ratio (L/T) and liver position. Physical growth and motor/speech development were evaluated at 1.5 and 3 years of age. Data were expressed as the median (range). The mean GA at diagnosis was 29 (17-40) weeks. The LHR and L/T were 1.56 (0.37-4.23) and 0.11 (0.04-0.25), respectively. There were 48 patients with liver up. The mean GA at birth was 38 (28-42) weeks. The 90-day survival rate and intact discharge rate were 79 and 63%, respectively. Twelve patients had major morbidity at discharge, and 71% of these patients had physical growth or developmental retardation at 3 years of age. This multicenter study demonstrated that the 90-day survival rate of isolated prenatally diagnosed CDH was 79%, and that subsequent morbidity remained high. A new treatment strategy is needed to reduce the mortality and morbidity of severe CDH.
    Pediatric Surgery International 04/2011; 27(4):373-8. DOI:10.1007/s00383-010-2805-0 · 1.00 Impact Factor
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    ABSTRACT: To document outcome and to explore prognostic factors in fetal left congenital diaphragmatic hernia (CDH). This was a multicenter retrospective study of 109 patients with prenatally diagnosed isolated left CDH born between 2002 and 2007. The primary outcome was intact discharge, defined as discharge from hospital without major morbidities, such as a need for respiratory support including oxygen supplementation, tube feeding, parenteral nutrition or vasodilators. All patients were managed at perinatal centers with immediate resuscitation, gentle ventilation (mostly with high-frequency oscillatory ventilation) and surgery after stabilization. Prenatal data collected included liver and stomach position, lung-to-head ratio, gestational age at diagnosis and presence or absence of polyhydramnios. Stomach position was classified into four grades: Grade 0, abdominal; Grade 1, left thoracic; Grade 2, less than half of the stomach herniated into the right chest; and Grade 3, more than half of the stomach herniated into the right chest. Overall intact discharge and 90-day survival rates were 65.1% and 79.8%, respectively. Stomach herniation was classified as Grade 0 in 19.3% of cases, Grade 1 in 45.9%, Grade 2 in 13.8% and Grade 3 in 21.1%. Multivariate analysis revealed that liver position was the strongest prognostic variable for intact discharge, followed by stomach position. Based on our results, we divided patients into three groups according to liver (up vs. down) and stomach (Grade 0–2 vs. Grade 3) position. Intact discharge rates declined significantly from liver-down (Group I), to liver-up with stomach Grade 0–2 (Group II), to liver-up with stomach Grade 3 (Group III) (87.0%, 47.4% and 9.5% of cases, respectively). Current status and outcomes of prenatally diagnosed left CDH in Japan were surveyed. Stomach herniation into the right chest was not uncommon and its grade correlated with outcome. The combination of liver and stomach positions was useful to stratify patients into three groups (Group I–III) with different prognoses. Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd.
    Ultrasound in Obstetrics and Gynecology 03/2011; 37(3):277-82. DOI:10.1002/uog.8892 · 3.85 Impact Factor
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    ABSTRACT: An accurate prenatal assessment of the patients' severity is essential for the optimal treatment of individuals with congenital diaphragmatic hernia (CDH). The purpose of this study was to clarify the reliability of the lung to thorax transverse area ratio (L/T) as a prenatal predictive parameter. A multicenter retrospective cohort study was conducted on 114 isolated CDH fetuses with a prenatal diagnosis during the period between 2002 and 2007 at five participating centers in Japan. The relationship between the gestational age and the L/T was analyzed. The most powerful measurement point and accurate cutoff value of the L/T was determined by an analysis of a receiver operating characteristic curve, which was verified by comparing the patients' severity. There was a negative correlation between the gestational age and the L/T in the non-survivors, and no correlation in the survivors. There were significant differences in the parameters which represented the patients' severity including the respiratory and circulatory status, the surgical findings, and the final outcomes between the groups divided at 0.080 in the minimum value of the L/T during gestation. The L/T was not strongly influenced by the gestational age, and it was found to be a reliable prenatal predictive parameter in fetuses with isolated CDH.
    Pediatric Surgery International 01/2011; 27(1):39-45. DOI:10.1007/s00383-010-2725-z · 1.00 Impact Factor
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    ABSTRACT: This report describes a case of fetal mediastinal teratoma complicated by hydrops fetalis managed successfully by aspiration of the tumor cyst fluid. Fetal mediastinal teratomas are rare tumors that cause hydrops fetalis or fetal demise in the prenatal period and respiratory distress in the neonatal period. The patient presented with a large cystic mass in the thoracic cavity complicated by hydrops fetalis. The hydrops resolved after fetal aspiration of the tumor cyst fluid. The infant was born without respiratory distress, and tumor resection was performed at the age of 30 days. The postoperative course was uneventful, and the patient was in good health 6 months postoperatively.
    Journal of Pediatric Surgery 12/2010; 45(12):e21-4. DOI:10.1016/j.jpedsurg.2010.08.055 · 1.39 Impact Factor
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    ABSTRACT: We describe two female neonates who suffered from four gastrointestinal anomalies, including duodenal stenosis or atresia, malrotation, segmental dilatation of the colon, and anorectal malformation. Each patient was managed by two or three operations, resulting in good bowel movements. Since this is the first report of four gastrointestinal anomalies, these cases may provide clues to elucidate the etiology of gastrointestinal tract developmental abnormalities.
    Pediatric Surgery International 09/2009; 25(11):1003-5. DOI:10.1007/s00383-009-2459-y · 1.00 Impact Factor
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    ABSTRACT: The serial transverse enteroplasty (STEP) procedure is a safe and successful way to lengthen the small bowel in patients with short bowel syndrome. However, postoperative dilatation of the intestine may occur, which induces bacterial overgrowth and malabsorption leading to liver failure. We describe the case of an infant boy with short bowel syndrome caused by jejunal atresia requiring the STEP procedure twice. The first STEP improved the liver function, and the second STEP allowed 80% of the total calorie intake to be tolerated enterally. One should not hesitate to perform a second STEP if after the initial bowel lengthening procedure the patient develops small bowel dilatation that interferes with enteral nutrition.
    Pediatric Surgery International 09/2009; 25(11):1007-11. DOI:10.1007/s00383-009-2456-1 · 1.00 Impact Factor
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    ABSTRACT: The aim of this study was to study the prognostic significance of circulating tumor cells (CTC) and the appropriate indications for aggressive surgery in advanced neuroblastoma. Micrometastasis was sequentially explored using our reverse transcriptase-polymerase chain reaction method in 29 neuroblastoma patients (International Neuroblastoma Staging System stage 4, n = 24; stage 3, n = 5) who treated at our department with the united chemotherapeutic regimen since 1991. Their medical records and detection of CTC and/or the bone marrow micrometastasis were retrospectively reviewed then analyzed statistically. The overall survival rate was 58.6% (17/29). Circulating tumor cells were detected in 55.6% of the stage 4 patients, and all deaths were related to systemic metastases in the CTC-positive patients. The detection of CTC scarcely associated with MYCN amplification. In the patients showing MYCN amplification but no CTC, all deaths were related to local relapse or chemotherapy-associated complications. The survival rate was not significantly different between the patients with and without MYCN amplification (56.8% vs 52.7%). However, it was significantly lower in the patients with CTC and/or persistent bone marrow micrometastasis compared to those without detectable micrometastasis (33.8% vs 87.5%; P < .05). The presence of CTC and/or persistent micrometastasis may indicate a significantly high risk, regardless of MYCN amplification. Patients with MYCN amplification but no micrometastasis would be most benefited by highly intensive surgery.
    Journal of Pediatric Surgery 01/2009; 43(12):2182-5. DOI:10.1016/j.jpedsurg.2008.08.046 · 1.39 Impact Factor
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    ABSTRACT: We present a neonate with gastroschisis and evidence of bile aspiration in utero, who developed severe respiratory distress that did not respond to postnatal intensive respiratory care. Although rare, a newborn with gastroschisis may develop severe respiratory distress due to bile aspiration in utero. Given the poor outcome in this case, we suggest a possible role for prenatal diagnosis and therapy.
    Pediatric Surgery International 09/2008; 24(10):1157-9. DOI:10.1007/s00383-008-2223-8 · 1.00 Impact Factor
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    ABSTRACT: One of the most serious problems in patients with long-gap esophageal atresia or corrosive esophagitis is esophageal stricture, which may require esophageal resection and replacement. We describe two cases with persistent esophageal stricture successfully managed by high dose intravenous methylprednisolone following balloon dilatation. High-dose methylprednisolone with gradual tapering (daily 25, 15, 10, 5, 2 mg/kg for 4 days each) plus cimetidine and ampicillin for 1 week was intravenously administrated immediately after balloon dilatation of the esophageal stenosis. This was followed by oral prednisolone (daily 2, 1, 0.5 mg/kg for 1 week each) for persistent esophageal stricture. High dose intravenous methylprednisolone therapy was given to two patients. One patient was a 5-year-old boy with long-gap esophageal atresia who had undergone repair of the esophagus resulting in severe anastomotic stenosis of 3 cm in length. The other case was a 10-year-old boy with corrosive stenosis caused by alkali ingestion. Both patients had been requiring balloon dilatation of the esophagus with intralesional injection of dexamethasone every 3 weeks for more than 1 year to tolerate oral feeding. After the high-dose methylprednisolone protocol was initiated, the symptoms of dysphagia or choking dramatically improved in both patients, and they remained symptom-free for 8 and 7 months. There were complications of moon faces that resolved concomitantly with the withdrawal of oral prednisolone in both cases. High dose intravenous methylprednisolone in addition to intralesional injection of dexamethasone following balloon dilation is an effective therapeutic strategy for persistent esophageal strictures.
    Pediatric Surgery International 09/2008; 24(10):1161-4. DOI:10.1007/s00383-008-2224-7 · 1.00 Impact Factor
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    ABSTRACT: Although the prognosis of congenital diaphragmatic hernia (CDH) is determined by the degree of pulmonary hypoplasia, there may be an occult contribution of infection to outcomes. The purpose of this study is to evaluate the effects of our new supportive therapy to prevent infectious complications on mortality and morbidity of CDH. Among 57 cases with CDH treated between 2002 and 2007, 43 prenatally diagnosed isolated cases were enrolled in this study. All patients were managed by a lung-protective strategy and delayed surgery. Since January 2006, we have optimized our perioperative care to reduce infectious complications by using peripherally inserted central catheters and restriction of invasive procedures including extracorporeal membranous oxygenation (ECMO). The survival rate, intubation period, maximum serum C-reactive protein (CRP) level, and complications were compared before and after the introduction of refined supportive therapy. There were 25 cases (12 liver-up, 13 liver-down) treated before 2006 and 18 cases (8 liver-up, 10 liver-down) after 2006. ECMO was required for stabilization in five cases before 2006. The survival rates of total, liver-up, and liver-down cases improved from 60, 42, 77, to 83, 63, 100% after 2006, respectively. The intubation period was shortened from 37.8 +/- 24.3 to 22.2 +/- 10.8 days, and the maximum serum CRP level declined from 12.8 +/- 11.5 to 2.2 +/- 1.6 mg/dl after 2006. Nine cases developed sepsis before 2006 whereas no patients suffered from sepsis or pneumonia after 2006. The new supportive therapy with strict infection control improved survival rate of prenatally diagnosed CDH without using ECMO.
    Pediatric Surgery International 09/2008; 24(10):1105-9. DOI:10.1007/s00383-008-2226-5 · 1.00 Impact Factor
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    ABSTRACT: Fetal extralobar pulmonary sequestration (EPS) is sometimes complicated by a massive pleural effusion, leading to tension hydrothorax and fetal hydrops. The goal of this study was to examine sonographic signs of venous obstruction in fetal EPS with or without pleural effusion. Records of fetal ultrasound from 6 patients with EPS were reviewed with special attention to aberrant arterial and venous flow. The results were correlated with their clinical outcomes. Four of the 6 cases (cases 1-4) were complicated by massive pleural effusion and required fetal thoracentesis; thoracoamniotic shunt placement was required in 3 of these 4 patients (cases 1-3). The other 2 patients (cases 5 and 6) were not associated with pleural effusion despite the comparable size of the mass and did not require any treatment, either prenatally or postnatally. In cases 1-3, aberrant venous flow was difficult to detect and, even when detected, the arterial-to-venous flow velocity ratio was >6. This is in contrast to the uncomplicated cases 5 and 6 in whom aberrant venous flow was easily detected with an arterial-to-venous flow velocity ratio of 2-3. Arterial-to-venous flow velocity ratios of 3-6 were observed in case 4. This case was complicated by pleural effusion but not by fetal hydrops. These data support the hypothesis that venous obstruction is related to the production of pleural effusion in fetal EPS. Ample flow in the aberrant vein may indicate benign clinical behavior, while difficulty in detecting aberrant venous flow may be correlated with the development of massive pleural effusion.
    Fetal Diagnosis and Therapy 04/2008; 23(4):299-302. DOI:10.1159/000123617 · 2.94 Impact Factor
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    ABSTRACT: This report describes a case of Abernethy malformation associated with hepatopulmonary syndrome, which was resolved after shunt ligation. The clinical course indicated that hepatopulmonary syndrome can develop in Abernethy malformation in which liver function and portal pressure is normal, and liver transplantation is not the exclusive therapy for hepatopulmonary syndrome. The levels of endotoxin and endothelin 1 in the shunt blood were high, whereas those of tumor necrosis factor alpha and interleukin-1beta were within reference range. Although pathogenesis of hepatopulmonary syndrome remains unknown, the findings in this case suggest that bacterial translocation as well as elevated endothelin 1 may play a causal role in development of hepatopulmonary syndrome.
    Journal of Pediatric Surgery 03/2008; 43(2):e35-8. DOI:10.1016/j.jpedsurg.2007.11.001 · 1.39 Impact Factor
  • Tatsuo Kuroda · Morihiro Saeki · Nobuyuki Morikawa · Koji Watanabe ·
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    ABSTRACT: The purpose of the paper was to study the potential risk and management of adult biliary atresia (BA) patients. Clinical courses and biochemical data were retrospectively analyzed in 52 BA patients who were operated in our department and are now older than 15 years, with special emphasis on 47 patients who survived beyond the age of 20 years. Of these 47 patients, 45 (95.7%) are employed, receiving higher education, and/or married. Thirty-three patients showed favorable liver function at the age of 20 years with their native liver. Thereafter, 6 (18.2%) developed liver dysfunction after working hard or pregnancy. In these 6 patients, cholinesterase levels were significantly lower at the age of 20 years than those in the 27 stable patients (239.5 +/- 42.6 vs 285.8 +/- 47.5, P = .0216), whereas no significant difference was found in alanine aminotransferase, aspartate aminotransferase, or total protein levels at the age of 20 years or in any biochemical parameters at the age of 15 years. Of the 14 transplanted patients, 5 who underwent transplantation after the age of 20 years had relatively uneventful courses until the age of 20 years; however, their biochemical data already showed significant deterioration at puberty. Social activities and pregnancy should be managed individually according to the risk assessment for potential hepatic failure even in stable adult BA patients. A falling cholinesterase level after the age of 20 years may predict the risk for the late hepatic dysfunction.
    Journal of Pediatric Surgery 01/2008; 42(12):2106-9. DOI:10.1016/j.jpedsurg.2007.08.037 · 1.39 Impact Factor
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    ABSTRACT: To investigate the clinical features and pathologic diagnosis of prenatally diagnosed lung diseases. The medical records of 28 fetuses with prenatally diagnosed lung diseases were reviewed with regard to perinatal courses, ultrasonographic measurement of the lesion volume ratio, and the histopathology of lung tissue. Of the 23 fetuses with a prenatal diagnosis of congenital cystic adenomatoid malformation (CCAM), 4 required an emergency lobectomy (1 prenatally and 3 postnatally), whereas the other 14 showed uneventful courses after birth. The pathologic diagnosis of the resected lungs included CCAM (n = 4), lobar emphysema (n = 2), intralobar sequestration (n = 2), and bronchial atresia (n = 1). The peak value of the lesion volume ratio was significantly higher in the CCAM cases that required urgent surgery than in the non-CCAM cases (2.29 +/- 0.46 vs 1.20 +/- 0.21, P < .001). The ratio decreased after the 25th to the 30th week to less than 1.0 in the 5 non-CCAM cases, but remained higher than 2.0 in the 4 CCAM cases. Prenatally diagnosed extralobar pulmonary sequestration caused massive pleural effusion requiring a fetal intervention in 4 of the 5 fetuses. The prenatally diagnosed lung lesions include various kinds of pulmonary diseases. Regardless of the diseases, sequential ultrasonographic assessment may predict perinatal risks.
    Journal of Pediatric Surgery 01/2007; 41(12):2028-31. DOI:10.1016/j.jpedsurg.2006.08.001 · 1.39 Impact Factor
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    ABSTRACT: Fetal extralobar pulmonary sequestration (EPS) is sometimes complicated by massive pleural effusion (PE) leading to fetal hydrops. The underlying mechanisms as well as the origin of the fluid are not well understood. This study was performed to find a histological hallmark of an EPS with massive PE. We hypothesized that venous obstruction has a role to play in the mechanisms of fluid production by EPS. We recently experienced 3 cases of fetal EPS complicated by fetal hydrothorax requiring thoracentesis and eventually thoracoamniotic shunt placement. Total protein content and cell count were measured in the aspirates, which were compared with chylothorax cases (n = 5) requiring fetal shunt placement. After birth, all 3 infants required mass resection for the control of PE. The venous wall thickness was measured on pathology slides stained with Elastica van Gieson staining. Thickness of the media and adventitia was measured in approximately 40 veins per case. They were corrected by external diameter and expressed as percentage of medial thickness and percentage of adventitial thickness. An EPS not associated with PE but with congenital diaphragmatic hernia served as a control. Total protein and the cell count of the EPS related PE were 0.6 +/- 0.3 mg/dL and 28 +/- 14/microL (mean +/- SD), which were significantly lower than those of PE in chylothorax (2.2 +/- 0.2 mg/dL and 1900 +/- 1100/microL). Percentages of adventitial thickness of EPS with PE were 9.8% +/- 3.8%, 10.4% +/- 3.6%, and 8.3% +/- 3.7%, which were significantly increased compared with the control of 3.1% +/- 1.3% (P < .01). Percentages of medial thickness of EPS with PE were 7.0% +/- 1.9%, 7.3% +/- 1.4%, and 6.6% +/- 2.3%, which were significantly increased compared with the control of 2.3% +/- 0.7% (P < .01). We conclude that PE associated with EPS is the transudate rather than the lymph. The thickened media and the adventitia found in EPS with PE support the hypothesis that partial obstruction of the venous system leads to an increased transudate production, which ultimately leads to fetal hydrops.
    Journal of Pediatric Surgery 03/2006; 41(3):490-4. DOI:10.1016/j.jpedsurg.2005.10.080 · 1.39 Impact Factor
  • Tatsuo Kuroda · Morihiro Saeki · Nobuyuki Morikawa · Yasushi Fuchimoto ·
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    ABSTRACT: The aim of this study was to study the late clinical features of biliary atresia in relation to pregnancy. Of the 175 patients with biliary atresia operated on at our department, the data for 49 patients (18 males, 31 females) older than 15 years were reviewed and analyzed. Six patients delivered 7 newborns (5 with their native liver and 1 with a transplanted liver). Although one of the patients delivered twice after transplantation, another underwent transplantation after delivery. None of the 7 newborns showed any congenital anomalies; however, 6 were small-for-date newborns. Their birth weight tended to be inversely related to the mothers' age at delivery. An additional 13 patients required liver transplantation, of whom 1 died after transplantation and 1 died before transplantation. The remaining 30 patients showed uneventful courses without transplantation. The age at Kasai operation was significantly higher in the 5 patients who delivered babies with their native liver compared with that in these 30 patients (88.0 vs 68.6 days). A significant difference between these 5 patients and those who died or required transplantation before pregnancy was recognized even at the age of puberty in the alanine aminotransferase (36.5 +/- 26.5 vs 126.4 +/- 56.1 IU/L), aspartate aminotransferase (36.2 +/- 26.5 vs 114.2 +/- 48.9 IU/L), and choline esterase (249.7 +/- 64.8 vs 163.5 +/- 40.2 IU/L). The age at Kasai operation may not necessarily impact the late clinical course; however, liver function at puberty may predict the subsequent clinical course, including the safety of pregnancy. The management strategies should be revised after puberty.
    Journal of Pediatric Surgery 01/2006; 40(12):1852-5. DOI:10.1016/j.jpedsurg.2005.08.026 · 1.39 Impact Factor
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    ABSTRACT: The aims of this study were to analyze the outcomes of fetuses with congenital diaphragmatic hernia (CDH) treated by a lung-protective strategy using high-frequency oscillatory ventilation (HFOV) in a single center with a perinatology service and extracorporeal membrane oxygenation (ECMO) capability and to define the natural history of CDH in the era of lung-protective ventilation. A retrospective chart review of 30 neonates with CDH seen between April 2002 and October 2004 was conducted. All fetuses with a prenatal diagnosis were evaluated by fetal magnetic resonance imaging to define the liver position, and those with a significant volume of the liver in the chest were regarded as liver-up. Patients were managed by a lung-protective strategy using pressure-limited (maximum mean airway pressure [MAP], 18 cm H(2)O) HFOV. The patients were initially placed on HFOV with a fraction of inspired oxygen (Fio(2)) of 1.0 and a MAP of 12 cm H(2)O. Hypercapnea and preductal saturation as low as 85% were accepted. Inhaled nitric oxide and ECMO were introduced when the baby could not be oxygenated with a MAP of 18 cm H(2)O. Twenty-six neonates (22 inborns with prenatal diagnosis and 4 outborns) were treated with this protocol. Four cases were not treated or died in utero because of severe associated anomalies. Thirteen of the 14 liver-down cases survived without ECMO and were discharged home (93% survival). On the contrary, 4 of 12 liver-up cases survived (33% survival). ECMO was required for initial stabilization in 5 cases with 1 survivor. Liver-down CDH babies have a good chance for survival without ECMO by a planned delivery and the lung-protective strategy using HFOV. Liver herniation demonstrated by prenatal magnetic resonance imaging retains a poor prognostic value even with this approach.
    Journal of Pediatric Surgery 01/2006; 40(12):1827-32. DOI:10.1016/j.jpedsurg.2005.08.020 · 1.39 Impact Factor
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    ABSTRACT: Congenital bronchial atresia (CBA) usually presents incidentally in asymptomatic young male adults but is rarely diagnosed in children. The aim of this study was to clarify the clinical characteristics of CBA in childhood and to describe the spectrum of this condition. The clinical features in 29 patients with CBA, aged from 1 day to 13 years (median, 4 years), were reviewed retrospectively. Diagnosis was confirmed by pathological findings of a blind-ending bronchus associated with distal mucous-filled bronchocele surrounded by hyperinflated lung parenchyma. All but 1 patient were symptomatic. The most frequent symptom was productive cough and fever owing to recurrent pneumonia found in 26 children. Two infants suffered from respiratory distress. Chest x-ray showed various findings of infiltrative pneumonia, emphysema, and a large cyst. Computed tomography, bronchography, and bronchoscopy were useful modalities for demonstrating bronchocele associated with hyperinflated lung or proximal blind-ending bronchus even in infected cases. The right lower lobe was predominantly affected in 12 cases, followed by left or right upper lobe in 7 cases. Lobectomy or segmentectomy resulted in remarkable clinical improvement. Congenital bronchial atresia presents differently in children than in young adults. Modern imaging techniques and careful pathological analyses lead to an accurate diagnosis of bronchial atresia, which may be misdiagnosed as intralobar sequestration or pulmonary bronchial cysts. Bronchial atresia is a distinct pathological entity that accounts for recurrent pneumonia or respiratory distress in childhood, requiring surgical treatment.
    Journal of Pediatric Surgery 01/2006; 40(12):1822-6. DOI:10.1016/j.jpedsurg.2005.08.021 · 1.39 Impact Factor