Publications by authors named "Akiko Yokoi"

71 Publications

Long-Term Outcomes of Congenital Diaphragmatic Hernia: Report of a Multicenter Study in Japan.

Children (Basel) 2022 Jun 8;9(6). Epub 2022 Jun 8.

Department of Pediatric Surgery, Osaka Women's and Children's Hospital, Izumi 594-1101, Japan.

Background: Treatment modalities for neonates with congenital diaphragmatic hernia (CDH) have greatly improved in recent years, with a concomitant increase in survival. However, long-term outcomes restrict the identification of optimal care pathways for CDH survivors in adolescence and adulthood. Therefore, we evaluated the long-term outcomes within the Japanese CDH Study Group (JCDHSG).

Methods: Participants were born with CDH between 2006 and 2018 according to the JCDHSG. Participants were enrolled in the database at 1.5, 3, 6, and 12 years old. Follow-up items included long-term complications, operations for long-term complication, and home medical care.

Results: A total of 747 patients were included in this study, with 626 survivors (83.8%) and 121 non-survivors (16.2%). At 1.5, 3, 6, and 12 years old, 45.4%, 36.5%, 34.8%, and 43.6% developed complications, and 20.1%, 14.7%, 11.5%, and 5.1% of participants required home care, respectively. Recurrence, pneumonia, pneumothorax, gastroesophageal reflux disease, and intestinal obstruction decreased with age, and thoracic deformity increased with age.

Conclusions: As CDH survival rates improve, there is a need for continued research and fine-tuning of long-term care to optimize appropriate surveillance and long-term follow-up.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/children9060856DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9222080PMC
June 2022

Feasibility of Real-Time Central Surgical Review for Patients with Advanced-Stage Hepatoblastoma in the JPLT3 Trial.

Children (Basel) 2022 Feb 10;9(2). Epub 2022 Feb 10.

Department of Pediatric Surgery, Hiroshima University Hospital, Hiroshima 734-8551, Hiroshima, Japan.

In the JPLT3 study, a real-time central surgical reviewing (CSR) system was employed aimed at facilitating early referral of candidates for liver transplantation (LTx) to centers with pediatric LTx services. The expected consequence was surgery, including LTx, conducted at the appropriate time in all cases. This study aimed to review the effect of CSR on institutional surgical decisions in cases enrolled in the JPLT3 study. Real-time CSR was performed in cases in which complex surgeries were expected, using images obtained after two courses of preoperative chemotherapy. Using the cloud-based remote image viewing system, an expert panel consisting of pediatric and transplant surgeons reviewed the images and commented on the expected surgical strategy or the necessity of transferring the patient to a transplant unit. The results were summarized and reported to the treating institutions. A total of 41 reviews were conducted for 35 patients, and 16 cases were evaluated as possible candidates for LTx, with the treating institutions being advised to consult a transplant center. Most of the reviewed cases promptly underwent definitive liver surgeries, including LTx per protocol.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/children9020234DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8870682PMC
February 2022

Conservative treatment for an anterior cutaneous nerve entrapment syndrome case.

Pediatr Int 2022 Jan 6;64(1):e14715. Epub 2021 Oct 6.

Department of General Pediatrics, Hyogo Prefectural Kobe Children's Hospital, Kobe, Japan.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/ped.14715DOI Listing
January 2022

Feasibility of dose-dense cisplatin-based chemotherapy in Japanese children with high-risk hepatoblastoma: Analysis of the JPLT3-H pilot study.

Pediatr Blood Cancer 2022 02 4;69(2):e29389. Epub 2021 Oct 4.

Natural Science Center for Basic Research and Development, Hiroshima University, Hiroshima, Japan.

Background: The SIOPEL-4 study has demonstrated that dose-dense cisplatin-based chemotherapy dramatically improves outcome in children with high-risk hepatoblastoma in western countries. However, the feasibility and safety of this regimen have not been clarified in Japanese patients.

Methods: A pilot study, JPLT3-H, was designed to evaluate the safety profile of the SIOPEL-4 regimen in Japanese children with newly diagnosed hepatoblastoma with either metastatic disease or low alpha-fetoprotein.

Results: A total of 15 patients (three female) were enrolled. Median age was 2 years (range, 0-14). Three patients were PRETEXT II (where PRETEXT is PRETreatment EXTent of disease), six PRETEXT III, and six PRETEXT IV. All patients had lung metastasis, none had low alpha-fetoprotein. Eight patients completed the prescribed treatment, and seven patients discontinued therapy prematurely, four due to progressive disease and three due to causes other than severe toxicity. Grade 4 neutropenia was documented in most patients in preoperative cycles A1-3 (11/15 in A1, 9/11 in A2, and 7/11 in A3) and in all considering all cycles. Grade 3-4 thrombocytopenia and grade 3 anemia were also frequently observed. Patients experienced several episodes of grade 3 febrile neutropenia, but none had grade 4 febrile neutropenia or severe infections. One patient had grade 3 heart failure only in the first cycle. Other grade 3 or 4 toxicities were hypomagnesemia, anorexia, nausea, mucositis, liver enzyme elevation, fever, infection, and fatigue. There were no unexpected severe toxicities.

Conclusion: The toxicity profile of JPLT3-H was comparable to that of SIOPEL-4. Dose-dense cisplatin-based chemotherapy may be feasible among Japanese patients with high-risk hepatoblastoma.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/pbc.29389DOI Listing
February 2022

Best pre-ductal PaO prior to extracorporeal membrane oxygenation as predictor of mortality in patients with congenital diaphragmatic hernia: a retrospective analysis of a Japanese database.

Pediatr Surg Int 2021 Dec 6;37(12):1667-1673. Epub 2021 Sep 6.

Department of Pediatric Surgery, Osaka Women's and Children's Hospital, Osaka, Japan.

Purpose: Predicting lethal pulmonary hypoplasia in infants with congenital diaphragmatic hernia (CDH) before extracorporeal membrane oxygenation (ECMO) initiation is difficult. This study aimed to predict lethal pulmonary hypoplasia in patients with CDH prior to ECMO.

Methods: This was a multicenter cohort study involving neonates prenatally diagnosed with isolated unilateral CDH (born 2006-2020). Patients who required ECMO due to respiratory insufficiency were included in this study. Patients who underwent ECMO due to transient disorders were excluded from analysis. Blood gas analysis data within 24 h of birth were compared between survivors and non-survivors. Predictive abilities were assessed for factors with significant differences.

Results: Overall, 34 patients were included (18 survivors and 16 non-survivors). The best pre-ductal PaO was significantly lower in non-survivors than in survivors (50.4 [IQR 30.3-64.5] vs. 67.5 [IQR 52.4-103.2] mmHg, respectively; p = 0.047). A cutoff PaO of 42.9 mmHg had a sensitivity, specificity, and positive predictive value of 50.0%, 94.4%, and 88.9%, respectively, to predict mortality.

Conclusion: The best PaO within 24 h after birth predicted mortality following ECMO initiation. This should be shared to families and caregivers to optimize the best interests of the infants with CDH.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00383-021-04995-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8419806PMC
December 2021

Optimal timing of delivery for pregnancies with prenatally diagnosed congenital diaphragmatic hernia: a propensity-score analysis using the inverse probability of treatment weighting.

J Perinatol 2021 08 14;41(8):1893-1900. Epub 2021 Jun 14.

Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan.

Objective: To evaluate the optimal timing of neonates with prenatally diagnosed congenital diaphragmatic hernia (CDH).

Methods: Data from a retrospective cohort study conducted by the Japanese CDH Study Group between 2011 and 2018 were divided into two groups according to delivery timing: 36-37 and 38-41 weeks of gestation (wg). Death before 90 days as the primary outcome and the duration of hospitalization, oxygen therapy and tube feeding at discharge as the secondary outcomes were analyzed with generalized linear model applying inverse probability of treatment weighting method. We also performed layered analysis according to stomach position.

Result: Among 493 neonates with prenatally diagnosed, isolated and left CDH, 237 were born at 38-41wg. The duration of hospitalization was significantly shorter in those born at 38-41wg, especially among those with stomach malposition, and the other outcomes showed no difference.

Conclusions: Delivery at 38-41wg could be beneficial for those with high grade stomach position.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1038/s41372-021-01118-2DOI Listing
August 2021

Thoracoscopic repair of congenital diaphragmatic hernia in neonates: findings of a multicenter study in Japan.

Surg Today 2021 Oct 20;51(10):1694-1702. Epub 2021 Apr 20.

Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan.

Purpose: We compared the efficacy of thoracoscopic repair (TR) with that of open repair (OR) for neonatal congenital diaphragmatic hernia (CDH).

Methods: The subjects of this multicenter retrospective cohort study were 524 infants with left-sided isolated CDH, diagnosed prenatally, and treated at one of 15 participating hospitals in Japan between 2006 and 2018. The outcomes of infants who underwent TR and those who underwent OR were compared, applying propensity score matching.

Results: During the study period, 57 infants underwent TR and 467 underwent OR. Ten of the infants who underwent TR required conversion to OR for technical difficulties and these patients were excluded from the analysis. The survival rate at 180 days was similar in both groups (TR 98%; OR 93%). Recurrence developed after TR in 3 patients and after OR in 15 patients (TR 7%, OR 3%, p = 0.40). The propensity score was calculated using the following factors related to relevance of the surgical procedure: prematurity (p = 0.1), liver up (p < 0.01), stomach position (p < 0.01), and RL shunt (p = 0.045). After propensity score matching, the multivariate analysis adjusted for severity classification and age at surgical treatment revealed a significantly shorter hospital stay (odds ratio 0.50) and a lower incidence of chronic lung disease (odds ratio 0.39) in the TR group than in the OR group.

Conclusions: TR can be performed safely for selected CDH neonates with potentially better outcomes than OR.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00595-021-02278-6DOI Listing
October 2021

The usefulness of olive oil enema in children with severe chronic constipation.

J Pediatr Surg 2021 Jul 26;56(7):1141-1144. Epub 2021 Mar 26.

Department of Pediatric Surgery, Kobe Children's Hospital. 1-6-7, Minatojima Minamimach, Chuoku, Kobe, Japan 650-0047.

Purpose: Few reports have determined the efficacy of olive oil enemas for severe constipation. Here, we review our experience with olive oil enemas in children with severe chronic constipation.

Methods: In our outpatient pediatric surgery department, the charts of 118 patients prescribed with olive oil enemas between January 2010 and November 2019 were retrospectively reviewed. A 1-2 ml/kg olive oil enema was given either alone or followed several hours later by a glycerin enema. Ratings included "very effective (VE)," "effective (E)," "limited (L)," "ineffective (I)," and "unknown (U)."

Results: One hundred and fifteen (97.5%) patients were able to use olive oil enemas at home. Forty-nine had functional constipation; 43 had anorectal malformation; 40 had Hirschsprung disease; 12 had spina bifida; and 10 had other maladies. Used as an enema, olive oil was effective in treating fecal impaction in 77.6% of patients; as a lubricant, it was effective in treating 76.9% of patients. Efficacy for fecal disimpaction was similar among patients with different underlying disorders.

Conclusion: Olive oil enemas are useful for more than three-quarters of children with severe chronic constipation. Further study is warranted to add olive oil enemas as an adjunctive treatment in the management of severe constipation.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jpedsurg.2021.03.024DOI Listing
July 2021

Characteristics and treatment of congenital esophageal stenosis: A retrospective collaborative study from three Japanese children's hospitals.

J Pediatr Surg 2021 Oct 7;56(10):1771-1775. Epub 2021 Jan 7.

Department of Surgery, Kanagawa Children's Medical Center(,) Yokohama, Japan.

Background/purpose: There is no consensus on treatment strategy of congenital esophageal stenosis (CES). This study aimed to assess appropriateness of the treatment we have provided to patients with CES over the past four decades.

Methods: We carried out a retrospective chart review of 83 CES patients treated at three children's hospitals between 1973 and 2015. Each patient underwent an initial treatment with either surgery or a series of dilation that was followed by surgery if dilation failed to improve esophageal transit. Demographic data, course of treatment, outcomes, and complications were analyzed.

Results: During this initial treatment, 19 and 64 patients underwent surgery and dilation, respectively. Out of the 64 patients who underwent dilations as an initial treatment, 26 patients eventually required surgery. Out of all patients who required surgery (19 initial treatments + 26 failed dilations), 29 had tracheobronchial remnants and 16 had fibromuscular hypertrophy. Six patients experienced esophageal perforation during dilation and ten experienced anastomotic leakage after surgery. No patients had swallowing difficulties at the latest follow up, 141(9-324) months.

Conclusions: Dilation is recommended as an initial therapy, especially if histological diagnosis of CES is uncertain. Persistent swallowing difficulties after 2 series of dilation may be an indication for surgery.

Levels Of Evidence: level IV.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jpedsurg.2020.12.029DOI Listing
October 2021

Prognosis of conventional vs. high-frequency ventilation for congenital diaphragmatic hernia: a retrospective cohort study.

J Perinatol 2021 04 11;41(4):814-823. Epub 2020 Nov 11.

Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan.

Objective: To determine the appropriate initial ventilatory mode for neonatal congenital diaphragmatic hernia (CDH) by comparing patient prognosis following conventional mechanical ventilation (CMV) versus high-frequency oscillatory ventilation (HFO).

Study Design: This multicenter retrospective cohort study was performed at 15 participating hospitals in Japan between 2011 and 2016. The 328 eligible CDH infants were classified into CMV (n = 78) and HFO groups (n = 250) to compare mortality and incidence of bronchopulmonary dysplasia (BPD). Propensity score matching was applied to reduce confounding by indication.

Result: While crude mortality was significantly higher in the HFO than the CMV group, adjusted odds ratio (OR) did not show significant difference in mortality between groups (OR of HFO group: 0.98, 95% confidence interval (CI): 0.57-1.67). Adjusted OR of BPD incidence showed no significant difference between groups (OR of HFO group: 1.66, 95%CI: 0.50-5.49).

Conclusion: Initial ventilatory mode in CDH patients, whether CMV or HFO, does not affect prognosis.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1038/s41372-020-00833-6DOI Listing
April 2021

Optimal timing of surgery in infants with prenatally diagnosed isolated left-sided congenital diaphragmatic hernia: a multicenter, cohort study in Japan.

Surg Today 2021 Jun 10;51(6):880-890. Epub 2020 Oct 10.

Department of Pediatric Surgery, Osaka University School of Medicine Graduate School of Medicine, Osaka, Japan.

Purpose: To date, there is no compelling evidence of the optimal timing of surgery for congenital diaphragmatic hernia (CDH). This study aimed to establish the optimal timing of surgery in neonates with isolated left-sided CDH.

Methods: This multicenter cohort study enrolled 276 patients with isolated left-sided CDH at 15 institutions. Based on the timing of surgery, the patients were classified into four groups [< 24 h (G1), 24-47 h (G2), 48-71 h (G3), and ≥ 72 h (G4)]. The clinical outcomes were compared among the groups using a logistic regression model, after adjusting for potential confounders, such as disease severity.

Results: Multivariate analyses showed that G2 also had a lower mortality rate than the other groups. In mild and severe cases, there were no significant differences in mortality across the four groups. In moderate cases, G2 resulted in significantly increased survival rates, compared with G1.

Conclusion: The study results suggest that surgery within 24 h of birth is not recommended for patients with moderate severity CDH, that there is no benefit in the delay of surgery for more than 72 h in mild severity CDH patients, and that there is no definite optimal time to perform surgery in severe cases of CDH.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00595-020-02156-7DOI Listing
June 2021

Outcome and Late Complications of Hepatoblastomas Treated Using the Japanese Study Group for Pediatric Liver Tumor 2 Protocol.

J Clin Oncol 2020 08 18;38(22):2488-2498. Epub 2020 May 18.

Center for Integrated Medical Research, Hiroshima University Hospital, Hiroshima, Japan.

Purpose: We report here the outcomes and late effects of the Japanese Study Group for Pediatric Liver Tumors (JPLT)-2 protocol, on the basis of cisplatin-tetrahydropyranyl-adriamycin (CITA) with risk stratification according to the pretreatment extent of disease (PRETEXT) classification for hepatoblastoma (HB).

Patients And Methods: From 1999 to 2012, 361 patients with untreated HB were enrolled. PRETEXT I/II patients were treated with up-front resection, followed by low-dose CITA (stratum 1) or received low-dose CITA, followed by surgery and postoperative chemotherapy (stratum 2). In the remaining patients, after 2 cycles of CITA, responders received the CITA regimen before resection (stratum 3), and nonresponders were switched to ifosfamide, pirarubicin, etoposide, and carboplatin (ITEC; stratum 4). Intensified chemotherapeutic regimens with autologous hematopoietic stem-cell transplantation (SCT) after resection were an optional treatment for patients with refractory/metastatic disease.

Results: The 5-year event-free and overall survival rates of HB patients were 74.2% and 89.9%, respectively, for stratum 1, 84.8% and 90.8%%, respectively, for stratum 2, 71.6% and 85.9%%, respectively, for stratum 3, and 59.1% and 67.3%%, respectively, for stratum 4. The outcomes for CITA responders were significantly better than those for nonresponders, whose outcomes remained poor despite salvage therapy with a second-line ITEC regimen or SCT. The late effects, ototoxicity, cardiotoxicity, and delayed growth, occurred in 61, 18, and 47 patients, respectively. Thirteen secondary malignant neoplasms (SMNs), including 10 leukemia, occurred, correlating with higher exposure to pirarubicin and younger age at diagnosis.

Conclusion: The JPLT-2 protocol achieved up-front resectability in PRETEXT I/II patients with no annotation factors, and satisfactory survival in patients who were CITA responders in the remaining patients. However, outcomes for CITA nonresponders were unsatisfactory, despite therapy intensification with ITEC regimens and SCT. JPLT-2 had a relatively low incidence of cardiotoxicity but high rates of SMNs.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1200/JCO.19.01067DOI Listing
August 2020

Risk factors for pneumothorax associated with isolated congenital diaphragmatic hernia: results of a Japanese multicenter study.

Pediatr Surg Int 2020 Jun 28;36(6):669-677. Epub 2020 Apr 28.

Department of Pediatric Surgery, Kyushu University, Fukuoka, Japan.

Purpose: This study aimed to elucidate the clinical characteristics of neonates with congenital diaphragmatic hernia (CDH) associated with pneumothorax and evaluate the risk factors for the development of pneumothorax.

Methods: A retrospective cohort study was conducted in the 15 institutions participating in the Japanese CDH Study Group. A total of 495 neonates with isolated CDH who were born between 2011 and 2018 were analyzed in this study.

Results: Among the 495 neonates with isolated CDH, 52 (10.5%) developed pneumothorax. Eighteen (34.6%) patients developed pneumothorax before surgery, while 34 (65.4%) developed pneumothorax after surgery. The log-rank test showed that the cumulative survival rate was significantly lower in patients with pneumothorax than in those without pneumothorax. Univariate analysis revealed significant differences between patients with pneumothorax and those without pneumothorax with regard to the best oxygenation index within 24 h after birth, mean airway pressure (MAP) higher than 16 cmHO, diaphragmatic defect size, and need for patch closure. Multiple logistic regression analysis indicated that only the MAP was associated with an increased risk of pneumothorax.

Conclusions: The cumulative survival rate was significantly lower in isolated CDH patients with pneumothorax than in those without pneumothorax. A higher MAP was a risk factor for pneumothorax in CDH patients.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00383-020-04659-3DOI Listing
June 2020

An anterior cricoid split and long-term silastic T-tube stenting for children with severe subglottic stenosis.

Pediatr Surg Int 2020 Jun 22;36(6):697-700. Epub 2020 Apr 22.

Department of Pediatric Surgery, Kobe Children's Hospital, 1-6-7, Minatojima minamimachi, Chuoku, Kobe, 650-0047, Japan.

Purpose: The management of subglottic stenosis (SGS) remains challenging. Although laryngotracheal reconstruction with a costal cartilage graft (LTR) has been widely performed, restenosis with cicatricial tissue may require long-term stenting, especially in patients with severe SGS. An anterior cricoid split (ACS) with long-term stenting has been shown to be useful for patients with mild SGS. Thus, we evaluated the clinical outcomes of patients, including severe SGS, who underwent ACS compared to those with LTR.

Methods: A retrospective chart review was conducted in 25 patients with severe SGS (Grades III and IV) who underwent initial laryngoplasty (ACS or LTR) in our hospital from January 2009 to April 2018.

Results: 17 patients (8 with Grade III and 9 with Grade IV) underwent ACS, and 8 (6 with Grade III and 2 with Grade IV) underwent LTR. The median duration of stenting was 11 months (range: 0.8-50) in the ACS group and 12 months (range: 0.4-29) in the LTR group. Thirteen of 17 patients (76.5%) in the ACS group were decannulated, whereas 4 of 8 patients (50%) in the LTR group were decannulated (p = 0.2).

Conclusion: ACS might be useful even for children with severe SGS. The optimal duration of stenting should be investigated further.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00383-020-04657-5DOI Listing
June 2020

Switch to Extracorporeal Membrane Oxygenation During Cardiac and Tracheal Repair.

Ann Thorac Surg 2020 09 28;110(3):e181-e183. Epub 2020 Feb 28.

Department of Cardiovascular Surgery, Kobe Children's Hospital, Kobe, Japan.

Simultaneous repair of congenital tracheal and cardiovascular lesions remains challenging in small patients. We describe two infants weighing less than 3 kg who underwent successful tracheoplasty with concomitant correction of complex heart anomalies. In both operations, cardiopulmonary bypass was switched to extracorporeal membrane oxygenation after cardiac repair to optimize hemostatic function with transfusion and maintain activated clotting time at 200 to 240 seconds. Slide tracheoplasty was performed in a bloodless field, which prevented intraoperative hemorrhage from running down the divided lower trachea into the lung and causing airway obstruction. Both patients were weaned from extracorporeal support during surgery and extubated within 9 days.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.athoracsur.2020.01.037DOI Listing
September 2020

Novel Risk Score for Fetuses with Congenital Diaphragmatic Hernia Based on Ultrasound Findings.

Eur J Pediatr Surg 2020 Feb 10;30(1):51-58. Epub 2019 Oct 10.

Department of Pediatric Surgery, Osaka Women's and Children's Hospital, Izumi, Japan.

Introduction:  We aimed to establish and validate a risk score for fetuses with congenital diaphragmatic hernia (CDH) using only prenatal ultrasound findings.

Material And Methods:  Derivation (2011-2016,  = 350) and validation (2006-2010,  = 270) cohorts were obtained from a Japanese CDH study group database. Using a logistic regression analysis, we created a prediction model and weighted scoring system from the derivation dataset and calculated the odds ratio of an unsatisfactory prognosis (death within 90 days of life or hospitalization duration exceeding 180 days). Five adverse prognostic factors obtained using prenatal ultrasound, including an observed/expected lung area-to-head circumference ratio (o/eLHR) <25%, liver herniation occupying more than one-third of the thoracic space, thoracic stomach, right-side CDH, and severe malformations, were used as predictors. The obtained model was validated using the validation cohort.

Results:  The unsatisfactory prognosis prediction model was obtained based on the adjusted odds ratios. The C statistics of the model were 0.83 and 0.80 in the derivation and validation datasets, respectively. The five variables were weighted proportionally to their adjusted odds ratios for an unsatisfactory prognosis (o/eLHR <25%, 1 point; liver herniation occupying more than one-third of the thoracic space, 1 point; thoracic stomach, 1 point; right-side CDH, 2 points; and severe malformations, 3 points). Unsatisfactory prognosis rates for the low- (0-2 points), intermediate- (3-5 points), and high-risk (6-8 points) groups were 17, 46, and 100%, respectively ( < 0.001), in the validation cohort.

Conclusion:  Our simple risk score effectively predicted the prognosis of fetuses with CDH.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1055/s-0039-1698768DOI Listing
February 2020

Risk factors for the recurrence of perineal canal.

Pediatr Surg Int 2019 Oct 16;35(10):1137-1141. Epub 2019 Aug 16.

Department of Pediatric Surgery, Kobe Children's Hospital, 1-6-7 Minatojimaminamimachi, Chuo-ku, Kobe, 650-0047, Japan.

Purpose: The aim of this study was to investigate risk factors for recurrence in the perineal canal (PC).

Methods: Patients with PC who underwent operations were enrolled in this study and were divided into recurrence and non-recurrence groups. Preoperative infection, the age at the operation, the presence of colostomy and the treatment procedure for fistula were retrospectively investigated. Regarding the treatment procedure for fistula, either closure of the rectal wall with stitches or ligation of fistula in the rectum was performed. These factors were compared between the two groups.

Results: Six of 17 patients with PC who underwent surgical treatment had recurrence. There were no significant differences in the incidence of preoperative infection, age at operation or presence of colostomy (p = 0.60, 0.38, 1.00, respectively). In the recurrence group, all patients were treated by closure of the rectal wall. In the non-recurrence group, five were treated by the closure of the rectal wall with stitches and six by ligation of the fistula. There was a significant association between recurrence and the treatment procedure for fistula (p = 0.04).

Conclusion: Closure of the rectal wall with stitches is a risk factor for the recurrence of PC.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00383-019-04536-8DOI Listing
October 2019

An update in the management of pediatric sarcoma.

Curr Opin Pediatr 2019 06;31(3):368-377

Department of Surgery, Division of Pediatric Surgery, University of North Carolina Children's Hospital, Chapel Hill, North Carolina, USA.

Purpose Of Review: Nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) is a rare subgroup of malignancy in childhood that is composed of a variety of soft tissue and bony tumors. Prognosis for resectable localized disease is usually good and improved with systemic treatment. However, survival from locally advanced and metastatic disease remains poor. There have been numerous preclinical and clinical studies to define histopathology, biology, and genetic alteration of sarcomas. The purpose of this review is to clarify the progress in the management of NRSTS.

Recent Findings: Genomic analysis, including the use of next-generation sequencing, has revealed fusion transcripts or specific genetic alterations which provide diagnostic biomarkers and potential targets for novel therapies.

Summary: Most cases are sporadic, but some are associated with genetic predispositions. Most present as a painless mass and diagnosis is frequently delayed because of a low index of suspicion. There is a wide array of histopathological subtypes. Investigations usually involve core, incisional or excisional biopsy for tissue diagnosis, and cross-sectional and nuclear imaging for staging. Management of pediatric sarcoma is largely dependent on the patient's histopathological diagnosis, age, disease stage, and co-morbidities but usually involves a combination of systemic and local therapies. Preclinical studies and phase I/II trials of newer targeted therapies are ongoing.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1097/MOP.0000000000000767DOI Listing
June 2019

Triangular Cord Sign on Ultrasonography Does Not Indicate Disease Progression of Biliary Atresia.

Indian J Pediatr 2019 07 26;86(7):656-657. Epub 2019 Apr 26.

Department of Pediatric Surgery, Kobe Children's Hospital, 1-6-7, Minatojima-Minami-machi, Chuo-ku, Hyogo, 650-0047, Japan.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s12098-019-02967-8DOI Listing
July 2019

Repair of type IV laryngotracheoesophageal cleft (LTEC) on ECMO.

Pediatr Surg Int 2019 May 19;35(5):565-568. Epub 2019 Feb 19.

Department of Pediatric Surgery, Kobe Children's Hospital, 1-6-7 Minatojima Minami, Chuou-ku, Kobe, Hyogo, 650-0047, Japan.

Purpose: A type IV laryngotracheoesophageal cleft (LTEC) is a very rare congenital malformation. Type IV LTEC that extends to the carina have poor prognosis and are difficult to manage. We present our experience with surgical repair in such a case using extracorporeal membranous oxygenation (ECMO).

Methods: A male infant, who was diagnosed with Goldenhar syndrome, showed severe dyspnea and dysphagia. Laryngoscopy indicated the presence of LTEC. The patient was transferred to our institute for radical operation 26 days after birth. Prior to surgery, a balloon catheter was inserted in the cardiac region of stomach through the lower esophagus to block air leakage, to maintain positive pressure ventilation. We also performed observations with a rigid bronchoscope to assess extent of the cleft, and diagnosed the patient with type IV LTEC. After bronchoscopy, we could intubate the tracheal tube just above the carina. Under ECMO, repair of the cleft was performed by an anterior approach via median sternotomy.

Results: The patient was intubated via nasotracheal tube and paralysis was maintained for 2 weeks, using a muscle relaxant for the first 3 days. Two weeks after surgery, rigid bronchoscopy showed that the repair had been completed, and the tracheal tube was successfully extubated without tracheotomy.

Conclusions: Although insertion of a balloon catheter is a very simple method, it can separate the respiratory and digestive tracts. This method allowed for positive pressure ventilation and prevented displacement of the endotracheal tube until ECMO was established. As a result, we safely performed the operation and the post-operative course was excellent.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00383-019-04455-8DOI Listing
May 2019

A new approach to risk stratification using fetal MRI to predict outcomes in congenital diaphragmatic hernia: the preliminary retrospective single institutional study.

Transl Pediatr 2018 Oct;7(4):356-361

Department of Obstetrics, Perinatal Medical Center, Kobe Children's Hospital, Kobe, Japan.

Background: Congenital diaphragmatic hernia (CDH) is a condition with a wide range of severity. Prenatal diagnosis is essential to optimize postnatal management, especially for severe cases. The lung to head ratio (LHR) and liver herniation estimated by prenatal ultrasound has been used as prenatal predictors in CDH. However, reliability of these factors remains to be proven and prediction of outcome from prenatal imaging is still challenging. We propose our new stratification system using lung to liver signal intensity ratio (LLSIR) in fetal MRI, which has been shown to be related to pulmonary maturation.

Methods: Retrospective chart review was conducted on 25 infants with CDH treated from 2009 through 2016 in our hospital. We stratified patients according to fetal T2-weighted MRI as Grade I, detectable ipsilateral lung at the apex; Grade II, undetectable ipsilateral lung at the apex and contralateral LLSR ≥2.0; Grade III, undetectable ipsilateral lung at the apex and contralateral LLSR <2.0. To evaluate this stratification system, we analyzed survival, severity [inhaled nitric oxide (iNO) usage with or without extracorporeal membrane oxygenation (ECMO)], and requirement of patch closure.

Results: All 15 patients survived in Grade I, while 2 out of 6 died in Grade II, and 3 out of 4 died in Grade III (P=0.003). Four were severe in Grade I, and all in Grade II and III who survived (P=0.007). One needed patch in Grade I, and all in Grade II and III (OR: 414,238,332; 95% CI, 0-∞). Liver herniation was noted in five patients, and significantly associated with survival (P=0.04), however, neither with severity (P=1.00) nor with the requirement of patch closure (P=0.52).

Conclusions: The risk stratification algorithm using contralateral LLSIR in fetal MRI could be useful and more reliable than liver herniation to predict survival, severity, and need of patch closure. Further investigation is warranted.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.21037/tp.2018.09.01DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6212392PMC
October 2018

Congenital tracheal stenosis: what should we look at for successful tracheoplasty?

Authors:
Akiko Yokoi

Transl Pediatr 2018 Jul;7(3):229-232

Department of Pediatric Surgery, Kobe Children's Hospital, Kobe, Japan.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.21037/tp.2018.07.01DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6087833PMC
July 2018

Clinical features and risk factors of bile duct perforation associated with pediatric congenital biliary dilatation.

Pediatr Surg Int 2018 Oct 3;34(10):1079-1086. Epub 2018 Aug 3.

Division of Pediatric Surgery, Department of Surgery, Kobe University School of Medicine, Kobe, Japan.

Purpose: This study aimed to investigate the clinical features and risk factors of bile duct perforation in pediatric congenital biliary dilatation (CBD) patients.

Methods: CBD patients, whose initial symptom was abdominal pain, were enrolled in this study and were divided into perforated and non-perforated groups. The clinical features of the perforated group were investigated. Moreover, the age at operation, sex, and morphologic features of the extrahepatic bile duct were compared between the groups.

Results: Fifteen cases of bile duct perforation (10.4%) were identified among the 144 CBD patients who had abdominal pain. Majority of bile duct perforation occurred in patients aged < 4 years. The median duration from onset of abdominal pain to bile duct perforation was 6 (4-14) days. Age at onset [< 4 years old; P = 0.02, OR 13.9, (1.663, 115.3)], shape of extrahepatic bile duct [non-cystic type; P = 0.009, OR 8.36, (1.683, 41.5)], and dilatation of the common channel [P = 0.02, OR 13.6, (1.651, 111.5)] were risk factors of bile duct perforation.

Conclusions: Emergent bile duct drainage might be planned to prevent bile duct perforation if CBD patients have the abovementioned risk factors and experience persistent abdominal pain lasting for a few days from onset.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00383-018-4321-6DOI Listing
October 2018

Clinical outcomes after tracheoplasty in patients with congenital tracheal stenosis in 1997-2014.

J Pediatr Surg 2018 Nov 27;53(11):2140-2144. Epub 2017 Dec 27.

Department of Pediatric Surgery, Takatsuki General Hospital, Takatsuki, Japan.

Background: Mortality and morbidity of congenital tracheal stenosis (CTS) remain high. The aim of this study was to determine the factors predicting 12-month survival and 2-month successful extubation after tracheoplasty in patients with CTS.

Methods: Retrospective chart reviews were conducted in patients with CTS undergoing tracheoplasty at a single institution between 1997 and 2014. Patients' characteristics at disease onset and tracheoplasty were summarized. Twelve-month survival rate and 2-month extubation rate without tracheotomy after tracheoplasty were analyzed.

Results: We reviewed 81 patients' records. Multivariate analysis for 12-month survival revealed that older age (>2 months, hazard ratio [HR]: 0.08, 95% confidence interval [CI]: 0.02-0.36) or heavier body weight (>4.4 kg, HR: 0.13, 95% CI: 0.02-0.73) at tracheoplasty was a predictive factor for survival. Body weight at tracheoplasty (>8.2 kg, HR: 3.83, 95% CI: 1.88-7.79), preoperative balloon dilatation (HR: 0.30, 95% CI: 0.12-0.78), and carina involvement (HR: 0.36, 95% CI: 0.19-0.69) were predictive factors for successful extubation.

Conclusions: Although CTS management is individualized, age or body weight at tracheoplasty needs to be considered and assessed for survival, as well as preoperative balloon dilatation, and carina involvement for successful extubation.

Levels Of Evidence: Level III.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jpedsurg.2017.12.017DOI Listing
November 2018

Harlequin Syndrome.

Indian J Pediatr 2018 08 14;85(8):700-701. Epub 2017 Dec 14.

Department of Neurology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Japan.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s12098-017-2557-0DOI Listing
August 2018

The role of pulmonary metastasectomy for hepatoblastoma in children with metastasis at diagnosis: Results from the JPLT-2 study.

J Pediatr Surg 2017 Dec 4;52(12):2051-2055. Epub 2017 Sep 4.

Japan Children's Cancer Group (JCCG) liver tumor committee (JPLT).

Background/purpose: The purpose of this study was to clarify the role of pulmonary metastasectomy in hepatoblastomas with lung metastasis at diagnosis. We reviewed cases enrolled in the JPLT-2 study.

Methods: A total of 360 cases with hepatoblastoma were enrolled. The clinical courses and outcome of 60 cases with pulmonary metastasis at diagnosis were reviewed, focusing on metastasectomy.

Results: Induction chemotherapy resulted in eradication of nodules in 26, residual nodules in 33, and early treatment-related death in one. Of the 33 cases with residual nodules, 11 underwent complete resection of the lung lesions, and among these, progression was reported in five. Complete resection of the liver tumor was not achieved in two of these. Three underwent incomplete resection of lung nodules, eventually leading to progression. Twelve cases with incomplete or no liver tumor resection progressed regardless of the status of lung lesions. Contrarily, among patients who underwent complete resection of the liver tumor, half were cured without metastasectomy.

Conclusions: Metastasectomy for residual pulmonary nodules after induction chemotherapy is effective provided that the liver tumor could be completely resected.

Type Of Study: Prospective Cohort Study.

Level Of Evidence: Level II.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jpedsurg.2017.08.031DOI Listing
December 2017

A case of biliary atresia with pancreaticobiliary maljunction.

Surg Case Rep 2017 Sep 5;3(1):100. Epub 2017 Sep 5.

Department of Pediatric Surgery, Kobe Children's Hospital, 1-6-7 Minatojima Minamimachi, Chuo-ku, Kobe, Hyogo, 654-0081, Japan.

Background: The pathogenesis of biliary atresia (BA) is still unknown. There are several reports on the etiology of BA, including pancreaticobiliary maljunction (PBM). We experienced a case of Kasai type IIIa BA with PBM, in which we found elevation of pancreatic enzymes in the gallbladder. We evaluated whether PBM is related to the pathogenesis of BA based on our findings.

Case Presentation: The patient was born at 40 weeks of gestation. His body weight at birth was 2850 g. At the age of 4 days, he had an acholic stool and was referred to our hospital. Abdominal ultrasonography showed that triangular cord sign was negative. The gallbladder was isolated with a diameter of 19 mm, and it contracted in response to oral feeding. His ultrasonographic findings were atypical for BA, but his jaundice did not improve. Therefore, we performed an operation at the age of 56 days. Intraoperative cholangiography showed a common bile duct and pancreatic duct and a common channel patent, while the common hepatic duct or intrahepatic duct was not visualized. Bile in the gallbladder contained colorless fluid, which showed elevated lipase level (34,100 IU/L). We performed Kasai portoenterostomy under the diagnosis of Kasai type IIIa BA with PBM. The patient's postoperative course was uneventful, and he was discharged on day 30 after the operation. Histopathological evaluation showed that the lumens of the common bile duct and cystic duct were patent. However, the common hepatic duct was closed, and only bile ductules with diameters of less than 50 μm were isolated. Infiltration of lymphocytes was detected in the porta hepatis. No apparent inflammation was observed around the cystic duct, which was constantly exposed to pancreatic juice because of reflux through PBM.

Conclusions: Reflux of pancreatic juice through PBM might not be an etiological factor for BA, but might be associated with patency of the common and cystic bile ducts in Kasai type IIIa BA.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1186/s40792-017-0375-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5583132PMC
September 2017

Timing and outcome of stoma closure in very low birth weight infants with surgical intestinal disorders.

Surg Today 2017 Aug 28;47(8):1001-1006. Epub 2017 Feb 28.

Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Suita, Japan.

Purpose: Very low birth weight infants (VLBWIs) are at risk of surgical intestinal disorders including necrotizing enterocolitis (NEC), focal intestinal perforation (FIP), and meconium-related ileus (MRI). We conducted this study to verify whether the timing of stoma closure and that of enteral nutrition establishment after stoma closure in VLBWIs differ among the most common disorders.

Methods: A retrospective multicenter study was conducted at 11 institutes. We reviewed the timing of stoma closure and enteral nutrition establishment in VLBWIs who underwent stoma creation for intestinal disorders.

Results: We reviewed the medical records of 73 infants: 21 with NEC, 24 with FIP, and 25 with MRI. The postnatal age at stoma closure was 107 (28-359) days for NEC, 97 (25-302) days for FIP, and 101 (15-264) days for MRI (p = 0.793), and the postnatal age at establishment of enteral nutrition was 129 (42-381) days for NEC, 117 (41-325) days for FIP, and 128 (25-308) days for MRI (p = 0.855). The body weights at stoma closure were 1768 (620-3869) g for NEC, 1669 (1100-3040) g for FIP, and 1632 (940-3776) g (p = 0.614) for MRI. There were no significant differences among the three groups.

Conclusions: The present study revealed that the time and body weights at stoma closure and the postoperative restoration of bowel function in VLBWIs did not differ among the three diseases.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00595-017-1498-6DOI Listing
August 2017

The role of adjunctive procedures in reducing postoperative tracheobronchial obstruction in single lung patients with congenital tracheal stenosis undergoing slide tracheoplasty.

J Pediatr Surg 2017 May 27;52(5):677-679. Epub 2017 Jan 27.

Takatsuki General Hospital, Takatsuki, Japan.

Purpose: Congenital tracheal stenosis (CTS) and a single right lung treated with slide tracheoplasty (ST) has relatively high rates of mortality and morbidity. We report a single institution's experience with adjunctive procedures at the time of ST to reduce postoperative tracheal obstruction in patients with a single right lung and CTS.

Methods: With IRB approval, 8 patients with a single right lung and CTS who underwent ST in our institution between 2008 and 2016 were reviewed.

Results: Seven of 8 patients (88%) survived, with a median follow-up period of 35 (3-89) months. The only mortality was because of complications unrelated to the airway. Six of 8 patients underwent ST anterior to the aortic arch (tracheal translocation, TT). This was combined with tracheopexy concomitantly in 3 patients and subsequently in one patient. Two patients had insufficient tracheal length for TT. Both underwent aortopexy. Of three patients undergoing TT with tracheopexy concomitantly, two have been successfully extubated, and a third has required tracheostomy for subglottic stenosis. All patients undergoing TT without tracheopexy have experienced severe tracheobronchomalacia.

Conclusion: We conclude that adjunctive TT with tracheopexy might be associated with lower rates of tracheobronchial obstruction in patients with a single right lung undergoing ST for CTS.

Level Of Evidence: Level IV.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jpedsurg.2017.01.014DOI Listing
May 2017

Predictive factor for intraoperative tumor rupture of Wilms tumor.

Pediatr Surg Int 2017 Jan 1;33(1):91-95. Epub 2016 Nov 1.

Department of Hematology and Oncology, Kobe Children's Hospital, Kobe, Japan.

Purpose: For Wilms tumor, intraoperative tumor rupture with wide tumor spillage during surgical manipulation raises the classification to stage 3. Then, postoperative chemotherapy must be more intensive, and abdominal radiotherapy is added. Therefore, intraoperative tumor rupture should be avoided if possible. However, predictive factors for intraoperative tumor rupture have not been sufficiently described. Here we examined the risk factors for intraoperative tumor rupture.

Methods: Patients with Wilms tumor who underwent treatment according to the National Wilms Tumor Study or the Japanese Wilms Tumor Study protocol at our institution were reviewed retrospectively. Collected cases were categorized into two groups: the ruptured group and the non-ruptured group. Risk factors for intraoperative tumor rupture, including the ratio of the tumor area to the abdominal area in a preoperative single horizontal computed tomography slice (T/A ratio), were investigated in both groups.

Results: The two groups were not different in age, body weight, tumor laterality, sex, or histological distribution. The T/A ratio in the ruptured group was significantly higher than that in the non-ruptured group. Receiver operating characteristic curve analysis identified a discriminative value for a T/A ratio >0.5.

Conclusion: The T/A ratio can be a predictive factor for intraoperative tumor rupture of Wilms tumor.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00383-016-4000-4DOI Listing
January 2017
-->