照井 慶太

OBJECTIVES: Respiratory management is crucial for patients with congenital diaphragmatic hernia (CDH). This prospective study aimed to assess the optimal initial ventilatory mode for CDH by comparing conventional mechanical ventilation (CMV) and high-frequency oscillatory ventilation (HFO). METHODS: Fifteen participating institutes used CMV or HFO as the initial ventilation mode for CDH, we thus divided them into two groups (CMV and HFO). Clinical data were prospectively collected from patients with isolated left CDH born at a gestational age ≥ 34 weeks between 2017 and 2021. The primary outcome was mortality to discharge, with non-inferiority analysis. We also performed analyses stratified by disease severity. RESULTS: Of the 247 patients, 124 received CMV, and 123 received HFO. The mortality rates for CMV and HFO were 11.3% and 15.4%, respectively, which met the definition of non-inferiority. We observed no significant differences in BPD (CMV, 41% vs. HFO, 42%) or the combined outcome of mortality or BPD (48% vs. 51%). Among patients with mild and moderate disease, CMV was associated with less frequent switching of the ventilation mode, shorter mechanical ventilation time, lower use of inhaled nitric oxide (iNO) therapy, and a shorter duration of iNO. In severe cases, HFO was associated with less frequent switching of the ventilation mode and a reduced need for ECMO. CONCLUSIONS: The initial ventilation mode in CDH patients does not influence mortality or BPD outcomes. However, this study suggests that CMV may be more beneficial for mild-to-moderate cases, while HFO may be more advantageous for severe cases.

BACKGROUND: To retrospectively investigate the developmental outcomes at 3 years of age in patients with congenital diaphragmatic hernia (CDH) using a multicenter collaborative research approach. METHODS: We evaluated patients with CDH and no other malformations born between 2010 and 2016 in seven facilities in the Japanese CDH Research Group. The developmental quotient (DQ) at 3 years of age was evaluated using the Kyoto Scale of Psychological Development 2001, the most standardized scale in Japan. Factors associated with a DQ score < 85 were also analyzed. RESULTS: Of 196 patients, developmental assessments at 3 years of age were performed in 132 patients (67%). Among these, 99 patients (75%) had a DQ score ≥ 85, 25 (19%) had a DQ score between 70 and 84, and 8 (6%) had a DQ score < 70. Multivariate analysis showed that the observed/expected lung area-to-head circumference ratio was an independent predictor of a DQ score < 85, with an adjusted odds ratio of 0.62 (95% confidence interval: 0.40-0.96; p = 0.03). CONCLUSION: Generally, isolated CDH is associated with good developmental outcomes for survivors, even after intensive care. However, there is a risk of neurodevelopmental impairment if pulmonary hypoplasia is present. IMPACT: This research highlights the observed/expected lung area-to-head circumference ratio (o/e LHR) as a crucial indicator to predict neurodevelopmental outcomes in 3-year-old children diagnosed with isolated congenital diaphragmatic hernia (CDH). Our results provide robust evidence from a large multicenter cohort, emphasizing the importance of o/e LHR in early risk stratification and prolonged neurodevelopmental follow-up. These findings highlight the need for comprehensive management and tailored follow-up care in CDH patients, potentially improving clinical protocols and enhancing the quality of life and outcomes for affected children.

BACKGROUND: This study aimed to examine the incidence of undescended testis (UDT) in congenital diaphragmatic hernia (CDH) and clinical risk factors for developing UDT. METHODS: Male patients with CDH born between 2006 and 2021 were retrospectively assessed for UDT using the database of the Japanese CDH study group. The UDT risk factors were examined by comparing patients with and without UDT. RESULTS: Of the 605 boys with CDH, 408 were followed for at least one year and were included in this study. A total of 50 (12.3 %) of the 408 patients experienced UDT and orchidopexy was performed in 41 (10.2 %) patients. Twenty-four cases developed UDT on the same side of CDH, whereas only 6 cases developed UDT on the contralateral side of CDH; the remaining 20 cases were bilateral UDT. Comparison between the patients with and without UDT revealed significantly lower birth weight and lower Apgar Score in the patients with UDT. Furthermore, patients with UDT demonstrated a significantly higher rate of non-isolated, "liver-up", defect with C/D type, and patch closure. Multivariate analysis showed that the C/D type defect was deemed an independent risk factor for developing UDT. CONCLUSIONS: The incidence of UDT and the need for orchidopexy are elevated in boys with CDH. In patients with CDH, UDT may tend to occur on the same side as that affected by CDH. The diaphragmatic large defect was considered to be an independent risk factor for UDT in CDH although different factors are thought to be involved in testicular descent.

INTRODUCTION: Ewing sarcoma is a rare malignant tumor that primarily affects children and adolescents, and approximately 20% occur in the chest wall, where achieving local control is challenging due to the need for extensive resection and the proximity to vital organs. Adjuvant radiotherapy improves outcomes but carries a risk of severe complications such as radiation pneumonitis. Radioprotective spacers have been used for abdominal and pelvic tumors, but their use in chest wall tumors is rarely reported. We report a chest wall Ewing sarcoma case in which a radioprotective spacer using expanded polytetrafluoroethylene and a water-inflatable expander was placed to reduce radiation-related organ damage. CASE PRESENTATION: A 15-year-old female presented with a large Ewing sarcoma arising from the left chest wall, measuring 121 × 166 × 93 mm. After diagnosis by wedge biopsy and confirmation of EWSR1::FLI1 fusion, she received neoadjuvant chemotherapy based on the JESS04 protocol. Following significant tumor shrinkage, surgical resection was performed via a 20‑cm thoracotomy. En bloc removal included the tumor, the 5th and 6th ribs, and a portion of the adjacent lung. To reconstruct the chest wall and protect adjacent organs from high‑dose postoperative radiotherapy, a novel radioprotective spacer was inserted. The spacer was constructed by sandwiching a water‑inflatable tissue expander between two expanded polytetrafluoroethylene sheets. This design provided dual functionality: it reinforced the large chest wall defect and physically distanced the left lung from the radiation field. The expander was filled with 70 mL of water to create an adjustable spacer volume and could be deflated if postoperative symptoms occurred. The patient underwent intensity‑modulated radiation therapy with a total dose of 50.4 Gy without respiratory complications. After radiotherapy, the expander was removed, while the expanded polytetrafluoroethylene sheets remained in place to maintain chest wall integrity. At 4 years postoperatively, the patient remains disease‑free and symptom‑free. CONCLUSIONS: This case illustrates the feasibility and safety of combining a radioprotective spacer with structural chest wall reconstruction in pediatric sarcoma. The dual‑purpose design may offer an effective strategy for minimizing radiation‑related toxicity in thoracic tumors requiring multimodal treatment.

33歳男性。意識障害と呂律障害を主訴とした。臨床経過および精査により、短腸症候群に伴うD-乳酸アシドーシスと診断された。整腸剤の投与や抗菌薬による治療が行われたが、症状は繰り返され、頻回の入院を要したことから、母親をドナーとした糞便移植を行うこととなった。前処置の段階で症状は改善がみられたが、移植5日後にはD-乳酸アシドーシスの再燃が認められた。そこで、前処置による腸管洗浄が症状の緩和に有効と考え、数日おきにポリエチレングリコール製剤の投与を行った結果、症状は緩和した。その後、中心静脈カテーテルを再挿入後、中心静脈栄養を再開したところ、再開後はD-乳酸アシドーシスの再燃なく、4年が経過している。

PURPOSE: This study aimed to clarify surgical complications associated with open surgery for congenital diaphragmatic hernia (CDH). METHODS: We performed an exploratory data analysis of the clinical characteristics of surgical complications of neonates with CDH who underwent laparotomy or thoracotomy between 2006 and 2021. Data of these patients were obtained from the database of the Japanese CDH Study Group. RESULTS: Among 1,111 neonates with left or right CDH, 852 underwent open surgery (laparotomy or thoracotomy). Of these 852 neonates, 51 had the following surgical complications: organ injury (n = 48; 6% of open surgeries); circulatory failure caused by changes in the organ location (n = 2); and skin burns (n = 1). Injured organs included the spleen (n = 30; 62% of organ injuries), liver (n = 7), lungs (n = 4), intestine (n = 4), adrenal gland (n = 2), and thoracic wall (n = 2). Fourteen of the patients who experienced organ injury required a blood transfusion (2% of open surgeries). The adjusted odds ratio of splenic injury for patients with non-direct closure of the diaphragm was 2.2 (95% confidence interval, 1.1-4.9). CONCLUSION: Of the patients who underwent open surgery for CDH, 2% experienced organ injury that required a blood transfusion. Non-direct closure of the diaphragmatic defect was a risk factor for splenic injury.

PURPOSE: To elucidate the clinical significance of sarcopenia in children with neuroblastic tumors (NTs). METHODS: We conducted a retrospective observational study and analyzed the z-scores for height, body weight, body mass index, and skeletal muscle index (HT-z, BW-z, BMI-z, and SMI-z) along with the clinical characteristics of 36 children with NTs. SMI-z was calculated from 138 computed tomography scans at diagnosis, during treatment, and at follow-up. The International Neuroblastoma Risk Group classification was used to identify high-risk groups. We analyzed the data at diagnosis for prognostic analysis and changes over time after diagnosis in the HT-z, BW-z, BMI-z, and SMI-z groups. RESULTS: Among the four parameters at diagnosis, only SMI-z predicted overall survival (hazard ratio, 0.58; 95% confidence interval, 0.34-0.99). SMI-z, HT-z, and BW-z significantly decreased over time after diagnosis (P < 0.05), while BMI-z did not (P = 0.11). In surviving high-risk NT cases without disease, SMI-z, HT-z, and BW-z significantly decreased over time (P < 0.05), while BMI-z did not (P = 0.43). CONCLUSION: In children with NT, the SMI-z at diagnosis was a significant prognostic factor and decreased during treatment and follow-up along with HT-z and BW-z. Monitoring muscle mass is important because sarcopenia may be associated with growth impairment.

BACKGROUND: We aimed to investigate the clinical characteristics and outcomes of patients with isolated left-sided congenital diaphragmatic hernia (CDH) who developed preoperative pneumothorax and determine its risk factors. METHODS: We performed an international cohort study of patients with CDH enrolled in the Congenital Diaphragmatic Hernia Study Group registry between January 2015 and December 2020. The main outcomes assessed included survival to hospital discharge and preoperative pneumothorax development. The cumulative incidence of pneumothorax was estimated by the Gray test. The Fine and Gray competing risk regression model was used to identify the risk factors for pneumothorax. RESULTS: Data for 2858 neonates with isolated left-sided CDH were extracted; 224 (7.8%) developed preoperative pneumothorax. Among patients with a large diaphragmatic defect, those with pneumothorax had a significantly lower rate of survival to discharge than did those without. The competing risks model demonstrated that a patent ductus arteriosus with a right-to-left shunt flow after birth (hazard ratio [HR]: 1.78; 95% confidence interval [CI]: 1.21-2.63; p = 0.003) and large defects (HR: 1.65; 95% CI: 1.13-2.42; p = 0.01) were associated with an increased risk of preoperative pneumothorax. Significant differences were observed in the cumulative incidence of pneumothorax depending on defect size and shunt direction (p < 0.001). CONCLUSIONS: Pneumothorax is a significant preoperative complication associated with increased mortality in neonates with CDH, particularly in cases with large defects. Large diaphragmatic defects and persistent pulmonary hypertension were found to be risk factors for preoperative pneumothorax development. LEVEL OF EVIDENCE: LEVEL Ⅲ Retrospective Comparative Study.

PURPOSE: This pilot study evaluated indocyanine green-guided near-infrared fluorescence (ICG-NIRF) imaging of testicular blood flow to predict long-term testicular atrophy after testicular torsion (TT) surgery. METHODS: The subjects of this retrospective study were patients who underwent surgery for TT at our hospital between December, 2020 and July, 2022. After detorsion, testicular blood flow was evaluated by ICG-NIRF imaging and classified into three categories: fluorescence detected, no fluorescence detected, and fluorescence detected only in the tunica albuginea vessels. Testicular volume was measured by ultrasonography up to 12 months after surgery to evaluate long-term outcomes. RESULTS: Twelve patients were included in this analysis. We found a 100% correlation between the absence of ICG-NIRF signals and subsequent testicular atrophy. In three patients without an ICG-NIRF signal, the median testis size 12 months postoperatively was significantly smaller (16.5% of the contralateral testis; range 13-20%) than that in six patients with an ICG-NIRF signal (96%; 89-115%) (p = 0.013). Mild atrophy (74.5%; 73-76%) was also observed in the three patients for whom an ICG-NIRF signal was detected only in the tunica albuginea vessels. CONCLUSIONS: Our pilot study highlights the potential of ICG-NIRF imaging as a prognostic tool for guiding surgical decision-making for patients with TT, by predicting postoperative testicular atrophy.

PURPOSE: The study compares the surgical outcomes of very-early-onset ulcerative colitis (VEO-UC), which is a rare disease diagnosed in pediatric patients < 6 years, with those of older pediatric patients with ulcerative colitis (UC). METHODS: A retrospective observational study of 57 pediatric patients with UC was conducted at a single center. The study compared surgical complications and postoperative growth between the two groups. RESULTS: Out of the 57 patients, 6 had VEO-UC, and 5 of them underwent total colectomy. Compared with the surgical cases of older patients with UC (n = 6), the rate of postoperative complications in patients with VEO-UC (n = 5) was not significantly different, except for high-output ileostomy (80% vs. 0% at 3 weeks postoperatively, p = 0.02). The rate of postoperative central venous catheter (CVC) placement at > 90 days was higher in patients with VEO-UC (100% vs. 17%, p = 0.02). The median change in the Z-score of height before and 2 years after colectomy was not significantly different between VEO-UC and older patients (1.1 vs. 0.3, p = 0.13). CONCLUSION: With regard to complications and outcomes, total colectomy for VEO-UC patients and that for older pediatric UC patients is comparable. However, high-output ileostomy and the long duration of CVC placement may pose management challenges.

当院で新生児期に胸腔鏡下先天性横隔膜ヘルニア(CDH)修復術を行った7例(男児2例、女児5例)について検討した。当院ではCPSSで6～7点を手術の適応基準としており、1例は4点で基準を外れていたが、SIMVで換気が保たれていること、一酸化窒素の使用がなく、手術室に移動できることから、胸腔鏡手術は可能と判断した。いずれの症例も開胸・開腹に移行することなく、安全に胸腔鏡下CDH修復術を完遂できたが、1例で術中に針の出し入れによる肺損傷から気胸がみられ、胸腔ドレーン留置により改善した。尚、術後合併症は全例で認められなかった。