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To assess mid-/long-term postoperative quality of life (QOL) of esophageal atresia (EA) patients.Modified gastrointestinal quality-of-life index surveys were administered to postoperative EA patients who were at least 7 years old at evaluation to assess three topics about general lifestyle (GL), five topics about EA, and four topics about mental health (MH). For MH, caregivers were also interviewed, but separately. Subjects were divided according to age: children (7-12 years old), teenagers (13-19), and adults (20 and over) and compared according to Foker or Kimura elongation (FK) or bougienage stretching (BS).There were 22 patients evaluated. Responses for GL, EA, and MH did not differ significantly between age groups, but MH responses by caregivers for subjects who were children or teenagers scored significantly lower than responses they made themselves. For primary esophageal elongation technique (PET), age at esophagoesophagostomy was significantly higher in FK. Despite FK scoring 15.1 versus 12.4 for BS during EA evaluation, this difference was not statistically significant.Changes in QOL responses according to age were unremarkable. However, discrepancies in MH indicate that subjects felt better than their caregivers thought. PET did not appear to influence QOL.

During choledochal cyst (CC) excision, the hepaticojejunostomy anastomosis (HJA) can be performed conventionally (CHJA) or with a Carrel patch (CPA). CPA can increase CHD diameter to 10-13 mm, preventing anastomotic stenosis and intrahepatic bile duct (IHBD) stones but may be at risk for malignant transformation.The medical records of 83 cystic-type CC with CHD ≤ 9 mm followed up for at least 20 years were reviewed retrospectively. Available excised CC specimens (70/83) were re-examined blindly for pre-malignant changes. A questionnaire about suturing narrow lumens was conducted.All 83 had pancreaticobiliary maljunction. Group data were similar. Anastomoses were CPA (n = 43) and CHJA (n = 40). Mean diameter for CPA was 11.4 mm (range: 10-13 mm); for CHJA was 7.4 mm (range: 5-9 mm). Mean follow-up was 27.7 years (range: 20-42). Postoperative anastomotic stenoses were less after CPA: 1/43 (2.3%) versus 5/40 (12.5%) (p = 0.10), but CHJA had significantly more postoperative IHBD stones: 0% versus 4/40 (10.0%) (p 0.05). All IHBD stone patients had anastomotic stenosis. Excised specimens showed no pre-malignant cytology. Lumen diameter ≤ 9 mm was considered challenging by 10/10 surgical trainees and ≤ 7 mm by 16/22 pediatric surgeons.CPA appears to be oncologically safe because of the absence of malignant transformation for at least 20 years.

To assess mid-/long-term quality of life (QOL) of total colonic aganglionosis (TCA) patients.Modified pre-existing QOL assessment tools for general lifestyle (GL), bowel function (BF), and mental health (MH) were administered to postoperative TCA patients from five institutions, who were at least 7 years old to compare Duhamel (with pouch) and Swenson/Soave (without pouch) techniques between children (Ch 7-12 years old), teenagers (Tn 13-19), and adults (Ad 20 and over). For MH, caregivers were also interviewed, but separately. Maximum scores were 12 for GL/MH and 18 for BF.There were 32 subjects. GL and BF scores increased significantly from Ch (GL 4.8 ± 2.5, BF: 11.3 ± 4.6) to Tn (GL 7.8 ± 2.6, BF 16.2 ± 3.0); scores for MH did not change significantly. Mean caregiver MH scores were significantly lower than mean subject MH scores for all age groups (subject scores: 10.1, 10.7, 10.7 versus caregiver scores: 6.8, 7.8, 8.1 for Ch, Tn, Ad, respectively). PT technique/presence of a pouch did not influence the incidence of enterocolitis or QOL scores.MH responses showed subjects felt better than caregivers believed. This discrepancy could cause conflict despite steadily improving GL/BF. QOL was unaffected by PT technique/presence of a pouch.

Although pediatric tracheostomy has been a widely performed, life-saving procedure, its long-term outcomes have remained unclear. This study aimed to review outcomes after tracheostomy at a Japanese tertiary hospital and clarify candidates for and timing of decannulation.Hospital records of critically ill children who underwent tracheostomy from 2001 to 2014 were retrospectively reviewed, subsequently analyzing outcomes according to demographics, complications, and decannulation. After excluding those who were lost to follow-up or had irreversible neuromuscular impairment, the remaining patients were divided into the decannulation (D group) and nondecannulation (ND group) groups and compared.In total, 184 patients who underwent tracheostomy were analyzed (median age at operation: 0.5 y). The major indication for tracheostomy was irreversible neuromuscular impairment (46%). Surgery-related and overall mortality rates were 1% and 25%, respectively, while the successful decannulation rate was 21%. No significant difference in surgical indications or comorbidities was observed between the D (n = 39) and ND (n = 50) groups, except for infection (7 in D group versus 0 in ND group; P = 0.002) and chromosome-gene disorder (15% versus 34%; P = 0.04). The ND group had a significantly higher mortality rate than the D group (46% versus 3%; P 0.0001). The median time to decannulation was 3.6 years, while that for infection was 0.7 y.Patients who underwent tracheostomy at our institution due to temporary infections achieved more successful and earlier decannulation compared to other indications. Chromosome-gene disorder as a comorbidity can negatively affect decannulation.

Neonatal free air on X-ray images is generally due to intestinal perforation, and requires surgical intervention. However, some cases without intestinal perforation show free air on X-ray images. Pneumoperitoneum without perforation is caused by an air leak syndrome. We present here the case of a low-birth-weight infant with free air on X-ray images, who had no evidence of intestinal perforation intraoperatively.

Whether to perform surgical or conservative treatment for congenital tracheal stenosis (CTS) is controversial. Thus, the computed tomography (CT) criteria for conservative treatment of CTS were investigated. From 2005 to 2017, 28 CTS cases were included. The operative cases and preoperative death cases constituted the required intervention group (group I), and the non-operative surviving cases constituted the observation group (group O). The diameter of the tracheal narrowest part (DTNP) on CT was evaluated as a criterion for non-operative follow-up. Chest CT was performed 19 times in 19 group I cases and 18 times in 9 group O cases. The median age of the patients that underwent CT scan examinations was 3.4 months (range 0–25 months) in group I and 22 months (range 0–60 months) in group O. The cut-off values of the non-operative criteria were 40.8% (AUC: 0.82, p

Treatment is more intensive for stage III Wilms tumor (WT) than for stages I and II non-metastatic WTs. Various factors including tumor spillage, unresectability, and lymph node metastasis are responsible for stage III disease. The present study aimed to not identify clinical factors associated with the features of stage III WT to establish new treatment strategies. Of 166 patients with non-metastatic WT enrolled in the Japan Wilms Tumor Study (JWiTS)-2, 51 patients had stage III disease. The treatment protocol for JWiTS-2 was essentially the same as that in the National Wilms Tumor study (NWTS)-5. Local hospitals were surveyed to collect details of clinical findings related to stage III disease, and data regarding 45 (88%) patients were obtained. Nine patients with massive tumors underwent preoperative chemotherapy. Biopsy was performed in 6. Reduction in the tumor size was achieved in 8 of the 9 cases. Nephrectomy was finally performed in all of them. Thirty-six patients underwent primary nephrectomy. The reason for the stage III disease was lymph node metastasis (n = 9, 25%), tumor spillage (n = 20, 56%), and tumor extension/incomplete resection (n = 17, 47%). Some patients had more than one of these factors. Most patients were treated with the DD-4A regimen, and 43 (95.6%) of the 45 patients received abdominal radiation therapy. Tumors recurred in three patients (local, 1; metastasis, 2), and two patients died. Overall and relapse-free survival rates were 95.2% and 90.8%, respectively. The prognosis of stage III WT was good. In the next stage, the doses of chemotherapy and radiotherapy should be reduced to avoid late effects. The high rate of tumor spillage after primary resection suggests that preoperative chemotherapy should be started instead of aggressive tumor resection in the large tumor cases with surgical risks

Intestinal aganglionosis (IA) is so rare that the entity remains unclear. The aim of the present study was to compare the outcomes of patients with IA and those with total colonic aganglionosis (TCA). The hospital records were retrospectively reviewed from 1977 to 2018. Outcomes were analyzed for the IA group and the TCA group, including clinical presentation, initial management, and operative details. There were six patients were managed in IA (all male) and seven patients in TCA (4 male). The median age at the first operation was significantly younger in IA than TCA (2 days vs 24 days, p = 0.01). The gap between the intraoperative caliber change (CC) of the intestine and the initial stoma location was not significantly different (7.5 cm vs 12 cm, p = 0.61), but the rate of stoma dysfunction was significantly higher in IA (83% vs 0%, p = 0.005). The gap between the CC and the ganglionated bowel was significantly longer in IA (85 cm vs 10 cm, p = 0.003). Patients with IA appear to have a high risk for stoma dysfunction after the first operation because of the unexpected gap between the CC and normoganglia. The initial location of the stoma requires careful consideration.

This study assessed the necessity of postoperative nasogastric tube (NGT) use in acute pediatric cases of perforated appendicitis. All cases of acute pediatric perforated appendicitis managed with transumbilical laparoscopic-assisted appendectomy at our hospital from 2011 to 2017 were retrospectively reviewed. Sixty-two cases were selected and divided into two groups based on NGT placement. There were no significant differences between the two groups in most parameters of patient demographics, or surgical data. Notably, the mean time to first oral intake and to regular diet was significantly shorter in no-NGT group (1 day vs 3 days, P

Purpose: We compared laparoscopic redo fundoplications performed for failed laparoscopic Toupet fundoplication (LTF) and failed laparoscopic Nissen fundoplications (LNFs). Methods: Redo LTF (R-LTF; n = 4) and redo LNF (R-LNF; n = 6) performed between 2007 and 2014 were assessed retrospectively for severity of intraperitoneal adhesions on a scale of 0–3, identification/preservation of the anterior/posterior/hepatic branches of the vagus nerve (VN), complications, and outcome. Results: Redos were performed after a mean of 34 months in R-LTF and 32 months in R-LNF (P = ns) indicated for sliding hernia (n = 3; 2 with partial wrap dehiscence) and partial wrap dehiscence (n = 1) in R-LTF and sliding hernia (n = 6; 4 with partial wrap dehiscence) in R-LNF. The mean adhesion severity score was 1.5 in R-LTF and 2.5 in R-LNF (P < 0.05). The mean number of VN branches identified/preserved was 2.0 in R-LTF and 0.8 in R-LNF (P < 0.05). Mean operative times and mean blood loss were similar. Intraoperative complications were accidental local trauma (n = 1 in R-LTF and n = 3 in R-LNF, one requiring conversion to open repair) (P = ns). Gastric outlet obstruction developed in two R-LNF cases; both were managed conservatively. There have been no further recurrences to date. Conclusion: Although our series is small, adhesions were less, and identification/preservation of VN was easier during R-LTF.

Foreign body aspiration (FBA), with potentially life-threatening outcomes, is not unusual in the pediatric population. We report two cases of lobar bronchial radiolucent foreign bodies. Chest X-ray (CXR) showed a slight but significant finding of lobar emphysema without a significant mediastinal shift. This is possibly a key to suspecting foreign bodies. In the clinical field, a stepwise approach to detecting foreign bodies is commonly performed, from less invasive options such as CXR to computed tomography (CT). In this context, clinicians should scrupulously check CXRs when pediatric patients complain of respiratory symptoms, especially with potential FBA history.

A fecaloma is a mass of accumulated feces with a consistency much harder than that of a fecal impaction. It is most frequently observed in the rectum and sigmoid area, and associated complications include colonic obstruction, ulceration, bleeding, and perforation. A one-year-old, previously healthy boy with no history of chronic constipation was admitted because of vomiting and abdominal distension. An abdominal computed tomography scan showed small and large bowel distension due to multiple obstructive fecalomas in the transverse colon. As the fecalomas could not be resolved by laxatives, enemas, or colonic lavage, endoscopic disimpaction under general anesthesia was attempted. Repeatedly shaving the fecalomas with biopsy forceps finally resulted in gradual fragmentation with subsequent passage. Gastrointestinal food allergy was later suggested as the cause because eosinophilic infiltration was found in a biopsy specimen of the colon wall. Endoscopic disimpaction is an effective treatment approach for addressing fecalomas to avoid more invasive surgical intervention.

In postoperative cases of fundoplication, the gastric emptying ability is promoted and sometimes exhibits dumping syndrome. Dumping syndrome often goes unrecognized in children. Furthermore, the risk factors for postoperative dumping syndrome are unknown. This study aimed to investigate the risk factors of developing dumping syndrome after fundoplication. A retrospective chart review of all consecutive patients between January 2003 and March 2018 (190 patients) who had fundoplication at our clinic was conducted. Regarding the risk factors of dumping syndrome, gender, age and body weight at the time of surgery, neurological impairment, severe scoliosis, microgastria, chromosomal abnormalities, complex cardiac anomalies, gastrostomy, and laparoscopic surgery were retrospectively studied. 17 patients (9%) developed dumping syndrome post-operatively. Multivariate analysis showed that significant risk factors for dumping syndrome included: undergoing surgery within 12 months of age (adjusted OR 10.3, 95% CI 2.6–45.2), severe scoliosis (adjusted OR 19.3, 95% CI 4.4–91.1), and microgastria (adjusted OR 26.5, 95% CI 1.4–896.4). We identified that: age at fundoplication being within 12 months of age, severe scoliosis, and microgastria were risk factors for dumping syndrome after fundoplication, and that this information should be explaining to the family before conducting the fundoplication.

Aim: We reviewed intraoperative video recordings (IVRs) of laparoscopic percutaneous extraperitoneal closure (LPEC) for inguinal hernia in children blindly to assess performance. Methods: IVRs of 1...

INTRODUCTION Congenital tracheal stenosis (CTS) with a bilateral tracheal bronchus (TB) has not been reported as a subtype of CTS. A novel technique to manage CTS in patients with a bilateral TB is described. CASE REPORT An infant with tetralogy of Fallot underwent repair of cardiac anomaly at age 1 month. He experienced numerous cyanosis and episodes of transient respiratory arrest. Chest computed tomography (CT) demonstrated an aberrant bilateral upper lobe bronchus arising directly from the trachea and a stenotic trachea connecting the pseudo- carina to the true carina between the common right lower and left lower bronchus. On bronchoscopy, the diameter of the lumen of the narrowed segment was estimated to be less than 2 mm. Tracheal reconstruction was undertaken when he was 2 years of age. The surgical technique using a modified slide tracheoplasty for the correction of this anomaly are described. After surgery, the patient was extubated and has had no respiratory symptoms. DISCUSSION AND CONCLUSION The patient had unique anatomic considerations that made reconstruction challenging. Our technique of covering a stenotic section by normal trachea is a modification of the slide tracheoplasty technique and is useful for CTS with a unilateral and a bilateral TB.

Blue rubber bleb nevus syndrome (BRBNS) is a rare disease in which venous malformations (VMs) involve any body organ, most commonly the skin and the gastrointestinal (GI) tract. Treatment of BRBNS aims at preserving the GI tract as much as possible. Although there are several dozen case reports about BRBNS that describe short-term clinical courses, a few provide an account of long-term clinical course. Here, we report a case of BRBNS in a girl that required multiple abdominal surgeries due to the GI VMs and a recurrence at an interval of 14 years. The preferred approach for gastrointestinal VMs involves the complete resection of all lesions without residual VMs. It is important to bear in mind the possibility of delayed recurrence of GI VMs after surgical or endoscopic treatment.

The aim of this study was to investigate the risk factors for in hospital mortality in infants with esophageal atresia (EA) without critical chromosome abnormality disorders and develop a new prognostic classification to assess the influence of external risk factors on the prognosis of EA, which could provide more effective treatment strategies in clinical practice. A retrospective chart review of all consecutive patients between January 1994 and December 2017, which included 65 EA infants who were admitted to Shizuoka Children’s Hospital, was conducted. Based on multivariate analysis data and ROC analysis, the discrimination of the new prognostic classification was quantified and compared with that of the Spitz classification using the area under the ROC curve (AUC). Multiple logistic regression analysis showed that birth weight of

Background: The combination of congenital tracheal stenosis (CTS) and unilateral pulmonary agenesis is rare and fatal. Especially, right lung agenesis is associated with dextrocardia and often causes tracheal alignment disorder. The mortality is estimated with 12.5–65%. Slide Tracheoplasty (ST) has been improved the prognosis, however, the postoperative course of CTS with single lung remains unclear. Here, we describe the postoperative challenging situation with bronchial intervention and other devices. Case presentation: A 9-month-old girl with CTS, right lung agenesis, dextrocardia, and bilateral superior vena cava underwent ST anterior to the aortic arch. On postoperative day 50, tracheostomy was performed for long-term airway pressure support. Six months after the ST, she showed severe bronchomalacia at the bifurcation of the lobar bronchi, which was sandwiched between the descending aorta and left pulmonary artery. Direct bronchopexy and mobilization of left pulmonary artery resolved the severe bronchomalacia. Additionally, the kinked trachea caused a tracheal ulcer and frequent accidental decannulations. The ulcer was made above the aortic arch by contact with the tracheal cannula. The customized Montgomery T-tube solved both problems and enabled the stable management for airway. Her condition eventually improved, and she was discharged home after 14 months of hospitalization. Conclusion: This case presented a very rare and critical condition. This is the first report to address the severe condition after ST anterior to the aortic arch. Surgical interventions or other devices for treatment are thought to be modified according to each condition. Keywords: Conjenital tracheal stenosis, Unilateral pulmonary agenesis, Dextorcardia, Bronchomalacia, Direct bronchopexy, Customized Montgomery T-tube

We investigated the developmental changes in the unaffected contralateral lungs of patients with isolated left-sided congenital diaphragmatic hernia (CDH) using signal intensity ratios on prenatal magnetic resonance imaging (MRI) and determined whether these changes correlated with clinical outcomes. We performed 47 fetal MRI screens on 30 patients with isolated left-sided CDH. A cohort of 88 fetuses was selected as the control. We calculated the lung-to-liver signal intensity ratio (LLSIR) using region of interest analysis and compared LLSIR between the groups and between those in the CDH group with good and poor prognoses. In the control group, LLSIR increased as pregnancy progressed [regression line = 2.232 + 0.135 × (GW—23), r = 0.669]. In the CDH group, especially in the poor prognosis group, LLSIR did not significantly increase as pregnancy progressed [regression line for good prognosis = 1.827 + 0.092 × (gestational week—23), r = 0.733; regression line for poor prognosis = 1.731 + 0.025 × (gestational week—23), r = 0.634]. Fetal LLSIR on T2-weighted MRI is an accurate marker of fetal lung maturity that correlates with postnatal survival and can potentially be used as a prognostic parameter in CDH management.

Introduction We compared laparoscopic Toupet fundoplication (LTF) and laparoscopic Nissen fundoplication (LNF) in neurologically normal children. Methods Forty neurologically normal children who were followed up for more than 3 years after LTF (n = 22) or LNF (n = 18) were reviewed retrospectively. LTF and LNF were performed between 2006 and 2012. Results There were no significant differences in gender (LTF, 15 male and 7 female patients; LNF:, 12 male and 6 female patients), mean age at surgery (LTF vs LNF: 2.5 vs 2.3 years), mean weight at surgery (LTF vs LNF: 9.6 vs 8.9 kg), preoperative symptoms, preoperative pH monitoring (pH

This study investigated risk factors for developmental disorders after the repair of esophageal atresia with tracheoesophageal fistula (EA/TEF). The clinical charts of all infants with EA/TEF (gross type C) treated at our institution between 1993 and 2013 were analyzed. Patients with chromosomal aberrations were excluded. Forty-seven patients were divided into groups according to the presence (D) or absence (N) of developmental disorders. Patients were assessed with appropriate developmental examinations at age >3 years. Group D comprised 13 patients. The 34 patients in the group N had received a standard education. In groups D and N, gestational age was 36.5 versus 38.5 weeks, birth weight was 1808 versus 2662 g, and congenital cardiac anomalies occurred in 76.9 versus 23.5%. These differences reached significance. Operative data did not differ significantly. Overall complications occurred in 69.2 versus 29.4%, gastroesophageal reflux (GER) requiring fundoplication in 46.1 versus 8.8%, and oral ingestion difficulty in 61.5 versus 14.7%, which were significantly different. The frequencies of anastomotic leakage, anastomotic stricture, and recurrent fistula did not differ significantly. Gestation, birth weight, and cardiac anomalies could be risk factors for developmental disorders. Moreover, overall complications, GER, and oral ingestion may affect development.

Background The use of laparoscopic percutaneous extraperitoneal closure (LPEC) for pediatric inguinal hernia has recently been increasing. Recurrence and contralateral metachronous inguinal hernia (CMIH) are important problems for LPEC. This study analyzed the risk factors for recurrence and CMIH. Methods This study included 1530 patients. The mean follow-up period was 48 months. Of 1530 patients, 847 were boys and 683 were girls. The mean age at operation was 3.9 years. The asymptomatic contralateral internal ring was routinely observed during the operation, and when a patent processus vaginalis (PPV) was confirmed, prophylactic surgery was performed. Results Recurrence was seen in 0.48% of patients (8/1653 sides), all of whom were male (P = 0.01: male versus female). On multivariate analysis, age less than 1 year was the only risk factor for recurrence in male patients (hazard ratio: 4.54, 95% CI: 1.07–19.25, P = 0.04). CMIH was seen in 0.22% of the patients (3/1382), again only in male patients (P = 0.12: male versus female). As a result of intraoperative observation, 44.6% of patients were confirmed to have an asymptomatic contralateral PPV and underwent prophylactic LPEC. Female, age 1 year or older, right side, and surgeon's experience were identified as factors associated with asymptomatic contralateral PPV. Conclusions To prevent recurrence, surgeons need to be careful when operating on young male patients. Whereas no specific factor could be identified as a risk factor for CMIH, some factors associated with asymptomatic PPV were identified. Further study and discussion will be needed to identify correlations between CMIH and these factors for PPV. Level of evidence Level III.