The comorbidities of recurrent inguinal hernia in children: A systematic review.

BACKGROUND: Higher risk of recurrence has been reported in pediatric inguinal hernia patients with specific comorbidities. The purpose of this systematic review was to investigate which comorbidities predispose to recurrent pediatric inguinal hernias (RPIHs). METHODS: A comprehensive search of six databases was performed, reviewing the literature to date on RPIHs and the co-occurrence of comorbidities. English-language publications were considered for inclusion. The primary surgical technique (e.g., Potts procedure or laparoscopic repair) was not considered. RESULTS: Fourteen articles published between 1967 and 2021 fulfilled the inclusion criteria and did not meet the exclusion criteria. They reported a total of 86 patients diagnosed with RPIHs with 99 comorbidities. Thirty-six percent of patients had conditions with increased intra-abdominal pressure, such as ventriculoperitoneal shunt for hydrocephalus, posterior urethral valves, bladder exstrophy, seizure disorder, asthma, using continuous positive airway pressure for respiratory distress syndrome, and gastroesophageal reflux disease. Twenty-eight percent of patients had diseases with weakness of the anterior abdominal wall, specifically mucopolysaccharidosis, giant omphalocele, Ehlers-Danlos syndrome, connective-tissue disorders, and segmental spinal dysgenesis. CONCLUSIONS: The main comorbidities of RPIHs were conditions with increased intra-abdominal pressure and weakness of the anterior abdominal wall. Although these comorbidities are rare, the risk of recurrence must be noted.

Safety evaluation of a stepwise tracheostomy decannulation program in pediatric patients.

PURPOSE: In the event of failed tracheostomy decannulation, patients might have a tragic course of events. We retrospectively evaluated our stepwise tracheostomy decannulation program and examined its safety. METHODS: A 12-year retrospective study of pediatric patients was conducted. The decannulation program was performed on patients who had airway patency by laryngobronchoscopy and whose cannula could be capped during the day. A stepwise decannulation program was performed: continuous 48-h capping trial during hospitalization (Phase 1), removal of the tracheostomy tube for 48 h during hospitalization (Phase 2), and outpatient observation (Phase 3). If a persistent tracheocutaneous fistula existed, the fistula was closed by surgery (Phase 4). RESULTS: The 77 patients in the study underwent 86 trials. The age at the first time of the decannulation program was 6.5 ± 3.6 years. Sixteen trials failed (18.6%): 8 trials in Phase 1, 2 trials in Phase 2, 4 trials in Phase 3, and 2 trials in Phase 4. Most decannulation failures were due to desaturation in Phase 1/2 and dyspnea in Phase 3/4. The time to reintubation after decannulation was 15-383 days in Phase 3/4. CONCLUSIONS: Patients could fail at every phase of the program, suggesting that a stepwise decannulation program contributes to safety.

Pancreaticobiliary maljunction without biliary dilatation in pediatric patients.

PURPOSE: Pancreaticobiliary maljunction (PBM) without biliary dilatation is a condition in which dilatation of the bile duct is not seen in patients with PBM. Recently, the Japanese Study Group on Pancreaticobiliary Maljunction (JSGPM) published new diagnostic criteria for PBM. In these criteria, biliary dilatation is defined according to the standard diameter at each age. We reviewed cases of pediatric patients with PBM without biliary dilatation. METHODS: From 1992 to 2019, 134 patients with PBM were treated in our institution. Among these, 7 patients were retrospectively diagnosed with PBM without biliary dilatation. The clinical information was retrospectively assessed in these patients. RESULTS: Of the seven patients, six were female. All patients had symptoms similar to those of patients with congenital biliary dilatation. In all seven patients, the diagnosis of PBM was made before definitive surgery. Six patients had type B PBM, and one had type D PBM. All patients underwent extrahepatic bile duct resection and hepaticojejunostomy, and their symptoms resolved. One patient experienced postoperative complications of anastomotic leakage followed by anastomotic stricture. CONCLUSION: The present report revealed important clinical features of this entity. However, there are still some issues that need to be discussed, and further research is needed.

Oncologic safety of Carrel patch hepaticojejunostomy for treating cystic-type choledochal cyst in children based on 20-plus years follow-up.

AIM: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: CPA appears to be oncologically safe because of the absence of malignant transformation for at least 20 years.

Quality of life after type-A esophageal atresia surgery: changes over time and effect of pre-anastomotic elongation.

AIM: To assess mid-/long-term postoperative quality of life (QOL) of esophageal atresia (EA) patients. METHODS: 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). RESULTS: 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. CONCLUSION: 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.

Changes in postoperative quality of life of pediatric total colonic aganglionosis patients: effect of pull-through technique.

AIM: To assess mid-/long-term quality of life (QOL) of total colonic aganglionosis (TCA) patients. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Risk factors of dumping syndrome after fundoplication for gastroesophageal reflux in children.

PURPOSE: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

Long-Term Outcomes After Pediatric Tracheostomy-Candidates for and Timing of Decannulation.

BACKGROUND: 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. MATERIALS AND METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Long-term follow-up for anorectal function after anorectoplasty in patients with high/intermediate imperforate anus: a single center experience.

PURPOSE: The aim of this study was to evaluate the clinical outcomes and postoperative anorectal function in the patients with high/intermediate imperforate anus (HIA/IIA) treated in our institution. In addition, we report our novel procedure, laparoscopically assisted anorectoplasty with anterior perineal incision (LAARP-API), which allows direct approach to the anterior edge of the puborectal sling and muscle complex, and is useful for the creation of a pulling-through route for the rectal pouch. METHODS: From 1976 to 2016, 22 patients with HIA and 43 patients with IIA underwent sacroperineal/sacroabdominoperineal pull-through anorectoplasty (SP/SAP), Potts procedure (Potts), SP with API (SP-API) or LAARP-API. Clinical data and anorectal function of those patients were retrospectively evaluated using the Japanese clinical score. RESULTS: Of the 22 cases of HIA, 15 were treated by SAP, 2 were SP and 5 were LAARP-API. Of the 43 cases of IIA, one was treated by SAP, 31 were SP, two were Potts and nine were SP-API. The mean score of anorectal function of HIA/IIA both increased with age. In IIA, the score after SP-API was significantly higher than the score after SP. CONCLUSION: Long-term outcomes of our anorectoplasty for HIA/IIA are good with excellent anorectal function score.

Factors responsible for stage III disease in patients with Wilms tumor enrolled in the JWiTS-2 study.

BACKGROUND/OBJECTIVES: 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. DESIGN/METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Unexpected gap between intraoperative caliber change of the intestine and normoganglia in patients with intestinal aganglionosis.

PURPOSE: 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). METHODS: 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. RESULTS: 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). CONCLUSION: 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.

Non-operative management of congenital tracheal stenosis: criteria by computed tomography.

OBJECTIVES: 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. METHODS: 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. RESULTS: 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 < .01) normal for age of the trachea's narrowest part, and 41.6% normal for body weight (AUC: 0.92, p < .01), respectively. CONCLUSIONS: DTNP is 40% and more of the normal diameter appears necessary for non-surgical management. The present study suggests that the criteria for conservative management of CTS are that the DTNP is not less than 40% of the normal tracheal diameter, with a few symptoms.

Risk factors for the recurrence of perineal canal.

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.

A Comparison of Laparoscopic Redo Fundoplications for Failed Toupet and Nissen Fundoplications in Children.

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.

Serial intralesional triamcinolone acetonide injections for acquired subglottic stenosis in premature infants.

PURPOSE: Long-term intubation of premature infants sometimes induces acquired subglottic stenosis (SGS), causing glottic or supraglottic problems. These kinds of SGS often require tracheostomy and subsequently make decannulation difficult. The aim of our study was to clarify the efficacy of repeated intralesional steroid injections to the stenosis. METHODS: Six children with acquired SGS, who were treated with triamcinolone acetonide injections to the subglottic space just below the vocal folds between September 2015 and December 2017 were retrospectively reviewed. RESULTS: The patients' mean age was 4.3 (range 1.3-4.4) years, the mean gestational age at birth was 25 (23-28) weeks, and the mean birth weight was 591 (456-734) g. The degree of SGS was grade II in one patient and III in five patients, with both tracheostoma and glottic or supraglottic abnormalities. They generally underwent ten procedures, every 3-4 weeks. In most cases, the patency of the injected space improved by 25-220%, and the symptoms were relieved. One patient achieved decannulation, and another one underwent laryngotracheal reconstruction and decannulation. Two patients started using a speech cannula. There were no severe complications. CONCLUSION: Serial intralesional steroid injections are likely to be effective in improving the patency of acquired SGS.

New prognostic classification and managements in infants with esophageal atresia.

PURPOSE: 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. METHODS: 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). RESULTS: Multiple logistic regression analysis showed that birth weight of < 1606 g (adjusted OR, 13.16; 95% CI, 1.16-352.75), and complex cardiac anomalies (adjusted OR 22.39; 95% CI 2.45-569.14) were significant risk factors for death. We have created a new classification close to Spitz classification using the presence of complex cardiac anomalies and birth weight. The mortality rates were 0% for class I (n = 0/40), 7.1% for class II (n = 1/14), 33.3% for class III (n = 3/9), and 100% for class IV (n = 2/2). The AUC of the new classification was better than that of the Spitz classification (0.939 vs 0.812, respectively; p = 0.04). CONCLUSION: New prognostic classification can improve the stratification of EA patients and be a useful predictor of survival.

The fetal lung-to-liver signal intensity ratio on magnetic resonance imaging as a predictor of outcomes from isolated congenital diaphragmatic hernia.

PURPOSE: 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. METHODS: 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. RESULTS: 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]. CONCLUSION: 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.