Case Report: Robotic pylorus-preserving pancreatoduodenectomy for periampullary rhabdomyosarcoma in a 3-year-old patient.

Periampullary neoplasm is rare in pediatric patients and has constituted a strict indication for pancreatoduodenectomy (PD), which is a procedure sporadically reported in the literature among children. Robotic PD has been routinely performed for periampullary neoplasm in periampullary neoplasm, but only a few cases in pediatric patients have been reported. Here, we report the case of a 3-year-old patient with periampullary rhabdomyosarcoma treated with robotic pylorus-preserving PD and share our experience with this procedure in pediatric patients. A 3-year-old patient presented with obstructive jaundice and a mass in the pancreatic head revealed by imaging. A laparoscopic biopsy was performed. Jaundice progressed with abdominal pain and elevated alpha-amylase leading to urgent robotic exploration in which a periampullary neoplasm was revealed and pathologically diagnosed as rhabdomyosarcoma by frozen section examination. After pylorus-preserving PD, we performed a conventional jejunal loop following a child reconstruction, including an end-to-end pancreaticojejunostomy, followed by end-to-side hepaticojejunostomy and duodenojejunostomy. Delayed gastric emptying (DGE) presented with increasing drain from the nasogastric tube (NGT) a week after the surgery and improved spontaneously within 10 days. In a 13-month follow-up until the present, our case patient recovered well without potentially fatal complications, such as pancreatic fistula. Robotic PD in pediatric patients was safe and effective without intra- or postoperative complications.

Alterations of gut microbiota in infants with biliary atresia identified by 16S rRNA-sequencing.

BACKGROUND: Biliary atresia (BA) is a severe neonatal disease with progressive intra- and extra-hepatic bile ducts inflammation and hepatic fibrosis. Characterization of gut microbiome profiles in infants with biliary atresia can provide valuable information and potential disease biomarkers. Our study aims to explore the relationship between gut microbiota and biliary atresia. METHODS: 16 S ribosomal RNA (rRNA) gene sequencing was carried out to identify the differences in composition and diversity of gut microbiota between infants with BA and healthy subjects. A total of 31 infants with biliary atresia and 20 healthy subjects were recruited. RESULTS: The composition of gut microbiota in BA group was significantly different with the normal control group (P < 0.05) and the abundance ratio of Klebsiella/Bifidobacterium showed great potential for identification of BA (P < 0.01). In addition, the differential bacterial taxa were involved in lipid and vitamins metabolism. CONCLUSION: Our results could provide potential non-invasive biomarker for identification of biliary atresia and contribute to the treatment in terms of ameliorating microbiota dysbiosis.

Diamond-shaped versus side-to-side anastomotic duodenoduodenostomy in laparoscopic management of annular pancreas in children: a single-center retrospective comparative study.

Background: Annular pancreas is a rare congenital disorder that requires surgical management once diagnosed. Diamond-shaped and side-to-side duodenoduodenostomy are both popular worldwide nowadays in the surgical management of annular pancreas. Here we present our experience with laparoscopic management of annular pancreas in the last 5 years and compare the clinical results of the diamond-shaped versus side-to-side anastomotic techniques. Methods: Fifty-two patients diagnosed with annular pancreas who underwent duodenoduodenostomy at our medical center between January 2016 and April 2021 were included in the study. Forty-four patients underwent laparoscopic diamond-shaped duodenoduodenostomy (DS group) and eight underwent laparoscopic side-to-side duodenoduodenostomy (STS group). Clinical data, including surgical indices and early outcomes after surgery, with at least 19 months of follow-up, were collected and analyzed. Results: Of the 52 patients, 61.5% were prenatally diagnosed, and vomiting was the most common clinical manifestation after birth. The operative time and bleeding volume were 187.5 [interquartile range (IQR), 150-228)] min and 2 (IQR, 2-5) mL in the DS group, compared to 175 (IQR, 155-270) min and 2 (IQR, 2-4.25) mL in the STS group (P=0.89 and 0.32 respectively). The mean time from surgery to initial oral feeding and full oral feeding was 6 (IQR, 4-10) and 12 (IQR, 10-15) days in the DS group, compared to 8 (IQR, 4.75-11.25) and 14.5 (IQR, 13-16.75) days in the STS group (P=0.61 and 0.46 respectively). The mean hospital stay was 16 (IQR, 14-19) and 20 (IQR, 17.75-26) days in the DS and STS groups respectively (P=0.13). No severe complications such as anastomotic leakage, anastomotic stenosis, reoperation or unsuspected rehospitalization were noted in either group. Feeding intolerance was revealed in six cases in the DS group and two cases in the STS group, and there was no significant difference between the two groups (P=0.50). Conclusions: Both laparoscopic diamond-shaped and side-to-side techniques showed good clinical results in treating annular pancreas. The surgical technique, trans-anastomotic tube and early feeding are not likely to increase the risk of postoperative feeding intolerance.

Early bile drainage improves native liver survival in biliary atresia without cholangitis.

Objectives: To explore the outcomes and related factors in children without cholangitis after Kasai portoenterostomy (KPE). Methods: We retrospectively analyzed the data of infants with type III BA who underwent KPE from June 2016 to December 2021. We compared and analyzed the difference in native liver survival (NLS) rates in different types of cholangitis. We also investigated the relationship between the absence of cholangitis and the effect of early bile drainage (EBD) as well as the related factors affecting EBD efficacy. Results: A total of 145 children were included in this study. Among these children, 82 (56.6%, 82/145) had cholangitis, including 40 (48.8%, 40/82) with early cholangitis and 33 (40.2%, 33/82) with recurrent cholangitis. The median follow-up period was 29 months (range, 2-75 months). The NLS rates were 67.6%, 51.7%, 45.5% and 43.4% at 6 months, 1 year, 2 years and 5 years following KPE, while the NLS rates for infants without cholangitis after KPE were 68.3%, 50.8%, 46.0% and 46.0%, respectively. Higher gamma-glutamyl transferase (γ- GT) and total bile acid (TBA) before KPE were risk factors for cholangitis (P < 0.05). The NLS rate in recurrent cholangitis was significantly lower than that in occasional cholangitis (P < 0.01). Compared with the EBD-poor group, the NLS rate in the EBD-good group of infants was significantly increased (P < 0.001). EBD was significantly correlated with the occurrence and frequency of cholangitis (P < 0.05). Conclusions: Recurrent cholangitis was an important factor affecting NLS. For children without cholangitis after KPE, early bile drainage was better, and the NLS was longer.

Management of pancreatic kaposiform hemangioendothelioma with sirolimus in a pediatric patient: a case report and literature review.

Background: Kaposiform hemangioendothelioma (KHE) is a rare locally aggressive vascular tumor. The pancreas is not a common site of KHE, especially in pediatric patients. Given that no guidelines are available for the treatment of KHE, management is currently based on expert opinions and clinical experiences. Here, we report a case of pancreatic KHE with obstructive jaundice, which was treated successfully with oral sirolimus instead of radical surgery. Additionally, a literature review on pancreatic KHE was performed to summarize prior clinical experiences and the available treatments. Case Description: A 10-month-old Chinese male infant presented with obstructive jaundice without any signs of fever, abdominal pain, or distention. A detailed consultation revealed an uneventful history. The obstructive jaundice worsened significantly during 3 weeks of conservative therapy. A pancreatic mass was identified via radiological evidence, and a laparoscopic biopsy of the tumor was performed, which confirmed the diagnosis of pancreatic KHE based on histological findings. Oral sirolimus 0.8 mg/m2 twice daily was administered at a steady serum concentration of 5-15 ng/mL, which led to a shrinkage in tumor size and resolution of jaundice. The patient showed no evidence of recurrence after 1 year of follow-up and is still on sirolimus treatment, which has been tolerated well up to the time of this report. Conclusions: The pancreas is a rare location of KHE, which is a locally aggressive vascular tumor. Diagnosis is based on histological findings, and therapy should be multidisciplinary and individualized. Although sirolimus has been very successful in the treatment of KHE even without radical surgery, the possible risks of tumor recurrence and adverse effects warrant some caution.

Clinical Characteristics of Gastric Duplication in Children.

Background: To investigate the clinical characteristics of gastric duplication (GD) in children. Methods: The clinical data of 17 children with GD who were treated in our hospital from July 2015 to June 2021 were analyzed retrospectively. There were 8 males and 9 females, aged from 2 months to 11 years. All children underwent laparoscopic GD resections and postoperative pathological diagnosis was GD. In addition, we searched and analyzed the literature on GD in children from 1 January 2011 to 31 December 2021 from the PubMed, EMBASE, and Cochrane Library databases. Results: Gastric duplication was more common in females, with the most common cystic type occurring in the greater curvature of the stomach. Vomiting is the most common clinical manifestation. Ultrasound is an effective method for the early screening of GD. In this study, one patient who had multiple GDs underwent laparoscopic cystectomy and mucosectomy, one patient was converted to open surgery, and all other children underwent laparoscopic cystectomies. The time to oral intake was 2.3 ± 1.0 days (range: 1-4 days), and the postoperative hospital stay was 5.7 ± 1.7 days (range: 2-9 days). All children were followed up for 6-77 months and had an uneventful recovery with the resolution of the preoperative symptoms. Conclusion: Gastric duplication in children lacks specific clinical manifestations, and the preoperative diagnosis rate is not high, so surgical exploration combined with pathological examination is often needed to make a clear diagnosis. Laparoscopic cystectomy can achieve good therapeutic results.

Application of Embedding Hepaticojejunostomy in Children with Pancreaticobiliary Maljunction Without Biliary Dilatation.

Background: To investigate the effect of embedded hepaticojejunostomy in children with pancreaticobiliary maljunction (PBM) without biliary dilatation. Materials and Methods: The clinical data of 10 patients with nondilated PBM from February 2017 to July 2020 were retrospectively analyzed. Perioperative liver function indexes were compared. Results: All patients were diagnosed by magnetic resonance cholangiopancreatography (MRCP) combined with intraoperative cholangiography. There were 5 cases of Komi type I and 5 cases of type II; the diameter of the common bile duct was 4-9 mm (median: 6 mm); and the length of the common channel was 5-15 mm (median: 9.25 mm). The procedure for one patient with common duct stones was converted to open surgery. Laparoscopic cholecystectomy, common bile duct resection, and embedded hepaticojejunostomy were successfully performed in all 10 cases. The average operation time was 225 ± 96.64 min, and the intraoperative blood loss was 2-5 mL. The mean time to oral intake was 3.5 ± 1.65 days (range: 2-5 days), and the mean hospitalization duration was 6.2 ± 2.44 days (range: 5-8 days). The differences in liver function indexes in the perioperative period were statistically significant (P < .05). The patients were followed-up for 13 to 54 months (median: 40 months). All patients grew well and there was no bile duct dilatation, calculus, or cirrhosis on B-ultrasound examinations. Conclusions: The clinical manifestations of nondilated PBM are often concealed, and preoperative MRCP was important for obtaining a diagnosis. Laparoscopic cholecystectomy, common bile duct resection, and embedded hepaticojejunostomy are feasible for treating nondilated PBM.

Total versus conventional laparoscopic cyst excision and Roux-en-Y hepaticojejunostomy in children with choledochal cysts: a case-control study.

BACKGROUND: To compare the efficacy of total and conventional laparoscopic hepaticojejunostomy (TLH and CLH) in children with choledochal cysts (CDCs). METHODS: Data from patients undergoing TLH and CLH between August 2017 and December 2018 were retrospectively analyzed. Intraoperative blood loss, time for jejunum-cojejunum anastomosis, time to oral intake, postoperative hospital stay, hospitalization expenses, and postoperative complications were compared. RESULTS: All 55 patients (TLH = 30, CLH = 25) were successfully treated without conversion to open surgery. In the TLH and CLH groups, the time to oral intake was 3.57 ± 0.19 d and 4.56 ± 0.27 d, respectively (t = 3.07, P < 0.01), the postoperative hospital stay was 5.50 ± 0.28 d and 7.00 ± 0.74 d (t = 2.03, P < 0.05), and the hospitalization expenses were CNY 40,085 ± 2447 and CNY 26,084 ± 2776 (t = 3.79, P < 0.001). There were no significant differences in intraoperative blood loss (9.57 ± 3.28 ml vs 8.2 ± 1.13 ml, t = 0.37, P = 0.72) or time for jejunum-cojejunum anastomosis (80.5 ± 2.46 min vs 75.00 ± 2.04 min, t = 1.68, P = 0.10). The median follow-up periods of the TLH and CLH groups were 17 and 16 months, respectively. Overall complication rates were comparable between the two groups (10% vs 8%, χ2 = 0.07, P = 0.79). CONCLUSIONS: TLH in children with CDCs has the advantages of rapid gastrointestinal functional recovery and a short hospitalization. However, hospitalization is relatively expensive.

PDGFA gene rs9690350 polymorphism increases biliary atresia risk in Chinese children.

Biliary atresia (BA) is a genetic and severe fibro-inflammatory obliterative cholangiopathy of neonates. Platelet-derived growth factor subunit A (PDGFA), as one of participants in liver fibrosis, the overexpression of PDGFA through DNA hypomethylation may lead to the development of BA, but the pathogenesis is still unclear. We conducted a large case-control cohort to investigate the association of genetic variants in PDGFA with BA susceptibility in the Southern Chinese population (506 cases and 1473 controls). We observed that the G allele of rs9690350(G>C) in PDGFA was significantly associated with an increased risk of BA (OR = 1.24, 95% CI = 1.04-1.49, P=0.02). Additionally, the rs9690350 G allele increased the risk of non-cystic biliary atresia (OR = 1.26, 95% CI = 1.04-1.52, P=0.02) and was a genetic biomarker of severe manifestations after surgery. These findings indicate that the rs9690350 G allele is a PDGFA polymorphism associated with the risk of BA that may confer increased disease susceptibility.

Parental Self-efficacy and Health-related Outcomes Among Children with Hirschsprung Disease: A Cross-sectional Study.

PURPOSE: Little attention has been put to parental self-efficacy (PSE) on the home care management and its impact on the health-related outcome in children with Hirschsprung disease (HD) after surgery. The purpose of this study was to investigate the association between PSE and post-operative outcome and quality of life (QoL) in children with HD. DESIGN AND METHODS: This study adopted a cross-sectional study design. Children diagnosed with HD who had surgery during 2015 and 2018, and their parents were included. Parental self-efficacy, children's post-operative fecal continence and QoL were evaluated with validated questionnaires; post-operative readmission and adverse events were extracted from electronic medical record system. RESULTS: Of the eligible families, 69.6% (96/138) responded to the follow-up. The median children's age at surgery and current age were 16 (interquartile range: 10-32) and 45 (interquartile range: 39.7-57) months, respectively. The mean PSE score is 8.78 points, with the lowest score in the bowel habit training dimension (7.88 ± 2.28), followed by getting social support dimension (8.07 ± 2.64). Multivariable linear regression showed that PSE was associated with fecal continence (ß = 0.043, 95% CI 0.013-0.072), pediatric QoL total score (ß = 0.210, 95% CI 0.011-0.409) and social score (ß = 0.273, 95% CI 0.022-0.525). No associations were observed between PSE and weight z-score, height z-score, readmission or adverse events. CONCLUSIONS: PSE is correlated with fecal continence and QoL of children with HD. PRACTICE IMPLICATIONS: PSE should be considered when designing a parental education program, with the focus on bowel habit training and getting social support.

Bowel management program for pediatric postoperative fecal incontinence in China: A surgeon's experience.

The purpose of this article is to report the status of the efficacy of and long-term adherence to the Bowel Management Program (BMP) for fecal incontinence (FI) postoperation in China.Children over 3 years of age with FI postoperation referred to our medical center were included in the study. Evaluations were performed before and 2 years after their clinic visit. The cost of bowel care, improvement in incontinence, health-related quality of life, and family functioning with the BMP were analyzed.A total of 48 children with FI were included in our study, of whom 38 were boys. The median treatment fee was 660.1 dollars. The complications included abdominal pain (4 patients, 8%), occasional vomiting (2 patients, 4%), and hypoglycemia (1 patient, 2%). The incontinence status and health-related quality of life improved significantly after the BMP. Despite the good outcome of the BMP, half of the patients discontinued the program.The BMP is an effective approach to manage FI and improve the patients' quality of life. Poor long-term adherence is currently the main challenge affecting the BMP application in China.