Clinical features of intussusception in children secondary to small bowel tumours: a retrospective study of 31 cases.

BACKGROUND: Summarizing the clinical features of children with intussusception secondary to small bowel tumours and enhancing awareness of the disease. METHODS: Retrospective summary of children with intussusception admitted to our emergency department from January 2016 to January 2022, who underwent surgery and were diagnosed with small bowel tumours. Summarize the types of tumours, clinical presentation, treatment, and prognosis. RESULTS: Thirty-one patients were included in our study, 24 males and 7 females, with an age of onset ranging from 1 m to 11y 5 m. Post-operative pathology revealed 4 types of small intestinal tumour, 17 lymphomas, 10 adenomas, 4 inflammatory myofibroblastomas and 1 lipoma. The majority of tumours in the small bowel occur in the ileum (83.9%, 26/31). Abdominal pain, vomiting and bloody stools were the most common clinical signs. Operative findings indicated that the small bowel (54.8%, 17/31) and ileocolic gut were the main sites of intussusception. Two types of procedure were applied: segmental bowel resection (28 cases) and wedge resection of mass in bowel wall (3 cases). All patients recovered well postoperatively, with no surgical complications observed. However, the primary diseases leading to intussusception showed slight differences in long-term prognosis due to variations in tumor types. CONCLUSIONS: Lymphoma is the most common cause of intussusception in pediatric patients with small bowel tumours, followed by adenoma. Small bowel tumours in children tend to occur in the ileum. Therefore, the treatment of SBT patients not only requires surgeons to address symptoms through surgery and obtain tissue samples but also relies heavily on the expertise of pathologists for accurate diagnosis. This has a significant impact on the overall prognosis of these patients.

Adenomyoma of the small intestine in children: a 12-year experience in a tertiary referral center.

BACKGROUND: Adenomyoma of the small intestine is rare in children and the clinical characteristics is not clear. The study was to document the clinical characteristics and treatment of children with adenomyoma of the small intestine. METHODS: A retrospective study was conducted in children with intestinal adenomyoma from 2010 to 2022. We recorded age, gender, symptoms, location, tumour size and treatment options. RESULTS: Thirteen patients with adenomyoma of the small bowel were included. The median age was 20 months with a male-to-female ratio of 10:3 and more than half of the patients were younger than 2 years old. The mean tumour size was 2.0 cm. The lesion was found accidentally in one patient, and the others presented with symptoms of intussusception. A pathological lead point was found on ultrasound in seven patients. All tumours were located in the ileum, ranging from 24 to 260 cm proximal to the ileocecal valve. The tumour was found in an antimesenteric site in eight patients. Three patients suffered intestinal necrosis, and segmental resection of the ileum was performed. Three patients without intestinal necrosis underwent tumour rection, while intestinal resection and anastomosis were performed in the remaining seven. All patients recovered well except one, who developed intussusception 7 days after surgery; that patient underwent surgery and recovered uneventfully. CONCLUSIONS: Adenomyoma of the small intestine has a male predominance in children and intussusception is a common presentation. The ultrasound feature is a mass of mixed echogenicity containing several small cystic areas. Surgery is the primary treatment option and the procedure should be chosen based on intraoperative findings.

Clinical Characteristics of Pediatric Patients With Septic Shock Caused by Acute Appendicitis: A Case Series.

OBJECTIVES: This study aimed to explore the clinical characteristics of septic shock in pediatric patients caused by acute appendicitis. METHODS: This case series included patients with septic shock caused by acute appendicitis in Beijing Children's Hospital between January 2015 and December 2020. RESULTS: Six patients with septic shock caused by acute appendicitis were enrolled. One patient was an infant with extremely low weight; 2 patients were obese. The diagnosis was delayed in 4 patients (the time from onset to diagnosis was 5 days in 3 children and 4 days in 1 child). All patients had abnormally raised inflammatory markers (C-reactive protein 119.17 ± 48.36 mg/L, procalcitonin 129.95 ± 86.09 ng/mL). Severe abdominal infection was found in all patients. There was appendix perforation in 4 patients and diffused peritonitis in 3 patients. Two patients had metabolic diseases (Wilson disease and decreased biotinase activity, respectively). Five patients had an appendectomy and 1 patient received conservative treatment. Five patients were discharged in stable condition, while 1 patient died. CONCLUSIONS: Children with delayed diagnosis, abnormal body weight, significant elevation in inflammatory markers, and underlying metabolic disease may be at greater risk of complicated appendicitis and septic shock. EVIDENCEBASED MEDICINE: Level of Evidence: IV.

Experience in diagnosis and treatment of duodenal ulcer perforation in children.

BACKGROUND: This study aims to summarize our experience in diagnosis and treatment of pediatric duodenal ulcer perforation in a National Center for Children's Health. METHODS: Fifty-two children with duodenal perforation hospitalized in Beijing Children's Hospital Affiliated to Capital Medical University from January 2007 to December 2021 were retrospectively collected. According to the inclusion and exclusion criteria, patients with duodenal ulcer perforation were included in the group. They were divided into the surgery group and the conservative group according to whether they received surgery. RESULTS: A total of 45 cases (35 males and 10 females) were included, with a median age of 13.0 (0.3-15.4) years. Forty cases (40/45, 88.9%) were over 6 years old, and 31 (31/45, 68.9%) were over 12 years old. Among the 45 cases, 32 cases (32/45, 71.1%) were examined for Helicobacter pylori (HP), and 25 (25/32, 78.1%) were positive. There were 13 cases in the surgery group and 32 cases in the conservative group, without a significant difference in age between the two groups (P = 0.625). All cases in the surgery group and the conservative group started with abdominal pain. The proportion of history time within 24 h in the two groups was 6/13 and 12/32 (P = 0.739), and the proportion of fever was 11/13 and 21/32 (P = 0.362). The proportion of pneumoperitoneum in the surgery group was higher than that in the conservative group (12/13 vs. 15/32, P = 0.013). The fasting days in the surgery group were shorter than those in the conservative group (7.7 ± 2.92 vs. 10.3 ± 2.78 days, P = 0.014). There was no significant difference in the total hospital stay (13.6 ± 5.60 vs14.8 ± 4.60 days, P = 0.531). The operation methods used in the surgery group were all simple sutures through laparotomy (9 cases) or laparoscopy (4 cases). All patients recovered smoothly after surgery. CONCLUSION: Duodenal ulcer perforation in children is more common in adolescents, and HP infection is the main cause. Conservative treatment is safe and feasible, but the fasting time is longer than the surgery group. A simple suture is the main management for the surgery group.

Colocolic Intussusception in Children: A Case Series and Review of the Literature.

Background: Colocolic intussusception is a rare subtype of intussusception mostly caused by juvenile polyps. The treatment of colocolic intussusception caused by other pathologic lead points in children remains poorly understood. Method: A systematic literature review was performed between January 2000 and June 2021 to characterize the comprehensive treatment of colocolic intussusception in children. This report also included patients admitted to our center between January 2010 and June 2021 who were not previously reported in the literature. Results: We identified 27 patients in 20 studies in addition to 17 patients from our center for a total of 44 patients (median age, 4.4 years; 52.3% male). The lead point was identified in 40 patients (40/44, 90.9%). The most common lead point was juvenile polyps (19/44, 43.2%). A therapeutic enema was performed in 15 patients with colocolic intussusception caused by juvenile polyps and was successful in 9 patients (9/15, 60.0%). Colonoscopic polypectomy was subsequently performed in 8 patients and was successful in 7 patients (7/8, 87.5%). The other patient had undergone laparoscopic exploration, and no abnormality was found. Subsequently, the patient underwent open surgery. The patients with colocolic intussusception caused by other pathologic lead points almost underwent surgical treatment (15/21, 71.4%), including 13 open surgeries and 2 laparoscopic surgeries. Conclusion: A therapeutic enema followed by colonoscopic polypectomy is feasible to treat colocolic intussusception caused by juvenile polyps unless the patient has bowel perforation; however, surgery is sometimes needed. For patients with colocolic intussusception caused by other pathologic lead points, open surgery remains the primary treatment.

Combination of gamma-glutamyl transferase and liver stiffness measurement for biliary atresia screening at different ages: a retrospective analysis of 282 infants.

BACKGROUND: This study aims to explore the diagnostic accuracy of the combination of gamma-glutamyl transferase (GGT) and liver stiffness measurement (LSM) for biliary atresia (BA) screening at different ages. METHODS: Our retrospective study involved 282 infants under the age of 120 days with jaundice who were admitted into Beijing Children's Hospital between January 2016 to December 2018. The GGT and LSM levels of infants were obtained. A parallel test was used, and ROC curve was created to obtain cutoff values of GGT and LSM for BA infants at different ages. RESULTS: Of the 282 infants, 135 were diagnosed with BA and 147 were non-BA infants. In all age groups (A: ≤60 days; B: 61-90 days; C: 91-120 days), the LSM and GGT levels of the BA group were significantly higher than that of the non-BA group, P < 0.05. The cutoff value of GGT and LSM to diagnosis BA was 191.2 U/L, 213.2 U/L, 281.5 U/L and 7.5 kPa, 10.0 kPa, 11.0 kPa in groups A, B and C, respectively. The parallel test was used to determine a sensitivity of 97.3, 98.1 and 100% in group A, B and C when either GGT or LSM levels were met in BA infants. The sensitivities of parallel testing for group A and B were higher than LSM or GGT used alone. CONCLUSIONS: Cutoff values of GGT and LSM to screen BA increased with age. Parallel testing of GGT and LSM in infants who are younger than 90 days old can decrease the rate of BA misdiagnosis.

Liver stiffness measurement predicts the difficulty of Kasai procedure in biliary atresia: a single center retrospective analysis of 199 patients.

BACKGROUND: Kasai procedure is the standard initial treatment of infants with biliary atresia. The key to perform a successful surgery is to accurately remove the fibrous portal plate near the liver hilum. Yet how to estimate surgical difficulty pre-operatively remains unclear. This study aims to design an algorithm that predicts the difficulty of Kasai procedure using liver stiffness measurement (LSM). METHODS: One hundred ninety-nine patients were included from April 2012 to December 2016. The patients were all surgically diagnosed with biliary atresia. Group A comprised of patients with porta hepatis retraction (the angle between the plane of the fibrous porta plate and the plane of the medial liver closest to the plate was equal to or smaller than 90°), group B comprised of patients without porta hepatis retraction (the angle between the plane of the fibrous porta plate and the plane of the medial liver closest to the plate was greater than 90°). Liver function measurements and LSM were measured for all patients within three days before surgery. RESULTS: Our study included 19 cases in group A (9 males, 10 females) and 180 cases in group B (87 males, 93 females). LSM had statistical differences between the two groups, 28.10(14.90) kPa VS 10.89(7.10) kPa, P = 0.000. There was a significant relationship between LSM and operative age, TBA, AST, GGT (P = 0.000, 0.003, 0.003, 0.012, correlation coefficient = 0.323, 0.213, 0.207, 0.179). The AUROC of LSM was 0.919. When the cutoff value was 15.15 kPa(OR = 3.989; P = 0.000), the sensitivity, specificity, PPV, NPV and diagnostic accuracy were 0.947, 0.750, 0.285, 0.992 and 0.768, respectively. When the value was 23.75 kPa(OR = 3.483; P = 0.000), the sensitivity, specificity, PPV, NPV and diagnostic accuracy were 0.631, 0.950, 0.571, 0.960 and 0.919, respectively. CONCLUSIONS: LSM can be used to predict the difficulty in dissecting fibrous portal plate, and in turn, the difficulty of Kasai procedure. LSM > 23.75 kPa suggests a more complicated surgery.

Five-year native liver survival analysis in biliary atresia from a single large Chinese center: The death/liver transplantation hazard change and the importance of rapid early clearance of jaundice.

PURPOSE: This study aimed to analyze the change of death/liver transplantation hazard and biochemical indexes over time after Kasai procedure (KP) based on a retrospective biliary atresia (BA) cohort, and to evaluate the predictive value of early jaundice clearance rate to 5-year native liver survival (NLS). METHODS: A retrospective cohort with follow-up results of 139 BA patients from January 2009 to December 2012 was established, and the pre- and postoperative data were collected. NLS rates were estimated with Kaplan-Meier curves, and any differences between groups were tested by log-rank test. Hazard curve of death/liver transplantation was fitted with Weibull distribution, and hazards at certain time points were calculated. Trend charts of biochemical indexes were drawn to show any changes over time. Rate of jaundice clearance was indicated as the proportion of decreased total bilirubin level at a certain postoperative time point to preoperative total bilirubin level. In multivariate analysis for prediction of 5-year NLS, COX proportional hazard regression model was used and results were expressed as hazard ratios with 95% confidence intervals (CIs). The predictive value of early jaundice clearance rates for 5-year NLS was analyzed by receiver operating characteristic (ROC) curve, and a cut-off value of 4-week jaundice clearance rate was determined. RESULTS: The estimated 5-year NLS rate of the 139 patients was 58.0%. The patients had a high hazard of death/liver transplantation early after KP, which gradually decreased and stabilized at a lower level 1 year later. Most death/liver transplantation events occurred within 1 year after KP. The total bilirubin (TBIL), direct bilirubin (DBIL) and total bile acid (TBA) levels of successful Kasai group decreased continuously after KP, and the biggest decline was seen in the first month. The aspartate aminotransferase (AST), alanine aminotransferase (ALT) and γ-glutamyl transpeptidase (GGT) levels increased during the first month after KP and decreased continuously thereafter. All the biochemical indexes of successful Kasai group tended to stabilize within/close to normal range 1 year after KP. On the contrary, all the biochemical indexes of failed Kasai group fluctuated at obvious abnormal levels after KP. The estimated 5-year NLS rates of successful Kasai group and failed Kasai group were 90.1% and 10.7% (p = 0.000). The most significant clinical protective factor of 5-year NLS was 4-week jaundice clearance rate, revealed by COX proportional hazard regression model, and the HR was 0.089 (95%CI 0.018-0.432, p = 0.003). In predicting 5-year native liver survival, the largest area under ROC (AUROC) curve belonged to 4-week jaundice clearance rate, which was 0.731 (p = 0.000). A cut-off value of 0.457 was determined, with sensitivity 0.827, specificity 0.552, positive predictive value 0.720, and negative predictive value 0.696. NLS rates of patients divided by cut-off value showed significant statistical difference demonstrated by Kaplan-Meier curve and log-rank test (p = 0.000). CONCLUSIONS: The 5-year death/liver transplantation hazard of biliary atresia patients reduces greatly and stabilizes 1 year after KP. A successful KP enables patients to achieve long-term stable normal biochemical indexes. A rapid clearance of jaundice is of great positive significance to 5-year NLS, and the 4-week jaundice clearance rate is of some predictive value. TYPE OF STUDY: Prognosis study. LEVEL OF EVIDENCE: Level II, retrospective study.

Assessment of liver fibrosis by Fibroscan as compared to liver biopsy in biliary atresia.

AIM: To evaluate liver stiffness measurement (LSM) using non-invasive transient elastography (Fibroscan) in comparison with liver biopsy for assessment of liver fibrosis in children with biliary atresia (BA). METHODS: Thirty-one children with BA admitted to the Department of Pediatric Surgery of Beijing Children's Hospital from March 2012 to February 2013 were included in this study. Their preoperative LSM, liver biopsy findings, and laboratory results were studied retrospectively. RESULTS: The grade of liver fibrosis in all 31 patients was evaluated according to the METAVIR scoring system, which showed that 4 cases were in group F2, 20 in group F3 and 7 in group F4. There were 24 non-cirrhosis cases (F2-F3) and 7 cirrhosis cases (F4). In groups F2, F3 and F4, the mean LSM was 9.10 ± 3.30 kPa, 11.02 ± 3.31 kPa and 22.86 ± 12.43 kPa, respectively. LSM was statistically different between groups F2 and F4 (P = 0.002), and between groups F3 and F4 (P = 0.000), however, there was no statistical difference between groups F2 and F3 (P = 0.593). The area under the receiver operating characteristic curve of LSM for ≥ F4 was 0.866. The cut-off value of LSM was 15.15 kPa for ≥ F4, with a sensitivity, specificity, positive predictive value and negative predictive value of 0.857, 0.917, 0.750 and 0.957, respectively. CONCLUSION: Fibroscan can be used as a non-invasive technique to assess liver fibrosis in children with BA. The cut-off value of LSM (15.15 kPa) can distinguish cirrhotic patients from non-cirrhotic patients.

Anterior perineal fistulectomy in repairing H-type rectovestibular fistula with a normal anus in female children.

The purpose of this study was to review a 7-year operative experience of anterior perineal fistulectomy in repairing H-type rectovestibular fistula with a normal anus in female children. From January 2007 to December 2013, 105 female children with H-type rectovestibular fistula and a normal anus underwent anterior perineal fistulectomy in the Department of General Surgery, Beijing Children's Hospital, Capital Medical University. Clinical data were retrospectively analyzed. Patients' age ranged from 6 months to 13 years 10 months, with an average age 5 years 2 months. The clinical feature of this disease was a definite history of vestibular infection at about 30 days after birth and leakage of gas or loose stool through vestibular fistular orifice after the infection was controlled. All patients had a normal anus. Follow-up through telephone or outpatient visits from 6 months to 7 years (median, 5.2 years), showed that all the patients were continent and had regular bowel movements. Among the 105 patients, 97 (92.4%) gained primary healing with a satisfactory appearance; 8 (7.6%) patients relapsed, among which 5 healed after hip bathing treatment within 3 weeks, 2 required another anterior perineal fistulectomy 7 months later and were cured, and the last one had another anterior perineal fistulectomy 1 year 10 months later but relapsed again, which healed after hip bathing treatment within 3weeks after the second surgery. Anterior perineal fistulectomy is a simple, safe and reliable surgical method to treat H-type rectovestibular fistula with a normal anus.