Diagnosis of liver cirrhosis with two-dimensional shear wave elastography in biliary atresia before Kasai portoenterostomy.

PURPOSE: To evaluate the application value of two-dimensional shear wave elastography (2D-SWE) for non-invasive diagnosis of liver cirrhosis (LC) in patients with biliary atresia (BA) before Kasai portoenterostomy (KP), and the cutoff value of liver stiffness measurement (LSM) for diagnosing LC. METHODS: The clinical data of 51 patients with BA who were diagnosed via surgery and pathological results from May 2017 to December 2018 in the department of general surgery, Beijing Children's Hospital, Capital Medical University, were retrospectively analyzed. The liver tissue specimens obtained during KP were evaluated according to the METAVIR criteria. The LSM was obtained using the 2D-SWE technique before KP. RESULTS: There was a grade positive correlation between LSM and METAVIR staging, and the Spearman correlation coefficient was 0.432 (P = 0.002). The AUC for 2D-SWE diagnosing LC (METAVIR score S = 4) in patients with BA before KP was 0.843 (95% confidence interval 0.736 ~ 0.950). The best cutoff value was 16.05 kPa, and the corresponding sensitivity was 75.0%, specificity was 83.7%, positive predictive value (PPV) was 46.1%, negative predictive value (NPV) was 94.7%, and the accuracy was 82.4%. CONCLUSION: 2D-SWE can be used to noninvasively diagnose LC in patients with BA before KP, and the cutoff value is 16.05 kPa.

Vascular Malformation of the Colorectum in Children: Case Series of 27 Patients.

BACKGROUND: Vascular malformation of the colorectum is a rare disease that presents most commonly in early childhood. Some pull-through procedures have been performed for its treatment. However, laparotomy was routinely required. The aim of this study was to present features of this disease, as well as the outcomes of transanal endorectal pull-through (TEPT) with or without laparotomy. METHODS: A retrospective analysis was performed on consecutive patients with vascular malformation of the colorectum who underwent TEPT with or without laparotomy in our hospital between January 2010 and December 2019. Follow-up information included hematochezia, other bleeding and bowel function. Bowel function was assessed using the Rintala score. RESULTS: Twenty-seven patients were included (14 boys and 13 girls). Hematochezia and anemia were the main chief complaints. Perianal vasodilatation and prolapse of rectal mucosa with spurting blood were detected in 13 (48.1%) and 9 (33.3%), respectively. Eighteen patients (66.7%) underwent TEPT with laparotomy, and 9 patients underwent TEPT without laparotomy (including 5 TEPT under laparoscopic supervision) for lesions located beyond the distal sigmoid colon. Compared to TEPT with laparotomy, the length of resected bowel and operative time was significantly shorter in TEPT without laparotomy (p = 0.001 and p = 0.004). There was no statistical difference for intraoperative blood loss. Other vascular malformations were detected in 9 patients (33.3%); 3 were detected by laparoscopy. Three patients (11.1%) experienced postoperative complications. Follow-up was held with 24 patients (3 were lost to follow-up). The mean follow-up time was 51.9 ± 32.8 months. Four patients occasionally had bloody stools, without anemia. None had hematuria or vaginal bleeding. Median bowel function score was 20 (range 17-20). CONCLUSION: Hematochezia, anemia and hemorrhoid-like manifestations might be an indication for vascular malformation of the colorectum in children. TEPT was effective for vascular malformation of the colorectum, and TEPT without laparotomy was more suitable for the short lesions.

Etiology and surgical management of pediatric acute colon perforation beyond the neonatal stage.

PURPOSE: Acute colon perforation is a pediatric surgical emergency. We aimed to analyze the different etiologies and clinical characteristics of acute non-traumatic colon perforation beyond the neonatal period and to identify surgical management and outcomes. METHODS: This retrospective study included 18 patients admitted with acute colon perforation and who received surgical treatment. RESULTS: Age of patients ranged between 1 month and 15 years. Five patients swallowed foreign objects (two swallowed magnets), two had colon perforation secondary to a malignant tumor (both colorectal adenocarcinoma) and two were iatrogenic (one prior colonoscopy, one air enema for intussusception). There was one perforation due to chemotherapy and Amyand's hernia respectively. The remaining seven patients had unknown etiologies; five of them were diagnosed with colitis. Fifteen (83.3 %) patients underwent open laparotomy, among which four attempted laparoscopy first. Three (16.7 %) patients underwent laparoscopic surgery. Fourteen (77.8 %) patients received simple suture repairs and four (22.2 %) received colonic resections and anastomosis. Four (22.2 %) patients received a protective diverting colostomy and three (16.7 %) received an ileostomy. CONCLUSIONS: There is a wide range of etiology besides necrotizing enterocolitis and trauma, but a significant portion of children present with unknown etiology. Type of surgery elected should be dependent on the patient's etiology, disease severity and experience of surgeons.

Characteristics and treatments for pediatric ordinary and incarcerated inguinal hernia based on gender: 12-year experiences from a single center.

BACKGROUND: Congenital primary inguinal hernia is a common condition among children. Although much literature regarding inguinal hernia is available, large scale analysis are few, and rarely do they expand on gender difference or incarcerated hernias. METHODS: Patients with unilateral or bilateral inguinal hernia who were admitted to our hospital and received open inguinal hernia repair (OIHR) or laparoscopic inguinal hernia repair (LIHR) under general anesthesia were included. LIHR was performed using single-site laparoscopic percutaneous extraperitoneal closure (SLPEC). Medical records were retrospectively collected and analyzed. RESULTS: A total of 12,190 patients were included in this study. The ratio of male to female was 4.8:1. There was a total of 10,646 unilateral hernias (87.3%) and 1544 bilateral hernias (12.7%), with a corresponding ratio of 6.9:1. 12,444 hernia repair surgeries, 11,083 (89.1%) OIHR and 1361 (10.9%) LIHR, were held. OIHR had a shorter operative time than LIHR for all unilateral and female bilateral repair, unlike for bilateral male repair. There was no difference between OIHR and LIHR for ipsilateral recurrent hernia in males. There was a difference between OIHR and LIHR for metachronous contralateral hernia. Incarcerated inguinal hernia was associated with longer operative time, hospital stay and higher hospital costs. Females and patients under 1 year were more likely to present with incarcerated hernia. CONCLUSIONS: OIHR should be considered for male patients, especially for unilateral and complete inguinal hernia. LIHR is highly recommended for female patients. For incarcerated hernia, attention should be paid to patients under 1 year old, as they can be 60 times more susceptible, and females. Surgeons should also be aware of ovary hernias in females.

Barium enema findings in total colonic aganglionosis: a single-center, retrospective study.

BACKGROUND: Preoperative diagnosis of total colonic aganglionosis is important for the rational choice of treatment. The present study aimed to evaluate the diagnostic performance of radiographic signs on preoperative barium enema in patients with total colonic aganglionosis. METHODS: Forty-four patients [41 (3-659) days] with total colonic aganglionosis, including 17 neonatal patients, who received preoperative barium enema at Beijing Children's Hospital, from January 2007 to December 2019 were included. All radiographs were retrospectively restudied by 2 pediatric radiologists to ascertain radiographic signs including rectosigmoid index, transition zone, irregular contraction, gas-filled small bowel, microcolon, question-mark-shaped colon and ileocecal valve reflux. Kappa test was performed to assess the accuracy and consistency of the radiographic signs. RESULTS: The 2 radiologists showed slight agreement for gas-filled small bowel, microcolon and rectosigmoid index, fair agreement for transition zone and irregular contraction, and moderate agreement for question-mark-shaped colon and ileocecal valve reflux (Kappa values, 0.043, 0.075, 0.103, 0.244, 0.397, 0.458 and 0.545, respectively). In neonatal patients, the 2 radiologists showed moderate agreement for ileocecal valve reflux and substantial agreement for question-mark-shaped colon (Kappa values, 0.469 and 0.667, respectively). In non-neonatal patients, the 2 radiologists showed substantial agreement for ileocecal valve reflux (Kappa value, 0.628). In 36 patients with total colonic aganglionosis extending to the ileum, the accuracies of question-mark-shaped colon, ileocecal valve reflux and the combination of both were 47%, 53%, and 75%, respectively, in one radiologist and 53%, 50% and 72%, respectively, in the other radiologist. CONCLUSIONS: Ileocecal valve reflux is a relatively reliable radiographic sign for diagnosing total colonic aganglionosis and could improve the diagnostic accuracy upon combination with question-mark-shaped colon.

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.

Risk factors for therapy failure after incision and drainage alone for perianal abscesses in children.

Background: It is well known that recurrent perianal abscesses (PAs) and fistula-in-ano (FIA) are the main causes of therapy failure following incision and drainage (I&D) for PAs. But few studies have focused on the risk factors for therapy failure after I&D for PAs in children. In this study, we retrospectively examine the risk factors for therapy failure after I&D for PAs in children in a pediatric tertiary care institution. Methods: A retrospective review of all outpatient children with PA treated by I&D at Beijing Children's Hospital between January 2021 and December 2022 was performed. A follow-up was conducted in October 2023. Patients with other predisposing factors for perianal infection, such as inflammatory bowel disease, hematologic tumor, and anorectal surgery, were excluded from this study. Logistic regression yielding odds ratios (ORs) was used to assess the significance of variables for therapy failure. Results: Of 160 children initially identified, follow-up was available for 146, with a total of 172 treatments. A total of 91% of children were male. The median (interquartile range) age at I&D was 2 (1, 15) months. The median follow-up duration was 20 (14, 25) months. Therapy failure occurred in 25 (15%) treatments performed for the prevention of recurrence of PA and in 35 (20%) treatments for the prevention of development of FIA. In the univariate analysis, a history of PA (P = 0.001), history of I&D (P = 0.014), and multilocal occurrence (P = 0.003) were associated with therapy failure. A sitz bath after I&D (P = 0.016) and regular cleaning of the wound after I&D (P = 0.024) were associated with therapy success. In the multivariate analysis, a history of PA (P = 0.015, OR = 3.374) and multilocal occurrence (P = 0.012, OR = 4.649) were independently associated with therapy failure. Regular cleaning of the wound (P = 0.017, OR = 0.341) and sitz bath (P = 0.001, OR = 0.128) after I&D were independently associated with therapy success. Conclusions: A history of PA and multilocal occurrence were predictor factors for therapy failure before I&D. Regular cleaning of the wound and sitz bath after I&D were protective factors for therapy success. Therefore, regular cleaning of the wound and sitz bath after I&D should be emphasized in all children with PAs, especially in those with a history of PA and multilocal occurrence.

Acquired Rectourethral and Rectovaginal Fistulas in Children: A Systematic Review.

Background: Acquired rectourethral (RUF) or rectovaginal fistulas (RVF) in children are rare conditions in pediatric surgery. Prior literature are retrospective studies and based on a small number of patients. The managements and outcomes vary widely across different studies. No standard or recommended management has been universally adopted. The goal was to systematically summarize different causes, provide an overlook of current clinical trend and to derive recommendation from the literature regarding the etiology, managements, and outcomes of pediatric acquired RUF and RVF. Methods: PubMed, Embase, Cochrane databases were searched using terms: rectourethral fistula, recto-urethral fistula, urethrorectal fistula, urethro-rectal fistula, rectovaginal fistula. All studies were retrospective, in English, and included patients under the age of 18 years. Any series with congenital cases, adult (>18 years), <2 fistula cases less and obstetric related causes were excluded. The Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guideline was followed. Results: Of the 531 records identified, 26 articles with 163 patients (63 RUF and 100RVF) were fully analyzed. Most RUF resulted from trauma, most RVF were from infection of HIV. About 92 patients underwent 1 of 3 categories of definitive repair, including transanal (4.3%), trans-sphincteric (48.9%), and transperineal (30.4%). Tissue interposition flaps were used in 37.6% patients, while temporary fecal diversions were used in 63.9% patients. Fistula was successfully closed in 50.3% patients (98.4% RUF and 20% RVF). 89.1 and 79.7 % of surgical repair patients had optimal fecal and urinary functions, respectively. In the inflammatory bowel disease and HIV infection related RVF patient group, the closure rate was prohibitive poor. Conclusions: Most RVF are a sign of systematic diseases like HIV-infection or IBD and are associated with poor general conditions. While conservative treatment is recommended, stable patients can benefit from surgery. Further investigation is recommended if RVF are encountered without trauma or surgical history. RUF are likely to result from trauma or surgery, and transperineal or trans-sphincter approach can lead to closure and optimal function results. Fecal diversion and/or urinary diversion are helpful in some cases, while interposition technique may not be necessary. An objective scoring system for long-term follow-up and reporting consensus is needed to address treatment inconsistence.

Rectourethral and rectovesical fistula as serious and rare complications after Hirschsprung disease operation: Experience in seven patients.

AIM: To present the features and treatment of rectourethral fistula (RUF) and rectovesical fistula (RVF) after Hirschsprung disease (HD) operation. METHODS: A retrospective analysis was performed on patients with RUF and RVF after HD operation, who received repair surgery from a single surgeon between January 2005 and December 2019. Bowel function was assessed using the Rintala score. RESULTS: Seven patients were included. Six patients were referred to us after transanal endorectal pull-through (TEPT) in other centers; one RVF patient had fecal diversion at admission. Bladder-neck injury was detected during redo TEPT in our hospital in the remaining one patient and instant repair was given. 11 days later, RVF and sepsis were detected. Fecal and urine diversion was performed immediately. The fistula openings were prostatic urethra (3), membranous urethra (2), bladder triangle (1), and bladder-neck (1). Anastomotic stricture (4), bladder stone (3), hydronephrosis and ureterovesical junction obstruction (2), pelvic infection (2), distal colonic dilatation (1) and other fistulas (1) were identified before repair surgery. Fecal and urine diversion was performed before repair surgery in one RUF and one RVF patient respectively, to treat pelvic infection owing to fecal or urine leaks and accompanying problems. Five RUFs were repaired by transperineal approach, and two RVFs were repaired using the transabdominal and transanal approach respectively. Four patients with anastomotic stricture underwent redo TEPT with simultaneous fistula repair. Aside from one RUF patient, the fistula in all patients successfully resolved. Median follow up time was 81 months (range, 5-116 months). No recurrence was observed. Median bowel function score was 17.5 (range, 17-18). CONCLUSION: The location of fistula, presence of anastomotic stricture and the association of urinary complications have significant impacts on the treatment strategy in such rare complications after TEPT for HD. THE TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: Level IV.