INTRODUCTION:Cloacal malformations are a spectrum of complex congenital anomalies traditionally classified by common channel length, urethral length, Müllerian configuration, and sacral or spinal abnormalities. Classification guides reconstructive strategy and informs prognosis. However, a subset of patients present with atypical anatomy that defies standard classification systems, complicating surgical planning and prediction of outcomes. AIM:Our cloacal malformation multidisciplinary service utilises a pathway based on common channel length, Mullerian anomalies, urethral length and sacral spinal abnormalities. We aimed to assess the management and faecal continence outcomes of these unclassifiable cases. METHODS:We conducted a retrospective review of 82 patients referred for primary reconstruction from 2010, analysing categorical and numerical data using Fisher's exact and Mann-Whitney U tests. Patients were categorised as classic cloaca, posterior cloaca, and unclassifiable (exhibited complex/atypical anatomy such as indistinct of absent common channels, duplication of urogenital structures or associated covered cloacal exstrophy variants). RESULTS:Of 82 patients, 15 were unclassifiable. The latter group had more co-morbidities: 75 % (n = 6) with spinal anomalies requiring neurosurgery, and 93 % (n = 14) with renal anomalies. They were more likely to undergo laparotomy (n = 10) and had poorer faecal continence outcomes, with bowel function score of 6, compared to 13 in the classic cloaca group. Surgical management was more complex, with all patients requiring functional diversion procedures rather than standard anatomical reconstruction, representing a form of "non-anatomical reconstruction". CONCLUSION:Unclassified cloacal malformation group represent a significant challenge, with poorer functional outcomes likely due to greater anatomical complexity and coexisting anomalies. Successful management requires experience in cloacal reconstruction and operative planning tailored to each patient's unique anatomy.

BACKGROUND:There has been an increasing number of single-centre studies describing the long-term outcomes of patients with Hirschsprung disease. This study aimed to systematically review the literature on long-term bowel, urological, and sexual functional outcomes, fertility and quality of life in adults with Hirschsprung disease. METHODS:A PROSPERO-registered systematic review of the English literature was conducted for studies published up to July 2025 that reported functional outcomes beyond childhood (≥16 years) for patients who had undergone surgery for Hirschsprung disease. Centres were contacted individually for secondary analyses of patient-level data on bowel function score, Gastrointestinal Quality of Life Index, and Short Form 36 questionnaire. Data were analysed and compared with those from healthy controls in the studies retrieved and from a reference healthy population. Hirschsprung disease clinical and surgical variables were correlated with these outcomes of interest in a patient-level analysis. RESULTS:Fifty-three manuscripts fulfilled the inclusion criteria of 4277 papers retrieved. Patients with Hirschsprung disease had a greater likelihood of constipation (odds ratio 9.27, 95% confidence interval (c.i.) 4.78 to 18.06) and soiling (odds ratio 2.76, 1.96 to 3.89) compared with healthy controls. They scored lower on the Gastrointestinal Quality of Life Index (mean difference -5.21, 95% c.i. -9.53 to -0.89; P = 0.020). There were no significant differences in Short Form 36 domain scores except for physical functioning (mean difference -6.30, -8.74 to -3.87; P < 0.001). At a patient level, longer-segment disease (P < 0.001) and redo pull-through surgery (P = 0.002) were associated with a poorer bowel function score. Short form 36 scores were lower in women across six of eight domains; similarly, Gastrointestinal Quality of Life Index scores were lower in women (P < 0.001) and in patients with longer-segment disease (P < 0.001). CONCLUSION:Among patients with Hirschsprung disease, women, those with longer-segment disease, and patients who underwent redo surgery may be at risk of poorer quality of life.

Introduction Hepatobiliary surgery can be challenging due to the complex nature of variations in liver anatomy, particularly in small children. Congenital portosystemic shunts (CPS – Abernethy syndrome) are increasingly recognized with a range of clinical presentations and associated complications and can present anatomical challenges for operative shunt closure. Three-dimensional printing (3DP) has gained popularity due to its ability to produce accurate models with the specific anatomical characteristics for the individual patient. We used a 3DP model from original image sources for planning of surgical shunt closure in a child with CPS and for educating the family and surgical team preoperatively. Case presentation A 5-year-old male with autism and hyperammonemia of 80 μmol/L and was found to have an intrahepatic CPS. No tumors were seen within the liver. Physical examination was unremarkable. After treatment with Rifaximin to lower blood ammonia levels, his ability to concentrate and achieve milestones improved. Angiography with shunt occlusion showed an intrahepatic shunt between the left portal and the middle hepatic vein into the IVC. Intrahepatic portal branches were demonstrated with the implication that a one stage closure could be performed. A 3DP model was printed from the cross sectional imaging which allowed for more detailed planning of shunt ligation and to explain the surgical details to the parents. Surgical closure was performed with no complications. Conclusion 3DP models can be a useful tool for planning complex congenital porto-systemic shunt closure surgery.

Introduction: Surgical access to anorectal malformations (ARM) can be difficult from abdominal, perineal and transanal approaches. We present a case in which magnetic compression anastomosis was successfully employed to achieve a minimally invasive repair of high rectal atresia in a neonate. Case presentation: A female neonate born at 38 weeks and 5 days with no significant antenatal history and failure to pass meconium in the first 48 hours after birth developed abdominal distension and bilious vomiting on the third day of life. The patient was found on clinical examination and contrast enema to have rectal atresia just below the peritoneal reflection. A diverting colostomy was fashioned on day four of life. A distal colostogram at two months of age confirmed the diagnosis of high rectal atresia with minimal separation between the atretic ends. Magnetic compression anastomosis by insertion of a pair of magnets through the anus and mucous fistula was attempted to obviate the need for invasive surgery. Following a failed first attempt at the age of 11 months, a second attempt performed at the age of 15 months under endoscopic and fluoroscopic guidance successfully established luminal continuity. The colostomy was closed at the age of 16 months following a single topical application of mitomycin C. The patient recovered well and has excellent anorectal function three years postoperatively. Conclusion: Selected cases of rectal atresia may be treated by magnamosis. Fluoroscopic and endoscopic guidance may be required to ensure appropriate positioning of the magnets.

Introduction:Patients born with congenital porto-systemic shunts have been shown to have a high risk of benign and malignant liver tumors in otherwise healthy livers. This study aimed to evaluate the genetic landscape of liver tumors in patients with congenital porto-systemic shunts (CPSS) and correlate genotype with histological findings. Methods:Nodules from patients with CPSS and sporadic pediatric focal nodular hyperplasia (FNH) or FNH-like nodules were evaluated histologically and sequenced for a panel of 50 genes using next-generation sequencing. Results:Thirty-eight nodules from 17 patients with CPSS were histologically classified as hepatoblastomas (n = 2), hepatocellular carcinomas (n = 4), HNF-1α-inactivated hepatocellular adenomas (HCAs) (n = 2), β-catenin-activated HCAs (n = 5), unclassified HCAs (n = 9), and FNH-like nodules (n = 16). CTNNB1 variants were detected in 26/38 nodules (68%) across different histological categories (2/2 hepatoblastomas, 4/4 HCCs, 10/16 HCAs, 10/16 FNH-like nodules), but not in sporadic FNH or FNH-like nodules (0/10). Less frequent variants were identified in APOB, GNAS, HNF1A, SERPINA1, MAML2, PTCH1, G6PC, KMT2C, DICER1, AXIN1, IL6ST and the promoter region of TERT. Germline variants were identified in AXIN1, HFE, SERPINA1, and ZNF521. CTNNB1 variants affecting amino acid positions 32 and 33 are more common in malignant tumors. Multiple CTNNB1 variants were identified in 6/7 (86%) of patients with multiple nodules, but no intratumoral variation was found. Discussion:CPSS is strongly associated with nodules containing variants in CTNNB1, irrespective of the histological category. Areas in background liver containing these variants were also identified, and different variants could be identified in individual patients. The high proportion of CTNNB1 variants may explain the higher malignant potential of benign tumors found in CPSS.

BackgroundThis study describes the presentation and initial management of anorectal malformation (ARM); evaluating the frequency, causes and consequences of late diagnosis.MethodsA prospective, population cohort study was undertaken for newly diagnosed ARMs in the UK and Ireland from 01/10/2015 and 30/09/2016. Follow-up was completed at one year.Data are presented as n(%), appropriate statistical methods used. Factors associated with late diagnosis; defined as: detection of ARM either following discharge or more than 72 hours after birth were assessed with univariable logistic regression.ResultsTwenty six centres reported on 174 cases, 158 of which were classified according to the type of malformation and 154 had completed surgical data. Overall, perineal fistula was the most commonly detected anomaly 43/158 (27%); of the 41 of these children undergoing surgery, 15 (37%) had a stoma formed. 21/154 (14%, CI95{9-20}) patients undergoing surgery experienced post-operative complications. Thirty-nine (22%) were diagnosed late and 12 (7%) were detected >30 days after birth. Factors associated with late diagnosis included female sex (OR 2.06; 1.0-4.26), having a visible perineal opening (OR 2.63; 1.21-5.67) and anomalies leading to visible meconium on the perineum (OR 18.74; 2.47-141.73).56/174 (32%) had a diagnosis of VACTERL association (vertebral, anorectal, cardiac, tracheal, oesophageal, renal and limb); however, not all infants were investigated for commonly associated anomalies. 51/140 (36%) had a cardiac anomaly detected on echocardiogram.ConclusionThere is room for improvement within the care for infants born with ARM in the UK and Ireland. Upskilling those performing neonatal examination to allow timely diagnosis, instruction of universal screening for associated anomalies and further analysis of the factors leading to clinically unnecessary stoma formation are warranted.Level of EvidenceII (Prospective Cohort Study <80% follow-up)

Purpose To identify markers of previous ovarian torsion and outline the outcomes according to US appearance and operative management. Methods A retrospective single-centre review of neonatal ovarian cysts from January 2000 to January 2020. Data on postnatal cyst size and sonographic features and operative treatment were co-related with outcomes of ovarian loss and histology. Results 77 females were included with 22 simple and 56 complex cysts, one patient had bilateral cysts. 9/22 (41%) simple cysts regressed spontaneously in a median of 13 weeks (8–17). Complex cysts regressed spontaneously less frequently, 7/56(12%, P = 0.01), in 13 weeks (7–39). 38/56 (68%) complex and 12/22 (55%) simple cysts were treated operatively. 21/22 (95%) ovaries with initially simple cyst were salvaged compared to 20/56(36%) with initially complex cyst ( P < 0.001). A fluid-debris level in 23/26 complex cysts was most associated with ovarian loss ( P = 0.0006). Presence of viable ovarian stromal tissue was seen in 8/20 (40%) excised specimens during ovarian sparing procedures and in 5/30 (17%) oophorectomies for necrotic appearing ovaries. Conclusions Fluid-debris level on US is significantly associated with ovarian loss likely due to previous torsion. Simple cysts are viable and often regress spontaneously. The finding of viable ovarian stromal tissue in resected specimens supports attempting ovarian preservation wherever possible.

Objective Our systematic review aims to compare recurrence rates and complications of biological versus synthetic patches for the repair of congenital diaphragmatic herniae. Methods Studies from January 1, 1980 to April 25, 2020, with patients under the age of 16 years and with a minimum 6-month follow-up, were included from MEDLINE, Embase, and Cochrane databases. Funnel plots for recurrence rates were constructed for biological and synthetic patches. Subgroup analysis was performed for recurrence rate at the 1-year time-point and data were gathered on individual adverse events from relevant studies. Results A total of 47 studies with 986 patients (226 biological, 760 synthetic) were included. Funnel plot analysis determined overall recurrence rates of 16.7% for synthetic and 30.3% for biological patches. Subgroup analysis of 493 and 146 patients with synthetic and biological patches, respectively, showed recurrence rates of 9.9 and 26%, respectively. The most commonly used patch types—PTFE (polytetrafluoroethylene) and SIS (small intestinal submucosa)—had 11.5 and 33.3% recurrence, respectively. Adhesive bowel obstruction rates ranged from 4 to 29% in studies that systematically reported it for synthetic, and 7 to 35% for biological patches. Gastroesophageal reflux rates ranged from 25 to 48% in studies that systematically reported it for synthetic, and 21 to 42% for biological patches. Pectus deformity rates were reported as high as 80% for synthetic patches. Conclusion Biological patches appear to have higher recurrence rates than synthetic patches, while skeletal deformities are associated more commonly with synthetic patches. Results of biological patches are mainly using SIS and this may overestimate complications of current superior biological patches.

Biliary atresia (BA) is a chronic neonatal cholangiopathy characterized by fibroinflammatory bile duct damage. Reliable biomarkers for predicting native liver survival (NLS) following portoenterostomy (PE) surgery are lacking. Herein we explore the utility of 22 preidentified profibrotic molecules closely connected to ductular reaction (DR) and prevailing after successful PE (SPE), in predicting PE outcomes and liver injury. We used qPCR and immunohistochemistry in a BA cohort including liver samples obtained at PE (n = 53) and during postoperative follow-up after SPE (n = 25). Of the 13 genes over-expressed in relation to cholestatic age-matched controls at PE, only secretin receptor (SCTR) expression predicted cumulative 5-year NLS and clearance of jaundice. Patients in the highest SCTR expression tertile showed 34–55% lower NLS than other groups at 1–5 years after PE (P = 0.006–0.04 for each year). SCTR expression was also significantly lower [42 (24–63) vs 75 (39–107) fold, P = 0.015] among those who normalized their serum bilirubin after PE. Liver SCTR expression localized in cholangiocytes and correlated positively with liver fibrosis, DR, and transcriptional markers of fibrosis (ACTA2) and cholangiocytes (KRT7, KRT19) both at PE and after SPE. SCTR is a promising prognostic marker for PE outcomes and associates with liver injury in BA.

A choledochocele is a rare congenital abnormality of the extrahepatic bile duct characterised by localised dilatation of the distal common bile duct within the wall of the duodenum. We present two children with a long history of undiagnosed abdominal pain and subtle signs of cholestasis. Specific investigations included magnetic resonance cholangiopancreatography (MRCP) that led to a possible diagnosis of choledochocele, and then confirmatory cholangiograms (ERCP and open). Both children required intervention to unroof the dilatation allowing improved pancreato-biliary drainage and complete resolution of symptoms.Elephants have no gallbladder, instead they have a localised dilatation of the distal common bile duct within the wall of the duodenum – analogous to the situation described here.

Congenital portosystemic shunts (CPSS) are associated with multisystem complications, with the most common being liver tumors. The purpose of this study is to investigate the incidence of complications of CPSS, and to determine the natural history of liver tumors and their relationship with shunt closure. Single-center retrospective cohort study of patients with CPSS referred from 1990 to 2020. Data on demographics, laboratory, radiological and histological investigations, clinical evolution, and surgery were reviewed. Mann–Whitney for continuous data and Fisher’s exact test for categorical data were used. A p value of 0.05 was considered significant. 54 patients were investigated for CPSS with a median age of 1.1 years (IQR 0.2–11.8 years) at presentation—7 intrahepatic shunts resolved spontaneously and were excluded. Type 1 (without intrahepatic portal flow) had a higher rate of all hepatic tumors than Type 2 (partial intrahepatic portal flow) [18/22(82%) vs. 9/25(36%); p = 0.003); and malignant tumors (6/22(27%) vs 1/25(4%); (p = 0.04). Following shunt closure, 4/11(36%) of patients experienced partial and 3/11(27%) complete tumor regression. Pulmonary hypertension and hepatopulmonary syndrome affected 4(9%), and 3(6%) patients, respectively. Pulmonary complications affected 1 patient with Type 1 and 6 with Type 2 shunts (p = 0.1). Neurocognitive anomalies were identified in 16/47(34%) patients, 8/22(35%) with Type 1 shunts and 8/25(32%) with Type 2 shunts (p = 0.76). 9/47 (19%) required special needs schooling. Severity of portal venous deprivation (Type 1 CPSS) increases the risk of hepatic tumors and surgical closure is associated with a reduction in size or complete resolution of benign tumors.

Interleukin (IL)-8 (CXCL8), a chemokine involved in neutrophil recruitment, has been implicated in ductular reaction and liver fibrogenesis. We studied liver and serum IL-8 expression in a large biliary atresia (BA) cohort and explored its prognostic and pathophysiological potential. IL-8 expression was assessed in liver utilizing quantitative polymerase chain reaction (qPCR), immunohistochemistry and in situ hybridization and in serum using an enzyme-linked immunosorbent assay, among 115 BA patients, 10 disease controls and 68 normal controls. Results were correlated to portoenterostomy (PE) outcomes, biochemical and histological liver injury, transcriptional markers of fibrosis and cholangiocytes, and expression of other related cytokines. IL-8 was markedly overexpressed in liver and serum of BA patients at PE (n = 88) and in serum samples obtained during postoperative follow-up (n = 40). IL-8 expression in the liver was predominantly in cholangiocytes within areas of ductular reaction. Liver IL-8 mRNA expression correlated positively with its serum concentration, bile ductular proliferation, Metavir fibrosis stage, and transcriptional markers of activated myofibroblasts (ACTA2) and cholangiocytes (KRT19). Taken together, IL-8 may mediate liver injury in BA by promoting ductular reaction and associated liver fibrogenesis. Prognostic value of serum IL-8 to predict native liver survival was limited and confined to the postoperative period after PE.

Background and Aims: Outcomes after Kasai portoenterostomy (KPE) for biliary atresia remain highly variable for unclear reasons. As reliable early biomarkers predicting KPE outcomes are lacking, we studied the prognostic value of FGF19. Approach and Results: Serum and liver specimens, obtained from biliary atresia patients (N=87) at KPE or age-matched cholestatic controls (N=26) were included. Serum concentration of FGF19 and bile acids, liver mRNA expression of FGF19 , and key regulators of bile acid synthesis were related to KPE outcomes and liver histopathology. Immunohistochemistry and in situ hybridization were used for the localization of liver FGF19 expression. Serum levels (223 vs. 61 pg/mL, p <0.001) and liver mRNA expression of FGF19 were significantly increased in biliary atresia. Patients with unsuccessful KPE (419 vs. 145 pg/mL, p =0.047), and those subsequently underwent liver transplantation (410 vs. 99 pg/mL, p =0.007) had significantly increased serum, but not liver, FGF19, which localized mainly in hepatocytes. In Cox hazard modeling serum FGF19 <109 pg/mL predicted native liver survival (HR: 4.31, p <0.001) also among patients operated <60 days of age (HR: 8.77, p =0.004) or after successful KPE (HR: 6.76, p =0.01). Serum FGF19 correlated positively with increased serum primary bile acids ( R =0.41, p =0.004) and ductular reaction ( R =0.39, p =0.004). Conclusions: Increased serum FGF19 at KPE predicted inferior long-term native liver survival in biliary atresia and was associated with unsuccessful KPE, elevated serum primary bile acids, and ductular reaction.

AIMS:To outline the use of radiological investigations in patients with congenital portosystemic shunts (CPS) and to assess the degree of portal venous deprivation in relation to the development of liver tumors. METHODS:Single center retrospective cohort study of all patients with CPS referred from 1990 to 2016. Radiological investigations were reviewed for the presence of intrahepatic portal veins. Two groups were defined: Group 1 - without evidence of preservation of an intrahepatic portal venous system and, Group 2 - with evidence of intrahepatic portal venous flow. These groups were compared for the development of liver tumors, serum ammonia, and nature of subsequent surgical intervention. The ratio of infraceliac to supraceliac aortic diameter and hepatic enhancement in biphasic CT scans were also used to infer hepatic arterial in-flow and compared to age matched controls. Nonparametric tests were used throughout. A P value of 0.05 was considered significant. Data are quoted as median (IQR). RESULTS:45 patients (Group 1, n = 12: Group 2, n = 33) were investigated for CPS at a median age of 8 months (1 month-14 years). Liver tumors were more common in Group 1 than Group 2 [11/12 (92%) versus 10/33 (29%); P < 0.001]. Aortic ratio was significantly lower in patients with CPS compared to control (0.82 versus 0.96; P < 0.001), but there was no difference between patients with or without tumors (0.82 versus 0.82; P = 0.52). Enhancement of the liver parenchyma was greater in portal venous rather than arterial phases by a median difference of 28 (15-50) Hounsfield units in controls, compared to 15 (8.5-23.5) in CPS patients (P = 0.04). A single stage closure was possible in 2/6 (33%) operated patients in Group 1 and 14/20 (70%) in Group 2 (P = 0.32). CONCLUSIONS:Patients without radiological evidence of intrahepatic portal venous flow were significantly more likely to have associated hepatic tumors with a relative risk 3.1. LEVELS OF EVIDENCE:This manuscript includes data that are III and IV Levels-of-Evidence.

Pathological conditions affecting skeletal muscle function may lead to irreversible volumetric muscle loss (VML). Therapeutic approaches involving acellular matrices represent an emerging and promising strategy to promote regeneration of skeletal muscle following injury. Here we investigated the ability of three different decellularised skeletal muscle scaffolds to support muscle regeneration in a xenogeneic immune-competent model of VML, in which the EDL muscle was surgically resected. All implanted acellular matrices, used to replace the resected muscles, were able to generate functional artificial muscles by promoting host myogenic cell migration and differentiation, as well as nervous fibres, vascular networks, and satellite cell (SC) homing. However, acellular tissue mainly composed of extracellular matrix (ECM) allowed better myofibre three-dimensional (3D) organization and the restoration of SC pool, when compared to scaffolds which also preserved muscular cytoskeletal structures. Finally, we showed that fibroblasts are indispensable to promote efficient migration and myogenesis by muscle stem cells across the scaffolds in vitro. This data strongly support the use of xenogeneic acellular muscles as device to treat VML conditions in absence of donor cell implementation, as well as in vitro model for studying cell interplay during myogenesis.

Aim: We investigated the natural history of fetal ovarian cysts to estimate the risk of torsion according to size. Methods: Cases were identified from 1/1/2000 until 1/1/2015. Data were collected pre-and postnatally on cyst size and sonographic features until an outcome of surgery, torsion, or resolution. Fisher's exact test for categorical data and logistic regression for continuous data were used to test the significance of size on torsion; P value <0.05 was considered significant. Results: 37 patients with unilateral ovarian cysts were included. 12 (32%) resolved spontaneously prenatally, 14 (38%) resolved spontaneously postnatally, 5 (14%) underwent surgery postnatally and 6 (16%) cases underwent torsion. Rate of torsion increased with size from 0% (n = 0) in cysts <= 20 mm to 33% (n = 2) in cysts >50 mm; however, the overall trend failed to reach statistical significance (P = 0.1). Cysts of 0-40 mm had a significantly higher rate of spontaneous resolution (90% vs. 44% in >40 mm, P = 0.003), but the rate of torsionwas not significantly different (10% in 0-40 mmvs. 25% in >40 mm, P = 0.26). The median time to postnatal resolutionwas 10 (5-27) weeks in those treated conservatively. Conclusion: Cysts N40 mm are significantly less likely to resolve spontaneously; however torsion showed no significant correlation with cyst size. No complications were observed in cysts <20 mm. (C) 2018 Published by Elsevier Inc.

BACKGROUND Biliary atresia is a life-threatening disease characterised by progressive destruction of both intra- and extra-hepatic biliary ducts. The mainstay of treatment is Kasai portoenterostomy, as soon as the disease has been confirmed. Glucocorticosteroids are steroid hormones which act on the glucocorticoid receptor and have a range of metabolic and immunomodulatory effects. Glucocorticosteroids are used to improve the postoperative outcomes in infants who have undergone Kasai portoenterostomy. OBJECTIVES To assess the beneficial and harmful effects of glucocorticosteroid administration versus placebo or no intervention following Kasai portoenterostomy in infants with biliary atresia. SEARCH METHODS We searched the Cochrane Hepato-Biliary Group Controlled Trials Register, Cochrane Central Register of Controlled Trials (CENTRAL) in the Cochrane Library, MEDLINE Ovid, Embase Ovid, Science Citation Index Expanded (Web of Science), and online trial registries (last search: 20 December 2017) for randomised controlled trials. SELECTION CRITERIA We included randomised clinical trials which assessed glucocorticosteroids for infants who have undergone Kasai portoenterostomy. For harm, we also considered quasi-randomised studies, observational studies, and case-control studies that were identified amongst the search results. DATA COLLECTION AND ANALYSIS We used standard methodological procedures expected by Cochrane. We assessed the risk of bias for each trial according to prespecified domains. We analysed data using both random-effects and fixed-effect models. We performed the analyses using Review Manager 5.3 and Trial Sequental Analysis software. We considered a P value of 0.025 or less, two-tailed, as statistically significant. We planned to calculate risk ratios (RRs) for dichotomous outcomes, and the mean difference (MD) for continuous outcomes. For all association measures, we planned to use 95% confidence intervals (CIs) as well as Trial Sequential Analysis-adjusted CIs. We used Trial Sequential Analyisis to control the risks of random errors; however, we were often unable to implement this beyond calculating the required information size as there were few trials and data. We assessed the certainty of the evidence using GRADE. MAIN RESULTS We found two randomised controlled trials fulfilling the inclusion criteria of our review. The trials provided data for meta-analysis. We judged the two trials as trials at low risk of bias. The two trials randomised a total of 213 infants to glucocorticosteroids versus placebo. In our Trial Sequential Analysis, the required information size (that is, the meta-analytic sample size) was not reached for any outcome. Trials were funded by charities, public organisations, and received support from private sector companies, none of which seemed to have an interest in the outcome of the respective trials. The effect of glucocorticosteroids after Kasai portoenterostomy on all-cause mortality is uncertain; the confidence interval is consistent with appreciable benefit and harm (RR 1.00; 95% CI 0.14 to 6.90; low-certainty evidence). The results showed little or no difference in adverse effects between the use of glucocorticosteroids or placebo after Kasai portoenterostomy, however this analysis was based on a single trial and we have low certainty in the result (RR 1.02; 95% CI 0.87 to 1.20;). Available data suggest that the proportions of infants who do not clear their jaundice at six months is similar between the two groups (RR 0.89; 95% CI 0.67 to 1.17; low-certainty evidence). All-cause mortality or liver transplantation did not differ at two years between the two groups (RR 1.00; 95% CI 0.72 to 1.39; low-certainty evidence). There were no data regarding health-related quality of life.Our searches also yielded 19 observational studies, some of them containing limited information on harmful effects of glucocorticosteroid treatment. We presented the extracted information narratively. We identified one further ongoing trial with no currently available results. AUTHORS' CONCLUSIONS The two meta-analysed randomised clinical trials present insufficient evidence to determine the effects of using glucocorticosteroids versus placebo after Kasai portoenterostomy in infants with biliary atresia on any of the primary or secondary review outcomes. There is insufficient evidence to support glucocorticosteroid use in the postoperative management of infants with biliary atresia for long-term outcomes of all-cause mortality or liver transplantation. It is also unclear if glucocorticosteroids are able to reduce the numbers of infants who did not clear their jaundice by six months. Further randomised, placebo-controlled trials are required to be able to determine if glucocorticosteroids may be of benefit in the postoperative management of infants with biliary atresia treated with Kasai portoenterostomy. Such trials need to be conducted as multicentre trials.

Congenital portosystemic shunt (CPSS) is a congenital anomaly resulting in partial or complete diversion of the portal blood into the systemic circulation. The literature on the histological changes in livers of patients with CPSS is limited. Liver histology of 22 consecutive patients managed in our institution between 2001 and 2016 was reviewed. Twenty-one patients were children at the time of diagnosis. Thirty-two specimens were available and consisted of three explant livers and 29 biopsy samples from 19 patients. Sixteen samples were from wedge biopsies taken at the time of shunt closure. Thirteen were from core needle biopsies taken during clinical work-up. A variable proportion of portal tracts contained prominent thin-walled channels (PTWCs) and arterio-biliary dyads. The proportion of portal tracts containing triads, arterio-biliary dyads and biliary monads varied considerably in the different samples. Dilated inlet venules, increase in the number of portal arteries or the presence of portal arteries of increased size, deposition of copper-associated protein, sinusoidal dilatation, capillarization and intralobular individual arteries were present. Physiological nuclear vacuolation of periportal hepatocytes was absent in most samples from our paediatric patients. Presence of PTWCs, arterial-biliary dyads, increased arterial profiles in portal tracts and lobule and lack of the physiological periportal vacuolated hepatocytes in children are the most characteristic histological changes of CPSS in the liver periphery.

ObjectiveThe objective of the study is to compare outcomes of ultrasound-guided aspiration of fetal ovarian cysts with conservative management. MethodA systematic review of MEDLINE and Web of Science included studies reporting outcomes (prenatal and postnatal torsion, spontaneous resolution and surgery) of fetuses with ovarian cysts. Subgroup analysis was performed according to cyst diameter at diagnosis and cysts 40mm. ResultsNinety-two non-randomised studies reported on 380 cysts (324 observed and 56 aspirated in utero) in 365 fetuses. All studies were case reports or series with high heterogeneity and risk of bias. The overall spontaneous resolution rate of conservatively managed cysts was 46%, yet decreased with increasing cyst size. Risk of prenatal ovarian torsion in conservatively managed cases depended on cyst size and was particularly important in the range 30 to 59mm (15-34%). The rate of prenatal torsion in simple cysts 40mm was lower in aspirated than conservatively managed cysts (0% vs 10%, p=0.03). Aspirated cysts had lower rates of postnatal surgery (7%) compared with conservatively managed cysts (49%, p<0.001). ConclusionCysts 30 to 59mm were at highest risk of torsion. Simple cysts >40mm had lower rates of torsion when aspirated prenatally. Randomised studies and safety data are needed prior to routine prenatal ovarian cyst aspiration. (c) 2017 John Wiley & Sons, Ltd.

The use of adjuvant steroids following Kasai porteoenterostomy (KPE) for biliary atresia is controversial. The aim of this study was twofold: a systematic review of published literature and an update of the clinical Kings College Hospital series to look for evidence of an effect of age on the outcome in a group of BA infants treated with high-dose steroids. This clinical study included infants treated between January 2006 and June 2014 who underwent KPE by day 70 of life and who received high-dose steroids (oral prednisolone starting 5 mg/kg/day). They were subdivided into cohorts according to age at which KPE was performed. The outcome measured was clearance of jaundice (<20 µmol/L) by 6 months and native liver survival. R × C χ 2 analysis and log-rank tests were used, respectively, and P ≤ 0.05 was regarded as significant. 104 infants were included with a median age at KPE of 45 (range 12–70) days. 71/104 (67 %) cleared their jaundice by 6 months of age. Age-cohort analysis showed a trend ( P = 0.03) favouring early KPE (e.g. 100 % of 11 infants operated on <30 days clearing their jaundice compared to 66 % of those operated on between 61 and 70 days). There was a significant native liver survival benefit for those operated on <45 days (5 year NLS estimate 69 versus 46 %; P = 0.05). Clearance of jaundice is related to the age at KPE in infants who receive high-dose steroids. Native liver survival appears to be improved as a result of this. This is the first study to show tangible longer-term benefit from high-dose steroids in biliary atresia.