Finnish Pediatric Surgery Hub - From Centralization to Collective Learning and Sharing of Expertise.

AIM OF THE STUDY: Continuous surgical developments, growing awareness of patient representatives and limited health-care resources are pushing for innovative approaches to ensure equal high-quality pediatric surgical care. We aimed to describe early experiences and assess surgical safety of a novel nationwide pediatric surgery collaborative initiative. METHODS: In 2021, general pediatric surgeons representing all five independent university hospitals performing neonatal surgery in Finland initiated national collaboration, the Finnish pediatric surgery hub (FPSH), for sharing of surgical expertise and collective learning. For each case addressed by FPSH, place of care and surgical team were decided individually, and when deemed necessary, operations were performed in cooperation. Operations performed during 2021-2023 and associated early (<30 days) postoperative complications were analyzed according to Clavien-Madadi classification. RESULTS: Of the total 40 surgeries managed co-operatively by FPSH, 30 (75%) took place in local university hospitals and 10 in Helsinki University Hospital. There were 34 (85%) elective and 6 urgent cases, which were operated within median 1 (range, 1-3) days. Most frequent underlying diagnoses included anorectal malformations, esophageal atresia and Hirschsprung disease. Overall, 12 (30%) had any early postoperative complications, all Clavien-Madadi grade IIIB or lower, and five patients (13%) were reoperated. Rate or grade of complications was not associated with place of care. In addition to regular virtual case meetings, national care protocols and research projects were introduced. CONCLUSION: These preliminary findings suggest that our national collaborative initiative, FPSH, not only provided practical and safe framework for sharing of surgical expertise but also for collective learning. LEVEL OF EVIDENCE: III.

Distal Recurrent Tracheoesophageal Fistula after Repair of Esophageal Atresia-Incidence, Risk Factors, and Outcome.

OBJECTIVE: The aim of this study was to assess incidence, risk factors, and outcomes of distal recurrent tracheoesophageal fistula (RTEF) after repair of esophageal atresia (EA). METHODS: Ethical consent was obtained. Data collection was done by review of hospital records of 286 patients (268 in-house and 18 referred) who underwent repair of type C or D EA from 1980 to 2021. Spitz class, long-gap (tracheoesophageal fistula at carina), fundoplication, leakage, and stricture were assessed as RTEF risk factors. Outcome measures were long-term closure of RTEF, retainment of native esophagus, and survival. RESULTS: RTEF occurred in 23 patients (19 in-house) with type C (n = 22) or type D (n = 1) EA with median 4.4 (interquartile range [IQR]: 1.7-13) months after repair. Five patients had late RTEF 3.5 to 16 years after repair. Nineteen (7.3%) in-house patients developed RTEF. Presenting symptoms, age at diagnosis, and presence of anastomotic stricture (AS) are listed. No statistically significant risk factors were found, risk ratio of 0.5 to 2.7 (IQR: 0.1-8.7), p-value of 0.25 to 0.75. Detached closure clip and esophageal foreign body contributed to RTEF in two patients. Sixteen patients underwent rethoracotomy for closure of RTEF, 5 (22%) with AS eventually underwent esophageal reconstruction after a period with cervical esophagostomy. Two patients with late-manifested RTEFs underwent closure with laser cauterization. In 22 patients, treatment of RTEF succeeded, whereas 1 (4%) premature patient died of instant re-RTEF. CONCLUSION: RTEF had an incidence of 7% with diverse patterns of manifestation and predicting factors. Closure rate and patient survival were excellent, but RTEF with AS predicted loss of native esophagus.

Intrinsic Cellular Susceptibility to Barrett's Esophagus in Adults Born with Esophageal Atresia.

The prevalence of Barrett's esophagus (BE) in adults born with esophageal atresia (EA) is four times higher than in the general population and presents at a younger age (34 vs. 60 years). This is (partly) a consequence of chronic gastroesophageal reflux. Given the overlap between genes and pathways involved in foregut and BE development, we hypothesized that EA patients have an intrinsic predisposition to develop BE. Transcriptomes of Esophageal biopsies of EA patients with BE (n = 19, EA/BE); EA patients without BE (n = 44, EA-only) and BE patients without EA (n = 10, BE-only) were compared by RNA expression profiling. Subsequently, we simulated a reflux episode by exposing fibroblasts of 3 EA patients and 3 controls to acidic conditions. Transcriptome responses were compared to the differential expressed transcripts in the biopsies. Predisposing single nucleotide polymorphisms, associated with BE, were slightly increased in EA/BE versus BE-only patients. RNA expression profiling and pathway enrichment analysis revealed differences in retinoic acid metabolism and downstream signaling pathways and inflammatory, stress response and oncological processes. There was a similar effect on retinoic acid signaling and immune response in EA patients upon acid exposure. These results indicate that epithelial tissue homeostasis in EA patients is more prone to acidic disturbances.

The Outcome of Blood Transfusions in Conservative and Surgical Necrotizing Enterocolitis and Spontaneous Intestinal Perforation.

INTRODUCTION: In necrotizing enterocolitis (NEC), blood transfusions (BTs) are a disputed factor concerning the etiology and the outcome. We assessed retrospectively the effects of preonset BT on the outcome of NEC and spontaneous intestinal perforation (SIP). MATERIALS AND METHODS: With ethical consent, we reviewed the hospital records of 113 neonates from 2002 to 2019 with surgical NEC (NECs) (n = 57), conservatively treated NEC (NECc) (n = 20), and SIP (n = 36). The onset was defined as the day of surgery (NECs and SIP) or diagnosis (NECc). The effects of preonset BT (total, beyond 48 hours, and within 48 hours) were compared with the effects of birth weight (BW), gestational age (GA), intracerebral hemorrhage, respiratory distress syndrome, septicemia, and patent ductus arteriosus. Main outcome measure was 2-week mortality, and secondary measures were small intestinal loss and days on parenteral nutrition (PN). RESULTS: Overall mortality (8.9%) was predicted independently by BT total and BT beyond 48 hours, risk ratio (RR) = 1.1 to 1.2 (95% confidence interval [CI] = 1.0-1.4), p = 0.01 to 0.02, and BW and GA, RR = 0.7 to 1.0 (95% CI = 0.5-1.0), p = 0.02 to 0.04. Mortality in NECs (12%) was predicted by BT total and BT beyond 48 hours only, RR = 1.1 to 1.2 (95% CI = 1.0-1.5), p = 0.03 to 0.04. BT within 48 hours of onset did not predict mortality. No factors were related with secondary outcome measures or with mortality in patients with SIP or NECc. CONCLUSION: In NECs, preoperative BT and BT given more than 48 hours before surgery were correlated with slightly increased 2-week mortality but not with small intestinal loss or duration of PN.

Anastomotic Stricture in End-to-End Anastomosis-Risk Factors in a Series of 261 Patients with Esophageal Atresia.

AIM: To assess the risk factors for anastomotic stricture (AS) in end-to-end anastomosis (EEA) in patients with esophageal atresia (EA). METHODS: With ethical consent, hospital records of 341 EA patients from 1980 to 2020 were reviewed. Patients with less than 3 months survival (n = 30) with Gross type E EA (n = 24) and with primary reconstruction (n = 21) were excluded. Outcome measures were revisional surgery for anastomotic stricture (RSAS) and number of dilatations required for anastomotic patency without RSAS. The factors that were tested for risk of RSAS or dilatations were distal tracheoesophageal fistula (TEF) at the carina in C-type EA (congenital TEF [CTEF]), type A/B EA, antireflux surgery (ARS), anastomotic leakage, recurrent TEF, and Spitz group and congenital heart disease. MAIN RESULTS: A total of 266 patients, Gross type A (n = 17), B (n = 3), C (n = 237), or D (n = 9) underwent EEA (early n = 240, delayed n = 26). Early anastomotic breakdown required secondary reconstruction in five patients. Of the remaining 261 patients, 17 (6.1%) had RSAS, whereas 244 patients with intact end to end required a median of five (interquartile range: 2-8) dilatations for anastomotic patency. Main risk factors for RSAS or (> 8) dilatations were CTEF, type A/B, ARS, and anastomotic leakage that increased the risk of RSAS or dilatations from 4.6- to 11-fold. CONCLUSION: The risk of severe AS is associated with long-gap EA, significant gastroesophageal reflux, and anastomotic leakage.

Oesophageal atresia with very low birth weight: Clinical characteristics and long-term outcome.

AIM OF THE STUDY: An assessment of the clinical data and outcome of patients with oesophageal atresia (OA) with very low birth weight (VLBW) was completed. METHOD: With ethical consent, we reviewed the records of 327 successive patients with OA from 1980 to 2020. Main outcome measures survival and oesophageal repair were compared between patients with VLBW(≤1500 g) and with BW>1500 g. RESULTS: Thirty-four (10%) patients had VLBW. Gross types of OA in VLBW were similar as in other patients: A (15%/7%), B (3%/3%), C (78%/82%), D (3%/4%), E (0%/7%), F (0%/1%) (p = 0.16-0.99). In VLBW the incidence of congenital heart disease (CHD) (47%) and trisomy 13/18 and Cri du Chat (15%) were higher than in BW>1500 g (23% and 1%), (p = 0.001 both). In VLBW one-month mortality was 35% vs 4% in patients with BW>1500 g (p < 0.001), overall mortality 56% and 8% (p < 0.001), respectively. Cause of one-month mortality in VLBW (12 patients) were CHD w/wo chromosomal abnormality (n = 7), cerebral hemorrhage (n = 2), gastric perforation (n = 1), anastomotic leakage (n = 1) and pulmonary hemorrhage (n = 1). Of VLBW patients 79% and of other patients 99% underwent oesophageal repair (p < 0.001). Repair in VLBW patients included early (n = 18) or delayed (n = 5) end-to-end anastomosis and reconstruction (n = 4). Anastomotic complications occurred in 24% of patients with VLBW and in 17% with BW>1500 g, (p = 0.31). From 1980-2000 to 2001-2020 survival in VLBW changed from 11% to 81% (p = 0.002). During 2001 - 2020 all VLBW patients underwent repair. CONCLUSION: OA with VLBW had high incidence of life-threatening associated anomalies and decreased survival. Recently survival and rate of oesophageal repair have improved significantly.

Long-term Morbidity of Choledochal Malformations in Children.

OBJECTIVE: The aim of the study was to assess long-term morbidity in children operated for choledochal malformation (CM) by relating clinical complications to liver histopathology, follow-up imaging, liver stiffness, and biochemistry. METHODS: A single-center retrospective follow-up study including all CM patients (n = 55, 71% girls) treated during 1976 to 2018 was performed. Mann-Whitney U test and Spearman rank correlation were used for statistical analyses. RESULTS: During median follow-up of 5.8 (interquartile range, 2.5-12) years, 1 patient was lost to follow-up whereas all survived. Intraoperative liver biopsies showed fibrosis in 32%, and patients with Metavir stage ≥2 were younger at surgery (0.36 [0.11-1.9] vs 3.8 [0.72-10.5] years, P = 0.024) than those without fibrosis. Overall, 21% had long-term complications including cholangitis in 9 (>2 episodes in 5) patients, anastomotic stricture in 2 referred patients and adhesive volvulus or hepatocellular carcinoma in 1 each. Anastomotic strictures were successfully managed nonoperatively and hepatocellular carcinoma with thermoablation. In postoperative magnetic resonance cholangiography (MRCP) performed 6.4 (3.6-16) years after hepaticojejunostomy, diameters of both main intrahepatic ducts had decreased significantly to 3.0 (2.5-3.5) mm (P = 0.0001) but a distal cyst stump was remaining in 30% with a length of 6.0 (4.0-20) mm that associated with operation age (r = 0.71, P = 0.015) and fusiform CM type. Follow-up ultrasound revealed mild dilation of intrahepatic bile ducts in 6.3% and mildly to moderately elevated liver biochemistry in 23%, and liver stiffness (>7 kPa) in 22%. CONCLUSIONS: Whilst cholangitis was the most common postoperative problem, individual patients experienced other more significant complications and one quarter of patients showed evidence of underlying liver dysfunction.

Late Abdominal Reoperations after Surgery for Necrotizing Enterocolitis and Spontaneous Intestinal Perforation.

INTRODUCTION: The aim of this study was retrospective assessment of late major reoperations after surgery for necrotizing enterocolitis (NEC) and spontaneous intestinal perforation (SIP) in neonates. MATERIALS AND METHODS: Appropriate ethical consent was obtained. Data collection was by review of hospital records of 165 successive prematures who underwent surgery for NEC (n = 99) or SIP (n = 66) from 1986 to 2019. Outcome measures were late major abdominal surgeries or endoscopies that occurred after the primary surgical treatment, closure of enterostomies, and weaning from parenteral nutrition (PN). We assessed also risk factors for late abdominal surgeries. RESULTS: Hundred and twenty-six (76%) patients (NEC, n = 70, 71% and in SIP, n = 56, 85%) survived. Median follow-up was 13 (interquartile range: 5.9-23) years. Nineteen (15%) patients underwent late abdominal surgery with 0% mortality. Most common was surgery for small bowel obstruction (SBO) in 12(9.5%) patients (NEC, n = 5; 7.0%, SIP, n = 7; 13%, p = 0.36) with 10-year cumulative risk of 8.7 (95% confidence interval [CI]: 3.5-14). Long duration of PN and development of intestinal failure associated cholestasis (IFAC) increased the risk of SBO surgery, relative risk: 1.0-3.2 (95% CI: 1.0-9.1), p = 0.02-0.03. Other surgeries included serial transverse enteroplasty (NEC, n = 2), incisional hernia repair (n = 3), cholecystectomy (n = 1), and ovarian torsion (n = 1). Twenty (29%) NEC and four (7%) SIP patients required endoscopic assessment of postoperative symptoms (p = 0.003). CONCLUSION: Late abdominal reoperations occurred in 15% of patients with NEC and SIP with nil mortality. SBO was the main indication. Risk of SBO was similar between NEC and SIP. Long PN and IFAC increased the risk of SBO.

Prediction, identification and progression of histopathological liver disease activity in children with intestinal failure.

BACKGROUND & AIMS: Diagnostic criteria, progression risk and optimal monitoring for intestinal failure (IF)-associated liver disease (IFALD) remain undefined. We assessed predictors, non-invasive markers and progression of histopathological liver disease in patients with IF. METHODS: In total, 77 children with IF and median age of 1.7 years underwent diagnostic liver biopsy, which was repeated in 48 patients after 2.9 years with simultaneous evaluation of liver biochemistry, liver stiffness, serum citrulline (a surrogate for viable enterocyte mass), spleen size, esophageal varices and clinical data. Patients were staged according to histopathological liver disease activity: active IFALD (cholestasis and/or inflammation), chronic IFALD (significant fibrosis and/or steatosis), or no IFALD (none of these features). RESULTS: Diagnostic liver biopsy revealed active, chronic or no IFALD in 48%, 21% and 31% of patients. Active IFALD was segregated by low serum citrulline, parenteral nutrition (PN) dependency and young age, while weaning off PN and older age predicted chronic IFALD. Although the liver histopathology in most patients either normalized (52%) or transformed to a less reactive (chronic) disease stage (23%), 19% of patients retained and 6.3% progressed to an active cholestatic/inflammatory IFALD phenotype. Decreased serum citrulline and PN-dependency also predicted active IFALD in follow-up biopsies. Increased median liver biochemistry values and liver stiffness only associated with active IFALD, which was accurately identified by gamma-glutamyltransferase (GGT), citrulline and liver stiffness, their combinations reaching diagnostic and follow-up AUROC values above 0.90. CONCLUSIONS: Active IFALD, essentially predicted by intestinal disruption and PN-dependency, was accurately detected by GGT, liver stiffness and citrulline, which together with recent advances in clinical management options, provides new avenues for monitoring and targeted liver protection in patients with IF. LAY SUMMARY: Liver disease is a common and critical complication in patients with intestinal failure, who require intravenous nutrition for survival due to severe intestinal dysfunction. We showed that both intravenous nutrition dependency and intestinal disruption essentially predicted development of active histopathological liver disease, which persisted in 25% of patients during long-term follow-up and could be accurately detected without the need for liver biopsy. Identification of the active and potentially progressive histopathology offers new possibilities for monitoring and targeted liver protection in patients with intestinal failure.

Indications, Surgical Complications, and Long-Term Outcomes in Pediatric Esophageal Reconstructions with Pedicled Jejunal Interposition Graft.

INTRODUCTION: Several surgical techniques are available for pediatric esophageal reconstruction. We started to use pedicled jejunum interposition graft (PJIG) because other techniques had significant long-term complications. In this retrospective study, the indications, surgical complications, and long-term outcomes were assessed in patients with PJIG. MATERIALS AND METHODS: With ethical consent, we reviewed the hospital records of 14 patients (7 females) who from 2005 to 2019 underwent a total of 16 esophageal reconstructions with PJIG. RESULTS: Median age at PJIG was 1.6 (range: 0.2-15) years. Underlying conditions were esophageal atresia (EA) (n = 11) or native esophagus lost by trauma or infection (n = 3). Eight patients with EA underwent PJIG as primary reconstruction and three as a rescue operation after complications in primary repair. Significant surgical complications occurred in 43% of patients. Major reoperations in six (43%) patients included resection and reanastomosis of strictured proximal PJIG (n = 1) and redo PJIG after failure of the first operation (n = 2). Surgical mortality was nil. After a median follow-up of 6.5 (range: 0.7-14) years, 13 (93%) patients survived, and 1 died of congenital heart disease. PJIG failed in three (23%) survivors of whom two underwent graft removal because of life-threatening aspiration and one did not start oral feeds at all. Ten survivors (77%) have full enteral feeds. Respiratory function in the survivors is satisfactory. Two patients have moderate and three mild gastroesophageal reflux symptoms. CONCLUSION: PJIG was a functional option for a variety of conditions that required esophageal reconstruction. However, significant early and late complications required major surgical revisions.

Surgically treated ovarian lesions in preadolescent girls.

INTRODUCTION: The purpose of this study was to investigate the epidemiology and characteristics of surgically treated ovarian lesions in preadolescent girls. MATERIAL AND METHODS: This was a retrospective cohort study including all 0- to 11-year-old girls operated at a single center from 1999 to 2016 for ovarian cysts, neoplasms or torsions. Patient charts were reviewed for symptoms, preoperative radiological imaging, operative details and histopathology. RESULTS: We identified 78 girls, resulting in a population-based incidence of 4.2/100 000. Infants (n = 44) presented with benign cysts (42/44, 95%, one bilateral), a benign neoplasm (1/44, 2%) and a torsion without other pathology (1/44, 2%). Torsion was found in 25/29 (86%) ovaries with complex cysts and in 3/15 (21%) ovaries with simple cysts in preoperative imaging (P < 0.001). Most infants were symptomless. Lesions in 1- to 11-year-old girls (n = 34) included benign neoplasms (n = 21/34, 62%), malignant neoplasms (n = 5/34, 15%), a cyst with torsion (n = 1/34, 3%) and torsions without other pathology (n = 7/34, 21%). Torsion was more common in benign (17/21, 81%) than in malignant neoplasms (1/5, 20%) (P < 0.020). Ovarian diameter did not differ between ovaries with or without torsion (P = 0.238) or between benign and malignant neoplasms (P = 0.293). The duration of symptoms in lesions with or without torsion was similar. CONCLUSIONS: The majority of surgically treated ovarian lesions in preadolescent are benign lesions with torsion. Surgery should be ovary-preserving and performed without delay.

ERNICA Consensus Conference on the Management of Patients with Esophageal Atresia and Tracheoesophageal Fistula: Diagnostics, Preoperative, Operative, and Postoperative Management.

INTRODUCTION: Many aspects of the management of esophageal atresia (EA) and tracheoesophageal fistula (TEF) are controversial and the evidence for decision making is limited. Members of the European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) conducted a consensus conference on the surgical management of EA/TEF based on expert opinions referring to the latest literature. MATERIALS AND METHODS: Nineteen ERNICA representatives from nine European countries participated in the conference. The conference was prepared by item generation, item prioritization by online survey, formulation of a final list containing the domains diagnostics, preoperative, operative, and postoperative management, and literature review. The 2-day conference was held in Berlin in October 2018. Anonymous voting was conducted via an internet-based system. Consensus was defined when 75% of the votes scored 6 to 9. RESULTS: Fifty-two items were generated with 116 relevant articles of which five studies (4.3%) were assigned as level-1evidence. Complete consensus (100%) was achieved on 20 items (38%), such as TEF closure by transfixing suture, esophageal anastomosis by interrupted sutures, and initiation of feeding 24 hours postoperatively. Consensus ≥75% was achieved on 37 items (71%), such as routine insertion of transanastomotic tube or maximum duration of thoracoscopy of 3 hours. Thirteen items (25%) were controversial (range of scores, 1-9). Eight of these (62%) did not reach consensus. CONCLUSION: Participants of the conference reached significant consensus on the management of patients with EA/TEF. The consensus may facilitate standardization and development of generally accepted guidelines. The conference methodology may serve as a blueprint for further conferences on the management of congenital malformations in pediatric surgery.

ERNICA Consensus Conference on the Management of Patients with Esophageal Atresia and Tracheoesophageal Fistula: Follow-up and Framework.

INTRODUCTION: Improvements in care of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) have shifted the focus from mortality to morbidity and quality-of-life. Long-term follow-up is essential, but evidence is limited and standardized protocols are scarce. Nineteen representatives of the European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) from nine European countries conducted a consensus conference on the surgical management of EA/TEF. MATERIALS AND METHODS: The conference was prepared by item generation (including items of surgical relevance from the European Society for Pediatric Gastroenterology Hepatology and Nutrition (ESPGHAN)-The North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) guidelines on follow-up after EA repair), item prioritization, formulation of a final list containing the domains Follow-up and Framework, and literature review. Anonymous voting was conducted via an internet-based system. Consensus was defined as ≥75% of those voting with scores of 6 to 9. RESULTS: Twenty-five items were generated in the domain Follow-up of which 17 (68%) matched with corresponding ESPGHAN-NASPGHAN statements. Complete consensus (100%) was achieved on seven items (28%), such as the necessity of an interdisciplinary follow-up program. Consensus ≥75% was achieved on 18 items (72%), such as potential indications for fundoplication. There was an 82% concordance with the ESPGHAN-NASPGHAN recommendations. Four items were generated in the domain Framework, and complete consensus was achieved on all these items. CONCLUSION: Participants of the first ERNICA conference reached significant consensus on the follow-up of patients with EA/TEF who undergo primary anastomosis. Fundamental statements regarding centralization, multidisciplinary approach, and involvement of patient organizations were formulated. These consensus statements will provide the cornerstone for uniform treatment protocols and resultant optimized patient care.

Surgical treatment and major complications Within the first year of life in newborns with long-gap esophageal atresia gross type A and B - a systematic review.

BACKGROUND: The surgical repair of long-gap esophageal atresia (LGEA) is still a challenge and there is no consensus on the preferred method of reconstruction. We performed a systematic review of the surgical treatment of LGEA Gross type A and B with the primary aim to compare the postoperative complications related to the different methods within the first postoperative year. METHODS: Systematic literature review on the surgical repair of LGEA Gross type A and B within the first year of life published from January 01, 1996 to November 01, 2016. RESULTS: We included 57 articles involving a total of 326 patients of whom 289 had a Gross type A LGEA. Delayed primary anastomosis (DPA) was the most applied surgical method (68.4%) in both types, followed by gastric pull-up (GPU) (8.3%). Anastomotic stricture (53.7%), gastro-esophageal reflux (GER) (32.2%) and anastomotic leakage (22.7%) were the most common postoperative complications, with stricture and GER occurring more often after DPA (61.9% and 40.8% respectively) compared to other methods (p < 0.001). CONCLUSION: The majority of patients in this review were managed by DPA and postoperative complications were common despite the surgical method, with anastomotic stricture and GER being most common after DPA. LEVEL OF EVIDENCE: Systematic review of case series and case reports with no comparison group (level IV).

Combined management of perianal rhabdomyosarcoma with chemotherapy, radical surgery, and irradiation: A series of three consecutive children.

BACKGROUND AND AIM: We describe a series of three successive patients with perianal rhabdomyosarcoma (PRMS) from 2014 to 2017 managed with combined chemotherapy, radical surgery, and radiotherapy. METHODS: Ethical consent was obtained. Data including tumor presentation, treatment, and survival was collected from hospital reports. RESULTS: Two girls aged 15 and 16 years (patient #1 and #2) and one boy aged five years (patient #3) were referred because of a suspected perianal abscess. MRI showed large perianal tumors from 7 to 12 cm in diameter that surrounded or infiltrated the anal sphincters and were inconsistent with abscess. Tumor biopsies showed RMS of alveolar (#1 and #2) and embryonal (#3) types. Patient #1 had lymph node and bone metastases, patient #2 lymph node metastases, and patient #3 no metastases. Pretreatment staging, IRS Clinical Group, and Risk Groups were: Stage 4, II, high; Stage 3, GII, intermediate; and Stage 3, I, low, respectively. All underwent colostomy before neoadjuvant chemotherapy (CWS-RMS 2009 program). Neoadjuvant chemotherapy failed to clear the tumors from anal sphincters preventing anus-saving surgery, and all patients underwent abdominoperineal excision. All removed specimens had free margins with negative lymph nodes. After adjuvant chemotherapy and local radiation, the patients were tumor free after 48, 13, and 18 months. CONCLUSION: In PRMS local surgical control required abdominoperineal excision. Confusion between PRMS and abscess may cause unnecessary delay in management. LEVEL OF EVIDENCE: IV (Treatment Study, Case Series with no Comparison Group).

Long-term Single-centre Outcomes After Proctocolectomy With Ileoanal Anastomosis for Paediatric Ulcerative Colitis.

BACKGROUND AND AIMS: Childhood-onset ulcerative colitis [UC] requires total colectomy in one-quarter of patients at some point of their disease. The study objective was to evaluate long-term outcomes after proctocolectomy with ileoanal anastomosis [IAA] for paediatric UC. METHODS: Medical records of all children undergoing proctocolectomy with IAA for UC during 1985-2016 in Helsinki University Hospital were retrospectively assessed. Data on disease history, diagnostic and operative details, occurrence of surgical complications, functional outcome, postoperative diagnosis of Crohn's disease [CD] and pouch failure were collected. Risk factors for IAA failure were analysed with Cox regression. RESULTS: Of 87 patients, 85 [98%] had UC and 2 [2%] inflammatory bowel disease unclassified [IBD-U] preoperatively. Altogether 66% underwent two-stage and 34% underwent three-stage procedures. During 7.8 [4.1-14.5] years' follow-up, nine [10%] patients were diagnosed with postoperative CD. Postoperative leakages [n = 8, 9%] and strictures [n = 10, 11%] were equally common, whereas fistulas [78% vs 9%, p <0.001] and abscesses [56% vs 14%, p = 0.009] were more frequent among patients with later CD diagnosis. At latest follow-up, eight [9%] patients had been converted to a permanent ileostomy and others reported daytime stooling frequency of 5 [4-7] and 0.5 [0-1] at night. CD diagnosis (hazard ratio [HR] = 23.3, p = 0.005), postoperative abscesses [HR = 16.3, p = 0.013] and fistulas [HR = 20.9, p = 0.007] as well as three-stage surgery [p = 0.018] increased risk for ileostomy. CONCLUSIONS: For paediatric UC, long-term surgical and functional outcomes after proctocolectomy with IAA are reassuring. Need for three-stage surgery and occurrence of postoperative fistulas and abscesses, but not leakages or strictures, associate with postoperative CD diagnosis and the risk for ileostomy.

European Paediatric Surgeons' Association Survey on the Management of Pediatric Appendicitis.

AIM: To define patterns in the management of pediatric appendicitis. METHODS: A total of 169 delegates from 42 (24 European) countries completed a validated survey administered at the EUPSA 2017 annual congress. RESULTS: In the work-up of children with suspected acute appendicitis, most surgeons rely on full blood count (92%), C-reactive protein (82%), and abdominal ultrasonography (76%), but rarely on computed tomography scans or magnetic resonance imaging. In suspected simple appendicitis, most surgeons (76%) do not perform appendectomy at night in clinically stable patients and start antibiotic preoperatively (64%), but only 15% offer antibiotic therapy alone (no appendectomy). In suspected perforated appendicitis, 96% start antibiotic preoperatively, and 92% perform an appendectomy. Presence of phlegmon/abscess is the main contraindication to immediate surgery. In case of appendix mass, most responders (75%) favor a conservative approach and perform interval appendectomy always (56%) or in selected cases (38%) between 2 and 6 months from the first episode (81%). Children with large intraperitoneal abscesses are managed by percutaneous drainage (59% responders) and by surgery (37% responders). Laparoscopy is the preferred surgical approach for both simple (89%) and perforated appendicitis (81%). Most surgeons send the appendix for histology (96%) and pus for microbiology, if present (78%). At the end of the operation, 58% irrigate the abdominal cavity only if contaminated using saline solution (93%). In selected cases, 52% leave a drain in situ. CONCLUSION: Some aspects of appendicitis management lack consensus, particularly appendix mass and intraperitoneal abscess. Evidence-based guidelines should be developed, which may help standardize care and improve clinical outcomes.

Surgical repair of long-gap esophageal atresia: A retrospective study comparing the management of long-gap esophageal atresia in the Nordic countries.

BACKGROUND: Several surgical procedures have been described in the reconstruction of long-gap esophageal atresia (LGEA). We reviewed the surgical methods used in children with LGEA in the Nordic countries over a 15-year period and the postoperative complications within the first postoperative year. METHODS: Retrospective multicenter medical record review of all children born with Gross type A or B esophageal atresia between 01/01/2000 and 12/31/2014 reconstructed within their first year of life. RESULTS: We included 71 children; 56 had Gross type A and 15 type B LGEA. Delayed primary anastomosis (DPA) was performed in 52.1% and an esophageal replacement procedure in 47.9%. Gastric pull-up (GPU) was the most frequent procedure (25.4%). The frequency of chromosomal abnormalities, congenital heart defects and other anomalies was significantly higher in patients who had a replacement procedure. The frequency of gastroesophageal reflux (GER) was significantly higher after DPA compared to esophageal replacement (p = 0.013). At 1-year follow-up the mean body weight was higher after DPA than after organ interposition (p = 0.043). CONCLUSION: DPA and esophageal replacement procedures were equally applied. Postoperative complications and follow-up were similar except for the development of GER and the body weight at 1-year follow-up. Long-term results should be investigated. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: Level III.

Intestinal failure associated cholestasis in surgical necrotizing enterocolitis and spontaneous intestinal perforation.

BACKGROUND: Surgery for necrotizing enterocolitis (NEC) and spontaneous intestinal perforation (SIP) is often complicated by intestinal failure (IF) and intestinal failure associated cholestasis (IFAC). OBJECTIVE: Assessment of incidence, predictors, and mortality associated with IFAC in surgically treated NEC and SIP. METHODS: A retrospective observational study based on hospital records during 1986-2014 in the two largest Finnish neonatal intensive care units was performed. IFAC was defined as conjugated bilirubin >34 µmol/l (2.0 mg/dl) for ≥ two postoperative weeks while receiving parenteral nutrition (PN). RESULTS: In total 225 patients underwent surgery for NEC (n = 142; 63%) or SIP (n = 83; 37%). Included were 57 survivors with ≥two weeks PN. Sixty-five (42%) patients developed IFAC. Two-year survival with IFAC was 80% and without IFAC 89% (p = 0.13). Of the 65 patients with IFAC, all eight with unresolved IFAC died in comparison to six of 57 (11%) whose IFAC resolved (p < 0.0001), while IFAC resolved in all survivors. Survival among patients with resolved IFAC was 89% and with unresolved IFAC (n = 8) 0%, (p < 0.0001). IFAC lasted for median 83 (IQR 45-120) days and correlated with the duration of PN (R2 = 0.16, p = 0.03), delay of starting enteral feeds (R2 = 0.12, p = 0.05) and PN lipid emulsion (RR = 1.0 (95% CI = 1.0-1.1) (p = 0.02). In multivariate logistic regression analysis, IFAC development associated with septicemias and reoperations. CONCLUSIONS: 42% of prematures who underwent surgery for NEC or SIP developed IFAC. Reoperations and septicemias increased the risk of IFAC. None of the patients with unresolved IFAC survived, but IFAC did not increase overall mortality. TYPE OF STUDY: Retrospective prognosis study. LEVEL OF EVIDENCE: Level II.

Pneumonia after Repair of Esophageal Atresia-Incidence and Main Risk Factors.

INTRODUCTION: Esophageal atresia (EA) is associated with significant respiratory mortality. We aimed to assess incidence and predictive factors of EA associated pneumonia during the first 5 years of life. MATERIALS AND METHODS: Institutional ethical consent was obtained. Hospital records of patients with EA from 2002 to 2017 were reviewed. Episodes of pneumonia that were diagnosed in university or regional hospitals were included. For instance, respiratory infections other than pneumonia, anastomotic complications, aortopexy, fundoplication, major EA associated diseases, types of EA, and EA anastomosis dilatations were tested as potential risk factors for pneumonia. RESULTS: A total of 104 patients (56 males; type A 7, B 3, C 83, D 3, E 6, F 2) was included. Thirty-five (34%) patients had 94 episodes of pneumonia corresponding to median 2 (IQR [interquartile range]: 1-4) and 609 episodes per thousand patient years. Majority of pneumonias occurred before the age of 3 years. The cause of pneumonias could be identified as RS (respiratory syncytial) virus in 15(16%) and aspiration in seven (7.4%) episodes. In univariate analysis, pneumonia was predicted by occurrence and number of nonpneumonia respiratory infections, anastomotic reoperations, fundoplication, and number of EA anastomosis dilatations. In multivariate logistic regression analysis, significant risk factors for pneumonia were occurrence of nonpneumonia respiratory infections and number of anastomotic dilatations. CONCLUSION: Episodes of pneumonia occurred in one-third of patients with EA. After the final repair, the incidence of pneumonia was highest during the first 3 years of life. Patients with other acute respiratory infections and high number of dilatations were at the greatest risk.