Ernica Clinical Consensus Statements on Total Colonic and Intestinal Aganglionosis.

BACKGROUND: Hirschsprung disease is a congenital intestinal motility disorder characterized by an absence of enteric ganglion cells. Total colonic aganglionosis and near total or total intestinal aganglionosis, defined as absence of ganglion cells in the entire colon and with variable length of small bowel involved, are life-threatening conditions which affect less than 10 % of all patients with Hirschsprung disease. The aim of this project was to develop clinical consensus statements within ERNICA, the European Reference Network for rare congenital digestive diseases, on four major topics: Surgical treatment of total colonic aganglionosis, surgical treatment of total intestinal aganglionosis, management of poor bowel function in total colonic and/or intestinal aganglionosis and long-term management in total colonic and or intestinal aganglionosis. METHODS: A multidisciplinary panel of representatives from ERNICA centers was invited to participate. Literature was searched, using specified search terms, in Medline (ALL), Embase and Google Scholar. Abstracts were screened and full text publications were selected. The panel was divided in four groups that extracted data from the full text publications and suggested draft statements for each of the major topics. A modified Delphi process was used to refine and agree on the statements. RESULTS: The consensus statement was conducted by a multidisciplinary panel of 24 participants from 10 European countries, 45 statements reached consensus after 3 Delphi-rounds. The availability of high-quality clinical evidence was limited, and most statements were based on expert opinion. Another 25 statements did not reach consensus. CONCLUSIONS: Total colonic and total intestinal aganglionosis are rare variants of Hirschsprung disease, with very limited availability of high-quality clinical evidence. This consensus statement provides statements on the surgical treatment, management of poor bowel function and long-term management for these rare patients. The expert panel agreed that patients benefit from multidisciplinary and personalized care, preferably in an expert center. TYPE OF STUDY: Clinical consensus statement. LEVEL OF EVIDENCE: 3a.

Congenital Diaphragmatic Hernia: Perinatal Prognostic Factors and Short-Term Outcomes in a Single-Center Series.

Background: Many prognostic factors for CDH patients are described and validated in the current literature: the size of diaphragmatic defects, need for patch repair, pulmonary hypertension and left ventricular dysfunction are recognized as the most influencing outcomes. The aim of this study is to analyze the influence of these parameters in the outcome of CDH patients in our department and identify any further prognostic factors. Methods: An observational retrospective single-center study was conducted including all patients treated at our centre with posterolateral CDH between 01.01.1997 and 12.31.2019. The main outcomes evaluated were mortality and length of hospital stay. A univariate and multivariate analysis was performed. Results: We identified 140 patients with posterolateral CDH; 34.8% died before discharge. The overall median length of stay was 24 days. A univariate analysis confirmed that both outcomes are associated with the size of diaphragmatic defects, need for patch repair and presence of spleen-up (p < 0.05). A multivariate analysis identified that the need for patch repair and maximum dopamine dose used for cardiac dysfunction are independent parameters associated with the length of stay only (p < 0.001). Conclusions: In our series, the duration of hospitalization is longer for newborns with CDH treated with higher doses of dopamine for left ventricular dysfunction or needing patch repair in large diaphragmatic defects.

A survival analysis of cuffed tunneled silicon central venous catheters in children affected by short bowel syndrome: A lesson from the past.

BACKGROUND: Tunneled central venous catheters (CVC) are crucial in the management of children affected by short bowel syndrome (SBS). This work aims to investigate the outcomes of tunneled CVC and to identify factors influencing their survival. METHODS: All the children diagnosed with SBS and undergone a procedure of insertion of a tunneled CVC from 2010 to 2019 were included. Demographic data and surgical information about the procedures were collected. Regression models and Kaplan-Meier analysis were performed to estimate the survival. RESULTS: Eighteen patients, eight males (44%), with a median length of residual bowel measuring 72 cm (IQR 50-102 cm), were enrolled. Thirty-nine Broviac CVCs were inserted with a mean number of 2.2 CVCs per patient and 13365 line-days. The overall incidence of complications was 3.2/1000 line-days, and the incidence of central line associated bloodstream infections (CLABSI) was 1.1/1000 line-days. No episode of catheter thrombosis was reported. The median survival was 269 days (IQR 82-1814 days). The survival was negatively influenced by a younger age at insertion (R2 = 0.29; p < 0.001), 2.7 Fr diameter (median survival 76 days; p < 0.001) and the occurrence of complications (median survival 169 days; p = 0.002). The length of residual bowel was a mild risk factor for anticipated removal (OR 1.1; CI95 1.0-1.1; p = 0.05). CONCLUSION: CVC-related complications negatively influenced the survival of the line. An elder age at insertion together with a larger CVC diameter increased the survival of the line, while a shorter residual bowel was associated with an anticipated removal due to complications.

Complicated acute appendicitis in children: the importance of stewarding antibiotic prescriptions.

BACKGROUND: The aim was to assess the success of a three-drug regimen, consisting of cefazoline, metronidazole and gentamicine, for the antimicrobial treatment of complicated appendicitis and to investigate predictors of failure. METHODS: This retrospective study included patients who had undergone appendectomy for complicated appendicitis from 2013 to 2018. The shift to second-line antibiotics was considered a failure. The choice was based upon clinical deterioration. Patients were grouped into 2 groups: localized complicated appendicitis (LCA) and extensively complicated appendicitis (ECA) for the study purpose. Univariate and multivariate analysis were performed to identify predictors of failure. RESULTS: Ninety patients (65.2%) with LCA and 48 patients (35%) with ECA were included. Three-drug regimen failed in 50 patients (36%) with a higher rate in the ECA group (50%, p=0.017). In a multivariate analysis, this failure was found to be associated with ECA (adjusted OR 3.00 [1.2-7.4], p=0.041). Children with ECA experienced a longer hospital stay (median length 8 days, p < 0.001) and antimicrobial therapy (median length 8 days, p < 0.001). However, no difference in the rate of surgical site infections was found (p=0.514). CONCLUSIONS: The institutional antibiotic stewardship program highlighted a high failure rate for the old threedrug regimen. A new protocol should be recommended, especially for the patients affected by ECA.

Bowel function and associated risk factors at preschool and early childhood age in children with anorectal malformation type rectovestibular fistula: An ARM-Net consortium study.

BACKGROUND: Outcome of patients operated for anorectal malformation (ARM) type rectovestibular fistula (RVF) is generally considered to be good. However, large multi-center studies are scarce, mostly describing pooled outcome of different ARM-types, in adult patients. Therefore, counseling parents concerning the bowel function at early age is challenging. Aim of this study was to evaluate bowel function of RVF-patients at preschool/early childhood age and determine risk factors for poor functional outcome. METHODS: A multi-center cohort study was performed. Patient characteristics, associated anomalies, sacral ratio, surgical procedures, post-reconstructive complications, one-year constipation, and Bowel Function Score (BFS) at 4-7 years of follow-up were registered. Groups with below normal (BFS < 17; subgroups 'poor' ≤ 11, and 'fair' 11 < BFS < 17) and good outcome (BFS ≥ 17) were formed. Univariable analyses were performed to detect risk factors for outcome. RESULTS: The study included 111 RVF-patients. Median BFS was 16 (range 6-20). The 'below normal' group consisted of 61 patients (55.0%). Overall, we reported soiling, fecal accidents, and constipation in 64.9%, 35.1% and 70.3%, respectively. Bowel management was performed in 23.4% of patients. Risk factors for poor outcome were tethered cord and low sacral ratio, while sacral anomalies, low sacral ratio, prior enterostomy, post-reconstructive complications, and one-year constipation were for being on bowel management. CONCLUSIONS: Although median BFS at 4-7 year follow-up is nearly normal, the majority of patients suffers from some degree of soiling and constipation, and almost 25% needs bowel management. Several factors were associated with poor bowel function outcome and bowel management. LEVEL OF EVIDENCE: Level III.

Laparo-endoscopic combination for the safe extraction of an open safety pin in a 9-month-old child. Case report.

INTRODUCTION: and importance: Accidental ingestion of foreign bodies (FBs) is common among infants. In case of sharp FBs, the risk of accidental organ damage with potential life-threatening complications constitutes an absolute indication for removal. We present the case of a child, who, following the ingestion of an open safety pin, was successfully treated exclusively with minimally invasive techniques. CASE PRESENTATION: A 9-month-old male patient was admitted for hematemesis. An anteroposterior and lateral X-ray of the thorax and abdomen revealed the presence of an open safety pin in the epi-mesogastric region, without a precise localization. Upper and lower gastrointestinal endoscopy, fluoroscopy, and laparoscopy were combined in the same intervention to localize and safely remove the foreign body. The patient was dismissed on a postoperative day 1. CLINICAL DISCUSSION AND CONCLUSION: The two main pitfalls of this scenario were the initially uncertain location of the foreign body and the young age of the patient. A combination of different techniques was used to safely locate and remove the foreign body, reducing hospitalization and avoiding repeated radiological exposure. An experienced team in a tertiary paediatric surgical and endoscopic centre increases the chances of success and minimizes invasiveness and the risk of complications.

Bench thrombolysis and "autotransplantation" as a rescue treatment for venous thrombosis after living-donor kidney transplantation.

BACKGROUND: Allograft venous thrombosis is a severe complication after kidney transplantation (KT). Early diagnosis and prompt treatment are crucial in preserving the survival of the allograft. In this study, we aimed to describe an emergent strategy for the management of acute allograft venous thrombosis. CASE PRESENTATION: A 4-year-old girl, weighing 13.5 kg, was diagnosed with bilateral congenital renal hypodysplasia, urogenital sinus and anorectal malformation. The patient was referred to our department for living-donor KT. Her mother was eligible as a donor, presenting a body weight ratio of 1:4.5. Thrombosis of the inferior vena cava (ICV) was also identified, without any predisposing factor for thrombophilia. KT was performed by an extraperitoneal approach without complications. Venous anastomosis required a human vascular graft sutured to the ICV, and renal artery was anastomosed to the aorta. On postoperative day (POD) 8, acute abdominal pain and hematuria led to the diagnosis of an allograft venous thrombosis. An emergent laparotomy was required to explant the allograft, followed by bench surgery. The allograft was irrigated with thrombolytic agents and lactated Ringer's solution and then after removing the venous vascular graft, it was reimplanted through vascular anastomosis with the ICV and aorta. The recovery of perfusion and function was good with diuresis since day 4 after re-surgery. At 2-year follow-up, the child presented normal allograft function with an estimated GFR of 65 ml/min/1.73 m2 . CONCLUSION: According to our experience, explantation of the kidney allograft, followed by irrigation with thrombolytics in bench surgery, and reimplantation resulted in unexpected optimal outcomes in the case of allograft venous thrombosis.

Ultrasound-guided percutaneous ventriculopleural shunt placement: a minimally invasive technique.

OBJECTIVE: Ventriculopleural shunt is still considered a third-line option for CSF diversion, when both peritoneal and atrial cavity are contraindicated. Different approaches have been used and in modern surgery, lesser invasive techniques are predominant. The goal of this manuscript is to present a minimally invasive placement of a pleural catheter. METHODS: We describe a minimally invasive approach to the pleural space using an a-traumatic peel-away introducer under ultrasonographic intraoperative control. Furthermore, consideration about complications, follow-up and advantages of the abovementioned technique will be discussed. CONCLUSIONS: Percutaneous US guided placement for pleural catheter is a safer and modern minimally invasive approach to the pleural space. Pleural effusion is the predominant complication, encountered especially in younger children.

Video-assisted thoracoscopy in compromised pediatric patients.

The use of video-assisted techniques (VATs) in the paediatric field has become increasingly more frequent, based on reports of prompter recovery following VATs in respect to standard techniques. Specific advantages have been documented, in particular for pediatric patients undergoing chemioradiotherapic treatment. We retrospectively reviewed data of severely compromised patients who underwent VAT for lung wedge resections and biopsies carried out in our center over a 7-year period. As far as the area of therapeutic tumor resection is concerned, the present data are consistent with the view that thoracoscopy is both an effective and safe tool in diagnostic procedures.

Amniotic fluid stem cell migration after intraperitoneal injection in pup rats: implication for therapy.

PURPOSE: Despite being commonly used in clinical practice, the intraperitoneal (i.p.) route has been rarely used for cell delivery. We evaluated the capacity of amniotic fluid stem (AFS) cells, administered i.p., to diffuse systemically and to integrate into tissues of healthy newborn rats. METHODS: AFS cells were obtained from pregnant GFP + Sprague-Dawley rats by c-kit selection. Wild-type Sprague-Dawley newborn rats were divided into two groups receiving i.p.: (1) 2 x 10(6) AFS cells (n = 12); (2) of phosphate buffer saline (PBS) (n = 2) at 24 and 48 h after birth. Animals were either killed at 96 h of life, and organs collected for gfp amplification, or at 3 weeks of life and tissues isolated for green fluorescence protein (GFP) immunofluorescence. RESULTS: No adverse effects were observed after i.p. injection of PBS or AFS cells. Gfp was amplified in at least one organ in all rats injected with AFS cells except one (11/12). The intestine was the organ found most frequently positive (67%) followed by liver (25%), spleen (16%), heart (16%), lungs (16%), femur (8%) and brain (0%). Immunohistochemistry confirmed PCR results. CONCLUSION: In the short term, the i.p. administration of AFS cells, is a safe procedure and allows their migration, homing and integration into various organs of healthy newborn rats.

Captopril reduces the severity of bowel damage in a neonatal rat model of necrotizing enterocolitis.

BACKGROUND/PURPOSE: Selective mesenteric ischemia may result from activation of the renin-angiotensin system during periods of shock and is implicated in the pathogenesis of neonatal necrotizing enterocolitis (NEC). We investigated the effectiveness of captopril, an angiotensin-converting enzyme inhibitor, in reducing the severity of bowel damage in a neonatal rat model of NEC. METHODS: Necrotizing enterocolitis was induced by a combination of gavage feeding of hypertonic formula, hypoxia, and oral lipopolysaccharide (LPS). Rats were randomly divided into 3 groups: group A, control (breast fed; n = 20); group B, NEC (gavage/hypoxia/LPS; n = 31); group C, NEC with captopril 20 mg/kg per dose with the formula for 4 days (gavage/hypoxia/LPS/captopril; n = 35). Pups were killed after 4 days. Incidence of NEC was evaluated microscopically. RESULTS: Severity of bowel damage was higher in the NEC group compared to controls and was reduced by administration of captopril. Dilatation of the intestinal vasculature was observed in the captopril group. There were no cases of NEC in the controls; the incidence increased to 55% in NEC group and reduced to 29% by captopril. CONCLUSIONS: In this model of neonatal NEC, captopril supplementation of formula reduces the severity of intestinal damage and the incidence of NEC, presumably by affecting mesenteric blood flow.

[Antireflux surgery in neurologically impaired children]. / La plastica antireflusso nel paziente pediatrico neuroleso.

OBJECTIVE: To evaluate results of Nissen fundoplication in a neurologically impaired children population versus normal, considering symptoms improvement, general conditions, parents satisfaction, facility to assist the patient. METHODS: 57 patients were analysed, 38 neurologically impaired children (NL),19 neurologically normal children (NNL), that underwent fundoplication during six-year period (Feb '95-Dec '00). Mean age at surgery was 6,8 years (range 3 month- 18 years) for NL; 4,7 years for NNL (1 month- 15 years). We contact 31 family (19 NL, 12 NNL) before and after surgery and we subject them a questionnaire. For data analysis chi-squared test and Mann-Witney Rank Sum test has been used. RESULTS: A significantly greater reduction of the vomit and regurgitation have been observed in both groups (p<0.5). Drooling was significantly reduced only in the NNL group (p<0.5) than in the NL group. Cough doesn't improved significantly in both groups (p>0.5). Major respiratory symptoms and respiratory infections improved significantly only in NL group (p<0.5). The parents satisfaction is high and patients management results easier. CONCLUSIONS: The Nissen fundoplication in NL children is an effective procedure in order to obtain improvement on vomit; major respiratory symptoms and quality of caregivers management.