Autologous Intestinal Reconstruction Surgery in Short Bowel Syndrome: Which, When, and Why.

Short bowel syndrome (SBS), secondary to any natural loss or after any extensive bowel resection for congenital malformations or acquired disease, is the most common cause of intestinal failure in children. Extensive introduction of parenteral nutrition (PN) has dramatically changed the outcome of these patients, allowing for long-term survival. The main goal in children with SBS remains to be increasing enteral tolerance and weaning from PN support. Post resection intestinal adaptation allows for achievement of enteral autonomy in a subset of these patients, but the inability to progress in enteral tolerance exposes others to long-term complications of PN. Autologous intestinal reconstruction surgery (AIRS) can facilitate the fulfilment of enteral autonomy, maximizing the absorptive potential of the remaining gut. All the different intestinal reconstruction techniques, from simple procedures like tapering, reversed segments, and colon interposition, to more complex lengthening procedures (LILT: longitudinal intestinal lengthening and tailoring, STEP: serial transverse enteroplasty, and SILT: spiral intestinal lengthening and tailoring) and techniques designed for peculiar problems like controlled intestinal tissue expansion or duodenal lengthening are presented. AIRS indications, clinical applications, and results reported in the literature are reviewed.

Technique for replacement of Groshong® peripherally inserted central venous catheters (PICCs) in children.

BACKGROUND: The use of peripherally inserted central catheters (PICCs) in children is increasingly common due to their certain advantages and low rate of adverse events. Valved PICCs, when displaced, must be removed and new a PICC must be placed in another vein. We present our PICC replacement technique over a Seldinger guidewire using the same entrance vein. METHODS: This prospective study involved all consecutive patients with a previously inserted Groshong PICC (Bard Access Systems Inc., Salt Lake City, UT, USA) requiring replacement due to displacement managed at our institution between January 2018 and January 2019. All procedures were performed at the patients' bedside, under local anesthesia. All the steps of the procedure were described. RESULTS: The procedure was performed in 19 patients (mean 10.4 years, range 5-18 years) in a mean time of 12 minutes (range 10-35). In all cases, the maneuver was quick and painless. No complications were encountered during the procedure and at follow-up. CONCLUSIONS: Our preliminary experience suggests that the PICC replacement technique using the same entrance vein is simple, safe, and feasible, and provides clear benefits to preserve children's venous patrimony.

Endoscopic treatment of periampullary duodenal duplication cysts in children: Four case reports and review of the literature.

BACKGROUND: Duodenal duplications are rare congenital anomalies of the gastrointestinal tract. As the periampullary variant is much rarer, literature is scant and only few authors have reported their experience in diagnosis and treatment, particularly with operative endoscopy. CASE SUMARY: To report our experience with the endoscopic treatment in a series of children with periampullary duodenal duplication cysts, focusing on the importance of obtaining an accurate preoperative anatomic assessment of the malformations. The pediatric periampullary duodenal duplication cyst literature is reviewed. We conducted a systematic review according to the PRISMA guidelines. The PubMed database was searched for original studies on "duodenal duplication", "periampullary duplication" or "endoscopic management" published since 1990, involving patients younger than 18 years of age. Eligible study designs were case report, case series and reviews. We analyzed the data and reported the results in table and text. Fifteen eligible articles met the inclusion criteria with 16 patients, and analysis was extended to our additional 4 cases. Median age at diagnosis was 13.5 years. Endoscopic treatment was performed in 10 (50%) patients, with only 2 registered complications. CONCLUSION: Periampullary duodenal duplication cysts in pediatric patients are very rare. Our experience suggests that an accurate preoperative assessment is critical. In the presence of sludge or stones inside the duplication, endoscopic retrograde cholangio-pancreatography is mandatory to demonstrate a communication with the biliary tree. Endoscopic treatment resulted in a safe, minimally invasive and effective treatment. In periampullary duodenal duplication cyst endoscopically treated children, long-term follow-up is still necessary considering the potential malignant transformation at the duplication site.

Extended Adhesion-Sparing Liver Eversion during Kasai Portoenterostomy for Infants with Biliary Atresia.

BACKGROUND: Despite the fact that Kasai portoenterostomy (KPE) is the primary treatment for biliary atresia (BA), liver transplantation (LT) remains the ultimate surgery for two-thirds of these patients. Their true survival rate with the native liver reflects the original KPE and the burden of post-operative complications. We report an original modification of the adhesion-sparing liver eversion (ASLE) technique during KPE that facilitates the total native hepatectomy at time of transplantation. METHODS: All consecutive patients with BA who underwent KPE at our department and subsequent LT at Paediatric Liver Transplant Centre at Papa Giovanni XXIII Hospital between 2010-2018 were retrospectively enrolled. All patients underwent ASLE during KPE. Patients' demographic data, type of KPE, total transplant time (TTT), hepatectomy time (HT), intra-operative packed red blood cells and plasma transfusions, intra- and post-operative complications were noted. RESULTS: 44 patients were enrolled. Median TTT and HT were 337 and 57 min, respectively. The median volume of packed red blood cell transfusion was 95 mL. No patients presented bowel perforation during the procedure or in the short post-operative course. No mortality after LT was recorded. CONCLUSIONS: In addition to the well-known advantages of the standard liver eversion technique, ASLE reduces the formation of intra-abdominal adhesions, lowering significantly the risk of bowel perforation and bleeding when liver transplantation is performed for failure of KPE.

Hepatic hemangioendothelioma of infancy: clinical features of a large cohort of patients and proposed management.

PURPOSE: The management of hepatic hemangioendothelioma (HHE) may be challenging. We aimed to review a large cohort of children who presented to our centers with symptomatic HHE in the last 16 years. METHODS: We collected age at presentation, clinical features, histology, diagnostic process, management and outcome. RESULTS: Twenty seven patients (male/female 5/22), median age 13 days (1-1530) presented with hepatomegaly (24/27), cardiac failure (10/27), cutaneous hemangiomas (8/27), fever and anemia (6/27 each), vomiting (5/27), splenomegaly (4/27). The lesion was focal, multifocal, or diffuse in 9 patients of each group. The management included medical treatment (8/27), embolization (8/27), resection (3/27), observation (6/27), transplantation (2/27). After 16 months' follow-up (30 days-11 years), 23/27 (85%) were alive. Diffuse lesions (4/4), cardiac failure (4/4), type II histology (4/4), age older than 6 months at diagnosis (3/4) predicted mortality (all p < 0.01). Histology showed type 1 lesion in 3/8, type 2 in 3/8, and type 3 in 2/8 with foci of angiosarcoma. CONCLUSION: Most patients with symptomatic HHE can be managed successfully with a combination of medical, radiological and surgical treatments. Patients with diffuse lesions, late presentation, cardiac failure and type II histology have a poor outcome. LEVEL OF EVIDENCE: Diagnostic level IV. Therapeutic level IV.

The coagulation profile as a marker for acute appendicitis in the paediatric population: Retrospective study.

INTRODUCTION: Appendicitis is the commonest and most frequently misdiagnosed acute abdominal surgical illness in the paediatric population worldwide. The aim of this study is to evaluate the role of coagulation profile in acute appendicitis (AA) in children. MATERIALS AND METHODS: we retrospectively collected data of patients submitted to appendectomy from 2011 to 2017. According to histopathology, patients were divided into three groups: not histologically confirmed AA (NAA), simple AA (SAA) and complicated AA (CAA). White blood cell (WBC) count, relative neutrophilia (Neutr%), C-reactive protein (CRP), prothrombin time ratio (PTratio), activated partial thromboplastin time ratio (APTTratio) and fibrinogen (Fib) were compared among groups. RESULTS: Three hundred and seven patients were included: 57 NAA, 184 SAA and 66 CAA. WBC was significantly different among groups: CAA (mean 16.67 × 103/ml), SAA (14.73 × 103/ml, P= 0.01) and NAA (10.85 × 103/ml, P< 0.0001). Significant differences were found for Neutr% (mean CAA 81.14 vs. SAA 77.03 P= 0.006, vs. NAA 63.86 P< 0.0001) and CRP (mean NAA 2.56, SAA 3.26, CAA 11.58, P< 0.0001). PTratio and Fib increased with the severity of AA receiver operator characteristic curves were similar for CRP (0.739), Fib (0.726), WBC (0.746) and Neutr% (0.754), while for PTratio and aPTTratio were 0.634 and 0.441, respectively. CONCLUSIONS: extrinsic coagulation pathway is altered in AA, especially in CAA. Coagulation can be useful in the diagnostic and perioperative anaesthetic management of AA in children. Fib seems to have the highest accuracy.

Feasibility of a Standardized Management for Primary Spontaneous Pneumothorax in Children and Adolescents: A Retrospective Multicenter Study and Review of the Literature.

Introduction: The management of primary spontaneous pneumothorax (PSP) in children still remains controversial. The current literature shows a lack of scientific evidence supporting a standardized management. Materials and Methods: A multicenter retrospective study was performed, patients admitted for PSP in five Pediatric Surgical Units over a 9-year period (from 2008 to 2017) were included. Patient data were reviewed for each case. Management and outcomes were statistically analyzed. Results: Overall 159 patients (135 males and 24 females) were included in this study. During the first hospital admission, 62/159 patients (39%) were conservatively managed with oxygen therapy; 95/159 patients (60%) required a chest drain insertion; 2/159 patients (1%) underwent surgery because of a bilateral pneumothorax. Thoracoscopy was performed in 75/159 (47%) patients after the second hospital admission or for persistent air leak during the first access. Postoperatively, 8/75 (11%) patients developed recurrent pneumothorax requiring chest drain insertion in 3/8 (37%) cases or aspiration in 1/8 (13%), whereas 4/8 (50%) patients were conservatively managed. All of the patients but one, were confirmed to be affected by emphysematous-like changes at histology. Conclusions: Despite the limitations of being a retrospective study, we suggest that the early surgical management in children with PSP is feasible and safe and it seems to significantly reduce the recurrence rate. To the best of our knowledge there are no pediatric guidelines for the management of PSP, therefore, we support the need for prospective studies to create the evidence-based pillars for correct and standardized management of this condition.

Total Oesophagogastric Dissociation in Neurologically Impaired Children: 18 Years' Experience and Long-term Follow-up.

OBJECTIVES: Total oesophagogastric dissociation (TOGD) is an alternative antireflux surgery for neurologically impaired children because of a 16% to 38% fundoplication failure rate. This study evaluates TOGD's feasibility and its long-term efficacy both as a Primary and as a "Rescue" procedure after failed fundoplication. METHODS: Thirty patients (18 boys) who underwent TOGD between 2000 and 2018 in 2 Italian Centres were retrospectively reviewed. Twenty-three were Primary procedures and 7 were "Rescue" ones. Inclusion criteria were severe neurodisability, intractable gastroesophageal reflux, and dysphagia. RESULTS: Preoperatively, all children had regurgitation, vomiting or retching, and 93% had unsafe swallowing and aspiration, with recurrent chest infections/aspiration pneumonia. Median relative weight was 77% (48%--118%). All patients were taking antireflux medication before surgery. Median age at TOGD was 6.48 years (0.69--22.18). Median follow-up was 3.5 years (0.6-17.7). No recurrence of gastroesophageal reflux (GER) and vomiting was recorded. The number of chest infections and length of hospital stay showed a significative decrease (P value <0.0001 for both), whereas median relative weight reached 101% (P value 0.002). Parents'/caregivers' perception of outcome showed a significative improvement. Six patients (20%) experienced early complications and 3 required surgical intervention. Three late complications (10%) also required surgery. There was no surgery-related mortality. CONCLUSION: TOGD is an effective procedure with an acceptably low complication rate for children with severe neurological impairment and is followed by a major improvement in general health and quality of life for children and families. There was no substantial difference in outcome between Primary and "Rescue" procedures.

Correction to: Acute epididymo-orchitis due to Salmonella Typhi in a young man from Bangladesh.

The original version of this article unfortunately contained a mistake. Arnaldo Caruso was not listed among the authors.

Acute epididymo-orchitis due to Salmonella Typhi in a young man from Bangladesh.

S. typhi infection rarely involves the genitourinary system. We report the first described case of acute epididymo-orchitis due to S. typhi in a 14-year-old boy from Bangladesh. A high index of suspicion should be maintained when evaluating patients coming from endemic countries also in case of unusual sites of infection.

Adjuvant therapy of cytomegalovirus IgM + ve associated biliary atresia: Prima facie evidence of effect.

AIM OF STUDY: CMV-IgM + ve associated biliary atresia (CMV-BA) is a distinct etiological subgroup characterized by older age at presentation and a greater degree of inflammation and hepatic fibrosis, leading to a worse outcome. We report our experience with adjuvant antiviral therapy after Kasai portoenterostomy (KPE). METHODS: Single-center prospective database identification of CMV-IgM + ve associated BA managed between 2003 and 2017. Since 2011, IV ganciclovir (5 mg/kg b.d.) and/or oral valganciclovir (520 mg/m2 b.d.) were started in the early postoperative period in selected cases and continued until negativity of CMV DNA load [Anti-Viral Therapy (AVT) Group 1]. Clearance of jaundice was defined as achieving a total bilirubin ≤20 µmol/L in post-KPE period and tested with a Fisher test; native liver survival (NLS) and overall actuarial survival (OS) were compared with untreated BA CMV IgM + ve patients (Group 2) using a Log-Rank test. A P value of <0.05 was regarded as significant. Data are quoted as median (IQ range). RESULTS: During the 14-year period, 376 infants with histologically confirmed BA were treated; of those 38(10%) were CMV IgM + ve at presentation. One child was considered too late at presentation for KPE and underwent primary liver transplantation while another only started AVT one month after KPE. Both were excluded from survival analysis. Therefore 36 underwent KPE [AVT Group 1 (n = 8) and Control Group 2 (n = 28)]. Overall age at surgery was 67(53-77) days. There was no difference in age at surgery (P = 0.26); bilirubin (P = 0.12); or AST (P = 0.15) between Group 1 and Group 2. Viral load data were available in 16 with a trend towards higher counts in the AVT group 1 [4935 (2668-18,817) vs. 1296 (253-10,471) c/ml; P = 0.06]. Clearance of jaundice was higher in AVT Group 1 (75% vs 21%, P = 0.009). There was no difference in OS (P = 0.24) but NLS was improved in the AVT Group 1 (75% vs. 25% at 2 years; P = 0.04). CONCLUSIONS: Although this finding may be regarded as preliminary, adjuvant antiviral therapy appeared to improve outcome in infants with CMV IgM + ve BA. LEVEL OF EVIDENCE: III.

Biliary atresia: 20-40-year follow-up with native liver in an Italian centre.

INTRODUCTION: Biliary atresia (BA) represents the leading indication for liver transplantation in childhood. Only few studies reported the outcome of patients who survived more than 20 years on their native liver, and up to date there are no Italian data available. We reported our 40-year single centre experience with long-term follow-up of BA patients. MATERIALS AND METHODS: All consecutive patients who underwent Kasai portoenterostomy (KPE) for BA managed at our Institution between 1975 and 1996 were retrospectively reviewed. Native liver (NLS) and overall survival (OS) were analyzed with Kaplan-Meyer curves and LogRank test. A p value of <.05 was regarded as significant. Quality of life of patients currently surviving with their native liver was assessed through a quality of life questionnaire. RESULTS: During the 22-year period of the study 174 patients underwent surgery (median age 60 days). Clearance of jaundice at 6 months from surgery was achieved in 90 patients (51.7%). NLS was 41% at 5 years, 32% at 10 years, 17.8% at 20 years and 14.9% at 40 years. Cholangitis was recorded in 32%, hepatocellular carcinoma in 0.5%. Twenty-six patients (14.9%) survived with their liver more than 20 years; 84.6% had normal serum bilirubin level and 23% had esophageal varices. Quality of life was comparable with the healthy Italian population in all but one patient. CONCLUSIONS: Our Italian experience confirms KPE represents the cornerstone of treatment for children with BA. Multidisciplinary and meticulous lifelong post-operative follow-up should be guaranteed for these patients because of the possibility of late-onset cholangitis, portal hypertension, hepatic deterioration and liver malignant tumors. TYPE OF THE STUDY: retrospective case series. LEVEL OF EVIDENCE: IV.

Intrahepatic bile duct primary cilia in biliary atresia.

AIM: The etiopathogenesis of non-syndromic biliary atresia (BA) is obscure. The primary aim was to investigate intrahepatic bile duct cilia (IHBC) in BA at diagnosis and its correlation with clinical outcome. The secondary aim was to analyze IHBC in routine paraffin-embedded liver biopsies using conventional scanning electron microscopy (SEM). METHODS: Surgical liver biopsies taken at diagnosis from 22 BA infants (age range, 39-116 days) and from eight children with non-BA chronic cholestasis (age range, 162 days -16.8 years) were evaluated for IHBC by immunofluorescence (IF) and SEM. A minimum 18-month follow-up after surgery was available for all patients. RESULTS: By IF, cilia were present in 6/8 (75%) non-BA but only in 3/22 (14%) BA cases, and cilia were reduced or absent in 19/22 (86%) BA and 2/8 (25%) non-BA livers (P < 0.01). In BA, cilia presence was found to be associated with clearance of jaundice at 6-month follow-up (P < 0.05). However, high overall survival rates with native liver, >90% at 12 months, and >70% at 24 months post-surgery, were recorded regardless of cilia presence/absence at diagnosis. Electron microscopy was able to detect bile ducts and cilia in routine liver biopsies, revealing significant abnormalities in 100% BA livers. CONCLUSIONS: The presence of IHBC in BA livers at the diagnosis was associated with resolution of cholestasis, although was not predictive of short-term survival with native liver. Scanning electron microscopy represents a powerful new tool to study routine liver biopsies in biliary disorders. Cilia dysfunction in BA pathogenesis and/or disease progression warrants further investigation.

Use of peripherally inserted central venous catheters (PICCs) in children receiving autologous or allogeneic stem-cell transplantation.

INTRODUCTION: The aim of our study was to present our experience with the use of peripherally inserted central catheters (PICCs) in pediatric patients receiving autologous or allogenic blood stem-cell transplantation. The insertion of the device in older children does not require general anesthesia and does not require a surgical procedure. METHODS: From January 2014 to January 2017, 13 PICCs were inserted as a central venous device in 11 pediatric patients submitted to 14 autologous or allogeneic stem-cell transplantation, at the Bone Marrow Transplant Unit of the Children's Hospital of Brescia. The mean age of patients at the time of the procedure was 11.3 years (range 3-18 years). PICCs remained in place for an overall period of 4104 days. All PICCs were positioned by the same specifically trained physician and utilized by nurses of our stem-cell transplant unit. RESULTS: No insertion-related complications were observed. Late complications were catheter ruptures and line occlusions (1.2 per 1000 PICC days). No rupture or occlusion required removal of the device. No catheter-related venous thrombosis, catheter-related bloodstream infection (CRBSI), accidental removal or permanent lumen occlusion were observed. Indications for catheter removal were completion of therapy (8 patients) and death (2 patients). Three PICCs are currently being used for blood sampling in follow-up patients after transplantation. CONCLUSIONS: Our data suggest that PICCs are a safe and effective alternative to conventional central venous catheters even in pediatric patients with high risk of infectious and hemorrhagic complications such as patients receiving stem-cell transplantation.

"Trident" preputial plasty for phimosis in childhood.

OBJECTIVE: To evaluate the functional and cosmetic result of modified-triple incision preputial plasty for surgical management of phimosis in children. MATERIALS AND METHODS: This prospective study involved all consecutive children (age 3-15) with phimosis, managed at our Institution in a one-year period. All patients unable to retract the foreskin and symptomatic with episodes of posthitis or balanoposthitis or ballooning of the foreskin with voiding were initially included. After a two-month trial of topic corticosteroids treatment, for patients refractory to conservative ointment treatment, "trident" preputial plasty was offered. This procedure combines two lateral Z-plasty and one middle Y-V plasty. All the procedures were performed as a day care, in general anaesthesia by two senior surgeons. Outpatient follow-up controls were scheduled at 1 and 2 week, 1, 6 and 12 months after surgery, respectively. RESULTS: A total of 41 patient were enrolled. Mean operative time was 24 minutes (range 15-43). At 12-month follow-up, all but one patients (97.6%) was able to retract the prepuce. Cosmetic and functional results were satisfactory. CONCLUSION: In selected group of children with phimosis, the "trident" preputial plasty provided excellent cosmetic and functional result. Adequate retraction of the prepuce by the patients soon after surgery is mandatory.