Organizational aspects of pediatric anesthesia and surgery between two waves of Covid-19.

BACKGROUND: The initial wave of the Covid-19 pandemic has hit Italy, and Lombardy in particular, with violence, forcing to reshape all hospitals' activities; this happened even in pediatric hospitals, although the young population seemed initially spared from the disease. "Vittore Buzzi" Children's Hospital, which is a pediatric/maternal hospital located in Milan (Lombardy Region), had to stop elective procedures-with the exception of urgent/emergent ones-between February and May 2020 to leave space and resources to adults' care. We describe the challenges of reshaping the hospital's identity and structure, and restarting pediatric surgery and anesthesia, from May on, in the most hit area of the world, with the purpose to avoid and contain infections. Both patients and caregivers admitted to hospital have been tested for Sars-CoV-2 in every case. METHODS: Observational cohort study via review of clinical charts of patients undergoing surgery between 16th May and 30th September 2020, together with SARS-CoV -2 RT-PCR testing outcomes, and comparison to same period surgeries in 2019. RESULTS: An increase of approximately 70% in pediatric surgeries (OR 1.68 [1.33-2.13], P < .001) and a higher increase in the number of surgeries were reported (OR 1.75 (1.43-2.15), P < .001). Considering only urgent procedures, a significant difference in the distribution of the type of surgery was observed (Chi-squared P-value < .001). Sars-CoV-2-positive patients have been 0.8% of total number; 14% of these was discovered through caregiver's positivity. CONCLUSION: We describe our pathway for safe pediatric surgery and anesthesia and the importance of testing both patient and caregiver.

Developmental histology of the portal plate in biliary atresia: observations and implications.

PURPOSE: The key characteristic of biliary atresia (BA) is obliteration of the extrahepatic bile ducts at the level of the porta hepatis. We aimed to relate the immunohistochemical features of remnant biliary ductules at the porta hepatis with clinical features and outcomes. METHODS: Samples were immunostained with anti-cytokeratin 20 (CK20), vimentin and alpha-smooth muscle actin (aSMA). Primary outcome was set as clearance of jaundice (bilirubin ≤ 20 µmol/L) following Kasai portoenterostomy (KPE). RESULTS: Eighty-two cases were classified into syndromic BA (n = 10), cystic BA (n = 7), CMV IgM+ BA (n = 9) and isolated BA (n = 56). CK20 expression was confirmed in 40/82 (49%), and vimentin expression in 19/82 (23%). aSMA was negative in all cases studied. CK20 expression was less common in isolated BA (n = 20/56, 36%) compared to CMV IgM+ BA (n = 8/9, 89%), cystic BA (n = 7/7, 100%) (isolated BA vs non-isolated BA, P = 0.0008). There was no difference in vimentin expression among the sub-groups (isolated BA vs. non-isolated BA; P = 0.39). CoJ was achieved in 52/82 (63%) overall with significant difference depending simply on sub-group [e.g. syndromic BA 9/10 (90%)]. CK20 expression was associated with a diminished rate of CoJ in the entire cohort [CK20+ 32/56 (57%) vs. CK20- 20/26 (77%); P = 0.04]. By contrast no correlation was observed between vimentin expression and CoJ (P = 0.13). CONCLUSION: CK20+ expression was associated with reduced clearance of jaundice in BA and a trend towards reduced native liver survival.

GERD surgery in non-neurologic patients: Modified Laparoscopic Hill-Snow Repair is a valid alternative to Nissen fundoplication. Results of a 20 years of follow-up.

Nowadays laparoscopic Nissen fundoplication represents the gold standard in surgical treatment of complicated Gastro-Esophageal-Reflux Disease (GERD), above all in cerebral palsy patients. In non-neurological patients without gastrostomy Nissen fundoplication can create some problems (gas bloat syndrome, dysphagia). Laparoscopic Hill-Snow repair is an established surgical alternative, but it is reported only in adult population. We describe our modification of Hill-Snow technique and our experience in a large series of non-neurological children in order to report its effectiveness and applicability in pediatric patients affected by complicated GERD. Between 2000 and 2022, 319 children underwent surgical correction of gastro-esophageal reflux at our Department. All were affected by complicated gastro-esophageal reflux unresponsive to PPI (Proton Pump Inhibitors). 251 underwent laparoscopic Nissen fundoplication; 68 non-neurological patients underwent laparoscopic Hill-Snow repair. Of these 68 children 48 were males (71%) and 20 females (29%); median age was 5years (3 months-11 years). Weight range was 4-37kg. 52 patients (76.5%) presented the following symptoms: retrosternal pain, dysphagia, regurgitation, coughing, failure to thrive, persisting reflux esophagitis. 16 (23.5%) had chronic respiratory problems (aspiration, apneic-spells, dysphagia, coughing, choking, gagging). For 8 (11.8%) symptoms were expression of chronic recurrent gastric volvulus. All underwent modified-laparoscopic-Hill-Snow repair. Contrast study showed sliding hiatal hernia in 55 patients (81%), while endoscopy demonstrated 16 cases of histologically severe esophagitis (23.5%) and 52 of mild esophagitis (76.5%). No intraoperative/postoperative complications were recorded. 60patients had a complete follow-up (range 1-20 years). 60/68 patients were evaluated with barium-swallow-study at 6-12 months; 40/68 patients with upper-gastrointestinal-endoscopy at 12months. No relapse was reported. 50 patients (73.7%) were symptom-free. 18 (26.3%) referred occasional epigastric pain, associated with vomit in 2 cases. 64 (94.1%) referred ability to vomit; 4 temporary difficulty to swallow (average 30 days). All patients reported being able to burp. 3(4.5%) presented episodes of gas-air-bloat during the first 2 months with spontaneous resolution. No case of dumping syndrome was recorded. This technique's modification yields excellent results in term of relapse and side effects at long-term follow-up. We reported the first and largest pediatric series in non-neurological children with encouraging results.

Surgical Approach of Wandering Spleen in Infants and Children: A Systematic Review.

Introduction: Splenopexy has been proposed as the treatment of choice in case of wandering spleen (WS). We report our experience and review the current literature focusing on surgical management and outcomes of children affected by WS. Materials and Methods: Data regarding demographics, clinical manifestations, diagnosis, and treatment of children treated for WS at our Institution were analyzed. Systematic review was registered on Prospero (CRD42018089971). Scientific databases were searched using defined keywords. Articles were selected using predefined exclusion and inclusion criteria. Analysis was conducted adding our center's cases. Results: One hundred sixty-six articles were included in the review, 197 cases were analyzed, 3 of which unpublished. Female/male ratio was 1.5:1 and median age at diagnosis was 8 years. Most frequent clinical manifestation was isolated abdominal pain (42.6%). Torsion of splenic pedicle was diagnosed in 56.3%. Among surgical procedures, 39% underwent splenopexy and 54.8% underwent splenectomy. In case of splenopexy, the most commonly used techniques were using of a mesh (45.5%) or creation of a retroperitoneal pouch (30.9%). In 48.2% of splenopexies, minimally invasive surgery (MIS) was used. Splenopexy was effective in 94.8% (88% considering only cases with a spleen torsion). Conclusion: WS is a rare condition potentially leading to torsion of the spleen. This entity has to be kept in mind as a differential diagnosis in case of abdominal pain. Splenopexy should be the treatment of choice; its success rate in terms of preserved spleens can be affected by the presence of a torted organ. Retroperitoneal pouch or mesh fixation are the most preferred techniques. Authors recommend MIS approach.

Retroperitoneoscopic Nephrectomy in Pediatric Patients.

Minimally invasive surgery (MIS) in pediatric population is becoming more and more popular among pediatric urologists and now it can be considered the best treatment for nephroureterectomy that is one of the most frequent urological procedures. The main indications to MIS ureteronephrectomy in children are congenital or acquired benign conditions such as nonfunctional and/or damaging kidney, renal dysplasia, and destroyed kidneys due to obstructive or refluxing uropathy. Since ever in open urological surgery, the lateral retroperitoneal approach represents the typical way to approach this kind of surgery. Although different surgical approaches can be chosen (i.e., posterior retroperitoneal, lateral retroperitoneal, laparoscopic transperitoneal, and robotic approach), the lateral retroperitoneal approach represents the typical way to perform this kind of surgery, also with minimally invasive techniques. In this article, we illustrate the technique of retroperitoneoscopic nephroureterectomy in infants and children, reporting our experience with this technique in the past 20 years.

Effectiveness of Articulating Linear Stapler for Total and Partial Laparoscopic Splenectomy in Children.

Purpose: We performed a retrospective study to determine the effectiveness and feasibility of articulating linear stapler in laparoscopic total splenectomy (LTS) and laparoscopic partial splenectomy (LPS), focusing on technical laparoscopic skills that could help pediatric surgeons to avoid intra- and postoperative complications. Methods: Retrospective cohort study of children younger than 18 years who underwent laparoscopic spleen surgery between January 2008 and March 2020. Age, sex, indication for surgery, operative time (OT), intra- and postoperative complications, and postoperative length of hospital stay (LHS) were analyzed. Data from parenchymal resection and vessels sealing techniques were obtained. Results: Thirty patients, 19 LTS and 11 LPS, were included. The mean age of the patients was 10.9 years, and 16 patients were male and 14 were female. For hematologic diseases, LTS was the elective surgery, associated with cholecystectomy in 5 cases. LPS was the common procedure for splenic cysts. The stapler was used in LTS to close the hilum vessels and in LPS for parenchymal resection. No statistically significant differences in OT were observed comparing LTS and LPS. Two conversions occurred in LTS; none in LPS. The mean LHS was 6 days in both groups. No recurrence or major complications appeared in both groups at 1-12 years of follow-up. In particular for LPS, there are no relapse of cyst neither reduction in splenic function. Conclusions: This study shows the effectiveness, feasibility, and safety of mechanic stapler in splenic surgery both for hilum vessels sealing and for parenchymal resection. The use of this device can reduce risk of hemorrhagic recurrences or major surgical complications improving the safety of the operation.

Appendicitis in Children in a Large Italian COVID-19 Pandemic Area.

Introduction: The coronavirus disease 2019 (COVID-19) pandemic has dramatically changed the routine activities of pediatric surgical centers, and it determined the reduction of admissions in the pediatric emergency departments (PED). We reviewed the records of patients affected by acute appendicitis (AA) during the COVID-19 pandemic period in a large Italian COVID-19 pandemic area. Methods: Data regarding demographics, age, macroscopic and microscopic findings, and time between symptom onset and PED admission of patients affected by confirmed AA in the period between March and April 2020 were considered. The data were compared with those obtained during the same period of 2019, 2018, and 2017 in the included centers. Data were quoted as median (range) or absolute number. Non-parametric statistical tests were used to compare groups. A p ≤ 0.05 was regarded as significant. Since only anonymous data have been used and the data storage meets current data protection regulations, ethical committee approval was not required for this study. Results: Eighty-six patients underwent surgical appendectomy for AA between February 20th, 2020 and April 20th, 2020; 32.5% were complicated appendicitis and 67.5% were uncomplicated. Fifty-three patients were males and 33 were females. Patients' age ranged from 3 to 17 years and the median age was 10 years. The median time between the onset of symptoms and the admission in PED was 1.85 days. The average time between the symptom onset and PED admission was 1.8 days. Conclusions: Although fear from the COVID-19 pandemic determined a delayed diagnosis of serious pediatric diseases, the increasing prevalence and severity of AA were not demonstrated in the most COVID-19-affected areas of Italy.

Esophageal Atresia: Nutritional Status and Energy Metabolism to Maximize Growth Outcome.

BACKGROUND: Long-term negative sequelae of esophageal atresia (EA) may induce poor growth and impaired nutritional status in childhood. We describe the nutritional profile and energy metabolism of children with repaired EA to identify malnutrition risk factors and optimize growth management. METHODS: Twenty-one children (>4 years) were included, and anthropometric measurements, nutritional assessment, and energy metabolism were considered. The subjects were defined as undernourished if they met BMI < -2 standard deviation (SD). To grade undernutrition, we defined the prevalence of underweight, stunting, and wasting (cut-off level of <-2 SD). Medical records were reviewed for the type of EA and surgery and perinatal data. RESULTS: Malnutrition was detected in 28.6% of children. Underweight was detected in 23.8% of patients (all with undernutrition p < 0.01). Wasting was noted in 28.6% of patients, of these 5 children were undernourished (p < 0.001) and stunting was noticed in only one patient with malnutrition (p = 0.5). Resting expenditure energy (REE) was lower in undernourished subjects compared to subjects with adequate nutritional status (p < 0.001). Malnutrition was associated to: type of EA (p = 0.003, particularly type A and C); intervention including deferred anastomosis due to long-gap repair (p = 0.04) with/or without jejunostomy (p = 0.02), gastric pull-up (p = 0.04), primary anastomosis (p = 0.04), pyloromyotomy in long-gap (p < 0.01); small for gestational age condition (p = 0.001). CONCLUSIONS: undernutrition risk factors, beyond the type of malformation, surgery, and perinatal factors, must be early considered to personalize nutritional programming. Energy metabolism is important to monitor the nutritional requirements. The management of nutritional issues is surely a contributory factor able to counteract the poor growth of children with EA.

Effects of an antimicrobial stewardship intervention on perioperative antibiotic prophylaxis in pediatrics.

Purpose: This study aims to determine the effectiveness of an Antimicrobial Stewardship Program based on a Clinical Pathway (CP) to improve appropriateness in perioperative antibiotic prophylaxis (PAP). Materials and methods: This pre-post quasi-experimental study was conducted in a 12 month period (six months before and six months after CP implementation), in a tertiary Pediatric Surgical Centre. All patients from 1 month to 15 years of age receiving one or more surgical procedures were eligible for inclusion. PAP was defined appropriate according to clinical practice guidelines. Results: Seven hundred sixty-six children were included in the study, 394 in pre-intervention and 372 in post-intervention. After CP implementation, there was an increase in appropriate PAP administration, as well as in the selection of the appropriate antibiotic for prophylaxis, both for monotherapy (p = 0.02) and combination therapy (p = 0.004). Even the duration of prophylaxis decreased during the post-intervention period, with an increase of correct PAP discontinuation from 45.1 to 66.7% (p < 0.001). Despite the greater use of narrow-spectrum antibiotic for fewer days, there was no increase in treatment failures (10/394 (2.5%) pre vs 7/372 (1.9%) post, p = 0.54). Conclusions: CPs can be a useful tool to improve the choice of antibiotic and the duration of PAP in pediatric patients.

"Rendezvous" Procedure in Children with Cholecysto-Choledocholithiasis.

Introduction: Cholecysto-choledocolithiasis is a rare entity in children and its management is still challenging and controversial. The laparoendoscopic rendezvous (LERV) procedure, consisting of laparoscopic cholecystectomy and simultaneous endoscopic retrograde cholangiopancreatography for the management of symptomatic choledocholithiasis is well described in adult patients. However, in the literature, few reports about its application in the pediatric population have been recorded. Aim of the Study: The aim of the present study is to report our first successful cases of symptomatic cholecysto-choledocholithiasis LERV treatment. Methods: Two girls suffering of hemolytic disease presented to our third referral center with acute abdominal pain due to cholecysto-choledocholithiasis. Preoperative, perioperative, and postoperative data were retrospectively reviewed. Results: Surgery was performed without complications. The girls were dismissed once re-alimentation and re-canalization were achieved and had no other episodes of biliary cholic. Reported advantages of LERV include: a shorter in-hospital stay, a reduction in the number of procedures and anesthesia, and a reduced overall risk of complications. Conclusions: The promising result with our 2 cases suggest that, when performed in highly specialized centers, LERV is a safe procedure, which leads to considerable benefits, despite logistic and organizational difficulties.

Congenital choledochal malformation: search for a marker of epithelial instability.

PURPOSE: There is a predisposition to the development of malignancy in congenital choledochal malformation (CCM) although the degree of risk is unknown. We investigated the role of CA19-9 in bile and the MIB-1 (Ki-67) epithelial proliferation index as markers of an at risk choledochal epithelium at the time of definitive surgery. METHODS: Bile collected at surgery was analyzed for levels of amylase (as a surrogate of pancreatic reflux) and CA19-9. Immunohistochemical staining for CA19-9 and MIB-1 index (expressed as %) was performed on resected specimens. Data are quoted as median (IQR) and differences assessed using non-parametric statistics. A P value of 0.05 was regarded as significant. RESULTS: Our study group consisted of 78 children with CCM (Type 1 fusiform, n=34; Type 1 cystic, n=30 and Type 4, n=14). Median bile CA19-9 was 159,400 (6-1×10(6)) kU/L. There was no correlation with bile amylase (P=0.49) or biliary pressure (P=0.17) but modest correlation with bilirubin (rs=0.24; P=0.02). In contrast, bile amylase was correlated with plasma γ-glutamyl transpeptidase (P=0.02), alkaline phosphatase (P=0.05) and aspartate aminotransferase (P=0.02); and inversely correlated with biliary pressure (rs=-0.38; P<0.0008). Epithelial expression of CA19-9 and MIB-1 was assessed in 43 specimens. CA19-9 was diffusely expressed on all choledochal epithelium. MIB-1 expression was divided into: high expression (>40%) n=3; moderate (20-40%) n=5, low (6-20%) n=7 and very low (≤5%) n=28. There was no correlation with choledochal pressure (P=0.87), CA19-9 (P=0.51) or bile amylase (P=0.55). CONCLUSION: Biliary CA19-9 levels were grossly (and unexpectedly) raised in choledochal malformation and appear to arise from biliary rather than pancreatic epithelium. MIB-1 confirms that a small proportion (19%) has marked epithelial proliferation but no clinical correlates could be identified.

Vaginal replacement in the pediatric age group: a 34-year experience of intestinal vaginoplasty in children and young girls.

BACKGROUND/PURPOSE: The absence of vagina is rare in the pediatric population. It can occur as a result of congenital malformations such as an aplasia of mullerian ducts (46,XX Mayer-Rokitansky-Küster-Hauser syndrome) or a complete androgen insensitivity syndrome (46,XY testicular feminizing syndrome). Intersex patients, who underwent reassessment of a female sex, need a genital reconstruction toward a feminine phenotype. Patients with congenital adrenogenital syndrome with high urogenital sinus could have a severe hypoplastic vagina. In all these cases, a vaginal replacement is required. We reviewed our experience of vaginal replacement using a sigmoid conduit. METHODS: In 34 years, we evaluated 47 patients. The observation period was from 1 to 34 years (mean: 12 years). The preoperative diagnosis was Mayer-Rokitansky-Küster-Hauser syndrome in 17 cases, androgen insensitivity syndrome in 24 cases, adrenogenital syndrome with high urogenital sinus in 5 and 1 patient was affected by penile agenesis. Forty-six patients were treated with vaginal reconstruction by interposition of sigmoid colon. Only in 1 case we performed a vaginal construction with an ileal loop: in this case, the sigmoid colon was extremely dilated by a chronic constipation secondary to a high anorectal malformation corrected at birth. RESULTS: The outcome for 47 patients is excellent: 18 are sexually active and 4 are married. Only 1 patient with adrenogenital syndrome died of endocrine problems. Complications occurred in 17 cases: in 1 patient a necrosis of the replaced vagina occurred, thus requiring vaginal exeresis; now she is waiting for a second operation. Another patient had an abdominal abscess, which was surgically treated. In 12 cases a second procedure was required: 6 had stenotic new-vaginal introitus, 4 had new-vaginal prolapse, and 2 had intestinal obstruction. CONCLUSIONS: We believe that the preferable technique for vaginal replacement is the use of intestinal conduit. The sigmoid colon is the best intestinal tract to be used owing to its size, location and preserved blood supply. Our experience leads us to believe that the sigmoid segment is the segment of choice, although we consider ileal loop as a good alternative when the sigmoid colon is not available.