Delayed diagnosis in Hirschsprung disease.

INTRODUCTION: Diagnostic delay in Hirschsprung disease is uncommon. Different definitions have been proposed but that of a diagnosis achieved after 12 months of age seems to be the most reliable and resorted to. Some authors reported a worse outcome in case of delay. Our study aims at providing the most relevant features of a series of patients who received a delayed diagnosis of Hirschsprung disease. MATERIALS AND METHODS: All consecutive patients admitted to our Center with a delayed diagnosis of Hirschsprung diseases between January 2017 and July 2023 have been retrospectively enrolled. Demographic data, phenotype, genotype, surgical complications, and outcome were assessed and compared to those of literature. A number of variables were also compared to those of a series of patients admitted during the same study period without a delayed diagnosis. RESULTS: A total of 45 patients were included (16.4% out of a series of 346 patients with data regarding age at diagnosis). Male to female ratio was 3.1:1. Median age at diagnosis was 41 months with a wide variation (range between 17 months and 58 years). All patients but 2 suffered from classic rectosigmoid aganglionosis. Normal meconium passage (58%) was reported in a significantly higher number of patients compared to what observed in a series without diagnostic delay (p = 0.0140). All other variables (associated anomalies, preoperative enterocolitis, complications, and functional outcome) proved not to have statistically significant differences compared to a series of patients without a diagnostic delay. CONCLUSIONS: The results of our study underline that a significant percentage of patients are basically missed in the neonatal period mostly due to mild symptoms. Overall outcome does not differ from that of patients without diagnostic delay. Nonetheless, we underline the importance of a throughout investigation of all patients with meconium delay/failure and that of adopting a low threshold for performing rectal suction biopsies in constipated children to avoid misdiagnosis to serve the best for our patients.

Pediatric intensive bowel continence rehabilitation training: a retrospective study to evaluate feasibility, tolerance and effectiveness.

BACKGROUND: Pediatric continence dysfunction is not uncommon. It causes long-term disability, impairing quality of life, activities and relationships with pears and can affect until adulthood. A high-risk population are children with Hirschsprung's disease and congenital anorectal malformation. Conservative medical and surgical management of continence dysfunction in this population is deeply described, while the rehabilitation issues are still unexplored. Aim of this study is to preliminary verify the feasibility, tolerance and effectiveness of an intensive technological aided and individualized pelvic floor rehabilitation program for pediatric patients. METHODS: This is a single-center, retrospective observational study. The assessment was performed by collecting demographic data, general and local physical examination and scoring assessment tools (Rintala Continence Score and Wexner Score). The study was conducted in the Rehabilitation Unit of the pediatric Giannina Gaslini Institute, a tertiary care pediatric hospital in Genoa (Italy) between September 2015 to August 2019. We enrolled 31 children; 25 male (80.6%) and six females (19%), aged between 5 and 14 years (mean age 9 years) at the beginning of the training. Twenty children (65.5%) had Hirschsprung's disease, and 11 children (34.5%) had a congenital anorectal malformation. The rehabilitation training program was customized on the compromised function, the anatomic characteristics, the child's age and compliance. The training was aimed at improving tone, strength, endurance of the pelvic floor, compliance and rectal sensitivity, and also the frequency of the bowel movements. All patients enrolled in the study underwent an intensive outpatient treatment lasted 5 days for children older than 7 years; 10 days for younger. The intensive rehabilitation treatment was followed by a continuous home training program. RESULTS: Twenty-nine children (96.8%) completed the training. A global improvement is observed in continence functioning in all the cohort at T1 (P<0.0001), maintained at T3 (P<0.0001) at both Rintala Continence Score and Wexner Score. No adverse effects have been referred. CONCLUSIONS: Our specific pediatric training program for pelvic floor rehabilitation is effective and safe for children with continence dysfunctions after pelvic surgery due to Hirschsprung's disease and anorectal malformations. The continence rehabilitation multimodal program should be integrated in the care of children with continence dysfunctions. It cooperates in the prevention of the long-term health global impairment and also in the reduction of social economic effort.

Robotic surgery in Hirschsprung disease: a unicentric experience on 31 procedures.

Robotic surgery has been increasingly applied to Hirschsprung patients with encouraging results. We report the results of a 5 year unicentric experience. All consecutive HSCR patients older than 12 months who underwent a surgical procedure with robotic approach between September 2017 and August 2022 were prospectively included. We collected data regarding demographics, extent of aganglionosis, associated anomalies, indications to surgery, and a number of perioperative data such as surgical details, intraoperative and perioperative complications, length of surgery, length of hospital stay, and functional outcome. A total of 28 patients underwent 31 robotic procedures during the study period. Median age at surgery was 82 months. Eleven primary Totally Robotic Soave Pull-Through, 12 redoes, 5 innervative mapping, 2 redundant rectal pouch excision, and 1 Miles' procedures have been performed. Median console time was 145 min. No conversion to either laparoscopy nor to laparotomy was required. Median length of hospital stay was 6 days. Two patients experienced complications requiring reiterative surgery. One patient experienced mild postoperative enterocolitis. Normal continence was achieved by 70% of patients after a median of 16 months postoperatively (80% for primary pull-throughs, 55% for redoes). To conclude, robotic surgery for older HSCR patients proved to be feasible, safe, and effective. Patients with complex surgical requirements seem to benefit most from this promising approach. Provided the economic burden is addressed and solved, robotic surgery will represent an excellent alternative for the surgical treatment of HSCR patients.

European Pediatric Surgeons' Association Survey on the Use of Splenic Embolization in Blunt Splenic Trauma in Children.

INTRODUCTION: This article assesses (1) access to splenic embolization (SE), (2) indications for SE, and (3) post-embolization management in high-grade splenic trauma in children. MATERIALS AND METHODS: An online questionnaire was sent in 2021 to all members of European Pediatric Surgeons' Association. RESULTS: There were a total of 157 responses (50 countries, 83% academic hospitals). Among them, 68% have access to SE (SE) and 32% do not (nSE). For a hemodynamic stable patient with high-grade isolated splenic trauma without contrast extravasation (CE) on computed tomography (CT) scan, 99% SE and 95% nSE respondents use nonoperative management (NOM). In cases with CE, NOM decreases to 50% (p = 0.01) and 51% (p = 0.007) in SE and nSE centers, respectively. SE respondents report a significant reduction of NOM in stable patients with an associated spine injury requiring urgent surgery in prone position, both without and with CE (90 and 28%, respectively). For these respondents, in stable patients the association of a femur fracture only tends to decrease the NOM, both without and with CE (93 and 39%, respectively). There was no significant difference in NOM in group nSE with associated injuries with or without CE. After proximal SE with preserved spleen vascularization on ultrasound Doppler, 44% respondents prescribe antibiotics and/or immunizations. CONCLUSION: Two-thirds of respondents have access to SE. For SE respondents, SE is used even in stable patients when CE showed on initial CT scan and its use increased with the concomitant need for spinal surgery. There is currently a variation in the use of SE and antibiotics/immunizations following SE.

Urological Impact of Epididymo-orchitis in Patients with Anorectal Malformation: An ARM-Net Consortium Study.

INTRODUCTION: To investigate the current experience of the ARM-Net Consortium in the management of epididymo-orchitis (EO) in patients with anorectal malformations (ARMs), and to identify specific risk factors and the need for urological care involvement. MATERIALS AND METHODS: We retrospectively collected data of EO in patients with ARM between 2015 and 2019. Data on urological aspects, ARM type, surgical approach, associated anomalies, diagnosis, and treatment of EO were collected and analyzed. RESULTS: Twenty-nine patients were reported by 12 centers. Twenty-six patients with EO (90%) had ARM with a rectourinary fistula. Median age at first EO was 2 years (range: 15 days-27 years). Twenty patients (69%) experienced multiple EO, and 60% of recurrences were ipsilateral. Associated urological anomalies included vesicoureteral reflux (48%), urethral anomalies (41%), neurogenic bladder (41%), and ectopic vas (10%). A positive urine culture during EO was present in 69%. EO was treated with antibiotics (90%), limiting surgical exploration to 14%. Prevention of recurrences included surgery (bulking agents 15%, vasectomy 15%, and orchiectomy 5%) and antibiotic prophylaxis (20%). CONCLUSION: Urologists may encounter patients with EO in ARM patients, frequently with positive urine culture. An appropriate urologic work-up for most ARM patients is necessary to identify and treat underlying risk factors. A practical scheme for the work-up is suggested for a close collaboration between pediatric surgeons and urologists.

Pathways and microbiome modifications related to surgery and enterocolitis in Hirschsprung disease.

BACKGROUND: Hirschsprung disease (HSCR) is a congenital anomaly of the enteric nervous system. Abnormal microbiome composition was reported in HSCR patients. In this study, we addressed and analyzed microbiome modifications with relation tosurgery and HSCR associated enterocolitis (HAEC). METHODS: The faecal microbiome of 31 HSCR patients (overall 64 samples) was analyzed. HAEC was diagnosed and classified according to a combination of Pastor's and Elhalabi's criteria. Stool samples were analyzed by 16S sequencing (7 out of 9 polymorphic regions). Compositional and relative abundance profiles, as well as the functional potentials of the microbial community, were analyzed with the marker gene sequencing profiles using PICRUSt. RESULTS: The relative abundance of Bacteroidetes showed a severe decrease with slow recovery after surgery. Conversely, Proteobacteria transiently increased their abundance. Noteworthy, a strong linkage has been found between Proteobacteria descendants and HAEC occurrences. The inferred functional analysis indicated that virulence factors and fimbriae or pili might be associated with HAEC. CONCLUSIONS: Our study, addressing microbiome dynamics, demonstrated relevant changes after surgical manipulation. Alpha-diversity analyses indicated that surgery deeply affects microbiome composition. Proteobacteria and Enterobacteriaceae seem to play a pivotal role in HAEC occurrences. Several virulence factors, such as fimbriae or pili, might explain the HAEC-predisposing potential of selected microbiomes. These results suggest some innovative therapeutic approaches that deserve to be tested in appropriate clinical trials.

European Pediatric Surgeons' Association Survey on the Management of Primary Spontaneous Pneumothorax in Children.

AIM: To evaluate the practice patterns of the European Pediatric Surgeons' Association (EUPSA) members regarding the management of primary spontaneous pneumothorax (PSP) in children. METHODS: An online survey was distributed to all members of EUPSA. RESULTS: In total, 131 members from 44 countries participated in the survey. Interventional approach (78%) is the most common choice of treatment in the first episode, and most commonly, chest tube insertion (71%) is performed. In the case of a respiratory stable patient, 60% of the responders insert chest tubes if the pneumothorax is more than 2 cm. While 49% of surgeons prefer surgical intervention in the second episode, 42% still prefer chest tube insertion. Main indications for surgical treatment were the presence of bullae more than 2 cm (77%), and recurrent pneumothorax (76%). Eighty-four percent of surgeons prefer thoracoscopy and perform excision of bullae with safe margins (91%). To prevent recurrences, 54% of surgeons perform surgical pleurodesis with pleural abrasion (55%) and partial pleurectomy (22%). The responders who perform thoracoscopy use more surgical pleurodesis and prefer shorter chest tube duration than the surgeons performing open surgery (p < 0.05). CONCLUSION: Most of the responders prefer chest tube insertion in the management of first episode of PSP and perform surgical treatment in the second episode in case of underlying bullae more than 2 cm and recurrent pneumothorax. The surgeons performing thoracoscopy use more surgical pleurodesis and prefer shorter chest tube duration than the responders performing open surgery. The development of evidence-based guidelines may help standardize care and improve outcomes in children with PSP.

Minimally Invasive Partial Spleen Resection Preserving the Lower Pole in Children: A Technical Report on Feasibility and Safety.

Background: Laparoscopic partial splenectomy is a surgical option for removing part of the spleen with a pathological mass in a way that ensures preservation of the spleen's physiological functions. We aimed to evaluate the efficacy and feasibility of this procedure, performed while preserving the blood supply from the left gastroepiploic artery, in children. Materials and Methods: Following institutional review board approval, this retrospective study was conducted on patients who, between January 2015 and December 2019, underwent laparoscopic partial splenectomy with preservation of blood supply from the left gastroepiploic vessels. In the article, we described patient indications for surgery, surgical technique, surgery time, complications, and follow-up outcomes. Results: Eleven patients (mean age: 12.3 ± 3.4 years) underwent laparoscopic partial splenectomy. Indications for surgery included nonparasitic cysts (n = 8), pseudocysts (n = 2), and hamartomas (n = 1). The mean benign mass diameter was 60 ± 22 mm. The mean operative time was 193.2 ± 55.1 minutes. The mean size of the retained lower pole was 31.1% ± 3.8%. There was no conversion to open splenectomy or any significant complication. The mean postoperative stay was 9 ± 0.7 days. No thrombosis of the splenic and portal veins was noted in the follow-up period, and no splenic remnant infarction occurred. Conclusions: Laparoscopic partial splenectomy with preservation of blood supply arising from the left gastroepiploic vessels seems to be safe in children. However, a larger study is needed to confirm our results.

Congenital anomalies of the kidney and urinary tract in a cohort of 280 consecutive patients with Hirschsprung disease.

BACKGROUND: Congenital anomalies of the kidney and urinary tract (CAKUT) have been underestimated in Hirschsprung disease (HSCR). This paper aims at reporting results of patients with HSCR who underwent kidney and urinary tract assessment. METHODS: Patients seen between December 2005 and November 2020 underwent a complete kidney and urinary tract diagnostic workup. Data regarding CAKUT, gender, length of aganglionosis, familial history, HSCR-associated enterocolitis (HAEC), RET genotype, and outcome were collected. RESULTS: Out of 472 patients, 280 completed the workup and represented the focus. Male to female ratio was 3.24:1. Familial cases accounted for 9.8% of patients. RET mutations were detected in 19.8%. We encountered a total of 61 patients with 70 nephrological issues (21.8%), including 28 hypoplasia/dysplasia, 12 hydronephrosis, 11 vesicoureteric reflux, 7 duplex collecting system, 2 kidney agenesis, 2 horseshoe kidney, and 8 miscellanea, involving 91 kidneys without side preponderance (50 right, 41 left). Of these 61 patients, 20 (7.1% of the whole series) required medical or surgical treatment. When comparing patients with and without CAKUT, familial history proved to occur with a significantly lower frequency in the former as did better patient perspectives of outcome. CONCLUSIONS: We confirmed that all diagnostic workups in HSCR should include a complete kidney and urinary tract diagnostic workup. Our study suggests that genes other than RET could play a role in determining CAKUT. Given worse patient perspectives of outcome, CAKUT seems to significantly interfere with quality of life thus confirming the need for early diagnosis and tailored prevention strategies.

European Pediatric Surgeon' Association Survey on the Management of Short-Bowel Syndrome.

INTRODUCTION: The aim of this study was to assess the management of short-bowel syndrome (SBS) at the time of primary surgery, and the strategies used to facilitate enteral autonomy depending on the institutional expertise. MATERIALS AND METHODS: An online questionnaire was sent in 2019 to members of The European Pediatric Surgeons' Association. RESULTS: Among the 65 responding members (26 countries, 85% from university hospitals), 57% manage less than three new patients with SBS per year (group A), and 43% at least three patients (group B). The cut-off of three patients treated yearly used in our study was defined after statistical analysis of different cut-offs. A multidisciplinary intestinal rehabilitation program is significantly more frequent in group B than in group A (85 and 53%, respectively; p = 0.009). Considering the primary surgical management of multiple intestinal atresia and congenital ultra-short bowel with jejunal atresia, primary surgical strategies to optimize bowel length are more often used in group B than group A (p = 0.09 and p = 0.04, respectively). A minimum of one intestinal lengthening procedure every 2 to 3 years is significantly more frequent in group B than group A (95 and 45%, respectively; p = 0.0013). Among the strategies used to promote intestinal adaptation, group B (35%) uses significantly more often glucagon-like peptide 2 analogs than group A (10%) (p = 0.02). CONCLUSION: Based on our survey, a minimum number of SBS patients treated yearly is required to manage this challenging disease according to up-to-date medical and surgical strategies. However, whatever their level of expertise is in managing SBS, most of pediatric surgeons are involved in the primary surgery. Medical education programs about SBS should be more largely available to pediatric surgeons.

Safety of Veress needle for laparoscopic entry in children: Myth or reality?

INTRODUCTION: Recent reports suggested that blind laparoscopic entry techniques, including Veress needle (VN), might increase the risks of potentially fatal complications. MATERIALS AND METHODS: All consecutive patients who underwent laparoscopy in two Pediatric Hospitals with the use of a Veress needle during a 14-year period have been included. In all cases the first trocar was a radially expanding one (STEP). Complications related to the insertion technique are reported as well as those related to the whole laparoscopic technique. RESULTS: A total of 3463 patients younger than 18 years of age underwent laparoscopy between January 2006 and December 2019. Of these, 205 (5.9%) were younger than 6 months of age at surgery. Two-hundred-eighty-four patients (8.2%) previously underwent abdominal surgery. During first trocar insertion no major or minor vascular injuries occurred. Two patients (0.06%) experienced bowel lesions. Nine (0.26%) experienced failed entry. Fourteen patients (0.4%) experienced postoperative issues related to trocars positioning, namely, 9 omental eviscerations through port site insertion and 5 cases of hemoperitoneum owing to epigastric vessels lesion during operative trocar positioning. No other issues strictly related to laparoscopic entry technique have been recorded during the study period. No specific risk factors predisposing to complications have been identified but the presence of a positive history of previous abdominal procedures proved to be significantly related to a higher occurrence of bowel injury during Veress needle insertion (p = 0.0067). DISCUSSION AND CONCLUSIONS: Although with a number of biases and limitations, our study suggests that creation of pneumoperitoneum with VN combined to first trocar entry with STEP technology in children can represent a safe alternative. An exception is represented by patients who underwent previous abdominal surgeries who should be approached with caution, possibly with an open approach. Anyway, given the relatively poor quality of high-quality studies on this regard, we strongly support the implementation of well-designed RCT in children in order to answer this delicate topic. TYPE OF STUDY: Retrospective. LEVEL OF EVIDENCE: IV.

Guidelines of the Italian Society of Videosurgery in Infancy (SIVI) for the minimally invasive treatment of fetal and neonatal ovarian cysts.

In the last three decades, fetal ovarian cysts were diagnosed more frequently, due to technological improvement and the increasing use of prenatal screening ultrasound. Nonetheless, treatment uncertainties are still present, either prenatally or postnatally. Recently, significant innovations on diagnosis and treatment have been proposed and a more conservative, minimally invasive approach may be offered to the Pediatrician or the Surgeon who face with this condition during prenatal or neonatal age. (...).

Guidelines of the Italian Society of Videosurgery (SIVI) in Infancy for the minimally invasive treatment of Hypertrophic Pyloric Stenosis in neonates and infants.

The most appropriate treatment for the infantile Hypertrophic Pyloric Stenosis (HPS) is still debated. The non-surgical conservative treatment with oral or intravenous administration of atropine does not enjoy a widespread appreciation for several factors (...).

Bedside surgery in the newborn infants: survey of the Italian society of pediatric surgery.

INTRODUCTION: This is the report of the first official survey from the Italian Society of Pediatric Surgery (ISPS) to appraise the distribution and organization of bedside surgery in the neonatal intensive care units (NICU) in Italy. METHODS: A questionnaire requesting general data, staff data and workload data of the centers was developed and sent by means of an online cloud-based software instrument to all Italian pediatric surgery Units. RESULTS: The survey was answered by 34 (65%) out of 52 centers. NICU bedside surgery is reported in 81.8% of the pediatric surgery centers. A lower prevalence of bedside surgical practice in the NICU was reported for Southern Italy and the islands than for Northern Italy and Central Italy (Southern

Minimally Invasive Redo Pull-Throughs in Hirschsprung Disease.

Introduction: To reoperate a patient with Hirschsprung disease (HSCR) can be technically demanding and most surgeons would resort to conventional laparotomy. This article describes a series of patients with postoperative obstructive symptoms who underwent minimally invasive redo pull-throughs (MIRPT) (either laparoscopic or robotic) to assess the role of minimally invasive surgery (MIS) in complicated HSCR patients. Patients and Methods: All consecutive HSCR patients with postoperative obstructive symptoms, who underwent MIRPT with fast track concepts of care between January 2012 and January 2020, have been included. Data regarding indications, surgical details, complications, and outcome have been compared to those of a series of patients who underwent conventional laparotomic redo. Results: Sixteen patients were included. Male to female ratio was 4.3:1. Median age at surgery was 78 months. Eleven patients underwent laparoscopic redo and 5 underwent robotic redo. Median length of follow-up was 49 months. Reasons for redoing were transition zone pull-through, residual aganglionosis, anastomotic retraction or leak, rectal diverticulum, and refractory anastomotic stricture. No major intraoperative complication occurred. No conversion to laparotomy was required. One patient experienced cuff stricture requiring laparoscopic release. Two patients reported bouts of enterocolitis postoperatively. Compared to classic laparotomic redo pull-throughs (49 patients with complete data), overall complications were significantly less frequent, accounting for 1 and 21 events, respectively (6% versus 43%) (P = .0067). Continence after a median of 21 months postoperatively scored excellent to good in 9 out of 12 patients, who were assessed on this regard (75%), without statistically significant differences. Conclusions: MIRPT proved to be effective and safe in HSCR patients complaining postoperative obstructive symptoms. Robotic surgery could play a crucial. Our study confirms that complicated HSCR cases can be safely managed by means of MIS, applying concepts of fast track care to serve the best for our patients.

Management of COVID-19-Positive Pediatric Patients Undergoing Minimally Invasive Surgical Procedures: Systematic Review and Recommendations of the Board of European Society of Pediatric Endoscopic Surgeons.

Introduction: Hospital response to the COVID-19 outbreak has involved the cancellation of elective, deferrable surgeries throughout Europe in order to ensure capacity for emergent surgery and a selection of elective but non-deferrable surgeries. The purpose of this document is to propose technical strategies to assist the pediatric surgeons to minimize the potential aerosolization of viral particles in COVID-19 patients undergoing urgent or emergent surgical treatment using laparoscopic approaches, based on the currently available literature. The situation and recommendations are subject to change with emerging information. Materials and Methods: The Scientific Committee and the Board of the European Society of Pediatric Endoscopic Surgeons gathered together in order to address the issue of minimally invasive surgery during this COVID-19 pandemic. A systematic search through PubMed, Embase, and World Wide Web of the terms "COVID-19," "Coronavirus," and "SARS-CoV-2" matched with "pneumoperitoneum," "laparoscopy," "thoracoscopy," "retroperitoneoscopy," and "surgery" was performed. Non-English language papers were excluded. A PRISMA report was performed. Criticalities were identified and a consensus was achieved over a number of key aspects. Results: We identified 121 documents. A total of 11 full-text documents were assessed to address all concerns related to the adoption of minimally invasive surgery. All aspect of pediatric minimally invasive surgery, including elective surgery, urgent surgery, laparoscopy, thoracoscopy, retroperitoneoscopy, and pneumoperitoneum creation and maintainance were extensively addressed through systematic review. A consensus regarding urgent laparoscopic procedures, setting and operation techniques was obtained within the Committee and the Board. Conclusions: The ESPES proposes the following recommendations in case minimally invasive surgery is needed in a COVID-19 positive pediatric patients: (1) consider conservative treatment whenever safely possible, (2) dedicate a theater, columns and reusable laparoscopic instrumentation to COVID-19 pediatric patients, (3) prefer disposable instrumentation and cables, (4) use low CO2 insufflation pressures, (5) use low power electrocautery, (6) prefer closed-systems CO2 insufflation and desufflation systems, and (7) avoid leaks through ports. These recommendations are subject to change with emerging information and might be amended in the near future.

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.

Totally robotic soave pull-through procedure for Hirschsprung's disease: lessons learned from 11 consecutive pediatric patients.

INTRODUCTION: Since Hirschsprung's disease (HSCR) already proved to benefit from robotic surgery, we aimed at describing a wider series of patients with this rare disease who were operated on with a robotic approach. PATIENTS AND METHODS: All consecutive HSCR patients who underwent totally robotic soave pull-through (TRSPT), between October 2015 and June 2019, have been included. Ethical Committee approval was obtained. Data regarding clinical features, technical details, complications, hospital stay, and functional outcome have been prospectively collected for each patient. RESULTS: Eleven patients have been included. Mean age at surgery was 29 months. Median length of surgery was 420 min. Median console time was 180 min. Six patients suffered from rectosigmoid aganglionosis, three from long HSCR (extending up to the hepatic flexure), two from total colonic aganglionosis. No major intraoperative complications occurred. Four patients (three of whom carrying a stoma) experienced minor mucosal tearing during dissection. One anastomotic stricture required dilatation under general anesthesia and two cuff strictures required cuff release (both occurring in patients who experienced intraoperative mucosal tearing). Follow-up lasted a median of 12 months. One patient experienced mild postoperative enterocolitis. Continence scored excellent-to-good in all patients who could be assessed on that regard (7 out of 11). CONCLUSIONS: Provided a number of technical key points are respected, the outcome of TRSPT for HSCR is promising. Younger patients, particularly those carrying a stoma, proved to be technically demanding and deserve a longer learning curve. Accurate preoperative bowel preparation, correct trocar placement and patient positioning proved to be crucial aspects of treatment. To conclude, TRSPT is particularly suitable for older HSCR patients, even those requiring a redo, and represents a valid alternative to available surgical option for this delicate subgroup of HSCR patients.

Hirschsprung disease and Down syndrome: From the reappraisal of risk factors to the impact of surgery.

INTRODUCTION: The association of Hirschsprung disease (HSCR) and Down Syndrome (DS) is not uncommon (HSCR+DS). This paper aims at reporting the results of a 24-year series focusing on surgical approach, complications and long term outcome. MATERIALS AND METHODS: The notes of all patients admitted with a diagnosis of HSCR+DS have been retrospectively reviewed. Surgical details, intraoperative complications, long term issues and functional outcome have been recorded. The results have been compared to those of patients without DS and were assessed based on surgical approach. RESULTS: A total of 23 HSCR+DS out of a series of 385 HSCR (6%) have been included. Preoperative enterocolitis (HAEC) was reported by 32%. Associated anomalies were detected in more than half of the patients. In particular, Congenital Heart Defects (CHDs) were reported by 57%. Postoperative complications (mostly symptomatic anal sphincter achalasia) were experienced by 55%. Constipation was experienced by 30%; severe continence issues, by 53%. One patient suffering from severe CHDs died. With regard to complications, only symptomatic anal achalasia requiring intrasphincteric BoTox injection was significantly more frequent in HSCR+DS (30% vs 10%, p = 0.0071). Similarly, continence proved to be significantly worse in HSCR+DS. DISCUSSION: With the exception of symptomatic anal achalasia, HSCR+DS patients proved not to have a higher likelihood of complications compared to HSCR alone. On the other hand, functional results in the long term are worse. As a consequence, long term follow up and personalized rehabilitation programs are warranted for this delicate subset of HSCR patients. LEVEL OF EVIDENCE: Level III.