Combined Radial Incision and Steroid Injection for Refractory Colorectal Stenosis After Pull-Through Surgery in Hirschsprung Disease: An Innovative Conservative Treatment.

BACKGROUND: Anastomotic stenosis after pull-through surgery remains a challenge in the management of Hirschsprung disease. Based on the management of esophageal stenosis, we evaluated the efficacy of combined radial incision and steroid injection for the treatment of refractory colorectal anastomotic stenosis after pull-through. IMPACT OF INNOVATION: Combined radial incision and steroid injection is an alternative conservative treatment of refractory anastomotic stenosis after pull-though for Hirschsprung disease, avoiding a potential complicated redo pull-though surgery. TECHNOLOGY MATERIALS AND METHODS: We included patients with recto-sigmoid Hirschsprung disease that developed a refractory anastomotic stenosis after a laparoscopic-assisted Swenson pull-through at Robert-Debré Children University Hospital, Paris, France. Refractory stenosis was defined as obstructive symptoms associated with an anastomotic stenosis upon rectal exam without improvement after serial anal dilatations. Under general anesthesia, an injection of 10mg delayed-action steroid per quadrant was combined with a radial incision of the stenosis using monopolar cautery. PRELIMINARY RESULTS: Combined radial incision and steroid injection was performed in 4 children, for either early or late refractory stenosis. This resulted in improvement of refractory anastomotic stenosis, avoiding a redo pull-through in 75% of patients. One child presented with transient improvement after combined radial incision and steroid injection but developed recurrent stenosis despite additional combined radial incision and steroid injection and redo pull-through. The median follow-up was 29 months. CONCLUSION AND FUTURE DIRECTIONS: We observed a clinical improvement in all the patients after combined radial incision and steroid injection. Steroids injection should be considered as a potential alternative therapy for anastomotic stenosis.

Intraperitoneal insufflation of carbon dioxide rescues intestinal damage in an experimental murine model of colitis.

OBJECTIVES: Necrotizing enterocolitis (NEC) is a severe neonatal surgical condition, associated with a prolonged pro-inflammatory state, leading to high mortality and morbidity rates. Carbon dioxide (CO2 ) insufflation during laparoscopy may have an anti-inflammatory effect. We aimed to evaluate the effects of CO2 -insufflation on experimental colitis. METHODS: Acute colitis was induced in 6-week-old Balb/c mice by the administration of 2%-dextran sulfate-sodium (DSS) during 7 days (n = 45). On Day 4, two groups received intraperitoneal insufflation (duration: 30 mn, pressure: 5 mmHg) of CO2 ("DSS+CO2 ") or air ("DSS+air"). A group received no insufflation ("DSS"). Groups were compared for clinical severity using the disease activity index (DAI-body weight loss, stool consistency, and bleeding), histological severity (histopathological activity index, colon length, and ulcerations), colonic mucosecretion, and inflammation. RESULTS: DAI was significantly decreased in DSS+CO2 group, compared to DSS (p < 0.0001) or DSS+air (p < 0.0001) groups. Colon length was increased in DSS+CO2 treated mice compared to DSS (p = 0.0002). The histopathological activity index was lower in DSS+CO2 (vs. DSS, p = 0.0059/vs. DSS+air, p = 0.0389), with decreased ulcerations (3.77 vs. 10.7, p = 0.0306), and persistent mucosecretion with increased mucin-secreting cells. CONCLUSIONS: CO2 -insufflation attenuates DSS-induced colitis and improves both clinical and histological scores. Laparoscopy with CO2 insufflation represents a therapeutic anti-inflammatory strategy for NEC.

Complications after Heller myotomy in children: a national multicenter study on the impact of prior endoscopic dilatation and identification of risk factors.

BACKGROUND: Although esophageal achalasia has been historically treated by Heller myotomy, endoscopic esophageal dilatations are nowadays often the first-line treatment in children. The aim was to assess whether performing an endoscopic dilatation before a Heller myotomy is associated with higher risks of esophageal perforation in children. METHODS: A retrospective multicentric study was performed, including children that underwent a Heller myotomy (2000-2022, 10 centers). Two groups were compared based on the history of previous dilatation before myotomy. Outcomes esophageal perforation (intra-operative or secondary) and post-operative complications requiring surgery (Clavien-Dindo III). Statistics Comparisons using contingency tables or Kruskal-Wallis when appropriate. Statistical significance: p-value < 0.05. RESULTS: A Heller myotomy was performed in 77 children (median age: 11.8 years), with prior endoscopic dilatation in 53% (n = 41). A laparoscopic approach was used in 90%, with associated fundoplication in 95%. Esophageal perforation occurred in 19% of children (n = 15), including 12 patients with intra-operative mucosal tear and 3 with post-operative complications related to an unnoticed esophageal perforation. Previous endoscopic dilatation did not increase the risk of esophageal perforation (22% vs 17%, OR: 1.4, 95%CI: 0.43-4.69). Post-operative complications occurred in 8% (n = 6), with similar rates regardless of prior endoscopic dilatation. Intra-operative mucosal tear was the only risk factor for post-operative complications, increasing the risk of complications from 5 to 25% (OR: 6.89, 95%CI: 1.38-31.87). CONCLUSIONS: Prior endoscopic dilatations did not increase the risk of esophageal perforation or postoperative complications of Heller myotomy in this cohort of children with achalasia. Mucosal tear was identified as a risk factor for post-operative complications.

Enucleation for intestinal duplications: a comparative study with intestinal resection and anastomosis.

PURPOSE: Duplication enucleation (DE) has been described as an alternative to intestinal resection with primary anastomosis (IRA) for intestinal duplications, but no comparative study exists. The aim of this study was to compare both surgical procedures for intestinal duplication. METHODS: A retrospective study was performed, including all children treated for intestinal duplication (2005-2023). Patients that underwent DE were compared to those that underwent IRA. Statistical significance was determined using p < 0.05. Ethical approval was obtained. RESULTS: A total of 51 patients (median age: 5 months) were treated for intestinal duplication, including 27 patients (53%) that underwent DE and 24 IRA (47%). A cystic image was detected prenatally in 19 patients (70%) with DE and 11 patients (46%) with IRA (p = 0.09). Enucleation was performed using laparoscopy in 7 patients (14%). Patients that underwent DE had shorter time to first feed (1 vs 3 days, p = 0.0001) and length of stay (4 vs 6 days, p < 0.0004) compared to IRA. A muscular layer was identified in 68% of intestinal resection specimens. CONCLUSION: Compared to intestinal resection with anastomosis, duplication enucleation is associated with decreased postoperative length of stay and delay to first feeds without increasing post-operative complications. Regarding histological analysis, enucleation seems feasible in most cases.

Usefulness of routine early œsophagogram after primary repair of œsophageal atresia: a multicenter study.

BACKGROUND: Advances in surgical and neonatal care have led to improved survival of patients with œsophageal atresia (OA) over time. Morbidity remains significant, with one-third of patients being affected by a postoperative complication. Several aspects of management are not consensual, such as the use of œsophagogram before starting oral feeding. METHODS: We conducted a multicenter retrospective study, including all children with OA that underwent a primary anastomosis in the first days of life, between 2012 and 2018 in five French centers, to determine the usefulness of postoperative œsophagogram during the 10 days after early primary repair of OA to diagnose the anastomotic leak and congenital œsophageal stenosis. RESULTS: Among 225 included children, 90 (40%) had a routine œsophagogram and 25 (11%) had an anastomotic leak, clinically diagnosed before the scheduled œsophagogram in 24/25 (96%) children at median postoperative day 4. Ten patients had associated congenital œsophageal stenosis diagnosed on the œsophagogram in only 30% of cases. CONCLUSION: Early œsophagogram is rarely useful in the diagnosis of an anastomotic leak, which is clinically diagnosed before performing an œsophagogram in the majority of cases. The need for a postoperative œsophagogram should be evaluated on a case-by-case basis. IMPACT: Early œsophagogram is not helpful in the diagnosis of an anastomotic leak in the majority of cases. An anastomotic leak is most often diagnosed clinically before performing an œsophagogram. Early postoperative œsophagogram could be helpful for the diagnosis of congenital œsophageal stenosis. However, dysphagia occurs later and early diagnosis of congenital œsophageal stenosis has no impact on the management and outcome of asymptomatic children. Indication of postoperative œsophagogram has to be evaluated on a case-by-case basis.

Concomitant gastrostomy tube insertion during laparoscopic Nissen fundoplication for gastro-esophageal reflux disease: analysis of risk factors for fundoplication failure.

BACKGROUND: Laparoscopic Nissen fundoplication (LNF) and gastrostomy tube (GT) placement may be performed concomitantly in children with gastro-esophageal reflux disease (GERD) and failure to thrive. We aimed to evaluate the rate and risk factors for LNF failure in children undergoing concomitant LNF/GT. METHODS: A retrospective multi-institutional cohort study was conducted, reviewing patients that underwent LNF (2005-2014). Data collected included patient demographics, comorbidities, and type of GT (laparoscopy- or endoscopy-assisted). The primary outcome measure was LNF failure. Data was compared using contingency tables or Mann-Whitney tests, when appropriate. An exploratory analysis by Kaplan-Meier survival and Cox proportional hazards analysis was performed to determine predictors of time to LNF failure after LNF/GT. RESULTS: Of 189 children that underwent LNF, 99 (52%) had a concomitant GT (55% laparoscopy-, 45% endoscopy-assisted). LNF failed in 15% after LNF/GT and in 17% after LNF alone (p = 0.84), at a median age of 23 months (IQR 8-41). Using univariate analysis, we found that a younger age at the time of surgery (p = 0.05), prematurity (p = 0.0018), esophageal atresia (p = 0.01), and endoscopy-assisted GT (p = 0.02) were potential predictors of LNF failure after LNF/GT. After multivariate regression analysis, prematurity (p = 0.007) remained significantly associated with LNF failure after LNF/GT. No predictive factors for LNF failure after LNF alone were identified. CONCLUSIONS: Concomitant GT insertion and LNF is a common practice, as half of the children that undergo LNF also received GT insertion. Children born preterm or with esophageal atresia comprise a fragile population at high-risk of LNF failure after LNF/GT. Prospective, multicentric studies are needed to evaluate the best GT technique to use in children undergoing LNF.

The role of a pediatric tertiary care center in avoiding collateral damage for children with acute appendicitis during the COVID-19 outbreak.

PURPOSE: To evaluate the impact of the COVID-19 pandemic-related lockdown on management and outcomes of children with acute appendicitis. METHODS: A retrospective cohort study was conducted, including children treated for acute appendicitis (January 20th-May 11th, 2020). The data regarding the severity of appendicitis and outcome were collected and compared for two time periods, before and after the nationwide lockdown (March 17th, 2020). RESULTS: The number of cases of acute appendicitis increased by 77% during the lockdown (n = 39 vs. n = 69, p = 0.03). During the lockdown, children treated for appendicitis were older (11.1 vs. 8.9 years, p = 0.003), and were more likely to live more than 5 km away from our institution (77% vs. 52%, p = 0.017). Less children had previously consulted a general practitioner (15% vs. 33%, p = 0.028), whereas more children were transferred from other hospitals (52% vs. 31%, p = 0.043). There was no difference in terms of length of hospital stay, rate of postoperative intra-abdominal abscess, ER visits, and readmissions between both periods. Three children (4%) were diagnosed with COVID-19 and appendicitis. CONCLUSIONS: Despite an increase in the number of children with appendicitis managed at our hospital during the COVID-19-related lockdown, management, and outcome remained similar. Although our pediatric center was strongly affected by this pandemic, maintaining our prior practice strategies for acute appendicitis avoided the occurrence of collateral damage for those children.

Pulmonary hypertension secondary to congenital diaphragmatic hernia: factors and pathways involved in pulmonary vascular remodeling.

Congenital diaphragmatic hernia (CDH) is a severe birth defect that is characterized by pulmonary hypoplasia and pulmonary hypertension (PHTN). PHTN secondary to CDH is a result of vascular remodeling, a structural alteration in the pulmonary vessel wall that occurs in the fetus. Factors involved in vascular remodeling have been reported in several studies, but their interactions remain unclear. To help understand PHTN pathophysiology and design novel preventative and treatment strategies, we have conducted a systematic review of the literature and comprehensively analyzed all factors and pathways involved in the pathogenesis of pulmonary vascular remodeling secondary to CDH in the nitrofen model. Moreover, we have linked the dysregulated factors with pathways involved in human CDH. Of the 358 full-text articles screened, 75 studies reported factors that play a critical role in vascular remodeling secondary to CDH. Overall, the impairment of epithelial homeostasis present in pulmonary hypoplasia results in altered signaling to endothelial cells, leading to endothelial dysfunction. This causes an impairment of the crosstalk between endothelial cells and pulmonary artery smooth muscle cells, resulting in increased smooth muscle cell proliferation, resistance to apoptosis, and vasoconstriction, which clinically translate into PHTN.

Assessment of the nitrofen model of congenital diaphragmatic hernia and of the dysregulated factors involved in pulmonary hypoplasia.

PURPOSE: To study pulmonary hypoplasia (PH) associated with congenital diaphragmatic hernia (CDH), investigators have been employing a fetal rat model based on nitrofen administration to dams. Herein, we aimed to: (1) investigate the validity of the model, and (2) synthesize the main biological pathways implicated in the development of PH associated with CDH. METHODS: Using a defined strategy, we conducted a systematic review of the literature searching for studies reporting the incidence of CDH or factors involved in PH development. We also searched for PH factor interactions, relevance to lung development and to human PH. RESULTS: Of 335 full-text articles, 116 reported the incidence of CDH after nitrofen exposure or dysregulated factors in the lungs of nitrofen-exposed rat fetuses. CDH incidence: 54% (27-85%) fetuses developed a diaphragmatic defect, whereas the whole litter had PH in varying degrees. Downregulated signaling pathways included FGF/FGFR, BMP/BMPR, Sonic Hedgehog and retinoid acid signaling pathway, resulting in a delay in early epithelial differentiation, immature distal epithelium and dysfunctional mesenchyme. CONCLUSIONS: The nitrofen model effectively reproduces PH as it disrupts pathways that are critical for lung branching morphogenesis and alveolar differentiation. The low CDH rate confirms that PH is an associated phenomenon rather than the result of mechanical compression alone.

Congenital Diaphragmatic Eventration: Should we Maintain Surgical Treatment? A Retrospective Multicentric Cohort Study.

BACKGROUND: The aims of this study were to describe the characteristics of children with congenital diaphragmatic eventration (CDE) and compare the outcomes of surgical and conservative treatment of pediatric CDE in France. METHODS: Retrospective study on cohort data conducted in 22 paediatric surgery departments, including patients less than 16 years of age diagnosed with CDE between 2010 and 2021. Patients with surgical or conservative treatment were compared. RESULTS: 139 patients were included, with a median age of 8 [1-16] months. CDE occurred in boys in 68.3% and was right-sided in 66.7% of the cases. Indication for treatment depended essentially on respiratory symptoms and level of the diaphragmatic dome. The initial treatment was a surgical, with a diaphragmatic plication, in 87 cases (62%) and conservative, consisting of clinical follow-up in 52 children (38%). Of the latter, 25 children underwent surgery secondarily. Intra- and early post-operative complications occurred in 32 children (29%) and eventration recurrence in 8 children (7%). With a median follow-up of 28 months, the median level of diaphragmatic dome improved from the 6th to the 9th back rib, and the rate of respiratory symptoms decreased from 64% to 14% in the overall cohort of patients. CONCLUSIONS: Diaphragmatic plication is effective in symptomatic patients with a dome level above the 6th posterior rib, but is associated with a 29% complication rate and 7% of recurrence. CLINICALTRIALS: NCT04862494, April 28, 2021. LEVEL OF EVIDENCE: level III treatment study.

Morbidity related to major lung thoracoscopic resections in children.

In pediatric thoracic surgery, reported predictors for increased risk are symptoms and active/previous infections (RAP). We investigated the adverse events related to Video-Assisted Thoracic Surgery (VATS) in pediatric patients when considering RAP predictors. A retrospective analysis of pediatric VATS major lung resections in 2008-2021 was conducted at three institutions. We employed the pediatric surgical risk calculator to define patients' preoperative predicted risk (PredR). Postoperative complications were classified according to the Thoracic Morbidity & Mortality (TM&M) system. The observed TM&M rate (ObsR) and the PredR were compared. A subgroup analysis by RAP predictors was conducted. 37 patients (54% female) were included. Mean age and weight were 5.8 years and 22.8 kg. 56.7% had respiratory symptoms, 38.9% active infection and 59.5% history of infections (RAP subpopulations). VATS procedures were lobectomy (n=32), segmentectomy (n=3), bilobectomy (n=1) and pneumonectomy (n=1). The conversion rate was 5.4%. The mean PredR was of 4.43% (±1.8) and the overall ObsR was 45.94% with a median severity of II (I-III). This difference was significant and a higher PredR was not associated with complications development. PredR does not show association among the RAP vs non-RAP group. ObsR showed positive association with RAP, even if it reached statistical significance only for "respiratory symptoms" risk factor. ObsR reflected the number of bronchiectasis patients in our series (n=9), aligning with the hypothesis of "earlier and safer surgery". The risk calculator underestimates VATS morbidity. Multicentre studies will clarify the correlation between inflammation and surgical adverse events.

Results of uretero-ureteral anastomosis in pathological duplex kidney.

INTRODUCTION: Duplex renal collecting system or duplex kidney (DK) commonly is associated with uropathy, with upper pole obstruction or lower pole vesicoureteral reflux (VUR). Its management is variable, ranging from therapeutic abstention to total nephrectomy. In case of damage to a single renal pole, uretero-ureteral anastomosis (UUA) is one of the surgical techniques for preserving the pathological pole. It can be performed by laparotomy, laparoscopy, or both. The aims of this study are to report the results of UUA in pathological DK, and compare outcomes depending on the surgical approach. METHODS: This is a retrospective analysis study over 20 years, from April 2002 to July 2022, including all children from 0 to 15 years old who underwent UUA for a DK. Outcome measure included per- and post-operative complications, the occurrence of urinary tract infections and ultrasound measurements during follow-up. RESULTS: Thirty-three children underwent an UUA over 20 years. The median age at diagnosis was 25 days (range: 1 day-12 years). The median age at surgery was 13 months (range: 2 months - 13 years). The sex ratio was 0.22. Prenatal diagnosis was made in 87.9 % of cases (n = 29). The left kidney was affected in 72.7 % of cases. Twenty-seven children (81.8 %) had an ectopic ureteral opening of the upper pole ureter; four children (12.1 %) had ureterocele of the upper pole and 2 children (6.1 %) had vesicoureteral reflux of the inferior pole. UUA was performed by inguinal approach (laparotomy) in 17 children (51.5 %), by laparoscopy in 9 cases (27.3 %), and by laparoscopy combined with laparotomy in 7 cases (21.2 %). The complication rate was 12.1 % (n = 4), including recurrent pyelonephritis with superior polar hydronephrosis (n = 2); iatrogenic injury of the lower pole ureter (n = 1) and a urinoma (n = 1), that all required surgical management with a polar nephrectomy. Between each surgical approach, there were no significant differences in operative time, hospital stay, complications. CONCLUSION: UUA is an effective therapeutic option in duplex kidneys as it allows the preservation of the pathological pole with low complication rates, regardless of the surgical approach.

Serial Transverse Enteroplasty (STEP) for Short Bowel Syndrome (SBS) in Children: A Multicenter Study on Long-term Outcomes.

OBJECTIVES: Short Bowel Syndrome (SBS), secondary to various underlying diseases, is one of the main causes of intestinal failure in children. Surgical management by serial transverse enteroplasty (STEP), is feasible in selected cases, but results on long-term follow-up are scarce. The aim of this study was to report long-term outcomes of the STEP procedure in children with SBS. METHODS: We performed a multicenter national retrospective study reviewing medical charts of children who underwent a STEP technique between 2000 and 2022 in 6 university hospitals. Collected data included demographics, SBS history, surgical procedures, nutritional support (enteral or parenteral), STEP management, complications, and outcomes (nutritional support, digestive symptoms, growth). RESULTS: STEP was performed in 36 SBS, resulting from 14 gastroschisis, 10 intestinal atresia, 8 necrotizing enterocolitis (NEC), 2 midgut volvulus, and 2 intestinal Hirschsprung disease. Median age at first STEP was 10.8 months [4.5; 63.8]. Bowel length significantly increased (47 vs 70 cm, p < 0.01), with a median gain of 16 cm [11; 25] or 34%. At surgery, 33 children had parenteral nutrition (PN). A second STEP was performed in 11 patients, at a median age of 4.2 years [2.8; 6.8]. One patient underwent a third STEP. Two children required intestinal transplantation. Median follow-up was 7 years [4.4;11.4]. Out of 33 children requiring PN before surgery, 14 children were weaned off PN. The remaining 19 children still required PN, but their dependency decreased by 19%. Out of 17 children presenting severe intestinal dilations and bacterial overgrowth, 12 became asymptomatic. Out of 20 with chronic obstructive symptoms, 8 patients still required supplementary PN whereas 12 achieved complete enteral autonomy, including 3 requiring supplementary enteral support. CONCLUSION: STEP technique remains a surgical option in the management of these children, enabling a decrease in PN dependency, resulting in weaning off PN in some cases, as well as an improvement of clinical symptoms. TYPE OF STUDY: Retrospective multicenter observational study. LEVEL OF EVIDENCE: Level 3.

Fetal hypoplastic lungs have multilineage inflammation that is reversed by amniotic fluid stem cell extracellular vesicle treatment.

Antenatal administration of extracellular vesicles from amniotic fluid stem cells (AFSC-EVs) reverses features of pulmonary hypoplasia in models of congenital diaphragmatic hernia (CDH). However, it remains unknown which lung cellular compartments and biological pathways are affected by AFSC-EV therapy. Herein, we conducted single-nucleus RNA sequencing (snRNA-seq) on rat fetal CDH lungs treated with vehicle or AFSC-EVs. We identified that intra-amniotically injected AFSC-EVs reach the fetal lung in rats with CDH, where they promote lung branching morphogenesis and epithelial cell differentiation. Moreover, snRNA-seq revealed that rat fetal CDH lungs have a multilineage inflammatory signature with macrophage enrichment, which is reversed by AFSC-EV treatment. Macrophage enrichment in CDH fetal rat lungs was confirmed by immunofluorescence, flow cytometry, and inhibition studies with GW2580. Moreover, we validated macrophage enrichment in human fetal CDH lung autopsy samples. Together, this study advances knowledge on the pathogenesis of pulmonary hypoplasia and further evidence on the value of an EV-based therapy for CDH fetuses.

Robotic-assisted colectomy in children: a comparative study with laparoscopic surgery.

The aim of this study was to compare outcomes of laparoscopic and robotic-assisted colectomy in children. All children who underwent a colectomy with a laparoscopic (LapC) or robotic-assisted (RobC) approach in our institution (January 2010-March 2023) were included. Demographics, surgical data, and post-operative outcomes within 30 days were collected. Additional cost related to the robotic approach was calculated. Comparisons were performed using Fisher tests for categorical variables and Mann-Whitney tests for continuous variables. A total of 55 colectomies were performed: 31 LapC and 24 RobC (median age: 14.9 years). Main indications included: inflammatory bowel disease (n = 36, 65%), familial adenomatous polyposis (n = 6, 11%), sigmoid volvulus (n = 5, 9%), chronic intestinal pseudo-obstruction (n = 3, 5%). LapC included 22 right, 4 left, and 5 total colectomies. RobC included 15 right, 4 left, and 5 total colectomies. Robotic-assisted surgery was associated with increased operative time (3 h vs 2.5 h, p = 0.02), with a median increase in operative time of 36 min. There were no conversions. Post-operative complications occurred in 35% of LapC and 38% of RobC (p = 0.99). Complications requiring treatment under general anesthesia (Clavien-Dindo 3) occurred in similar rates (23% in LapC vs 13% in RobC, p = 0.49). Length of hospitalization was 10 days in LapC and 8.5 days in RobC (p = 0.39). The robotic approach was associated with a median additional cost of 2156€ per surgery. Robotic-assisted colectomy is as safe and feasible as laparoscopic colectomy in children, with similar complication rates but increased operative times and cost.

Scarless laparoscopic incisions in Pfannenstiel (slip): the first 50 cases using an innovative approach in pediatric robotic surgery.

Incisions in the supra-pubic region have been described robotic-assisted surgery to improve cosmetic results in adults, but seldom in children. We aimed to present an innovative trocar placement in the Pfannenstiel line, named Scarless Laparoscopic Incisions in Pfannenstiel (SLIP), and evaluate its feasibility for various intra-abdominal procedures and its cosmetic results in pediatric robotic surgery. We performed a monocentric prospective study, including children undergoing robotic-assisted surgeries using a SLIP approach (July 2019-September 2021). Data regarding demographics, surgery, and outcome were collected and reported as median (range), or number (percentage). Cosmetic results were evaluated with a questionnaire. A SLIP approach was performed in 50 children (24 cholecystectomies, 12 splenectomies, 2 cholecystectomies and splenectomies, 9 colonic resections, 2 choledochal cyst resections, and 1 pancreatic pseudocyst resection). Median age was 11 years (2-18) and median weight 35 kg (10.5-80). Conversion to laparoscopy occurred in two cases. Post-operative complications occurred in 5 patients (10%), after colectomies [intrabdominal abscess (n = 3), stoma dysfunction (n = 1), parietal abscess (n = 1)], of which 3 (6%) required reintervention (intrabdominal abscess n = 2, stoma dysfunction n = 1). Regarding scars, 68% (n = 28) of parents and patients reported the maximal score of 5/5 for global satisfaction and 63% (n = 26) had all scars hidden by underwear. SLIP approach is versatile and can be used in supra- and infra-mesocolic robotic-assisted procedures. The low complication rate shows its safety in both young children and teenagers and does not increase operative difficulties. It results in high patient satisfaction regarding scars, and a scarless abdomen.

Hirschsprung disease.

Hirschsprung disease (HSCR) is a rare congenital intestinal disease that occurs in 1 in 5,000 live births. HSCR is characterized by the absence of ganglion cells in the myenteric and submucosal plexuses of the intestine. Most patients present during the neonatal period with the first meconium passage delayed beyond 24 h, abdominal distension and vomiting. Syndromes associated with HSCR include trisomy 21, Mowat-Wilson syndrome, congenital central hypoventilation syndrome, Shah-Waardenburg syndrome and cartilage-hair hypoplasia. Multiple putative genes are involved in familial and isolated HSCR, of which the most common are the RET proto-oncogene and EDNRB. Diagnosis consists of visualization of a transition zone on contrast enema and confirmation via rectal biopsy. HSCR is typically managed by surgical removal of the aganglionic bowel and reconstruction of the intestinal tract by connecting the normally innervated bowel down to the anus while preserving normal sphincter function. Several procedures, namely Swenson, Soave and Duhamel procedures, can be undertaken and may include a laparoscopically assisted approach. Short-term and long-term comorbidities include persistent obstructive symptoms, enterocolitis and soiling. Continued research and innovation to better understand disease mechanisms holds promise for developing novel techniques for diagnosis and therapy, and improving outcomes in patients.