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.

Efficiency of non-operative management for pectus deformities in children using an X-ray-free protocol.

OBJECTIVES: The aim of this study was to explore the correlation between the Haller index (HI), the external depth of protrusion and the external Haller index (EHI) for both pectus excavatum (PE) and pectus carinatum (PC) and to assess the variation in the HI during this first year of non-operative treatment for pectus deformities in children. METHODS: From January 2018 to December 2022, all children treated for PE by vacuum bell and for PC by compression therapy at our institution were evaluated by external gauge, 3D scanning (iPad with Structure Sensor and Captevia-Rodin4D) and magnetic resonance imaging (MRI). The main objectives were to assess the effectiveness of the treatment during the first year and to compare the HI determined by MRI to the EHI evaluated with 3D scanning and external measurements. The HI determined by MRI was compared to the EHI evaluated with 3D scanning and external measurements at M0 and M12. RESULTS: A total of 118 patients (80 PE and 38 PC) had been referred for pectus deformity. Of these, 79 met the inclusion criteria (median age 13.7 years, 8.6-17.8). There was a statistically significant difference in the external measurements of the depth for PE between M0 and M12: 23.0 ± 7.2 vs 13.8 ± 6.1 mm, respectively, P < 0.05, and for PC 31.1 ± 10.6 vs 16.7 ± 8.9 mm, respectively, P < 0.01. During this first year of treatment, the reduction in the external measurement increased more rapidly for PE compared with PC. We found a strong correlation between the HI by MRI and the EHI by 3D scanning for PE (Pearson coefficient = 0.910, P < 0.001) and for PC (Pearson coefficient = 0.934, P < 0.001). A correlation between the EHI by 3D scanning and the external measurements by profile gauge was found for PE (Pearson coefficient = 0.663, P < 0.001) but not for PC. CONCLUSIONS: Excellent results were observed as soon as the sixth month for both PE and PC. Measurement of protrusion is a reliable monitoring tool at clinical consultation but caution is required for PC as it does not appear to be correlated to the HI by MRI.

Comparison of the Learning Curve for Robot-Assisted Laparoscopic Pyeloplasty Between Senior and Junior Surgeons.

Introduction: The widespread use of robotic surgery has provided surgeons a high-quality and alternative method to perform pyeloplasty. Indeed, robot-assisted technology has made it a lot easier to obtain the advanced technical skills required to perform this procedure. The learning curve (LC) reflects the increase in learning as a function of experience. Robot-assisted laparoscopic pyeloplasty (RALP) represents a well-standardized and reproducible procedure with an LC that allows reliable results to readily be obtained. The aim of our study was to compare the RALP LC of senior versus junior surgeons. Materials and Methods: We reviewed all of the RALP procedures performed at three pediatric surgery centers between November 2007 and November 2018. Three senior surgeons and 4 junior surgeons performed the robotic procedures. Neither the senior nor the junior surgeons had previous experience with robotic surgery; they had experience with conventional laparoscopic procedures but not with laparoscopic pyeloplasty. The primary metric that we selected to evaluate competence acquisition was a composite outcome defined by a combination of operative time (OT), complications, and surgical success. The complications were rated according to the Clavien-Dindo classification expressed by a complication factor; surgical success was expressed as a success factor, and we used a cumulative sum (CUSUM) analysis to determine the LC. The CUSUM method, through its multioutcome approach, is a very effective way to evaluate LCs. Results: Between November 2007 and November 2018, 3 senior surgeons and 4 junior surgeons performed 88 RALP procedures. The included patients had a median age of 6.1 years (range 7 months-16 years). The median duration of follow-up was 6.4 years (range 14 months-12 years). The median OT was 198.86 minutes (range 106-335 minutes). By CUSUM analysis of the composite outcome, we found that despite the junior surgeons having performed fewer procedures than the senior surgeons, their LCs exhibited an earlier inflection point (Fig. 1) followed by a constant rate of improvement in proficiency, thus indicating a more rapid learning process. The median composite scores for the senior surgeons and the junior surgeons were 299 (range 210-370) and 193 (range 131-255), respectively, after seven procedures. Conclusion: Assuming proper exposure to robotics and an adequate case volume, we demonstrated that junior surgeons can readily achieve comparable levels of expertise compared with senior practitioners in the field of pediatric RALP. It can be assumed that the LC in robotic pyeloplasty is not only directly influenced by the individual surgical experience but also by the experience of the surgical team.

Management of lower urinary tract fibroepithelial polyps in children.

INTRODUCTION: Fibroepithelial polyps (FEP) of the lower urinary tract are relatively common in adults but rare in children, with fewer than 250 cases reported in the literature to date. OBJECTIVE: The aim of this study was to address the experience of FEP management in children. STUDY DESIGN: A retrospective multicenter review was undertaken in children with defined FEP of the lower urinary tract managed between 2008 and 2018. The data at 18 pediatric surgery centers were collected. Their demographic, radiological, surgical, and pathological information were reviewed. RESULTS: A total of 33 children (26 boys; 7 girls) were treated for FEP of the lower urinary tract at 13 centers. The most common presentation was urinary outflow as hematuria (41%), acute urinary retention (25%), dysuria (19%), or urinary infections (28%). A prenatal diagnosis was made for three patients with hydronephrosis. Almost all of the children (94%) underwent ultrasound imaging of the urinary tract as the first diagnostic examination, 23 (70%) of them also either had an MRI (15%), cystourethrography (25%), computerized tomography (6%), or cystoscopy (45%). Two of these children (6%) had a biopsy prior to the surgery. The median preoperative delay was 7.52 (range: 1-48) months. Most of the patients were treated endoscopically, although four (12.1%) had open surgery and two (6.1%) had an additional incision for specimen extraction. The median hospital stay was 1.5 (range: 1-10) days. There were no recurrences and no complications after a median follow-up of 13 (range: 1-34) months. DISCUSSION: The main limitation of our study is the retrospective design, although it is the largest one for this pathology. CONCLUSION: This series supports sonography as the most suitable diagnosis tool before endoscopy to confirm the diagnosis and to perform the resection for most FEP in children. This report confirms the recognized benign nature in the absence of recurrences. LEVEL OF EVIDENCE: Level V.

Predictors of the Performance of Early Antireflux Surgery in Esophageal Atresia.

OBJECTIVE: To identify predictors of and factors associated with the performance of antireflux surgery during the first year of life in children born with esophageal atresia. STUDY DESIGN: All patients were included in a French registry for esophageal atresia. All 38 multidisciplinary French centers completed questionnaires about perinatal characteristics and one-year outcome for children born with esophageal atresia. RESULTS: Of 835 infants with esophageal atresia born in France from 2010 to 2014, 682 patients, excluding those with long-gap esophageal atresia, were included. Three patients had type I, 669 had type III, and 10 had type IV esophageal atresia. Fifty-three children (7.8%) received fundoplication during the first year of life. The median age at the time of the end-to-end esophageal anastomosis was 1.1 day (range 0-15). Multivariate analysis identified three perioperative factors that predicted the need for early antireflux surgery: anastomotic tension (P = .004), associated malformations (P = .019), and low birth weight (P = .018). Six other factors, measured during the first year of life, were associated with the need for antireflux surgery: gastroesophageal reflux (P < .001), anastomotic stricture (P < .001), gastrostomy (P < .001), acute life-threatening event (P = .002), respiratory complications (P = .045), and poor nutritional status (P < .001). CONCLUSIONS: Gastroesophageal reflux disease, low birth weight, poor nutrition, and surgical anastomosis difficulties predicted the performance of antireflux surgery in the first year of life in infants with esophageal atresia.

A Simulation Model to Support Laparoscopic Pyloromyotomy Teaching.

INTRODUCTION: A key concern regarding laparoscopic pyloromyotomy (LP) lies with the process of learning this skill. The learning processes for open pyloromyotomy and LP appear to be different, with an earlier increased risk of perforation or incomplete pyloromyotomy (IP) for LP. Our aim was to develop a simple simulation tool to reduce these specific complications. MATERIALS AND METHODS: A model of hypertrophic pyloric stenosis was created and inserted into a pediatric laparoscopic surgery simulator. A cohort of experts completed a six-item questionnaire, using a 4-point scale regarding the model's realistic nature and accuracy. Evaluation of the LP procedure was based on a dedicated Objective Structured Assessment of Technical Skills score. Surgical residents and students were enrolled for the final evaluation to assess the relative performance of trainees who had practiced with this model (Group 1) versus those who had observed its use (Group 2). RESULTS: Reproducibility of the model construction was considered to be satisfactory. The experts agreed that the model accurately simulated essential components of LP (mean 3.03 ± 0.7). They scored significantly better than the residents (27.2 ± 1.8 versus 22.8 ± 2.9; P < .001), with a lower rate of complications. Group 1 (39 trainees) performed significantly better than Group 2 (26 trainees), with a significant decrease in the risk of an IP (P < .05). CONCLUSIONS: This model appears to be sufficiently accurate to teach LP. In light of this, it can be considered to be an efficient tool for LP simulation teaching in our fellows' educational program.

Comparison of 8 and 5 mm robotic instruments in small cavities : 5 or 8 mm robotic instruments for small cavities?

INTRODUCTION: Robotic surgery has seen increasing use in the field of pediatric surgery. Our clinical experience suggested instrument size can impact on the surgical ability. This study aimed to compare the performance of robot-assisted laparoscopic skills in confined spaces using either 5 or 8 mm instruments. METHODS: A preclinical randomized crossover study design was implemented. 24 assessors performed three different reproducible drill procedures (M1: peg transfer, M2: circle cutting, M3: intracorporeal suturing). To assess surgical proficiency in confined working spaces, these exercises were performed with 5 and 8 mm instruments of the da Vinci® Surgical Systems Si in a cubic box with 60 mm-sized edges. Each performance was recorded and evaluated by two reviewers using both objective structured assessment of technical skills (OSATS) and global evaluative assessment of robotic skills (GEARS) scores. Parietal iatrogenic impacts and instrument collisions were specifically analyzed using a dedicated scoring system. RESULTS: Regardless of their experience, trainees performed significantly better when using 8 mm instruments in terms of OSATS scores (20.5 vs. 18.4; p < 0.01) and GEARS scores (23.4 vs. 21.9; p < 0.01) for most items, except for "depth perception" and "autonomy." The 8 mm performances involved significantly less parietal box damage (4.1 vs. 3.4; p < 0.01), and tool collisions (4.1 vs. 3.2; p < 0.01). CONCLUSIONS: In light of the better performances with 8 mm tools for specific tasks and parietal sparing constraints in restricted spaces, this study indicates that 5 mm instruments can be deemed to be less effective for reconstructive procedures in small children.

Small Cell Carcinoma of the Ovary, Hypercalcemic Type: Report of a Bilateral Case in a Teenager Associated with SMARCA4 Germline Mutation.

Small cell carcinoma of the ovary, hypercalcemic type (SCCOHT), is a highly aggressive neoplasm that typically occurs in young females. Paraneoplastic hypercalcemia is associated in two thirds of the cases. Recent studies demonstrated that this rare tumor harbors the same molecular features of malignant rhabdoid tumor secondary to SMARCA4/BRG1 mutations. We illustrate herein a typical bilateral case of SCCOHT with comprehensive molecular characterization in a 14-year-old girl. We also discuss the value of SMARCA4 immunostaining in the diagnostic approach of undifferentiated ovarian and pelvic malignancies.

Portal vein thrombosis after laparoscopic splenectomy during childhood.

Portal vein thrombosis (PVT) is a rare but potentially life-threatening complication of laparoscopic splenectomy (LS) and can lead to bowel ischemia or portal hypertension. In childhood, this complication is reported in 5-10 % of the cases whereas it can be up to 50 % in adult population. Our aim was to evaluate PVT incidence after LS and associated risks factors. A retrospective chart review identified 37 children who underwent elective LS from 2005 to 2013. The main indications were spherocytosis or sickle cell disease. Median age and weight were respectively 7.4 years and 25.1 kg. Thromboembolic prophylaxis was not routinely given. Duration of surgery was 129 min and hospital length of stay 4 days. Doppler ultrasound scan (USS) was performed post-operatively in 26 cases. Post-operative course was uneventful in all but one patient. She was a 17 year-old girl previously operated for an ovarian tumor with hyperandrogenism. Histopathology revealed a splenic lymphoma. At day 4, a systematic USS showed a PVT extending in the portal branches. Therapeutic low molecular weight heparin was used and then transitioned to fluindione for 3 months. Follow-up USS performed at 1 and 4 months demonstrated complete resolution of the PVT. PVT after pediatric LS is a rare event in our series. Clinician should be cautious in oncologic cases and if very large spleen or if thrombocythemia >650.10(9)/L is present. If detected early, PVT can be treated efficiently. We therefore recommend a systematic USS during the first postoperative week.

Prenatal intestinal obstruction affects the myenteric plexus and causes functional bowel impairment in fetal rat experimental model of intestinal atresia.

BACKGROUND: Intestinal atresia is a rare congenital disorder with an incidence of 3/10,000 birth. About one-third of patients have severe intestinal dysfunction after surgical repair. We examined whether prenatal gastrointestinal obstruction might effect on the myenteric plexus and account for subsequent functional disorders. METHODOLOGY/PRINCIPAL FINDINGS: We studied a rat model of surgically induced antenatal atresia, comparing intestinal samples from both sides of the obstruction and with healthy rat pups controls. Whole-mount preparations of the myenteric plexus were stained for choline acetyltransferase (ChAT) and nitric oxide synthase (nNOS). Quantitative reverse transcription PCR was used to analyze mRNAs for inflammatory markers. Functional motility and permeability analyses were performed in vitro. Phenotypic studies were also performed in 8 newborns with intestinal atresia. In the experimental model, the proportion of nNOS-immunoreactive neurons was similar in proximal and distal segments (6.7±4.6% vs 5.6±4.2%, p = 0.25), but proximal segments contained a higher proportion of ChAT-immunoreactive neurons (13.2±6.2% vs 7.5±4.3%, p = 0.005). Phenotypic changes were associated with a 100-fold lower concentration-dependent contractile response to carbachol and a 1.6-fold higher EFS-induced contractile response in proximal compared to distal segments. Transcellular (p = 0.002) but not paracellular permeability was increased. Comparison with controls showed that modifications involved not only proximal but also distal segments. Phenotypic studies in human atresia confirmed the changes in ChAT expression. CONCLUSION: Experimental atresia in fetal rat induces differential myenteric plexus phenotypical as well as functional changes (motility and permeability) between the two sides of the obstruction. Delineating these changes might help to identify markers predictive of motility dysfunction and to define guidelines for post-surgical care.

Infantile hypertrophic pyloric stenosis: are viruses involved?

Infantile hypertrophic pyloric stenosis (IHPS) is characterized by abnormal thickening of the internal circular muscle layer. IHPS is known to be due to a combination of genetic and environmental factors, but its precise causes and pathophysiology are poorly understood. The objective of the study is to determine the prevalence of the principal viruses targeting the respiratory and digestive tracts in children with IHPS. Nasopharyngeal fluids, stools, vomit, and surgical pyloric muscle fragments and swabs were tested by cell culture, viral antigen assay and PCR. IHPS was diagnosed in 23 boys and 8 girls with a mean (± SD) age of 42 ± 15 days (range 20-88 days). There was no seasonal pattern of diagnosis. Twenty-two children (71%) lost weight (mean 246 ± 164 g, range 30-600 g) after the onset of vomiting, and five (16.1%) were dehydrated. Seven (22.6%) infants had been exposed to an infectious contact within 15 days before admission, and one on the day of admission (3.2%). Ear, nose and throat samples and pyloric muscle specimens were negative for all the viruses tested. An adenovirus type 3 was recovered from one stool sample, and RT-PCR was positive for an enterovirus on one vomit sample. This study suggests that the principal viruses targeting the respiratory and digestive tracts are not responsible for IHPS.