Surgical management of ovarian masses in children and adolescents: experience of an academic institution in France.

PURPOSE: Surgical management of ovarian masses in girls still challenging. The aim of the study is to report an 8-year experience in managing children with ovarian masses, and to demonstrate the advantages and the limitations of laparoscopy for such lesions. METHODS: Data of girls aged less than 18 years operated because of an ovarian mass between January 2015 and February 2023 were retrospectively reviewed. Patients were divided into two groups: group A including children operated by laparoscopy, and Group B of patients who underwent open surgery. RESULTS: Eighty-eight children were enrolled. Laparoscopy was performed in 56 patients (63.6%). Group A patients had smaller tumor size (53.6±38.5 vs. 122.2±75.4 mm, P<0.0001), shorter operative time (50.4±20.3 vs. 71.5±36.5 min, P = 0.004), reduced length of hospital stay (1.4±1.1 vs. 3±2.3 days, P<0.0001), and absence of postoperative complications. Only 3 cases (5.7%) of recurrence were seen exclusively within patients followed for benign tumors during a mean follow-up period of 4.6±3 years. CONCLUSION: Laparoscopy should be done in benign ovarian lesions or/and if a torsion is seen. For tumors at high risk of malignancy, laparoscopy can be performed to establish a clear macroscopic diagnosis, for staging of the disease, and resection of small tumors. Conversion to open surgery is indicated in case of doubt.

Long-Term Functional Outcomes of an Anorectal Malformation French National Cohort.

OBJECTIVES: The present study aimed to assess long-term functional outcomes of children with anorectal malformations (ARMs) across a network of expert centers in France. METHODS: Retrospective cross-sectional study of patients ages 6-30 years that had been surgically treated for ARM. Patient and ARM characteristics (eg, level, surgical approach) and functional outcomes were assessed in the different age groups. RESULTS: Among 367 patients, there were 155 females (42.2%) and 212 males (57.8%), 188 (51.2%) cases with, and 179 (48.8%) higher forms without, perineal fistula. Univariate and multivariate statistical analyses with logistic regression showed correlation between the level of the rectal blind pouch and voluntary bowel movements (odds ratio [OR] = 1.84 [1.31-2.57], P < 0.001), or soiling (OR = 1.72 [1.31-2.25], P < 0.001), which was also associated with the inability to discriminate between stool and gas (OR = 2.45 [1.28-4.67], P = 0.007) and the presence of constipation (OR = 2.97 [1.74-5.08], P < 0.001). Risk factors for constipation were sacral abnormalities [OR = 2.26 [1.23-4.25], P = 0.01) and surgical procedures without an abdominal approach (OR = 2.98 [1.29-6.87], P = 0.01). Only the holding of voluntary bowel movements and soiling rates improved with age. CONCLUSION: This cross-sectional study confirms a strong association between anatomical status and functional outcomes in patients surgically treated for ARM. It specifically highlights the need for long-term follow-up of all patients to help them with supportive care.

Preoperative 3D reconstruction images for paediatric tumours: Advantages and drawbacks.

RATIONALE: Three-dimensional reconstruction (3DR) of preoperative images may improve the presurgical assessment of tumours prior to removal. We aimed to analyse the advantages and discrepancies of preoperative 3DR in paediatric tumours. METHODS: We conducted a prospective observational study from 2016 to 2019, including patients with thoraco-abdominal tumours having predictable surgical risks on preoperative images (encasement of vessels posing vascular risks, ie, neuroblastic and soft tissue tumours or parenchyma preservation of the invaded organ, ie, liver and kidney). A comparison of 2D/3DR and surgical findings was performed. RESULTS: Twenty-four patients, with a median age at surgery of 68.2 months (13 days-203 months), were operated on for neuroblastoma (n = 7), renal tumour (n = 7), hepatic tumour (n = 4) and others (n = 6; bone sarcoma of the iliac branch, abdominal lymph nodes of a recurrent testicular germ cell tumour, pseudoinflammatory tumour of the omentum, thoracic lipoblastoma, desmoplastic tumour, solid and pseudopapillar tumour of the pancreas). Reconstruction was of poor quality in two patients with renal tumours because computed tomography (CT) had no excretory phase. Discrepancies between 3DR and surgical findings occurred in two patients, one because of poor assessment of caliceal infiltration by renal nodules and the other because of inadequate reconstruction of renal vein thrombosis. For all the other tumours, 3DR improved the visualisation and precise location of vessels during surgery. CONCLUSION: High-quality preoperative images are mandatory to provide the best 3DR. In the majority of cases, 3DR is of significant help during surgery to better identify vascular structures within tumours and preserve parenchyma.

Is laterality of congenital diaphragmatic hernia a reliable prognostic factor? French national cohort study.

OBJECTIVES: The objective of this study was to assess whether the laterality of congenital diaphragmatic hernia (CDH) was a prognostic factor for neonatal survival. METHODS: This was a cohort study using the French national database of the Reference Center for Diaphragmatic Hernias. The principal endpoint was survival after hospitalization in intensive care. We made a comparative study between right CDH and left CDH by univariate and multivariate analysis. Terminations and stillbirths were excluded from analyses of neonatal outcomes. RESULTS: A total of 506 CDH were included with 67 (13%) right CDH and 439 left CDH (87%). Rate of survival was 49% for right CDH and 74% for left CDH (P < .01). Multivariate analysis showed two factors significantly associated with mortality: thoracic herniation of liver (OR 2.27; IC 95% [1.07-4.76]; P = .03) and lung-to-head-ratio over under expected (OR 2.99; IC 95% [1.41-6.36]; P < .01). Side of CDH was not significantly associated with mortality (OR 1.87; IC 95% [0.61-5.51], P = .26). CONCLUSION: Rate of right CDH mortality is more important than left CDH. Nevertheless after adjusting for lung-to-head-ratio and thoracic herniation of liver, right CDH does not have a higher risk of mortality than left CDH.

Our Laparoscopic Surgical Technique and Experience in Treating Pediatric Inguinal Hernia Over the Past Decade.

BACKGROUND: Over the past decade, laparoscopic hernia repair was the most performed operation in our department. Equally, it compromises 15% of all pediatric operations performed. We aim, in this study, to review all the cases performed and extrapolate important information like reoccurrences, the incidence of metachronous inguinal hernia, complications amongst other information. MATERIAL AND METHODS: All patients under the age of 18 whom underwent elective laparoscopic hernia repair between 03/01/2007 till the 18/05/2016 were included in our study. We recorded important clinical features and studied their post-operative follow up. Equally reoccurrences, the incidence of metachronous inguinal hernia, complications and other parameters were recorded and studied. RESULTS: A total of 916 patients were operated on during the defined study period. There was a 0.17% reoccurrence rate and a 0.46% incidence of metachronous inguinal hernia. Equally a contralateral patent processus vaginalis was diagnosed and closed in 17.10%. There were no postoperative complications and we had a 0% postoperative hydrocele rate. CONCLUSION: Laparoscopic hernia repair is safe and carries all the benefits of minimally invasive surgery. We recommend that it is offered to patients and would like to refute previously claimed reports that it carries a higher reoccurrence rate or takes a long time to perform. Our reoccurrence rate of 0.17% is actually lower than many published reoccurrence rates after open repair.

Cytoreductive surgery and hyperthermic intraperitoneal perfusion with chemotherapy in children with peritoneal tumor spread: A French nationwide study over 14 years.

BACKGROUND: Efficacy and role of cytoreductive surgery (CRS) and hyperthermic peritoneal perfusion with chemotherapy (HIPEC) remain poorly documented in pediatric tumors. METHODS: This retrospective national study analyzed all pediatric patients with peritoneal tumor spread treated by CRS and HIPEC as part of a multimodal therapy in France from 2001 to 2015. RESULTS: Twenty-two patients (nine males and 13 females) were selected. The median age at diagnosis was 14.8 years (4.2-17.6). Seven had peritoneal mesotheliomas; seven, desmoplastic small round cells tumors (DSRCT); and eight, other histologic types. A complete macroscopic resection (CC-0, where CC is completeness of cytoreduction) was achieved in 16 (73%) cases. Incomplete resections were classified as CC-1 in four (18%) cases and CC-2 in two (9%) cases. Fourteen (64%) patients had complications within 30 days from HIPEC, requiring an urgent laparotomy in eight (36%) cases. Thirteen (59%) patients received adjuvant chemotherapy and four (18%) received total abdominal radiotherapy after surgery. Sixteen (72%) patients had relapse after a median time of 9.6 months (1.4-86.4) and nine (41%) eventually died after a median time of 5.3 months (0.1-36.1) from relapse. Six (27%) patients (four mesotheliomas, one pseudopapillary pancreatic tumor, and one DSRCT) were alive and in complete remission after a median follow-up of 25.0 months (5.3-78.2). The mean overall survival (OS) and disease-free survival (DFS) were 57.5 months (95% CI [38.59-76.32]) and 30.9 months (95% CI [14.96-46.77]). Patients with a peritoneal mesothelioma had a significantly better OS (p = 0.015) and DFS (p = 0.028) than other histologic type. CONCLUSIONS: In this national series, outcomes of HIPEC are encouraging for the treatment of peritoneal mesothelioma in children.

Endoloop versus endostapler: what is the best option for appendiceal stump closure in children with complicated appendicitis? Results of a multicentric international survey.

BACKGROUND: There is a limited and conflicting evidence about the most appropriate method for appendiceal stump closure during laparoscopic appendectomy (LA). We aimed to compare endoloop (EL) versus endostapler (ES) for stump closure during LA for complicated perforated appendicitis in children. METHODS: We retrospectively reviewed the records of 708 patients (463 boys and 245 girls with an average age of 9.8 years) who underwent LA for complicated appendicitis in 5 international centers of Pediatric Surgery over a 5-years period (January 2011-December 2016). The appendix was perforated with localized peritonitis in 470 cases and diffuse peritonitis in 238 patients. EL was used in 374 cases (G1), whereas ES was adopted in 334 cases (G2). RESULTS: No intra-operative complication occurred in both groups but 5 conversions to open surgery were reported in G1 (1.3%) and 4 in G2 (1.1%) (OR 1.1; 95% CI 0.30-4.19). Use of EL was significantly associated with higher incidence of intra-abdominal abscess (OR 1.36; 95% CI 0.84-2.18), postoperative ileus (OR 3.61; 95% CI 0.76-17.11), and re-operations/readmissions (OR 6.46; 95% CI 1.46-28.62) compared to ES. The average cost of supplies for LA was significantly higher in G2 (€ 915.60) compared to G1 (€ 578.36) (p = 0.0001). The average cost of re-operations/readmissions was significantly higher in G1 (€ 4.091,39) compared to G2 (€ 2.127,88) (p = 0.0001) (OR 1.72; 95% CI 1.47-2.01). CONCLUSIONS: Our study is the first in the pediatric population to demonstrate that the method used for appendiceal stump closure may influence the outcome of LA in complicated appendicitis. Although ES is more expensive compared to EL, our results demonstrated that appendix stump closure should be performed using ES rather than EL in complicated perforated appendicitis since its use was associated with a lower incidence of postoperative intra-abdominal abscess and postoperative ileus and lower re-operations and readmissions rates and costs.

Laparoscopic Management of a Rudimentary Uterine Horn.

STUDY OBJECTIVE: To demonstrate a technique of laparoscopic management of a rudimentary horn in a 12-year-old girl. DESIGN: A step-by-step explanation of the surgery using a video (instructive video) approved by the local institutional review board. SETTING: A university hospital (University Hospital of Strasbourg, Strasbourg, France). PATIENT: A 12-year-old girl with a uterine rudimentary horn. INTERVENTION: We describe a case of a 12-year-old girl who had no medical history. She had her first menstruation at 11 years old with major left pelvic pain occurring each month. Ultrasonography showed a duplication of the uterus with a liquid collection on the left side; this type of malformation is called an accessory and cavitated uterine mass. Medical treatment was initiated with progestin. Magnetic resonance imaging showed a left noncommunicating rudimentary horn with a unicornuate uterus. No other malformation was present, particularly in the kidneys. A primary vaginal endoscopy was performed showing a single cervix without vaginal malformation. It was decided to perform a laparoscopic excision of the left rudimentary horn. We placed a 10-mm optical port into the umbilicus and 3 accessory 5-mm trocars. Evaluation of the abdominal cavity showed 2 normal adnexas with normal ovaries. We decided to start with a left salpingectomy using the Ligasure device (Medtronic, Minneapolis, MN), staying close to the tube to preserve ovarian vascularization. The remnant fimbria must be removed to avoid cancerization. Then, the vesicouterine septum was divided until we reached the cervix to dissect the bladder from the rudimentary horn. The broad ligament was fenestrated in order to push the left ureter laterally .The utero-ovarian pedicle was transected with the Ligasure device; the left ovary was preserved and vascularized by the left infundibulopelvic ligament. We then dissected the left uterine artery. The posterior peritoneum was opened. The resection of the rudimentary horn was performed by means of a monopolar hook. The dissection was performed slowly with selective coagulation until we reached the cavity of the horn, with old blood flowing out. The entire cavity was removed, and we confirmed the absence of communication with the other part of the uterus. Uterine reconstruction was performed with inverted separated stiches of a 2-0 braided suture, and, finally, an antiadhesion barrier was placed. CONCLUSIONS: Laparoscopic management of a uterine rudimentary horn is feasible with satisfactory uterine reconstruction. This is not the first case of this surgery performed by laparoscopy. A similar case has been published in 2015 [1], and recently another video [2] has been published describing 2 other cases.

Current perspectives in robot-assisted surgery.

From classical surgery to robot-assisted surgery (RAS), there has been a long way allowed by the improvements achieved in minimally invasive surgery (MIS). The last three decades have witnessed a prodigious development of MIS, and especially in the field of laparoscopic pediatric surgery but there are several limitations in the use of conventional laparoscopic surgery and RAS was developed to relieve these drawbacks. This new technology enables today the performance of a wide variety of procedures in children with a minimally invasive approach. As for all new technologies, an objective evaluation is essential with the need to respond to several questions: is the technology feasible?, is the technology safe?, is the technology efficient?, does it bring about benefits compared with current technology?, what are the procedures derived from most benefits of robotic assistance?, how to assume the transition from open surgery to minimally invasive access for RAS? In the first part of this article, some details are provided about technical concerns. Then, the implementation process with its organization, pitfalls, successes, and issues from human resources and financial standpoints is described. The learning curve is also analyzed, and a special focus on small children weighing less than 15 kg is developed. Finally, the concept of evaluation of this emerging technology is evocated and financial concerns are developed.

Long-term functional renal outcomes after retroperitoneoscopic upper pole heminephrectomy for duplex kidney in children: a multicenter cohort study.

BACKGROUND: Retroperitoneoscopic upper pole heminephrectomy (RUHN) in duplex kidney in children remains a challenging procedure with a need for postoperative functional assessment of the remnant lower pole. We aimed to calculate the incidence of long-term functional renal outcomes in these children and examine the effect of age on those outcomes. METHODS: A multicenter retrospective cohort study of 9 years included all patients undergoing RUHN and evaluated by renal ultrasound (US) and dimercaptosuccinic acid (DMSA) scintigraphy pre and postoperatively. Patients were divided in two age groups of ≤12 and >12 months. Standard follow-up assessed pre-, intra- and postoperative outcomes using clinical review, US and DMSA. RESULTS: Standard RUHN in lateral position was performed in 30 patients. Five cases were excluded (2 lacks of postoperative DMSA, 3 conversions). Indications for RUHN were non-functioning upper moieties (n = 25) caused by ureterocele (n = 11), ectopic distal implantation of the ureter with incontinence (n = 6) or evolving severe ureterohydronephrosis (n = 8). Mean age at surgery was 30 ± 27 months, operation time 116 ± 52 min and hospital stay 2.8 ± 1 days. Long-term follow-up (mean, 7.2 ± 2.7 years) with US and DMSA showed that none of the 25 patients had complete loss of lower pole renal function. Mean lower pole renal function directly related to RUHN was not significantly different after versus before RUHN for the entire cohort (n = 24; 39.7 ± 7.90 % vs. 41.7 ± 6.74 %; p = 0350), for the ≤12-month (n = 6; 39.3 ± 4.18 vs. 41.3 ± 5.47; p = 0.493) and the >12-month groups (n = 18; 39.8 ± 8.90 vs. 41.9 ± 7.25; p = 0.443). Four patients (17 %) had partial loss of function (mean function loss, 9.3 ± 5.85 %; median age, 13 months). The number and type of complications between the two age groups were not statistically different. Overall, 29 % (n = 7/24) of the patients presented with medium-term (17 %) and long-term (17 %) complications directly related to RUHN. CONCLUSIONS: RUHN is a demanding yet efficient technique that is safe for the lower pole at any age. Systematic postoperative DMSA is not mandatory as long as US remains normal.

Technical standardization of laparoscopic repair of Morgagni diaphragmatic hernia in children: results of a multicentric survey on 43 patients.

BACKGROUND: This study aimed to standardize the surgical correction technique of congenital Morgagni diaphragmatic hernia (CMDH), analyzing the results of an international multicentric survey. METHODS: The medical records of 43 patients (29 boys, 14 girls) who underwent laparoscopic repair of CMDH in 8 pediatric surgery units in a 5-year period were retrospectively reviewed. Their average age was 3.3 years. Ten patients (23.2%) presented associated malformations: 9 Down syndrome (20.9%) and 1 palate cleft (2.3%). Thirty-five patients (81.4%) were asymptomatic, whereas 8 patients (18.6%) presented symptoms such as respiratory distress, cough or abdominal pain. As for preoperative work-up, all patients received a chest X-ray (100%), 15/43 (34.8%) a CT scan, 8/43 (18.6%) a barium enema and 4/43 (9.3%) a US. RESULTS: No conversion to open surgery was reported. Average operative time was 61.2 min (range 45-110 min). In 38/43 (88.3%) patients, a trans-parietal stitch was positioned in order to reduce the tension during the repair. In 14/43 cases (32.5%), the sac was resected; in only 1/43 case (2.3%) a dual mesh of goretex was adopted to reinforce the closure. Average hospital stay was 2.8 days. The average follow-up was 4.2 years, and it consisted in annual clinical controls and chest X-ray. We recorded 2 complications (4.6%): one small pleural opening that required no drain and one recurrence (2.3%), re-operated in laparoscopy, with no further recurrence. CONCLUSIONS: To the best of our knowledge, this is the largest series published in the literature on this topic. Laparoscopic CMDH repair is well standardized: The full-thickness anterior abdominal wall repair using non-resorbable suture with interrupted stitches is the technique of choice. Postoperative outcome was excellent. Recurrence rate was very low, about 2% in our series. We believe that children with CMDH should be always treated in laparoscopy following the technical details reported in this paper.

Prevalence of Barrett Esophagus in Adolescents and Young Adults With Esophageal Atresia.

OBJECTIVE: To study the prevalence of Barrett esophagus (BE) (gastric and/or intestinal metaplasia) in adolescents treated for esophageal atresia (EA). SUMMARY OF BACKGROUND DATA: EA patients are at high risk of BE. METHODS: This multicenter prospective study included EA patients aged 15 to 19 years. All eligible patients were proposed an upper endoscopy with multistaged esophageal biopsies under general anesthesia. Histological suspicion of metaplasia was confirmed centrally. RESULTS: One hundred twenty patients [mean age, 16.5 years (±1.4)] were included; 70% had been treated for gastroesophageal reflux disease (GERD) during infancy. At evaluation, 8% were undernourished, 41% had received antireflux surgery, and 41% presented with GERD symptoms, although only 28% were receiving medical treatment. Esophagitis was found at endoscopy in 34% and confirmed at histology in 67%. BE was suspected after endoscopy in 37% and was confirmed by histology for 43% of patients (50 gastric and 1 intestinal metaplasia). No endoscopic or histological anomalies were found at the anastomosis site. BE was not significantly related to clinical symptoms. In multivariate analysis, BE was associated with EA without fistula (P = 0.03), previous multiple antireflux surgery (P = 0.04), esophageal dilation (P = 0.04), suspicion of BE at endoscopy (P < 0.001), and histological esophagitis (P = 0.02). CONCLUSIONS: Patients with EA are at high risk of persistent GERD and BE. The development of BE is related to GERD history. Long-term systematic follow-up of the esophageal mucosa including multistaged biopsies is required, even in asymptomatic patients. (NCT02495051).

Recurrence of Solid Pseudopapillary Neoplasms of the Pancreas: Results of a Nationwide Study of Risk Factors and Treatment Modalities.

BACKGROUND: Solid pseudopapillary neoplasms of the pancreas (SPPN) can relapse very late, but little is known about risk factors for recurrence and optimal treatment. We aimed to identify risk factors for recurrence and to analyze treatment modalities in all French pediatric cases of SPPN over the past 20 years. MATERIAL AND METHODS: Data were collected from pediatric oncologists and surgeons, and also from adult pancreatic surgeons in order to identify late recurrences. RESULTS: Fifty-one patients (41 girls) were identified. Median age at diagnosis was 13.1 years [8.7-17.9]. Abdominal pain was the commonest presenting symptom (32/49, 65%). The tumor was located in the pancreatic head in 24 patients (47%). Preoperative biopsy or cytology was performed in 14 cases (28%). All patients were operated with a median of 23 days [0-163] after diagnosis. The rate of postoperative morbidity was 29%. With a median follow-up of 65 months [0.3-221], the overall and event-free survival was 100% and 71%, respectively. Seven patients (13.7%) relapsed with a median of 43 months [33-94] after initial surgery. Six were treated surgically, either alone (n = 3) or with perioperative chemotherapy (n = 2) or hyperthermic intraperitoneal chemotherapy (n = 1). One patient in whom further treatment was not feasible was still alive at last news. Risk factors for recurrence were positive surgical margins (P = 0.03) and age less than 13.5 years at diagnosis (P = 0.03). CONCLUSIONS: SPPN recurrence in this pediatric series was a rare and late event that did not undermine overall survival. Complete surgical removal of recurrent tumors appears to be the best option.

Staged gastroschisis closure using Alexis wound retractor: first experiences.

INTRODUCTION: The aim of this study is to analyze the effectiveness of an Alexis wound retractor (AWR) device for staged gastroschisis closures. PATIENTS AND METHODS: AWR device was used to cover unreduced viscera of a gastroschisis when primary abdominal wall closure was not convenient. The eviscerated organs were covered with one of the two spring-loaded rings of the AWR inserted underneath the abdominal wall. Gradual reduction was guaranteed through careful traction on the external ring. We retrospectively analyzed the prenatal, post-natal and operative data of the first patients treated with AWR and report their post-operative outcomes. RESULTS: The AWR device was used for staged closure in eight cases. Complete reduction and fascial closure were performed at a median of 3.5 ± 1.6 days. Ventilatory support was necessary for 4.0 ± 3 days and full parenteral feeds for 7.5 ± 6.1 days after fascial closure. Median full enteral feeding was observed at 18 ± 12.5 days after closure allowing discharge in a median period of 30.5 ± 15.6 days after closure. CONCLUSION: The AWR device is not only a safe and efficient silo for a progressive reduction of severe gastroschisis, but also an interesting tool for continuous stretching leading to an increase of the peritoneal cavity volume, enhancing the equalizing of the viscero-abdominal disproportion.

Observational, medical or surgical management of vesicoureteric reflux.

UNLABELLED: The clinical management of vesicoureteric reflux includes observational, medical and surgical procedures. The choice of management is often a joint decision made between the paediatric nephrologist and urologist. The use of prophylactic antibiotics has become increasingly debated. In recent years, the surgical treatment of reflux (including endoscopic intervention or ureteral reimplantation) has mainly been limited to cases of high-grade reflux. There are several important risk factors that influence the final outcome which need to be identified and treated. The aim of reflux management is no longer to treat imperatively, but rather to avoid renal damage. It is perhaps time to revise the classic saying 'diagnosed reflux - treated reflux' with a new objective 'diagnosed reflux - evaluated reflux'. CONCLUSION: The management and follow-up of childhood reflux is a joint decision between the paediatric urologist and nephrologist and should be decided on a case-by-case basis.

Pectus Arcuatum: A Pectus Unlike Any Other.

BACKGROUND: Pectus arcuatum is often mistaken for a type of pectus carinatum. However, pectus arcuatum is a unique clinical form of pectus caused by premature obliteration of the sternal sutures (manubrial sternum, four sternebrae and xiphoïd process), whereas pectus carinatum is due to abnormal growth of the costal cartilage. In order to better describe pectus arcuatum, we analysed the files of patients with pectus arcuatum followed in our centers. METHODS: Multicenter retrospective study of young patients' files diagnosed with pectus arcuatum. RESULTS: The clinical diagnosis of pectus arcuatum was made in 34 patients with a mean age at diagnosis of 10.3 years (4-23 years). A chest profile X-ray or a CT scan was performed in 16 patients (47%) and confirmed the diagnosis of PA by the presence of a sternal fusion. It was complete in 12 patients. A malformation was associated in 35% of cases (Noonan syndrome 33%, scoliosis 25% or cardiopathy 16%). 11 patients (32%) had a family history of skeletal malformation. Orthopedic treatment was initiated in 3 patients without any success. 11 patients underwent surgical correction, which was completed in 7 of them. CONCLUSION: The diagnosis of pectus arcuatum is based on clinical experience and if necessary, on a profile chest X-ray showing the fusion of the sternal pieces. It implies the search for any associated malformations (musculoskeletal, cardiac, syndromic). Bracing treatment is useless for pectus arcuatum. Corrective surgery, based on a sternotomy associated with a partial chondro-costal resection, can be performed at the end of growth. LEVEL OF EVIDENCE: IV.

A 16-year bicentric retrospective analysis of ovarian tissue cryopreservation in pediatric units: indications, results, and outcome.

Background: Cancer treatments of the last decades improve the survival rate of children and adolescents. However, chemo- and radiotherapy result in gonadal damage, leading to acute ovarian failure and sterility. The preservation of fertility is now an integral part of care of children requiring gonadotoxic treatments. Ovarian tissue cryopreservation (OTC) is an effective fertility preservation option that allows long-term storage of primordial follicles, subsequent transplantation, and restoration of endocrine function and fertility. The efficacy of this technique is well-demonstrated in adults but the data are scarce for pediatric patients. Currently, OTC represents the only possibility of preserving the potential fertility in prepubertal girls. Procedure: This is a retrospective study of OTC practice of two French centers from January 2004 to May 2020. A total of 72 patients from pediatric units underwent cryopreservation of ovarian tissue before gonadotoxic therapy for malignant or non-malignant diseases. The ovarian cortex was cut into fragments and the number of follicles per square millimeter was evaluated histologically. The long-term follow-up includes survival rate and hormonal and fertility status. Results: The mean age of patients at OTC was 9.3 years [0.2-17] and 29.2% were postpubertal; 51 had malignant diseases and 21 had non-malignant diseases. The most frequent diagnoses included acute leukemia, hemoglobinopathies, and neuroblastoma. Indication for OTC was stem cell transplantation for 81.9% (n = 59) of the patients. A third of each ovary was collected for 62.5% (n = 45) of the patients, a whole ovary for 33.3% (n = 24) of the patients, and a third of one ovary for 4.2% (n = 3) of the patients. An average of 17 fragments [5-35] per patient was cryoconserved. A correlation was found between the age of the patients and the number of fragments (p < 0.001). More fragments were obtained from partial bilateral harvesting than from whole ovary harvesting (p < 0.05). Histological analysis of ovarian tissue showed a median of 6.0 primordial follicles/mm2 [0.0-106.5] and no malignant cells were identified. A negative correlation was found between age and follicular density (p < 0.001). Median post-harvest follow-up was 92 months [1-188]. A total of 15 girls had died, 11 were still under treatment for their pathology, and 46 were in complete remission. Of all patients, 29 (40.2%) were subjected to a hormonal status evaluation and 26 were diagnosed with premature ovarian insufficiency (POI) (p < 0.001). One patient had undergone thawed ovarian tissue transplantation. Conclusion: OTC should be proposed to all girls with high risk of developing POI following gonadotoxic therapies in order to give them the possibility of fertility and endocrine restoration.

Changes in the clinical management of 5α-reductase type 2 and 17ß-hydroxysteroid dehydrogenase type 3 deficiencies in France.

Objectives: To examine the changes in diagnostic practices and clinical management of patients with 5α-reductase type 2 (SRD5A2) or 17ß-hydroxysteroid dehydrogenase type 3 (HSD17B3) deficiency since molecular diagnoses became available. Methods: Clinical, laboratory, and therapeutic data were retrieved from the medical records of 52 patients with a molecular diagnosis of SRD5A2 (n = 31) or HSD17B3 (n = 21) deficiency. Temporal trends regarding age at assessment and initial sex assignment over 1994-2020 were qualitatively analyzed. Age at molecular diagnosis was compared between two subgroups of patients according to their year of birth. Results: Fifty-eight percent (n = 30) patients were diagnosed during the perinatal period, 33% (n = 17) during infancy, and 9% (n = 5) during adolescence or adulthood. Over the studied period, the patients' age at initial assessment and diagnosis frankly decreased. The median (range) age at diagnostic confirmation was 10.5 (0-53.2) years for patients born before 2007 and 0.4 (0-9.3) years for those born in 2007 or later (P = 0.029). Genetic testing identified 27 different variants for the SRD5A2 gene (30% novel, n = 8) and 18 for the HSD17B3 gene (44% novel, n = 8). Before 2002, most patients were initially assigned as females (95%, n = 19), but this proportion dropped for those born later (44%, n = 14; P < 0.001). The influence of initial genital appearance on these decisions seemingly decreased in the most recent years. Therapeutic interventions differed according to the sex of rearing. Ten percent (n = 2) patients requested female-to-male reassignment during adulthood. Conclusion: This study showed, over the past two decades, a clear trend toward earlier diagnosis and assignment of affected newborns as males.

Outcome of alimentary tract duplications operated on by minimally invasive surgery: a retrospective multicenter study by the GECI (Groupe d'Etude en Coeliochirurgie Infantile).

BACKGROUND: Alimentary tract duplications (ATD) are a rare cause of intestinal obstruction in childhood. There are many case reports but few series about laparoscopy or thoracoscopy for ATD. The aim of our study was to report the outcome of minimally invasive surgery (MIS) for ATD. METHODS: This was a retrospective multicenter study from the GECI (Groupe d'Etude en Coeliochirurgie Infantile). We reviewed the charts of 114 patients operated on by MIS for ATD from 1994 to 2009. RESULTS: Sixty-two patients (54 %) had a prenatal diagnosis. Forty-nine patients (43 %) were symptomatic before surgery: 33 of those patients (63 %) with postnatal diagnosis compared to 16 (25 %) with prenatal diagnosis (P < 0.01). In this last group, the median age at onset of symptoms was 16 days (range = 0-972). One hundred and two patients had laparoscopy (esophageal to rectal duplications) and 12 patients had thoracoscopy for esophageal duplications. The mean operative time was 90 min (range = 82-98). There were 32 (28 %) resection anastomoses, 55 (48 %) enucleations, and 27 (24 %) unroofings. The conversion rate was 32 %, and in a multivariate analysis, it was significantly higher, up to 41 % for patients weighing <10 kg (P < 0.01). Ten patients (8 %) had unintentional perioperative opening of the digestive tract during the dissection. Eight patients had nine postoperative complications, including six small bowel obstructions. The median length of hospital stay was 4 days (range = 1-21) without conversion and 6 days (range = 1-27) with conversion (P = 0.01). The median follow-up was 3 months (range = 1-120). Eighteen of the 27 patients who underwent partial surgery had an ultrasound examination during follow-up. Five (18 %) of them had macroscopic residue. CONCLUSION: This study showed that MIS for ATD is feasible with a low rate of complications. Patients with prenatal diagnosis should have prompt surgery to prevent symptoms, despite a high rate of conversion in small infants.

Anti-reflux surgery in children with congenital diaphragmatic hernia: A prospective cohort study on a controversial practice.

INTRODUCTION: Gastro-esophageal reflux disease (GERD) is the most frequent long-term morbidity of congenital diaphragmatic hernia (CDH) survivors. Performing a preventive fundoplication during CDH repair remains controversial. This study aimed to: (1) Analyze the variability in practices regarding preventive fundoplication; (2) Identify predictive factors for fundoplication. (3) Evaluate the impact of preventive fundoplication on gastro-intestinal outcomes in children with a CDH patch repair; METHODS: This prospective multi-institutional cohort study (French CDH Registry) included CDH neonates born in France between January 1st, 2010-December 31st, 2018. Patch CDH was defined as need for synthetic patch or muscle flap repair. Main outcome measures included need for curative fundoplication, tube feed supplementation, failure to thrive, and oral aversion. RESULTS: Of 762 CDH neonates included, 81 underwent fundoplication (10.6%), either preventive or curative. Median follow-up was 3.0 years (IQR: 1.0-5.0). (1) Preventive fundoplication is considered in only 31% of centers. The rates of both curative fundoplication (9% vs 3%, p = 0.01) and overall fundoplication (20% vs 3%, p < 0.0001) are higher in centers that perform preventive fundoplication compared to those that do not. (2) Predictive factors for preventive fundoplication were: prenatal diagnosis (p = 0.006), intra-thoracic liver (p = 0.005), fetal tracheal occlusion (p = 0.002), CDH-grade C-D (p < 0.0001), patch repair (p < 0.0001). After CDH repair, 8% (n = 51) required curative fundoplication (median age: 101 days), for which a patch repair was the only independent predictive factors identified upon multivariate analysis. (3) In neonates with patch CDH, preventive fundoplication did not decrease the need for curative fundoplication (15% vs 11%, p = 0.53), and was associated with higher rates of failure to thrive (discharge: 81% vs 51%, p = 0.03; 6-months: 81% vs 45%, p = 0.008), tube feeds (6-months: 50% vs 21%, p = 0.02; 2-years: 65% vs 26%, p = 0.004), and oral aversion (6-months: 67% vs 37%, p = 0.02; 1-year: 71% vs 40%, p = 0.03). CONCLUSIONS: Children undergoing a CDH patch repair are at high risk of requiring a curative fundoplication. However, preventive fundoplication during a patch repair does not decrease the need for curative fundoplication and is associated with worse gastro-intestinal outcomes in children. LEVEL OF EVIDENCE: II - Prospective Study.