Diagnosis and Management of Congenital H-Type Tracheoesophageal Fistula: Results of a National Survey.

BACKGROUND: Congenital h-type tracheoesophageal fistula (H-TEF) without esophageal atresia (EA) represents about 4% of congenital esophageal anomalies. The diagnosis is challenging, and surgery is considered curative. The aim was to report a national survey on the diagnosis, management, and outcome of patients with congenital H-TEF. METHODS: Following approval of the Italian Society of Pediatric Surgery, a survey was sent to all Pediatric Surgery Units to retrospectively collect H-TEF treated in the period 2010-2022. Descriptive analysis was performed, and results are given as prevalence, mean ± standard deviation (SD), or median and interquartile range (IQR). RESULTS: The survey was sent to 65 units. Seventeen responded with one or more cases; 78 patients were diagnosed with H-TEF during the study period. Associated malformations were present in 43%, mostly cardiac (31%). The most frequent symptoms were cough (36%), bronchopneumonia (24%), and dysphagia (19%). H-TEF was detected by tracheobronchoscopy (90%), and/or upper GI (58%), and/or esophagoscopy (32%). The median age at diagnosis was 23 days (1 day-18 years). The most common approach was cervicotomy (76%), followed by thoracoscopy (14%) and thoracotomy (9%). The fistula underwent ligation and section of the fistula in 90% of the patients and clip closure and section in 9%. In one patient, the fistula was cauterized endoscopically. H-TEF preoperative cannulation was performed in 68% of cases, and a drain was placed in 26%. One month after surgery, 13% of the patients had mild persisting symptoms, mainly hypophonia. Recurrence occurred in 5%, and a second recurrence occurred in 1%. CONCLUSIONS: H-TEF prevalence was six cases/year, consistent with the expected rate of five cases/year in our country. The diagnosis was challenging, sometimes delayed, and, in most patients, required multiple examinations. Fistula ligation and section through cervicotomy were the most frequent treatment. Long-term outcomes are good, and recurrence is a rare event.

Congenital lung malformations: a nationwide survey on management aspects by the Italian Society of Pediatric Surgery.

INTRODUCTION: Over the years, congenital lung malformations (CLM) management remains a controversial topic in pediatric thoracic surgery. The Italian Society of Pediatric Surgery performed a national survey to study the current management variability among centers, trying to define national guidelines and a standardized approach of children with congenital lung malformations. METHODS: Following a National Society approval, an electronic survey including 35 items on post-natal management was designed, focusing on surgical, anesthesiology, radiology and pneumology aspects. The survey was conducted contacting all pediatric surgical units performing thoracic surgery. RESULTS: 39 pediatric surgery units (97.5%) participated in the study. 13 centers (33.3%) were classified as high-volume (Group A), while 26 centers (66.7%) were low volume (Group B). Variances in diagnostic imaging protocols were observed, with Group A performing fewer CT scans compared to Group B (p = 0.012). Surgical indications favored operative approaches for asymptomatic CLM and pulmonary sequestrations in both groups, while a wait-and-see approach was common for congenital lobar emphysema. Surgical timing for asymptomatic CLM differed significantly, with most high-volume centers operating on patients younger than 12 months (p = 0.02). Thoracoscopy was the preferred approach for asymptomatic CLM in most of centers, while postoperative long-term follow-up was not performed in most of the centers. CONCLUSION: Thoracoscopic approach seems uniform in asymptomatic CLM patients and variable in symptomatic children. Lack of uniformity in surgical timing and preoperative imaging assessment has been identified as key areas to establish a common national pattern of care for CLM.

Management and outcomes of acute appendicitis in children during the COVID-19 pandemic: a systematic review and meta-analysis.

The COVID-19 pandemic has changed the way to manage the emergencies, as people faced fear of the hospitals, with possible delay in the diagnosis. Moreover, clinicians had to rearrange protocols for diagnosis and treatment. We aimed to assess whether COVID-19 pandemic influenced severity of inflammation, management, and outcomes of acute appendicitis (AA), when compared to the pre-COVID era. Using defined search strategy, two independent investigators identified those studies comparing pediatric AA during COVID-19 pandemic versus the pre-COVID-19 period. Meta-analysis was performed using RevMan 5.3. Data are mean ± SD. Of 528 abstracts, 36 comparative studies were included (32,704pts). Time from symptoms onset to surgery was longer during the pandemics compared to the pre-COVID-19 (1.6 ± 0.9 versus 1.4 ± 0.9 days; p < 0.00001). Minimally Invasive Surgery was similar during COVID-19 (70.4 ± 30.2%) versus control period (69.6 ± 25.3%; p = ns). Complicated appendicitis was increased during the pandemics (35.9 ± 14.8%) compared to control period (33.4 ± 17.2%; p < 0.0001). Post-operative complications were comparable between these two groups (7.7 ± 6.5% versus 9.1 ± 5.3%; p = ns). It seems that the COVID-19 pandemic influenced the time of diagnosis, severity of inflammation, and type of surgery. However, the number of post-operative complications was not different between the two groups, leading to the conclusion that the patients were correctly managed. LEVEL OF EVIDENCE: Level 3 Meta-analysis on Level 3 studies.

Pneumovesicoscopic management of bladder neoplasms in children: three case reports.

Urothelial bladder neoplasms (UBN) are uncommon in children and are poorly understood. Their management is contentious, and there are currently no pediatric guidelines available, making it difficult to envision a surgical approach that can be defined as the gold standard for the treatment of these diseases. Pneumovesicoscopy, which has already been used to treat other urological diseases, could be a promising treatment option for selected cases of this group of pathologies. We present our experience with three pediatric UBN cases in which pneumovesicoscopy was used for complete excision of a perimeatal papilloma in two cases and biopsy of a botryoid rhabdomyosarcoma in one. The pneumovesicoscopic approach, in our experience, provided a viable alternative technique for the management of selected cases of UBN.

Rectal atresia and rectal stenosis: the ARM-Net Consortium experience.

PURPOSE: To assess the number, characteristics, and functional short-, and midterm outcomes of patients with rectal atresia (RA) and stenosis (RS) in the ARM-Net registry. METHODS: Patients with RA/RS were retrieved from the ARM-Net registry. Patient characteristics, associated anomalies, surgical approach, and functional bowel outcomes at 1 and 5-year follow-up were assessed. RESULTS: The ARM-Net registry included 2619 patients, of whom 36 (1.3%) had RA/RS. Median age at follow-up was 7.0 years (IQR 2.3-9.0). Twenty-three patients (63.9%, RA n = 13, RS n = 10) had additional anomalies. PSARP was the most performed reconstructive surgery for both RA (n = 9) and RS (n = 6) patients. At 1-year follow-up, 11/24 patients with known data (45.8%, RA n = 5, RS n = 6) were constipated, of whom 9 required stool softeners and/or laxatives. At 5-year follow-up, 8/9 patients with known data (88.9%, RA n = 4, RS n = 4) were constipated, all requiring laxatives and/or enema. CONCLUSION: RA and RS are rare types of ARM, representing 1.3% of patients in the ARM-Net registry. Additional anomalies were present in majority of patients. Different surgical approaches were performed as reconstructive treatment, with constipation occurring in 46% and 89% of the patients at 1 and 5-year follow-up. However, accurate evaluation of long-term functional outcomes remains challenging.

Perinatal diagnosis of congenital urogenital sinus abnormality.

Anomalies of the urogenital sinus, which is a transient feature of the early human embryological development, are rare birth defects. Urogenital sinus abnormalities commonly present as pelvic masses, hydrometrocolpos, or ambiguous genitalia and most commonly occur within the context of congenital adrenal hyperplasia. Anomalies of the urogenital sinus requires surgical repair. We experienced a case of a female newborn with congenital urogenital sinus abnormality in which the early diagnosis helped us to prevent complications by decompressing the vagina soon after birth. Antibiotic prophylaxis was sufficient to avoid infections and to decompress the genitourinary system, thus allowing a deferred elective surgery to correct the sinus.

Pediatric primary spontaneous pneumothorax: a comparison of treatment at pediatric surgery vs. thoracic surgery departments.

Management of pediatric Primary Spontaneous Pneumothorax (PSP) is controversial and based on guidelines on adults. Therapeutic strategies include: observation, needle aspiration, chest drain, or surgery. We aimed to assess: i) differences in the management of PSP in pediatric vs. adult departments; ii) risk of recurrence associated to each therapeutic choice; iii) management of "large" pneumothorax (i.e. >3cm at the apex on chest X-Ray); iv) role of CT scan in addressing the treatment. We reviewed all PSP treated at Pediatric Surgery Unit (PSU) and Thoracic Surgery Unit for adults (TSU) in a 10-year period (2011 to 2020). We included a total of 42 PSP: 30/42 1st episodes and 12/42 recurrences. Among the 30/42 1st episodes, 15/30 were managed in the PSU and 15/30 in the TSU. Observation was significantly most common among PSU patients (9/15, 60%) vs. TSU cases (1/15, 6.7%; p=0.005]. Chest drain placement was reduced in PSU (3/15, 20%) vs. TSU (12/15, 80%; p=0.002). Observational was associated with a reduced risk of recurrence (0/10, 0%) compared to chest drain (7/15, 46.7%; p=0.01). Management of 20/42 "large" pneumothorax was: 4/20 (20%) observation, 10/20 (50%) chest drain, 2/20 (10%) needle aspiration, 4/20 (20%) surgery. Twentythree/ 29 PSP (79.3%) underwent CT-scan after the first episode. Bullae were detected in 17/23 patients and 5/17 (29.4%) had seven episodes of recurrence. PSP patients treated by PSU were more likely to receive clinical observation. Those managed by TSU were mostly treated by chest drain. Observation seems an effective choice for clinically stable PSP, with low risk of recurrence at a mid-term follow-up. CT-scan seems not to detect those patients at higher risk of recurrence.

Current Status of Pediatric Robot-Assisted Surgery in Italy: Epidemiologic National Survey and Future Directions.

Background: Pediatric robot-assisted surgery (RAS) is gaining increasing acceptance. We aimed to assess the diffusion of pediatric RAS in Italy, the training period, indications, preliminary outcomes, and limitations. Materials and Methods: An online questionnaire-based survey was performed. The data about robotic activity of 9 Italian Pediatric Surgery units were collected and analyzed. Results: Most of the participating centers (7/9, 77.8%) started RAS less than 5 years ago with only 2 centers (22.2%) performing RAS since 2010. The training included dry-lab in 5/9 centers (55.5%), wet-lab in 5/9 centers (55.5%), and robot simulator in 7/9 centers (77.8%), followed by an exam to obtain a certificate. The average duration of training was 23.7 hours (range 5-50). A total of 209 robotic procedures was performed in all centers during the period 2010-2018 and included 119 urological (56.9%), 31 gynecological (14.8%), 41 gastrointestinal (19.6%), 12 oncological (5.7%), and 6 other (2.8%) procedures. The docking time significantly fell down after 18 robotic procedures (P = .001). Intraoperative complications were recorded in 4 cases (1.9%). Conversion to laparoscopy was needed in 4 cases (1.9%) whereas conversion to open was required in 6 cases (2.8%). Postoperative complications occurred in 17/209 cases (8.1%) and were graded Clavien I-II in 14 cases (6.7%) and Clavien IIIb in only 4 cases (1.9%). Conclusions: Our study confirmed that RAS has still a limited diffusion in Italy for pediatric patients. Before starting robotic activity, pediatric surgeons have to obtain a certificate after a virtual and experimental training period. A mentorship clinical period of 10 cases under supervision of a proctor is also needed. The main indications in children remain reconstructive urological procedures. RAS is safe in children but its applications are currently limited to patients older than 2 years and with a weight >15 kg, due to the size of robotic ports.

Ovarian torsion in the pediatric population: predictive factors for ovarian-sparing surgery-an international retrospective multicenter study and a systematic review.

STUDY OBJECTIVE: Ovarian torsion (OT) in pediatric age is a challenging condition to diagnose and treat. To date, there is still no clear consensus about its management. Our aim was to assess some possible associated factors that can help surgeons in decision-making. DESIGN: We conducted a retrospective multicentric study of pediatric OT surgically treated between 2010 and 2020 in six Italian and German institutions, comparing our findings with a literature review of the last 10 years (2010-2020). PARTICIPANTS: Patients aged 0-18 years with a diagnosis of OT intraoperatively confirmed and surgically treated at the involved institutions. RESULTS: Ninety-seven patients with a mean age at diagnosis of 8.37 years were enrolled in the study. Severe abdominal pain was present in 82 patients (84.5%). Eighty children (82.5%) presented an enlarged ovary with an US diameter > 5 cm and only 32 (40%) of them underwent conservative surgery. A laparoscopic approach was performed in 60 cases (61.9%) although in 15 (15.5%) conversion to open surgery was deemed necessary. A functional cyst was present in 49 patients (50.5%) while 11 children (11.3%) suffered from OT on a normal ovary. CONCLUSIONS: Our results showed that a post-menarchal age (p = .001), a pre-operative US ovarian size < 5 cm, (p = .001), the presence of severe abdominal pain (p = .002), a laparoscopic approach (p < .001), and the presence of a functional cyst (p = .002) were significantly associated with conservative surgery.

Ureteropelvic junction obstruction in infants: Open or minimally invasive surgery? A systematic review and meta-analysis.

Introduction: The historical gold standard treatment for ureteropelvic junction obstruction (UPJO) was the open Anderson-Hynes dismembered pyeloplasty (OP). Minimally invasive surgery (MIS) procedures, including laparoscopic pyeloplasty (LP) and robot-assisted laparoscopic pyeloplasty (RALP), have been reported to achieve better outcomes (i.e., decreased morbidity, reduced postoperative pain, superior esthetic results, and shortened length of hospital stay, LOS), with a success rate similar to OP. The main limitation of the MIS approach is the age and weight of patients, limiting these procedures to children >1 year. This study aims to evaluate the feasibility and benefits of MIS pyeloplasty compared to OP to surgically treat UPJO in children <1 year of age. Materials and methods: A systematic review was independently performed by two authors. Papers comparing both techniques (MIS pyeloplasty vs. OP) in infants were included in the meta-analysis. Data (mean ± DS or percentage) were analyzed using Rev.Man 5.4 A p < 0.05 was considered significant. Results: Nine studies (eight retrospective and one prospective) meet the inclusion criteria. A total of 3,145 pyeloplasties have been included, with 2,859 (90.9%) OP and 286 (9.1%) MIS. Age at operation was 4.9 ± 1.4 months in OP vs. 5.8 ± 2.2 months in MIS, p = ns. Weight at surgery was 6.4 ± 1.4 kg in OP vs. 6.9 ± 1.4 kg in MIS, p = ns. Operative time was 129.4 ± 24.1 min for OP vs. 144.0 ± 32.3 min for MIS, p < 0.001. LOS was 3.2 ± 1.9 days for OP vs. 2.2 ± 0.9 days for MIS, p < 0.01. Postoperative complications were present in 10.0 ± 12.9% of OP vs. 10.9 ± 11.6% in MIS, p = ns. Failure of surgery was 5.2 ± 3.5% for OP vs. 4.2 ± 3.3% for MIS, p = ns. Conclusion: The development of miniaturized instruments and technical modifications has made MIS feasible and safe in infants and small children. MIS presented a longer operative time than OP. However, MIS seemed effective for treating UPJO in infants, showing shortened LOS compared to OP. No differences have been reported with regard to the incidence of postoperative complications and failure of pyeloplasty. Given the low quality of evidence of the meta-analysis according to the GRADE methodology, we would suggest limiting MIS procedures in infants to only those high-volume centers with experienced surgeons.

Bowel function and associated risk factors at preschool and early childhood age in children with anorectal malformation type rectovestibular fistula: An ARM-Net consortium study.

BACKGROUND: Outcome of patients operated for anorectal malformation (ARM) type rectovestibular fistula (RVF) is generally considered to be good. However, large multi-center studies are scarce, mostly describing pooled outcome of different ARM-types, in adult patients. Therefore, counseling parents concerning the bowel function at early age is challenging. Aim of this study was to evaluate bowel function of RVF-patients at preschool/early childhood age and determine risk factors for poor functional outcome. METHODS: A multi-center cohort study was performed. Patient characteristics, associated anomalies, sacral ratio, surgical procedures, post-reconstructive complications, one-year constipation, and Bowel Function Score (BFS) at 4-7 years of follow-up were registered. Groups with below normal (BFS < 17; subgroups 'poor' ≤ 11, and 'fair' 11 < BFS < 17) and good outcome (BFS ≥ 17) were formed. Univariable analyses were performed to detect risk factors for outcome. RESULTS: The study included 111 RVF-patients. Median BFS was 16 (range 6-20). The 'below normal' group consisted of 61 patients (55.0%). Overall, we reported soiling, fecal accidents, and constipation in 64.9%, 35.1% and 70.3%, respectively. Bowel management was performed in 23.4% of patients. Risk factors for poor outcome were tethered cord and low sacral ratio, while sacral anomalies, low sacral ratio, prior enterostomy, post-reconstructive complications, and one-year constipation were for being on bowel management. CONCLUSIONS: Although median BFS at 4-7 year follow-up is nearly normal, the majority of patients suffers from some degree of soiling and constipation, and almost 25% needs bowel management. Several factors were associated with poor bowel function outcome and bowel management. LEVEL OF EVIDENCE: Level III.

Surgical management of ovarian teratomas in childhood: a multicentric study on 110 cases and a literature review.

AIM OF THE STUDY: To compare a multicentric surgical experience on ovarian teratomas in childhood with the current management trends. DESIGN: A retrospective multicentric pediatric ovarian teratomas surgically treated between January 2000 and August 2020 at four Italian institutions. PubMed database was used to search for Reviews and Systematic Reviews published between January 2010 and August 2020: 15 manuscripts reported 3633 ovarian neoplasms in pediatric age, 1219 (33,5%) of which were ovarian teratomas. RESULTS: A hundred-ten patients with a mean age at diagnosis of 11.8 years were enrolled. Mature cystic teratomas accounted for the 90% of the masses. At surgery, 78 were oophorectomies and 32 were ovary sparing surgeries. Laparoscopy occurred in 16.3% of the surgeries.As regarding the current management trends, the mean age at diagnosis was 11.9 years and 80.5% of the cases were represented by mature teratomas. Of 430 procedures, 331 were oophorectomies while 99 were ovary sparing surgeries and laparoscopy was performed in 23.8% of cases. CONCLUSIONS: Ovary-sparing surgery with laparoscopic approach is increasingly offered as standard treatment for benign masses that fit the criteria for mature teratomas, in the attempt to achieve the best compromise between the preservation of fertility and the prevention of recurrences. Awareness should be raised among pediatric surgeons to reduce unnecessary radical surgery.

Minimally invasive vascular hitch to treat pediatric extrinsic ureteropelvic junction obstruction by crossing polar vessels: A systematic review and meta-analysis.

INTRODUCTION: Vascular hitch (VH) gained an increasing success in treating ureteropelvic junction obstruction (UPJO) by crossing vessels (CV) in pediatrics. AIMS OF THE STUDY: We aimed: (i) to compare laparoscopic VH versus laparoscopic dismembered pyeloplasty (DP) to treat UPJO by CV; (ii) to review possible amelioration given by a robot-assisted procedure. METHODS: Using defined search strategy, three investigators identified all studies on laparoscopic VH. Those studies comparing VH versus DP or versus robot-assisted VH were included in the meta-analysis. The meta-analysis was conducted using RevMan 5.3. Data are mean ± SD. RESULTS: Systematic review - Of 2783 titles/abstracts screened, 43 full-text articles were analyzed. Twelve studies on laparoscopic VH (298 pts) reported 98.3% success rate, with 1.3% intra-operative complications. Meta-Analysis - Five studies compared laparoscopic VH versus laparoscopic DP (277 pts). Operative time was reduced in VH (102.5 ± 47.5min) compared to DP (165.7 ± 53.7min; p < 0.00001). Complications were similar (VH 4/119 pts, 3.4 ± 1.2% versus DP 15/158 pts, 9.5 ± 6.8%; p = ns). Hospital stay was shortened in VH (1.1 ± 0.9dd) versus DP (3.3 ± 3.2dd; p < 0.0001; Summary Figure). The success rate was comparable (VH 115/118 pts, 97.5 ± 1.6% versus DP 157/158 pts, 99.4 ± 0.5%; p = ns). Two prospective studies compared robot-assisted VH to laparoscopic VH (53 pts). No differences were found among complications (robot-assisted VH 0/13 pts, 0% versus laparoscopic VH 1/40 pts, 2.5%; p = ns) and success rate (robot-assisted VH 13/13 pts, 100% versus laparoscopic VH 39/40 pts, 97.5%; p = ns). DISCUSSION: Several studies have been reported long-term results of laparoscopic VH in children. However, few papers demonstrated its superiority over laparoscopic DP to treat extrinsic UPJO. In the present study, we found similar incidence of complications and success rates when comparing VH versus DP. Nonetheless, the operative time and the length of hospital stay were significantly reduced in VH compared to DP. An increasing number of surgeons performed robotic-assisted VH, reporting promising outcomes. However, only a couple of studies compared robot-assisted VH to laparoscopic VH, with a similar incidence of complications and success rate in both procedures. The main limitations of the study were related to the slight number of papers included and to their quality, since all of them were retrospective studies or prospectively followed-up cohort of patients. CONCLUSIONS: Laparoscopic VH seems to be a safe and reliable procedure to treat UPJO by CV. The procedure appeared quicker than laparoscopic DP, with shortened hospital stay. Further studies are needed to corroborate these results and to establish amelioration given by a robot-assisted procedure.

Secondary hydrosalpinx in adolescents: a challenging decision-making process for surgical choice and future fertility preservation.

Hydrosalpinx in pre-pubertal children and non-sexually active adolescents is a rare finding with several etiology and negative impact on future female fertility. The therapeutic approach in these specific populations is debated and it must consider its etiology and adult guidelines focused on fertility issues, which suggest laparoscopic salpingectomy. We described two adolescent cases (15 years asymptomatic and 13 years with abdominal pain) presenting a monolateral hydrosalpinx secondary to surgery for Hirschsprung's disease and complicated appendicitis, respectively. Both patients underwent to uncomplicated robotic-assisted salpingectomy, with uneventful follow-up and preserved ovarian function. Robotic-assisted salpingectomy for hydrosalpinx secondary to previous surgical conditions is a safe and careful approach for adolescents in order to preserve ovarian vascularization, function and future fertility.

Esophageal Atresia and Associated Duodenal Atresia: A Cohort Study and Review of the Literature.

INTRODUCTION: Esophageal atresia (EA) is associated with duodenal atresia (DA) in 3 to 6% of cases. The management of this association is controversial and literature is scarce on the topic. MATERIALS AND METHODS: We aimed to (1) review the patients with EA + DA treated at our institution and (2) systematically review the English literature, including case series of three or more patients. RESULTS: Cohort study: Five of seventy-four patients with EA had an associated DA (6.8%). Four of five cases (80%) underwent primary repair of both atresia, one of them with gastrostomy placement (25%). One of five cases (20%) had a delayed diagnosis of DA. No mortality has occurred. Systematic Review: Six of six-hundred forty-five abstract screened were included (78 patients). Twenty-four of sixty-eight (35.3%) underwent primary correction of EA + DA, and 36/68 (52.9%) underwent staged correction. Nine of thirty-six (25%) had a missed diagnosis of DA. Thirty-six of sixty-eight underwent gastrostomy placement. Complications were observed in 14/36 patients (38.9 ± 8.2%). Overall mortality reported was 41.0 ± 30.1% (32/78 patients), in particular its incidence was 41.7 ± 27.0% after a primary treatment and 37.0 ± 44.1% following a staged approach. CONCLUSION: The management of associated EA and DA remains controversial. It seems that the staged or primary correction does not affect the mortality. Surgeons should not overlook DA when correcting an EA.

Inter- and Intraobserver Variation in the Assessment of Preoperative Colostograms in Male Anorectal Malformations: An ARM-Net Consortium Survey.

Aim: Male patients with anorectal malformations (ARM) are classified according to presence and level of the recto-urinary fistula. This is traditionally established by a preoperative high-pressure distal colostogram that may be variably interpreted by different surgeons. The aim of this study was to evaluate the inter- and intraobserver variation in the assessment by pediatric surgeons of preoperative colostograms with respect to the level of the recto-urinary fistula. Materials and Methods: Sixteen pediatric surgeons from 14 European centers belonging to the ARM-Net Consortium twice scored 130 images of distal colostograms taken in sagittal projection at a median age of 66 days of life (range: 4-1,106 days). Surgeons were asked to classify the fistula in bulbar, prostatic, bladder-neck, no fistula, and "unclear anatomy" example. Their assessments were compared with the intraoperative findings (kappa) for two scoring rounds with an interval of 6 months (intraobserver variation). Agreement among the surgeons' scores (interobserver variation) was also calculated using Krippendorff's alpha. A kappa over 0.75 is considered excellent, between 0.40 and 0.75 fair to good, and below 0.40 poor. Surgeons were asked to score the images in "poor" and "good" quality and to provide their years of experience in ARM treatment. Results: Agreement between the image-based rating of surgeons and the intraoperative findings ranges from 0.06 to 0.45 (mean 0.31). Interobserver variation is higher (Krippendorff's alpha between 0.40 and 0.45). Years of experience in ARM treatment does not seem to influence the scoring. The mean intraobserver variation between the two rounds is 0.64. Overall, the quality of the images is considered poor. Images categorized as having a good quality result in a statistically significant higher kappa (mean: 0.36 and 0.37 in the first and second round, respectively) than in the group of bad-quality images (mean: 0.25 and 0.23, respectively). Conclusions: There is poor agreement among experienced pediatric colorectal surgeons on preoperative colostograms. Techniques and analyses of images need to be improved in order to generate a homogeneous series of patients and make comparison of outcomes reliable.

A Rare Case of Giant Mesenteric Lipoblastoma in a 6-Year-Old Child and Review of the Literature.

Giant mesenteric lipoblastoma is a rare benign tumor arising from the adipocytes. It can mimic malignant tumors, and its diagnosis is difficult before surgery. Imaging studies could lead the diagnosis but not confirm it. Those tumors arising in the abdomen are usually larger and can cause symptoms of compression. Surgical excision is the treatment of choice, and a long-term follow-up is necessary to detect local recurrences. Only a few cases of lipoblastomas arising from the mesentery are reported in literature. We present a case of a rare giant lipoblastoma arising from the mesentery of a 6-year-old girl, with a history of postprandial abdominal pain.

Successful lung response after surgical repair in an infant with right-sided congenital diaphragmatic hernia.

Congenital diaphragmatic hernia is a rare condition associated with pulmonary complications as the abdominal viscera herniated into the chest may affect lungs development. We present the case of a male newborn baby with a prenatal diagnosis of a posterolateral defect (Bochdalek hernia) involving the right side. The infant underwent surgical repair at 3 days of life, and the post-surgery chest X-ray did not reveal morpho-structural alterations of the lungs and diaphragmatic profile. Our clinical case shows that patients may have a better lung outcome despite an initial unfavorable picture. Prenatal diagnosis is essential in identifying infants with congenital diaphragmatic hernia, especially those cases at higher risk for the worse outcomes, to optimize their clinical and surgical management.

Improving standard of pediatric surgical care in a low resource setting: the key role of academic partnership.

BACKGROUND: An epidemiological transition is interesting Sub-Saharan Africa increasing the burden of non-communicable diseases most of which are of surgical interest. Local resources are far from meeting needs and, considering that 50% of the population is less than 14 years of age, Pediatric surgical coverage is specially affected. Efforts are made to improve standards of care and to increase the number of Pediatric surgeons through short-term specialist surgical Missions, facilities supported by humanitarian organization, academic Partnership, training abroad of local surgeons. This study is a half term report about three-years Partnership between the University of Chieti- Pescara, Italy and the University of Gezira, Sudan to upgrade standard of care at the Gezira National Centre for Pediatric Surgery (GNCPS) of Wad Medani. Four surgical Teams per year visited GNCPS. The Program was financed by the Italian Agency for Development Cooperation. METHODS: The state of local infrastructure, current standard of care, analysis of caseload, surgical activity and results are reported. Methods utilized to assess local needs and to develop Partnership activities are described. RESULTS: Main surgical task of the visiting Team were advancements in Colorectal procedures, Epispadias/Exstrophy Complex management and Hypospadias surgery (20% of major surgical procedures at the GNCPS). Intensive care facilities and staff to assist more complex cases (i.e. neonates) are still defective. Proctoring, training on the job of junior surgeons, anaesthetists and nurses, collaboration in educational programs, advisorship in hospital management, clinical governance, maintenance of infrastructure together with training opportunities in Italy were included by the Program. Despite on-going efforts, actions have not yet been followed by the expected results. More investments are needed on Healthcare infrastructures to increase health workers motivation and prevent brain drain. CONCLUSIONS: The key role that an Academic Partnership can play, acting through expatriated Teams working in the same constrained contest with the local workforce, must be emphasized. Besides clinical objectives, these types of Global Health Initiatives address improvement in management and clinical governance. The main obstacles to upgrade standard of care and level of surgery met by the Visiting Team are scarce investments on health infrastructure and a weak staff retention policy, reflecting in poor motivation and low performance.

Perineal Groove: An Anorectal Malformation Network, Consortium Study.

OBJECTIVE: To review the Anorectal Malformation Network experience with perineal groove (PG) focusing on its clinical characteristics and management. STUDY DESIGN: Data on patients with PG managed at 10 participating Anorectal Malformation Network centers in 1999-2019 were collected retrospectively by questionnaire. RESULTS: The cohort included 66 patients (65 females) of median age 1.4 months at diagnosis. The leading referral diagnosis was anal fissure (n = 20 [30.3%]): 23 patients (34.8%) had anorectal malformations. Expectant management was practiced in 47 patients (71.2%). Eight (17%) were eventually operated for local complications. The median time to surgery was 14 months (range, 3.0-48.6 months), and the median age at surgery was 18.3 months (range, 4.8-58.0 months). In the 35 patients available for follow-up of the remaining 39 managed expectantly, 23 (65.7%) showed complete or near-complete self-epithelization by a mean age 15.3 months (range, 1-72 months) and 4 (11.4%) showed partial self-epithelization by a mean age 21 months (range, 3-48 months). Eight patients showed no resolution (5 were followed for ≤3 months). Nineteen patients (28.7%) were primarily treated with surgery. In total, 27 patients were operated. Dehiscence occurred in 3 of 27 operated patients (11.1%). CONCLUSIONS: PG seems to be an underestimated anomaly, frequently associated with anorectal malformations. Most cases heal spontaneously; therefore, expectant management is recommended. When associated with anorectal malformations requiring reconstruction, PG should be excised in conjunction with the anorectoplasty.