Short and Long-Term Outcomes of PSARP versus LAARP and Single versus Staged Repair for Infants with High-Type Anorectal Malformations: A Systematic Review and Meta-Analysis.

BACKGROUND: We aimed to compare among patients with high-type anorectal malformations (ARM): (i) short- and long-term outcomes of laparoscopic-assisted anorectoplasty (LAARP) compared to classic posterior sagittal anorectoplasty (PSARP) and (ii) the results of single-stage versus staged PSARP. METHODS: Using a defined search strategy, two independent investigators systematically reviewed the English literature. PRISMA guidelines were followed, and meta-analysis was performed using RevMan5.3. RESULTS: Of 567 abstracts screened, 7 papers have been included (254 pts; 121 PSARP, 133 LAARP) in the first systematic review and meta-analysis. The length of hospitalization was shortened in LAARP versus PSARP (10.9 versus 14.4 days; p < 0.0001). PSARP and LAARP were comparable in terms of early postoperative complications (28.9% versus 24.7%; p = ns) and rectal prolapse (21.6% versus 17.5%; p = ns). At long-term follow-up, the presence of voluntary bowel movements (74.0% versus 83.5%; p = ns) and the incidence of soiling (45.5% versus 47.6%; p = ns) were similar in both PSARP and LAARP. Six papers (297 pts) were included in the second systematic review, with three comparative studies included in the meta-analysis (247 pts; 117 one-stage, 130 staged procedures). No significant difference in terms of presence of voluntary bowel movements after single-stage versus staged procedures (72.6% versus 67.3%; p = ns) has been detected. CONCLUSIONS: LAARP seems to be a safe and effective procedure, showing short- and long-term outcomes similar to PSARP. One-stage PSARP could be a safe alternative to the classic three-stage procedure, even for those infants with high-type ARM. Further and larger comparative studies would be needed to corroborate these partial existing data.

Neonatal Intestinal Segmental Volvulus: What Are the Differences with Midgut Volvulus?

OBJECTIVE: Intestinal volvulus in the neonate is a surgical emergency caused by either midgut volvulus (MV) with intestinal malrotation or less commonly, by segmental volvulus (SV) without intestinal malrotation. The aim of our study was to investigate if MV and SV can be differentiated by clinical course, intraoperative findings, and postoperative outcomes. METHODS: Using a defined search strategy, two investigators independently identified all studies comparing MV and SV in neonates. PRISMA guidelines were followed, and a meta-analysis was performed using RevMan 5.3. RESULTS: Of 1,026 abstracts screened, 104 full-text articles were analyzed, and 3 comparative studies were selected (112 patients). There were no differences in gestational age (37 vs. 36 weeks), birth weight (2,989 vs. 2,712 g), and age at presentation (6.9 vs. 3.8 days). SV was more commonly associated with abnormal findings on fetal ultrasound (US; 65 vs. 11.6%; p < 0.00001). Preoperatively, SV was more commonly associated with abdominal distension (32 vs. 77%; p < 0.05), whereas MV with a whirlpool sign on ultrasound (57 vs. 3%; p < 0.01). Bilious vomiting had similar incidence in both (88 ± 4% vs. 50 ± 5%). Intraoperatively, SV had a higher incidence of intestinal atresia (2 vs. 19%; p < 0.05) and need for bowel resection (13 vs. 91%; p < 0.00001). There were no differences in postoperative complications (13% MV vs. 14% SV), short bowel syndrome (15% MV vs. 0% SV; data available only from one study), and mortality (12% MV vs. 2% SV). CONCLUSION: Our study highlights the paucity of studies on SV in neonates. Nonetheless, our meta-analysis clearly indicates that SV is an entity on its own with distinct clinical features and intraoperative findings that are different from MV. SV should be considered as one of the differential diagnoses in all term and preterm babies with bilious vomiting after MV was ruled out-especially if abnormal fetal US and abdominal distension is present.

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.

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.

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.

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.

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.

Management of Spontaneous Pneumothorax in Children: A Systematic Review and Meta-Analysis.

INTRODUCTION: Management of primary spontaneous pneumothorax (PSP) is mainly based on adults. Data are controversial with regards to its management in children. We aimed to assess: (1) the length of hospital stay (LOS) between conservative management (i.e., observation with O2 administration), aspiration/chest drain, and surgical management; (2) the risk of recurrence after nonsurgical treatment versus surgery; (3) the risk of recurrence in the presence of bullae. MATERIALS AND METHODS: Using a defined search strategy, three independent investigators identified all the studies on the management of PSP in children. Case reports, opinion articles, and gray literature publications were excluded. The study was conducted according to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. A meta-analysis was performed using RevMan 5.3. Data are expressed as mean ± SD. RESULTS: Of 3,089 abstracts screened, 95 full-text were analyzed, 23 were included in the quantitative analysis, and 16 were included in the meta-analysis (1,633 patients). LOS was similar between conservative and surgical management (6.2 ± 0.8 days vs. 5.9 ± 1.4 days; p = ns). Recurrence of PSP was significantly higher among children with a nonsurgical management (32%) versus those surgically treated (18%; p = 0.002). The incidence of recurrence was slightly higher in patients managed by aspiration/chest drain (34%) compared with those with a conservative management (27%; p = 0.05). Risk of recurrence in patients with or without documented bullae was not significantly different (26 vs. 38%, respectively; p = ns). CONCLUSION: Given the lack of a standardized management of pediatric PSP, the present study seems to demonstrate a better outcome in children treated with surgery as first-line of management. LEVEL OF EVIDENCE: This is a Level III study.

Contrast-enhanced ultrasonography (CEUS) in the follow-up of pediatric abdominal injuries: value and timing.

AIMS: Primary aim was to investigate the value and safety of contrast-enhanced ultrasonography (CEUS) during follow-up (FU) of splenic, hepatic and renal post-traumatic injuries in a pediatric population. Secondary aim was to extrapolate appropriate timing of FU-CEUS. METHODS: In a retrospective study, post-traumatic parenchymal injuries diagnosed with CT or CEUS, were subjected to non-operative management and followed with CEUS. RESULTS: Forty-six patients were enrolled, with isolated or combined injuries, for a total of 30 splenic, 15 hepatic and 12 renal injuries. At admission 42/46 patients underwent CT and 4/46 underwent CEUS. During FU a total of 65 CEUS were performed: 16 within 72 h to check delayed active bleeding or parenchymal rupture; 24 between 5 and 10 days post admission, to pose indication to active mobilization or to discharge; 21 between 20 and 60 days post admission to document complete healing of the lesion or pose indication to discharge in most severe injuries. No complications related to CEUS were encountered. CONCLUSIONS: CEUS is valuable and safe to follow patients with post-traumatic abdominal injuries, even if further data are needed for renal injuries. We propose a tailored approach based on injury grade and clinical course: in the first 3 days only in case of delayed bleeding or rupture suspect; between 5 and 10 days post trauma to ensure a safe active mobilization and/or pose indication to discharge, and over 20-30 days post trauma to pose indication to discharge in most severe injuries or document complete healing and permit return to sport activities.

Duodenal atresia and associated intestinal atresia: a cohort study and review of the literature.

PURPOSE: To determine the true incidence of associated intestinal atresia (AIA) in infants with duodenal atresia (DA) and to analyze whether the surgical approach, open versus laparoscopic, would impact on patient outcome when AIA is present. METHODS: Cohort study We review all DA infants treated at our institution (2001-2016) and analyzed the outcome of those with AIA. Systematic review/meta-analysis Using a defined search strategy and according to PRISMA guidelines, two investigators independently identified all studies on DA and searched cases of AIA to determine its incidence. Data are mean ± SD. RESULTS: Cohort study Of 140 DA infants, 10 (7%) had AIA (4 type I, 4 type III, 2 type II). All type I AIA (webs) were found in the duodenum. Systematic review/meta-analysis Of 840 studies, 18 were included (2026 infants). The incidence of AIA was 2.8 ± 1.6%. The incidence of missed AIA was 0.8 ± 2.4%. Three comparative studies (759 infants) showed higher risk of missed AIA following laparoscopic (2.9 ± 2.4%) than open repair (0.3 ± 0.1%; p < 0.01). CONCLUSIONS: The incidence of AIA in DA infants is low and the risk of missing it is higher at laparoscopy than at laparotomy. Regardless the approach, surgeons should carefully investigate bowel continuity to avoid the risk of missing AIA.

Intestinal malrotation in infants with omphalocele: A systematic review and meta-analysis.

PURPOSE: The management of intestinal malrotation in infants with omphalocele varies among surgeons. Herein, we aimed to determine whether infants with omphalocele should be investigated for malrotation. METHODS: Using a defined search strategy, three investigators identified all studies reporting patients with omphalocele and malrotation. Outcome measures included: 1. incidence of malrotation; 2. correlation with the abdominal size defect in patients with omphalocele; 3. risk of volvulus in those not investigated for malrotation; 4. incidence of adhesive bowel obstruction in those who underwent Ladd's procedure. The meta-analysis was conducted according to PRISMA guidelines and using RevMan 5.3. RESULTS: Of 111 articles analyzed, 12 (3888 children) reported malrotation in 136 patients (3.5%). Malrotation was equally found in patients with major (15.2%) and minor (13.6%; p = 0.52) omphalocele. A volvulus was more common in children who had Ladd's procedure (8%) than in those who did not (1%; p = 0.03). Adhesive bowel obstruction rate was similar in both groups (5% vs. 3%; p = 0.21). CONCLUSION: The incidence of malrotation in infants with omphalocele is low but probably underreported, and is not influenced by the size of the defect. At present, there is no evidence in the literature to support investigations to detect malrotation in infants with omphalocele. TYPE OF STUDY: Therapeutic. LEVEL OF EVIDENCE: III.

Unilateral Papillary Cystadenoma of the Epididymis as a First Presentation of Von Hippel-Lindau Disease.

Association between papillary cystadenoma of the epididymis (PCE) and Von Hippel-Lindau Disease (VHLD) is well known and stronger for bilateral tumors. Unilateral PCE occurs either as a sporadic tumor without evidence of VHLD or in the context of a known diagnosis of VHLD, indeed it has never been reported as the first manifestation of VHLD. In contrast, we report the case of a boy with an apparently isolated, unilateral PCE that resulted to be the first manifestation of an unknown VHLD. Thus, we recommend screening for VHLD in patients with a new diagnosis of unilateral PCE, especially if the patients are young.