Gastric-type adenocarcinoma of the endocervix: Potentially overcoming resistant behavior with surgery.

Objective: Gastric-type adenocarcinoma of the endocervix (GAS) is a rare form of human papillomavirus-independent cervical cancer commonly described as an insidious disease bearing a poor prognosis. Based on scarce data, uncertainty persists pertaining to its oncologic management. Method: All cases of well-differentiated GAS treated at our institution from 2010 to 2021 were reviewed. Clinical characteristics, diagnostic tests results and oncologic outcomes were recorded and analyzed. Kaplan-Meier curves and log rank test were performed to compare survival curves between patients with tumors confined to the cervix (group 1: up to stage IB3) versus locally advanced or metastatic (group 2: stages II to IV). Results: Cervical cytologies and biopsies yielded low detection rates (38 and 42% respectively) leading to 87% of patients with locally advanced or metastatic disease at diagnosis. Median overall survival (OS) was 40.0 ± 15.9 months with a clear dichotomy in survival when comparing patients with disease confined to the cervix to those with higher stages (respectively 59.0 vs 12.0 months, p = 0.047). None of the 5 patients initially managed with concurrent chemoradiotherapy (CCRT) responded to treatment but fortunately 3 of the latter achieved remission after surgery. Conclusion: Well-differentiated GAS did not show favorable response to chemotherapy and radiation. Surgical resection seems to be a cornerstone in the management of this disease, as all patients who achieved remission were treated with surgery, either upfront or after suboptimal response to CCRT. We suggest considering aggressive upfront surgery when feasible. If CCRT is selected to avoid upfront exenterative procedures, rapid evaluation of tumor response is recommended.

Impact of Selective Fetal Growth Restriction on Laser Therapy Outcomes in Twin-Twin Transfusion Syndrome.

INTRODUCTION: The aim of this study was to determine if outcomes of fetoscopic laser photocoagulation in isolated twin-twin transfusion syndrome (TTTS) differ from TTTS with concomitant selective fetal growth restriction (sFGR). METHODS: This is a retrospective cohort study of all cases of TTTS treated at the CHU Sainte-Justine between February 2006 and January 2020. Data were collected from maternal, obstetrical, and neonatal chart review. RESULTS: A total of 149 patients were included in our study. Forty-seven patients (31.5%) had a pregnancy complicated by TTTS and sFGR. Mean gestational age at diagnosis and at treatment was 20+4 weeks and 20+6 weeks for TTTS alone, and 20+5 weeks and 21+2 weeks with concomitant sFGR. The presence of concomitant sFGR negatively impacted survival. Double survival in the TTTS + sFGR was 48.9% (23/47) versus 68.6% (70/102) in the TTTS-only group (p = 0.021). Fetal donor survival was 59.6% (28/47) in the TTTS + sFGR group and 84.3% (86/102) in the TTTS-only group (p = 0.001). However, the survival of at least one twin did not differ between the two groups: 93.6% (44/47) in the TTTS + sFGR group versus 92.2% (94/102) in the TTTS-only group (p = 0.751). The presence of type 2-3 sFGR (OR = 0.56; 95% CI 0.32-0.96, p = 0.033) and gestational age at laser therapy (OR = 1.17; 95% CI 1.01 = 1.36, p = 0.036) were independently associated with dual survival. CONCLUSION: sFGR is independently associated with decreased double survivorship at the expense of the donor in TTTS undergoing laser therapy. Type 2 or 3 sFGR and early gestational age at treatment are especially at risk. A larger cohort is needed to validate our results.

Prenatal markers of atypical neurodevelopment in children with congenital heart defects.

BACKGROUND: A growing body of literature demonstrates that survivors of congenital heart defects (CHD) are at increased risk of neurodevelopmental delay, which frequently manifests as motor delay during the first year of life. OBJECTIVE: The aim of this study was to determine prenatal predictors of an early atypical neurodevelopment. This information could help assist decision-making during prenatal counseling. STUDY DESIGN: In this retrospective cohort study, we evaluated the records of 75 children with CHD followed at the Clinique d'Investigation Neuro-Cardiaque (CINC) of the CHU Ste-Justine born between 2013 and 2016. The neurodevelopmental outcome was determined using the Alberta Infant Motor Scale (AIMS) at 4 months. Associations between prenatal factors and atypical neurodevelopment (AIMS < 10th percentile) were assessed using bivariate and multivariate analyses. RESULTS: Forty-four infants (58.7%) had atypical neurodevelopment. When there was no extra cardiac anomaly seen on prenatal ultrasound, a head to abdominal ratio (HC/AC) below 1.1 was associated with a four-fold increased risk of atypical neurodevelopment (OR = 4.54; 95% CI = 1.24-16.64 p = .023). There was no difference in identified genetic anomaly in both groups. However, there was a trend toward more extra cardiac anomalies in infants with atypical neurodevelopment (27.3%) compared to 9.7% in those with typical neurodevelopment (p = .061). CONCLUSION: Our study shows that early atypical neurodevelopment affects the majority of children with CHD and highlights the importance of post-natal monitoring by a specialized team. A thorough prenatal ultrasound is important to screen for those at higher risk i.e. those with extra cardiac anomaly and HC/AC below 1.1. A larger cohort is needed to validate those results.

Prenatal presentation of glutaric aciduria type II: A case report with radiologic, clinical, biochemical, molecular, and pathological phenotyping.

We know that glutaric aciduria type II is an inborn metabolism. This case report highlights that polycystic kidneys with hepatomegaly in prenatal ultrasound are suggestive of glutaric aciduria type II and it identifies a new variant as pathogenic.

Expectant Management of Monochorionic-Triamniotic Triplets Complicated by Selective In Utero Growth Restriction: Report of 2 Cases.

The optimal management of monochorionic-triamniotic (MCTA) triplet pregnancies is not clearly established, and there is no literature to guide management of MCTA complicated with selective intrauterine growth restriction (sIUGR). This gap in knowledge and the concern for higher risk of severe complications have led some medical societies to recommend selective termination of nontrichorionic triplet pregnancies. We sought to report the favourable outcomes of two MCTA complicated by sIUGR expectantly managed at Sainte-Justine Hospital, Montreal, Canada. The first case is of a 42-year-old woman with spontaneous MCTA triplets diagnosed at 18 weeks with type II sIUGR who opted for expectant management. The second patient was a 22-year-old woman with a spontaneous MCTA triplet pregnancy diagnosed at 18 weeks with type III sIUGR. Our experience shows that close serial ultrasounds could potentially allow physicians to foresee fetal deterioration. In our opinion, expectant management should be considered as a management option for MCTA complicated by sIUGR.

Comparison of diagnostic imaging modalities for the evaluation of pancreatic duct injury in children: a multi-institutional analysis from the Pancreatic Trauma Study Group.

PURPOSE: Determining the integrity of the pancreatic duct is important in high-grade pancreatic trauma to guide decision making for operative vs non-operative management. Computed tomography (CT) is generally an inadequate study for this purpose, and magnetic resonance cholangiopancreatography (MRCP) is sometimes obtained to gain additional information regarding the duct. The purpose of this multi-institutional study was to directly compare the results from CT and MRCP for evaluating pancreatic duct disruption in children with these rare injuries. METHODS: Retrospective study of data obtained from eleven pediatric trauma centers from 2010 to 2015. Children up to age 18 with suspected blunt pancreatic duct injury who had both CT and MRCP within 1 week of injury were included. Imaging findings of both studies were directly compared and analyzed using descriptive statistics, Chi square, Wilcoxon rank-sum, and McNemar's tests. RESULTS: Data were collected for 21 patients (mean age 7.8 years). The duct was visualized more often on MRCP than CT (48 vs 5%, p < 0.05). Duct disruption was confirmed more often on MRCP than CT (24 vs 0%), suspected based on secondary findings equally (38 vs 38%), and more often indeterminate on CT (62 vs 38%). Overall, MRCP was not superior to CT for determining duct integrity (62 vs 38%, p = 0.28). CONCLUSIONS: In children with blunt pancreatic injury, MRCP is more useful than CT for identifying the pancreatic duct but may not be superior for confirmation of duct integrity. Endoscopic retrograde cholangiogram (ERCP) may be necessary to confirm duct disruption when considering pancreatic resection. LEVEL OF EVIDENCE: III.

Proposed clinical pathway for nonoperative management of high-grade pediatric pancreatic injuries based on a multicenter analysis: A pediatric trauma society collaborative.

BACKGROUND: Guidelines for nonoperative management (NOM) of high-grade pancreatic injuries in children have not been established, and wide practice variability exists. The purpose of this study was to evaluate common clinical strategies across multiple pediatric trauma centers to develop a consensus-based standard clinical pathway. METHODS: A multicenter, retrospective review was conducted of children with high-grade (American Association of Surgeons for Trauma grade III-V) pancreatic injuries treated with NOM between 2010 and 2015. Data were collected on demographics, clinical management, and outcomes. RESULTS: Eighty-six patients were treated at 20 pediatric trauma centers. Median age was 9 years (range, 1-18 years). The majority (73%) of injuries were American Association of Surgeons for Trauma grade III, 24% were grade IV, and 3% were grade V. Median time from injury to presentation was 12 hours and median ISS was 16 (range, 4-66). All patients had computed tomography scan and serum pancreatic enzyme levels at presentation, but serial enzyme level monitoring was variable. Pancreatic enzyme levels did not correlate with injury grade or pseudocyst development. Parenteral nutrition was used in 68% and jejunal feeds in 31%. 3Endoscopic retrograde cholangiopancreatogram was obtained in 25%. An organized peripancreatic fluid collection present for at least 7 days after injury was identified in 59% (42 of 71). Initial management of these included: observation 64%, percutaneous drain 24%, and endoscopic drainage 10% and needle aspiration 2%. Clear liquids were started at a median of 6 days (IQR, 3-13 days) and regular diet at a median of 8 days (IQR 4-20 days). Median hospitalization length was 13 days (IQR, 7-24 days). Injury grade did not account for prolonged time to initiating oral diet or hospital length; indicating that the variability in these outcomes was largely due to different surgeon preferences. CONCLUSION: High-grade pancreatic injuries in children are rare and significant variability exists in NOM strategies, which may affect outcomes and effective resource utilization. A standard clinical pathway is proposed. LEVEL OF EVIDENCE: Therapeutic/care management, level V (case series).