The role of fetal hemoglobin in the artificial placenta: A premature ovine model.

INTRODUCTION: A radical paradigm shift in the treatment of premature infants failing conventional treatment is to recreate fetal physiology using an extracorporeal Artificial Placenta (AP). The aim of this study is to evaluate the effects of changing fetal hemoglobin percent (HbF%) on physiology and circuit function during AP support in an ovine model. METHODS: Extremely premature lambs (n = 5) were delivered by cesarean section at 117-121 d estimated gestational age (EGA) (term = 145d), weighing 2.5 ± 0.35 kg. Lambs were cannulated using 10-14Fr cannulae for drainage via the right jugular vein and reinfusion via the umbilical vein. Lambs were intubated and lungs were filled with perfluorodecalin to a meniscus with a pressure of 5-8 cm H2O. The first option for transfusion was fetal whole blood from twins followed by maternal red blood cells. Arterial blood gases were used to titrate AP support to maintain fetal blood gas values. RESULTS: The mean survival time on circuit was 119.6 ± 39.5 h. Hemodynamic parameters and lactate were stable throughout. As more adult blood transfusions were given to maintain hemoglobin at 10 mg/dL, the HbF% declined, reaching 40% by post operative day 7. The HbF% was inversely proportional to flow rates as higher flows were required to maintain adequate oxygen saturation and perfusion. CONCLUSIONS: Transfusion of adult blood led to decreased fetal hemoglobin concentration during AP support. The HbF% was inversely proportional to flow rates. Future directions include strategies to decrease the priming volume and establishing a fetal blood bank to have blood rich in HbF.

Thoracoscopic Patch Repair of Congenital Diaphragmatic Hernia: Can Smaller Incisions Treat Larger Defects?

INTRODUCTION: Thoracoscopic CDH repair is increasingly performed for Type A and small Type B defects that are amenable to primary repair. However, the thoracoscopic approach is controversial for larger defects necessitating a patch due to technical complexity, intraoperative acidosis, and recurrence risk. We aim to compare clinical outcomes between thoracoscopic and open patch repair of Type B/C defects, using a standardized technique. METHODS: This is a single-center retrospective review of thoracoscopic and open CDH patch repairs January 2017-December 2021. We excluded primary repairs, Type D hernias, repairs on ECMO, recurrent repairs. Various preoperative, intraoperative, and postoperative variables were compared. Primary outcome was recurrence rate. Secondary outcomes included intraoperative pH and pCO2, operative time, and complication rates. RESULTS: Twenty-nine patients met inclusion criteria (open = 13, thoracoscopic = 16). The open cohort had lower o/e total fetal lung volume (29 vs 41.2%, p = 0.042), higher preoperative peak inspiratory pressures (24 vs 20 cm H2O, p = 0.007), were more frequently Type C defects (92.3 vs 31%, p = 0.002) and had liver "up" in left-sided hernias (46 vs 0%, p < 0.0001). Intraoperatively, median lowest pH and highest pCO2 did not differ; neither did overall median pH or pCO2. Operative times were similar (153 vs 194 min, p = 0.113). No difference in recurrence rates was identified, however postoperative complications were higher in the open group. There were no mortalities. CONCLUSIONS: Although we demonstrate higher disease severity of patients undergoing open repair, thoracoscopic patch repair for Type B/C defects is safe and effective in patients with favorable physiologic status, alleviating concerns for intraoperative acidosis, operative length, and risk of recurrence. LEVEL OF EVIDENCE: II.

Beyond Parity: Gender Diversity of National and International Pediatric Surgery Societal Leadership.

PURPOSE: Despite trends toward equal gender representation among medical school graduates, surgical trainees and fellows, diversity in higher levels of pediatric surgery is largely unstudied. This study aims to quantify gender diversity among leadership of pediatric surgical associations and societies across the globe. METHODS: National and international pediatric surgical organizations were identified from the websites of the American Pediatric Surgical Association (APSA) and World Federation of Associations of Pediatric Surgery (WOFAPS). Compositional gender data of current and past organizational leadership was collected by examining publicly available archives of executive membership rosters. If roster pictures were not available, member names were input into social media sites and other search engines to ensure accurate gender denotation. Univariate analyses of organizational metrics and aggregate data of 5-year time intervals were performed via Fischer's Exact Test with significance of p < 0.05. RESULTS: Nineteen pediatric surgical organizations were included for study analysis. Of 189 current organizational leaders, 50 (26.4%) are women. Eight organizations (42.1%) have less than 20% of leadership positions filled by women members, while two executive boards have no women members. Four organizations (22.2%) have a current woman seated as president/chairperson. Historical gender distribution stratified by organization demonstrates a range of 0-7.8% (p = 0.99), with one organization having yet to elect a woman president/chairperson. Longitudinally, women presidential representation remained consistently low (5-11%) across all time intervals from 1993 to 2022 (p = 0.35). CONCLUSIONS: Despite advances in diversity in medical school graduates, surgical training, and workforce recruitment, there are still significant disparities in gender representation within pediatric surgery societal leadership. LEVEL OF EVIDENCE: IV.

The Michigan Action Progress System (MAPS): Enhancing a Culture of Professionalism, Accountability, and Psychological Safety.

OBJECTIVE: To prioritize trainee well-being, promote professionalism, and allow individuals to raise concerns without fear of retribution, one surgical department created an innovative process by which individuals can raise concerns and obtain subsequent support. DESIGN AND SETTING: The University of Michigan Department of Surgery implemented the Michigan Action Progress System (MAPS) in February 2021. PARTICIPANTS: General Surgery residents, faculty, and staff voluntarily participate in MAPS. RESULTS: Since implementation, there have been 26 entries into MAPS. Petitioners included students (10, 38%), residents and fellows (7, 27%), staff (1, 4%), faculty (1, 4%), and anonymous petitioners (7, 27%). Concerns regarding racism (1, 4%), bullying (11, 52%), gender discrimination (1, 4%), and other incidents (8, 38%) were addressed though MAPS. CONCLUSIONS: We have successfully implemented an innovative system that focuses on the needs of the user, consolidates handling of concerns, and emphasizes transparency, documentation, education, and improvement to promote a culture of professionalism and accountability.

Neonatal pneumothorax in congenital diaphragmatic hernia: Be wary of high ventilatory pressures.

Background: Patients with congenital diaphragmatic hernia (CDH) require invasive respiratory support and higher ventilator pressures may be associated with barotrauma. We sought to evaluate the risk factors associated with pneumothorax in CDH neonates prior to repair. Methods: We retrospectively reviewed newborns born with CDH between 2014 and 2019 who developed a pneumothorax, and we matched these cases to patients with CDH without pneumothorax. Results: Twenty-six patients were included (n=13 per group). The pneumothorax group required extracorporeal life support (ECLS) more frequently (85% vs 54%, p=0.04), particularly among type A/B defects (31% vs 7%, p=0.01). The pneumothorax group had higher positive end-expiratory pressure (PEEP) within 1 hour of birth (p=0.02), at pneumothorax diagnosis (p=0.003), and at ECLS (p=0.02). The pneumothorax group had a higher mean airway pressure (Paw) at birth (p=0.01), within 1 hour of birth (p=0.01), and at pneumothorax diagnosis (p=0.04). Using multiple logistic regression with cluster robust SEs, higher Paw (OR 2.2, 95% CI 1.08 to 3.72, p=0.03) and PEEP (OR 1.8, 95% CI 1.15 to 3.14, p=0.007) were associated with an increased risk of developing pneumothorax. There was no difference in survival (p=0.09). Conclusions: Development of a pneumothorax in CDH neonates is independently associated with higher Paw and higher PEEP. A pneumothorax increases the likelihood of treated with ECLS, even with smaller defect.

Preoperative virtual video visits only: a convenient option that should be offered to caregivers beyond the pandemic.

Objective: During the COVID-19 pandemic, our group implemented preoperative video visits (VVs) to limit physical contact. The aim of this study was to determine caregivers' and providers' perceptions of this practice and to determine feasibility for continuation. Methods: All patients who had only a preoperative VV prior to an elective surgery were identified from March-October 2020. Caregivers, surgeons, and clinic staff were surveyed about their experiences. Results: Thirty-four preoperative VVs were followed by an elective surgery without a preceding in-person visit. Of the 31 caregiver surveys completed, the majority strongly agreed that the VV was more convenient (87%, n=27). Eighty-one percent (n=25) strongly agreed or agreed that the VV saved them money. Ninety-four percent (n=29) strongly agreed or agreed that they would choose the VV option again. Caregivers saved an average travel distance of 60.3 miles one way (range 6.1-480). Of the 13/17 providers who responded, 77% (n=10) expressed that the practice should continue. Conclusions: Virtual health became a necessity during the pandemic, and caregivers were overwhelmingly satisfied. Continuing VVs as an option beyond the pandemic may be a reasonable and effective way to help eliminate some of the hurdles that impede healthcare-seeking behavior and should be offered.

Image-based prenatal predictors of postnatal survival, extracorporeal life support, and defect size in right congenital diaphragmatic hernia.

OBJECTIVE: To determine the association between prenatal ultrasound (US) and magnetic resonance imaging (MRI) characteristics in right congenital diaphragmatic hernia (RCDH) with postnatal outcome. STUDY DESIGN: CDH Study Group data were reviewed for all RCDH infants (n = 156) born between 2015 and 2019. Prenatal US and MRI lung size measurements were correlated with survival, extracorporeal life support (ECLS), and defect size. RESULT: Overall survival was 64.1%. ECLS was required in 40.4%. US and MRI-based prenatal assessment of pulmonary hypoplasia does not predict survival. Prenatal measurement of lung size using either US or MRI correlates with ECLS use. Only MRI-based measures of lung size are associated with defect size. CONCLUSION: Image-based prenatal predictors of survival, ECLS, and defect size are of limited value in RCDH. Extrapolation of prenatal survival and morbidity indicators from left to right-sided CDH is not appropriate. There is an urgent need to develop RCDH prenatal prediction models.

Image-based prenatal predictors correlate with postnatal survival, extracorporeal life support use, and defect size in left congenital diaphragmatic hernia.

OBJECTIVE: To evaluate the association between prenatal imaging predictors of patients with left-sided congenital diaphragmatic hernia (LCDH) and postnatal outcomes. STUDY DESIGN: CDH study group data were reviewed for LCDH infants born 2015-2019. Prenatal ultrasound (US) and magnetic resonance imaging (MRI) data were collected and correlated with postnatal information including CDHSG defect size (A through D or non-repair (NR)). RESULTS: In total, 929 LCDH patients were included. Both US and MRI imaging predictors correlated with postnatal survival (72.2%) and ECLS use (29.6%). Logistic regression models confirmed increased survival and decreased ECLS use with larger values for all predictors. Importantly, all prenatal values evaluated showed no significant difference between defect size D and NR patients. CONCLUSIONS: This is the largest cohort of LCDH patients and demonstrates that prenatal imaging factors correlate with postnatal outcomes and confirms that patients in the non-repair group are prenatally similar to type D defects.

A single institution's experience with the management of peripheral bronchial atresia.

PURPOSE: Peripheral bronchial atresia is a pulmonary abnormality diagnosed on postnatal computed tomography after prenatal imaging reveals a congenital lung lesion. Debate regarding management of this abnormality prompted us to review our institution's practice patterns and outcomes. METHODS: All patients diagnosed with bronchial atresia were assessed from 6/2014 to 7/2020. Pediatric radiologists were surveyed to delineate computed tomography criteria used to diagnose peripheral bronchial atresia. Criteria were applied in an independent blinded review of postnatal imaging. Data for patients determined to have peripheral bronchial atresia and at least an initial pediatric surgical evaluation were analyzed. RESULTS: Twenty-eight patients with bronchial atresia received at least an initial pediatric surgical evaluation. Expectant management was planned for 22/28 (79%) patients. Two patients transitioned from an expectant management strategy to an operative strategy for recurrent respiratory infections; final pathology revealed bronchial atresia in both. Six patients were initially managed operatively; final pathology revealed bronchial atresia (n = 3) or congenital lobar overinflation (n = 3). CONCLUSIONS: Peripheral bronchial atresia can be safely managed expectantly. A change in symptoms is suspicious for alternate lung pathology, warranting further workup and consideration for resection. LEVEL OF EVIDENCE: Level IV.

The in-utero diagnosis of choledochal cyst: can postnatal imaging predict benefit from early surgical intervention?

BACKGROUND: Infants prenatally suspected of having a choledochal cyst (CDC) typically undergo ultrasound imaging shortly after birth. This study sought to evaluate features on the initial postnatal ultrasound (IPU) that could identify newborns at risk for early complications. METHODS: Following IRB approval, patients from four US fetal centers with prenatal suspicion for CDC and postnatal imaging from 2000 to 2017 were reviewed. Imaging and clinical courses were assessed. RESULTS: Forty-two patients had prenatal ultrasounds suspicious for CDC. Nineteen (45.2%) were excluded due to diagnostic revision (n = 9), cyst resolution (n = 5), lack of IPU measurements (n = 3), or lack of follow-up (n = 2). The 23 remaining patients were included in the study. Of these, five (21.7%) developed symptoms at a median age of 16.5 days (IQR 16-19 days), and 18 (78.3%) remained asymptomatic throughout the first year after birth. Five patients (21.7%) had cysts ≥ 4.5 cm on IPU (Symptomatic: n = 3; Asymptomatic: n = 2). Eighteen patients (78.3%) had cysts < 4.5 cm on IPU (Symptomatic: n = 2; Asymptomatic: n = 16). An IPU cyst size ≥ 4.5 cm was associated with neonatal symptom manifestation (p = 0.048), with 88.9% specificity (95% CI 65.3-98.6%) and 60% sensitivity (95% CI 14.7-94.7%). CONCLUSIONS: In newborns with prenatally diagnosed CDC, a cyst size ≥ 4.5 cm on IPU is associated with symptom development during the first month after birth and therefore early cyst excision is recommended.

Current status of subspecialization in pediatric surgery: A focus on fetal surgery.

BACKGROUND/PURPOSE: Fetal surgery is a growing field within pediatric surgery. We sought to understand practice patterns of pediatric surgeons who subspecialize in fetal surgery. METHODS: A survey was sent to all active non-trainee surgeons within the American Pediatric Surgical Association. Respondents were stratified based on self-reported fetal affiliations. RESULTS: Of 1015 surveys, we received 405 responses (40%). Fetal surgery practices were described by 77 self-designated fetal center members. Centers provide prenatal consultation (99%), diagnostic imaging (84%), care/delivery coordination (83%), and/or fetal surgery/procedures (52%). The majority (56%) of fetal programs are directed by surgery and maternal fetal medicine. Pediatric surgeons are represented on the fetal team in 96% of centers. Prenatal consultations are primarily seen by any pediatric surgeon in the group (53%), with the pediatric surgeon on call operating on/caring for the baby postnatally in the majority (64%), regardless of who performed the prenatal consultation. Only 29% of fetal center members performed a fetal operation in the last year. Yearly fetal case numbers vary widely per procedure, with the most common being complex twin procedures and needle-based sclerotherapy. CONCLUSIONS: Fetal centers vary by services offered, team composition, and interventions performed, with few surgeons performing a wide range of fetal surgery. LEVEL OF EVIDENCE: Level III.

Overcoming COVID-19: Strategies to Mitigate the Perpetuated Gender Achievement Gap.

Sex inequity in academic achievement was well documented before the COVID-19 pandemic, and evolving data suggest that women in academic surgery are disproportionately disadvantaged by the pandemic. This perspective piece reviews currently accepted solutions to the sex achievement gap, with their associated shortcomings. We also propose innovative strategies to overcoming barriers to sex equity in academic medicine that broadly fall into three categories: strategies to mitigate inequitable caregiving responsibilities, strategies to reduce cognitive load, and strategies to value uncompensated, impactful work. These approaches address inequities at the system-level, as opposed to the individual-level, lifting the burden of changing the system from women.

Autoamputated ovarian mass causing a partial bowel obstruction in an infant with literature review.

An autoamputated ovary (AO) is an extremely rare circumstance with a few reports in the literature. We present a case of a 3-month-old girl with a history of vague abdominal symptoms as well as a known ovarian mass, which was initially suspected to be benign and regressing. On laparoscopy for pain and obstructive symptoms, she was found to have an AO. The pathology was consistent with necrotic and torsed ovarian tissue with superimposed infection and possibly a cystic non-malignant teratoma. AOs are thought to originate from a torsion and are typically diagnosed incidentally. This is an interesting case of an AO causing a partial small bowel obstruction.

Fetal endoscopic tracheal occlusion for congenital diaphragmatic hernia: a narrative review of the history, current practice, and future directions.

Fetal intervention for fetuses with congenital diaphragmatic hernia (CDH) has been investigated for over 30 years and is summarized in this manuscript. The review begins with a discussion of the history of fetal intervention for this severe congenital anomaly beginning with open fetal surgery with repair of the anatomical defect, shifting towards tracheal occlusion via open surgery techniques, and finally fetoscopic endoluminal balloon tracheal occlusion using a percutaneous approach. The current technique of fetal endoscopic tracheal occlusion (FETO) is described in detail with steps of the procedure and complementary figures. The main outcomes of single-institutional studies and multiple systematic reviews are examined and discussed. Despite these studies, the fetal community agrees that FETO remains investigational at this time as there is insufficient evidence to recommend it as the standard of care for CDH. A randomized controlled trial, The Tracheal Occlusion to Accelerate Lung Growth (TOTAL) trial, has been designed to attempt to answer this question in an elaborate, international, multi-institutional study and is described in the text. Finally, future directions of fetal intervention for antenatally diagnosed CDH are discussed, including options for non-isolated CDH, the Smart-TO balloon for nonoperative reversal of occlusion, and transplacental sildenafil for treatment of pulmonary hypertension prior to birth.

Fetal hemoglobin levels in premature newborns. Should we reconsider transfusion of adult donor blood?

PURPOSE: The aim of this study was to assess the percent decrease in fetal hemoglobin (HbF) after transfusion of adult-derived donor packed red blood cell (pRBC) units in extremely low gestational age newborns (ELGANs). METHODS: Control percent fetal hemoglobin (%HbF) levels were measured in newborn cord blood or peripheral blood samples in non-transfused patients prior to elective surgery. ELGANs were followed prospectively and %HbF was measured on residual post-test complete blood count (CBC) specimens. ELGAN %HbF values were compared to the control population and transfusions were recorded. RESULTS: Initial mean %HbF in ELGANs (n=16) was 92.2±1.3% (range 90.2-95.1%), which is similar to the control group (n=25). Mean levels dropped to 61.1±11.1% (range 34.2-73.2%) after a single pRBC transfusion (n=9) and to a mean of 35.6±6.3% after an additional transfusion (n=5). %HbF levels trended upwards if no additional transfusions were given, but levels still remained lower than expected for gestational age through discharge (n=85 samples). CONCLUSIONS: Percent fetal hemoglobin concentrations in ELGANs decrease precipitously after transfusion with adult donor pRBCs. Further studies are needed to evaluate the benefit of maintaining higher fetal hemoglobin concentrations in these patients and whether administration of HbF rather than adult donor pRBCs would improve patient outcomes.