Risk of meningomyelocele mediated by the common 22q11.2 deletion.

Meningomyelocele is one of the most severe forms of neural tube defects (NTDs) and the most frequent structural birth defect of the central nervous system. We assembled the Spina Bifida Sequencing Consortium to identify causes. Exome and genome sequencing of 715 parent-offspring trios identified six patients with chromosomal 22q11.2 deletions, suggesting a 23-fold increased risk compared with the general population. Furthermore, analysis of a separate 22q11.2 deletion cohort suggested a 12- to 15-fold increased NTD risk of meningomyelocele. The loss of Crkl, one of several neural tube-expressed genes within the minimal deletion interval, was sufficient to replicate NTDs in mice, where both penetrance and expressivity were exacerbated by maternal folate deficiency. Thus, the common 22q11.2 deletion confers substantial meningomyelocele risk, which is partially alleviated by folate supplementation.

Immunohistochemical and Histopathological Characterization of Spina Bifida Defect Tissues Removed After Prenatal and Postnatal Surgical Repair.

Background: Myelomeningocele or spina bifida is an open neural tube defect that is characterized by protrusion of the meninges and the spinal cord through a deformity in the vertebral arch and spinous process. Myelomeningocele of post-natal tissue is well described; however, pre-natal tissue of this defect has no known previous histologic characterization. We compared the histology of different forms of pre-natal myelomeningocele and post-natal myelomeningocele tissue obtained via prenatal intrauterine and postnatal surgical repairs. Methods: Pre-and post-natal tissues from spina bifida repair surgeries were obtained from lipomyelomeningocele, myeloschisis, and myelomeningocele spina bifida defects. Tissue samples were processed for H&E and immunohistochemical staining (KRT14 and p63) to assess epidermal and dermal development. Results: Prenatal skin near the defect site develops with normal epidermal, dermal, and adnexal structures. Within the grossly cystic specimens, histology shows highly dense fibrous connective tissue with complete absence of a normal epidermal development with a lack of p63 and KRT14 expression. Conclusion: Tissues harvested from prenatal and postnatal spina bifida repair surgeries appear as normal skin near the defect site. However, cystic tissues consist of highly dense fibrous connective tissue with complete absence of normal epidermal development.

Prevalence of Chronic Middle Ear Effusion in Cases of Cleft Palate.

Chronic middle ear effusion is generally present in children with cleft palate (CP) associated with or without cleft lips. The aim of our study was to assessment of how common middle ear effusion is in patients with cleft palates and to evaluate the presence of these symptoms by performing a Basic Audiological Evaluation (BAE). A retrospective randomized study was conducted on 50 children (29 male and 21 female) aged 2 to 16 years who had CPs (associated with or without cleft lips) with symptoms of middle ear effusion. The study was conducted from March 2021 to February 2022. Data review included the results of otoscopic findings and BAE. The Fundamentals of BAE comprise the testing of middle ear function with tympanometry and a pure tone audiometry to determine the kind and degree of hearing impairment. Regarding the BAE, we found that 70% of the children with normal hearing, 24% with conductive type of hearing loss, and 6% with mixed type of hearing loss. The tympanometric results revealed that 66% of the children with type A tympanogram, 24% with type C tympanogram, and 10% with type B tympanogram. The contralateral stapedial reflex was present in 60% of the patients while in 40% of cases not present. The results confirmed the great prevalence of chronic middle ear effusion in children with CPs. Furthermore, the hearing impairment associated with middle ear effusion was visible, demonstrating that middle ear effusion was linked with the prevalence of moderate conductive type of hearing loss. The OME in cases of CP necessitates early prediction and in turn early treatment.

Role of Loupes Magnification in Tracheal Resection and Anastomosis.

Tracheal resection and anastomosis is characterized in the last years by significant innovations which are well codified and standardized. Although the mortality rate is markedly reduced, the operation is still not free from risk of complications such as recurrent laryngeal nerve injury, anastomosis dehiscence, granulation tissue formation and restenosis. Pearson FG, Cooper ID, Nelems JL (1975) Primary tracheal anastomosis after resection of the cricoide cartilage with preservation of the recurrent laryngeal nerves. J Thorac Cardiovasc Surg 70:806-16. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-023-04115-3.

Percutaneous versus Open Fetal Surgical Intervention for Sacrococcygeal Teratomas: Is Less Really More?

INTRODUCTION: Sacrococcygeal teratomas (SCTs) may require in utero intervention for survival. Open surgical intervention (OSI) was first described, but increasing reports of percutaneous intervention (PI) with variable indications and outcomes exist. We reviewed the literature for all published cases of fetal SCT intervention and compared OSI to PI cohorts. METHODS: A keyword search of PubMed was conducted. Inclusion criteria were as follows: data available per individual fetus including gestational age at intervention, type of intervention, primary indication, survival, gestational age at birth, and complications. Complications were grouped into categories: placenta/membrane, procedural, or hemorrhagic. Failure was defined as little/no improvement or recurrence of the primary indication. χ2 analysis was performed for solid tumor PI versus OSI to assess significant trends in these intervention groups. A meta-analysis was not feasible due to small numbers and heterogeneity. RESULTS: Twenty-seven articles met inclusion criteria. In the PI group, 38 fetuses underwent intervention for solid tumors, 21 for cystic tumors, and 3 for solid and cystic tumor components. Among fetuses with solid tumors, OSI was associated with lower need for multiple interventions (0% vs. 31.6%, p = 0.01) and higher survival to discharge (50% vs. 39.5%, p = 0.02). A fetal intervention was performed in the absence of hydrops/early hydrops in 45% of fetuses receiving PI, compared to 21% receiving OSI. Failure to resolve the primary indication was higher in the PI group (55.9% vs. 11.1% OSI, p = 0.02). The overall complication rates were high in both groups (90% OSI, 87% PI), though bleeding was unique to the PI group (26.5%). Preemptive cyst drainage, for purely cystic tumors, was universally successful and associated with a low complication risk (18.2%). CONCLUSIONS: For solid tumors, OSI appears to be superior with regard to survival to discharge, fewer interventions, and lower failure rates. PIs to drain a cyst may facilitate delivery or preempt future complications, though consideration should be given to long-term oncologic outcomes.

The establishment of national diagnostic reference levels for adult SPECT-CT in Saudi Arabia.

This study aims to introduce national diagnostic reference levels (NDRLs) for adult hybrid single photon emission computed tomography (SPECT-CT) in nuclear medicine (NM) departments in the Kingdom of Saudi Arabia. The administered activity (AA) of radiopharmaceuticals, volume-weighted computed tomography dose index (CTDIvol) and dose length product (DLP) for ten hybrid SPECT/CT examinations were collected and analysed for one year. The median of AA, CTDIvoland DLP for each dose quantity was derived and the suggested national DRLs were determined based on the 75thpercentile for all identified SPECT-CT examinations. A comparison of the defined adult NDRLs in Saudi Arabia with the published data of other countries was performed. Although there are no significant variations of the proposed NDRL of AA between countries, the proposed NDRLs of the integrated CT metrics exceed the published data in most procedures. NM departments are urged to consider optimisation for both image quality and radiation protection.

Determinants of parental seasonal influenza vaccine hesitancy in the Eastern Mediterranean region: A cross-sectional study.

Background: Seasonal influenza vaccine can reduce the risk of influenza-associated hospitalizations and deaths among children. Given that parents are the primary decision makers, this study examined the parental attitude toward childhood influenza vaccine and identified determinants of vaccine hesitancy (VH) in the Eastern Mediterranean region (EMR). Methods: A cross-sectional study was conducted using an anonymous online survey in 14 EMR countries. Parents of children aged 6 months to 18 years were included. The Parent Attitude about Childhood Vaccines (PACV) was used to assess VH. Chi square test and independent t-test were used to test for association of qualitative and quantitative variables, respectively. A structural equations model (SEM) was used to identify direct and indirect determinants of parental VH. Results: Almost half of the parents were hesitant about vaccinating their children against influenza (50.8%). Parental VH was significantly higher among older mothers (37.06 ± 8.8 years, p = 0.006), rural residents (53.6%, p < 0.001), high-income countries residents (50.6%, p < 0.001), and mothers with higher educational levels (52.1%, p < 0.001). Parents of school-aged children (5-9 years) (55.6%, p < 0.001), children free from any comorbidities (52.5%, p < 0.001), children who did not receive routine vaccination at all (51.5%, p = 0.03), children who were not vaccinated against COVID-19 (54.3%, p < 0.001), in addition to parents who were not vaccinated against influenza (57.1%, p < 0.001) were significantly associated with increased likelihood of VH. Parents who were depending on healthcare provider as a source of information regarding vaccines were less likely to report VH (47.9%, p < 0.001), meanwhile those who used social media as their source of health information showed a significantly higher VH (57.2%, p < 0.001). The SEM suggested that mother's age, residence, country income level, child gender, total number of children and source of information regarding vaccines had a direct effect on VH. Meanwhile, parents vaccinated against influenza, children completely or partially vaccinated with routine vaccines and children vaccinated against Coronavirus disease 2019 (COVID-19) had an indirect effect on VH. Conclusion: A high proportion of included parents were hesitant to vaccinate their children against seasonal influenza. This attitude is due to many modifiable and non-modifiable factors that can be targeted to improve vaccination coverage.

Improvement of Pancreatic Steatosis and Indices of Insulin Resistance After Metabolic Surgery.

Purpose: Obesity is associated with fat accumulation in ectopic sites such as the pancreas, the so-called pancreatic steatosis (PS). Bariatric surgery has been shown to be associated with reducing pancreatic fat. This study investigated the effect of laparoscopic sleeve gastrectomy (LSG) on pancreatic volume and its fat content and glucose homeostasis. Methods: The study enrolled 54 patients subjected to LSG. Metabolic variables and pancreatic exocrine function were assessed immediately before surgery and 12 months after. MRI of the abdomen was performed to measure pancreatic fat content and its total volume and visceral adipose tissue (VAT). Results: Surgery resulted in a significant reduction in body weight and BMI. HbA1c, fasting insulin, C-peptide levels, HOMA-IR, and Hs-CRP levels decreased significantly. Surgery resulted in significant improvement in lipid profile except for HDL-cholesterol and liver function tests. Total VAT volume decreased significantly. Total pancreas volume decreased by a mean of 9.0 cm3 (95% CI: 6.6-11.3). The median change of pancreatic fat was -26.1% (range: -55.6 to 58.3%). Pancreatic lipase decreased significantly (P < 0.001). There was a positive correlation between the percentage of total weight loss and decrease in pancreatic fat volume (r = 0.295, P = 0.030). Conclusion: Weight loss after LSG is associated with a reduction of total VAT volume, total pancreatic volume, and pancreatic fat content. These changes are associated with improved glucose homeostasis, reduced systemic inflammation, and decreased pancreatic lipase secretion.

Comparative study between skin micrografting (Meek technique) and meshed skin grafts in paediatric burns.

BACKGROUND: Globally, burn injuries are the 3rd principal cause of death due to injury among children aged 1-9 years. Yet, the management of paediatric burns is always challenging; due to limited donor sites and the cosmetic appearance that will affect the child later in life, either at the donor or the recipient site. Skin grafts may need to be expanded to minimise donor skin size or in patients with limited donor sites. Multiple techniques were described for graft expansion, mainly the mesher and the Meek technique. PATIENTS AND METHODS: A prospective comparative randomised study was done from January 2019 to June 2020 on 40 paediatric burn patients with deep dermal and full-thickness burns. Patients were divided into two groups, Meek and meshed groups. The skin graft take, epithelialization time, total time of the surgery and the aesthetic outcomes (using the POSAS - Patient and observer scar assessment scale) in each group were evaluated at three months postoperatively. RESULTS: The percentage of take in the Meek group (84.25%) was significantly better than with the meshed group (71.5%) (P = 0.006). Epithelialization time was better for the Meek group (27.11 days) compared to the meshed group (33.5 days) (P = 0.176). In addition, infection rates were lower in the Meek group (25%) than the meshed group (40%) (P = 0.311). Subjectively POSAS scar assessment scale exhibited better results for the Meek group with a mean score of 3.17 & for the meshed group was 4.2 (P = 0.048). The observer's overall score was as well better for the Meek group with a mean overall opinion score of 2.89 & for the meshed group was 4.1 (P = 0.003). The operative time was longer with the Meek technique than the traditional mesher (P < 0.001). CONCLUSION: The Meek technique for expanding the skin grafts is useful in covering burn wounds with greater expansion rate, more accessible application, better graft take & a better scar appearance than the traditional mesher. Still, the Meek technique has a considerable learning curve, longer procedure time & is more expensive.

Recognition and management of traumatic fetal injuries.

Trauma during pregnancy is the leading non-obstetric cause of morbidity and mortality, and accounts for five per 1000 fetal deaths. Direct fetal injury due to trauma during pregnancy is rare, and limited information is available about how to optimize fetal outcomes after injury. Early recognition and appropriate management of direct fetal trauma may improve outcomes for the fetus. There are currently no available guidelines to direct management of the injured fetus. We provide a detailed literature review of the management and outcomes of direct fetal injury following blunt and penetrating injury during pregnancy, and describe a suggested initial approach to the injured pregnant patient with a focus on evaluation for fetal injury. We identified 45 reported cases of blunt trauma resulting in direct fetal injury, with 21 surviving past the neonatal period, and 33 of penetrating trauma resulting in direct fetal injury, with 24 surviving past the neonatal period. Prenatal imaging identified fetal injury in 19 cases of blunt trauma and was used to identify bullet location relative to the fetus in 6 cases. These reports were used to develop management algorithms for the injured fetus.

Preliminary Results of a Reverse Thermal Gel Patch for Fetal Ovine Myelomeningocele Repair.

BACKGROUND: Prenatal surgical closure of Myelomeningocele (MMC) is considered part of the current age armamentarium. Clinical data has demonstrated the need for innovative patches to maximize the benefits and decrease the risks of this approach. Our team has developed a minimally invasive reverse thermal gel (RTG) patch with cellular scaffolding properties. Here, we demonstrate the initial gross and microscopic histological effects of this RTG patch in the fetal ovine model of MMC. MATERIALS AND METHODS: A fetal ovine MMC defect was created at 68-75 days gestation, RTG patch application or untreated at 100-103 days, and harvest at 135-140 days. The RTG was applied to the defect and secured in place with an overlay sealant. Defect areas underwent gross and microscopic analysis for inflammation and skin development. Brains were analyzed for hindbrain herniation and hydrocephalus. RESULTS: The untreated fetus (n = 1) demonstrated an open defect lacking tissue coverage, evidence of spinal cord injury, increased caspase-3, Iba1 and GFAP in spinal cord tissues, and hindbrain herniation and ventricular dilation. RTG treated fetuses (n = 3) demonstrated defect healing with well-organized dermal and epidermal layers throughout the entire healed tissue area overlaying the defect with minimal inflammation, reduced caspase-3, Iba1 and GFAP in spinal cord tissues, and no hindbrain herniation or ventricular dilation. CONCLUSION: An RTG patch applied to MMC defects in fetal sheep promoted skin coverage over the defect, was associated with minimal inflammation of the spinal cord tissues and prevented brain abnormalities. The present findings provide exciting results for future comprehensive radiological, functional, and mechanistic evaluation of the RTG.

The need for early Kasai portoenterostomy: a Western Pediatric Surgery Research Consortium study.

PURPOSE: The purpose of this study was to investigate factors impacting transplant-free survival among infants with biliary atresia. METHODS: A multi-institutional, retrospective cohort study was performed at nine tertiary-level children's hospitals in the United States. Infants who underwent Kasai portoenterostomy (KP) from January 2009 to May 2017 were identified. Clinical characteristics included age at time of KP, steroid use, surgical approach, liver pathology, and surgeon experience. Likelihood of transplant-free survival (TFS) was evaluated using logistic regression, adjusting for patient and surgeon-level factors. Secondary outcomes at 1 year included readmission, cholangitis, reoperation, mortality, and biliary clearance. RESULTS: Overall, 223 infants underwent KP, and 91 (40.8%) survived with their native liver. Mean age at surgery was 63.9 days (± 24.7 days). At 1 year, 78.5% experienced readmission, 56.9% developed cholangitis, 3.8% had a surgical revision, and 5 died. Biliary clearance at 3 months was achieved in 76.6%. Controlling for patient and surgeon-level factors, each additional day of age toward operation was associated with a 2% decrease in likelihood of TFS (OR 0.98, 95% CI 0.97-0.99). CONCLUSION: Earlier surgical intervention by Kasai portoenterostomy at tertiary-level centers significantly increases likelihood for TFS. Policy-level interventions to facilitate early screening and surgical referral for infants with biliary atresia are warranted to improve outcomes.

The novel fetal MRI O/E CLV versus O/E LHR in predicting prognosis in congenital diaphragmatic hernias: can we teach an old dog new tricks?

PURPOSE: In congenital diaphragmatic hernia (CDH), ultrasound (U/S) measurements of the contralateral lung commonly provide the observed-to-expected lung-to-head ratio (O/E LHR) and are used to determine the severity of pulmonary hypoplasia. Fetal magnetic resonance imaging (MRI) measurement of the observed-to-expected total lung volume (O/E TLV) has been used as an adjunct to O/E LHR in predicting outcomes. Since O/E LHR only measures the contralateral lung, we sought to investigate if MRI measurements of the contralateral lung volume (O/E CLV) can accurately predict outcomes in CDH. We hypothesize that O/E CLV is a better predictor of CDH outcomes than O/E LHR. METHODS: We identified all infants with a prenatal diagnosis of CDH at our fetal center who had both MRI and U/S measurements. Using lung volume ratios of right-left 55:45, we calculated O/E CLV from O/E TLV. We used receiver-operating characteristic (ROC) curves to calculate the area under the curve (AUC) to compare the predictive accuracy of O/E CLV to O/E LHR for ECMO support, as well as survival to both discharge and 1 year. RESULTS: Seventy-four patients had complete prenatal imaging with 39% requiring ECMO support. The median O/E CLV was 48.0% and the median O/E LHR was 42.3%. O/E CLV was a better predictor of the need for ECMO support (AUC 0.81 vs. 0.74). O/E CLV was a better predictor of survival to discharge (AUC 0.84 vs. 0.64) and 1-year survival (AUC 0.83 vs. 0.63) than O/E LHR. CONCLUSION: O/E LHR is a well-validated standard for predicting outcomes and guiding prenatal counseling in CDH. We provide evidence that fetal MRI measurements of the contralateral lung volume corrected for gestational age were more accurate in predicting the need for ECMO and survival. Future prospective studies validating O/E CLV regarding outcomes and ECMO utilization are warranted. LEVEL OF EVIDENCE: Level III, retrospective comparative study.

Open versus laparoscopic pyloromyotomy for pyloric stenosis.

BACKGROUND: Infantile hypertrophic pyloric stenosis (IHPS) is a disorder of young children (aged one year or less) and can be treated by laparoscopic (LP) or open (OP) longitudinal myotomy of the pylorus. Since the first description in 1990, LP is being performed more often worldwide. OBJECTIVES: To compare the efficacy and safety of open versus laparoscopic pyloromyotomy for IHPS. SEARCH METHODS: We conducted a literature search on 04 February 2021 to identify all randomised controlled trials (RCTs), without any language restrictions. We searched the following electronic databases: MEDLINE (1990 to February 2021), Embase (1990 to February 2021), and the Cochrane Central Register of Controlled Trials (CENTRAL). We also searched the Internet using the Google Search engine (www.google.com) and Google Scholar (scholar.google.com) to identify grey literature not indexed in databases. SELECTION CRITERIA: We included RCTs and quasi-randomised trials comparing LP with OP for hypertrophic pyloric stenosis. DATA COLLECTION AND ANALYSIS: Two review authors independently screened references and extracted data from trial reports. Where outcomes or study details were not reported, we requested missing data from the corresponding authors of the primary RCTs. We used a random-effects model to calculate risk ratios (RRs) for binary outcomes, and mean differences (MDs) for continuous outcomes. Two review authors independently assessed risks of bias. We used GRADE to assess the certainty of the evidence for all outcomes. MAIN RESULTS: The electronic database search resulted in a total of 434 records. After de-duplication, we screened 410 independent publications, and ultimately included seven RCTs (reported in 8 reports) in quantitative analysis. The seven included RCTs enrolled 720 participants (357 with open pyloromyotomy and 363 with laparoscopic pyloromyotomy). One study was a multi-country trial, three were carried out in the USA, and one study each was carried out in France, Japan, and Bangladesh. The evidence suggests that LP may result in a small increase in mucosal perforation compared with OP (RR 1.60, 95% CI 0.49 to 5.26; 7 studies, 720 participants; low-certainty evidence). LP may result in up to 5 extra instances of mucosal perforation per 1,000 participants; however, the confidence interval ranges from 4 fewer to 44 more per 1,000 participants. Four RCTs with 502 participants reported on incomplete pyloromyotomy. They indicate that LP may increase the risk of incomplete pyloromyotomy compared with OP, but the confidence interval crosses the line of no effect (RR 7.37, 95% CI 0.92 to 59.11; 4 studies, 502 participants; low-certainty evidence). In the LP groups, 6 cases of incomplete pyloromyotomy were reported in 247 participants while no cases of incomplete pyloromyotomy were reported in the OP groups (from 255 participants). All included studies (720 participants) reported on postoperative wound infections or abscess formations. The evidence is very uncertain about the effect of LP on postoperative wound infection or abscess formation compared with OP (RR 0.59, 95% CI 0.24 to 1.45; 7 studies, 720 participants; very low-certainty evidence). The evidence is also very uncertain about the effect of LP on postoperative incisional hernia compared with OP (RR 1.01, 95% CI 0.11 to 9.53; 4 studies, 382 participants; very low-certainty evidence). Length of hospital stay was assessed by five RCTs, including 562 participants. The evidence is very uncertain about the effect of LP compared to OP (mean difference -3.01 hours, 95% CI -8.39 to 2.37 hours; very low-certainty evidence). Time to full feeds was assessed by six studies, including 622 participants. The evidence is very uncertain about the effect of LP on time to full feeds compared with OP (mean difference -5.86 hours, 95% CI -15.95 to 4.24 hours; very low-certainty evidence). The evidence is also very uncertain about the effect of LP on operating time compared with OP (mean difference 0.53 minutes, 95% CI -3.53 to 4.59 minutes; 6 studies, 622 participants; very low-certainty evidence). AUTHORS' CONCLUSIONS: Laparoscopic pyloromyotomy may result in a small increase in mucosal perforation when compared with open pyloromyotomy for IHPS. There may be an increased risk of incomplete pyloromyotomy following LP compared with OP, but the effect estimate is imprecise and includes the possibility of no difference. We do not know about the effect of LP compared with OP on the need for re-operation, postoperative wound infections or abscess formation, postoperative haematoma or seroma formation, incisional hernia occurrence, length of postoperative stay, time to full feeds, or operating time because the certainty of the evidence was very low for these outcomes. We downgraded the certainty of the evidence for most outcomes due to limitations in the study design (most outcomes were susceptible to detection bias) and imprecision. There is limited evidence available comparing LP with OP for IHPS. The included studies did not provide sufficient information to determine the effect of training, experience, or surgeon preferences on the outcomes assessed.

Congenital diaphragmatic hernias: Severe defect grade predicts the need for fundoplication.

Over one-third of infants with congenital diaphragmatic hernia (CDH) eventually require a Nissen fundoplication (NF). We examined pre- and intraoperative predictors for need of a NF in children undergoing CDH repair to elucidate, which patients will need a later NF.A retrospective analysis of all consecutive patients undergoing CDH repair at our institution from 2008 to 2018 was performed. Patients who underwent a NF were compared to those who did not (noNissen). Logistic regression analysis was performed to find independent predictors for NF in patients undergoing CDH repair. Severe Defect Grade was defined as defect >50% of the hemidiaphragm and intrathoracic liver.One hundred twenty-six patients were included, 42 (33%) underwent NF at a median of 61 days after CDH repair. Intrathoracic liver was more frequent in the NF (71%) versus noNissen (45%) group (P = .008). Absence of >50% of the hemidiaphragm was more frequent in the NF group (76% vs 31%, P < .001). Severe Defect Grade emerged as independent predictor for NF (odds ratio 7, 95% confidence interval 3-16, P < .001).Severe Defect Grade emerged as independent predictor for NF after CDH repair.

Outcomes of Airway Management in Micrognathia and Retrognathia Patients Born at Fetal versus Nonfetal Centers.

OBJECTIVES: There is a paucity of evidence to guide the perinatal management of difficult airways in fetuses with micrognathia. We aimed to (1) develop a postnatal grading system based on the extent of airway intervention required at birth to assess the severity of micrognathic airways and (2) compare trends in airway management and outcomes by location of birth [nonfetal center (NFC), defined as a hospital with or without an NICU and no fetal team, versus fetal center (FC), defined as a hospital with an NICU and fetal team]. METHODS: We retrospectively reviewed the prenatal and postnatal records of all neonates diagnosed with micrognathia from January 2010 to April 2018 at a quaternary children's hospital. We developed a novel grading scale, the Micrognathia Grading Scale (MGS), to grade the extent of airway intervention at birth from 0 (no airway intervention) to 4 (requirement of EXIT or advanced airway instrumentation for airway securement). RESULTS: We identified 118 patients with micrognathia. Eighty-nine percent (105/118) were eligible for grading using the MGS. When the MGS was applied, the airway grades were as follows: grade 0 (30%), grade 1 (10%), grade 2 (9%), grade 3 (48%), and grade 4 (4%). A quarter of micrognathic patients with grade 0-2 airways had postnatal hospital readmissions for airway obstruction after birth, of which all were born at NFC. Over 40% of patients with grade 3-4 micrognathic airways required airway intervention within 24 h of birth. Overall, NFC patients had a readmission rate of (27%) for airway obstruction after birth compared to FC patients (17%). CONCLUSIONS: Due to the high incidence of grade 3-4 airways on the MGS in micrognathic patients, fetuses with prenatal findings suggestive of micrognathia should be referred to a comprehensive fetal care center capable of handling complex neonatal airways. For grade 0-2 airways, infants frequently had postnatal complications necessitating airway intervention; early referral to a multidisciplinary team for both prenatal and postnatal airway management is recommended.

Thromboelastography-guided management of coagulopathy in neonates with congenital diaphragmatic hernia supported by extracorporeal membrane oxygenation.

PURPOSE: Congenital diaphragmatic hernia (CDH) can cause severe hemodynamic deterioration requiring support with extracorporeal membrane oxygenation (ECMO). ECMO is associated with hemorrhagic and thromboembolic complications. In 2015, we standardized anti-coagulation management on ECMO, incorporating thromboelastography (TEG) as an adjunct to manage hemostasis of CDH patients. The purpose of this study is to evaluate our blood product utilization, choice of blood product use in response to abnormal TEG parameters, and the associated effect on bleeding and thrombotic complications. METHODS: We retrospectively reviewed all CDH neonates supported by ECMO between 2008 and 2018. Blood product administration, TEG data, and hemorrhagic and thrombotic complications data were collected. We divided subjects into two groups pre-2015 and post-2015. RESULTS: After 2015, platelet transfusion was administered for a low maximum amplitude (MA) more frequently (77% compared to 65%, p = 0.0007). Cryoprecipitate was administered less frequently for a low alpha-angle (28% compared to 41%, p = 0.0016). There was no difference in fresh frozen plasma use over time. After standardizing the use of TEG, we observed a significant reduction in hemothoraces (18% compared to 54%, p = 0.026). CONCLUSION: Institutional standardization of anti-coagulation management of CDH neonates on ECMO, including the use of goal-directed TEG monitoring may lead to improved blood product utilization and a decrease in bleeding complications in neonates with CDH supported by ECMO. LEVEL OF EVIDENCE/TYPE OF STUDY: Level III, Retrospective comparative study.

Fetal surgery and neonatal ICU admissions.

PURPOSE OF REVIEW: Fetal intervention is a dynamic field with wide-reaching implications on neonatal ICU admissions and the care of neonates with congenital anomalies. The aim of this review is to summarize recent advancements in fetal surgery and provide a broad understanding of how these topics interrelate. RECENT FINDINGS: Advancements in prenatal imaging and diagnosis have dramatically expanded the indications for fetal intervention. Most recently, there has been a large focus on evaluating minimally invasive strategies of fetal intervention, notably fetoscopic surgery, and the use of stem cells for fetal treatment of myelomeningocele. With the advances in fetal intervention, neonatal care has adapted to the needs of these various patients to help improve the outcomes of this unique population. SUMMARY: Fetal intevention relies on a multidisciplinary team from prenatal imaging and maternal fetal medicine to fetal surgery and postnatal subspecialty care, particuarly neonatology. Fetal intervention uniquely involves two patients, both mother and fetus, and therefore, has unique risks and considerations, particularly in the advancement of the field. As the number of conditions suited to fetal intervention grows, awareness and advancement of the postnatal intensive care necessary for these patients are essential.

Evidence according to Cochrane Systematic Reviews on Alterable Risk Factors for Anastomotic Leakage in Colorectal Surgery.

Anastomotic leakage reflects a major problem in visceral surgery, leading to increased morbidity, mortality, and costs. This review is aimed at evaluating and summarizing risk factors for colorectal anastomotic leakage. A generalized discussion first introduces risk factors beginning with nonalterable factors. Focus is then brought to alterable impact factors on colorectal anastomoses, utilizing Cochrane systematic reviews assessed via systemic literature search of the Cochrane Central Register of Controlled Trials and Medline until May 2019. Seventeen meta-anaylses covering 20 factors were identified. Thereof, 7 factors were preoperative, 10 intraoperative, and 3 postoperative. Three factors significantly reduced the incidence of anastomotic leaks: high (versus low) surgeon's operative volume (RR = 0.68), stapled (versus handsewn) ileocolic anastomosis (RR = 0.41), and a diverting ostomy in anterior resection for rectal carcinoma (RR = 0.32). Discussion of all alterable factors is made in the setting of the pre-, intra-, and postoperative influencers, with the only significant preoperative risk modifier being a high colorectal volume surgeon and the only significant intraoperative factors being utilizing staples in ileocolic anastomoses and a diverting ostomy in rectal anastomoses. There were no measured postoperative alterable factors affecting anastomotic integrity.