Optimal Timing of Inguinal Hernia Repair in Premature Infants: A NSQIP-P Study.

INTRODUCTION: Among premature infants, the incidence of inguinal hernias is reported to be as high as 30%. Despite being one of the most commonly performed procedures, the optimal setting of inguinal hernia repair (IHR) that is inpatient versus outpatient remains debatable. We sought to compare the 30-day outcomes of each approach by querying the National Surgical Quality Improvement Program-Pediatric database. MATERIALS AND METHODS: A retrospective cohort study comparing inpatient versus outpatient IHR using the National Surgical Quality Improvement Program-Pediatric database from 2013 to 2019 was performed. Demographic and clinical data were initially compared using univariate analysis. Continuous variables are presented as median and interquartile range and categorical variables are presented as n (%). Subsequently, cohorts were propensity matched using clinically and statistically significant patient characteristics. RESULTS: 928 patients underwent IHR, 634 (68.3%) while inpatient, 294 (31.7%) following hospital discharge. Inpatient IHR was associated with lower age at the time of surgery (120 versus 147 d; P < 0.0001), younger gestational age (27 versus 33 wk; P < 0.0001), decreased probability of repair in elective setting (87.2% versus 97.3%; P < 0.0001), and increased preoperative supplemental oxygen need (42% versus 4.4%; P < 0.0001). Comparison of propensity matched cohorts revealed that inpatient IHR was associated with increased procedure time (82 versus 51 min; P < 0.0001) and anesthetic duration (146 versus 102 min; P < 0.0001), wound infection rates (3.8% versus 0%; P = 0.007), blood transfusions (4.2% versus 0.5%; P = 0.036), unplanned intubations (2.8% versus 0%; P = 0.03), ventilator days (0 versus 0; range [0,30 versus 0,2]; P = 0.002), reoperation rate (5.6% versus 0%; P < 0.001), postoperative hospital length of stay (4 versus 1 d; P < 0.0001), and unplanned readmissions (8.9% versus 0.9%; P = 0.002). CONCLUSIONS: Inpatient IHR in premature neonates were associated with different postoperative outcomes than outpatient IHR. At least in the elective setting among premature infants, outpatient IHR can be considered safe in select patients while we await higher quality prospective data.

Short-term outcomes of thoracoscopic versus open lobectomy for congenital lung malformations.

PURPOSE: Thoracoscopic and open approaches for the management of congenital lung malformations (CLM) has been debated. The aim of this study is to compare 30-day outcomes for non-emergent lobectomies in children. METHODS: The National Surgical Quality Improvement Program-Pediatric database was queried for patients undergoing CLM resection from 2013 to 2020. Outcomes were compared by operative technique in an intention-to-treat model and then propensity matched. RESULTS: 2157 patients met inclusion criteria and underwent non-emergent pulmonary lobectomy for CLM. The intended operative approach was thoracoscopic in 57.7% of patients. Patients in the open group compared to the thoracoscopic were more likely to be born premature, have chronic lung disease, require preoperative oxygen support, and be ventilator dependent. After propensity matching, there was no statistically significant difference in 30-day mortality, unplanned readmission, and other complications between the thoracoscopic and open groups. Thoracoscopic approach was associated with a shorter length of stay. The proportion of cases approached via thoracoscopy increased over time from 48.8% in 2013 to 69.9% in 2020. CONCLUSIONS: This large multicenter retrospective matched analysis demonstrates thoracoscopic lobectomy in children has similar favorable 30-day outcomes and shorter length of stay for the non-emergent management of CLM, compared to open thoracotomy. LEVEL OF EVIDENCE: Level III.

Single site versus conventional laparoscopic appendectomy: some pain for no gain?

INTRODUCTION: The optimal laparoscopic appendectomy approach is not clear, comparing single site laparoscopic appendectomy (SILA) to conventional 3-port appendectomy (CLA). We investigated outcomes in pediatric patients comparing SILA to CLA: length of operation, length of stay, time to resumption of regular diet, follow up, rehospitalization, and cost. METHODS: Data was collected from children 1 to 18 years with appendectomy at Loma Linda University from 2018 to 2020, operated by two surgeons. Analysis utilized two-sample T, chi-squared, and Fisher's exact tests. RESULTS: Of 173 patients, 77 underwent SILA and 96 had CLA. There was no gender, age, or race difference between groups. Mean WBC was 17.5 × 103/mL in SILA group, compared to 15.3 × 103/mL in CLA group (P = 0.004). Operative time was 47.0 SILA compared to 49.5 minutes CLA (P = 0.269). Of SILA cases, 55.8% were simple appendicitis, while 53.3% of the CLA cases were simple (P = 0.857). Regular diet was resumed after 1.7 days in the SILA group, 1.1 days in CLA (P = 0.018). Length of stay was 2.9 days for SILA, 2.4 days for CLA (P = 0.144). Seven children required hospital readmission, 5 SILA and 2 CLA (P = 0.244). Five of the children who returned had intra-abdominal abscesses, of whom 4 had SILA. There was no difference in cost. CONCLUSIONS: The operative techniques had similar outcomes and operative times. There was a trend toward more intra-abdominal abscesses in the SILA group. Further study and longer follow up is needed to determine if there is an advantage to one laparoscopic approach over another.

Inguinal hernias in premature neonates: exploring optimal timing for repair.

PURPOSE: Inguinal hernias have been reported in as many as 10-30% premature neonates, making inguinal herniorrhaphy (IHR) one of the most commonly performed surgical procedures. The timing of surgery remains controversial. The purpose of this report is to compare outcomes of IHR while in the NICU (inpatient) versus repair following discharge (outpatient) to determine optimal timing. METHODS: Premature neonates having undergone IHR over a 5-year period were identified and a retrospective case cohort analysis was performed. RESULTS: 263 patients underwent IHR during the 5-year study period with 115 (43.7%) having surgical repair inpatient (IP; prior to discharge) and 148 having outpatient herniorrhaphy (OP). Patients with IHR performed IP had significantly lower birth weight (p < 0.001), gestational age (p < 0.001), longer duration of surgery (p = 0.01) and were more likely to have post-operative ventilator dependence following repair; however, there were no differences in the rate of recurrence (p = 0.44) and incarceration (p = 0.45). CONCLUSION: Our study demonstrated no significant differences in the rates of incarceration or recurrence, following in- or out-patient IHR. These findings suggest that IHR can potentially be offered as an outpatient procedure following hospital discharge in appropriate patients. The optimal timing of IHR in premature infants remains elusive and will likely require additional multicenter investigation.

Esterified Trehalose Analogues Protect Mammalian Cells from Heat Shock.

Trehalose is a disaccharide produced by many organisms to better enable them to survive environmental stresses, including heat, cold, desiccation, and reactive oxygen species. Mammalian cells do not naturally biosynthesize trehalose; however, when introduced into mammalian cells, trehalose provides protection from damage associated with freezing and drying. One of the major difficulties in using trehalose as a cellular protectant for mammalian cells is the delivery of this disaccharide into the intracellular environment; mammalian cell membranes are impermeable to the hydrophilic sugar trehalose. A panel of cell-permeable trehalose analogues, in which the hydrophilic hydroxyl groups of trehalose are masked as esters, have been synthesized and the ability of these analogues to load trehalose into mammalian cells has been evaluated. Two of these analogues deliver millimolar concentrations of free trehalose into a variety of mammalian cells. Critically, Jurkat cells incubated with these analogues show improved survival after heat shock, relative to untreated Jurkat cells. The method reported herein thus paves the way for the use of esterified analogues of trehalose as a facile means to deliver high concentrations of trehalose into mammalian cells for use as a cellular protectant.

Publication Trends in Craniofacial Distraction: A Bibliometrical Analysis.

PURPOSE: The purpose of this study was to analyze the craniofacial distraction literature published over the last 50 years and to determine various trends in publications. METHODS: A literature search was conducted in November and December 2015. The date search range was 1965 to 2015. Databases searched included Medline, Web of Science, Biosis, SciELO, Data Citation, and Zoologic Records. Data were collected on distraction type, author specialty, date of publication, country, state (if United States), number of citations, journal name, journal type, and Le Fort type (for midfacial distractions). RESULTS: Total number of craniofacial distraction publications was 1729. Cranial distraction accounted for (11%), midfacial (11%), and mandibular (78%). Largest increase in publications was in the 1990s, with 48 publications from 1991 to 1995 rising to 261 publications from 1996 to 2000. Among the cranial distraction publications, Plastic and Reconstructive Surgery (PRS) (67%) were the most frequent authors but among the midfacial and mandibular distraction publications, Oral and Maxillofacial Surgery (OMFS) were the most frequent authors (68% and 64%, respectively). Total number of citations was 26,281 with OMFS (50.4%) and PRS (37%) being cited most frequently. Oral and Maxillofacial Surgery was cited most for mandibular and midfacial distraction, and PRS was cited most for cranial distraction. CONCLUSION: Research on craniofacial distraction has significantly increased since the 1970s, with mandibular distraction accounting for the majority of this rise. Among specialties, OMFS and PRS account for the majority of the literature. The United States leads the publication. Authors tend to publish distraction literature in their corresponding journal specialty, with the exception of PRS who publishes most frequently in OMFS journals.

Paget-Schroetter syndrome in 21 children: outcomes after multidisciplinary care.

OBJECTIVE: To review the presentation, management, and outcomes of Paget-Schroetter syndrome (PSS) in children and propose a multidisciplinary treatment algorithm involving pediatric and vascular surgery, interventional radiology, and hematology. STUDY DESIGN: Patients with PSS presenting between 2003 and 2013 were reviewed. Demographics, symptoms, therapies, and functional outcomes were noted. Data from early patients informed the development of a multidisciplinary treatment algorithm applied to later patients. RESULTS: Of 21 patients, mean ± SD age was 16 ± 1.6 years and 11 (52%) were male. Of patients with complete presentation data, common symptoms were edema (84%), discoloration (58%), and pain (58%). Thrombophilia workup revealed one heterozygote for factor V Leiden, 2 patients with factor VIII elevation and 1 patient with mildly low antithrombin. The most recent 8 patients were treated according to an algorithm developed by a multidisciplinary working group through experience with the first 13 cases. All patients underwent a venogram, endovascular intervention (including 15 receiving catheter-directed thrombolysis), and operative ipsilateral thoracic outlet decompression (first rib resection, anterior scalenectomy, and venolysis). Postoperative complications included hemothorax (2), pneumothorax (1), and recurrent thrombosis (2). Follow up duration was 12 ± 9.5 months. Symptoms recurred transiently in 1 patient. CONCLUSION: Pediatric patients with PSS can be treated successfully using a multidisciplinary treatment algorithm including anticoagulation, catheter-directed thrombolysis, and operative decompression of the thoracic outlet. Early outcomes are promising.

Predictors of Enteral Autonomy in Children with Intestinal Failure: A Multicenter Cohort Study.

OBJECTIVES: In a large cohort of children with intestinal failure (IF), we sought to determine the cumulative incidence of achieving enteral autonomy and identify patient and institutional characteristics associated with enteral autonomy. STUDY DESIGN: A multicenter, retrospective cohort analysis from the Pediatric Intestinal Failure Consortium was performed. IF was defined as severe congenital or acquired gastrointestinal diseases during infancy with dependence on parenteral nutrition (PN) >60 days. Enteral autonomy was defined as PN discontinuation >3 months. RESULTS: A total of 272 infants were followed for a median (IQR) of 33.5 (16.2-51.5) months. Enteral autonomy was achieved in 118 (43%); 36 (13%) remained PN dependent and 118 (43%) patients died or underwent transplantation. Multivariable analysis identified necrotizing enterocolitis (NEC; OR 2.42, 95% CI 1.33-4.47), care at an IF site without an associated intestinal transplantation program (OR 2.73, 95% CI 1.56-4.78), and an intact ileocecal valve (OR 2.80, 95% CI 1.63-4.83) as independent risk factors for enteral autonomy. A second model (n = 144) that included only patients with intraoperatively measured residual small bowel length found NEC (OR 3.44, 95% CI 1.36-8.71), care at a nonintestinal transplantation center (OR 6.56, 95% CI 2.53-16.98), and residual small bowel length (OR 1.04 cm, 95% CI 1.02-1.06 cm) to be independently associated with enteral autonomy. CONCLUSIONS: A substantial proportion of infants with IF can achieve enteral autonomy. Underlying NEC, preserved ileocecal valve, and longer bowel length are associated with achieving enteral autonomy. It is likely that variations in institutional practices and referral patterns also affect outcomes in children with IF.

Preservation of biochemical liver function with low-dose soy-based lipids in children with intestinal failure-associated liver disease.

OBJECTIVES: Intestinal failure-associated liver disease (IFALD) contributes to significant morbidity in pediatric patients with intestinal failure (IF); however, the use of parenteral nutrition (PN) with a fish oil-based intravenous (IV) emulsion (FO) has been associated with biochemical reversal of cholestasis and improved outcomes. Unfortunately, FO increases the complexity of care: because it can be administered only under Food and Drug Administration compassionate use protocols requiring special monitoring, it is not available as a 3-in-1 solution and is more expensive than comparable soy-based IV lipid emulsion (SO). Because of these pragmatic constraints, a series of patient families were switched to low-dose (1 g kg(-1) day(-1)) SO following biochemical resolution of cholestasis. The present study examines whether reversal of cholestasis and somatic growth are maintained following this transition. METHODS: The present study is a chart review of all children with IFALD who switched from FO to SO following resolution of cholestasis. Variables are presented as medians (interquartile ranges). Comparisons were performed using the Wilcoxon signed-rank test. RESULTS: Seven patients ages 25.9 (16.2-43.2) months were transitioned to SO following reversal of cholestasis using FO. At a median follow-up of 13.9 (4.3-50.1) months, there were no significant differences between pretransition and post-transition serum alanine and aspartate aminotransferases, direct bilirubin, and weight-for-age z scores. Because of recurrence of cholestasis, 1 patient was restarted on FO after 4 months on SO. CONCLUSIONS: Biochemical reversal of IFALD and growth were preserved after transition from FO to SO in 6 of 7 (86%) patients. Given the challenges associated with the use of FO, SO may be a viable alternative in select patients with home PN.

Tight Glycemic Control With Insulin Does Not Affect Skeletal Muscle Degradation During the Early Postoperative Period Following Pediatric Cardiac Surgery.

OBJECTIVE: Critical illness is associated with significant catabolism, and persistent protein loss correlates with increased morbidity and mortality. Insulin is a potent anticatabolic hormone; high-dose insulin decreases skeletal muscle protein breakdown in critically ill pediatric surgical patients. However, insulin's effect on protein catabolism when given at clinically utilized doses has not been studied. The objective was to evaluate the effect of postoperative tight glycemic control and clinically dosed insulin on skeletal muscle degradation in children after cardiac surgery with cardiopulmonary bypass. DESIGN: Secondary analysis of a two-center, prospective randomized trial comparing tight glycemic control with standard care. Randomization was stratified by study center. PATIENTS: Children 0-36 months who were admitted to the ICU after cardiac surgery requiring cardiopulmonary bypass. INTERVENTIONS: In the tight glycemic control arm, insulin was titrated to maintain blood glucose between 80 and 110 mg/dL. Patients in the control arm received standard care. Skeletal muscle breakdown was quantified by a ratio of urinary 3-methylhistidine to urinary creatinine. MEASUREMENTS AND MAIN RESULTS: A total of 561 patients were included: 281 in the tight glycemic control arm and 280 receiving standard care. There was no difference in 3-methylhistidine to creatinine between groups (tight glycemic control, 249 ± 127 vs standard care, 253 ± 112, mean ± SD in µmol/g; p = 0.72). In analyses restricted to the patients in tight glycemic control arm, higher 3-methylhistidine to creatinine correlated with younger age, as well as lower weight, weight-for-age z score, length, and body surface area (p < 0.005 for each) and lower postoperative day 3 serum creatinine (r = -0.17; p = 0.02). Sex, prealbumin, and albumin were not associated with 3-methylhistidine to creatinine. During urine collection, 245 patients (87%) received insulin. However, any insulin exposure did not impact 3-methylhistidine to creatinine (t test, p = 0.45), and there was no dose-dependent effect of insulin on 3-methylhistidine to creatinine (r = -0.03; p = 0.60). CONCLUSION: Although high-dose insulin has an anabolic effect in experimental conditions, at doses necessary to achieve normoglycemia, insulin appears to have no discernible impact on skeletal muscle degradation in critically ill pediatric cardiac surgical patients.

Consensus-Based Guidelines for Acute Myeloid Leukemia Management in Gulf Cooperation Council Countries: Addressing Unmet Regional Needs and the Changing AML Landscape.

PURPOSE: AML is a heterogeneous hematologic malignancy. Region-specific recommendations for AML management can enhance patient outcomes. This article aimed to develop recommendations for the Gulf Cooperation Council (GCC) countries. METHODS: Ten AML panel members from Kuwait, Oman, Qatar, and the United Arab Emirates (KOQU) participated in a modified two-round Delphi process. The panel first identified the unmet regional needs and finalized a list of core variables. Next, they voted on iterative statements drawn from international recommendations and provided feedback via a questionnaire. Consensus voting ≤70% was discussed, and additional clinical decision making statements were suggested. At round closure, a consensus vote took place on revised statements. RESULTS: The panel reached ≥97.8% consensus on AML management. The panel agreed to use international risk stratification categories for personalized treatment of AML. The presence of ≥10% blasts for recurrent genetic abnormalities was required for a diagnosis of AML. Key consensus was reached for different treatment stages. The panel noted that older patients pose a challenge because of poor cytogenetics and genetic anomalies and require different treatment approaches. The panel recommended venetoclax-hypomethylating agents; fludarabine, cytarabine, idarubicin, and granulocyte colony-stimulating factor; and targeted therapy for AML relapsed/refractory disease. Supportive care is considered on the basis of prevailing organisms and drug resistance. CONCLUSION: The GCC KOQU's consensus-based recommendations for managing AML include an evidence-based and region-specific framework.

Intersecting Realms of Skin and Neurology in Systemic Lupus Erythematosus: A Systematic Review of Dermatological and Neurological Manifestations and Their Impact on Long-Term Prognosis.

Systemic Lupus Erythematosus (SLE) is a complex autoimmune disease characterized by diverse manifestations, notably in dermatological and neurological domains. This review aims to synthesize the current understanding of these manifestations and their impact on long-term prognosis. Adhering to PRISMA guidelines, we conducted a comprehensive search across multiple databases, focusing on studies exploring SLE's dermatological and neurological aspects. Selected studies were analyzed to understand their epidemiology, pathophysiology, clinical presentation, and impact on prognosis. Six pivotal studies were reviewed, highlighting the severity of neuropsychiatric SLE, the progression of skin diseases, and their systemic implications. Notably, studies underscored the role of high disease activity and specific antibodies in the development of neuropsychiatric symptoms and the progression of cutaneous manifestations. The review emphasizes the need for an interdisciplinary approach to managing SLE, considering the interplay between its dermatological and neurological manifestations. It suggests that tailored treatment strategies, early detection, and comprehensive care are crucial for improving patient outcomes. This synthesis provides a foundation for future research to develop integrated care protocols and advance patient care in SLE.

Assessment and Management of Inguinal Hernias in Children.

Inguinal hernia repair is one of the most commonly performed surgical procedures in children. However, a number of controversial issues, such as optimal timing and approach to repair, continue to be debated. This publication reviews the available data pertaining to these controversial issues and identifies best practices in management of inguinal hernias in children.

Use of Image Guidance During Pediatric Venovenous Extracorporeal Membrane Oxygenation Cannulation: A Survey of the American Pediatric Surgical Association.

This study evaluated practices for image guidance during placement of bicaval dual-lumen (BCDL) venovenous extracorporeal membrane oxygenation (VV-ECMO) cannulas in pediatric and adolescent patients and elucidates reasoning behind surgeon practices. A survey covering VV-ECMO practice and opinions was distributed by the American Pediatric Surgical Association (APSA) to all attending members. A total of 110 pediatric surgeons responded (11.3%). During initial BCDL cannula placement, 67.7% of surgeons reported using bimodal imaging with either fluoroscopy (38.4%) or x-ray (29.3%) plus echocardiography. Although 37.4% of surgeons used serial x-rays during cannula placement, only 5.9% believed it was best practice to do so ( P < 0.0001). Rather, 60.4% believed that fluoroscopy was the standard. Among surgeons not using fluoroscopy, 27.6% (13.3% of respondents) reported fluoroscopy added unnecessary complexity or that they preferred another modality. More frequently, reasons for not using fluoroscopy are related to resource limitations. Echocardiography use to confirm cannula position was considered best practice by 92.1% of surgeons, with 86.9% utilization. Therefore, most pediatric surgeons use multimodal imaging during cannulation and consider it best practice. Fluoroscopy is preferred, but its use is frequently limited by hospital resources. Echocardiography is widely available and used. These data represent increased consensus among surgeons and present opportunities for modernization of hospital resources and standards.

Discontinuation of the OriGen® Dual-Lumen Right Atrial Cannula Decreased Venovenous ECMO Usage in Neonates and Older Children: A Survey of the American Pediatric Surgical Association.

INTRODUCTION: Dual-lumen cannulas for venovenous (VV)-ECMO are widely used in pediatric patients. The popular OriGen® dual-lumen right atrial cannula was discontinued in 2019 without a comparable replacement. METHODS: A survey covering VV-ECMO practice and opinions was distributed to attending members of the American Pediatric Surgical Association. RESULTS: 137 pediatric surgeons responded (14%). Prior to discontinuation of the OriGen®, 82.5% offered VV-ECMO to neonates, and 79.6% cannulated with the OriGen®. Following its discontinuation, those that offered only venoarterial (VA)-ECMO to neonates increased to 37.6% from 17.5% (p = 0.0002). An additional 33.8% changed their practice to sometimes use VA-ECMO when VV-ECMO was indicated. Reasons for not incorporating dual-lumen bi-caval cannulation into practice included risk of cardiac injury (51.7%), inexperience with bi-caval cannulation in neonates (36.8%), difficulty with placement (31.0%), and recirculation and/or positioning problems (27.6%). For the pediatric/adolescent population, 95.5% of surgeons offered VV-ECMO prior to OriGen® discontinuation. Few switched to exclusive VA-ECMO (1.9%) when the OriGen® was discontinued, but 17.8% of surgeons began to incorporate selective use of VA-ECMO. CONCLUSION: Discontinuation of the OriGen® cannula drove pediatric surgeons to alter their cannulation practices, dramatically increasing VA-ECMO use for neonatal and pediatric respiratory failure. These data may suggest a need for targeted education accompanying major technological shifts. LEVEL OF EVIDENCE: Level IV.

A 12-Year-Old Girl with Juvenile Granulosa Cell Tumor of the Ovary, Presenting with Adolescent Hyperprolactinemia, Galactorrhea, and Amenorrhea.

BACKGROUND Juvenile-type granulosa cell tumors (JGCTs) are a rare subtype of sex cord stromal tumor with a characteristic histology that is commonly found in the first 3 decades of life. It most commonly presents with symptoms of hyperestrogenism, which may present as precocious pseudopuberty or as menstruation-related symptoms, allowing for early detection of the tumor. CASE REPORT We present the case of a 12-year-old girl who presented to her primary care provider (PCP) with secondary amenorrhea with intermittent abdominal pain, who underwent an ultrasound for further evaluation, which revealed a large incidental pelvic mass. She was admitted to the Emergency Department (ED) and had findings of galactorrhea and hyperprolactinemia on examination. Imaging studies demonstrated a large ovarian mass measuring 15.0×9.0×18.8 cm that was resected, and subsequent pathology results showed JGCT stage 1A. CONCLUSIONS Prognosis of granulosa cell tumors (GCT) largely depends on its initial size, stage at diagnosis, residual tumors after surgery, and the subtype of GCT. If the patient is of reproductive age, fertility-sparing surgical options must be considered and patients must be regularly monitored for recurrence. JGCTs can present with minimal to no symptoms of precocious puberty in young girls but may present with amenorrhea, which may be considered normal for their developmental age. Although JGCTs are rare, they are important to include in differential diagnoses of younger female patients with abdominal pain, especially if accompanied by hormonal irregularities.

What animal model should I use to study necrotizing enterocolitis?

Unfortunately, we are all too familiar with the statement: "Necrotizing enterocolitis remains the leading cause of gastrointestinal surgical emergency in preterm neonates". It's been five decades since the first animal models of necrotizing enterocolitis (NEC) were described. There remains much investigative work to be done on identifying various aspects of NEC, ranging from the underlying mechanisms to treatment modalities. Experimental NEC is mainly focused on a rat, mouse, and piglet models. Our aim is to not only highlight the pros and cons of these three main models, but to also present some of the less-used animal models that have contributed to the body of knowledge about NEC. Choosing an appropriate model is essential to conducting effective research and answering the questions asked. As such, this paper reviews some of the variations that come with each model.

Milky pericardial effusion causing tamponade in a neonate after extracorporeal membrane oxygenation cannulation.

Cardiac tamponade is a known life-threatening complication of extracorporeal membrane oxygenation (ECMO), often secondary to hemopericardium from major vascular or cardiac perforation. We present the unique case of a neonate with a milky pericardial effusion causing tamponade after ECMO cannulation, managed successfully with pericardial window. Understanding ECMO physiology and its effect on the classic presentation of tamponade is critical and can prevent delays in diagnosis. While hemopericardium is most commonly seen in these cases, findings of a non-bloody, milky effusion should prompt further workup for infection, chylopericardium or total parenteral nutrition-associated pericardial effusion, as the appropriate management can mitigate immediate and potential long-term sequelae.

Establishment of a successful robotic pediatric general surgery practice.

Robotic-assisted surgery (RAS) has a variety of theoretical advantages, including tremor filtration, optimal visualization, and improvement of surgeon ergonomics. Though it has achieved wide application in pediatric urology, the majority of pediatric general surgeons do not employ RAS. This study reports our institution's experience with RAS on a pediatric general surgery team. Following IRB approval, a retrospective review of all pediatric patients at our academic children's hospital who underwent RAS between 2017 and 2022 for pediatric general surgical conditions was performed. Patient demographics, operation performed, operating time, complications, and recovery were evaluated. A total of 159 children underwent RAS, increasing from 10 patients in 2017 to 59 patients in 2022. The median age and weight were 15.3 years and 76.4 kg, and 121 (76.1%) were female. The application of RAS was successful in all cases. There were no intraoperative complications and no conversions to an open approach. Eleven patients (6.9%) had unplanned presentations to the emergency department within 30 days. Five of these patients (3.1%) required admission to the hospital. This study demonstrates that the application of RAS in an academic pediatric general surgery practice is feasible and safe. The application of RAS to pediatric general surgery should continue to increase as operative teams increase their experience and comfort.Level of evidence Level IV.

Multiple Perianal Epidermal Cysts Found in a Case of Lowe Syndrome: A Case Report and Review of the Literature.

BACKGROUND Lowe syndrome, also known as oculocerebrorenal syndrome, is a rare genetic condition caused by an X-linked mutation of the OCRL1 gene, with an estimated prevalence in the general population of approximately 1 in 500 000. It is a multisystem disorder most commonly affecting the eyes, central nervous system, and kidneys. These commonly manifest as congenital cataracts, intellectual disability, and proximal renal dysfunction (Fanconi-type). Epidermal lesions are an uncommon manifestation of this condition, and the association is not completely understood. CASE REPORT Here we present a case of a 9-year-old boy with Lowe syndrome who presented with multiple cystic masses found in the perianal region. An excision was then performed to remove the masses and found that the lesions were epidermal cysts, which are infrequently found in Lowe syndrome. After excision, the patient recovered uneventfully without complications. CONCLUSIONS While epidermal cysts are an uncommon manifestation that have been documented, our case remains unique given the location and associated symptoms of the lesions. At presentation, the constellation of pain and perianal masses was concerning for a malignant etiology. However, after diagnostic imaging was performed, these lesions were found to be epidermal cysts, an infrequent manifestation of Lowe syndrome. Few previous case reports described cystic lesions in association with Lowe syndrome, and none, to our knowledge, have described multiple symptomatic perianal lesions. This case is important to consider because epidermal cystic lesions can be found with this presentation and should be considered on differential diagnoses for dermatologic findings in Lowe syndrome patients.