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.

Are pediatric surgery fellowship websites ready for the changing paradigms in the virtual interview era?

Purpose: With the COVID-19 pandemic, in-person fellowship interviews were curtailed, leading candidates to seek information from other resources. Our main purposes were (1) to determine what information recent participants in the match needed to evaluate programs and (2) to assess which of these were available online. Methods: A focus group of ten recent graduates/applicants identified information that was important in choosing a fellowship program. In August 2020 and December 2021, websites belonging to the American Pediatric Surgical Association (APSA) and individual programs were assessed. Results: Recent applicants identified 55 pieces of information considered important to their decision making. Of 57 pediatric surgery fellowships, 98% were listed on APSA's website. Program descriptions on APSA's website listed on average 60% of program information desired by applicants. All listed fellowship director, accreditation status, faculty list, and current fellow(s). Other descriptors frequently noted were alumni (95%), graduate's board performance (83%), ECMO exposure (77%), and curriculum (70%). Information desired but less frequently available were fellow case logs (63%), trauma center designation (53%), burn center designation (40%), research opportunities (30%), candidate interview assistance (25%), and supplemental fellowships (12%). There were 7% of program descriptions that were not updated for at least a year. Conclusions: APSA and individual program websites were complimentary. Websites often lacked data that applicants sought to inform their rank list. To best adapt to the evolving virtual interview paradigm, we suggest reporting key information on a central APSA website with more nuanced information available via links to program specific websites. Supplementary Information: The online version contains supplementary material available at 10.1007/s44186-023-00104-w.

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.

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.

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.

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.

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.

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.

The Current Pediatric Surgery Job Market: A Perspective of Recent Fellowship Graduates.

PURPOSE: This study describes the job market from the perspective of recent pediatric surgery graduates. METHODS: An anonymous survey was circulated to the 137 pediatric surgeons who graduated from fellowships 2019-2021. RESULTS: The survey response rate was 49%. The majority of respondents were women (52%), Caucasian (72%), and had a median student debt burden of $225,000. Considering job opportunities, respondents strongly emphasized camaraderie (93%), mentorship (93%), case mix (85%), geography (67%), faculty reputation (62%), spousal employment (57%), compensation (51%), and call frequency (45%). 30% were satisfied with the employment opportunities available, and 21% felt strongly prepared to negotiate for their first job. All respondents were able to secure a job. Most jobs were university-based (70%) or hospital employed (18%) positions where surgeons covered median of two hospitals. 49% wanted protected research time, and 12% of respondents were able to secure substantial, protected research time. The median compensation for university-based jobs was $12,583 below the median AAMC benchmark for assistant professors for the corresponding year of graduation. CONCLUSION: These data highlight the ongoing need for assessment of the pediatric surgery workforce and for professional societies and training programs to further assist graduating fellows in preparing to negotiate their first job. TYPE OF STUDY: Survey LEVEL OF EVIDENCE: Level V.

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.

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.

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.

Prenatal administration of heparin-binding epidermal growth factor-like growth factor in an experimental model of necrotizing enterocolitis decreased both incidence and severity of the disease.

Background: Necrotizing enterocolitis (NEC) is the leading gastrointestinal cause of death in premature infants and causes long-term disabilities. Previously, enteral heparin-binding epidermal growth factor-like growth factor (HB-EGF) administered after birth demonstrated decreased incidence and severity of NEC in a neonatal animal model of NEC. We investigated the potential prophylactic strategy of preventing NEC using prenatally administered HB-EGF. Methods: An HB-EGF (800 µg/kg/dose) dose was injected into pregnant rats via tail vein or intraperitoneal route 2 hours prior to delivery. After cesarean section (C-section) at 21 days' gestation, the rat pups were subjected to the NEC protocol by inducing stressors: hypoxia, hypothermia, hypertonic feeds, and orogastric gavage of lipopolysaccharide (2 mg/kg). Postnatally, pups were monitored for 96 hours and assessed for the development of clinical and postmortem histological NEC. Results: The experimental NEC incidence in untreated, stressed rat pups was 66%. Compared with untreated pups, the maternal administration of HB-EGF correlated with a significant NEC incidence and severity decrease in rat pups. The strongest decrease was seen when HB-EGF was administered via the intraperitoneal route 2 hours prior to C-section (66% vs 31%, *p<0.05). Prenatal HB-EGF administration significantly increased pups' survival after NEC protocol exposure, with the greatest benefit observed in the group that received HB-EGF intraperitoneally 2 hours before delivery. Conclusions: Prenatal administration of HB-EGF decreases the incidence and severity of NEC, preserves gut barrier function and increases survival. This may represent a novel prophylactic clinical strategy for NEC offered to mothers at risk of delivering a premature infant.

Anatomy and embryology of abdominal wall defects.

Abdominal wall defects are one of the most frequently encountered human congenital anomalies. They are seen in as many as 1 in 2,000 live births with evidence to suggest that their incidence is increasing. While often discussed together abdominal wall defects consist mainly of two entities namely gastroschisis and omphalocele. There are marked differences in their theories of embryo-pathogenesis, clinical presentation/anatomy and overall outcomes. There is no clear consensus explaining the precise embryological mechanisms leading to the development of abdominal wall defects. Many clinicians and embryologists have attempted to explain the genesis of congenital abdominal wall defects because of failure of progression of various phases of normal embryonic development. This review summarizes the mechanisms involved in normal and abnormal development of the ventral abdominal wall leading to the development of gastroschisis and omphalocele.

Objective Personality Determination: A Useful Addition to the Traditional Process of Pediatric Surgical Fellow Selection.

BACKGROUND: Selection of pediatric surgical trainees is a multifaceted process heavily influenced by in-person interviews to determine personality makeup and institutional compatibility. We present our experience in utilizing a validated personality inventory in the selection of our candidates. METHODS: All applicants selected for an in-person interview for the 2020 Pediatric Surgery Match were offered the Big Five Inventory (BFI) questionnaire. An initial rank order list (ROL) was formulated employing application information and in-person interview score. A reformulated ROL was created after incorporating BFI data. Correlation of specific personality characteristics vs position on the ROL was assessed for both the initial ROL and the reformulated ROL. RESULTS: Thirty-four applicants were interviewed and 24 (70.6%) were ranked. Linear regression analysis identified a lower neuroticism score directly related to a higher position on the initial ROL {R = .4626, P = .023}. The reformulated ROL identified a higher conscientiousness score {R = .5331; P = .007} and a lower neuroticism score {R = -.4383; P = .032} correlated with ascending the final ROL. CONCLUSIONS: The self-administered BFI adds objectivity to personality determination gleamed from the standard face-to-face interview. Conscientiousness and neuroticism had a significant impact on the final ROL position. Adding objective personality data appears to provide additional aid in this difficult process.

To Wrap or Not? Utility of Anti-reflux Procedure in Infants Needing Gastrostomy Tubes.

Purpose: Gastrostomy tube (GT) insertion is commonly performed in children with failure to thrive. Pediatric patients' frequently have gastroesophageal reflux (GER) and discerning pathological GER can be challenging. Moreover, there is some evidence that GT insertion may lead to worsening GER and to avoid a subsequent anti-reflux procedure (ARP), though controversial some surgeons advocate considering an ARP concomitantly. The purpose of this report is to assess outcomes in infants who underwent a GT vs. GT with ARP. Methods: Retrospective review of all infants who had a GT placed at a single institution from 2009-2014. The patients were then divided into two cohorts based on the index operation i.e., GT vs GT with ARP and outcomes compared. Results: 226 operations (104 GT, 122 GT with ARP) were performed. The cohorts were similar in gender, gestational age, race, weight, median age, LOS, and proportion of neurologically impaired patients. Preoperative GER was significantly higher in the GT with ARP cohort (91 vs. 18%). No difference in the rate of immediate complications was noted between the two groups. Postoperative increase in anti-reflux medications was significantly higher in the GT cohort (p = 0.01). Post-op GER needing a secondary procedure (ARP or GJ tube) was noted in 21/104 (20%) patients. Those needing an additional procedure vs. those with GT alone were similar in the proportion of patients with pre-op GER, neurologic impairment, type of feeds, and age. Conclusion: Identifying patients who would benefit from a concomitant ARP remains challenging. A fifth of GT patients needed a subsequent procedure despite most high-risk patients having already undergone an ARP. Since the overall rate of complications remained similar, initial GT approach can be considered reasonable.

The impact of regionality and hospital status on mortality associated with non-accidental trauma.

INTRODUCTION: Non-accidental trauma (NAT) affects 2 per 100,000 children annually in the US and may go unrecognized. The aim of this study to quantify the burden of NAT and to evaluate regional variations in mortality. METHODS: The Kids Inpatient Database (2000-2012) was queried for pediatric patients presenting with a diagnosis of NAT. Data was obtained on demographic, clinical and hospital-level characteristics. Primary outcome measure was mortality. Multivariable logistic regression models for age, sex, race/ethnicity, insurance status, income quartile, hospital volume, region (Northeast, South, West and Midwest), teaching status, and injury severity scores. RESULTS: NAT represented 1.92% (n = 15,999) of all trauma patients. Mortality rates were 3.98% for patients presenting with NAT. African American children had a higher likelihood of mortality compared to White children (OR[95%CI]:1.35[1.03-1.79]), however, this effect was not statistically significant for patients being treated at designated children's hospitals (OR[95%CI]:1.23(0.78-1.95) and urban facilities (OR[95%CI]:1.30[0.99-1.72]). Statistically significant regional variations in mortality, lost significance for patients treated at designated children's hospitals (p > 0.05). CONCLUSION: NAT has devastating consequences and is associated with a high mortality rate. Treatment at designated children's hospitals results in the loss of variation in mortality, resulting in diminished disparities and improved outcomes. These findings align with current trends towards the "regionalization of pediatric health care" and reflects the value of regional transfer centers that are.

Thoracoscopic segmentectomy for a large previously undiagnosed CPAM presenting as a spontaneous pneumothorax: A case report.

INTRODUCTION: Incidentally found congenital pulmonary airway malformations (CPAM) in older children are extremely rare and have traditionally been managed with minimally invasive versus open lobectomy of the affected lobe. PRESENTATION OF CASE: In this report, we present a 11-year-old male who presented with a recurrent spontaneous pneumothorax and was found to have a large symptomatic CPAM confined to a single segment of the right lower lobe. The patient was successfully treated with thoracoscopic segmentectomy without any residual disease seen on follow up imaging. DISCUSSION: Minimally invasive thoracoscopic approach has many advantages over open approach including better pain control, reduced hospital length of stay, and decreased intraoperative blood loss. With increasing use of minimally invasive approaches, lung-sparing surgery has demonstrated to be a viable and an attractive option for definitive resection of CPAM, without compromising resection margins and/or future lung function. CONCLUSION: This report demonstrates that minimally invasive lung-sparing surgical treatment of a large CPAM is feasible in older children.

The Utility of Gallbladder Absence on Ultrasound for Children With Biliary Atresia.

Background: Biliary Atresia (BA) is congenital condition, where infant intra- and extrahepatic bile ducts become obliterated, leading to cholestasis, and cirrhosis if untreated. This study aims to assess the predictive measure of absent gallbladder on ultrasounds (US) performed in infants with cholestasis for diagnosing BA. Method: After Institutional Review Board approval, retrospective chart reviews of 61 infants with cholestasis found 43 (70.5%) were diagnosed with BA. A pediatric radiologist provided interpretations of all ultrasounds in a blinded fashion. Statistical analysis was used to assess the utility of absence of gallbladder on US in predicting BA, confirmed intraoperatively. Results: Absent gallbladder on US predicts absent gallbladder with 77% accuracy, 92% sensitivity, 73% specificity, PPV 43%, and NPV 97% (P < 0.001, Fisher exact test). To diagnose BA, absent gallbladder on US has 66% accuracy, 53% sensitivity, 94% specificity, 96% PPV, and 46% NPV (P < 0.001, Fisher exact test). Conclusion: Sonographic gallbladder absence has high specificity and PPV, indicating utility for BA diagnosis; however, it is not useful for ruling out BA given its low sensitivity.