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Abdominal surgical emergencies are relatively common in neonates. Some of them are related to congenital diseases such as intestinal atresia and intestinal malrotation, whereas some are entirely postnatal conditions such as necrotizing enterocolitis and gastric perforation. While there is a wide range of clinical severity for these conditions, outcomes are most favorable with prompt identification and expeditious treatment. In this review, we describe the most common neonatal abdominal surgical emergencies, highlight the signs that can help with early detection, and explain the approach to diagnosis and management.

Thoracoscopic bilateral T3 sympathectomy for primary focal palmar hyperhidrosis in children has excellent short-term outcomes. However, data in the literature, on the long-term outcomes of the operation are scarce.We conducted a retrospective institutional review of all children and adolescents undergoing T3 bilateral thoracoscopic sympathectomy for primary focal palmar hyperhidrosis between June 2013 and October 2020. We composed a quality of life (QoL) questionnaire evaluating the patient's perception of how much the hyperhidrosis affected their daily life in multiple domains. The questionnaire was completed before the operation and at every postoperative follow-up visit.We operated on 58 patients with a median age of 15 (6-25) years. There were no intraoperative or postoperative complications, and all patients had immediate complete postoperative resolution of their palmar hyperhidrosis. Fifty-three patients (91.4%) had long-term follow-up data available with a median of 2.5 (range 0.1-7.5) years. Two patients (3.4%) experienced recurrence of their palmar hyperhidrosis. Nine patients (15.5%) experienced compensatory hyperhidrosis and required occasional medical management with oral anticholinergics. Two patients reported regretting having undergone the operation. Overall, the mean QoL score improved remarkably, from 42/100 before the operation to 92/100 at 1 month, 89/100 at six months to a year, 97/100 between two and four years, and 80/100 ≥ 5 years after the operation.Thoracoscopic bilateral T3 sympathectomy has a high success rate for primary palmar hyperhidrosis in children in the mid- to long-term. Compensatory sweating and recurrence can occur years after the operation, so long-term follow up is mandatory.IV.

Introduction Lung biopsy is infrequently utilized in the population of infants with severe bronchopulmonary dysplasia (BPD). Yet, its presentation may overlap with other infant diffuse lung diseases, including those within the spectrum of childhood interstitial lung diseases (chILD). Lung biopsy might differentiate between these entities, or discern those with an extremely poor prognosis. Both might alter the clinical management of a subset of infants diagnosed with BPD. Methods In this tertiary referral center, we drew on a retrospective cohort of 306 preterm infants with severe BPD. Of these, nine underwent lung biopsy between 2012 and 2017. Our aim was to assess the indication of lung biopsy, the prior clinical history, safety of the procedure, and describe the biopsy findings. Finally, we considered management decisions in relation to the biopsy results in these patients. Results The average gestational age and birth weight of the 9 patients were 30±3 (range 27-34) weeks and 1421±571 (range 611-2140) grams. All infants had serial echocardiograms to assess for pulmonary hypertension, genetic testing, and computed tomography angiography (CTA) prior to biopsy. All patients showed moderate to severe alveolar simplification and 8 had some degree of pulmonary interstitial glycogenosis (PIG) ranging from focal to diffuse. Following biopsy, 2 infants with findings of PIG received high dose systemic steroids and 2 separate infants had care redirected. Conclusion In our cohort, lung biopsy was safe and well tolerated. Findings from lung biopsy may aid decision making in selected patients when used as a part of a step-wise diagnostic algorithm.

Central conducting lymphatic anomaly (CCLA) due to congenital maldevelopment of the lymphatics can result in debilitating and life-threatening disease with limited treatment options. We identified 4 individuals with CCLA, lymphedema, and microcystic lymphatic malformation due to pathogenic, mosaic variants in KRAS. To determine the functional impact of these variants and identify a targeted therapy for these individuals, we used primary human dermal lymphatic endothelial cells (HDLECs) and zebrafish larvae to model the lymphatic dysplasia. Expression of the p.Gly12Asp and p.Gly13Asp variants in HDLECs in a 2‑dimensional (2D) model and 3D organoid model led to increased ERK phosphorylation, demonstrating these variants activate the RAS/MAPK pathway. Expression of activating KRAS variants in the venous and lymphatic endothelium in zebrafish resulted in lymphatic dysplasia and edema similar to the individuals in the study. Treatment with MEK inhibition significantly reduced the phenotypes in both the organoid and the zebrafish model systems. In conclusion, we present the molecular characterization of the observed lymphatic anomalies due to pathogenic, somatic, activating KRAS variants in humans. Our preclinical studies suggest that MEK inhibition should be studied in future clinical trials for CCLA due to activating KRAS pathogenic variants.

Mesenteric lymphatic malformations result from abnormal proliferation of disorganized mesenteric lymphatic channels. Sclerotherapy is often preferred over surgery as it is less invasive and has lower post-procedure morbidity. Sclerotherapy has been described as durable and effective with a low complication rate. We describe a serious complication from sclerotherapy of a lymphatic malformation extending from the mesentery through the bowel wall, highlighting the spectrum of this pathology and the need for multidisciplinary management of complex cases.

To compare the outcomes and hospital charges of patients who underwent laparoscopic appendectomy for non-perforated appendicitis and were discharged home either shortly after the operation after being admitted for overnight observation. Postoperative (30-day) emergency department (ED) visits, hospital readmissions, and reoperations were compared between patients who were discharged shortly after surgery (same-day discharge [SDD] group) and patients who were discharged after spending one night in the hospital (overnight observation group). Study period: July/2015 to June/2019. Patients with perforated appendicitis and/or who spent > 1 night in the hospital were excluded from the study. We did 1957 laparoscopic appendectomies within the 4-year study period. After excluding all non-eligible cases, 930 patients were included in the overnight observation group, and 511 in the SDD group. Mean age and mean operative time were similar in both groups: 11.5 (SD 3.6)/11.8 (SD 3.5) years, and 35 (SD 13)/33 (SD 12) minutes, respectively. There were 24 (2.6%) ED visits within the overnight observation group. Sixteen patients (1.7%) were discharged from the ED, and 8 (0.9%) required a re-admission. There were 11 (2.1%; P = 0.61) ED visits within the SDD group. Six patients (1.1%; P = 0.41) were discharged from the ED, and 5 (1%; P = 0.82) required a readmission. Six of the 11 ED visits within the SDD group occurred on the 5th postoperative day or later, whereas five (1%) occurred within the first 3 days post appendectomy. These five patients would have likely benefited from an overnight admission and were erroneously discharged on the same day of the appendectomy. There were no reoperations in the overnight observation group, but there were 3 reoperations in the SDD group (0.6%, P = 0.01). The reasons for the reoperations (two bowel obstructions and one bowel perforation) were in no way related to the time of the original discharge. The mean hospital charges per patient in the SDD group and the overnight observation group were significantly different: $32,450 and $35,420, respectively (> 9% margin, P

Placement of ventriculoperitoneal shunts (VPS) can be challenging in children with previous VPS, previous abdominal surgery, or complex abdominal pathology. Laparoscopy can help identify intra-abdominal adhesions and determine the optimal site for insertion of the distal VPS catheter. We analyzed the feasibility and safety of laparoscopy assisted VPS placement (lapVPS) in children.A retrospective review from January/2015 to December/2018 was performed. Laparoscopy was performed via a 5 mm trans-umbilical port. Once the optimal entry site was identified, the distal end of the VPS was inserted by Seldinger technique under direct laparoscopic guidance.One hundred ten lapVPS procedures were reviewed. Median age was 1 (IQR 0.3-9.37) year. Fifty-five (50%) patients were1 year, and 15 (13.6%) were ≤28 days old. The mean age of the neonates was 14.7 (SD 7.6, range 4-28) days, and the mean weight was 3 (SD 0.39, range 2.4-3.7) kg. Thirty-one (28.2%) lapVPS were initial VPS placements, whereas 79 (71.8%) were subsequent VPS placements. Thirty-nine (35.5%) patients had a history of abdominal surgery or complex abdominal pathology. Median operative time was 36 (IQR 28-56) minutes. One hundred seven (97.3%) patients underwent successful lapVPS. Two (1.8%) patients underwent diagnostic laparoscopy, and lapVPS was deemed infeasible due to intraabdominal adhesions. One (0.9%) patient had an intestinal perforation from trocar placement. Three patients developed 5 postoperative complications that required a reoperation.Laparoscopy is a valuable tool to assess the suitability of the peritoneal cavity to accommodate a VPS. LapVPS is safe and can be performed in children of all ages.Level IV.

Background Children with suspected appendicitis generally undergo an ultrasound (most commonly) or a CT scan (rarely) as the first imaging study. At our hospital, patients with non-diagnostic ultrasound or CT scan results undergo a non-contrast (unenhanced), non-sedated MRI. We aimed to determine the accuracy of this study for this purpose in a large cohort of children. Methods A retrospective review of all unenhanced, non-sedated MRIs done for suspected appendicitis was performed from January 2014 to December 2018. MRI reports were correlated with pathology reports in cases that underwent appendectomy, and with clinical outcomes if no operation was done (clinical follow up: 30d). No patient was treated for appendicitis non-operatively. Results Three hundred fifty unenhanced, non-sedated MRIs were done and reviewed with median age: 12 (3 to 18) years. Sixty-five (18.6%) MRIs were positive for appendicitis, and 62 of those underwent appendectomy (3 excluded clinically). Pathology was positive in 59/62 cases. 256 (73.1%) MRIs were negative for appendicitis. Six cases underwent appendectomy (persistent symptoms). Pathology was positive in 2/6 cases. The overall diagnostic accuracy was: sensitivity 96.7% (95% CI: 88.6–99.6), specificity 97.7% (95% CI: 95.0–99.1), PPV: 90.8% (95% CI: 81.6–95.6; false positives 6/65), and NPV: 99.2% (95% CI: 97.0–99.8; false negatives 2/254). Twenty-nine (8.3%) MRIs were non-diagnostic. None of those 29 cases had appendicitis (4 negative pathology, 25 excluded clinically). Conclusions The unenhanced, non-sedated MRI is highly accurate for the diagnosis of appendicitis in children. It should be considered as an alternative to CT in the work-up of patients with suspected appendicitis to eliminate the risks associated with ionizing radiation. Level of Evidence Level IV.

Background Thoracic duct (TD) outflow obstruction causes high morbidity and mortality in newborns. It can be congenital/idiopathic or acquired (secondary to central venous thrombosis or injury during cardiothoracic surgery). Re-routing the TD to the venous system by microsurgical techniques to restore lymphatic flow is a potential surgical solution. We present a series of newborns and infants who underwent thoracic duct-to-vein anastomosis (TDVA) to restore TD outflow. Materials and methods A retrospective review of all TDVA September 2015–March 2019 was performed. All patients underwent extensive pre-operative imaging evaluation by dynamic MRI and fluoroscopic lymphangiography. The TDVAs were done under high-power microscopy. Results Eight patients underwent TDVA. Age at surgery was 1 to 9 months. Four patients had a history of cardiac surgery (one with complete thrombosis of the central venous system), one patient had a history of ECMO and thrombosis of the SVC, and three patients had a history of fetal hydrothorax and non-immune hydrops. Six patients had a successful TDVA with restoration of the lymphatic flow through the TD and clinical improvement. Two patients had a technically adequate TDVA but without improvement of the flow due to persistently high central venous pressure. Five patients remain alive, two patients died from complications of the lymphatic disorder, and one patient died from an unrelated cause. Conclusions Patients with congenital or acquired TD outlet obstruction for whom no improvement is achieved by non-surgical interventions may benefit from TDVA. A thorough understanding of the anatomy and physiology of each patient is critical for the success of the operation. Level of Evidence Level IV.

OBJECTIVES: Protein-losing enteropathy (PLE) is a disorder of intestinal lymphatic flow resulting in leakage of protein-rich lymph into the gut lumen. Our primary aim was to report the imaging findings of dynamic contrast magnetic resonance lymphangiography (DCMRL) in patients with PLE. Our secondary objective was to use these imaging findings to characterize lymphatic phenotypes. METHODS: Single-center retrospective cohort study of patients with PLE unrelated to single-ventricle circulation who underwent DCMRL. We report imaging findings of intranodal (IN), intrahepatic (IH), and intramesenteric (IM) access points for DCMRL. RESULTS: Nineteen patients 0.3–58 years of age (median 1.2 years) underwent 29 DCMRL studies. Primary intestinal lymphangiectasia (PIL) was the most common referring diagnosis (42%). Other etiologies included constrictive pericarditis, thoracic insufficiency syndrome, and genetic disorders. IN-DCMRL demonstrated a normal central lymphatic system in all patients with an intact thoracic duct and localized duodenal leak in one patient (1/19, 5%). IH-DCMRL detected a duodenal leak in 12/17 (71%), and IM-DCMRL detected duodenal leak in 5/6 (83%). Independent of etiology, lymphatic leak was only visualized in the duodenum. CONCLUSIONS: In patients with PLE, imaging via DCMRL reveals that leak is localized to the duodenum regardless of etiology. Comprehensive imaging evaluation with three access points can provide detailed information about the site of duodenal leak.

To compare the outcomes and hospital charges of patients who underwent laparoscopic appendectomy for non-perforated appendicitis and were discharged home either shortly after the operation after being admitted for overnight observation.Postoperative (30-day) emergency department (ED) visits, hospital readmissions, and reoperations were compared between patients who were discharged shortly after surgery (same-day discharge [SDD] group) and patients who were discharged after spending one night in the hospital (overnight observation group).July/2015 to June/2019. Patients with perforated appendicitis and/or who spent 1 night in the hospital were excluded from the study.We did 1957 laparoscopic appendectomies within the 4-year study period. After excluding all non-eligible cases, 930 patients were included in the overnight observation group, and 511 in the SDD group. Mean age and mean operative time were similar in both groups: 11.5 (SD 3.6)/11.8 (SD 3.5) years, and 35 (SD 13)/33 (SD 12) minutes, respectively. There were 24 (2.6%) ED visits within the overnight observation group. Sixteen patients (1.7%) were discharged from the ED, and 8 (0.9%) required a re-admission. There were 11 (2.1%; P = 0.61) ED visits within the SDD group. Six patients (1.1%; P = 0.41) were discharged from the ED, and 5 (1%; P = 0.82) required a readmission. Six of the 11 ED visits within the SDD group occurred on the 5th postoperative day or later, whereas five (1%) occurred within the first 3 days post appendectomy. These five patients would have likely benefited from an overnight admission and were erroneously discharged on the same day of the appendectomy. There were no reoperations in the overnight observation group, but there were 3 reoperations in the SDD group (0.6%, P = 0.01). The reasons for the reoperations (two bowel obstructions and one bowel perforation) were in no way related to the time of the original discharge. The mean hospital charges per patient in the SDD group and the overnight observation group were significantly different: $32,450 and $35,420, respectively ( 9% margin, P 0.01).Healthy children who undergo laparoscopic appendectomy for non-perforated appendicitis can be discharged home during the same day of the operation after a short period of observation. This approach is safe and does not result in more postoperative ED visits or hospital readmissions. In addition, there is a significant financial benefit when patients are discharged early.Level III-retrospective comparative treatment study.

Purpose To report the long-term core outcome set of patients with simple gastroschisis. Methods This was a retrospective chart review of all patients with simple gastroschisis managed at our hospital between August 2008 and July 2016. We collected all data included in the core outcome set developed for the standardization of gastroschisis outcomes reporting. We conducted a phone survey of the patients' parents using the PedsQL™ Pediatric Quality of Life Inventory, Cognitive Functioning Scale, and Gastrointestinal Symptoms Scale (GSS). Additionally, parents reported their subjective evaluation of the patients' cosmetic result and overall quality of life. Results There were 124 patients included in the study. The majority (76.5%) was born prematurely at a median gestational age of 36 (range 27.6–38) weeks. At neonatal discharge (median 36 days [18–150] days) most patients were below the 10th percentile for height (81.4%) and weight (87%). Their growth, however, normalized during early childhood. Seven patients (5.6%) required at some point an operation for acute abdominal complications. One-third of patients required long-term treatment for constipation and one-third of patients required long-term treatment for gastroesophageal reflux disease (GERD). Thirty-five parents participated in the phone survey. Mean parent-reported quality of life score was better than healthy controls (87.5% vs. 82.3%, p = 0.049). Cognitive functions and gastrointestinal symptoms scores were similar to healthy controls. All patients are alive. Conclusion Growth restriction in patients with simple gastroschisis is common at birth and during the neonatal period, but it improves during the first three years of life. Abdominal operations are rarely needed in patients with simple gastroschisis. GERD and constipation, on the other hand, are common and often require long-term medical management. The overall parent-reported quality of life of patients with simple gastroschisis is excellent. Level of evidence Level II.

The perfect balance between safety, cosmesis, and cost effectiveness in a world with ever growing healthcare costs has yet to be found for nonperforated appendicitis. The aim is to present our data regarding safety and cost effectiveness of the transumbilical extracorporeal laparoscopic-assisted appendectomy technique.A retrospective review was performed for all laparoscopic appendectomies for acute appendicitis from October 2014 to October 2016. All cases of perforated appendicitis were excluded (visible hole/abscess/free pus). Included cases were divided into two groups by operative technique: transumbilical (TU) or laparoscopic 3-port (L3P). Operating room charges were billed in 30-min intervals, and hospital charges billed per night in-house. The technique was that the appendix is identified with the laparoscope, grabbed with a grasper that is inserted parallel to the laparoscope, and exteriorized through the umbilicus. The appendectomy is completed extracorporeally.A total of 494 cases of nonperforated appendicitis were included in the study. One surgeon attempted all cases with the TU technique (n=161), and all other surgeons used the L3P technique (n=333), which required an endostapler and a vascular sealing device. The TU technique was successful in 99 of the attempted cases. The mean operative time of the TU cases and the L3P cases was 21 (8-43) and 37 (12-73) min, respectively (P0.001). The mean hospital stay for the TU and the L3P cases was 1.6 (1-5) days (one-night admission) and 2.4 (1-14) days (two-night admission), respectively (P0.001). There were no operative complications or readmissions in either group. The overall cost of the L3P cases was 30% higher than the cost of the TU cases.The transumbilical extracorporeal laparoscopic-assisted technique was as safe as the laparoscopic 3-port technique. It offered all the advantages of a minimally invasive procedure, was associated with a significantly shorter hospital stay, and was remarkably more cost effective than the standard laparoscopic 3-port technique.III.

Background Only a few isolated cases in the literature exist to guide management of bilateral congenital lobar emphysema (CLE). Here, we review our experience in infants with bilateral CLE. Methods A case series of all infants presenting with bilateral CLE from 2014 to 2015 in a single institution. Results Four patients underwent intervention, with all having right middle lobe (RML) and left upper lobe (LUL) affected. Preoperative planning with computed tomography angiography (CTA) chest allowed a tailored approach based on specific radiologic features. All patients also underwent bronchoscopy to evaluate the anatomy and to assess for alternative causes of airway compression. Three patients underwent unilateral lobectomies, two RML and one LUL. All are growing normally and on room air more than one year later. The last patient underwent a staged procedure beginning with left upper lobectomy followed by right middle lobectomy two weeks later after exhibiting rebound hyperexpansion of the remaining diseased lobe. Thoracoscopy was precluded by mass effect in all patients. No patients underwent emergent lobectomies. One patient had pulmonary interstitial glycogenosis (PIG) in the setting of CLE, first reported case of bilateral CLE with PIG. Conclusions This study supports a staged, image-guided, physiology-based operative approach to bilateral CLE. Excision of both diseased lobes does not appear to be mandatory, at least in the short-term follow up, and comports with a "the least intervention that is the most effective" philosophy. CTA is critical for planning, but the role of V/Q scan is not defined. Thoracoscopy appears to have no role. Level of evidence Treatment Study, Level IV.

Pulmonary lymphatic flow disorders involve the abnormal lymphatic flow via lymphatic channels to the lungs and pleural space. Plastic bronchitis and chylothorax are the main complications of this abnormal lymphatic perfusion, which has been termed pulmonary lymphatic perfusion syndrome (PLPS). Following lymphatic access, dynamic contrast MR lymphangiography is the imaging modality of choice to diagnose these disorders. Management includes medical therapy, percutaneous interventions under fluoroscopy, and surgical interventions.

Aim of the Study Pediatric surgery trainees at our institution perform 15 to 20 supervised laparoscopic pyloromyotomies during their junior year, and are allowed to perform the operation independently without supervision during their senior year. We reviewed the outcomes of laparoscopic pyloromyotomies performed by senior trainees operating without supervision and compared them to experienced pediatric surgeons. Method We did a retrospective reviewed of all unsupervised laparoscopic pyloromyotomies (n = 90) performed by the last 12 pediatric surgery fellows (2012–2018) during their senior year, and the most recent 90 consecutive laparoscopic pyloromyotomies performed by 9 experienced pediatric surgeons. Statistical significance was determined by T-test and Fisher's exact test. Data is expressed as mean (SD) or median (range). A p value of ≤ 0.05 was considered significant. Results Mean age at surgery was 4.7 (SD: 1.6) and 5 (SD: 2.3) weeks in the trainees and surgeons group, respectively (p = 0.38). Mean operative time was 28 (SD: 13) minutes in the trainees group vs. 25 (SD: 10) minutes in the surgeons group (p = 0.09). Intraoperative complications occurred in 3 of 90 (2.7%) cases in the trainees group (three mucosal perforations, all detected during the operation, one repaired laparoscopically, two repaired open), and none in the surgeons group (p = 0.11). One postoperative complication requiring reoperation occurred in the trainees group (omentum eviscerated through an incision site), while none occurred in the surgeons group (p = 0.36). No incomplete pyloromyotomies occurred in either group. The median length of postoperative hospital stay was 1 (1 to 10) and 1 (1 to 6) days in the trainees and surgeons group, respectively (p = 0.63). Conclusion Senior trainees at high-volume training programs can perform unsupervised laparoscopic pyloromyotomies safely as a mean to promote surgical autonomy without compromising patient outcomes. Level of Evidence III

Nevus comedonicus syndrome (NCS) is a rare epidermal nevus syndrome characterized by ocular, skeletal, and central nervous system anomalies. We present a 23-month-old boy with a history of a congenital pulmonary airway malformation (CPAM) of the lung and a congenital cataract who developed progressive linear and curvilinear plaques of dilated follicular openings with keratin plugs (comedones) on parts of his scalp, face, and body consistent with nevus comedonicus. MRI of the brain demonstrated an aneurysm of the right internal carotid artery. Genetic testing identified NEK9 c.1755_1757del (p.Thr586del) at mean allele frequency of 28% in the nevus comedonicus. This same mutation was present in the CPAM tissue. This is the first case of a CPAM in a patient with an epidermal nevus syndrome. This case expands the phenotype of nevus comedonicus syndrome to include CPAM and vascular anomalies.

Introduction: Experts of pediatric endosurgery recommend early thoracoscopic resection of congenital pulmonary airway malformations (CPAM) even in asymptomatic infants due to the risk of l...

Primary focal hyperhidrosis (PH) is a condition in which the sweat glands of certain areas of the body produce more sweat than what would be expected at any given circumstance for adequate thermoregulation. The condition generally affects the soles, the axillae, and the palms, bringing deleterious consequences from psychological, functional, and social perspectives. The actual prevalence of the condition is unknown, mostly due to underreporting, but it has been estimated to be between 2 and 4% in the general Caucasian population. The etiology of PH is largely unknown, although the frequently positive family history suggests a genetic component [1, 2]. Why it affects only certain areas of the body and why each patient has a different combination of areas affected remain a complete mystery. PH affects males and females, tends to begin in early childhood, and is more prevalent in the Asian population than in the Caucasian population. The excessive sweating occurs regardless of the temperature of the environment, tends to worsen in stressful situations, and in general does not occur during sleep.

Pulmonary hypoplasia and hypertension is a leading cause of morbidity and mortality in congenital diaphragmatic hernia (CDH). The etiologic insult occurs early in gestation highlighting the potential of prenatal interventions. We evaluated prenatal pharmacologic therapies in the nitrofen CDH model. Olive oil or nitrofen were administered alone or with dexamethasone (DM), sildenafil, or DM+sildenafil to pregnant rats. Newborn pups were assessed for lung function, structure and pulmonary artery (PA) flow and resistance. Prenatal DM treatment of CDH pups increased alveolar volume density (Vva), decreased interalveloar septal thickness, increased tidal volumes and improved ventilation without improving oxygenation or PA resistance. Sildenafil decreased PA resistance and improved oxygenation without improving ventilation or resulting in significant histologic changes. DM+sildenafil decreased PA resistance, improved oxygenation and ventilation while increasing Vva and decreasing interalveolar septal and pulmonary arteriole medial wall thickness. Lung and body weights were decreased in pups treated with DM and/or sildenafil. Prenatal DM or sildenafil treatment increased pulmonary compliance and decreased pulmonary vascular resistance respectively, and was associated with improved neonatal gas exchange but had a detrimental effect on lung and fetal growth. This study highlights the potential of individual and combined prenatal pharmacologic therapies for CDH management.

Present the outcomes of patients younger than 2years who underwent laparoscopic fundoplication, highlighting the failure rate and need for redo fundoplication.Retrospective review of patients2years who underwent laparoscopic fundoplication between January 2009 and December 2014.458 infants younger than 2years underwent laparoscopic fundoplication in the 6-year period (360 Nissen, 77 Toupet and 21 Thal fundoplications). Median age at surgery was 5 (1-23) months. Median follow-up was 3 (1-6) years. The conversion rate was 0.87% (4 of 458 cases). Patients did not undergo routine studies to assess the incidence of postoperative GER but were instead followed clinically. Failure of the fundoplication was determined when a patient was unable to gain weight and/or protect the airway while receiving gastric feedings because of GER. The failure rate in our experience was 2.6% (12 redo out of 458 cases [11/360 Nissen, 1/77 Toupet and 0/21 Thal]). All failed cases occurred because of migration of the fundoplication, confirmed preoperatively by a contrast study. Median time between the initial fundoplication and the redo was 13 (5-27) months. There were no failures within a contemporaneous group of 101 patients2years who underwent open fundoplication.The need for a redo fundoplication after a laparoscopic fundoplication was an uncommon event in our experience (12 of 458 cases). Our results contrast with published studies that report higher failure rates. Case volume per surgeon may explain in part the dissimilar results among studies.III.

To evaluate the incidence of respiratory failure requiring ECMO in newborns with gastroschisis (GC), compare it to the incidence in the general population, review the surgical outcomes of newborns with GC requiring ECMO and compare them to newborns with GC not requiring ECMO. This is a retrospective review of all neonatal admissions for GC from December 2010 to September 2015. 110 newborns with GC were admitted to our NICU between 12/2010 and 9/2015; 36 were term. Four cases, all term, all prenatally diagnosed, all outborn, developed respiratory failure requiring ECMO secondary to meconium aspiration syndrome (MAS). This 11% (4/36 term GC) represents a 300-fold increase in the incidence of MAS-associated respiratory failure requiring ECMO compared to the general population of term newborns (0.037%). Median time on ECMO was 12 (9–20) days. The time to achieve full enteral feedings in the GC/ECMO group was twice the time of the 106 newborns in the GC/non-ECMO group [median: 70 (48–77) vs. 35 (16–270) days, respectively]. Time to hospital discharge was three times longer in the GC/ECMO group compared to the GC/non-ECMO group (median: 42 [20–282] versus 125 [69–223] days, respectively). All patients survived. The incidence of respiratory failure requiring ECMO is remarkably higher in patients with GC than in the general population and much higher in the subgroup of term GC. While infrequent, the possibility of this event supports the concept that fetuses with GC benefit from being delivered at tertiary centers with immediate pediatric surgery and ECMO capabilities.

Aim of the study Pediatric surgery trainees at our program are allowed to perform unsupervised laparoscopic appendectomies during their last year of training to promote independent operative decision-making skills. We reviewed the outcomes of laparoscopic appendectomies done by senior trainees without supervision and compared them to experienced pediatric surgeons. Methods We reviewed 500 laparoscopic appendectomies performed without supervision by the last 10 pediatric surgery trainees during their last year of training (first 50 cases of each trainee). We compared the outcomes of those 500 cases to the outcomes of 200 laparoscopic appendectomies performed by eight experienced pediatric surgeons (last 25 cases of each surgeon). Data are expressed as mean (SD), unless otherwise indicated. A P value of ≤ 0.05 was regarded as significant. Main results Median age in the “trainees” and “surgeons” groups was 11 (range 2–22) and 12 (2–20) years, respectively (P = 0.35). The proportion of perforated appendicitis was similar: 98/500 (19.6%) in the trainees group and 42/200 (21%) in the surgeons group, respectively (P = 0.75). Mean operative time was 41 (SD 14.5) min in the trainees group vs. 39 (SD 16.1) min in the surgeons group (P = 0.05). Minor intraoperative complications occurred in 3/500 (0.6%) cases in the trainees group vs. 1/200 (0.5%) in the surgeons group (P = 0.69). No major complications occurred in either group. Mean hospital stay was 2 (range 0.5–26) and 2.3 (range 0.5–18) days in the trainees and surgeons groups, respectively (P = 0.25). There were 13/500 vs. 5/200 readmissions (P = 0.92), and 1/500 vs. 1/200 reoperations in the trainees and surgeons groups, respectively (P = 0.91). Conclusion Allowing senior pediatric surgery trainees to perform laparoscopic appendectomies without supervision to stimulate surgical autonomy is safe and does not compromise patient outcomes. Level of evidence III.

With the expanding horizon of microsurgical techniques, novel treatment strategies for lymphatic abnormalities are increasingly reported. Described in this article is the first reported use of lymphovenous anastomosis surgery to manage recalcitrant chylothoraces in infants. Chylothorax is an increasingly common postoperative complication after pediatric cardiac surgery, with a reported incidence of up to 9.2 percent in infants. Although conservative nutritional therapy has a reported 70 percent success rate in this patient population, failed conservative management leading to persistent chylothorax is associated with a significant risk of multisystem complications and mortality. Once conservative medical strategies are deemed unsuccessful, surgical or radiologic interventions, such as percutaneous thoracic duct embolization or ligation, are often attempted. However, these procedures lack high-level evidence in the infant population and remain a challenge, given the small size of the lymphatic vessels. As such, we report our experience with performing lymphovenous anastomoses in two infants who had developed refractory chylothoraces secondary to thoracic duct injury following cardiac surgery for congenital cardiac anomalies. In addition, this article reviews the relevant pathophysiology of chylothoraces, current treatment algorithm following failed conservative management, and potential role of the microsurgeon in the multidisciplinary management of this life-threatening problem. As part of the evolving microsurgery frontier, physiologic operations, such as lymphovenous anastomosis, may have a considerable role in the management of refractory pediatric chylothoraces. In our experience, lymphovenous anastomosis can restore normal lymphatic circulation within 1 to 2 weeks, liberate patients from mechanical ventilation, and enable expeditious return to enteral feeding. Clinical question/level of evidence Therapeutic, V.

At fetal MR, congenital lung lesions are usually T2 hyperintense with respect to normal lung parenchyma. Some lesions, however, demonstrate unusual patterns of T2 hypointensity, sometimes in a rosette-like pattern. These lesions usually present a diagnostic conundrum. To evaluate the imaging findings and pathological characterization of fetal solid lung lesions with elements showing T2-hypointense signal with respect to lung. This is a retrospective study of lung lesions with elements showing T2 hypointensity treated prenatally and postnatally at our center and with available pathological evaluation. Prenatal imaging evaluation included US and MR; postnatal evaluation consisted of pathological examination of the lesion. We also performed prenatal and postnatal chart review. Six cases met study criteria. Areas of decreased echogenicity/T2-hypointense signal were more conspicuous at MR than US. At pathology, these areas correlated with immature parenchymal development and increased mesenchymal tissue. Five of these lesions were congenital pulmonary airway malformations (CPAM); one was a congenital peribronchial myofibroblastic tumor (CPMT). The lesions did not significantly change in size after steroid administration. They were all large in volume and were associated with increased amniotic fluid. All cases of CPAM underwent premature delivery (one of them weeks after fetal surgical resection of the lesion for worsening hydrops); the fetus with CPMT was delivered at term. The neonate with CPMT succumbed shortly after birth secondary to lung hypoplasia; the remaining five neonates survived. The differential diagnoses of prenatal lung lesions that contain unusual T2-hypointense elements include CPAM and CPMT. The T2-hypointense areas appear to correlate with increasing degree of immaturity at histology. None of the lesions significantly changed in size after prenatal administration of steroids. All cases with CPAM lesions did well despite persistent polyhydramnios and premature birth. The single case of CPMT, however, resulted in neonatal demise shortly after birth secondary to pulmonary hypoplasia. It is important that fetal radiologists, obstetricians and fetal surgeons alike are aware of these lesions so that appropriate diagnosing and parental counseling can be reached.

To evaluate if gestational age (GA), mode of delivery and abdominal wall closure method influence outcomes in uncomplicated gastroschisis (GTC). Retrospective review of NICU admissions for gastroschisis, August 2008–July 2016. Primary outcomes were: time to start enteral feeds (on-EF), time to discontinue parenteral nutrition (off-PN), and length of stay (LOS). A total of 200 patients with GTC were admitted to our NICU. Patients initially operated elsewhere (n = 13) were excluded. Patients with medical/surgical complications (n = 62) were analyzed separately. The study included 125 cases of uncomplicated GTC. There were no statistically significant differences in the outcomes of patients born late preterm (34 0/7–36 6/7; n = 70) and term (n = 40): on-EF 19 (5–54) versus 17 (7–34) days (p = 0.29), off-PN 32 (12–101) versus 30 (16–52) days (p = 0.46) and LOS 40 (18–137) versus 37 (21–67) days (p = 0.29), respectively. Patients born before 34 weeks GA (n = 15) had significantly longer on-EF, off-PN and LOS times compared to late preterm patients: 26 (12–50) days (p = 0.01), 41 (20–105) days (p = 0.04) and 62 (34–150) days (p

Transient hypoglycemia in the newborn period is common and generally associated either with immaturity of the glucose regulatory pathways (which responds to frequent feeds and resolves spontaneously within hours), or with stress-associated hyperinsulinism (which responds well to hyperglycemic drugs and resolves spontaneously within the first few weeks or months of life). Congenital Hyperinsulinism (HI) is the most frequent cause of persistent, long-term hypoglycemia in newborns and infants, and can lead to severe and irreversible brain damage and developmental delay. It is a rare congenital disorder of the glucose metabolism that has an estimated incidence of 1–1.4 cases per 50,000 live births, leading to about 80–120 new cases in the United States each year. An incidence as high as 1 in 2500 live births has been reported in populations with high consanguinity like Arabians and Ashkenazi Jews. Inappropriate oversecretion of insulin is the hallmark of HI, and the genetic background is quite variable. Depending on the genetic mutation, babies with HI may be treated medically or may require surgery either as a palliative treatment or as a definitive cure.

Objective The objective of the study is to describe our initial 100 attempted infant thoracoscopic lobectomies for asymptomatic, prenatally diagnosed lung lesions, and compare the results to contemporaneous age-matched patients undergoing open lobectomy. Background Infant thoracoscopic lobectomy is a technically challenging procedure, which has only gained acceptance worldwide in recent years. Methods This is a retrospective review of all patients undergoing thoracoscopic or open lung lobectomy between March 2005 and January 2014. Included were all asymptomatic infants younger than 4months. Excluded were patients undergoing emergent lobectomy and patients with isolated extralobar bronchopulmonary sequestrations. Results A total of 100 attempted thoracoscopic lobectomies were compared with 188 open lobectomies. In the thoracoscopic group, mean age and weight at surgery were 7.3weeks and 4.8kg, mean operative time was 185minutes, and mean hospital stay was 3days. Twelve cases were converted to open (12%). Ten conversions occurred within the first third of the series and none in the last third. There were no mortalities. There were no differences between the thoracoscopic and open groups in perioperative complications or hospital stay. There was a significant difference in the operative time: 111minutes vs. 185minutes (open vs. thoracoscopic; p 2 ) and lower mean peripheral capillary oxygen saturation (SpO 2 ) in the thoracoscopic group versus the open group (51.7 versus 38.6mmHg and 97.5 versus 99.1%, respectively). Conclusion In high volume centers, the learning curve of thoracoscopic lobectomy can be overcome and the procedure can be performed with equivalent outcomes and, in our opinion, superior cosmetic results to open lobectomy.

Thoracoscopic repair of congenital diaphragmatic hernia (CDH) has become a popular approach. As there is an ongoing discussion on whether the benefits of the thoracoscopic repair outweigh the potential side effects, we aimed to investigate the opinion and experience of the members of the International Pediatric Endosurgery Group (IPEG) on this topic.An online survey was conducted between October and December 2013 on behalf of the IPEG Research Committee. All 536 IPEG members were contacted by e-mail and asked to complete an anonymous questionnaire that included 28 items on the management of CDH.One hundred sixty-one pediatric surgeons completed the questionnaire. Contraindications to thoracoscopic repair included the following: patient on extracorporeal membrane oxygenation (ECMO) (78%); preoperative need for ECMO (42%); right-sided hernia (15%); liver in chest (32%); weight2.5 kg (35%); and persistent right-to-left shunting (41%). Fifty-two percent of participants said that during thoracoscopic CDH repair they would tolerate any pH. In contrast, 48% indicated that they would only tolerate pH/partial pressure of CO2 (pCO2) levels down/up to 7.2/80 mm Hg (range: pH, 6.9-7.3; pCO2, 55-100 mm Hg). If a patch was needed, 39% of participants said they would continue thoracoscopically, 31% would convert, and 31% stated that the decision would depend on the size of the defect. Fifty-seven percent of participants reported recurrences after thoracoscopic repair, which occurred within 6 months in 43% of the cases, after 6 months in 37%, and both before and after in 20% of the cases. Overall, 50% of surgeons stated that CDH can be repaired equally by thoracoscopy and open approaches, and 50% disagreed with this statement.Thoracoscopic CDH repair is currently being performed by 89% of all participating IPEG members. ECMO, either active or previously, and persistent right-to-left shunting are the main deterrents to thoracoscopic repair. The fact that only 50% of surgeons stated that CDH can be repaired equally by thoracoscopy and open surgery suggests that future studies should focus on identifying the appropriate patient population.

Background/Purpose The purpose of this study is to describe the prenatal findings and postnatal outcomes of fetuses with axillary lymphatic malformations. Methods Retrospective chart review of fetuses with the prenatal diagnosis of isolated axillary lymphatic malformation detected between 2009 and 2013. Results There were 8 fetuses diagnosed with isolated axillary lymphatic malformation. Median gestational age at diagnosis was 20.5 (19–28) weeks. All fetuses were evaluated by serial ultrasound and ultrafast fetal MRI. Two pregnancies were electively terminated. All continued pregnancies reached term, and all fetuses were delivered by cesarean section. None of the fetuses developed polyhydramnios or hydrops fetalis. Only one patient had an associated malformation (coarctation of the aorta). All patients were evaluated postnatally by MRI. Treatment included sclerotherapy only (1), sclerotherapy followed by surgical resection (1), surgical resection only (3), and observation (1). The median postnatal hospital stay was 8 (6–15) days. Three cases recurred after the initial treatment, two after surgery and one after sclerotherapy. On a median follow up of 2.1years, all patients have some degree of visible residual disease. There were no deaths. Conclusions Prenatal diagnosis of axillary lymphatic malformation is increasing with improved technology. Axillary lymphatic malformations are usually isolated developmental anomalies that do not affect fetal health. Postnatal management options include surgery, sclerotherapy, and observation. Recurrences and residual disease after all types of treatment are frequent. This should be communicated to the parents at the time of prenatal counseling.

Purpose The purpose of this study was to review the outcomes and technical details of EXIT procedures performed in fetuses with large cervical lymphatic malformations. Methods A retrospective chart review of fetuses with a prenatal diagnosis of cervical lymphatic malformation evaluated at our center between 1995 and 2013 was performed. Results We evaluated a total of 112 fetuses with a prenatal diagnosis of cervical lymphatic malformation. Thirteen of the 112 fetuses (11%) were delivered by an EXIT procedure. Criteria to deliver by EXIT were: 1) deviation/compression/obstruction of the airway, and 2) involvement of the floor of the mouth. Two fetuses developed hydrops. Five fetuses developed polyhydramnios. Eleven EXITs were performed electively at term (n=7; 37–38 weeks) or late pre-term (n=4; 34–36/6weeks), whereas two patients underwent emergency EXIT at 33 and 38weeks, respectively. The airway was accessed successfully in 12 of 13 cases. Laryngoscopy only was sufficient in 7, rigid bronchoscopy was required in 4, and 1 required a tracheostomy. In one case with a massive lymphatic malformation of the face, neck, and airway, a tracheostomy was not attempted, and the fetus expired. Four patients had invasion of the larynx by the lymphatic malformation. Five patients required a tracheostomy later. Median time from fetal exposure to intubation was 8 (2–29) min. Median total EXIT time was 105.5 (67–142) min. Median maternal blood loss was 800 (300–1000) ml. Median maternal hospital stay was 4 (3–6) days. Conclusion The EXIT procedure allows controlled airway access in fetuses with cervical lymphatic malformations and evidence of airway impairment on prenatal images.

To evaluate the outcomes of neonates with complex gastroschisis (GC), and correlate outcomes with each type of complication.Retrospective review of patients with complex GC owing to prenatal and/or postnatal abdominal complications; 2008-2016. Primary outcomes: time to discontinue parenteral nutrition (off-PN), length of stay (LOS) and neonatal survival.We treated 58 patients with complex gastroschisis owing to abdominal complications, which were: intestinal necrosis at birth (n=9), intestinal atresia (n=16), medical necrotizing enterocolitis (NEC) (n=15), surgical NEC (n=1), in utero volvulus (n=1), vanishing gastroschisis (n=2), severe intestinal dysmotility (n=1), delayed abdominal closure (n=3), abdominal compartment syndrome (n=2) and hiatal hernia/severe gastroesophageal reflux disease (GERD; n=11). The off-PN time and LOS of the whole group were 92 (35-255) and 119 (42-282) days, significantly longer than those of a demographically equivalent contemporaneous series of 125 patients with uncomplicated gastroschisis (off-PN 32 [12-105] days [p0.001]; LOS 41 [18-150] days [p0.001]). Patients with intestinal necrosis at birth or with intestinal atresia had the longest off-PN and LOS times (133 [38-255] / 157 [43-282] and 114 [36-222] / 143 [42-262] days, respectively), followed by patients with complications of the abdominal wall closure (n=5) (69 [43-93] / 89 [58-110] days), patients with hiatal hernias/severe GERD who required fundoplication (63 [35-84] / 89 [57-123] days) and patients who developed medical NEC (67 [35-103] / 76 [50-113] days). Short-bowel syndrome/PN-dependence occurred in 6/58 (10%) patients (2 vanishing gastroschisis, 1 in utero volvulus, 2 intestinal atresias and 1 bowel necrosis at birth). There were no neonatal mortalities.Gastroschisis can be complicated by a wide variety of prenatal and postnatal events. The most severe outcomes occur in patients with bowel necrosis at birth, intestinal atresias, or vanishing gastroschisis. Complications, however, did not affect neonatal survival in our experience.III.

Abdominal wall defects range from the mild umbilical cord hernia to the highly complex limb-body wall syndrome. The most common defects are gastroschisis and omphalocele, and the rarer ones include the exstrophy complex, pentalogy of Cantrell and limb-body wall syndrome. Although all have a common feature of viscera herniation through a defect in the anterior body wall, their imaging features and, more important, postnatal management, differ widely. Correct diagnosis of each entity is imperative in order to achieve appropriate and accurate prenatal counseling and postnatal management. In this paper, we discuss fetal abdominal wall defects and present diagnostic pearls to aid with diagnosis.

To present our experience in the care of infants with Beckwith-Wiedemann syndrome (BWS) who required pancreatectomy for the management of severe Congenital Hyperinsulinism (HI).We did a retrospective chart review of patients with BWS who underwent pancreatectomy between 2009 and 2012.Four patients with BWS and severe HI underwent pancreatectomy, 3 females and one male. Eight other BWS patients with HI could be managed medically. The diagnosis of BWS was established by the presence of mosaic 11p15 loss of heterozygosity and uniparental disomy in peripheral blood and/or pancreatic tissue. All patients had hypoglycemia since birth that did not respond to medical management with diazoxide or octreotide, and required glucose infusion rates of up to 30 mg/kg/min. Preoperative 18-F-DOPA PET/CT scans showed diffuse uptake of the radiotracer throughout an enlarged pancreas in three patients and a normal sized pancreas with a large area of focal uptake in the pancreatic body in one patient. None of the patients had mutations in the ABCC8 or KCNJ1 genes that are typically associated with diazoxide-resistant HI. Age at surgery was 1, 2, 4, and 12 months and the procedures were 85%, 95%, 90%, and 75% pancreatectomy, respectively, with the pancreatectomy extent tailored to HI severity. Pathologic analysis revealed marked diffuse endocrine proliferation throughout the pancreas that occupied up to 80% of the parenchyma with scattered islet cell nucleomegaly. One patient had a small pancreatoblastoma in the pancreatectomy specimen. The HI improved in all cases after the pancreatectomy, with patients being able to fast safely for more than 8 h. All patients are under close surveillance for embryonal tumors. One patient developed a hepatoblastoma at age 2.The pathophysiology of HI in BWS patients is likely multifactorial and is associated with a dramatic increase in pancreatic endocrine tissue. Severe cases of HI that do not respond to medical therapy improve when the mass of endocrine tissue is reduced by subtotal or near-total pancreatectomy.