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Introduction ECMO is a support modality for refractory critical illness. This study reviews the incidence and utility of central venous line (CVL) placement at pediatric ECMO decannulation. Methods A single-institution retrospective study of patients undergoing open neck decannulation from 2015 to 2019. Patients were divided into two groups: ≤ 28-days and > 28-days. Results Of 65 patients, 31% had a CVL placed at decannulation. Sepsis and pneumonia were the most common indications for ECMO in the older-group compared to CDH in neonates. The most common indications for CVL were hemodialysis (45%), monitoring (25%), and access (25%). 89% of neonates had an access line placed, whereas 73% of the older group received hemodialysis catheters. Median CRRT requirement was 20 days. 85% of lines were functional at time of removal or death. None were removed for infection. 40% of the patients not receiving a CVL at decannulation required one within 30 days. Conclusion 69% of patients did not have a CVL placed at decannulation, however 40% required a CVL within 30 days. Most lines placed at decannulation remained functional and none were removed for infection. Decannulation removes the circuit as a route for vascular access, but it also presents an opportunity to safely place an essential CVL.

Congenital tracheo-esophageal fistula/esophageal atresia (TEF/EA) with concomitant pulmonary agenesis is exceedingly rare and has a high mortality rate. While there are several reported cases of successful repair, all but one patient had right-sided pulmonary agenesis. In the case of left-sided pulmonary agenesis, the patient had incomplete agenesis and underwent repair through a left thoracotomy. We present the first successful repair of TEF/EA with complete left-sided pulmonary agenesis. This patient also underwent elective pre-operative veno-venous extracorporeal membrane oxygenation (ECMO) and subsequent repair of the TEF/EA. We discuss the management, anesthesia risks, and role of periprocedural ECMO in pediatric patients who are high anesthetic risk.

Surgical trainees are expected to demonstrate family-centered care. However, it is unclear if residents know how to address psychosocial issues of pediatric patients and their families. Our aim was to evaluate surgical trainees’ knowledge of family dynamics. Over a six-month period, trainees (n = 16) were surveyed regarding their comfort and familiarity with the psychosocial aspects of patient care and family dynamics. Residents recorded their comfort level with managing various behaviors using a Likert scale, and indicated which family issues they felt least prepared to handle. Most trainees lacked knowledge of family adjustment phases (50%), relational triangles (78%), developmental stages of families (40%), ambiguous loss (75%), ABCX model of family stress (100%), and the SPIKES model (88%). Excluding anxiety and sadness, almost half of residents felt unprepared for dealing with a variety of challenging behaviors. Finally, trainees were least comfortable with breaking bad news. A Family Dynamics curriculum could potentially increase resident management skills and improve patient care.

BACKGROUND: Cross-sectional imaging increases accuracy in diagnosing appendicitis. We hypothesized that a radiation-free imaging pathway of ultrasonography selectively followed by MRI would not change clinical end points compared with computed tomography (CT) for diagnosis of acute appendicitis in children. METHODS: We retrospectively reviewed children ( RESULTS: Six hundred sixty-two patients had imaging for suspected appendicitis (group A = 265; group B = 397, of which 136 [51%] and 161 [41%], respectively, had positive imaging for appendicitis). Negative appendectomy rate was 2.5% for group A and 1.4% for group B. Perforation rate was similar for both groups. Time from triage to antibiotic administration and operation did not differ between groups A and B. There was higher proportion of positive imaging and appendectomies in group A and thus more negative imaging tests in group B (ultrasonography and MRI), but diagnostic accuracy of the 2 imaging pathways was similar. CONCLUSIONS: In children with suspected acute appendicitis, a radiation-free diagnostic imaging of ultrasonography selectively followed by MRI is feasible and comparable to CT, with no difference in time to antibiotic administration, time to appendectomy, negative appendectomy rate, perforation rate, or length of stay.

Over the last 15 years thoracoscopic lobectomy for congenital and acquired lesions has become an accepted modality in pediatric thoracic surgery. There is still debate about the need to perform a complete lobectomy for some of these lesions, and some advocate observation rather than resection, despite possible long-term complications of untreated lesions. High-resolution computed tomography (CT) scans and physical findings at the time of surgery, along with new advanced techniques, now allow for discrete partial anatomic resections, which may preserve normal lung. This study evaluates the feasibility and early results using these techniques in selected cases.With institutional review board approval, the records of all patients undergoing thoracoscopic lung resection were reviewed. From January 2006 to December 2012, 23 patients, ranging from 1 month to 16 years of age and weighing 3.8-42 kg, underwent thoracoscopy for planned resection. Pathology was congenital cystic lung disease in 19 patients, bronchiectasis in 3 patients, and arteriovenous malformation in 1 patient. In each case findings on CT scan and at the time of surgery warranted consideration of lung-preserving surgery. Procedures were performed through three ports using single lung ventilation and CO2 insufflation to achieve lung collapse. The LigaSure™ device (Covidien, Norwalk, CT) was the primary instrument used to seal and divide the lung parenchyma and seal vessels.All procedures were completed successfully thoracoscopically. An anatomic segmental resection was achieved in 22 of 23 cases. Operative time ranged from 30 to 300 minutes (mean, 120 minutes). Segmental resections included the left upper lobe apical/posterior (n=4), lingula (n=3), left lower lobe superior (n=5), medial or posterior basal (n=3), right middle lobe medial (n=1), right upper lobe apical (n=1), right lower lobe superior (n=4), and posterior basal (n=2). Two patients had more than one segment excised. Chest tubes were left in for 24 hours in 16 cases, 48 hours in 4 cases, and 5 days in 1 case. Hospital stay ranged from 1 to 6 days (mean, 2 days). Follow-up CT scans obtained at 1-6 years (mean, 28 months) show no residual disease in 20 of 21 patients. One patient underwent a nonanatomic resection and had evidence of recurrent congenital pulmonary airway malformation at the 4-year follow-up. This patient underwent a secondary thoracoscopic resection.Thoracoscopic lung-conserving therapy is technically feasible and safe in infants and children. The magnification provided by a thoracoscopic approach makes identification of segmental anatomic planes easier, aiding in safe dissection and resection. Anatomic resection appears to be associated with a low morbidity. It may be appropriate in the case of bilateral or extensive disease or in cases where the diseased tissue is clearly limited to an anatomic segment. Continued long-term follow-up is needed.

Purpose Lung-to-head ratio (LHR) has been used for antenatal evaluation of infants with congenital diaphragmatic hernia (CDH). We hypothesized that LHR was predictive of acute and chronic pulmonary hypertension in infants with CDH. Methods Echocardiograms on all inborn infants with CDH (December 2001–March 2011) were reviewed. Echocardiograms at 1 and 3months post-repair and most recent follow-up were assessed for presence of pulmonary hypertension (PAH). LHR, gestational age, birth weight, extracorporeal membrane oxygenation (ECMO), and death rate were obtained. Bivariate and multivariate analyses were performed. Results 106 infants with CDH had LHR obtained at median 28weeks gestation (median LHR=1.25 [range 0.4–5.3]). Median follow-up was 26.6months (range 4.6–97.5). The long-term incidence of pulmonary hypertension was 16%. LHR was significantly associated with pulmonary hypertension at one month (p=0.0001) but not at 3months (p=0.22) or long-term (p=0.54). LHR was predictive of ECMO use (p=0.01) and death (p=0.001). Conclusions The overall incidence of PAH in infants with CDH decreases over time. Prenatal LHR predicts PAH at one month but not long-term in infants with CDH. The ability for LHR to predict PAH at one month but not long term may suggest remodeling of the pulmonary vasculature over time.

Mycoplasma pneumoniae is one of the most common agents causing respiratory disease in children. The most common extra-pulmonary manifestations of M. pneumoniae include central nervous system involvement, with stroke being an uncommon but devastating consequence. We present a 13-year-old girl with severe respiratory disease requiring extracorporeal membrane oxygenation, who developed ischemic stroke associated with clinical and serologic evidence of M. pneumoniae. A case of M. pneumoniae causing this degree of respiratory failure associated with stroke has not been previously reported. Prompt recognition of severe mycoplasmal infection may allow for earlier treatment and concomitant evaluation of neurologic injury.

Lymphatic malformations (LMs) are vascular anomalies thought to arise from dysregulated lymphangiogenesis. These lesions impose a significant burden of disease on affected individuals. LM pathobiology is poorly understood, hindering the development of effective treatments. In the present studies, immunostaining of LM tissues revealed that endothelial cells lining aberrant lymphatic vessels and cells in the surrounding stroma expressed the stem cell marker, CD133, and the lymphatic endothelial protein, podoplanin. Isolated patient-derived CD133+ LM cells expressed stem cell genes (NANOG, Oct4), circulating endothelial cell precursor proteins (CD90, CD146, c-Kit, VEGFR-2), and lymphatic endothelial proteins (podoplanin, VEGFR-3). Consistent with a progenitor cell identity, CD133+ LM cells were multipotent and could be differentiated into fat, bone, smooth muscle, and lymphatic endothelial cells in vitro. CD133+ cells were compared to CD133− cells isolated from LM fluids. CD133− LM cells had lower expression of stem cell genes, but expressed circulating endothelial precursor proteins and high levels of lymphatic endothelial proteins, VE-cadherin, CD31, podoplanin, VEGFR-3 and Prox1. CD133− LM cells were not multipotent, consistent with a differentiated lymphatic endothelial cell phenotype. In a mouse xenograft model, CD133+ LM cells differentiated into lymphatic endothelial cells that formed irregularly dilated lymphatic channels, phenocopying human LMs. In vivo, CD133+ LM cells acquired expression of differentiated lymphatic endothelial cell proteins, podoplanin, LYVE1, Prox1, and VEGFR-3, comparable to expression found in LM patient tissues. Taken together, these data identify a novel LM progenitor cell population that differentiates to form the abnormal lymphatic structures characteristic of these lesions, recapitulating the human LM phenotype. This LM progenitor cell population may contribute to the clinically refractory behavior of LMs.

Fig. Preduodenal portal vein identified during exploration for biliary atresia. Duo, Duodenum; P, pancreas; L, liver; S, stomach; PDPV, preduodenal portal vein. AN 11-WEEK-OLD FEMALE INFANT presented with jaundice and hyperbilirubnemia. Her mother reported a history of yellow-colored eyes, lightcolored stools, and dark urine since shortly after birth. She was a full-term infant with an initial admission to the neonatal intensive care unit for bradycardia. An echocardiogram showed a noncompaction cardiomyopathy. A hepatic panel preformed at her primary pediatrician’s office was significant for total bilirubin of 7.3 mg/dL and direct bilirubin 4.2 mg/dL; she was referred for further evaluation. Preoperative abdominal ultrasonography showed a shrunken gallbladder and no other abnormality. A percutaneous liver biopsy was performed and revealed bile ductular proliferation and cholestasis, which was thought to be consistent with biliary atresia. A Kasai Roux-en-Y hepatoportoenterostomy for a presumptive diagnosis of biliary atresia was therefore recommended. After the patient’s abdomen was entered, inspection of the left upper quadrant was notable for the absence of the spleen and further exploration showed intestinal malrotation and a preduodenal portal vein (Figure). The dissection of the portal plate was accomplished without difficulty. The narrowed base of the mesentery was broadened as much as possible, and an appendectomy was performed. Construction of the Roux limb and the hepatoportoenterostomy was then performed.

Introduction: Anterior diaphragmatic hernias of the Morgagni type are the least common type of congenital diaphragmatic hernia and account for about 6% of all cases. Morgagni hernias often present as an incidental finding on a chest radiograph. Most pediatric surgeons would advocate for elective repair in asymptomatic patients. Laparoscopic repair of Morgagni hernia in children has been previously described.1–3 As the hernia itself produces minimal symptoms, postoperative cosmesis is often of great concern to the parents of the affected child. Percutaneous suturing techniques have been previously described for single-incision laparoscopic surgery. We present our technique of virtually scarless single-incision pediatric endosurgical (SIPES) repair of a Morgagni hernia. Case: The patient was a 20-month-old female child who presented to our hospital with fever and persistent cough. A chest radiograph was obtained, showing an anterior diaphragmatic hernia. The finding was confirmed on computed tomogr...

Purpose To assess the efficacy and accuracy of ultrasonography (US) and selective computed tomography (CT) in the diagnosis of acute appendicitis in children. Methods A retrospective review of all ultrasound evaluations for appendicitis from July 1, 2003, to June 30, 2010, was conducted at two urban pediatric centers. Beginning in 2003, a multi-disciplinary diagnostic protocol was implemented to reduce radiation exposure employing US as the initial imaging modality followed by CT for non-diagnostic US studies in patients with an equivocal clinical presentation. The imaging, operative findings, and pathology of 802 patients (365 females, 437 males, age less than 18years) with suspected appendicitis were reviewed. The sensitivity, specificity, predictive value, and negative appendectomy rate of the protocol were analyzed. A telephone survey was conducted of patients discharged without a diagnosis of appendicitis to evaluate the missed appendicitis rate. Results Of the 601 pediatric appendectomies performed, a total of 275 (46%) were diagnosed by protocol. The selective protocol had a sensitivity of 94.2%, specificity of 97.5%, positive predictive value of 95.2%, and negative predictive value of 97.0%. The negative appendectomy rate was 1.82%, and the missed appendicitis rate was 0%. No patient discharged after only ultrasound evaluation without undergoing surgery reported missed appendicitis on the survey (41.7% response rate). Protocol use increased from 6.7% to 88.3%. US was the sole imaging modality in 630 of all 802 patients (78.6%). Conclusions US followed by selective CT for the diagnosis of acute appendicitis is useful and accurate. This has important implications in the reduction of childhood radiation exposure.

Infants with tracheoesophageal fistulas may present with complex cardiac disease that may obviate or disrupt a safe operative repair. Here we present a case of an infant who developed cardiac instability during esophageal atresia repair, precluding formal anastomosis after approximation of the distal and proximal esophageal segments. Postoperatively, anastomosis of the esophagus was achieved using an image-guided technique with subsequent dilation. This approach may provide an alternative approach for establishment of esophageal continuity in patients who are high-risk operative candidates.

Mycoplasma pneumoniae is one of the most common agents causing respiratory disease in children. The most common extra-pulmonary manifestations of M. pneumoniae include central nervous system involvement, with stroke being an uncommon but devastating consequence. We present a 13-year-old girl with severe respiratory disease requiring extracorporeal membrane oxygenation, who developed ischemic stroke associated with clinical and serologic evidence of M. pneumoniae. A case of M. pneumoniae causing this degree of respiratory failure associated with stroke has not been previously reported. Prompt recognition of severe mycoplasmal infection may allow for earlier treatment and concomitant evaluation of neurologic injury.