James E. Stein

Outcome analysis of neonates with congenital diaphragmatic hernia treated with venovenous vs venoarterial extracorporeal membrane oxygenation.

PURPOSE Venoarterial extracorporeal membrane oxygenation (ECMO) (VA) is used more commonly in neonates with congenital diaphragmatic hernia (CDH) than venovenous ECMO (VV). We hypothesized that VV may result in comparable outcomes in infants with CDH requiring ECMO. METHODS We retrospectively analyzed the Extracorporeal Life Support Organization (ELSO) database (1991-2006). Multivariate logistic regression analyses were used to compare VV- and VA-associated mortality. RESULTS Four thousand one hundred fifteen neonates required ECMO, with an overall mortality rate of 49.6%. Venoarterial ECMO was used in 82% and VV in 18% of neonates. Pre-ECMO inotrope use and complications were equivalent between VA and VV. The mortality rate for VA and VV was 50% and 46%, respectively. After adjusting for birth weight, gestational age, prenatal diagnosis, ethnicity, Apgar scores, pH less than 7.20, Paco(2) greater than 50, requiring high-frequency ventilation, and year of ECMO, there was no difference in mortality between VV vs VA. Renal complications and on-ECMO inotrope use were more common with VV, whereas neurologic complications were more common with VA. The conversion rate from VV to VA was 18%; conversion was associated with a 56% mortality rate. CONCLUSION The short-term outcomes of VV and VA are comparable. Patients with CDH who fail VV may be predisposed to a worse outcome. Nevertheless, VV offers equal benefit to patients with CDH requiring ECMO while preserving the native carotid.

Contemporary management of lipoblastoma

PURPOSE Lipoblastoma is a rare, benign, adipose tissue tumor. We report the largest single institution experience managing these uncommon neoplasms. METHODS We retrospectively reviewed 32 cases of lipoblastoma entered in the pathology database at our institution between January 1991 and August 2005. We conducted a comprehensive literature review of lipoblastoma and summarized the results of the largest series published. RESULTS Most patients presented with an enlarging, palpable, firm, nontender, mobile mass. The male-to-female ratio was 1.9:1. The anatomical distribution was trunk (n = 12), extremity (n = 12), groin (n = 5), and neck (n = 3). Average age at resection was 2.8 years (range, 2.6 months to 12 years). Thirty-one cases were completely excised, although 1 patient underwent staged partial excision to preserve nerve function. Chromosomal analysis performed in selected patients revealed characteristic aberrations in chromosome 8. Complications included keloid formation (n = 3), wound infection/dehiscence (n = 2), wound seroma (n = 1), and transient brachial plexus neurapraxia (n = 1). Average follow-up was 7.4 months (range, 1 day to 6.5 years); 2 patients were lost to follow-up. There were no recurrences. CONCLUSIONS A staged approach with meticulous sparing of the neurovascular bundle provides excellent functional outcome for patients with large tumors. Nonmutilating surgical excision is the treatment of choice.

Thoracoscopic Repair of Neonatal Diaphragmatic Hernia

INTRODUCTION The use of minimally invasive surgery (MIS) in the neonatal population is increasing. Thoracoscopic intervention for congenital diaphragmatic hernia (CDH) is no exception. In this report, we describe our initial experience with thoracoscopic repair of left-sided diaphragmatic defects in neonates. MATERIALS AND METHODS We performed retrospective chart reviews on all neonates who underwent thoracoscopic repair of CDH between November 2004 and January 2008. Neonates that underwent thoracoscopic repair were physiologically stable with resolved pulmonary hypertension and minimal to moderate ventilatory support. They had no associated cardiac anomalies. RESULTS We identified 15 neonates with CDH who underwent thoracoscopic repair during the study period. Ten neonates underwent primary repair of the diaphragmatic defect. Five neonates with large defects required closure with a synthetic patch, which was placed thoracoscopically. The average operating room time was 134 minutes. There were no instances of intraoperative respiratory or cardiac instability. Three patients had a recurrence. One recurrence was seen after thoracoscopic patch repair. Two recurrences occurred following primary repair of left diaphragmatic hernias. There were no deaths. Follow-up has been 4-40 months. CONCLUSIONS Neonatal MIS for CDH should be limited to stable patients. The ideal candidate is the newborn without associated anomalies, not requiring extracorporeal membrane oxygenation, on minimal ventilatory support, and without evidence of pulmonary hypertension. It is technically possible to perform thoracoscopic repair with a patch.

Association of Escherichia coli O157:H7 with necrotizing enterocolitis in a full-term infant.

Necrotizing enterocolitis (NEC) is the most common gastrointestinal emergency of the neonate. NEC is predominantly seen in premature infants; however, in rare instances it can affect full-term infants as well. Although the pathogenesis of NEC remains elusive, it is well established that bacterial colonization is required for development of this disease. In this report, we present a case of a full-term infant, who developed a very aggressive form of NEC and was found to have Escherichiacoli (E.coli) O157:H7 both in stool and blood cultures. Unfortunately, despite aggressive surgical and intensive care management, this infant suffered pan-intestinal necrosis and expired. We were not able to establish the route of transmission. To our knowledge, this is the first report of the association of E.coli O157:H7 with NEC.

The Role of Extracorporeal Membrane Oxygenation Simulation Training at Extracorporeal Life Support Organization Centers in the United States

Introduction Extracorporeal membrane oxygenation (ECMO) requires a multidisciplinary healthcare team. The Extracorporeal Life Support Organization publishes training guidelines but leaves specific requirements up to each institution. Simulation training has shown promise, but it is unclear how many institutions have incorporated simulation techniques into ECMO training to date. Methods We sent an electronic survey to ECMO coordinators at Extracorporeal Life Support Organization sites in the United States. Participants were asked about training practices and the use of simulation for ECMO training. Descriptive results were reported as the percentage of total responses for each question. Logistic regression was used to identify characteristics associated with simulation use. Results Of 94 responses (62% response rate), 46% had an ECMO simulation program, whereas 26% report a program is in development. Most (61%) have been in operation for 2 to 5 years. Sixty-three percent use simulation for summative assessment, and 76% have multidisciplinary training. Access to a simulation center [odds ratio (OR) = 4.7, 95% confidence interval (CI) = 1.7–12.5], annual ECMO caseload of greater than 20 (OR = 2.5, 95% CI = 1.5–5.8), and having a pediatric cardiothoracic intensive care unit (OR = 2.8, 95% CI = 1.2–6.7) are each associated with increased likelihood of mannequin-based ECMO simulation. Common scenarios include pump failure (93%), oxygenator failure (90%), and circuit rupture (76%). Discussion Extracorporeal membrane oxygenation simulation is growing but remains in its infancy. Centers with access to a simulation center, higher caseloads, and pediatric cardiothoracic intensive care units are more likely to have ECMO simulation programs. Extracorporeal membrane oxygenation simulation is felt to be beneficial, and further work is needed to delineate best training practices for ECMO providers.

The Swiss cheese model of adverse event occurrence—Closing the holes

Traditional surgical attitude regarding error and complications has focused on individual failings. Human factors research has brought new and significant insights into the occurrence of error in healthcare, helping us identify systemic problems that injure patients while enhancing individual accountability and teamwork. This article introduces human factors science and its applicability to teamwork, surgical culture, medical error, and individual accountability.

Respiratory failure in postpneumonectomy syndrome complicated by thoracic lordoscoliosis: treatment with prosthetic implants, partial vertebrectomies, and spinal fusion.

Study Design. This study investigated the case of a 17-year-old girl with postpneumonectomy syndrome, complicated by a thoracic lordoscoliosis, who was successfully treated with prosthetic implants, partial vertebrectomies, and anteroposterior spinal fusion. Objective. To report a unique case and describe the authors’ method of treatment. Summary of Background Data. Postpneumonectomy syndrome is an uncommon complication of pneumonectomy. Many case reports describe successful treatment with insertion of prosthetic implants into the empty hemithorax to shift the mediastinum to its original position. Thoracic lordoscoliosis reportedly has contributed to pulmonary compromise, but no cases have shown its occurrence in the setting of postpneumonectomy syndrome. Methods. The patient was observed at the National Children’s Hospital in Tokyo, referred to Children’s Hospital in Los Angeles, California for surgical correction, and followed in Tokyo for the next year. Results. Two prosthetic implants with an injection port for further expansion were positioned in the right hemithorax to restore the mediastinum to its normal position. Anterior discectomies, partial vertebrectomies, and fusion of T5–T10 was performed concurrently. Then 5 days later, posterior spinal fusion of T1–T12 with instrumentation and bone graft were performed to correct the thoracic lordoscoliosis and increase the chest cavity space. At 1 month after the surgery, the patient was extubated after being ventilator dependent for 5 months. At the time of operation, the girl was ventilator dependent and nonambulatory, but 1 year later could participate in all activities of daily living without any oxygen supplementation. Conclusions. Postpneumonectomy syndrome can be treated successfully with prosthetic implants to restore the normal position of the mediastinum. Thoracic lordoscoliosis can complicate the syndrome and may be corrected to help restore normal pulmonary function.

Increased carotid artery intima-media thickness following venoarterial extracorporeal membrane oxygenation in the neonatal period

Objective:To compare left carotid intima-media thickness (CIMT) and biochemical markers for atherogenesis in neonatal venoarterial extracorporeal membrane oxygenation (ECMO) survivors with normal controls during childhood.Methods:Venoarterial ECMO survivors and healthy patients between 12 and 18 years of age were enrolled in a matched control prospective study. ECMO survivors were matched to controls based on chronological age and percentage of body mass index (BMI). Measured CIMT of the posterior left carotid artery and CIMT values corrected for carotid artery size were used for data analysis.Results:Thirty-one neonatal venoarterial ECMO survivors were matched to 31 healthy controls. No significant differences were found between ECMO survivors and controls for age, weight, percentage of BMI, total fat composition, lipid profiles, ultrasensitive C-reactive protein or homocysteine levels. Significant differences between ECMO and controls patients were found in systolic, diastolic and mean left CIMT.Conclusion:Compared with controls, the thickness of the left carotid intima media is significantly increased at the age of 12 to 18 years in neonatal venoarterial ECMO survivors. The clinical significance of this increased CIMT is unknown. ECMO centers may want to incorporate assessment of CIMT in their follow-up protocols.

Hepatocellular malignant neoplasm, NOS: a clinicopathological study of 11 cases from a single institution

The primary aim of this study is to characterize hepatocellular malignant neoplasm, NOS (HEMNOS), a new provisional entity describing a subset of paediatric hepatocellular tumours, which have histological features of neither typical hepatoblastoma (HB) nor hepatocellular carcinoma (HCC).

The biological prosthesis is a viable option for abdominal wall reconstruction in pediatric high risk defects

BACKGROUND Our aim was to explore the indications for and outcome of biological prostheses to repair high risk abdominal wall defects in children. METHODS A retrospective chart review was performed of all cases of abdominal wall reconstruction in a single institution between 2007 and 2015. Demographic and clinical variables, technique and complications were described and compared between prosthesis types. RESULTS A total of 23 patients underwent abdominal wall reconstruction using a biological prosthesis including 17 neonates. The main indication was gastroschisis (17 patients) followed by ruptured omphalocele and miscellaneous conditions. Alloderm™ was most commonly used followed by Surgisis™, Strattice™, Flex-HD™ and Permacol™. In 22 cases wounds were contaminated or infected. Open bowel/stomas were present in 9 cases. Skin was not closed in 11 cases. Post-operative complication rate was 30% and hernia recurrence rate was 17% after a mean follow-up time of 16 months. CONCLUSIONS The use of a biological prosthesis may offer advantages over a synthetic mesh in pediatric high risk abdominal wall defects. The surgeon should be ready to consider its use in selected cases.