Rebecka L. Meyers

Predictive power of pretreatment prognostic factors in children with hepatoblastoma: A report from the Children's Oncology Group

PRETEXT is used to stratify risk in children with hepatoblastoma by the Liver Tumor Strategy Group (SIOPEL) of the International Society of Pediatric Oncology (SIOP). A recent analysis excluding patients that did not survive neoadjuvant chemotherapy, concluded that PRETEXT was superior to Childrens Oncology Group (COG) stage for predicting survival. Puzzled by this result, we made a similar comparison of PRETEXT and COG stage. This time, however, we include all patients, and we compare predictive value at diagnosis, instead of after neoadjuvant chemotherapy.

Routine use of a SILASTIC spring-loaded silo for infants with gastroschisis: a multicenter randomized controlled trial.

BACKGROUND Retrospective studies have suggested that routine use of a preformed silo for infants with gastroschisis may be associated with improved outcomes. We performed a prospective multicenter randomized controlled trial to test this hypothesis. METHODS Eligible infants were randomized to (1) routine bedside placement of a preformed Silastic spring-loaded silo, with gradual reduction and elective abdominal wall closure, or (2) primary closure. RESULTS There were 27 infants in each group. There was no significant difference between groups with respect to age, weight, sex, Apgar scores, prenatal diagnosis, or mode of delivery. The total number of days on the ventilator was lower in the spring-loaded silo group, although it did not reach statistical significance (3.2 vs 5.3, P = .07). There was no significant difference between groups with respect to length of time on total parenteral nutrition, length of stay, or incidence of sepsis and necrotizing enterocolitis. CONCLUSION Routine use of a preformed silo was associated with similar outcomes to primary closure for infants with gastroschisis but with a strong trend toward fewer days on the ventilator. Use of a preformed silo has the advantage of permitting definitive abdominal wall closure in a more elective setting.

Towards an international pediatric liver tumor consensus classification: proceedings of the Los Angeles COG liver tumors symposium

Liver tumors are rare in children, and their diagnoses may be challenging particularly because of the lack of a current consensus classification system. Systematic central histopathological review of these tumors performed as part of the pediatric collaborative therapeutic protocols has allowed the identification of histologic subtypes with distinct clinical associations. As a result, histopathology has been incorporated within the Children’s Oncology Group (COG) protocols, and only in the United States, as a risk-stratification parameter and for patient management. Therefore, the COG Liver Tumor Committee sponsored an International Pathology Symposium in March 2011 to discuss the histopathology and classification of pediatric liver tumors, and hepatoblastoma in particular, and work towards an International Pediatric Liver Tumors Consensus Classification that would be required for international collaborative projects. Twenty-two pathologists and experts in pediatric liver tumors, including those serving as central reviewers for the COG, European Société Internationale d’Oncologie Pédiatrique, Gesellschaft für Pädiatrische Onkologie und Hämatologie, and Japanese Study Group for Pediatric Liver Tumors protocols, as well as pediatric oncologists and surgeons specialized in this field, reviewed more than 50 pediatric liver tumor cases and discussed classic and newly reported entities, as well as criteria for their classification. This symposium represented the first collaborative step to develop a classification that may lead to a common treatment-stratification system incorporating tumor histopathology. A standardized, clinically meaningful classification will also be necessary to allow the integration of new biological parameters and to move towards clinical algorithms based on patient characteristics and tumor genetics, which should improve future patient management and outcome.

Tracheobronchial injuries after blunt chest trauma in children : Hidden pathology

BACKGROUND Blunt thoracic injuries in children are unique because the pliability of the chest wall allows transmission of massive external force directly into the mediastinum. Children presenting after blunt chest trauma may have complete disruption of the airway with little external sign of injury. Without prompt diagnosis and appropriate treatment, the risk for progressive respiratory failure is high. METHODS Four children with tracheobronchial injuries were referred to a pediatric trauma center from 1994 to 1997. All children, age 18 months to 13 years, suffered unusual crush injuries. All diagnoses were based on unresolved pneumothorax or pneumomediastinum. RESULTS Bronchoscopy identified the location of injury as posterior trachea (n = 1) and right mainstem bronchus (n = 2). A tertiary bronchial injury (n = 1) was missed by initial tracheogram and subsequent bronchoscopy but identified during surgical exploration. All children survived after thoracotomy and primary repair of the injury. CONCLUSIONS Tracheobronchial disruption is a rare, life-threatening injury. Suspicion should be high when pneumomediastinum and pneumothorax are refractory to adequate pleural drainage. Flexible bronchoscopy with intubation distal to the injury may be necessary to prevent loss of the airway. Advance preparation should include setups for bronchoscopy, thoracotomy, and cardiopulmonary bypass. Patient survival depends on preparation and prompt surgical intervention.

Hepatoblastoma state of the art: pathology, genetics, risk stratification, and chemotherapy.

Purpose of review As a rare pediatric tumor, hepatoblastoma presents challenges to the individual practitioner as no center will see more than a handful of cases each year. Recent findings The Childrens Hepatic tumor International Collaborative (CHIC) effort has fostered international cooperation in this rare childrens tumor, leading to the establishment of a large international collaborative dataset, the CHIC database, which has been interrogated to refine risk stratification and inform treatment options. Apace with this effort has been the international collaboration of pediatric pathologists working together to establish a new international histopathologic consensus classification for pediatric liver tumors as a whole, with particular focus on the histological subtypes of hepatoblastoma. Summary International collaborative efforts in hepatoblastoma have led to a new international histopathologic consensus classification, refinements in risk stratification, advances in chemotherapy, and a better understanding of surgical resection options forming the foundation for the development of an upcoming international therapeutic trial.

Elimination of soybean lipid emulsion in parenteral nutrition and supplementation with enteral fish oil improve cholestasis in infants with short bowel syndrome.

BACKGROUND Parenteral nutrition-associated liver disease (PNALD) is a potentially fatal complication for children with intestinal failure. Fish oil-based lipid emulsions have shown promise for the treatment of PNALD but are not readily available. Six cases are presented in which cholestasis resolved after soybean lipid emulsion (SLE) was removed from parenteral nutrition (PN) and enteral fish oil was given. METHODS A retrospective review at a tertiary childrens hospital (July 2003 to August 2008) identified 6 infants with intestinal failure requiring PN for >6 months who developed severe hepatic dysfunction that was managed by eliminating SLE and providing enteral fish oil. RESULTS Twenty-three infants with short bowel syndrome requiring prolonged PN developed cholestasis. SLE was removed in 6 of these patients, and 4 of the 6 received enteral fish oil. Standard PN included 2-3 g/kg/d SLE with total PN calories ranging from 57 to 81 kcal/kg/d at the time of SLE removal. Hyperbilirubinemia resolved after elimination of SLE within 1.8-5.4 months. Total PN calories required to maintain growth generally did not change. CONCLUSIONS Temporary elimination of SLE and supplementation with enteral fish oil improved cholestasis in PN-dependent infants. Further trials are needed to evaluate this management strategy.

Hepatoblastoma state of the art: pre-treatment extent of disease, surgical resection guidelines and the role of liver transplantation.

Purpose of review This is part two of a two-part state of the art – hepatoblastoma. International hepatoblastoma specialists were brought together to highlight advances, controversies, and future challenges in the treatment of this rare pediatric tumor. Recent findings Pretreatment extent of disease (PRETEXT) is a grouping system introduced as part of the multicenter international childhood liver tumors strategy group, SIOPEL-1, study in 1990. The system has been refined over the ensuing years and has now come to be adopted for risk stratification by all of the major pediatric liver tumor multicenter trial groups. PRETEXT is being intensively studied in the current Childrens Oncology Group (COG) AHEP-0731 trial in an attempt to validate interobserver reproducibility and ability to monitor response to neoadjuvant chemotherapy, and determine surgical resectability. PRETEXT is now used to identify those patients who are at risk for having an unresectable tumor and who should be referred to a liver specialty center with transplant capability early in their treatment schema. Summary International collaborative efforts in hepatoblastoma have led to increased refinements in the use of the PRETEXT and post-treatment extent to define prognosis and surgical resectability. PRETEXT criteria which suggest a possible need for liver transplantation are discussed in detail.

Critical review of controversial issues in the management of advanced pediatric liver tumors

Hepatocellular carcinoma (HCC) and hepatoblastoma (HB) are the most common primary tumors of liver in children. The management of patients with locally advanced, unresectable disease or those with extra‐hepatic distant metastases provides substantial challenges to pediatric oncologists, hepatologists, and surgeons. Herein, we critically debate the two sides of three specific controversies: (1) the role of chemotherapy in the treatment of advanced pediatric HCC; (2) the indications for liver transplantation in children with HCC, specifically, the appropriateness of using adult Milan criteria; and (3) the role of liver trasplantation in children with unresectable HB that present with metastatic disease. Pediatr Blood Cancer 2011;56:1013–1018.

Decompressive laparotomy for abdominal compartment syndrome in children: before it is too late

PURPOSE Abdominal compartment syndrome (ACS) in children is an infrequently reported, rapidly progressive, and often lethal condition underappreciated in the pediatric population. This underrecognition can result in a critical delay in diagnosis causing increased morbidity and mortality. This study examines the clinical course of patients treated for ACS at our institution. METHODS A review of children requiring an emergency laparotomy (n = 264) identified 26 patients with a diagnosis of ACS. ACS was defined as sustained intraabdominal hypertension (bladder pressure >12 mm Hg) that was associated with new onset organ dysfunction or failure. RESULTS Patients ranged in age from 3 months to 17 years old and were cared for in the pediatric intensive care unit (PICU). Twenty-seven percent (n = 7) were transferred from referring hospitals, 50% (n = 13) were admitted directly from the emergency department, and 23% (n = 6) were inpatients before being transferred to PICU. Admission diagnoses included infectious enterocolitis (n = 12), postsurgical procedure (n = 10), and others (n = 4). Patients progressed to ACS rapidly, with most requiring decompressive laparotomy within 8 hours of PICU admission (range, <1-96 hours). Preoperatively, all patients had maximum ventilatory support and oliguria, 85% (n = 22) required vasopressors/inotropes, and 31% (n = 8) required hemodialysis. Mean bladder pressure was 25 mm Hg (range, 12-44 mm Hg). In 42% (n = 11), cardiac arrest preceeded decompressive laparotomy. All patients showed evidence of tissue ischemia before decompressive laparotomy with an average preoperative lactate of 8 (range, 1.2-20). Decompressive laparotomy was done at the bedside in the PICU in 13 patients and in the operating room in 14 patients. Abdominal wounds were managed with open vacuum pack or silastic silo dressings. Physiologic data including fluid resuscitation, oxygen index, mean airway pressure, vasopressor score, and urine output were recorded at 6-hour intervals beginning 12 hours before decompressive laparotomy and extending 12 hours after operation. The data demonstrate improvement of all physiologic parameters after decompressive laparotomy except for urine output, which continued to be minimal 12 hours post intervention. Mortality was 58% (n = 15) overall. The only significant factor related to increased mortality was bladder pressure (P = .046; odds ratio, 1.258). Cardiac arrest before decompressive laparotomy, need for hemodialysis, and transfer from referring hospital also trended toward increased mortality but did not reach significance. CONCLUSION Abdominal compartment syndrome in children carries a high mortality and may be a consequence of common childhood diseases such as enterocolitis. The diagnosis of ACS and the potential need for emergent decompressive laparotomy may be infrequently discussed in the pediatric literature. Increased awareness of ACS may promote earlier diagnosis, treatment, and possibly improve outcomes.

Neurologically intact children with an isolated skull fracture may be safely discharged after brief observation

PURPOSE The management of children presenting with an isolated skull fracture (ISF) posttrauma is highly variable. We sought to estimate the risk of neurologic deterioration in children with a Glasgow coma score (GCS) 15 and ISF to reduce unnecessary hospital admissions. METHODS A retrospective review at a level I pediatric trauma referral center was conducted for patients with ISF on head computed tomography from 2003 to 2008. Patients were excluded for injury greater than 24 hours prior, GCS less than 15, intracranial pathology, significant fracture depression, or complex fractures involving facial bones or skull base. RESULTS A total of 235 patients were identified with an ISF. The median age was 11 months, with falls accounting for 87% of the injuries. One hundred seventy-seven patients were admitted, and 58 patients were discharged from the emergency department after a period of observation (median, 3.3 hours). Median length of stay for those admitted to the hospital was 18.2 hours. One patient developed vomiting following overnight observation and a repeat computed tomography scan demonstrated a small extra-axial hematoma that required no intervention. The mean total costs for patients discharged from the emergency department were