Max R. Langham

Congenital diaphragmatic hernia : Epidemiology and outcome

Congenital diaphragmatic hernia is a relatively common birth defect. It affects about 1114 babies a year in the United States. Reported survival averages 60% but may be significantly lower. We do not understand the etiology of CDH. Its association with other anomalies and several distinct patterns of presentation suggest that more than one cause may exist. There is a high degree of variability in both treatment and outcomes, but no data exist to allow a rigorous comparison of the efficacy of various treatment strategies. Stratification of patients into more homogeneous groups will be a necessary prerequisite for the design of meaningful comparative trials. The incidence of the lesion prevents any single institution from accruing sufficient patients to conduct such a trial. An ad hoc multicenter study group (the Congenital Diaphragmatic Hernia Study Group) has been formed for this purpose. This organization has begun collecting data with an initial goal of developing a stratification scheme. Prospective data collection should allow verification of several of the estimates made in this article. Current data make it clear that CDH represents a major cause of perinatal morbidity and mortality.

Current surgical management of congenital diaphragmatic hernia: A report from the congenital diaphragmatic hernia study group

BACKGROUND Repair of congenital diaphragmatic hernia (CDH) has changed from an emergent procedure to a delayed procedure in the last decade. Many other aspects of management have also evolved since the first successful repair. However, most reports are from single institutions. The lack of a large multicenter database has hampered progress in the management of congenital diaphragmatic hernia (CDH) and makes determination of the current standard difficult. METHODS The CDH study group was formed in 1995 to collect data from multiple institutions in North America, Europe, and Australia. Participating centers completed a registry form on all live-born infants with CDH during 1995 and 1996. Demographic information, data about surgical management, and outcome were collected for all patients. RESULTS Sixty-two centers participated, with 461 patients entered. Overall survival was 280 of 442 patients (63%) where survival was recorded. The defect was left-sided in 78%, right-sided in 21%, and bilateral in 1%. A subcostal approach was used in 91% of patients, with pleural drainage used in 76%. A patch of some kind was used in just over half (51%) of the patients, with polytetrafluoroethylene being the most commonly used material (81%) in those patients with a patch. The mean surgical time was 102 minutes, with an average blood loss of 14 mL (range, 0 to 500 mL). The overwhelming majority of patients underwent repair between 6:00 AM and 6:00 PM (289 of 329, 88%). Nineteen percent of patients had surgical repair on extracorporeal membrane oxygenation (ECMO) at a mean time of 170 hours into the ECMO course (range, 10 to 593 hours). The mean age at surgery in patients not treated with ECMO was 73 hours (range, 1 to 445 hours). CONCLUSIONS The multicenter nature of this report makes it a snapshot of current management. The data would indicate that prosthetic patching of the defect has become common, that after-hours repair is infrequent, and that delayed surgical repair has become the preferred approach in many centers. Furthermore, the mean survival rate of 63% indicates that despite decades of individual effort, the CDH problem is far from solved. This highlights the need for a centralized database and cooperative multicenter studies in the future.

Postoperative outcomes of extremely low birth-weight infants with necrotizing enterocolitis or isolated intestinal perforation: A prospective cohort study by the NICHD Neonatal Research Network

Objective:Purposes of this study were: 1) to compare mortality and postoperative morbidities (intra-abdominal abscess, wound dehiscence, and intestinal stricture) in extremely low birth weight (ELBW) infants who underwent initial laparotomy or drainage for necrotizing enterocolitis (NEC) or isolated intestinal perforation (IP); 2) to determine the ability to distinguish NEC from IP preoperatively and the importance of this distinction on outcome measures; and 3) to evaluate the association between extent of intestinal disease determined at operation and outcome measures. Background:ELBW infants who undergo operation for NEC or IP have a postoperative, in-hospital mortality rate of approximately 50%. Whether to perform laparotomy or drainage initially is controversial. Also unknown is the importance of distinguishing NEC from IP and the current ability to make this distinction based on objective data available prior to operation. Methods:A prospective, multicenter cohort study of 156 ELBW infants at 16 neonatal intensive care units (NICU) within the NICHD Neonatal Research Network. Results:Among the 156 enrolled infants, 80 underwent initial peritoneal drainage and 76 initial laparotomy. Mortality rate was 49% (76 of 156). Ninety-six patients had a preoperative diagnosis of NEC and 60 had presumed IP. There was a high level of agreement between the presumed preoperative diagnosis and intraoperative diagnosis in patients undergoing initial laparotomy (kappa = 0.85). The relative risk for death with a preoperative diagnosis of NEC (versus IP) was 1.4 (95% confidence interval, 0.99–2.1, P = 0.052). The overall incidence of postoperative intestinal stricture was 10.3%, wound dehiscence 4.4%, and intra-abdominal abscess 5.8%, and did not significantly differ between groups undergoing initial laparotomy versus initial drainage. Conclusions:Survival to hospital discharge after operation for NEC or IP in ELBW neonates remains poor (51%). Patients with a preoperative diagnosis of NEC have a relative risk for death of 1.4 compared with those with a preoperative diagnosis of IP. A distinction can be made preoperatively between NEC and IP based on abdominal radiographic findings and the patients age at operation. Future randomized trials that compare laparotomy versus drainage would likely benefit from stratification of treatment assignment based on preoperative diagnosis.

Detrimental Effects of Standard Medical Therapy in Congenital Diaphragmatic Hernia

OBJECTIVE To evaluate the impact of a nonstandard ventilation strategy on survival in congenital diaphragmatic hernia (CDH). BACKGROUND Despite recent advances, including nitric oxide, CDH remains an unsolved problem with a mortality rate of 35% to 50%. Hyperventilation and alkalization remain common therapies. METHODS In 1992, the authors prospectively abandoned hyperventilation and alkalization. Patients are lightly sedated and ventilated with the lowest pressure providing adequate chest movement, and the rate is set to patient comfort. Nitric oxide and extracorporeal membrane oxygenation (ECMO) are reserved for life-threatening instability. Surgical repair is delayed 1 to 5 days. Sixty consecutive patients are compared with 29 previous patients treated with hyperventilation and alkalization, 13 before and 16 after the availability of ECMO. RESULTS Overall, 47 of 60 patients (78%) in study era 3 survived compared with 2 of 13 (15%) in the hyperventilation era and 7 of 16 (44%) in the hyperventilation/ECMO era (p < 0.0001). The disease severity and the incidence of associated anomalies did not differ between groups. To compare management strategies, patients who had treatment withheld because of lethal associated conditions were then removed from analysis. Peak inspiratory pressure and arterial pH were lower (p < 0.0001) and Paco2 was higher (p < 0.05) in era 3 than in the previous eras. The rate of pneumothorax (1.9%) decreased (p < 0.0001). In era 3, survival was 47 of 53 (89%) treated patients, and 23 of 25 inborn patients with isolated CDH survived (92%). CONCLUSIONS Nonstandard ventilatory support of patients with CDH has led to significantly improved survival rates. This study sets a survival benchmark and strongly suggests the negative effects of hyperventilation and alkalization.

Combined Resection of the Liver and Inferior Vena Cava for Hepatic Malignancy

Objective:The objective of this paper is to review the results of combined resection of the liver and inferior vena cava for hepatic malignancy. The morbidity and mortality along with preliminary survival data are assessed in order to determine the utility of this aggressive approach to otherwise unresectable tumors. Summary Background Data:Involvement of the inferior vena cava has traditionally been considered a contraindication to resection for advanced tumors of the liver because the surgical risks are high and the long-term prognosis is poor. Progress in liver surgery allows resection in some cases. Methods:Twenty-two patients undergoing hepatic resection from 1997 to 2003, that also required resection and reconstruction of the inferior vena cava (IVC), were reviewed. The median age was 49 years (range 2 to 68 years). Resections were carried out for: hepatocellular carcinoma (n = 6), colorectal metastases (n = 6), cholangiocarcinoma (n = 5), gastrointestinal stromal tumor (n = 2), hepatoblastoma (n = 2), and squamous cell carcinoma in 1 patient. Liver resections performed included 13 right trisegmentectomies, 6 right lobectomies extended to include the caudate lobe, and 3 left trisegmentectomies. Complex ex vivo procedures were performed in 2 cases using venovenous bypass while the other 20 cases were performed using varying degrees of vascular isolation. In situ cold perfusion of the liver was used in 1 case. The IVC was reconstructed with ringed Gore-Tex tube graft (n = 14), primarily (n = 6), or with Gore-Tex patches (n = 2). Results:There were 2 perioperative deaths (9%). One cirrhotic patient died of liver failure 3 weeks post operatively and 1 patient with cholangiocarcinoma died of pulmonary hemorrhage secondary to a cavitating pulmonary infection after aspiration pneumonia 6 weeks after resection. Six patients had evidence of postoperative liver failure that resolved with supportive management and 2 patients required temporary dialysis. All vascular reconstructions were patent at last follow-up. With median follow-up of 26 months, 5 patients have died of recurrent malignancy at 44, 40, 32, 26, and 24 months, while an additional patient is alive with disease at 31 months. Actuarial 1-, 3-, and 5-year survivals were 85%, 60%, and 33%, respectively. Conclusions:IVC involvement by hepatic malignancy does not necessarily preclude resection. Liver resection with reconstruction of the inferior vena cava can be performed in selected cases. The increased risk associated with the procedure appears to be balanced by the possible benefits, particularly when the lack of alternative curative approaches is considered.

Hepatic undifferentiated (embryonal) sarcoma arising in a mesenchymal hamartoma.

We report the case of a hepatic undifferentiated (embryonal) sarcoma (UES) arising within a mesenchymal hamartoma (MH) in a 15-year-old girl. Mapping of the tumor demonstrated a typical MH transforming gradually into a UES composed of anaplastic stromal cells. When evaluated by flow cytometry, the MH was diploid and the UES showed a prominent aneuploid peak. Karyotypic analysis of the UES showed structural alterations of chromosome 19, which have been implicated as a potential genetic marker of MH. The histogenesis of MH and UES is still debated, and reports of a relationship between them, although suggested on the basis of histomorphologic similarities, have never been convincing. The histologic, flow cytometric, and cytogenetic evidence reported herein suggests a link between these two hepatic tumors of the pediatric population.

Hepatic vein reconstruction for resection of hepatic tumors

Summary Background DataInvolvement of the hepatic veins requiring reconstruction has traditionally been considered a contraindication to resection for advanced tumors of the liver because the surgical risks are high and the long-term prognosis poor. Recent advances in liver surgery gleaned from split and live donor liver transplantation that necessitate hepatic vein reconstruction can be applied to hepatic resection in some cases. MethodsSixteen patients who underwent hepatic resection requiring hepatic vein reconstruction from 1996-2001 were reviewed. The mean age was 43 years (range 2–61). Nine patients were resected for hepatocellular carcinoma (HCC), five patients for colorectal metastases, and one patient each for hepatoblastoma and cholangiocarcinoma. In six patients with HCC and cirrhosis, the right hepatic vein was reconstructed to provide venous outflow to liver segments not adequately drained by a remaining major hepatic vein. Four of these six patients required the use of Gore-Tex (W. L. Gore & Associates, Inc., Newark, DE) interposition grafts. In the 10 other cases the entire venous outflow from the remnant liver was reconstructed or reimplanted into the inferior vena cava primarily (n = 8) or using segments of the portal vein from the resected side of the liver as a graft (n = 2). Ex-vivo procedures with the use of veno-venous bypass were required in two cases and in-situ cold perfusion of the liver was used in one case. ResultsThere were two perioperative deaths (12%). One patient died of liver failure 3 weeks after right trisegmentectomy with reconstruction of the left hepatic vein and one patient died at 3 months after resection due to sepsis from a segment of small bowel that perforated into a diaphragmatic hernia. Four patients had evidence of postoperative liver failure that resolved with supportive management and one patient required temporary dialysis. All vascular reconstructions were patent at last followup. With median followup of 23 months, 3 patients have died of recurrent malignancy at 14, 18 and 30 months, while an additional patient went on to die of progressive liver failure at 22 months. Actuarial 1 and 3 year survival was 88% and 50% respectively. ConclusionHepatic vein involvement by hepatic malignancy does not necessarily preclude resection. Liver resection with reconstruction of the hepatic veins can be performed in selected cases. The increased risk associated with the procedure appears to be balanced by the possible benefits, particularly when the lack of alternative curative approaches is considered.

Early vs interval appendectomy for children with perforated appendicitis.

OBJECTIVE To compare the effectiveness and adverse event rates of early vs interval appendectomy in children with perforated appendicitis. DESIGN Nonblinded randomized trial. SETTING A tertiary-referral urban childrens hospital. PATIENTS A total of 131 patients younger than 18 years with a preoperative diagnosis of perforated appendicitis. INTERVENTIONS Early appendectomy (within 24 hours of admission) vs interval appendectomy (6-8 weeks after diagnosis). MAIN OUTCOME MEASURES Time away from normal activities (days). Secondary outcomes included the overall adverse event rates and the rate of predefined specific adverse events (eg, intra-abdominal abscess, surgical site infection, unplanned readmission). RESULTS Early appendectomy, compared with interval appendectomy, significantly reduced the time away from normal activities (mean, 13.8 vs 19.4 days; P < .001). The overall adverse event rate was 30% for early appendectomy vs 55% for interval appendectomy (relative risk with interval appendectomy, 1.86; 95% confidence interval, 1.21-2.87; P = .003). Of the patients randomized to interval appendectomy, 23 (34%) had an appendectomy earlier than planned owing to failure to improve (n = 17), recurrent appendicitis (n = 5), or other reasons (n = 1). CONCLUSIONS Early appendectomy significantly reduced the time away from normal activities. The overall adverse event rate after early appendectomy was significantly lower compared with interval appendectomy. TRIAL REGISTRATION clinicaltrials.gov Identifier: NCT00435032.

Hepatocellular carcinoma in 2 young adolescents with chronic hepatitis C.

Hepatitis C virus (HCV) infection is a global health problem affecting 170 million individuals worldwide. In the United States, there are approximately 7 million adults and 100,000 children chronically infected with HCV (1,2). The importance of HCV infection stems from its proclivity to cause insidious liver damage over many years, including chronic hepatitis, cirrhosis, and liver cancer. In adults, HCV infection is a leading cause for liver cancer worldwide (3). The financial burden of this viral infection is staggering, with projected medical costs of

Standardized reporting for congenital diaphragmatic hernia – An international consensus

10.7 billion in adults from 2010 to 2019 and approximately