Embryonal rhabdomyosarcoma of the ampulla of vater with long-term survival following pancreaticoduodenectomy

Rhabdomyosarcoma of the biliary tree is a rare cause of biliary tract obstruction in childhood. A 3-year-old child is reported here after presenting with obstructive jaundice secondary to an embryonal rhabdomyosarcoma of the ampulla of Vater. He underwent pancreaticoduodenectomy followed by adjuvant chemotherapy and irradiation. He is now well and free of disease 5 years following treatment. This child appears to be the first long-term survivor who has required pancreaticoduodenal resection for this lesion.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/28946/1/0000783.pd

Idiopathic gastric infarction in childhood

The case of a 6-year-old female with gastric infarction is presented. Despite a careful evaluation, no etiology could be determined in this child or, retrospectively, in several similar children described by other authors in the past. Vascular compromise was present although it is not possible to know whether this was a primary or a secondary event. It is important to recognize that gastric infarction can occur in children without obvious explanation.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/47178/1/383_2004_Article_BF00182780.pd

The role of oxygen radicals in immune complex injury

In this review we will summarize our current understanding of the mediation of immune complex induced tissue injury. Comparisons will be made between the mediation of IgG versus IgA immune complex injury with emphasis on the role that reactive oxygen products derived from leucocytic phagocytic cells play in the initiation of the tissue injury.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/27557/1/0000601.pd

Anti-tumor necrosis factor antibody augments edema formation in caerulein-induced acute pancreatitis,

The pathogenesis of acute pancreatitis is incompletely defined, but the outcome is determined in part by an acute inflammatory process. Pancreatitis-associated inflammation appears to play a role in the local retroperitoneal injury as well as in the associated dysfunction of remote organs such as the lung. Tumor necrosis factor (TNF) appears to be a proximal mediator of the inflammatory response. In this study, anti-TNF antibody was administered to rats with caerulein-induced pancreatitis to determine if the observed increases in pancreatic and pulmonary microvascular permeability were related to plasma TNF activity. In contrast to the expected findings, blockade of TNF activity was found to increase the amount of edema formation in both the pulmonary and pancreatic microvascular beds. The mechanism is not known; however, blockade of TNF-induced down regulation of phagocytic cell activity, ablation of TNF-dependent feedback inhibition of other cytokines, failure of induction of endogenous antioxidant systems, or inactivation of the TNF control of microvascular tone are all possible explanations. This is potentially an important observation as clinical strategies are now being developed to modify the inflammatory response in ways presumed advantageous to an injured host.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/29016/1/0000045.pd

Redo fundoplication in infants and children with recurrent gastroesophageal reflux

The Nissen fundoplication is well established as the surgical treatment for medically refractory gastroesophageal reflux (GER) in childhood. Recurrent GER following fundoplication is a challenging problem with a reported incidence ranging from 0% to 12%. From January 1974 to January 1989, 286 children have been treated for GER with Nissen fundoplication and gastrostomy tube placement at the University of Michigan C.S. Mott Children's Hospital; 242 of these children have been followed for an average of 30 months, the remaining 44 have been lost to follow-up. Twenty-nine children (12%) have developed recurrent reflux following fundoplication. Medical management with thickened upright feelings, gastrostomy feedings, or gastrojejunostomy tube feedings has been successful for 11 children with control of reflux symptoms. Five additional children who were treated nonoperatively died of coexistant medical problems within 2 months following documentation or recurrent reflux. The remaining 13 children have required redo fundoplication for wrap disruption or herniation, and an additional six children, initially treated at other institutions, have also undergone redo fundoplications. One other child treated at this hospital required redo fundoplication for a postoperative partial gastric volvulus causing gastric outlet obstruction. Of the 20 children who have undergone a second Nissen fundoplication, 16 (80%) are doing well without recurrent GER. Four children have developed recurrent GER with wrap disruption; 1 is doing well following a third fundoplication, 2 have been managed successfully with continuous feedings via gastrojejunostomy feeding tubes, and a fourth child died of complications related to a recurrent tracheoesophageal fistula. Conservative management with gastrojejunal tube feedings should be considered in the initial management of children with recurrent GER following fundoplication. If necessary, redo Nissen fundoplication, although technically difficult, is associated with a good outcome in the majority of children and is indicated for refractory recurrent GER following a failed antireflux procedure.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/29266/1/0000324.pd

Use of high-frequency jet ventilation in the management of congenital tracheoesophageal fistula associated with respiratory distress syndrome

Two preterm infants (28 weeks, 960 g; 32 weeks, 1,870 g) with very large tracheoesophageal fistulas suffered from respiratory distress syndrome and falled to respond to conventional mechanical ventilation despite placement of a decompressive gastrostomy. Pulmonary air leaks developed in both, resulting in transdiaphragmatic pneumoperitoneum, and significant gas flow occurred through the gastrostomy tube despite placement under water-seal. High-frequency jet ventilation was instituted in each case and resulted in improved pulmonary gas exchange at lower mean airway pressures (12.0 to 6.7 cm H2O; 11.0 to 8.0 cm H2O) and in prompt resolution of air leaks. Both patients remained refractory to reinstitution of conventional ventilation until division of the fistula in the first patient and complete resolution of the respiratory distress syndrome in the second.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/28945/1/0000782.pd

Hepatocellular oxidant stress following intestinal ischemia-reperfusion injury,

Reperfusion of ischemic intestine results in acute liver dysfunction characterized by hepatocellular enzyme release into plasma, reduction in bile flow rate, and neutrophil sequestration within the liver. The pathophysiology underlying this acute hepatic injury is unknown. This study was undertaken to determine whether oxidants are associated with the hepatic injury and to determine the relative value of several indirect methods of assessing oxidant exposure in vivo. Rats were subjected to a standardized intestinal ischemia-reperfusion injury. Hepatic tissue was assayed for lipid peroxidation products and oxidized and reduced glutathione. There was no change in hepatic tissue total glutathione following intestinal ischemia--reperfusion injury. Oxidized glutathione (GSSG) increased significantly following 30 and 60 min of reperfusion. There was no increase in any of the products of lipid peroxidation associated with this injury. An increase in GSSG within hepatic tissue during intestinal reperfusion suggests exposure of hepatocytes to an oxidant stress. The lack of a significant increase in products of lipid peroxidation suggests that the oxidant stress is of insufficient magnitude to result in irreversible injury to hepatocyte cell membranes. These data also suggest that the measurement of tissue GSSG may be a more sensitive indicator of oxidant stress than measurement of products of lipid peroxidation.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/29014/1/0000043.pd

Pulmonary endothelial cell ATP depletion following intestinal ischemia

Multiple organ failure (MOF) is known to follow systemic inflammatory mediator activation associated with intestinal ischemia--reperfusion injury. In particular, the pulmonary microvasculature appears to be susceptible to MOF-related injury. This study was designed to evaluate the hypothesis that non-cellular plasma factors associated with intestinal ischemia without reperfusion also mediate pulmonary endothelial cell injury. Male Sprague--Dawley rats had intestinal ischemia induced by microvascular clip occlusion of the superior mesenteric artery for 30, 60, 90, or 120 min. Following each period of ischemia, plasma samples were obtained from the portal vein. Time-matched sham-operated animals served as controls. Monolayers of cultured rat pulmonary artery endothelial cells were then incubated with the plasma samples and ATP levels determined using a luciferin--luciferase assay. A 51Cr-release assay using labeled endothelial cells was performed under identical conditions to assess cytotoxicity. Endothelial cell ATP levels were 1.99 +/- 0.23 x 10-11 mole/[mu]g DNA in sham preparations. After a 4-hr incubation in plasma from the 90 and 120 min ischemia groups, cellular ATP levels fell significantly to 1.07 +/- 0.23 x 10-11 mole/[mu]g DNA, respectively (P P = NS). All animals survived 24 hr in the sham, 30, and 60 min groups. However, survival was 40 and 0% in the 90 and 120 min groups, respectively (P in vitro data with in vivo survival.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/30022/1/0000390.pd

Efficacy of anterior gastric fundoplication in the treatment of gastroesophageal reflux in infants and children

Anterior gastric fundoplication (AGF) has been performed at the University of Michigan since 1988. To objectively evaluate the long-term efficacy of the AGF, the authors performed a study of children who had undergone AGF between June 1988 and June 1990 (n = 46). Six of them died of unrelated causes. Twenty-two consented to follow-up evaluation that included parental interview, physical examination, upper gastrointestinal series (UGI), 24-hour esophageal pH probe monitoring (EpH), and a liquid-phase gastric emptying study. Twenty patients (74%) remained asymptomatic, patients exhibited gastroesophageal reflux (GER) by UGI, and three others by EpH. Three children were noted to have delayed gastric emptying. These results compare favorably with data previously reported from this institution of a 5-year follow-up of children after Nissen fundoplication. There is a trend toward improved efficacy (87% v 74%; P = .12), decreased reoperation rate (4% v 14%; P = .11), and less severe complications. The present study shows that AGF is effective treatment for GER when evaluated by objective studies and is comparable in therapeutic efficacy and safety to the Nissen fundoplication.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/31949/1/0000902.pd

Choledochocele: an unusual form of choledochal cyst

Choledochocele, or type III choledochal cyst, is a rare anomaly. Two children with choledochocele, both younger than any previously reported patient, were recently cared for at the University of Michigan Medical Center and prompted a literature review of this subject. Since 1974, 40 cases of choledochocele have been reported with enough clinical information for critical review. Ten of these patients were 21 years of age or younger. Presenting symptoms were not specific for choledochocele; they were generally interpreted to result from other biliary or gastrointestinal disorders that are more common for each age group. In pediatric patients the most frequent signs and symptoms of choledochocele were abdominal pain (70%), nausea and/or vomiting (60%), jaundice (30%), and acute pancreatitis (30%). While two-thirds of adult patients with choledochocele had undergone prior cholecystectomy (with stones rarely found), this was observed only once in children. Obstructive symptoms led to evaluation of the stomach and duodenum with either barium upper GI series or endoscopy in all children. These demonstrated an extrinsic mass effect in 90% of the patients. Endoscopic retrograde cholangiopancreatography identified a choledochocele in all cases in which the study was successfully executed. Intravenous cholangiography was sensitive in children, but less so in the adult patients reviewed. Other imaging efforts (computerized tomography, ultrasound, radionuclide scanning) were less dependable. Transduodenal marsupialization is the treatment of choice for patients of all ages and was provided in both of these newly reported children.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/47183/1/383_2004_Article_BF00179633.pd