Improved survival in children with esophageal perforation.

OBJECTIVE: To analyze the cause, location, signs and symptoms, presence of underlying disease, time interval to diagnosis, treatment, and morbidity and mortality in 24 children (19 boys and 5 girls) with esophageal perforation who were treated from 1975 to 1995. DESIGN: Data were collected retrospectively from hospital and office records. SETTING: A tertiary care children's hospital. RESULTS: The average age at diagnosis was 58 months (range, 1 day to 19 years). Fourteen children had underlying esophageal disease (atresia, n = 7 and gastroesophageal reflux, n = 7). Iatrogenic perforations occurred in 17 children: 8 during dilatation, 5 during an antireflux procedure, 2 during endoscopy, and 2 after passage of a feeding tube. Trauma was the cause of perforation in 6 children. In 2 cases the cause was unknown. Perforation occurred in the thoracic esophagus in 12 cases, abdominal esophagus in 7, cervical esophagus in 5, and involved both the thoracic and abdominal esophagus in 1. Signs and symptoms included dysphagia (15 patients), dyspnea (14), fever (12), cyanosis (8), abdominal pain (6), chest pain (5), and subcutaneous emphysema (3). Management of esophageal perforation included nonoperative management (7 patients), drainage alone (1), primary closure (16), and resection and diversion (1). Two perforations occurred in 1 child. Complications occurred in 11 (44%) of the 25 cases and were more common after delayed diagnosis (73%). The average hospital stay was 20 days. There was 1 death (4%) attributed to esophageal perforation. CONCLUSIONS: Morbidity and mortality are directly related to delays in diagnosis and therapy. Most cases of esophageal perforation in children can be closed primarily and the esophagus salvaged despite delayed presentation. The mortality rate in children with esophageal perforation (4%) is significantly less than that for adults (25%-50%).

Avoidance of stomas and delayed anastomosis for bowel necrosis: the 'clip and drop-back' technique.

Necrotizing enterocolitis (NEC) and midgut volvulus (MGV) often are associated with extensive bowel necrosis. These cases may require extensive enterectomy and the formation of high or multiple stomas, and frequently are complicated by short bowel syndrome, excessive fluid losses, fistulas, stenosis, and skin breakdown. This report describes a "clip and drop-back" technique, followed by delayed anastomosis performed 48 to 72 hours later. The technique was successful in five severely ill infants (3 NEC, 2 MGV) with extensive necrosis, bowel perforation(s), and peritonitis, who required either a high stoma near the ligament of Treitz or multiple resections and enterostomies. This method removes obvious necrotic perforated bowel, controls contamination, avoids stomas (and their inherent complications in this age group), and preserves bowel length. All five babies survived. The technique is a useful addition to the pediatric surgeon's operative armamentarium in selective cases.

Favorable outcome in children with Beckwith-Wiedemann syndrome and intraabdominal malignant tumors.

Children with Beckwith-Wiedemann syndrome (BWS) have an increased risk of developing Wilms' tumors, hepatoblastomas, and adrenal tumors. This study evaluates disease-free survival in children with BWS and intraabdominal tumors. Sixteen tumors occurred in 13 children with BWS (8 boys, 5 girls). Diagnoses included Wilms' tumor (10) (2 bilateral, 20%), hepatoblastoma (2), bladder rhabdomyosarcoma (1), and adrenal cortical tumor (1). In the 10 children with Wilms' tumor, the average age at diagnosis was 3.5 years (range, 7 months to 5 years). Nine of 10 had initial tumor resection, chemotherapy, and radiation therapy (when indicated). One child with bilateral disease had tumor biopsy, chemotherapy, and partial nephrectomy. Tumors were classified as stage I (5), stage II (2), stage IV (1) and stage V (2), all with favorable histology. Disease-free survival rate was 100% with median follow-up of 9 years (range, 4 to 22 years). One patient had a left adrenal tumor detected during screening sonography 11 years after Wilms' tumor resection. Two infants with advanced-stage hepatoblastoma responded to chemotherapy, allowing subsequent complete hepatic resection. Both tumors had unfavorable histology. Both completed postoperative chemotherapy and have no evidence of disease (NED) with normal alpha-fetoprotein levels at 21 and 12 months, respectively, after tumor detection. One patient with stage III (group 3) bladder rhabdomyosarcoma underwent partial cystectomy following chemoradiation and is alive (NED) after 20 months. Children with BWS should be screened at regular intervals (every 3 to 6 months) for renal, adrenal, and hepatic tumors. The exact duration of screening is not yet determined.(ABSTRACT TRUNCATED AT 250 WORDS)

The role of spiral (helical) computerized tomography with three-dimensional reconstruction in pediatric solid tumors.

The ability to accurately assess tumor size and orientation to surrounding vital structures is an important consideration during preoperative evaluation. The authors report on nine children with solid tumors (hepatoblastoma [1], neuroblastoma [2], adrenal cortical carcinoma [2], liver adenoma [1], primitive neuroectodermal tumor [PNET] [1], and stage V Wilms' tumor [2]) for whom tumor resectability was questioned because of the tumors' close proximity to major blood vessels (noted through conventional radiographic imaging). The children had scanning with spiral volumetric acquisition computerized tomography, (CT) which obtains images during continuous rotation of the x-ray source while the patient moves at a constant velocity through the gantry. This technique is rapid (18 to 30 seconds), and is similar with respect to radiation exposure; little or no sedation is required, and the contrast dose is lower than that of conventional CT. Three-dimensional reconstruction of spiral CT imaging provided useful information that allowed successful resection in all nine cases. The authors suggest that spiral CT may become an important imaging modality in the preoperative evaluation of pediatric solid tumors and that further evaluation of this new methodology is warranted.

Pentoxifylline protects splanchnic prostacyclin synthesis during mesenteric ischemia/reperfusion.

This study examines the hypothesis that pentoxifylline protects splanchnic PGI2 synthesis during severe mesenteric ischemia/reperfusion injury. Anesthetized Sprague-Dawley rats (300 grams) were subjected to sham or superior mesenteric artery occlusion for 20 minutes followed by 30 minutes of reperfusion. The ischemia/reperfusion groups received either enteral allopurinol (10 mg/kg) daily for 5 days prior to ischemia, PTX (50 mg/kg) 10 minutes prior to ischemia or carrier. The superior mesenteric artery was cannulated and removed with its intact intestine (SV + SI). The SV + SI was perfused in vitro with oxygenated Krebs buffer. The venous effluent was collected and assayed for release of 6-keto-PGF1 alpha, PGE2 and thromboxane B2 by enzyme immunoassay. Severe mesenteric ischemia/reperfusion decreased SV + SI 6-keto-PGF1 alpha release by 40% compared to the sham group but did not alter release of PGE2 or thromboxane B2. Pretreatment of the animals with PTX and not allopurinol preserved SV + SI 6-keto-PGF1 alpha release at all times of perfusion to a level similar to the sham group. These data showed that severe mesenteric ischemia/reperfusion injury abolished release of endogenous splanchnic PGI2. PTX exerted a protective effect against severe mesenteric ischemia/reperfusion injury by maintaining release of splanchnic PGI2, a potent endogenous splanchnic vasodilator.

Burn induced cardiac dysfunction is reduced by pentoxifylline.

Thermal injury impairs cardiac contractility and slows isovolumic relaxation; these myocardial defects persist despite adequate fluid resuscitation. Results of previous studies suggest that myocardial dysfunction occurring after thermal injury results from the byproducts of activated neutrophils and other inflammatory mediators. Pentoxifylline (PTX) (Hoechst-Roussel Pharmaceuticals) has been shown to modulate many of the deleterious effects mediated by the neutrophil. In the current study, isolated coronary perfused hearts of rats were used to determine if PTX improved cardiac dysfunction after burn injury. Parameters measured included left ventricular pressure (LVP), maximal rate of LVP rise (+dP/dt max) and fall (-dP/dt max). Full thickness scald burns averaging 40 percent of total body surface area (burn groups, n = 22) or zero percent for sham burns (n = 10) were produced using a template device. Ten rats with burns were not fluid resuscitated and served as untreated burns. An additional 12 burned rats received PTX intraperitoneally (50 milligrams per kilogram) 30 minutes, six hours and 20 hours after thermal insult. The results of ex vivo studies confirmed significant burn mediated cardiac dysfunction as indicated by a decrease in LVP (55 +/- 4 millimeters of mercury, p < 0.001), +/- dP/dt max (1,063 +/- 119 millimeters of mercury per second; 874 +/- 82 millimeters of mercury per second, p < 0.001) and a downward shift of LV function curves from those obtained for sham-burn hearts. However, hearts from burned rats treated with PTX had significantly higher LVP (76 +/- 3 millimeters of mercury, p < 0.001) and +/- dP/dt max (1,790 +/- 54 millimeters of mercury per second; 1,334 +/- 50 millimeters of mercury per second, p < 0.001) compared with hearts from untreated burned rats and generated LV function curves comparable with those calculated for sham-burned rats. The current data indicate that PTX attenuates postburn cardiac dysfunction and suggest a potential role for the adjunctive use of PTX after thermal injury.

Allopurinol prevents intestinal permeability changes after ischemia-reperfusion injury.

Under normal conditions the intestinal mucosa is impermeable to potentially harmful materials from the intestinal lumen. Mucosal disruption promotes bacterial translocation, which is postulated to be a fuel source for sepsis and multiorgan failure. We have previously demonstrated that mesenteric ischemia-reperfusion (I/R) injury increases intestinal permeability (IP); however, the mechanism remains unclear. This study was designed to examine the hypothesis that changes in IP, after I/R injury, are mediated by xanthine oxidase-generated, oxygen-derived free radicals. Thirty-three Sprague-Dawley rats (weighing 300 to 400 g) were included in this study. Group 1 (n = 10) received enteral allopurinol, a xanthine oxidase inhibitor, 10 mg/kg daily for 1 week prior to mesenteric ischemia. Group 2 consisted of 11 untreated, ischemic animals. Groups 1 and 2 were subjected to superior mesenteric artery occlusion with interruption of collateral flow for 20 minutes to produce ischemic injury to the intestine. An additional 12 rats (group 3), served as nonischemic controls (sham). A loop of distal ileum was isolated and cannulated proximally and distally to allow luminal perfusion with warmed Ringer's lactate at 1 mL/min. IP was determined in all groups by quantitatively measuring the plasma-to-luminal clearance of chromium (51Cr)-labeled ethylenediaminetetraacetate (EDTA) at baseline, during ischemia and 20, 40, and 60 minutes after reperfusion. Complete ischemia produced significant increases in IP over baseline values in the untreated rats (group 2, baseline: 0.49 +/- 0.006, ischemia: 0.149 +/- 0.039) compared with sham rats (baseline: 0.41 +/- 0.006; ischemia: 0.047 +/- 0.009) or allopurinol-treated rats (baseline: 0.098 +/- 0.020, ischemia: 0.073 +/- 0.012, P less than .001).(ABSTRACT TRUNCATED AT 250 WORDS)