Edward P. Tagge

Use of an ethanol lock to prevent catheter-related infections in children with short bowel syndrome ☆

BACKGROUND Children with short bowel syndrome (SBS) requiring central venous catheters (CVCs) may experience frequent catheter-related infections (CRIs). Treatment strategies include antibiotic- and ethanol-containing locks, with CVC removal if the CRI cannot be cleared. Ethanol lock therapy has been reported for CRI treatment in children but not for CRI prevention. METHODS Medical records of children with SBS receiving cycled home parenteral nutrition via a silicone CVC and who received a daily 70% ethanol lock at some time during their therapy were reviewed retrospectively. MAIN RESULTS Ten patients had 26 CVCs for a total of 3556 catheter-days and received a daily ethanol lock for 4 to 14 hours during a total of 3018 catheter-days. Before ethanol lock therapy (n = 5), there were 6 CRIs in 538 catheter-days (rate, 11.15 per 1000 catheter-days). During ethanol lock therapy in the same 5 patients, the CRI rate decreased to 2.06 per 1000 catheter-days (4 CRIs in 1936 catheter-days). In the 5 patients with no ethanol lock-free period, the CRI rate was 1.85 per 1000 catheter-days. Overall, CRI rate with ethanol lock therapy was 1.99 per 1000 catheter-days (2 CRIs in 1081 catheter-days). Four patients developed 6 CRIs during ethanol lock therapy. Four of these CRIs were cleared with systemic anti-infective and ethanol lock therapy; 2 CVCs were removed owing to infection. No adverse reactions were reported during ethanol instillation. CONCLUSION A daily 70% ethanol lock for CRI prevention was safe and effective in a series of 10 patients with SBS.

Diphtheria toxin fused to human interleukin-3 is toxic to blasts from patients with myeloid leukemias

Leukemic blasts from patients with acute phase chronic myeloid leukemic and refractory acute myeloid leukemia are highly resistant to a number of cytotoxic drugs. To overcome multi-drug resistance, we engineered a diphtheria fusion protein by fusing human interleukin-3 (IL3) to a truncated form of diphtheria toxin (DT) with a (G4S)2 linker (L), expressed and purified the recombinant protein, and tested the cytotoxicity of the DTLIL3 molecule on human leukemias and normal progenitors. The DTLIL3 construct was more cytotoxic to interleukin-3 receptor (IL3R) bearing human myeloid leukemia cell lines than receptor-negative cell lines based on assays of cytotoxicity using thymidine incorporation, growth in semi-solid medium and induction of apoptosis. Exposure of mononuclear cells to 680 pM DTLIL3 for 48 h in culture reduced the number of cells capable of forming colonies in semi-solid medium (colony-forming units leukemia) ⩾10-fold in 4/11 (36%) patients with myeloid acute phase chronic myeloid leukemia (CML) and 3/9 (33%) patients with acute myeloid leukemia (AML). Normal myeloid progenitors (colony-forming unit granulocyte–macrophage) from five different donors treated and assayed under identical conditions showed intermediate sensitivity with three- to five-fold reductions in colonies. The sensitivity to DTLIL3 of leukemic progenitors from a number of acute phase CML patients suggests that this agent could have therapeutic potential for some patients with this disease.

Impact of Segmental Grafts on Pediatric Liver Transplantation- A Review of the United Network for Organ Sharing Scientific Registry Data (1990-1996)

PURPOSE The aim of this study was to assess the relative impact of segmental grafts from cadaveric and living donors on outcomes in 3,409 pediatric transplants (<18 years) between 1990 and 1996. METHODS Analysis of the United Network for Organ Sharing (UNOS) Scientific registry data from 1990 to 1996 was performed. RESULTS Liver grafts consisted of 2,636 whole grafts (WLG), 246 liver donor grafts (LDG), 89 split liver graft (SLG), and 438 reduced-size grafts (RSG). Although the number of pediatric transplants were unchanged between 1990 and 1996, segmental grafts made up an increasing proportion from 14.5% to 29.2%, and WLG decreased proportionately. The increase among segmental grafts occurred for LDG (threefold), followed by SLG (53%) and RSG (50%). One-year graft and patient survival rates for 3,409 transplants were 69.7% and 81.9%, respectively and were significantly higher (P<.001) in nonhospitalized patients than in hospitalized patients (79.8% and 91.3% v 61.0% and 73.7%). LDG graft survival (75.9%) was comparable with WLG(70.9%) but significantly better at 1 year than SLG (60.3%, P = .007) and RSG (61.1%, P = .001), even after excluding retransplants and ICU patients. Patient survival rates were not different statistically between groups. A separate analysis of outcomes in recipients less than 1 year of age suggested significantly better graft and patient survivals for LDG (83.3% and 89.4%) than for WLG (62.3% and 76.5%) and RSG (62.7% and 75%). CONCLUSIONS Segmental liver grafts from cadaveric and living donors constitute an increasing proportion of pediatric transplants. Survival rates of cadaveric segmental graft are inferior to those of live donor segmental grafts even after adjustment for medical condition. Live donor grafts demonstrate consistently superior graft and patient outcomes in pediatric recipients less than 1 year of age, and should be promoted aggressively as a solution to the critical shortage of size matched grafts in small recipients.

CHILDHOOD PLEUROPULMONARY BLASTOMA: CAUTION AGAINST NONOPERATIVE MANAGEMENT OF CONGENITAL LUNG CYSTS

Pulmonary blastoma is a rare and aggressive malignant tumor that affects children and adults. Recently a 3-year-old boy with a 2-year history of bilateral unilocular pulmonary cysts was transferred for evaluation of a cough and high spiking fever. A chest radiogram showed left pulmonary consolidation with pleural effusion, but thoracentesis was unsuccessful. Computerized tomography (CT) was suggestive of a pulmonary abscess, but CT-guided drainage did not yield any purulent fluid. Percutaneous biopsies were performed, and the cytology showed malignant cells. During thoracotomy, a large tumor involving the left lower lobe and pleural space was found, and a biopsy was performed. A frozen section showed blastemal and mesenchymal components devoid of neoplastic epithelium, consistent with the pleural variant of pulmonary blastoma. A left lower lobectomy, with tumor decortication of the pleural space, achieved total gross tumor removal. The child received aggressive multiagent chemotherapy, and midway through it he underwent elective excision of the opposite lung cyst. It has been 17 months since the lobectomy; he is off chemotherapy and has no evidence of disease. A review of the literature showed that a large number of pediatric pulmonary blastomas are associated with cystic lung disease. Because total tumor removal offers the only chance of a good long-term outcome, surgical excision or close follow-up of pulmonary cysts in children is strongly recommended.

Outcome After Surgery Alone or With Restricted Use of Chemotherapy for Patients With Low-Risk Neuroblastoma: Results of Children's Oncology Group Study P9641

PURPOSE The primary objective of Childrens Oncology Group study P9641 was to demonstrate that surgery alone would achieve 3-year overall survival (OS) ≥ 95% for patients with asymptomatic International Neuroblastoma Staging System stages 2a and 2b neuroblastoma (NBL). Secondary objectives focused on other low-risk patients with NBL and on those who required chemotherapy according to protocol-defined criteria. PATIENTS AND METHODS Patients underwent maximally safe resection of tumor. Chemotherapy was reserved for patients with, or at risk for, symptomatic disease, with less than 50% tumor resection at diagnosis, or with unresectable progressive disease after surgery alone. RESULTS For all 915 eligible patients, 5-year event-free survival (EFS) and OS were 89% ± 1% and 97% ± 1%, respectively. For patients with asymptomatic stage 2a or 2b disease, 5-year EFS and OS were 87% ± 2% and 96% ± 1%, respectively. Among patients with stage 2b disease, EFS and OS were significantly lower for those with unfavorable histology or diploid tumors, and OS was significantly lower for those ≥ 18 months old. For patients with stage 1 and 4s NBL, 5-year OS rates were 99% ± 1% and 91% ± 1%, respectively. Patients who required chemotherapy at diagnosis achieved 5-year OS of 98% ± 1%. Of all patients observed after surgery, 11.1% experienced recurrence or progression of disease. CONCLUSION Excellent survival rates can be achieved in asymptomatic low-risk patients with stages 2a and 2b NBL after surgery alone. Immediate use of chemotherapy may be restricted to a minority of patients with low-risk NBL. Patients with stage 2b disease who are older or have diploid or unfavorable histology tumors fare less well. Future studies will seek to refine risk classification.

Tracheoesophageal compression from aortic arch anomalies: Analysis of 30 operatively treated children

During a 16-year period (1976 to 1992), 30 children underwent surgery for tracheoesophageal compression caused by aortic arch anomalies. The age range was 3 days to 12 years (median, 3 months); 19 (63%) were male, and 20 (67%) were white. Of the 30 patients, 10 had a double aortic arch (the left was atretic in 6), 5 had a left-sided arch with an aberrant right subclavian artery (4) or innominate artery (1), and 15 had a right-sided arch with an aberrant left subclavian artery (14) or with mirror-image branching (1). There was no mortality during hospitalization or within 30 days of surgery. The left ductus arteriosus was divided in 26 of the 30 patients. Among the 10 patients with a double aortic arch, division of the atretic left arch (6), the lesser left arch (3), or the lesser right arch (1) was carried out. All 4 patients with a left arch and aberrant right subclavian artery had division of the artery. Of 14 patients with a right arch and aberrant left subclavian artery, only 3 underwent division of the artery. Of the 30 patients, anterior arteriopexy was performed in 9 (30%), and reoperation for persistent symptoms was necessary in 4 (13%). The second operation usually consisted of aortopexy or tracheopexy. Of the 4 patients with associated cardiac anomalies, 2 underwent simultaneous cardiac repair. The duration of hospital stay for the 30 patients ranged from 4 to 148 days (median, 6 days). Excluded from this series are patients with vascular rings who were asymptomatic and patients with the pulmonary vascular sling syndrome.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute chest syndrome in the postoperative sickle cell patient

BACKGROUND/PURPOSE Acute chest syndrome (ACS), a phenomenon of pulmonary sequestration in sickle cell disease (SCD) patients, is frequently missed in the postoperative SCD child. The constellation of symptoms range from fever and respiratory distress to abdominal discomfort. In its most fulminate state, the syndrome has been reported in some series to carry almost a 25% to 50% mortality rate in the postoperative patient. The incidence in pediatric patients in the era of minimally invasive surgery is unknown. METHODS Since December 1995, 63 episodes of ACS have been documented in the nearly 500 SCD children seen at our institution. Six of 63 episodes occurred within 2 weeks after a surgical procedure under general anesthesia. During this period, 59 operations were performed by the pediatric surgery service on SCD patients with an ACS incidence of 10.2%. Careful review of the preoperative, intraoperative, and postoperative management of these patients was performed. RESULTS All six received preoperative oxygen saturation monitoring and intravenous fluid (IVF) hydration. One half of these patients required transfusion to achieve a hemoglobin level of greater than 10 mg/dL. Documentation of intraoperative temperature, hypoxia, volume status, and hypercarbia as well as any atypical perioperative events were monitored and reviewed. All patients received postoperative oxygen supplementation and IVF hydration. Onset of ACS ranged from 1 hour to 7 days postoperatively. Only one of six was thought to be of microbial etiology (elevated mycoplasma titers), and all patients received prophylactic antibiotic and aggressive pulmonary therapy. Overall length of hospitalization was increased with an average stay of 6.1 days. There were no postsurgical ACS deaths. CONCLUSIONS Despite close attention and avoidance of known risk factors for development of postoperative SCD complications, ACS occurred with an incidence much higher than previously reported in the literature (0.4% v 10.2%). Interestingly, five of six cases were after laparoscopic procedures suggesting that the advantages of laparoscopy, such as reduced postoperative pain, do not extrapolate to decreased incidence of ACS.

Multidisciplinary approach to the treatment of pediatric pancreaticobiliary disorders

A multidisciplinary approach using traditional open surgery, endoscopic retrograde cholangiopancreatography (ERCP) and laparoscopic surgery has revolutionized the care of the adult with pancreaticobiliary disease. This study focuses on a similar collaborative effort to diagnose and treat children with pancreaticobiliary disorders. Charts of all patients treated on the pediatric surgery service between June 1990 and May 1995, who also underwent ERCP, were abstracted for disease process, presenting symptoms, laboratory evaluation, surgical or endoscopic procedures, and eventual outcome. Twenty-six children were identified, ranging from 6 months to 19 years of age. Pancreaticobiliary disorders included pancreas divisum (n = 1), choledochal cyst (n = 4), pancreaticobiliary trauma (n = 4), cholelithiasis and choledocholithiasis (n = 17). The pancreaticobiliary tree was successfully visualized by ERCP in 25 of 26 (96%) patients. Fifteen of these patients also underwent attempted therapeutic endoscopic procedures, with 13 (87%) performed successfully. Three patients with choledochal cyst had stents placed preoperatively for cholangitis, all of whom have undergone successful choledochal cyst excision. Two trauma patients underwent attempted stenting of a bile leak and bile duct stricture, respectively, both of which were unsuccessful, necessitating surgical correction. Seventeen patients with cholelithiasis underwent ERCP to rule out choledocholithiasis. Ten patients were found to have common duct stones, and all stones were endoscopically extracted, including those in a 6-month-old child. Overall survival rate was 96% (25 of 26), with the one death occurring in a trauma patient unrelated to his pancreaticobiliary disorder. A multidisciplinary approach using traditional open surgery, ERCP and laparoscopic surgery can successfully treat even young children with pancreaticobiliary disorders. In experienced hands, diagnostic ERCP and therapeutic endoscopic intervention can be performed successfully in most pediatric patients, greatly simplifying the surgical management of these potentially complex problems.

A new method of treatment for complete tracheal rings in an infant: Endoscopic laser division and balloon dilation☆

PURPOSE The authors describe a new technique for management of complete tracheal rings in infants. METHODS The procedure consists of rigid bronchoscopy with KTP laser division, in the posterior midline, of the complete rings and gradual advancement of the bronchoscope aided by endoscopic balloon dilation. CONCLUSIONS The laser division, coupled with balloon dilation, allows for controlled separation of the cartilages posteriorly. The anterior esophageal wall buttresses the posterior tracheal separation.

Endoscopic electrohydraulic lithotripsy in the management of pancreatobiliary lithiasis

AbstractBackground: Clinical evaluation of intraoperative endoscopy with electrohydraulic lithotripsy (EHL) in the management of 13 patients with pancreatobiliary lithiasis was undertaken. Methods: Ten patients with chronic pancreatitis with intraductal lithiasis in the head and three with biliary lithiasis (one choledochal, one cystic, one right intrahepatic) underwent intraoperative endoscopy with EHL. Shock waves were applied by visual contact with a 3-Fr gauge EHL probe until all stones were fragmented and irrigated free. All pancreatitis patients had failed ERCP attempts to stent their pancreatic ducts secondary to ductal lithiasis. Patients with pancreatic stones underwent lateral pancreatojejunostomy. Biliary stone patients underwent laparoscopic cholecystectomy with common duct exploration (two cases) and open cholecystectomy with choledochoduodenostomy (one case). Results: Intraductal stone eradication was successful in all patients. Transampullary visualization of the duodenum was achieved in eight cases. Average EHL time was 65 min. There was no evidence of postoperative pancreatitis, cholangitis, or retained common duct stones. Conclusion: Intraoperative pancreatobiliary endoscopy with EHL is safe and effective in the eradication of pancreatic and bile duct stones. This novel technique represents a valuable adjunct in the management of chronic fibrocalcific pancreatitis with ductal lithiasis in the head region and in the open and laparoscopic management of intra- and extrahepatic bile duct stones.