Thoracoscopic-assisted placement of azygos vein central venous catheter in a child.

Loss of central venous access in intestinal failure patients is a potentially fatal complication, and an indication for intestinal transplantation. Thrombosis of the superior vena cava (SVC) has historically been considered a contraindication to small bowel transplantation; however, unconventional central venous access can facilitate survival and eventual transplant procedure in patients with end-stage central venous access. We describe a technique for azygos vein central catheter insertion utilizing thoracoscopic guidance in a 14-year-old girl with thrombosis of the SVC and chronic idiopathic pseudo-obstruction syndrome awaiting multivisceral transplantation. The technique is simplified by utilizing carbon dioxide (CO(2)) insufflation of the thoracic cavity to collapse the lung instead of double-lumen endotracheal tube placement, and no postoperative chest tube drainage of the pleural space is required. Thoracoscopic-assisted central access can also be used in children requiring chronic hemodialysis with limited venous sites due to thrombosis or small size of vessels.

Multifocal angiomyolipoma in a patient with tuberous sclerosis.

Angiomyolipomas are rare lesions that occur most commonly in the kidney, but also can occur in the liver and rarely, in the lymph nodes. Its rare association with tuberous sclerosis has been suggested, but never proved. We report a patient with multiple visceral angiomyolipomas as well as visceral vessel aneurysms and tuberous sclerosis. The association of the three lesions suggests a possible common connective-tissue defect.

Modulation of multidrug resistance with antisense oligodeoxynucleotide to mdr1 mRNA.

BACKGROUND: P-glycoprotein (pgp), a 170-kDa adenosine triphosphate-dependent membrane drug efflux pump encoded by the mdr1 gene, mediates cross-resistance in tumor cells to structurally unrelated cancer drugs. We investigated the capacity for modulating multidrug resistance by selectively inhibiting synthesis of Pgp using an antisense oligodeoxynucleotide complementary to the initiation codon of mdr1 messenger RNA. METHODS: By continuous culture of K562 in 100 nM vincristine, a resistant cell line, K562/VCR100, was derived with high expression of Pgp (95.9% of cells) and an IC50 40-fold greater than that of the parental cell line. The K562/VCR100 cells were treated with 10 microM of 15-mer antisense and sense phosphorothioate oligodeoxynucleotides. Modulation of multidrug resistance was analyzed using a daunorubicin/tritiated thymidine incorporation assay and flow cytometric assessment of cellular rhodamine 123 accumulation. RESULTS: Treatment of K562/VCR100 with the antisense oligodeoxynucleotide led to a doubling in daunorubicin growth inhibition at 1 microgram/ml and a tripling of growth inhibition at 0.6 micrograms/ml (p < 0.0023); a 58% reduction in the daunorubicin IC50 (p < 0.02); and an increased rate of rhodamine-123 accumulation (p = 0.02) compared with treatment with sense oligodeoxynucleotide or media controls. CONCLUSIONS: These results suggest that antisense oligodeoxynucleotides may serve as a useful adjunct in the treatment and prevention of multidrug resistance during cancer chemotherapy.

Totally implantable vascular access devices in 131 pediatric oncology patients.

The use of totally implantable vascular access devices (TIVAD) has gained acceptance in oncology patients, with lower overall complications and maintenance costs than percutaneous silastic catheters. We inserted 135 TIVAD in 131 selected pediatric oncology patients (mean age 8.9 years) for chemotherapy of 68 solid tumors, 39 leukemias, and 24 lymphomas. Patients were required to have an absolute neutrophil count of 1,000/µl prior to TIVAD insertion. The cumulative duration of access was 45,098 days, with a mean of 334 days per device (range 5 to 981 days). At the time of review, 53 (39%) TIVAD were functioning without complication, 69 (51%) were removed at the end of therapy or were functioning at the time of death, and 13 (9.6%) were removed due to complications. Complications (n = 23) included 12 episodes of septicemia and 4 pocket infections for an infection rate of 11.8% (1 in 2,819 access days). Infections were more common in patients with leukemia compared to all others (P <0.001). Coagulase-negative staphylococci were isolated in 10 of the 16 infections; 7 infections resolved with antibiotic therapy. Mechanical complications were associated with 7 (5.2%) devices (1 in 6,443 access days). These data suggest that in selected non-neutropenic pediatric oncology patients, TIVAD can be utilized with minimal morbidity in the delivery of long-term chemotherapy.

P-glycoprotein status of favorable-histology Wilms' tumor predicts treatment outcome.

Patients with advanced-stage favorable-histology (FH) Wilms' tumor have a 4-year relapse-free survival rate of 70% to 90% after resection and chemotherapy of actinomycin D, vincristine, and doxorubicin. These three agents are actively pumped out of cells by P-glycoprotein (Pgp). The authors studied whether Wilms' tumor expresses Pgp and if the degree of Pgp expression correlates with treatment outcome. At the time of diagnosis, eight blinded paraffin-embedded FH and four anaplastic (ANA) Wilms' tumor sections were immunogold-labeled with a Pgp monoclonal antibody (17F9). Four of the FH-tumor patients had had relapse (FH+) according to the National Wilms' Tumor Study-3 protocol. Negative-relapse FH-tumor patients (FH-) had at least 6 years of follow-up. All 12 Wilms' tumors stained positive for Pgp. Both differentiated tubular structures and blastemal elements expressed Pgp. By the pathologist's score and the computerized cell image analysis, the degree of Pgp staining was greater at the time of diagnosis in FH+ tumors than in FH- tumors (P < .03; Mann-Whitney test). There was no statistically significant difference between ANA and FH+ or FH- tumors. These results show that both FH and ANA Wilms' tumors express Pgp, with higher levels of Pgp expression found in FH patients who had relapse. Current chemotherapeutic protocols, using Pgp-sensitive agents, may not be optimal for all FH Wilms' tumor patients.

Criteria for safe cost-effective pediatric trauma triage: prehospital evaluation and distribution of injured children.

In an effort to maximize staff utilization, all pediatric trauma patients were triaged by emergency room personnel to one of two tiers, based on information reported by prehospital providers over radiotelephones. A total of 952 patients less than 15 years of age were evaluated during a 1-year period. The triage criteria had a sensitivity of 86% in predicting which trauma patients would require operating room and/or pediatric intensive care, while maintaining a specificity of 90%. Fifteen patients died; however, by TRISS methodology there were no unexpected deaths and four unexpected survivors. All eventual deaths were initially captured from field data by the severely injured triage criteria. The study data suggest that physician-controlled two-tiered field triage criteria can safely serve to maximize staff utilization in the emergency room.

Chronic and recurrent appendicitis are uncommon entities often misdiagnosed.

Those having chronic and recurrent appendicitis represent a small portion of patients with disorders of the appendix. We present a series of nine patients who underwent appendectomy for chronic or recurrent appendicitis at The Johns Hopkins Hospital, Baltimore, Maryland, between July 1984 and October 1992. There were seven women and two men (median age of 30 years, range of 15 to 63 years). All patients presented with pain in the right lower quadrant or lower abdomen of three or more weeks duration (mean of 16.0 +/- 8.4 months, range of three weeks to seven years), had no alternative diagnosis to account for the symptoms, had pathologic evidence of chronic inflammation or fibrosis of the appendix and had complete relief of the symptoms after appendectomy. Although the patients presented herein had clinical and pathologic evidence for recurrent or chronic appendicitis, careful review of the course of each patient before surgical referral revealed at least one episode of acute pain in the abdomen consistent with acute appendicitis managed by nonoperative means. This suggests that, while recurrent acute appendicitis and chronic appendicitis do occur, they can be avoided by the accurate diagnosis and operative management of acute appendicitis. We conclude that acute appendicitis can resolve spontaneously and recur repeatedly in the same individual; in the evaluation of a patient with abdominal pain, a history of prior similar episodes of pain should never dissuade one from considering the diagnosis of acute appendicitis, and recurrent acute appendicitis and chronic appendicitis should be considered in the differential diagnosis of recurrent pain in the lower abdomen.

Rupture of the right diaphragm following blunt trauma in an infant: case report.

Traumatic diaphragmatic rupture is a rare childhood injury and is often difficult to diagnose. This is particularly true in infants. We present the case of a 3-month-old infant with traumatic rupture of the right diaphragm that became clinically apparent only after extubation. The diagnosis can often be made on the basis of chest radiography and clinical signs. Surgical treatment is required. Some phrenic nerve injuries can be anticipated and late sequelae may result.

Limited role of barium enema examination preceding colostomy closure in trauma patients.

Whether trauma patients should undergo barium enema (BE) examination of the colon prior to colostomy closure has recently been questioned. To ascertain the utility of BE and its impact on postoperative course in this patient population, we reviewed 86 trauma patients who underwent colostomy closure during a 12-year period at our institution. There were 82 males and four females with an average age of 28 years. Ninety-five percent of the injuries were the result of penetrating trauma. Sixteen patients had rectal injuries. Fifteen of these had BE greater than 6 weeks post-trauma and all showed healing of the injury. Of the 70 patients with colonic injuries, 43 (group 1) had BE prior to colostomy closure. Ninety-eight percent (n = 42) of these studies were negative. The only positive finding did not affect the planned surgical procedure. Group 2 (n = 27) did not have a BE prior to colostomy closure. Overall complication rates were not significantly different between group 1 (18.6%) and group 2 (29.6%). We conclude that BE prior to colostomy closure for colonic injuries yields little useful information and does not affect the morbidity rate prior to colostomy closure. Its routine usage should be abandoned. The role of barium enema in assessing rectal injury status is less clear because of the small number in our series, but probably offers no advantage over proctoscopy.

The night after surgery. Postoperative management of the pediatric outpatient--surgical and anesthetic aspects.

Outpatient or "ambulatory" anesthesia and surgery has revolutionized the way surgery is practiced in the United States. Safe, reliable, inexpensive, and convenient outpatient surgery is an attractive option for parents, children, health care providers, and insurers.

Morbidity and timing of colostomy closure in trauma patients.

The reported morbidity of colostomy closure in trauma patients varies from 5 to 27 per cent. Low morbidity rates are cited as a factor favouring colostomy creation and against expanded indications for primary repair in the treatment of colonic injuries. In order to assess the morbidity of colostomy closure, we reviewed all colonic injuries from 1979 to 1991 at our institutions. In all, 86 trauma patients who underwent colostomy creation and closure were identified. There were 82 men and four women with an age range of 16 to 74 years (mean 28.1 years). Of these, 95 per cent (N = 82) resulted from penetrating trauma. Of the patients, 63 per cent (N = 54) received end colostomies and 81 per cent (N = 70) of the patients had associated injuries. Of the patients, 38 per cent (N = 33) had a complication with their initial operation. There were no deaths after colostomy closure, but a total morbidity of 24.4 per cent (N = 21) was noted. There were 11 anastomotic complications (two of which required repeat laparotomy) and nine wound infections. The average length of stay was 10.4 days. Morbidity was concentrated in the group who had complications at their initial hospitalization. This was especially true if these patients underwent closure earlier than 3 months after injury. Conversely, if the first operation was uncomplicated, waiting longer than 3 months to perform colostomy closure did not improve results further.

Use of the pulmonary artery catheter to reduce operative complications.

Despite several prospective, randomized trials that demonstrated reductions in operative morbidity and mortality rates, routine use of invasive monitoring has not achieved widespread acceptance. This probably comes from skepticism about the validity of some of the studies along with known complications of the pulmonary artery catheter. The major studies are reviewed and a rational approach to monitoring is presented.

Atypical thrombotic and septic complications of totally implantable venous access devices in patients with cystic fibrosis.

The use of vascular access systems in patients with cystic fibrosis (CF) is well accepted, with lower overall complications and maintenance costs than percutaneous silastic catheters. We report our 6 year experience with 22 infusaports in 15 CF patients. Our patients had indwelling catheters for an average of 539 days per catheter (range, 14-2,224 days). These infusaports were used for home antibiotic therapy, blood sampling, and total parenteral nutrition. The overall complication rate was relatively low, 1 in every 1,483 catheter days. Infectious complications were extremely infrequent at a rate of 1 in 5,929 catheter days. The rate of mechanical complications was 1 in 1,976 catheter days. However, superior vena caval syndrome or deep venous thrombosis was associated with 3 of 22 catheters (13.6%). Due to this high incidence of major thrombotic events with the attendant risk of pulmonary embolism, all patients with CF using infusaports and without evidence of liver disease or bleeding problems receive aspirin prophylaxis.