Patent ductus arteriosus ligation in premature infants: who really benefits, and at what cost?

Purpose

Patent ductus arteriosus (PDA) ligation in premature infants has been shown to have low surgical morbidity and mortality. Ligation goals include prompt improvement in cardiorespiratory failure, with rapid wean from mechanical ventilation; less risk of prolonged mechanical ventilation and subsequent chronic lung disease (CLD); and survival to discharge. This study was designed to examine true morbidity after ligation and elucidate which preoperative factors might predict favorable outcomes.

Methods

Institutional review board-approved retrospective review of 197 infants less than 38 weeks of gestational age (GA), undergoing PDA ligation via thoracotomy between January 1, 1992, and January 1, 2004. Chronic lung disease defined as need for supplemental oxygen at 36 weeks corrected GA. Student t and χ² tests were used.

Results

Mean GA was 27 weeks (range, 23-35 weeks), birth weight was 957 g (range, 440-3170 g); infants underwent ligation at 16 days of life (range, 1-132 days). Duration of surgery was 50.5 minutes (range,13-150 minutes). Mean postoperative times were 27 days to extubation, 60 days to wean from supplemental oxygen, and 84 days to discharge. Early extubation (within 10 days of ligation) occurred in only 54 patients (30%). Only 44 (22%) survived to discharge without CLD. Forty patients (20%) died, with respiratory failure the most common cause (70%). In general, early extubation, survival without CLD and survival to discharge were associated with greater GA and birth weight, higher Apgar scores, greater age and weight at surgery, no preoperative intraventricular hemorrhage, lack of ventilator dependence, and lower ventilator settings (P < .05). Preoperative amount and duration of indomethacin use, chest x-ray findings, and echocardiographic assessment of ductus size did not predict favorable outcomes (all P > .05).

Conclusions

Most premature infants currently undergoing PDA ligation at our institution do not experience the anticipated rapid improvements in cardiorespiratory status and go on to develop CLD. Few preoperative variables (including radiographic and echocardiographic assessments) definitively predict outcomes.     

Patterns of cerebral injury in a series of infants with congenital diaphragmatic hernia utilizing magnetic resonance imaging

Background

Congenital diaphragmatic hernia (CDH) is a condition with significant mortality and respiratory morbidity. Long-term neurodevelopmental outcome is not yet well documented.

Methods

The authors examined all cases of CDH at their institution over a 12-month period and performed magnetic resonance imaging (MRI) of the brain postoperatively.

Results

Eight of 10 patients survived to have an MR brain scan. All 8 patients had cerebral injury detected on MRI, with a predominance of white matter and deep nuclear gray matter injury.

Conclusions

Cerebral injury detected with MRI is common in infants with congenital diaphragmatic hernia. The long-term significance of these MRI abnormalities is unknown, although the need for good long-term neurodevelopmental follow-up of infants with CDH is highlighted.     

Acquired intestinal aganglionosis after a lytic infection with varicella-zoster virus

Background and Purpose

In this report, we present the first case of an immunologically impaired child surviving a lytic varicella-zoster virus infection affecting the enteric nervous system. In histological findings, myenteric and submucous enteric ganglia were nearly completely absent owing to virus infection.

Methods

A 3-year-old girl with acute lymphoblastic leukemia and generalized varicella-zoster infection developed an ileus. She underwent multiple laparotomies in which histological sections of the entire small intestine could be obtained.

Results

The histological evaluation of these samples showed a generalized aganglionosis with inflammatory residuals. A more detailed immunohistochemical analysis using neuronal (PGP, enolase), glial (S100), and lymphocytic (LCA) antibodies demonstrated a nearly complete neuronal loss.

Conclusion

To our knowledge, this is the first case of a secondary intestinal aganglionosis after varicella-zoster virus infection.     

Effect of graft size on graft fracture rate after anterior lumbar spinal fusion in a sheep model

Study design

Sheep anterior spinal fusion model with autogenous and xenogenous bone grafts.

Objective

To investigate whether the relationship between cross-sectional area of the bone graft and area of the adjacent vertebral endplates has an effect on graft fracture rate.

Summary of background data

Anterior spondylodesis with autogenous iliac crest transplant is a frequently performed operation to stabilize spinal motion segments but to date no precise recommendations with respect to minimum graft size are available in the literature.

Methods

Anterior spondylodesis using autogenous and xenogenous grafts of constant size in combination with an angular stable plate (Macs TL^®). Autogenous iliac crest graft was inserted in eight sheep and xenogenic, commercially available bovine graft (Tutobone^®) in the additional eight animals.The surface areas of the endplates of the fused intervertebral space were calculated using CT scans and contact radiographs of the specimens obtained after 24 weeks. The graft itself was evaluated for fractures and osteolysis.

Results

A fracture occurred in tricortical, autogenous grafts if the graft cross-sectional area was less than 21% of the area of the adjacent endplates. All xenogenic grafts fractured and therefore a comparable value could not be determined.

Conclusion

The results clearly indicate that the relation between graft cross-sectional area and endplate area defines the survival or fracture of the graft in anterior spinal fusion. Although it is difficult to directly apply the results to the clinical situation it is suggested to choose a sufficiently large graft, in order to reduce the risk of autogenous graft fracture in anterior spondylodesis.     

Medical treatment of infantile hypertrophic pyloric stenosis: should we always slice the "olive"?

Background/Purpose

Laparoscopic pyloromyotomy has recently gained wide acceptance as the optimum treatment of infantile hypertrophic pyloric stenosis (IHPS). However, medical treatment may be superior to laparoscopic surgery in invasiveness. The efficacy of our regimen of intravenous atropine therapy for IHPS was assessed in comparison with surgical treatment.

Methods

Medical treatment was initially chosen for 52 (61%) of 85 infants with IHPS at our institute between 1996 and 2004. Atropine was given intravenously at 0.01 mg/kg 6 times a day before feeding. When vomiting ceased and the infants were able to ingest 150 mL/kg per day of formula after stepwise increases in the feeding volume, they were given 0.02 mg/kg atropine 6 times a day orally, and the dose was decreased stepwise.

Results

Of the 52 patients, 45 (87%) ceased projectile vomiting with treatment using intravenous (median, 7 days) and subsequent oral (median, 44 days) atropine administration. The median hospital stay was 13 days (6-36), and no significant complications were encountered during atropine therapy. The remaining 7 patients required surgery. Of 40 who underwent surgery, 4 had wound infections and 1 with hemophilia had postoperative hemorrhagic shock. The patients who underwent successful atropine therapy had body weights comparable with those who underwent surgery at the age of 1 year.

Conclusions

The high success rate of intravenous atropine therapy for IHPS suggests that this therapy is an effective alternative to pyloromyotomy if the length of the hospital stay and the necessity of continuing oral atropine medication are accepted.     

Total parenteral nutrition-associated hyperglycemia correlates with prolonged mechanical ventilation and hospital stay in septic infants

Purpose

We studied the effects of total parenteral nutrition (TPN)-associated hyperglycemia on the clinical outcome in premature septic infants in the neonatal intensive care unit.

Methods

The charts of all premature infants weighing less than 1500 g upon admission to the neonatal intensive care unit between January 1, 2002, and December 31, 2002, with sepsis, ventilator dependence, and feeding intolerance were studied. Maximum serum glucose concentrations were compared with duration of TPN, mechanical ventilation, hospital length of stay, and survival using Pearson regression analysis and Student's t test.

Results

Thirty-seven patients met the search criteria. The average caloric intake for all infants at the time of blood culture-proven sepsis was 83 ± 19 kcal/kg per day. The maximum serum glucose concentration (milligrams per deciliter) after having positive blood cultures (sepsis) was positively correlated with the duration of TPN (r = 0.45, P = .005), length of dependence on mechanical ventilation (r = 0.45, P = .006), and hospital length of stay (r = 0.36, P = .03). The average maximum serum glucose level was significantly higher in the nonsurviving infants (241 ± 46 vs 141 ± 48, P < .0001).

Conclusion

Hyperglycemia correlated with prolonged ventilator dependency and increased hospital length of stay in premature septic infants. Avoidance of excessive nutrient delivery and tight glycemic control during periods of acute metabolic stress may improve outcome in this patient population.     

Preliminary observations of the use of high-frequency jet ventilation as rescue therapy in infants with congenital diaphragmatic hernia

Background/Purpose

Congenital diaphragmatic hernia (CDH) is associated with mortality of 10% to 50%. Several investigators have reported outcomes from centers using high-frequency oscillatory ventilation in their management of CDH, but there are no recent reports on use of high-frequency jet ventilation.

Methods

During the study period from January 2001 until August 2007, infants with CDH who were cared for at Duke University Medical Center received high-frequency jet ventilation as a rescue mode of high-frequency ventilation. We compared actual survival with predicted survival for infants treated only with conventional ventilation vs those rescued with high-frequency jet ventilation after failing conventional ventilation.

Results

Survival for the 16 infants that received high-frequency jet ventilation was predicted to be 63%; actual survival was 75%. Survival for the 15 infants that received only conventional ventilation was predicted to be 83%; actual survival was 87%. We observed no significant survival benefit for high-frequency jet ventilation, 8.0% (95 confidence interval, −22.0% to 38.1%; P = .59).

Conclusions

Although our sample size was small, we conclude with consideration of the absolute results, the degree of illness of the infants, and the biologic plausibility for the intervention that high-frequency jet ventilation is an acceptable rescue ventilation mode for infants with CDH.     

The second STEP: the feasibility of repeat serial transverse enteroplasty

Background

Serial transverse enteroplasty (STEP) lengthens and tapers dilated bowel. Redilation of the STEP segment occurs in some patients with intestinal failure. The feasibility of a repeat STEP procedure in a pig model is evaluated.

Methods

Six pigs underwent reversal of an intestinal segment distal to the ligament of Treitz. At 6-week intervals after reversal, each animal had 2 STEP procedures on the bowel proximal to the reversed segment. Necropsy was performed up to 6 weeks after repeat STEP.

Results

Bowel length increased by 11.3 ± 3.9 cm and bowel diameter decreased from a mean of 5.3 ± 0.8 to 1.8 ± 0.4 cm (P < .0001) after the first STEP. After repeat STEP, bowel length increased by 16.7 ± 13.3 cm (P < .01), and the bowel was tapered from a mean of 5.4 ± 0.9 to 2.2 ± 0.4 cm (P < .01). Five pigs did well after repeat STEP, and 1 pig had early necropsy for bowel obstruction. None had histologic evidence of bowel ischemia in the repeat STEP segment.

Conclusions

A second STEP operation is feasible in a pig model and may be considered to optimize bowel length and function in select patients with intestinal failure.     

The burden of incisional hernia in necrotizing pancreatitis: how can we improve?

Background

Necrotizing pancreatitis (NP) patients frequently require pancreatic debridement, and have risk factors for incisional hernia (IH). However, no published data exist regarding the incidence of IH in NP. The aim of the current study was to define the incidence of and identify risk factors for developing IH after pancreatic debridement.

Methods

Hernia presence was determined by clinical examination and patient interview. Technical and clinical considerations were noted: type of incision, closure, suture material, age, body mass index (BMI), diabetes mellitus (DM), preoperative albumin, and number of operations.

Results

Sixty-three (42%) of 149 debrided patients with NP developed IH. IH patients were older (P < .05). No differences in surgical technique or clinical risk factors were seen between groups.

Conclusion

The incidence of IH in NP patients requiring operative debridement is substantially higher than that in patients undergoing routine laparotomy. Innovative fascial closure techniques such as primary fascial buttress with nonsynthetic mesh should be considered.     

Gastroschisis: ward reduction compared with traditional reduction under general anesthesia

Background/Purpose

In gastroschisis it is proposed that gut reduction may be achieved without intubation or general anesthesia (GA) through ward reduction. The authors aimed to determine if ward reduction decreased morbidity and duration of treatment.

Methods

Infants born from January 1, 1995, to December 31, 2001, with gastroschisis were managed with either reduction under GA in the operating theatre (OT group)—up to September 1999, or ward reduction (when eligible) in the neonatal unit without GA/ventilation (ward reduction [WR] group)—from September 1999.

Results

Of the 37 infants, 31 were eligible for ward reduction—15 from the OT group, 16 from the WR group. All infants in the OT group had at least 1 episode of ventilation and 1 GA: 62% of infants in the WR group avoided ventilation (P = .0002) and 81% avoided GA (P < .0001). Infants who had ward reduction had significantly shorter durations of ventilation and oxygen therapy. Septicemia occurred in 31% of the WR group and 7% of the OT group (P = .17). Infants who had ward reduction left intensive care 16 days earlier (P = .02) and tended to reach full enteral feeds 8 days sooner (P = .06) and be discharged from hospital 15 days earlier (P = .05).

Conclusions

Infants who had ward reduction do better in terms of avoiding GA/ventilation, establishing feeds, and going home earlier. A randomized, controlled trial comparing the 2 approaches is feasible, safe, and worthwhile.     

Biological Changes After Liver Transplantation According to the Presence or Not of Graft Steatosis

Aim

To assess the consequences of graft steatosis on postoperative liver function as compared with normal liver grafts.

Patients and methods

From January 2005 to December 2007, liver transplant patients were prospectively included, excluding those who experienced arterial or biliary complications or presented acute rejection. All patients had a surgical biopsy after reperfusion. Patients were compared according to the rate of macrovacuolar steatosis: namely above or below 20%.

Results

Fifty-three patients were included: 10 in the steatosis group and 43 in the control group. No significant difference was observed in terms of morbidity, mortality, and primary non- or poor function. Nevertheless, biological changes after the procedure were significantly different during the first postoperative week. Prothrombin time, serum bilirubin, and transaminases were significantly increased among the steatosis group compared with the control group (P < .05).

Conclusion

This case-controlled study including a small number of patients, described postoperative biological changes among liver transplantations with steatosis in the graft.     

Kasai portoenterostomy: 12-year experience with a novel adjuvant therapy regimen

Aim

The role of adjuvant therapy with corticosteroids and choleretics after Kasai portoenterostomy for biliary atresia (BA) remains uncertain. Experience with a novel postoperative adjuvant therapy regimen is reported.

Methods

Between 1994 and 2006, 71 infants with BA were referred. Four died from uncorrectable congenital heart disease/cardiorespiratory failure without undergoing portoenterostomy, 7 underwent primary liver transplantation (3 referred ≥19 weeks of age), and 60 underwent portoenterostomy at a median of 51 (10-104) days. Of these, 55 (92%) had type 3 BA and 6 had the BA splenic malformation syndrome. Fifty (83%) received the following adjuvant therapy beginning on postoperative day 5: oral dexamethasone 0.3 mg/kg bd for 5 days, 0.2 mg/kg bd for 5 days, and 0.1 mg/kg bd for 5 days together with oral ursodeoxycholic acid 5 mg/kg bd and phenobarbitone 5 mg/kg nocte, both of which were continued for 1 year. All infants received routine perioperative prophylactic antibiotics.

Results

Overall, 42 of 60 (70%) infants cleared their jaundice (bilirubin <20 μmol/L): 38 of 50 (76%) with the dexamethasone/ursodeoxycholic acid regimen compared with 4 of 10 (40%) not receiving this adjuvant treatment. There were 4 late deaths after portoenterostomy: 2 from associated congenital disorders and 2 after liver transplantation. Of the remaining 56 children, 39 (70%) are currently alive with their native liver at a median follow-up of 3.3 years and 17 are alive after liver transplantation. Surgical complications occurred in 3 after portoenterostomy: adhesive bowel obstruction (2) and an anastomotic leak. One infant had gastrointestinal bleeding that may have been related to dexamethasone, but this resolved with ranitidine. There were no perioperative septic complications.

Conclusion

In this series, adjuvant postoperative treatment with a short course of oral dexamethasone and longer-term ursodeoxycholic acid significantly improved the outcome after Kasai portoenterostomy.     

The usefulness of videomanometry for studying pediatric esophageal motor disease

Purpose

Abnormalities in esophageal motor function underlie various symptoms in the pediatric population. Manometry remains an important tool for studying esophageal motor function, whereas its analyses have been conducted with considerable subjective interpretation. The usefulness of videomanometry with topographic analysis was examined in the current study.

Methods

Videomanometry was conducted in 5 patients with primary gastroesophageal reflux disease (GERD), 4 with postoperative esophageal atresia (EA), 1 with congenital esophageal stenosis (CES), and 1 with diffuse esophageal spasms (DES). Digitized videofluoroscopic images were recorded synchronously with manometric digital data in a personal computer. Manometric analysis was conducted with a view of concurrent esophageal contour and bolus transit.

Results

Primary GERD patients showed esophageal flow proceeding into the stomach during peristaltic contractions recorded manometrically, whereas patients with EA/CES frequently showed impaired esophageal transit during defective esophageal peristaltic contractions. A characteristic corkscrew appearance and esophageal flow in a to-and-fro fashion were seen with high-amplitude synchronous esophageal contractions in a DES patient. The topographic analysis showed distinctive images characteristic of each pathological condition.

Conclusions

Videomanometry is helpful in interpreting manometric data by analyzing concurrent fluoroscopic images. Topographic analyses provide characteristic images reflecting motor abnormalities in pediatric esophageal disease.     

Perturbation of Fgf10 signal pathway in mouse embryonic palate by dexamethasone and vitamin B12 in vivo

Background/Purpose

The Fgf10 signaling pathway plays an important role in early stages of mouse embryonic palatal development, which is associated with cell proliferation and differentiation. The objective of this study was to assess whether dexamethasone and vitamin B₁₂ affected the Fgf10 signal pathway of mouse embryonic palate.

Materials and Methods

Immunohistochemical studies were performed for expression of Fgf10, Fgfr2b, and sonic hedgehog and for cell proliferation and apoptosis of mouse embryonic palate.

Results

The expression of Fgf10, Fgfr2b, and sonic hedgehog was changed in mouse embryonic palate after dexamethasone and vitamin B₁₂ treatment, resulting in reduced and restored proliferation of mesenchymal cells.

Conclusions

Dexamethasone and vitamin B₁₂ affected the Fgf10 signaling pathway and cell proliferation of mouse embryonic palate. Cell apoptosis was not altered after dexamethasone and vitamin B₁₂ exposure.     

Smoking status is a risk factor for recurrence after transurethral resection of non-muscle-invasive bladder cancer

Background

Cigarette smoking is the most well-established risk factor for developing bladder cancer.

Objective

To investigate the role of smoking status on the clinical outcome of patients with non-muscle-invasive bladder cancer.

Design, setting, and participants

Data obtained during a prospective phase 3 study with three schedules of epirubicin were used for statistical analysis. Smoking status (obtained when entering the study), other prognostic variables, and clinical outcome measures of 718 patients were analyzed. Mean follow-up was 2.5 yr.

Measurements

The primary outcome measure was recurrence-free survival (RFS).

Results and limitations

Demographics were similar for nonsmokers versus ex-smokers and current smokers, except for gender (p < 0.001) and grade (p = 0.022). In univariate analyses, RFS was significantly shorter in male patients (p = 0.020), in patients with a history of recurrences (p < 0.003), in patients with multiple tumors (p < 0.004), in patients with a history of intravesical therapy (p = 0.037), and in ex-smokers and current smokers (p = 0.005). In multivariate analyses, a history of recurrences, multiplicity, and smoking status remained significant factors for predicting RFS. Gender and initial therapy were no longer a significant influence on RFS.Because progression was uncommon (n = 25) and follow-up was short and focused only on recurrences, no conclusion can be drawn on progression-free survival. A limitation of the study were the questionnaires. They were only used when entering the study, and there were no questions about passive smoking and other causal factors.

Conclusions

In this prospective study, the significance of known factors (history of recurrences and number of tumors) in predicting RFS was confirmed. Another significant factor that appears to predict RFS is smoking status: ex-smokers and current smokers had a significantly shorter RFS compared with nonsmokers.     

Do patients undergoing parathyroidectomy for primary hyperparathyroidism in San Francisco, CA, and Bursa, Turkey, differ?

Background

To compare the preoperative clinical symptoms and associated conditions and findings in operations in age- and sex-matched patients with primary hyperparathyroidism (PHPT) living in the two different regions of the world to determine whether PHPT differs in various countries.

Methods

Fifty-two patients from Bursa, Turkey were matched with 52 age and sex matched patients from San Francisco, USA. Patients' preoperative symptoms, biochemical and radiologic findings and surgical procedures were documented.

Results

More patients in the American Group (15%) had preoperatively persistant or recurrent hyperparathyroidism, P = 0.016. Serum parathyroid hormone levels were higher in Turkish group (546 ± 75.33 pg/mL) than in American group (146 ± 75.33 pg/mL). More Turkish patients had osteoporosis (P < 0.05). The size of parathyroid adenomas was significantly greater in Turkish patients (25.2 ± 1.18 mm) than in American patients (17.5 ± 1.18 mm), P < 0.001.

Conclusions

Patients with PHPT from Bursa, Turkey have higher plasma parathyroid hormone levels, larger parathyroid adenomas and more severe bone disease than in age and sex matched patients with PHPT in San Francisco.     

Prospective randomized trial of paravertebral block for patients undergoing breast cancer surgery

Background

The goal of the current study was to evaluate the effect of regional anesthesia using paravertebral block (PVB) on postoperative pain after breast surgery.

Methods

Patients undergoing unilateral breast surgery without reconstruction were randomized to general anesthesia (GA) only or PVB with GA and pain scores assessed.

Results

Eighty patients were randomized (41 to GA and 39 to PVB with GA). Operative times were not significantly different between groups. Pain scores were lower after PVB compared to GA at 1 hour (1 vs 3, P = .006) and 3 hours (0 vs 2, P = .001) but not at later time points. The overall worst pain experienced was lower with PVB (3 vs 5, P = .02). More patients were pain-free in the PVB group at 1 hour (44% vs 17%, P = .014) and 3 hours (54% vs 17%, P = .005) postoperatively.

Conclusions

PVB significantly decreases postoperative pain up to 3 hours after breast cancer surgery.     

Structural changes of smooth muscle in congenital ureteropelvic junction obstruction

Background/Purpose

Ureteropelvic junction (UPJ) obstruction is the most common cause of congenital hydronephrosis. Previous studies have reported that the excess amount of collagen restricting mobility and resiliency of the UPJ is the result of an impaired collagen production by anomalous smooth muscle cells (SMCs). Our purpose was to evaluate the role of SMC differentiation in the pathogenesis of UPJ obstruction.

Methods

Surgical specimens of UPJ from 21 patients (8 girls/13 boys) who were subjected to dismembered pyeloplasty were examined immunohistochemically using monoclonal antibodies against smooth muscle (SM) myosin heavy chain isoforms including SM1, SM2, and SMemb. The age ranged from 1 month to 13 years. Ureteropelvic walls taken from 14 forensic autopsy cases, with no urological abnormalities, served as age-matched control group.

Results

The immunohistochemical expression of SM1 and SM2 in UPJ obstruction was significantly increased when compared with controls (P < .05). In contrast, there was no statistical difference of expression of SMemb.

Conclusion

Our findings supported the hypothesis that the primary anomaly in UPJ obstruction may be attributed to a malfunction of SMCs in the ureter.     

Primary hepatic sarcomas in children--a single-center experience over 19 years

Background/ Purpose

Primary hepatic sarcomas are rare and account for about 13% of primary hepatic neoplasms. There are few reported series of pediatric hepatic sarcomas, and the aim was to review our experience.

Methods

A retrospective analysis of cases managed from 1988 to 2007 by the pediatric liver unit in Birmingham, UK, was conducted.

Results

Nineteen children were identified. These presented with sudden abdominal pain (n = 6), obstructive jaundice (n = 3), incidental mass (n = 3), and chronic pain/distension (n = 3). Vascular involvement was identified in 3, and 6 had pulmonary metastases. Three patients had primary resection, and 3 only a biopsy. Thirteen had a biopsy followed by chemotherapy and resection. Surgery included extended hepatectomy (n = 11), hepatectomy (n = 3), and nonanatomical resections (n = 2). There was 1 major intraoperative complication. Median inpatient stay was 7 days. One biliary leak developed 4 weeks postoperatively. Five of the 16 patients who underwent resection of the primary tumor died. Eleven were alive at a median follow-up of 3 years.

Conclusion

This is a challenging group of patients. Local control remains pivotal to successful treatment. Good results can be achieved in a specialist center with multidisciplinary approach.     

Abdominal involvement in pediatric heart and lung transplant recipients with posttransplant lymphoproliferative disease increases the risk of mortality

Background

Posttransplant lymphoproliferative disease (PTLD) is a serious complication in transplant recipients. Abdominal PTLD has been reported, but the prognosis remains undefined. The purpose of this study was to identify the incidence, predisposing factors, and outcome of abdominal PTLD in pediatric cardiothoracic transplant patients.

Methods

Retrospective chart review of 134 transplant patients (50 heart, 77 lung, 7 heart/lung) at our institution (1995-2005).

Results

Posttransplant lymphoproliferative disease was diagnosed in 14 patients. Most were Epstein-Barr virus naive initially, but all had seroconverted when diagnosed with PTLD. Eight had abdominal involvement; 4 required surgical interventions—1 for intussusception and for bowel perforation, 2 for bowel perforation, and 1 for tumor debulking. All had lifelong follow-up, with an average follow-up of 3 years. Of 8 patients with abdominal PTLD, 4 died of complications related to PTLD, whereas 1 of 6 patients with extraabdominal PTLD died of PTLD.

Conclusions

Epstein-Barr virus infection after transplantation is a major risk factor for PTLD. Pediatric patients with PTLD who present with abdominal involvement are more likely to die of PTLD than those without abdominal disease. Delay in diagnosis may contribute to the high mortality. Therefore, prompt evaluation and surveillance for possible abdominal PTLD may decrease mortality associated with this devastating problem.