Pedunculated focal nodular hyperplasia in a healthy toddler.

Focal nodular hyperplasia (FNH) is a benign hepatic tumor rarely seen in pediatric patients, with most cases reported in school-aged children with a history of malignancy, liver disease, chemotherapy, or hematopoietic stem cell therapy. Despite having advanced radiographic imaging, diagnosing FNH before surgical resection can be difficult. We report a rare case of pedunculated FNH presenting as a large abdominal mass palpated on physical exam in a healthy 3-year-old girl with no history of malignancy or underlying liver disease. Ultrasound, computed tomography, and magnetic resonance imaging (MRI) did not demonstrate the typical imaging characteristics of FNH, because the mass was pedunculated with a poorly visualized central scar. Because approximately 75% of all primary hepatic tumors in this age group are malignant, this report also discusses the importance of adding hepatobiliary phase imaging with MRI to avoid, if possible, the need for biopsy or surgical resection of a benign hepatic tumor.

Mechanisms of systemic adaptation to univentricular Fontan conversion.

OBJECTIVE: After univentricular Fontan conversion, systemic venous pressure serves as the sole driving force for transpulmonary blood flow. Consequently, systemic venous return is markedly altered and ventricular filling is subnormal. The mechanisms and time course of systemic adaptation to Fontan conversion are incompletely understood. We hypothesized that acute elevation in systemic venous pressure induces an adaptive response similar to conversion to a univentricular Fontan circulation. METHODS: Adjustable vessel occluders were placed around the superior and inferior vena cavae in juvenile sheep. After 1-week recovery, occluders were tightened to acutely increase and maintain systemic venous pressure at 15 mm Hg (n = 6), simulating 1-stage Fontan conversion. Control animals (n = 4) received identical surgery, but venous pressure was not manipulated. RESULTS: Cardiac index decreased significantly (3.9 ± 1.0 mL/min/m(2) to 2.7 ± 0.7 mL/min/m(2), P < .001) and then normalized to control at 2 weeks. Circulating blood volume increased (100 ± 9.4 mL/kg vs 85.5 ± 8.4 mL/kg, P = .034) as a persistent response. Cardiac reserve improved and was not different from control by week 3. Resting heart rate decreased in both groups. Oxygen extraction (arteriovenous oxygen difference) and neurohormonal mediators increased transiently and then normalized by week 2. CONCLUSIONS: Adaptation to global elevation in systemic venous pressure to Fontan levels is complete within 2 weeks. Increased blood volume and reduced heart rate are persistent responses. Increased oxygen extraction and neurohormonal up-regulation are temporary responses that normalize with recovery of cardiac output. With improved physiologic understanding of systemic adaptation to Fontan conversion, approaches to single-ventricle palliation can be more objectively assessed and optimized.

Pediatric blunt abdominal injury: age is irrelevant and delayed operation is not detrimental.

BACKGROUND: During the past 40 years, management of solid organ injury in pediatric trauma patients has shifted to highly successful nonoperative management. Our purpose was to characterize children requiring operative intervention. We hypothesized that older children would be more likely to require operative intervention. In particular, we wanted to examine potential outcome disparities between children who were operated upon immediately and those in whom attempted nonoperative management failed. Additionally, we asked whether attempted nonoperative management, when failed, put children at higher risk for mortality or morbidities such as increased blood product transfusions or lengths of stays. METHODS: Retrospective cohorts from seven Level I pediatric trauma centers were identified. Blunt splenic, hepatic, renal, or pancreatic injuries were documented in 2,944 children <1 to 19 years of age from January 1993 to December 2002. Data collected included demographics, hemodynamics, blood transfusions, Glasgow Coma Scale score, Injury Severity Score, hospital length of stay (LOS), intensive care unit (ICU) LOS, and mortality. Analysis involved 140 (4.8%) of 2,944 patients requiring operation. Two cohorts were characterized: (1) immediate operation (IO), defined as laparotomy 3 hours after arrival (n = 59; 42%). RESULTS: Comparing the two cohorts, no age differences were found. Compared with F-NOM, IO had significantly worse hemodynamics, Injury Severity Score, and Glasgow Coma Scale score and was associated with liver injuries. Pancreatic injuries were significantly associated with F-NOM. While controlling for injury severity to compare IO versus F-NOM, linear regression revealed equivalent blood transfusions, ICU LOS, hospital LOS, and mortality rates. CONCLUSION: IO and F-NOM are rare events and independent of age. When operated upon for appropriate physiology, the timing of operation in pediatric solid organ injury is irrelevant and not detrimental with respect to blood transfusion, mortality, ICU and hospital LOS, and resource utilization.

Malone antegrade continence enema (MACE) for fecal incontinence in imperforate anus improves quality of life.

The MACE procedure has been used in patients with imperforate anus (IA) to improve fecal continence. Our aim was to assess the impact of the MACE on the quality of life (QOL) in children with IA and fecal incontinence. A retrospective review was performed of children with IA that underwent the MACE procedure between 1997 and 2004. Patients and their parents were contacted by telephone survey regarding continence and its psychosocial effects before and after MACE. The same survey was given to the patients' teachers. Responses to 15 questions were compiled and a QOL score calculated and significance evaluated by t-test (P < or = 0.05). IRB approval was obtained. Thirty-two patients were identified with a mean age at operation of 9 years (4-19 years) and mean follow-up of 3.8 years (7 months to 8 years). Four patients had a low malformation, 8 were intermediate, 15 were high, and 5 had a cloacal anomaly. Twenty patients had documented sacral/spinal anomalies, including five with tethered cord. Post-MACE complications included stenosis in 16 (50%), with 11 requiring an operative revision at a mean of 21.7 months (2 months to 6 years), takedown in one at 4 years and volvulus in one at 18 months. Prior to the MACE, 18/25 (72%) had poor QOL scores. Post-MACE QOL results were similar between patients, parents and teachers. Patients' mean QOL score improved from 59.9 to 26.3% (P < 0.001), with parents from 59.7 to 26.4% (P < 0.001). QOL score improved >50% in nine families, 25-50% in ten and <25% in six. All patients and parents interviewed reported an improvement in their QOL following the MACE. This procedure should be offered to children with IA with the expectation of significant improvement in QOL.

Incidence of abdominal wall defects is related to surface water atrazine and nitrate levels.

BACKGROUND: Gastroschisis and omphalocele are congenital abdominal wall defects (AWD). Atrazine and nitrates are common agricultural fertilizers. METHODS: The Centers for Disease Control and Prevention natality data set was used to collect data for patients with AWD born between January 1990 and December 2002. Similar data were obtained from the Indiana State Department of Health. An estimated date of conception was calculated by birth date and gestational age. Surface water nitrate and atrazine levels for Indiana were collected from US Geological Survey data. Midwest was defined as Indiana, Illinois, Iowa, Ohio, and Nebraska. Statistical analysis was performed by chi2 test and Pearson correlation for P < or = .05. RESULTS: The Centers for Disease Control and Prevention identified 9871 children with AWD in 1990 and in 1995-2001 of 35,876,519 live births (rate 2.75/10(5)). In Indiana, 358 children from 1990-2001 had AWD of 1,013,286 live births (rate 3.53/10(5)). The AWD rate in Indiana was significantly higher than the national rate in 1996 (P = .0377), 1998 (P = .0005), and 2001 (P = .0365) and significantly higher than the Midwest rate in 1998 (P = .0104). Monthly comparison demonstrated a positive correlation of AWD rate and mean atrazine levels (P = .0125). CONCLUSION: Indiana has significantly higher rates of AWD compared with national rates. Increased atrazine levels correlate with increased incidence of AWD.

Pediatric pancreatic trauma: predictors of nonoperative management failure and associated outcomes.

BACKGROUND: Nonoperative management (NOM) is an accepted treatment of pediatric solid organ injuries and is typically successful. Blunt pancreatic trauma tends to require operative intervention more frequently. We sought to identify predictors of failure of NOM and compare the outcome of operative management against NOM. METHODS: A retrospective analysis was performed from January 1993 to December 2002 of all children with blunt pancreatic injuries from the trauma registries of 7 designated level 1 pediatric trauma centers. Failure of NOM was defined as the need for intraabdominal operative intervention. Injuries were graded I to V, and ductal injury was defined as grades III to V. Parameters included mechanism of injury, injury severity score (ISS), organ grade, Glasgow Coma Scale score, and outcome. Data were analyzed by Fisher exact test and Mann-Whitney U test, with mean values +/- SD and significance of P < .05. RESULTS: Pancreatic injuries were present in 173 (9.2%) of 1823 patients. Of these, 43 (26.0% [43/173]) required an operation. Valid morbidity data was obtained in 118 of 173 patients. ISS was significantly higher in all patients treated operatively. Patients with an injury of grade III to V failed NOM more frequently than all patients with pancreatic injury (P =.0169). Length of stay was longer, and the incidence of pseudocysts, drainage procedures, and pancreatitis was higher in NOM patients, although not significant. CONCLUSIONS: Patients with pancreatic injuries had a NOM failure rate of 26.0%. ISS and injury grades III to V were predictors of NOM failure. Patients with pancreatic ductal injury require more aggressive management.

The failure of nonoperative management in pediatric solid organ injury: a multi-institutional experience.

BACKGROUND: Nonoperative management (NOM) is the accepted treatment of most pediatric solid organ injuries (SOI) and, is typically successful. We sought to elucidate predictors of, and the time course to, failure in the subset of children suffering SOI who required operative intervention. METHODS: A retrospective analysis was performed from January 1997 through December 2002 of all pediatric patients (age 0-20 years) with a SOI (liver, spleen, kidney, pancreas) from the trauma registries of seven designated, level I pediatric trauma centers. Failure of NOM was defined as the need for intra-abdominal operative intervention. Data reviewed included demographics, injury mechanism, injury severity (ISS, AIS, SOI grade, and GCS), and outcome. For the failures of NOM, time to operation and relevant clinical variables were also abstracted. A summary AIS (sAIS) was calculated for each patient by summing the AIS values for each SOI, to account for multiple SOI in the same patient. Univariate and multivariate analyses were employed, and significance was set at p < 0.05. RESULTS: A total of 1,880 children were identified. Of these, 62 sustained nonsurvivable head injuries that precluded assessment of NOM outcome and were thus excluded. The remaining 1,818 patients comprised the overall study population. There were 1,729 successful NOM patients (controls -- C) and 89 failures (F), for an overall NOM failure rate of 5%. For isolated organ injuries, the failure rates were: kidney 3%, liver 3%, spleen 4%, and pancreas 18%. There were 14 deaths in the failure group from nonsalvageable injuries (mean ISS = 54 +/- 15). The two groups did not differ with respect to mean age or gender. An MVC was the most common injury mechanism in both groups. Only bicycle crashes were associated with a significantly increased risk of failing NOM (RR = 1.76, 95% CI = 1.02-3.04, p < 0.05). Injury severity and organ specific injuries were associated with NOM failure. When controlling for ISS and GCS, multivariate regression analysis confirmed that a sAIS > or = 4, isolated pancreatic injury, and >1 organ injured were significantly associated with NOM failure (p < 0.01). The median time to failure was 3 hours (range, 0.5-144 hours) with 38% having failed by 2 hours, 59% by 4 hours, and 76% by 12 hours. CONCLUSIONS: Failure of NOM is un common (5%) and typically occurs within the first 12 hours after injury. Failure is associated with injury severity and multiplicity, as well as isolated pancreatic injuries.