Peritoneal dialysis catheter outcomes in infants initiating peritoneal dialysis for end-stage renal disease.

BACKGROUND: End-stage renal disease (ESRD) although rare among infants presents many management challenges. We sought to evaluate factors associated with PD catheter failure among infants initiated on chronic PD. METHODS: A retrospective chart review of all children under two years of age who had PD catheters placed for initiation of chronic PD from 2002 to 2015. Data was extracted for catheter related events occurring within 12 months of catheter placement. Cox and Poisson regression models were used to delineate factors associated catheter complications. RESULTS: Twenty-five infants with median age 18 days had PD catheters placed for chronic dialysis. Common complications included leakage around the exit site (31%), blockage (26%), migration or malposition (23%), catheter-related infections (18%), and other complications (2%). Predictors of initial PD catheter failure were age less than one month at catheter placement (hazard ratio (HR) 7.77, 95% CI, 1.70-35.39, p = 0.008), use of catheter within three days of placement (HR 5.67, 95% CI, 1.39-23.10, p = 0.015) and presence of a hernia (HR 8.64, 95% CI, 1.19-62.36, p = 0.033). In an adjusted Poisson regression model, PD catheter use within three days of placement was the only predictor of any catheter complication over the12 months of follow up. CONCLUSIONS: Use of PD catheters within three days of placement was associated with catheter failure. We recommend that when possible, catheters should be allowed to heal for at least three days prior to use to reduce risk of complications and improve catheter survival.

Evaluating quality of life of extracorporeal membrane oxygenation survivors using the pediatric quality of life inventory survey.

PURPOSE: This study assesses the impact of extracorporeal membrane oxygenation (ECMO) associated morbidities on long-term quality of life (QOL) outcomes. METHODS: A single center, retrospective review of neonatal and pediatric non-cardiac ECMO survivors from 1/2005-7/2016 was performed. The 2012 Pediatric Quality of Life Inventory™ (PedsQL™) survey was administered. Clinical outcomes and QOL scores between groups were compared. RESULTS: Of 74 patients eligible, 64% (35 NICU, 12 PICU) completed the survey. Mean time since ECMO was 5.5±3years. ECMO duration for venoarterial (VA) and venovenous (VV) were similar (median 9 vs. 7.5days, p=0.09). VA ECMO had higher overall complication rate (64% vs. 36%, p=0.06) and higher neurologic complication rate (52% vs. 9%, p=0.002). ECMO mode and ICU type did not impact QOL. However, patients with neurologic complications (n=15) showed a trend towards lower overall QOL (63/100±20 vs. 74/100±18, p=0.06) compared to patients without neurologic complications. A subset analysis of patients with ischemic or hemorrhagic intracranial injuries (n=13) had significantly lower overall QOL (59/100±19 vs. 75/100±18, p=0.01) compared to patients without intracranial injuries. CONCLUSION: Neurologic complication following ECMO is common, associated with VA mode, and negatively impacts long-term QOL. Given these associations, when clinically feasible, VV ECMO may be considered as first line ECMO therapy. TYPE OF STUDY: Retrospective review. LEVEL OF EVIDENCE: II.

Use of venovenous ECMO for neonatal and pediatric ECMO: a decade of experience at a tertiary children's hospital.

BACKGROUND: Advances in extracorporeal membrane oxygenation (ECMO) have led to increased use of venovenous (VV) ECMO in the pediatric population. We present the evolution and experience of pediatric VV ECMO at a tertiary care institution. METHODS: A retrospective cohort study from 01/2005 to 07/2016 was performed, comparing by cannulation mode. Survival to discharge, complications, and decannulation analyses were performed. RESULTS: In total, 160 patients (105 NICU, 55 PICU) required 13 ± 11 days of ECMO. VV cannulation was used primarily in 83 patients with 64% survival, while venoarterial (VA) ECMO was used in 77 patients with 54% survival. Overall, 74% of patients (n = 118) were successfully decannulated; 57% survived to discharge. VA ECMO had a higher rate of intra-cranial hemorrhage than VV (22 vs 9%, p = 0.003). Sixteen VA patients (21%) had radiographic evidence of a cerebral ischemic insult. No cardiac complications occurred with the use of dual-lumen VV cannulas. There were no differences in complications (p = 0.40) or re-operations (p = 0.85) between the VV and VA groups. CONCLUSION: Dual-lumen VV ECMO can be safely performed with appropriate image guidance, is associated with a lower rate of intra-cranial hemorrhage, and may be the preferred first-line mode of ECMO support in appropriately selected NICU and PICU patients. LEVEL OF EVIDENCE: II.

Perioperative determinants of transient hypocalcemia after pediatric total thyroidectomy.

PURPOSE: We seek to identify risk factors associated with hypocalcemia after pediatric total thyroidectomy. METHODS: We retrospectively reviewed patients younger than 21years undergoing total thyroidectomy between January 2002 and January 2016. We defined hypocalcemia as any serum calcium <8mg/dl or ionized calcium <1.0mmol/L. Perioperative risk factors were identified through multivariate logistic regression. RESULTS: Ninety-one children underwent total thyroidectomy. The average age was 13.7±4.4years, and 77% were female. Transient hypocalcemia was diagnosed in 34% (n=31) of patients. There was one case of permanent hypoparathyroidism. Predictors of transient hypocalcemia included age (OR 0.87, 95% CI 0.8-0.97, p=0.01), lymphadenectomy (OR 6.6, 95% CI 1.7-31.6, p=0.01), and hyperthyroidism (OR 13.3, 95% CI 1.3-1849, p=0.03). Patients with malignancy undergoing central (OR 7.1, 95% CI 1.5-33.4, p=0.01) or central plus lateral lymphadenectomy (OR 6.33, 95% CI 1.0-40.1, p=0.05) had significantly increased risk for transient hypocalcemia. Malignancy, MEN2A/B, goiter, preoperative calcium supplementation, incidental parathyroid removal, and postoperative PTH <15pg/ml were not associated with transient hypocalcemia. CONCLUSIONS: Younger age, hyperthyroidism, and concomitant lymphadenectomy during total thyroidectomy increase risk of developing transient hypocalcemia. Malignant cases with central or central plus lateral lymphadenectomy are also at increased risk. Aggressive perioperative management of hypocalcemia should be initiated in patients with these risk factors. LEVEL OF EVIDENCE: 2b.

Diagnostic Performance of US for Differentiating Perforated from Nonperforated Pediatric Appendicitis: A Prospective Cohort Study.

Purpose To prospectively evaluate the diagnostic performance of ultrasonography (US) for differentiating perforated from nonperforated pediatric appendicitis and to investigate the association between specific US findings and perforation. Materials and Methods This HIPAA-compliant study had institutional review board approval, and the need for informed consent was waived. All abdominal US studies performed for suspected pediatric appendicitis at one institution from July 1, 2013, to July 9, 2014, were examined prospectively. US studies were reported by using a risk-stratified scoring system (where a score of 1 indicated a normal appendix; a score of 2, an incompletely visualized normal appendix; a score of 3, a nonvisualized appendix; a score of 4, equivocal; a score of 5a, nonperforated appendicitis; and a score of 5b, perforated appendicitis). The diagnostic performance of US studies designated 5a and 5b was calculated. The following US findings were correlated with perforation at multivariate analysis: maximum appendiceal diameter, wall thickness, loss of mural stratification, hyperemia, periappendiceal fat inflammation, periappendiceal fluid, lumen contents, and appendicolith presence. The number of symptomatic days prior to presentation was recorded. Surgical diagnosis and clinical follow-up served as reference standards. Results A total of 577 patients with a diagnosis of appendicitis at US met the study criteria (468 with a score of 5a; 109 with a score of 5b). Appendicitis was correctly identified in 573 (99.3%) of 577 patients. US performance in the detection of perforated appendicitis (5b) was as follows: a sensitivity of 44.0% (80 of 182), a specificity of 93.1% (364 of 391), a positive predictive value of 74.8% (80 of 107), and a negative predictive value of 78.1% (364 of 466). Statistically significant associations with perforated appendicitis were longer duration of symptoms (odds ratio [OR] = 1.46, P < .0001), increased maximum diameter (OR = 1.29, P < .0001), simple periappendiceal fluid (OR = 2.08, P = .002), complex periappendiceal fluid (OR = 18.5, P < .0001), fluid-filled lumen (OR = 0.34, P = .002), and appendicolith (OR = 1.67, P = .02). Conclusion US is highly specific but nonsensitive for perforated pediatric appendicitis. Several US findings are significantly associated with perforation, especially the presence of complex periappendiceal fluid. © RSNA, 2016.

Searching for certainty: findings predictive of appendicitis in equivocal ultrasound exams.

BACKGROUND: Ultrasound (US) is the preferred imaging modality for evaluating suspected pediatric appendicitis. However, borderline appendiceal enlargement or questionable inflammatory changes can confound interpretation and lead to equivocal exams. OBJECTIVE: The purpose of this study was to determine which findings on equivocal US exams are most predictive of appendicitis. MATERIALS AND METHODS: All US exams performed for suspected pediatric appendicitis from July 1, 2013, through July 9, 2014, were initially interpreted using a risk-stratified scoring system. Two blinded pediatric radiologists independently reviewed US exams designated as equivocal and recorded the following findings: increased wall thickness, loss of mural stratification, peri-appendiceal fat inflammation, peri-appendiceal fluid, appendicolith and maximum appendiceal diameter. A third pediatric radiologist resolved discrepancies. US features were correlated with the final diagnosis via multivariate analysis. RESULTS: During the study period, 162/3,750 (4.3%) children had US exams initially interpreted as equivocal (mean age 9.8 +/- 3.8 years). Five outpatients were lost to follow-up. Forty-eight of the remaining 157 (30.6%) children had an operative diagnosis of appendicitis. Findings significantly associated with appendicitis were loss of mural stratification (odds ratio [OR] = 6.7, P=0.035), peri-appendiceal fat inflammation (OR = 10.0, P<0.0001) and appendicolith (OR = 15.8, P=0.025). While appendiceal diameter tended to be larger in patients with appendicitis, the difference was not statistically significant. CONCLUSION: Loss of mural stratification, peri-appendiceal fat inflammation and an appendicolith are significant predictors of appendicitis in children with otherwise equivocal US exams. While maximum appendiceal diameter is not statistically associated with appendicitis in our study, mean appendiceal diameter of 6.7 mm in those without appendicitis suggests that the customary upper normal limit of 6 mm is too sensitive.

Assessing quality of life in pediatric gastroschisis patients using the Pediatric Quality of Life Inventory survey: An institutional study.

BACKGROUND: The purpose of this study was to quantify quality of life (QOL) outcomes in gastroschisis children using a validated QOL inventory survey. METHODS: A chart review and prospective survey (2012 Pediatric Quality of Life Inventory™ (PedsQL™)) were performed for gastroschisis patients from 2005 to 2011. Demographics and scores were compared between patents with simple versus complicated gastroschisis and patients with and without bowel resection. RESULTS: One hundred nineteen patients (>2years of age) were identified. Twenty-eight families participated in the prospective survey with an average patient age of 5.8±2.3years. There were 11 complicated and 17 simple cases. Children with simple gastroschisis had lower rates of reoperation (0.06% versus 90%, p<0.001) and bowel resection (12% and 64%, p=0.004) than complicated patients. Average QOL scores for children with simple and complicated gastroschisis were 81.69/100±19.50 and 78.7/100±20.14 (p=0.70), respectively. Scores were also similar in children with and without bowel resection (74.72/100±19.94 and 83.29/100±19.10, p=0.171). Cronbach's alpha correlation was 0.912 for the overall survey. DISCUSSION: Despite increased need for reoperation and bowel resection, at >2years of age, PedsQL™ scores were similar between patients with simple and complicated gastroschisis. This study provides preliminary data on QOL outcomes for antenatal counseling of gastroschisis families.

Outcomes after peritoneal dialysis catheter placement.

BACKGROUND: The purpose of this study was to review surgical outcomes after elective placement of peritoneal dialysis (PD) catheters in children with end-stage renal disease. METHODS: Children with PD catheters placed between February 2002 and July 2014 were retrospectively reviewed. Outcomes were catheter life, late (>30days post-op) complications (catheter malfunction, catheter malposition, infection), and re-operation rates. Comparison groups included laparoscopic versus open placement, age<2, and weight<10kg. Univariate and multivariate analysis were performed. RESULTS: One hundred sixteen patients had 173 catheters placed (122 open, 51 laparoscopic) with an average patient age of 9.7±6.3years. Mean catheter life was similar in the laparoscopic and open groups (581±539days versus 574±487days, p=0.938). The late complication rate was higher for open procedures (57% versus 37%, p=0.013). Children age<2 or weight<10kg had higher re-operation rates (64% versus 42%, p=0.014 and 73% versus 40%, p=0.001, respectively). Adjusted for age and weight, open technique remained a risk factor for late complications (OR 2.44, 95% CI 1.20-4.95) but not re-operation. DISCUSSION: Laparoscopic placement appears to reduce the rate of late complications in children who require PD dialysis catheters. Children <2years age or <10kg remain at risk for complications regardless of technique.

Feeding gastrostomy in children with complex heart disease: when is a fundoplication indicated?

PURPOSE: Malnutrition is common among children with complex heart disease (CHD). Feeding gastrostomies are often used to improve the nutritional status of such patients. Our purpose was to evaluate a cohort of children with CHD following open Stamm gastrostomy without fundoplication. METHODS: We reviewed all CHD patients who underwent feeding gastrostomy placement from 1/1/2004 to 4/7/2015. Demographic data, cardiac diagnoses, operative details, post-operative complications, and the need for GJ feeding and fundoplication were examined. RESULTS: Open Stamm gastrostomy was performed in 111 patients. Median age at surgery was 37 weeks (3 weeks-13.7 years); average weight was 5.3 ± 4.9 kg. Thirty-four patients (30 %) experienced a total of 37 minor complications, including tube dislodgement after stoma maturation (20), superficial surgical site infection (13), mechanical failure (3), and bleeding (1). Three patients experienced a major complication (need for return to the OR or peri-operative death <30 days). Three patients required a subsequent fundoplication. Fifty-six surviving patients (62 %) continue gastrostomy feeds, of which 7 (13 %) patients require GJ feeds. CONCLUSION: Children with CHD tolerate an open Stamm gastrostomy well with minimal major complications. These results support very selective use of fundoplication in infants and children with CHD who require a feeding gastrostomy.

Management of appendiceal carcinoid tumors in children.

BACKGROUND: Appendiceal carcinoid tumors, also know as well-differentiated neuroendocrine neoplasms, are rare lesions detected incidentally after appendectomy in children. There are limited data about the natural history of these tumors, and guidelines regarding family counseling and need for additional surgery or follow-up imaging are not established in the pediatric age group. The purpose of this study was to review our institutional experience with appendiceal carcinoid tumors to provide data that might improve management. METHODS: After institutional review board approval, the charts of all patients treated at our institution for an appendiceal carcinoid tumor between 2002 and 2014 were reviewed. Data collected included patient demographics, pathologic details, postoperative management, and follow-up information. Descriptive analyses were performed. RESULTS: Twenty-eight patients were identified, which represents an incidence of 0.2% of children undergoing appendectomy during that time interval. The mean age at surgery was 13.8 ± 2.1 y; 54% were females. Two patients had symptoms suspicious for carcinoid syndrome at presentation, though none had evidence of metastatic disease. The mean tumor size was 0.73 ± 0.4 cm. Five patients (18%) underwent subsequent ileocecectomy or right hemicolectomy because of pathologic findings of invasion of the mesoappendix (n = 4) or lymphovascular invasion and subserosal extension (n = 1), two of whom had residual disease in the resected specimen (one in a lymph node). No recurrences have been detected at mean follow-up of 1.8 y. CONCLUSIONS: Appendiceal carcinoid tumors are discovered incidentally in about 0.2% of children undergoing appendectomy. Based on findings from a large contemporary series, we can conclude that these tumors are generally small and demonstrate lymphovascular invasion or mesenteric extension in fewer than 20% of cases. Prospective, multicenter studies are necessary to better define the indication for ileocecectomy and follow-up imaging protocols.

Correlating surgical and pathological diagnoses in pediatric appendicitis.

BACKGROUND: The stratification of appendicitis into simple and complex variants has far-reaching implications. While the operative diagnosis made by the surgeon dictates clinical management, the pathologic diagnosis often differs and is frequently used for coding and reimbursement. The purpose of this study was to examine discrepancies between the operative and pathologic diagnoses with subsequent correlation to clinical outcomes. METHODS: Patients with acute appendicitis from July 2011 to July 2012 were identified. Diagnoses included simple (normal, acute, and suppurative) and complex (gangrenous and perforated). We evaluated the inter-rater reliability between pathologic and operative diagnoses in the five appendicitis categories. Clinical outcomes of deep and superficial surgical site infections were evaluated according to the pathologic and surgical diagnosis. RESULTS: During the study period, we identified 1166 patients with acute appendicitis. The surgeon and pathologist agreed on the specific diagnosis (acute, suppurative, gangrenous, perforated, normal) in 48% of patients (kappa 0.289, 95% CI 0.259-0.324, p=0.001). Agreement on disease severity (simple vs. complex) improved to 82%. The operative diagnosis more accurately predicted infectious complications than the pathologic diagnoses. CONCLUSION: Significant discordance exists between surgical and pathologic diagnoses. While the relevance of this discordance to clinical outcomes is still not clear, a potential for incorrect hospital coding and subsequent reimbursement exists. Future quality improvement projects should focus on standardizing the surgical and pathologic diagnoses.