Monica Langer

Improved survival in a multidisciplinary short bowel syndrome program

PURPOSE Pediatric short bowel syndrome (SBS) remains a management challenge with significant mortality. In 1999, we initiated a multidisciplinary pediatric intestinal rehabilitation program. The purpose of this study was to determine if the multidisciplinary approach was associated with improved survival in this patient population. METHODS The Center for Advanced Intestinal Rehabilitation includes dedicated staff in surgery, gastroenterology, nutrition, pharmacy, nursing, and social work. We reviewed the medical records of all inpatients and outpatients with severe SBS treated from 1999 to 2006. These patients were compared to a historical control group of 30 consecutive patients with severe SBS who were treated between 1986 and 1998. RESULTS Fifty-four patients with severe SBS managed by the multidisciplinary program were identified. Median follow-up was 403 days. The mean residual small intestinal length was 70 +/- 36 vs 83 +/- 67 cm in the historical controls (P = NS). Mean peak direct bilirubin was 8.1 +/- 7.9 vs 9.0 +/- 7.4 mg/dL in controls (P = NS). Full enteral nutrition was achieved in 36 (67%) of 54 patients with severe SBS vs 20 (67%) of 30 patients in the control group (P = NS). The overall survival rate, however, was 89% (48/54), which is significantly higher than in the historical controls (70%, 21/30; P < .05). CONCLUSIONS A multidisciplinary approach to intestinal rehabilitation allows for fully integrated care of inpatients and outpatients with SBS by fostering coordination of surgical, medical, and nutritional management. Our experience with 2 comparable cohorts demonstrates that this multidisciplinary approach is associated with improved survival.

Long-term bowel function and quality of life in children with Hirschsprung's disease.

BACKGROUND/PURPOSE Little is known about the quality of life (QOL) of children with Hirschsprungs disease (HD) as they grow older. The purpose of this study was to measure the QOL and bowel function of these children as they mature. METHODS All children who were surgically treated for HD at British Columbia Childrens Hospital, Vancouver, British Columbia, Canada between 1986 and 2003 were invited to participate. Each family was sent 3 previously validated questionnaires exploring current QOL and bowel function. RESULTS Fifty-one families participated (49%), with children between the ages of 3 and 21 years. Fecal continence improved significantly with age (P = .04) and was the strongest predictor of QOL scores of all variables in our study. There was no statistically significant difference in QOL scores between children with HD and healthy children, although a clinically relevant impairment in QOL may be present, especially in psychosocial scores. CONCLUSIONS Fecal continence is an important predictor of overall QOL in children surgically treated for HD. Although continence tends to improve with age, a number of older children still have ongoing continence problems, and they seem to be a group at risk for impaired QOL. Our study indicates that interventions for children with incontinence may offer gains in QOL as well as bowel function.

Adrenal insufficiency in the critically ill neonate and child.

Purpose of review Adrenal insufficiency, common in critically ill patients of all ages, has recently gained prominence as a significant pathologic entity in pediatrics. This review describes the current diagnostic approach to detecting adrenal insufficiency and the clinical consequences in critically ill children and infants. It also discusses the current therapeutic approach to adrenal insufficiency in critically ill patients. Recent findings Relative adrenal insufficiency and its clinical implications have recently come into focus with observational studies demonstrating a high prevalence in pediatric septic shock patients and a significant associated morbidity. Neonatal studies have clarified diagnostic testing and defined clinical outcomes associated with adrenal insufficiency in preterm infants. Comparisons of bioavailable and total cortisol levels demonstrate the utility of total cortisol testing in pediatric septic shock patients. Summary Adrenal insufficiency contributes to morbidity in critically ill neonates and children. Diagnostic testing by adrenocorticotropin stimulation tests should be done in patients unresponsive to standard treatment of shock. Prospective, randomized clinical trials in critically ill neonates and children with adrenal insufficiency are required to determine if these populations will benefit from glucocorticoid replacement therapy.

Preservation of intestinal motility after the serial transverse enteroplasty procedure in a large animal model of short bowel syndrome

INTRODUCTION Serial transverse enteroplasty (STEP) has been shown to improve bowel function in short bowel syndrome. The effect of the STEP procedure on intestinal motility is not known, but some have hypothesized that it could disrupt bowel innervation and thus impair intestinal motility. METHODS Growing Yorkshire pigs (n = 7) underwent 3 operations at 6-week intervals: (1) reversal of 50 cm of jejunum, (2) 90% bowel resection +/- STEP to the proximal dilated bowel (4 STEP, 3 control), and (3) implantation of serosal strain gauges. At each operation, baseline and post-octreotide small intestinal motility was studied with continuously perfused manometry catheters using non-anticholinergic anesthesia. In addition, awake monitoring was performed using strain gauge analysis 1 week after the third operation. Characteristics of phase III of the migrating motor complex (MMC) were compared between and within groups using t test, chi(2), and analysis of variance, with significance set at P < .05. RESULTS Manometry data from the third surgery revealed no differences between groups or compared with baseline within groups for the presence and characteristics of phase III of the MMC. Specifically, the mean amplitude and frequency of phase III after octreotide, and both the mean baseline and mean octreotide-stimulated motility indices were equivalent. The duration of phase III after octreotide stimulation was significantly increased in the STEP animals, suggesting a potential benefit of the STEP procedure. Strain gauge analysis, performed in awake animals, confirmed no differences between the groups for basal and octreotide-stimulated characteristics of phase III of the MMC. CONCLUSIONS These preliminary data suggest that the STEP procedure in a porcine model of short bowel syndrome does not interfere with baseline or hormonally stimulated motility within the small bowel. These findings further support the STEP procedure as a safe option for the surgical management of short bowel syndrome.

Outcomes and unmet need for neonatal surgery in a resource-limited environment: Estimates of global health disparities from Kampala, Uganda

PURPOSE Reported outcomes of neonatal surgery in low-income countries (LICs) are poor. We examined epidemiology, outcomes, and met and unmet need of neonatal surgical diseases in Uganda. METHODS Pediatric general surgical admissions and consults from January 1, 2012, to December 31, 2012, at a national referral center in Uganda were analyzed using a prospective database. Outcomes were compared with high-income countries (HICs), and met and unmet need was estimated using burden of disease metrics (disability-adjusted life years or DALYs). RESULTS 23% (167/724) of patients were neonates, and 68% of these survived. Median age of presentation was 5days, and 53% underwent surgery. 88% survived postoperatively, while 55% died without surgery (p<0.001). Gastroschisis carried the highest mortality (100%) and the greatest mortality disparity with HICs. An estimated 5072 DALYs were averted by neonatal surgery in Uganda (met need), with 140,154 potentially avertable (unmet need). Approximately 3.5% of the need for neonatal surgery is met by the health system. CONCLUSIONS More than two thirds of surgical neonates survived despite late presentation and lack of critical care. Epidemiology and outcomes differ greatly with HICs. A high burden of hidden mortality exists, and only a negligible fraction of the population need for neonatal surgery is met by health services.

Parathyroid carcinoma in more than 1,000 patients: A population-level analysis.

BACKGROUND Parathyroid carcinoma (PC) is a rare malignancy with a moderate prognosis. The staging system, prognostic indicators, and optimal surgical management are still under debate. This large cohort explores prognostic factors for PC. METHODS 1,022 cases of PC in the 1998-2011 National Cancer Data Base that underwent surgery were examined for predictors of lower overall survival (OS) and relative risk (RR) of death at 5 years. RESULTS The 5-year OS was 81.1% in 528 patients with ≥ 60 months of follow-up. The overall cohort was mainly non-Hispanic (96.5%), white (77.4%), and insured (94.3%), with a median age of 57 years. Mean OS was lower and RR of death greater in older (P < .001), black (P = .007) patients with a secondary malignancy (P = .015) and ≥ 2 comorbidities (P = .005), whose surgical specimen had positive surgical margins (P = .026) or positive lymph nodes (P < .001). Multivariate cox regression demonstrated that positive lymph nodes (hazard ratio [HR], 6.47; 95% CI, 1.81-23.11) and older age (HR, 2.35; 95% CI, 1.25-4.43) were associated with lower OS. CONCLUSION PC is a rare malignancy with a 5-year OS of 81.1%. Positive lymph nodes and older age predict lower OS and an increased risk of death.

The Global Paediatric Surgery Network: A Model of Subspecialty Collaboration Within Global Surgery

Attention to surgical conditions in lowand middle-income countries (LMICs) has increased in recent years. Because half of the population in the world’s poorest countries are children [1], paediatric surgical conditions compose a significant proportion of the global burden of disease (BoD), and there are critical shortages in workforce and skills to treat these diseases in LMICs. Several populationbased studies have highlighted the magnitude of the need for paediatric surgery and the limited capacity, both in human resources and in infrastructure, to tackle the problem [2, 3]. Africa, in particular, has a grave shortage of paediatric surgeons. The number of fully trained paediatric surgeons ranges from 1 in Malawi (population 13 million) to 120 in Egypt (population of 80 million). In more than

Endoloop versus endostapler closure of the appendiceal stump in pediatric laparoscopic appendectomy.

BACKGROUND There is little information available to inform choice of technique for appendiceal stump control in pediatric laparoscopic appendectomy (LA). We compared complications (stump leak, intra-abdominal abscess formation [IAA], surgical site infection [SSI]) in children undergoing LA for perforated (PA) and nonperforated appendicitis (NPA) by technique of appendiceal stump control. METHODS All children who underwent LA for confirmed acute appendicitis between 2006 and 2009 were reviewed. Choice of stump control (endoloop [EL] or endostapler [ES]) was determined by surgeon preference. Interactions between stump closure techniques and other potential confounders (intra-abdominal drain, irrigation, different antibiotic regimens) were explored using a logistic regression model. RESULTS Of 242 patients undergoing LA, 57 (23.6%) had PA. In the PA group the appendiceal stump was closed with EL in 47 (82.5%) patients, while in the NPA group EL was used in 161 (87%) patients. Among PA patients, IAA was more common in the ES than the EL group (5 of 10 [50%] v. 6 of 47 [12.7%]). There was no significant difference in rates of SSI. Among NPA patients, there were no differences in rates of IAA or SSI. There were no stump leaks in either group. Logistic regression analysis confirmed the predictive effect of ES use on IAA formation in PA (adjusted odds ratio 7.09; 95% confidence interval 1.08-46.13; p = 0.042). CONCLUSION Our data suggest that in most cases of PA, the appendiceal stump can be safely controlled with EL. Within the PA group, the higher rates of IAA seen in ES patients may be attributable to the quality of the appendiceal stump rather than the technique of closure.

Pediatric surgery as an essential component of global child health

Recent initiatives in global health have emphasized universal coverage of essential health services. Surgical conditions play a critical role in child health in resource-poor areas. This article discusses (1) the spectrum of pediatric surgical conditions and their treatment; (2) relevance to recent advances in global surgery; (3) challenges to the prioritization of surgical care within child health, and possible solutions; (4) a case example from a resource-poor area (Uganda) illustrating some of these concepts; and (5) important child health initiatives with which surgical services should be integrated. Pediatric surgery providers must lead the effort to prioritize childrens surgery in health systems development.

Colorectal carcinoma in pediatric patients: A comparison with adult tumors, treatment and outcomes from the National Cancer Database

BACKGROUND Pediatric colorectal cancer (CRC) is rare. Comparison with adult CRC tumors, management, and outcomes may identify opportunities for improvement in pediatric CRC care. STUDY DESIGN CRC patients in the National Cancer Data Base from 1998 to 2011, were grouped into Pediatric (≤21years), early onset adult (22-50) and older adult (>50) patients. Groups were compared with χ(2) and survival analysis. RESULTS A total of 918 pediatric (Ped), 157,779 early onset adult (EA), and 1,304,085 older adults (OA) were identified (p<0.01 for all comparisons). Patients ≤50 presented more frequently with stage 3 and 4 disease (Ped: 62.0%, EA: 49.7%, OA: 37.3%) and rectal cancer (Ped: 23.6%, EA: 27.5%, OA: 19.2%). Pediatric histology was more likely signet ring, mucinous, and poorly differentiated. Initial treatment was usually surgery, but patients ≤50 were more likely to have radiation (Ped: 15.1%, EA: 18.6%, and OA: 9.2%) and chemotherapy (Ped: 42.0%, EA: 38.2%, and OA: 22.7%). Children and older adults showed poorer overall survival at 5years when compared to early onset adults. Adjusting for covariates, age ≤21 was a significant predictor of mortality for colon and rectal cancers (colon HR: 1.22, rectal HR: 1.69). CONCLUSIONS This is the largest cohort of pediatric CRC patients, revealing more aggressive tumor histology and behavior in children, particularly in rectal cancer. Despite standard oncologic treatment, age ≤21 was a significant predictor of mortality. This is likely owing to worse tumor biology rather than treatment disparities and may signal the need for different therapeutic strategies.