Prevention of Parenteral Nutrition-associated Cholestasis Using Reduced Dose Soybean Lipid Emulsion: A Multicenter Randomized Trial.

INTRODUCTION: Reducing soybean lipid emulsion (SLE) dose may prevent parenteral nutrition-associated cholestasis (PNAC) but effects on growth and neurodevelopment are unknown. The purpose of this study was to evaluate the effect of reduced dose SLE on growth and neurodevelopment. METHODS: Surgical neonates at 4 centers were randomized to standard SLE (3 g/kg/day) or reduced SLE (1 g/kg/day) over a 12-week period. Bilirubin levels and growth parameters were measured baseline and weekly while on study. The effects of time and group on direct bilirubin and growth were evaluated with a linear mixed effects model. Neurodevelopmental outcomes were assessed at 12- and 24-months corrected gestational age. RESULTS: Twenty-one individuals were randomized (standard dose = 9, reduced dose = 12). Subjects in the reduced dose group had slower rates of direct bilirubin increase and overall levels decreased earlier than those in the standard dose group. There was a trend toward a faster direct bilirubin decrease in the reduced dose group (p = 0.07 at day 84). There were no differences in the rates of change in weight (p = 0.352 at day 84) or height Z-scores (p = 0.11 at day 84) between groups. One subject in the reduced dose group had abnormal neurodevelopmental testing at 24 months. CONCLUSIONS: Surgical neonates randomized to a reduced dose of SLE had improved trends in direct bilirubin levels without clinically significant differences in overall growth and neurodevelopment. TYPE OF STUDY: Randomized Controlled Trial. LEVEL OF EVIDENCE: II.

Neonatal pneumothorax in congenital diaphragmatic hernia: Be wary of high ventilatory pressures.

Background: Patients with congenital diaphragmatic hernia (CDH) require invasive respiratory support and higher ventilator pressures may be associated with barotrauma. We sought to evaluate the risk factors associated with pneumothorax in CDH neonates prior to repair. Methods: We retrospectively reviewed newborns born with CDH between 2014 and 2019 who developed a pneumothorax, and we matched these cases to patients with CDH without pneumothorax. Results: Twenty-six patients were included (n=13 per group). The pneumothorax group required extracorporeal life support (ECLS) more frequently (85% vs 54%, p=0.04), particularly among type A/B defects (31% vs 7%, p=0.01). The pneumothorax group had higher positive end-expiratory pressure (PEEP) within 1 hour of birth (p=0.02), at pneumothorax diagnosis (p=0.003), and at ECLS (p=0.02). The pneumothorax group had a higher mean airway pressure (Paw) at birth (p=0.01), within 1 hour of birth (p=0.01), and at pneumothorax diagnosis (p=0.04). Using multiple logistic regression with cluster robust SEs, higher Paw (OR 2.2, 95% CI 1.08 to 3.72, p=0.03) and PEEP (OR 1.8, 95% CI 1.15 to 3.14, p=0.007) were associated with an increased risk of developing pneumothorax. There was no difference in survival (p=0.09). Conclusions: Development of a pneumothorax in CDH neonates is independently associated with higher Paw and higher PEEP. A pneumothorax increases the likelihood of treated with ECLS, even with smaller defect.

Hepatic Function in Premature Lambs Supported by the Artificial Placenta and Total Parenteral Nutrition.

The artificial placenta (AP) promotes organ development and reduces organ injury in a lamb model of extreme prematurity. This study evaluates hepatic outcomes after AP support with total parenteral nutrition (TPN) administration. Premature lambs (116-121 days estimated gestational age; term = 145) were cannulated for 7 days of AP support. Lambs received TPN with SMOFlipid (n = 7) or Intralipid (n = 5). Liver function and injury were compared between the two groups biochemically and histologically. Groups were compared by ANOVA with Tukey's multiple comparisons or linear-mixed effects models. From baseline to day 7, total bilirubin (Intralipid 2.6 ± 2.3 to 7.9 ± 4.4 mg/dl; SMOFlipid 0.3 ± 0.1 to 5.5 ± 2.3 mg/dl), alanine aminotransferase, and gamma-glutamyl transferase increased in both groups ( p < 0.001 for all). Direct bilirubin (0.3 ± 0.2 to 1.8 ± 1.4 mg/dl; p = 0.006) and AST (27 ± 5 to 309 ± 242 mg/dl; p < 0.001) increased in SMOFlipid group (not measured in Intralipid group). On liver histology, Intralipid showed more cholestasis than SMOFlipid; both groups showed more than tissue controls. The Intralipid group alone showed hepatocyte injury and had more congestion than controls. Lambs supported by the AP with TPN administration maintain normal hepatic function and sustain minimal hepatic injury. SMOFlipid is associated with decreased cholestasis and hepatic injury versus Intralipid.

Interdisciplinary Care and a Focus on Fertility Preservation When Multi-cystic Ovaries Cause Ovarian Torsion: A Case of a 9-year-old Girl with Severe, Undiagnosed Hypothyroidism.

BACKGROUND: Ovarian torsion can occur in Van Wyk Grumbach syndrome, a disorder characterized by severe primary hypothyroidism and ovarian enlargement. To date, all documented cases of torsion in this setting describe oophorectomy, which has significant hormonal and fertility implications. CASE: A 9-year-old pubertal girl presented to the emergency room with abdominal pain. Magnetic resonance imaging demonstrated bilateral, multi-cystic ovaries. Operative laparoscopy confirmed unilateral adnexal torsion, and detorsion without oophorectomy was accomplished. Postoperative laboratory tests revealed severe primary hypothyroidism. Ovarian size was reduced with hormone replacement therapy. SUMMARY AND CONCLUSION: This case demonstrates that prompt interdisciplinary intervention and awareness of severe hypothyroidism as a cause of ovarian torsion related to enlarged, multi-cystic ovaries may reduce the rate of oophorectomy, allowing preservation of pediatric patients' future fertility, and reducing morbidity postoperatively through prompt, long-term thyroid supplementation.

Safety of delayed decannulation of venoarterial cannulas in patients with congenital diaphragmatic hernia.

BACKGROUND: The practice of "cutting-away" from venoarterial extracorporeal life support (ECLS) and leaving indwelling heparinized cannulas prior to decannulation is controversial. This study aims to determine the safety and efficacy of this strategy in patients with congenital diaphragmatic hernia (CDH) who require ECLS. METHODS: A single-center retrospective review of electronic health records was performed on all patients with CDH who underwent elective ECLS decannulation between January 2014 and September 2018. Descriptive statistics are presented as medians with interquartile range. RESULTS: Seventy-three percent (19/26) of patients who underwent venoarterial ECLS for CDH were electively decannulated. After a median ECLS run of 10.7 days [6.1-19.5], patients were "cut-away" for a median of 26 h [19.8-43] prior to decannulation. One patient required re-initiation at 36 h for a pulmonary hypertensive crisis (5%). There were no major bleeding or embolic events while "cut-away", and four (21%) patients had clots removed from the cannulas without clinical sequelae. One patient was recannulated 16 days following initial decannulation. CONCLUSIONS: Our data suggests that "cutting-away" from ECLS in patients with congenital diaphragmatic hernia is safe and allows a period of observation without significant complications. This strategy may be particularly helpful in patients at risk for recannulation, but better prognostic criteria are needed. LEVEL OF EVIDENCE: Level IV. TYPE OF STUDY: Treatment Study.

A standardized guideline for antibiotic prophylaxis in surgical neonates.

Infection following surgical procedures leads to increased morbidity and mortality in all populations. Guidelines to aid providers in the proper use of prophylactic antibiotics exist for adults, but are rare in the neonatal surgical population. A recent emphasis on appropriate antibiotic stewardship had led to the development of more guidelines without a coincident increase in surgical site infection. Robust data from randomized, controlled trials, however, remain sparse.

Ovarian masses in the child and adolescent: An American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee systematic review.

BACKGROUND: The treatment of ovarian masses in pediatric patients should balance appropriate surgical management with the preservation of future reproductive capability. Preoperative estimation of malignant potential is essential to planning an optimal surgical strategy. METHODS: The American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee drafted three consensus-based questions regarding the evaluation and treatment of ovarian masses in pediatric patients. A search of PubMed, the Cochrane Library, and Web of Science was performed and Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were followed to identify articles for review. RESULTS: Preoperative tumor markers, ultrasound malignancy indices, and the presence or absence of the ovarian crescent sign on imaging can help estimate malignant potential prior to surgical resection. Frozen section also plays a role in operative strategy. Surgical staging is useful for directing chemotherapy and for prognostication. Both unilateral oophorectomy and cystectomy have been used successfully for germ cell and borderline ovarian tumors, although cystectomy may be associated with higher rates of local recurrence. CONCLUSIONS: Malignant potential of ovarian masses can be estimated preoperatively, and fertility-sparing techniques may be appropriate depending on the type of tumor. This review provides recommendations based on a critical evaluation of recent literature. TYPE OF STUDY: Systematic review of level 1-4 studies. LEVEL OF EVIDENCE: Level 1-4 (mainly 3-4).

Time to appendectomy for acute appendicitis: A systematic review.

OBJECTIVE: The goal of this systematic review by the American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee was to develop recommendations regarding time to appendectomy for acute appendicitis in children within the context of preventing adverse events, reducing cost, and optimizing patient/parent satisfaction. METHODS: The committee selected three questions that were addressed by searching MEDLINE, Embase, and the Cochrane Library databases for English language articles published between January 1, 1970 and November 3, 2016. Consensus recommendations for each question were made based on the best available evidence for both children and adults. RESULTS: Based on level 3-4 evidence, appendectomy performed within 24h of admission in patients with acute appendicitis does not appear to be associated with increased perforation rates or other adverse events. Based on level 4 evidence, time from admission to appendectomy within 24h does not increase hospital cost or length of stay (LOS). Data are currently limited to determine an association between the timing of appendectomy and parent/patient satisfaction. CONCLUSIONS: There is a paucity of high-quality evidence in the literature regarding timing of appendectomy for patients with acute appendicitis and its association with adverse events or resource utilization. Based on available evidence, appendectomy performed within the first 24h from presentation is not associated with an increased risk of perforation or adverse outcomes. TYPE OF STUDY: Systematic Review of Level 1-4 studies.

Ovarian torsion in pediatric and adolescent patients: A systematic review.

OBJECTIVE: Ovarian torsion in pediatric patients is a rare event and is primarily managed by pediatric general surgeons. Torsion can be treated with detorsion of the ovary or oopherectomy. Oopherectomy is the most common procedure performed by pediatric general surgeons for ovarian torsion. The purpose of this systematic review by the American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee was to examine evidence from the medical literature and provide recommendations regarding the optimal treatment of ovarian torsion. METHODS: Using PRISMA guidelines, six questions were addressed by searching Medline, Cochrane, Embase Central and National clearing house databases using relevant search terms. Risks of ovarian detorsion including thromboembolism and malignancy, indications for oophoropexy, benefits of detorsion including recovery of function and subsequent fertility, and recommended surveillance after detorsion were evaluated. Consensus recommendations were derived for each question based on the best available evidence. RESULTS: Ninety-six studies were included. Risks of ovarian detorsion such as thromboembolism and malignancy were reviewed, demonstrating minimal evidence for unknowingly leaving a malignancy behind in the salvaged ovary and no evidence in the literature of thromboembolic events after detorsion of a torsed ovary. There is no clear evidence supporting the benefit of oophoropexy after a single episode of ovarian torsion. The gross appearance of the ovary does not correlate with long-term ovarian viability or function. Pregnancies have occurred in patients after detorsion of an ovary both spontaneously and with harvested oocytes from previously torsed ovaries. The consensus recommendation for imaging surveillance following ovarian detorsion is an ultrasound at 3months postprocedure but sooner if there is a concern for malignancy. CONCLUSION: There appears to be overwhelming evidence supporting ovarian detorsion rather than oopherectomy for the management of ovarian torsion in pediatric patients. Ovarian salvage is safe and is the preferred treatment for ovarian torsion. Most salvaged ovaries will maintain viability after detorsion. TYPE OF STUDY: Systematic review of level 3-4 studies. LEVEL OF EVIDENCE: 3-4.

Avalon catheters in pediatric patients requiring ECMO: Placement and migration problems.

PURPOSE: The Avalon dual-lumen venovenous catheter has several advantages, but placement techniques and management have not been adequately addressed in the pediatric population. We assessed our institutional outcomes and complications using the Avalon catheter in children. METHODS: We reviewed all pediatric patients who had Avalon catheters placed for respiratory failure at our institution, excluding congenital heart disease patients, from April 2009 to March 2016. All patients were managed using our standard ECMO protocol, and cannula position was followed by daily chest x-ray and intermittent echocardiography (ECHO). Data included demographics, diagnosis, PRISM3 predicted mortality, ECMO duration, complications, and survival. The primary outcome was the need for catheter repositioning. RESULTS: Twenty-five patients were included, with mean age 8.3±6.9years and 15±22days of ECMO support. Overall survival was 68% (17/25). Fourteen patients (56%) underwent placement with fluoroscopy in addition to ultrasound and ECHO, primarily after 2013. Overall, thirteen patients (52%) had problems with cannula malposition. 9 of these (69%) required cannula repositioning. Three of 14 (21%) cannulas placed with fluoroscopy required repositioning, compared to 7/11 (64%) placed without fluoroscopy (p=0.05). CONCLUSIONS: Complications are common with the Avalon catheter in children. Safe percutaneous access requires ultrasound guidance, and use of intraoperative fluoroscopy in addition to echocardiography decreases malposition rates. LEVEL OF EVIDENCE: IV (Prognosis study).

Treatment of congenital pulmonary airway malformations: a systematic review from the APSA outcomes and evidence based practice committee.

PURPOSE: Variation in management characterizes treatment of infants with a congenital pulmonary airway malformation (CPAM). This review addresses six clinically applicable questions using available evidence to provide recommendations for the treatment of these patients. METHODS: Questions regarding the management of a pediatric patient with a CPAM were generated. English language articles published between 1960 and 2014 were compiled after searching Medline and OvidSP. The articles were divided by subject area and by the question asked, then reviewed and included if they specifically addressed the proposed question. RESULTS: 1040 articles were identified on initial search. After screening abstracts per eligibility criteria, 130 articles were used to answer the proposed questions. Based on the available literature, resection of an asymptomatic CPAM is controversial, and when performed is usually completed within the first six months of life. Lobectomy remains the standard resection method for CPAM, and can be performed thoracoscopically or via thoracotomy. There is no consensus regarding a monitoring protocol for observing asymptomatic lesions, although at least one chest computerized tomogram (CT) should be performed postnatally for lesion characterization. An antenatally identified CPAM can be evaluated with MRI if fetal intervention is being considered, but is not required for the fetus with a lesion not at risk for hydrops. Prenatal consultation should be offered for infants with CPAM and encouraged for those infants in whom characteristics indicate risk of hydrops. CONCLUSIONS: Very few articles provided definitive recommendations for care of the patient with a CPAM and none reported Level I or II evidence. Based on available information, CPAMs are usually resected early in life if at all. A prenatally diagnosed congenital lung lesion should be evaluated postnatally with CT, and prenatal counseling should be undertaken in patients at risk for hydrops.

Taking a STEP back: Assessing the outcomes of multiple STEP procedures.

PURPOSE: Short bowel syndrome (SBS) is a highly morbid condition primarily because of parenteral nutrition (PN)-associated complications. Bowel lengthening via serial transverse enteroplasty (STEP) has become standard of care. While initial STEPs have resulted in weaning from PN, outcomes of repeated STEPs (ReSTEPs) are not well described. We investigated outcomes of initial STEP compared to ReSTEP procedures. METHODS: This retrospective review of STEPs included 17 children and a total of 24 procedures. Demographics, complications, hospital readmission rates, postoperative costs, and PN weaning were analyzed. RESULTS: Neither patient-specific data nor the etiology of SBS was predictive of requiring a ReSTEP. PN weaning was more likely in the year following a first STEP (18% wean rate vs. 0% for ReSTEP, p>.05). No ReSTEP patients reached enteral autonomy. Enteral nutrition (%EN) increases were greater after first STEP compared to ReSTEP (26.0% vs. 4.7%, p=0.03). This trend was true for bowel length as well, where first STEPs resulted in a 51% increase in bowel length compared to a 20% increase after in ReSTEP (p=0.02). CONCLUSIONS: ReSTEPs failed to result in significant PN weaning, with no ReSTEP patients achieving enteral autonomy during follow-up. Given its higher costs, smaller bowel length gains, and limited ability to produce enteral autonomy, surgeons should carefully consider performing ReSTEP procedures. LEVEL OF EVIDENCE: Level III.

Surgical management of gastroesophageal reflux disease (GERD) in children: A systematic review.

OBJECTIVE: The goal of this systematic review by the American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee was to derive recommendations from the medical literature regarding the surgical treatment of pediatric gastroesophageal reflux disease (GERD). METHODS: Five questions were addressed by searching the MEDLINE, Cochrane, Embase, Central, and National Guideline Clearinghouse databases using relevant search terms. Consensus recommendations were derived for each question based on the best available evidence. RESULTS: There was insufficient evidence to formulate recommendations for all questions. Fundoplication does not affect the rate of hospitalization for aspiration pneumonia, apnea, or reflux-related symptoms. Fundoplication is effective in reducing all parameters of esophageal acid exposure without altering esophageal motility. Laparoscopic fundoplication may be comparable to open fundoplication with regard to short-term clinical outcomes. Partial fundoplication and complete fundoplication are comparable in effectiveness for subjective control of GERD. Fundoplication may benefit GERD patients with asthma, but may not improve outcomes in patients with neurologic impairment or esophageal atresia. Overall GERD recurrence rates are likely below 20%. CONCLUSIONS: High-quality evidence is lacking regarding the surgical management of GERD in the pediatric population. Definitive conclusions regarding the effectiveness of fundoplication are limited by patient heterogeneity and lack of a standardized outcomes reporting framework. TYPE OF STUDY: Systematic review of level 1-4 studies. LEVEL OF EVIDENCE: Level 1-4 (mainly level 3-4).

Lipid management in pediatric intestinal failure.

PURPOSE OF REVIEW: Lipids are a crucial component in parenteral nutrition but their prolonged use can be associated with parenteral nutrition-associated liver disease (PNALD), which may lead to significant morbidity and mortality. The purpose of this review is to discuss the current state of lipid management in pediatric intestinal failure, especially as it relates to the prevention of PNALD. RECENT FINDINGS: Lipid restriction has emerged as a successful strategy in both the treatment and prevention of PNALD in parenteral nutrition-dependent children. There is concern, however, that lipid restriction can lead to essential fatty acid deficiency. This, in turn, may affect neurodevelopmental outcomes. A wide variety of lipid emulsion formulations is available throughout the world although only one, a soybean-based product, is Food and Drug Administration-approved for use in the pediatric population. To date, there are no definitive data favoring one lipid emulsion composition over another. SUMMARY: Pediatric intestinal failure patients are at high risk for the development of PNALD. Although this may be managed, and even prevented, with lipid restrictive strategies, the development of essential fatty acid deficiency in this setting remains a concern. Further studies are needed to determine the ideal lipid emulsion and dose to be used in parenteral nutrition-dependent children.

Pediatric intestinal failure: Predictors of metabolic bone disease.

PURPOSE: The purpose of this study was to identify risk factors for the development of metabolic bone disease (MBD) in pediatric intestinal failure (IF). METHODS: A retrospective single-center study of 36 pediatric IF patients who were screened for MBD was performed. Bone mineral density (BMD) was measured using dual-energy X-ray absorptiometry (DXA). Simple regression analysis was initially performed to screen predictors, followed by multivariate step-wise linear regression analysis to identify risk factors of MBD. RESULTS: Mean lumbar spine BMD Z-score was -1.16 ± 1.32, and 50.0% of patients had a BMD Z-score less than -1.0. Deficiency of 25-hydroxyvitamin-D (25-OHD <30 ng/ml) was present in the 63.8% of patients, while 25.0% had hyperparathyroidism (intact parathyroid hormone (PTH)>55 pg/ml). Seven patients (19.4%) had bone pain, of which 4 (11.1%) suffered a pathologic fracture. Using multivariate analysis, parenteral nutrition (PN) duration predicted decreased BMD (B=-0.132, p=0.006). Serum 25-OHD nonsignificantly correlated with BMD Z-score (B=0.024, p=0.092). Interestingly, repeat DXA after increasing vitamin D supplementation showed no improvement in BMD Z-score (-1.18 ± 1.49 vs -1.36 ± 1.47, p=0.199). CONCLUSIONS: Pediatric IF is associated with a significant risk of MBD, which is predicted by the duration of PN-dependence. These findings underscore the importance of BMD monitoring. Better therapies for treating IF-associated MBD are needed.

Enteral autonomy in pediatric short bowel syndrome: predictive factors one year after diagnosis.

PURPOSE: This study examined predictors of achieving enteral autonomy among pediatric short bowel syndrome (SBS) patients remaining on parenteral nutrition (PN) beyond one year. METHODS: A retrospective single-institution study of 171 pediatric SBS patients (defined as ≥50% small bowel (SB) loss or ≥60 days of PN with onset before 6 weeks of age) was performed. Multivariate Cox proportional hazards analysis was conducted, with subgroup analysis of patients on PN for ≥1 year (n=59). Primary outcome was successful wean from PN. RESULTS: Over a follow-up of 4.1±4.8 years, 64.3% of children weaned from PN. Mortality was 15.2%. Presence of ≥10% expected SB length (hazard ratio [HR] 6.48, p=0.002) or an ileocecal valve (ICV; HR, 2.86, p<0.001) predicted PN weaning. Of those on PN ≥1 year, the wean rate was 50.8%, and ICV no longer predicted weaning (p=0.153). Predictors among those on PN ≥1 year were: ≥10% expected SB length (HR, 8.27, p=0.010), intestinal atresia (HR, 4.26, p=0.011), and necrotizing enterocolitis (NEC, HR, 2.84, p=0.025). CONCLUSIONS: SBS children on PN ≥1 year continue to wean from PN, and those with ≥10% of predicted SB length, NEC, or atresia are more likely to do so. These findings may help direct management and advice for these challenging patients.

Cloaca and hydrocolpos: laparoscopic-, cystoscopic- and colposcopic-assisted vaginostomy tube placement.

Hydrocolpos presenting at birth in a neonate with a cloacal malformation may lead to massive distension, with compression of adjacent structures. At times, the hydrocolpos requires urgent drainage. Additionally, these neonates need a divided colostomy to divert their fecal stream and prevent genitourinary contamination. We present a novel approach by which these two procedures can be performed as a single operation guided by a combination of cystoscopy, colposcopy, and laparoscopy. This provides a minimally invasive technique with excellent outcome and potentially superior visualization of the necessary intra-abdominal structures.

Drug shortage-associated increase in catheter-related blood stream infection in children.

BACKGROUND: Ethanol lock therapy (ELT) has been shown to reduce the incidence of catheter-related blood stream infections (CRBSI) in intestinal failure (IF) patients. Dosing and frequency remains undefined. Scrutiny of pharmaceutical facilities by the Food and Drug Administration led to the voluntary shutdown of the sole supplier of ethanol, resulting in a nationwide shortage. To conserve supply, we reduced ELT frequency from a daily regimen. We examined the impact that reduction in ELT frequency had on CRBSI in pediatric IF patients. METHODS: We retrospectively reviewed our parenteral nutrition-dependent IF children. Primary outcome measure was CRBSI per 1000 catheter days after ELT frequency reduction. Data were compared (paired t test) to the same group over 1 year before ethanol shortage and to historical controls. RESULTS: During the shortage 13 outpatients received ELT. Eight met study criteria. Mean ± SD age was 9.1 ± 7.8 years. Mean CRBSI rate per 1000 catheter days was 0.7 ± 1.3 before ELT shortage. This increased to 6.2 ± 2.5 after frequency reduction (P < .001). This CRBSI rate was similar to historical IF children not on ELT (8.0 ± 5.4). Seven children developed CRBSI after frequency reduction, 6 requiring hospitalization, 2 to the ICU. Mean length of stay (15.5 days) averaged $104,783(± 111,034) in hospital charges. Organisms included Gram-negatives (6), methicillin-resistant Staphylococcus aureus (1), and Candida spp (1). CONCLUSIONS: ELT frequency reduction resulted in complete failure in CRBSI prophylaxis. The nationwide shortage of this drug has been costly both financially and in patient morbidity.

Biliary duplication cyst with heterotopic gastric mucosa resulting in obstruction of the biliary system: a case report.

Biliary tract duplication cysts with heterotopic gastric mucosa are rare congenital anomalies, with our case representing only the fourth reported case in the literature. An 8-year-old girl with several months of abdominal pain was found to have a complex cystic mass communicating with the biliary system via the common hepatic duct. Intraoperatively, inflammation caused by the cystic mass was found to have resulted in a Mirizzi-like syndrome, with a nearly complete obstruction at the confluence of the left and right hepatic ducts. Histopathologic examination of the biliary mass revealed it to be a duplication cyst lined by heterotopic gastric mucosa with secondary ulceration and fibrosis. Biliary duplication cysts are a rare but important process that should be considered in a child with a mass in the portal triad and biliary obstruction.