Endoscopic Management of a Double Duodenal Web: A Case Report of a Rare Alimentary Anomaly.

Duodenal webs are a rare clinical entity with the presentation of a double duodenal web being exceedingly uncommon. Management of duodenal webs traditionally involves duodenal web excision with duodenoduodenostomy, which is usually performed via a laparoscopic or an open approach. We report the case of a 6-month-old child who presented with progressively worsening bilious emesis with imaging findings concerning for a duodenal web. Endoscopic evaluation was performed that identified 2 webs in the fourth portion of the duodenum. These were managed completely endoscopically with balloon dilation. Although surgery is the mainstay of treatment of duodenal webs, this patient was successfully managed by endoscopic intervention without the need for open or laparoscopic excision, which has not been previously described for double duodenal webs. This work demonstrates the safety and efficacy of endoscopic management for infants with this anomaly.

Pediatric Pedestrian Injuries: Striking Too Close to Home.

BACKGROUND: Pediatric pedestrian injuries (PPI) are a major public health concern. This study utilized geospatial analysis to characterize the risk and injury severity of PPI. METHODS: A retrospective chart review of PPI patients (age < 18) from a level 1 trauma center was performed (2013-2020). A geographic information system geocoded injury location to home and other public landmarks. Incidents were aggregated to zip codes and the Local Indicators of Spatial Association statistic tested for spatial clustering of injury rates per 10,000 children. Predictors for increased injury severity were assessed by logistic regression. RESULTS: PPI encompassed 6% (n = 188) of pediatric traumas. Most patients were black (54%), male (58%), >13 years (56%), and with Medicaid insurance (68%). Nine zip codes comprised a statistically significant cluster of PPI. Nearly half (40%) occurred within a quarter mile of home; 7% occurred at home. Most (65%) PPI occurred within 1 mile of a school, and 45% occurred within a quarter mile of a park. Nearly all (99%) PPI occurred within a quarter mile of a major intersection and/or roadway. Using admission to ICU as a marker for injury severity, farther distance from home (OR 1.060, 95% CI 1.001-1.121, p = 0.045) and age <13 years (3.662, 95% CI 1.854-7.231, p < 0.001) were independent predictors of injury severity. CONCLUSIONS: There are significant sociodemographic disparities in PPI. Most injuries occur near patients' homes and other public landmarks. Multidisciplinary injury prevention collaboration can help inform policymakers, direct local safety programs, and provide a model for PPI prevention at the national level. LEVEL OF EVIDENCE: Level IV.

Performance of islets of Langerhans conformally coated via an emulsion cross-linking method in diabetic rodents and nonhuman primates.

Polyethylene glycol (PEG)-based conformal coating (CC) encapsulation of transplanted islets is a promising ß cell replacement therapy for the treatment of type 1 diabetes without chronic immunosuppression because it minimizes capsule thickness, graft volume, and insulin secretion delay. However, we show here that our original CC method, the direct method, requiring exposure of islets to low pH levels and inclusion of viscosity enhancers during coating, severely affected the viability, scalability, and biocompatibility of CC islets in nonhuman primate preclinical models of type 1 diabetes. We therefore developed and validated in vitro and in vivo, in several small- and large-animal models of type 1 diabetes, an augmented CC method-emulsion method-that achieves hydrogel CCs around islets at physiological pH for improved cytocompatibility, with PEG hydrogels for increased biocompatibility and with fivefold increase in encapsulation throughput for enhanced scalability.

National burden of pediatric abusive injuries: patterns vary by age.

PURPOSE: Child abuse is often unrecognized by healthcare practitioners. This study sought to characterize pediatric abuse injury patterns and associated mortality rates in a nationwide cohort. METHODS: The Kids' Inpatient Database (1997-2012) was queried for patients < 18 years old with a diagnosis of child abuse (utilizing ICD-9 codes for abusive head trauma [AHT], neglect, physical, emotional, sexual, and other abuse). Demographic factors, mortality, and injury patterns were compared with demographic factors using standard statistical tests. RESULTS: > 39,000 children were hospitalized for abuse from 1997 to 2012. The majority were Caucasian (36%), male (51%) and < 4 years old (70%). Most sustained physical abuse (53%), followed by AHT (14%), sexual (9%) and emotional abuse (2%). Multiple injuries were sustained by 44% of patients. Mortality was 4% (n = 1476). Burns (2%) and intrathoracic (2%) injuries had the highest mortality compared to other injuries (21% and 19%, respectively; both P < 0.001). Emotional abuse (5%) and neglect (1%) were highest in those ≥ 13 years old (both P  = 0.001). CONCLUSION: Physical injuries are less common in adolescents (13-18 years) hospitalized for abuse. However, they are more likely to report emotional abuse and neglect, which has not been compared in previous studies. Intrathoracic injuries and AHT are associated with significant mortality.

Liquid Nitrogen Applied at Point of Sale: A New Presentation of Gastric Perforation in Children.

ABSTRACT: The August 30, 2018, US Food and Drug Administration advisory warned consumers to avoid eating, drinking, or handling food products prepared with liquid nitrogen (LN) (US FDA. Safety Alerts & Advisories: FDA Advises Consumers to Avoid Eating, Drinking, or Handling Food Products Prepared with Liquid Nitrogen at the Point of Sale. US Food and Drug Administration). We report on the case of a pediatric patient sustaining gastric perforation after ingestion of LN applied to food at the point of sale in the United States. "Dragon's Breath" is a popular snack of cereal puffs coated in LN giving the allusion of breathing smoke on ingestion. Instructions provided by vendors include avoidance of touching or drinking the liquid in the bottom of the cup. We report on a case of a 9-year-old girl presenting with peritonitis and gross pneumoperitoneum after consumption of Dragon's Breath with injury conferred secondary to LN ingestion. Intraoperative finding of a large perforation along the lesser curvature of the stomach was repaired primarily with an omental overlay. Her postoperative course was complicated by pneumonia and a surgical site infection. She was discharged 13 days after admission. A review of the literature of previous case reports of LN ingestion by intentional or accidental means is provided. This case report and review of the literature bring awareness of the dangers posed to pediatric patients exposed to LN applied at the point of sale.

A retrospective comparison of outcomes for open vs. laparoscopic surgical techniques in pediatric ulcerative colitis.

BACKGROUND: Ulcerative colitis (UC) is an aggressive disease in the pediatric population and a cause of significant, lifelong morbidity. The aim of this study is to compare surgical complications in pediatric patients undergoing laparoscopic vs. open surgical treatment for UC. METHODS: We queried the Kids' Inpatient Database (KID) for all cases of UC undergoing surgical treatment in 2009 and 2012. We identified patients who received total colectomy without proctectomy (n=413) or total proctocolectomy (n=196) and performed univariate and multivariate analyses comparing laparoscopic vs. open procedures. RESULTS: In pediatric UC patients undergoing total colectomy without proctectomy, open procedures were associated with more complications than laparoscopic, including fluid and electrolyte disorders (40% vs. 28%), surgical wound dehiscence (6% vs. 2%), septicemia (18% vs. 2%), and gastrointestinal disorders (16% vs. 7%) among others, all P<0.05. Likewise, in patients with UC undergoing total proctocolectomy, there were more complications in open vs. laparoscopic technique, including increased transfusion requirements (25% vs. 7%, P=0.001) and significantly more gastrointestinal upset, including nausea, vomiting, and diarrhea (11% vs. 1%, P=0.003). In multivariate analysis, patients who underwent total colectomy with or without proctectomy had an increased risk of experiencing any complication when their procedure was performed in an open or non-elective fashion (all odds ratio >2.4; all P<0.001). CONCLUSIONS: The laparoscopic approach was associated with significantly lower rates of surgical complications in pediatric patients undergoing total colectomy with or without proctectomy for UC. These findings demonstrate that laparoscopic technique compares favorably, and may be preferable, to the open approach in selected pediatric patients with UC.

The limited role of ultrasound in the surgical assessment of solid pediatric breast lesions.

INTRODUCTION: Ultrasound (US) is an adjunct to history and clinical exam (CE) in the assessment of pediatric breast lesions. We sought to investigate the reliability of US and CE to predict final pathologic diameter (P). METHODS: A single institutional retrospective analysis of patients aged ≤18 years who underwent breast mass resection was performed. Data was collected and analyzed using SPSS. RESULTS: 88 patients met inclusion criteria with an average age at surgery of 16 ± 1.5 years. No malignancies were encountered. The largest mean diameter measured by final pathology (MPØ) for all lesions was 4.1 ± 2.6 cm. Pathology encountered were fibroadenoma (83%, MPØ 3.7 ± 1.7 cm), juvenile fibroadenoma (10%, MPØ 7.0 ± 5.4 cm), and low-grade phyllodes tumor (3%, MPØ 6.2 ± 3.8 cm). 67 patients had documented CE measurement with a mean diameter of 3.4 ± 1.8 cm. 62 patients underwent US with a mean diameter of 3.3 ± 1.6 cm. US and CE were accurate in determining P by Cronbach Alpha reliability testing. CONCLUSION: US and CE are reliable measurements of P. The surgical utility of US when considering pediatric breast lesions is limited and should be individualized following pediatric surgical evaluation and CE.

Protocol-Driven Surgical Care of Necrotizing Enterocolitis and Spontaneous Intestinal Perforation.

BACKGROUND: There is no clear consensus on the optimal operative management of premature infants with surgical necrotizing enterocolitis (sNEC) or spontaneous intestinal perforation (SIP); thus, a protocol was developed to guide surgical decision making regarding initial peritoneal drainage (PD) versus initial laparotomy (LAP). We sought to evaluate outcomes after implementation of the protocol. METHODS: Pre-post study including multiple urban hospitals. Premature infants with sNEC/SIP were accrued after implementation of surgical protocol-directed care (June 2014-June 2019). Patients with a birth weight of <750 g and less than 2 wk of age without pneumatosis or portal venous gas were treated with PD on perforation. PD patients received subsequent LAP for clinical deterioration or continued meconium/bilious drainage. Postprotocol characteristics and outcomes were compared with institutional historical controls. Significance set at P < 0.05. RESULTS: Preprotocol and postprotocol cohorts comprise 35 and 73 patients, respectively. There was a statistically significant difference in age at intervention between historical control PD (14 ± 13 d) and postprotocol PD (9 ± 4 d) groups (P = 0.01), PD patient's birth weight (716 ± 212 g versus 610 ± 141 g, P = 0.02) and estimated gestational age of LAP patients (27 ± 1.7 wk versus 31 ± 4 wk, P = 0.002). PD was definitive surgery in 27% (12 of 44) of postprotocol patients compared with 13% (3 of 23) historical controls. A trend in improved survival postprotocol occurred in all PD infants (73% versus 65%), all LAP (75% versus 70%), and for initial PD and subsequent LAP (82% versus 67%). CONCLUSIONS: Utilization of a surgical protocol in sNEC/SIP is associated with improved success of PD as definitive surgery and improved survival.

Waardenburg Syndrome Type IV De Novo SOX10 Variant Causing Chronic Intestinal Pseudo-Obstruction.

Waardenburg syndrome (WS) type IV is characterized by pigmentary abnormalities, deafness and Hirschsprung's disease. This syndrome can be triggered by dysregulation of the SOX10 gene, which belongs to the SOX (SRY-related high-mobility group-box) family of genes. We discuss the first known case of a SOX10 frameshift mutation variant defined as c.895delC causing WS type IV without Hirschsprung's disease. This female patient of unrelated Kuwaiti parents, who tested negative for cystic fibrosis and Hirschsprung's disease, was born with meconium ileus and malrotation and had multiple surgical complications likely due to chronic intestinal pseudo-obstruction. These complications included small intestinal necrosis requiring resection, development of a spontaneous fistula between the duodenum and jejunum after being left in discontinuity, and short gut syndrome. This case and previously reported cases demonstrate that SOX10 gene sequencing is a consideration in WS patients without aganglionosis but with intestinal dysfunction.

Predictors of in-hospital mortality in newborn conjoined twins.

BACKGROUND: Conjoined twins are rare developmental anomalies. There is a paucity of literature other than case reports and small case series. The aim of this study was to examine national outcomes and identify predictors of mortality in newborn conjoined twins. METHODS: We reviewed data on newborn conjoined twins from the Kids' Inpatient Database (1997-2012). RESULTS: A total of 240 patients were identified for a nationally weighted incidence of 1 per 100,000 live births. The majority of conjoined twins were female (n = 190 [81%]). The most commonly associated anomalies were cardiac (n = 87 [36%]), gastrointestinal (n = 41 [17%]), and abdominal wall (n = 32 [13%]) defects. Fifty-six (23%) patients underwent operative procedures, including 28 (12%) neonatal separation surgeries. The overall mortality rate was 61%; most deaths occurred within 24 hours (99 of 146 [68%]) to 48 hours (129 of 146 [88%]) after birth. Mortality was higher in female compared with male children (66% vs 38%, P = .025), premature compared with full-term children (72% vs 44%, P = .007), and in children with extremely low birth weight (95% vs 59%, P = .002). Congenital diaphragmatic hernias were seen in 15 (6%) patients and were uniformly fatal (100% vs 58%, P = .029). Mortality was highest in hospitals not designated as children's hospitals (72%) compared with children's hospitals (44%) (P = .007). CONCLUSION: Conjoined twins are rare anomalies who are susceptible to extremely high perinatal mortality, especially in female children, those who are premature, or those who have low birth weight. These data support caring for these complex patients at hospitals equipped to care for this fragile population.

Analysis of water sports injuries admitted to a pediatric trauma center: a 13 year experience.

BACKGROUND: Unintentional injury is the leading cause of death in children and adolescents. Injuries occurring during boating and recreational water sports are poorly described in the literature. Herein, we compare injuries from water sports to those resulting from motor vehicle collisions, which are better described in existing literature. METHODS: A retrospective review of 1935 consecutive pediatric trauma patients, as defined by age < 18 years, admitted to a single level-1 pediatric trauma center between January 2000 and August 2013 was performed. Patients were divided into two cohorts based on the mechanism of injury: water sports injury (WSI) or motor vehicle collision (MVC). Demographics, injury descriptors, and outcomes were reviewed for each patient. Categorical variables were compared by Chi square or Fisher's exact test, and continuous by t test or Mann-Whitney U test. Parametric data are reported as mean ± standard deviation and nonparametric as median (interquartile range). Significance was set at alpha level 0.05. RESULTS: A total of 18 pediatric patients were admitted for WSI and 615 for MVC during the study period. Among those with WSI, mean age was 12 ± 4 years, mean Injury Severity Score (ISS) was 11 ± 10, and mean Revised Trauma Score (RTS) was 7.841(IQR 6.055-7.841). 44% of WSI occurred by personal watercraft (Jet Ski, WaveRunner), 39% by boat, and 17% by other means (e.g., diving, tubing, kite surfing). Overall, the most common WSI included skin/soft-tissue lacerations (59%), head injury/concussion (33%), tendon/ligament lacerations (28%), and extremity fractures (28%). Compared to 615 patients admitted for MVC, age, sex, race, Glasgow Coma Scale, ISS, RTS, spleen and liver laceration rates, neurosurgical consultation, ICU admission, ICU and total length of stay, and mortality were similar. Patients with WSI were more likely to be tourists (44% vs. 5%, p < 0.001). Those with WSI showed a significantly higher requirement for any surgical intervention (61% vs. 15%, p = 0.001). The rate of open fracture (28% vs. 6%, p = 0.006) and, subsequently, orthopedic procedures (39% vs. 17%, p = 0.027) were also higher in the WSI group. CONCLUSION: Overall, water sports injuries are similar in in-hospital mortality to motor vehicle collisions. They are more likely to result in penetrating trauma and more likely to require surgical intervention. Primary and secondary prevention strategies should specifically target personal watercraft usage and tourist populations.

A pediatric carotid body tumor.

Carotid body tumors are rare childhood extra-adrenal paragangliomas. We present an 8-year old female with a neck mass mistaken as reactive lymphadenopathy. Computed tomography and magnetic resonance angiography, as well as preoperative embolization and balloon test occlusion, were utilized for planning and management. Surgical excision of the tumor was successful and pathological examination revealed a benign paraganglioma. Surgical treatment is curative for these benign lesions, however rare cases have presented years later with metastatic disease. Therefore, a child, like our patient, will require lifelong surveillance for the development of potential metastasis.

Nationwide overview of survival and management of appendiceal tumors in children.

INTRODUCTION: There remains a paucity of literature on survival related to pediatric appendiceal tumors. The purpose of this study was to determine the incidence, surgical management, and survival outcomes of appendiceal tumors in pediatric patients. METHODS: The Surveillance, Epidemiology, and End Results (SEER) Registry was analyzed for pediatric appendiceal tumors from 1973 to 2011. Parameters analyzed were: tumor type, surgical management (appendectomy vs. extensive resection), tumor size, and lymph node sampling. Chi-square analysis for categorical and Student's t test for continuous data were used. RESULTS: Overall, 209 patients had an appendiceal tumor, including carcinoid (72%), appendiceal adenocarcinoma (16%), and lymphoma (12%). Patients undergoing appendectomy vs. extensive resection had similar 15-year survival rates (98% vs. 97%; p=0.875). Appendectomy vs. extensive resection conferred no 15-year survival advantage when patients were stratified by tumor type, including adenocarcinoma (87% vs. 89%; p=0.791), carcinoid (100% vs. 100%; p=0.863), and lymphoma (94% vs. 100%; p=0.639). There was no significant difference in 15-year survival between tumor size groups ≥2 and <2cm (both 100%) and presence or absence of lymph node sampling (96% and 97%; p=0.833) for all patients with a carcinoid tumor. CONCLUSION: Appendectomy may be adequate for pediatric appendiceal tumors. Extensive resection may be of limited utility for optimizing patient survival, placing patient at greater operative risk. TYPE OF STUDY: Retrospective Prognostic Study. LEVEL OF EVIDENCE: III.

Incidence and outcomes of pediatric extremity melanoma: A propensity score matched SEER study.

BACKGROUND: There is a paucity of literature on treatment of melanoma in children with surgical management extrapolated from adult experience. The incidence and clinical outcomes of pediatric extremity melanoma were studied. METHODS: SEER registry was analyzed between 1973 and 2010 for patients <20years old with extremity melanoma. Multivariate and propensity-score matched analyses were performed to identify independent predictors of survival. RESULTS: Overall, 917 patients were identified with an age-adjusted incidence of 0.2/100,000 persons, annual percent change 0.96. Most had localized disease (77%), histology revealing melanoma-not otherwise specified (52%). Surgical procedures performed included wide local excision (50%), excisional biopsy (32%), lymphadenectomy (LA) (28%), and sentinel lymph node biopsy (SLNB) (15%). Overall, 30-year disease specific mortality was 7% with lower survival for extremity melanoma (90%), males (89%), nodular histology (69%), and distant disease (36%) (all P<0.05). Post-treatment multivariate analysis revealed localized disease (HR 9.76; P=0.006) as an independent prognosticator of survival; earlier diagnostic years 1988-1999 (HR 2.606; P=0.017) were a negative prognosticator of survival. Propensity-score matched analysis found no difference in survival between SLNB/LA vs no sampling for regional/distant disease. CONCLUSIONS: Pediatric extremity melanoma in SEER demonstrate no survival advantage between children undergoing sampling procedures vs no sampling for regional/distant disease. TYPE OF STUDY: Retrospective, prognostic study. LEVEL OF EVIDENCE: III.

Looped suture versus stapler device in pediatric laparoscopic appendectomy: a comparative outcomes and intraoperative cost analysis.

BACKGROUND: Appendiceal ligation during pediatric laparoscopic appendectomy (LA) may be performed using looped suture versus stapler. Controversy regarding the utility of either method exists. Clinical outcomes and cost analysis of LA with both methods were compared. METHODS: All pediatric LA were performed from fiscal years 2013 and 2014 by two pediatric surgeons. While one surgeon used looped suture, the other used stapler exclusively. chi-Square tests were performed to analyze associations. RESULTS: Two hundred thirty-eight cases were analyzed where looped suture versus stapler LA was performed in 46% and 54% of patients, respectively. Operating room costs were $317.10 and $707.12/person for looped suture and stapler LA, respectively (P<0.0001). Difference in cost of $390.02/person was attributed solely to ligation type. On bivariate analysis, rate of in-hospital complications, length of stay, return-to-ER and readmission within 30 days did not significantly differ between groups. CONCLUSION: A comparative analysis of looped suture versus stapler device during LA for pediatric appendicitis revealed that postoperative complications, length of stay, ER visits and readmissions were not significantly different. Looped suture LA was significantly more cost efficient than stapler LA. In pediatric appendicitis, appendiceal ligation during LA may be performed safely and cost effectively with looped suture versus stapler. TYPE OF STUDY: Cost effectiveness LEVEL OF EVIDENCE: III.

Accessory pancreatic lobe in association with a gastric duplication cyst.

Gastric duplication cysts are an extremely rare anomaly with few reported cases in association with accessory pancreatic tissue. Diagnosis can be challenging given a presentation of recurrent pancreatitis and resemblance to pancreatic pseudocysts. We report the case of a 6-year old boy with multiple episodes of pancreatitis who was discovered to have an accessory pancreatic lobe connected to a gastric duplication cyst, successfully treated with surgical excision.

Splenic Cysts.

Splenic cysts are rare in the United States but more common in regions of the world where Echinococcus is endemic. Cysts are typically classified as true cysts or pseudocysts. True cysts can be parasitic or nonparasitic in origin, whereas most pseudocysts are a result of previous trauma. Recent recognition of features shared by true cysts and pseudocysts suggests the classification system may need to be revised. The prevalence of splenic cysts has increased secondary to the widespread use of abdominal imaging and successful nonoperative management of traumatic splenic injuries. Treatment previously consisted primarily of total splenectomy. However, recognition of the importance of the spleen throughout a patient's life has led to changes in the management of splenic disease. Advances in the testing and preoperative localization of splenic lesions have also led to increased efforts in splenic conservation. [Pediatr Ann. 2016;45(7):e251-e256.].

Neonatal kaposiform hemangioendothelioma of the spleen associated with Kasabach-Merritt phenomenon.

Kaposiform hemangioendothelioma is a rare locally aggressive vascular tumor that usually manifests during early childhood. Typically the lesion presents with skin, soft tissue and bone involvement and is characterized histologically by ill-defined nodularity and the presence of spindle cells with resemblance to Kaposi's sarcoma. We report a rare neonatal case of a splenic kaposiform hemangioendothelioma associated with Kasabach-Merritt phenomenon that was diagnosed with radiographic imaging. Because of the rapid onset of thrombocytopenia and anemia, the patient required urgent splenectomy with subsequent resolution of the blood dyscrasias.