Amina M. Bhatia

Cytokine levels in the preterm infant with neonatal intestinal injury.

OBJECTIVES The purpose of this study is to characterize the cytokine response of preterm newborns with surgical necrotizing enterocolitis (NEC) or spontaneous intestinal perforation (SIP) before surgical treatment and to relate these finding to intestinal disease (NEC vs. SIP). STUDY DESIGN The study was a 14-month prospective, cohort study of neonates undergoing surgery or drainage for NEC or SIP or surgical ligation of patent ductus arteriosus (PDA). Multiplex cytokine detection technology was used to analyze six inflammatory markers: interleukin-2, interleukin-6 (IL-6), interleukin-8 (IL-8), interleukin-1 β (IL-1β), interferon-gamma, and tumor necrosis factor-α (TNF-α). RESULTS Patients with NEC had much higher median preoperative levels of IL-6 (NEC: 8,381 pg/mL; SIP: 36 pg/mL; PDA: 25 pg/mL, p < 0.001), IL-8 (NEC: 18,438 pg/mL; SIP: 2,473 pg/mL; PDA: 1,110 pg/mL, p = 0.001), TNF-α (NEC: 161 pg/mL; SIP: 77 pg/mL; PDA: 71 pg/mL, p < 0.001), and IL-1β (NEC: 85 pg/mL; SIP: 31 pg/mL; PDA: 24 pg/mL, p = 0.001). Patients with NEC totalis (NEC-totalis had the highest levels of IL-8 and were significantly different from infants with limited NEC (28,141 vs. 11,429 pg/mL, p = 0.03). CONCLUSION Surgical NEC is a profoundly more proinflammatory disease than SIP. The cytokine profiles of patients with SIP are closer to those of a nonseptic surgical neonate.

Pseudoangiomatous stromal hyperplasia of the breast in pediatric patients: an underrecognized entity.

Pseudoangiomatous stromal hyperplasia (PASH) is a benign lesion consisting of mammary stromal proliferation with anastomosing slits mimicking vascular spaces. Grossly, it most often resembles fibroadenoma, but may commonly be confused with angiosarcoma and other types of benign vascular proliferations. While PASH has been described in female and male adults since the mid-1980s, there have been only a few accounts in the pediatric population. We present a series of 12 pediatric patients with PASH, including a 3-year-old male, who we believe to be the youngest patient to present with this entity. In our study, PASH was found in 12% of tumors diagnosed preoperatively as fibroadenomas and in 12% of cases diagnosed preoperatively as gynecomastia. Our series documents that PASH is not uncommon in pediatric breast pathology and delineates important differences between adult and pediatric presentations of this entity.

Pathogenesis of NEC: Role of the innate and adaptive immune response

Necrotizing enterocolitis (NEC) is a devastating disease in premature infants with high case fatality and significant morbidity among survivors. Immaturity of intestinal host defenses predisposes the premature infant gut to injury. An abnormal bacterial colonization pattern with a deficiency of commensal bacteria may lead to a further breakdown of these host defense mechanisms, predisposing the infant to NEC. Here, we review the role of the innate and adaptive immune system in the pathophysiology of NEC.

Heterogeneity in trauma registry data quality: Implications for regional and national performance improvement in trauma

BACKGROUND Led by the American College of Surgeons Trauma Quality Improvement Program, performance improvement efforts have expanded to regional and national levels. The American College of Surgeons Trauma Quality Improvement Program recommends 5 audit filters to identify records with erroneous data, and the Georgia Committee on Trauma instituted standardized audit filter analysis in all Level I and II trauma centers in the state. STUDY DESIGN Audit filter reports were performed from July 2013 to September 2014. Records were reviewed to determine whether there was erroneous data abstraction. Percent yield was defined as number of errors divided by number of charts captured. RESULTS Twelve centers submitted complete datasets. During 15 months, 21,115 patient records were subjected to analysis. Audit filter captured 2,901 (14%) records and review yielded 549 (2.5%) records with erroneous data. Audit filter 1 had the highest number of records identified and audit filter 3 had the highest percent yield. Individual center error rates ranged from 0.4% to 5.2%. When comparing quarters 1 and 2 with quarters 4 and 5, there were 7 of 12 centers with substantial decreases in error rates. The most common missed complications were pneumonia, urinary tract infection, and acute renal failure. The most common missed comorbidities were hypertension, diabetes, and substance abuse. CONCLUSIONS In Georgia, the prevalence of erroneous data in trauma registries varies among centers, leading to heterogeneity in data quality, and suggests that targeted educational opportunities exist at the institutional level. Standardized audit filter assessment improved data quality in the majority of participating centers.

Cardiaplication: a novel surgical technique for refractory gastroesophageal reflux in the pediatric population.

INTRODUCTION We encountered 3 patients with severe gastroesophageal reflux disease and tubular stomachs precluding fundoplication. Here, we report the use of an innovative technique, cardiaplication, as an alternative approach for antireflux surgery. METHODS Three infants with medically refractory gastroesophageal reflux disease (GERD) were referred for fundoplication. In each case, the patients anatomy prevented a traditional fundoplication from being performed. A cardiaplication was performed by invaginating the cardia of the stomach at the angle of His and securing the invaginated tissue with interrupted silk suture. The plication tubularized the cardia of the stomach, essentially increasing the intra-abdominal portion of the esophagus and altering the angle of His. The imbrication also creates a flapper valve over the distal esophagus, further limiting potential reflux. The charts for the infants who received cardiaplication were reviewed. Radiographic studies and clinical notes for the presence of persistent reflux were evaluated. RESULTS Cardiaplication was completed in 3 patients with GERD. All cases were initiated laparoscopically and one was converted to an open procedure secondary to dense adhesive disease. Each child was initiated on feeds between postoperative day 2 and 3. Two of the 3 patients were tolerating goal feeds with-in 2 days. The third patient reached goal feeds on day 16. Postoperative imaging (upper gastrointestinal series [UGI]) was obtained in 2 of the 3 patients. At follow-up (13, 7, and 4 months), all 3 patients are clinically free of symptoms of GERD. Delayed radiographic imaging has confirmed that the patients are no longer refluxing. CONCLUSION Based on preliminary findings, cardiaplication appears to be a safe and effective surgical technique for the management of severe GERD in infants. We performed cardiaplication out of necessity; however, after further testing this may prove to be an optimal approach, as it can be performed without disruption of the hiatus.

Structural airway abnormalities contribute to dysphagia in children with esophageal atresia and tracheoesophageal fistula

BACKGROUND Long-term dysphagia occurs in up to 50% of repaired esophageal atresia and tracheoesophageal fistula (EA/TEF) patients. The underlying factors are unclear and may include stricture, esophageal dysmotility, or associated anomalies. Our purpose was to determine whether structural airway abnormalities (SAA) are associated with dysphagia in EA/TEF. METHODS We conducted a retrospective chart review of children who underwent EA/TEF repair in our hospital system from 2007 to 2016. Children with identified SAA (oropharyngeal abnormalities, laryngeal clefts, laryngomalacia, vocal cord paralysis, and tracheomalacia) were compared to those without airway abnormalities. Dysphagia outcomes were determined by the need for tube feeding and the modified pediatric Functional Oral Intake Scale (FOIS) at 1 year. RESULTS SAA was diagnosed in 55/145 (37.9%) patients with EA/TEF. Oropharyngeal aspiration was more common in children with SAA (58.3% vs. 36.4%, p=0.028). Children with SAA were more likely to require tube feeding both at discharge (79.6% vs. 48.3%, p<0.001) and at 1 year (52.7% vs. 13.6%, p<0.001) and had lower mean FOIS (4.18 vs. 6.21, p<0.001). In the logistic regression model adjusting for gestational age, long gap EA, and esophageal stricture, the presence of SAA remained a significant risk factor for dysphagia (OR 4.17 (95% CI 1.58-11.03)). CONCLUSION SAA are common in children with EA/TEF and are associated with dysphagia, even after accounting for gestational age, esophageal gap and stricture. This study highlights the need for a multidisciplinary approach, including early laryngoscopy and bronchoscopy, in the evaluation of the EA/TEF child with dysphagia. LEVEL OF EVIDENCE Level II retrospective prognostic study.

Right paraduodenal hernia leading to bowel strangulation

Up to half of all internal hernias are caused by paraduodenal hernia, a rare congenital midgut malrotation that accounts for less than 1% of all intestinal obstructions. The diagnosis may arise from an incidental finding on abdominal imaging or the patient may present with abdominal pain, vomiting, and obstipation. Early recognition and management of this disease entity are keys because serious complications such as bowel ischemia and infarction may result from a delay in diagnosis. We present a case involving a 14-year-old boy with gangrenous small bowel secondary to right paraduodenal hernia.