Repair of Tracheoesophageal Fistula via Median Sternotomy Approach.

Tracheoesophageal fistula (TEF) and esophageal atresia (EA) are rare congenital anomalies occurring in approximately 1 in 2500 to 3500 neonates. We present a neonatal patient diagnosed with EA/TEF in conjunction with pulmonary agenesis requiring definitive repair via median sternotomy. The child was born at 33 weeks gestational age with post-delivery respiratory distress necessitating intubation. A nasogastric tube was unable to be passed. After subsequent imaging, TEF and pulmonary agenesis were diagnosed. During planned staged repair with ligation of TEF via standard right thoracotomy approach, significant ventilatory compromise was encountered. Due to concern for ventilatory compromise and anatomical variance limiting visualization, a median sternotomy approach was utilized for definitive repair. This exposure and repair were successful and may be considered for cases with complex pulmonary malformation limiting standard thoracotomy. To our knowledge, this is only the second reported case of a successful TEF/EA repair using a median sternotomy approach.

Outcomes by disease onset, sex, and intervention in neonates with SIP and surgical NEC.

BACKGROUND: Outcomes of infants following surgical necrotizing enterocolitis (NEC) and spontaneous intestinal perforation (SIP) categorized by the age of onset, interventions, and sex are not well defined. METHODS: Retrospective comparison of infants categorized by age of onset (NEC at <10, 10-20, and >20 days) and SIP at <7 versus ≥7 days), sex, and intervention [Penrose Drain (PD) vs. laparotomy]. RESULTS: A total of 114 infants had NEC and 37 had SIP. On multinomial logistic regression, infants with NEC/SIP onset >20 days had significantly lower odds of small bowel involvement (aOR = 0.07, 95% CI: 0.01-0.33, p = 0.001), higher necrosis (aOR = 3.59, 95% CI: 1.34-9.65, p = 0.012) and higher CRP (p = 0.004) than onset <10 days. Initial laparotomy was associated with more bowel loss (24.1 cm [12.3; 40.6] vs.12.1 [8.00; 23.2]; p = 0.001), small and large intestine involvement (47.1% vs 17.2%; p = 0.01), and ileocecal valve resection (42% vs. 19.4%; p = 0.036) than initial PD therapy. Females underwent fewer small bowel resections (52.3% vs 73.6%; p = 0.025) but had higher surgical morbidity (53.7% vs. 24.7%.; p = 0.001) than males. CONCLUSION: Clinical, radiological, and histopathological presentation and outcomes in preterm infants with surgical NEC/SIP are associated with age of disease onset, sex, and initial intervention. IMPACT: Neonates with surgical NEC onset >20 days had more severe necrosis, inflammation, kidney injury, and bowel loss than those with <10 days. Initial laparotomy was associated with later age onset, more bowel loss, and ileocecal valve resection compared to initial PD treatment, but not with differences in mortality or length of stay. Female sex was associated with lower maturity, more placental malperfusion, less often small bowel involvement, lower pre-NEC hematocrit as well as higher surgical morbidity than males. Whether the management of surgical NEC and SIP should differ by the age of onset requires further investigation.

Postoperative Outcomes, and Growth and Brain Injury Outcomes in Spontaneous Intestinal Perforation vs Surgical Necrotizing Enterocolitis in Preterm Infants.

OBJECTIVE: To compare the clinical outcomes in preterm infants following surgical necrotizing enterocolitis (sNEC) and spontaneous intestinal perforation (SIP). METHODS: Retro-spective comparison of clinical information in preterm infants with sNEC and SIP admitted between January, 2013 and December 31, 2018. The clinical outcomes were compared in two groups, including postoperative and brain injury detected on brain magnetic resonance imaging (MRI) after clinical and histopathological confirmation of the SIP and the NEC diagnosis. RESULTS: 114 infants had sNEC, and 37 had SIP. Infants with SIP had lower median gestational age [25.1 weeks (23.5, 27.1) vs 26.6 (24.4, 31.0), P=0.03], an earlier mean (SD) age of disease onset [10.1 (11.3) days vs 19.6 (17.9); P<0.001] and lower maternal chorioamnionitis on placental pathology [4 (23.5%) vs 22 (68.8%); P=0.007), received more often Penrose drain therapy (54% vs 33%; P=0.03), had less median (IQR) bowel length loss [3.3 cm (1.72, 4.38) vs 21.4 (9.55, 35.3); P=<0.001] and had more often intact ileocecal valve (91.4% vs 65.7%; P=0.006] compared to those with sNEC. In addition, those with sNEC had lower median (IQR) weight z scores at the time of discharge [-1.88 (-2.80, -1.09) vs -1.14 (-2.22, -0.44); P=0.036] than SIP. There were no significant differences in postoperative ileus, duration of parenteral nutrition, surgical morbidity, length of stay, mortality, white matter, and grey matter injury on brain MRI at term equivalent age in preterm infants with SIP and sNEC. CONCLUSION: In our cohort, preterm infants with SIP and sNEC did not show significant differences in postoperative morbidity and brain MRI abnormalities at term equivalent age. sNEC had lower discharge weight z scores. Larger prospective studies are needed for confirmation of these findings.

Clinical and Growth Correlates of Retinopathy of Prematurity in Preterm infants with Surgical Necrotizing Enterocolitis and intestinal Perforation.

Background: we sought to determine the clinical and growth parameters associated with retinopathy of prematurity (ROP) in infants with necrotizing enterocolitis (NEC) and spontaneous ileal perforation (SIP). Methods: Retrospective cohort study comparing clinical information before and following NEC/SIP onset in neonates with and without severe ROP (Type 1 and 2). Results: Those with severe ROP (32/109, 39.5%) had lower GA, BW, chorioamnionitis, later median onset of ROP diagnosis and received Penrose drain and had higher AKI, poor weight z scores, poor linear growth, longer duration of ventilation and higher FIo2 than those without ROP following NEC/SIP. The GA and diagnosis at later age remained significant for any ROP on multi regression modelling. Conclusion: The surgical NEC/SIP infants with severe ROP were more likely to be younger, smaller, had AKI, had higher oxygen exposure and poor weight gain and linear growth than those without severe ROP.

Population Density and Triage of Pediatric Firearm Injuries in a Rural Trauma System.

BACKGROUND: Rural pediatric firearm injuries require regional pediatric and trauma expertise. We evaluated county-level population density associations with transport, hospital interventions, and patient outcomes at a Level I pediatric trauma center serving a rural, statewide catchment area. MATERIAL AND METHODS: The trauma registry of the only in-state pediatric trauma center was reviewed for firearm injuries in patients < 18 between 1/2013 and 3/2020. County-level population density was classified according to the United States Office of Management and Budget definitions for rural, micropolitan, and metropolitan areas. RESULTS: 364 patients were identified, including 7 patients who were re-injured. Mean age was 11.3 ± 4.5 y and patients were 79.4% male. 59.3% were transferred from a referring hospital. Median injury severity score was 5 (IQR 1-10); 88.0% required trauma center admission, and 48.2% required operative intervention. 7.4% were injured in a rural county, 46.4% in a micropolitan county, and 46.2% in a metropolitan county. Patients from rural counties were more likely to be unintentionally injured (72.0%) than those from micropolitan (54.4%) or metropolitan counties (44.0%, P = .04). While need for inpatient admission and length of stay were similar, those transported from rural counties had significantly longer transport times (P < .01) and less frequent need for operative intervention (P = .03), as well as trends toward lower injury severity (P = .08) and mortality (P = .06). CONCLUSION: Management of pediatric firearm injury is a unique challenge with significant regional variability. Opportunities exist for outreach, telehealth, and decision support to ensure equitable distribution of resources in rural trauma systems. LEVEL OF EVIDENCE: Epidemiological, Level III.

Traumatic Spigelian Hernia in a Pediatric Patient Following a Bicycle Injury.

Blunt abdominal trauma due to bicycle accident is a common occurrence in the pediatric population; however, traumatic abdominal wall hernia as the result of blunt trauma is a rare presentation. Abdominal wall injuries can be isolated or associated with multiple intra-abdominal pathologies. A high index of suspicion is essential for prompt intervention, especially when there is concurrent intra-abdominal pathology. We present a case of a traumatic spigelian hernia in a pediatric patient following a handlebar injury with the intraoperative discovery of a bucket-handle type mesenteric injury to the jejunum.

Burkitt Lymphoma Presenting as Perforated Appendicitis.

Appendicitis is the most common indication for an emergent abdominal operation in the pediatric population. Fewer than .015% of patients who undergo appendectomy for presumed appendicitis are diagnosed with primary lymphoma after evaluation of pathology specimen. 1 Of these primary lymphomas, 29.5% are Burkitt lymphoma. Burkitt lymphoma is an aggressive B-cell lymphoma characterized by translocation and dysregulation of the c-Myc gene. Intraabdominal extranodal Burkitt lymphoma has a polymorphic presentation that includes bowel obstruction, intussusception, and appendicitis. Here we report a case of an adolescent patient who was initially admitted for medical management of perforated appendicitis but was diagnosed with Burkitt lymphoma during his hospital course.

Surgical Control of Bleeding From Ovarian Torsion in the Setting of Immune Thrombocytopenic Purpura Without Splenectomy.

Immune thrombocytopenic purpura (ITP) is a disorder caused by autoimmune antibodies which target glycoprotein IIb/IIIa complex or other platelet membrane antigens leading to platelet destruction. These platelets are then cleared by the spleen resulting in thrombocytopenia. Immune thrombocytopenic purpura affects about 1 to 6.4 cases in 100 000 children making it one of the most common causes of symptomatic thrombocytopenia in the pediatric population. It is rare that children or adolescents present with serious bleeding due to ITP. Common presentations include petechiae, bleeding gums, or bruising. Bleeding requiring hospitalization or transfusions is unusual and only occurs in approximately 5% of children. Even more uncommon is the presentation of severe bleeding complications requiring surgery for resolution. We present a case of a 17-year-old girl with acute ITP complicated by intraperitoneal hemorrhage and refractory thrombocytopenia due to ovarian cyst requiring oophorectomy.

The Ideal Candidate for Subcutaneous Endoscopically Assisted Ligation (SEAL) of the Internal Ring for Pediatric Inguinal Hernia Repair.

Subcutaneous endoscopically assisted ligation (SEAL) technique is an effective and minimally invasive approach for indirect inguinal hernia repair in children. Not all patients are candidates for SEAL because of technical limitations. We hypothesized that preoperatively assessed patient-level factors may predict technical feasibility of SEAL repair. We performed a retrospective review of all patients who underwent indirect inguinal hernia repair between June 2012 and December 2014. All patients younger than two years and any patient older than two years who had a concomitant umbilical hernia were considered candidates for diagnostic laparoscopy with SEAL repair. We compared patients who had SEAL repair with those who had diagnostic laparoscopy with conversion to open repair. Univariate statistics was performed using the chi-squared and Student's t test. One hundred forty-one patients underwent diagnostic laparoscopy with intent to perform a SEAL repair. Seventeen patients were lost to follow-up. Of the remaining 124 patients, 66 had SEAL repairs, 35 had open repairs, and 23 had a SEAL repair with contralateral open repair. Patient age, BMI, gender, history of prematurity, and history of incarcerated hernia were similar between the SEAL and open groups. Sixty-two per cent of hernias were able to be repaired with SEAL technique. Hernia recurrence was seen in 3 of 123 total SEAL repairs and in 1 of 74 open repairs. The recurrence rate for SEAL repairs (2.4%) was not significantly different from the recurrence rate for open repairs (1.4%). No preoperative patient-level factors predicted technical inability to perform a successful SEAL repair. In this series, the recurrence risk of SEAL compared with open repair was low and not statistically significant. For practitioners with minimally invasive experience, SEAL should be considered a safe and successful option for inguinal hernia repair in pediatric patients undergoing routine diagnostic laparoscopy.

Wound complications after chemo-port placement in children: Does closure technique matter?

PURPOSE: Wound dehiscence after chemo-port placement is a rare but potentially significant complication. We hypothesize that by using a simple running skin closure technique during chemo-port placement the rate of wound dehiscence and overall wound complications can be significantly decreased. METHODS: IRB approval was obtained and patients <18years that received a tunneled central line with port from June 2012 to April 2016 were analyzed. Data collected on patients included patient demographics, skin closure type, and wound complications within 30days. Chi-square was performed to examine the univariate association with skin closure technique and wound dehiscence. Logistic regression was performed to examine the multivariable association between skin closure type and wound dehiscence and to compute odds ratios. RESULTS: There were 259 ports placed in this cohort: 125 used simple running skin closure technique, and 134 used the subcuticular skin closure. Patients were found to not have any difference in rate of dehiscence or overall wound complications based on gender, age, location of port, or use of steroids or chemotherapy within 1week of port placement. When compared, only 1 case (0.80%) in the simple running group vs 10 cases (7.46%) in the subcuticular group experienced a wound dehiscence [unadjusted OR=14.07 (1.69, 116.99) p=0.0144]. When comparing overall wound complications the simple running group had 3 (2.4%) versus 12 (8.96%) in the subcuticular group [unadjusted OR=4.78 (1.27, 17.94) p=0.0203]. When adjusting for port-number both dehiscence and overall wound complications remained statistically significant. CONCLUSION: We conclude that the simple running skin closure for chemo-port placement in children has superior outcomes in regards to prevention of dehiscence and overall wound related complications when compared to the subcuticular technique.

Stapled intestinal anastomoses with endoscopic staplers in premature infants.

PURPOSE: The safety and effectiveness of a stapled intestinal anastomosis in adults, children, and infants is well documented. However, in neonates it is not well validated. We hypothesized that premature infants who received a stapled bowel anastomosis utilizing endoscopic staplers had similar outcomes compared to patients with a handsewn anastomosis. METHODS: A retrospective study was performed reviewing premature infants who underwent an intestinal anastomosis over a 4-year period. Patients greater than 36weeks gestational age at birth or a weight greater than 5kg at surgery were excluded. Patient demographics, type of intestinal anastomosis, and anastomotic related complications within 3months were collected and analyzed. RESULTS: Sixty-five patients underwent 71 operations involving an intestinal anastomosis: 33 cases were handsewn, and 38 cases were stapled. Groups were noted to have differences in age, weight, and diagnosis. Complications including leak and anastomotic stricture did not differ between groups. Reports of blood per rectum after surgery were more common in the stapled group (24% versus 6%, p=0.0522), but this did not reach statistical significance. CONCLUSION: There were no significant differences in anastomotic complications when comparing the handsewn and stapled intestinal anastomosis techniques in premature infants weighting less than 5kg. TYPE OF STUDY: Treatment Study. LEVEL OF EVIDENCE: III.

Neonate With Imperforate Anus and Meconium Streak Along the Penile Shaft.

We present a neonatal male with a rectopenile fistula in the setting of imperforate anus. Reported cases of fistulae exiting on the penile shaft are exceedingly rare. The patient had a diverting colostomy performed within the first 48 hours of life and imaging later revealed no communication with the urinary tract. At 4 months of age the child was brought to the operative suite for penile exploration and posterior sagittal anorectoplasty. The patient's colostomy was reversed 4 months later. We discuss the embryology and management of imperforate anus as well as literature review.

Epiploic Appendagitis: A Rare Cause of Acute Abdominal Pain in Children. Report of a Case and Review of the Pediatric Literature.

A 9-year-old boy presented with acute onset of abdominal pain and vomiting. History, physical examination, and initial laboratory testing failed to provide a diagnosis. A computed tomography scan revealed the rare finding of epiploic appendagitis. We review the literature of this rare, but increasingly recognized, condition that mimics appendicitis and needs to be considered in the child with acute abdominal pain.

Gastric metastasis from an alveolar soft part sarcoma in a child: case report and review of the literature.

The authors report a child with alveolar soft part sarcoma who developed significant anemia due to gastrointestinal blood loss. Evaluation revealed the source of bleeding as a gastric metastasis, which was successfully removed. A brief review of gastrointestinal involvement by alveolar soft part sarcoma is discussed.

Resection of a giant hepatic adenoma in an eight-year-old girl.

Hepatic adenomas are benign tumors typically diagnosed in women of reproductive age, however, these tumors occur in the pediatric population although rare. We present the case of a giant hepatic adenoma in an 8-year-old female without established risk factors with progressive abdominal pain, and po intolerance, biliary obstruction, anemia, and fever. Right trisegmentectomy afforded extirpation of the 16 × 14.5 × 8.5 cm mass. The postoperative course was complicated by transient encephalopathy and a bile leak which resolved with appropriate care. The patient is well over 1 year from resection. She demonstrates age appropriate mental and physical function without disease recurrence or complication.