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Background: In order to improve transparency within the patient selection process, a transplant listing advisory committee was formed within the Boston Children's Hospital Pediatric Transplant Center. Its mission is to promote equity in access to organ transplantation by ensuring that the institutional transplant selection criteria are fair, unbiased, and nondiscriminatory. The committee conducts comprehensive case and data review of individual characteristics and reviews in aggregate to identify potential systems bias. Methods: Charts for 256 patients evaluated for transplant from 3/2016 to 3/2019 were reviewed. Among these, 64 (25%) patients were declined for transplant. Univariate logistic regression analysis was used to identify demographic variables and vulnerable status factors associated with being declined. Odds ratios (OR) are reported. Results: Among all patients, median age was 8.5 years and 58% were male. Asian patients were more likely to be declined than White patients (OR = 5.3, Wald p =.007). Socioeconomic factors that affected likelihood of listing decline included concerns for caregivers' ability to manage and understand care requirements (OR = 3.8, p =.011), caregiver employment status (OR = 1.9, p =.042), and use of public assistance programs (OR = 2.2, p =.05). Patients with severe neurodevelopmental delay were more likely to be declined for listing (OR = 3.7, p =.019). Conclusion: This analysis identified areas of potential bias related to race, socioeconomic status, and neurodevelopmental delay where initiatives can be targeted. Advisory committees are an important aspect of evaluating equity in transplant center selection policy and practice. [ABSTRACT FROM AUTHOR]

Background Midaortic syndrome is often associated with refractory hypertension. The aim of our study was to better understand the short- and medium-term outcomes in this patient population utilizing a multidisciplinary management approach. Methods We conducted a review of patients with midaortic syndrome treated at our institution over the past 30 years. Results Fifty-three patients presented at a median age of 6.7 years (birth to 28.7 years). Thirty-five patients (66 %) underwent invasive management (percutaneous techniques: 21; surgical techniques: 5; both: 9). Percutaneous interventions were acutely successful in decreasing the gradient across the obstruction and degree of luminal stenosis. However, freedom from reintervention was 58 % at 1 year and 33 % at 5 years. Freedom from reintervention after a surgical procedure was longer: 83 % at 1 year and 72 % at 10 years. At the most recent follow-up, the majority of patients (69 %) were normotensive. The median duration between time of presentation and achievement of blood pressure control was 5.7 (0.4-21.1) years. The median number of anti-hypertensive medications was 1 (0-5). Conclusions A multidisciplinary management strategy which couples comprehensive medical management with catheter-based and surgical interventions can lead to adequate blood pressure control and preservation of end-organ function in patients with midaortic syndrome. Keywords Midaortic syndrome * Hypertension * Renal artery stenosis * Atypical coarctation of the aorta * Abdominal coarctation * middle aortic syndrome, Introduction Narrowing of the abdominal aorta was first described by Quain in 1848 [1], but the term middle-aortic syndrome was originally used by Sen et. al. [2] in a report [...]

Introduction: Fetal dermal wound healing is characterized by a minimal inflammatory response, restoration of normal dermal architecture, and scarless repair. We previously showed that the production of proinflammatory cytokines interleukin-6 (IL-6) and interleukin-8 (IL-8) are diminished in fetal wound repair. IL-10 is an anti-inflammatory cytokine that deactivates macrophages, decreasing production of IL-6 and IL-8. We hypothesized that the diminished IL-6 and IL-8 levels, and minimal inflammation seen in scarless fetal dermal wound repair may be due to increased production of IL-10. To test this hypothesis, we developed a new model of fetal wound repair in normal and IL-10 "knockout" mice. Methods: Fifteen day gestation fetal mouse skin was obtained from either normal C57BL/6 mice or transgenic C57BL/6 mice in which the IL-10 gene has been "knocked out". The skin was grafted to the back of the same strain adult mice. After 1 week the grafts were incisionally wounded and marked with India ink. Grafts were harvested at 1 week and histochemically analyzed for scar using Masson's Trichrome stain. Results: At one week, the normal C57BL/6 fetal skin wounds demonstrated minimal inflammation and the normal dermal reticular collagen pattern at the site of the wound, consistent with scarless repair. In contrast, the wounds in the C57BL/6 IL-10 knockout fetal skin healed with significant inflammation and abnormal collagen deposition at the site of the wound., consistent with scar formation. Conclusion: In a new murine model of fetal wound healing, fetal skin on adult syngeneic mice heals without scar, suggesting scarless repair is intrinsic to fetal skin. The absence of IL-10 in fetal skin results in scar formation. Intrinsic lack of IL-10 in dermal tissue may permit continued amplification of the proinflammatory cytokine cascade, resulting in continued stimulation of fibroblasts in the wound, and abnormal collagen deposition. IL-10 is necessary for scarless wound repair to occur in normal fetal skin., Kenneth W. Liechty, MD, Heung B. Kim, MD, N. Scott Adzick, MD, FAAP, Timothy M. Crombleholme, MD, FAAP.; The Children's Institute for Surgical Science at The Children's Hospital of Philadelphia, [...]

Routine use of transanastomotic biliary stents (RTBS) for biliary reconstruction in liver transplantation ( LT) is controversial, with conflicting outcomes in adult randomized trials. Pediatric literature contains limited data. This study is a retrospective review of 99 patients who underwent first LT (2005-2014). In 2011, RTBS was discontinued at our center. This study describes biliary complications following LT with and without RTBS. 56 (56%) patients had RTBS. Median age at LT was 1.9 yr ( IQR 0.7, 8.6); 55% were female. Most common indication for LT was biliary atresia (36%). Most common biliary reconstruction was Roux-en-Y choledochojejunostomy (75% with RTBS, 58% without RTBS, p = 0.09). Biliary complications (strictures, bile leaks, surgical revision) occurred in 23% without significant difference between groups (20% with RTBS, 28% without RTBS, p = 0.33). Patients with RTBS had routine cholangiography via the tube at 6-8 wk; thus, significantly more patients with RTBS had cholangiograms (91% vs. 19%, p < 0.0001). There was no difference in the number of patients who required therapeutic intervention via endoscopic or percutaneous transhepatic cholangiography (11% with RTBS, 19% no RTBS, p = 0.26). Routine use of RTBS for biliary reconstruction in pediatric LT may not be necessary, and possibly associated with need for costlier, invasive imaging without improvement in outcomes. [ABSTRACT FROM AUTHOR]

Abstract: Background: Since its introduction as an alternative intestinal lengthening technique, serial transverse enteroplasty (STEP) has been increasingly used as the surgical treatment of choice for patients with refractory short bowel syndrome (SBS). While primary STEP for the treatment of congenital conditions was proposed in the original description of the procedure, emphasis was placed on a delayed or staged approach to these patients. To date, a comprehensive review of the outcomes from this sub-population has not been reported by the International STEP Data Registry. Methods: A retrospective review of the International STEP Data Registry was performed to identify all patients who underwent STEP as a primary operative procedure for the treatment of congenital SBS. Changes in pre- and post-STEP values were assessed using paired t-tests with significance set at p<0.05. Data are presented as mean ± standard deviation. Results: Fifteen patients underwent primary STEP for congenital SBS between September 1, 2004, and April 10, 2012. Thirteen patients had follow-up information available. Causes of congenital SBS included closing gastroschisis, small bowel atresia, and midgut volvulus. Twelve patients had pre- and post-STEP bowel measurements taken. Average pre- and post-STEP bowel lengths were 32 ± 16 cm and 47 ± 22 cm, respectively. Intestinal length was increased by a mean of 15 ± 12 cm for a relative small bowel length increase of 50.4 ± 27.3% (p<0.001). Only one patient required an ostomy at the time of primary STEP. A second patient required a temporary ostomy at 3months of age that was later closed. There was one death from intestinal failure associated liver disease (IFALD). Another patient experienced IFALD progression and required liver and intestinal transplantation. The most commonly reported complication following primary STEP was obstruction or bowel re-dilatation requiring additional operative interventions. Nine patients underwent second STEP procedures under these circumstances. Eight patients remain dependent on parenteral nutrition, while three patients achieved enteral autonomy. Conclusions: Primary STEP is a feasible and safe surgical option for the treatment of congenital conditions resulting in SBS. Primary STEP establishes early bowel continuity, creates intestinal length from congenitally dilated bowel, and appears to obviate the need for interval stomas and their associated loss of bowel length in neonates with congenital SBS. However, with recent changes in SBS management emphasizing intestinal rehabilitation, additional studies are needed to assess the long-term impact on intestinal adaptation of STEP performed in the neonatal period prior to adoption of this technique. [Copyright &y& Elsevier]

Abstract: Traumatic injury has been the most common cause of morbidity and mortality in the pediatric population, but injuries to the abdominal aorta are rare. We reviewed 2 children who underwent exploratory laparotomy for blunt aortic injury in which intra-operative ultrasound guided the surgical approach. Intra-operative ultrasound allowed us to minimize resection of normal aorta and achieve primary repair. In the pediatric population, achieving primary repair is ideal, as synthetic grafts will likely need future revisions as children grow. Here we present the use of intra-operative ultrasound in maximizing the chance at primary repair of the aorta following blunt injury. [Copyright &y& Elsevier]

Background/Purpose: Aortopexy is an established treatment method for severe tracheomalacia. It can be performed by thoracotomy or thoracoscopically. The purpose of this study is to review our experience with thoracoscopic aortopexy. Methods: Thoracoscopic aortopexy was performed for treatment of tracheomalacia in five patients. A left-sided approach with three ports was used. The aorta was sutured to the sternum with a series of transversely oriented simple interrupted Prolene sutures. Knots were tied subcutaneously. Pre- and postprocedure bronchoscopy was used to verify improvement in tracheal patency. The study protocol was approved by the Institutional Review Board. A retrospective chart review was performed and indications, perioperative course, and, where available, long-term results were noted. Results: We treated four girls and one boy. Ages were between 6 weeks and 2 years. Weights were between 3.2 and 10 kg. Resolution of symptoms was achieved in all patients. There was no morbidity or mortality associated with the procedure. Length of hospital stay after aortopexy ranged between 4 and 59 days. Follow-up ranged between 34 and 5 months. Conclusions: Thoracoscopic aortopexy is an effective treatment for severe tracheomalacia not controlled by conservative measures. It can be safely performed even in small infants as long as equipment and skill for pediatric thoracoscopy are available. The authors believe that thoracoscopic exposure offers advantages over open technique and is cosmetically more pleasing to patients and their families. It may result in less pulmonary complications, shorter hospital stay, and less narcotic requirement compared to open thoracotomy. [ABSTRACT FROM AUTHOR]

Abstract: Focal nodular hyperplasia (FNH) is a benign, poorly understood hepatic tumor that is rare in children. Although there is no evidence for malignant degeneration, FNH can occur adjacent to a malignancy. Here, the case of a 4-year-old boy with a hepatic mass and history of stage IV neuroblastoma is presented. Initial imaging and core-needle biopsy were consistent with FNH. However, after left lateral segmentectomy, pathologic examination revealed a malignant tumor most consistent with small cell undifferentiated hepatoblastoma as well as 3 foci of FNH in the surrounding parenchyma. [Copyright &y& Elsevier]