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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]

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]

Ho DT, Rodig NM, Kim HB, Lidov HGW, Shapiro FD, Raju GP, Kang PB. Rapid reversal of uremic neuropathy following renal transplantation in an adolescent. Pediatr Transplantation 2011. © 2011 John Wiley & Sons A/S. Abstract: Clinical and pathologic studies on adults with uremic neuropathy are numerous, but less is known about this disorder in children and adolescents. We report the clinical, electrophysiologic, and pathologic findings in an adolescent female with uremic neuropathy. Electrophysiologic findings were consistent with a primarily axonal sensorimotor polyneuropathy. Sural nerve biopsy revealed areas of focal depletion in myelin sheaths and loss of axons. Axonal degeneration with secondary myelin changes appears to be the characteristic pathology in this case, one of the youngest to our knowledge for which nerve biopsy data are available. Our patient experienced dramatic recovery after renal transplantation, similar to the reports of older patients. [ABSTRACT FROM AUTHOR]

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]

We present a case of a three-yr-old child with a history of multisystem Langerhans cell histiocytosis treated with systemic chemotherapy, who developed progressive liver failure and received an orthotopic split liver transplant while continuing on chemotherapy. One month following transplant, he developed acute graft-vs.-host disease of the skin and gastrointestinal tract. Peripheral blood chimerism studies post-transplant demonstrated an increasing predominance of donor lymphocytes and granulocytes. Shortly after, the patient developed vitiligo, and two yr after transplantation, the patient developed skin manifestations of psoriasis. We discuss and review the current literature, which demonstrates that chimerism following liver transplantation is rare and in our patient may be related to his profound immunosuppression around the time of liver transplant as well the development of acute graft-versus-host disease. While autoimmune disease can occur after solid organ and stem cell transplant, our patient developed skin manifestations of autoimmunity after liver transplantation, which is also rarely described. [ABSTRACT FROM AUTHOR]

McGuire MM, Jones BA, Hull MA, Misra MV, Smithers CJ, Feins NR, Jenkins RL, Lillehei CW, Harmon WE, Jonas MM, Kim HB. Combined en bloc liver-double kidney transplantation in an infant with IVC thrombosis. Pediatr Transplantation 2011: 15: E142-E144. © 2010 John Wiley & Sons A/S. Abstract: We report a case of a pediatric en bloc liver-double kidney transplant in a patient with IVC thrombosis below the renal veins. The patient is an 11-month-old girl diagnosed with congenital nephrotic syndrome at two months of age. Multifocal liver masses were identified on routine ultrasound at eight months of age. Alpha fetoprotein level was 55 319. Biopsy confirmed hepatoblastoma. CT scan confirmed multiple lesions in both lobes, which would require liver transplantation for resection. She was also found to have thrombosis of her infrarenal IVC secondary to multiple central lines. She was listed for combined liver-kidney transplant and began chemotherapy. After four cycles of chemotherapy, she underwent bilateral nephrectomies followed by a combined en bloc liver-double kidney transplant from a size matched donor. In order to provide adequate venous outflow from the kidneys in the absence of a recipient infrarenal IVC, the donor liver and kidneys were procured en bloc with a common arterial inflow via the infrarenal aorta and common outflow via the suprahepatic IVC. Kidney transplantation in the absence of adequate recipient venous drainage may require unusual vascular reconstruction techniques. This case demonstrates a novel approach in patients who may require combined liver-kidney transplantation. [ABSTRACT FROM AUTHOR]