Itx in children still is a high-risk procedure but has now become a viable option for children who otherwise have no hope for survival. Control of respiratory infection is of particular importance in the younger children.
Rapidly evolving literature describes case reports of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), and the myriad of clinical manifestations of this infection known as coronavirus disease 2019. 1 Few studies have clinically characterized the disease in the pediatric population 2 and none in immunosuppressed children. At least one clinical report suggests that immunosuppressed patients are not at increased risk for severe disease or mortality. 3 In this report, we describe the clinical course and treatment of a 6-month-old female who underwent living related donor liver transplant (LT) from her mother and tested positive for SARS-CoV-2 four days post-operatively. In the interest of rapidly circulating information to our community, we previously published a partial description of this case focused on histopathology. 4 For the benefit of a wider pediatric transplant audience, we hope to provide more detailed information regarding the clinical course as it relates to available treatment and immunosuppression management.
By preserving part of the native liver, auxiliary partial orthotopic liver transplantation (APOLT) provides the advantage of potential immunosuppression (ISP) withdrawal if the native liver recovers but has had limited acceptance, especially in the United States, due to technical complications and low rates of native liver regeneration. No previous study has evaluated APOLT specifically for preadolescent children with fulminant hepatic failure (FHF). This population might benefit especially based on greater capacity for liver regeneration. Data from 13 preadolescent children who underwent APOLT were compared to 13 matched controls who underwent orthotopic liver transplantation (OLT) for FHF from 1996 to 2013. There were no significant differences in patient demographics or survival between the 2 groups. However, all surviving OLT recipients (10/13) remain on ISP, while all but 1 surviving APOLT recipient (12/13) showed native liver regeneration, and the first 10 recipients (76.9%) are currently off ISP with 2 additional patients currently weaning. In our experience, APOLT produced excellent survival and high rates of native liver regeneration in preadolescent children with FHF. This represents the largest series to date to report such outcomes. Liberating these children from lifelong ISP without the downside of increased surgical morbidity makes APOLT an attractive alternative. In conclusion, we therefore propose that, with the availability of technical expertise and with the technical modifications above, APOLT for FHF should be strongly considered for preteenage children with FHF.
Abstract. Ganglioneuromas are slow growing, clinically silent benign tumors for which surgery is considered to be the standard treatment. However, surgical excision in cases where surrounding structures are involved can be challenging. The present study reports a novel technique of ex vivo excision for the management of a retroperitoneal ganglioneuroma in a 21-year old patient, that appeared to be inoperable using standard surgical resection. Preoperative investigations revealed a large tumor with encasement of the origins of the superior mesenteric artery (SMA) and bilateral renal arteries. Initially, to prevent the need to explant the liver, the distal SMA (with takeoff of the replaced common hepatic artery) was anastomosed to the splenic artery. The bulk of the tumor along with the bilateral kidneys was mobilized from the retroperitoneum, and the aorta and inferior vena cava (IVC) were cross-clamped above and below the tumor and divided. The two kidneys were dissected free of the tumor at the back-table and were auto-transplanted in a standard technique following the reconstruction of the aorta and IVC. The patient tolerated surgery well and a one-year postoperative follow-up did not show any sign of tumor recurrence. Although technically demanding, ex vivo resection and auto-transplantation of the involved organs can be introduced as a final option for the treatment of tumors that are un-resectable using standard surgical techniques. IntroductionGanglioneuromas (GNs) are rare, slow-growing benign tumors arising from the sympatho-adrenal neuroendocrine system (1,2). GNs typically occur in children and adolescents, with up to 60% of patients <20 years old at the time of diagnosis. Additionally, females are more likely to be affected than males (3). GNs are the benign ends of a wide spectrum of peripheral neuroblastic tumors, which also include neuroblastoma, ganglioneuroblastoma and ganglioneuroblastoma intermixed (2,4). GN can be diagnosed de novo in healthy patients or in specific cases result from spontaneous or chemo-or radiotherapy induced maturation of less-differentiated neuroblastic tumors (neuroblastoma and ganglioneuroblastoma) (1,5). In addition, certain studies have presented an association between GNs and specific familial diseases including neurofibromatosis type 2 and multiple endocrine neoplasia type 2 (6,7). While GNs can occur anywhere along the sympathetic nervous system, the two most common locations of occurrence are the posterior mediastinum and retroperitoneum (6). Retroperitoneal tumors may originate from the adrenal glands or extra-adrenal tissues (6). There are a limited number of studies reporting tumor occurrence in uncommon locations, including the tongue, bladder, uterus and skin (8-10).Although complete surgical excision is considered to be the treatment of choice in the management of these tumors, occasionally it can be challenging and in specific cases (such as when large tumors are involving the surrounding vital organs) it is not a feasible option. The current case report pre...
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