Complex lymphatic malformations in pediatrics: a review of treatment options.

INTRODUCTION: Lymphatic malformations (LMs) are low-flow lesions resulting from abnormalities in the development of lymphatics. The management of these lesions is complex and involve the collaboration of many specialties. The purpose of this review is to summarize current knowledge regarding the different therapeutic options used in complex lymphatic malformations, analyzing their indications, efficacy and complications. EVIDENCE ACQUISITION: A search was made using the algorithm: "(lymphatic abnormality OR lymphatic malformation OR lymphangioma OR cystic hygroma) AND (extensive OR giant OR complex) AND (therapeutics OR treatment) AND (child OR children)". Of the 120 articles found, 53 were included. EVIDENCE SYNTHESIS: Historically, surgery was the treatment of choice for this type of lesions. However, excision was often incomplete, associated with high rates of recurrence and severe complications. The use of sclerotherapy emerged as a minimal invasive option appropriate in selected cases as a single or adjuvant therapy. Inhibitors of the mammalian target of rapamycin, such as sirolimus, now play a central role in the treatment of complex malformations resistant to sclerotherapy, recurrent after surgery or more extensive malformations that affect vital structures. Other therapeutic options as sildenafil and laser ablation are also recognized as effective in selected cases. CONCLUSIONS: Looking through the literature over the last decade authors realize that surgery had gradually been replaced by less invasive options such as sirolimus with or without adjuvant sclerotherapy. In conclusion, each treatment option seems to have its own indications and characteristics, which must be considered in therapeutic decision and individualized for each patient.

Auxiliary liver transplantation for management of acute liver failure in children - Systematic review.

INTRODUCTION: Liver transplantation (LT) remains the standard of care in the treatment of acute pediatric liver failure (PALF) for the replacement of a severely damaged native liver in patients who are unlikely to recover. However, this is burdened by the consequences of long-term immunosuppression. Auxiliary partial liver orthotopic transplantation (APOLT) has emerged as a possible improved approach, by providing a graft that assures liver function until the regeneration of the native liver occurs, and then allows for possible progression to immunosuppression withdrawal. No previous systematic review has assessed APOLT for PALF. The aim of this work is to provide information on survival, postoperative complications, and withdrawal of immunosuppression after APOLT for PALF. METHODS: The study was carried out according to the recommendations of the preferred report items for systematic reviews and meta-analyzes (PRISMA). We searched several electronic databases until October 31st, 2020, using the search terms "acute liver failure", "auxiliary liver transplant" and the MESH term "liver failure, acute". All types of clinical publications that presented results on APOLT for PALF, in English or Portuguese, and restricted to humans and for children under 18 years old were included. The following exclusion criteria were applied: "follow-up time <6 months", "does not report complications" and "does not report immunosuppression regimen (double vs triple)". Demographic data, clinical characteristics at the time of surgery and postoperative results were analyzed. RESULTS: A total of 14 references (including 45 patients) were selected, including 3 case series (6-20 patients) and 11 case reports. Of the 45 subjects, 33 (73.3%) were male and 12 (26.7%) female. In most cases (n = 30; 66.7%), the cause of PALF was undetermined. All patients underwent APOLT. Their median age was 9 (range 0.6-17) years. In the postoperative period, the immunosuppression regimen was double in 34 (75.6%) and triple in 11 (24.4%) individuals. The main postoperative complications were rejection and infection. Over a follow-up period of 6 months to 14 years, 10 (22.2%) patients died. The main cause of death was sepsis (70%). Six (13.3%) patients were retransplanted. Of the survivors (n = 35), 68.6% achieved complete withdrawal from the immunosuppression regimen. CONCLUSION: Based on current published evidence, APOLT for the treatment of PALF is a safe option, with an acceptable rate of complications and mortality. It has the great advantage of providing an immunosuppression-free life in the majority (68.6%) of survivors.

Successful liver transplantation using a whole liver graft with gallbladder agenesis: First report in pediatric liver transplantation.

INTRODUCTION: Gallbladder agenesis (GA) is a rare congenital condition, occurring in approximately 40/100.000. It is likely due to an embryologic mishap in the development of the gallbladder bud and can be associated with other congenital variations in biliary anatomy. However, the liver likely suffers no functional impairment and can be safely used for transplantation. To the best of our knowledge, this is the first case report describing a pediatric liver transplantation (PLT) using a graft with GA. CASE REPORT: A 10-year-old boy with methylmalonic aciduria underwent isolated liver transplant with a deceased graft from a donor with no relevant medical or surgical history and normal laboratory tests. During the back-table liver preparation procedure, no evidence of gallbladder was found, raising the possibility of a GA, confirmed by intraoperative cholangiography. The liver transplantation procedure was uneventful despite the particularly rare combination of biliary tree anatomic distribution found in the cholangiography. At 1 year of follow-up, there were no clinical, laboratory, or imagological signs of bile leaks or anastomotic site stricture. DISCUSSION: The present report highlights the importance of the accurate knowledge of the vasculobiliary anatomic variation, particularly in extremely rare cases, such as GA, and in complex hepatobiliary procedures, such as PLT. In our opinion, grafts with GA should not be discarded for transplantation.

Restrição física da mobilidade no doente internado em neurocirurgia: Que intervenção? / Physical restriction of mobility in the neurosurgery inpatient: Which intervention?

No quotidiano do seu trabalho o enfermeiro é permanentemente confrontado com a necessidade de tomar decisões que afetam de forma direta e/ou indireta o doente/família. Escolher de entre várias hipóteses aquela que no momento, segundo o julgamento clínico parece ser a mais adequada para responder ao problema identificado para atingir os objetivos, pode por vezes constituir um dilema para o enfermeiro, principalmente quando o tema em causa é a aplicação de medidas de restrição física da mobilidade (RFMob). Com esta dissertação pretendemos, elaborar um guia orientador da tomada de decisão do enfermeiro na aplicação de medidas de RFMob, em doentes internados num serviço de neurocirurgia, de um Hospital Central. Trata-se de um estudo descritivo, na vertente estudo de caso, inserido no paradigma qualitativo. Os dados foram colhidos através da aplicação de um questionário a enfermeiros de um serviço de neurocirurgia e da observação direta dos doentes, com alteração do estado de consciência, sujeitos a medidas de RFMob, no período de 11 de setembro a 31 novembro de 2014. Os resultados obtidos mostram que as principais razões que levam os enfermeiros em neurocirurgia a recorrer a medidas de RFMob, estão relacionadas com a segurança do doente e dos envolventes, com as respostas que o doente apresenta, nomeadamente o risco de queda, confusão, agitação e a remoção de dispositivos médicos. Apesar dos enfermeiros conhecerem os riscos da aplicação deste tipo de medidas, os resultados parecem indiciar que os profissionais percecionam os benefícios decorrentes da aplicação destas medidas como superiores aos riscos. Os resultados mostram ainda que os enfermeiros recorrem a medidas alternativas, como a orientação para a realidade, contenção farmacológica, técnicas de comunicação e gestão do ambiente físico antes de aplicar medidas de RFMob, assim como, iniciam a aplicação de medidas pelas menos restritivas, de acordo com a orientação da Direcção Geral da Saúde (DGS).

Amyand's Hernia in a 6-Week-Old Infant: A Delayed Diagnosis.

In Amyand's hernia, the hernia sac contains the appendix. This can be normal and accidentally found during herniotomy or inflamed and present as acute scrotum, although the latter is less frequent. We present a case of a male infant with scrotal abscess resulting from Amyand's hernia, with appendicitis and periappendicular abscess.