Esplenectomía en enfermedades hemolíticas. Influencia de la enfermedad de Gilbert en la aparición de complicaciones biliares / No disponible

Introducción: En pacientes con enfermedades hemolíticas (EH) se recomienda esplenectomía entre 6-12 años. En aquellos con enfermedad de Gilbert (EG) asociada se ha descrito mayor riesgo de complicaciones biliares (CB), sin establecerse edad quirúrgica óptima. Nuestro objetivo es cuantificar el riesgo de CB en pacientes con EH y EG para valorar el beneficio de esplenectomía temprana. Material y métodos: Estudio retrospectivo de las esplenectomías realizadas en pacientes con EH entre 2000-2017. Se analizó la incidencia de CB, su repercusión clínica (ingreso o tratamiento invasivo) y momento de aparición. Se consideraron dos grupos: pacientes con EG y sin EG. Se obtuvieron curvas de supervivencia y se compararon mediante log-rank test. Resultados: Se realizaron 44 esplenectomías, 15 de ellas (34,1%) en pacientes con EH+EG. La edad mediana en la cirugía fue 10,3 años (rango 5,4-14,8). Veintinueve (65,9%) presentaron CB. El 50% de los pacientes con EG las presentaron antes de los 8 años vs.10,5 años en los casos sin EG (log-rank 3,9; p= 0,05). Los pacientes con EG presentaron más CB (86,7% vs. 55,2%; c2= 4,37, p= 0,037). En el grupo EH+EG, 8 casos (53%) necesitaron ingreso vs. 8 (31%) en el grupo sin EG (c2= 2, p= 0,1). El tratamiento invasivo fue necesario en 2 pacientes (13%) del grupo EH+EG y 2 pacientes (7,6%) del grupo sin EG (c2= 0,3, p= 0,6). Conclusiones: En nuestra serie, la incidencia de CB fue superior en los pacientes con EG. Existió una tendencia a la presentación más temprana de CB en este grupo, pero ni este dato ni su repercusión clínica nos permiten recomendar la esplenectomía temprana

Introduction: In patients with hemolytic disorders (HD) splenectomy is recommended between 6-12 years. A higher risk of biliary complications (BC) has been described in those with associated Gilbert’s disease (GD), but the ideal surgical age has not been stablished yet. Our aim is to quantify the risk of BC in patients with HD and GD to assess the benefit of early splenectomy. Material and methods: Retrospective study of splenectomies performed in patients with HD between 2000-2017. The incidence of BC, its clinical consequences (admission or invasive treatment) and time of onset were analyzed. Two groups were considered: patients with GD and without GD. Survival curves were obtained and compared with log-rank test. Results: Fourty-four patients underwent splenectomy, 15 of them (34.1%) with HD+GD. The median age at surgery was 10.3 years (range 5.4-14.8). Twenty-nine (65.9%) had BC. Half of the patients with GD had BC before 8 years vs. 10,5 years in the cases without GD (log-rank 3.9, p= 0.05). Patients with GD had more BC (86.7% vs. 55.2%; c2= 4.37, p= 0.037). In the HD+GD group, 8 cases (53%) required admission vs.8 patients (31%) in the group HD without GD (c2= 2, p= 0.1). Invasive treatment was performed in 2 patients (13%) in the HD+GD group and 2 others (7.6%) in the group HD without GD (c2= 0.3, p= 0.6). Conclusions: In our series, the BC incidence was higher in patients with HD and GD. There was a trend towards an earlier presentation of BC in this group, but neither this data nor its clinical consequences allow us to recommend early splenectomy

Perioperative Anesthetic Management of Patients Having Liver Transplantation for Uncommon Conditions.

This review focuses on the perioperative anesthetic management of patients having liver transplantation (LT) performed for several uncommon indications or in combination with rare pathology. Conditions discussed in the article include Alagille syndrome, hypertrophic cardiomyopathy, Gilbert's syndrome, porphyria, Wilson's disease, and Budd-Chiari syndrome. In comparison to other indications, LT in these settings is infrequent because of the low incidence of these pathologies. Most of these conditions (with the exception of Gilbert syndrome) are associated with a high probability of significant perioperative complications and increased mortality and morbidity. Experience in management of these unusual conditions is only gained over time. Developing clinical pathways for patients with these conditions should result in outcomes similar to LT performed for more common indications.

Liver transplantation from living donors with Gilbert's syndrome is a safe procedure for both donors and recipients.

Liver transplantation (LT) has become a favorable therapeutic option for patients with end-stage liver diseases. Gilbert's syndrome (GS) is a benign condition characterized by intermittent mild jaundice due to unconjugated hyperbilirubinemia. It is not obvious whether living-donor liver transplantation (LDLT) from a donor with GS could result in a normal outcome for both the recipient and the donor. We aimed to determine whether right lobe hepatectomy is a safe procedure for living donors with GS and LT recipients. Between September 2011 and March 2015, 305 LDLT procedures using right lobe grafts were performed at Atasehir Memorial Hospital, Istanbul, Turkey. Nineteen of 305 LT candidates who had been diagnosed with GS were included in the current study. After a 12-h overnight fast, total and indirect bilirubin levels of donors and recipients were measured. The median follow-up after transplant was 16 months (range 3-36 months). The median age of donors was 25 (range 20-55 yr). Four donors (21%) were female, and 15 donors (89%) were male. The median age of donors was 51 (range 23-68 yr). Eleven recipients (57%) were female, and 8 (43%) were male. The median preoperative total bilirubin level of donors was 1.69 mg/dL (range 1.26-2.43 mg/dL) (normal range <1.2 mg/dL). The median total bilirubin level of donors on postoperative day 7 was 1.04 mg/dL (range 0.71-3.23 mg/dL). As our study has included a large number of donors with GS, it produced reliable evidence that right lobe hepatectomy is a safe procedure for living donors with GS and LT recipients.

Interplay of co-inherited diseases can turn benign syndromes in a deadly combination: haemoglobinopathy and bilirubin transport disorder.

We present a case about a 25-year-old male patient suffering from a rare genetic disorder called Mizuho haemoglobin. He was admitted to the Intensive Care Unit with acute liver and renal failure. During admission he also developed a cardiac tamponade twice. Finally he received a liver transplantation. Hereafter the patient stabilised and his liver and renal functions improved. His symptoms could not be explained solely by his known disease. After searching the literature, similarities between his symptoms and a rare complication of sickle cell disease were found. Molecular diagnostics showed that the patient also suffered from Gilbert's syndrome. Due to his chronic haemolysis, symptoms of this other disease were masked. This stresses the importance of always looking for other causes if symptoms or changes cannot be explained by a known rare disorder.

Usefulness of the prognostic score for donor safety in living donor liver transplantation.

This study sought to determine whether a prognostic score is a useful indicator of donor safety using 13 consecutive donors enrolled for liver transplantation. The donor operations were right hepatic lobectomies (n = 10) and left hepatic lobectomies (n = 3). The postoperative maximal level of serum total bilirubin was used to assess the magnitude of surgical stress. Variables such as donor age, percentage of liver resection (PLR), indocyanine green 15-minute retention rate (ICGR15), operative blood loss, operation time, prognostic score and graft weight were evaluated as predictors of the magnitude of surgical stress. The PLR and prognostic score (PS) were calculated according to the following formulae: PLR (%) = 100*Graft weight (g)/standard liver volume of the donor (mL); PS = -84.6 + 0.933*PLR (%) +1.11*ICGR15 (%) +0.999*age (years); Standard liver volume (mL) = 706.2*body surface area (m2) + 2.39. No serious complications occurred after the donor operations. Maximal bilirubin ranged from 1.9 to 10.9 mg/dL. There were no mortalities, although there were two morbidities, bile leakage and prolonged liver dysfunction. Postoperative hyperbilirubinemia was observed in two donors and in one Gilbert's syndrome donor. Linear regression analysis of each variable indicated poor correlations between those variables and maximal bilirubin. However, close correlations were seen between maximal bilirubin and both donor age and PS except for the three patients who showed postoperative hyperbilirubinemia. In these uneventful donors, statistical formulae were obtained as follows: maximal bilirubin (PMB) = 0.271 + 0.056*donor age (correlation coefficient 0.612, P < .008), PMB = 1.541 + 0.059*PS (correlation coefficient 0.597, P < .009). In conclusion, PS is useful to predict maximal bilirubin and to ensure donor safety.