Successful Simultaneous Anatomic Subtotal Splenectomy During Pediatric Living-Donor Liver Transplantation: A Case Report.

Some liver transplant recipients may present with persistent hypersplenism post transplant. Persistent thrombocytopenia and leukopenia may put immunosuppressed transplant recipients at a higher risk of serious, even life-threatening bleeding and infection and limit the use of some drugs (such as mycophenolate mofetil) that could reduce white blood cell and platelet counts during the postoperative period. Herein, we report the first case, to the best of our knowledge, of successful simultaneous anatomic subtotal splenectomy during living donor liver transplantation in a 6-year-old girl with biliary atresia, cirrhotic portal hypertension, severe hypersplenism, and massive splenomegaly. The normalization of platelet and leukocyte counts observed in this patient on the third and first day of the transplant were maintained within the reference ranges during the whole follow-up period, with no significant remnant splenic regrowth and occurrence of serious procedure-related complications. Currently at 48 months of follow-up, the patient has remained in good general condition with normal liver allograft function and peripheral blood cell counts. Our case demonstrates that simultaneous anatomic subtotal splenectomy during liver transplantation may be an effective and feasible treatment for the prevention of persistent hypersplenism post transplant, achieving a long-term desired hematological response, in a selective category of pediatric cirrhotic patients with severe hypersplenism and massive splenomegaly.

A novel method for the angiographic estimation of the percentage of spleen volume embolized during partial splenic embolization.

PURPOSE: To evaluate the efficacy of estimating the volume of spleen embolized in partial splenic embolization (PSE) by measuring the diameters of the splenic artery and its branches. MATERIALS AND METHODS: A total of 43 liver cirrhosis patients (mean age, 62.19±9.65 years) with thrombocytopenia were included. Among these, 24 patients underwent a follow-up CT scan which showed a correlation between angiographic estimation and measured embolized splenic volume. Estimated splenic embolization volume was calculated by a method based on diameters of the splenic artery and its branches. The diameters of each of the splenic arteries and branches were measured via 2D angiographic images. Embolization was performed with gelatin sponges. Patients underwent follow-up with serial measurement of blood counts and liver function tests. The actual volume of embolized spleen was determined by computed tomography (CT) measuring the volumes of embolized and non-embolized spleen two months after PSE. RESULTS: PSE was performed without immediate major complications. The mean WBC count significantly increased from 3.81±1.69×10(3)/mm(3) before PSE to 8.56±3.14×10(3)/mm(3) at 1 week after PSE (P<0.001). Mean platelet count significantly increased from 62.00±22.62×10(3)/mm(3) before PSE to 95.40±46.29×10(3)/mm(3) 1 week after PSE (P<0.001). The measured embolization ratio was positively correlated with estimated embolization ratio (Spearman's rho [ρ]=0.687, P<0.001). The mean difference between the actual embolization ratio and the estimated embolization ratio was 16.16±8.96%. CONCLUSIONS: The method provides a simple method to quantitatively estimate embolized splenic volume with a correlation of measured embolization ratio to estimated embolization ratio of Spearman's ρ=0.687.

Formula to predict platelet count after partial splenic arterial embolization in patients with hypersplenism.

PURPOSE: To establish a formula to guide appropriate embolization volume for postprocedural platelet gain following partial splenic arterial embolization (PSE) for hypersplenism. MATERIALS AND METHODS: The hepatic volume (Vh) and splenic volume (Vsp) were measured by using 2-mm-thick computed tomography images before and after PSE in 20 patients with various chronic liver diseases. A formula was derived from the relationship between the platelet count increase ratio (dPlt%) and the organ volumes, which was then evaluated in another cohort. RESULTS: After an embolization of a median of 72.1% of the spleen (interquartile range, 38.2%-93.8%), the dPlt% was 67.7% ± 40.0 and significantly correlated with the increasing ratio of Vh to Vsp (P = .019, ρ = 0.52). Because the difference in Vh/Vsp ratio after PSE was significantly correlated with the spleen embolization ratio (eVsp%; P = .0003, ρ = 0.72), the estimated dPlt% could be derived from the Vh/Vsp ratio before PSE and the eVsp%. The estimated dPlt% was significantly correlated with the actual dPlt% (P = .0003, ρ = 0.72). When the formula was evaluated in another cohort of 14 cases, another strict correlation was observed (P < .0001, ρ = 0.92). CONCLUSIONS: These data suggest that platelet count after PSE can be predicted before the procedure by using the Vh/Vsp ratio and the anticipated spleen embolization volume. The use of such a prediction can prevent too much or too little embolization, thereby leading to an improvement in the risk/return trade-off in PSE.

Low-dose palliative splenic irradiation in haematolymphoid malignancy.

Patients are treated with palliative splenic irradiation (SI) to relieve pain, volume effects and the clinical consequences of hypersplenism. The case records of 19 patients treated with palliative SI at our centre, from April 2003 to November 2004, were reviewed. Twenty-two courses of SI were identified. The radiation doses delivered ranged from 150 to 800 cGy (median 450 cGy). The fraction sizes ranged from 25 to 100 cGy. Parallel-opposed anteroposterior-posteroanterior portals were the most common field arrangement. The target volume was reduced in 18 out of 22 courses. The percentage of field reduction ranged from 0 to 59.57% (mean 24.82%). Twelve of 14 courses were successful in achieving symptom palliation. Of the six patients who received SI for a combination of splenic symptoms and abnormal blood tests, five had symptomatic palliation but only one patient responded haematologically. Of two patients who were started on palliative SI for abnormal haematology alone, only one responded. In summary, 17 of 20 (85%) courses of SI initiated for symptom control resulted in effective palliation. Only two of eight (25%) courses of SI started for abnormal blood counts produced a desired response. To conclude, SI offers an effective and well-tolerated palliative treatment option.

Late complications in long-term survivors of biliary atresia.

Ninety patients with biliary atresia surviving more than 5 years were analysed with respect to late complications occurring after the age of 4 years. Thirty-five had complications including cholangitis, portal hypertension, hypersplenism, gastrointestinal bleeding, and esophageal varices. These complications occurred at various times. The background factors of late complications were past history of cholangitis soon after the operation, advanced age at operation, re-operation, high portal pressure at initial operation, and a long interval before disappearance of jaundice after surgery. These factors are mostly related to the first operation and its postoperative course. Therefore, we stress that late complications can be prevented by intensive treatment of the patient at the time of the first operation.

Evolving therapy of hairy cell leukemia.

The rationale for antileukemic therapy in hairy cell leukemia is to reduce the significant risk of infection and other potential serious complications. Corticosteroids have limited value; both corticosteroids and chemotherapy are associated with substantial risks of infection. The mainstay of therapy has been splenectomy. Improvement is seen in 50% to 70% of patients with cytopenias; although the impact of splenectomy on survival has not been clearly demonstrated, prolonged hematologic improvement can occur. Splenectomy presumably alleviates the pancytopenic effect of hypersplenism by removing the preferred site of leukemic cell proliferation. Human interferon represents a major advance in management. Favorable results with natural leukocyte alpha interferon have been confirmed by data with biosynthetic (recombinant) alpha interferon. Importantly, the incidence of infection has been clearly shown to decrease, suggesting improved survival in patients with advanced hairy cell leukemia. Many questions regarding interferon therapy remain unanswered, including optimal dose, optimal duration, and maintenance therapy after maximal response. The mechanism of action is unclear, but possibly interferon modulates as yet unidentified lymphokines or growth factors. In vitro evidence suggests a direct antiproliferative effect of type I interferon on hairy cells. Preliminary data suggest that although toxicity issues, including induction of immunodeficiency and renal insufficiency require further clarification, deoxycoformycin, an adenosine deaminase inhibitor, is also highly effective and holds substantial promise as an important therapeutic modality.

Effect of portacaval anastomosis on hypersplenism.

Leukopenia, thrombocytopenia, and hemolytic anemia occur commonly in advanced cirrhosis. Some investigators have reported that portacaval anastomosis (PCA) abolished hypersplenism while others have not found PCA to be uniformly beneficial. We compared the frequency of hypersplenism before and after admission to a controlled investigation of the effects of PCA in 52 unoperated control subjects and 38 patients with patent PCA. The two groups were followed for an average period of 5 1/2 years. On admission to the study leukopenia was present in about 2% of patients, thrombocytopenia in 6%, and hemolytic anemia in 4%. Splenomegaly was present in 48% and hypersplenism in 11%. After randomization splenomegaly disappeared more frequently in the shunted group. In addition, fewer patients with PCA developed splenomegaly for the first time after inclusion into the study than did unoperated control subjects. Leukopenia, thrombocytopenia, and hemolytic anemia, when present at inclusion into the study, disappeared with equal frequency in the shunted and unshunted patients, and appeared with equal frequency in both groups after randomization in previously unaffected patients. In no instance was hypersplenism clinically significant nor was splenectomy considered or carried out in any of these 90 patients. In additional uncontrolled studies we observed that therapeutic PCA did not affect hypersplenism differently from prophylactic PCA. We conclude that PCA has neither clinically nor statistically significant effects on hypersplenism.