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Congenital mitral stenosis is a rare anomaly. We report successful surgical management of congenital mitral stenosis in a patient with Kabuki syndrome. A 15-month-old male with Kabuki syndrome was referred and echocardiography showed isolated congenital mitral stenosis. The valve was not repairable as it was showing severe stenotic features in multilevel fashion. Valve was replaced with mechanical valve. Postoperative course was uneventful without any rhythm problem and he has been doing well clinically during the 25 months follow-up with regular international normalized ratio checks.

Both perivalvular leakage and kinked prosthetic graft may cause hemolysis. A 72-year-old man was refereed to our hospital because of hemolytic anemia. He has past histories of total aortic arch replacement and repeat aortic valve replacement for aortic aneurysm and prosthetic valve endocarditis. Pre-operative examinations demonstrated aortic valve perivalvular leakage and severe graft kinking of the elephant trunk. Repeat aortic valve replacement and axillo-femoral bypass were performed successfully. Hemolysis got better after the operation and the patient discharged home in stable condition.

A 66-year-old man presented with hemolytic anemia, which required frequent blood transfusion, 6 months after surgical repair of an ascending aortic pseudoaneurysm. Hemolysis was attributed to luminal stenosis caused by graft kinking by laboratory test, CT and four-dimensional magnetic resonance imaging. First, an Excluder cuff was placed at the stenotic site under rapid pacing, but it migrated distally. Thereafter a Palmaz XL stent was placed at the stenotic site, which led to resolution of anemia. In this case, a Palmaz XL stent was successfully used to treat hemolytic anemia caused by graft kinking following ascending aortic surgery., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: Children undergoing splenectomy for hemolytic anemia often have cholelithiasis, which may or may not be symptomatic. It is unclear whether concurrent cholecystectomy increases length of stay or morbidity after splenectomy. The purpose of this study was to compare morbidity among children undergoing laparoscopic splenectomy alone versus splenectomy with concurrent cholecystectomy in patients with hemolytic anemia., Methods: We retrospectively evaluated children with hemolytic anemia undergoing non-traumatic laparoscopic splenectomy in the National Surgical Quality Improvement Program-Pediatric database (2012-2020). Outcomes were compared for patients undergoing splenectomy alone (n = 1010) versus splenectomy with cholecystectomy (n = 371). Pearson's Chi-square and Student's t-tests were utilized as appropriate. Propensity score-matching was completed, controlling for eight demographic and clinical variables., Results: 1381 patients were identified, 73.1% undergoing splenectomy alone and 26.9% splenectomy with cholecystectomy. Splenectomy with cholecystectomy patients were older (10.9 years vs. 8.4 years, p < 0.01), more likely to have hereditary spherocytosis (56.1% vs. 40.8%, p < 0.01), less likely to have sickle cell disease (12.1% vs. 33.5%, p < 0.01), more likely ASA class 1 or 2 (49.3% vs. 42.1%, p < 0.01), and had similar preoperative hematocrit levels (29.6 vs. 29.3, p = 0.33). The splenectomy with cholecystectomy group was less likely to receive preoperative blood transfusions (13.5% vs. 25.4%, p < 0.01). There were 360 pairs selected on propensity score-matching, and splenectomy with cholecystectomy was associated with increased operative time (182 min vs. 145 min, p < 0.01) and decreased occurrences of a postoperative transfusion (4.2% vs. 8.9%, p = 0.01). Length of stay after surgery (2.5 days vs. 2.3 days, p = 0.13), composite morbidity (3.9% vs. 3.4%, p = 0.69), and 30-day readmission rates (3.3% vs. 7.4%, p = 0.08) were all similar., Conclusions: Splenectomy with cholecystectomy is associated with similar postoperative morbidity, length of stay and readmission rates compared to splenectomy alone. These data support the safety of concurrent cholecystectomy with splenectomy for children with cholelithiasis in the setting of hemolytic anemia., Type of Study: Retrospective Cohort Study., Level of Evidence: Level III., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Clinically insignificant hemolytic anemia is occasionally a complication of prosthetic valve replacement. However, hemolysis related to kinked grafts is a very rare complication after central repair for acute aortic dissection., Case Presentation: A 42-year-old man had undergone replacement of the ascending aorta and a root repair for type A aortic dissection 6 months previously. Laboratory data showed mild hemolysis 5 months later, and he began to complain of fatigue on exertion. The serum hemoglobin level reduced to 8.6 g/dL, and lactate dehydrogenase levels increased to 3071 IU/L with gross change in urine color, indicating hemoglobinuria. We diagnosed mechanical hemolytic anemia caused by a kinked graft and planned a repeat operation. The kinked graft was resected and graft-graft anastomosis was performed. Postoperatively, the clinical course was uneventful, and the hemolytic anemia completely resolved., Conclusion: We herein report a case of hemolytic anemia caused by kinking of the graft 6 months after acute aortic dissection repair. The diagnosis was swiftly made, and the patient was successfully managed with redo surgery., (© 2022. The Author(s).)

A 67-year-old male underwent ascending aortic replacement for Stanford type A acute aortic dissection four years ago. Residual false lumen in the aortic root and mild to moderate aortic regurgitation were noted postoperatively. Two and a half years later, he presented with hemolytic anemia and shortness of breath. Computed tomography (CT) revealed aneurysmal aortic root and severely kinked vascular prosthesis, and echocardiography showed severe aortic regurgitation. It was considered that the proximal residual false lumen gradually enlarged, and the graft was pushed up distally and kinked, resulting in hemolytic anemia. Surgical treatment was indicated because of deteriorating shortness of breath due to hemolytic anemia (Hb 6.7 g/dl, LDH 1,528 U/l) and aortic regurgitation. Aortic root replacement was successfully performed and hemolytic anemia improved immediately after surgery.

Background: Intractable, mechanical hemolytic anemia (IMHA) is a rare catastrophic complication following mitral valve surgery. We analyzed patient characteristics and IMHA management by reoperations after mitral valve surgery., Methods: We collected medical records from mitral valve patients requiring reoperation due to IMHA., Inclusion Criteria: hemoglobin < 100 g/L; positive hemolysis tests and echocardiography results; and exclusion of other hemolysis causes., Results: Data from 25 IMHA cases included 10 (40%) early onset (1.3 (0.3,3.0) months) and 15 (60%) late onset (120 (24,204) months) cases. Early IMHA etiologies included surgical defects (6, 60%), uncontrolled infection (3, 30%) and Bechet's disease (1, 10%). Late IMHA etiologies included degeneration (13, 87%), new infection (1, 7%) and trauma (1, 7%). There were more mechanical valves (15, 88%) than bio-valves (2, 12%); the main valvular dysfunction was paravalvular leak (16, 64%). IMHA manifestations included jaundice (18, 72%), dark urine (21, 84%), heart failure (16, 64%), acute kidney injury (11, 44%), hepatomegaly (15, 60%), splenomegaly (15, 60%) and pancreatitis (1, 4%). Laboratory results showed decreased hemoglobin (70 ± 14 g/L) and increased bilirubin (72 ± 57 μmol/L), lactate dehydrogenase (2607 ± 2142 IU/L) and creatinine (136 ± 101 μmol/L) levels. Creatinine level negatively correlated with hemoglobin level (B = -3.33, S.E. B = 1.31, Exp(B) = 368.15, P = 0.018). Preoperative medications included iron supplements (20, 80%), erythropoietin (16, 64%) and beta-blocker (22, 88%). Two patients died of cardiac causes before reoperation. The other 23 underwent reoperation with long surgical times (aortic cross clamp 124 ± 50 min, cardiopulmonary bypass 182 ± 69 min) and blood transfusions (red blood cells 6 (6, 8) units, plasma 600 (400,800) ml, platelet 1(0,2) units). Postoperative complications included cardiac dysfunction (5, 22%), arrhythmia (10, 43%), sepsis (6, 26%), pulmonary infection (5, 22%), gastrointestinal bleeding (3, 13%), cerebral hemorrhage (2, 9%), chronic renal dysfunction (1, 4%) and surgical hemorrhage (1, 4%). Five (33%) patients died after reoperation from cardiac dysfunction (3, 60%), septic shock (1, 20%) and self-discharge (1, 20%)., Conclusions: IMHA induces severe multi-organ dysfunction, contributing to high mortality. Perioperative management should focus on etiological treatment, organ protection, and blood management.

Hemolytic anemia is a well-recognized complication in patients with left-sided mechanical heart valves. It is rare to see hemolysis with a bioprosthetic valve in the right ventricular outflow tract. We report a 4-year-old-girl with history of truncus arteriosus status-post repair who developed hemolytic anemia as a result of a calcified and obstructed bioprosthetic right ventricular to pulmonary artery-valved conduit. The hemolysis was alleviated by replacing the obstructed conduit with a larger valved conduit.

Hemolysis is a well-recognized complication after prosthetic valve replacement, especially with perivalvular leaks. Hemolytic anemia associated with mitral valve (MV) repair is less common. We report the case of a young man with severe hemolytic anemia caused by turbulence of blood flow through a very small quadrangle orifice due to early failure of MV repair. The patient underwent redo MV biologic prosthesis replacement and tricuspid valve annuloplasty. The hemolysis completely disappeared few months later. In this case, we describe a new presentation of mechanical hemolysis due to early failure of MV repair that has not been described in the literature., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Backgrounds: Hemolysis related to a kinked prosthetic graft or inner felt strip is a very rare complication after aortic surgery. We describe herein a case of hemolytic anemia that developed due to aortic flap of the dissection and inversion of an inner felt strip that was applied at the proximal anastomosis of a replaced ascending aorta 10 years previously., Case Presentation: A 74-year-old woman presented with consistent hemolytic anemia 10 years after replacement of the ascending aorta to treat Stanford type A acute aortic dissection. The cause of hemolysis was attributed to mechanical injury of red blood cells at a site of stenosis caused by aortic flap of the dissection and inversion of the felt strip used for the proximal anastomosis. Repeated resection of the strip and graft replacement of the ascending aorta resolved this problem., Conclusions: We considered that blood flow disrupted by a jet of blood at the site of the proximal inner felt strip was the cause of severe hemolysis, we describe rare hemolytic anemia at the site of aortic flap and inverted felt strip after replacement of the ascending aorta.

We describe the case of an 82-year-old woman who had undergone aortic mechanical valve replacement for aortic stenosis with a small annulus, and coronary artery bypass grafting. Four years after the operation, she began to experience hemolysis. Prosthetic valve obstruction was observed but there was no paravalvular leakage or aortic regurgitation through the mechanical valve. We elected to perform apicoaortic bypass in this patient with severe hemolytic anemia secondary to a mechanical valve malfunction., (© The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.)

Objectives: The elephant trunk technique for aortic dissection is useful for reducing false lumen pressure; however, a folded vascular prosthesis inside the aorta can cause haemolysis. The purpose of this study was to investigate whether an elephant trunk in a small-calibre lumen can cause haemolysis., Methods: Inpatient and outpatient records were retrospectively reviewed., Results: Two cases of haemolytic anaemia after aortic surgery using the elephant trunk technique were identified from 2011 to 2013. A 64-year-old man, who underwent graft replacement of the ascending aorta for acute Stanford type A aortic dissection, presented with enlargement of the chronic dissection of the descending aorta and moderate aortic regurgitation. A two-stage surgery was scheduled. Total arch replacement with an elephant trunk in the true lumen and concomitant aortic valve replacement were performed. Postoperatively, he developed severe haemolytic anaemia because of the folded elephant trunk. The anaemia improved after the second surgery, including graft replacement of the descending aorta. Similarly, a 61-year-old man, who underwent total arch replacement for acute Stanford type A aortic dissection, presented with enlargement of the chronic dissection of the descending aorta. Graft replacement of the descending aorta with an elephant trunk inserted into the true lumen was performed. The patient postoperatively developed haemolytic anaemia because of the folded elephant trunk, which improved after additional stent grafting into the elephant trunk., Conclusions: A folded elephant trunk in a small-calibre lumen can cause haemolysis. Therefore, inserting an elephant trunk in a small-calibre true lumen during surgery for chronic aortic dissection should be avoided., (© The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: Haemolytic anaemia after acute aortic dissection surgery is extremely rare. We report 4 cases of haemolytic anaemia with different aetiologies., Methods: Four patients underwent emergency operation for acute type A aortic dissection and subsequently developed haemolytic anaemia., Results: Case 1: a 41-year old man underwent hemiarch replacement. We performed total arch replacement 3 years postoperatively, which revealed that haemolytic anaemia was induced by proximal anastomotic stenosis caused by inverted internal felt strip. Case 2: a 28-year old man diagnosed with Marfan syndrome underwent total arch replacement. Five months postoperatively, we noted severe stenosis at the previous distal anastomotic site, which caused the haemolytic anaemia, and performed descending thoracic aortic replacement for a residual dissecting aneurysm. Case 3: a 49-year old man underwent hemiarch replacement. Three years postoperatively, we performed total arch replacement for a residual dissecting aortic arch aneurysm and repaired a kinked graft responsible for haemolytic anaemia. Case 4: a 42-year old man underwent total arch replacement. Eighteen months later, we performed descending thoracic aortic replacement. We repaired a portion of the ascending aorta as haemolityc anaemia was induced by kinking of a total arch replacement redundant graft., Conclusions: All the haemolityc anaemia patients were successfully released after surgical reintervention.

A patient with a history of chronic lymphocytic leukaemia and a previous splenectomy underwent full blood count analysis in a general hospital. Her medical care had previously taken place in a different institution. A CELL- DYN Sapphire analyser measured her lymphocyte count at ten-fold higher than her known baseline. The sample was sent to her previous hospital, where the laboratory utilises an ADVIA-2120i analyser. The results of this analysis were in keeping with her baseline. The spurious result appears to be related to red cell lysis resistance following splenectomy; however, this resistance appeared to be specific to the analytical method used.

A 26-yr-old male patient reported worsened dyspnea, dizziness one year after an emergency Bentall operation for type A aortic dissection. There was evidence of hemolytic anemia and aortogram revealed a significant stenosis at the distal anastomosis site. During the reoperation, we found the inner felt at the distal anastomosis was inverted causing a significant stenosis. The reoperation successfully resolved this problem. Here, we report a rare case of hemolytic anemia caused by an inverted inner felt after Bentall operation.

Although hemolytic anemia after mitral valve replacement (MVR) is well recognized, hemolytic anemia associated with mitral valve repair is an uncommon condition. Furthermore, persistent and severe hemolytic anemia despite of medical treatment subsequently requiring reoperation is extremely rare. We here report three cases of intractable hemolytic anemia after mitral valve repair leading to MVR. Collision of regurgitant jet into the annuloplasty ring was the speculated mechanism of hemolysis in all cases. After MVR, all of them experienced immediate resolution of the hemolytic anemia., (© 2013, Wiley Periodicals, Inc.)

Paravalvular leak (PVL) is an uncommon yet serious complication associated with surgical prosthetic valve implantation. Paravalvular leak can have significant clinical consequence such as congestive heart failure, haemolytic anaemia, and infective endocarditis. Recently, transcatheter therapy has been applied to the treatment of this disorder with reasonable procedural and clinical success. This review discusses the current state of PVLs, the utilization of multi-modality imaging in their diagnosis and treatment, and the available therapeutic options. Further aim of this review is to examine transcatheter therapy of PVLs including the principles, outcomes, and procedural-related complications.

Hemolysis is a potential complication of mitral valve repair. We report a case of hemolytic anemia after mitral valve repair successfully treated with re-repair, and review the literature on reoperation after failure of mitral valve repair with associated hemolysis., (© 2013 Wiley Periodicals, Inc.)

A 59-year-old male who had undergone aortic and mitral valve replacement with Starr-Edwards ball valves 27 years ago was admitted to our hospital for hemolytic anemia and heart failure. Echocardiography revealed prosthetic valve failure with a high-pressure gradient and small effective orifice area. The Starr-Edwards ball valves were successfully replaced with bileaflet mechanical valves. The explanted valves revealed no structural abnormalities.

Introduction: Laparoscopic splenectomy using pneumoperitoneum has been performed since 1992. The gasless abdominal wall-lifting method for laparoscopic splenectomy was introduced as an alternative. This retrospective study was undertaken to compare results using the two techniques., Methods: Between 1995 and 2010, 54 patients underwent laparoscopic splenectomy at a single institution; 30 underwent the procedure using the gasless technique and 24 using pneumoperitoneum. There were no significant differences between the two groups regarding age, sex or BMI, but more patients underwent concurrent operations in the pneumoperitoneum group. The abdominal wall-lift system with subcutaneous K-wires was used for the gasless method., Results: Intraoperative blood loss was similar in the two groups (193.0 ± 196.7 mL gasless, 217.3 ± 296.6 mL pneumoperitoneum; P > 0.05), but operative time (182.1 ± 92.1 min, 135.1 ± 46.1 min; P < 0.05), and resected spleen weight (306.1 ± 297.7 g, 138 ± 81.0 g; P < 0.05) were significantly different. In the gasless group, additional procedures included conversion (n = 1), mini-laparotomy (n = 2), and CO(2) insufflation (n = 2). Excluding the concurrent living-related kidney donor patients, hospital stay was similar (6.9 ± 2.5 days, 6.3 ± 2.0 days, P > 0.05)., Conclusion: Although gasless laparoscopic splenectomy is feasible, there are disadvantages, particularly the restricted operative working space in some patients. These results suggest that either technique may be used on an individual basis in patients undergoing laparoscopic splenectomy., (© 2011 Japan Society for Endoscopic Surgery, Asia Endosurgery Task Force and Blackwell Publishing Asia Pty Ltd.)

We report a case of a patient affected by compound heterozygosis for two PK-LR gene mutations: p.R486W (c.1456C>T) and p.M403I (c.1209G>A). Our patient suffered from an initial moderate hemolytic anemia which subsequently evolved into a severe form after mitral prosthetic valve replacement for valve regurgitation. Thereafter, the clinical features evolved into a worsening of anemia, heart failure and pulmonary hypertension, in the absence of valve dysfunction. This clinical picture improved only after an intensive transfusion regimen. This case highlights aspects concerning the intricate balance between the risks and benefits of a mechanical prosthetic valve implant in PK-deficient patients., (Copyright © 2011. Published by Elsevier Inc.)

Paravalvular leakage (PVL) remains an unavoidable late complication after valve surgery. We indicate surgery only cases with progressive congestive heart failure and/or hemolytic anemia. We review our clinical experiences of PVL surgery. Between 1992 and 2009 we experienced 8 cases of aortic PVL-6 subjects after primary aortic valve replacement (AVR) and 2 subjects after re-AVR-and 10 subjects with mitral PVL-5 cases after primary mitral valve replacement (MVR) and 5 cases after re-MVR. Mitral PVL began in the later phases after surgery and had more severe symptoms, because of heart failure and/or hemolytic anemia, than aortic PVL. Aortic PVL occurred more frequently because of laxation of sutured threads without frequent sites. Conversely, mitral PVL was mainly caused by cutting annulus tissue around the anterior commisurae after primary MVR, and by a valve-on-valve structure on the middle scallop of the posterior leaflet or circumferentially after re-MVR. All operations were performed safely and all patients were discharged uneventfully. No cases experienced recurrence of PVL in the follow-up period. The symptoms of PVL became exacerbated, and our surgical indications based on these symptoms were validated. Valve-on-valve replacement, which was a major cause of PVL after re-MVR, should be avoided in a re-MVR procedure. Cautious follow-up is necessary, even in the late phase after surgery, especially for patients who have undergone MVR.

We report on a 29-year-old woman with severe hemolytic anemia following mitral valve annuloplasty. Although hemolysis due to mechanical prosthetic mitral valve is well recognized, hemolytic anemia associated with mitral valve repair is an uncommon condition. Reoperation may be considered if the patient has serious and persistent anemia. Although valve replacement is suggested to be a unique intervention, it may not be the solution every time because of mechanical effects. Various mechanisms of hemolysis related to mitral valve repair were suggested, but sufficient and precise data is not available. In this case, we tried to emphasize whether all hemolytic anemias that occur after mitral valve repair require surgical treatment., (Copyright © 2010 Wiley Periodicals, Inc.)

Aortic interposition grafting combined with aortic root replacement (conduit) is widely performed to manage thoracic aortic aneurysms. Intravascular hemolysis without clinical significance is occasionally observed as a complication after prosthetic valve replacement. Symptomatic lysis of red blood cells (RBCs) is rare and primarily attributed to mechanical damage as result of high shear stress, turbulent flow, and physical interaction. We report a case of severe hemolytic anemia shortly after mechanical conduit implantation. The RBC damage was not related to the valve prosthesis and resolved completely after replacement of a section of the kinked Dacron tube graft to correct a fold.

Until now 40 robotic splenectomies were performed in our department, the first case being done on February 25, 2008. Our data show that robotic splenectomy with the DaVinci surgical system is technically feasible and safe, with good results and without complications. The main advantages are a better tridimensional view and an increased versatility of the surgical instruments. The DaVinci system allows an accurate dissection around the splenic hilum and preservation of the splenic remnant vessels in partial splenectomy. Robotic splenectomy will probably not replace the laparoscopic splenectomy for the most common indications like ITP, hemolytic anemia. It may be a very useful surgical tool in difficult splenectomy: partial splenectomy, splenectomy in liver cirrhosis, splenic tumors or malignant hemopathies. In these cases the robotic approach may shorten the operative time, decrease the blood loss and the risk of remorrhagic complications during surgery and even make possible a minimally invasive splenectomy very difficult to be performed by classical laparoscopy.

A primigravida with eclampsia and hemolytic anemia, elevated liver enzymes, and low platelet count (HELLP syndrome) developed intra-abdominal compartment syndrome requiring a decompressive laparotomy, underlining the importance of including abdominal compartment syndrome in the differential diagnosis in pregnant women., ((c) Thieme Medical Publishers.)

Most of the hemoglobin variants are the result of single amino acid replacement in one of the globin chains. In many cases, these hemoglobinopathies are harmless, while in others they determine alterations in the physical and chemical properties, raising clinical manifestations of variable severity. In the unstable hemoglobinopathies, the changes reduce solubility, inducing the formation of precipitates of denaturated hemoglobin (Heinz bodies), which damage the membrane and finally destroy the red blood cells prematurely. Up to now, more than 150 different unstable hemoglobins have been described; most of them cause chronic hemolysis, increased by infections or drugs. We report the clinical presentation of an unstable hemoglobin (hemoglobin Hammersmith) in a girl with severe hemolytic anemia, splenomegaly and red blood cell requirement.

Cor triatriatum is a congenital malformation of the heart which is usually presented with heart failure. We have dealt with an unusual presentation of a two-month-old child with cor triatriatum presented with hemolytic anemia.

Atypical hemolytic uremic syndrome is often associated with mutations in genes encoding complement regulatory proteins and secondary disorders of complement regulation. Progression to kidney failure and recurrence with graft loss after kidney transplantation are frequent. The most common mutation is in the gene encoding complement factor H. Combined liver-kidney transplantation may correct this complement abnormality and prevent recurrence when the defect involves genes encoding circulating proteins that are synthesized in the liver, such as factor H or I. Good outcomes have been reported when surgery is associated with intensified plasma therapy. A consensus conference to establish treatment guidelines for atypical hemolytic uremic syndrome was held in Bergamo in December 2007. The recommendations in this article are the result of combined clinical experience, shared research expertise, and a review of the literature and registry information. This statement defines groups in which isolated kidney transplantation is extremely unlikely to be successful and a combined liver-kidney transplant is recommended and also defines those for whom kidney transplant remains a viable option. Although combined liver-kidney or isolated liver transplantation is the preferred therapeutic option in many cases, the gravity of risk associated with the procedure has not been eliminated completely, and assessment of risk and benefit requires careful and individual attention.

We present a patient with persistent severe hemolysis following mitral valve repair which resolved after valve replacement with bioprosthesis. The posterior portion of the annuloplasty ring was retained due to severe calcifications of the posterior mitral valve annulus. All chordae were preserved to avoid disruption of the mitral annulus.

We report three cases of hemolytic anemia caused by anastomotic stenosis after surgical treatment for aortic dissection in which internal and external Teflon (DuPont, Wilmington, DE) felt strips were used for reinforcement of the aortic stump. To detect this complication, laboratory findings typical of red cell fragmentation syndrome as well as appropriate imaging modalities are necessary. As a precaution, it is necessary to be meticulous when stitching the internal felt strip.

A 60-year old man presented with hemolytic anemia, heart failure, and renal dysfunction 4 years after ascending and total arch replacement for Stanford type A aortic dissection. The hemolysis was caused by graft compression by a pseudoaneurysm arising from a proximal anastomosis. Reoperation of resection of pseudoaneurysm and repair of ascending aorta and arch successfully resolved these problems.

Hemolytic anemia related to intracardiac prosthetic materials has been described. We describe a case of a 74-year-old female with a history of hypertrophic obstructive cardiomyopathy that had a postoperative ventricular septal defect closed with a muscular ventricular septal defect occluder. The device caused intractable hemolytic anemia that required surgical removal and repair. We describe a rare cause of hemolysis complicating transcatheter closure of a post-traumatic ventricular septal defect.

In cases of accessory splenic tissue in postsplenectomy patients, it is of utmost importance to localize the accessory spleen prior to surgery. Several studies have shown the feasibility of laparoscopic resection of accessory splenic tissue using preoperative scintigraphy. We present the cases of three postsplenectomy patients with accessory splenic tissue causing relapsing hematologic disease. Accessory spleens were diagnosed and localized preoperatively by positive uptake of heat-damaged Tc99m-labeled red blood cells using scintigraphy. Two patients with relapse of immune thrombocytopenic purpura and one with hemolytic anemia underwent handheld gamma probe-assisted laparoscopic accessory splenectomy. One patient with immune thrombocytopenic purpura recovered his platelet count at 3-year follow-up. The other patient had a relapse of disease within 3 months despite successful removal of the accessory spleen. The patient with hemolytic anemia had postoperative relapse; two accessory spleens were identified on radionuclide investigation. The use of intraoperative nuclear imaging can greatly aid in localization and provide confirmation of complete laparoscopic excision of the nuclear focus. The technique is especially useful in cases of a small accessory spleen, by avoiding a major open procedure and contributing to good postoperative results.

Objective: To define indications and outcome in haematologic cases undergoing splenectomy., Study Design: A retrospective review of clinical records from consecutive patients having open or laparoscopic removal of the spleen in an academic centre in the private sector. Endpoints were survival, operating time, spleen size, histopathology, requirements for blood or related products complications and average costs., Results: In the total group (n = 69) there were two deaths. Referrals were for immune thrombocytopaenia (41%), acquired haemolytic anaemia (10%), myeloproliferative syndrome (9%), acute or chronic leukaemia (19%), lymphoma (13%) and a miscellaneous group (8%), comprising cholelithiasis, aplasia or as a diagnostic procedure for otherwise unexplained splenomegaly. An open midline approach was predicated by spleens greater than twice normal size and a history of any bleeding disorder. Here the mean operating time was 83 min (range 40-295) whereas for laparoscopy this was 251 min (range 181-272)., Summary: Careful stratification between the two options facilitated optimum haemostasis and consequently reduced requirement for packed red cells and platelets. Neither underlying pathology nor the choice of treatment influenced morbidity or mortality. Overall local experience is consistent with published international standards of surgical practice. Outcome is directly proportional to the number of each procedure carried out by a single team, observance of consistent protocols for preoperative evaluation and standardized proactive management through the recovery period.

Hereditary stomatocytosis is an autosomal dominant, congenital hemolytic form of anemia, and thromboembolic complications may ensue after a splenectomy is performed in a patient with hereditary stomatocytosis. However, little is known about the relationship between pulmonary hypertension and hereditary stomatocytosis. We describe here a rare case of a patient with hereditary stomatocytosis who developed pulmonary hypertension due to thromboembolic disease after splenectomy. At the first presentation, he was diagnosed with hemolytic anemia, but the cause was unknown, and he was also diagnosed with pulmonary hypertension. Thereafter, we linked his pulmonary hypertension with hemolytic anemia because his red blood cells appeared as leptocytes, acanthytes, and stomatocytes on scanning electron microscopy. It is important to confirm the diagnosis of hemolytic anemia, and splenectomy should be performed with due consideration of whether patients have hemolytic anemia.

Thromboembolic complications following splenectomy for hematologic diseases occur in up to 10% of patients and may range from portal vein thrombosis (PVT) to pulmonary embolism (PE) and deep vein thrombosis (DVT). Up to now there exist no recommendations for the duration and intensity of prophylactic anticoagulation, which usually follows local institutional protocols. We report on three consecutive patients with severe portal vein thrombosis and/or pulmonary embolism--one with fatal outcome--7 to 35 days after splenectomy for autoimmune hemolytic anemia, immunothrombocytopenia, and indolent lymphoma, respectively. Incidence and pathophysiology of thromboembolic events (TE) in this patient group as well as prophylactic anticoagulation will be discussed, including a review of the current literature on this topic., (Copyright 2004 Wiley-Liss, Inc.)