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Surgical management of children with hemoglobinopathies.

Sickle hemoglobinopathies include sickle cell disease, sickle-C disease, and sickle-beta thalassemia. Patients with these disorders commonly suffer a multitude of destructive events to vital organs, especially to the central nervous system, the spleen, the kidney, the lung, and the heart as a result of microvascular plugging by the sickled erythrocytes. Thoughtful preparation for anesthesia and operation, especially when directed by experienced individuals, can greatly reduce the hazard of inducing the sickle crises that formerly plagued individuals with sickle hemoglobinopathies who faced major operations under general anesthesia. The patient must be free of any acute illness, especially one involving the respiratory system. Adequate hydration preoperatively combined with avoiding perioperative hypoxia, hypothermia, and acidosis, the triggers for sickling, will go far toward avoiding sickle-induced complications. Modern transfusion therapy, consisting of multiple small transfusions of Hb A erythrocytes administered over several weeks prior to the operation, not only corrects the chronic anemia but suppresses erythropoiesis of cells containing Hb S in the patient's bone marrow and leaves him or her with a majority of cells containing Hb A. This provides a safety net in case a sickle-inducing insult occurs despite the best efforts to avoid one. Individuals with sickle hemoglobinopathies may require any of the operations common to all children, for example, herniorrhaphy, appendectomy, tonsillectomy, and circumcision, but a significant number will develop calcium bilirubinate cholelithiasis and possibly cholecystitis as a result of the continual increased load of bile salts resulting from the shortened lifespan of the cells containing Hb S. Also, although most individuals with Hb S will gradually suffer splenic infarction by late childhood, a significant number of infants will experience acute splenic sequestration crisis, a life-threatening entity, the recurrence of which is prevented by splenectomy. Several publications have demonstrated that such surgical procedures can be performed in large numbers of patients with sickle hemoglobinopathies without deaths and with minimal morbidity.

Adolescent↗

[Abdominal complications of heart surgery].

Abdominal complications after cardiac surgery (excluding "medical" jaundice) are rare. Twenty six cases were observed out of a total of 7 847 operations (0.33%) performed between 1973 and 1980. The causes were very diverse; the most common being gastroduodenal ulceration, usually acute (9 cases). Other cases included intestinal (3 cases of postoperative ileus, 4 cases of mesenteric infarction, 2 cases of necrosing enterocolitis), biliary (2 cases of acute cholecystitis) and splenic pathology (2 cases of splenic infarction, one associated with necrosing enterocolitis). Anticoagulant therapy was implicated in 3 cases. Diagnosis is difficult in the immediate postoperative period, some complications only being recognised at autopsy. The clinical signs may be misleading and the interpretation of complementary investigations difficult. Therefore, the possibility of abdominal complications must be kept in mind, especially in patients with one or more predisposing factors. Excluding accidents due to anticoagulant therapy the following factors were associated with an increased risk of abdominal complications: previous history of gastro intestinal pathology (ulcer, gall stone, alcoholism) the nature of the underlying cardiac disease (coronary artery and aortic valve disease), cardiopulmonary bypass, and, above all, per- and postoperative incidents: hypovolaemia, low output syndrome (half the patients in this series) respiratory and infectious complications. The inappropriate use of vasoconstricting agents may also play a role. The majority of abdominal complications seemed to be related to ischaemia and anoxia in the splanchnic territory, which explains the important role of the preceding factors. The prognosis of abdominal complications after cardiac surgery was poor, mortality reaching 50 to 100% in some causes: in this series, 12 of the patients died. This justifies certain prophylactic measures: strict selection of patients, diagnosis and treatment of associated abdominal pathology before operation, prevention of low output states, respiratory and infectious complications ... and careful examination of the abdomen after operation to ensure the early diagnosis and treatment of complications, should they develop.

Acute Disease↗

Gastric varices with splenic vein occlusion treated by splenic arterial embolization.

A 53-year-old man was admitted to our hospital in August 1997 with enlarged gastric varices. Computed tomography (CT) showed splenic vein occlusion, gastric varices, and extra-gastric wall collateral veins. Color flow images of gastric varices were clearly visualized, and the velocity in the gastric varices was 19.6 cm/s via endoscopic color Doppler ultrasonography (ECDUS). The patient was diagnosed with gastric varices according to angiographic findings of splenic vein occlusion, and splenic arterial embolization was performed. Two weeks after the splenic arterial embolization, CT showed peripheral areas of low attenuation in the spleen, due to splenic infarction, with 70% of the spleen volume showing low attenuation. Eight months after the splenic arterial embolization, ECDUS revealed a decrease in gastric variceal color flow images, with the velocity in the gastric varices being 10.3 cm/s.

Chronic Disease↗

Recurrent cerebral embolism in a familial amyloid polyneuropathy patient who received partial liver transplantation from a living donor.

Cerebral embolism in relation to cardiac amyloidosis has not been widely noted. A 47-year-old woman who had been suffering from familial amyloid polyneuropathy (FAP) for 7 years was treated with partial liver transplantation from a living donor and her early postoperative course was uneventful. During the 391st to 613th postoperative day she experienced recurrent cerebral infarctions, but clinical examinations revealed no disorders capable of producing cerebral embolism. At autopsy splenic infarction and intracardiac thrombi adhering to the mitral valve and left atrium were found, and these areas showed severe amyloid deposition. Amyloid heart is considered to be one possible cause of systemic embolism.

Amyloid Neuropathies↗

Complications of orthotopic liver transplantation: spectrum of findings with helical CT.

Orthotopic liver transplantation has become the treatment of choice for patients with end-stage nonmalignant liver disease. The surgical techniques and immunosuppressive therapy for this procedure have improved considerably. Nevertheless, there are still significant complications, particularly those of vascular origin, which can lead to graft failure and require retransplantation unless prompt treatment is instituted. These complications include arterial and venous thrombosis and stenosis; arterial pseudoaneurysm; biliary leakage, stricture, and obstruction; liver ischemia, infarction, and abscess; fluid collections and hematomas; lymphoproliferative disorders; recurrent tumors; hepatitis C virus infection; and splenic infarction. Since the clinical presentation of posttransplantation complications is frequently nonspecific and varies widely, imaging studies are critical for early diagnosis. Helical computed tomography (CT) is a valuable complement to ultrasonography (US) in the postoperative period and is a safe, accurate, and noninvasive method of demonstrating hepatic vessels (hepatic artery, portal vein, hepatic veins, and inferior vena cava) and evaluating nonvascular complications (in the hepatic parenchyma and bile duct abnormalities) and extrahepatic tissues. Knowledge and early recognition of these complications is essential for graft salvage, and CT can provide valuable information, particularly for patients with indeterminate US results or in whom US examination is difficult.

Aneurysm, False↗

Laparoscopic exclusion of a splenic artery aneurysm using a lateral approach permits preservation of the spleen.

Splenic artery aneurysms have commonly accepted therapeutic indications. Operative therapy for splenic artery aneurysms has traditionally consisted of aneurysm resection with a possible splenectomy. Recently, laparoscopic splenic artery aneurysm exclusion has been shown to provide adequate therapy without incurring the morbidity associated with aneurysmectomy and splenectomy. However, an anterior approach to exclusion may result in short gastric and left gastroepiploic artery disruption leading to splenic infarction. Preservation of the spleen can best be assured by a lateral approach, which preserves collateral splenic perfusion. A 51-year-old woman with a distal splenic artery aneurysm, who underwent an uncomplicated laparoscopic splenic artery aneurysm exclusion using a lateral approach, is described.

Aneurysm↗

[Laparoscopic splenic surgery--report of 17 personal cases].

Since the beginning of the decade we can remark an increasing of publications about laparoscopic approach in splenic surgery. We report about 17 unselected own cases (8 males, 9 females). Main indications were 12 hematologic diseases: 9 idiopathic thrombopenic purpuras, 1 hemolytic anemia, 1 Hodgkin's disease and 1 recurrent splenic infarction with hypersplenism and extramedulary hemapoiesis, further 1 unclear sepsis with focal lesions and splenomegaly, 1 dysontogenetic splenic cyst, 1 splenic co-affection of Wegener's disease, and 2 traumatic lesions. We performed in 13 cases asplenectomy, under these 1 time combined with a laparoscopic hernioplasty and 1 time with a complete staging, further in 1 case a partial splenic resection, in 1 case a diagnostic excision and in 2 cases a hemostyptic management without splenectomy. We had to convert in no case, the lethality rate was also 0. As a complication we noted one postoperative bleeding, we could managing also laparoscopically. Our mean operating time of 137 min was evident shorter than the published ones by other authors. Because of our good results and the excellent tolerance by patients we can recommend this proceeding. Nevertheless, it should be limited for surgeons with a large experience at laparoscopic centers.

Adolescent↗

Splenic artery aneurysm associated with systemic lupus erythematosus: report of a case.

We herein report on a 64-year-old Japanese female patient who presented with a splenic artery aneurysm (SAA) associated with systemic lupus erythematosus (SLE). The saccular aneurysm, which measured 3 cm in diameter, was located in the proximal third of the splenic artery from the pancreas with a portosystemic shunt. A double ligation of the splenic artery (the distal and proximal sides of the aneurysm) was performed without a splenectomy. The postoperative course showed acute pancreatitis without either splenic infarction or portal thrombus. To our knowledge, the closed association of SLE with an aneurysmal dilatation of the splenic artery has not been previously reported. Both the pathogenesis and the management of SAA associated with SLE are discussed following the presentation of this case. This is the first reported case of SAA associated with SLE.

Aneurysm↗

[Diagnostic methods in torsion of the spleen].

Torsion of a spleen is a frequent complication of a rare entity--a wandering spleen. A wandering spleen is revealed occasionally, with physical and/or sonographic exam. However, it is often overseen. It has very unspecific simptoms, and can present a diagnostic problem. Splenic torsion manifets itself like an acute abdomen. We have presented a rare case of splenic torsion with unusual clinical findings, followed by a splenic infarction. Due to their promptness, sensitivity, uniqueness and availability, two diagnostic methods have definitive primacy in visualisation of splenic torsion: Color-Doppler sonography and CT with intravenous application of contrast medium.

Adolescent↗

Complications associated with transcatheter arterial embolization for hepatic tumors.

Transcatheter arterial embolization (TAE) is widely used in the treatment of hepatic tumors. A total of 2,300 TAE procedures were performed with a 2-15-mL injection of a mixture or suspension of anticancer drugs and iodized oil, followed by administration of gelatin sponge particles. One or two chemotherapeutic drugs, including doxorubicin hydrochloride (10-30 mg), epirubicin hydrochloride (10-30 mg), mitomycin C (10-20 mg), and cisplatin (25-100 mg), were used for each procedure. Complications were encountered in 4.4% of cases (n = 102) and were related to the use of chemoembolic agents or the manipulation of a catheter or guide wire. These complications included acute hepatic failure (n = 6), liver infarction (n = 4) or abscess (n = 5), intrahepatic biloma (n = 20), multiple intrahepatic aneurysms (n = 6), cholecystitis (n = 7), splenic infarction (n = 2), gastrointestinal mucosal lesions (n = 5), pulmonary embolism or infarction (n = 4), tumor rupture (n = 1), variceal bleeding (n = 3), and iatrogenic dissection (n = 35) or perforation (n = 4) of the celiac artery and its branches. Knowledge of these complications is important for correct diagnosis and appropriate management.

Angiography↗

Laparoscopic distal pancreatectomy combined with preservation of the spleen for cystic neoplasms of the pancreas.

The precise role of laparoscopy in the resection of cystic neoplasms of the pancreas (CyNP) remains unknown. In addition, the question of spleen-preserving distal pancreatectomy is controversial. This report evaluates the feasibility and outcome of laparoscopic spleen-preserving distal pancreatectomy (LapSPDP) in 19 patients (17 women and 2 men) with CyNP. A prospective comparison was made between 11 consecutive patients (group I) with splenic vessel preservation (SVP) and 8 patients (group II) without SVP (Warshaw technique). This study used color-Doppler ultrasound (CDUS) as a tool to identify patients at high risk for postoperative splenic complications. The mean tumor size was, in both groups, 5 cm. In group I, with an intent-to-treat basis of SVP, only in 54.5% of patients the spleen was preserved with an intact splenic artery and vein; in the remainder, conversion to the Warshaw technique was required for intraoperative bleeding. Evaluation of intraoperative factors showed that the mean operative time was significantly shorter (165 vs. 222 minutes) and the mean blood loss significantly lower (225 vs. 495 mL) in the group of LapSPDP with the Warshaw technique. No patients required blood transfusion in both groups. The overall conversion rate was 0%. The overall rate of pancreatic fistula was 15% and it was classified as biochemical leak (no clinical symptomatology). Overall splenic complications were observed in 16.6% of patients but occurred only in three patients undergoing LapSPDP with the Warshaw technique; CDUS showed in 2 patients a focal splenic infarct; the third patient had an initial hospital stay of 5 days, was readmitted 2 days later for a massive splenic necrosis, and splenectomy was performed. The overall hospital stay was 5.7 days. At mean follow up of 22 months (range 6-42), there have been no local recurrences.

Adult↗

Transcatheter embolization of acute non-variceal gastrointestinal hemorrhage.

OBJECTIVE: To determine the efficacy and safety of transcatheter embolization of nonvariceal gastrointestinal hemorrhage. DESIGN: Descriptive analytical study. PLACE AND DURATION OF STUDY: Radiology Department, The Aga Khan University Hospital, Karachi, Pakistan, from October 1999 to August 2004. SUBJECTS AND METHODS: The study included 27 patients who underwent angiography for suspected gastrointestinal hemorrhage. The presumptive diagnosis was based on the findings of endoscopy in 08 patients, scintigraphy in 05 patients and on clinical findings only in 14 patients. Nine patients had gastroduodenal hemorrhage, 04 patients had hemobilia and 14 patients had lower gastrointestinal bleeding. Five patients had comorbidities. One patient had uremia, 2 had hepatitis C, 1 had cirrhosis and 1 had coagulopathy. Technical success, clinical success, complications and patient survival were assessed. RESULTS: Nine patients had upper gastrointestinal hemorrhage, 4 patients had hemobilia and 14 patients had lower gastrointestinal bleeding. Etiology of bleeding was known in 15 patients and unknown in 12 patients. Clinical success was achieved in 23 (85.18%) of 27 patients. Two patients had early rebleeding. One of these was managed surgically and recovered. The other had severe coagulopathy and was unfit for surgery. Two patients had bowel infarction, one of these was managed surgically and recovered and the other died of complication of procedure. One patient had small splenic infarcts and was managed conservatively. Overall 85.18% success was achieved for the embolization procedure. Two of 27 patients died. One patient died because of recurrence of bleeding and other died because of infarction of right hemi-colon which occurred as complication of embolization. CONCLUSION: Transcatheter embolization is effective and safe in patients with nonvariceal gastrointestinal hemorrhage.

Embolization, Therapeutic↗

Spleen-preserving laparoscopic distal pancreatectomy after division of the splenic vessels.

A 37-year-old woman with a history of syncope was hospitalized with a diagnosis of hypoglycemia due to insulinoma. Computed tomography (CT) and magnetic resonance imaging revealed an enhanced solid mass, 1.5 cm in diameter, at the tail of the pancreas. Angiography via the splenic artery revealed a hypervascular mass. Because the tumor was located deep in the pancreatic parenchyma, laparoscopic distal pancreatectomy was performed. The pancreas was exposed by dissecting the greater omentum, and the tumor was located by intraoperative ultrasonography. After division of the splenic artery, the pancreas, main pancreatic duct, and splenic vein were transected with an endoscopic linear stapler. The pancreatic pedicle was divided at the splenic hilum to preserve the spleen. The postoperative course was uneventful except for the appearance of splenic infarction on a CT scan 2 weeks after surgery but without any overt symptoms. Spleen-preserving laparoscopic distal pancreatectomy by division of splenic vessels is a feasible treatment option for benign pancreatic disease.

Adult↗

Decreased splenic enhancement on CT in traumatized hypotensive patients.

Three patients with transient episodes of hypotension following blunt abdominal trauma incurred in motor vehicle accidents were examined by computed tomography within 6 hours of injury. The examinations were done using a contrast material injection technique that ordinarily leads to greater splenic than hepatic attenuation. None of the patients had splenic injury evident on autopsy, surgery, or clinical follow-up study (one case each), nor did they have other characteristic features of splenic infarction. However, in each case the spleen was less enhanced than the liver, leading to an erroneous impression in one patient that the splenic artery had been disrupted. Physiologic studies have shown that splenic perfusion decreases with sympathetic stimulation; this may have been the cause of the diminished enhancement. Decreased splenic enhancement should be interpreted cautiously in traumatized hypotensive patients.

Adult↗

Wandering spleen: anatomic and radiologic considerations.

Wandering spleen is a rare clinical entity and remains an elusive clinical diagnosis, particularly in the pediatric patient. Among the imaging modalities (computerized tomography, magnetic resonance imaging, nuclear medicine, ultrasonography, plain films) used in the diagnosis of wandering spleen, ultrasonography is the least invasive and most effective. Elective splenopexy remains the treatment of choice when the diagnosis is made before splenic infarction. We present two cases of wandering spleen manifested as a lower abdominal mass, and we discuss the surgical anatomy of the spleen and splenic ligaments and the hypothetical responsibility of these ligaments for the genesis of this clinical entity.

Adolescent↗

Wandering spleen: a challenging diagnosis.

Wandering spleen is rare, particularly in children, and diagnosis is difficult. It usually occurs at 20 to 40 years of age, and most cases are seen in women. Diagnosis is difficult because of lack of symptoms, unless splenic torsion has occurred. Patients usually have an asymptomatic abdominal mass, an acute abdomen, or, most commonly, a mass associated with pain. Laboratory data are nonspecific, but the diagnosis can be confirmed by imaging studies; computed tomography and duplex ultrasonography are preferred modalities. Treatment is operative because of complications of splenic infarction and possible splenectomy. Splenopexy is the treatment of choice for a noninfarcted wandering spleen. Splenectomy should be done only when there is no evidence of splenic blood flow after detorsion of the spleen. We review our experience with wandering spleen in two pediatric patients, one treated with splenopexy and the other with splenectomy.

Abdominal Pain↗

Ultrasonography of splenic abnormalities.

AIM: This report gives a comprehensive overview of ultrasonography of splenic abnormalities. Certain ultrasonic features are also discussed with pathologic correlation. METHODS: We review the typical ultrasonic characteristics of a wide range of splenic lesions, illustrating them with images obtained in our institution from 2000 to 2003. One hundred and three patients (47 men, 56 women), with a mean age of 54 years (range 9-92 years), were found to have an abnormal ultrasonic pattern of spleen. RESULTS: We describe the ultrasonic features of various splenic lesions such as accessory spleen, splenomegaly, cysts, cavernous hemangiomas, lymphomas, abscesses, metastatic tumors, splenic infarctions, hematomas, and rupture, based on traditional gray-scale and color Doppler sonography. CONCLUSION: Ultrasound is a widely available, noninvasive, and useful means of diagnosing splenic abnormalities. A combination of ultrasonic characteristics and clinical data may provide an accurate diagnosis. If the US appearance alone is not enough, US may also be used to guide biopsy of suspicious lesions.

Adolescent↗