Outcome of splenectomy in children younger than 4 years with sickle cell disease

Purpose

Removal of the spleen in patients younger than 4 years has been reported to carry an increased risk of postsplenectomy sepsis and has not been universally accepted. We reviewed our experience with splenectomy in children with acute splenic sequestration crisis (ASSC) younger than 4 years.

Methods

The study involved retrospective review of demographic and operative data, number of ASSC, operative complications, infections, and death.

Results

From 1993 to 2008, 53 patients (28 males, 25 females) younger than 4 years had open (43.8%) or laparoscopic (56.6%) splenectomy after one or more events of ASSC. Six (11.3%) were younger than 18 months, 28 (52.8%) were 18 to 24 months old, and 21 (39.6%) were 24 to 48 months old. Operative complications were diaphragm laceration (laparoscopy, n = 3; 5.7%) and reoperation for bleeding (open, n = 1; 1.8%). Length of stay was similar for laparoscopic (3.6 days) vs open (3.8 days) splenectomy. Mean postoperative follow-up was 5.6 years. In 353 postsplenectomy admissions, 3 (5.7%) patients had positive blood cultures requiring treatment. Three (5.7%) patients died within the 15-year study period; one (1.8%) had documented pneumococcal sepsis.

Discussion

The advantage of early splenectomy may outweigh the risks of long-term transfusion. Splenectomy in young children with sickle cell disease carries a low risk of postsplenectomy sepsis with appropriate vaccination and prophylactic antibiotics. We conclude that splenectomy in young children with ASSC is safe and effective, especially with penicillin prophylaxis and improved vaccination strategies.     

Indications and complications of splenectomy for children with sickle cell disease

Background

Sickle cell anemia (SCA), which is characterized by high hemoglobin (Hb) F level and persistent splenomegaly into the older age group (up to 18 years of age) or even adults, is one of the commonest hemoglobinopathies in the Eastern Province of Saudi Arabia. This makes them liable to develop splenic complications requiring splenectomy. This is a review of our experience in the management of 134 children with SCA who had splenectomy as part of their management at our hospital, with emphasis given to the indications and complications of splenectomy.

Patients and methods

The medical records of all children who had splenectomy at our hospital were retrospectively reviewed for the following: age at splenectomy, sex, Hb electrophoresis, indication for splenectomy, preoperative investigations, type of surgery, spleen weight, histology, perioperative management, and postoperative complications.

Results

From 1990 to 2004, 170 children with various hematologic disorders had splenectomy at our hospital. Of these, 134 had SCA (118 had sickle cell disease and 16 had sickle-β-thalassemia). Recurrent acute splenic sequestration crisis (ASSC) was the commonest indication for splenectomy in 103 (76.9%) patients, followed by hypersplenism in 18 (13.4%). Seven (5.2%) of our patients had splenectomy for splenic abscess (SA) and 2 had splenectomy for massive splenic infarction; 103 (61 boys, 42 girls) patients with a mean age of 7.6 years (range, 1.8-13 years) had splenectomy for ASSC. Their mean Hb F level was 20.5% (range, 9.2%-39.6%). Thirty-two of them had major attacks. Their Hb levels at the time of admission ranged from 1.4 to 4.1 g/dL (mean, 2.5 g/dL). The remaining 71 had minor recurrent attacks. Eighteen had splenectomy for hypersplenism and all had a significant increase in their blood parameters after splenectomy. Seven had splenectomy for SA. In 5 patients, Salmonella was the causative organism; in 1, it was Enterobacter sakazaki, whereas in 1, no organisms were identified. Two of our patients had splenectomy for massive splenic infarction because of persistent left upper quadrant abdominal pain, and 1 had splenectomy for splenomegaly with a nonfunctioning spleen. Twenty-eight (21%) of our patients had splenectomy and cholecystectomy. In 4 patients, this was because of symptomatic gallstones, whereas in the other 24, it was because of asymptomatic gallstones discovered on ultrasound. There was no mortality, but 8 (6%) developed postoperative complications.

Conclusions

With good perioperative management, splenectomy in children with SCA is not only safe, but also beneficial in treating SA, reducing the patients' transfusion requirements, eliminating the risks of ASSC, and eliminating the discomfort and mechanical pressure of the enlarged spleen. Abdominal ultrasound should be done routinely preoperatively for all children with SCA undergoing splenectomy, and if gallstones are discovered, they should undergo concomitant cholecystectomy. This is even so for asymptomatic gallstones. The addition of cholecystectomy to splenectomy does not increase the morbidity, but eliminates the subsequent complications of gallstones and simplifies their future management in case of abdominal crisis as the possibility of cholecystitis is eliminated.     

Splenic syndrome in patients at high altitude with unrecognized sickle cell trait: splenectomy is often unnecessary.

BACKGROUND: The health risks associated with sickle cell trait are minimal in this sizable sector of the world's population, and many of these patients have no information about their sickle cell status. Splenic syndrome at high altitude is well known to be associated with sickle cell trait, and unless this complication is kept in mind these patients may be subjected to unnecessary surgery when they present with altitude-induced acute abdomen. METHODS: Four patients were admitted to the surgical ward with a similar complaint of acute severe left upper abdominal pain after arrival to the mountainous resort city of Abha, Saudi Arabia. All were subjected to splenectomy because of lack of suspicion regarding sickle cell status. RESULTS: Histologic examination of the spleen showed all patients had sickle cells in the red pulp. On further assessment all were found to have sickle cell trait with splenic infarction. In a similar study of 6 patients with known sickle cell disease who had comparable problems when they travelled to the Colorado mountains, all made an uncomplicated recovery with conservative management. CONCLUSIONS: In ethnically vulnerable patients with splenic syndrome, sickle cell trait should be ruled out before considering splenectomy. These patients could respond well to supportive management, and splenectomy would be avoided.     

Is splenectomy for massive splenomegaly safe in children?

PURPOSE: To study and analyze the causes, etiology, morbidity, mortality and therapeutic value of splenectomy performed for massive splenomegaly in children. METHODS: The medical records of 115 children less than 18 years old who had splenectomy for various hematological disorders were reviewed. Twenty of them had splenectomy for massive splenomegaly (spleen weight > or =1,000 g). The records of these were reviewed for age at operation, gender, hematological diagnosis, indication for splenectomy, operative procedures, postoperative complications, and outcome. RESULTS: Twenty children had splenectomy for massive splenomegaly. There were 16 males and 4 females. Their ages ranged from 4 to 15 years (mean 11.2). Twelve had sickle cell disease, 5 had sickle-beta-thalassemia, 1 had beta-thalassemia major, 1 had thalassemia intermediate, and 1 had chronic myeloid leukemia. The indications for splenectomy were hypersplenism in 11, recurrent splenic sequestration crisis in 8, and splenic abscess in 1. The transfusion requirements in the patient with beta-thalassemia major decreased markedly postoperatively from 18 transfusions/year to only 4 transfusions/year; and for those with hypersplenism, there was a marked improvement in their blood parameters following splenectomy. The patient with thalassemia intermediate required no more blood transfusions. There was no mortality. The immediate postoperative morbidity was 10% for those with massive splenomegaly compared with 6.3% for those with splenomegaly <1,000 g. CONCLUSIONS: With good perioperative management, splenectomy in children with massive splenomegaly is both safe and effective.     

Differential diagnosis and therapy of acute abdomen in sickle cell crisis. A rare case in visceral surgery

Surgical therapy of the acute abdomen often allows only limited time for differential diagnosis to confirm the indication for surgery. Under consideration of clinical aspects and case history both common and rare causes of an acute abdomen should be investigated without undue loss of time. Differential diagnostic considerations and eventual therapy are presented in the following case of a 25-year-old Afro-american who developed multiorgan failure after an initial course of lower-back pain. In addition to the clinical setting of an acute abdomen the patient presented with acute respiratory failure and laboratory signs of severe hemolysis in combination with newly detected splenomegaly. The indication for splenectomy was made following CT-proven complete splenic infarction due to repeated acute squestration. Histologic examination of the spleen together with hemoglobin electrophoresis confirmed the clinical assumption of unusually late primary manifestation of a sickle cell crisis. In the underlying case, the hemoglobinopathy was in fact the less common form of combined sickle-cell-beta-thalassemia. A ten-day course of intensive care therapy was necessary to treat ongoing multiorgan failure due to persistent sickle cell crisis. Current diagnostic and therapeutic procedures in connection with sickle cell crisis as a rare cause of an acute abdomen with the necessity for surgical intervention are presented.     

Urinary retention in sickle cell syndromes

Four patients with sickle cell disease noted the development of urinary retention during an acute painful crisis. The acute urinary retention resolved with improvement in the painful sickle cell crisis. No evidence of anatomic urologic defect, systemic neurologic abnormality, or infection was documented in these patients. Patients with sickle cell disease in painful crisis should be observed for signs of acute urinary retention.     

Partial splenectomy in children under 4 years of age with hemoglobinopathy

Background/Purpose: The risk of septicemia in postsplenectomy pediatric patients is approximately 2%. This risk is twice as great for children less than 4 years of age. In the first year of life this risk can be 30% or higher. Partial splenectomy is an alternative in patients with hemoglobinopathies. The authors attempt to assess the outcome of pediatric patients less than 4 years of age undergoing partial splenectomy using the Argon beam.Methods: In a tertiary care, university affiliated, dedicated children's hospital seven patients underwent partial splenectomy (PS) by the same surgeon from May 1993 to September 1995. The PS performed for trauma was excluded. Included were patients with hemoglobinopathies. Therefore, six patients were evaluated. Follow-up was from 6 months to 2 years. Pre- and postoperative blood transfusions, length of operation, estimated blood loss, length of hospital stay, postoperative complications, perfusion, and function of remnant spleen were evaluated.Results: Indications for splenectomy included sequestration crisis and hypersplenism. Percent splenectomy ranged from 65% to 75%. Average hospital stay was 6.3 days. Postoperative splenic function, measured by Pitt count and radionuclide spleen scan, was normal. Postoperatively there were no systemic infections, overwhelming postsplenectomy sepsis (OPSS), torsion of the splenic remnant, left upper quadrant fluid collections, or subphrenic abscesses. There were no deaths.Conclusions: Partial splenectomy is a safe and effective procedure in children less than 4 years of age with hemoglobinopathies. The procedure as described yields minimal blood loss and retains immune competence. Partial splenectomy greatly reduces, and in some cases eliminates, the need for blood transfusions. PS should be considered the procedure of choice in children less than 4 years of age with sickle cell anemia or β-thalassemia who require splenectomy.     

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.     

The natural history of asymptomatic osteonecrosis of the femoral head in adults with sickle cell disease

BACKGROUND: Osteonecrosis of the femoral head is a frequent complication in adult patients with sickle cell disease. However, little is known about the natural history of asymptomatic lesions. METHODS: One hundred and twenty-one patients (121 hips) with sickle cell disease and asymptomatic osteonecrosis of the femoral head that was contralateral to a hip with symptomatic osteonecrosis were identified with magnetic resonance imaging between 1985 and 1995. The lesions were graded with use of the Steinberg classification system. The patients were followed with annual plain radiographs. The mean duration of follow-up was fourteen years. RESULTS: At the time of the initial evaluation, fifty-six hips were classified as Steinberg stage 0, forty-two hips were classified as Steinberg stage I, and twenty-three hips were classified as Steinberg stage II. At the time of the most recent follow-up, pain had developed in 110 previously asymptomatic hips (91%) and collapse had occurred in ninety-three hips (77%). Symptoms always preceded collapse. Of the fifty-six hips that were classified as Steinberg stage 0 at the time of the initial evaluation, forty-seven (84%) had symptomatic osteonecrosis and thirty-four (61%) had collapse at the time of the most recent follow-up. Of the forty-two asymptomatic stage-I hips, forty (95%) became symptomatic within three years and thirty-six (86%) had collapse of the femoral head. Of the twenty-three asymptomatic stage-II hips, all became symptomatic within two years and all collapsed; the mean interval between the onset of pain and collapse was eleven months. At the time of the final follow-up, ninety-one hips (75%) had intractable pain and required surgery. CONCLUSIONS: Untreated asymptomatic osteonecrosis of the femoral head in patients with sickle cell disease has a high likelihood of progression to pain and collapse. Because of the high prevalence of complications after total hip arthroplasty in patients with this disease, consideration should be given to early surgical intervention with other procedures in an attempt to retard progression of the disease.     

Sickle cell nephropathy: an update on pathophysiology,diagnosis, and treatment

Sickle cell nephropathy is a major complication of sickle cell disease. It manifests in different forms, including glomerulopathy, proteinuria, hematuria, and tubular defects, and frequently results in end-stage renal disease (ESRD). Different pathophysiologic mechanisms have been proposed to explain the development of nephropathy in SCD, where hemolysis and vascular occlusion are the main contributors in the manifestations of this disease. Markers of renal injury, such as proteinuria and tubular dysfunction, have been associated with outcomes among patients with sickle cell nephropathy and provide means for early detection of nephropathy and screening prior to progression to renal failure. In small-sized clinical trials, hydroxyurea has demonstrated to be effective in slowing the progression to ESRD. Dialysis and renal transplantation represent the last resort for patients with sickle cell nephropathy. Nevertheless, despite the availability of diagnostic and therapeutic strategies, sickle cell nephropathy remains a challenging and under-recognized complication for patients with sickle cell disease.     

Laparoscopic splenic procedures in children: experience in 231 children

OBJECTIVES: The purpose of this report is to evaluate the efficacy of and complications observed after laparoscopic splenic procedures in children. METHODS: Review of a prospective database at a single institution (1995-2006) identified 231 children (129 boys; 102 girls; average age 7.69 years) undergoing laparoscopic splenic procedures. RESULTS: Two hundred twenty-three children underwent laparoscopic splenectomy (211 total; 12 partial) by the lateral approach. Indication for splenectomy was hereditary spherocytosis (111), immune thrombocytopenic purpura (36), sickle cell disease (SCD) (51), and other (25). Four (2%) required conversion to an open procedure. Eight additional laparoscopic splenic procedures were performed: splenic cystectomy for epithelial (4) or traumatic (2) cyst, and splenopexy for wandering spleen (2). Average length of stay was 1.5 days. Complications (11% overall, 22% in SCD patients) included ileus (5), bleeding (4), acute chest syndrome (5), pneumonia (2), portal vein thrombosis (1), priapism (1), hemolytic uremic syndrome (1), diaphragm perforation (2), colonic injury (1), missed accessory spleen (1), trocar site hernia (1), subsequent total splenectomy after an initial partial (1), and recurrent cyst (1). Subsequent operations were open in 3 (colon repair, hernia, and missed accessory spleen) and laparoscopic in 2 (completion splenectomy, and cyst excision). There were no deaths, wound infections, or instances of pancreatitis. CONCLUSIONS: Laparoscopic splenic procedures are safe and effective in children and are associated with low morbidity, higher complication rate in SCD, low conversion rate, zero mortality, and short length of stay. Laparoscopic splenectomy has become the procedure of choice for most children requiring a splenic procedure.     

Perioperative management in children with sickle cell disease undergoing laparoscopic surgery.

OBJECTIVE: The aim of this study was to evaluate our experience with laparoscopic surgery in children with sickle cell disease. METHODS: A retrospective chart review was performed to analyze the indication for surgery, perioperative management, surgical technique, complications, duration of hospitalization, and outcome. One pediatric surgeon performed all procedures. RESULTS: Thirteen children underwent laparoscopic surgery for the following indications: symptomatic cholelithiasis/cholecystitis in 9; recurrent splenic sequestration in 3; and hypersplenism/symptomatic cholelithiasis in 1. The 7 boys and 6 girls had a median age of 7.8 years. Patients undergoing splenectomy only were younger than those undergoing cholecystectomy (median age, 3.6 years versus 11.5 years, respectively). Four children underwent endoscopic retrograde cholangiopancreatography (ERCP) and sphincterotomy because of common bile duct dilatation and stones. Twelve patients received packed red blood cell transfusions prior to surgery. The median operative time was 150 minutes, and the median hospitalization was 3 days. Four patients suffered postoperative complications (2 with acute chest syndrome, 1 with recurrent abdominal pain, and 1 with priapism). The patient with abdominal pain was found to have a retained stone in the common bile duct, which was retrieved via endoscopic retrograde cholangiopancreatography and sphincterotomy. All complications resolved with medical management. CONCLUSIONS: Laparoscopic surgery is safe in children with sickle cell disease. Meticulous attention to perioperative management, transfusion guidelines, and pulmonary care may decrease the incidence of acute chest syndrome.     

Laparoscopic versus open splenectomy in children

BACKGROUND: The authors have reviewed their initial experience with laparoscopic splenectomy (LS) to identify the indications, success rate, and complications associated with this procedure compared with a series of children undergoing open splenectomy (OS) during the same time period. METHODS: The records of 51 children who underwent splenectomy from 1993 through 1998 were reviewed retrospectively. RESULTS: Thirty-five patients aged 1 to 17 years (mean, 9.4 years) underwent LS for the following indications: ITP (n = 20), sickle cell disease or thalassemia (n = 6), hereditary spherocytosis (n = 5), other hematologic disorders (n = 4). Seventeen patients aged 2 to 17 years (mean, 11.8 years) underwent OS during the same time period for ITP (n = 4), sickle cell disease or thalassemia (n = 4), hereditary spherocytosis (n = 5), and other indications (n = 4). Concomitant cholecystectomy was performed in 4 of 35 LS and 4 of 17 OS. Accessory spleens were identified in 10 of 35 LS and 2 of 17 OS cases. Eleven spleens were enlarged in the LS group, and 8 were enlarged in the OS group. One LS required conversion to an open procedure because the spleen did not fit in the bag. No other cases were converted. Median estimated blood loss was 50 mL for both the LS and OS groups. The only intraoperative complication in the LS group was a splenic capsular tear, which had no effect on the successful laparoscopic removal of the spleen. No patient in either group required a blood transfusion. The LS patients had a shorter length of hospital stay (1.8 +/- 1 versus 4.0 +/- 1 day, P = .0001). Total hospital charges were not significantly different. Follow-up ranged from 6 to 40 months. One LS patient died 47 days postoperatively from unrelated causes. Two LS patients had recurrent ITP; accessory spleens were found in one and resected laparoscopically. CONCLUSION: LS in children can be performed safely with a low conversion rate (2.9%) and is associated with a shorter hospital stay and comparable total hospital cost when compared with OS.     

Laparoscopic cholecystectomy in sickle cell disease

STUDY AIM: Sickle cell affection is a public health problem in Africa. The aims of this prospective study were to evaluate the early results of laparoscopic cholecystectomy in sickle cell patients in Senegal. METHOD: From January 1998 to June 2002 all the sickle cell patients undergoing a laparoscopic cholecystectomy were included. Intra- and post-operative protocol (blood transfusion if Hb < 9 g/dl, rehydration, oxygenotherapy) was standardized. RESULTS: Forty-two patients with sickle cell of types SS-33 and AS9 were operated upon by same surgeon. One case of conversion due to an effraction of biliary junction was reported. One homozygote patient died post-operatively because of peritonitis. Two acute thoracic syndromes, three vaso-occlusive crisis, and two cases of wound infection constituted the post-operative morbidity. No case of complication was noted in those who underwent pre-operative transfusion. CONCLUSION: Laparoscopic cholecystectomy can be carried out in sickle cell patients with biliary lithiasis provided that general anaesthetic rules are respected.     

Tramadol infusion for the pain management in sickle cell disease: a case report

We present the analgesic management of a 4-year-old child who suffered from severe abdominal and leg pain during his first vaso-occlusive crisis with sickle cell disease, diagnosed as beta/S disease when he was 1 year old. His mother and father were carriers of beta-thalassemia and hemoglobin S, respectively. He had an upper respiratory tract infection in which a vaso-occlusive crisis was precipitated. On admission to hospital, fever, severe abdominal and leg pain were noted. Hemoglobin was 4 g x dl(-1) with accompanying prominent reticulocytosis and acute spleen enlargement. These findings indicated a sequestration crisis as well as vaso-occlusive disease. He was transfused with packed red cells. Paracetamol (40-60 mg x kg(-1) x day(-1)) and ibuprofen (20 mg x kg(-1) x day(-1)) were administered to relieve pain. The child experienced moderate to severe pain (Oucher score 60-80) despite nonopioid analgesics, so a tramadol infusion (0.25 mg x kg(-1) x h(-1)) was started. During the tramadol infusion no morphine was required, the intensity of pain gradually decreased (Oucher score 20) and the child was able to move his legs. At the end of 3 days splenomegaly regressed, no fever and pain were observed and the infusion was stopped. In conclusion, tramadol infusion i.v. (0.25 mg x kg(-1) x h(-1)) combined with nonopioids was effective to relieve moderate to severe pain due to vaso-occlusive crisis and can be recommended before using morphine in a pediatric sickle cell crisis.     

The early diagnosis of splenic abscess

Five cases of splenic abscess seen between 1970 and 1984 are reviewed. The predisposing factors included preceding pyogenic infection, sickle cell disease, and contiguous disease in the pancreas. Abdominal pain and fever were the most frequent presenting symptoms. The most common physical finding was left upper quadrant (LUQ) abdominal tenderness. All patients were treated with splenectomy. In one patient percutaneous drainage was attempted prior to splenectomy but failed. The mortality rate was 20 per cent. Radiologic procedures developed in the last ten years make possible the early diagnosis and treatment of splenic abscess. The treatment of choice remains antibiotics followed by splenectomy.     

Long-term follow-up of partial splenectomy in Gaucher's disease.

Seven children with Gaucher's disease who underwent partial splenectomy were followed for 7 to 8 years. None of the children had systemic sepsis or symptoms related to liver enlargement. Bone crisis occurred in only two children who had experienced bone crisis prior to partial splenectomy. This contrasts with the development of bone crises in five of six children who underwent total splenectomy at a similar age and who had previously been free of bone symptoms. Partial splenectomy should be regarded as a temporary solution in the treatment of hypersplenism and the mechanical compression related to the huge spleen. In 71% of the patients, massive enlargement and a severe to moderate degree of pancytopenia occurred again after 3 to 8 years; total splenectomy was required in three patients.     

Splenectomy versus spleen-preserving surgery for splenic echinococcosis

BACKGROUND/AIMS: The spleen is the third most common location of hydatid disease after liver and lung. The aim of this study was to analyse the long-term outcome of surgical treatment of patients with splenic echinococcosis comparing splenectomy with spleen-preserving surgery. METHODS: During a period of 25 years (1976-2001), 19 (5.4%) patients with splenic echinococcosis were treated in our department out of 349 patients with abdominal hydatid disease. In 16 patients the spleen was the only organ involved, while in 3 patients the liver was also affected. RESULTS: Eleven patients had splenectomy and in the other 8 the spleen was preserved: enucleation (n = 4), partial cystectomy and omentoplasty (n = 2) and cystojejunal Roux-en-Y anastomosis (n = 2). One (6%) patient died in the early postoperative period and 5 (29%) patients had postoperative complications. There was no significant difference between the splenectomy and spleen-preserving groups concerning median hospital stay and postoperative complication rate. The median follow-up in 15 patients was 52 (range 6-300) months. Two patients (13%) developed recurrence of the disease requiring re-operation at 2 and 3 years, respectively. Recurrence occurred in 1 (12%) patient in the splenectomy group and in 1 (14%) out of 7 patients in the spleen-preserving group. CONCLUSION: In the present series it was possible to preserve the spleen in 8 (42%) of 19 patients, without significant increase of recurrent echinococcosis.     

Pediatric laparoscopic splenectomy: benefits of the anterior approach

Introduction: Splenectomy is frequently performed in children for various hematologic and autoimmune disorders. We reviewed our indication for splenectomy, our technique of laparoscopic, splenectomy, and our results. The aim of this study was to show if an anterior approach with elective vessel endoligature in a patient in decubitus position was a better and safer approach than the other techniques previously published. Patients and methods: Between January 1996 and December 2002, 54 children underwent laparoscopic splenectomy. We use the anterior pedicle approach and elective vessel endoligature in order to prevent persistence of any exogenous material. Sixteen children also had a concomitant cholecystectomy. Their age ranged between 1 and 18 years (mean 7.5 years). Twenty children had hereditary spherocytosis, 15 were affected by sickle cell disease, six had an idiopathic thrombocytopenia, four had beta-thalassemia, and nine another hemolytic disease. Results: Mean operative time was 150 min (range, 115–230 min). Hospital stays ranged from 3 to 15 days (mean: 5.4). Seven patients had complications (four pneumonia and three deep abscess with successful antibiotic treatment). Median follow-up was 42 months (2 months–6 years) without problems regarding procedure. Discussion: With this technique consisting in first elective arterial and secondary venal ligature, we preserved blood splenic sequestration and pancreatic tail lesion. The procedure is safer. For us, considering the low complication rate for this type of patient, and the advantages of a small abdominal trauma in the postoperative period, laparoscopy with anterior approach for elective splenectomy and eventual cholecystectomy in hematologic disorders is the best technique.     

Physical therapy alone compared with core decompression and physical therapy for femoral head osteonecrosis in sickle cell disease. Results of a multicenter study at a mean of three years after treatment

BACKGROUND: Osteonecrosis of the femoral head is a common complication in patients with sickle cell disease, and collapse of the femoral head occurs in 90% of patients within five years after the diagnosis of the osteonecrosis. However, the efficacy of hip core decompression to prevent the progression of osteonecrosis in these patients is still controversial. METHODS: In a prospective multicenter study, we evaluated the safety of hip core decompression and compared the results of decompression and physical therapy with those of physical therapy alone for the treatment of osteonecrosis of the femoral head in patients with sickle cell disease. Forty-six patients (forty-six hips) with sickle cell disease and Steinberg Stage-I, II, or III osteonecrosis of the femoral head were randomized to one of two treatment arms: (1) hip core decompression followed by a physical therapy program or (2) a physical therapy program alone. Eight patients withdrew from the study, leaving thirty-eight who participated. RESULTS: Seventeen patients (seventeen hips) underwent decompression combined with physical therapy, and no intraoperative or immediate postoperative complications occurred. Twenty-one patients (twenty-one hips) were treated with physical therapy alone. After a mean of three years, the hip survival rate was 82% in the group treated with decompression and physical therapy and 86% in the group treated with physical therapy alone. According to a modification of the Harris hip score, the mean clinical improvement was 18.1 points for the patients treated with hip core decompression and physical therapy compared with 15.7 points for those treated with physical therapy alone. With the numbers studied, the differences were not significant. CONCLUSIONS: In this randomized prospective study, physical therapy alone appeared to be as effective as hip core decompression followed by physical therapy in improving hip function and postponing the need for additional surgical intervention at a mean of three years after treatment.