Splenic complications of the sickling syndromes and the role of splenectomy.

PURPOSE: To analyze the authors' experience with splenectomy for sickling disorders and evaluate the indications, complications, and outcome. PATIENTS AND METHODS: Over a period of 10 years (1987-1997), 113 patients with sickling disorders (100 with sickle cell disease and 13 with sickle-beta-thalassemia) had splenectomy at the authors' hospital as part of their management. The indications for splenectomy were hypersplenism (26 patients), major splenic sequestration crisis (MSSC) (23 patients), minor recurrent splenic sequestration crisis (MRSSC) (50 patients), splenic abscess (12 patients), and massive splenic infarction (2 patients). RESULTS: Splenectomy in patients with sickle cell disease (SCD) and sickle-beta-thalassemia (S-beta-Thal) was beneficial in reducing their transfusion requirements and its attendant risks, eliminating the discomfort from mechanical pressure of the enlarged spleen, and avoiding the risks of acute splenic sequestration crisis. It also was curative for patients with splenic abscess and massive splenic infarction. Twenty-four patients with SCD (24%) had splenectomy and cholecystectomy caused by concomitant gallstones. There was no mortality, and the postoperative morbidity was 7%. CONCLUSIONS: With careful perioperative management, splenectomy is both safe and beneficial in a select group of patients with SCD and S-beta-Thal.

Splenectomy for hematological diseases: The Qatif Central Hospital experience.

BACKGROUND: In the Eastern Province of Saudi Arabia, an area known for various hemoglobinopathies, splenectomy is performed rather frequently. This study is an analysis of our experience with splenectomy performed for various hematological disorders between 1988 and 1997, outlining the indications, complications and outcome. PATIENTS AND METHODS: This is a retrospective analysis of all patients who had splenectomy at our hospital during this period. One hundred and forty-three patients were treated for various hematological disorders at our hospital. These disorders included sickle cell disease (SCD) (100 patients), sickle ss-thalassemia (S-ss-thal) (13 patients), ss-thalassemia major (15 patients), Hb H disease (3 patients), idiopathic thrombocytopenic purpura (ITP) (5 patients), Gaucherâs disease (2 patients), hereditary spherocytosis (1 patient), autoimmune hemolytic anemia (1 patient), thalassemia intermediate (2 patients) and chronic myeloid leukemia (1 patient). RESULTS: The indications for splenectomy in those with SCD and S-ss-thal were: hypersplenism (26 patients), major splenic sequestration crisis (23 patients), minor recurrent splenic sequestration crisis (50 patients), splenic abscess (12 patients), and massive splenic infarction (2 patients). Splenectomy in these patients was beneficial in reducing their transfusion requirements and its attendant risks, eliminating the discomfort from mechanical pressure of the enlarged spleen, avoiding the risks of acute splenic sequestration crisis, and managing splenic abscess. For those with thalassemia, total splenectomy was beneficial in reducing their transfusion requirements, while partial splenectomy was beneficial only as a temporary measure, as regrowth of splenic remnant in these patients subsequently led to increase in their transfusion requirements. Those with ITP, hereditary spherocytosis, and autoimmune hemolytic anemia showed excellent response following splenectomy. There was no mortality, and the postoperative morbidity was 5.6%. CONCLUSION: With careful perioperative management, splenectomy is both safe and beneficial in a selected group of patients with hematological diseases.

Splenic abscess and sickle cell disease.

This is a report of our experience with 10 cases of splenic abscess in patients with sickle cell disease (SCD). All presented with fever and abdominal pain and were found to have a tender enlarged spleen. Two were found to have a ruptured spleen and five of them were septicemic on presentation. Although both ultrasound and CT-scan of the abdomen were of diagnostic value, we found CT-scan more accurate and reliable in the diagnosis of splenic abscess. Ultrasound and/or CT-scan should be used routinely in the evaluation of SCD patients who present with fever and abdominal pain, especially if they have a tender enlarged spleen. Diagnostic aspiration under CT-scan or ultrasound guidance should be used in doubtful cases to differentiate between splenic abscess and a large splenic infarct. All our patients were managed by peri operative antibiotics and splenectomy with no mortality. Salmonella was the commonest causative organism. Although CT-guided aspiration of splenic abscess is being advocated recently, we feel splenectomy should be the treatment of choice in patients with SCD as there is no point in preserving a non-functioning spleen that is present in the majority of patients. CT-guided aspiration may be employed as a temporary measure for those patients who are at high surgical risk with unilocular abscess.

Elective, postoperative ventilation in the management of esophageal atresia and tracheoesophageal fistula.

The management of esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) has undergone many changes. As a result of recent advances in neonatal intensive care and pediatric anesthesia, the survival of infants with EA and TEF has improved markedly, but the occurrence of anastomotic complications has remained constant. To overcome this problem, various techniques and suture materials have been used. This review of 20 consecutive cases of EA/TEF stresses the importance and influence of non-reversal of anesthesia, paralysis, and elective ventilation for protection of the esophageal anastomosis following repair of EA and TEF.

Laparoscopic versus open cholecystectomy in children.

Twenty-one consecutive laparoscopic cholecystectomies (LC) were compared with 29 consecutive open cholecystectomies (OC). Sickle-cell disease (SCD) was the most common reason for cholecystectomy in both groups. The average length of operative time for LC was significantly longer than that of OC (P=0.0149). In 1 patient there was conversion from LC to OC due to severe adhesions. Common bile duct (CBD) stones were diagnosed in 8 (27.6%) of the OC group; in 4 of them the diagnosis was made preoperatively by ultrasound, in 4 by intraoperative cholangiogram. All 8 patients required CBD exploration, and 2 had additional transduodenal sphincteroplasties. In the LC group 5 patients (23.8%) had CBD stones. All had (ERCP) endoscopic retrograde cholangiopancreatography sphincterotomy, and stone extraction followed by LC. ERCP is a necessary adjunct to treatment if LC is to be contemplated. Six patients in the OC group developed complications, while only 4 patients in the LC group developed minor complications. The length of hospitalization after LC was significantly shorter than after OC (P=0.0150). LC is the procedure of choice in the management of cholelithiasis in children, especially those with SCD.

Splenectomy in patients with sickle-cell disease.

BACKGROUND: Splenic complications of sickle-cell disease (SCD) are associated with morbidity, and in some it may lead to mortality. This paper presents our experience with 43 patients with SCD who had splenectomy as part of their management. PATIENTS AND METHODS: The records of 43 patients with SCD who had splenectomy were examined for age at operation, sex, hemoglobin (Hb) electrophoresis, indication for splenectomy, pre- and postoperative medications, operative procedures, and postoperative complications. RESULTS: The indications for splenectomy were acute splenic sequestration crisis (ASSC) in 21 patients, hypersplenism in 15, and splenic abscess in 7. In 17 patients, the spleen was also found to be massively enlarged causing discomfort and intervening with everyday activity. For those with hypersplenism, there was a significant postoperative increase in total Hb (P < 0.0001), hematocrit (P < 0.0001), white blood cells (P < 0.0001), and platelet count (P < 0.0001). CONCLUSIONS: With careful perioperative management and proper follow-up, splenectomy in patients with SCD is beneficial in reducing their transfusion requirements and its attendant risks, eliminating the discomfort from mechanical pressure of the enlarged spleen, avoiding the risks of ASSC, and managing splenic abscess.

Perianal abscess and fistula in ano in infancy and childhood: a clinicopathological study.

This is a retrospective study of 78 children with perianal abscess and/or fistula in ano presenting during a 6 1/2-year period. Sixty-five were males and 13 females. Their ages at presentation ranged from 22 days to 18 years (median 1.7 year), and the majority of males were below 2 years of age. The 13 females all presented with perianal abscess, the majority of which grew Staphylococcus aureus (69.2%). On follow-up, none of them developed fistula in ano. Twenty-two of the 65 males (33.8%) presented initially with fistula in ano. The remaining 43 presented with perianal abscess. Four of them were found to have fistula in ano at the time of incision and drainage and on follow-up, and 14 others developed fistula in ano. Of the 40 cases of fistula in ano, all were males; 25 were on the right side and 9 on the left side, 5 had bilateral fistula in ano, and 1 had two fistulas on the left side at 3 and 5 o'clock positions. Gut-derived organisms were isolated from 88.4% of the males with perianal abscess. There appears to be a causal relationship between perianal abscess and fistula in ano.

Splenectomy and acute splenic sequestration crises in sickle cell disease.

Acute splenic sequestration crises (ASSC) is one of the complications of sickle cell disease (SCD) that can be life-threatening due to loss of blood volume. Over a 5-year period, we have treated 19 patients ranging in age from 4 to 32 years with ASSC. There were 14 males and 5 females; 17 had homozygous SCD and the other 2 had sickle thalassemia. Two patients presented with severe anemia and acute circulatory collapse; 1 of them developed residual weakness of his limbs and decreased visual acuity. Nine patients underwent splenectomy after major episodes of sequestration while the remaining 10 had recurrent minor episodes of sequestration. The clinical features and the role of splenectomy are discussed.

Cholelithiasis in children with sickle cell disease.

Abdominal ultrasonography was performed on 305 children with sickle cell disease (SCD) (285 SS and 20 S-beta-thalassemia) to establish the prevalence of cholelithiasis in Saudi children with SCD. Their ages ranged from 1 to 18 years (mean 10.45 years). Gallstones were demonstrated in 60 children, giving a prevalence of 19.7%. An additional 50 patients (16.4%) had only biliary sludge. The youngest patient with gallstones was 3 years old. There was a correlation between the presence of gallstones and increasing age. Patients with gallstones were also found to have higher serum bilirubin levels, but their hemoglobin, hematocrit, reticulocyte count, hemoglobin S, and hemoglobin F levels were not significantly different from those of patients without gallstones.

Osteomyelitis and septic arthritis in sickle cell disease in the eastern province of Saudi Arabia.

Patients with sickle cell disease are more susceptible to osteomyelitis and septic arthritis than the population at large. Seventy eight patients with these conditions were admitted to our hospital from April 1988 to March 1991. Thirty had sickle cell disease, 14 had the sickle cell trait and 34 had normal electrophoresis. The tibia, followed by the femur and humerus were the bones most commonly affected, and the knee was the joint most often involved. Salmonella was the commonest organism in osteomyelitis and septic arthritis in sickle cell disease, whereas staphylococcus was commonest in normal patients and those with the sickle cell trait. Antibiotics that cover these two organisms must be considered in patients with sickle cell disease who are suspected of having osteomyelitis or septic arthritis.