Outcomes of Splenectomy in Children with Sickle Cell Disease

Background

Children with splenic sequestration, related to sickle cell disease, are managed acutely with blood transfusions followed by an elective splenectomy to prevent recurrent episodes. The objective of this study was to review the outcomes of splenectomy in children with sickle cell disease as a single center experience.

Methods

A retrospective chart review of children with sickle cell disease who had splenectomy between 1999 and 2014 at the University of South Alabama was performed. Data on demographics, sequestration episodes, post-operative complications, bacteremia, transcranial doppler ultrasonography and death were collected.

Results

A total of 52 patients (36 with Hemoglobin SS, 7 with Hemoglobin SC, 5 with S-Beta Thalassemia Plus, and 4 with S-Beta Thalassemia Zero) received splenectomy during the study period. Mean age at first splenic sequestration event was 39 months. The mean age of splenectomy was 5 years (Minimum: 18 months; Maximum: 18 years). There were 24 males and 28 females. Over 95 percent of patients were on penicillin prophylaxis. In only 73 percent of patients, proof of completed vaccination including pneumococcal polysaccharide, pneumococcal conjugate and meningococcal conjugate vaccines, could be found. Average post-splenectomy follow-up was 7.4 years.

The post-operative complications included fever in 4 patients, acute chest syndrome in 4 patients, lobar pneumonia in 2 patients, pleural effusion in 1 patient, atelectasis on chest radiograph in 2 patients and surgical wound abscess in 1 patient. One patient had an intra-abdominal bleed which required reoperation. The average number of hospitalizations for vaso-occlusive pain crises was 3.3 per year prior to splenectomy and 2.2 per year during the 2 years following splenectomy (p=0.04). Mean platelet count before splenectomy was 267/m³ compared to 533/m³ at 1 year after splenectomy (p<0.05). Differences in mean white blood cell and reticulocyte counts before and after splenectomy were not statistically significant. Only 2 of the patients had culture proven bacteremia, but both of them occurred prior to their splenectomy. One of the patients grew Staphylococcus hominis and the other grew Staphylococcus lugdunensis. No true bacteremia were reported in patients after splenectomy. None of the patients developed stroke while four (~8%) patients developed critical transcranial doppler ultrasonography velocities (≥200 cm/s) and were started on chronic blood transfusions. The mean time-average maximum velocity before splenectomy was 127 cm/sec and increased to 151 cm/sec at 2 years after splenectomy (p=0.002). Among the splenectomized patients, 18 (35%) of them had been started on Hydroxyurea.

Discussion

Our results indicate that with proper vaccination and penicillin prophylaxis, the risk of infection after splenectomy can be controlled. The mean hemoglobin levels did not change after splenectomy but our patients had fewer hospitalizations for pain crises after splenectomy. The cerebral blood flow velocity increased after splenectomy. This might imply that more patients will require chronic blood transfusions for stroke prevention after splenectomy. We conclude that splenectomy is a safe and effective modality for management of life threatening splenic sequestrations in children with sickle cell disease.