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Forster, González E.1; Morros, Faura A.1; Cazalilla, Moron G.1; Meda, Mesegué M.1; Llobet, Ruiz A.1

doi: 10.1097/01.HS9.0000567692.48535.db
Publication Only: Sickle cell disease

1Pediatric Hematology, Hospital Sant Joan de Déu, Barcelona, Spain

Corresponding author.

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Although children with sickle cell disease (SCD) often undergo surgery, there are limited current epidemiological data for this pediatric population. Sickle cell patients present a high risk of postoperative complications and have a higher morbidity and mortality than the general population after surgery. During the perioperative period there are certain situations that can trigger an increase in falciformation and vaso-occlusive crises that lead to complications mainly of respiratory type. Our protocol of perioperative general care includes: transfusion of packed red blood cells to obtain a hemoglobin value of 10 g/dL, hyperhydration, avoiding hypoxia and cold, strict control of pain, early ambulation and incentivized spirometry.

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To study the prevalence and severity of postoperative complications in pediatric patients with SCD (HbSS/Sβ0 o SC) followed in our center in a period of 22 years

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We reviewed the medical records of patients with SCD who underwent surgery between January 1997 and January 2019. We described characteristics of patients, the type of surgery that was performed and the postoperative complications they presented.

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From 1997 to 2019 out of 91 pediatric patients followed in our center with SCD, 38 patients underwent surgery (41.8%). A total of sixty-one interventions were performed in these patients (1.6 surgeries per patient). The median age at the moment of surgery was 7.5 years. The most frequent procedure was otorhinolaryngological surgery (21.3%), followed by trauma surgery (18%), splenectomy (14.7%), herniorrhaphy (11.4%), cholecystectomy (8.2%), circumcision (6.6%) and others (19.6%). Postoperative complications were observed in 7 out of the 61 surgical interventions: 4 acute thoracic syndromes, 2 bronchospasm crises and 1 vaso-occlusive crisis. In 4 of the 7 cases, complications occurred after abdominal surgeries. Most complications occurred within 24-48 hours after the surgery (1 day median with a range of 0 to 6 days). In 4/7 of the perioperative complications patients were previously receiving hydroxyurea, 3/7 were not receiving hydroxyurea at the moment of the surgery; two because of medical indications and one patient arrived from country of origin where the medication was not administered. Three patients required admission to the intensive care unit (ICU). Two patients required exchange transfusion and respiratory support. There were no strokes and no deaths.



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We observed a high rate of surgical procedures in our patients. The burden of surgical disease in SCD children differs from the general pediatric population. In our series the most frequent indication for surgery was tonsillectomy and adenoidectomy. Despite the perioperative care described, we observed a prevalence of postsurgical complications of 11.5%. The majority of the complications were of respiratory type coinciding with the existing literature, practically half of them required admission to the ICU and a 28.5% underwent exchange transfusion and required respiratory support after the event. The incidence of death and stroke is nule in our series. There are few adequate trials to determine optimal perioperative management in these patients. Future research should determine how to predict and decrease perioperative complications.

Copyright © 2019 The Authors. Published by Wolters Kluwer Health Inc., on behalf of the European Hematology Association.