Surgery in children with homozygous sickle cell anaemia

Area of Science:

• Pediatric Surgery
• Hematology
• Sickle Cell Disease Management

Background:

• Sickle cell anaemia (SCA) presents unique challenges in pediatric surgical patients.
• SCA complications can mimic acute surgical conditions, complicating diagnosis and management.

Purpose of the Study:

• To review surgical procedures performed on children with SCA.
• To discuss preparation strategies, including transfusions and splenectomy, for SCA patients undergoing surgery.
• To provide recommendations for surgical preparation in acute and routine cases.

Main Methods:

• Retrospective analysis of 31 surgical procedures in children (up to 16 years) with SCA from 1952-1977.
• Categorization of surgeries into emergency, minor, and major procedures.
• Review of preparation protocols, including pre-operative transfusions and indications for splenectomy.

Main Results:

• Six emergency operations were performed for SCA complications.
• Seventeen minor operations were well-tolerated.
• Eight major surgeries included five splenectomies for hypersplenism and transfusion needs.

Conclusions:

• Careful pre-operative preparation, including planned transfusions, is crucial for children with SCA undergoing surgery.
• Splenectomy is indicated for hypersplenism and high transfusion requirements in SCA patients.
• Guidelines for preparing SCA patients for both emergency and elective surgery are essential.