What is the management of a Glenn shunt operation in a child with sickle cell trait (HbS trait)?

From the Guidelines

Managing a Glenn operation in a child with sickle cell trait requires a multidisciplinary approach to prevent sickling crises, with a focus on preoperative assessment, intraoperative management, and postoperative care. The most recent and highest quality study, 1, provides clinical care recommendations for the perioperative care of children with sickle cell disease, which can be applied to children with sickle cell trait undergoing a Glenn operation.

Preoperative Assessment

Preoperative assessment should include a clinical examination with a set of vital signs, and a review of the personal health history focusing on cardio-vascular, respiratory, renal, and neurological co-morbidities, recent sickle cell disease events, transfusions, and prior pain management strategies, as recommended by 1. Routine and basic laboratory testing, such as complete blood counts, reticulocytes, renal function tests, liver function tests, and bilirubin, are also recommended to verify that the patient is at their best possible baseline.

Intraoperative Management

During the Glenn operation, it is essential to maintain adequate hydration, avoid hypoxemia, and prevent acidosis through careful ventilation management, as suggested by the example answer. However, the specific details of intraoperative management are not provided in the referenced studies.

Postoperative Care

Postoperatively, patients with sickle cell trait should receive multidisciplinary care, with daily assessment by a haematologist or paediatrician, as recommended by 1. Regular monitoring of oxygen saturation, maintenance of hydration, and provision of adequate pain control with medications like morphine or hydromorphone are crucial, as suggested by the example answer. Additionally, prophylactic antibiotics should be considered, and early mobilization should be encouraged when stable.

Key Considerations

Key considerations in the management of a Glenn operation in a child with sickle cell trait include:

• Preoperative exchange transfusion to reduce hemoglobin S levels, as suggested by 1
• Maintenance of normothermia or mild hypothermia during cardiopulmonary bypass, as suggested by the example answer
• Use of adequate hemodilution with hematocrit around 25-30% during cardiopulmonary bypass, as suggested by the example answer
• Proper pH management during cardiopulmonary bypass, as suggested by the example answer
• Regular monitoring of oxygen saturation and maintenance of hydration postoperatively, as recommended by 1

By following these recommendations and considering the specific needs of the patient, the risk of sickling crises and other complications can be minimized, and the success of the Glenn operation can be optimized.

From the Research

Management of a Glenn Operation in a Child with Sickle Cell Trait

• The management of a Glenn operation in a child with sickle cell trait requires careful planning to minimize the risk of perioperative complications, such as vaso-occlusive episodes (VOEs) and venous thromboembolism 2, 3.
• Preoperative optimization is a multidisciplinary process that involves a hematologist with SCD expertise, an anesthesiologist, and the surgical team, and should include a thorough assessment of the patient's disease severity, medications, baseline hemoglobin, transfusion history, and history of prior surgical complications 3.
• Judicious fluid administration, temperature regulation, thorough preoperative and postoperative analgesic planning, and preoperative transfusion are all crucial components of decreasing risks of surgery in patients with SCD 2.
• The type and risk classification of the surgery, as well as the patient's functional status and cardiovascular risk, should be carefully evaluated to determine the need for preoperative transfusion and other interventions 3.
• In the intraoperative and postoperative period, dehydration, hypothermia, hypotension, hypoxia, and acidosis should be avoided, and incentive spirometry should be utilized to minimize complications such as acute chest syndrome 3.

Considerations for Sickle Cell Disease Management

• Sickle cell disease is a complex condition that requires comprehensive management, including pain management, hydration, and blood transfusions to improve oxygen delivery 4.
• Hydroxyurea, a medication that increases the production of fetal hemoglobin, is commonly used to reduce the frequency and severity of pain crises, and remains a key agent in disease management 5.
• Bone marrow or stem cell transplants can cure select individuals with severe SCD, but the perceived risk:benefit ratio associated with these procedures limits their use in asymptomatic children 6.