ANESTHETIC MANAGEMENT IN BETA THALASSEMIA, DILATED HEART DISEASE, AND

HYPERSPLENISM PATIENT UNDERWENT SPLENECTOMY

Erlangga Prasamya*,Djayanti Sari and Yunita Widyastuti**
*Resident; **Consultant
Department of Anesthesiology and Intensive Therapy
Medical Faculty of Universitas Gadjah Mada / Dr. Sardjito Hospital Yogyakarta

Background: Survival of thalassemia patients have dramatically increased recent years along
with advancement in medical treatment. Thalassemic problems such as anemia, splenomegaly,
difficult airway management due to extramedullary erythropoiesis, iron deposition in multiple
organ, and nefrotoxicity due to chelating agent are often encountered by anesthesiologist.
Therefore anaesthesiologist should be able to identify those problems and take proper measures
to provide safe anethetic management.

Objective:
To identify and manage thalassemia problems related to anesthesia

Case: A 15-yo. male patient planned for splenectomy. Patient was well treated by pediatric
hematologist and cardiologist. Vital signs within normal range. Patient has cardiac enlargement
with murmurs, and schuffner VII spleen enlargement, but no difficult airway problem.
Echocardiography revealed dilatation in all chamber, mild pulmonary valve insufficiency, trivial
mitral insufficency, good LV contractility with 65% ejection fraction. Patient was assessed with
ASA physical status III. Anesthesia conduct under general endotracheal intubation with invasive
monitor (CVP & IABP). During the procedure, patient had stable hemodynamic parameters and
controlled bleeding. Patient was admitted to ICU for 24 hours.

Discussion: Multidicipline approach in thalassemia is the key of succesful preoperative care as

multiple organ systems may be affected by three main pathology: thalassemia itself, iron
overload and chelating therapy. Patients may develop cardiomyopathy, pulmonary hypertension,
restrcitive lung dysfunction due to splenomegaly, heart failure and renal toxicity. When general
anaesthesia is administered, the risk of difficult intubation due to orofacial malformation should
be evaluate carefully. Cardiovascular depression due to negative inotropic and vasodilating
effects of general anaesthesia should be minimised.
Conclusion: Problems in thalassemia should treated optimally before proceed to surgery.
Multidiscipline approach is needed.
Keyword: Pediatric, Beta Mayor Thalassemia, Anesthesia Management, Splenecetomy