CASE OF THE WEEK – “Surgical management of splenomegaly secondary to sickle cell thalassemia” by Dr. Ali Al Ghrebawi, HOD & Consultant, General Surgery, NMC Royal Hospital Sharjah

CASE OF THE WEEK – “Surgical management of splenomegaly secondary to sickle cell thalassemia” by Dr. Ali Al Ghrebawi, HOD & Consultant, General Surgery, NMC Royal Hospital Sharjah

Surgical management of splenomegaly secondary to sickle cell thalassemia

Case:

A 16 year old female with sickle cell thalassemia with elevated HbF complicated by leucocytosis, thrombocytopenia, palpitations, splenomegaly and chronic calcular cholecystitis. She had a history of multiple blood transfusions with subsequent development of allo antibodies which made further transfusions difficult. Given her history, the decision to proceed with surgery was made by Dr. Ali Al Ghrebawi in liaison with Dr. Abdul Rahman Agha El Kinge.

Pre-operative optimisation:

She received the first doses of meningococcal and pneumococcal vaccines 2 weeks prior to surgery and was admitted on 5/6/2021 with Hb 5.3 g/dl, HCT 18.2%, Platelets 101,000/L and a Reticulocytes 25.39%. Ultrasound evaluation of the abdomen showed mild hepatomegaly, distended gallbladder with a 1.0cm calculus and an enlarged spleen that measures 22 cm in size. Pre-operative optimisation included the transfusion of 4 units PRBCs, VTE prophylaxis and symptomatic management. On 8/6/2021, Hb 11.2 g/dl, HCT 35.3% and Platelets 124,000.

Operation:

The patient underwent laparoscopic cholecystectomy + splenectomy on 9/6/2021 with a total blood loss of only 20ml. The resected spleen weighed 1500 grams (normal 140 grams in females).

Post-operative Care/Course:

The first night post-op was spent in the ICU followed by a transfer to the surgical ward on Day 1.

The patient was started on I/V antibiotics along with VTE prophylaxis and symptomatic management.

On Day 2 post op, the abdominal drain was removed. Hb – 10.8 g/dl and Platelets 210,000 and the patient was discharged on the 4th post operative day.

On 23/6/2021 the patient was seen in the OPD reportedly feeling comfortable and pain free. She received the second planned doses of meningococcal and pneumococcal vaccines and surgical wounds were dry and healing well.

CASE OF THE WEEK – “Surgical management of splenomegaly secondary to sickle cell thalassemia” by Dr. Ali Al Ghrebawi, HOD & Consultant, General Surgery, NMC Royal Hospital Sharjah