Laparoscopic splenectomy for hereditary spherocytosis
EAES Academy. Fassari A. 07/05/22; 366523; P240
Dr. Alessia Fassari
Contributions
Contributions
Abstract
Aim: Hereditary spherocytosis (HS) is the most common inherited hemolytic anaemia. The disease is caused by a defect in the erythrocyte cell membrane causing abnormal, spherical shape of red blood cells (so-called microspherocyte). The classical clinical features of haemolysis are anaemia, jaundice, and splenomegaly. Symptoms can develop in infancy, but some people with HS have no symptoms or minor symptoms and are diagnosed later in life. Surgical removal of the spleen is used as a cure for HS in case of severe anaemia and poor QoL as a consequence of recurrent haemolytic crises.
Materials and Methods: A 38-year-old man was admitted for severe anaemia with increased need for red cells transfusions, history of haemolytic crisis, mild jaundice and significant splenomegaly. Blood smear, reticulocytes count, bilirubin concentration, osmotic fragility test and direct Coombs test were studied. The diagnosis was confirmed by a positive flow cytometric analysis of eosin-5-maleimide (EMA)-labelled red blood cells (RBCs). Preoperative ultrasonography showed severe splenomegaly (22 x 15,5 x 9 cm) without cholelithiasis. The patient received immunisation for Haemophilus influenzae, Streptococcus pneumoniae and Meningococcus 2 weeks before surgery.
Splenectomy was performed through a 4-port laparoscopic approach and a Gagner oblique position on the right flank with thorax section of the table. The procedure of splenectomy began with the division of the splenocolic ligament followed by the section of the gastrosplenic ligament including the short gastric vessels. An accessory spleen was found and removed. The splenic hilum was dissected: first, splenic artery was skeletonized, clipped and divided, then splenic vein was carefully dissected from the pancreatic tail.
The splenorenal dissection and the division of lateral attachments to the spleen was furthered cephalad along the entire length of the spleen, with care not to enter the Gerota fascia. The splenophrenic ligament division allowed a complete spleen mobilisation. Although these planes are relatively avascular, the use of an energized device such as the ultrasonic activated shears can minimize tissue oozing. The specimen was removed with a retrieval bag through a minilaparotomy in the left flank connecting the left subcostal 12 mm-trocar incisions.
Finally the operating field was thoroughly rinsed and a drain left in situ.
Results: Postoperative course was uneventful. Bilirubin level decreased in the early postoperative period. Haemoglobin and RBC improved, and the effect was maintained during 4 months of follow-up. Doppler ultrasonography on postoperative day 7 was negative for portal splenic vein thrombosis.
Conclusions: Laparoscopic splenectomy is an effective technique when performed in patients with hereditary spherocytosis even in the case of large or massive splenomegaly. Proper technique and adequate surgical team training and experience are essential to perform the procedure safely.
Materials and Methods: A 38-year-old man was admitted for severe anaemia with increased need for red cells transfusions, history of haemolytic crisis, mild jaundice and significant splenomegaly. Blood smear, reticulocytes count, bilirubin concentration, osmotic fragility test and direct Coombs test were studied. The diagnosis was confirmed by a positive flow cytometric analysis of eosin-5-maleimide (EMA)-labelled red blood cells (RBCs). Preoperative ultrasonography showed severe splenomegaly (22 x 15,5 x 9 cm) without cholelithiasis. The patient received immunisation for Haemophilus influenzae, Streptococcus pneumoniae and Meningococcus 2 weeks before surgery.
Splenectomy was performed through a 4-port laparoscopic approach and a Gagner oblique position on the right flank with thorax section of the table. The procedure of splenectomy began with the division of the splenocolic ligament followed by the section of the gastrosplenic ligament including the short gastric vessels. An accessory spleen was found and removed. The splenic hilum was dissected: first, splenic artery was skeletonized, clipped and divided, then splenic vein was carefully dissected from the pancreatic tail.
The splenorenal dissection and the division of lateral attachments to the spleen was furthered cephalad along the entire length of the spleen, with care not to enter the Gerota fascia. The splenophrenic ligament division allowed a complete spleen mobilisation. Although these planes are relatively avascular, the use of an energized device such as the ultrasonic activated shears can minimize tissue oozing. The specimen was removed with a retrieval bag through a minilaparotomy in the left flank connecting the left subcostal 12 mm-trocar incisions.
Finally the operating field was thoroughly rinsed and a drain left in situ.
Results: Postoperative course was uneventful. Bilirubin level decreased in the early postoperative period. Haemoglobin and RBC improved, and the effect was maintained during 4 months of follow-up. Doppler ultrasonography on postoperative day 7 was negative for portal splenic vein thrombosis.
Conclusions: Laparoscopic splenectomy is an effective technique when performed in patients with hereditary spherocytosis even in the case of large or massive splenomegaly. Proper technique and adequate surgical team training and experience are essential to perform the procedure safely.
Aim: Hereditary spherocytosis (HS) is the most common inherited hemolytic anaemia. The disease is caused by a defect in the erythrocyte cell membrane causing abnormal, spherical shape of red blood cells (so-called microspherocyte). The classical clinical features of haemolysis are anaemia, jaundice, and splenomegaly. Symptoms can develop in infancy, but some people with HS have no symptoms or minor symptoms and are diagnosed later in life. Surgical removal of the spleen is used as a cure for HS in case of severe anaemia and poor QoL as a consequence of recurrent haemolytic crises.
Materials and Methods: A 38-year-old man was admitted for severe anaemia with increased need for red cells transfusions, history of haemolytic crisis, mild jaundice and significant splenomegaly. Blood smear, reticulocytes count, bilirubin concentration, osmotic fragility test and direct Coombs test were studied. The diagnosis was confirmed by a positive flow cytometric analysis of eosin-5-maleimide (EMA)-labelled red blood cells (RBCs). Preoperative ultrasonography showed severe splenomegaly (22 x 15,5 x 9 cm) without cholelithiasis. The patient received immunisation for Haemophilus influenzae, Streptococcus pneumoniae and Meningococcus 2 weeks before surgery.
Splenectomy was performed through a 4-port laparoscopic approach and a Gagner oblique position on the right flank with thorax section of the table. The procedure of splenectomy began with the division of the splenocolic ligament followed by the section of the gastrosplenic ligament including the short gastric vessels. An accessory spleen was found and removed. The splenic hilum was dissected: first, splenic artery was skeletonized, clipped and divided, then splenic vein was carefully dissected from the pancreatic tail.
The splenorenal dissection and the division of lateral attachments to the spleen was furthered cephalad along the entire length of the spleen, with care not to enter the Gerota fascia. The splenophrenic ligament division allowed a complete spleen mobilisation. Although these planes are relatively avascular, the use of an energized device such as the ultrasonic activated shears can minimize tissue oozing. The specimen was removed with a retrieval bag through a minilaparotomy in the left flank connecting the left subcostal 12 mm-trocar incisions.
Finally the operating field was thoroughly rinsed and a drain left in situ.
Results: Postoperative course was uneventful. Bilirubin level decreased in the early postoperative period. Haemoglobin and RBC improved, and the effect was maintained during 4 months of follow-up. Doppler ultrasonography on postoperative day 7 was negative for portal splenic vein thrombosis.
Conclusions: Laparoscopic splenectomy is an effective technique when performed in patients with hereditary spherocytosis even in the case of large or massive splenomegaly. Proper technique and adequate surgical team training and experience are essential to perform the procedure safely.
Materials and Methods: A 38-year-old man was admitted for severe anaemia with increased need for red cells transfusions, history of haemolytic crisis, mild jaundice and significant splenomegaly. Blood smear, reticulocytes count, bilirubin concentration, osmotic fragility test and direct Coombs test were studied. The diagnosis was confirmed by a positive flow cytometric analysis of eosin-5-maleimide (EMA)-labelled red blood cells (RBCs). Preoperative ultrasonography showed severe splenomegaly (22 x 15,5 x 9 cm) without cholelithiasis. The patient received immunisation for Haemophilus influenzae, Streptococcus pneumoniae and Meningococcus 2 weeks before surgery.
Splenectomy was performed through a 4-port laparoscopic approach and a Gagner oblique position on the right flank with thorax section of the table. The procedure of splenectomy began with the division of the splenocolic ligament followed by the section of the gastrosplenic ligament including the short gastric vessels. An accessory spleen was found and removed. The splenic hilum was dissected: first, splenic artery was skeletonized, clipped and divided, then splenic vein was carefully dissected from the pancreatic tail.
The splenorenal dissection and the division of lateral attachments to the spleen was furthered cephalad along the entire length of the spleen, with care not to enter the Gerota fascia. The splenophrenic ligament division allowed a complete spleen mobilisation. Although these planes are relatively avascular, the use of an energized device such as the ultrasonic activated shears can minimize tissue oozing. The specimen was removed with a retrieval bag through a minilaparotomy in the left flank connecting the left subcostal 12 mm-trocar incisions.
Finally the operating field was thoroughly rinsed and a drain left in situ.
Results: Postoperative course was uneventful. Bilirubin level decreased in the early postoperative period. Haemoglobin and RBC improved, and the effect was maintained during 4 months of follow-up. Doppler ultrasonography on postoperative day 7 was negative for portal splenic vein thrombosis.
Conclusions: Laparoscopic splenectomy is an effective technique when performed in patients with hereditary spherocytosis even in the case of large or massive splenomegaly. Proper technique and adequate surgical team training and experience are essential to perform the procedure safely.
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