Asplenia

(see also Ivemark syndrome type I)


Congenital absence of the spleen. It can be isolated but is usually associated with other malformations such as situs solitus or situs inversus or ambiguous. This malformation is more common in boys than in girls.

Possible associated malformations  (see also heterotaxy):

-        cardiac: 99 %: persistent left superior vena cava (46-71 %); lack of coronary sinus (80-85 %); total anomalous pulmonary venous return (64-72 %); dextrocardia (36-41 %); congenital atrioventricular block (84-92 %); sometimes hypoplasia of one of the ventricles (44-55 %)

-        pulmonary: 2 trilobate lungs  (right !) (81-93 %); tracheal bronchus

-        liver: median liver  (76-91 %), atresia of the extrahepatic bile ducts (10 %), preduodenal portal vein .


Anesthetic implications: 

preoperative echocardiography; complex cardiac malformation; high incidence of left superior vena cava; the aorta and the inferior vena cava can be located on the same side of the vertebral axis; risk of single coronary artery. Disorder of thermoregulation ?


References : 

-          Williams GD, Feng A. 
Heterotaxy syndrome : implications for anesthesia management. 
J Cardiothorac Vascul Anesth 2010; 24: 834-44.

-        Leung WK, Jahr JS, Hotz J et al. 
Nonmalignant hyperthermia on induction of anesthesia in a pediatric patient undergoing bidirectional Glenn procedure. 
J Clin Anesth 1998; 10: 427-31.

-        Ming Z, hong S, Biao J. 
Asplenia syndrome with bilateral tracheal bronchi. 
Circulation 2008; 118: 196-7.


Updated: November 2019