Familial asplenia, other malformations, and sudden death

Two families have been presented. In one, asplenia occurred with and without gastrointestinal malformations. In the other, cardiovascular malformations occurred in one member and Ivemark syndrome in the other. All three children with asplenia died in infancy, two with evidence of sepsis. Sudden death in infancy may be due to sepsis in a child with isolated asplenia syndrome and impaired resistance to infection. Both isolated asplenia and some cases of congenital heart disease without asplenia may be related to Ivemark syndrome. New infants born in families with a history of congenital cardiovascular malformations, visceral heterotaxy, or other malformations associated with Ivemark syndrome should be tested for asplenia, primarily by searching for Howell-Jolly bodies. Infans with asplenia should be protected with prophylactic antibiotics. Pneumococcal polysaccharide vaccine is indicated at the age of 2 years. It would appear worthwhile for pediatric surgical or pediatric cardiologic services to perform a screening program for Howell-Jolly bodies.