Arleta Zajączkowska-Sadlok, Kazimierz Koziołkiewicz, Piotr Korbal
CaseRepClinPractRev 2002; 3(1):24-27
Background: Cantrell’s pentalogy is a rare congenital defect syndrome, including hernia of the umbilical cord, diaphragmatic defect, abnormalities of the sternum structure, pericardial defect and heart defect. Only 6 cases
of children with the above syndrome who survived were described before 1996. Now, advances in intensive care and new surgical techniques allow to correct more congenital defects, thus making possible further development
of the child.
Case report: The paper presents a case of a neonate, born after full-term pregnancy, operated on on the first day of life because of omphalocele, in whom detection of other anomalies contributed to the diagnosis of Cantrell’s
pentalogy with complex heart defect in the form of Fallot’s tetralogy. The post-operative course was additionally complicated by necrotizing enterocolitis, requiring exteriorization of the small intestine and long-term complete
parenteral feeding. The enterostomy was closed in the 2nd month of life. Because of retardation of psychomotor development, due probably to numerous repeated hospitalizations, the child underwent rehabilitation according to Vojta’s method, which resulted in gradual development of motor and cognitive functions. The patient required close co-operation between the parents and physicians representing many medical disciplines.
Cardiosurgical consultation led to qualification of the boy for surgical correction of heart defects which is to be performed at the age of 1 year. Then the patient is scheduled for reconstruction of the abdominal integument.
Conclusions: Therefore, Cantrell’s pentalogy seems to be one of the defect syndromes, in which survival and nearly normal development of the child is possible owing to advances in pediatric surgery, intensive care and
Keywords: pentalogy of Cantrell, necrotizing enterocolitis, hernia of umbilical cord