Incomplete pentalogy of Cantrell: a case report and review

BACKGROUND: The pentalogy of Cantrell is a rare congenital malformation characterized by five component defects in the anterior abdominal wall, lower sternum, anterior diaphragm, and diaphragmatic pericardium and congenital heart disease. The occurrence of the five features is quite rare. The pentalogy of Cantrell is classified as complete, partial, and incomplete. Few studies have described the successful treatment of neonates with the pentalogy of Cantrell, with even fewer publications about an incomplete defect. CASE REPORT: We report the successful surgical treatment of a newborn boy with an incomplete pentalogy of Cantrell. In this patient, the diaphragmatic hernia was eliminated at the first stage, and a temporary container for umbilical cord hernia was made by suturing the silastic sac to the edges of the defect in the anterior abdominal wall for subsequent gradual immersion of the hernia contents into the abdominal cavity. These steps made it possible to reduce intra-abdominal and, accordingly, intrathoracic pressures, provide favorable conditions for the healing of the diaphragm, and thus stabilize the child’s respiratory and cardiovascular systems. Subsequently, the cardiovascular system was examined under more favorable conditions and intracardiac defects were excluded. Moreover, the proposed technique made it possible to safely perform the second stage of surgical correction, i.e., radical plasty of the anterior abdominal wall on day 14 of life with full restoration of the normal anatomical and physiological relationships, by which time the diaphragm and mediastinum had taken their correct topographic position. The literature review provides data from 32 sources. CONCLUSION: The pentalogy of Cantrell is a severe congenital malformation with a high risk of poor outcomes. Reporting of all possible variants of the pentalogy of Cantrell (complete, partial, or incomplete), regardless of the outcome, is important for the accumulation of experience in treating such patients, which by focusing on the clinical situation and the combination of various defects in the pentalogy of Cantrell will improve the strategy and prognosis for this defect.