Selective management of omphalocele

A retrospective study of the results of selective treatment of infants born with omphalocele was undertaken. This analysis reveals that the mortality of patients with omphalocele is associated with additional congenital anomalies and low birth weight. Fifty-four infants formed the study group. An analysis of these patients allows a logical choice of treatment. The overall mortality for patients with omphalocele was 43 per cent. Exclusion of 11 patients who received no definitive treatment revealed a mortality rate of 28 per cent (12 of 43 patients). Low birth weight affected survival adversely. Seventy-five per cent of the infants who weighed less than 1500 g died, whereas only 33 per cent of those who weighed over 2500 g at birth failed to survive. Eleven infants with obvious chromosomal abnormalities, extrophy of the cloaca, or other severe multiple congenital anomalies also had omphalocele. After consultations of neonatologists, geneticists, and family, these patients received no definitive treatment for omphalocele, and all died as the result of associated problems. The presence of additional major congenital anomalies affected survival adversely. Only two of 19 infants (11 per cent) with no anomalies died, whereas 15 of 19 (80 per cent) of those with major cardiovascular anomalies died. Five of eight infants (63 per cent) with major gastrointestinal anomalies died. The size of the defect was an important factor in survival. The overall mortality rate of patients with defects less than 5 cm in greatest dimension was 33 per cent; if the defect was greater than 5 cm, the mortality rate was 48 per cent. When only those patients who were treated were considered, it was found that no patient with a defect under 5 cm died, but 40 per cent of those with defects over 5 cm died. Perinatal rupture did not appear to increase the mortality rate of those who had surgery. If the omphalocele was ruptured at birth, the mortality rate was 1 7 per cent. Patients with intact omphalocele who were treated had a mortality rate of 30 per cent. Seven patients received topical applications of either 1 per cent merbromin solution, 1 per cent novidone-iodine solution, or 0.5 per cent silver nitrate solution instead of surgical treatment. This treatment was elected in the case of infants with associated severe life-threatening anomalies and of those with defects so large as to preclude primary fascial closure, skin closure, or delayed fascial closure. One infant was given topical therapy in an effort to avoid blood transfusion. Thirty-six patients underwent surgical treatment and 28 (78 per cent) of them survived. Three patients (8 per cent) died from operative complications. One infant had uncontrollable hemorrhage from the liver. This patient had a fascial defect greater than 5 cm and perinatal rupture of the omphalocele sac, in consequence of which a “silo” technique was deemed the only available method of treatment. A large portion of the liver was outside the abdomen. In the preparation of a fascial edge to which the silo could be attached, the contiguous liver capsule was torn, which resulted in uncontrollable hemorrhage. Two patients had primary fascial closure and died from progressive acidosis and respiratory failure. One of these infants also had bowel necrosis, probably from over-compression. Five patients (14 per cent) died from congenital heart disease or sepsis. All patients with defects less than 5 cm who underwent surgery survived. Patients who had primary closure of the fascia had very little morbidity. No patient had a purulent wound infection. Similarly, infants who had only skin closure had no major wound complications. The silo technique has been useful in obtaining closure of the abdominal defect when it was large. In two of these patients, the silo separated from the fascial margins. One patient had conversion from a silo to skin closure. The other was treated with topical 1 per cent povidone-iodine solution, and skin coverage was obtained by wound contraction and epithelialization.