Repair of Ruptured Omphalocele Sac in the Neonatal Period and Beyond.

Conservative management of giant omphalocele in the neonate period is a known strategy to allow tissue growth aiding in anatomical closure. However, rupture of the covering sac is considered an absolute contraindication for continuing conservative management. We report a case where a ruptured sac of giant omphalocele was ingeniously sutured to restore its integrity, and conservative management continued. The giant omphalocele later became a huge ventral hernia and was gradually reduced and primary closure was achieved with multiple surgeries over a period of 4 years. Copyright:

INTRODUCTION

Conservative management of giant omphaloceles in the neonatal period to form a ventral hernia is an established management strategy. The advantages of such an approach include avoidance of a major surgical procedure during the neonatal period, avoidance of silo construction and its attendant complications, and importantly allowing growth of the abdominal cavity which helps in the reduction of omphalocele contents with minimal rise in intra-abdominal complications.[1] However, rupture of the sac is considered to be a contraindication to continuation of conservative management because of the risks of desiccation, perforation, and infection of exposed intra-abdominal contents. We here present a case where the integrity of the ruptured sac was ingenuously restored by simple suturing. Conservative management was continued to form a ventral hernia which was managed subsequently.

CASE REPORT

A full-term female baby with giant omphalocele was delivered normally at a small rural hospital. The omphalocele sac ruptured intrapartum exposing the entire abdominal contents, including the liver and the small bowel [Figure 1a]. The baby reached a referral hospital within 3 h of birth. Considering the general condition of the baby, a large volume of the extruded viscera, and limited facilities for intensive care, the attending surgeon found the baby unfit to attempt a major procedure such as primary repair or silo construction. The surgeon sutured the edges of the ruptured sac to restore its integrity [Figure 1b]. The resutured sac was then painted daily with dilute povidone-iodine solution which resulted in epithelization and escharification forming a ventral hernia. It was in this condition that the child was referred to us [Figure 1c]. At the age of 15 months, closure of the omphalocele was attempted, but due to the large size, a subcutaneous silo was made, and gradual reduction was planned [Figure 1d]. Unfortunately, the skin sloughed off the mesh, and this led to a paucity of covering skin. To obtain tissue for closure, 2 months following the silo construction, two tissue expanders were inserted subcutaneously, laterally in the abdomen [Figure 2a]. Two months later, one of the tissue expanders had to be removed following infection, and 1 month later, partial reduction of the silo was done with the expanded skin. One month following this, the second expander was removed, and the silo was reduced and closed, although with a mesh [Figure 2b]. Unfortunately, the recovery was complicated with a small fecal fistula. The child was subsequently discharged with the mesh in situ and a small controlled fecal fistula, for the subcutaneous tissue to grow in the intervening period before the mesh could be removed [Figure 2c]. Finally, 2 years later, resection and anastomosis of the fistula and removal of the mesh with primary closure of the sheath were achieved [Figure 2d].

Figure 1

(a) Ruptured omphalocele sac with extruded viscera; (b) resutured sac in the neonate; (c) ventral hernia at 15 months; (d) subcutaneous silo at 15 months

Figure 2

(a) Tissue expanders for skin expansion; (b) reduction of the silo with mesh; (c) fecal fistula with extruded mesh before final closure; (d) final result after the closure of sheath

DISCUSSION

Giant omphaloceles have been defined variably based on the size or the contents. A size of defect >5 cm or the presence of liver in the sac is accepted by most to define a giant omphalocele.[2] These present a unique challenge for management in the neonatal period. Primary reduction and closure are technically challenging because of the small volume of the abdominal cavity. Even when the reduction is possible, there are significant morbidity and mortality due to abdominal compartment syndrome, respiratory and circulatory compromise. Provisions for strict monitoring, prolonged mechanical ventilation, and parenteral nutrition are important for postoperative recovery.

The introduction of silo repair attempted to reduce the risks of abdominal compartment syndrome. However, this has its own complications, including infections, tear or dislodgement of the silo, and fascial disruption. In addition, this may require intensive monitoring, prolonged ventilation, and parenteral nutrition on a case to case basis.[2]

Conservative management of giant omphaloceles was described first in 1899 by Ahlfeld using alcohol as a topical agent.[3] Since then, a variety of agents have been used to aid in escharification and epithelization of the omphalocele to form a ventral hernia. This approach offers several benefits. It avoids a major surgery in the neonatal period and all the complications of reduction of contents in a small abdomen. There is no risk of abdominal hypertension, respiratory or renal compromise, and minimal chances of infection. Early feeds can be instituted, and minimum monitoring is required. In developing countries like ours where access to tertiary-level health care is difficult, this approach can be life-saving for many babies with omphalocele.

However, tear of the omphalocele sac any time during conservative management has been considered to be an absolute indication for shifting to surgical management because it can result in desiccation and perforation of the contents. In our case, surgical management would be associated with unacceptable risks because of unavailability of intensive care. Transfer of the patient to a higher center was not possible immediately. Without much option, the torn sac was resutured, as a life-saving procedure and conservative management continued. The omphalocele was successfully epithelialized into a ventral hernia. The paucity of the tissues was such that even later, it required multiple surgeries to achieve a successful closure of the sheath. Of course, infection contributed to the complications later during closure of the ventral hernia; this prolonged the duration of treatment and increased the morbidity; nevertheless, these could be managed successfully, finally resulting in the discharge of a healthy child.

This strategy for ruptured omphalocele has been described very rarely in the literature. Akakpo-Numado et al. and Patil et al. reported the same initial approach in neonates with ruptured omphalocele sac. However, the surgical approach for closure of the ventral hernia was not presented.[45] Our case's success story endorses this alternative approach in the management strategies of ruptured giant omphaloceles in the neonatal period. It is especially advantageous in the peripheral centers of developing countries in an emergency situation. The procedure in itself does not complicate further management of the child at a later time. This option is safe and simple and may be carried out even if a specially trained pediatric surgeon is not present.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.