Surgical paper XIV
Successful primary repair of congenital omphalocele
A Case Report
Case Presentation
The patient, a male newborn named Shao, was born at home on May 14, 1983. He was brought to the emergency department four hours post-birth due to the presence of an omphalocele, a condition where part of his abdominal organs were protruding out of the abdomen, enclosed in a transparent membranous sac.
Clinical Examination
Upon examination, the infant weighed 3010 grams and had no other associated congenital malformations. The umbilical cord was ligated at a distance of 10 cm. The transparent sac contained portions of the liver, the majority of the stomach, and sections of the small and transverse intestines. There were no signs of strangulation or vascular compromise. The sac was intact and not discolored, and the abdominal wall defect at the umbilicus measured 6 cm (Figures 1 and 2).
Surgical Intervention
The surgery was performed under local anesthesia. The omphalocele sac was excised, and the skin edges were trimmed and dissected laterally. Successful herniation of the protruding organs was achieved, followed by layered suturing (Figure 3). The patient experienced neither respiratory distress nor circulatory complications. He passed gas and stool on the evening following surgery, and sutures were removed as scheduled. The wound healed without complications.
Follow-up
One-and-a-half years post-surgery, the child displayed normal development, stable gait, and no intellectual disabilities. He had begun to recognize and call out to people.
Discussion
Congenital omphalocele is an embryonic developmental disorder resulting from the failure of synchronous development between the abdominal cavity and viscera. Approximately 40% of cases present with additional congenital malformations. The condition is relatively rare, occurring in 1 in 7,000 births. The literature contains limited case reports, with one study reporting only 22 instances. Delayed surgical intervention can result in desiccation, necrosis, and rupture of the omphalocele sac, exposing the organs to infection and significantly increasing mortality risk. The surgical approach, either primary or staged repair, depends on the size of the omphalocele and the developmental status of the abdominal cavity. Our case, although categorized as a "giant" omphalocele due to the 6 cm abdominal wall defect and involvement of multiple organs, was successfully managed through primary repair owing to the absence of other deformities and favorable overall development.
References
- She Yaxiong: Pediatric Surgery. 1979; p. 296
Figures: Embryonal Omphalocele
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Figure 1: External appearance of the omphalocele with a 6 cm abdominal wall defect at the umbilical pedicle.
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Figure 2: Organs involved in the omphalocele: liver, stomach, small intestine, and transverse colon.
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Figure 3: Appearance following successful primary repair.
1988/04/01 Changhang Hospital, Li Mingjie
from 先天性脐膨出一期修补成功一例