2020 年 2 巻 2 号 p. 61-68
BACKGROUND
Omphalocele is frequently detected prenatally worldwide. Nevertheless, little is known about patient demographics, practice pattern and outcomes.
METHODS
We examined patient demographics, treatment options, and outcomes for patients with omphalocele with and without severe chromosomal abnormalities (trisomy 13 and 18), July 2010–March 2016, using a nationwide database in Japan.
RESULTS
Of 399 patients with omphalocele, 89 had trisomy 13 or 18. The average birthweight was 2,449 g, and the average gestational age was 35 weeks; 65% had other congenital associated anomalies, including circulatory and chromosomal anomalies. About 85% of the patients received abdominoplasty. The median length of mechanical ventilation was 5 (0–30) days. The median length of stay (interquartile range) was 45 (21–94) days. Overall, 52% of the patients were discharged to home without home medical care; this percentage was 65% among those without trisomy 13 or 18. In-hospital mortality was 20% for the total sample of patients, while it was 49% for those with trisomy 13 or 18.
CONCLUSIONS
The present study revealed the patient demographics, treatment practices, and discharge status of infants with omphalocele in Japan. This information is useful in aiding decision making on therapeutic strategies by medical staffs and the families of patients with omphalocele.
