I. Introduction

Intestinal atresia occurs in approximately 10% to 20% of patients with gastroschisis ^[1] . Treatment of such cases is often difficult and the association of intestinal atresia is reported to increase the mortality of gastroschisis cases ^[1-3] . However, the optimum surgical management still remains a matter of debate. We herein describe the successful treatment of two such gastroschisis cases, including one accompanied by an intestinal perforation.

II. Case

Case 1

Gastroschisis with intestinal atresia was diagnosed by ultrasound at 30 weeks of gestation in a female fetus; the infant was delivered vaginally at 37 weeks of gestation, and weighed 2434 grams. Severe intestinal edema and adhesion prohibited primary abdominal wall closure and she underwent silo repair two hours after birth. At that time, the intestinal atresia site could not be ascertained. Radical abdominal wall closure and delayed primary intestinal anastomosis was performed nine days after birth. At the second surgery, the intestinal edema had decreased and the adhesions were dissolved; the procedure for the combined abnormality progressed well without difficulty. Prior to this radical procedure, only a nasogastric tube was necessary for decompression. The patient did well postoperatively and was discharged at 85 days of age.

Case 2

A 2502-gram female infant was delivered vaginally at 36 weeks of gestation; no anomalies had been noted during the prenatal course. At birth, gastroschisis with an intestinal perforation was found. Seven hours after birth, the first operation was performed. At that time, the intestinal abnormality, which caused perforation, could not be ascertained because of the intestinal edema and adhesions. Therefore, we decided to perform a staged repair. The first operation included a silo repair for the abdominal wall defect, and a tube drainage, which was inserted into the intestine via the perforation site. Additional soft tubes were placed inside of the silo pouch to drain overflowed intestinal contents ^{(Fig. 1, 2)}. Five days after birth, a second operation was performed. At surgery, the intestinal edema and adhesion was improved and intestinal atresia was suspected as the cause of the perforation; however, the specific atretic site was not apparent. We performed a closure of the abdominal wall defect and made an enterostomy at the perforation site. A barium enema at 63 days after birth revealed a microcolon. At 70 days of age, a third operation was performed. At surgery, the colonic atresia was confirmed and the perforation site was found at the caecum; an intestinal anastomosis was made. The patient did well postoperatively and was discharged 35 days after the last operation (105 days of age).

III. Discussion

Currently, gastroschisis is recognized as a disease with a good prognosis and the mortality rate has been reported to be 0 to 8 % ^[1,2,4,5] . However, if the condition is complicated by intestinal atresia, its management and treatment becomes more difficult. Several investigators have identified intestinal atresia as a marker for increased mortality in gastroschisis ^[1-3] . Furthermore, intestinal perforation, which makes the situation even more complicated, occurs in 12.5 to 20 % of gastroschisis cases with intestinal atresia ^[2,3,6] . In Japan, 36 cases of gastroschisis, accompanied with intestinal atresia, have been reported, and seven cases (13.9 %) were complicated with an intestinal perforation. Strategies for these conditions are not yet established, and it still remains controversial about the timing of intestinal anastomosis and the indication of enterostomies.

Case 1 had an intestinal atresia at the jejunum, which had been diagnosed prenataly. At birth, the thickened and edematous intestine contraindicated the performance of a primary anastomosis. Therefore, we chose a delayed primary anastomosis, using a nasogastric tube for intestinal decompression. Prior reports have suggested that if the intestine is of good quality with an isolated atresia and limited proximal dilation, primary anastomosis can be selected. If excessive intestinal adhesion and edema (peel) prohibits a primary anastomosis, delayed primary anastomosis is preferred than enterostomy, since stomas might lead to major complications ^[1-3,6,7] . The advantage of delayed primary anastomosis is that the bowel softens rapidly within 7 to 14 days once returned to the abdominal cavity, making the anastomosis much easier ^[2] . In our case, we could not perform primary abdominal closure and a silo was placed initially. Nine days later, the intestinal peel was improved markedly and we were able to perform a delayed primary anastomosis. However, only in cases in which the proximal intestine is grossly dilated, enterostomy must be considered to allow maximal deflation of the proximal intestine ^[3] .

Case 2 had ascending colonic atresia with a caecal perforation. Excessive intestinal peel made it impossible to perform both a primary abdominal closure and an enterostomy; therefore, a silo repair was required. It is generally considered that in perforated cases, stoma construction is required for safe management ^[2] . However, stoma construction is recommended to take place at the time of abdominal closure, ^[1] since the combination of silos and stomas makes the management complicated and increases the risk of infections. Therefore, in perforated cases that require silo repair, it is necessary to avoid the leakage of intestinal content from the perforation site until abdominal wall closure and stoma construction becomes possible. Oversewing the perforation site may be one strategy for this condition ^[2] . We considered that oversewing an edematous, thickened, and dilated intestine might carry a risk of re-perforation. As an alternative strategy for local control of the perforation site, we inserted a drainage tube into the intestine via the perforation site and successfully drained the intestinal content. As a result, the intestinal peel was lessened and safe stoma construction could be performed at the second surgery. We recommend this strategy in perforated cases that require a staged abdominal closure.