Closed gastroschisis: total parenteral nutrition–free
survival with aggressive attempts at bowel preservation and
Sarah A. Voglera, Stephen J. Fentona, Eric R. Scaifeb, Linda S. Bookc,
Daniel Jacksonc, Peter F. Nicholb, Rebecka L. Meyersb,⁎
aDepartment of Surgery, University of Utah, Salt Lake City, UT 84132, USA
bDivision of Pediatric Surgery, Primary Children's Medical Center, University of Utah, Salt Lake City, UT 84113, USA
cDivision of Pediatric Gastroenterology, Primary Children's Medical Center, University of Utah,
Salt Lake City, UT 84113, USA
Received 28 January 2008; accepted 8 February 2008
Abdominal wall defect;
Background: In infants with gastroschisis antenatal closure of the umbilical defect results in a proximal
atresia with ischemia and/or volvulus of the extracorporeal midgut. It has been described as “closed
gastroschisis” or “vanishing midgut.”
Methods: A 10-year review of 219 gastroschisis patients identified 10 infants with this rare complication.
Results: In these 10 infants, the extracorporeal midgut was invariably matted and fibrosed. In 3 cases, the
midgut had completely “vanished.” In the remaining 7 cases, the remnant midgut was surgically reduced
into the abdominal cavity with care not to compromise the diminutive vascular pedicle. Abdominal
underwent a serial transverse enteroplasty. Mean residual length of salvaged small bowel was 79 cm with
of stay of 121 days and mean hospital charge of $287,094. Six of the 7 long-term survivors have been
completely weaned off total parenteral nutrition.
Conclusion: A nihilistic attitude toward infants with closed gastroschisis may not be uniformly
supported because in the majority of these infants' long-term independence from total parenteral
nutrition was achieved.
© 2008 Elsevier Inc. All rights reserved.
Inexplicably, the prevalence of gastroschisis has increased
significantly in recent years , and as the number of cases
has increased, the number of complex cases has grown
apace. Perhaps the most complex situation arises in infants
with spontaneous antenatal closure of the gastroschisis
umbilical defect. Although advances in nutritional manage-
ment and neonatal and surgical care have combined to
improve survival in infants with gastroschisis to greater than
90% , infants with antenatal closure of the umbilical
defect have been recognized in the literature to have
substantially worse outcomes. The generally discouraging
Presented at the 59th Annual Meeting of the Section on Surgery,
American Academy of Pediatrics, San Francisco, CA, October 25-27, 2007.
⁎Corresponding author. Tel.: +1 801 662 2950.
E-mail address: email@example.com (R.L. Meyers).
0022-3468/$ – see front matter © 2008 Elsevier Inc. All rights reserved.
Journal of Pediatric Surgery (2008) 43, 1006–1010
outcomes reported in 18 cases in the literature have been
variably labeled as “closing/closed gastroschisis,” “midgut
atresia,” “total intestinal atresia,” and “vanishing bowel/
midgut” [2-7]. Antenatal closure of the umbilical defect
strangulates the herniated midgut, which can result in
mortality secondary to short gut syndrome and cholestatic
liver failure. Our recent experience with better than expected
outcomes in some of these infants prompted us to review
these cases (Figs. 1 and 2).
After obtaining approval from the institutional review
board (IRB #16717) at the University of Utah, Salt Lake
City, Utah, a review was conducted of all infants born with
gastroschisis between January 1996 and March 2007. The
medical records were reviewed for patient demographics
(maternal history, prenatal evaluations, gestational age at
birth, birth weight, and sex) and details of gastroschisis and
involved intestine, operative procedures and findings, nutri-
tional support, ventilator requirements, hospital course, and
overall outcomes (mortality, intrahospital complications,
length of stay, outpatient follow-up). These data were collec-
ted and entered into a database in continuous, ordinal, and
Between January 1996 and March 2007, we treated 219
infants with gastroschisis, 33 (15%) of whom were complex
cases as defined as having an associated atresia, stricture, or
perforation. In 10 of these 33 complex cases, the umbilical
defect had closed before birth, causing strangulation of the
herniated bowel. Ischemic damage was noted in the variably
mummified, matted, and fibrosed remnant of extracorporeal
midgut. Nine of 10 were diagnosed with gastroschisis by
prenatal ultrasound, and in 5 of these 9, the prenatal
ultrasound had recognized an associated atresia. The mat-
ernal age ranged from 15 to 25 years (average, 19 years). The
mother was primigravida in 50% of the cases. The
gestational age of the infant was more than 32 weeks in all
cases, with most being more than 34 weeks. Nine of the
infants were born by spontaneous vaginal delivery, and one
infantwas born by cesarean delivery because offetal distress.
Six of the patients were male and 4 were female.
Overall, these 10 infants underwent an average of 2.5
operative procedures (range, 1-4). Given the alarming
appearance of the herniated bowel at birth, none were con-
sidered candidates for a bedside preformed silo. All were
taken immediately to the operating room for exploratory
laparotomy and careful inspection of the remnant midgut. In
7 infants, we suspected that the amorphous fibrotic glob of
however, the midgut had completely vanished or infarcted
(Figs. 1 and 2).
In these 3 cases “of vanishing bowel,” the remaining
length of small bowel from pylorus to atresia measured less
than 10, 10, and 23.5 cm, respectively. The findings of
extreme short bowel and the implications of this diagnosis
were discussed in multidisciplinary consultation with the
family, neonatology, and gastroenterology. In these 3 cases,
the families were offered the full spectrum of treatment
options ranging from “comfort care only” to intestinal trans-
plantation . Both families whose infant had duodenum
only (V10 cm from pylorus) declined aggressive care. Both
of these infants died at home under hospice care a few days
later. The third infant with a “vanished” midgut had 23.5 cm
of intraabdominal duodenum/jejunum, the family elected to
pursue aggressive care. At initial surgery the dilated proxi-
mal atresia was anastomosed to the distal microcolon. This
infant underwent early institution of enteral feeds and
subsequently had second surgery for a serial transverse
enteroplasty procedure (STEP) at 6 weeks of age. He was
discharged home on total parenteral nutrition (TPN) at
4 months old. The TPN has subsequently been weaned as
tolerated. Enteral nutrition has been supplemented with fish
oil since infancy in an attempt to ameliorate TPN cholestasis.
In 7 infants with closed gastroschisis, some atretic midgut
remained, and we made aggressive attempts to salvage some
portion of this strangulated bowel (Fig. 3). The umbilical
defect was carefully reopened, taking great care not to twist
or kink the diminutive pedicle of fragile blood supply. In 5 of
7 infants, the strangulated remnant of midgut was reduced
back into the abdominal cavity at this initial operation, with
no attempt to reestablish intestinal continuity. One of these
5 infants had 25 cm of frankly necrotic bowel resected at this
first operation; 4 had a proximal decompressive duodenost-
omy/jejunostomy. Several weeks later (range, 52-82 days),
these infants underwent second-look laparotomy, careful
dissection of the adhesions and fibrosis of remnant midgut,
resection of strictured segments of bowel, and restitution of
Two of the 7 infants in whom we attempted to salvage the
midgut had a silo placed within the surgically reopened
umbilical defect at the initial operation. The dilated and
fibrosed midgut was reduced back into the abdominal cavity
for the next 7 to 10 days. These infants underwent lapa-
rotomy, resection of strictured segments of bowel, and resto-
ration of bowel continuity 20 and 54 days later, respectively.
The infant with the shortest amount of remaining small
bowel (40 cm) underwent subsequent laparotomy for STEP
for bowel lengthening and amelioration of bacterial over-
growth in the dilated proximal bowel.
Overall, 8 infants survived the initial hospitalization, with
a mean length of stay of 121 days (range, 36-205 days) and a
mean hospital charge of $287,094 (range, $86,310-522,817).
The mean residual length of salvaged small bowel was 79 cm
(range, 24-180 cm), with retention of the distal half of the
colon (hindgut). Three of 8 were weaned off TPN before
discharge, 5 were sent home with TPN supplementation, one
died while still receiving TPN, and one remains on minimal
TPN supplementation at 11 months old. All others were
completely weaned off TPN. All infants developed some
degree of TPN cholestasis, one died of complications related
to liver failure and sepsis awaiting transplantation, and none
underwent transplantation. The 7 long-term survivors are
Infants with vanishing midgut or closed gastroschisis
Infant Length of proximal
Total length of
Description of gastroschisisLength of
b10 TC to anus 1 Vanishing midgut Comfort
Off TPN, app.
Off TPN, app.
Death at 6 mo
2 10 53RC to anus 2Closed: sphere of matted
bowel with small vascular pedicle
Closed: ischemic, fibrotic,
Closed: small vascular stalk
with matted ball of bowel
Closed: ischemic ball of bowel
with 25 cm of necrotic bowel
Closed: sphere of matted bowel,
stalk of mesentery
3 25 62Ileum to
TC to anus 2
4 19 44 113
5 25170 Ileum to
TC to anus 4
6 1040 205 Off TPN, supp
Off TPN, app.
7 15 180LC to anus 4 Closed: inflamed, matted bowel,
8 1010 LC to anus 1 Comfort
8192060TC to anus 4Closed: bouquet of pink bowel
with small vascular stalk
Vanishing midgut 10 23.523.5TC to anus 2 133 Weaning TPN,
TC, transverse colon; RC, right colon; LC left colon.
1008S.A. Vogler et al.
gaining weight and achieving appropriate developmental
milestones. The overall mortality was 30%—2 of 3 (66%) in
vanishing midgut and 1 of 7 (14%) in closed gastroschisis
(Tables 1 and 2).
There is a broad spectrum of sequelae from antenatal
narrowing of the gastroschisis umbilical ring defect. At the
most severe and lethal end of this spectrum, the tiny vascular
pedicle is lost, either by extrinsic compression or volvulus,
and the herniated midgut undergoes ischemic infarction and
is gradually resorbed or mummified. This scenario was
termed vanishing midgut by Johnson et al  and Bromley
et al . Infants born with gastroschisis and vanishing
midgut have nearly a 70% mortality according to current
literature in combination with our data. There have been only
6 cases of vanishing gut reported in the literature, with all but
2 cases resulting in fatality [4,5]. In the case reported by
Barsoom et al , the infant was found to have 10 cm of
functional bowel on initial exploration, and a Bianchi bowel
lengthening procedure was performed at 5 months old. The
patient remained hospitalized at 8 months old because of
end-stage liver failure, and it is unknown if the patient is still
alive. The case reported by Davenport et al  also
underwent the Bianchi bowel lengthening procedure and
developed end-stage liver disease but underwent liver
transplantation at 12 months and was still alive at 2.5 years
old. This series includes 3 infants with “vanishing midgut”.
In 2 infants, only duodenum remained, and both of these
families elected not to pursue aggressive medical care.
However, our third case of vanishing midgut had a 23.5-cm
length of a proximal atresia, underwent primary anastomosis
at initial exploration, and subsequently had a STEP at 6
weeks old. This infant was discharged to home on
supplemental TPN, which has since been weaned signifi-
cantly; at 11 months old, he continues to slowly adapt.
Next in severity are those cases of complete umbilical ring
closure with just a tiny stalk containing a diminutive vascular
pedicle attached to an island of extracorporeal midgut. This
has been termed closed gastroschisis and closing gastro-
schisis . The extracorporeal midgut appears matted,
fibrosed, and, in some cases, necrotic. The intraabdominal
bowel will include dilated proximal bowel with a duodenal
or jejunal atresia and, typically, a distal atresia at the level of
the transverse colon. There have been 12 cases of closing
gastroschisis reported in the literature, dating back to
Kiesewetter  in 1957. Based on these cases, which
have been published mostly as single case reports, this
scenario has 75% mortality [9-16]. Most of these case reports
described dilated proximal small bowel, approximately 15 to
25 cm in length, a small amount of viable bowel involved in
the extracorporeal midgut, and a distal atresia at the level of
the transverse colon. In most of these cases, because of the
very limited amount of viable bowel, no aggressive medical
or surgical care was pursued, and the infants died during the
In this series, the mortality of the 7 infants with closed
gastroschisis was only 14%, highlighting the importance of
avoiding a uniformly fatalistic attitude when treating these
patients. Vigilance and patience by the surgeon and gastro-
the best chance of survival. These 7 infants all survived their
initial hospitalization and were successfully discharged to
home. The one death that occurred was caused by compli-
cations of cholestatic liver failure and sepsis at 6 months old.
The other 6 cases were discharged to home after an average
hospitalization of121days,3abdominal surgical procedures,
and a hospital cost of $298,768. After their initial exploratory
laparotomy, these patients remained in the neonatal intensive
care (NICU) for an average of 58 days before returning to the
operating room for reinspection of the reduced midgut and
anastomosis. While in the NICU, meticulous attention was
given toward nutritional management, including TPN and
enteral feeds. We believe that waiting several weeks before
returning to the operating room allowed the fibrosis time to
soften and the residual bowel time to heal. Although dense
adhesions were encountered during the second operation, the
bowel could be safely separated and inspected for any
a 10- to 25-cm length of a proximal duodenal/jejunal atresia,
so aggressive attempts to salvage their strangulated midgut
were vital. By patiently waiting for the injured midgut to
soften and either heal or fibrose, a significant amount of
bowel was able to be saved. Ultimately, the mean length of
salvaged small bowel, including the proximal duodenal/
jejunal atresia plus salvaged midgut, was 79 cm.
Although the cause of gastroschisis, in general, remains a
subject of much investigation, the neonatal and surgical
management of these infants continues to become better
defined . Familiarity with the broad array of clinical
sequelae from gastroschisis and the use of prenatal
ultrasound have allowed more insight into management
and possible treatments. Meanwhile, technological advance-
ments in neonatal care and greater knowledge about
Total parenteral nutrition and enteral feeds
Patient Total TPN days Diet at discharge
TPN and TF
TPN and PO
TPN and PO
TPN and PO
1009 Closed gastroschisis
nutritional support continue to decrease the number of lethal Download full-text
outcomes from complicated gastroschisis. This series of 10
patients with antenatal closure of their abdominal wall defect
highlights the importance of maximizing the advancements
that are available in neonatal surgical and nutritional care to
preserve the greatest extent of involved small intestine.
Ultimately, a nihilistic attitude toward these infants is not
uniformly supported because most of the infants in this series
achieved survival with long-term independence from TPN.
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