Hindawi Publishing Corporation
Case Reports in Pediatrics
Volume 2015, Article ID 129098, 4 pages
http://dx.doi.org/10.1155/2015/129098
Case Report
The Combination of Gastroschisis, Jejunal Atresia, and
Colonic Atresia in a Newborn
Zachary Bauman1 and Victor Nanagas Jr.2
1
Henry Ford Macomb Hospital, Clinton Township, MI 48038, USA
2
Dayton Children’s Hospital, Dayton, OH 45404, USA
Correspondence should be addressed to Zachary Bauman; zbauman1@hfhs.org
Received 24 February 2015; Revised 29 May 2015; Accepted 30 May 2015
Academic Editor: Ursula Kiechl-Kohlendorfer
Copyright © 2015 Z. Bauman and V. Nanagas Jr. This is an open access article distributed under the Creative Commons Attribution
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
We encountered a rare case of gastroschisis associated with jejunal atresia and colonic atresia. In our case, the jejunal atresia was not
discovered for 27 days after the initial abdominal wall closure. The colonic atresia was not discovered for 48 days after initial repair of
the gastroschisis secondary to the rarity of the disorder. Both types of atresia were repaired with primary hand-sewn anastomoses.
Other than the prolonged parenteral nutrition and hyperbilirubinemia, our patient did very well throughout his hospital course.
Based on our case presentation, small bowel atresia and colonic atresia must be considered in patients who undergo abdominal wall
closure for gastroschisis with prolonged symptoms suggestive of bowel obstruction. Our case report also demonstrates primary
enteric anastomosis as a safe, well-tolerated surgical option for patients with types of intestinal atresia.
1. Introduction shortly after he was born secondary to previously diagnosed
gastroschisis. The child was born to a 17-year-old gravida
Atresia of the colon is an uncommon cause of intestinal 1 para 1 female. The pregnancy was uncomplicated other
obstruction in the newborn patient, with classic etiologic fac- than a traumatic event that occurred at 17 weeks resulting in
tors resulting from intrauterine mesenteric vascular obstruc- placental abruption but no loss of the pregnancy. The mother
tion associated with internal hernia, volvulus, intussuscep- did receive appropriate prenatal care. The child was born via
tion, or strangulation in tight gastroschisis [1]. It occurs an uncomplicated vaginal delivery with APGAR scores of 9
between 1.8 and 15 percent of all patients affected by atresia and 9 at one and five minutes, respectively. There is no family
of the bowel, with most studies quoting less than 10 percent history of gastroschisis or intestinal atresia. The mother’s only
of patients reviewed [2]. Reported incidences range from medical history included celiac disease.
1 in 1500 to 1 in 66,000 live births [3, 4]. Furthermore,
approximately 5 to 15 percent of all infants born with Upon presentation to the NICU, the child was examined
gastroschisis will have intestinal stenosis or atresia resulting by the surgical service and deemed to be an appropriate
in increased time to full enteral feeds, prolonged parenteral candidate for reduction of the gastroschisis and primary
nutrition, increased hospital stay, and increased mortality closure of the abdominal wall defect. Only a small amount
[5, 6]. Isolated colonic atresia is an extremely rare condition of bowel was protruding from the abdominal wall defect
with limited information published about management and without signs of compromised bowel viability. The patient
outcomes. Here, we report an even greater rarity in which a was subsequently taken to the operating room where a
patient born with a known gastroschisis was later found to nasogastric tube was placed for decompression. Furthermore,
have both jejunal atresia and colonic atresia. the rectum of the patient was emptied of meconium by
gentle anal dilatation and irrigation, providing standard of
care prior to reduction and closure of the abdomen for
2. Case Report
the gastroschisis. The patient’s gastroschisis was reduced
A 35-week-old gestational age male weighing 2,495 grams and the abdominal wall was primarily closed without any
was admitted to our neonatal intensive care unit (NICU) complications. The extracorporeal bowel was found to be
2 Case Reports in Pediatrics
mildly edematous with minimal inflammatory peel and no
signs of atresia. The patient was transferred back to the
NICU where he was extubated within the first 24 hours
postoperatively. A peripherally inserted central catheter was
placed on postoperative day one and the patient was started
on total peripheral nutrition (TPN), awaiting return of bowel
function before starting enteral feeds. Postoperatively, the
patient never developed any signs or symptoms of abdominal
compartment syndrome or necrotizing enterocolitis. The
patient was continued on IV antibiotics for a total of 72 hours
from the date of surgery.
The patient remained hemodynamically stable with naso-
gastric decompression and parenteral nutrition. He contin-
ued to demonstrate high output from the nasogastric tube,
which, over the course of his first three weeks of life, became
more mucus-like and nonbilious. His abdomen remained
mildly distended but, as Snyder et al. pointed out, patients
can often suffer from severe ileus for up to 3 to 4 weeks Figure 1: Upper gastrointestinal small bowel follow-through at
after closure of the gastroschisis [7]. Further studies have also 4 hours showing dilated loops of proximal small bowel and no
demonstrated prolonged ileus from closure of a gastroschisis. progression of contrast through to the colon, suggestive of small
bowel obstruction.
A study from 2000 by Driver et al. showed the median time
to full oral feedings and resolution of ileus was 30 days
(range: 5 to 160 days) [8] and a 2011 study by Bradnock et
al. showed a median duration of 21 days (range: 9 to 39 developed elevated bilirubin levels due to the extended period
days) to reach full oral intake [9]. For this reason, we never of time receiving TPN. At this point, we attributed the
became alarmed as we felt our patient was just suffering a prolonged ileus to the previous two extensive surgeries expe-
prolonged ileus. However, as the patient approached four rienced by the patient; however, in retrospect, we probably
weeks since the initial gastroschisis closure, the concern for observed the patient too long this second time. The average
a possible bowel obstruction became evident as the patient postoperative ileus for jejunal atresia is approximately 5 days
never developed any further bowel function since the original with a range of 3 to 10 days [10]. Additional imaging, such as
passage of meconium at the initial surgery. Therefore, an an abdominal ultrasound, may have been beneficial during
upper gastrointestinal study with small bowel follow-through this time to help determine why the patient was not having
was obtained (Figure 1). The study demonstrated multiple bowel function. Nonetheless, a barium enema was obtained
dilated loops of proximal small bowel, consistent with a small (Figure 2) after our allotted observation time demonstrat-
bowel obstruction. Therefore, on day of life 27, the decision ing no progression of contrast beyond the midtransverse
was made to take the patient back to the operating room for colon as well as significant microcolon, highly suggestive of
exploratory laparotomy. colonic obstruction. Furthermore, an upper gastrointestinal
After an extensive enterolysis, it was found that the patient study with small bowel follow-through was again performed
had type III proximal jejunal atresia, which is when the blind (Figure 3). It demonstrated patency of the previously created
ends of bowel are separated by a V-shaped defect of the jejunojejunostomy and propagation of contrast into the distal
mesentery [1]. Eight centimeters of the distal atretic jejunum small bowel and proximal colon but was also suggestive of
was resected secondary to questionable viability; however, the a distal colonic obstruction. Due to a work-up and clinical
remaining bowel appeared healthy and without inflammatory presentation highly suggestive of a colonic obstruction, the
peel. A catheter was inserted into the distal atretic enterotomy patient was taken back to the operating room on day of life
and sterile normal saline was injected, demonstrating patency 48 for a second exploratory laparotomy.
of the bowel through to the ascending colon. At this point, Again, extensive enterolysis was performed after entrance
a primary end-to-end hand-sewn enteroenterostomy was into the abdominal cavity. The previous jejunojejunostomy
created followed by a proximal jejunal plication secondary to with the proximal plication was found to be patent. The
the size difference of the two ends of bowel. The abdomen was bowel was followed to the colon where we discovered type
primarily closed and the patient was transferred to the NICU II transverse colonic atresia, which is when the blind ends
in a stable condition. of bowel are separated by a fibrous cord [1]. An enterotomy
Once again, the patient did well postoperatively. His TPN was made in the distal atretic segment and 60cc of normal
was continued and the nasogastric tube remained in place saline was injected into the distal colon with conformation
for decompression secondary to the anticipated postoperative of patency demonstrated by fluid excreted by the anus. The
ileus. Three weeks following the second surgery, there was decision was made to perform a hand-sewn end-to-end
again concern for an obstructive process, as the patient anastomosis by spatulating the distal atretic segment. Once
was still demonstrating high, nonbilious output from the this was completed, the abdomen was closed and the patient
nasogastric tube with chronic abdominal distension and no was transferred to the NICU in a stable condition. The patient
progression of bowel function. Furthermore, the patient also tolerated the procedure well.
Case Reports in Pediatrics 3
Diagnosis of intestinal atresia at the initial presentation of
gastroschisis can be difficult secondary to a thick inflamma-
tory peel covering the bowel [7], as well as poor visualization
of bowel within the peritoneal cavity. Similar to the review by
Kronfli et al., our missed intestinal atresia patient continued
to demonstrate abdominal distension with prolonged feeding
intolerance and high nasogastric tube output [5]. We were,
however, able to recognize this obstructive jejunal atresia
within 27 days of life, as opposed to the 41–57-day range cited
in this review [5].
Our management of this patient was certainly not ideal as
the patient required three operations in total. We believe this
to be related to the rarity of having all three anomalies simul-
taneously. The classic surgical approach to managing patients
with both gastroschisis and small bowel atresia has been to
create a stoma in the acute setting with delayed primary
anastomosis after a period of nasogastric decompression [5].
Figure 2: Barium enema showing microcolon and no progression of A similar surgical approach has been described for colonic
contrast proximal to the midtransverse colon suggestive of colonic atresia in which a primary anastomosis is performed for
obstruction. colonic atresia found proximal to the splenic flexure, and a
colostomy with delayed anastomosis is performed for colonic
atresia distal to the splenic flexure [1]. More recently, bowel
resection with primary anastomosis has been demonstrated
as a reasonable treatment for patients with gastroschisis and
intestinal atresia [1, 5]. Of course, this is only possible when
the bowel is healthy enough to undergo primary anastomosis,
which is left to the judgment of the pediatric surgeon [7].
In our case presentation, the types of intestinal atresia
were not initially diagnosed at abdominal wall closure sec-
ondary to a lack of bowel protruding through the abdom-
inal wall defect and a low suspicion. Unfortunately, during
our patient’s second operation, transverse colon atresia was
missed resulting in a third operation at day of life 48.
Etensel et al. demonstrated in their meta-analysis of 224
cases of colonic atresia a statistically significant increase
in mortality if time from birth to colonic atresia repair
is greater than 72 hours [1]. Although our case does not
support this increase in mortality from delayed repair, our
patient was not without multiple comorbidities including
Figure 3: Upper gastrointestinal small bowel follow-through show-
hyperbilirubinemia, chronic TPN requirements, extended
ing no progression of contrast beyond the right side of the abdomen
after 4 days suggestive of obstruction. Previous enteroenterostomy
period of nasogastric tube insertion with high volume output,
appears patent. constant electrolyte abnormalities, inappropriate weight gain,
and intestinal motility dysfunction.
Isolated colonic atresia is very uncommon, meaning its
association with gastroschisis and jejunal atresia is even more
The patient was continued on TPN and his nasogastric so. Although the management of gastroschisis and intestinal
tube remained in place. A few days after the final surgery, the atresia still remains contentious among various pediatric
patient finally had his first bowel movement. Enteric tubes surgeons, the ultimate goal of restoring normal bowel func-
feeds were introduced slowly as his TPN was weaned. With tion remains the focus of care for these patients. Healthcare
the introduction of enteric feeds and weaning of TPN, the providers must have a high suspicion of intestinal atresia
patient’s hyperbilirubinemia gradually resolved. associated with gastroschisis, something we lacked through-
out this case, if neonates demonstrate intractable bilious vom-
3. Discussion iting, abdominal distension, and feeding intolerance on phys-
ical examination. We delayed the second operation because
Although the incidence of gastroschisis, jejunal atresia, and what we felt was a prolonged ileus from the gastroschisis
colonic atresia found concomitantly in the same patient is itself, but that was obviously an incorrect assumption. Once
not well defined in the current literature, only a signal case the jejunal atresia was discovered, diagnostic dilemmas such
report has been described where one of a pair of dichorionic, as the false positive intraoperative saline injection test, not
diamniotic twins was found to have all three anomalies [6]. completely imaging the rectum and colon prior to surgery,
4 Case Reports in Pediatrics
and the initial passage of meconium during the initial [5] R. Kronfli, T. J. Bradnock, and A. Sabharwal, “Intestinal atresia
gastroschisis closure plagued us causing the patient to require in association with gastroschisis: a 26-year review,” Pediatric
a third operation. Additionally, once the jejunal atresia was Surgery International, vol. 26, no. 9, pp. 891–894, 2010.
discovered, we should have had a higher suspicion for colonic [6] M. A. Saxonhouse, D. W. Kays, D. J. Burchfield, R. Hoover,
atresia as jejunal atresia is associated with a greater number of and S. Islam, “Gastroschisis with jejunal and colonic atresia,
simultaneous congenital malformations than ileal atresia [10]. and isolated colonic atresia in dichorionic, diamniotic twins,”
The diagnosis of gastroschisis is fairly obvious and often Pediatric Surgery International, vol. 25, no. 5, pp. 437–439, 2009.
diagnosed prior to birth. Intestinal atresia, however, can [7] C. L. Snyder, K. A. Miller, R. J. Sharp et al., “Management
be more challenging, especially in the presence of other of intestinal atresia in patients with gastroschisis,” Journal of
pathologic abnormalities. Although the use of both upper Pediatric Surgery, vol. 36, no. 10, pp. 1542–1545, 2001.
and lower contrast gastrointestinal studies should be included [8] C. P. Driver, J. Bruce, A. Bianchi, C. M. Doig, A. P. Dickson, and
in the work-up of intestinal atresia, simply monitoring of J. Bowen, “The contemporary outcome of gastroschisis,” Journal
of Pediatric Surgery, vol. 35, no. 12, pp. 1719–1723, 2000.
bowel movements is extremely sensitive in assessing bowel
obstruction. As clinicians, we were misled by the passage of a [9] T. J. Bradnock, S. Marven, A. Owen et al., “Gastroschisis: one
year outcomes from national cohort study,” British Medical
small amount of meconium at the initial surgery followed by
Journal, vol. 343, no. 7832, Article ID d6749, p. 1036, 2011.
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[10] V. C. Shakya, C. S. Agrawal, P. Shrestha, P. Poudel, S. Khaniya,
various pathologies. Utilizing bowel movement frequency
and S. Adhikary, “Management of jejunoileal atresias: an expe-
with appropriate imaging modalities will allow for early
rience at eastern Nepal,” BMC Surgery, vol. 10, article 35, 2010.
and more accurate diagnosis of obstructive pathology in
neonates, which would have been very beneficial for the
patient described in our case report. Once intestinal atresia
is diagnosed, time should not be wasted in repairing the
obstructive pathology in order to decrease morbidity and
mortality. When patients with such anomalies are managed
appropriately, outcomes are generally favorable.
Disclosure
Drs. Zachary Bauman, D.O., and Victor Nanagas, M.D., had
full access to all the information in the case report and take
responsibility for the integrity of the information and the
accuracy of the information analysis.
Conflict of Interests
There is no conflict of interests or financial interests to
disclose for any of the contributing authors.
Authors’ Contribution
All authors contributed substantially to this project. All
authors involved in this project collectively reviewed and
agreed upon the information as presented. Furthermore, all
authors reviewed and approved the decision to submit this
paper for publication.
References
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clinicopathological insight into its etiology,” European Journal
of Pediatric Surgery, vol. 20, no. 2, pp. 102–105, 2010.
[3] C.-T. Hsu, S.-S. Wang, J.-F. Houng, P.-J. Chiang, and C.-
B. Huang, “Congenital colonic atresia: report of one case,”
Pediatrics and Neonatology, vol. 51, no. 3, pp. 186–189, 2010.
[4] S. Kim, S. Yedlin, and O. Idowu, “Colonic atresia in monozy-
gotic twins,” The American Journal of Medical Genetics, vol. 91,
no. 3, pp. 204–206, 2000.
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