- Email: [email protected]
The risk of midgut volvulus in patients with abdominal wall defects: A multi-institutional study
Journal of Pediatric Surgery xxx (2016) xxx–xxx
Contents lists available at ScienceDirect
Journal of Pediatric Surgery journal homepage: www.elsevier.com/locate/jpedsurg
The risk of midgut volvulus in patients with abdominal wall defects: A multi-institutional study Jason A Fawley a,b, Abdelhafeez H. Abdelhafeez c, Jessica A. Schultz a,b, Allison Ertl a, Laura D. Cassidy a,b, Shawn St Peter d, Amy J. Wagner a,b,⁎ a
Medical College of Wisconsin, Milwaukee, WI Children's Hospital of Wisconsin, Milwaukee, WI The Children's University Hospital, Dublin, Ireland d Children's Mercy Hospital, Kansas City, MO b c
a r t i c l e
i n f o
Article history: Received 29 September 2016 Accepted 20 October 2016 Available online xxxx Key words: Midgut volvulus Omphalocele Gastroschisis Ladd procedure
a b s t r a c t Background: The management of malrotation in patients with congenital abdominal wall defects has varied among surgeons. We were interested in investigating the risk of midgut volvulus in patients with gastroschisis and omphalocele to help determine if these patients may benefit from undergoing a Ladd procedure. Methods: A retrospective chart review was performed for all patients managed at three institutions born between 1/1/2000 and 12/31/2008 with a diagnosis of gastroschisis or omphalocele. Patient charts were reviewed through 12/31/2012 for occurrence of midgut volvulus or need for second laparotomy. Results: Of the 414 patients identified with abdominal wall defects, 299 patients (72%) had gastroschisis, and 115 patients (28%) had omphalocele. The mean gestational age at birth was 36.1 ± 2.3 weeks, and the mean birth weight was 2.57 ± 0.7 kg. There were a total of 8 (1.9%) cases of midgut volvulus: 3 (1.0%) patients with gastroschisis compared to 5 patients (4.4%) with omphalocele (p = 0.04). Conclusions: Patients with omphalocele have a greater risk of developing midgut volvulus, and a Ladd procedure should be considered during definitive repair to mitigate these risks. Level of evidence: III; retrospective comparative study © 2016 Elsevier Inc. All rights reserved.
The normal rotation of the intestine follows precise embryologic events that result in a fixed intestinal configuration with the duodenojejunal junction in the left upper quadrant and the cecum in the right lower quadrant [1]. Normal intestinal configuration allows for the midgut to be anchored by a wide mesenteric base. Patients with abdominal wall defects have a disruption in embryologic rotational events, which results in malrotation of the intestines with potential for a narrow mesenteric base and midgut volvulus. Midgut volvulus is a devastating event associated with significant morbidity and mortality. The Ladd procedure is the procedure of choice for correction of malrotation. Performing a Ladd procedure in patients with abdominal wall defects is variable among surgeons [2–5]. This variability is a result of the belief that adhesions from repair of the abdominal wall defect should be sufficient to provide bowel fixation and prevent midgut volvulus. Additionally, manipulation of friable bowel may pose risk to the patient [2,3]. However, the ability of adhesions to provide bowel fixation remains uncertain. There is little evidence regarding the incidence of midgut volvulus in patients with abdominal wall defects.
⁎ Corresponding author at: Medical College of Wisconsin, Division of Pediatric Surgery, 999 N 92nd Street Suite 320, Milwaukee, WI 53226. Tel.: +1 414 266 6550. E-mail address: [email protected] (A.J. Wagner).
Furthermore, gastroschisis patients may have more protective adhesions because of inflammation and increased handling. However, this is not true in the case of omphalocele and may predispose these patients to midgut volvulus. In a single-institution study examining midgut volvulus in subjects with abdominal wall defects, 3% of those with omphaloceles developed midgut volvulus. Conversely, none of those with gastroschisis developed midgut volvulus [6]. However, because of the relatively low incidence of abdominal wall defects, we sought to expand on these data by performing a multi-institutional study. The aim of this study was to examine the occurrence of midgut volvulus in patients with abdominal wall defects and identify a group who may be at a greater risk. 1. Methods Data were collected from three participating institutions: Children's Hospital of Wisconsin (Milwaukee, WI), Children's Mercy Hospital (Kansas City, MO), and The Children's University Hospital (Dublin, Ireland). After institutional review board approval, charts of all patients with an ICD-9 diagnosis code for gastroschisis (756.73) and omphalocele (756.72) were retrospectively reviewed. Patients were included if their birth dates were between the ranges of 1/1/2000 through 12/31/2008. The patients' charts were reviewed including all emergency room visits,
http://dx.doi.org/10.1016/j.jpedsurg.2016.10.014 0022-3468/© 2016 Elsevier Inc. All rights reserved.
Please cite this article as: Fawley JA, et al, The risk of midgut volvulus in patients with abdominal wall defects: A multi-institutional study, J Pediatr Surg (2016), http://dx.doi.org/10.1016/j.jpedsurg.2016.10.014
2
J.A. Fawley et al. / Journal of Pediatric Surgery xxx (2016) xxx–xxx
hospital admissions, and outpatient clinic appointments, through 12/31/2012 to assess if these patients developed midgut volvulus. Patients were excluded if they had an abdominal wall defect that was not gastroschisis or omphalocele, if they died prior to surgical repair of their defect, or if their primary surgical repair occurred at an outside facility. Small omphalocele was defined as b 4 cm defect, and giant omphalocele was defined as N 4 cm defect or defect containing the liver [7]. Midgut volvulus was defined as twisting of the midgut around its mesentery in the setting of malrotated bowel without predisposing abnormalities including adhesions, congenital band, meconium ileus, or mass. Electronic medical records were reviewed for patient demographics, outcomes including details of surgical repair (primary vs delayed, need for patch, Ladd procedure completed on final repair), and surgical complications requiring a second unplanned laparotomy (midgut volvulus, adhesive bowel obstruction, internal hernia, abdominal compartment syndrome). Primary outcome was defined as the occurrence of midgut volvulus. Secondary outcomes were need for unplanned laparotomy and death. Data were collected and stored in the Research Electronic Data Capture (REDCap™) system. Patient factors and outcomes were compared between the gastroschisis and omphalocele groups using the chi-square test and Fisher's exact test for categorical variables and the Wilcoxon rank-sum test for continuous variables (* in Tables 1 and 4 indicate Fisher's exact test). P values ≤0.05 were considered statistically significant. All statistical analyses were performed using SAS 9.3 software (SAS Institute, Cary, NC). 2. Results During the defined study period, there were a total of 414 patients with an abdominal wall defect. Of these, 299 (72.2%) patients had a diagnosis of gastroschisis, while 115 (27.8%) had a diagnosis of omphalocele. Of those with omphaloceles, 54 (50.0%) had a small omphalocele, while 36 (31.3%) had a giant omphalocele. There were 25 (21.7%) with an unknown size of the omphalocele. Neonatal characteristics are summarized in Table 1. The mean gestational age at delivery was 36.1 ± 2.3 weeks. Those with gastroschisis were delivered earlier than those with an omphalocele (36.0 ± 1.9 vs 36.5 ± 2.9; p = 0.001). The mean birth weight was 2.57 ± 0.7 kg in gastroschisis patients having a statistically significantly lower birth weight (2.49 ± 0.55 vs 2.90 ± 0.9; p = b 0.0001). Furthermore, the mortality rate was statistically greater in those with an omphalocele (4.0% vs 11.3%; p = 0.005) (Table 2). In the study cohort, there were a total of 8/414 (1.9%) subjects who developed midgut volvulus (Table 3). The operation performed was reduction of volvulus and bowel resection as indicated. There was a statistically significant difference in the occurrence of midgut volvulus between those with gastroschsis and those with omphalocele.
In omphalocele patients, 5/115 (4.4%) developed midgut volvulus, whereas 3/299 (1.0%) of subjects with gastroschisis developed midgut volvulus (p = 0.04). Of the omphalocele patients who developed midgut volvulus, 2/5 (40%) were categorized as small and 3/5 (60%) had giant omphaloceles. Furthermore, the median age that midgut volvulus occurred was 1098 days (range, 21–2285 days). However, the occurrence of midgut volvulus tended to cluster at two time points. Four of the patients had midgut volvulus within or just after the first year of age. The other four developed midgut volvulus around 5–6 years of age. Three of those who developed midgut volvulus required a bowel resection. However, volvulus was not a cause of short bowel syndrome for any patients. Additionally, volvulus was not a cause of mortality in this cohort. Only 8/414 (5.1%) of the study cohort underwent a prophylactic Ladd procedure at some point during repair of the abdominal wall defect. There was a difference in the rates of Ladd procedures between the two groups. Of those with gastroschisis, 3/299 (1.0%) had a Ladd procedure, whereas 5/115 (4.3%) of those with omphaloceles underwent a Ladd procedure. All Ladd procedures were performed on an elective basis. The decision to perform a Ladd procedure seemed to be solely based on surgeon preference. All Ladd procedures were performed at definitive closure. Furthermore, a single institution in the study performed all 8 of the Ladd procedures (Children's Hospital of Wisconsin). None of the patients who underwent a Ladd procedure later developed midgut volvulus. Additionally, none of those who underwent a Ladd procedure required a laparotomy secondary to an adhesive bowel obstruction. A significant portion of the study cohort required a laparotomy for nonvolvulus-related complications (Table 4). A total of 57/414 (13.7%) required a laparotomy for any complication with an equal rate between gastroschisis and omphaloceles (14.0% vs 13.0%; p = 0.874). The most common complication among the entire cohort requiring a laparotomy was adhesive bowel obstruction occurring in 25/414 (6.0%) of the total population. There was not a difference in the rate of adhesive bowel obstruction between gastroschisis and omphalocele (6.6% vs 4.4%; p = 0.491). However, the rate of adhesive bowel obstruction was equal to that of midgut volvulus in patients with omphalocele (4.4% vs 4.4%). This was not the case for patients with gastroschisis, with bowel obstruction being 6 times more common (6.6% vs 1.0%). 3. Discussion Midgut volvulus is a catastrophic complication of malrotation, which is inherent in patients with abdominal wall defects. Although the association between congenital abdominal wall defects and intestinal rotational anomalies is well established, performing a Ladd procedure is not standard of care [2–5]. This is because of operative challenges and perhaps the belief that adhesions are sufficient enough to provide bowel fixation [2,3].
Table 1 Demographics. Patient demographics Sex Male Female Gestational age Birth weight Ethnicity Caucasian African American Hispanic Asian Other/unknown Midgut volvulus Neonatal death Ladd procedure for incidental malrotation
Total (n = 414)
Gastroschisis (n = 299)
Omphalocele (n = 115)
204 (49.3%) 210 (50.7%) 36.1 ± 2.3 2.57 ± 0.7
143 (47.8%) 156 (52.2%) 36.0 ± 1.9 2.49 ± 0.55
61 (53.0%) 54 (47.0%) 36.5 ± 2.9 2.90 ± 0.9
278 (67.1%) 48 (11.6%) 46 (11.1%) 11 (2.7%) 31 (7.5%) 8 (1.9%) 25 (6.0%) 8 (1.9%)
195 (65.2%) 32 (10.7%) 41 (13.7%) 9 (3.0%) 22 (7.4%) 3 (1.0%) 12 (4.0%) 3 (1.0%)
83 (72.2%) 16 (13.9%) 5 (4.4%) 2 (1.7%) 9 (7.8%) 5 (4.4%) 13 (11.3%) 5 (4.3%)
p-value 0.3416
0.0013 b0.0001 0.0773
0.0408* 0.0053 0.0408
Please cite this article as: Fawley JA, et al, The risk of midgut volvulus in patients with abdominal wall defects: A multi-institutional study, J Pediatr Surg (2016), http://dx.doi.org/10.1016/j.jpedsurg.2016.10.014
J.A. Fawley et al. / Journal of Pediatric Surgery xxx (2016) xxx–xxx
3
Table 2 Causes of mortality. Gastroschisis
Omphalocele
Total number
Cause of mortality (n)
Total number
Cause of mortality (n)
12/299 (4.0%)
Cardiac failure (1) Pseudomonas peritonitis (1) Short bowel syndrome (2) Hemorrhagic complications of liver failure (2) Liver failure (2) NEC (2) Unknown (2)
13/115 (11.3%)
Pentalogy of Cantrell, withdrawal of care (1) Ruptured giant omphalocele, sepsis (1) Abdominal compartment syndrome, multiorgan failure (1) NEC (2) Sepsis (2) Respiratory failure (4) Unknown (2)
Table 3 Midgut volvulus characteristics. Diagnosis
Type of repair
Small omphalocele Giant omphalocele Giant omphalocele Giant omphalocele Small omphalocele Gastroschisis
Primary 21 Delayed primary closure at 1 year 1954 Primary (fascial reapproximation at 1 yr) 2109 Primary closure (skin only) 250 Primary 59 Primary 1802
Age at the time of midgut volvulus (days) Bowel ischemia or resection Other GI complications No 60 cm bowel resection Bowel resection No No Bowel resection
Gastroschisis Gastroschisis
Silo Silo
No No
394 2285
Patients with gastroschisis may have more protective adhesions than those with omphalocele. Gastroschisis patients have violation of the peritoneal cavity as a result of their disease process. The bowel is exposed to amniotic fluid during intrauterine life, which may be associated with bowel inflammation and fibrinous peel formation. Furthermore, these patients have frequent handling of the bowel during repair. The incidence of midgut volvulus in the general population ranges from 1.7 to 60 per 100,000 [8]. In the current study, all diagnoses of midgut volvulus were based on the surgeons reporting of intraoperative findings. Midgut volvulus occurred in 1% of those with gastroschisis. However, midgut volvulus occurred 4 times more frequently in those with omphalocele with a rate of 4.4%. This is about 67 times more frequent than the highest estimations of the general public. In addition, the rate of volvulus in those with omphalocele was equivalent to the rate of adhesive bowel obstruction. This finding was not observed in those with gastroschisis. The rate of adhesive bowel obstruction was six times more frequent than the rate of volvulus in gastroschisis patients. These data may support the hypothesis that the disease process of gastroschsis allows for greater bowel fixation as evidenced by the decreased rate of midgut volvulus and suggest that patients with omphalocele may glean greater benefit from a Ladd procedure. However, while patients with omphalocele may benefit from a Ladd procedure, there are challenges worth addressing. One of the operative challenges in omphalocele is visceroabdominal disproportion. These patients lack abdominal domain and as a result, there is not enough peritoneal space to accommodate the return of abdominal contents. As the child grows, the peritoneal space increases and
No No No No No Complete SBO with bowel resection; short gut syndrome No No
allows for return of the abdominal contents. During this time of growth, the integrity of the omphalocele sac is critical, as there is an increased morbidity with violation of the sac [9]. Therefore, the benefit of violating the omphalocele sac to perform a Ladd procedure would have to outweigh the risk. Our data do not support this. Every case of midgut volvulus occurred after completion of definitive repair. Furthermore, in the general population, up to 75% of volvulus occurs within the first month of life and another 15% occurring within the first year of life [10,11]. In our study population, 63% of cases of midgut volvulus occurred outside of the first year of life. Based on these findings, it would be acceptable to keep the omphalocele sac intact and perform a Ladd procedure during definitive repair. A true Ladd's procedure involves the steps of taking Ladd's bands down; widening the mesentery; placing the small bowel on the right, colon in the left; and an appendectomy. Based on review of the operative notes, there was a subset of gastroschisis patients who had the mesentery widened at the time of closure but did not have the other steps of a Ladd's procedure completed. These patients were not counted as having a Ladd's in the current study. However, performing a Ladd procedure is not itself without risk. One of the consequences of performing a Ladd procedure is the formation of adhesions. This can lead to bowel obstruction and in severe cases, bowel ischemia. Therefore, increasing the frequency of Ladd procedures in this patient population may increase the incidence of small bowel obstruction. Therefore, while there is benefit in performing a Ladd procedure to mitigate the risk of midgut volvulus, one must be cognizant that this may increase the risk of future small bowel obstruction.
Table 4 Complication-related laparotomy.
Need for laparotomy for complications Findings at laparotomy Volvulus Obstruction secondary to adhesions Stricture or atresia Perforation or NEC Enterocutaneous fistula Abdominal compartment syndrome Internal hernia
Total (n = 414)
Gastroschisis (n = 299)
Omphalocele (n = 115)
p-value
57 (13.7%)
42 (14.0%)
15 (13.0%)
0.874
8 (1.9%) 25 (6.0%) 9 (2.2%) 8 (1.9%) 2 (0.5%) 1 (0.25%) 4 (1.0%)
3 (1.0%) 20 (6.6%) 7 (2.3%) 7 (2.3%) 1 (0.33%) 0 (0%) 4 (1.3%)
5 (4.4%) 5 (4.4%) 2 (1.7%) 1 (0.9%) 1 (0.9%) 1 (0.9%) 0 (0%)
0.0408* 0.491* 1.0* 0.452* 0.478* 0.277* 0.579*
Please cite this article as: Fawley JA, et al, The risk of midgut volvulus in patients with abdominal wall defects: A multi-institutional study, J Pediatr Surg (2016), http://dx.doi.org/10.1016/j.jpedsurg.2016.10.014
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We acknowledge that a major limitation of this study is its retrospective design. We performed a chart review of all emergency room visits, hospital admissions, and outpatient clinic visits during the defined study period. However, patients did not have additional follow-up specifically to assess for the development of midgut volvulus. It is possible that subjects in this study could have developed midgut volvulus that was managed by another institution. Despite these limitations, to our knowledge, this is the largest study to date examining the occurrence of midgut volvulus in patients with congenital abdominal wall defects.
4. Conclusion The management of intestinal rotational anomalies in congenital abdominal wall defects has varied largely because of lack of evidence. However, patients with omphalocele are at a greater risk of developing midgut volvulus. A Ladd procedure should be considered for these patients during definitive repair to mitigate the risk of midgut volvulus.
References [1] Kluth D, Jaeschke-Melli S, Fiegel H. The embryology of gut rotation. Semin Pediatr Surg 2003;4:275–9. [2] Abdelhafeez A, Alagtal M, Tareen F, et al. The incidence of symptomatic malrotation post gastroschisis repair. Eur J Pediatr Surg 2011;6:375–6. [3] Rescorla FJ, Shedd FJ, Grosfeld JL, et al. Anomalies of intestinal rotation in childhood: analysis of 447 cases. Surgery 1990;4:710–5 [discussion 715-6]. [4] Pacilli M, Spitz L, Kiely EM, et al. Staged repair of giant omphalocele in the neonatal period. J Pediatr Surg 2005;5:785–8. [5] Sinha CK, Kader M, Dykes E, et al. An 18 years' review of exomphalos highlighting the association with malrotation. Pediatr Surg Int 2011;11:1151–4. [6] Abdelhafeez AH, Schultz JA, Ertl A, et al. The risk of volvulus in abdominal wall defects. J Pediatr Surg 2015;4:570–2. [7] Kumar HR, Jester AL, Ladd AP. Impact of omphalocele size on associated conditions. J Pediatr Surg 2008;12:2216–9. [8] Iwuagwu O, Deans GT. Small bowel volvulus: a review. J R Coll Surg Edinb 1999;3:150–5. [9] Baird R, Gholoum S, Laberge JM, et al. Management of a giant omphalocele with an external skin closure system. J Pediatr Surg 2010;7:E17–20. [10] Ford EG, Senac Jr MO, Srikanth MS, et al. Malrotation of the intestine in children. Ann Surg 1992;2:172–8. [11] Stewart DR, Colodny AL, Daggett WC. Malrotation of the bowel in infants and children: a 15 year review. Surgery 1976;6:716–20.
Please cite this article as: Fawley JA, et al, The risk of midgut volvulus in patients with abdominal wall defects: A multi-institutional study, J Pediatr Surg (2016), http://dx.doi.org/10.1016/j.jpedsurg.2016.10.014
Contents lists available at ScienceDirect
Journal of Pediatric Surgery journal homepage: www.elsevier.com/locate/jpedsurg
The risk of midgut volvulus in patients with abdominal wall defects: A multi-institutional study Jason A Fawley a,b, Abdelhafeez H. Abdelhafeez c, Jessica A. Schultz a,b, Allison Ertl a, Laura D. Cassidy a,b, Shawn St Peter d, Amy J. Wagner a,b,⁎ a
Medical College of Wisconsin, Milwaukee, WI Children's Hospital of Wisconsin, Milwaukee, WI The Children's University Hospital, Dublin, Ireland d Children's Mercy Hospital, Kansas City, MO b c
a r t i c l e
i n f o
Article history: Received 29 September 2016 Accepted 20 October 2016 Available online xxxx Key words: Midgut volvulus Omphalocele Gastroschisis Ladd procedure
a b s t r a c t Background: The management of malrotation in patients with congenital abdominal wall defects has varied among surgeons. We were interested in investigating the risk of midgut volvulus in patients with gastroschisis and omphalocele to help determine if these patients may benefit from undergoing a Ladd procedure. Methods: A retrospective chart review was performed for all patients managed at three institutions born between 1/1/2000 and 12/31/2008 with a diagnosis of gastroschisis or omphalocele. Patient charts were reviewed through 12/31/2012 for occurrence of midgut volvulus or need for second laparotomy. Results: Of the 414 patients identified with abdominal wall defects, 299 patients (72%) had gastroschisis, and 115 patients (28%) had omphalocele. The mean gestational age at birth was 36.1 ± 2.3 weeks, and the mean birth weight was 2.57 ± 0.7 kg. There were a total of 8 (1.9%) cases of midgut volvulus: 3 (1.0%) patients with gastroschisis compared to 5 patients (4.4%) with omphalocele (p = 0.04). Conclusions: Patients with omphalocele have a greater risk of developing midgut volvulus, and a Ladd procedure should be considered during definitive repair to mitigate these risks. Level of evidence: III; retrospective comparative study © 2016 Elsevier Inc. All rights reserved.
The normal rotation of the intestine follows precise embryologic events that result in a fixed intestinal configuration with the duodenojejunal junction in the left upper quadrant and the cecum in the right lower quadrant [1]. Normal intestinal configuration allows for the midgut to be anchored by a wide mesenteric base. Patients with abdominal wall defects have a disruption in embryologic rotational events, which results in malrotation of the intestines with potential for a narrow mesenteric base and midgut volvulus. Midgut volvulus is a devastating event associated with significant morbidity and mortality. The Ladd procedure is the procedure of choice for correction of malrotation. Performing a Ladd procedure in patients with abdominal wall defects is variable among surgeons [2–5]. This variability is a result of the belief that adhesions from repair of the abdominal wall defect should be sufficient to provide bowel fixation and prevent midgut volvulus. Additionally, manipulation of friable bowel may pose risk to the patient [2,3]. However, the ability of adhesions to provide bowel fixation remains uncertain. There is little evidence regarding the incidence of midgut volvulus in patients with abdominal wall defects.
⁎ Corresponding author at: Medical College of Wisconsin, Division of Pediatric Surgery, 999 N 92nd Street Suite 320, Milwaukee, WI 53226. Tel.: +1 414 266 6550. E-mail address: [email protected] (A.J. Wagner).
Furthermore, gastroschisis patients may have more protective adhesions because of inflammation and increased handling. However, this is not true in the case of omphalocele and may predispose these patients to midgut volvulus. In a single-institution study examining midgut volvulus in subjects with abdominal wall defects, 3% of those with omphaloceles developed midgut volvulus. Conversely, none of those with gastroschisis developed midgut volvulus [6]. However, because of the relatively low incidence of abdominal wall defects, we sought to expand on these data by performing a multi-institutional study. The aim of this study was to examine the occurrence of midgut volvulus in patients with abdominal wall defects and identify a group who may be at a greater risk. 1. Methods Data were collected from three participating institutions: Children's Hospital of Wisconsin (Milwaukee, WI), Children's Mercy Hospital (Kansas City, MO), and The Children's University Hospital (Dublin, Ireland). After institutional review board approval, charts of all patients with an ICD-9 diagnosis code for gastroschisis (756.73) and omphalocele (756.72) were retrospectively reviewed. Patients were included if their birth dates were between the ranges of 1/1/2000 through 12/31/2008. The patients' charts were reviewed including all emergency room visits,
http://dx.doi.org/10.1016/j.jpedsurg.2016.10.014 0022-3468/© 2016 Elsevier Inc. All rights reserved.
Please cite this article as: Fawley JA, et al, The risk of midgut volvulus in patients with abdominal wall defects: A multi-institutional study, J Pediatr Surg (2016), http://dx.doi.org/10.1016/j.jpedsurg.2016.10.014
2
J.A. Fawley et al. / Journal of Pediatric Surgery xxx (2016) xxx–xxx
hospital admissions, and outpatient clinic appointments, through 12/31/2012 to assess if these patients developed midgut volvulus. Patients were excluded if they had an abdominal wall defect that was not gastroschisis or omphalocele, if they died prior to surgical repair of their defect, or if their primary surgical repair occurred at an outside facility. Small omphalocele was defined as b 4 cm defect, and giant omphalocele was defined as N 4 cm defect or defect containing the liver [7]. Midgut volvulus was defined as twisting of the midgut around its mesentery in the setting of malrotated bowel without predisposing abnormalities including adhesions, congenital band, meconium ileus, or mass. Electronic medical records were reviewed for patient demographics, outcomes including details of surgical repair (primary vs delayed, need for patch, Ladd procedure completed on final repair), and surgical complications requiring a second unplanned laparotomy (midgut volvulus, adhesive bowel obstruction, internal hernia, abdominal compartment syndrome). Primary outcome was defined as the occurrence of midgut volvulus. Secondary outcomes were need for unplanned laparotomy and death. Data were collected and stored in the Research Electronic Data Capture (REDCap™) system. Patient factors and outcomes were compared between the gastroschisis and omphalocele groups using the chi-square test and Fisher's exact test for categorical variables and the Wilcoxon rank-sum test for continuous variables (* in Tables 1 and 4 indicate Fisher's exact test). P values ≤0.05 were considered statistically significant. All statistical analyses were performed using SAS 9.3 software (SAS Institute, Cary, NC). 2. Results During the defined study period, there were a total of 414 patients with an abdominal wall defect. Of these, 299 (72.2%) patients had a diagnosis of gastroschisis, while 115 (27.8%) had a diagnosis of omphalocele. Of those with omphaloceles, 54 (50.0%) had a small omphalocele, while 36 (31.3%) had a giant omphalocele. There were 25 (21.7%) with an unknown size of the omphalocele. Neonatal characteristics are summarized in Table 1. The mean gestational age at delivery was 36.1 ± 2.3 weeks. Those with gastroschisis were delivered earlier than those with an omphalocele (36.0 ± 1.9 vs 36.5 ± 2.9; p = 0.001). The mean birth weight was 2.57 ± 0.7 kg in gastroschisis patients having a statistically significantly lower birth weight (2.49 ± 0.55 vs 2.90 ± 0.9; p = b 0.0001). Furthermore, the mortality rate was statistically greater in those with an omphalocele (4.0% vs 11.3%; p = 0.005) (Table 2). In the study cohort, there were a total of 8/414 (1.9%) subjects who developed midgut volvulus (Table 3). The operation performed was reduction of volvulus and bowel resection as indicated. There was a statistically significant difference in the occurrence of midgut volvulus between those with gastroschsis and those with omphalocele.
In omphalocele patients, 5/115 (4.4%) developed midgut volvulus, whereas 3/299 (1.0%) of subjects with gastroschisis developed midgut volvulus (p = 0.04). Of the omphalocele patients who developed midgut volvulus, 2/5 (40%) were categorized as small and 3/5 (60%) had giant omphaloceles. Furthermore, the median age that midgut volvulus occurred was 1098 days (range, 21–2285 days). However, the occurrence of midgut volvulus tended to cluster at two time points. Four of the patients had midgut volvulus within or just after the first year of age. The other four developed midgut volvulus around 5–6 years of age. Three of those who developed midgut volvulus required a bowel resection. However, volvulus was not a cause of short bowel syndrome for any patients. Additionally, volvulus was not a cause of mortality in this cohort. Only 8/414 (5.1%) of the study cohort underwent a prophylactic Ladd procedure at some point during repair of the abdominal wall defect. There was a difference in the rates of Ladd procedures between the two groups. Of those with gastroschisis, 3/299 (1.0%) had a Ladd procedure, whereas 5/115 (4.3%) of those with omphaloceles underwent a Ladd procedure. All Ladd procedures were performed on an elective basis. The decision to perform a Ladd procedure seemed to be solely based on surgeon preference. All Ladd procedures were performed at definitive closure. Furthermore, a single institution in the study performed all 8 of the Ladd procedures (Children's Hospital of Wisconsin). None of the patients who underwent a Ladd procedure later developed midgut volvulus. Additionally, none of those who underwent a Ladd procedure required a laparotomy secondary to an adhesive bowel obstruction. A significant portion of the study cohort required a laparotomy for nonvolvulus-related complications (Table 4). A total of 57/414 (13.7%) required a laparotomy for any complication with an equal rate between gastroschisis and omphaloceles (14.0% vs 13.0%; p = 0.874). The most common complication among the entire cohort requiring a laparotomy was adhesive bowel obstruction occurring in 25/414 (6.0%) of the total population. There was not a difference in the rate of adhesive bowel obstruction between gastroschisis and omphalocele (6.6% vs 4.4%; p = 0.491). However, the rate of adhesive bowel obstruction was equal to that of midgut volvulus in patients with omphalocele (4.4% vs 4.4%). This was not the case for patients with gastroschisis, with bowel obstruction being 6 times more common (6.6% vs 1.0%). 3. Discussion Midgut volvulus is a catastrophic complication of malrotation, which is inherent in patients with abdominal wall defects. Although the association between congenital abdominal wall defects and intestinal rotational anomalies is well established, performing a Ladd procedure is not standard of care [2–5]. This is because of operative challenges and perhaps the belief that adhesions are sufficient enough to provide bowel fixation [2,3].
Table 1 Demographics. Patient demographics Sex Male Female Gestational age Birth weight Ethnicity Caucasian African American Hispanic Asian Other/unknown Midgut volvulus Neonatal death Ladd procedure for incidental malrotation
Total (n = 414)
Gastroschisis (n = 299)
Omphalocele (n = 115)
204 (49.3%) 210 (50.7%) 36.1 ± 2.3 2.57 ± 0.7
143 (47.8%) 156 (52.2%) 36.0 ± 1.9 2.49 ± 0.55
61 (53.0%) 54 (47.0%) 36.5 ± 2.9 2.90 ± 0.9
278 (67.1%) 48 (11.6%) 46 (11.1%) 11 (2.7%) 31 (7.5%) 8 (1.9%) 25 (6.0%) 8 (1.9%)
195 (65.2%) 32 (10.7%) 41 (13.7%) 9 (3.0%) 22 (7.4%) 3 (1.0%) 12 (4.0%) 3 (1.0%)
83 (72.2%) 16 (13.9%) 5 (4.4%) 2 (1.7%) 9 (7.8%) 5 (4.4%) 13 (11.3%) 5 (4.3%)
p-value 0.3416
0.0013 b0.0001 0.0773
0.0408* 0.0053 0.0408
Please cite this article as: Fawley JA, et al, The risk of midgut volvulus in patients with abdominal wall defects: A multi-institutional study, J Pediatr Surg (2016), http://dx.doi.org/10.1016/j.jpedsurg.2016.10.014
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Table 2 Causes of mortality. Gastroschisis
Omphalocele
Total number
Cause of mortality (n)
Total number
Cause of mortality (n)
12/299 (4.0%)
Cardiac failure (1) Pseudomonas peritonitis (1) Short bowel syndrome (2) Hemorrhagic complications of liver failure (2) Liver failure (2) NEC (2) Unknown (2)
13/115 (11.3%)
Pentalogy of Cantrell, withdrawal of care (1) Ruptured giant omphalocele, sepsis (1) Abdominal compartment syndrome, multiorgan failure (1) NEC (2) Sepsis (2) Respiratory failure (4) Unknown (2)
Table 3 Midgut volvulus characteristics. Diagnosis
Type of repair
Small omphalocele Giant omphalocele Giant omphalocele Giant omphalocele Small omphalocele Gastroschisis
Primary 21 Delayed primary closure at 1 year 1954 Primary (fascial reapproximation at 1 yr) 2109 Primary closure (skin only) 250 Primary 59 Primary 1802
Age at the time of midgut volvulus (days) Bowel ischemia or resection Other GI complications No 60 cm bowel resection Bowel resection No No Bowel resection
Gastroschisis Gastroschisis
Silo Silo
No No
394 2285
Patients with gastroschisis may have more protective adhesions than those with omphalocele. Gastroschisis patients have violation of the peritoneal cavity as a result of their disease process. The bowel is exposed to amniotic fluid during intrauterine life, which may be associated with bowel inflammation and fibrinous peel formation. Furthermore, these patients have frequent handling of the bowel during repair. The incidence of midgut volvulus in the general population ranges from 1.7 to 60 per 100,000 [8]. In the current study, all diagnoses of midgut volvulus were based on the surgeons reporting of intraoperative findings. Midgut volvulus occurred in 1% of those with gastroschisis. However, midgut volvulus occurred 4 times more frequently in those with omphalocele with a rate of 4.4%. This is about 67 times more frequent than the highest estimations of the general public. In addition, the rate of volvulus in those with omphalocele was equivalent to the rate of adhesive bowel obstruction. This finding was not observed in those with gastroschisis. The rate of adhesive bowel obstruction was six times more frequent than the rate of volvulus in gastroschisis patients. These data may support the hypothesis that the disease process of gastroschsis allows for greater bowel fixation as evidenced by the decreased rate of midgut volvulus and suggest that patients with omphalocele may glean greater benefit from a Ladd procedure. However, while patients with omphalocele may benefit from a Ladd procedure, there are challenges worth addressing. One of the operative challenges in omphalocele is visceroabdominal disproportion. These patients lack abdominal domain and as a result, there is not enough peritoneal space to accommodate the return of abdominal contents. As the child grows, the peritoneal space increases and
No No No No No Complete SBO with bowel resection; short gut syndrome No No
allows for return of the abdominal contents. During this time of growth, the integrity of the omphalocele sac is critical, as there is an increased morbidity with violation of the sac [9]. Therefore, the benefit of violating the omphalocele sac to perform a Ladd procedure would have to outweigh the risk. Our data do not support this. Every case of midgut volvulus occurred after completion of definitive repair. Furthermore, in the general population, up to 75% of volvulus occurs within the first month of life and another 15% occurring within the first year of life [10,11]. In our study population, 63% of cases of midgut volvulus occurred outside of the first year of life. Based on these findings, it would be acceptable to keep the omphalocele sac intact and perform a Ladd procedure during definitive repair. A true Ladd's procedure involves the steps of taking Ladd's bands down; widening the mesentery; placing the small bowel on the right, colon in the left; and an appendectomy. Based on review of the operative notes, there was a subset of gastroschisis patients who had the mesentery widened at the time of closure but did not have the other steps of a Ladd's procedure completed. These patients were not counted as having a Ladd's in the current study. However, performing a Ladd procedure is not itself without risk. One of the consequences of performing a Ladd procedure is the formation of adhesions. This can lead to bowel obstruction and in severe cases, bowel ischemia. Therefore, increasing the frequency of Ladd procedures in this patient population may increase the incidence of small bowel obstruction. Therefore, while there is benefit in performing a Ladd procedure to mitigate the risk of midgut volvulus, one must be cognizant that this may increase the risk of future small bowel obstruction.
Table 4 Complication-related laparotomy.
Need for laparotomy for complications Findings at laparotomy Volvulus Obstruction secondary to adhesions Stricture or atresia Perforation or NEC Enterocutaneous fistula Abdominal compartment syndrome Internal hernia
Total (n = 414)
Gastroschisis (n = 299)
Omphalocele (n = 115)
p-value
57 (13.7%)
42 (14.0%)
15 (13.0%)
0.874
8 (1.9%) 25 (6.0%) 9 (2.2%) 8 (1.9%) 2 (0.5%) 1 (0.25%) 4 (1.0%)
3 (1.0%) 20 (6.6%) 7 (2.3%) 7 (2.3%) 1 (0.33%) 0 (0%) 4 (1.3%)
5 (4.4%) 5 (4.4%) 2 (1.7%) 1 (0.9%) 1 (0.9%) 1 (0.9%) 0 (0%)
0.0408* 0.491* 1.0* 0.452* 0.478* 0.277* 0.579*
Please cite this article as: Fawley JA, et al, The risk of midgut volvulus in patients with abdominal wall defects: A multi-institutional study, J Pediatr Surg (2016), http://dx.doi.org/10.1016/j.jpedsurg.2016.10.014
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J.A. Fawley et al. / Journal of Pediatric Surgery xxx (2016) xxx–xxx
We acknowledge that a major limitation of this study is its retrospective design. We performed a chart review of all emergency room visits, hospital admissions, and outpatient clinic visits during the defined study period. However, patients did not have additional follow-up specifically to assess for the development of midgut volvulus. It is possible that subjects in this study could have developed midgut volvulus that was managed by another institution. Despite these limitations, to our knowledge, this is the largest study to date examining the occurrence of midgut volvulus in patients with congenital abdominal wall defects.
4. Conclusion The management of intestinal rotational anomalies in congenital abdominal wall defects has varied largely because of lack of evidence. However, patients with omphalocele are at a greater risk of developing midgut volvulus. A Ladd procedure should be considered for these patients during definitive repair to mitigate the risk of midgut volvulus.
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Please cite this article as: Fawley JA, et al, The risk of midgut volvulus in patients with abdominal wall defects: A multi-institutional study, J Pediatr Surg (2016), http://dx.doi.org/10.1016/j.jpedsurg.2016.10.014