Journal of Pediatric Surgery 50 (2015) 570–572
Contents lists available at ScienceDirect
Journal of Pediatric Surgery journal homepage: www.elsevier.com/locate/jpedsurg
The risk of volvulus in abdominal wall defects☆ Abdelhafeez H. Abdelhafeez a,b,⁎, Jessica A. Schultz a,b, Allison Ertl a, Laura D. Cassidy a,b, Amy J. Wagner a,b a b
Medical College of Wisconsin, Milwaukee, WI, USA Children's Hospital of Wisconsin, Milwaukee, WI, USA
a r t i c l e
i n f o
Article history: Received 23 April 2014 Received in revised form 22 December 2014 Accepted 22 December 2014 Key words: Abdominal wall defects Gastroschisis Omphalocele Ladd’s Volvulus
a b s t r a c t Background: Congenital abdominal wall defects are associated with abnormal intestinal rotation and fixation. A Ladd's procedure is not routinely performed in these patients; it is believed intestinal fixation is provided by adhesions that develop post-repair of the defects. However, patients with omphalocele may not have adequately protective postoperative adhesions because of difference in the inflammatory state of the bowel wall and in repair strategy. The aim of this study is to describe the occurrence of midgut volvulus in patients with gastroschisis or omphalocele. Methods: A retrospective chart review was performed for all patients managed in a single institution 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 206 patients identified with abdominal wall defects, 142 patients (69%) had gastroschisis and 64 patients (31%) had omphalocele. Patients' follow up ranged from 4 years to 13 years. The median gestational age was 36 weeks (26–41 weeks) and the median birth weight was 2.42 kg (0.8–4.87 kg). None of the patients with gastroschisis developed midgut volvulus, however two patients (3%) with omphalocele developed midgut volvulus. Conclusions: No patients with gastroschisis developed midgut volvulus. Therefore, the current practice of not routinely performing a Ladd's procedure is a safe approach during surgical repair of gastroschisis. The two cases of volvulus in patients with omphalocele may be related to less bowel fixation. It is necessary to examine current practice in regards to the need for assessing the risk of volvulus during omphalocele closure and counseling of these patients. This assessment may be achieved via routine examination of the width of the small bowel mesenteric base, whenever feasible; however, the sample size is relatively small to draw any definitive conclusions. Published by Elsevier Inc.
Midgut volvulus is a catastrophic event associated with mortality and severe morbidities. Abnormal bowel rotation and fixation are the underlying causes of midgut volvulus that can lead to bowel necrosis, short bowel syndrome, and death. The association between congenital abdominal wall defects and abnormal bowel rotation is well established. As a result, patients with abdominal wall defects are at risk of developing midgut volvulus [1]. However, the incomplete documentation of bowel rotation status in the patient records in many retrospective studies limited the ability to accurately assess the exact incidence of malrotation [2–5]. Ladd’s procedure is the standard operative intervention to prevent midgut volvulus in the setting of malrotation [6]. Current practice, in regards to prevention of midgut volvulus in patients with abdominal wall defects, is variable [2–5]. A Ladd’s procedure is not routinely performed in the majority of these patients as it is thought that post repair adhesions are enough to provide bowel fixation and prevent midgut volvulus [2,3]. Moreover, the practical operative challenges faced when managing patients with large omphalocele and gastroschisis at ☆ No funding was received to produce this research. ⁎ Corresponding author at: Division of Pediatric Surgery, Medical College of Wisconsin, Children's Hospital of Wisconsin. Tel.: +1 414 702 2077; fax: +1 414 266 6579. E-mail address: [email protected] (A.H. Abdelhafeez). http://dx.doi.org/10.1016/j.jpedsurg.2014.12.017 0022-3468/Published by Elsevier Inc.
the initial management phase may make a Ladd’s procedure hazardous. The lack of both abdominal domain and a suitable abdominal wall substitute curtails operative goals in many patients with large omphalocele to avoid breach of the sac and stimulation of its epithelialization until satisfactory growth of the abdominal cavity is established. In patients with gastroschisis, the small size of the defect and the associated inflammation and fragility of the bowel wall make a Ladd’s procedure an unsafe endeavor. This is not the case in small omphalocele, since their abdominal domain is adequate and their bowel wall is normal. There is little evidence in the literature regarding the incidence of midgut volvulus in patients with abdominal wall defects. Gastroschisis patients may have more protective postoperative adhesions because the initial repair involves handling of the bowel and violation of the peritoneal cavity. Additionally, the exposure to amniotic fluid prenatally is associated with bowel wall inflammation and fibrinous peel formation and may also induce adhesion formation in gastroschisis patients. Conversely, the peritoneal cavity and bowel of patients with omphalocele may be subjected to less handling and less stimulation of adhesion formation when able to undergo primary closure [4]. Giant omphalocele patients treated with desiccation techniques to allow epithelialization will not have any manipulation of the bowel. The bowel wall of patients with an intact omphalocele sac is not exposed to the amniotic fluid and does not show fibrinous peel or inflammatory
A.H. Abdelhafeez et al. / Journal of Pediatric Surgery 50 (2015) 570–572
571
Table 1 Gastroschisis vs. omphalocele demographic data.
Prenatal diagnosis Cardiac defectsa (not including PFO and PDA) Associated complex anomalies ASA score (median, range) Hospital length of stay (days) (median, range) Age at final repair (days) (median, range) Mortality a
Gastroschisis (N = 142)
Omphalocele (N = 64)
p-value
Total (N = 206)
122 (86%) 6 (4%) 13 (9%) 3 (3–5) 27 (3–184) 0 (0–49) 7 (5%)
50 (78%) 21 (33%) 20 (31%) 3 (1–5) 19 (0–882) 2 (0–2874) 7 (11%)
0.1634 b0.0001 b0.0001 0.1967 0.0091 0.0001 0.1369
172 (84%) 27 (13%) 33 (16%) 3 (1–5) 26 (0–882) 1 (0–2874) 14 (6%)
n = 204.
changes. The aim of this study was to examine the occurrence of midgut volvulus in patients with abdominal wall defects and to determine whether this is higher in patients with omphalocele. 1. Methods After Institutional Review Board approval (CHW IRB #13/53), data were abstracted from medical records for patients with the ICD-9 diagnosis code for gastroschisis, omphalocele or other congenital anomalies of the abdominal wall with birth dates ranging from 1/1/2000 through 12/31/2008 and follow-up data were obtained through 12/31/2012. Patients were excluded if their diagnosis was not gastroschisis or omphalocele, if they received only palliative management, or if their primary surgical repair occurred at an outside facility. Small omphalocele was defined as b 4 cm defect and large omphalocele was defined as N 4 cm defect. Primary repair is attempted in all patients with small omphalocele or gastroschisis. Silo bags were used when primary repair was not safe. Patients with large omphalocele were treated with desiccation at the initial stage, and then delayed repair was planned when their abdominal domain growth was judged to be adequate. Data were collected and stored in the Research Electronic Data Capture (REDCap TM) system [7]. Patient factors were compared between the gastroschisis and omphalocele groups using the chi-square test for categorical variables and the Wilcoxon rank-sum test for continuous variables. P values ≤ 0.05 were considered statistically significant. All statistical analysis was performed using SAS 9.3 software (SAS Institute, Cary, NC). 2. Results Out of the 237 possible patients, 206 patients with abdominal wall defects met the inclusion criteria. The median gestational age was 36 weeks (26–41 weeks) and the median birth weight was 2.42 kg (0.8–4.87 kg). A prenatal diagnosis was established in 172 patients (84%). Patients with omphalocele had a significantly higher percentage of cardiac defects, than patients with gastroschisis (33% vs. 4%). There were also a higher percentage of associated complex anomalies in patients with omphalocele than gastroschisis (31% vs. 9%). Patients with gastroschisis had a significantly longer hospital stay (p = 0.0091) and a shorter time to final repair (p = 0.0001), mostly within the first three days of life (Table 1). There were 142 patients diagnosed with gastroschisis (69%) and 64 patients had omphalocele (31%), of which 39 patients had small defect (61%), mostly repaired primarily except 2 patients who died prior to repair, and 23 patients (39%) had large omphalocele, all were managed initially with desiccation. Primary repair was feasible in 93 patients with gastroschisis, while 48 patients required initial silo bag insertion and 1 patient died prior to repair. Length of survivors’ follow-up ranged from 4 years to 13 years and the median was 7.4 years. Subsequent laparotomy was required in 35 patients (17%) to treat complications, mostly for adhesive small bowel obstruction (Table 2). Ladd’s procedure was performed prophylactically in 20 patients (9%), none of which developed volvulus. It was not clear why these 20 patients had prophylactic Ladd’s procedure, however this
appeared to be due to interpersonal practice variability. These Ladd’s procedures were performed at the time of laparotomies for a different indication, mostly at the time of division of adhesion for bowel obstruction. None of the patients with gastroschisis developed midgut volvulus, and two patients (3%) with omphalocele developed midgut volvulus (Table 3). Postnatal volvulus was not a cause of mortality or short bowel syndrome in this group, however it resulted in a 60 cm bowel resection in one of the patients. The mortality of patients with omphalocele was 11% and primarily due to associated cardiac defects. Mortality in gastroschisis patients was 5% and primarily due to bowel loss and sepsis. Five patients received only palliative management and were excluded, 60% of these had severe associated complex anomalies and none had volvulus.
3. Discussion The overall risk of midgut volvulus in abdominal wall defects is low, however it is relatively higher in patients with omphalocele. Even though the percentage of midgut volvulus in patients with omphalocele still seems low, the associated mortality and morbidity make it a clinically significant problem. The incidence in the general population is much lower ranging from 1.7 to 60 per 100,000 [8]. We acknowledge the single-institution nature of this study as being a limitation; however, it is second to only one study that also included more than 200 patients [3]. Rescola and colleagues, more than two decades ago, examined the incidence of volvulus in 220 patients with abdominal wall defects. They reported two cases of volvulus in patients with omphalocele (2.7%) with 100% mortality of those two patients. None of their gastroschisis patients developed volvulus [3]. Considering the identical findings of two equivalently large single-institution studies (i.e. our study and the Rescola et al. study), we can afford to make some careful generalizations; nonetheless, a contemporary multi-institution, multinational study is required to further validate any conclusion. Given our location as a tertiary care facility and the area we serve, it can be reasonably assumed that patients would come back to our center for a crisis such as midgut volvulus. Table 2 Ladd’s procedure, complications and mortality. Gastroschisis (N = 142) Ladd’s procedure for incidental malrotation Need for laparotomy for complications: Findings at laparotomy: Volvulus Obstruction secondary to adhesions Stricture or atresia Perforation or NEC Enterocutaneous fistula Abdominal compartment syndrome
Omphalocele (N = 64)
Total (N = 206)
14 (9%)
6 (9%)
20 (9%)
25 (18%)
10 (16%)
35 (17%)
0 16 (11%)
2 (3%) 4 (6%)
2 (1%) 20 (9%)
6 (4%) 3 (2%) 0 0
1 (1%) 1 (1%) 1 (1%) 1 (1%)
7 (3%) 4 (2%) 1 (0.5%) 1 (0.5%)
572
A.H. Abdelhafeez et al. / Journal of Pediatric Surgery 50 (2015) 570–572
Table 3 Demographic data of the two patients who developed midgut volvulus. Diagnosis
Type of repair
Age at the time of midgut volvulus
Bowel ischemia or resection
Other GI complications
Small omphalocele Large omphalocele
Primary Delayed stage repair at age 1 year
3 weeks 5 years
No Ischemia, 60 cm bowel resection
No No
The risk of midgut volvulus is related to the width of the mesenteric base rather than the mere finding of abnormal rotation. The main objective of a Ladd’s procedure is to broaden a narrow mesenteric base. Interestingly, some patients with abnormal bowel rotation will have a relatively wide mesenteric base and, as a result, low mechanical risk for volvulus and perhaps less benefit of Ladd’s procedure [9]. Although determining the width of the base of the mesentery would subject bowel to further handling and may increase the incidence of ileus and postoperative adhesions, we feel that examination of the mesenteric base should be performed at the time of initial repair in patients with small omphalocele as it is technically straightforward and the benefits outweigh the risks. The risks of violating the abdominal cavity in large omphalocele at the initial management phase outweigh the benefit of examining the bowel mesentery in patients managed with topical desiccation technique. However, the mesenteric base should be examined and addressed appropriately at subsequent staged repair when there is satisfactory abdominal domain growth. Our data suggest that the benefits of examining the mesenteric base at the time of repair of gastroschisis is questionable since the occurrence of midgut volvulus is very low, and excessive physical manipulation of friable inflamed bowel wall is hazardous. The risk of volvulus should be discussed with all families who have a child with an abdominal wall defect. This should be emphasized to those who are being managed with desiccation of the sac as timely presentation in the event of midgut volvulus could be life saving. We feel that patients with omphalocele and narrow bowel mesentery should have a Ladd’s procedure when technically feasible and safe; either at
the time of primary closure in patients with small defects or at the time of staged closure in large omphalocele. A multicenter study should be performed to increase the sample size and to generate a more accurate estimate of the difference in the incidence of midgut volvulus between gastroschisis and omphalocele. References [1] Stockmann PT. Malrotation. In: Oldham KT, Colombani PM, Foglia RP, Skinner MA, editors. Principles and practice of pediatric surgery. Philadelphia: Lippincott Williams & Wilkins; 2005. p. 1287–8. [2] Abdelhafeez A, Alagtal M, Tareen F, et al. The incidence of symptomatic malrotation post gastroschisis repair. Eur J Pediatr Surg 2011;21:375–6. http://dx.doi.org/10. 1055/s-0031-1286342. [3] Rescorla FJ, Shedd FJ, Grosfeld JL, et al. Anomalies of intestinal rotation in childhood: analysis of 447 cases. Surgery 1990;108:710–5 [discussion 715–16]. [4] Pacilli M, Spitz L, Kiely EM, et al. Staged repair of giant omphalocele in the neonatal period. J Pediatr Surg 2005;40:785–8. [5] Sinha CK, Kader M, Dykes E, et al. An 18 years' review of exomphalos highlighting the association with malrotation. Pediatr Surg Int 2011;27:1151–4. http://dx.doi.org/10. 1007/s00383-011-2930-4. [6] Danny CL, Samuel DS. Malrotation. In: Ashcraft KW, Holcomb GW, Murphy JP, editors. Pediatric surgery. Philadelphia: Saunders Elsevier; 2010. p. 416–24. [7] Harris PA, Taylor R, Thielke R, et al. Research electronic data capture (REDCap) — a metadata-driven methodology and workflow process for providing translational research informatics support. J Biomed Inform 2009;42:377–81. http://dx.doi.org/10. 1016/j.jbi.2008.08.010. [8] Iwuagwu O, Deans GT. Small bowel volvulus: a review. J R Coll Surg Edinb 1999;44: 150–5. [9] Newman B, Koppolu R, Murphy D, et al. Heterotaxy syndromes and abnormal bowel rotation. Pediatr Radiol 2014;44:542–51. http://dx.doi.org/10.1007/s00247-0132861-4.
Contents lists available at ScienceDirect
Journal of Pediatric Surgery journal homepage: www.elsevier.com/locate/jpedsurg
The risk of volvulus in abdominal wall defects☆ Abdelhafeez H. Abdelhafeez a,b,⁎, Jessica A. Schultz a,b, Allison Ertl a, Laura D. Cassidy a,b, Amy J. Wagner a,b a b
Medical College of Wisconsin, Milwaukee, WI, USA Children's Hospital of Wisconsin, Milwaukee, WI, USA
a r t i c l e
i n f o
Article history: Received 23 April 2014 Received in revised form 22 December 2014 Accepted 22 December 2014 Key words: Abdominal wall defects Gastroschisis Omphalocele Ladd’s Volvulus
a b s t r a c t Background: Congenital abdominal wall defects are associated with abnormal intestinal rotation and fixation. A Ladd's procedure is not routinely performed in these patients; it is believed intestinal fixation is provided by adhesions that develop post-repair of the defects. However, patients with omphalocele may not have adequately protective postoperative adhesions because of difference in the inflammatory state of the bowel wall and in repair strategy. The aim of this study is to describe the occurrence of midgut volvulus in patients with gastroschisis or omphalocele. Methods: A retrospective chart review was performed for all patients managed in a single institution 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 206 patients identified with abdominal wall defects, 142 patients (69%) had gastroschisis and 64 patients (31%) had omphalocele. Patients' follow up ranged from 4 years to 13 years. The median gestational age was 36 weeks (26–41 weeks) and the median birth weight was 2.42 kg (0.8–4.87 kg). None of the patients with gastroschisis developed midgut volvulus, however two patients (3%) with omphalocele developed midgut volvulus. Conclusions: No patients with gastroschisis developed midgut volvulus. Therefore, the current practice of not routinely performing a Ladd's procedure is a safe approach during surgical repair of gastroschisis. The two cases of volvulus in patients with omphalocele may be related to less bowel fixation. It is necessary to examine current practice in regards to the need for assessing the risk of volvulus during omphalocele closure and counseling of these patients. This assessment may be achieved via routine examination of the width of the small bowel mesenteric base, whenever feasible; however, the sample size is relatively small to draw any definitive conclusions. Published by Elsevier Inc.
Midgut volvulus is a catastrophic event associated with mortality and severe morbidities. Abnormal bowel rotation and fixation are the underlying causes of midgut volvulus that can lead to bowel necrosis, short bowel syndrome, and death. The association between congenital abdominal wall defects and abnormal bowel rotation is well established. As a result, patients with abdominal wall defects are at risk of developing midgut volvulus [1]. However, the incomplete documentation of bowel rotation status in the patient records in many retrospective studies limited the ability to accurately assess the exact incidence of malrotation [2–5]. Ladd’s procedure is the standard operative intervention to prevent midgut volvulus in the setting of malrotation [6]. Current practice, in regards to prevention of midgut volvulus in patients with abdominal wall defects, is variable [2–5]. A Ladd’s procedure is not routinely performed in the majority of these patients as it is thought that post repair adhesions are enough to provide bowel fixation and prevent midgut volvulus [2,3]. Moreover, the practical operative challenges faced when managing patients with large omphalocele and gastroschisis at ☆ No funding was received to produce this research. ⁎ Corresponding author at: Division of Pediatric Surgery, Medical College of Wisconsin, Children's Hospital of Wisconsin. Tel.: +1 414 702 2077; fax: +1 414 266 6579. E-mail address: [email protected] (A.H. Abdelhafeez). http://dx.doi.org/10.1016/j.jpedsurg.2014.12.017 0022-3468/Published by Elsevier Inc.
the initial management phase may make a Ladd’s procedure hazardous. The lack of both abdominal domain and a suitable abdominal wall substitute curtails operative goals in many patients with large omphalocele to avoid breach of the sac and stimulation of its epithelialization until satisfactory growth of the abdominal cavity is established. In patients with gastroschisis, the small size of the defect and the associated inflammation and fragility of the bowel wall make a Ladd’s procedure an unsafe endeavor. This is not the case in small omphalocele, since their abdominal domain is adequate and their bowel wall is normal. There is little evidence in the literature regarding the incidence of midgut volvulus in patients with abdominal wall defects. Gastroschisis patients may have more protective postoperative adhesions because the initial repair involves handling of the bowel and violation of the peritoneal cavity. Additionally, the exposure to amniotic fluid prenatally is associated with bowel wall inflammation and fibrinous peel formation and may also induce adhesion formation in gastroschisis patients. Conversely, the peritoneal cavity and bowel of patients with omphalocele may be subjected to less handling and less stimulation of adhesion formation when able to undergo primary closure [4]. Giant omphalocele patients treated with desiccation techniques to allow epithelialization will not have any manipulation of the bowel. The bowel wall of patients with an intact omphalocele sac is not exposed to the amniotic fluid and does not show fibrinous peel or inflammatory
A.H. Abdelhafeez et al. / Journal of Pediatric Surgery 50 (2015) 570–572
571
Table 1 Gastroschisis vs. omphalocele demographic data.
Prenatal diagnosis Cardiac defectsa (not including PFO and PDA) Associated complex anomalies ASA score (median, range) Hospital length of stay (days) (median, range) Age at final repair (days) (median, range) Mortality a
Gastroschisis (N = 142)
Omphalocele (N = 64)
p-value
Total (N = 206)
122 (86%) 6 (4%) 13 (9%) 3 (3–5) 27 (3–184) 0 (0–49) 7 (5%)
50 (78%) 21 (33%) 20 (31%) 3 (1–5) 19 (0–882) 2 (0–2874) 7 (11%)
0.1634 b0.0001 b0.0001 0.1967 0.0091 0.0001 0.1369
172 (84%) 27 (13%) 33 (16%) 3 (1–5) 26 (0–882) 1 (0–2874) 14 (6%)
n = 204.
changes. The aim of this study was to examine the occurrence of midgut volvulus in patients with abdominal wall defects and to determine whether this is higher in patients with omphalocele. 1. Methods After Institutional Review Board approval (CHW IRB #13/53), data were abstracted from medical records for patients with the ICD-9 diagnosis code for gastroschisis, omphalocele or other congenital anomalies of the abdominal wall with birth dates ranging from 1/1/2000 through 12/31/2008 and follow-up data were obtained through 12/31/2012. Patients were excluded if their diagnosis was not gastroschisis or omphalocele, if they received only palliative management, or if their primary surgical repair occurred at an outside facility. Small omphalocele was defined as b 4 cm defect and large omphalocele was defined as N 4 cm defect. Primary repair is attempted in all patients with small omphalocele or gastroschisis. Silo bags were used when primary repair was not safe. Patients with large omphalocele were treated with desiccation at the initial stage, and then delayed repair was planned when their abdominal domain growth was judged to be adequate. Data were collected and stored in the Research Electronic Data Capture (REDCap TM) system [7]. Patient factors were compared between the gastroschisis and omphalocele groups using the chi-square test for categorical variables and the Wilcoxon rank-sum test for continuous variables. P values ≤ 0.05 were considered statistically significant. All statistical analysis was performed using SAS 9.3 software (SAS Institute, Cary, NC). 2. Results Out of the 237 possible patients, 206 patients with abdominal wall defects met the inclusion criteria. The median gestational age was 36 weeks (26–41 weeks) and the median birth weight was 2.42 kg (0.8–4.87 kg). A prenatal diagnosis was established in 172 patients (84%). Patients with omphalocele had a significantly higher percentage of cardiac defects, than patients with gastroschisis (33% vs. 4%). There were also a higher percentage of associated complex anomalies in patients with omphalocele than gastroschisis (31% vs. 9%). Patients with gastroschisis had a significantly longer hospital stay (p = 0.0091) and a shorter time to final repair (p = 0.0001), mostly within the first three days of life (Table 1). There were 142 patients diagnosed with gastroschisis (69%) and 64 patients had omphalocele (31%), of which 39 patients had small defect (61%), mostly repaired primarily except 2 patients who died prior to repair, and 23 patients (39%) had large omphalocele, all were managed initially with desiccation. Primary repair was feasible in 93 patients with gastroschisis, while 48 patients required initial silo bag insertion and 1 patient died prior to repair. Length of survivors’ follow-up ranged from 4 years to 13 years and the median was 7.4 years. Subsequent laparotomy was required in 35 patients (17%) to treat complications, mostly for adhesive small bowel obstruction (Table 2). Ladd’s procedure was performed prophylactically in 20 patients (9%), none of which developed volvulus. It was not clear why these 20 patients had prophylactic Ladd’s procedure, however this
appeared to be due to interpersonal practice variability. These Ladd’s procedures were performed at the time of laparotomies for a different indication, mostly at the time of division of adhesion for bowel obstruction. None of the patients with gastroschisis developed midgut volvulus, and two patients (3%) with omphalocele developed midgut volvulus (Table 3). Postnatal volvulus was not a cause of mortality or short bowel syndrome in this group, however it resulted in a 60 cm bowel resection in one of the patients. The mortality of patients with omphalocele was 11% and primarily due to associated cardiac defects. Mortality in gastroschisis patients was 5% and primarily due to bowel loss and sepsis. Five patients received only palliative management and were excluded, 60% of these had severe associated complex anomalies and none had volvulus.
3. Discussion The overall risk of midgut volvulus in abdominal wall defects is low, however it is relatively higher in patients with omphalocele. Even though the percentage of midgut volvulus in patients with omphalocele still seems low, the associated mortality and morbidity make it a clinically significant problem. The incidence in the general population is much lower ranging from 1.7 to 60 per 100,000 [8]. We acknowledge the single-institution nature of this study as being a limitation; however, it is second to only one study that also included more than 200 patients [3]. Rescola and colleagues, more than two decades ago, examined the incidence of volvulus in 220 patients with abdominal wall defects. They reported two cases of volvulus in patients with omphalocele (2.7%) with 100% mortality of those two patients. None of their gastroschisis patients developed volvulus [3]. Considering the identical findings of two equivalently large single-institution studies (i.e. our study and the Rescola et al. study), we can afford to make some careful generalizations; nonetheless, a contemporary multi-institution, multinational study is required to further validate any conclusion. Given our location as a tertiary care facility and the area we serve, it can be reasonably assumed that patients would come back to our center for a crisis such as midgut volvulus. Table 2 Ladd’s procedure, complications and mortality. Gastroschisis (N = 142) Ladd’s procedure for incidental malrotation Need for laparotomy for complications: Findings at laparotomy: Volvulus Obstruction secondary to adhesions Stricture or atresia Perforation or NEC Enterocutaneous fistula Abdominal compartment syndrome
Omphalocele (N = 64)
Total (N = 206)
14 (9%)
6 (9%)
20 (9%)
25 (18%)
10 (16%)
35 (17%)
0 16 (11%)
2 (3%) 4 (6%)
2 (1%) 20 (9%)
6 (4%) 3 (2%) 0 0
1 (1%) 1 (1%) 1 (1%) 1 (1%)
7 (3%) 4 (2%) 1 (0.5%) 1 (0.5%)
572
A.H. Abdelhafeez et al. / Journal of Pediatric Surgery 50 (2015) 570–572
Table 3 Demographic data of the two patients who developed midgut volvulus. Diagnosis
Type of repair
Age at the time of midgut volvulus
Bowel ischemia or resection
Other GI complications
Small omphalocele Large omphalocele
Primary Delayed stage repair at age 1 year
3 weeks 5 years
No Ischemia, 60 cm bowel resection
No No
The risk of midgut volvulus is related to the width of the mesenteric base rather than the mere finding of abnormal rotation. The main objective of a Ladd’s procedure is to broaden a narrow mesenteric base. Interestingly, some patients with abnormal bowel rotation will have a relatively wide mesenteric base and, as a result, low mechanical risk for volvulus and perhaps less benefit of Ladd’s procedure [9]. Although determining the width of the base of the mesentery would subject bowel to further handling and may increase the incidence of ileus and postoperative adhesions, we feel that examination of the mesenteric base should be performed at the time of initial repair in patients with small omphalocele as it is technically straightforward and the benefits outweigh the risks. The risks of violating the abdominal cavity in large omphalocele at the initial management phase outweigh the benefit of examining the bowel mesentery in patients managed with topical desiccation technique. However, the mesenteric base should be examined and addressed appropriately at subsequent staged repair when there is satisfactory abdominal domain growth. Our data suggest that the benefits of examining the mesenteric base at the time of repair of gastroschisis is questionable since the occurrence of midgut volvulus is very low, and excessive physical manipulation of friable inflamed bowel wall is hazardous. The risk of volvulus should be discussed with all families who have a child with an abdominal wall defect. This should be emphasized to those who are being managed with desiccation of the sac as timely presentation in the event of midgut volvulus could be life saving. We feel that patients with omphalocele and narrow bowel mesentery should have a Ladd’s procedure when technically feasible and safe; either at
the time of primary closure in patients with small defects or at the time of staged closure in large omphalocele. A multicenter study should be performed to increase the sample size and to generate a more accurate estimate of the difference in the incidence of midgut volvulus between gastroschisis and omphalocele. References [1] Stockmann PT. Malrotation. In: Oldham KT, Colombani PM, Foglia RP, Skinner MA, editors. Principles and practice of pediatric surgery. Philadelphia: Lippincott Williams & Wilkins; 2005. p. 1287–8. [2] Abdelhafeez A, Alagtal M, Tareen F, et al. The incidence of symptomatic malrotation post gastroschisis repair. Eur J Pediatr Surg 2011;21:375–6. http://dx.doi.org/10. 1055/s-0031-1286342. [3] Rescorla FJ, Shedd FJ, Grosfeld JL, et al. Anomalies of intestinal rotation in childhood: analysis of 447 cases. Surgery 1990;108:710–5 [discussion 715–16]. [4] Pacilli M, Spitz L, Kiely EM, et al. Staged repair of giant omphalocele in the neonatal period. J Pediatr Surg 2005;40:785–8. [5] Sinha CK, Kader M, Dykes E, et al. An 18 years' review of exomphalos highlighting the association with malrotation. Pediatr Surg Int 2011;27:1151–4. http://dx.doi.org/10. 1007/s00383-011-2930-4. [6] Danny CL, Samuel DS. Malrotation. In: Ashcraft KW, Holcomb GW, Murphy JP, editors. Pediatric surgery. Philadelphia: Saunders Elsevier; 2010. p. 416–24. [7] Harris PA, Taylor R, Thielke R, et al. Research electronic data capture (REDCap) — a metadata-driven methodology and workflow process for providing translational research informatics support. J Biomed Inform 2009;42:377–81. http://dx.doi.org/10. 1016/j.jbi.2008.08.010. [8] Iwuagwu O, Deans GT. Small bowel volvulus: a review. J R Coll Surg Edinb 1999;44: 150–5. [9] Newman B, Koppolu R, Murphy D, et al. Heterotaxy syndromes and abnormal bowel rotation. Pediatr Radiol 2014;44:542–51. http://dx.doi.org/10.1007/s00247-0132861-4.
