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Splenic and pancreatic torsion after giant omphalocele repair
Journal of Pediatric Surgery Case Reports 25 (2017) 40e43
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.jpscasereports.com
Splenic and pancreatic torsion after giant omphalocele repair Gilgamesh J. Eamer a, Yasser Alfraih b, Nina Stein c, Karen Bailey b, * a
Department of Surgery, 2D WC Mackenzie Health Sciences Centre, 8440 112 St NW, University of Alberta, Edmonton, Alberta, T6G 2B7, Canada Department of Surgery, Faculty of Health Sciences, McMaster University, Canada c Department of Radiology, Faculty of Health Sciences, McMaster University, Canada b
a r t i c l e i n f o Article history: Received 29 July 2017 Accepted 4 August 2017 Available online 7 August 2017
1. Introduction Giant omphalocele is a congenital defect resulting from the failure of intestinal rotation beyond the first stage of rotation resulting in failure of abdominal contents to re-enter the abdominal cavity [1] and the abdominal wall to close at the umbilical ring in utero [2,3]. It results in a 3-part membranous covering over eviscerated abdominal contents that can include all gastrointestinal organs including liver, pancreas and spleen [1]. Omphalocele is considered to be giant when the defect is larger than 5 cm. When the normally retroperitoneal organs are included, they become intraperitoneal and therefore lack their usual ligaments attaching them to adjacent structures. Omphalocele can be associated with conditions such as wandering spleen [4], gastric or midgut volvulus [5,6] and hepatic torsion [7]. The organs are also frequently not found in their usual intraabdominal locations [8]. The incidence ranges between 1 in 4000e6000 live births and carries a 13%e28% mortality [2,3,9]. Omphaloceles are frequently associated with other midline defects that can be either supra-umbilical (cephalic) or infra-umbilical (caudal). Frequency of associated defects range from 27% to 74% including chromosomal (Trisomy 13, 17, 18 and 21), multiple congenital anomalies and recognized conditions (including Beckwith-Wiedemann, Goltz and fetal valproate syndromes, OEIS and Pentalogy of Cantrell) [10]. Commonly associated anomalies include limb deficiencies, cardiac defects, diaphragmatic hernia, ileal atresia, renal agenesis and anal atresia [10]. It is also associated with lower maternal age and its prevalence may be increasing [10]. Here, we report a case of a splenic and distal pancreatic torsion in a child with a history of a giant omphalocele.
* Corresponding author. Division of Pediatric Surgery, McMaster Children's Hospital, 1200 Main St West, 4E4, Hamilton, ON, L8N 3Z5, Canada. E-mail address: [email protected] (K. Bailey).
2. Case report The patient is a 6-year-old boy who presented with an eight-day history of progressive left-lower quadrant abdominal pain out of keeping with physical exam findings. He was born at 37þ5 weeks gestational age by elective caesarean section with a prenatally diagnosed giant omphalocele. The omphalocele included his small and large bowel, stomach and liver. He had a bifid sternum inferiorly, but no other associated caudal or cephalic defects. He was initially treated with silver sulfadiazine cream and dressings in the newborn period. He subsequently underwent an abdominal wall reconstruction with Gore-Tex mesh to repair his resultant ventral hernia. He most recently underwent a planned staged abdominal wall closure with partial removal of the mesh and advancement of the left later abdominal wall muscles. This was performed approximately 3 months prior to presentation. The patient began experiencing left lower quadrant abdominal pain 8 days prior to presentation to the emergency department. He developed progressive left hip pain to the point that he was unable to walk due to the pain. During the course of this presentation, the patient was assessed by several physicians who were unable to identify a cause for either his hip or lower abdominal pain the day prior, but the pain had subsided prior to discharge home. When he returned to our centre his mother reported that he broke into sweats and frequently doubled over due to the pain despite his high pain tolerance. Furthermore, he was not his usual active self and was anorexic. He had not traveled recently, had no sick contacts and his vaccinations were up-to-date. He was not exposed to smoking in his household. He had prior episodes of pain that had been much less severe and had been occurring episodically since his last surgery 3 months prior. The episodes of pain were associated with nausea and non-bloody, non-bilious vomiting and we could not elicit a temporal relationship with the pain and any activity. He had an abdominal ultrasound and urinalysis three weeks before presenting; neither revealed any abnormal findings aside from intraabdominal organs that were not in their usual locations. In particular there was no evidence of cholelithiasis. Physical assessment revealed a boy who was small for his age, in obvious discomfort. His vital signs were stable and he was afebrile. Pulmonary and cardiac exams were unremarkable. He had a very tender board-like left abdomen with similar tenderness to his left
http://dx.doi.org/10.1016/j.epsc.2017.08.002 2213-5766/© 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
G.J. Eamer et al. / Journal of Pediatric Surgery Case Reports 25 (2017) 40e43
hip and left gluteus muscle. There was no costovertebral tenderness. He was able to move his legs in normal range of motion, although it did cause him significant discomfort. Laboratory investigations identified leukocytosis (24), elevated C-reactive protein (11.9) and a lipase above measurable levels (>1200), liver enzymes and bilirubin were within normal limits. Abdominal x-ray was unremarkable with no evidence of free air or obstruction. In consultation with radiology a computed tomography scan was obtained. His liver and spleen were ectopic (Fig. 1), and there was a splenic volvulus with 720-degree rotation of the splenic vessels including the pancreatic tail (Fig. 2). The spleen had no normal perfusion in keeping with splenic infarct most likely due to a wandering spleen which was located in the left lower abdomen (Fig. 3). There was also note of edematous changes to the pancreatic tail suggestive of pancreatitis without focal fluid collection. Our findings were discussed with the patient and his mother and operative and non-operative management was described. Due to the presence of mesh, the risk of prolonged involution time and the significant pain being experienced by the patient surgical management was undertaken. Ongoing staged anterior abdominal reconstruction presented a challenge for operative planning. Multiple previous midline incisions and the presence of a midline liver with overlying Gore-Tex mesh made a midline incision inappropriate. Left abdominal transverse incisions would place the blood supply to the skin flap required for reconstruction at risk. Consequently, we chose a curvilinear left sided incision that avoided the major visible vessels. There were minimal adhesions in the left hemi-abdomen and we easily delivered the spleen and pancreatic tail which had none of the usual splenic ligaments (Fig. 4). Detorting the spleen did not return blood flow to the torted spleen and pancreatic tail, so a splenectomy and distal pancreatectomy was performed. The splenic vessels were ligated with silk ties. The distal pancreas was divided with a vascular stapler and over sewn with a polypropylene suture. A drain was placed adjacent to the over-sewn pancreatic staple line. The post-operative course was unremarkable. The drain was removed on post-operative day three and he was discharged on
Fig. 1. Axial computed tomography of the upper abdomen. The liver and spleen are not closely associated with the hemi-diaphragms.
41
Fig. 2. Axial computed tomography of the mid abdomen demonstrates a whirlpool appearance of the splenic vessels.
post-operative day five. He was tolerating a regular diet, his pain was adequately controlled with oral acetaminophen and he was mobilizing with assistance. At his four-week follow-up the pain had resolved, he had returned to his usual activity level and had begun his post-splenectomy prophylaxis. Pathologic examination confirmed the intra-operative diagnosis: necrotic changes in both the spleen and pancreatic tail. 3. Discussion We conducted a systematic search using MEDLINE for omphalocele and spleen or wandering spleen or torsion or pancreatitis;
Fig. 3. Axial computed tomography demonstrating a midline liver with overlying Gore-Tex mesh and the infarcted ectopic spleen.
42
G.J. Eamer et al. / Journal of Pediatric Surgery Case Reports 25 (2017) 40e43
allowed a rapid and clear assessment of the underlying pathology. Surgical intervention on these patients with omphalocele can also be very challenging in light of multiple prior abdominal surgeries, implanted surgical mesh and the resultant adhesions. The congenital malformations associated with intra-abdominal adhesions, malrotation and defective abdominal wall significantly increases risks compared to a standard splenectomy and distal pancreatectomy. Additionally, ongoing reconstruction of the abdominal wall meant the blood supply to the skin flaps being used for repair were critical as further loss of abdominal domain due to flap necrosis could result in an uncorrectable abdominal wall defect. Fortunately, the surgical approach chosen on this case resulted in a straight forward surgery without complication during the immediate post operative period. 4. Conclusion
Fig. 4. Intraoperative image of spleen with volvulus incorporating the tail of the pancreas and left lower quadrant oblique incision.
we identified 24 articles, including three relevant articles [4,11,12]. We also searched the national library of medicine for omphalocele and splenic infarct and did identify additional reports of splenic torsion in wandering spleen [13e15] but no additional reports in omphalocele. Neither search identified previous reports with a similar presentation. The three case reports identified presented cases of torsion of a wandering spleen associated with omphalocele, however, none presented with pancreatitis in addition to splenic torsion. We also identified reports of wandering spleen causing pancreatic and splenic volvulus without a history of omphalocele [13e15]. It has also been demonstrated that wandering spleen is a known entity among patients born with giant omphaloceles [16] and is due, in part, to the non-rotation of the gastrointestinal tract in-utero. Wandering spleen and splenic torsion are particularly challenging to diagnose without imaging. The atypical location of the spleen and vague symptoms of abdominal pain without significant gastrointestinal symptoms can lead to significant confusion. In fact, all case reports of splenic torsion that we identified were not considered until after radiographic imaging had been completed. We have identified three case reports of splenic volvulus or torsion leading to pancreatitis and splenic necrosis and three reports of splenic torsion in the setting of omphalocele however there are no reports of the triad of torsion with pancreatitis and a history of omphalocele. Given the history of episodic pain in the preceding three months, it is likely that the patient was experiencing intermittent and self-limiting torsion of the spleen. Ultrasonography was previously unable to identify this as it was likely a transient event. Loss of blood flow would most likely only have been present during the painful episodes. During his acute admission, his peritonitic abdomen and young age would have made ultrasound difficult to perform. Additionally, the increase in lipase and the clinical findings suggested the main differential diagnosis was necrotising pancreatitis. Combined, these circumstances prompted computed tomography being the initial investigation to be performed, which
In summary, we report the case of a young boy with a wandering spleen secondary to a giant omphalocele who presented with atypical abdominal pain from a splenic and distal pancreatic torsion resulting in necrosis of the spleen and pancreatitis. Given the known association of wandering spleen and the possibility of abnormal location for all intra-abdominal organs in patients with giant omphalocele, it is important to develop a broad differential diagnosis, trust laboratory findings and consider organ systems that wouldn't typically cause pain in the location these patients report pain. When the clinical picture is unclear, it is crucial the seek the advice of a skilled pediatric radiologist to assist with further diagnostic work-up. Surgical management is also ideally performed by a skilled pediatric surgeon in light of the associated congenital anomalies. Conflicts of interest There are no conflicts to declare. References [1] Gardner CE, Durham NC. The surgical significance of anamolies of intestinal rotation. Ann Surg 1950;131:879e96. [2] Bauman B, Stephens D, Gershone H, Bongiorno C, Osterholm E, Acton R, et al. Management of giant omphaloceles: a systematic review of methods of staged surgical vs. nonoperative delayed closure. J Pediatr Surg 2016;51:1725e30. [3] Lee SL, Beyer TD, Kim SS, Waldhausen JHT, Healey PJ, Sawin RS, et al. Initial nonoperative management and delayed closure for treatment of giant omphaloceles. J Pediatr Surg 2006 Nov;41:1846e9. [4] Yilmaz O, Genc A, Ozcan T, Aygoren RS, Taneli C. Unusual association of omphalocele and wandering spleen. Eur Surg Res 2008;41:331e3. [5] Abdelhafeez AH, Schultz JA, Ertl A, Cassidy LD, Wagner AJ. The risk of volvulus in abdominal wall defects. J Pediatr Surg 2015;50:570e2. Elsevier B.V. [6] Fawley JA, Abdelhafeez AH, Schultz JA, Ertl A, Cassidy LD, Peter SS, et al. The risk of midgut volvulus in patients with abdominal wall defects: a multiinstitutional study. J Pediatr Surg 2017;52:26e9. Elsevier Inc. [7] Ammouche C, Moog R, Lacreuse I, Gomes C, Kauffmann I, Becmeur F. Volvulus du foie d’evolution fatale 16 mois apres la cure d'une omphalocele geante. Arch Pediatr 2012;19:260e3. [8] van Eijck FC, Klein WM, Boetes C, Aronson DC, Wijnen RMH. Has the liver and other visceral organs migrated to its normal position in children with giant omphalocele? A follow-up study with ultrasonography. Eur J Pediatr 2010 May 29;169:563e7. Springer-Verlag. [9] Fisher R, Attah A, Partington A, Dykes E. Impact of antenatal diagnosis on incidence and prognosis in abdominal wall defects. J Pediatr Surg 1996 Apr;31:538e41. [10] Stoll C, Alembik Y, Dott B, Roth M-P. Omphalocele and gastroschisis and associated malformations. Am J Med Genet Part A 2008 May 15;146A:1280e5. Wiley Subscription Services, Inc., A Wiley Company. [11] Williams JL, Bush D, Wright PG. Omphalocele and ectopic spleen. J Clin Ultrasound 1987 Jul 1;15:409e11. Wiley Subscription Services, Inc., A Wiley Company. [12] Sheikh F, Kim ME, Zamora IJ, Olutoye OO. Non-operative management of a rare diagnosis of splenic torsion in a child with a history of giant omphalocele: a case report and literature review. Patient Saf Surg BioMed Cent 2014 Mar 7;8:12.
G.J. Eamer et al. / Journal of Pediatric Surgery Case Reports 25 (2017) 40e43 [13] Aswani Y, Anandpara KM, Hira P. Wandering spleen with torsion causing pancreatic volvulus and associated intrathoracic gastric volvulus. An unusual triad and cause of acute abdominal pain. J Pancreas 2015;16:78e80. [14] Sheflin JR, Lee CM, Kretchmar KA. Torsion of wandering spleen and tail of pancreas. Am J Roentgenol 1984;142:100e1. [15] Jia LW, Hoong CW, Wei TK. Torsion of the wandering spleen and pancreatic
43
tail precipitating diabetic ketoacidosis in a patient with prader willi syndrome: a case report. J ASEAN Fed Endocr Soc 2016 May 31;31:45e9. [16] Masui D, Fukahori S, Asagiri K, Ishii S, Saikusa N, Hashizume N, et al. Wandering spleen associated with omphalocele in a neonate: an unusual case with non-operative management. J Pediatr Surg Case Rep 2017;24:8e11.
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.jpscasereports.com
Splenic and pancreatic torsion after giant omphalocele repair Gilgamesh J. Eamer a, Yasser Alfraih b, Nina Stein c, Karen Bailey b, * a
Department of Surgery, 2D WC Mackenzie Health Sciences Centre, 8440 112 St NW, University of Alberta, Edmonton, Alberta, T6G 2B7, Canada Department of Surgery, Faculty of Health Sciences, McMaster University, Canada c Department of Radiology, Faculty of Health Sciences, McMaster University, Canada b
a r t i c l e i n f o Article history: Received 29 July 2017 Accepted 4 August 2017 Available online 7 August 2017
1. Introduction Giant omphalocele is a congenital defect resulting from the failure of intestinal rotation beyond the first stage of rotation resulting in failure of abdominal contents to re-enter the abdominal cavity [1] and the abdominal wall to close at the umbilical ring in utero [2,3]. It results in a 3-part membranous covering over eviscerated abdominal contents that can include all gastrointestinal organs including liver, pancreas and spleen [1]. Omphalocele is considered to be giant when the defect is larger than 5 cm. When the normally retroperitoneal organs are included, they become intraperitoneal and therefore lack their usual ligaments attaching them to adjacent structures. Omphalocele can be associated with conditions such as wandering spleen [4], gastric or midgut volvulus [5,6] and hepatic torsion [7]. The organs are also frequently not found in their usual intraabdominal locations [8]. The incidence ranges between 1 in 4000e6000 live births and carries a 13%e28% mortality [2,3,9]. Omphaloceles are frequently associated with other midline defects that can be either supra-umbilical (cephalic) or infra-umbilical (caudal). Frequency of associated defects range from 27% to 74% including chromosomal (Trisomy 13, 17, 18 and 21), multiple congenital anomalies and recognized conditions (including Beckwith-Wiedemann, Goltz and fetal valproate syndromes, OEIS and Pentalogy of Cantrell) [10]. Commonly associated anomalies include limb deficiencies, cardiac defects, diaphragmatic hernia, ileal atresia, renal agenesis and anal atresia [10]. It is also associated with lower maternal age and its prevalence may be increasing [10]. Here, we report a case of a splenic and distal pancreatic torsion in a child with a history of a giant omphalocele.
* Corresponding author. Division of Pediatric Surgery, McMaster Children's Hospital, 1200 Main St West, 4E4, Hamilton, ON, L8N 3Z5, Canada. E-mail address: [email protected] (K. Bailey).
2. Case report The patient is a 6-year-old boy who presented with an eight-day history of progressive left-lower quadrant abdominal pain out of keeping with physical exam findings. He was born at 37þ5 weeks gestational age by elective caesarean section with a prenatally diagnosed giant omphalocele. The omphalocele included his small and large bowel, stomach and liver. He had a bifid sternum inferiorly, but no other associated caudal or cephalic defects. He was initially treated with silver sulfadiazine cream and dressings in the newborn period. He subsequently underwent an abdominal wall reconstruction with Gore-Tex mesh to repair his resultant ventral hernia. He most recently underwent a planned staged abdominal wall closure with partial removal of the mesh and advancement of the left later abdominal wall muscles. This was performed approximately 3 months prior to presentation. The patient began experiencing left lower quadrant abdominal pain 8 days prior to presentation to the emergency department. He developed progressive left hip pain to the point that he was unable to walk due to the pain. During the course of this presentation, the patient was assessed by several physicians who were unable to identify a cause for either his hip or lower abdominal pain the day prior, but the pain had subsided prior to discharge home. When he returned to our centre his mother reported that he broke into sweats and frequently doubled over due to the pain despite his high pain tolerance. Furthermore, he was not his usual active self and was anorexic. He had not traveled recently, had no sick contacts and his vaccinations were up-to-date. He was not exposed to smoking in his household. He had prior episodes of pain that had been much less severe and had been occurring episodically since his last surgery 3 months prior. The episodes of pain were associated with nausea and non-bloody, non-bilious vomiting and we could not elicit a temporal relationship with the pain and any activity. He had an abdominal ultrasound and urinalysis three weeks before presenting; neither revealed any abnormal findings aside from intraabdominal organs that were not in their usual locations. In particular there was no evidence of cholelithiasis. Physical assessment revealed a boy who was small for his age, in obvious discomfort. His vital signs were stable and he was afebrile. Pulmonary and cardiac exams were unremarkable. He had a very tender board-like left abdomen with similar tenderness to his left
http://dx.doi.org/10.1016/j.epsc.2017.08.002 2213-5766/© 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
G.J. Eamer et al. / Journal of Pediatric Surgery Case Reports 25 (2017) 40e43
hip and left gluteus muscle. There was no costovertebral tenderness. He was able to move his legs in normal range of motion, although it did cause him significant discomfort. Laboratory investigations identified leukocytosis (24), elevated C-reactive protein (11.9) and a lipase above measurable levels (>1200), liver enzymes and bilirubin were within normal limits. Abdominal x-ray was unremarkable with no evidence of free air or obstruction. In consultation with radiology a computed tomography scan was obtained. His liver and spleen were ectopic (Fig. 1), and there was a splenic volvulus with 720-degree rotation of the splenic vessels including the pancreatic tail (Fig. 2). The spleen had no normal perfusion in keeping with splenic infarct most likely due to a wandering spleen which was located in the left lower abdomen (Fig. 3). There was also note of edematous changes to the pancreatic tail suggestive of pancreatitis without focal fluid collection. Our findings were discussed with the patient and his mother and operative and non-operative management was described. Due to the presence of mesh, the risk of prolonged involution time and the significant pain being experienced by the patient surgical management was undertaken. Ongoing staged anterior abdominal reconstruction presented a challenge for operative planning. Multiple previous midline incisions and the presence of a midline liver with overlying Gore-Tex mesh made a midline incision inappropriate. Left abdominal transverse incisions would place the blood supply to the skin flap required for reconstruction at risk. Consequently, we chose a curvilinear left sided incision that avoided the major visible vessels. There were minimal adhesions in the left hemi-abdomen and we easily delivered the spleen and pancreatic tail which had none of the usual splenic ligaments (Fig. 4). Detorting the spleen did not return blood flow to the torted spleen and pancreatic tail, so a splenectomy and distal pancreatectomy was performed. The splenic vessels were ligated with silk ties. The distal pancreas was divided with a vascular stapler and over sewn with a polypropylene suture. A drain was placed adjacent to the over-sewn pancreatic staple line. The post-operative course was unremarkable. The drain was removed on post-operative day three and he was discharged on
Fig. 1. Axial computed tomography of the upper abdomen. The liver and spleen are not closely associated with the hemi-diaphragms.
41
Fig. 2. Axial computed tomography of the mid abdomen demonstrates a whirlpool appearance of the splenic vessels.
post-operative day five. He was tolerating a regular diet, his pain was adequately controlled with oral acetaminophen and he was mobilizing with assistance. At his four-week follow-up the pain had resolved, he had returned to his usual activity level and had begun his post-splenectomy prophylaxis. Pathologic examination confirmed the intra-operative diagnosis: necrotic changes in both the spleen and pancreatic tail. 3. Discussion We conducted a systematic search using MEDLINE for omphalocele and spleen or wandering spleen or torsion or pancreatitis;
Fig. 3. Axial computed tomography demonstrating a midline liver with overlying Gore-Tex mesh and the infarcted ectopic spleen.
42
G.J. Eamer et al. / Journal of Pediatric Surgery Case Reports 25 (2017) 40e43
allowed a rapid and clear assessment of the underlying pathology. Surgical intervention on these patients with omphalocele can also be very challenging in light of multiple prior abdominal surgeries, implanted surgical mesh and the resultant adhesions. The congenital malformations associated with intra-abdominal adhesions, malrotation and defective abdominal wall significantly increases risks compared to a standard splenectomy and distal pancreatectomy. Additionally, ongoing reconstruction of the abdominal wall meant the blood supply to the skin flaps being used for repair were critical as further loss of abdominal domain due to flap necrosis could result in an uncorrectable abdominal wall defect. Fortunately, the surgical approach chosen on this case resulted in a straight forward surgery without complication during the immediate post operative period. 4. Conclusion
Fig. 4. Intraoperative image of spleen with volvulus incorporating the tail of the pancreas and left lower quadrant oblique incision.
we identified 24 articles, including three relevant articles [4,11,12]. We also searched the national library of medicine for omphalocele and splenic infarct and did identify additional reports of splenic torsion in wandering spleen [13e15] but no additional reports in omphalocele. Neither search identified previous reports with a similar presentation. The three case reports identified presented cases of torsion of a wandering spleen associated with omphalocele, however, none presented with pancreatitis in addition to splenic torsion. We also identified reports of wandering spleen causing pancreatic and splenic volvulus without a history of omphalocele [13e15]. It has also been demonstrated that wandering spleen is a known entity among patients born with giant omphaloceles [16] and is due, in part, to the non-rotation of the gastrointestinal tract in-utero. Wandering spleen and splenic torsion are particularly challenging to diagnose without imaging. The atypical location of the spleen and vague symptoms of abdominal pain without significant gastrointestinal symptoms can lead to significant confusion. In fact, all case reports of splenic torsion that we identified were not considered until after radiographic imaging had been completed. We have identified three case reports of splenic volvulus or torsion leading to pancreatitis and splenic necrosis and three reports of splenic torsion in the setting of omphalocele however there are no reports of the triad of torsion with pancreatitis and a history of omphalocele. Given the history of episodic pain in the preceding three months, it is likely that the patient was experiencing intermittent and self-limiting torsion of the spleen. Ultrasonography was previously unable to identify this as it was likely a transient event. Loss of blood flow would most likely only have been present during the painful episodes. During his acute admission, his peritonitic abdomen and young age would have made ultrasound difficult to perform. Additionally, the increase in lipase and the clinical findings suggested the main differential diagnosis was necrotising pancreatitis. Combined, these circumstances prompted computed tomography being the initial investigation to be performed, which
In summary, we report the case of a young boy with a wandering spleen secondary to a giant omphalocele who presented with atypical abdominal pain from a splenic and distal pancreatic torsion resulting in necrosis of the spleen and pancreatitis. Given the known association of wandering spleen and the possibility of abnormal location for all intra-abdominal organs in patients with giant omphalocele, it is important to develop a broad differential diagnosis, trust laboratory findings and consider organ systems that wouldn't typically cause pain in the location these patients report pain. When the clinical picture is unclear, it is crucial the seek the advice of a skilled pediatric radiologist to assist with further diagnostic work-up. Surgical management is also ideally performed by a skilled pediatric surgeon in light of the associated congenital anomalies. Conflicts of interest There are no conflicts to declare. References [1] Gardner CE, Durham NC. The surgical significance of anamolies of intestinal rotation. Ann Surg 1950;131:879e96. [2] Bauman B, Stephens D, Gershone H, Bongiorno C, Osterholm E, Acton R, et al. Management of giant omphaloceles: a systematic review of methods of staged surgical vs. nonoperative delayed closure. J Pediatr Surg 2016;51:1725e30. [3] Lee SL, Beyer TD, Kim SS, Waldhausen JHT, Healey PJ, Sawin RS, et al. Initial nonoperative management and delayed closure for treatment of giant omphaloceles. J Pediatr Surg 2006 Nov;41:1846e9. [4] Yilmaz O, Genc A, Ozcan T, Aygoren RS, Taneli C. Unusual association of omphalocele and wandering spleen. Eur Surg Res 2008;41:331e3. [5] Abdelhafeez AH, Schultz JA, Ertl A, Cassidy LD, Wagner AJ. The risk of volvulus in abdominal wall defects. J Pediatr Surg 2015;50:570e2. Elsevier B.V. [6] Fawley JA, Abdelhafeez AH, Schultz JA, Ertl A, Cassidy LD, Peter SS, et al. The risk of midgut volvulus in patients with abdominal wall defects: a multiinstitutional study. J Pediatr Surg 2017;52:26e9. Elsevier Inc. [7] Ammouche C, Moog R, Lacreuse I, Gomes C, Kauffmann I, Becmeur F. Volvulus du foie d’evolution fatale 16 mois apres la cure d'une omphalocele geante. Arch Pediatr 2012;19:260e3. [8] van Eijck FC, Klein WM, Boetes C, Aronson DC, Wijnen RMH. Has the liver and other visceral organs migrated to its normal position in children with giant omphalocele? A follow-up study with ultrasonography. Eur J Pediatr 2010 May 29;169:563e7. Springer-Verlag. [9] Fisher R, Attah A, Partington A, Dykes E. Impact of antenatal diagnosis on incidence and prognosis in abdominal wall defects. J Pediatr Surg 1996 Apr;31:538e41. [10] Stoll C, Alembik Y, Dott B, Roth M-P. Omphalocele and gastroschisis and associated malformations. Am J Med Genet Part A 2008 May 15;146A:1280e5. Wiley Subscription Services, Inc., A Wiley Company. [11] Williams JL, Bush D, Wright PG. Omphalocele and ectopic spleen. J Clin Ultrasound 1987 Jul 1;15:409e11. Wiley Subscription Services, Inc., A Wiley Company. [12] Sheikh F, Kim ME, Zamora IJ, Olutoye OO. Non-operative management of a rare diagnosis of splenic torsion in a child with a history of giant omphalocele: a case report and literature review. Patient Saf Surg BioMed Cent 2014 Mar 7;8:12.
G.J. Eamer et al. / Journal of Pediatric Surgery Case Reports 25 (2017) 40e43 [13] Aswani Y, Anandpara KM, Hira P. Wandering spleen with torsion causing pancreatic volvulus and associated intrathoracic gastric volvulus. An unusual triad and cause of acute abdominal pain. J Pancreas 2015;16:78e80. [14] Sheflin JR, Lee CM, Kretchmar KA. Torsion of wandering spleen and tail of pancreas. Am J Roentgenol 1984;142:100e1. [15] Jia LW, Hoong CW, Wei TK. Torsion of the wandering spleen and pancreatic
43
tail precipitating diabetic ketoacidosis in a patient with prader willi syndrome: a case report. J ASEAN Fed Endocr Soc 2016 May 31;31:45e9. [16] Masui D, Fukahori S, Asagiri K, Ishii S, Saikusa N, Hashizume N, et al. Wandering spleen associated with omphalocele in a neonate: an unusual case with non-operative management. J Pediatr Surg Case Rep 2017;24:8e11.