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Noncommunicating isolated enteric duplication cyst in childhood
Journal of Pediatric Surgery (2009) 44, E9–E10
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Noncommunicating isolated enteric duplication cyst in childhood Punit Srivastava a , Ajay N Gangopadhyay a,⁎, Vijayendra kumar a , Vijay D. Upadhyaya a , Shiv P. Sharma a , Richa Jaiman b , Zaheer Hasan a a
Department of Pediatric Surgery IMS, BHU, Varanasi, 221005 UP, India Department of Surgery, S. Medical College, Agra 228005, India
b
Received 22 December 2008; revised 17 March 2009; accepted 18 March 2009
Key words: Noncommunicating; Isolated duplication cyst; Ileum
Abstract Duplications of the alimentary tract are spherical or tubular structures lined by epithelium similar to intestine that are firmly attached to or share the wall of the alimentary tract and have a common blood supply with the adjacent segment of the bowel. Completely isolated duplication cysts are an extremely rare variety of gastrointestinal duplications with their own exclusive blood supply and do not communicate with the intestine. There are only 4 cases reported in the English literature—3 in childhood and 1 in an adult, and all are male. We report a case of noncommunicating isolated ileal duplication cyst in a 3-year-old female child that presented as a mass in the right iliac fossa, mimicking an intussusception. The duplication had its own blood supply arising from the terminal ileal mesentery. This report describes the first female patient with this condition and reviews the English literature. © 2009 Elsevier Inc. All rights reserved.
Enteric duplication cysts are hollow, epithelium-lined, cystic, spherical, or tubular structures that are basically attached to the wall of the gastrointestinal tract (often sharing the serosa) and supplied by common mesenteric blood vessels. Completely isolated duplication cysts are an extremely rare variety of gastrointestinal duplications with their own blood supply and do not communicate with the normal bowel segment [1,2]. We present the first case of such an entity in a 3-year-old female.
1. Case report A 3-year-old female child weighing 12 kg presented to us with a history of recurrent pain in right lower abdomen ⁎ Corresponding author. E-mail address: [email protected] (A.N. Gangopadhyay). 0022-3468/$ – see front matter © 2009 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2009.03.041
Fig. 1 Isolated noncommunicating ileal duplication cyst with normal adjacent ileum.
E10
Fig. 2 Excised specimen of ileal duplication cyst with its own vascular pedicle.
of 6 months that was relieved by medication. The pain was not associated with vomiting or fever. One month previously, her mother noticed a vague abdominal mass in right lower abdomen. Abdominal examination revealed a large, mobile nontender mass present in the right iliac fossa extending toward umbilicus, which had bowel-like consistency mimicking intussusception. Abdominal ultrasound examination showed a distended tortuous bowel-like mass filled with thick fluid in right ileac fossa extending toward umbilicus. Upper gastrointestinal contrast study was not contributory. At laparotomy, there was large 25 × 6-cm tubular fluid-filled cystic bowel-like mass extending from 5 cm proximal to ileocecal junction toward the root of mesentery that was blind ended, which did not communicate with the bowel segment and had its own blood supply arising from ileocecal mesentery (Fig. 1). The entire cyst was excised without disturbing the normal bowel or mesenteric anatomy (Fig. 2). The histologic examination confirmed this was an enteric duplication cyst. The postoperative course was uneventful, and child was discharged and has remained well in follow-up.
2. Discussion Gastrointestinal duplications are uncommon congenital lesions that can occur anywhere from the mouth to the anus and have a reported incidence of 1 in 4500 [3]. Ladd [4] coined the term alimentary tract duplications in 1937, which more effectively described the clinical and pathologic aspects of these lesions. Duplications found in the small intestine are the most frequent site of enteric duplications and most commonly occur in the ileum. These usually share a common wall with the normal intestine and have a common blood supply so it is mandatory to remove the adjacent bowel segment along with duplication cyst. This is in contrast to
P. Srivastava et al. Meckel's diverticulum that occurs on the antimesenteric border of the ileum [1]. In our case, the isolated duplication cyst rested on the mesentery with a separate vascular pedicle and no luminal communication with the adjacent alimentary segments. Several hypotheses have been postulated for the pathogenesis of enteric duplications. The hypothesis of traction between gut endoderm and overlying structures suggests a mechanism of dorsal enteric duplication and diverticula formation [5]. Intrauterine ischemic insult to the bowel has also been suggested as a cause of duplications [6]. The accurate diagnosis of duplication cyst was made on the macroscopic appearance of bowel as well as the typical histopathologic characteristics, composed of distinct muscle wall layers and an epithelial lining. The clinical presentation of the cyst is variable, most are documented in the neonatal or infancy period, but in our case, the cyst remained asymptomatic for more than 2 years of life, and symptoms were subsequently dependent on enlargement in size or complication such as torsion on a pedicle, volvulus, rupture, and bleeding because of the presence of ectopic gastric and pancreatic mucosa [7]. Unrecognized, asymptomatic cysts may be the site of adenocarcinoma during adult life. The enteric duplication cyst can be associated with malrotation [8] but not in our case. [1]. The ideal treatment of alimentary tract duplications is complete resection, which is usually accomplished by resection of the adjacent bowel and mesentery. However, in the case of completely isolated duplication cysts, resection can be accomplished safely without requiring bowel resection as demonstrated in our case. As Menon et al [7] concluded in their report, we would also suggest that anatomical knowledge of this form of isolated duplication cyst would help surgeons choose the optimal surgical procedure without resection of adjacent bowel segment to lessen the risk of postoperative morbidity. Early surgical intervention is mandatory to prevent complication.
References [1] Sinha A, Ojha S, Sarin YK. Completely isolated, noncontiguous duplication cyst. Eur J Pediatr Surg 2006;16(2):127-9. [2] Lund DP. Alimentary tract duplications. In: Grosfeld JL, O’Neill JA, Fonkalsrud EW, Coran AG, editors. Pediatric surgery. 6th ed. St Louis (Mo): Mosby; 2006. p. 1389-98. [3] Schalamon J, Schleef J, Hollwarth ME. Experience with gastrointestinal duplications in childhood. Langenbeck's Arch Surg 2000;385:402-5. [4] Ladd WE. Duplications of the alimentary tract. South Med J 1937;30:363-71. [5] Skandalakis JE, Gray SW, editors. Embryology for surgeons. 2nd ed. Baltimore (Md): Williams and W and INS; 1994. p. 226-8. [6] Favara BE, Franciosi RA, Akers DR. Enteric duplications. 37cases: a vascular theory pathogenesis. Am J Dis Child 1971;122:501-6. [7] Menon P, Na KL, Rao A, Vaiphei K. Isolated enteric duplication cysts. J Pediatr Surg 2004;39(8):5-7. [8] Stringer MD, Spitz L, Abel R, et al. Management of alimentary tract duplication in children. Br J Surg 1995;82:74-8.
www.elsevier.com/locate/jpedsurg
Noncommunicating isolated enteric duplication cyst in childhood Punit Srivastava a , Ajay N Gangopadhyay a,⁎, Vijayendra kumar a , Vijay D. Upadhyaya a , Shiv P. Sharma a , Richa Jaiman b , Zaheer Hasan a a
Department of Pediatric Surgery IMS, BHU, Varanasi, 221005 UP, India Department of Surgery, S. Medical College, Agra 228005, India
b
Received 22 December 2008; revised 17 March 2009; accepted 18 March 2009
Key words: Noncommunicating; Isolated duplication cyst; Ileum
Abstract Duplications of the alimentary tract are spherical or tubular structures lined by epithelium similar to intestine that are firmly attached to or share the wall of the alimentary tract and have a common blood supply with the adjacent segment of the bowel. Completely isolated duplication cysts are an extremely rare variety of gastrointestinal duplications with their own exclusive blood supply and do not communicate with the intestine. There are only 4 cases reported in the English literature—3 in childhood and 1 in an adult, and all are male. We report a case of noncommunicating isolated ileal duplication cyst in a 3-year-old female child that presented as a mass in the right iliac fossa, mimicking an intussusception. The duplication had its own blood supply arising from the terminal ileal mesentery. This report describes the first female patient with this condition and reviews the English literature. © 2009 Elsevier Inc. All rights reserved.
Enteric duplication cysts are hollow, epithelium-lined, cystic, spherical, or tubular structures that are basically attached to the wall of the gastrointestinal tract (often sharing the serosa) and supplied by common mesenteric blood vessels. Completely isolated duplication cysts are an extremely rare variety of gastrointestinal duplications with their own blood supply and do not communicate with the normal bowel segment [1,2]. We present the first case of such an entity in a 3-year-old female.
1. Case report A 3-year-old female child weighing 12 kg presented to us with a history of recurrent pain in right lower abdomen ⁎ Corresponding author. E-mail address: [email protected] (A.N. Gangopadhyay). 0022-3468/$ – see front matter © 2009 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2009.03.041
Fig. 1 Isolated noncommunicating ileal duplication cyst with normal adjacent ileum.
E10
Fig. 2 Excised specimen of ileal duplication cyst with its own vascular pedicle.
of 6 months that was relieved by medication. The pain was not associated with vomiting or fever. One month previously, her mother noticed a vague abdominal mass in right lower abdomen. Abdominal examination revealed a large, mobile nontender mass present in the right iliac fossa extending toward umbilicus, which had bowel-like consistency mimicking intussusception. Abdominal ultrasound examination showed a distended tortuous bowel-like mass filled with thick fluid in right ileac fossa extending toward umbilicus. Upper gastrointestinal contrast study was not contributory. At laparotomy, there was large 25 × 6-cm tubular fluid-filled cystic bowel-like mass extending from 5 cm proximal to ileocecal junction toward the root of mesentery that was blind ended, which did not communicate with the bowel segment and had its own blood supply arising from ileocecal mesentery (Fig. 1). The entire cyst was excised without disturbing the normal bowel or mesenteric anatomy (Fig. 2). The histologic examination confirmed this was an enteric duplication cyst. The postoperative course was uneventful, and child was discharged and has remained well in follow-up.
2. Discussion Gastrointestinal duplications are uncommon congenital lesions that can occur anywhere from the mouth to the anus and have a reported incidence of 1 in 4500 [3]. Ladd [4] coined the term alimentary tract duplications in 1937, which more effectively described the clinical and pathologic aspects of these lesions. Duplications found in the small intestine are the most frequent site of enteric duplications and most commonly occur in the ileum. These usually share a common wall with the normal intestine and have a common blood supply so it is mandatory to remove the adjacent bowel segment along with duplication cyst. This is in contrast to
P. Srivastava et al. Meckel's diverticulum that occurs on the antimesenteric border of the ileum [1]. In our case, the isolated duplication cyst rested on the mesentery with a separate vascular pedicle and no luminal communication with the adjacent alimentary segments. Several hypotheses have been postulated for the pathogenesis of enteric duplications. The hypothesis of traction between gut endoderm and overlying structures suggests a mechanism of dorsal enteric duplication and diverticula formation [5]. Intrauterine ischemic insult to the bowel has also been suggested as a cause of duplications [6]. The accurate diagnosis of duplication cyst was made on the macroscopic appearance of bowel as well as the typical histopathologic characteristics, composed of distinct muscle wall layers and an epithelial lining. The clinical presentation of the cyst is variable, most are documented in the neonatal or infancy period, but in our case, the cyst remained asymptomatic for more than 2 years of life, and symptoms were subsequently dependent on enlargement in size or complication such as torsion on a pedicle, volvulus, rupture, and bleeding because of the presence of ectopic gastric and pancreatic mucosa [7]. Unrecognized, asymptomatic cysts may be the site of adenocarcinoma during adult life. The enteric duplication cyst can be associated with malrotation [8] but not in our case. [1]. The ideal treatment of alimentary tract duplications is complete resection, which is usually accomplished by resection of the adjacent bowel and mesentery. However, in the case of completely isolated duplication cysts, resection can be accomplished safely without requiring bowel resection as demonstrated in our case. As Menon et al [7] concluded in their report, we would also suggest that anatomical knowledge of this form of isolated duplication cyst would help surgeons choose the optimal surgical procedure without resection of adjacent bowel segment to lessen the risk of postoperative morbidity. Early surgical intervention is mandatory to prevent complication.
References [1] Sinha A, Ojha S, Sarin YK. Completely isolated, noncontiguous duplication cyst. Eur J Pediatr Surg 2006;16(2):127-9. [2] Lund DP. Alimentary tract duplications. In: Grosfeld JL, O’Neill JA, Fonkalsrud EW, Coran AG, editors. Pediatric surgery. 6th ed. St Louis (Mo): Mosby; 2006. p. 1389-98. [3] Schalamon J, Schleef J, Hollwarth ME. Experience with gastrointestinal duplications in childhood. Langenbeck's Arch Surg 2000;385:402-5. [4] Ladd WE. Duplications of the alimentary tract. South Med J 1937;30:363-71. [5] Skandalakis JE, Gray SW, editors. Embryology for surgeons. 2nd ed. Baltimore (Md): Williams and W and INS; 1994. p. 226-8. [6] Favara BE, Franciosi RA, Akers DR. Enteric duplications. 37cases: a vascular theory pathogenesis. Am J Dis Child 1971;122:501-6. [7] Menon P, Na KL, Rao A, Vaiphei K. Isolated enteric duplication cysts. J Pediatr Surg 2004;39(8):5-7. [8] Stringer MD, Spitz L, Abel R, et al. Management of alimentary tract duplication in children. Br J Surg 1995;82:74-8.