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Isolated enteric duplication cysts
Isolated Enteric Duplication Cysts By Prema Menon, K.L.N. Rao, and Kim Vaiphei Chandigarh, India
Two rare cases of completely isolated duplication cysts are reported. A large but asymptomatic tubular cyst hanging from a narrow pedicle arising from the base of the terminal ileal mesentery was removed in a 6-day-old boy along with correction of associated malrotation. Multiple isolated duplication cysts located in the thorax and abdomen were excised in a 10-week-old infant.
Both children remained asymptomatic after surgery. J Pediatr Surg 39:E27. © 2004 Elsevier Inc. All rights reserved.
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cyst with no intraspinal component.3 The child also had multiple thoracic vertebral anomalies. The abdomen was explored first, and multiple cysts were found lying in the small bowel mesentery. The proximal cyst, lying close to the duodeno-jejunal flexure, was 4 ⫻ 2.5 cm long and had a cordlike blind extension into the pancreas. There was another 22-cm-long cyst in the ileal mesentery with multiple constricted areas. There were incomplete mucosal folds forming valvelike structures in the constricted areas. Both the cysts were excised without sacrificing any bowel, because they did not share any common wall with the adjacent intestine. The blood supply appeared to arise from the retroperitoneal region. The cysts contained thick, mucoid material. One week after the laparotomy, a right-sided thoracotomy was performed. A thick-walled, 5 ⫻ 4-cm mucus-filled cyst was removed from the paravertebral retropleural region. It had no attachment to any part of the gastrointestinal tract nor was it communicating with the spinal canal. The histopathology of all the cysts was typical of a duplication cyst with all the layers of intestine. The mucosa was denuded in large areas (Fig 3). The child is now 1.6 years old and is asymptomatic.
OMPLETELY ISOLATED duplication cysts are extremely rare, and only 2 cases have been reported in the literature so far, one in a 7-day-old baby and another in a 28-year-old man.1,2 We report 2 further cases, one of multiple cysts, which has not been reported in the literature so far. CASE REPORT
Case 1 A full-term newborn boy presented on day 6 of life with history of abdominal distension noticed since birth. The antenatal period had been uneventful. He was born to a gravida 2 mother by cesarean section for meconium-stained liquor with a birth weight of 3 kg. The baby had passed meconium and urine within 24 hours of birth. He was taking feedings normally and on examination was active and alert with a heart rate of 120 beats per minute. A large cystic, nontender mass with side-to-side mobility was palpable, which was causing significant abdominal distension and prominence of both the flanks. An ultrasonography (USG) and computed tomography (CT) scan confirmed an intraperitoneal cystic mass but could not comment on the origin or the exact nature of the swelling. At laparotomy, a tense tubular cystic mass resembling small bowel in external appearance and measuring 26.5 ⫻ 7 cm was found occupying the entire abdomen. It was extending from the right lumbar region to the left iliac fossa in an inverted U-shaped manner and was attached to the base of the terminal ileal mesentery by a narrow 4-cm-long pedicle. There was a small v-shaped defect in the mesentery at this site. The lesion was excised (Fig 1) in toto. There was associated malrotation for which Ladds’ procedure was done. The small bowel length was within normal limits. The cyst was unilocular and contained thin blood-stained fluid. The histopathologic examination showed an extremely thin wall with 2 distinct muscle layers and mostly denuded focally flattened lining epithelium, compatible with a duplication cyst (Fig 2). The postoperative period was uneventful. The child remains asymptomatic 8 months after surgery.
Case 2 A 10-week-old boy was admitted with an asymptomatic right-sided abdominal lump noticed since birth. The USG and CT scan showed multiple cysts in the abdomen as well as a mediastinal cystic mass in the right paravertebral gutter with walls characteristic of a duplication
Journal of Pediatric Surgery, Vol 39, No 8 (August), 2004: E27
INDEX WORDS: Completely isolated duplication cyst, abdominal masses, multiple duplication cysts, enteric duplications.
DISCUSSION
Duplication cysts are rare anomalies, which usually present during infancy. One of the accepted diagnostic criteria is the close proximity to some part of the gastrointestinal tract with which it also shares its blood supply. No discernible communication or connection with the adjacent alimentary tract but the presence of typical histopathologic features of a duplication cyst would qualify for a diagnosis of a completely isolated duplication cyst. Isolated, intraspinal, neurenteric cysts may also fall into the same group by this definition.4 In the 2 cases From the Departments of Pediatric Surgery and Histopathology, PostGraduate Institute of Medical Education and Research, Chandigarh, India. Address reprint requests to Dr K.L.N. Rao, Professor and Head, Department of Pediatric Surgery, P.G.I.M.E.R., Chandigarh, 160012, India. © 2004 Elsevier Inc. All rights reserved. 1531-5037/04/3908-0036$30.00/0 doi:10.1016/j.jpedsurg.2004.04.040
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of secretions and therefore warrant early operative removal after diagnosis. Because the lining epithelium is that of the gastrointestinal tract, bleeding can also occur into the cyst in the presence of gastric or pancreatic epithelium. Both enteric duplication cysts and mesenteric cysts are known to be associated with malrotation.5-7 One of the hypotheses regarding formation of mesenteric cysts is that they may result from lymphatic obstruction secondary to chronic volvulus.7 A similar course in the presence of a tubular duplication cyst mainly lying separately in the mesentery may have resulted in the cyst in the first case gradually separating from the adjacent bowel and its mesentery.8 The small defect at the base of the mesentery in this case appears to further add to this theory. In the second case, it would appear that part of the embryonic gut was sequestered at a very early stage in fetal life. There appears to be a distinct spectrum in duplication cysts regarding the blood supply and the area of attachment to the bowel. Apart from the classical description
Fig 1. Operative specimen shows intact long tubular cyst measuring 26.5 ⴛ 7 cm with its narrow ligated pedicle in the centre.
reported earlier, the cysts were found hanging by a thin vascular stalk from the mesentery in the vicinity of the duodenojejunal junction with no connection to the adjacent bowel.1,2 Our first case showed similar features; the stalk, however, was attached to the base of the mesentery of the terminal ileum, about 10 cm from the ileocecal junction. In the second case, multiple cysts were lying in the small bowel mesentery; neither were communicating with the adjacent bowel or sharing its blood supply. The diagnosis of duplication cyst was made on the macroscopic appearance as well as the typical histopathologic features, which comprises distinct muscle wall layers and lining epithelium. Of the 2 previous reported cases, one was similar to our case, presenting in an asymptomatic baby boy and operated on 1 week after birth.1 The only additional finding in our first case was the presence of malrotation, which has not been reported previously in completely isolated cysts. A large cyst attached by a narrow mesentery can undergo torsion. In case of inflammation and adherence to the adjacent intestine it can also precipitate volvulus. They are also prone to rupture because of accumulation
Fig 2. Photomicrograph of the cyst wall comprising smooth muscle with a lining of flattened epithelial cells (H & E staining, original magnification ⴛ 240).
ISOLATED ENTERIC DUPLICATION CYSTS
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with shared common wall and blood supply, at the other end of the spectrum are cysts completely isolated from the bowel and having an independent blood supply as seen in our 2 cases. Another group lies in between, where part of the bowel lies independently and part of it shares a common wall.8 Awareness of varied presentations of duplication cysts would help in appropriate management during surgery without any confusion. REFERENCES
Fig 3. Low-power photomicrograph of the cyst shows the wall comprising 2 distinct smooth muscle layers, submucosa, muscularis mucosa, and grossly denuded mucosa (H & E staining, original magnification ⴛ 45).
1. Steiner Z, Mogilner J: A rare case of completely isolated duplication cyst of the alimentary tract. J Pediatr Surg 34:12841286, 1999 2. Kim SK, Lim HK, Lee SJ, et al: Completely isolated enteric duplication cyst: Case report. Abdom Imaging 28:12-14, 2003 3. Teele RL, Henschke CI, Tapper D: The radiographic and ultrasonographic evaluation of enteric duplication cysts. Pediatr Radiol 10:9-12, 1980 4. Kumar R, Jain R, Rao KM, et al: Intraspinal neurenteric cysts—Report of three paediatric cases. Childs Nerv Syst 17:584588, 2001 5. Stringer MD, Spitz L, Abel R, et al: Management of alimentary tract duplication in children. Br J Surg 82:74-78, 1995 6. Basu R, Forshall I, Rickham PP: Duplication of the alimentary tract. Br J Surg 62:269-274, 1960 7. Bentley JFR: Mesenteric cysts with malrotated intestine. Br Med J 2:223, 1970 8. Norris RW, Brereton RJ, Wright VM, et al: A new surgical approach to duplications of the intestine. J Pediatr Surg 21:167-170, 1986
Two rare cases of completely isolated duplication cysts are reported. A large but asymptomatic tubular cyst hanging from a narrow pedicle arising from the base of the terminal ileal mesentery was removed in a 6-day-old boy along with correction of associated malrotation. Multiple isolated duplication cysts located in the thorax and abdomen were excised in a 10-week-old infant.
Both children remained asymptomatic after surgery. J Pediatr Surg 39:E27. © 2004 Elsevier Inc. All rights reserved.
C
cyst with no intraspinal component.3 The child also had multiple thoracic vertebral anomalies. The abdomen was explored first, and multiple cysts were found lying in the small bowel mesentery. The proximal cyst, lying close to the duodeno-jejunal flexure, was 4 ⫻ 2.5 cm long and had a cordlike blind extension into the pancreas. There was another 22-cm-long cyst in the ileal mesentery with multiple constricted areas. There were incomplete mucosal folds forming valvelike structures in the constricted areas. Both the cysts were excised without sacrificing any bowel, because they did not share any common wall with the adjacent intestine. The blood supply appeared to arise from the retroperitoneal region. The cysts contained thick, mucoid material. One week after the laparotomy, a right-sided thoracotomy was performed. A thick-walled, 5 ⫻ 4-cm mucus-filled cyst was removed from the paravertebral retropleural region. It had no attachment to any part of the gastrointestinal tract nor was it communicating with the spinal canal. The histopathology of all the cysts was typical of a duplication cyst with all the layers of intestine. The mucosa was denuded in large areas (Fig 3). The child is now 1.6 years old and is asymptomatic.
OMPLETELY ISOLATED duplication cysts are extremely rare, and only 2 cases have been reported in the literature so far, one in a 7-day-old baby and another in a 28-year-old man.1,2 We report 2 further cases, one of multiple cysts, which has not been reported in the literature so far. CASE REPORT
Case 1 A full-term newborn boy presented on day 6 of life with history of abdominal distension noticed since birth. The antenatal period had been uneventful. He was born to a gravida 2 mother by cesarean section for meconium-stained liquor with a birth weight of 3 kg. The baby had passed meconium and urine within 24 hours of birth. He was taking feedings normally and on examination was active and alert with a heart rate of 120 beats per minute. A large cystic, nontender mass with side-to-side mobility was palpable, which was causing significant abdominal distension and prominence of both the flanks. An ultrasonography (USG) and computed tomography (CT) scan confirmed an intraperitoneal cystic mass but could not comment on the origin or the exact nature of the swelling. At laparotomy, a tense tubular cystic mass resembling small bowel in external appearance and measuring 26.5 ⫻ 7 cm was found occupying the entire abdomen. It was extending from the right lumbar region to the left iliac fossa in an inverted U-shaped manner and was attached to the base of the terminal ileal mesentery by a narrow 4-cm-long pedicle. There was a small v-shaped defect in the mesentery at this site. The lesion was excised (Fig 1) in toto. There was associated malrotation for which Ladds’ procedure was done. The small bowel length was within normal limits. The cyst was unilocular and contained thin blood-stained fluid. The histopathologic examination showed an extremely thin wall with 2 distinct muscle layers and mostly denuded focally flattened lining epithelium, compatible with a duplication cyst (Fig 2). The postoperative period was uneventful. The child remains asymptomatic 8 months after surgery.
Case 2 A 10-week-old boy was admitted with an asymptomatic right-sided abdominal lump noticed since birth. The USG and CT scan showed multiple cysts in the abdomen as well as a mediastinal cystic mass in the right paravertebral gutter with walls characteristic of a duplication
Journal of Pediatric Surgery, Vol 39, No 8 (August), 2004: E27
INDEX WORDS: Completely isolated duplication cyst, abdominal masses, multiple duplication cysts, enteric duplications.
DISCUSSION
Duplication cysts are rare anomalies, which usually present during infancy. One of the accepted diagnostic criteria is the close proximity to some part of the gastrointestinal tract with which it also shares its blood supply. No discernible communication or connection with the adjacent alimentary tract but the presence of typical histopathologic features of a duplication cyst would qualify for a diagnosis of a completely isolated duplication cyst. Isolated, intraspinal, neurenteric cysts may also fall into the same group by this definition.4 In the 2 cases From the Departments of Pediatric Surgery and Histopathology, PostGraduate Institute of Medical Education and Research, Chandigarh, India. Address reprint requests to Dr K.L.N. Rao, Professor and Head, Department of Pediatric Surgery, P.G.I.M.E.R., Chandigarh, 160012, India. © 2004 Elsevier Inc. All rights reserved. 1531-5037/04/3908-0036$30.00/0 doi:10.1016/j.jpedsurg.2004.04.040
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MENON, RAO, AND VAIPHEI
of secretions and therefore warrant early operative removal after diagnosis. Because the lining epithelium is that of the gastrointestinal tract, bleeding can also occur into the cyst in the presence of gastric or pancreatic epithelium. Both enteric duplication cysts and mesenteric cysts are known to be associated with malrotation.5-7 One of the hypotheses regarding formation of mesenteric cysts is that they may result from lymphatic obstruction secondary to chronic volvulus.7 A similar course in the presence of a tubular duplication cyst mainly lying separately in the mesentery may have resulted in the cyst in the first case gradually separating from the adjacent bowel and its mesentery.8 The small defect at the base of the mesentery in this case appears to further add to this theory. In the second case, it would appear that part of the embryonic gut was sequestered at a very early stage in fetal life. There appears to be a distinct spectrum in duplication cysts regarding the blood supply and the area of attachment to the bowel. Apart from the classical description
Fig 1. Operative specimen shows intact long tubular cyst measuring 26.5 ⴛ 7 cm with its narrow ligated pedicle in the centre.
reported earlier, the cysts were found hanging by a thin vascular stalk from the mesentery in the vicinity of the duodenojejunal junction with no connection to the adjacent bowel.1,2 Our first case showed similar features; the stalk, however, was attached to the base of the mesentery of the terminal ileum, about 10 cm from the ileocecal junction. In the second case, multiple cysts were lying in the small bowel mesentery; neither were communicating with the adjacent bowel or sharing its blood supply. The diagnosis of duplication cyst was made on the macroscopic appearance as well as the typical histopathologic features, which comprises distinct muscle wall layers and lining epithelium. Of the 2 previous reported cases, one was similar to our case, presenting in an asymptomatic baby boy and operated on 1 week after birth.1 The only additional finding in our first case was the presence of malrotation, which has not been reported previously in completely isolated cysts. A large cyst attached by a narrow mesentery can undergo torsion. In case of inflammation and adherence to the adjacent intestine it can also precipitate volvulus. They are also prone to rupture because of accumulation
Fig 2. Photomicrograph of the cyst wall comprising smooth muscle with a lining of flattened epithelial cells (H & E staining, original magnification ⴛ 240).
ISOLATED ENTERIC DUPLICATION CYSTS
e7
with shared common wall and blood supply, at the other end of the spectrum are cysts completely isolated from the bowel and having an independent blood supply as seen in our 2 cases. Another group lies in between, where part of the bowel lies independently and part of it shares a common wall.8 Awareness of varied presentations of duplication cysts would help in appropriate management during surgery without any confusion. REFERENCES
Fig 3. Low-power photomicrograph of the cyst shows the wall comprising 2 distinct smooth muscle layers, submucosa, muscularis mucosa, and grossly denuded mucosa (H & E staining, original magnification ⴛ 45).
1. Steiner Z, Mogilner J: A rare case of completely isolated duplication cyst of the alimentary tract. J Pediatr Surg 34:12841286, 1999 2. Kim SK, Lim HK, Lee SJ, et al: Completely isolated enteric duplication cyst: Case report. Abdom Imaging 28:12-14, 2003 3. Teele RL, Henschke CI, Tapper D: The radiographic and ultrasonographic evaluation of enteric duplication cysts. Pediatr Radiol 10:9-12, 1980 4. Kumar R, Jain R, Rao KM, et al: Intraspinal neurenteric cysts—Report of three paediatric cases. Childs Nerv Syst 17:584588, 2001 5. Stringer MD, Spitz L, Abel R, et al: Management of alimentary tract duplication in children. Br J Surg 82:74-78, 1995 6. Basu R, Forshall I, Rickham PP: Duplication of the alimentary tract. Br J Surg 62:269-274, 1960 7. Bentley JFR: Mesenteric cysts with malrotated intestine. Br Med J 2:223, 1970 8. Norris RW, Brereton RJ, Wright VM, et al: A new surgical approach to duplications of the intestine. J Pediatr Surg 21:167-170, 1986