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Cecal duplication causing a disappearing abdominal mass in an infant
Cecal Duplication Causing a Disappearing Abdominal Mass in an Infant By Jose´ Luiz Martins, Edson K. Cury, Antonio S. Petrilli, Elaine C.S. Martins, and Guidugli Neto Sa˜o Paulo, Brazil
Duplications of the alimentary tract are rare malformations. A case of a 2-year-old girl with an intestinal intussusception caused by a cystic duplication of the cecum is presented. This case report is justified by its clinical features and the difficult diagnosis, which was only confirmed during surgery. Ultrasonography had documented an abdominal mass that then disappeared and later reappeared, leading to surgical indication. During surgery, the intussusception within the abdomen was reduced, and right hemicolectomy was performed around the mass in the interior part of the cecum and appendix, with ileocolic anastomosis. On resecting the
cecum, a cystic duplication measuring 4 ⫻ 3 cm was observed, with a granular mucous layer, an ulcerated hole in its interior, and inflammatory reaction. The patient had an uneventful recovery and was discharged on the fourth postoperative day. J Pediatr Surg 36:1581-1583. Copyright © 2001 by W.B. Saunders Company.
D
appendix was observed to have lymphoid hyperplasia. Inspection of the interior of the abdominal cavity showed a normal uterus and ovaries. The patient had an uneventful recovery and was discharged on the fourth postoperative day.
UPLICATIONS of the alimentary tract are rare malformations.1-4 The small intestine is the most common site, whereas rectal, duodenal, gastric, thoracoabdominal, and cecal duplications are extremely rare. CASE REPORT A 2-year-old white girl born in 1995 had presented with a roundshaped abdominal mass in the right hypochondrium during a routine examination at the age of 6 months. Ultrasonography showed a cystic mass that was diagnosed as intestinal duplication. The mass shrank after 1 month and was no longer palpable. A second ultrasound scan showed a significant reduction in size (from 3 to 1.6 cm in diameter). Five months later, the mass became undetectable by ultrasonography. After 1 year, the mass again became detectable by ultrasonography, measuring 2.5 ⫻ 1.7 ⫻ 2.0 cm, and surgery was indicated. While awaiting surgery, the girl suddenly presented with abdominal pain, sweating, and paleness, with intermissions of a few minutes without pain or any other symptoms. There was a palpable mass in the upper part of the abdomen. Intestinal sounds increased in number. Rectal finger examination showed no feces, blood, or mucus. After her admission into Albert Einstein Hospital, she underwent abdominal ultrasonography (Fig 1), and the diagnosis of intestinal intussusception caused by a cystic intestinal mass was confirmed. Laparotomy was performed by means of a right transverse infraumbilical incision through the muscular layers, showing an ileocecocolic intussusception. The intussusception within the abdomen was reduced, and right hemicolectomy was performed around the mass in the interior part of the cecum and appendix with ileocolic anastomosis. The cecum containing the mass was resected, together with part of the terminal ileum and ascending colon, with an incision measuring 11 ⫻ 5 cm, as shown in Fig 2. After opening up the cecum, a cystic duplication measuring 4 ⫻ 3 cm was observed (Fig 2B) with a granular mucous layer, an ulcerated hole in its interior, and inflammatory reaction (Fig 3 A & B). This inflammatory reaction could have been responsible for the involution and reappearance of the cystic mass. The Journal of Pediatric Surgery, Vol 36, No 10 (October), 2001: pp 1581-1583
INDEX WORDS: Cecal duplication, intestinal duplication, intestinal intussusception.
DISCUSSION
Duplications are rare congenital cystic or tubular abnormalities of the gastrointestinal tract that can occur anywhere from the mouth to the anus. Colonic duplications occurs in 13% of these cases.2 Although they can present at any age, more than 80% of the cases present before the age of 2 years. The classic presentation of duplications is in the bowel mesentery, sharing a common muscle wall and blood supply with the associated bowel. The presentation depends on the location and size of the duplication and other factors such as the presence of heterotopic gastric mucosa. The patient usually presents with acute intestinal obstruction; palpable abdominal mass; vague abdominal pain over prolonged periods, or intermittent gastrointestinal bleeding, nausea, vomiting, abdominal distension, and constipation. An ectopic gastric mucosa is found in approximately one third of the duplications, predisposing From the Section of Pediatric Surgery, Department of Surgery, Federal University of Sa˜o Paulo, Paulista School of Medicine, Sa˜o Paulo, SP, Brazil. Address reprint requests to Jose´ Luiz Martins, MD, Rua dos Otonis 131, Vila Clementino, 04025-000 Sa˜o Paulo, SP, Brazil. Copyright © 2001 by W.B. Saunders Company 0022-3468/01/3610-0024$35.00/0 doi:10.1053/jpsu.2001.27057 1581
1582
MARTINS ET AL
Fig 1. Transverse ultrasonography view showing the concentric rings of the intussusceptum within the intussuscipiens.
Fig 3. (A) Ulcerated hole inside the cystic duplication. (B) Histologic section of the resected duplicate wall, with mucosa, enlarged submucosa and muscle layer in the background (H&E, original magnification ⴛ 220).
Fig 2. (A) Resected cecum with the mass in its lumen. (B) Cystic duplication in cecal lumen.
the cyst mucosa to ulceration. Neoplastic changes have been reported in colon duplications. Ultrasonography has proved to be an excellent method for diagnosis and screening of enteric duplications, distinguishing cystic abdominal masses from solid structures. A diagnosis of enteric duplication is suggested when the classic ultrasound triple layer effect appears. Computerized tomography (CT) and magnetic resonance imaging scans (MRI) also show abdominal duplications. A correct preoperative diagnosis is rarely made for patients with symptomatology from an enteric duplication. Less than 25% of cases are correctly diagnosed preoperatively. Frequent misdiagnoses include appendicitis, peptic ulcer disease, intussusception,5,6 and intestinal malrotation. From the fact that in this case the abdominal mass disappeared, an ovarian cystic mass could have been suspected. However, no abnormality was observed in the ovaries of the patient operated on, thus, excluding the possibility of involution of an ovarian cystic mass. This case report shows that cecal duplication can diminish or even disappear on physical or ultrasound
CECAL DUPLICATION
1583
examination, depending on the degree of communication between the mass and the intestine. It is, therefore, important to bear this fact in mind when treating such
patients. For this reason, there is a danger that many patients will perhaps receive a misdiagnosis until much later in life.7,8
REFERENCES 1. Heiss K: Intestinal duplications, in Oldham KT, Colombani PM, Foglia RP (eds): Surgery of Infants and Children: Scientific Principles and Practice. Philadelphia, PA, Lippincott-Raven Publishers, 1997, pp 1265-1274 2. Bond SJ, Groff DB: Gastrointestinal duplications, in O’Neill JA, Rowe MI, Grosfeld JL, et al (eds): Pediatric Surgery. St Louis, MO, Mosby, 1998, pp 1257-1267 3. Stringer MD, Spitz L, Abel R, et al: Management of alimentary tract duplication in children. Br J Surg 82:74-78, 1995 4. Deeths TM, Kilo C, Deeths CM: Duplication of the colon: A case report. Mo Med, 88:289-291, 1991 5. Kum CK, Prabhakaran K, Lee YS, et al: Cystic duplication of the
caecum mimicking intussusception. J Singapore Paediatr Soc 33:37-39, 1991 6. Pinus J, Cury EK, Pereira EE, et al: Duplicidade de ceco. Apresentac¸a˜o de quatro casos e revisa˜o da literatura. Ars Me´dica 18:4-7, 1993 7. Kataoka H, Katsumi K, Kutsuna T, et al: An adult case of ileo-cecal intestinal duplication associated with intussusception, which is difficult to be differentiated from submucosal tumor. Nippon Shoakakibyo Gakkai Zasshi 91:1027-31, 1994 8. Choong CK, Robertson RW, Beasley SW, et al: Congenital caecal cystic duplication presenting with caecal volvulus in elderly woman. Int J Colorectal Dis 12:256-258, 1997
Duplications of the alimentary tract are rare malformations. A case of a 2-year-old girl with an intestinal intussusception caused by a cystic duplication of the cecum is presented. This case report is justified by its clinical features and the difficult diagnosis, which was only confirmed during surgery. Ultrasonography had documented an abdominal mass that then disappeared and later reappeared, leading to surgical indication. During surgery, the intussusception within the abdomen was reduced, and right hemicolectomy was performed around the mass in the interior part of the cecum and appendix, with ileocolic anastomosis. On resecting the
cecum, a cystic duplication measuring 4 ⫻ 3 cm was observed, with a granular mucous layer, an ulcerated hole in its interior, and inflammatory reaction. The patient had an uneventful recovery and was discharged on the fourth postoperative day. J Pediatr Surg 36:1581-1583. Copyright © 2001 by W.B. Saunders Company.
D
appendix was observed to have lymphoid hyperplasia. Inspection of the interior of the abdominal cavity showed a normal uterus and ovaries. The patient had an uneventful recovery and was discharged on the fourth postoperative day.
UPLICATIONS of the alimentary tract are rare malformations.1-4 The small intestine is the most common site, whereas rectal, duodenal, gastric, thoracoabdominal, and cecal duplications are extremely rare. CASE REPORT A 2-year-old white girl born in 1995 had presented with a roundshaped abdominal mass in the right hypochondrium during a routine examination at the age of 6 months. Ultrasonography showed a cystic mass that was diagnosed as intestinal duplication. The mass shrank after 1 month and was no longer palpable. A second ultrasound scan showed a significant reduction in size (from 3 to 1.6 cm in diameter). Five months later, the mass became undetectable by ultrasonography. After 1 year, the mass again became detectable by ultrasonography, measuring 2.5 ⫻ 1.7 ⫻ 2.0 cm, and surgery was indicated. While awaiting surgery, the girl suddenly presented with abdominal pain, sweating, and paleness, with intermissions of a few minutes without pain or any other symptoms. There was a palpable mass in the upper part of the abdomen. Intestinal sounds increased in number. Rectal finger examination showed no feces, blood, or mucus. After her admission into Albert Einstein Hospital, she underwent abdominal ultrasonography (Fig 1), and the diagnosis of intestinal intussusception caused by a cystic intestinal mass was confirmed. Laparotomy was performed by means of a right transverse infraumbilical incision through the muscular layers, showing an ileocecocolic intussusception. The intussusception within the abdomen was reduced, and right hemicolectomy was performed around the mass in the interior part of the cecum and appendix with ileocolic anastomosis. The cecum containing the mass was resected, together with part of the terminal ileum and ascending colon, with an incision measuring 11 ⫻ 5 cm, as shown in Fig 2. After opening up the cecum, a cystic duplication measuring 4 ⫻ 3 cm was observed (Fig 2B) with a granular mucous layer, an ulcerated hole in its interior, and inflammatory reaction (Fig 3 A & B). This inflammatory reaction could have been responsible for the involution and reappearance of the cystic mass. The Journal of Pediatric Surgery, Vol 36, No 10 (October), 2001: pp 1581-1583
INDEX WORDS: Cecal duplication, intestinal duplication, intestinal intussusception.
DISCUSSION
Duplications are rare congenital cystic or tubular abnormalities of the gastrointestinal tract that can occur anywhere from the mouth to the anus. Colonic duplications occurs in 13% of these cases.2 Although they can present at any age, more than 80% of the cases present before the age of 2 years. The classic presentation of duplications is in the bowel mesentery, sharing a common muscle wall and blood supply with the associated bowel. The presentation depends on the location and size of the duplication and other factors such as the presence of heterotopic gastric mucosa. The patient usually presents with acute intestinal obstruction; palpable abdominal mass; vague abdominal pain over prolonged periods, or intermittent gastrointestinal bleeding, nausea, vomiting, abdominal distension, and constipation. An ectopic gastric mucosa is found in approximately one third of the duplications, predisposing From the Section of Pediatric Surgery, Department of Surgery, Federal University of Sa˜o Paulo, Paulista School of Medicine, Sa˜o Paulo, SP, Brazil. Address reprint requests to Jose´ Luiz Martins, MD, Rua dos Otonis 131, Vila Clementino, 04025-000 Sa˜o Paulo, SP, Brazil. Copyright © 2001 by W.B. Saunders Company 0022-3468/01/3610-0024$35.00/0 doi:10.1053/jpsu.2001.27057 1581
1582
MARTINS ET AL
Fig 1. Transverse ultrasonography view showing the concentric rings of the intussusceptum within the intussuscipiens.
Fig 3. (A) Ulcerated hole inside the cystic duplication. (B) Histologic section of the resected duplicate wall, with mucosa, enlarged submucosa and muscle layer in the background (H&E, original magnification ⴛ 220).
Fig 2. (A) Resected cecum with the mass in its lumen. (B) Cystic duplication in cecal lumen.
the cyst mucosa to ulceration. Neoplastic changes have been reported in colon duplications. Ultrasonography has proved to be an excellent method for diagnosis and screening of enteric duplications, distinguishing cystic abdominal masses from solid structures. A diagnosis of enteric duplication is suggested when the classic ultrasound triple layer effect appears. Computerized tomography (CT) and magnetic resonance imaging scans (MRI) also show abdominal duplications. A correct preoperative diagnosis is rarely made for patients with symptomatology from an enteric duplication. Less than 25% of cases are correctly diagnosed preoperatively. Frequent misdiagnoses include appendicitis, peptic ulcer disease, intussusception,5,6 and intestinal malrotation. From the fact that in this case the abdominal mass disappeared, an ovarian cystic mass could have been suspected. However, no abnormality was observed in the ovaries of the patient operated on, thus, excluding the possibility of involution of an ovarian cystic mass. This case report shows that cecal duplication can diminish or even disappear on physical or ultrasound
CECAL DUPLICATION
1583
examination, depending on the degree of communication between the mass and the intestine. It is, therefore, important to bear this fact in mind when treating such
patients. For this reason, there is a danger that many patients will perhaps receive a misdiagnosis until much later in life.7,8
REFERENCES 1. Heiss K: Intestinal duplications, in Oldham KT, Colombani PM, Foglia RP (eds): Surgery of Infants and Children: Scientific Principles and Practice. Philadelphia, PA, Lippincott-Raven Publishers, 1997, pp 1265-1274 2. Bond SJ, Groff DB: Gastrointestinal duplications, in O’Neill JA, Rowe MI, Grosfeld JL, et al (eds): Pediatric Surgery. St Louis, MO, Mosby, 1998, pp 1257-1267 3. Stringer MD, Spitz L, Abel R, et al: Management of alimentary tract duplication in children. Br J Surg 82:74-78, 1995 4. Deeths TM, Kilo C, Deeths CM: Duplication of the colon: A case report. Mo Med, 88:289-291, 1991 5. Kum CK, Prabhakaran K, Lee YS, et al: Cystic duplication of the
caecum mimicking intussusception. J Singapore Paediatr Soc 33:37-39, 1991 6. Pinus J, Cury EK, Pereira EE, et al: Duplicidade de ceco. Apresentac¸a˜o de quatro casos e revisa˜o da literatura. Ars Me´dica 18:4-7, 1993 7. Kataoka H, Katsumi K, Kutsuna T, et al: An adult case of ileo-cecal intestinal duplication associated with intussusception, which is difficult to be differentiated from submucosal tumor. Nippon Shoakakibyo Gakkai Zasshi 91:1027-31, 1994 8. Choong CK, Robertson RW, Beasley SW, et al: Congenital caecal cystic duplication presenting with caecal volvulus in elderly woman. Int J Colorectal Dis 12:256-258, 1997