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Adenomyomatous hamartoma causing ileoileal intussusception in a young child
CLINICAL IMAGING 1993;17:183-185
183
ADENOMYOMATOUS HAMARTOMA CAUSING ILEOILEAL INTUSSUSCEPTION IN A YOUNG CHILD NEELA LAMKI, MD, FRCPC, CYNTHIA L. WOO, ALFRED B. WATSON, JR., MD, AND HAN-SEOB
An unusual case of adenomyomatous hamartoma of ileum leading to ileo-ileal intussusception in a 22month-old child is reported. KEY WORDS:
Intussusception; Small bowel neoplasm: Small bowel tumor; Adenomatous hamartoma; Myoepithelial hamartoma; Ileal intussusception; Ileum neoplasm INTRODUCTION A case of ileoileal intussusception in a 22-month-old boy caused by a small bowel tumor is reported. This is the first reported case of an adenomyomatous hamartoma causing ileoileal intussusception in a young child. CASE REPORT A 22-month-old boy was seen in the emergency room with a three-day history of crampy abdominal pain and vomiting. Physical examination revealed a distended abdomen with high-pitched bowel sounds and rebound tenderness. Rectal exam demonstrated acholic, guaic-negative stool. Supine and upright films of the abdomen showed dilated small bowel with air fluid levels consistent with small bowel obstruction. A bar-
From the Departments of Radiology (N.L., C.L.W., A.B.W.) and Pathology (H.S.K.), Baylor College of Medicine, Houston, Texas. Address reprint requests to: Neela Lamki, MD, Director of Radiologic Education, Professor of Clinical Radiology, Department of Radiology, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030. Received January 5, 1993; accepted January 19, 1993. 0 1993 Elsevier Science Publishing Co., Inc. 655 Avenue of the Americos, 0699-7071/93/$6.00
New York, NY 10010
MD,
KIM,
MD, PHD
ium enema demonstrated no colonic lesions, but refly into the terminal ileum revealed a coiled springappearing filling defect in the distal ileum consistent with an ileo-ileal intussusception (Figure 1). The visualized ileal mucosa appeared edematous. The intussusception could not be reduced using the hydrostatic pressure from the barium with the barium bag raised to three feet above the table top.
FIGURE 1. Film from barium enema showing coiled-spring filling defect (arrow) in distal ileum consistent with ileoileal intussusception.
CLINICAL
IMAGING
VOL. 17, NO. 3
FIGURE 3. Multiloculated, various-sized cystic spaces in the muscularis propria are lined by a single layer of columnar epithelium. A basal portion of the small intestinal mucosa is seen in the left upper corner.
DISCUSSION
B FIGURE 2. (A) Laminal view of the resected ileal segment showing raised submucosal lesions. (J3) Cross section of the ileum through the hamartoma. Note relatively intact mucosa and serosa and cystic structure within the tumor.
At exploratory laparotomy an ileoileal intussusception was discovered. Upon reduction a submucosal or subserosal small bowel mass was noted as a lead point. The lead point and approximately 2 cm of normal small bowel proximal and distal to it were excised. The remaining ileum was reanastamosed. The patient’s postoperative course was uncomplicated and he was discharged on postoperative day six in good condition. Gross examination of the surgical specimen revealed edematous small bowel with hemorrhagic exudate on the serosal surface and two adjacent submucosal masses measuring 1.5 and 1.2 cm in diameter (Figure 2A and B). On microscopic sections (Figure 3), the masses were within the inner portion of the muscularis propria and consisted of epithelial-lined cysts surrounded by hypertrophic smooth muscle. The cysts were lined by tall columnar epithelium. The microscopic features were consistent with an adenomyoma [also known as myoepithelial hamartoma) of the gastrointestinal tract.
Intussesception most commonly occurs in infants and children between the ages of 6 months and 2 years, but can be seen in older children and adults. The majority of intussusceptions are ileocolic, but ileoileal and ileoileocolic intussusceptions can also occur. The etiology of intussusception in infants and children is often obscure. Neonatal intussusception and intussusception in children over 2 years old and adults are usually associated with a lead point mass, and consequently are harder to reduce by barium enema. The lead point mass may be a Meckel’s diverticulum (most common), polyp, tumor, or lymphoid hyperplasia. Small bowel tumors are quite rare. In one series of 292 children with intussusception, 10 (3.5%) were caused by small bowel tumors (1). These included adenomatous polyps, hamartomas of F’eutz-Jeghers syndrome, hemangiomas, and lymphangiosarcoma. Other tumors previously reported are leiomyosarcoma, angiosarcoma, embryonic tumor, and non-Hodgkin’s lymphoma. Hamartoma, also known as adenomyoma or myoepithelial hamartoma of the small intestine, is rare and has been reported only twice before in the literature as a cause of intussusception in adults (2,~). One case of adenomatous hamartoma of the small bowel causing jejunoileal atresia in an infant is reported, but no histologic information is provided (4). The hamartoma of Peutz-Jeghers syndrome has been frequently implicated in intussusception, but histologically it is distinct. This is the first reported case of an adenomyomatous hamartoma causing ileoileal intussusception in a young child.
JULY-SEPTEMBER
1993
The authors wish to thank Becky Baxter and Lori Burlin for their secretarial assistance.
REFERENCES 1. Puri P, Guiney EJ. Small bowel tumours causing intussusception in childhood. Br J Surg 1985;72:493-494.
ILEOILEAL
INTUSSUSCEPTION
PROM A TUMOR
185
2. Gal R, Kalkow Z, Nobel M. Adenomyomatous hamartoma of the small intestine: a rare cause of intussusception in an adult. Am J Gastmenterol 1966;80:1209-1211. 3. Hadley GP, SimpsonRI. J Surg 1983;70:281.
Adult intussusception
in the tropics. Br
4. Roserman E, Maayan C, Lernau 0. Leiomyomatous hamartosis with congenital jejunoileal atresia. Isr J Med Sci 1980;16:775-779.
183
ADENOMYOMATOUS HAMARTOMA CAUSING ILEOILEAL INTUSSUSCEPTION IN A YOUNG CHILD NEELA LAMKI, MD, FRCPC, CYNTHIA L. WOO, ALFRED B. WATSON, JR., MD, AND HAN-SEOB
An unusual case of adenomyomatous hamartoma of ileum leading to ileo-ileal intussusception in a 22month-old child is reported. KEY WORDS:
Intussusception; Small bowel neoplasm: Small bowel tumor; Adenomatous hamartoma; Myoepithelial hamartoma; Ileal intussusception; Ileum neoplasm INTRODUCTION A case of ileoileal intussusception in a 22-month-old boy caused by a small bowel tumor is reported. This is the first reported case of an adenomyomatous hamartoma causing ileoileal intussusception in a young child. CASE REPORT A 22-month-old boy was seen in the emergency room with a three-day history of crampy abdominal pain and vomiting. Physical examination revealed a distended abdomen with high-pitched bowel sounds and rebound tenderness. Rectal exam demonstrated acholic, guaic-negative stool. Supine and upright films of the abdomen showed dilated small bowel with air fluid levels consistent with small bowel obstruction. A bar-
From the Departments of Radiology (N.L., C.L.W., A.B.W.) and Pathology (H.S.K.), Baylor College of Medicine, Houston, Texas. Address reprint requests to: Neela Lamki, MD, Director of Radiologic Education, Professor of Clinical Radiology, Department of Radiology, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030. Received January 5, 1993; accepted January 19, 1993. 0 1993 Elsevier Science Publishing Co., Inc. 655 Avenue of the Americos, 0699-7071/93/$6.00
New York, NY 10010
MD,
KIM,
MD, PHD
ium enema demonstrated no colonic lesions, but refly into the terminal ileum revealed a coiled springappearing filling defect in the distal ileum consistent with an ileo-ileal intussusception (Figure 1). The visualized ileal mucosa appeared edematous. The intussusception could not be reduced using the hydrostatic pressure from the barium with the barium bag raised to three feet above the table top.
FIGURE 1. Film from barium enema showing coiled-spring filling defect (arrow) in distal ileum consistent with ileoileal intussusception.
CLINICAL
IMAGING
VOL. 17, NO. 3
FIGURE 3. Multiloculated, various-sized cystic spaces in the muscularis propria are lined by a single layer of columnar epithelium. A basal portion of the small intestinal mucosa is seen in the left upper corner.
DISCUSSION
B FIGURE 2. (A) Laminal view of the resected ileal segment showing raised submucosal lesions. (J3) Cross section of the ileum through the hamartoma. Note relatively intact mucosa and serosa and cystic structure within the tumor.
At exploratory laparotomy an ileoileal intussusception was discovered. Upon reduction a submucosal or subserosal small bowel mass was noted as a lead point. The lead point and approximately 2 cm of normal small bowel proximal and distal to it were excised. The remaining ileum was reanastamosed. The patient’s postoperative course was uncomplicated and he was discharged on postoperative day six in good condition. Gross examination of the surgical specimen revealed edematous small bowel with hemorrhagic exudate on the serosal surface and two adjacent submucosal masses measuring 1.5 and 1.2 cm in diameter (Figure 2A and B). On microscopic sections (Figure 3), the masses were within the inner portion of the muscularis propria and consisted of epithelial-lined cysts surrounded by hypertrophic smooth muscle. The cysts were lined by tall columnar epithelium. The microscopic features were consistent with an adenomyoma [also known as myoepithelial hamartoma) of the gastrointestinal tract.
Intussesception most commonly occurs in infants and children between the ages of 6 months and 2 years, but can be seen in older children and adults. The majority of intussusceptions are ileocolic, but ileoileal and ileoileocolic intussusceptions can also occur. The etiology of intussusception in infants and children is often obscure. Neonatal intussusception and intussusception in children over 2 years old and adults are usually associated with a lead point mass, and consequently are harder to reduce by barium enema. The lead point mass may be a Meckel’s diverticulum (most common), polyp, tumor, or lymphoid hyperplasia. Small bowel tumors are quite rare. In one series of 292 children with intussusception, 10 (3.5%) were caused by small bowel tumors (1). These included adenomatous polyps, hamartomas of F’eutz-Jeghers syndrome, hemangiomas, and lymphangiosarcoma. Other tumors previously reported are leiomyosarcoma, angiosarcoma, embryonic tumor, and non-Hodgkin’s lymphoma. Hamartoma, also known as adenomyoma or myoepithelial hamartoma of the small intestine, is rare and has been reported only twice before in the literature as a cause of intussusception in adults (2,~). One case of adenomatous hamartoma of the small bowel causing jejunoileal atresia in an infant is reported, but no histologic information is provided (4). The hamartoma of Peutz-Jeghers syndrome has been frequently implicated in intussusception, but histologically it is distinct. This is the first reported case of an adenomyomatous hamartoma causing ileoileal intussusception in a young child.
JULY-SEPTEMBER
1993
The authors wish to thank Becky Baxter and Lori Burlin for their secretarial assistance.
REFERENCES 1. Puri P, Guiney EJ. Small bowel tumours causing intussusception in childhood. Br J Surg 1985;72:493-494.
ILEOILEAL
INTUSSUSCEPTION
PROM A TUMOR
185
2. Gal R, Kalkow Z, Nobel M. Adenomyomatous hamartoma of the small intestine: a rare cause of intussusception in an adult. Am J Gastmenterol 1966;80:1209-1211. 3. Hadley GP, SimpsonRI. J Surg 1983;70:281.
Adult intussusception
in the tropics. Br
4. Roserman E, Maayan C, Lernau 0. Leiomyomatous hamartosis with congenital jejunoileal atresia. Isr J Med Sci 1980;16:775-779.