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Primary (retractile) mesenteritis in a child
Primary (Retractile)
Mesenteritis
in a Child
By Carl F. Davis, Philip C. Guzzetta, and Kathleen Patterson Washington, l We report a case of primary retractile mesenteritis presenting as acute abdominal pain requiring surgery in a 3-year-old boy. At laparotomy, a hemoperitoneum was discovered and the diagnosis of primary mesenteritis was made with the aid of frozen section biopsy of the small bowel mesentery. No resection was necessary, and he made an uneventful recovery and remains well on follow-up. Copyright o 1992 by W.B. Saunders Company INDEX WORDS:
Mesenteritis,
primary (retractile).
I
DIOPATHIC (retractile) mesenteritis is a benign, usually self-limited thickening of the intestinal mesentery. We present a case of this disease in a 3-year-old child. CASE REPORT A 3-year-old black boy presented to Children’s National Medical Center with a 1Zhour history of severe abdominal pain. He was awakened from a nap earlier in the day by the pain. Other gastrointestinal symptoms included anorexia and one episode of vomiting. He had a normal bowel movement earlier that day. There was no history of trauma. He had no prodromal symptoms. Past medical history was only relevant for a seizure disorder for which he took phenobarbital. On physical examination, he was distressed and had a tender abdomen. No mass could be palpated.
Preoperative Studies Blood results included: hematocrit, 28%; leukocyte count, 22,500 (88% segs, 2% bands); electrolytes, normal; amylase and liver function tests, normal. X-rays of the abdomen and chest were nondiagnostic. A contrast enema was performed to rule out intussusception, and it demonstrated free flow into the terminal ileum with filling of the appendix. He was then prepared for urgent laparotomy.
DC
the involved mesentery. The sigmoid colon was adherent to the small bowel mesentery at one site. Thickening of the peritoneum in the right lateral reflection and around the epiploic foramen was also noted. Fat necrosis was seen in the gastrocolic and greater omentum. The pancreas was fully exposed and was seen to be normal, as was the retroperitoneum. Multiple biopsies of the mesentery and thickened peritoneum were obtained for frozen section analysis. No resection was performed, and the abdomen was closed. Return of bowel function was slightly delayed, but the patient was discharged 7 days postoperatively. He has been followed as an outpatient and is thriving. No mass can be palpated, and the abdomen is soft and nontender. Abdominal ultrasound 2 months following surgery was normal.
Pathology Findings Biopsy fragments from the mesentery and thickened peritoneum examined at frozen section and after formalin fixation and paraffin embedding showed an inflammatory and fibrosing process surrounding and replacing the fat. The inflammation was spotty and composed primarily of lymphocytes and foamy macrophages with occasional hemosiderin-laden macrophages (Fig 1). DISCUSSION
Idiopathic (retractile) mesenteritis is an uncommon condition, with under 200 cases reported since that of Jura in 1924.1e4It is usually seen in middleaged and elderly patients, with only 8 cases being reported in children,2-4 to which we add our case. The large number of terms used to describe the disease has led to much confusion as to the classification and the exact incidence of mesenteritis.5,6 The disease has been called: retractile mesenteritis, sclerosing mesenteritis, multifocal subperitoneal sclero-
Operative Findings Under general anesthesia, a firm, irregular, &cm-diameter mass was palpated in the epigastric region. At laparotomy, a hemoperitoneum (150 to 200 mL) was evacuated. A single arterial bleeding point near the origin of the middle colic artery was found and undersewn. A thorough examination of the abdomen showed a thickened mesentery to the entire small bowel and transverse colon. This had caused marked shortening and retraction of the involved gut. As a result, the entire small bowel was held centrally and anteriorly in the abdomen. No blood vessels could be seen in
From the Departments of Pediatric Szuge~ and Pathology, Children S National Medical Center, and the Department of Child Health and Development, George Washington University, Washington, DC. Address reprint requests to Philip C. Guzzetta, MD, Department of Pediatric Surgery, Children S National Medical Center, I I1 Michigan Ave NW, Washington, DC 20010. Copyright 0 1992 by WB. Saunders Company 0022.3468/92/2712-0024$03.00/O
1544
Fig 1. Section of mesentery with reactive fibroblastic proliferation, hemorrhage, and a chronic inflammatory infiltrate, seen at higher magnification in the lower right corner insert (H&E, original magnificatidn X50, insert x200).
JournalofPediatricSurgery,
Vol27, No 12 (December), 1992:pp 1544-1545
1545
PRIMARY MESENTERITIS
sis, mesenteric lipodystrophy, mesenteric panniculitis, and sclerosing lipogranulomatosis. In a recent attempt to provide a working classification, Remmele et al differentiated between primary and secondary mesenteritis, the latter having recognized associated conditions.5 Although changes similar to primary mesenteritis have been reproduced in laboratory settings by ingestion of bacteria or bacterial toxins or by trauma to the mesentery, in the clinical setting no precipitating factors are usually identifiable. Idiopathic mesenteritis may be the result of a number of different insults to the mesentery. Many treatment options have been reported.5 These include steroids, antibiotics, azathioprine, irradiation, and surgery for localized lesions. Because spontaneous remission is the most common outcome.
adjunctive therapy should be limited to those patients with progression of symptoms. Surgical resection for intestinal obstruction is occasionally necessary. The present patient is the youngest reported with this condition. His presentation of primary mesenteritis with hemoperitoneum is very unusual. This may have been as a result of minor, unnoticed trauma injuring the relatively noncompliant transverse mesocolon at its base, while the mesenteric fibrosis may have prevented retraction of the vessel ends. Despite the extent of the mesenteric involvement, the patient has made an uneventful and apparently total recovery. Although this is an unusual condition, it is important to recognize that the usual outcome is spontaneous resolution, and thus management should be conservative.
REFERENCES 1, Jura V: Sulla mesenterite retrattile e sclerosante. Policlinico (sez-prat) 31:575-581, 1924 2. Spark RB, Yakovac WC, Wagget J: Retractile sclerosing mesenteritis. Case report. Clin Pediatr 10:119-122, 1971 3. Reske M. Namiki H: Sclerosing mesenteritis: Report of two cases. Am J Clin Path01 64:661-667. 1975 4. Jona JZ. Glickman M. Cohen RD: Sclerosing mesenteritis in a child. Arch Surg 122:735, 1987
5. Remmele itis. mesenteric
W, Muller-Lobeck fibrosis
four cases, pathology,
H, Paulus W: Primary
and mesenteric and classification.
tibromatosis. Pathol
mesenterReport
of
Res Pratt
184:77-
(sclerosing)
mesen-
85. 1989 6. Kelly JK, Hwang W: Idiopathic teritis and its differential 19x9
diagnosis.
retractile
Am J Surg Path01 13:513-521.
Mesenteritis
in a Child
By Carl F. Davis, Philip C. Guzzetta, and Kathleen Patterson Washington, l We report a case of primary retractile mesenteritis presenting as acute abdominal pain requiring surgery in a 3-year-old boy. At laparotomy, a hemoperitoneum was discovered and the diagnosis of primary mesenteritis was made with the aid of frozen section biopsy of the small bowel mesentery. No resection was necessary, and he made an uneventful recovery and remains well on follow-up. Copyright o 1992 by W.B. Saunders Company INDEX WORDS:
Mesenteritis,
primary (retractile).
I
DIOPATHIC (retractile) mesenteritis is a benign, usually self-limited thickening of the intestinal mesentery. We present a case of this disease in a 3-year-old child. CASE REPORT A 3-year-old black boy presented to Children’s National Medical Center with a 1Zhour history of severe abdominal pain. He was awakened from a nap earlier in the day by the pain. Other gastrointestinal symptoms included anorexia and one episode of vomiting. He had a normal bowel movement earlier that day. There was no history of trauma. He had no prodromal symptoms. Past medical history was only relevant for a seizure disorder for which he took phenobarbital. On physical examination, he was distressed and had a tender abdomen. No mass could be palpated.
Preoperative Studies Blood results included: hematocrit, 28%; leukocyte count, 22,500 (88% segs, 2% bands); electrolytes, normal; amylase and liver function tests, normal. X-rays of the abdomen and chest were nondiagnostic. A contrast enema was performed to rule out intussusception, and it demonstrated free flow into the terminal ileum with filling of the appendix. He was then prepared for urgent laparotomy.
DC
the involved mesentery. The sigmoid colon was adherent to the small bowel mesentery at one site. Thickening of the peritoneum in the right lateral reflection and around the epiploic foramen was also noted. Fat necrosis was seen in the gastrocolic and greater omentum. The pancreas was fully exposed and was seen to be normal, as was the retroperitoneum. Multiple biopsies of the mesentery and thickened peritoneum were obtained for frozen section analysis. No resection was performed, and the abdomen was closed. Return of bowel function was slightly delayed, but the patient was discharged 7 days postoperatively. He has been followed as an outpatient and is thriving. No mass can be palpated, and the abdomen is soft and nontender. Abdominal ultrasound 2 months following surgery was normal.
Pathology Findings Biopsy fragments from the mesentery and thickened peritoneum examined at frozen section and after formalin fixation and paraffin embedding showed an inflammatory and fibrosing process surrounding and replacing the fat. The inflammation was spotty and composed primarily of lymphocytes and foamy macrophages with occasional hemosiderin-laden macrophages (Fig 1). DISCUSSION
Idiopathic (retractile) mesenteritis is an uncommon condition, with under 200 cases reported since that of Jura in 1924.1e4It is usually seen in middleaged and elderly patients, with only 8 cases being reported in children,2-4 to which we add our case. The large number of terms used to describe the disease has led to much confusion as to the classification and the exact incidence of mesenteritis.5,6 The disease has been called: retractile mesenteritis, sclerosing mesenteritis, multifocal subperitoneal sclero-
Operative Findings Under general anesthesia, a firm, irregular, &cm-diameter mass was palpated in the epigastric region. At laparotomy, a hemoperitoneum (150 to 200 mL) was evacuated. A single arterial bleeding point near the origin of the middle colic artery was found and undersewn. A thorough examination of the abdomen showed a thickened mesentery to the entire small bowel and transverse colon. This had caused marked shortening and retraction of the involved gut. As a result, the entire small bowel was held centrally and anteriorly in the abdomen. No blood vessels could be seen in
From the Departments of Pediatric Szuge~ and Pathology, Children S National Medical Center, and the Department of Child Health and Development, George Washington University, Washington, DC. Address reprint requests to Philip C. Guzzetta, MD, Department of Pediatric Surgery, Children S National Medical Center, I I1 Michigan Ave NW, Washington, DC 20010. Copyright 0 1992 by WB. Saunders Company 0022.3468/92/2712-0024$03.00/O
1544
Fig 1. Section of mesentery with reactive fibroblastic proliferation, hemorrhage, and a chronic inflammatory infiltrate, seen at higher magnification in the lower right corner insert (H&E, original magnificatidn X50, insert x200).
JournalofPediatricSurgery,
Vol27, No 12 (December), 1992:pp 1544-1545
1545
PRIMARY MESENTERITIS
sis, mesenteric lipodystrophy, mesenteric panniculitis, and sclerosing lipogranulomatosis. In a recent attempt to provide a working classification, Remmele et al differentiated between primary and secondary mesenteritis, the latter having recognized associated conditions.5 Although changes similar to primary mesenteritis have been reproduced in laboratory settings by ingestion of bacteria or bacterial toxins or by trauma to the mesentery, in the clinical setting no precipitating factors are usually identifiable. Idiopathic mesenteritis may be the result of a number of different insults to the mesentery. Many treatment options have been reported.5 These include steroids, antibiotics, azathioprine, irradiation, and surgery for localized lesions. Because spontaneous remission is the most common outcome.
adjunctive therapy should be limited to those patients with progression of symptoms. Surgical resection for intestinal obstruction is occasionally necessary. The present patient is the youngest reported with this condition. His presentation of primary mesenteritis with hemoperitoneum is very unusual. This may have been as a result of minor, unnoticed trauma injuring the relatively noncompliant transverse mesocolon at its base, while the mesenteric fibrosis may have prevented retraction of the vessel ends. Despite the extent of the mesenteric involvement, the patient has made an uneventful and apparently total recovery. Although this is an unusual condition, it is important to recognize that the usual outcome is spontaneous resolution, and thus management should be conservative.
REFERENCES 1, Jura V: Sulla mesenterite retrattile e sclerosante. Policlinico (sez-prat) 31:575-581, 1924 2. Spark RB, Yakovac WC, Wagget J: Retractile sclerosing mesenteritis. Case report. Clin Pediatr 10:119-122, 1971 3. Reske M. Namiki H: Sclerosing mesenteritis: Report of two cases. Am J Clin Path01 64:661-667. 1975 4. Jona JZ. Glickman M. Cohen RD: Sclerosing mesenteritis in a child. Arch Surg 122:735, 1987
5. Remmele itis. mesenteric
W, Muller-Lobeck fibrosis
four cases, pathology,
H, Paulus W: Primary
and mesenteric and classification.
tibromatosis. Pathol
mesenterReport
of
Res Pratt
184:77-
(sclerosing)
mesen-
85. 1989 6. Kelly JK, Hwang W: Idiopathic teritis and its differential 19x9
diagnosis.
retractile
Am J Surg Path01 13:513-521.