eISSN: 2084-9869
ISSN: 1233-9687
Polish Journal of Pathology
Current issue Archive Manuscripts accepted About the journal Supplements Editorial board Abstracting and indexing Subscription Contact Instructions for authors Ethical standards and procedures
SCImago Journal & Country Rank
3/2013
vol. 64
 
Share:
Share:
more
 
 

Quiz
Correct answer to the quiz. Check your diagnosis
Brown bowel syndrome (intestinal lipofuscinosis) – a case report and review of the literature

Magdalena Białas
,
Sergiusz Demczuk
,
Grzegorz Dyduch
,
Grażyna Drabik
,
Małgorzata Chrupek
,
Krzysztof Okoń

Pol J Pathol 2013; 64 (3): 228-231
Online publish date: 2013/10/21
Article file
- Brown bowel.pdf  [1.11 MB]
Get citation
ENW
EndNote
BIB
JabRef, Mendeley
RIS
Papers, Reference Manager, RefWorks, Zotero
AMA
APA
Chicago
Harvard
MLA
Vancouver
 
 

Introduction

Brown bowel syndrome (intestinal lipofuscinosis, intestinal ceroidosis, ceroid pigmentation) is brown discoloration of the intestinal wall caused by deposition of insoluble, light brown, granular pigment (lipofuscin) within the smooth muscle cells of the muscularis propria and muscularis mucosae of any bowel segment [1]. It occurs in patients with various malabsorption syndromes including celiac disease and Whipple’s disease, causing deficiency of fat soluble vitamins, especially vitamin E [2]. Brown bowel syndrome is a rare entity which may be asymptomatic but also may have serious clinical implications. One of them is massive intestine dilation causing sub-obstructive symptoms necessitating partial intestinal resection [3]. Although BBS is very rare, it should be taken into consideration in cases with major intestinal dilatation in patients without mechanical obstruction suffering from a malabsorption syndrome.

Case report

An 11-year-old boy (born on time with signs of evisceration, operated on the first day of life) diagnosed with unspecific malabsorption syndrome, IgA deficiency and unspecific myopathy was admitted to the hospital. He had a history of recurrent diarrhoea and symptoms of functional intestinal disturbances suggestive of partial intestinal obstruction lasting for several months prior to admission. The above listed symptoms worsened during the last three weeks. The boy also complained about colicky abdominal pain.

Physical examination revealed an afebrile, cachectic boy with body mass as well as height below the 3rd percentile. His abdomen was distended but bowel sounds were easily heard. Ultrasound and computed tomography showed the first small intestinal loop distended up to 10 cm at a distance of 15 cm. The sigmoid colon was slightly extended as well. The dilated intestinal loop was palpable through the skin. Laparotomy revealed a dilated, darker than usual first jejunal loop. Peristaltic movements of the dilated loop were preserved, but they were described as severely disorganised. No perforation was seen. Resection of an 18 cm long segment of the small intestine was done. The material was sent for histological examination.

Microscopically, small intestinal wall structure was preserved and intestinal villi of normal length were seen (Fig. 1). At higher magnification light-brown, granular pigment was visible in longitudinal and circular muscle coats of muscularis propria and in muscularis mucosae of the intestinal wall (Fig. 2A-B). The same pigment was present in macrophages within sinusoids of the small mesenteric lymph node. Brown pigment stained strongly with periodic acid Schiff (PAS) (Fig. 3) and was resistant to diastase digestion. Stains for iron and melanin were negative. The diagnosis of BBS (intestinal lipofuscinosis) was made. The patient’s postoperative course was uncomplicated.

Discussion

When brown discoloration of any part of the gastrointestinal tract is macroscopically visible, one of the intestinal pigmentation conditions must be suspected. Intestinal pigmentation is seen in BBS, Whipple’s disease and melanosis coli. In BBS granular pigment is accumulated mostly in smooth muscle cells of muscularis propria and muscularis mucosae. Rarely accumulation of pigment is also present in mesenteric lymph node macrophages. The most frequently affected part of the bowel is the small intestine. In Whipple’s disease and melanosis coli brown pigment is present almost exclusively within macrophages in intestinal lamina propria. The pigment in BBS is lipofuscin [4, 5]. It stains strongly with PAS and is resistant to diastase digestion. It also stains with methylene blue, methenamine silver and Masson-Fontana [6, 7]. In some cases of BBS intestinal wall discoloration may be very discrete or even invisible on gross inspection and the diagnosis is made only on the grounds of histological findings in a bowel specimen or in biopsy material.

Brown bowel syndrome was first described by Wagner [8]. Later the association between BBS, malabsorption syndromes and vitamin E deficiency was found [9]. Foster proposed that BBS is a kind of smooth muscle mitochondrial myopathy resulting in lipofuscin accumulation within smooth muscle mitochondria, which occurred in patients with vitamin E deficiency [5]. Vitamin E acts as a mitochondrial antioxidant. Accumulation of lipofuscin (ceroid) usually occurs in smooth muscle of the small or large intestine and in lymph node macrophages. Rarely brown, granular pigment can be seen in smooth muscles of the oesophageal wall and in walls of small vessels [6, 10]. Vitamin E plays a role in protection and stabilisation of mitochondrial membranes and prevents membranes’ damage by free radicals. Vitamin E deficiency accelerates peroxidation of unsaturated membrane lipids and leads to mitochondrial malfunction and degeneration with lipofuscin accumulation in intestinal smooth muscle cells. Damaged mitochondria are not able to provide a sufficient energy supply for smooth muscle, which may result in impaired function of smooth muscle and muscle myopathy with subsequent decrease in intestinal tone leading to massive dilatation of any part of the bowel. Therefore BBS may present clinically as pseudo-obstruction necessitating surgical intervention [2, 10-13].

Brown bowel syndrome is three times more common in males than in females (Table I). It is found in association with various malabsorption syndromes and nutritional disturbances, causing vitamin E deficiency, especially coeliac disease, chronic pancreatitis, liver cirrhosis, Crohn’s disease, tropical sprue, intestinal lymphangiectasia, Whipple’s disease and cystic fibrosis (Table I).

Brown bowel syndrome is not a primary disease, but rather a consequence of long-standing malabsorption of fat-soluble vitamins, especially vitamin E. Treatment with vitamin E supplementation in most cases will not reduce the amount of already deposited lipofuscin in muscle cells but it can prevent further progression of lipofuscin accumulation and subsequent complications.

The identification of lipofuscin accumulation and BBS diagnosis had two implications for the patient: a malabsorption syndrome should be searched and the vitamin E level should be checked.

Conclusions

Brown bowel syndrome is a rare condition which probably is a non-specific morphologic marker of other systemic diseases causing vitamin E deficiency. In children and young adults, especially in cases with heavy lipofuscin accumulation, it can be associated with

massive intestinal dilation. BBS should be considered in patients with malabsorption syndromes without

inflammatory bowel disease when massive intestinal dilation occurred.

References

 1. Fenoglio-Preiser CM. Gastrointestinal Pathology. An Atlas and Text. Wolters Kluwer, Lippincott Williams and Wilkins, Philadelphia 2008; 437-439.

 2. Connolly CE, Kennedy M, Stevens FM, McCarthy CF. Brown bowel syndrome occurring in coeliac disease in the west of Ireland. Scand J Gastroenterol 1994; 29: 91-94.

 3. Robinson MH, Dowling BL, Clark JV, Mason CH. Brown bowel syndrome: an unusual cause of massive dilatation of the colon. Gut 1989; 30: 882-884.

 4. Ghadially FN, Walley VM. Pigments of the gastrointestinal tract: a comparison of light microscopic and electron microscopic findings. Ultrastruct Pathol 1995; 19: 213-219.

 5. Foster CS. The brown bowel syndrome: a possible smooth muscle mitochondrial myopathy. Histopathology 1979; 3: 1-17.

 6. Stamp GW, Evans DJ. Accumulation of ceroid in smooth muscle indicates severe malabsorption and vitamin E deficiency. J Clin Pathol 1987; 40: 798-802.

 7. Ward HC, Leake J, Milla PJ, Spitz L. Brown bowel syndrome: a late complication of intestinal atresia. J Pediatr Surg 1992; 27: 1593-1595.

 8. Wagner E. Uber eine eigenthumliche primare Fettmetamorphose der Muskelhaut des Dunndarms. Arch Heilkunde 1861; 2: 455-459.

 9. Pappenheimer AM, Victor A. Ceroid pigment in human tissues. Am J Pathol 1946; 22: 395-412.

10. Ruchti C, Eisele S, Kaufmann M. Fatal intestinal pseudo-obstruction in brown bowel syndrome. Arch Pathol Lab Med 1990; 114: 76-81.

11. Boller M, Fiocchi C, Brown CH. Pseudoobstruction in ceroidosis. AJR Am J Roentgenol 1976; 127: 277-279.

12. Horn T, Svendsen LB, Nielsen R. Brown bowel syndrome. Review of literature and presentation of cases. Scand J Gastroenterol 1990; 25: 66-72.

13. Michaely HJ, Daroca PJ, Plavsic BM. Brown bowel syndrome – an unusual etiology of pseudo-obstruction of small intestine. Rofo 2003; 175: 1143-1144.

14. Lambert JR, Luk SC, Pritzker KP. Brown bowel syndrome in Crohn’s disease. Arch Pathol Lab Med 1980; 104: 201-205.

15. Lee H, Carlin AM, Ormsby AH, Lee MW. Brown bowel syndrome secondary to jejunoileal bypass: the first case report. Obes Surg 2009; 19: 1176-1179.

16. Drake WM, Winter TA, Price SK, O'Keefe SJ. Small bowel intussusception and brown bowel syndrome in association with severe malnutrition. Am J Gastroenterol 1996; 91: 1450-1452.

17. Hurley JP, Leary R, Connolly CE, Keeling P. Massive lower gastrointestinal bleeding in association with brown bowel syndrome. J R Soc Med 1991; 84: 437-438.

18. Hosler JP, Kimmel KK, Moeller DD. The “brown bowel syndrome”: a case report. Am J Gastroenterol 1982; 77: 854-855.

19. Narváez Rodríguez I, Herrera Justiniano JM, Márquez Galán JL, et al. The brown bowel syndrome: a case report. Rev Esp Enferm Dig 1993; 83: 281-283.

20. Kaiserling E, Schäffer R, Weckermann J. Brown bowel syndrome with manifestation in the gastrointestinal tract and thyroid gland. Pathol Rec Pract 1988; 183: 65-79.



Address for correspondence



Magdalena Białas
, MD

Department of Pathomorphology

ul. Grzegórzecka 16

31-351 Kraków

tel. +48 12 421 15 64

e-mail: mbialas7@gmail.com
Copyright: © 2013 Polish Association of Pathologists and the Polish Branch of the International Academy of Pathology This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0) License (http://creativecommons.org/licenses/by-nc-sa/4.0/), allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.
Quick links
© 2022 Termedia Sp. z o.o. All rights reserved.
Developed by Bentus.