- Case report
- Open Access
Distinguishing between limited systemic scleroderma-associated pseudo-obstruction and peritoneal dissemination
© Saigusa et al.; licensee Springer. 2015
Received: 6 November 2014
Accepted: 22 December 2014
Published: 24 February 2015
A 78-year-old woman receiving treatment for limited systemic scleroderma (SSc) underwent high anterior resection and partial liver resections for rectosigmoid colon cancer with multiple liver metastases. A year after surgery, an abdominal computed tomography (CT) demonstrated suspicion for peritoneal dissemination with an increase in ascites, and 18F-fluorodeoxy glucose-positron emission tomography-CT was suggestive of carcinomatosis. We began to decompress the small intestine and administer octreotide. However, the intestinal obstruction did not improve. Although intestinal pseudo-obstruction caused by limited SSc was considered as a differential diagnosis, we performed an exploratory laparotomy because the possibility of peritoneal dissemination-associated obstruction could not be excluded. We observed a moderate amount of serous ascites and dilatation of the small intestine that was white in color, hard, and with limited contractility. There was no evidence of peritoneal dissemination nor of mechanical obstruction. Our experience thus shows the difficulty of distinguishing SSc-associated intestinal pseudo-obstruction from peritoneal dissemination.
Intestinal pseudo-obstruction is caused by several diseases including connective tissue disorders, hypothyroidism, Chagas’ disease, diabetes, and Parkinson’s disease. Systemic scleroderma (SSc) is a chronic disorder of the connective tissue characterized by inflammation, fibrosis, and degeneration of the skin and blood vessels and is known to cause intestinal pseudo-obstruction [1-5]. The radiographic findings of intestinal pseudo-obstruction caused by SSc are a hide-bound bowel sign or accordion sign on abdominal X-ray or contrast study and pneumatosis cystoides intestinalis on abdominal computed tomography (CT) [3,6-8]. However, these findings cannot exclude mechanical obstruction such as that caused by postoperative adhesions or peritoneal dissemination. Although 18F-fluorodeoxy glucose-positron emission tomography (FDG PET-CT) is a useful tool to detect peritoneal metastases, low-grade FDG uptake should be carefully assessed because it is difficult to differentiate inflammation or fibrotic change from tumor recurrence [9-12]. We report a case in which it was difficult to distinguish between SSc-associated pseudo-obstruction and peritoneal dissemination in a SSc patient after surgery for stage IV colon cancer.
Gastrointestinal involvement is the most common non-dermatologic complication in patients with SSc [3,13]. Gastrointestinal dysfunction can severely and negatively impact quality of life and is associated with a poor prognosis [14-18]. Limited SSc is a subtype of systemic scleroderma that was previously referred to as CREST syndrome (calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias). Patients with limited SSc develop sclerosis of the skin distal to their elbows, knees, and face and are more likely to develop pulmonary hypertension. Patients with another subtype of SSc, diffuse SSc, develop not only distal but also proximal sclerosis and tend to have more significant visceral organ involvement than those with limited SSc. However, gastrointestinal involvement is common in both subtypes and the severity of cutaneous and gastrointestinal manifestations often does not correlate [3,19,20]. Pathologic manifestations in patients with SSc are due to progressive fibrosis of intestinal smooth muscle. Physiologically, inflammation of the myenteric ganglia and fibrosis of gastrointestinal muscle have been found in patients with SSc [21,22]. Small intestinal hypomotility causes luminal dilatation and stasis of luminal contents resulting in a pseudo-obstruction, leading to stasis-related malabsorption. Secondary small intestinal bacterial outgrowth affects almost half of patients with SSc [5,23-25]. It has been reported that octreotide is the most potent prokinetic drug for pseudo-obstruction [26,27]. However, octreotide did not improve motility in the present case. Moreover, erythromycin, which mimics motilin, known as the prokinetic hormone, was also not effective, despite the fact that several authors have reported upon its effectiveness for pseudo-obstruction [28,29]. We think that the lack of effectiveness of these drugs is due to the progressive fibrosis of the small intestine and malabsorption by small intestinal bacterial overgrowth. Although we tried enteral nutrition in the form of an elemental diet via the enterostomy with concomitant administration of antibiotics such as metronidazole [30,31], the nutrition status of our patient did not improve. We think that early diagnosis and treatment for pseudo-obstruction is important, along with the collaboration of rheumatologists and gastroenterologists in patients with gastrointestinal involvement of SSc. Although we lack a complete understanding of the progressive nature of the disease, the presence of carcinoma in addition to adjuvant chemotherapy may compound the situation .
On radiographic examination, abdominal X-ray did not show the typical appearance of SSc-associated pseudo-obstruction such as the hide-bound bowel sign [3,6-8]. Although we observed pneumatosis cystoides intestinalis in the descending colon 2 months before exploratory laparotomy, this finding was not noted during the surgery. Given the increase in ascites and occurrence of obstructive symptoms, most would suspect peritoneal recurrence in a patient with stage IV colon cancer after surgery. FDG PET-CT has the potential to improve detection of peritoneal carcinomatosa [9-12]. However, FDG uptake is also present in cases of fibrosis and inflammation, and it is difficult to distinguish this pathology from peritoneal metastasis [9-12]. Although diffuse low-grade FDG uptake (up to the maximum standardized uptake value of 2.3) along the small intestinal wall was observed, we were unable to definitively diagnose the current case as one of intestinal pseudo-obstruction. Several authors reported that evaluation for radiation-induced fibrosis and retroperitoneal fibrosis is helpful [33-35]. On the other hand, Nishiyama et al. have reported that FDG PET-CT is useful to evaluate the degree of disease associated with connective tissue disorder . In the current case, we think that elevated FDG uptake along the small intestinal wall was indicative of inflammation and fibrosis.
In conclusion, our experience suggests that it is difficult to distinguish LSSc-associated intestinal pseudo-obstruction from peritoneal dissemination. Although FDG PET-CT is an effective tool for detection of peritoneal metastasis, comprehensive assessment of the findings in a patient with both SSc and advanced gastrointestinal cancer is necessary.
Written informed consent was obtained from the patient’s husband for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
- Sjogren RW. Gastrointestinal features of scleroderma. Curr Opin Rheumatol. 1996;8:569–75.View ArticlePubMedGoogle Scholar
- Connor FL, Di Lorenzo C. Chronic intestinal pseudo-obstruction: assessment and management. Gastroenterology. 2006;130:S29–36.View ArticlePubMedGoogle Scholar
- Domsic R, Fasanella K, Bielefeldt K. Gastrointestinal manifestations of systemic sclerosis. Dig Dis Sci. 2008;53:1163–74.View ArticlePubMedGoogle Scholar
- Munoz-Yague MT, Solis-Munoz P, Salces I, Ballestin C, Colina F, Ibarrola C, et al. Chronic intestinal pseudo-obstruction: a diagnosis to be considered. Rev Esp Enferm Dig. 2009;101:336–42.View ArticlePubMedGoogle Scholar
- Savarino E, Mei F, Parodi A, Ghio M, Furnari M, Gentile A, et al. Gastrointestinal motility disorder assessment in systemic sclerosis. Rheumatology (Oxford). 2013;52:1095–100.View ArticleGoogle Scholar
- Pickhardt PJ. The “hide-bound” bowel sign. Radiology. 1999;213:837–8.View ArticlePubMedGoogle Scholar
- Hokama A, Ihama Y, Kishimoto K, Yara S, Kinjo F, Fujita J. Clinical images: chronic intestinal pseudoobstruction and the “hide-bound” bowel sign. Arthritis Rheum. 2007;56:1724.View ArticlePubMedGoogle Scholar
- Seket B, Kaczmarek D, Tiffet O. Intestinal pseudo-obstruction and pneumatosis cystoides intestinalis in a scleroderma patient. J Am Coll Surg. 2007;205:180–1.View ArticlePubMedGoogle Scholar
- De Gaetano AM, Calcagni ML, Rufini V, Valenza V, Giordano A, Bonomo L. Imaging of peritoneal carcinomatosis with FDG PET-CT: diagnostic patterns, case examples and pitfalls. Abdom Imaging. 2009;34:391–402.View ArticlePubMedGoogle Scholar
- Anthony MP, Khong PL, Zhang J. Spectrum of (18)F-FDG PET/CT appearances in peritoneal disease. AJR Am J Roentgenol. 2009;193:W523–9.View ArticlePubMedGoogle Scholar
- Puranik AD, Purandare NC, Agrawal A, Shah S, Rangarajan V. Imaging spectrum of peritoneal carcinomatosis on FDG PET/CT. Jpn J Radiol. 2014;32:571–8.View ArticlePubMedGoogle Scholar
- Buscombe J. PET imaging of inflammation. Q J Nucl Med Mol Imaging. 2014;58:284–9.PubMedGoogle Scholar
- Szamosi S, Szekanecz Z, Szucs G. Gastrointestinal manifestations in Hungarian scleroderma patients. Rheumatol Int. 2006;26:1120–4.View ArticlePubMedGoogle Scholar
- Jacobsen S, Ullman S, Shen GQ, Wiik A, Halberg P. Influence of clinical features, serum antinuclear antibodies, and lung function on survival of patients with systemic sclerosis. J Rheumatol. 2001;28:2454–9.PubMedGoogle Scholar
- Scussel-Lonzetti L, Joyal F, Raynauld JP, Roussin A, Rich E, Goulet JR, et al. Predicting mortality in systemic sclerosis: analysis of a cohort of 309 French Canadian patients with emphasis on features at diagnosis as predictive factors for survival. Medicine (Baltimore). 2002;81:154–67.View ArticleGoogle Scholar
- Steen VD, Medsger Jr TA. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum. 2000;43:2437–44.View ArticlePubMedGoogle Scholar
- Forbes A, Marie I. Gastrointestinal complications: the most frequent internal complications of systemic sclerosis. Rheumatology (Oxford). 2009;48 Suppl 3:36–9.Google Scholar
- Khanna D, Nagaraja V, Gladue H, Chey W, Pimentel M, Frech T. Measuring response in the gastrointestinal tract in systemic sclerosis. Curr Opin Rheumatol. 2013;25:700–6.View ArticlePubMedGoogle Scholar
- Fett N. Scleroderma: nomenclature, etiology, pathogenesis, prognosis, and treatments: facts and controversies. Clin Dermatol. 2013;31:432–7.View ArticlePubMedGoogle Scholar
- Di Ciaula A, Covelli M, Berardino M, Wang DQ, Lapadula G, Palasciano G, et al. Gastrointestinal symptoms and motility disorders in patients with systemic scleroderma. BMC Gastroenterol. 2008;8:7.View ArticlePubMed CentralPubMedGoogle Scholar
- Malandrini A, Selvi E, Villanova M, Berti G, Sabadini L, Salvadori C, et al. Autonomic nervous system and smooth muscle cell involvement in systemic sclerosis: ultrastructural study of 3 cases. J Rheumatol. 2000;27:1203–6.PubMedGoogle Scholar
- Roberts CG, Hummers LK, Ravich WJ, Wigley FM, Hutchins GM. A case-control study of the pathology of oesophageal disease in systemic sclerosis (scleroderma). Gut. 2006;55:1697–703.View ArticlePubMed CentralPubMedGoogle Scholar
- Marie I. Gastrointestinal involvement in systemic sclerosis. Presse Med. 2006;35:1952–65.View ArticlePubMedGoogle Scholar
- Sjolund K, Bartosik I, Lindberg G, Scheja A, Wildt M, Akesson A. Small intestinal manometry in patients with systemic sclerosis. Eur J Gastroenterol Hepatol. 2005;17:1205–12.View ArticlePubMedGoogle Scholar
- Parodi A, Sessarego M, Greco A, Bazzica M, Filaci G, Setti M, et al. Small intestinal bacterial overgrowth in patients suffering from scleroderma: clinical effectiveness of its eradication. Am J Gastroenterol. 2008;103:1257–62.View ArticlePubMedGoogle Scholar
- Soudah HC, Hasler WL, Owyang C. Effect of octreotide on intestinal motility and bacterial overgrowth in scleroderma. N Engl J Med. 1991;325:1461–7.View ArticlePubMedGoogle Scholar
- Marie I, Ducrotte P, Denis P, Hellot MF, Levesque H. Outcome of small-bowel motor impairment in systemic sclerosis - a prospective manometric 5-yr follow-up. Rheumatology (Oxford). 2007;46:150–3.View ArticleGoogle Scholar
- Emmanuel AV, Shand AG, Kamm MA. Erythromycin for the treatment of chronic intestinal pseudo-obstruction: description of six cases with a positive response. Aliment Pharmacol Ther. 2004;19:687–94.View ArticlePubMedGoogle Scholar
- Minami T, Nishibayashi H, Shinomura Y, Matsuzawa Y. Effects of erythromycin in chronic idiopathic intestinal pseudo-obstruction. J Gastroenterol. 1996;31:855–9.View ArticlePubMedGoogle Scholar
- Lock G, Holstege A, Lang B, Scholmerich J. Gastrointestinal manifestations of progressive systemic sclerosis. Am J Gastroenterol. 1997;92:763–71.PubMedGoogle Scholar
- Itou H, Iizuka M, Shindo K, Konno S, Watanabe S. A case of pseudoobstruction of the intestine associated with scleroderma dramatically responding to antibiotics. J Gastroenterol. 2006;41:711–2.View ArticlePubMedGoogle Scholar
- Denton CP, Black CM, Abraham DJ. Mechanisms and consequences of fibrosis in systemic sclerosis. Nat Clin Pract Rheumatol. 2006;2:134–44.View ArticlePubMedGoogle Scholar
- Terashima K, Shioyama Y, Nomoto S, Ohga S, Nonoshita T, Ohnishi K, et al. A case of radiation fibrosis appearing as, mass-like consolidation after SBRT with elevation of serum CEA. Case Rep Med. 2010;2010:986706.PubMed CentralPubMedGoogle Scholar
- Nakajima N, Sugawara Y, Kataoka M, Hamamoto Y, Ochi T, Sakai S, et al. Differentiation of tumor recurrence from radiation-induced pulmonary fibrosis after stereotactic ablative radiotherapy for lung cancer: characterization of 18F-FDG PET/CT findings. Ann Nucl Med. 2013;27:261–70.View ArticlePubMedGoogle Scholar
- Niccoli Asabella A, Nicoletti A, Altini C, Notaristefano A, Lastilla G, Rubini G. 18F-FDG positron emission tomography/computed tomography in the diagnosis and post-therapeutic treatment in a patient with an early stage of retroperitoneal fibrosis. Mol Imaging Radionucl Ther. 2013;22:60–2.View ArticlePubMed CentralPubMedGoogle Scholar
- Nishiyama Y, Yamamoto Y, Dobashi H, Kameda T. Clinical value of 18F-fluorodeoxyglucose positron emission tomography in patients with connective tissue disease. Jpn J Radiol. 2010;28:405–13.View ArticlePubMedGoogle Scholar
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