International Journal of Rheumatic Diseases 2013; 16: 595–598
APLAR GRAND ROUND CASE
Successful treatment of extensive intestinal perforations from Behcet’s disease involving the whole gut: a case report Shu-Cheng ZHANG and Wei-Lin WANG Department of Pediatric Surgery, Shengjing Hospital of China Medical University, Shenyang, Liaoning, China
Abstract Intestinal perforation resulting from Behcet’s disease has been previously well recognized. To date, fewer than 100 cases of this nature have been reported. Nearly the entire digestive system can be involved, including esophageal, gastric, duodenal, ileum, colon and anal tissues. Most of the reported perforations are single or assembled. Herein, an 8-year-old Chinese girl who developed Behcet’s disease with extensive intestinal perforations throughout the gut was successfully treated and is presented. The diagnosis of Behcet’s disease was established based on the typical presentations of recurrent oral aphthous ulcers, genital ulcers, typical eye lesions and a positive skin prick test. The extensive intestinal perforations were confirmed by laparotomy. Taking her developmental requirements into consideration, a simple perforation repair was carried out, followed by routine administration of immunosuppressive treatment, and the symptoms were temporarily relieved. Unfortunately, another ileocecal perforation occurred after 1.5 years, and an ileocolectomy was carried out. After the procedure, the girl recovered well. Clinicians should be aware of Behcet’s disease as a unique source of intestinal perforation. Although it is encountered rarely in clinical settings, this condition should be considered in each case of intestinal perforation. An ileocolectomy was the necessary choice for radical treatment, even in the case of a pediatric patient. Key words: clinical aspects, education, pediatric rheumatology.
INTRODUCTION Intestinal perforation resulting from Behcet’s disease has been previously well recognized.1–12 Nearly the entire digestive tract, including esophageal,3 gastric,4 duodenal,5,6 ileum,5,7–10 colon10–12 and anal tissues3 can be involved in this condition. Herein, we report an 8-year-old girl who developed Behcet’s disease with extensive intestinal perforations throughout the gut. The girl was successfully treated, and herein the diagnosis and treatment are presented.
CASE REPORT An 8-year-old girl was admitted to our hospital for intermittent abdominal pain for 1 month with a Correspondence: Professor Wei-Lin Wang, Department of Pediatric Surgery, Shengjing Hospital of China Medical University, 36 SanHao Street, Heping District, Shenyang 110004, China. Email: [email protected]
sudden exacerbation and an elevated temperature for 2 days. Nausea and vomiting were simultaneously present. Upon physical examination, tenderness with rebound tenderness and muscle tension were detected by palpation of the whole abdomen. The laboratory examination revealed a white blood cell count of 22.1 9 109/L and a C-reactive protein (CRP) of 129 mg/L. Abdominal radiology revealed a small amount of free gas under the diaphragm, suggesting an intestinal perforation. An emergency laparotomy was immediately carried out. During the operation, extensive intestinal perforation was confirmed, involving nearly the whole gut, with a more serious tendency noted in the ileocecal area (Fig. 1a,b). The diagnosis was established as extensive intestinal perforation. The primary disease was unclear; however, Behcet’s disease was suspected. A simple repair was conducted for the eight macroscopic perforations without a self-healing trend, and an indwelling peritoneal drain was left in place. On the second postoperative day, a consultation
© 2013 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd
S.-C. Zhang and W.-L. Wang
(a) (a)
(b) (b)
Figure 1 (a) Lots of red sclerosis with a pale center, measuring 0.5 9 0.5 cm, were observed in the intestinal serosa in almost the whole gut; the lesion was particularly serious in the ileocecal area where the largest diameter was about 1.5 cm. Some sclerosis had been enclosed by the omentum and other intestines, and some had been punctured. (b) When the intestine was dissected, a series of ulcers could be seen on the mucosa; parts of them had penetrated through the submucosa, muscularis and serosal surface.
was carried out with a dermatologist, an ophthalmologist, a pathologist, a rheumatologist and a digestive pediatrician. The medical history was carefully reviewed by the expert group. A painful recurrent oral ulcer was noted with an occurrence of once every 1–2 months for 2 years; it was self-healing each time without any treatment. Genital ulcers had been found on the patient’s labia minora at least three times over 2 years, which was mistaken as a perineal injury from novice cycling. At the same time, the girl had presented red eyes, periorbital pain, tears and other eye symptoms during the onset period prior to the admission. Upon physical examination, a large ulcer was observed on the oral mucosa, and multiple ulcers were observed on the genital mucosa (Fig. 2a,b). An intense and indurative
596
Figure 2 (a) Oral aphthous ulcers; (b) genital ulcer.
erythema with a small pustule was observed around the pinhole after intravenous injection, suggesting the skin prick test was positive. The signs of anterior uveitis, including conjunctival injection, ciliary flush, anterior chamber cells and flare, and fine keratic precipitates, were all obtained. Upon laboratory examination, the antistreptolysin O test, rheumatoid factor and antinuclear antibody, Weil–Felix reaction, purified protein derivative, polyarteritis nodosa (PAN), systemic lupus erythematosus, Wegener, perinuclear antineutrophil cytoplasmic antibodies (p-ANCA), cytoplasmic ANCA and anti corneum antibody (ASCA) were all negative. The final diagnosis of intestinal Behcet’s disease was then established. A glucocorticoid (prednisone, 20 mg/day) was administered for 5 days followed by gradual tapering of the dose. Oral methotrexate (5 mg/day) administration was also carried out once per week for 3 months. The patient was discharged on the eighth postoperative day. After that, she experienced an event-free period of 1.5 years. Unfortunately, another intestinal perforation was confirmed by abdominal film due to a sudden and severe abdominal
International Journal of Rheumatic Diseases 2013; 16: 595–598
Whole gut perforations in Behcet’s disease
pain, and the perforation was accompanied by oral and genital ulcers. A single ileocecal perforation was then confirmed and ileocolectomy was performed. During the operation, the ileocecum, along with 15 cm of the ileum, was removed. Simultaneously, glucocorticoid and oral methotrexate were administered for another 3 months. After that, the patient received a regular monthly follow-up. At present, no recurrence has been observed.
of the disease can be traced back 2 years; the symptoms of Behcet’s disease, such as the aphthous and genital ulcers and ocular symptoms, had already been presented, but they had not drawn attention. All the symptoms did not receive enough attention until the extensive intestinal perforations were confirmed by laparotomy and Behcet’s disease was taken into consideration. However, at that time vasculitis and thrombosis in the intestinal vessels due to Behcet’s disease had been so severe that the intestinal ulcers and perforations appeared inevitable. The diagnosis was a key point in this case. Due to the lack of specific serological and pathological features, the diagnosis of Behcet’s disease is mainly based on symptoms. Unfortunately, there are still no international standards for pediatric Behcet’s disease. Therefore, the diagnosis required the consideration of cases in adults. In this patient, the recurrent aphthous ulcers, recurrent genital ulcers, and typical ocular and cutaneous lesions were all presented, meeting the consensus standards for Behcet’s disease, allowing for the final diagnosis.1,2 Intestinal Behcet’s disease should be differentiated from inflammatory bowel disease.13–15 For example, the oral and digestive ulcers could present in Crohn’s disease, but the typical digestive lesions of Crohn’s disease are characterized by hyperplasia in segments. In most cases, pebble-like lesions in the ileum can be found. Neither of these characteristics are present in intestinal Behcet’s disease.13 Ulcerative colitis could be regarded as another important differentiation because of its similar digestive symptoms and the presence of lower digestive ulcers. However, these ulcers are mainly located in the distal colon; they can develop from the anus to the ileum and the condition is known as ‘irrigated ileitis’. A barium enema or colonoscopy can be used for the differential diagnosis.
DISCUSSION As an unusual presentation of Behcet’s disease, intestinal perforations have been well described before. To date, fewer than 100 cases of this nature have been reported3–12 (Table 1). Intestinal perforations in Behcet’s disease can develop throughout the digestive tract, such as the esophagus, gastric tissue, intestine and colon, and most of the perforations are single or assembled. The extensive involvement seen in the present patient is relatively rare. Her intestinal involvement was so extensive that perforations throughout the gut from the jejunum to the distal colon were observed. This extensive involvement might be related to the delayed diagnosis, which may be related to two factors. First, Behcet’s disease is mostly seen in countries along the ancient Silk Road from Eastern Asia to the Mediterranean Basin, and young adults between the second and fourth decades are the principally affected population. The present case was an 8-year-old girl from a rural Chinese area; both the race and age were outside those of the mainly affected population. The lower morbidity and the lack of knowledge of Behcet’s disease were the main reason for the delayed diagnosis. Second, the protracted course of the disease was also another important factor in the delay of the diagnosis. The onset
Table 1 The latest documented cases of reported intestinal Behcet’s disease with intestinal perforations Author
Date
Case
Age
Morimoto Y et al. Shin DY et al. Altintas E et al. Matsumoto K et al. Haller C et al. Hizawa K et al. Moon CM et al. Pirildar T et al. Sekmen U et al. Turan M et al. Isik B et al. Hamza M et al.
2005 2009 2009 2001 2003 2002 2010 2001 2008 2005 2005 1994
1 1 1 1 1 1 33 1 1 1 3 3
65 55 27 65 33 57 12–70 34 31 47
International Journal of Rheumatic Diseases 2013; 16: 595–598
20–30
Area
Sex
Involved organs
Japan Korea Turkey Japan Chad Japan Korea Turkey Turkey Turkey Turkey France
M M M F M F
Esophageal Gastic, colon Duodenal, ileum Duodenum Anus, cecum Ileum
F M M
Ileum, colon Colon Sigmoid colon Ileum, colon
M
597
S.-C. Zhang and W.-L. Wang
Another key point in this case was the treatment. For the treatment of intestinal Behcet’s disease, according to the ‘EULAR (European League Against Rheumatism) recommendations for the management of Behcet’s disease’, ileocolectomy or hemicolectomy should be carried to prevent reperforations whenever intestinal perforation is found.16,17 However, not all intestinal perforations are suitable for extensive ileocolectomy or hemicolectomy. For example, if the whole gut from the jejunum to the colon were involved as in this case, it would have been impossible to remove the ileocecum only. Most of all, the presented case was an 8-year-old girl who was still in development. Performing an extensive ileocolectomy or hemicolectomy as recommended may result in short bowel syndrome and lead to a series of development disorders later in life. However, in the presence of intestinal perforations, an emergency strategy must be performed. With the developmental requirements and the extensive intestinal involvement taken into consideration, a palliative strategy instead of the standard ileocolectomy was carried out in the present case; a simple intestinal repair was performed with a peritoneal drainage left, and then the symptoms were temporarily relieved. Unfortunately, due to the existence of ileocecum, reperforation appears to be inevitable; another ileocecal perforation occurred after 1.5 years, and the ileocolectomy had to be carried out. After that, the girl recovered well and no recurrence has been observed. This case supports the necessity of ileocolectomy for the treatment of intestinal perforations in Behcet’s disease. Hence, clinicians should be aware of Behcet’s disease as a unique source of intestinal perforation. Although it is encountered rarely in clinical settings, this condition should be considered in each case of intestinal perforation. An ileocolectomy was the necessary choice for radical treatment, even in the case of a pediatric patient.
ACKNOWLEDGMENTS There are no commercial or proprietary interests related to any drug, device or equipment mentioned in the submitted article, and institutional review board approval was received.
REFERENCES 1 Reprinted from International Study Group for Behcet’s Disease (1990) Criteria for diagnosis of Behcet’s disease. Lancet 335, 1078–80. 2 International Team for the Revision of the International Criteria for Behcet’s Disease (2006) Revision of the Inter-
598
3
4
5
6
7
8
9
10
11
12
13
14
15
16
17
national Criteria for Behcet’s Disease (ICBD). Clin Exp Rheumatol 24(Suppl 42), S14–5. Morimoto Y, Tanaka Y, Itoh T, Yamamoto S, Kurihara Y, Nishikawa K (2005) Esophagobronchial fistula in a patient with Behcet’s disease: report of a case. Surg Today 35, 671–6. Shin DY, Cheon JH, Park JJ et al. (2009) Serial episodes of gastric and cecal perforation in a patient with Behcet’s disease involving the whole gastrointestinal tract: a case report. Korean J Gastroenterol 53(2), 106–10. Altintasß E, Senli MS, Polat A, Sezgin O (2009) A case of Behcet’s disease presenting with massive lower gastrointestinal bleeding. Turk J Gastroenterol 20(1), 57–61. Matsumoto K, Yamada Y, Takeda R et al. (2001) A case of intestinal Behcßet disease with duodenal ulcer perforation. Nippon Shokakibyo Gakkai Zasshi 98, 953–8. Haller C, Guenot C, Odman M, Bruttin JM, Rosso R (2003) Recurrent anal abscess and cecal perforation as a first presentation of Behcet’s disease. Gastroenterol Clin Biol 27, 940–3. Hizawa K, Kai H, Hasegawa H et al. (2002) Ileal perforation in diffuse intestinal Behcet disease: report of a case. Surg Today 32, 167–9. Moon CM, Cheon JH, Shin JK et al. (2010) Prediction of free bowel perforation in patients with intestinal Behcet’s disease using clinical and colonoscopic findings. Dig Dis Sci 55, 2904–11. Pirildar T, Keser G, Tuncß E, Alkanat M, Tuncßyurek M, Do ganavsßargil E (2001) An unusual presentation of Behcet’s disease: intestinal perforation. Clin Rheumatol 20(1), 61–2. Sekmen U, Muftuoglu T, Sagiroglu J, Gungor O (2008) Multiple perforations along the entire colon as a complication of intestinal Behcet’s disease: a rare case. Saudi J Gastroenterol 14(2), 85–7. Turan M, Sen M, Koyuncu A, Aydin C, Arici S (2003) Sigmoid colon perforation as an unusual complication of Behcet’s syndrome: report of a case. Surg Today 33, 383–6. Marchetti F, Trevisiol C, Ventura A (2002) Intestinal involvement in children with Behcet’s disease. Lancet 359, 2115. Tabata M, Tomomasa T, Kaneko H, Morikawa A (1999) Intestinal Behcet’s disease: a case report and review of Japanese reports in children. J Pediatr Gastroenterol Nutr 29, 477–81. Portuesi MG, Lancia M, Ciuffreda L (2010) Problem of differential diagnosis between Crohn’s disease and colitis in Behcet’s disease: a case report. Reumatismo 62, 127–30. Chou SJ, Chen VT, Jan HC, Lou MA, Liu YM (2007) Intestinal perforations in Behcet’s disease. J Gastrointest Surg 11, 508–14. Wechsler B, L^e Thi Huong DB, Saadoun D (2009) EULAR recommendations for the management of Behcet’s disease: evidence-based or experience-based medicine. Rev Med Interne 30, 939–41.
International Journal of Rheumatic Diseases 2013; 16: 595–598
Copyright of International Journal of Rheumatic Diseases is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
APLAR GRAND ROUND CASE
Successful treatment of extensive intestinal perforations from Behcet’s disease involving the whole gut: a case report Shu-Cheng ZHANG and Wei-Lin WANG Department of Pediatric Surgery, Shengjing Hospital of China Medical University, Shenyang, Liaoning, China
Abstract Intestinal perforation resulting from Behcet’s disease has been previously well recognized. To date, fewer than 100 cases of this nature have been reported. Nearly the entire digestive system can be involved, including esophageal, gastric, duodenal, ileum, colon and anal tissues. Most of the reported perforations are single or assembled. Herein, an 8-year-old Chinese girl who developed Behcet’s disease with extensive intestinal perforations throughout the gut was successfully treated and is presented. The diagnosis of Behcet’s disease was established based on the typical presentations of recurrent oral aphthous ulcers, genital ulcers, typical eye lesions and a positive skin prick test. The extensive intestinal perforations were confirmed by laparotomy. Taking her developmental requirements into consideration, a simple perforation repair was carried out, followed by routine administration of immunosuppressive treatment, and the symptoms were temporarily relieved. Unfortunately, another ileocecal perforation occurred after 1.5 years, and an ileocolectomy was carried out. After the procedure, the girl recovered well. Clinicians should be aware of Behcet’s disease as a unique source of intestinal perforation. Although it is encountered rarely in clinical settings, this condition should be considered in each case of intestinal perforation. An ileocolectomy was the necessary choice for radical treatment, even in the case of a pediatric patient. Key words: clinical aspects, education, pediatric rheumatology.
INTRODUCTION Intestinal perforation resulting from Behcet’s disease has been previously well recognized.1–12 Nearly the entire digestive tract, including esophageal,3 gastric,4 duodenal,5,6 ileum,5,7–10 colon10–12 and anal tissues3 can be involved in this condition. Herein, we report an 8-year-old girl who developed Behcet’s disease with extensive intestinal perforations throughout the gut. The girl was successfully treated, and herein the diagnosis and treatment are presented.
CASE REPORT An 8-year-old girl was admitted to our hospital for intermittent abdominal pain for 1 month with a Correspondence: Professor Wei-Lin Wang, Department of Pediatric Surgery, Shengjing Hospital of China Medical University, 36 SanHao Street, Heping District, Shenyang 110004, China. Email: [email protected]
sudden exacerbation and an elevated temperature for 2 days. Nausea and vomiting were simultaneously present. Upon physical examination, tenderness with rebound tenderness and muscle tension were detected by palpation of the whole abdomen. The laboratory examination revealed a white blood cell count of 22.1 9 109/L and a C-reactive protein (CRP) of 129 mg/L. Abdominal radiology revealed a small amount of free gas under the diaphragm, suggesting an intestinal perforation. An emergency laparotomy was immediately carried out. During the operation, extensive intestinal perforation was confirmed, involving nearly the whole gut, with a more serious tendency noted in the ileocecal area (Fig. 1a,b). The diagnosis was established as extensive intestinal perforation. The primary disease was unclear; however, Behcet’s disease was suspected. A simple repair was conducted for the eight macroscopic perforations without a self-healing trend, and an indwelling peritoneal drain was left in place. On the second postoperative day, a consultation
© 2013 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd
S.-C. Zhang and W.-L. Wang
(a) (a)
(b) (b)
Figure 1 (a) Lots of red sclerosis with a pale center, measuring 0.5 9 0.5 cm, were observed in the intestinal serosa in almost the whole gut; the lesion was particularly serious in the ileocecal area where the largest diameter was about 1.5 cm. Some sclerosis had been enclosed by the omentum and other intestines, and some had been punctured. (b) When the intestine was dissected, a series of ulcers could be seen on the mucosa; parts of them had penetrated through the submucosa, muscularis and serosal surface.
was carried out with a dermatologist, an ophthalmologist, a pathologist, a rheumatologist and a digestive pediatrician. The medical history was carefully reviewed by the expert group. A painful recurrent oral ulcer was noted with an occurrence of once every 1–2 months for 2 years; it was self-healing each time without any treatment. Genital ulcers had been found on the patient’s labia minora at least three times over 2 years, which was mistaken as a perineal injury from novice cycling. At the same time, the girl had presented red eyes, periorbital pain, tears and other eye symptoms during the onset period prior to the admission. Upon physical examination, a large ulcer was observed on the oral mucosa, and multiple ulcers were observed on the genital mucosa (Fig. 2a,b). An intense and indurative
596
Figure 2 (a) Oral aphthous ulcers; (b) genital ulcer.
erythema with a small pustule was observed around the pinhole after intravenous injection, suggesting the skin prick test was positive. The signs of anterior uveitis, including conjunctival injection, ciliary flush, anterior chamber cells and flare, and fine keratic precipitates, were all obtained. Upon laboratory examination, the antistreptolysin O test, rheumatoid factor and antinuclear antibody, Weil–Felix reaction, purified protein derivative, polyarteritis nodosa (PAN), systemic lupus erythematosus, Wegener, perinuclear antineutrophil cytoplasmic antibodies (p-ANCA), cytoplasmic ANCA and anti corneum antibody (ASCA) were all negative. The final diagnosis of intestinal Behcet’s disease was then established. A glucocorticoid (prednisone, 20 mg/day) was administered for 5 days followed by gradual tapering of the dose. Oral methotrexate (5 mg/day) administration was also carried out once per week for 3 months. The patient was discharged on the eighth postoperative day. After that, she experienced an event-free period of 1.5 years. Unfortunately, another intestinal perforation was confirmed by abdominal film due to a sudden and severe abdominal
International Journal of Rheumatic Diseases 2013; 16: 595–598
Whole gut perforations in Behcet’s disease
pain, and the perforation was accompanied by oral and genital ulcers. A single ileocecal perforation was then confirmed and ileocolectomy was performed. During the operation, the ileocecum, along with 15 cm of the ileum, was removed. Simultaneously, glucocorticoid and oral methotrexate were administered for another 3 months. After that, the patient received a regular monthly follow-up. At present, no recurrence has been observed.
of the disease can be traced back 2 years; the symptoms of Behcet’s disease, such as the aphthous and genital ulcers and ocular symptoms, had already been presented, but they had not drawn attention. All the symptoms did not receive enough attention until the extensive intestinal perforations were confirmed by laparotomy and Behcet’s disease was taken into consideration. However, at that time vasculitis and thrombosis in the intestinal vessels due to Behcet’s disease had been so severe that the intestinal ulcers and perforations appeared inevitable. The diagnosis was a key point in this case. Due to the lack of specific serological and pathological features, the diagnosis of Behcet’s disease is mainly based on symptoms. Unfortunately, there are still no international standards for pediatric Behcet’s disease. Therefore, the diagnosis required the consideration of cases in adults. In this patient, the recurrent aphthous ulcers, recurrent genital ulcers, and typical ocular and cutaneous lesions were all presented, meeting the consensus standards for Behcet’s disease, allowing for the final diagnosis.1,2 Intestinal Behcet’s disease should be differentiated from inflammatory bowel disease.13–15 For example, the oral and digestive ulcers could present in Crohn’s disease, but the typical digestive lesions of Crohn’s disease are characterized by hyperplasia in segments. In most cases, pebble-like lesions in the ileum can be found. Neither of these characteristics are present in intestinal Behcet’s disease.13 Ulcerative colitis could be regarded as another important differentiation because of its similar digestive symptoms and the presence of lower digestive ulcers. However, these ulcers are mainly located in the distal colon; they can develop from the anus to the ileum and the condition is known as ‘irrigated ileitis’. A barium enema or colonoscopy can be used for the differential diagnosis.
DISCUSSION As an unusual presentation of Behcet’s disease, intestinal perforations have been well described before. To date, fewer than 100 cases of this nature have been reported3–12 (Table 1). Intestinal perforations in Behcet’s disease can develop throughout the digestive tract, such as the esophagus, gastric tissue, intestine and colon, and most of the perforations are single or assembled. The extensive involvement seen in the present patient is relatively rare. Her intestinal involvement was so extensive that perforations throughout the gut from the jejunum to the distal colon were observed. This extensive involvement might be related to the delayed diagnosis, which may be related to two factors. First, Behcet’s disease is mostly seen in countries along the ancient Silk Road from Eastern Asia to the Mediterranean Basin, and young adults between the second and fourth decades are the principally affected population. The present case was an 8-year-old girl from a rural Chinese area; both the race and age were outside those of the mainly affected population. The lower morbidity and the lack of knowledge of Behcet’s disease were the main reason for the delayed diagnosis. Second, the protracted course of the disease was also another important factor in the delay of the diagnosis. The onset
Table 1 The latest documented cases of reported intestinal Behcet’s disease with intestinal perforations Author
Date
Case
Age
Morimoto Y et al. Shin DY et al. Altintas E et al. Matsumoto K et al. Haller C et al. Hizawa K et al. Moon CM et al. Pirildar T et al. Sekmen U et al. Turan M et al. Isik B et al. Hamza M et al.
2005 2009 2009 2001 2003 2002 2010 2001 2008 2005 2005 1994
1 1 1 1 1 1 33 1 1 1 3 3
65 55 27 65 33 57 12–70 34 31 47
International Journal of Rheumatic Diseases 2013; 16: 595–598
20–30
Area
Sex
Involved organs
Japan Korea Turkey Japan Chad Japan Korea Turkey Turkey Turkey Turkey France
M M M F M F
Esophageal Gastic, colon Duodenal, ileum Duodenum Anus, cecum Ileum
F M M
Ileum, colon Colon Sigmoid colon Ileum, colon
M
597
S.-C. Zhang and W.-L. Wang
Another key point in this case was the treatment. For the treatment of intestinal Behcet’s disease, according to the ‘EULAR (European League Against Rheumatism) recommendations for the management of Behcet’s disease’, ileocolectomy or hemicolectomy should be carried to prevent reperforations whenever intestinal perforation is found.16,17 However, not all intestinal perforations are suitable for extensive ileocolectomy or hemicolectomy. For example, if the whole gut from the jejunum to the colon were involved as in this case, it would have been impossible to remove the ileocecum only. Most of all, the presented case was an 8-year-old girl who was still in development. Performing an extensive ileocolectomy or hemicolectomy as recommended may result in short bowel syndrome and lead to a series of development disorders later in life. However, in the presence of intestinal perforations, an emergency strategy must be performed. With the developmental requirements and the extensive intestinal involvement taken into consideration, a palliative strategy instead of the standard ileocolectomy was carried out in the present case; a simple intestinal repair was performed with a peritoneal drainage left, and then the symptoms were temporarily relieved. Unfortunately, due to the existence of ileocecum, reperforation appears to be inevitable; another ileocecal perforation occurred after 1.5 years, and the ileocolectomy had to be carried out. After that, the girl recovered well and no recurrence has been observed. This case supports the necessity of ileocolectomy for the treatment of intestinal perforations in Behcet’s disease. Hence, clinicians should be aware of Behcet’s disease as a unique source of intestinal perforation. Although it is encountered rarely in clinical settings, this condition should be considered in each case of intestinal perforation. An ileocolectomy was the necessary choice for radical treatment, even in the case of a pediatric patient.
ACKNOWLEDGMENTS There are no commercial or proprietary interests related to any drug, device or equipment mentioned in the submitted article, and institutional review board approval was received.
REFERENCES 1 Reprinted from International Study Group for Behcet’s Disease (1990) Criteria for diagnosis of Behcet’s disease. Lancet 335, 1078–80. 2 International Team for the Revision of the International Criteria for Behcet’s Disease (2006) Revision of the Inter-
598
3
4
5
6
7
8
9
10
11
12
13
14
15
16
17
national Criteria for Behcet’s Disease (ICBD). Clin Exp Rheumatol 24(Suppl 42), S14–5. Morimoto Y, Tanaka Y, Itoh T, Yamamoto S, Kurihara Y, Nishikawa K (2005) Esophagobronchial fistula in a patient with Behcet’s disease: report of a case. Surg Today 35, 671–6. Shin DY, Cheon JH, Park JJ et al. (2009) Serial episodes of gastric and cecal perforation in a patient with Behcet’s disease involving the whole gastrointestinal tract: a case report. Korean J Gastroenterol 53(2), 106–10. Altintasß E, Senli MS, Polat A, Sezgin O (2009) A case of Behcet’s disease presenting with massive lower gastrointestinal bleeding. Turk J Gastroenterol 20(1), 57–61. Matsumoto K, Yamada Y, Takeda R et al. (2001) A case of intestinal Behcßet disease with duodenal ulcer perforation. Nippon Shokakibyo Gakkai Zasshi 98, 953–8. Haller C, Guenot C, Odman M, Bruttin JM, Rosso R (2003) Recurrent anal abscess and cecal perforation as a first presentation of Behcet’s disease. Gastroenterol Clin Biol 27, 940–3. Hizawa K, Kai H, Hasegawa H et al. (2002) Ileal perforation in diffuse intestinal Behcet disease: report of a case. Surg Today 32, 167–9. Moon CM, Cheon JH, Shin JK et al. (2010) Prediction of free bowel perforation in patients with intestinal Behcet’s disease using clinical and colonoscopic findings. Dig Dis Sci 55, 2904–11. Pirildar T, Keser G, Tuncß E, Alkanat M, Tuncßyurek M, Do ganavsßargil E (2001) An unusual presentation of Behcet’s disease: intestinal perforation. Clin Rheumatol 20(1), 61–2. Sekmen U, Muftuoglu T, Sagiroglu J, Gungor O (2008) Multiple perforations along the entire colon as a complication of intestinal Behcet’s disease: a rare case. Saudi J Gastroenterol 14(2), 85–7. Turan M, Sen M, Koyuncu A, Aydin C, Arici S (2003) Sigmoid colon perforation as an unusual complication of Behcet’s syndrome: report of a case. Surg Today 33, 383–6. Marchetti F, Trevisiol C, Ventura A (2002) Intestinal involvement in children with Behcet’s disease. Lancet 359, 2115. Tabata M, Tomomasa T, Kaneko H, Morikawa A (1999) Intestinal Behcet’s disease: a case report and review of Japanese reports in children. J Pediatr Gastroenterol Nutr 29, 477–81. Portuesi MG, Lancia M, Ciuffreda L (2010) Problem of differential diagnosis between Crohn’s disease and colitis in Behcet’s disease: a case report. Reumatismo 62, 127–30. Chou SJ, Chen VT, Jan HC, Lou MA, Liu YM (2007) Intestinal perforations in Behcet’s disease. J Gastrointest Surg 11, 508–14. Wechsler B, L^e Thi Huong DB, Saadoun D (2009) EULAR recommendations for the management of Behcet’s disease: evidence-based or experience-based medicine. Rev Med Interne 30, 939–41.
International Journal of Rheumatic Diseases 2013; 16: 595–598
Copyright of International Journal of Rheumatic Diseases is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
