Unusual presentation of Crohn's Disease.
Objective of reporting this unusual case of Crohn's Disease is twofold. Firstly in western literature there appears to be an impression that it is very rare condition in Asian countries, although it perhaps is true but the fact remains that it does exist here both in children and adults. Main reason why it does not get detected, may be that because it gets drowned in the overwhelming prevalent tuberculous disease in our country as the symptoms in two conditions are somewhat similar. Second reason for reporting this case is the symptoms in this patient were predominantly gastrointestinal bleeding requiring several transfusions, which is not a predominant presenting feature in Crohn's disease.
KEY WORDS: Crohn's disease, Unusual presentation, Rectal bleeding, Misdiagnosis.
Crohn,s disease is a chronic inflammatory bowel disease with remitting and relapsing episodes. It is characterized by the fact that it almost always involves ileum, often has skip lesions, and often colon may be involved. It is four times more common in adults but also occurs in children, under the age of 15 years.1 Diagnosis is entertained on the basis of recurrent symptoms of abdominal pain and failure to thrive which dictates further laboratory and radiologic investigations.1 There is no single diagnostic test1,2, which may provide 100% accuracy. Combination of various factors is taken into account to arrive at a most plausible diagnosis.1,2 Its management is predominantly medical. Generally different complications dictate surgical intervention.
Here we are reporting the unusual symptoms of Crohn's disease in a 12 years old girl who presented to us with episodes of several rectal bleeding along with abdominal pain periodically over a period of three to four months. She had been to several hospitals and treated symptomatically and for abdominal tuberculosis without relief.
A 12 years old girl was admitted in our department with the complaints of abdominal pain, loose stools and with episodes of fresh gastrointestinal bleeding on and off for the last three to four months. There were also signs of failure to thrive.
She had history of multiple admissions to various hospitals with similar complaints requiring multiple blood transfusions. On the basis of the signs and symptoms she was diagnosed as a case of abdominal tuberculosis and was started on antituberculous therapy (ATT) without any relief and her abdominal pain and bleeding episodes continued to recur. Failure of response to treatment required admission to our department and this raised the question for the diagnosis to be reconsidered.
After basic hematological investigations we performed colonoscopy which did not show any lesion in the colon, because of our suspicion of it not being tuberculous pathology, we performed a minilaprotomy for diagnostic purpose, where there were no evidence of tuberculous lesions and only regional ileitis in terminal ileum with markedly enlarged adjacent mesenteric lymph nodes were noted.
Lymph node and omental biopsies were taken which further confirmed it not being a case of tuberculosis. Only nonspecific inflammatory process was noted. Patient was started on conservative line of treatment for Crohn,s disease which included aminosalicylic acid (ASA) and steroids.
However episodes of large GI hemorrhage continued, her hemoglobin dropping to low levels of 6 -7 g/dl. This required multiple blood transfusions. This forced us to perform definitive procedure. Terminal ileum, caecum and a small portion of ascending colon was removed and end to end ileocolic anastomosis was performed. No skip lesion was noted at the time of laparotomy.
Following surgery there was quick recovery and the improvement in general well being of patient. In the follow up of 6 months she has remained well without requiring any blood transfusions or any further treatment. Histopathology of excised ileum and caecum confirmed the diagnosis of Crohn's disease with non-involvement of caecum and excised portion of colon with disease free margin of proximal 3 cm of ileum.
Regular follow up has been done, especially due to the fact that these patients may develop recurrent disease with skip lesions.
Crohn's disease, previously believed to affect young adults, but considering the recent studies 50% of population affected by crohn's is under the age of 15 years.3 It is thought to be rare in Asians, although in the recent years there has been noticeable increase in number of inflammatory bowel disease (IBD) patients.4
There seems to be delay in the diagnosis of Crohn's disease particularly in children. Firstly the possibility is often not entertained in patients of this age group as incidence is low5, somewhat same thing happened in our case, secondly the disease may manifest with features of extra intestinal involvement, or with rare symptoms like Gastrointestinal (GI) bleeding which is very infrequent feature of Crohn's disease, although it is a prominent feature in ulcerative colitis.6-8 The most likely regional involvement in Crohn's disease is ileocolic variety which is about in 55% of cases, only colon alone is involved in 34.7% and isolated ileal disease is seen in just 9.7% cases.
Our patient presented with abdominal pain and recurrent profuse GI bleeding. She had already undergone several investigations in other hospitals, as mentioned previously which led to confusion in diagnosis, keeping in view the wide prevalence of tuberculosis in our country, it was assumed to be abdominal tuberculosis. Therefore she was put on a course of anti-tuberculous chemotherapy, without any relief of symptoms. Because of this parents sought further medical advice and at this point she was brought to us. Considering the history, we reconsidered her diagnosis, other than tuberculosis, which led us to re-investigate the patient. It is important here to mention that the girl presented with a rare presenting predominant symptom of Crohn's disease, in addition to distinct selective involvement of terminal ileum with sparing of colon.
The exploration and naked eye appearance of regional ileitis on mini-laparotomy along with contrast study giving a definitive picture of Crohn's with ileal involvement lead us to appropriate diagnosis, and after confirmation with histopathology enabled us to put her on the treatment of the Crohn's disease. Furthermore failure of conservative measure and continued episodes of bleeding required excision of diseased part of intestine.
The reason for reporting this case is that unless critical evaluation is entertained, sometimes prevalence of a common disease in an area can mask or overshadow the rare diseases with similar signs and symptoms. This may sometimes result in wrong and hazardous treatment.
In conclusion the purpose of reporting this case are two folds, firstly that the rare conditions are often missed especially when the sign and symptoms of the disease may be some what similar to commonly prevalent condition in that region. Secondly highest index of suspicion is needed especially when there are rare types of signs and symptoms as it was in our case where the predominant feature was repeated episodes was GI bleeding.
1. IBD Working Group of the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN). Inflammatory bowel disease in children and adolescents: recommendations for diagnosis -the Porto criteria. J Pediatr Gastroenterol Nutr 2005;41(1):1-7.
2. Zhou ZY, Luo HS. Differential diagnosis between Crohn's disease and intestinal tuberculosis in China. Int J Clin Pract 2006;60(2):212-214.
3. Turunen P, Kolho KL, Auvinen A, Iltanen S, Huhtala H, Ashorn M. Incidence of inflammatory bowel disease in Finnish children, 1987-2003. Inflamm Bowel Dis 2006;12(8):677-683.
4. Adi AJ, Lloyd GJ. Crohns disease: a case report. Nig Q J Hosp Med 2010;20(1):46-48.
5. Langholz E, Munkholm P, Krasilnikoff PA, Binder V. Inflammatory bowel diseases with onset in childhood. Clinical features, morbidity, and mortality in a regional cohort. Scand J Gastroenterol 1997;32(2):139-147.
6. Treepongkaruna S, Pienvichit P, Sornmayura P, Pornkul R, Wisedopas N, Phuapradit P. Inflammatory bowel disease in Thai children: presentations and outcomes of treatment. Asian Pac J Allergy Immunol 2006;24(1):73-79.
7. Veroux M, Angriman I, Ruffolo C, Barollo M, Buffone A, Madia C, et al. Severe gastrointestinal bleeding in Crohn's disease. Ann Ital Chir 2003;74(2):213-215.
8. Buller HA. Problems in diagnosis of IBD in children. Neth J Med 1997;50(2):S8-11.
1. Dr. Samreen Khushbakht, Postgraduate Student in Paediatric Surgery, 2. Naeem Khan, Professor of Paediatric Surgery, 1, 2: Department of Paediatric Surgery, KRL Hospital G-9-1 Mauve Area, Islamabad. Correspondence: Samreen Khushbakht, Post Graduate Student in Pediatric Surgery, Department of Paediatric Surgery, KRL Hospital, G-9-1 Mauve Area, Islamabad-Pakistan. E-mail: email@example.com, Received for Publication: March 19, 2012, 1st Revision Received: May 9, 2012, 2nd Revision Received: June 15, 2012, Final Revision Received: June 18, 2012
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|Author:||Khushbakht, Samreen; Khan, Naeem|
|Publication:||Pakistan Journal of Medical Sciences|
|Date:||Sep 30, 2012|
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