Crohn's Disease Presenting as Palatal Ulcer - MedIND

57

Clinical Brief

Crohn’s Disease Presenting as Palatal Ulcer R. Ganesh, N. Suresh, S. Ezhilarasi, Sarala Rajajee and Malathi Sathiyasekaran Kanchi Kamakoti CHILDS Trust Hospital, Chennai, India

Abstract. Crohn’s disease (CD) in children younger than 5 years of age is termed as early onset inflammatory bowel disease (EO-IBD). We report a 4 yr 6 mo-old child with EO-IBD, who presented with palatal ulcer, an extra intestinal manifestation of Crohn’s disease as the dominant feature. [Indian J Pediatr 2006; 73 (3) : 229-231] E-mail : [email protected] Key words: Crohn’s disease; EO-IBD; Children; Steroids

Crohn’s disease (CD) is a chronic inflammatory bowel disease characterized by transmural granulomatous inflammation involving any part of the gastrointestinal tract in a discontinuous manner. The incidence of pediatric CD in US varies from 0.2-8.5 per 1,00,000, and children less than 5 years of age constitute 4% of the pediatric CD.1 This “early onset IBD (EO-IBD)” has been recognized as a special subset of IBD2 because of its great potential to affect the growth and development. A recent study from Chennai had reported the clinical manifestations of Crohn’s disease in children and adolescents.3 This case report is to highlight the unusual age and presentation of Crohn’s disease. CASE REPORT A 4-year-6 month-old-boy presented with fever, palatal ulcer and odynophagia of one-month duration. He also had crampy abdominal pain associated with passage of loose stools, occasionally tinged with blood for 2 weeks with loss of weight. There was no history of arthralgia, rash, pedal edema, jaundice or contact with tuberculosis. On examination, he was febrile, weighed 17 kg and his height was 102 cms. An irregular ulcer 1x2 cm with raised margins was seen on the soft palate. There was no significant lymphadenopathy. General and systems examination were normal. Investigations showed a Hb of 11.9 gm/dl; leukocyte count was 10,900 cells/cu. mm with neutrophilic predominance. Reactive protein was positive 96 mg/dl

Correspondence and Reprint requests : Dr. Malathi Sathiyasekeran, M.D (Ped), D.C.H, DM, Consultant Pediatric Gastroenterologist, No 1,16 th Cross Street, Indra Nagar, Chennai 600 020, Tamil Nadu, India.

Indian Journal of Pediatrics, Volume 73—March, 2006

and erythrocyte sedimentation rate (ESR) was 40 mm at 1 hour. Chest skiagram and ultrasound abdomen were normal. Antinuclear antibodies, antibodies to HIV and herpes simplex viral antigens were negative. Liver function tests (LFT) and renal function tests (RFT) were normal. Cultures of blood, urine and bone marrow showed no growth. Upper GI endoscopy showed erythematous antral gastritis, and biopsy revealed focal active granulomatous gastritis (Fig 1). Ileocolonoscopy showed multiple aphthous ulcers in sigmoid, descending and transverse colon. Rectum and terminal ileum were normal. Biopsy from these lesions revealed non-caseating granulomas, consistent with Crohn’s disease (Fig. 2). Biopsy from oral ulcer was not taken; however, smear for AFB and fungal elements were negative. A diagnosis of early onset Crohn’s disease with moderate to severe activity was made. The child was treated with IV methyl prednisolone, followed by oral prednisolone 2 mg/kg/ day, tapered over 6 weeks and stopped. Mesalamine 50 mg/kg/day was also started. Supplemental enteral

Fig. 1. Photomicrograph of antral mucosa with features of focally active granulomatous gastritis (H & E 40 X)

229

58

R. Ganesh et al

Fig. 2. Photomicrograph of colonic mucosa with increase in lamina propria cellularity, few granuloma and multinucleated giant cell. (H & E 200 X)

nutrition was advised for one week, followed by an adequate calorie home-made diet. On initiating therapy, his fever settled within 48 hours, appetite improved, stools became normal; the ulcer healed in 4 days and he gained 2 kg weight in one month. This child is being presented for the uncommon age and unusual mode of presentation of Crohn’s disease. DISCUSSION Inflammatory Bowel Disease (IBD) is an important cause of chronic morbidity in childhood and adolescence 4. Crohn’s disease (CD) and ulcerative colitis (UC) are the two important entities of IBD. The median age of presentation of CD is 7.9 years, and 4% of pediatric CD occurs below the age of 5.1 This is probably the youngest case of CD reported from India. The pathogenesis of CD is still unclear and most likely due to interplay of genetic, environmental and immune factors. The CD susceptible gene NOD2/CARD 15 (Caspase Activation Recruitment Domain) has been identified on chromosome 16 and mutations in this region is associated more with EO-IBD.5 The common gastrointestinal manifestations are abdominal pain, diarrhea, fever and weight loss.6 The location and extent of disease involvement of the gastrointestinal tract determine the clinical presentation of CD. Extra-intestinal manifestations are seen in 25-35% of children with IBD and may outweigh those related to the GIT by several years. Recurrent aphthous ulcer is the most common oral manifestation of IBD and is a frequent finding in CD. The other disease-specific oral manifestations include mucosal tags, lip swelling, fissures, buccal mucosal swelling or cobble stoning, deep linear ulcerations and localised mucogingivitis.7 This child had only a palatal ulcer and none of the reported lesions. The oral lesions being directly visible is more 230

accessible for tissue diagnosis. However, if other sites show a characteristic granuloma, then oral biopsy is not clinically justified. 7 The diagnosis of CD is by a combination of clinical, laboratory, endoscopic and histological findings. The rectum is usually spared in CD and the inflammation is asymmetric with deep, irregular ulcers surrounded by normal looking mucosa. Focal enhanced gastritis and identification of microgranuloma on antral biopsy has also helped in increasing the diagnostic yield. 8 The role of serological assay in the diagnostic armamentarium of IBD is not very significant. The goals of treatment for children with CD are to achieve the best clinical and laboratory control with least possible side effects from medications, to promote growth through adequate nutrition, and to permit the child to function as normal as possible. The medical management of CD consists of 5-aminosalicylic acid (5ASA) with or without steroids depending on the activity of the disease. Immunosuppressive drugs like azathioprine, 6 mercaptopurine, cyclosporin A and methotrexate are used when there is failure to respond to corticosteroid or if the child is steroid-dependent. 9 Infliximab has proved effective in short-term treatment of moderate to severe CD in children. Nutritional therapy is an important modality for treatment during disease activity and growth failure. Surgery is indicated in the presence of obstruction, hemorrhage or perforation. CD in children unlike in adults has a significant impact on its nutrition, growth and development in addition to the psychological stress.10 It is therefore essential that an early diagnosis is made and appropriate treatment is initiated. The total management of a child with CD is a multidisciplinary approach involving the pediatrician, pediatric gastroenterologist, nutritionist, psychologist and surgeon. REFERENCES 1. Baldassano RN, Piccoli DA. Inflammatory bowel disease in pediatric and adolescent patients. Gastroenterol Clin North Am 1999; 28: 445-455. 2. Mamula P, Telega GW, Markowitz JE, Brown KA, Russo PA, Piccoli DA et al. Inflammatory bowel disease in children 5 years of age and younger. Am J Gastroenterol 2002; 97 : 2005 2010. 3. Malathi S, BhaskarRaju B, Shivbalan So, Rajarajan K. Pediatic Crohn’s Disease in South India. Indian Pediatr 2005; 42 : 459 463. 4. Cuffari C, Darbari A. Inflammatory bowel disease in the pediatric and adoloscent patient. Gastroenterol Clin N Am 2002; 31 : 275-291. 5. Bonen DK, Cho JK. The genetics of inflammatory bowel disease. Gastroenterol 2003; 124 : 521-536. 6. Hyams JS. Crohn’s disease. In Wyllie R, Hyams JS, eds. Pediatric Gastrointestinal Disease: Pathophysiology, Diagnosis, Management. Philadelphia; WB Saunders 1999:401-18. 7. Pittock S, Drumm B, Fleming P, Mc Dermott M, Imrie C, Flint S et al. The oral cavity in Crohn’s disease. J Pediatr 2002; 138(5) : 767-771. 8. Abdullah BA, Gupta SK, Croffie JM, Pfefferkorn MD,


59

Crohn's Disease Molleston JP, Corkins MR et al. The role of oesophagoduodenoscopy in the initial evaluation of childhood inflammatory bowel disease: A 7 year study. J Pediatr Gastroenterol 2002; 35; 636-640. 9. Markowitz J, Grancher K, Kohn N, Daum F. Immunomo dulatory therapy for pediatric inflammatory bowel disease :

changing patterns of use, 1999-2000. Am J Gastroenterol 2002; 97(4): 928-932. 10. Mamula P, Markowitz JE, Baldassano RN. Inflammatory bowel disease in early childhood and adoloscence:special considerations. Gastroenterol clin North Am 2003; 32 : 967-995.

Notes and News

Pediatric Advanced Life Support (PALS) Provider Course Christian Medical College and Hospital, Ludhiana, Punjab

Intensive care chapter, IAP of Punjab State Branch is organizing PALS provider course on 29, 30th April, 2006 at Christian Medical College and Hospital, Ludhiana, Punjab. The workshop is limited to 40 pediatricians on first come first served basis. The course fee is Rs 2000. For further details, please contact the undersigned Dr Jugesh Chhatwal, Prof and Head of Pediatrics, Christian Medical College and Hospital, Ludhiana, Punjab; E-mail: [email protected]; Mobile : 9316919718. Dr Puneet Aulakh Pooni, Secretary, ICC-IAP, Punjab State Branch, Assist Prof of Pediatrics, Dayanand Medical College and Hospital, Ludhiana. E-mail : [email protected]; Mobile : 9815539292

MAHAPEDICON 2006

Organised by: IAP Solapur Branch (3rd, 4th and 5th November 2006)

Conference Secretariat: Dr Hemant S. Sathe, Sathe Nursing Home, 131, Murarji Peth, Monalisa Chambers, Lucky Chowk, Solapur. Ph: (0217) 2728767; Fax : (0217) 2723448

8th National Congress on Pediatric Critical Care NCPCC Bangalore NOVEMBER 10-12, 2006 Contact : Dr Girish HC, Organizing Secretary, NCPCC Bangalore, KK Hospital, 979, 25th Main Road BSK 1st Stage, Bangalore-560050; Mobile : 98452-72933; Website : www.ncpccbangalore.com; E-mail : [email protected]

ICAAICON 2006 40th National Conference on Allergy, Asthma and Applied Immunology (Thurday 7th to Sunday 10th December, 2006) Venue : Desh Bhagat Yaadgar Hall, Jalandhar, Organising Secretary : Dr. H.J. Singh, Ranjit Hospital, Patel Chowk, Jalandhar, Tele.: 0181-2620600, Fax : 2620700; Cell : 09814217738. E-mail : [email protected]; Website : www.ICAAICON2006.com Please register with Dr. H.J. Singh Indian Journal of Pediatrics, Volume 73—March, 2006

231

60

Notes and News

20th CONGRESS OF ASIAN ASSOCIATION OF PEDIATRIC SURGEONS WITH EXECUTIVE NOVEMBER 12-15, 2006

Meeting of WOFAPS, at Taj Hotel and Convention Center, New Delhi. Organizing Chairman, Prof. D.K. Gupta, Head, Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi 110029. Fax : 011-2658 8663, 26588641. Website : www.aaps2006.com; E-mail : [email protected] November 11-12, 2006 Pre-congress Live Operative Workshop on Anorectal Malformation (led by Prof Alberto Pena), at AIIMS, New Delhi. Pediatric intensive nursing care, including resuscitation November 12, 2006, Conference Hall, AIIMS, New Delhi. November 16-17, 2006 Post Congress Live Operative Workshop on Pediatric Urology (VUR, Intersex and Hypospadias) (Led by Prof Prem Puri, Prof Snodgrass, Mr. A. Bracka, Prof. John Hutson, Prof. Asopa) at AIIMS, New Delhi.

27 UP PEDIACON 2006

VP Chapter of PEDICON in being organised by the Department of Pediatrics K.G.M.U. on 14th

and 15th October 2006

Organising Secretary: Prof. K.L. Srivastava, H.O.D., Department of Pediatrics, K.G.M.U., Lucknow 226003. Ph: (R) 0522-2480060; (o) 0522-2257243, Fax : 91-522-2258410, Mobile : 9415016634

STATE-OF-THE-ART WORKSHOP ON NEONATAL RESUSCITATION A State-of-the-art workshop on Neonatal Resuscitation (as per latest 2006 guidelines) is being organized by the Department of Pediatrics, AIIMS, on Sunday 2nd April 2006 (9.00 am to 5.00 pm). The workshop is targeted for practicing pediatricians/obstetricians and postgraduate students. Registration would be limited to 40 participants on the first-come-first-served basis. Please send a draft of Rs. 500/- drawn in favour of “CME Programme in Neonatalogy”, payable at New Delhi latest by 15 th March, 2006. Participants will be provided with latest resource book at least two weeks before the workshop. Please note there will be no provision for spot registration. For further details, please contact Dr. Ramesh Agarwal, Assistant Professor, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi-110029. Tel-001-26593621; Fax : 011-26862663, E-mail : [email protected]

LAKESIDE EDUCATION TRUST 24th Annual CME ON Sunday 23rd July 2006, At Hotel Atria Allergic Disorders in Pediatric Practice For Details Contact : Dr. H. Paramesh, Chairman, Lakeside Education Trust, Pediatrician-in–Chief & Pediatric Pulmonologist, C/o Lakeside Medical Center & Hospital, 33/4, Meanee Avenue Road, Near Ulsoor Lakeside Medical Center and Hospital, Bangalore–560042. Phone : 080-25303677, 25304276, 25566738, 25366723, 25512934 Fax : 25303677, E-mail : [email protected]

232
