Introduction
Inflammatory bowel diseases (IBDs), consisting of Crohn’s disease (CD) and ulcerative colitis (UC), are chronic bowel relapsing inflammatory disorders [
1]. CD can affect any segment of the gastrointestinal tract (usually, the terminal ileum or the perianal region) with a transmural inflammation with sporadic pattern. On the other hand, UC causes mucosal inflammation and is limited to the colon. The complications such as abscesses, fistulas, and strictures take place in CD more than in UC [
2].
IBDs begin most commonly during puberty and young adulthood, and rarely in infants [
3,
4]. Approximately 25% of patients with IBDs present before the age of 20 years [
5]. The peak onset of IBD among children is in adolescence, with 4% present before age 5 years and 18% before age 10 years [
6]. Very early-onset IBD (VEO-IBD) occurs before the age of 6 years; infantile IBD occurs before the age of 2 years and is extremely rare in infants under 1 year of age [
7‐
9]. Genetic, familial, and external environmental factors are implicated [
10,
11]. Childhood-onset IBD clinical characteristics usually progress rapidly and more aggressively in comparison with the features of the adult cohort, hence early diagnosis is imperative [
8,
12]. Thus, any child with persistent diarrhea, hematochezia, failure to thrive, and/or poor feeding should have IBD considered in the differential diagnosis. The diagnosis of IBD is based on a set of clinical, biochemical, stool, endoscopic, cross-sectional imaging, and histological investigations [
13].
We report herein a case series of 7-month-, 11-month-, and 12-month-old infants that presented with diarrhea, hematochezia, and pale appearance and were finally diagnosed with infantile IBD and treated.
Discussion
Even though the prevalence of IBD in children is expanding around the world [
14,
15], it is still extremely rare, so less than 1% of the pediatric population develop neonatal or infantile IBD [
16]. Between the types of IBD, ulcerative colitis appeared to be less common than Crohn’s disease in previous studies [
17‐
20].
Although the causes of IBD remain unclear, it is probably due to a combination of hereditary and environmental factors in addition to immune dysfunction [
21]. With regard to infants, IBD is frequently related to a serious course, broad colonic injury [
22], and perianal lesions [
20]. This is consistent with our series showing that the infants presented in cases 1 and 2 suffered from perianal lesions including ulcers and rash.
The patients presented in this series were diagnosed with Crohn’s disease in the second case, and ulcerative colitis in the first and third cases at ages 11, 7, and 12 months, respectively. All of the patients had suffered from severe diarrhea and pallor due to blood loss in feces.
The infants in case 1 and 2 were malnourished and failed to thrive. This actively demonstrates that this disease, when affecting infants, can cause weight loss and fatigue. Perianal lesions such as strictures and fistulas can be seen with the progression of the disease due to inadequate control [
23], as in our first two cases. Diagnosis of IBD may be difficult, especially in infants, owing to its similar symptoms to some other more common diseases such as allergy to milk, infections, and lymphoid follicular hyperplasia [
24]. This diagnosis requires several tests such as blood tests, stool studies, imaging, and endoscopy. What we noticed in case 1 was that calprotectin was significantly high and compatible with the diagnosis of inflammatory bowel disease (IBD), unlike what was shown in case 2, in which the calprotectin level was in the normal range and not compatible with the diagnosis of IBD. Upper and lower endoscopy with biopsies is considered the gold standard for IBD diagnosis [
25]. This was a typical example here where colonoscopy and the pathological findings in biopsies affirmed the diagnosis of infantile IBD in these three children presented in this series.
Even after being put on conservative treatment and going through immunosuppressive therapy, the infant in case 2 required surgery with resection of the colon. A previous study showed that IBD patients with gene mutation tend to have early onset of symptoms and more resistance to medical management [
26]. Our patient also showed severe and early onset of the symptoms, where he suffered from recurrent lower gastrointestinal bleeding at just 1 month of birth and was accepted into our department at the age of 7 months because of accompanying symptoms that worsened his health. Moreover, he also did not respond well to medical treatment. Shim
et al. reported that some gene mutations are accompanied by an increased risk of failure of medical treatment and undergoing early surgical interventions. Most of the patients presented with these mutations were diagnosed with Crohn’s disease, the same as our patient presented here who needed a surgical intervention [
25]. In conclusion, our patient is most likely to have a gene mutation. However, we could not relate this finding to our case because gene testing is unavailable in our country owing to financial limitations and war consequences. As for the remaining two patients, they responded well under treatment of corticosteroids, 5-acetylsalicylic acid, and antibiotics (i.e., metronidazole) and they were followed up for a few months after this treatment. However, cases 1 and 3 might show recurrence in the future, which may necessitate surgical resection of the bowel. Thus, close follow-up is needed.
Steroids were prescribed for the three cases for a short period as osteoporosis, diabetes, and disturbance in growth are consequences of long use. A previous study showed that steroid utilization is only suggested for a brief period in children [
24].
Neglected IBD for a long time in addition to late diagnosis could result in severe outcomes and complications [
23]. Despite the rarity of IBD in pediatric patients, early and prompt diagnosis is crucial to reduce potential severe consequences.
Conclusion
IBDs begin rarely in infants. Approximately 25% of patients with IBDs present before the age of 20 years. Very early-onset IBD (VEO-IBD) occurs before the age of 6 years; infantile IBD occurs before the age of 2 years and is extremely rare in infants under 1 year of age. So, early diagnosis and ruling out infantile IBD despite its rarity are recommended. Over and above that, new drugs such as vedolizumab, golimumab, and less invasive treatment methods should also be taken into consideration for better response and adequate remission with improved quality of life.
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