Background: The togetherness of invagination with celiac disease is an extremely rare condition especially in children. However, invagination may be the presenting symptom of celiac disease. Moreover, recurrent invaginations have been also reported in patients with celiac disease. Aim: To increase the awareness of clinicians about togetherness of these conditions. Case Presentation: Herein, we will discuss three children with diagnosis of celiac disease who presented with invagination and intestinal pseudo-obstruction. Conclusion: Children with pseudo-obstruction and invagination without an underlying etiology should be evaluated for the presence of celiac disease, especially if they have accompanying growth retardation or anemia and if they are at an unusual age for invagination. Further studies are warranted to elucidate the exact relationship of invagination with celiac disease.
Invagination is one of the most common causes of gastrointestinal obstruction in children [
Celiac disease is an autoimmune enteropathy affecting genetically predisposed patients [
Herein, we will discuss three children with diagnosis of celiac disease who presented with invagination and intestinal pseudo-obstruction.
By this way we aimed to increase the awareness of clinicians about togetherness of these conditions.
A nine-year-old boy was admitted to the hospital with abdominal pain and bilious vomiting for three days. His vital signs were normal. His weight was 20 kg (<5th percentile) and height was 124 cm (10th percentile). In physical examination, abdominal distention and diffuse tenderness on palpation were detected. Moreover, a mass lesion on the left lower quadrant was palpated. Laboratory test results were as follows: hemoglobin (Hgb), 9 g/dl (14 - 18); white blood count (WBC) , 22 × 103/uL (4.5 - 11), C-reactive protein (CRP), 4.8 mg/dL (0 - 5), and Na 128 mmol/L (134 - 145). There was free air under the diaphragm in standing direct abdominal graph (SDAG) together with multiple air-fluid levels (
ileo-ileal invagination of 5.8 cm in length in the left lower quadrant, minimal free fluid between intestinal loops, an extensive dilatation between colonic segments (
A six-year-old girl was admitted to the hospital with colic type abdominal pain and recurrent diarrhea. These self-limited episodes were lasting about one week. She was cachectic in appearance with a history of anorexia. Her weight was 17 kg (10th-25th percentile) and her height was 110 cm (25 percentile). Her abdomen was dis- tended without any tenderness on palpation. Laboratory test results were as follows: Hemoglobin, 10.2 g/dl (14 - 18); WBC, 13.5 × 103 /uL (4.5 - 11), CRP, 10 (0 - 5) mg/dL, and Na 132 mmol/L (135 - 145). There were multiple fluid-air levels in SDAG. In abdominal ultrasound, distention in intestinal loops, and a reductable, transient ileo-ileal donut-shaped invagination with a 4 cm diameter was present in right upper quadrant. Control sonograms obtained after reduction of invagination were unremarkable. Her serum tTG IgG, and tTG IgA levels were 87 U/ml, and 100 U/ml., respectively. Endoscopic duodenal biopsy results revealed celiac disease with Marsh III histopathological appearance-intraepithelial lymphocytosis. With GFD, the asymptomatic patient gained 7 kg of weight within 1 year.
A ten-year-old boy was admitted to the hospital with abdominal pain, constipation, vomiting and distention for 6 months. It was learnt that he had ileus attacks, and the last attack was 2 months ago which did not require surgical interventions. In physical examination, abdomen was distended with diffuse tenderness on palpation. In auscultation, the abdominal sounds were hyperactive. His Hgb level was 9.8 g/dl and CRP, erythrocyte sedimen- tation rate and electrolyte levels were within normal limits . His serum tTG IgG, andtTG IgA levels were 56 U/ ml, and 68 U/ml, respectively . There was an air fluid level in abdominal X-ray as a sign of intestinal obstruction (
We have presented three children admitted to the hospital with the invagination symptoms and concomitant diagnosis of CD. Invagination is the prolabation of a part of intestinal loops into the lumen of the adjacent segment.
It is generally an idiopathic condition reported more commonly in children [
Gluten sensitive enteropathy, CD, is an autoimmune reaction to the gliadin fraction of gluten present in cereals as barley, rye and wheat [
On the other hand, CD patients may present with atypical symptoms such as infertility, abdominal distention
or constipation. The prevalence of acute abdominal pain in CD patients is 3% and chronic or recurrent abdo- minal pain may also be the presenting symptom of CD [
The invagination prevalence among CD patients was reported as 1.2 - 18 percent. Hizal et al. reported that transient invagination may accompany 20% of CD patients and Reilly et al. reported that in children with CD risk of invagination increased 10 - 20 times [
In conclusion, children with pseudo-obstruction and invagination without an underlying etiology should be evaluated for the presence of CD especially if they have accompanying growth retardation or anemia and if they are at an unusual age for invagination. Further studies are warranted to elucidate the exact relationship of invagination with CD.
Written informed consent was obtained from the patients’s legal guardians 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.
The authors declare that they have no competing interests
Sevgi Buyukbese Sarsu designed the study and acquired the data; drafted the article and revised it critically for important intellectual content. Mustafa Demirci made radiologic examination.
Sevgi Buyukbese Sarsu,Mustafa Demirci, (2016) Unusual Initial Presentation Of Celiac Disease in Children: Three Case Reports. Open Journal of Pediatrics,06,53-59. doi: 10.4236/ojped.2016.61010