CASE REPORT
Desmosis coli as an extremely rare cause of constipation in children – case report and mini-review
1
Department of Paediatric Gastroenterology and Nutrition, Medical University of Warsaw, Warsaw, Poland
2
Department of Paediatric Surgery, Medical University of Warsaw, Warsaw, Poland
Submission date: 2022-12-29
Final revision date: 2023-03-13
Acceptance date: 2023-03-15
Publication date: 2023-08-30
Corresponding author
Jakub Michał Kucharski
Jakub Michał Kucharski, PhD, Department of Paediatric Gastroenterology and Nutrition, Medical University of Warsaw, Warsaw, Poland
Jakub Michał Kucharski, PhD, Department of Paediatric Gastroenterology and Nutrition, Medical University of Warsaw, Warsaw, Poland
Pediatr Pol 2023;98(3):254-257
KEYWORDS
TOPICS
ABSTRACT
Desmosis coli (DC) is an extremely rare condition of unknown pathogenesis. Only a few cases have been reported in the literature. It is characterised by the absence of connective tissue between muscle layers in the colon. Chronic constipation is the main manifestation of DC; thus, it largely resembles Hirschsprung disease. Moreover, neither DC nor Hirschsprung disease responds to conventional treatment. This paper describes a case of a patient with a diagnosis of DC. It was written to highlight the importance of proper diagnosis and to raise awareness of this uncommon disorder. A mini-review of the literature on DC is also provided.
REFERENCES (15)
1.
Steutel NF, Zeevenhooven J, Scarpato E, et al. Prevalence of functional gastrointestinal disorders in European infants and toddlers. J Pediatr 2020; 221: 107-114.
2.
Dybowska A, Kupniewska P, Sobacka A, Kroczek K, Krogulska A, Gałązka P. A child with chronic functional constipation – a challenge for a pediatrician alone or a surgeon as well? Case seriesand literature review. Pediatr Pol 2022; 97: 125-132.
3.
Chanpong A, Borrelli O, Thapar N. Hirschsprung disease and paediatric intestinal pseudo-obstruction. Best Pract Res Clin Gastroenterol 2022; 56-57: 101765.
4.
Desale J, Shah H, Kumbhar V, Sandlas G. Desmosis coli – a case report and review of the literature. Dev Period Med 2017; 21: 390-392.
5.
Hübner U, Meier-Ruge W, Halsband H. Four cases of Desmosis coli: severe chronic constipation, massive dilatation of the colon, and hypoperistalsis due to of changes in the colonic connective-tissue net. Pediatr Surg Int 2002; 18: 198-203.
6.
Marshall DG, Meier-Ruge WA, Chakravarti A, Langer JC. Chronic constipation due to Hirschsprung’s disease and Desmosis coli in a family. Pediatr Surg Int 2002; 18: 110-114.
7.
Meier-Ruge WA. Desmosis of the colon: a working hypothesis of primary chronic constipation. Eur J Pediatr Surg 1998; 8: 299-303.
8.
Szymańska S, Markiewicz-Kijewska M, Pyzlak M, et al. Pathogenesis of chronic constipation in a Polish group of paediatric patients – an attempt to create the optimal histopathological diagnostic protocol. Prz Gastroenterol 2019; 14: 109-111.
9.
Thapar N, Saliakellis E, Benninga MA, et al. Paediatric intestinal pseudo-obstruction: evidence and consensus-based recommendations from an ESPGHAN-Led Expert Group. J Pediatr Gastroenterol Nutr 2018; 66: 991-1019.
10.
Gfroerer S, Rolle U. Pediatric intestinal motility disorders. World J Gastroenterol 2015; 21: 9683-9687.
11.
Rajalakshmi T, Makhija P, Babu MK, Kini U. Intestinal neuronal dysplasia type A. Indian J Pediatr 2003; 70: 839-841.
12.
Toledo de Arruda Lourenção PL, Terra SA, Ortolan EVP, Rodrigues MAM. Intestinal neuronal dysplasia type B: a still little known diagnosis for organic causes of intestinal chronic constipation. World J Gastrointest Pharmacol Ther 2016; 7: 397-405.
13.
Nogueira A, Campos M, Soares-Oliveira M, et al. Histochemical and immunohistochemical study of the intrinsic innervation in colonic dysganglionosis. Pediatr Surg Int 2001; 17: 144-151.
14.
Wymer KM, Anderson BB, Wilkens AA, Gundeti MS. Megacystis microcolon intestinal hypoperistalsis syndrome: case series and updated review of the literature with an emphasis on urologic management. J Pediatr Surg 2016; 51: 1565-1573.
15.
Obata S, Fukahori S, Yagi M, et al. Internal anal sphincter achalasia: data from a nationwide survey of allied disorders of Hirschsprung’s disease in Japan. Surg Today 2017; 47: 1429-1433.
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