{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,1]],"date-time":"2025-10-01T16:35:22Z","timestamp":1759336522690,"version":"build-2065373602"},"reference-count":14,"publisher":"MDPI AG","issue":"19","license":[{"start":{"date-parts":[[2025,10,1]],"date-time":"2025-10-01T00:00:00Z","timestamp":1759276800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.mdpi.com"],"crossmark-restriction":true},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Obstruction differential diagnosis involves tumors, \u201cacute abdomen\u201d, and chronic pseudo-obstruction (CIPO). Pediatric CIPO cases have different backgrounds than adults\u2019 and impairs development. The cases are rare; diagnosis and treatment are still not well established. Diagnosis is complex; clinical, radiological, molecular, and manometric pathologic data are essential. The performance of broad radiological investigations and manometry is cumbersome in a small intestine. Herein, we present cases of a 14-year-old girl and 11-year-old boy with visceral myopathies (VMs). Presented cases show unique hardship in the analysis of standing and contrast bedside X-ray images\u2014the colon distension alone speaks to Hirschsprung, and the clinicians could not confirm suspected short-segment disease for a long time. VMs are usually diagnosed up to 12 months of life and accompanied by other organ dysfunctions, which are herein absent. The key features here were also the involvement of the small intestine, lack of distant colon contraction, and for the long-lasting case in the boy, loss of haustration. The initial diagnosis relied on clinical data (vomiting, malabsorption, &gt;6-month obstruction, and uncharacteristic biochemical tests), radiology (lack of tumor, enlargement of diameter, and fluid in small and large intestines), and manometry (presence of propagation wave and of anal inhibitory reflex in recto\u2013anal manometry). Examination of intestinal muscle biopsies involved hematoxylin-eosin, trichrome-Masson staining, and immunohistochemistry. The characteristics were fibrosis, small vacuoles, muscle layer thinning, and decreased expression of smooth muscle actin and desmin. The localization of biopsies was chosen after X-ray examination, due to interruption and with various degree changes. The final diagnosis was put forward after the analysis of all accessible data. The diagnosis of VM underlines the importance of interdisciplinary co-work. An earlier intestine muscle biopsy and well-designed molecular panel might fasten the process of diagnosis. Deeper exploration of phenotype\u2013genotype correlation of various VM presentations in the future is crucial for personalized treatment.<\/jats:p>","DOI":"10.3390\/diagnostics15192503","type":"journal-article","created":{"date-parts":[[2025,10,1]],"date-time":"2025-10-01T14:27:42Z","timestamp":1759328862000},"page":"2503","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Infrequent, but Not Intricate Radiological and Pathological Diagnosis of Chronic Intestinal Pseudo-Obstruction\u2014Presented in a Two Pediatrics Cases of the Visceral Myopathy"],"prefix":"10.3390","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8191-4239","authenticated-orcid":false,"given":"Monika","family":"Kujdowicz","sequence":"first","affiliation":[{"name":"Department of Pathomorphology, Jagiellonian University Medical College, Grzeg\u00f3rzecka 16, 31-531 Krakow, Poland"},{"name":"Department of Pathomorphology, University Children\u2019s Hospital of Krakow, Wielicka 265, 30-663 Krakow, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gra\u017cyna","family":"Drabik","sequence":"additional","affiliation":[{"name":"Department of Pathomorphology, University Children\u2019s Hospital of Krakow, Wielicka 265, 30-663 Krakow, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Damian","family":"M\u0142ynarski","sequence":"additional","affiliation":[{"name":"Department of Pathomorphology, University Children\u2019s Hospital of Krakow, Wielicka 265, 30-663 Krakow, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katarzyna","family":"J\u0119drzejowska","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, Institute of Pediatrics, Jagiellonian University Medical College, Wielicka 265, 30-663 Krakow, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4907-0520","authenticated-orcid":false,"given":"Wojciech","family":"G\u00f3recki","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, Institute of Pediatrics, Jagiellonian University Medical College, Wielicka 265, 30-663 Krakow, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna","family":"Wierdak","sequence":"additional","affiliation":[{"name":"Nutritional Treatment Department, University Children\u2019s Hospital of Krakow, Wielicka 265, 30-663 Krakow, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kamila","family":"P\u0142achno","sequence":"additional","affiliation":[{"name":"Nutritional Treatment Department, University Children\u2019s Hospital of Krakow, Wielicka 265, 30-663 Krakow, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7197-3334","authenticated-orcid":false,"given":"J\u00f3zef","family":"Kobos","sequence":"additional","affiliation":[{"name":"Department of Pathomorphology Central Clinical Hospital, Medical University of Lodz, Pomorska 251, 92-213 Lodz, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,10,1]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1148\/rg.210165","article-title":"Fluoroscopic Evaluation of Duodenal Diseases","volume":"42","author":"Johnson","year":"2022","journal-title":"Radiographics"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"933","DOI":"10.2214\/ajr.143.5.933","article-title":"Radiologic and histologic differentiation of neuromuscular disorders of the gastrointestinal tract: Visceral myopathies, visceral neuropathies, and progressive systemic sclerosis","volume":"143","author":"Rohrmann","year":"1984","journal-title":"Am. 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Biomolecules, 12.","DOI":"10.3390\/biom12121849"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"110908","DOI":"10.1016\/j.ijscr.2025.110908","article-title":"Delayed diagnosis and management of adult Hirschsprung\u2019s disease: Case report and literature review","volume":"128","author":"Jabareen","year":"2025","journal-title":"Int. J. Surg. Case Rep."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"e14715","DOI":"10.1111\/nmo.14715","article-title":"Chronic intestinal pseudo-obstruction in adults: A practical guide to identify patient subgroups that are suitable for more specific treatments","volume":"36","author":"Basilisco","year":"2024","journal-title":"Neurogastroenterol. Motil."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"G919","DOI":"10.1152\/ajpgi.00066.2021","article-title":"Visceral myopathy: Clinical syndromes, genetics, pathophysiology, and fall of the cytoskeleton","volume":"320","author":"Hashmi","year":"2021","journal-title":"Am. J. Physiol. Gastrointest. Liver Physiol."}],"container-title":["Diagnostics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-4418\/15\/19\/2503\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,1]],"date-time":"2025-10-01T14:32:08Z","timestamp":1759329128000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-4418\/15\/19\/2503"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,10,1]]},"references-count":14,"journal-issue":{"issue":"19","published-online":{"date-parts":[[2025,10]]}},"alternative-id":["diagnostics15192503"],"URL":"https:\/\/doi.org\/10.3390\/diagnostics15192503","relation":{},"ISSN":["2075-4418"],"issn-type":[{"value":"2075-4418","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,10,1]]}}}