{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,8]],"date-time":"2026-06-08T06:58:22Z","timestamp":1780901902108,"version":"3.54.1"},"reference-count":58,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2026,6,8]],"date-time":"2026-06-08T00:00:00Z","timestamp":1780876800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Background and Clinical Significance: Chronic intestinal pseudo-obstruction (CIPO) clinically presents as a mechanical obstruction. When patients present with signs of impending ischemia, emergency exploratory laparotomy is indicated. Differentiating CIPO from other etiologies preoperatively is highly challenging, making intraoperative findings and definitive histopathology crucial for an accurate diagnosis. This report aims to elucidate a novel macroscopic presentation of myopathic CIPO and explore the potential biomechanical mechanisms underlying the observed structural anomaly. Case Presentation: A 37-year-old male with no prior abdominal surgery presented with a high-grade bowel obstruction and early signs of ischemia. Emergency laparotomy revealed a unique \u201cbraided dough twist\u201d anomaly of the distal small bowel, prompting a segmental resection. Subsequent histopathological and immunohistochemical evaluations revealed severe outer longitudinal muscle atrophy with an intact enteric nervous system, confirming myopathic CIPO. Following surgery, the patient recovered uneventfully with significant nutritional improvement. Conclusions: Myopathic CIPO can manifest as a striking \u201cbraided\u201d bowel anomaly\u2014a macroscopic presentation previously undocumented in the literature. This case illustrates that determining the optimal extent of resection relies heavily on intraoperative visual and tactile assessment, and that comprehensive histopathological profiling is essential for establishing a definitive diagnosis and elucidating the underlying biomechanical mechanisms of this unique structural anomaly.<\/jats:p>","DOI":"10.3390\/diagnostics16121762","type":"journal-article","created":{"date-parts":[[2026,6,8]],"date-time":"2026-06-08T05:43:46Z","timestamp":1780897426000},"page":"1762","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Unmasking the \u201cBraided\u201d Bowel: Chronic Intestinal Pseudo-Obstruction\u2014A Case Report"],"prefix":"10.3390","volume":"16","author":[{"ORCID":"https:\/\/orcid.org\/0009-0002-3895-5518","authenticated-orcid":false,"given":"Chun-Heng","family":"Hung","sequence":"first","affiliation":[{"name":"Department of Surgery, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan 704302, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4947-1463","authenticated-orcid":false,"given":"Chung-Ta","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Pathology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan 704302, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1511-0080","authenticated-orcid":false,"given":"Po-Chuan","family":"Chen","sequence":"additional","affiliation":[{"name":"Division of Colorectal Surgery, Department of Surgery, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan 704302, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,6,8]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1097\/MCG.0000000000001047","article-title":"Pathophysiology, Diagnosis, and Management of Chronic Intestinal Pseudo-Obstruction","volume":"52","author":"Downes","year":"2018","journal-title":"J. Clin. Gastroenterol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"S22","DOI":"10.1016\/j.jviscsurg.2022.01.001","article-title":"Small Intestine Motility Disorders: Chronic Intestinal Pseudo-Obstruction","volume":"159","author":"Billiauws","year":"2022","journal-title":"J. Visc. Surg."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"882","DOI":"10.1136\/gut.2009.200444","article-title":"The London Classification of Gastrointestinal Neuromuscular Pathology: Report on Behalf of the Gastro 2009 International Working Group","volume":"59","author":"Knowles","year":"2010","journal-title":"Gut"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"749","DOI":"10.1097\/PAS.0b013e31819b381a","article-title":"The Role of Immunohistochemistry in Idiopathic Chronic Intestinal Pseudoobstruction (CIPO): A Case-Control Study","volume":"33","author":"Amiot","year":"2009","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_5","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_6","doi-asserted-by":"crossref","first-page":"e13231","DOI":"10.1111\/nmo.13231","article-title":"Chronic Intestinal Pseudo-Obstruction: Progress in Management?","volume":"29","author":"Karunaratne","year":"2017","journal-title":"Neurogastroenterol. Motil."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1016\/j.gtc.2019.08.005","article-title":"Management of the Patient with Chronic Intestinal Pseudo-Obstruction and Intestinal Failure","volume":"48","author":"Pironi","year":"2019","journal-title":"Gastroenterol. Clin. N. Am."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"638","DOI":"10.1007\/s12262-022-03507-z","article-title":"A Rare Case of Acute on Chronic Intestinal Pseudo-Obstruction Secondary to Primary Visceral Myopathy","volume":"85","author":"Philip","year":"2023","journal-title":"Indian J. Surg."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1587","DOI":"10.1007\/s00330-008-0913-1","article-title":"Chronic Intestinal Pseudo-Obstruction in Adult Patients: Multidetector Row Helical CT Features","volume":"18","author":"Merlin","year":"2008","journal-title":"Eur. Radiol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"e12191","DOI":"10.1111\/nmo.12191","article-title":"Non-Transplantation Surgical Approach for Chronic Intestinal Pseudo-Obstruction: Analysis of 63 Adult Consecutive Cases","volume":"25","author":"Sabbagh","year":"2013","journal-title":"Neurogastroenterol. Motil."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"8","DOI":"10.5056\/jnm241031","article-title":"Surgical and Therapeutic Interventions for Chronic Intestinal Pseudo-Obstruction: A Scoping Review","volume":"31","author":"Dalby","year":"2025","journal-title":"J. Neurogastroenterol. Motil."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"G431","DOI":"10.1152\/ajpgi.00182.2016","article-title":"Regulation and Dysregulation of Esophageal Peristalsis by the Integrated Function of Circular and Longitudinal Muscle Layers in Health and Disease","volume":"311","author":"Mittal","year":"2016","journal-title":"Am. J. Physiol. Gastrointest. Liver Physiol."},{"key":"ref_13","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."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1007\/978-3-031-05843-1_2","article-title":"Clinical and Pathological Features of Severe Gut Dysmotility","volume":"Volume 1383","author":"Bianco","year":"2022","journal-title":"The Enteric Nervous System II"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"660","DOI":"10.1002\/jpn3.70334","article-title":"Clinical, Manometric, Genetic, and Histologic Associations in Pediatric Intestinal Pseudo-Obstruction: A Case Series","volume":"82","author":"Wolfson","year":"2026","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_16","doi-asserted-by":"crossref","unstructured":"Bianco, F., Lattanzio, G., Lorenzini, L., Mazzoni, M., Clavenzani, P., Calz\u00e0, L., Giardino, L., Sternini, C., Costanzini, A., and Bonora, E. (2022). Enteric Neuromyopathies: Highlights on Genetic Mechanisms Underlying Chronic Intestinal Pseudo-Obstruction. Biomolecules, 12.","DOI":"10.3390\/biom12121849"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"587","DOI":"10.1111\/joim.70078","article-title":"Collagen and Microvascular Alterations Contribute to Neuromuscular Degeneration and Disease Progression in Chronic Intestinal Pseudo-Obstruction","volume":"299","author":"Boschetti","year":"2026","journal-title":"J. Intern. Med."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1007\/s11894-020-0748-8","article-title":"Small Bowel Dysmotility, Pseudoobstruction, and Functional Correlation with Histopathology: Lessons Learned","volume":"22","author":"Gonzalez","year":"2020","journal-title":"Curr. Gastroenterol. Rep."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"230","DOI":"10.1097\/MOG.0000000000000630","article-title":"Update on Chronic Intestinal Pseudo-Obstruction","volume":"36","author":"Zenzeri","year":"2020","journal-title":"Curr. Opin. Gastroenterol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"e12945","DOI":"10.1111\/nmo.12945","article-title":"Chronic Intestinal Pseudo-Obstruction in Children and Adults: Diagnosis and Therapeutic Options","volume":"29","author":"Lauro","year":"2017","journal-title":"Neurogastroenterol. Motil."},{"key":"ref_21","doi-asserted-by":"crossref","unstructured":"Chen, Y., Chen, X., Ou, C., Chen, Y., Li, J., Li, J., Liu, Y., and Li, X. (2025). Phenotype and Genotype in Hereditary Chronic Intestinal Pseudo-Obstruction with Small Intestine Involvement. Front. Med., 12.","DOI":"10.3389\/fmed.2025.1632816"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"e72","DOI":"10.1097\/PAI.0000000000000314","article-title":"Two Cases of Chronic Intestinal Pseudo-Obstruction: A Comparison of Staining Characteristics of Enteric Visceral Myopathy With Hirschsprung Disease","volume":"24","author":"Chaffin","year":"2016","journal-title":"Appl. Immunohistochem. Mol. Morphol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"273","DOI":"10.5056\/jnm16101","article-title":"Chronic Intestinal Pseudo-Obstruction: Clinical and Manometric Characteristics in the Chilean Population","volume":"23","author":"Landskron","year":"2017","journal-title":"J. Neurogastroenterol. Motil."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1007\/s10237-023-01773-8","article-title":"Mechanical Experimentation of the Gastrointestinal Tract: A Systematic Review","volume":"23","author":"Durcan","year":"2024","journal-title":"Biomech. Model. Mechanobiol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"110576","DOI":"10.1016\/j.matdes.2022.110576","article-title":"Biomechanical Constitutive Modeling of the Gastrointestinal Tissues: A Systematic Review","volume":"217","author":"Patel","year":"2022","journal-title":"Mater. Des."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"647","DOI":"10.1038\/s41419-020-02822-1","article-title":"Caveolin1 and YAP Drive Mechanically Induced Mesothelial to Mesenchymal Transition and Fibrosis","volume":"11","author":"Strippoli","year":"2020","journal-title":"Cell Death Dis."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"3068","DOI":"10.1038\/s41467-020-16893-3","article-title":"Post-Surgical Adhesions Are Triggered by Calcium-Dependent Membrane Bridges between Mesothelial Surfaces","volume":"11","author":"Fischer","year":"2020","journal-title":"Nat. Commun."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"1018","DOI":"10.1016\/j.fertnstert.2016.09.005","article-title":"Mesothelial Cells and Peritoneal Homeostasis","volume":"106","author":"Mutsaers","year":"2016","journal-title":"Fertil. Steril."},{"key":"ref_29","doi-asserted-by":"crossref","unstructured":"Zwicky, S.N., Stroka, D., and Zindel, J. (2021). Sterile Injury Repair and Adhesion Formation at Serosal Surfaces. Front. Immunol., 12.","DOI":"10.3389\/fimmu.2021.684967"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"705","DOI":"10.1007\/s10439-018-1992-z","article-title":"The Turning Point for Morphomechanical Remodeling During Complete Intestinal Obstruction in Rats Occurs After 12\u201324 h","volume":"46","author":"Sun","year":"2018","journal-title":"Ann. Biomed. Eng."},{"key":"ref_31","unstructured":"American College of Surgeons International Relations Committee (2021). Management of Large Bowel Obstruction. Gastrointestinal Surgical Emergencies, American College of Surgeons."},{"key":"ref_32","doi-asserted-by":"crossref","unstructured":"Gilbert-Kawai, E.T., and Wittenberg, M.D. (2014). Laplace\u2019s Law and Tension. Essential Equations for Anaesthesia, Cambridge University Press.","DOI":"10.1017\/CBO9781139565387"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"e3588","DOI":"10.1002\/cnm.3588","article-title":"Biomechanical Characterization of the Small Intestine to Simulate Gastrointestinal Tract Chyme Propulsion","volume":"38","author":"Carvalho","year":"2022","journal-title":"Int. J. Numer. Method. Biomed. Eng."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"1225","DOI":"10.1016\/j.rcl.2015.06.008","article-title":"Bowel Obstruction","volume":"53","author":"Gore","year":"2015","journal-title":"Radiol. Clin. N. Am."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1007\/s11894-017-0566-9","article-title":"A Systematic Review of the Clinical Presentation, Diagnosis, and Treatment of Small Bowel Obstruction","volume":"19","author":"Cappell","year":"2017","journal-title":"Curr. Gastroenterol. Rep."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"1069","DOI":"10.1007\/s10439-016-1739-7","article-title":"Shear Modulus of the Partially Obstructed Rat Small Intestine","volume":"45","author":"Sun","year":"2017","journal-title":"Ann. Biomed. Eng."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"336","DOI":"10.1007\/s10620-006-9431-7","article-title":"Biomechanical Remodeling of the Chronically Obstructed Guinea Pig Small Intestine","volume":"52","author":"Storkholm","year":"2007","journal-title":"Dig. Dis. Sci."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"430","DOI":"10.1111\/cge.13895","article-title":"Novel ACTG2 Variants Disclose Allelic Heterogeneity and Bi-Allelic Inheritance in Pediatric Chronic Intestinal Pseudo-Obstruction","volume":"99","author":"Matera","year":"2021","journal-title":"Clin. Genet."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"e13371","DOI":"10.1111\/nmo.13371","article-title":"Variants in ACTG2 Underlie a Substantial Number of Australasian Patients with Primary Chronic Intestinal Pseudo-Obstruction","volume":"30","author":"Ravenscroft","year":"2018","journal-title":"Neurogastroenterol. Motil."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1177\/1066896918786586","article-title":"ACTG2-Associated Visceral Myopathy With Chronic Intestinal Pseudoobstruction, Intestinal Malrotation, Hypertrophic Pyloric Stenosis, Choledochal Cyst, and a Novel Missense Mutation","volume":"27","author":"Collins","year":"2019","journal-title":"Int. J. Surg. Pathol."},{"key":"ref_41","doi-asserted-by":"crossref","unstructured":"Ta, M.-C., Cazals-Hatem, D., Billiauws, L., Amiot, A., Bellaiche, M., Berrebi, D., Coffin, B., Corcos, O., Ecochard-Dugelay, E., and Goulet, O. (2026). Histogenetic Classification Predicts Outcomes in 130 Adults with Chronic Intestinal Pseudo-Obstruction. Am. J. Gastroenterol., ahead of print.","DOI":"10.14309\/ajg.0000000000003980"},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"eadn6615","DOI":"10.1126\/sciadv.adn6615","article-title":"Molecular Mechanisms Linking Missense ACTG2 Mutations to Visceral Myopathy","volume":"10","author":"Ceron","year":"2024","journal-title":"Sci. Adv."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"1482","DOI":"10.1053\/j.gastro.2012.08.045","article-title":"Segregation of a Missense Variant in Enteric Smooth Muscle Actin \u03b3-2 with Autosomal Dominant Familial Visceral Myopathy","volume":"143","author":"Lehtonen","year":"2012","journal-title":"Gastroenterology"},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"641","DOI":"10.1002\/humu.23960","article-title":"Recurrent Arginine Substitutions in the ACTG2 Gene Are the Primary Driver of Disease Burden and Severity in Visceral Myopathy","volume":"41","author":"Larson","year":"2020","journal-title":"Hum. Mutat."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1016\/S0016-5085(19)32221-8","article-title":"Studies of Idiopathic Intestinal Pseudoobstruction. I. Hereditary Hollow Visceral Myopathy: Clinical and Pathological Studies","volume":"73","author":"Schuffler","year":"1977","journal-title":"Gastroenterology"},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"306","DOI":"10.1097\/00000658-197903000-00009","article-title":"Surgical Treatment in Familial Visceral Myopathy","volume":"189","author":"Anuras","year":"1979","journal-title":"Ann. Surg."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"752","DOI":"10.1097\/00000658-198012000-00011","article-title":"Chronic Idiopathic Intestinal Pseudo-Obstruction. A Surgical Approach","volume":"192","author":"Schuffler","year":"1980","journal-title":"Ann. Surg."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"825","DOI":"10.1016\/S0046-8177(82)80079-8","article-title":"Pathologic Features of Familial Visceral Myopathy","volume":"13","author":"Mitros","year":"1982","journal-title":"Hum. Pathol."},{"key":"ref_49","first-page":"93","article-title":"Familial Visceral Myopathy with Carcinoma of Unknown Primary","volume":"32","author":"Darvishian","year":"2002","journal-title":"Ann. Clin. Lab. Sci."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1186\/1755-7682-3-35","article-title":"Emergency Surgery in Chronic Intestinal Pseudo-Obstruction Due to Mitochondrial Neurogastrointestinal Encephalomyopathy: Case Reports","volume":"3","year":"2010","journal-title":"Int. Arch. Med."},{"key":"ref_51","first-page":"645349","article-title":"Acute Abdomen and Perforated Bowel with a Rare Pathology: Nonfamilial Visceral Myopathy","volume":"2011","author":"Burcharth","year":"2011","journal-title":"Case Rep. Surg."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"554","DOI":"10.1111\/j.1440-1827.2012.02842.x","article-title":"An Adult Case of Midgut Volvulus in Familial Visceral Myopathy","volume":"62","author":"Nakajima","year":"2012","journal-title":"Pathol. Int."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1016\/j.ijscr.2017.02.040","article-title":"Acute Large Bowel Pseudo-Obstruction Due to Atrophic Visceral Myopathy: A Case Report","volume":"33","author":"Wrenn","year":"2017","journal-title":"Int. J. Surg. Case Rep."},{"key":"ref_54","first-page":"e8003","article-title":"A Rare Case of Chronic Small Bowel Pseudo-Obstruction","volume":"12","author":"Lim","year":"2020","journal-title":"Cureus"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"e240923","DOI":"10.1136\/bcr-2020-240923","article-title":"Recurrent Spontaneous Small Bowel Perforations with a Rare Pathology: Non-Familial Visceral Myopathy","volume":"14","author":"Shi","year":"2021","journal-title":"BMJ Case Rep."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"e242579","DOI":"10.1136\/bcr-2021-242579","article-title":"Chronic Intestinal Psuedo-Obstruction and MIDD, a Rare Cause of Acute Abdomen: Implications in Emergency Surgery","volume":"14","author":"Underwood","year":"2021","journal-title":"BMJ Case Rep."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"5021","DOI":"10.1177\/00031348231191198","article-title":"Eosinophilic Myenteric Ganglionitis Presenting as Sigmoid Volvulus: A Brief Report","volume":"89","author":"Kim","year":"2023","journal-title":"Am. Surg."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1007\/s00384-026-05093-y","article-title":"Paediatric Caecal Volvulus, a Rare Presentation of African Degenerative Leiomyopathy\u2014A Case Report","volume":"41","author":"Palmisani","year":"2026","journal-title":"Int. J. Color. 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