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Cross-sectional imaging and lymphoscintigraphy have historically dominated assessment, while transabdominal B-mode intestinal ultrasound (IUS)\u2014a cornerstone modality for inflammatory bowel disease\u2014has remained almost unmapped in PIL. We conducted a PRISMA-ScR scoping review (Open Science Framework preregistration gfbc9) of all primary studies reporting transabdominal B-mode IUS in PIL, searching three bibliographic platforms (PubMed\/MEDLINE, Ovid Multifile, Scopus) supplemented by forward and backward citation chasing. Studies using only extra-intestinal ultrasound, endoscopic ultrasound, those describing secondary lymphangiectasia or focal lymphangioma, and those using ultrasound only as procedural guidance for lymphangiography were excluded at full-text stage. Fifteen primary studies, published 1986\u20132026 across thirteen countries, met eligibility (nine paediatric, six adult). All fifteen reported per-patient B-mode IUS findings extractable for synthesis. A consistent sonographic signature emerged: diffuse, regular, slightly hypoechoic small-bowel-wall thickening with preserved five-layer stratification and prominent valvulae conniventes; dilated fluid-filled loops with reduced peristalsis; oedematous mesentery, variable ascites; and characteristically absent mesenteric lymphadenopathy (with two notable exceptions framed only as a research hypothesis). Diagnostic accuracy as a screening tool has been evaluated only in one study (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200920: accuracy 80%, 95% CI 56.3\u201394.3%). We illustrate this signature in a 42-year-old woman with Hennekam syndrome and report quantitative B-mode IUS values as anecdotal single-observation\u2014single sonographer, single device, single fasted time-point, mid-jejunal segment to motivate, prospective standardisation.\n                  <\/jats:p>","DOI":"10.1007\/s40477-026-01181-5","type":"journal-article","created":{"date-parts":[[2026,7,29]],"date-time":"2026-07-29T18:12:07Z","timestamp":1785348727000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Intestinal ultrasound in primary intestinal lymphangiectasia (Waldmann\u2019s disease): a PRISMA-ScR scoping review with an illustrative adult case"],"prefix":"10.1007","author":[{"ORCID":"https:\/\/orcid.org\/0009-0001-9816-4171","authenticated-orcid":false,"given":"Luisa","family":"Bertin","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-9141-269X","authenticated-orcid":false,"given":"Miriana","family":"Zanconato","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-9612-6560","authenticated-orcid":false,"given":"Camilla","family":"Cavagna","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3164-8243","authenticated-orcid":false,"given":"Brigida","family":"Barberio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-7376-0063","authenticated-orcid":false,"given":"Cristina","family":"Caranfil","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0076-8549","authenticated-orcid":false,"given":"Cristina","family":"Bezzio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8791-191X","authenticated-orcid":false,"given":"Patrizia","family":"Burra","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3187-2894","authenticated-orcid":false,"given":"Edoardo Vincenzo","family":"Savarino","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1133-1502","authenticated-orcid":false,"given":"Fabiana","family":"Zingone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,7,29]]},"reference":[{"key":"1181_CR1","doi-asserted-by":"publisher","first-page":"3429","DOI":"10.1093\/ibd\/izaf196","volume":"31","author":"V Jairath","year":"2025","unstructured":"Jairath V, Adsul S, Allocca M, Danese S, Dubinsky MC, de Oliveira MF, Ma C, Kucharzik T, Novak KL, Panaccione R, Bautista IR, Sands BE, Taylor SA, Wilkens R, Maaser C (2025) Integrating intestinal ultrasound to clinical trials in patients with Crohn\u2019s disease: opportunities and challenges. 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EVS has served as speaker for Abbvie, Agave, AGPharma, Alfasigma, Aurora Pharma, CaDiGroup, Celltrion, Dr Falk, EG Stada Group, Fenix Pharma, Fresenius Kabi, Galapagos, Janssen, JB Pharmaceuticals, Innovamedica\/Adacyte, Malesci, MayolyBiohealth, Omega Pharma, Pfizer, Reckitt Benckiser, Sandoz, SILA, Sofar, Takeda, Tillots, Unifarco; has served as a consultant for Abbvie, Agave, Alfasigma, Biogen, Bristol-Myers Squibb, Celltrion, DiademaFarmaceutici, Dr. Falk, Fenix Pharma, Fresenius Kabi, Janssen, JB Pharmaceuticals, Merck & Co, Nestl\u00e9, Reckitt Benckiser, Regeneron, Sanofi, SILA, Sofar, Synformulas GmbH, Takeda, Unifarco; he received research support from Pfizer, Reckitt Benckiser, SILA, Sofar, Unifarco, Zeta Farmaceutici. FZ has served as a speaker for EG Stada Group, Fresenius Kabi, Janssen, Pfizer, Takeda, Unifarco, Malesci, and Kedrion and has served as a consultant for Galapagos. BB has served as a speaker for Abbvie, Agave, Alfasigma, AGpharma, Janssen, Lilly, MSD, Pfizer, Sofar, Takeda, and Unifarco. LB has served as a speaker for Takeda and Edra Spa. The remaining authors (MZ, CC, CCa, PB) declare that they have no competing interests.","order":1,"name":"Ethics","label":"Conflict of interest","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"This study was performed in line with the principles of the Declaration of Helsinki. The illustrative single-case component was conducted under the standard institutional consent framework of the Azienda Ospedale Universit\u00e0 Padova (Modulo 1CR informed consent), in compliance with Regional Decree DGR Veneto 1633\/2022 and Internal Operational Instruction Rev. 00 dated 24 April 2024. The scoping-review component, based exclusively on previously published material, did not require separate ethics-committee approval.","order":2,"name":"Ethics","label":"Ethical approval","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Written informed consent was obtained from the patient described in the illustrative case for participation in the clinical examination, including ultrasound performance and clinical-data collection.","order":3,"name":"Ethics","label":"Consent to participate","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The patient described in the illustrative case provided written informed consent for publication of her anonymised clinical, imaging and genetic data, including the ultrasonographic images shown in Fig.\u00a0\n                      \n                      .","order":4,"name":"Ethics","label":"Consent to publish","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Anthropic\u2019s Claude was used for AI-assisted language refinement. All conceptualisation, data extraction, methodological decisions, scientific interpretation and final wording are the authors\u2019 own; no LLM contribution meets the JUS authorship criteria. The authors are accountable for the final version of the text.","order":5,"name":"Ethics","label":"Use of large language models","group":{"name":"EthicsHeading","label":"Declarations"}}]}}