Publicação
Functional shoulder girdle reconstruction in paediatric desmoid tumour
| Resumo: | Desmoid tumours are rare fibroblastic neoplasms that, although benign, may be locally aggressive, invasive, and disabling. In children with Gardner syndrome, they typically arise in the abdominal wall, whereas scapular involvement is uncommon and surgically challenging because of the risk of scapular winging after resection of stabilizing muscles. We report the case of a 4-year-old boy with Gardner syndrome and multifocal desmoid disease, including a large tumour of the left shoulder girdle. Radical resection required sacrifice of several stabilizers, and scapular stability was preserved using a dynamic reconstructive technique: a trapezius flap elongated with lumbosacral fascia, passed through a bone tunnel in the scapula and anchored to adjacent muscles. Despite recurrence requiring re-excision and chemotherapy, the patient remained disease-free at 4-year follow-up, with preserved scapular stability. This case underscores the importance of dynamic reconstruction techniques, avoidance of mutilating resections, and multidisciplinary management in paediatric multifocal desmoid disease. |
|---|---|
| Autores principais: | Albuquerque, Maria |
| Outros Autores: | Constantino, Cláudia; Martins, Pedro; Casal, Diogo; Casal, Diogo |
| Assunto: | desmoid tumour Gardner syndrome trapezius flap winged scapula Surgery |
| Ano: | 2025 |
| País: | Portugal |
| Tipo de documento: | artigo |
| Tipo de acesso: | acesso aberto |
| Instituição associada: | Universidade Nova de Lisboa |
| Idioma: | inglês |
| Origem: | Repositório Institucional da UNL |
| _version_ | 1868982352464576512 |
|---|---|
| author | Albuquerque, Maria |
| author2 | Constantino, Cláudia Martins, Pedro Casal, Diogo Casal, Diogo |
| author2_role | author author author author |
| author_facet | Albuquerque, Maria Constantino, Cláudia Martins, Pedro Casal, Diogo Casal, Diogo |
| author_role | author |
| contributor_name_str_mv | NOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM) Oxford University Press RUN |
| country_str | PT |
| creators_json_txt | [{\"Person.name\":\"Albuquerque, Maria\"},{\"Person.name\":\"Constantino, Cláudia\"},{\"Person.name\":\"Martins, Pedro\"},{\"Person.name\":\"Casal, Diogo\"},{\"Person.name\":\"Casal, Diogo\",\"Person.identifier.orcid\":\"0000-0002-5537-9340\"}] |
| datacite.contributors.contributor.contributorName.fl_str_mv | NOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM) Oxford University Press RUN |
| datacite.creators.creator.creatorName.fl_str_mv | Albuquerque, Maria Constantino, Cláudia Martins, Pedro Casal, Diogo Casal, Diogo |
| datacite.date.Accepted.fl_str_mv | 2025-10-01T00:00:00Z |
| datacite.date.available.fl_str_mv | 2025-11-18T21:10:59Z |
| datacite.date.embargoed.fl_str_mv | 2025-11-18T21:10:59Z |
| datacite.rights.fl_str_mv | http://purl.org/coar/access_right/c_abf2 |
| datacite.subjects.subject.fl_str_mv | desmoid tumour Gardner syndrome trapezius flap winged scapula Surgery |
| datacite.titles.title.fl_str_mv | Functional shoulder girdle reconstruction in paediatric desmoid tumour |
| dc.contributor.none.fl_str_mv | NOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM) Oxford University Press RUN |
| dc.creator.none.fl_str_mv | Albuquerque, Maria Constantino, Cláudia Martins, Pedro Casal, Diogo Casal, Diogo |
| dc.date.Accepted.fl_str_mv | 2025-10-01T00:00:00Z |
| dc.date.available.fl_str_mv | 2025-11-18T21:10:59Z |
| dc.date.embargoed.fl_str_mv | 2025-11-18T21:10:59Z |
| dc.format.none.fl_str_mv | application/pdf |
| dc.identifier.none.fl_str_mv | http://hdl.handle.net/10362/191008 |
| dc.language.none.fl_str_mv | eng |
| dc.rights.none.fl_str_mv | http://purl.org/coar/access_right/c_abf2 |
| dc.subject.none.fl_str_mv | desmoid tumour Gardner syndrome trapezius flap winged scapula Surgery |
| dc.title.fl_str_mv | Functional shoulder girdle reconstruction in paediatric desmoid tumour |
| dc.type.none.fl_str_mv | http://purl.org/coar/resource_type/c_6501 |
| description | Desmoid tumours are rare fibroblastic neoplasms that, although benign, may be locally aggressive, invasive, and disabling. In children with Gardner syndrome, they typically arise in the abdominal wall, whereas scapular involvement is uncommon and surgically challenging because of the risk of scapular winging after resection of stabilizing muscles. We report the case of a 4-year-old boy with Gardner syndrome and multifocal desmoid disease, including a large tumour of the left shoulder girdle. Radical resection required sacrifice of several stabilizers, and scapular stability was preserved using a dynamic reconstructive technique: a trapezius flap elongated with lumbosacral fascia, passed through a bone tunnel in the scapula and anchored to adjacent muscles. Despite recurrence requiring re-excision and chemotherapy, the patient remained disease-free at 4-year follow-up, with preserved scapular stability. This case underscores the importance of dynamic reconstruction techniques, avoidance of mutilating resections, and multidisciplinary management in paediatric multifocal desmoid disease. |
| dirty | 0 |
| eu_rights_str_mv | openAccess |
| format | article |
| fulltext.url.fl_str_mv | https://run.unl.pt/bitstreams/2dacfb39-e335-443b-a4e7-7d79cdcdfec7/download |
| id | run_c0fe1b91dbb63e2bae63aa07e50e5d1c |
| identifier.url.fl_str_mv | http://hdl.handle.net/10362/191008 |
| inst_facet_str | urn:organizationAcronym:unl{{{_:::_}}}Universidade Nova de Lisboa |
| instacron_str | unl |
| institution | Universidade Nova de Lisboa |
| instname_str | Universidade Nova de Lisboa |
| language | eng |
| network_acronym_str | run |
| network_name_str | Repositório Institucional da UNL |
| oai_identifier_str | oai:run.unl.pt:10362/191008 |
| organization_str_mv | urn:organizationAcronym:unl |
| person_str_mv | Albuquerque, Maria Constantino, Cláudia Martins, Pedro Casal, Diogo Casal, Diogo Casal, Diogo https://www.ciencia-id.pt/301F-64FA-1E13 301F-64FA-1E13 http://orcid.org/0000-0002-5537-9340 0000-0002-5537-9340 |
| publishDate | 2025 |
| repo_facet_str | urn:repositoryAcronym:run{{{_:::_}}}Repositório Institucional da UNL |
| reponame_str | Repositório Institucional da UNL |
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| service_str_mv | urn:repositoryAcronym:run |
| spelling | engenDesmoid tumours are rare fibroblastic neoplasms that, although benign, may be locally aggressive, invasive, and disabling. In children with Gardner syndrome, they typically arise in the abdominal wall, whereas scapular involvement is uncommon and surgically challenging because of the risk of scapular winging after resection of stabilizing muscles. We report the case of a 4-year-old boy with Gardner syndrome and multifocal desmoid disease, including a large tumour of the left shoulder girdle. Radical resection required sacrifice of several stabilizers, and scapular stability was preserved using a dynamic reconstructive technique: a trapezius flap elongated with lumbosacral fascia, passed through a bone tunnel in the scapula and anchored to adjacent muscles. Despite recurrence requiring re-excision and chemotherapy, the patient remained disease-free at 4-year follow-up, with preserved scapular stability. This case underscores the importance of dynamic reconstruction techniques, avoidance of mutilating resections, and multidisciplinary management in paediatric multifocal desmoid disease.application/pdfenFunctional shoulder girdle reconstruction in paediatric desmoid tumourAlbuquerque, MariaConstantino, CláudiaMartins, PedroCasal, DiogoPersonalCasal, DiogoDSpacehttp://dspace.org/items/851d9536-1288-403b-b6ad-7282fd830917DSpacehttp://dspace.org/items/851d9536-1288-403b-b6ad-7282fd830917CasalDiogoCiência IDhttps://www.ciencia-id.pt301F-64FA-1E13ORCIDhttp://orcid.org0000-0002-5537-9340Scopus Author IDhttps://www.scopus.com35434625000NOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM)Oxford University PressHostingInstitutionOrganizationalRUNe-mailmailto:run@unl.ptrun@unl.ptISSNIsPartOf2042-8812URNIsPartOfPURE: 134840214URNIsPartOfPURE UUID: fc533ee4-73d3-4fb2-9d50-0b698b543d4eURNIsPartOfScopus: 105020047938DOIIsPartOf10.1093/jscr/rjaf8302025-11-18T21:10:59Z2025-10-012025-10-01T00:00:00ZHandlehttp://hdl.handle.net/10362/191008http://purl.org/coar/access_right/c_abf2open accessdesmoid tumourGardner syndrometrapezius flapwinged scapulaSurgery1005211 bytesliteraturehttp://purl.org/coar/resource_type/c_6501journal articlehttp://purl.org/coar/access_right/c_abf2application/pdffulltexthttps://run.unl.pt/bitstreams/2dacfb39-e335-443b-a4e7-7d79cdcdfec7/download |
| spellingShingle | Functional shoulder girdle reconstruction in paediatric desmoid tumour Albuquerque, Maria desmoid tumour Gardner syndrome trapezius flap winged scapula Surgery |
| status | SINGLETON |
| subject.fl_str_mv | desmoid tumour Gardner syndrome trapezius flap winged scapula Surgery |
| title | Functional shoulder girdle reconstruction in paediatric desmoid tumour |
| title_full | Functional shoulder girdle reconstruction in paediatric desmoid tumour |
| title_fullStr | Functional shoulder girdle reconstruction in paediatric desmoid tumour |
| title_full_unstemmed | Functional shoulder girdle reconstruction in paediatric desmoid tumour |
| title_short | Functional shoulder girdle reconstruction in paediatric desmoid tumour |
| title_sort | Functional shoulder girdle reconstruction in paediatric desmoid tumour |
| topic | desmoid tumour Gardner syndrome trapezius flap winged scapula Surgery |
| topic_facet | desmoid tumour Gardner syndrome trapezius flap winged scapula Surgery |
| url | http://hdl.handle.net/10362/191008 |
| visible | 1 |