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A multi-step approach to overcome challenges in the management of head and neck lymphatic malformations, and response to treatment.
Trevisan, Valentina; De Corso, Eugenio; Viscogliosi, Germana; Onesimo, Roberta; Cina, Alessandro; Panfili, Marco; Perri, Lucrezia; Agazzi, Cristiana; Giorgio, Valentina; Rigante, Donato; Vento, Giovanni; Papacci, Patrizia; Paradiso, Filomena Valentina; Silvaroli, Sara; Nanni, Lorenzo; Resta, Nicoletta; Castori, Marco; Galli, Jacopo; Paludetti, Gaetano; Zampino, Giuseppe; Leoni, Chiara.
Afiliación
  • Trevisan V; Center for Rare Diseases and Birth Defects, Department of Woman and Child Health and Public Health, Fondazione Policlinico Universitario "A. Gemelli" IRCCS, Largo A. Gemelli 8, Rome, 00168, RM, Italy.
  • De Corso E; Unit of Otorhinolaryngology, Fondazione Policlinico Universitario "A. Gemelli" IRCCS, Rome, RM, Italy.
  • Viscogliosi G; UOC Radiodiagnostica e Neuroradiologia, Dipartimento di Diagnostica per Immagini, Fondazione Policlinico Universitario A. Gemelli IRCCS, Radioterapia, Rome, Italy.
  • Onesimo R; Center for Rare Diseases and Birth Defects, Department of Woman and Child Health and Public Health, Fondazione Policlinico Universitario "A. Gemelli" IRCCS, Largo A. Gemelli 8, Rome, 00168, RM, Italy.
  • Cina A; UOC Radiodiagnostica e Neuroradiologia, Dipartimento di Diagnostica per Immagini, Fondazione Policlinico Universitario A. Gemelli IRCCS, Radioterapia, Rome, Italy.
  • Panfili M; UOC Radiodiagnostica e Neuroradiologia, Dipartimento di Diagnostica per Immagini, Fondazione Policlinico Universitario A. Gemelli IRCCS, Radioterapia, Rome, Italy.
  • Perri L; Center for Rare Diseases and Birth Defects, Department of Woman and Child Health and Public Health, Fondazione Policlinico Universitario "A. Gemelli" IRCCS, Largo A. Gemelli 8, Rome, 00168, RM, Italy.
  • Agazzi C; Center for Rare Diseases and Birth Defects, Department of Woman and Child Health and Public Health, Fondazione Policlinico Universitario "A. Gemelli" IRCCS, Largo A. Gemelli 8, Rome, 00168, RM, Italy.
  • Giorgio V; Center for Rare Diseases and Birth Defects, Department of Woman and Child Health and Public Health, Fondazione Policlinico Universitario "A. Gemelli" IRCCS, Largo A. Gemelli 8, Rome, 00168, RM, Italy.
  • Rigante D; Center for Rare Diseases and Birth Defects, Department of Woman and Child Health and Public Health, Fondazione Policlinico Universitario "A. Gemelli" IRCCS, Largo A. Gemelli 8, Rome, 00168, RM, Italy.
  • Vento G; Università Cattolica del Sacro Cuore, Rome, RM, Italy.
  • Papacci P; Università Cattolica del Sacro Cuore, Rome, RM, Italy.
  • Paradiso FV; Neonatology Unit, Department of Woman and Child Health and Public Health, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, 00168, Italy.
  • Silvaroli S; Neonatology Unit, Department of Woman and Child Health and Public Health, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, 00168, Italy.
  • Nanni L; Scuola di Specializzazione in Chirurgia Pediatrica, Università Cattolica Sacro Cuore, Roma, Italy.
  • Resta N; Scuola di Specializzazione in Chirurgia Pediatrica, Università Cattolica Sacro Cuore, Roma, Italy.
  • Castori M; Università Cattolica del Sacro Cuore, Rome, RM, Italy.
  • Galli J; Scuola di Specializzazione in Chirurgia Pediatrica, Università Cattolica Sacro Cuore, Roma, Italy.
  • Paludetti G; Department of Biomedical Sciences and Human Oncology (DIMO), Medical Genetics, University of Bari "Aldo Moro", Bari, Italy.
  • Zampino G; Division of Medical Genetics, Fondazione IRCCS-Casa Sollievo della Sofferenza, Foggia, San Giovanni Rotondo, Italy.
  • Leoni C; Unit of Otorhinolaryngology, Fondazione Policlinico Universitario "A. Gemelli" IRCCS, Rome, RM, Italy.
Orphanet J Rare Dis ; 19(1): 276, 2024 Jul 23.
Article en En | MEDLINE | ID: mdl-39044220
ABSTRACT

BACKGROUND:

Lymphatic malformations are vascular developmental anomalies varying from local superficial masses to diffuse infiltrating lesions, resulting in disfigurement. Patients' outcomes range from spontaneous regression to severe sequelae notwithstanding appropriate treatment. The current classification guides, in part, clinicians through the decision-making process, prognosis prediction and choice of therapeutic strategies. Even though the understanding of molecular basis of the disease has been recently improved, a standardized management algorithm has not been reached yet.

RESULTS:

Here, we report our experience on five children with different lymphatic anomalies of the head and neck region treated by applying a multidisciplinary approach reaching a consensus among specialists on problem-solving and setting priorities.

CONCLUSIONS:

Although restitutio ad integrum was rarely achieved and the burden of care is challenging for patients, caregivers and healthcare providers, this study demonstrates how the referral to expert centres can significantly improve outcomes by alleviating parental stress and ameliorating patients' quality of life. A flow-chart is proposed to guide the multidisciplinary care of children with LMs and to encourage multidisciplinary collaborative initiatives to implement dedicated patients' pathways.
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Texto completo: 1 Colección: 01-internacional Banco de datos: MEDLINE Asunto principal: Anomalías Linfáticas Límite: Child / Child, preschool / Female / Humans / Infant / Male Idioma: En Revista: Orphanet J Rare Dis Asunto de la revista: MEDICINA Año: 2024 Tipo del documento: Article País de afiliación: Italia

Texto completo: 1 Colección: 01-internacional Banco de datos: MEDLINE Asunto principal: Anomalías Linfáticas Límite: Child / Child, preschool / Female / Humans / Infant / Male Idioma: En Revista: Orphanet J Rare Dis Asunto de la revista: MEDICINA Año: 2024 Tipo del documento: Article País de afiliación: Italia