Recurrent laryngeal lymphangiomatosis in a patient with Proteus syndrome: A case report
Key Clinical Message Proteus syndrome is a rare genetic disease characterized by an asymmetrical growth of individual parts of the body and has only been described in single cases. This patient presented with recurrent manifestations of a laryngeal and hypopharyngeal lymphangioma, which were treated...
| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2023-10-01
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| Series: | Clinical Case Reports |
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| Online Access: | https://doi.org/10.1002/ccr3.8073 |
| _version_ | 1797650450119393280 |
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| author | Christina Sauter Veronika Kolb Nikolaus Bosch Cornelia Hornberger Peter Karl Plinkert Ralph Hohenberger |
| author_facet | Christina Sauter Veronika Kolb Nikolaus Bosch Cornelia Hornberger Peter Karl Plinkert Ralph Hohenberger |
| author_sort | Christina Sauter |
| collection | DOAJ |
| description | Key Clinical Message Proteus syndrome is a rare genetic disease characterized by an asymmetrical growth of individual parts of the body and has only been described in single cases. This patient presented with recurrent manifestations of a laryngeal and hypopharyngeal lymphangioma, which were treated with laser surgery, systemic therapy, and sclerotherapy. The reported data depict the diagnosis and treatment in the department of otorhinolaryngology, head and neck surgery of the university hospital Heidelberg from 2019 until May 2023. The recurrent endoscopy of the upper airway was performed using a flexible HD‐endoscope and the Visera Elite video tower from Olympus, Hamburg. The 29‐year old female patient initially presented in February 2019 with stridor and exertional dyspnea due to a lymphatic malformation of the left larynx and hypopharynx. In April 2019 there was no improvement by sclerotherapy with Picibanil, so that systemic therapy with the PIK3CA inhibitor alpelisib was initiated (03–07/2020) and discontinued due to a high side effect profile. In the course of 2021–2023, three microlaryngoscopies with laser surgical resection and renewed sclerotherapy of the lymphangioma with Picibanil were carried out due to fluctuating findings. After these interventions a stable disease could be established until May 2023. Laser surgical therapy is currently described as the therapy of choice in lymphangiomas in the head and neck region and also showed the highest effectiveness in our patient. In case of airway obstruction in particular, it can bring rapid symptom relief. Alternatively, and with a lower surgical risk, local improvements have been reported by sclerotherapy, which was less effective in the presented case. Rare syndromic diseases require multidisciplinary collaboration. In the case of laryngeal lymphangiomatosis, other treatment options should be considered in addition to surgical excision, especially in the case of recurrence. |
| first_indexed | 2024-03-11T16:00:32Z |
| format | Article |
| id | doaj.art-fb3e8cf6bfd34338b0a948a153eb9cf0 |
| institution | Directory Open Access Journal |
| issn | 2050-0904 |
| language | English |
| last_indexed | 2024-03-11T16:00:32Z |
| publishDate | 2023-10-01 |
| publisher | Wiley |
| record_format | Article |
| series | Clinical Case Reports |
| spelling | doaj.art-fb3e8cf6bfd34338b0a948a153eb9cf02023-10-25T07:12:45ZengWileyClinical Case Reports2050-09042023-10-011110n/an/a10.1002/ccr3.8073Recurrent laryngeal lymphangiomatosis in a patient with Proteus syndrome: A case reportChristina Sauter0Veronika Kolb1Nikolaus Bosch2Cornelia Hornberger3Peter Karl Plinkert4Ralph Hohenberger5Department of Otorhinolaryngology, Head and Neck Surgery University of Heidelberg Heidelberg GermanyDepartment of Otorhinolaryngology, Head and Neck Surgery University of Heidelberg Heidelberg GermanyDepartment of Otorhinolaryngology, Head and Neck Surgery University of Heidelberg Heidelberg GermanyDepartment of Otorhinolaryngology, Head and Neck Surgery University of Heidelberg Heidelberg GermanyDepartment of Otorhinolaryngology, Head and Neck Surgery University of Heidelberg Heidelberg GermanyDepartment of Otorhinolaryngology, Head and Neck Surgery University of Heidelberg Heidelberg GermanyKey Clinical Message Proteus syndrome is a rare genetic disease characterized by an asymmetrical growth of individual parts of the body and has only been described in single cases. This patient presented with recurrent manifestations of a laryngeal and hypopharyngeal lymphangioma, which were treated with laser surgery, systemic therapy, and sclerotherapy. The reported data depict the diagnosis and treatment in the department of otorhinolaryngology, head and neck surgery of the university hospital Heidelberg from 2019 until May 2023. The recurrent endoscopy of the upper airway was performed using a flexible HD‐endoscope and the Visera Elite video tower from Olympus, Hamburg. The 29‐year old female patient initially presented in February 2019 with stridor and exertional dyspnea due to a lymphatic malformation of the left larynx and hypopharynx. In April 2019 there was no improvement by sclerotherapy with Picibanil, so that systemic therapy with the PIK3CA inhibitor alpelisib was initiated (03–07/2020) and discontinued due to a high side effect profile. In the course of 2021–2023, three microlaryngoscopies with laser surgical resection and renewed sclerotherapy of the lymphangioma with Picibanil were carried out due to fluctuating findings. After these interventions a stable disease could be established until May 2023. Laser surgical therapy is currently described as the therapy of choice in lymphangiomas in the head and neck region and also showed the highest effectiveness in our patient. In case of airway obstruction in particular, it can bring rapid symptom relief. Alternatively, and with a lower surgical risk, local improvements have been reported by sclerotherapy, which was less effective in the presented case. Rare syndromic diseases require multidisciplinary collaboration. In the case of laryngeal lymphangiomatosis, other treatment options should be considered in addition to surgical excision, especially in the case of recurrence.https://doi.org/10.1002/ccr3.8073head and necklarynxlaser surgerylymphangiomatosisProteus syndromerare diseases |
| spellingShingle | Christina Sauter Veronika Kolb Nikolaus Bosch Cornelia Hornberger Peter Karl Plinkert Ralph Hohenberger Recurrent laryngeal lymphangiomatosis in a patient with Proteus syndrome: A case report Clinical Case Reports head and neck larynx laser surgery lymphangiomatosis Proteus syndrome rare diseases |
| title | Recurrent laryngeal lymphangiomatosis in a patient with Proteus syndrome: A case report |
| title_full | Recurrent laryngeal lymphangiomatosis in a patient with Proteus syndrome: A case report |
| title_fullStr | Recurrent laryngeal lymphangiomatosis in a patient with Proteus syndrome: A case report |
| title_full_unstemmed | Recurrent laryngeal lymphangiomatosis in a patient with Proteus syndrome: A case report |
| title_short | Recurrent laryngeal lymphangiomatosis in a patient with Proteus syndrome: A case report |
| title_sort | recurrent laryngeal lymphangiomatosis in a patient with proteus syndrome a case report |
| topic | head and neck larynx laser surgery lymphangiomatosis Proteus syndrome rare diseases |
| url | https://doi.org/10.1002/ccr3.8073 |
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