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Giant cavernous malformation of the posterior fossa with lymphangiomatous phenotype, associated with orbital venolymphatic anomaly in an 11-month-old patient: case report and literature review

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Abstract

The synchronous presentation of venolymphatic anomalies of the orbit and noncontiguous intracranial cavernous malformations is uncommon. Herein, we present a case of an 11-month-old female patient diagnosed with orbital venolymphatic anomaly associated with a large cavernous malformation in the posterior fossa, who underwent complete surgical resection of the latter. The immunohistochemical analysis was positive for podoplanin, a marker expressed by lymphatic endothelial cells, but not vascular endothelium. This exceptional finding suggests lymphatic involvement in the etiology of the lesion. In our review of the literature, we did not find similar cases in patients under 1 year of age.

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Authors and Affiliations

  1. Department of Pediatric Neurosurgery, Instituto Nacional de Salud del Niño San Borja, Lima, Peru

    Jose Daniel Flores-Sanchez, Ivethe Pregúntegui & Alberto Ramirez

  2. Department of Radiology, Instituto Nacional de Salud del Niño San Borja, Lima, Peru

    Carlos Ugas

  3. Department of Pathology, Instituto Nacional de Salud del Niño San Borja, Lima, Peru

    Carla Cruzado

  4. Genetics Service, Hospital Nacional Docente Madre Niño San Bartolome, Lima, Peru

    Julio A. Poterico

Authors
  1. Jose Daniel Flores-Sanchez
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  2. Ivethe Pregúntegui
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  3. Carlos Ugas
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  4. Carla Cruzado
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  5. Alberto Ramirez
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  6. Julio A. Poterico
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Contributions

All authors contributed to the study conception and design. Material preparation, data collection, and analysis were performed by Jose Daniel Flores-Sanchez, Ivethe Pregúntegui, Carlos Ugas, and Julio A. Poterico. The first draft of the manuscript was written by Jose Daniel Flores-Sanchez and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Jose Daniel Flores-Sanchez.

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Ethics approval

This case report is in accordance with the ethical standards and was approved by the ethics committee of the Instituto Nacional de Salud del Niño - San Borja. The study was conducted in accordance with the Declaration of Helsinki.

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The person responsible for the patient has consented to the submission of the case report to the journal.

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The authors declare no competing interests.

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Flores-Sanchez, J.D., Pregúntegui, I., Ugas, C. et al. Giant cavernous malformation of the posterior fossa with lymphangiomatous phenotype, associated with orbital venolymphatic anomaly in an 11-month-old patient: case report and literature review. Childs Nerv Syst 39, 289–293 (2023). https://doi.org/10.1007/s00381-022-05623-3

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  • DOI: https://doi.org/10.1007/s00381-022-05623-3

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