Cargando…

Haemophilic Pelvic Pseudotumour: A New Surgical Option

Background: Haemophilia is an inherited coagulopathy caused by the absence or dysfunction of clotting factor VIII or IX. Clinical manifestations are generally secondary to recurrent bleeding episodes mainly in the musculoskeletal system. Bleeding symptoms appear early in life and, when the disease i...

Descripción completa

Detalles Bibliográficos
Autores principales: Pasta, Gianluigi, Ruggieri, Roberta, Annunziata, Salvatore, Gallese, Alessandro, Gagliardi, Vincenzo Pio, Cuzzocrea, Fabrizio, Ghiara, Matteo, Russo, Mariaconcetta, Preti, Paola Stefania, Santi, Roberto Mario, Mosconi, Mario, Benazzo, Francesco
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8536013/
https://www.ncbi.nlm.nih.gov/pubmed/34682949
http://dx.doi.org/10.3390/healthcare9101269
_version_ 1784587922124570624
author Pasta, Gianluigi
Ruggieri, Roberta
Annunziata, Salvatore
Gallese, Alessandro
Gagliardi, Vincenzo Pio
Cuzzocrea, Fabrizio
Ghiara, Matteo
Russo, Mariaconcetta
Preti, Paola Stefania
Santi, Roberto Mario
Mosconi, Mario
Benazzo, Francesco
author_facet Pasta, Gianluigi
Ruggieri, Roberta
Annunziata, Salvatore
Gallese, Alessandro
Gagliardi, Vincenzo Pio
Cuzzocrea, Fabrizio
Ghiara, Matteo
Russo, Mariaconcetta
Preti, Paola Stefania
Santi, Roberto Mario
Mosconi, Mario
Benazzo, Francesco
author_sort Pasta, Gianluigi
collection PubMed
description Background: Haemophilia is an inherited coagulopathy caused by the absence or dysfunction of clotting factor VIII or IX. Clinical manifestations are generally secondary to recurrent bleeding episodes mainly in the musculoskeletal system. Bleeding symptoms appear early in life and, when the disease is severe (when plasma factor VIII or IX activity is <1% of normal), joint and muscle bleeding may occur spontaneously. A pseudotumour is a recurrent, chronic, encapsulated, slowly expanding, muscle hematoma. Haemophilic pseudotumour is a rare complication of haemophilia which occurs, as a condition either from repeated spontaneous bleeding or coming from a traumatic origin, in 1–2% of haemophilic patients. Case report: A 32-year-old man with severe haemophilia A referred to our Clinic with a massive right iliac wing pseudotumour complicated by Staphylococcus aureus superinfection and skin fistulisation. In this report we describe the medical management and surgical treatment by the adoption of a novel surgical technique which involves the use of a pedicle-screw and rod system (PSRS), a polyglycolic acid MESH and bone cement in order to build up an artificial ilium-like bony mass. This case report highlights the importance of interdisciplinary approach and the efficacy of eradicating surgery as treatment, especially in the case of large and long-lasting lesions.
format Online
Article
Text
id pubmed-8536013
institution National Center for Biotechnology Information
language English
publishDate 2021
publisher MDPI
record_format MEDLINE/PubMed
spelling pubmed-85360132021-10-23 Haemophilic Pelvic Pseudotumour: A New Surgical Option Pasta, Gianluigi Ruggieri, Roberta Annunziata, Salvatore Gallese, Alessandro Gagliardi, Vincenzo Pio Cuzzocrea, Fabrizio Ghiara, Matteo Russo, Mariaconcetta Preti, Paola Stefania Santi, Roberto Mario Mosconi, Mario Benazzo, Francesco Healthcare (Basel) Case Report Background: Haemophilia is an inherited coagulopathy caused by the absence or dysfunction of clotting factor VIII or IX. Clinical manifestations are generally secondary to recurrent bleeding episodes mainly in the musculoskeletal system. Bleeding symptoms appear early in life and, when the disease is severe (when plasma factor VIII or IX activity is <1% of normal), joint and muscle bleeding may occur spontaneously. A pseudotumour is a recurrent, chronic, encapsulated, slowly expanding, muscle hematoma. Haemophilic pseudotumour is a rare complication of haemophilia which occurs, as a condition either from repeated spontaneous bleeding or coming from a traumatic origin, in 1–2% of haemophilic patients. Case report: A 32-year-old man with severe haemophilia A referred to our Clinic with a massive right iliac wing pseudotumour complicated by Staphylococcus aureus superinfection and skin fistulisation. In this report we describe the medical management and surgical treatment by the adoption of a novel surgical technique which involves the use of a pedicle-screw and rod system (PSRS), a polyglycolic acid MESH and bone cement in order to build up an artificial ilium-like bony mass. This case report highlights the importance of interdisciplinary approach and the efficacy of eradicating surgery as treatment, especially in the case of large and long-lasting lesions. MDPI 2021-09-26 /pmc/articles/PMC8536013/ /pubmed/34682949 http://dx.doi.org/10.3390/healthcare9101269 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Case Report
Pasta, Gianluigi
Ruggieri, Roberta
Annunziata, Salvatore
Gallese, Alessandro
Gagliardi, Vincenzo Pio
Cuzzocrea, Fabrizio
Ghiara, Matteo
Russo, Mariaconcetta
Preti, Paola Stefania
Santi, Roberto Mario
Mosconi, Mario
Benazzo, Francesco
Haemophilic Pelvic Pseudotumour: A New Surgical Option
title Haemophilic Pelvic Pseudotumour: A New Surgical Option
title_full Haemophilic Pelvic Pseudotumour: A New Surgical Option
title_fullStr Haemophilic Pelvic Pseudotumour: A New Surgical Option
title_full_unstemmed Haemophilic Pelvic Pseudotumour: A New Surgical Option
title_short Haemophilic Pelvic Pseudotumour: A New Surgical Option
title_sort haemophilic pelvic pseudotumour: a new surgical option
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8536013/
https://www.ncbi.nlm.nih.gov/pubmed/34682949
http://dx.doi.org/10.3390/healthcare9101269
work_keys_str_mv AT pastagianluigi haemophilicpelvicpseudotumouranewsurgicaloption
AT ruggieriroberta haemophilicpelvicpseudotumouranewsurgicaloption
AT annunziatasalvatore haemophilicpelvicpseudotumouranewsurgicaloption
AT gallesealessandro haemophilicpelvicpseudotumouranewsurgicaloption
AT gagliardivincenzopio haemophilicpelvicpseudotumouranewsurgicaloption
AT cuzzocreafabrizio haemophilicpelvicpseudotumouranewsurgicaloption
AT ghiaramatteo haemophilicpelvicpseudotumouranewsurgicaloption
AT russomariaconcetta haemophilicpelvicpseudotumouranewsurgicaloption
AT pretipaolastefania haemophilicpelvicpseudotumouranewsurgicaloption
AT santirobertomario haemophilicpelvicpseudotumouranewsurgicaloption
AT mosconimario haemophilicpelvicpseudotumouranewsurgicaloption
AT benazzofrancesco haemophilicpelvicpseudotumouranewsurgicaloption