Cargando…

Penile prosthesis implant for primary erectile dysfunction in patient with Klippel-Trenaunay syndrome complicated by consumptive coagulopathy: A case report

RATIONALE: Klippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by cutaneous port wine capillary malformations, varicose veins with hemihypertrophy of soft tissue and bone. Pelvic and retroperitoneal vascular malformations have been described up to the 30% of patients with KTS whi...

Descripción completa

Detalles Bibliográficos
Autores principales: Bettocchi, Carlo, Spilotros, Marco, Lucarelli, Giuseppe, Utano, Emanuele, Sebastiani, Francesco, Bittner, Lukas, Ditonno, Pasquale, Battaglia, Michele
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer Health 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6708871/
https://www.ncbi.nlm.nih.gov/pubmed/31393387
http://dx.doi.org/10.1097/MD.0000000000016741
_version_ 1783446076777299968
author Bettocchi, Carlo
Spilotros, Marco
Lucarelli, Giuseppe
Utano, Emanuele
Sebastiani, Francesco
Bittner, Lukas
Ditonno, Pasquale
Battaglia, Michele
author_facet Bettocchi, Carlo
Spilotros, Marco
Lucarelli, Giuseppe
Utano, Emanuele
Sebastiani, Francesco
Bittner, Lukas
Ditonno, Pasquale
Battaglia, Michele
author_sort Bettocchi, Carlo
collection PubMed
description RATIONALE: Klippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by cutaneous port wine capillary malformations, varicose veins with hemihypertrophy of soft tissue and bone. Pelvic and retroperitoneal vascular malformations have been described up to the 30% of patients with KTS while hemangiomas of the urinary tract have been reported in 6% PATIENT CONCERNS: A 30-year-old man with KTS was referred to our center for primary erectile dysfunction (ED) associated with varicosities of unusual distribution and asymmetry of the lower limbs. Furthermore, he suffered from hypertension and autosomal dominant polycystic kidney disease. During penile prosthesis implantation, a significant intraoperative bleeding (1 liter) due to large scrotal venous malformations and profuse bleeding from both corpora was recorded. One month later, the day after the first prosthesis training session, the patient returned with swelling in the penoscrotal region. A large inguino-scrotal hematoma was drained. There was a complete bilateral dehiscence of corpora cavernosa with a spread venous bleeding in the scrotum. DIAGNOSES: CT scan showed hypertrophy of the right hypogastric artery with severe vascular malformations: the right pudendal artery was massively dilated with early visualization of venous drainage without evidence of arteriovenous fistulae; regular bulbocavernous capillary blush; right upper gluteus artery hypertrophic and dilated. Multiple twisting and aneurysms of the right internal pudenda artery were bleeding from multiple points. Cystoscopy showed a fistula between the proximal urethra and the penoscrotal dartos. Coagulation tests revealed the presence of factor XIII deficiency INTERVENTIONS: The patient underwent several procedures including percutaneous scleroembolization of the internal pudendal arteries, removal of the penile implant, recombinant factor XIII (FXIII) administration, and cord blood platelet gel application. OUTCOMES: The patient was discharged after almost 3 months in hospital, hemodynamically stable. LESSONS: Experience regarding management of ED in KTS patient is limited and in case of concomitant factor XIII deficiency, the clinical scenario can be life-threatening. A multidisciplinary approach including a urologist, an interventional radiologist and a hematologist in our experience represented the key approach in case of severe bleeding following surgery for ED.
format Online
Article
Text
id pubmed-6708871
institution National Center for Biotechnology Information
language English
publishDate 2019
publisher Wolters Kluwer Health
record_format MEDLINE/PubMed
spelling pubmed-67088712019-10-01 Penile prosthesis implant for primary erectile dysfunction in patient with Klippel-Trenaunay syndrome complicated by consumptive coagulopathy: A case report Bettocchi, Carlo Spilotros, Marco Lucarelli, Giuseppe Utano, Emanuele Sebastiani, Francesco Bittner, Lukas Ditonno, Pasquale Battaglia, Michele Medicine (Baltimore) Research Article RATIONALE: Klippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by cutaneous port wine capillary malformations, varicose veins with hemihypertrophy of soft tissue and bone. Pelvic and retroperitoneal vascular malformations have been described up to the 30% of patients with KTS while hemangiomas of the urinary tract have been reported in 6% PATIENT CONCERNS: A 30-year-old man with KTS was referred to our center for primary erectile dysfunction (ED) associated with varicosities of unusual distribution and asymmetry of the lower limbs. Furthermore, he suffered from hypertension and autosomal dominant polycystic kidney disease. During penile prosthesis implantation, a significant intraoperative bleeding (1 liter) due to large scrotal venous malformations and profuse bleeding from both corpora was recorded. One month later, the day after the first prosthesis training session, the patient returned with swelling in the penoscrotal region. A large inguino-scrotal hematoma was drained. There was a complete bilateral dehiscence of corpora cavernosa with a spread venous bleeding in the scrotum. DIAGNOSES: CT scan showed hypertrophy of the right hypogastric artery with severe vascular malformations: the right pudendal artery was massively dilated with early visualization of venous drainage without evidence of arteriovenous fistulae; regular bulbocavernous capillary blush; right upper gluteus artery hypertrophic and dilated. Multiple twisting and aneurysms of the right internal pudenda artery were bleeding from multiple points. Cystoscopy showed a fistula between the proximal urethra and the penoscrotal dartos. Coagulation tests revealed the presence of factor XIII deficiency INTERVENTIONS: The patient underwent several procedures including percutaneous scleroembolization of the internal pudendal arteries, removal of the penile implant, recombinant factor XIII (FXIII) administration, and cord blood platelet gel application. OUTCOMES: The patient was discharged after almost 3 months in hospital, hemodynamically stable. LESSONS: Experience regarding management of ED in KTS patient is limited and in case of concomitant factor XIII deficiency, the clinical scenario can be life-threatening. A multidisciplinary approach including a urologist, an interventional radiologist and a hematologist in our experience represented the key approach in case of severe bleeding following surgery for ED. Wolters Kluwer Health 2019-08-09 /pmc/articles/PMC6708871/ /pubmed/31393387 http://dx.doi.org/10.1097/MD.0000000000016741 Text en Copyright © 2019 the Author(s). Published by Wolters Kluwer Health, Inc. http://creativecommons.org/licenses/by-nc-nd/4.0 This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc-nd/4.0
spellingShingle Research Article
Bettocchi, Carlo
Spilotros, Marco
Lucarelli, Giuseppe
Utano, Emanuele
Sebastiani, Francesco
Bittner, Lukas
Ditonno, Pasquale
Battaglia, Michele
Penile prosthesis implant for primary erectile dysfunction in patient with Klippel-Trenaunay syndrome complicated by consumptive coagulopathy: A case report
title Penile prosthesis implant for primary erectile dysfunction in patient with Klippel-Trenaunay syndrome complicated by consumptive coagulopathy: A case report
title_full Penile prosthesis implant for primary erectile dysfunction in patient with Klippel-Trenaunay syndrome complicated by consumptive coagulopathy: A case report
title_fullStr Penile prosthesis implant for primary erectile dysfunction in patient with Klippel-Trenaunay syndrome complicated by consumptive coagulopathy: A case report
title_full_unstemmed Penile prosthesis implant for primary erectile dysfunction in patient with Klippel-Trenaunay syndrome complicated by consumptive coagulopathy: A case report
title_short Penile prosthesis implant for primary erectile dysfunction in patient with Klippel-Trenaunay syndrome complicated by consumptive coagulopathy: A case report
title_sort penile prosthesis implant for primary erectile dysfunction in patient with klippel-trenaunay syndrome complicated by consumptive coagulopathy: a case report
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6708871/
https://www.ncbi.nlm.nih.gov/pubmed/31393387
http://dx.doi.org/10.1097/MD.0000000000016741
work_keys_str_mv AT bettocchicarlo penileprosthesisimplantforprimaryerectiledysfunctioninpatientwithklippeltrenaunaysyndromecomplicatedbyconsumptivecoagulopathyacasereport
AT spilotrosmarco penileprosthesisimplantforprimaryerectiledysfunctioninpatientwithklippeltrenaunaysyndromecomplicatedbyconsumptivecoagulopathyacasereport
AT lucarelligiuseppe penileprosthesisimplantforprimaryerectiledysfunctioninpatientwithklippeltrenaunaysyndromecomplicatedbyconsumptivecoagulopathyacasereport
AT utanoemanuele penileprosthesisimplantforprimaryerectiledysfunctioninpatientwithklippeltrenaunaysyndromecomplicatedbyconsumptivecoagulopathyacasereport
AT sebastianifrancesco penileprosthesisimplantforprimaryerectiledysfunctioninpatientwithklippeltrenaunaysyndromecomplicatedbyconsumptivecoagulopathyacasereport
AT bittnerlukas penileprosthesisimplantforprimaryerectiledysfunctioninpatientwithklippeltrenaunaysyndromecomplicatedbyconsumptivecoagulopathyacasereport
AT ditonnopasquale penileprosthesisimplantforprimaryerectiledysfunctioninpatientwithklippeltrenaunaysyndromecomplicatedbyconsumptivecoagulopathyacasereport
AT battagliamichele penileprosthesisimplantforprimaryerectiledysfunctioninpatientwithklippeltrenaunaysyndromecomplicatedbyconsumptivecoagulopathyacasereport