AUTHOR=Cucinella Gaspare , Di Buono Giuseppe , Geraci Girolamo , Ricupati Federica , Gullo Giuseppe , Maienza Elisa , Romano Giorgio , Bonventre Giulia , Amato Giuseppe , Romano Giorgio , Buscemi Salvatore , Agrusa Antonino 

TITLE=Uterine Involvement in Klippel–Trenaunay Syndrome: A Rare But Relevant Event. Review of the Literature

JOURNAL=Frontiers in Surgery

VOLUME=Volume 9 - 2022

YEAR=2022

URL=https://www.frontiersin.org/journals/surgery/articles/10.3389/fsurg.2022.893320

DOI=10.3389/fsurg.2022.893320

ISSN=2296-875X

ABSTRACT=Introduction
Klippel Trenaunay syndrome is a rare vascular congenital disorder characterized by the classical triad of port-wine stains, abnormal overgrowth of soft tissues and bones and vascular malformations. The involvement of  the genitourinary tract and of the uterus in particular is extremely infrequent, but relevant for the possible consequences
Methods
We performed an extensive review of literature using the Pubmed, Scopus and ISI web of knowledge database to identify all cases of KTS with uterine involvement.  The search was done using the  MeSH term “Klippel-Trenaunay syndrome” AND “uterine” OR “uterus”. We considered only publications in English language with no limits of time. We selected a total of 11 records of KTS with uterine involvement considering also those affecting pregnant women
Results
Klippel-Trenaunay syndrome was described for the first time in 1900 in two patients with hemangiomatous lesions of the skin associated with varicose veins and asymmetric soft tissue and bone hypertrophy.  Uterine involvement is a rare condition and can cause severe menorrhagia. Diagnosis is based on physical signs and symptoms. CT scans and MRI are first choice exams to evaluate both the extension of the lesion and infiltration of deeper tissues before treatment. The management of Klippel-Trenaunay syndrome should be personalized and makes use of careful diagnosis, prevention and treatment of complications.
Conclusion
The syndrome of Klippel-Trenaunay is a rare vascular malformation with a wide variability of  manifestations. There are no univocal and clear guidelines that suggest the most adequate monitoring of the possible complications of the disease. Treatment is generally conservative but in presence of recurrent bleeding, surgery may be needed.