1 3
Int Urol Nephrol (2016) 48:1771–1775
DOI 10.1007/s11255-016-1365-1
UROLOGY - REVIEW
Congenital lymphovascular malformations with urological
symptoms: a report of two cases and review of the literature
Miklos Romics
1
· Geza Tasnadi
2
· Balint Sulya
3
· Andras Kiss
3
· Miklos Merksz
3
·
Peter Nyirady
1
Received: 2 March 2016 / Accepted: 30 June 2016 / Published online: 15 July 2016
© Springer Science+Business Media Dordrecht 2016
therapeutical success is graded by Browse’s scoring system.
According to that, our choices of management achieved the
best possible outcome in both cases below.
Conclusions Although conservative ways of therapy are
known and widely used, in more advanced cases surgi-
cal help is often needed to reach long-term improvement.
In situations where significant mass of chyle has accu-
mulated causing severe complaints, conservative therapy
should not to be started. Surgical solutions provide fast and
lasting improvement for patients suffering from congenital
lymphovascular malformations.
Keywords Lymphoedema · Lymphovascular ·
Malformations · Management
Introduction
Congenital lymphovascular malformations and lymph cir-
culatory disorders may be the rarest (only 2:1000 in the
3-year age group) and most complicated areas of vascu-
lar surgery and—through their symptoms—urology [1].
Obstructions in the abdominal lymphoid system can be
present since childbirth, and symptoms usually occur in the
early years. However, it is possible that the disorder stays
latent till adulthood.
Clinical manifestations are highly diverse: The vis-
ible symptoms can be different as the incompetent lym-
phovenous sections vary. First, these blocked lymph
veins get dilated and the deformed venous valves will
cease to prevent reverse lymph flow. The slowed lym-
phoid circulation will lead to the vein’s disruption and
lymph edema. After the lymph steps out of the circula-
tory system, it will penetrate into the peritoneum, bow-
els, thorax, scrotum, labia or the lower extremities and
Abstract
Objectives To collect and review the diagnostic and thera-
peutic solutions for primary and congenital lymphovascular
malformations leading to urological symptoms in child-
hood and also to find the most efficient therapeutic algo-
rithms managing such conditions.
Methods In our work, we assemble all the diagnostic and
therapeutic tools for lymphovascular malformations with
urological–urogenital symptoms and demonstrate the inter-
ventional therapeutic algorithms through two of our cases
where surgery (laparoscopic intervention and clipping of
the lymph vessel) had to be performed to stop lymphoid
leakage and restore anatomy.
Results In cases, where lymphovascular malformations
and urological–urogenital symptoms are both present,
* Miklos Romics
miklos_romics@gmx.com
Geza Tasnadi
meditas.bt@chello.hu
Balint Sulya
bsulya@gmail.com
Andras Kiss
kissandras@heimpalkorhaz.hu
Miklos Merksz
merksz@heimpalkorhaz.hu
Peter Nyirady
nyirady.peter@med.semmelweis-univ.hu
1
Department of Urology and Urooncology, Semmelweis
University, Ulloi Str 78/b, Budapest 1082, Hungary
2
Department of Surgery, Heim Pál Children’s Hospital,
Budapest, Hungary
3
Department of Urological Surgery, Heim Pál Children’s
Hospital, Budapest, Hungary