Klippel–Trenaunay syndrome is an uncommon genetic condition. The main pathology consists of arteriovenous malformations. It is generally asymptomatic but may present as soft tissue or bony hypertrophy. We hereby present a case of Klippel–Trenaunay syndrome of an 18 year old male patient coming with large venous malformations, lymphangiomas and A-V fistula at lower leg along with soft tissue hypertrophy of right foot. Patient was evaluated clinically and radiologically and a diagnosis of Klippel–Trenaunay syndrome was formed. Patient was given compression stockings and asked to followup regularly.

1. Klippel–Trenaunay syndrome – A case report

2. Case Report
KlippeleTrenaunay syndrome e A case report
Vikram Khanna a,*
, Sanjeev Kumar a
, Raju Vaishya b
a
Department of Orthopaedics, Eras Lucknow Medical College and Hospital, Lucknow, India
b
Department of Orthopaedics, Indraprastha Apollo Hospital, Delhi, India
a r t i c l e i n f o
Article history:
Received 24 February 2015
Accepted 12 March 2015
Available online xxx
Keywords:
KlippeleTrenaunay syndrome
Arteriovenous malformation
Soft tissue hypertrophy
a b s t r a c t
KlippeleTrenaunay syndrome is an uncommon genetic condition. The main pathology
consists of arteriovenous malformations. It is generally asymptomatic but may present as
soft tissue or bony hypertrophy. We hereby present a case of KlippeleTrenaunay syndrome
of an 18 year old male patient coming with large venous malformations, lymphangiomas
and A-V fistula at lower leg along with soft tissue hypertrophy of right foot. Patient was
evaluated clinically and radiologically and a diagnosis of KlippeleTrenaunay syndrome
was formed. Patient was given compression stockings and asked to followup regularly.
Copyright © 2015, Indraprastha Medical Corporation Ltd. All rights reserved.
1. Description
KlippeleTrenaunay syndrome is an uncommon yet distinct
clinical entity. It is a sporadic condition with multifactorial in-
heritance. There is no predilection for gender or any particular
ethnicity and it being a congenital condition, more and more
cases arenowbeingseen atbirth,childhood oradolescence.This
triad constitutes of soft tissue and bone hypertrophy, Cutaneous
Vascular Malformation and Atypical Venous abnormalities.1
We report a case of young 18-year-old boy with single
lower limb involvement with large venous malformations,
lymphangiomas and A-V fistula at lower leg along with soft
tissue hypertrophy of right foot (Figs. 1 and 2). On X-ray
osteopenic changes were seen along with soft tissue swelling
and on further radiological analysis MRI (T2-weighted MR
images) revealed markedly hyper intense soft tissue swelling
predominantly in subcutaneous tissue in right gluteal region,
extending inferiorly involving the thigh (Figs. 3e5). It also
involved significant part of muscles on the posterior and
postero-lateral aspect of thigh. The findings were suggestive
of arteriovenous malformation and KlippeleTrenaunay was
diagnosed. The patient was treated conservatively and was
told to apply elastic stockings and keep the limb elevated
whenever possible.
The mainstay of the treatment is conservative, surgical
procedure is used only in very distressing cases.2
These
Fig. 1 e Clinical picture showing venous malformations
and lymphangiomas.
* Corresponding author. 13, D-Road. Allahabad 211003, India. Tel.: þ91 7073647974.
E-mail address: 86.khanna@gmail.com (V. Khanna).
Available online at www.sciencedirect.com
ScienceDirect
journal homepage: www.elsevier.com/locate/apme
a p o l l o m e d i c i n e x x x ( 2 0 1 5 ) 1 e3
http://dx.doi.org/10.1016/j.apme.2015.03.004
0976-0016/Copyright © 2015, Indraprastha Medical Corporation Ltd. All rights reserved.
Please cite this article in press as: Khanna V, et al., KlippeleTrenaunay syndrome e A case report, Apollo Medicine (2015), http://
dx.doi.org/10.1016/j.apme.2015.03.004

3. cases are important to diagnose clinically and are associ-
ated with high-morbidity and mortality. This diagnosis
should be kept in mind and should be diagnosed with good
clinical acumen along with systematic radiological
investigations.
Fig. 3 e MRI image showing hyper-intense soft tissue
shadow in gluteal region.
Fig. 4 e MRI image showing extent till the lower thigh.
Fig. 2 e Clinical Picture showing hypertrophy of the foot.
Fig. 5 e MRI image showing the involvement of the
posterior glutei.
a p o l l o m e d i c i n e x x x ( 2 0 1 5 ) 1 e32
Please cite this article in press as: Khanna V, et al., KlippeleTrenaunay syndrome e A case report, Apollo Medicine (2015), http://
dx.doi.org/10.1016/j.apme.2015.03.004

4. 2. Learning points
Classical clinical and radiological picture of the condition.
Doctors should keep this diagnosis in mind when they see
a clinical and radiological picture and should not be
misdiagnosed.
Conflicts of interest
All authors have none to declare.
r e f e r e n c e s
1. Klippel M, Trenaunay P. Du Noevus Variquesosteo
hypertrophicus. Arch Gen Med (Paris). 1900;3:641e672.
2. Gloviczki P, Stanson AW, Stickler GB, et al. KlippeleTrenaunay
syndrome: the risks and benefits of vascular interventions.
Surgery. 1991;110:469e479.
a p o l l o m e d i c i n e x x x ( 2 0 1 5 ) 1 e3 3
Please cite this article in press as: Khanna V, et al., KlippeleTrenaunay syndrome e A case report, Apollo Medicine (2015), http://
dx.doi.org/10.1016/j.apme.2015.03.004

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