Generic selectors
Exact matches only
Search in title
Search in content
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Studies
Study Letter
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Tables
Technology
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF
Net letter
2009:75:5;552-552
doi: 10.4103/0378-6323.55428
PMID: 19736465

Klippel-Trenaunay syndrome in a new born with multiple congenital anomalies

Kamal Aggarwal1 , Sanjeev Gupta2 , Vijay Kumar Jain1 , Jyotsna Sen3 , Smiti Nanda4
1 Department of Dermatology, Venereology and Leprology, Post Graduate Institute of Medical Sciences and Research, Rohtak, India
2 Department of Dermatology, Venereology and Leprology, MM Institute of Medical Sciences and Research, Mullana, Ambala, India
3 Department of Radiodiagnosis, Post Graduate Institute of Medical Sciences and Research, Rohtak, India
4 Department of Obstetrics and Gynecology, Post Graduate Institute of Medical Sciences and Research, Rohtak, India

Correspondence Address:
Sanjeev Gupta
B-2, Near Shiv Mandir, MM Medical College Residential Complex, Mullana Ambala - 133203, Haryana
India
How to cite this article:
Aggarwal K, Gupta S, Jain VK, Sen J, Nanda S. Klippel-Trenaunay syndrome in a new born with multiple congenital anomalies. Indian J Dermatol Venereol Leprol 2009;75:552
Copyright: (C)2009 Indian Journal of Dermatology, Venereology, and Leprology

Sir,

Klippel-Trenaunay syndrome (KTS), as originally defined in 1900, is comprised of the triad of a port-wine stain extending the full length of a limb, venous varicosities of the same limb, either congenital or of onset in infancy and overgrowth of all the tissues of the affected limb, particularly bone. [1] Today, this term is generally used for any case where there is an association of port-wine stain and increased limb size, whether or not bony overgrowth is present and whether or not venous varicosities are apparent.[2] One or several macular telangiectatic vascular naevi are almost invariably present at birth. [3] In addition to these main features, a wide range of associated conditions and complications have been described. Other vascular malformations such as angiokeratomas and lesions resembling granuloma telangiectaticum are not uncommonly associated with KTS. [2]

A 26-year-old mother delivered a female infant by vaginal delivery at full term. There was no history of medication for any systemic disorder taken during pregnancy or of maternal illness. The father of the baby was a 28 -year- old male and he was in good health. The family history was unremarkable. Cutaneous examination of the baby, on the second day of birth, revealed a macular, telangiectatic vascular hemangioma of the ′port-wine′ variety, pink to purple in color, present in a splotchy distribution over the entire right lower extremity extending over the right gluteal region and onto the medial aspect of the right thigh and partially involving the genitals. The lesion was diffusely spread over the antero-lateral aspect of the right lower extremity with only a limited involvement of the medial aspect. The right limb was grossly edematous and hypertrophied and there was a perceptible difference in the limb lengths and mid-calf circumferences [Figure - 1]. Multiple, markedly dilated and tortuous venous varicosities were present in and around the right knee joint. Small brownish-black angiokeratomatous nodules were irregularly distributed over the underlying hemangioma. The right foot was distinctly bigger in size with respect to the left foot. Syndactyly was present in between the second and third, and the fourth and fifth right toes along with bilateral congenital talipes equinus varus deformity. A Doppler study performed showed a normal pattern of venous and arterial blood flow in the affected leg. Ultrasonography of the abdomen and chest X-ray were normal. Hematological profile including platelet count was normal. A diagnosis of Klippel-Trenaunay syndrome with congenital talipes equinus varus with syndactyly and angiokeratomas was made.

The etiology of KTS is unknown. Manifestations of this syndrome begin at birth, or shortly after birth with most of the patients displaying cutaneous hemangiomas. As the child begins to walk, varicosities appear and become more obvious. Limb and soft tissue hypertrophy usually manifests several years later and involves a single lower extremity. In our patient, all the three components, i.e. hemangioma/port-wine stain, varicosity and limb hypertrophy were present at birth which itself is a unique presentation and not reported till date. In addition, but relatively not frequent, congenital orthopedic anomalies such as polydactyly, syndactyly, oligodactyly, compensatory scoliosis [3] and hip dislocation can occur in some cases. Equinus deformity of the feet as present in our case too, is the rare orthopedic anomaly. Many patients with KTS can be managed conservatively with elastic support garments. The usual agents used for the treatment of hemangiomas, like prednisolone, interferon alpha are of no benefit in KTS. Recently, pulse dye laser therapy and surgical correction to debulk the excessive tissue have been found effective. [4] Most of the cases of KTS are diagnosed after the different cutaneous changes start manifesting as the child grows. Jalil et al . [5] have recently reported a case of KTS diagnosed at four years of age.

This case highlights the presence of various developmental defects apart from three classical components of KTS, which have been diagnosed just after birth. Awareness of the syndrome and knowledge of its associated anomalies are important for appropriate follow-up and subsequent management.

References
1.
Klippel M. Trenaunay P. Du naevus variqueux osteohypertrophique. Arch Gen Med Paris 1900;3:611-72.
[Google Scholar]
2.
Atherton DJ. Naevi and other developmental defects. In: Champion RH, Burton JL, Burns DA, Breathnach S, editors. Textbook of Dermatology. 6th ed. Oxford: Blackwell Science; 1998. p. 519-616.
[Google Scholar]
3.
Baskerville PA, Ackroyd JS, Lea Thomas M, Browse NL. The Klippel-Trenaunay syndrome: clinical, radiological and haemodynamic features and management. Br J Surg 1985;72:232-6.
[Google Scholar]
4.
Garzon MC, Huang JT, Enjolras O, Frieden IJ. Vascular malformations: Part1. J Am Acad Dermatol 2007;56:353-70.
[Google Scholar]
5.
Jalil J, Shafique M, Ghafoor T, Amin U. Klippel Trenaunay Syndrome. J Pak Med Assoc 2007;57:150-1.
[Google Scholar]

Fulltext Views
106

PDF downloads
160
Show Sections