Klippel Trenaunay Syndrome: a rare case report in a neonate
DOI:
https://doi.org/10.18203/2349-3291.ijcp20164624Keywords:
CLOVES syndrome, India, KTS, NewbornAbstract
Klippel Trenaunay Syndrome (KTS) is a cutaneous vascular malformation in combination with bone and soft tissue overgrowth, with or without lymphatic malformation. Symptoms appear by birth and approximately 90% of the cases are diagnosed by the age of 12 years. Prompt diagnosis at the earliest and conservative management with regular follow up is crucial in prognosis of KTS.
References
Klippel M. Trenaunay P, Variquex DN. Osteohypertrophies. Arch Gen Med. 1900;3:641-72.
Recommendations from the International Society for the study of Vascular Anomalies. Pediatrics. 2015;136:203.
Vahidnezhad H, Youssefian L, Vitto J. Klippel trenaunay belongs to the PIK3CA related overgrowth spectrum (PRO3). Exp Dermatol. 2016;25:17.
Frasier K, Giangola G, Rosen R. Endovascular radiofrequency ablation: a novel treatment of venous insufficiency in Klippel Trenaunay patients. J Vasc Surg. 2008;47:1339-45.
Mazoyer E, Enjolvas O, Bisdorff A. Coagulation disorders in patients with venous malformation of limbs and trunks: A case series of 118 patients. Arch Dermatol. 2008;144:861.
Servelle M. Klippel and trenaunay’s syndrome. 768 operated cases. Ann Surg Mar. 1985;201(3):365-73.
Steijen PM, Steensel MA. Paradominant inheritance, a hypothesis explaining occasional familial occurrence of sporadic syndromes. Am J Med Genet. 1999;85:359.
Luks VL, Kamitaki N, Vivero MP. Lymphatic and other vascular malformative / overgrowth disorders are caused by somatic mutations in PIK3CA. J Pediatr. 2015;166:1048.
Maari C, Frieden J. Klippel trenaunay syndrome: the importance of geographic stains in identifying lymphatic disease and risk of complications. J Am Acad Dermatol. 2004;52:391.
Mulliken JB, Young AE. Vascular birthmarks, hemagiomas and malformations. Saunders. Philadelphia. 1988
Redondo P, Bastarika G, Aguedo L. Foot or hand malformations related to deep venous system anomalies of the lower limb in klippel Trenaunay syndrome. J Am Acad Dermatol. 2009;61:621.
Redondo P, Martinez AL. Diagnose and management of extensive malformations of lower limbs: Part-I clinical diagnosis. J Am Acad Dermatoll. 2011;65:893.
Noreuil KM, Rios JJ, Parker VE. PIK3CA-related overgrowth spectrum: diagnostic and testing eligibility criteria, differential diagnosis and evaluation. Am J Med Genet. 2015;167:287.
Wassef M, Blei F, Adams D. Vascular anomalies classification: recommendations from the international society for the study of vascular anomalies. Pediatrics. 2015;136:203.
Lee MS, Lieng MG, Mulliken JB. Diffuse capillary malformation with overgrowth. A Clinical subtype of vascular anomalies with hypertrophy. J Am Acad Dermatol. 2013;69:589.
Gloriczki P, Driscoll DJ. Klippel Trenaunay syndrome: current management. Phlebology. 2007;22:291.
