Klippel Trenaunay Syndrome: A Case Report in an Adolescent Nigerian Boy

Authors

  • Anthonia Asanye Ikpeme University of Calabar Teaching Hospital, Radiology, University of Calabar, PMB 1115
  • Usang Edet Usang University of Calabar Teaching Hospital, Department of Surgery, University of Calabar, PMB 1115
  • Akan Wilson Inyang University of Calabar Teaching Hospital, Department of Surgery, University of Calabar, PMB 1115
  • Nchiewe Ani University of Calabar Teaching Hospital, Radiology, University of Calabar, PMB 1115

DOI:

https://doi.org/10.3889/oamjms.2015.036

Keywords:

Klippel-Trenaunay Weber syndrome, Adolescent, Port wine stain, infection

Abstract

AIM: This is to report a case of Klippel Trenauay Weber syndrome in a fifteen year old Nigerian boy. This is a rare syndrome and it is the first case to be reported in UCTH Calabar.

CASE PRESENTATION: Product of a full term uneventful pregnancy, delivered to non-consanguineous apparently healthy parents. At birth was noted to gradually develop swelling on the right leg, worse at the right foot. There was crossed hemi-hypertrophy with right leg bigger than the left. As child grew symptoms worsened, parents separated and eventually he was abandoned to the streets. He presented at University of Calabar Teaching Hospital for medical care at the age of fifteen years with lymphatic obstruction, persistent foul smelling drainage, lipodermatosclerosis of right foot as well psycho-social and financial constraints. The diagnosis was made with x-rays and Doppler studies of the lower limb vessels. He is currently being managed conservatively with compression dressings on the affected limbs, Antibiotics for the infection and analgesics. De-bulking surgery is being anticipated at this time.

CONCLUSION: This is a case of KTWS presenting in adolescence and due to its rarity in Nigeria, this report is to increase awareness.

Downloads

Download data is not yet available.

Metrics

Metrics Loading ...

Plum Analytics Artifact Widget Block

Author Biography

Anthonia Asanye Ikpeme, University of Calabar Teaching Hospital, Radiology, University of Calabar, PMB 1115

RADIOLOGY DEPARTMENT, CONSULTANT RADIOLOGIST.

References

Philips GN, Gordon DH, Martin EC, et al. The Klippel-Trenauncy Syndrome: Clinical and radiological aspects. Radiology. 1978; 128(2):429 – 34. DOI: https://doi.org/10.1148/128.2.429

Cha SH, Romeo MA, Nentze JA. Visceral manifestations of Kippel- Trenaunay Syndrome. Radiographs. 2005;25(6):1694-7. DOI: https://doi.org/10.1148/rg.256055042

Ha Youn Kim, Yun-Woo Chang, Dong Hwan Lee. Rectal and Splenic Vascular Malformation in Klippel – Trenauncy Weber Syndrome: A Case Report. J Korean Soc Radiol. 2012; 67(4):289-292. DOI: https://doi.org/10.3348/jksr.2012.67.4.289

Baskerville P.A. et al. The etiology of the Klippel Trenaunay syndrome. Ann Surg. 1985; 202:624-627. DOI: https://doi.org/10.1097/00000658-198511000-00015

Aelvoet GE, Jorens PG, Roden LM. Genetics aspects of the Klippel-Trenauncy Syndrome. Br J Dermatol. 1992; 126:603 – 607. DOI: https://doi.org/10.1111/j.1365-2133.1992.tb00107.x

Klippel M, Trenauncy P. Du. Naevus variqueux Osteohypertrophique. Archive generates de medicine. 1900; 3: 641 – 72.

Weber FP. Angioma formation in connection with hypertrophy of limbs and hemi-hypertrophy. British journal of Dermatology. 1907; 19: 231 – 5.

Weber FP. Hemangiectatic hypertrophy of limbs – congenital phlebar teriectans and so-called congenital varicose veins. British journal of children’s Diseases. 1918; 25:13.

Whelan AJ, Watson MS, Porter FD, Steiner RD. Klippel Trenaunay-Weber Syndrome associated with a 5:11 balanced translocation. Am J Med Genet. 1995; 59:492-4. DOI: https://doi.org/10.1002/ajmg.1320590416

Brooksaler F. The angio osteohypertrophy syndrome. Amj Dis CHILD. 1966; 112:161-164. DOI: https://doi.org/10.1001/archpedi.1966.02090110105012

Mendiratta V, Koranne RV, Sardana K, Hemal U, Solanki RS. Klippel trenaunay Parkes-Weber syndrome. Indian J Dermatol Venereol Leprol. 2004;70(2):119-22.

Marler Jj, Fishman SJ, Upton J, et al. Pre natal diagnosis of vascular anomalies J. Pediatr. Surg. 2002:37(3): 318 – 26. DOI: https://doi.org/10.1053/jpsu.2002.30831

Wiedemann, HR, Burgio GR, AI denhoft P, Kunze J, Kaufmann HJ, Schirg E. The proteus syndrome: partial gigantism of the hands and/feet, naevi, hemihypertrophy, subcutaneous tumors, macrocephaly or other skull anomalies and possible accelerated growth and visceral affectation. Eur J Pediatr. 1983; 140:5-12. DOI: https://doi.org/10.1007/BF00661895

Bodensteiner J, Roach FS, edetors. Sturge – Weber syndrome. Mount freedom (NJ): The Sturge-Weber Foundation, 1999:pp 1-95.

Published

2015-03-28

How to Cite

1.
Ikpeme AA, Usang UE, Inyang AW, Ani N. Klippel Trenaunay Syndrome: A Case Report in an Adolescent Nigerian Boy. Open Access Maced J Med Sci [Internet]. 2015 Mar. 28 [cited 2024 Apr. 23];3(2):322-5. Available from: https://oamjms.eu/index.php/mjms/article/view/oamjms.2015.036

Issue

Section

C- Case Reports