Malignant hypertension in a child with phakomatosis pigmentovascularis type II b

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5 Citations (Scopus)

Abstract

Phakomatosis pigmentovascularis (PPV) is a rare congenital syndrome characterized predominantly by cutaneous vascular malformations and pigmentary naevi. The most frequently reported form, Type II b, is associated with systemic involvement. Sturge-Weber Syndrome (SWS) with concomitant glaucoma, Klippel-Trenaunay Syndrome (KTS) and naevus of Ota have been frequently described, but there have only been two case reports with asymptomatic renal anomalies. Conclusion: We report the first case of PPV associated with SWS and naevus of Ota presenting with malignant hypertension secondary to bilateral renal artery stenosis. This case raises the possibility that the phakomatoses are not distinct entities but a spectrum of overlapping neurocutaneous, vascular and renal features.

Original languageEnglish
Pages (from-to)1589-1591
Number of pages3
JournalActa Paediatrica, International Journal of Paediatrics
Volume97
Issue number11
DOIs
Publication statusPublished - Nov 2008

Fingerprint

Neurocutaneous Syndromes
Malignant Hypertension
Nevus of Ota
Sturge-Weber Syndrome
Klippel-Trenaunay-Weber Syndrome
Kidney
Renal Artery Obstruction
Vascular Malformations
Nevus
Glaucoma
Blood Vessels
Skin

Keywords

  • Naevus of Ota
  • Oculodermal melanocytosis
  • Renal artery stenosis
  • Renal cysts
  • Sturge-Weber Syndrome (SWS)

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

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title = "Malignant hypertension in a child with phakomatosis pigmentovascularis type II b",
abstract = "Phakomatosis pigmentovascularis (PPV) is a rare congenital syndrome characterized predominantly by cutaneous vascular malformations and pigmentary naevi. The most frequently reported form, Type II b, is associated with systemic involvement. Sturge-Weber Syndrome (SWS) with concomitant glaucoma, Klippel-Trenaunay Syndrome (KTS) and naevus of Ota have been frequently described, but there have only been two case reports with asymptomatic renal anomalies. Conclusion: We report the first case of PPV associated with SWS and naevus of Ota presenting with malignant hypertension secondary to bilateral renal artery stenosis. This case raises the possibility that the phakomatoses are not distinct entities but a spectrum of overlapping neurocutaneous, vascular and renal features.",
keywords = "Naevus of Ota, Oculodermal melanocytosis, Renal artery stenosis, Renal cysts, Sturge-Weber Syndrome (SWS)",
author = "{P. Yoganathan}, Kanaheswari and {Abdul Hamid}, Hamzaini and Wong, {Sau Wei} and A. Zulfiqar",
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T1 - Malignant hypertension in a child with phakomatosis pigmentovascularis type II b

AU - P. Yoganathan, Kanaheswari

AU - Abdul Hamid, Hamzaini

AU - Wong, Sau Wei

AU - Zulfiqar, A.

PY - 2008/11

Y1 - 2008/11

N2 - Phakomatosis pigmentovascularis (PPV) is a rare congenital syndrome characterized predominantly by cutaneous vascular malformations and pigmentary naevi. The most frequently reported form, Type II b, is associated with systemic involvement. Sturge-Weber Syndrome (SWS) with concomitant glaucoma, Klippel-Trenaunay Syndrome (KTS) and naevus of Ota have been frequently described, but there have only been two case reports with asymptomatic renal anomalies. Conclusion: We report the first case of PPV associated with SWS and naevus of Ota presenting with malignant hypertension secondary to bilateral renal artery stenosis. This case raises the possibility that the phakomatoses are not distinct entities but a spectrum of overlapping neurocutaneous, vascular and renal features.

AB - Phakomatosis pigmentovascularis (PPV) is a rare congenital syndrome characterized predominantly by cutaneous vascular malformations and pigmentary naevi. The most frequently reported form, Type II b, is associated with systemic involvement. Sturge-Weber Syndrome (SWS) with concomitant glaucoma, Klippel-Trenaunay Syndrome (KTS) and naevus of Ota have been frequently described, but there have only been two case reports with asymptomatic renal anomalies. Conclusion: We report the first case of PPV associated with SWS and naevus of Ota presenting with malignant hypertension secondary to bilateral renal artery stenosis. This case raises the possibility that the phakomatoses are not distinct entities but a spectrum of overlapping neurocutaneous, vascular and renal features.

KW - Naevus of Ota

KW - Oculodermal melanocytosis

KW - Renal artery stenosis

KW - Renal cysts

KW - Sturge-Weber Syndrome (SWS)

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