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 language | English |
|---|---|
| Pages (from-to) | 1589-1591 |
| Number of pages | 3 |
| Journal | Acta Paediatrica, International Journal of Paediatrics |
| Volume | 97 |
| Issue number | 11 |
| DOIs | |
| Publication status | Published - Nov 2008 |
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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
Malignant hypertension in a child with phakomatosis pigmentovascularis type II b. / P. Yoganathan, Kanaheswari; Abdul Hamid, Hamzaini; Wong, Sau Wei; Zulfiqar, A.
In: Acta Paediatrica, International Journal of Paediatrics, Vol. 97, No. 11, 11.2008, p. 1589-1591.Research output: Contribution to journal › Article
}
TY - JOUR
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)
UR - http://www.scopus.com/inward/record.url?scp=53749100552&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=53749100552&partnerID=8YFLogxK
U2 - 10.1111/j.1651-2227.2008.00971.x
DO - 10.1111/j.1651-2227.2008.00971.x
M3 - Article
VL - 97
SP - 1589
EP - 1591
JO - Acta Paediatrica, International Journal of Paediatrics
JF - Acta Paediatrica, International Journal of Paediatrics
SN - 0803-5253
IS - 11
ER -