An interesting case of nevus flammeus with loss of vision and hemiparesis

Nevus flammeus is the most common benign congenital capillary malformation, often known as a port-wine stain. Sturge-Weber syndrome (SWS) is a congenital, sporadic, nonfamilial disease characterized by intracranial and ophthalmic vascular anomalies and nevus flammeus. It usually manifests as develop...

Full description

Saved in:
Bibliographic Details
Published in:Journal of family medicine and primary care 2022-05, Vol.11 (5), p.2214-2216
Main Authors: Narasimhan, Murali, Valarmathi, S, Ramakrishnan, Ramachandran, T. Durai, Priya, Guhan, Sivathangavel
Format: Article
Language:English
Subjects:
capillary malformation
Case Report
glaucoma
nevus flammeus
port-wine stain
sturge–weber syndrome
Citations: Items that this one cites
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Nevus flammeus is the most common benign congenital capillary malformation, often known as a port-wine stain. Sturge-Weber syndrome (SWS) is a congenital, sporadic, nonfamilial disease characterized by intracranial and ophthalmic vascular anomalies and nevus flammeus. It usually manifests as developmental delay, learning problems, paralysis, seizures, glaucoma and attention deficit, and hyperactivity disorder. A 29-year-old male patient presented with a reddish patch over the face since birth. He was found to have hemihypertrophy of face, hemiparesis of right limbs, and low intelligence quotient. On ophthalmic examination, the patient was found to have glaucoma and only perception of light in the left eye. Computed tomography brain showed atrophy of the left cerebral hemisphere and calcifications in the left frontal, parietal and occipital regions. With these findings, he was diagnosed as SWS type I. A multidisciplinary approach was followed for patient evaluation and management. This case also highlights the irreversible sequelae of this rare phacomatosis.
ISSN:2249-4863
2278-7135
DOI:10.4103/jfmpc.jfmpc_2054_21