A rare case of overlapping Sturge–Weber syndrome and Klippel–Trenaunay syndrome associated with bilateral refractory childhood glaucoma

نویسندگان

  • Manju R Pillai
  • P P Hasini
  • Ashish Ahuja
  • S R Krishnadas
چکیده

A 6-year-old girl presented with blurred vision and was found to have elevated intraocular pressure (IOP) and glaucomatous optic disc damage in both eyes. She also displayed capillary malformations on the face (port-wine stain), upper back and all four limbs, angiomatosis in the brain and had hypertrophy of the left upper and lower limbs typical of overlapping Sturge-Weber syndrome and Klippel-Trenaunay syndromes. She was initially managed with IOP lowering topical medications but required trabeculectomy in the right eye followed by Ahmed valve implantation in both eyes. Despite multiple measures over a 7-year period, her IOP still remained uncontrolled with gradual progression of the glaucomatous damage. This case exhibits a very rare occurrence of overlapping syndromes reported only a handful of times in literature. Most cases with Sturge-Weber syndrome have ipsilateral glaucoma affecting the eye on the same side as the port-wine stain. This case presented with bilateral refractory childhood glaucomas, which is exceedingly rare.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Report of a case of Klippel-Trenaunay syndrome associated with bilateral nevus of Ota

Port-wine stain is a vascular malformation characterized pathologically by ectasia of superficial dermal capillaris and clinically by persistent macular erythema. The association of a port-wine stain on a limb with soft tissue swelling, with or without bony overgrowth, is termed klippel-Trenaunay syndrome. Phakomatosis pigmento-vascularis is a combination of port-wine stain and cutaneous ...

متن کامل

Overlapping of Sturge Weber syndrome and Klippel Trenaunay Weber syndrome.

Sturge-Weber Syndrome (SWS) occurs sporadically with a frequency of approximately 1 in 50,000. SWS is a mesodermal phakomatosis. Klippel-Trenaunay Weber syndrome (KTWS) is another very rare phakomatosis. Overlap between SWS & KTWS is very rarely encountered. We report a 19 months old boy with features of both SWS and KTWS. The reported case had seizures, port wine haemangioma of the right side ...

متن کامل

An Infantile Case of Sturge-Weber Syndrome in Association with Klippel-Trenaunay-Weber Syndrome and Phakomatosis Pigmentovascularis

Sturge-Weber syndrome can be associated with facial port-wine stains and intracranial calcification, and concurrent Klippel-Trenaunay-Weber syndrome has been reported. Klippel-Trenaunay-Weber syndrome is a rare congenital mesodermal phakomatosis characterized by cutaneous hemangiomas, venous varicosities and soft tissue or bone hypertrophy of the affected extremities. This report is presented a...

متن کامل

Management of childhood glaucoma in Sturge Weber Syndrome: A challenge.

BACKGROUND Sturge-Weber syndrome (SWS) is a rare congenital neuro- oculo- cutaneous disorder. Glaucoma is one of the ocular abnormalities associated with it. Management of glaucoma in SWS is challenging. OBJECTIVE To report surgical and visual outcome of trabeculectomy in bilateral glaucoma in Sturge-Weber syndrome and the challenges encountered in its management. CASE DESCRIPTION A six-yea...

متن کامل

Klippel-Trenaunay and Sturge-Weber overlapping syndrome in a Saudi boy.

Sturge-Weber Syndrome (SWS) is a rare, sporadic neurocutaneous disorder. It is typically characterized by unilateral, posterior leptomeningeal angiomas that calcify, glaucoma, and facial portwine tains. Klippel-Trenaunay syndrome (KTS) is a rare congenital syndrome characterized by ipsilateral cutaneous capillary malformations, venous varicosities, and bony or soft tissue overgrowth of the affe...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:

دوره 65  شماره 

صفحات  -

تاریخ انتشار 2017