|Year : 1991 | Volume
| Issue : 2 | Page : 74-75
Groenblad Strandberg syndrome-A case report
S Harikrishnan, Tara Prasad Das, P Namperumalsamy
Aravind Eye Hospital & Postgraduate Institute of Ophthalmology, 1, Annanagar, Madurai - 625 020, India
Aravind Eye Hospital & Postgraduate Institute of Ophthalmology, 1, Annanagar, Madurai - 625 020
Source of Support: None, Conflict of Interest: None
A rare case of Groenblad Strandberg syndrome in which angioid streaks are associated with pseudoxanthoma elasticum is being reported. The pathology, clinical features, complications and treatment of angioid streaks are discussed in detail.
Keywords: Angioid streaks, Pseudoxanthoma elasticum, Groenblad Stran-dberg syndrome, Bruch′s membrane, Peau d′ orange.
|How to cite this article:|
Harikrishnan S, Das TP, Namperumalsamy P. Groenblad Strandberg syndrome-A case report. Indian J Ophthalmol 1991;39:74-5
| Introduction|| |
Angioid streaks are rarely encountered in clinical practice. In 50% of cases, it is not associated with any systemic problems. Its association with pseudoxanthoma elasticum is an established but rare entity. We are reporting one such case of angioid streaks associated with pseudoxanthoma elasticum (Groenblad Strandberg syndrome).
| Case report|| |
A 41 year old female came with a history of defective vision in the left eye which she noticed 10 years ago and defective vision in the right eye of 3 weeks duration. Wrinkling of the skin in front of the neck was noticed 25 years ago. She was not a known hypertensive. There was no history of epilepsy. Family history was not significant.
General examination : She was a moderately built female, with wrinkling of the skin and yellowish papules with hyperelasticity in the front and back of the neck. There were no similar lesions in the axillae, periumbilical region, antecubital fossae, or inguinal region; no hyperextensibility of joints, no bony deformities, acromegaly or lipomatosis. Pulse 72 per minute regular, BP 120/80 mm of Hg; cardiovascular, respiratory, gastrointestinal and central nervous systems were within normal limits. Ophthalmological examination revealed normal anterior segments in both eyes. FundUs examination revealed angioid streaks in the right eye; angioid streaks along with chorioretinal atrophy at the macula in the left eye.
Visual acuity was 6/9 in the right eye, 1/60 in the left eye, near vision improving to J2 with + 1.50 D sphere in the right eye. Retinoscopy was normal in both eyes. Intraocular tension was 17.3 mm of Hg Schiotz in both eyes. Central fields revealed a central scotoma in the left eye and enlargement of the blind spot in both eyes.
Fluorescein angiography showed irregular streaks radiating from the optic disc in both eyes and chorioretinal atrophy in the left eye.
| Discussion|| |
Angioid streaks (choroidal elastosis) are a peculiar and characteristic bilateral lesion in the fundus primarily due to degenerative changes affecting the elastic lamina which constitutes the mesodermal component of the Bruch's membrane. Angioid streaks were first described by Robert Doyne in 1889. They are found more in males, usually noticed after the 40th year of life'. Pseudoxanthoma elasticum is the commonest systemic association of angioid streaks constituting Groenblad Strandberg syndrome. Apart from eye and skin, pseudoxanthoma elasticum may affect the cardiovascular system and gastrointestinal bleeding can also occur. The other associated conditions of angioid streaks are fibroplasia hyperelastica (Ehlers Danlos syndrome), Osteitis deformans (Paget's disease), Sickle cell disease, cardiovascular disease with hypertension, diffuse lipomatosis, dwarfism, acromegaly, epilepsy and lead poisoning. Groenblad Strandberg syndrome is inherited as an autosomal dominant and autosomal recessive trait; the skin lesions in pseudoxanthoma elasticum appear as yellow papules usually on the neck, axilla, antecubital fossa or paraumbilical region. In our patient, characteristic skin lesions of pseudoxanthoma elasticum were found on the neck [Figure - 1].
Clinically angioid streaks appear as dark brown to reddish streaks radiating from the optic disc to the equator sometimes associated with a mosaic of white spots.
They are irregular with serrated edges and end abruptly; they may intercommunicate near the disc in a circumferential manner [Figure - 2]. They are seen beneath the retinal and above the choroidal vessels. In longstanding cases, the narrow streaks become wholly pigmented and the broader streaks become marginated by pigments and tend to become greyer or whiter due to hyalinisation. Visual acuity, peripheral fields, dark adaptation, colour vision and ERG are normal in early stages. Sometimes pericaecal scotoma or a linear scotoma corresponding to the streaks may be seen. After the age of 50 years, vision reduces to 6/60 or less. Fundus fluorescein angiography shows hyperfluorescence in the early phase due to atrophy of the retinal pigment epithelium overlying the streaks ,. Persistent fluorescence is a characteristic finding.
The common complications are
1. macular lesions: haemorrhages, pigmentary deposits, patches of atrophy and proliferative changes of disciform degeneration
2. coarse granulation of large areas of fundus by pigmentary deposits suggesting the appearance of peau d' orange or yellowish white spots scattered in the peripheral fundus
3. patches of choroidal atrophy with pigment proliferation or choroidal sclerosis
4. colloid bodies on Bruch's membrane or hyaline bodies on the disc
5. progressive atrophy of the pigment epithelium in the early stage. Fluorescein angiography in our patient revealed irregular streaks radiating from the optic disc in both eyes and evidence of chorioretinal atrophy in the macular region in the left eye [Figure - 2][Figure - 3].
The chorioretinal degeneration and atrophy produced marked reduction in visual acuity in the left eye in our patient.
The recognition of angioid streaks in early life is important since the patients can be advised regarding protection of their eyes from even minor trauma. They can also be advised regarding the possibility of a marked loss of central vision and the use of visual aids in such a situation. They may be instructed to seek ophthalmic evaluation for subtle visual abnormalities which may be manifestations of choroidal neovascularisation which if detected early may be treatable by photocoagulation .
| References|| |
Duke-Elders System of Ophth. Vol XI first Ed. Page 721-734.
Jack. J. Kanski. Eye in Systemic Diseases - 1986. page 114-116.
Basic & Clinical Science Course-American Academy of Ophthalmology. Section 4: Retina & vitreous 1989. page 68.
Gilbert Grand M., Michael J. Issertman.. Charles W Miller.: Angioid streaks associated with pseudoxanthoma elasticum in a 13-year old patient - Ophthalmology 1987: 94:197-200.
[Figure - 1], [Figure - 2], [Figure - 3]