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Arch Ophthalmol. 2002;120:862-864.

We describe the clinical course of 3 cases of circumscribed, choroidal hemangiomata that were associated with progressive exudative detachments during pregnancy.

Report of Cases
Case 1

A 31-year-old woman, gravida 1, visited our affiliation when 7 months pregnant with a 4-week history of blurred vision in the left eye. She had a cutaneous hemangioma of the left eyelid, dilated episcleral vessels, and amblyopia. Her visual acuity was 20/13 OD and 20/1200 OS. Left fundoscopy revealed an inferior temporal, orange choroidal mass with some subretinal fibrosis. Overlying the mass was a large inferior retinal detachment with shifting fluid (Figure 1). The mass (excluding the overlying fluid) was 12.0 x 12.0 mm wide and 7.4 mm high; it demonstrated high internal reflectivity on ocular ultrasound. Right fundoscopy results were unremarkable.

View larger version (27K): [in this window] [in a new window] Figure 1. Case 1. Left fundus photograph taken at 7 months' gestation showing a large inferior exudative detachment overlying the circumscribed choroidal hemangioma.

The diagnosis was a circumscribed choroidal hemangioma in Sturge-Weber syndrome associated with an exudative detachment. One month later, the exudative detachment had significantly increased in size. The patient's visual acuity subsequently deteriorated to light perception. She was at risk for developing a total retinal detachment, and therefore underwent an elective cesarean section at 37 weeks' gestation.

Five weeks' post partum, there was a marked reduction in the size of the retinal detachment (Figure 2), and her visual acuity improved to hand motions. She had one treatment of transpupillary diode thermotherapy. Five months later, the retinal detachment had completely resolved, and the hemangioma was replaced by scar tissue. Her pinhole corrected acuity improved to 20/80 OS.

View larger version (30K): [in this window] [in a new window] Figure 2. Case 1. Left fundus photograph taken at 5 weeks' post partum, prior to any thermotherapy. There is a marked reduction in the subretinal fluid in this eye, which had clinical evidence of a choroidal hemangioma with some subretinal fibrosis.

Case 2

A 26-year-old woman, gravida 1, was referred when 31 weeks pregnant. She had a congenital cutaneous angioma along the first division of the right trigeminal nerve involving the right upper eyelid, with dilated episcleral veins on the same side. Her visual acuity was 20/220 OD and 20/60 OS. Fundoscopy revealed a large dome-shaped detachment at the posterior pole associated with cystoid macular edema (Figure 3).

View larger version (27K): [in this window] [in a new window] Figure 3. Case 2. Right fundus photograph taken at 31 weeks' gestation showing a large dome-shaped detachment at the posterior pole, which is associated with retina striae and cystoid macular edema.

She had no visual symptoms because the right eye was amblyopic. Underlying the exudative detachment was a circumscribed, orange choroidal mass that showed high internal reflectivity on B-scan.

The diagnosis was a circumscribed choroidal hemangioma in Sturge-Weber syndrome associated with an exudative detachment. Two weeks later, her acuity deteriorated to counting fingers as the subretinal fluid extended beyond the posterior pole to detach the inferior retina.

No intervention was undertaken during her pregnancy, but the intention was to treat the patient with radiotherapy after the delivery. She was examined 4 months after childbirth. When visual acuity started to improve to 20/400 OD, the subretinal fluid was resolving spontaneously. At 9 months' post partum, visual acuity returned to 20/120 OD, and the retina was entirely flat. An isolated circumscribed choroidal hemangioma was seen at the right posterior pole with associated subretinal fibrosis (Figure 4). Four years later, the retina remains flat. During the course of follow-up the choroidal hemangioma has not changed in size. The patient has not had any further pregnancies.

View larger version (31K): [in this window] [in a new window] Figure 4. Case 2. Right fundus photograph taken at 9 months' post partum with no treatment. The retinal detachment has resolved; subretinal fibrosis is seen associated with the circumscribed choroidal hemangioma.

Case 3

A 19-year-old woman visited our affiliation 10 months after the birth of her first child. She complained of blurred vision and micropisa in the right eye, which started when she was 8 months pregnant. Her vision had not improved following delivery. Her visual acuity at initial visit was 20/60 OD and 20/20 OS. She had a dome-shaped, orange choroidal mass at the posterior pole and a large inferior exudative detachment. She did not have a facial angioma or any episcleral abnormality.

The diagnosis was a solitary, circumscribed choroidal hemangioma with an associated exudative detachment. The patient was treated with argon laser photocoagulation. One month later, her vision improved, but on fundal examination, the hemangioma had not responded, and the inferior detachment remained unchanged. Two further applications of argon laser photocoagulation were administered, but the retinal detachment did not resolve. The patient was lost to follow-up.

Five years after delivery, the patient returned with an acuity of no light perception, as there was a total retinal detachment. Eight months later, the eye became painful as the intraocular pressure rose to 41 mm Hg. A right enucleation was subsequently performed for uncontrolled secondary glaucoma. The pathology report confirmed the diagnosis of a choroidal hemangioma.

Comment
Little is known about the behavior of choroidal hemangioma in pregnancy. There is one previous report of leakage from a circumscribed choroidal hemangioma in pregnancy, and the fluid resolved following full-term normal delivery.¹

In our case series, exudative retinal detachments developed during the third trimester of pregnancy. All the mothers were primigravida, but none had symptoms or signs of pre-eclampsia. Blood pressure was monitored during the pregnancies. Cases 1 and 2 had Sturge-Weber syndrome. The most frequently described ocular manifestation in this syndrome is a flat diffuse choroidal hemangioma, giving the fundus the typical "tomato ketchup" appearance, but recently circumscribed elevated choroidal hemangiomatas have been described.^2-3

Isolated hemangiomas are known to grow very slowly.⁴ Shields et al⁵ compared ultrasound measurements of an enlarging circumscribed choroidal hemangioma with histology reports at enucleation. They demonstrated that enlargement was the result of venous congestion and was not due to cell multiplication.⁵ The altered hemodynamic state in pregnancy may result in engorgement of the vascular networks within a hemangioma, resulting in increased transudation of fluid into the subretinal space. This could explain the observed leakage from a choroidal hemangioma in the late stages of pregnancy. There may also be hormonal influence on the growth of vascular malformations; however, this remains speculative.

This series demonstrates that following delivery, the subretinal fluid tends to reabsorb, and the hemangioma ceases to leak. The retina was flat at 5 months' post partum following one episode of thermotherapy in case 1. The retina was flat at 9 months' post partum with no treatment in case 2. However, resolution of an exudative detachment after delivery is not always the rule, even following the supplementary argon laser photocoagulation treatment. The third patient suffered from a total retinal detachment and secondary glaucoma, and she finally required enucleation.

In summary, the effect of pregnancy on choroidal hemangiomas is unknown, although in select situations, there may be an apparent increased exudation and enlargement of the lesion. While 2 of these 3 cases stabilized during the postpartum timeframe, the clinical course may be quite variable.

AUTHOR INFORMATION
No grants or sponsors were used for this study.

The authors have no commercial interest or conflicting relationship.

Victoria M. Cohen, MRCOphth; Paul A. Rundle, FRCOphth; Ian G. Rennie, FRCS, FRCOphth
Sheffield, England

Corresponding author: Victoria Cohen, MRCOphth, Department of Ophthalmology, Addenbrooke's Hospital, Hills Road, Cambridge, England CB2 2QQ (e-mail: victoria{at}victoriac.freeserve.co.uk).

REFERENCES
1. Pitta C, Bergen R, Littwin S. Spontaneous regression of a choroidal haemangioma following pregnancy. Ann Ophthalmol. 1979;11:772-774. ISI | PUBMED 2. Scott IU, Alexandrakis G, Cordahi GJ, Murray TG. Diffuse and circumscribed choroidal hemangiomas in a patient with Sturge-Weber syndrome. Arch Ophthalmol. 1999;117:406-407. FREE FULL TEXT 3. Cheung D, Grey R, Rennie IG. Circumscribed choroidal hemangioma in a patient with Sturge Weber Syndrome. Eye. 2000;14:238-239. 4. Medlock RD, Augsburger JJ, Wilkinson CP, et al. Enlargement of circumscribed choroidal hemangiomas. Retina. 1991;11:385-388. PUBMED 5. Shields JA, Stephens RF, Eagle RC, et al. Progressive enlargement of a circumscribed choroidal hemangioma. Arch Ophthalmol. 1992;110:1276-1278. ABSTRACT

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