EURETINA 2021 Day 2: Sub-macular hemorrhage during vitrectomy and foveal hypoplasia

Raziyeh Mahmoudzadeh, MD
Retina Research Fellow
Wills Eye Hospital

Sub-macular hemorrhage during vitrectomy for retinal detachment: how to proceed

Lars Hattenbach, Ludwigshafen, Germany

Dr. Lars-Olof Hattenbach, MD, professor of ophthalmology and chairman of the department of ophthalmology at Ludwigshafen Hospital, Germany, on the second day of EURETINA 2021, discussed the best ways to manage unexpected subretinal/submacular hemorrhage during vitrectomy for retinal detachment. It can be triggered by several risk factors, including underlying systemic diseases (hypertension, diabetes), neovascular posterior segment disorders, treatment with anticoagulant or antiplatelet agents, age and poor general status.

Management of two cases was discussed in this presentation. The first one was a 77-year-old female with arterial hypertension and systemic steroid treatment for breast cancer who underwent vitrectomy for rhegmatogenous retinal detachment with proliferative vitreoretinopathy (PVR). She developed subretinal hemorrhage during vitrectomy, most likely as a result of retinectomy and shaving of the dense vitreous base with the removal of PVR membranes. To manage this situation, the infusion fluid pressure was elevated immediately, and diathermy at the retinectomy site was performed. Vitrectomy was then continued, followed by fluid-air exchange with drainage of subretinal blood using a soft tip needle. The surgery was completed with endolaser treatment of retinal breaks and silicone oil tamponade. On post-operative visits the remaining subretinal blood resolved continuously and visual acuity recovered to the baseline level. There is evidence that peri-operative anticoagulant use is a risk factor for the development of intra or post-operative hemorrhage in ocular surgeries. Surgeons should be aware that some patients develop delayed hemorrhage post-operatively that requires repeat vitrectomy and washout.

The second case was a 70-year-old female with atrial fibrillation on anticoagulation, hypertension and type 2 diabetes mellitus who developed spontaneous vitreous hemorrhage one week after vitrectomy and scleral fixation of an intraocular lens. Since there was no resolution of hyphema and vitreous hemorrhage, the decision was made for repeat the vitrectomy. After a lavage of the anterior chamber and vitreous cavity, a dense coagulated periretinal hemorrhage in the inferotemporal quadrant associated with retinal detachment and subretinal hemorrhage at the posterior pole was detected. After complete removal of the periretinal blood clots, diathermy and tamponade with heavy liquid, a drainage retinotomy was created and fluid-air-exchange with drainage of subretinal blood was performed. After successful reattachment of the retina, the endolaser was used to barricade the retinotomy site, followed by a silicone oil tamponade. One month postoperatively, the visual acuity recovered to 20/80 with no recurrence of ocular hemorrhage despite continued anticoagulant treatment.

In conclusion, subretinal hemorrhage can be successfully managed if it occurs. Overall, subretinal hemorrhage during vitrectomy for retinal detachment or other conditions is a rare complication that may be associated with several risk factors including anticoagulant use. However, prompt though not too aggressive removal of blood and successful retina reattachment provides excellent chances for visual recovery. So be prepared and don’t find yourself in a nightmare!

Foveal hypoplasia in ocular and systemic diseases

Kristina Hess, Bonn, Germany

Krisitina Hess, MD, from Bonn, Germany, currently working at the National Institute of Health (NIH) in the United States, discussed the ocular and systemic diseases that lead to foveal hypoplasia. A normal foveal pit is characterized as follows:  diameter of 2474±243 µm, depth of 131±22 µm, and 7/110 of normal eyes have persistent inner retinal layers that are not associated with any ocular or systemic diseases. The first condition associated with foveal hypoplasia is mutation of the PAX6 gene, which causes aniridia. It has a high phenotypical variability ranging from Peters anomaly to no visible changes depending on the mutation. Foveal hypoplasia is present in the majority of these cases and is mainly caused by lack of centrifugal displacement of the inner retinal layers away from the fovea.  Albinism is another well-known disease associated with foveal hypoplasia. It is caused by abnormalities in melanin biosynthesis and maturation of melanosomes. There are two forms of albinism. The oculocutaneos form (AR) includes hair and skin and ocular form (X-linked). The majority of these patients (>99%) have foveal hypoplasia, which is usually in higher grades, and there is a degree of phenotypic variability.  It is mostly due to delayed maturation of the visual system, and is associated with decreased visual acuity in these patients.

Foveal hypoplasia, optic nerve decussation defects, and anterior segment dysgenesis (FHONDA) in the absence of albinism is associated with SLC38A8. Most of these patients (90%) are clinically misdiagnosed as albinism or PAX6 associated foveal hypoplasia. Foveal hypoplasia is usually severe with the absence of outer nuclear layer widening and outer segment lengthening. Two other diseases result from collagen-associated mutations. The first is Stickler syndrome, caused by a mutation in collagen in the vitreous (collagen II). These patients have high myopia, altered vitreoretinal interface, and mild foveal hypoplasia associated with the missing vascular zone. The systemic associations of Stickler syndrome include hearing loss, hypermobile joints, flattened vertebrae, and flattened facial appearance. The second condition is Alport syndrome which is caused by mutations in collagen IV. Collagen IV is part of basement membranes in the body, and in the eye, it is part of the limiting membrane and Bruch’s membrane. Recently it has been reported that these patients can have mild foveal hypoplasia associated with absent foveal avascular zone. They can also have posterior polymorphous corneal dystrophy, anterior lenticonus, dot, and fleck retinopathy. The most important problem in these patients is renal failure and sensorineural hearing loss.

Foveal hypoplasia and peripheral vascular abnormalities can also be seen in retinopathy of prematurity and familial exudative vitreoretinopathy (FEVR). Foveal hypoplasia is found in about half of the patients with FEVR.  Incontinentia pigmenti is X-linked dominant and affected males mostly die in utero because of a severe form of this disease, and 90-95% of living patients are females. About 25-70% of these patients have ocular changes, including occlusive vascular alterations in the periphery and foveal hypoplasia centrally. The last condition is achromatopsia which results in dysfunction of all three cone classes. Foveal hypoplasia is present in about 50% of these patients, but the foveal avascular zone can be present despite the foveal hypoplasia. The foveal hypoplasia is atypical in these patients because of alterations in the outer retinal layers.

In summary, the causes of foveal hypoplasia are highly heterogeneous. Foveal hypoplasia and absence of foveal avascular zone are closely correlated, whereas the best-corrected visual acuity is not compellingly associated with foveal hypoplasia.