to report our experience in managing a case of bilateral optic disc oedema and unilateral cystoid macular oedema with a posterior subtenon ( pst ) injection of triamcinolone acetonide and intravitreal bevacizumab ( ivb ) in a patient with polyneuropathy , organomegaly , endocrinopathy , monoclonal gammopathy , and skin changes ( poems ) syndrome . a 50-year - old healthy woman complaining of bilateral blurred vision for several days was referred to the ophthalmologist . g / l and a vascular endothelial growth factor ( vegf ) concentration of 6.1 ng / ml . the cerebrospinal fluid protein content was 131 mg / dl . the diagnosis of poems syndrome was made based on the polyneuropathy , hepatosplenomegaly , iga lambda monoclonal proteinemia , and optic disc swelling . ten weeks thereafter , 2 ivb injections of 2.5 mg were administered to the left eye , with a 4-week interval . because of an incomplete recovery of her visual functions , diarrhoea and general malaise , melphalan , followed by autologous peripheral blood stem cell transplantation , was initiated . after systemic treatment , she had no visual complaints anymore , the optic disc oedema had resolved , and the vegf serum concentration had normalized . local treatment with ivb and pst injection of triamcinolone can be an option for ocular symptoms in poems syndrome . however , given the incomplete recovery of the ocular abnormalities and the other symptoms , systemic treatment remains standard . a 50-year - old woman was referred to us for bilateral blurred vision as well as dryness and irritation of her eyes for several days . except for a mild fatigue and a slight headache , she had no further complaints . her visual acuity was 1.0 in both eyes , and slit - lamp examination was normal . a fundus examination revealed bilateral optic disc swelling and small preretinal peripapillary splinter haemorrhages in the right eye ( fig . humphrey visual field testing showed an enlarged blind spot and an inferonasal deficit in both eyes ( right > left ) ( fig . fluorescein angiography demonstrated a bilateral late leakage of dye from the optic disc and pooling consistent with cystoid macular oedema ( cmo ) in the left eye . optical coherence tomography ( oct ) performed with cirrus hd - oct 6.0 showed an in - creased macular thickness with a small amount of subretinal fluid at the fovea in the left eye . general physical examination was normal except for absent ankle reflexes and a diminished sensation in the feet . laboratory tests showed an erythrocyte sedimentation rate of 30 mm / h , elevated levels of m - protein type iga of 1.4 g / l , and a vascular endothelial growth factor ( vegf ) concentration of 6.1 ng / ml ( n = 0.0280.966 mg cerebrospinal fluid pressure was normal , and the cerebral spinal fluid had a protein content of 131 mg / dl ( normal < 45 mg / dl ) . the diagnosis of polyneuropathy , organomegaly , endocrinopathy , monoclonal gammopathy , and skin changes ( poems ) syndrome was made based on polyneuropathy , hepatosplenomegaly , iga lambda monoclonal proteinemia , and the optic disc swelling . after 2 months , local treatment with 40 mg posterior sub - tenon ( pst ) injection of triamcinolone in both eyes was started because of continuous complaints of blurred vision . this was followed by 2 intravitreal bevacizumab ( ivb ) injections of 2.5 mg in the left eye at intervals of 4 weeks after 10 weeks . local therapy with pst injection had resulted in a small decrease of the optic disc swelling after 4 weeks . in the left eye , the macular oedema regressed , and the central retinal thickness , as measured by oct , diminished . the ivb treatment resulted in a slight regression of the optic disc oedema ( odo ) and a minimal decrease of the cmo ( table 1 ) . because of diarrhoea and general malaise and the fact that the blurred vision had diminished but not completely disappeared , melphalan was initiated . after systemic treatment , the patient had no further visual complaints and the odo had disappeared ( fig . poems syndrome is a rare and chronic paraneoplastic disorder characterized by polyneuropathy , organomegaly , endocrinopathy , m - protein and skin changes , resulting from an underlying plasma cell disorder . the acronym refers to some , but not all the features . poems syndrome is potentially fatal with a substantial deterioration in quality of life and a chronic clinical course [ 1 , 2 ] . odo , unilateral or bilateral , is present in 2955% of patients [ 2 , 3 ] . macular oedema secondary to optic disc leakage is rare and associated with macular detachment [ 4 , 5 ] . these patients usually report blurred vision [ 2 , 3 , 4 , 5 , 6 ] . in a study of 33 poems patients undergoing ophthalmologic examination , 5 patients reported diplopia , 15 patients had blurred vision , and 3 patients had ocular pain . bilateral ode was seen in about half of the cases , with about one third asymptomatic at the first examination . an elevated production of proangiogenic and proinflammatory cytokines are believed to play a central role . vegf and other cytokines are elevated , reflecting the clinical condition [ 1 , 7 ] . the mechanism underlying the formation of odo and cmo associated with poems syndrome is unclear . odo can result either from increased intracranial pressure or from a chronic overproduction of cytokines ( such as vegf ) which supports extravascular volume overload secondary to raised vascular permeability [ 7 , 8 , 9 ] . development of cmo might be attributed to the elevated serum vegf and not to the vegf secreted from retinal tissues . odo and cmo may result from an osmotic gradient created by the deposition of high - molecular - weight immunoglobulins within and beneath the neurosensory retina . anti - vegf antibodies such as bevacizumab are an effective treatment for ocular conditions associated with increased vegf levels . theoretically , they could also be used locally [ 1 , 8 ] . our case is the first in which bevacizumab was used intravitreally , resulting ( in combination with pst injection ) in a partial regression of odo and cmo . odo and cmo normalized however only after systemic treatment . because poems is a systemic condition , the ocular inhibition of vegf alone is insufficient . additionally , decreasing vegf serum concentration as a response to systemic treatment correlated with the resolving of optic disc swelling . pst has been widely used in ophthalmology for the treatment of intraocular proliferative , oedematous , and neovascular diseases . a reduction of macular oedema can be induced by the anti - inflammatory reaction of steroids as investigated in several studies [ 9 , 10 ] . in a case report describing pars plana vitrectomy and intraocular triamcinolone injections as a treatment for cmo , the macular thickness decreased . in the untreated eye , the macular thickness altered in relation to the serum vegf level . in conclusion , local treatment with ivb and pst injection of triamcinolone can be an option for ocular symptoms in poems syndrome , but always in combination with the standard treatment of this syndrome .
purposeto report our experience in managing a case of bilateral optic disc oedema and unilateral cystoid macular oedema with a posterior subtenon ( pst ) injection of triamcinolone acetonide and intravitreal bevacizumab ( ivb ) in a patient with polyneuropathy , organomegaly , endocrinopathy , monoclonal gammopathy , and skin changes ( poems ) syndrome.case reporta 50-year - old healthy woman complaining of bilateral blurred vision for several days was referred to the ophthalmologist . 
 an eye examination showed a bilateral optic disc swelling and small preretinal haemorrhages . 
 in addition , a peripheral neuropathy was found . 
 laboratory tests showed elevated levels of m - protein type iga of 1.4 
 g / l and a vascular endothelial growth factor ( vegf ) concentration of 6.1 ng / ml . the cerebrospinal fluid protein content was 131 mg / dl . 
 an abdominal ct scan revealed hepatosplenomegaly . 
 the diagnosis of poems syndrome was made based on the polyneuropathy , hepatosplenomegaly , iga lambda monoclonal proteinemia , and optic disc swelling . 
 as vision declined , 40 mg pst injection of triamcinolone was given bilaterally . 
 ten weeks thereafter , 2 ivb injections of 2.5 mg were administered to the left eye , with a 4-week interval . because of 
 an incomplete recovery of her visual functions , diarrhoea and general malaise , melphalan , followed by autologous peripheral blood stem cell transplantation , was initiated . 
 after systemic treatment , she had no visual complaints anymore , the optic disc oedema had resolved , and the vegf serum concentration had normalized.discussionlocal treatment with ivb and pst injection of triamcinolone can be an option for ocular symptoms in poems syndrome . 
 however , given the incomplete recovery of the ocular abnormalities and the other symptoms , systemic treatment remains standard .