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Bilateral Serous Retinal Detachment Due to Protein-Losing Enteropathy
Arch Ophthalmol. 2004;122:1067-1070.
Protein-losing enteropathy (PLE) refers to a group of disorders causing hypoproteinemia and edema in the absence of proteinuria or defects in protein synthesis.1 It is characterized by increased protein loss in the gastrointestinal tract and is commonly suggested by the presence of peripheral edema and low serum albumin and globulin levels, in the absence of renal and hepatic disease. We describe an unusual patient with a corticosteroid-responsive PLE who developed bilateral serous retinal detachments (RDs) coincident with a flare of her enteropathy. With appropriate treatment of the enteropathy, there was resolution of the serous detachments.
Report of a Case
A 47-year-old woman was diagnosed as having idiopathic PLE 8 years before referral to the Retina Service of the Department of Ophthalmology and Visual Sciences, University of Wisconsin–Madison.
In 1995, she was seen at the Mayo Clinic, Rochester, Minn, complaining of swelling over the face and lower extremities. The findings from the physical examination revealed anasarca. She reported a history of multiple episodes of edematous facies and lower extremity edema responsive to intermittent short courses of oral corticosteroid therapy.
Laboratory analysis revealed severe hypoalbuminemia, with serum albumin levels measuring 1.4 g/dL (normal range, 3.5-5.5 g/dL). Renal and liver function test results were unremarkable. The antinuclear antibody was mildly elevated, although a complete rheumatologic evaluation ruled out rheumatologic disease, including systemic lupus erythematosus.
Protein-losing enteropathy was suspected. Twenty-four-hour stool analysis revealed a high level of fecal  1-antitrypsin concentration at 642 mg/dL (118.13 µmol/L) (normal, <54 mg/dL [<9.94 µmol/L]),2 and 24-hour serum analysis for 1-antitrypsin clearance was 964 mL/24 h (normal, <27 mL/24 h). (Intestinal clearance of 1-antitrypsin has been shown to be a reliable diagnostic test for PLE.2) Esophagogastroduodenoscopy and gastric biopsy revealed a congestive gastropathy. A diagnosis of PLE was made.
Given the patient's history of responsiveness to corticosteroid administration, she was successfully treated with intravenous corticosteroids (methylprednisolone), intravenous furosemide, and albumin, with resolution of the anasarca. The PLE was subsequently well controlled with 10 mg of methylprednisolone (Medrol) every other day and 50 mg of mercaptopurine (Purinethol) daily for the subsequent 8 years.
The patient developed a systemic viral illness 4 weeks before being seen at the Department of Ophthalmology and Visual Sciences, University of Wisconsin–Madison clinic. As her upper respiratory symptoms began to resolve, she sought care from a primary care physician because of complaints of increased abdominal girth, profound anorexia, diarrhea, and shortness of breath.
The medical workup revealed abnormal liver function test results, including a low serum albumin level of 2.3 g/dL and elevated alkaline phosphatase (750 U/L), aspartate aminotransferase (147 U/L), and alanine aminotransferase (201 U/L). An abdominal ultrasound revealed no structural pathologic condition of the hepatobiliary system.
She was diagnosed as having a systemic viral syndrome causing exacerbation of her underlying PLE, primarily by interfering with hepatic protein synthesis. Epstein-Barr virus, cytomegalovirus, and hepatitis A, B, and C titers were negative.
During the systemic evaluation, the patient also complained of a gradual, progressive loss of the bilateral superior visual fields. She reported that her central vision was blurry in the morning on waking and when lying in a supine position. She denied pain, floaters, or photopsias. Her local ophthalmologist diagnosed bilateral inferior RDs and referred the patient for further evaluation and treatment.
Visual acuity was 20/40 OU. Intraocular pressures were normal. Slitlamp examination revealed anterior chamber cells and flare and moderate anterior vitreous cells. Dilated funduscopic examination revealed bilateral inferior bullous RDs when the patient was in an upright posture (Figure 1 and Figure 2). There was a superior subretinal fluid shift, involving the macula, with supine positioning. Shallow peripheral choroidal detachments were noted. Diffuse choroidal thickening was confirmed by ultrasonography (Figure 3).
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Figure 1. Right eye. A, At initial examination, a serous detachment is prominent inferiorly, with shallow fluid involving the macula. B, After treatment with systemic corticosteroids, there is resolution of the subretinal fluid. Retinal pigment epithelial clumping is present.
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Figure 2. Left eye. A, At initial examination, a serous detachment is present inferiorly, extending into the macula. B, One month after treatment, there is a resolution of the detachment.
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Figure 3. B-scan of right eye with patient in a supine position. Fluid shifts into the macula. Note the thickening of the choroid.
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Fluorescein angiography revealed deep-speckled hyperfluorescence in the inferior macula and inferior periphery (Figure 4). Mild choroidal leakage was noted late in the angiogram. No retinal vascular leakage or vessel wall staining suggesting retinal vasculitis was present.
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Figure 4. Late-phase fluorescein angiogram of the right eye. There is irregular, deep-speckled hyperfluorescence in the inferior macula and inferior periphery. No leakage is noted from the retinal vasculature.
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Following reevaluation by and discussion with the patient's primary care physician, treatment of the PLE exacerbation was initiated with an increase of methylprednisolone to 30 mg/d. There was a rapid resolution of her systemic edema, with a weight loss of 12 kg, and resolution of the shortness of breath and diarrhea. The dosage was tapered during the following 3 to 4 weeks to her baseline level of 10 mg of methylprednisolone every other day. The serous RDs gradually resolved during 5 months of follow-up, with her visual acuity improving to 20/25 OU (Figure 1 and Figure 2). There was persistent mild anterior chamber inflammation, with iris-lens synechiae formation. As the high-dose systemic corticosteroids were tapered, she required topical treatment with 1% prednisolone acetate. Anterior segment inflammation resolved during 5 months of follow-up.
Comment
There are multiple known causes3 of serous (exudative) RD, including inflammatory etiologies such as scleritis, vascular tumors (eg, diffuse choroidal hemangioma), neoplastic disease, or autoimmune pathologic conditions (eg, Vogt-Koyanagi-Harada syndrome [VKH]). To our knowledge, this is the first case reported of bilateral serous RD secondary to a PLE.
This patient had ocular features that are similar to the symptom complex in VKH. She had bilateral serous RD and diffuse choroidal thickening. Vogt-Koyanagi-Harada syndrome affects women more commonly than men, especially among those aged 20 to 50 years.4 As in VKH, the serous RD resolved on administration of systemic corticosteroids. However, despite these similarities, there are features not synchronous with a diagnosis of VKH.5 There was no disc hyperemia or development of a "sunset" fundus. There were no meningeal or neurologic symptoms. In addition, there were no cutaneous signs of alopecia, poliosis, or vitiligo. She was also not of ancestry typically affected by VKH.4
The neurosensory retina maintains attachment to the retinal pigment epithelium largely through dynamics of retinal fluid flow.2 Although no study has shown that lower intravascular oncotic pressure leads directly to serous RD, Negi and Marmor6 demonstrated the importance of oncotic pressure in maintaining the necessary fluid dynamics for intact retinal attachment. They showed that nonrhegmatogenous RDs induced by subretinal injection of fluids of higher osmolality took longer to resolve spontaneously than those induced by fluids of lower osmolality. Sustained reduced oncotic pressure in the choriocapillaris due to severe hypoalbuminemia could have led to a decreased vitreoretinal to choroidal fluid outflow in this patient, thereby causing bilateral serous RDs. This was exacerbated by the outflow of serous fluid from the vascular space as the oncotic pressure of the choroid fell due to wasting of intravascular protein.
Because the patient's medical systemic disease was appropriately treated with systemic corticosteroids, the hypoalbuminemia was reversed and normal pump function of the retinal pigment epithelium was able to remove the serous fluid from the subretinal space. This was coincident with a reversal of her systemic edema and a loss of more than 12 kg of fluid overload.
Protein-losing enteropathy is an unusual systemic disorder. Nevertheless, it must be considered in the differential diagnosis of causes of serous RD.
The authors have no relevant financial interest in this article.
AUTHOR INFORMATION
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Jayant Venkatramani, MBBS;
Justin L. Gottlieb, MD;
Thom S. Thomassen, MD;
Adamarie Multari, MD
Correspondence: Dr Gottlieb Retina Service, Department of Ophthalmology and Visual Sciences, University of Wisconsin–Madison, 2870 University Ave, Suite 206, Madison, WI 53705 (jlgottlieb{at}wisc.edu).
REFERENCES
1. Binder HJ. Disorders of absorption. In: Braunwald E, Fauci AS, Isselbacher KJ, Kasper DL, Hauser SL, Longo DL, Jameson JL, eds. Harrison's Online. Chap 286. Available at: http://www.harrisons.accessmedicine.com. Accessed May 26, 2003.
2. Florent C, L'Hirondel C, Desmazures C, et al. Intestinal clearance of 1-antitrypsin: a sensitive method for the detection of protein-losing enteropathy. Gastroenterology. 1981;81:777-780.
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3. Anand R, Tasman WS. Tasman nonrhegmatogenous retinal detachment. In: Ryan SJ, Wilkinson CP, eds. Retina. Vol 3. 3rd ed. St Louis, Mo: Mosby Inc; 2001:2076-2097.
4. Moorthy RS, Inomata H, Rao NA. Vogt-Koyanagi-Harada syndrome. Surv Ophthalmol. 1995;39:265-292.
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5. Snyder DA, Tessler HA. Vogt-Koyanagi-Harada syndrome. Am J Ophthalmol. 1980;90:69-75.
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6. Negi A, Marmor MF. Effects of subretinal and systemic osmolality on subretinal fluid resorption. Invest Ophthalmol Vis Sci. 1984;25:616-620.
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