Choroidal effusion and serous retinal detachment in protein-losing enteropathy from intestinal lymphangiectasia.

Protein-losing enteropathy (PLE) is a condition manifesting loss of serum proteins through the bowel lumen. One form of PLE involves congenital or acquired intestinal lymphangiectasia with pathologic dilation of lymphatic channels leading to leakage of lymph into the small bowel lumen with decreased protein absorption and loss of cellular elements such as lymphocytes, often associated with immune deficiency.1Fuss IJ. Strober W. et al.Protein-losing enteropathies.in: Strober W Mestecky J. Russell M. Mucosal immunology. Academic Press, New York2015: 1667-1694Google Scholar Other forms of PLE are associated with allergic, hypersensitivity, inflammatory, immunodeficiency, and infectious bowel diseases. Rarely, PLE can affect visual acuity through fluid retention in the eye.2Mafi M Khodabandeh A Riazi-Esfahani H Mirghorbani M. Bilateral central serous retinal detachment in protein-losing enteropathy.Korean J Ophthalmol. 2019; 33: 577-578Crossref PubMed Google Scholar, 3Venkatramani J Gottlieb JL Thomassen TS Multari A. Bilateral serous retinal detachment due to protein-losing enteropathy.Arch Ophthalmol. 2004; 122: 1067-1070Crossref PubMed Scopus (11) Google Scholar, 4Wong AHY Li STL Ho CK ou PF Bilateral serous macular elevation associated with lupus protein-losing enteropathy.J Ophthalmic Vis Res. 2017; 12: 245-246PubMed Google Scholar We report a patient with PLE related to intestinal lymphangiectasia with vision loss from choroidal effusion and serous retinal detachment. A 55-year-old Chinese female with a history of intestinal lymphangiectasia noted blurred vision in her right eye for 5 months. On initial presentation elsewhere, her visual acuity was 20/30 OD and 20/400 OS from previous traumatic macular choroidal rupture. Optical coherence tomography (OCT) revealed intra- and subretinal fluid in the macular region OD. Antibody tests for syphilis and tuberculosis were negative. There was no evidence of Vogt–Koyanagi–Harada syndrome, sympathetic ophthalmia, or tumour on fundus examination or magnetic resonance imaging. Oral prednisone (30 mg daily) failed to resolve the fluid. Pars plana vitrectomy with vitreous biopsy revealed a few cells of mature T-cell lineage with no B cells and no evidence of clonality. The patient was referred to the Wills Eye Hospital Ocular Oncology Service for evaluation. On examination 1 month later, the patient's visual acuity had dropped to counting fingers at 2 feet OD and 20/400 OS, with intraocular pressures of 10 mm Hg in each eye. Externally, there was upper and lower eyelid edema OU and pitting edema of both legs. The anterior segment was normal OU. Funduscopically, the unaffected left eye showed the known macular choroidal rupture with retinal pigment epithelium alterations and no hemorrhage. The affected right eye demonstrated an extensive choroidal effusion and serous retinal detachment (Fig. 1A) with documented positional shifting confirmed on ultrasonography (Fig. 1B). OCT of the right eye (Fig. 2A) showed intra- and subretinal fluid with retracted photoreceptors, suggesting chronicity, as well as dilation of choroidal vessels limited to Haller's layer (yellow arrows). OCT of the left eye (Fig. 2B) demonstrated the macular chorioretinal scarring from previous trauma and similar dilation of choroidal vessels limited to Haller's layer (yellow arrows). There was no evidence of intraocular tumour. Serum albumin measured 2.4 g/dL (range, 3.8–4.9 g/dL). Based on the intestinal lymphangiectasia and hypoalbuminemia, it was suspected that the choroidal effusion was transudative “third spacing” fluid. Injection of triamcinolone acetonide (40 mg/mL) to sub-Tenon's fascia and an increase in oral prednisone (60 mg daily) were prescribed as well as gastrointestinal consultation to reduce protein loss. Ultimate scleral window surgery was discussed. The patient was subsequently followed elsewhere. Surgical correction of the intestinal lymphangiectasia with local resection or lymphatic embolization was not pursued. The albumin level eventually returned to 3.8 g/dL (range, 3.8–4.9 g/dL) at 12-month follow-up, but the choroidal effusion and serous retinal detachment OD persisted with vision decline to light perception.Fig. 2(A) Optical coherence tomography of the right eye showed sub- and intraretinal fluid with retracted photoreceptors indicating chronicity as well as dilation of the choroidal vessels limited to Haller's layer (yellow arrows); (B) optical coherence tomography of the left eye showed macular chorioretinal scarring secondary to previous trauma and loss of choroidal vessels except at the level of dilation of Haller's layer, where they are relatively dilated (yellow arrows).View Large Image Figure ViewerDownload Hi-res image Download (PPT) PLE is a syndrome characterized by loss of serum proteins from the gastrointestinal tract.1Fuss IJ. Strober W. et al.Protein-losing enteropathies.in: Strober W Mestecky J. Russell M. Mucosal immunology. Academic Press, New York2015: 1667-1694Google Scholar The metabolic turnover of serum proteins is balanced between synthesis, endogenous catabolism, and excretion.1Fuss IJ. Strober W. et al.Protein-losing enteropathies.in: Strober W Mestecky J. Russell M. Mucosal immunology. Academic Press, New York2015: 1667-1694Google Scholar PLE results in a shift in this delicate equilibrium toward net protein loss, commonly leading to secondary pathologies related to protein deficit.1Fuss IJ. Strober W. et al.Protein-losing enteropathies.in: Strober W Mestecky J. Russell M. Mucosal immunology. Academic Press, New York2015: 1667-1694Google Scholar In this patient, there was bilateral eyelid and lower leg lymphedema as well as intraocular fluid retention, suggesting a diffuse systemic process. The intestinal lymphangiectasia likely led to PLE and the hypoalbuminemic state, thereby decreasing intravascular osmotic pressure and causing third spacing of fluid. This transudative fluid shift involved the skin of the eyelids and legs as well as the subretinal and choroidal spaces. There are only a few case reports documenting serous retinal detachment in PLE.2Mafi M Khodabandeh A Riazi-Esfahani H Mirghorbani M. Bilateral central serous retinal detachment in protein-losing enteropathy.Korean J Ophthalmol. 2019; 33: 577-578Crossref PubMed Google Scholar, 3Venkatramani J Gottlieb JL Thomassen TS Multari A. Bilateral serous retinal detachment due to protein-losing enteropathy.Arch Ophthalmol. 2004; 122: 1067-1070Crossref PubMed Scopus (11) Google Scholar, 4Wong AHY Li STL Ho CK ou PF Bilateral serous macular elevation associated with lupus protein-losing enteropathy.J Ophthalmic Vis Res. 2017; 12: 245-246PubMed Google Scholar Mafi et al.2Mafi M Khodabandeh A Riazi-Esfahani H Mirghorbani M. Bilateral central serous retinal detachment in protein-losing enteropathy.Korean J Ophthalmol. 2019; 33: 577-578Crossref PubMed Google Scholar observed serous retinal detachment in a patient with idiopathic PLE. Venkatramani et al.3Venkatramani J Gottlieb JL Thomassen TS Multari A. Bilateral serous retinal detachment due to protein-losing enteropathy.Arch Ophthalmol. 2004; 122: 1067-1070Crossref PubMed Scopus (11) Google Scholar similarly observed serous retinal detachment in a patient with idiopathic PLE exacerbated by concomitant viral infection. Wong et al.4Wong AHY Li STL Ho CK ou PF Bilateral serous macular elevation associated with lupus protein-losing enteropathy.J Ophthalmic Vis Res. 2017; 12: 245-246PubMed Google Scholar observed bilateral serous retinal detachment in patients with PLE secondary to systemic lupus erythematosus. Each case showed partial or full resolution of the retinal detachment following corticosteroid delivery. To our knowledge, our case of choroidal effusion and serous retinal detachment in a patient with PLE from intestinal lymphangiectasia is unique. The management of PLE-related choroid effusion typically involves systemic corticosteroids and specific focus on dietary modification with a high-protein, low-fat diet and occasionally cyclophosphamide (100 mg/day) or azathioprine (100 mg/day) immune modulation.2Mafi M Khodabandeh A Riazi-Esfahani H Mirghorbani M. Bilateral central serous retinal detachment in protein-losing enteropathy.Korean J Ophthalmol. 2019; 33: 577-578Crossref PubMed Google Scholar, 3Venkatramani J Gottlieb JL Thomassen TS Multari A. Bilateral serous retinal detachment due to protein-losing enteropathy.Arch Ophthalmol. 2004; 122: 1067-1070Crossref PubMed Scopus (11) Google Scholar, 4Wong AHY Li STL Ho CK ou PF Bilateral serous macular elevation associated with lupus protein-losing enteropathy.J Ophthalmic Vis Res. 2017; 12: 245-246PubMed Google Scholar Mafi et al.2Mafi M Khodabandeh A Riazi-Esfahani H Mirghorbani M. Bilateral central serous retinal detachment in protein-losing enteropathy.Korean J Ophthalmol. 2019; 33: 577-578Crossref PubMed Google Scholar observed visual improvement from counting fingers to 20/40 OD and 20/100 to 20/25 OS following oral prednisolone (30 mg/day) and a high-protein, low-fat diet. Venkatramani et al.3Venkatramani J Gottlieb JL Thomassen TS Multari A. Bilateral serous retinal detachment due to protein-losing enteropathy.Arch Ophthalmol. 2004; 122: 1067-1070Crossref PubMed Scopus (11) Google Scholar observed visual acuity improvement from 20/40 to 20/25 following an increase of methylprednisolone to 30 mg/day from the baseline dose of 10 mg every other day. Although response can vary relative to the severity and duration of the disease, corticosteroids remain a potential therapy for affected patients. However, primary intestinal lymphangiectasia, the cause of PLE in our patient, is often targeted with surgical intervention rather than corticosteroids alone, especially when imaging studies help to localize the area of affected bowel.5Kwon Y Kim MJ. The update of treatment for primary intestinal lymphangiectasia.Pediatr Gastroenterol Hepatol Nutr. 2021; 24: 413-422Crossref PubMed Scopus (3) Google Scholar These therapies include surgical resection of the affected bowel or therapeutic lymphatic embolization.5Kwon Y Kim MJ. The update of treatment for primary intestinal lymphangiectasia.Pediatr Gastroenterol Hepatol Nutr. 2021; 24: 413-422Crossref PubMed Scopus (3) Google Scholar In this patient, these surgical interventions were not pursued because the intestinal lymphangiectasia was diffuse rather than focal, so surgical correction was not indicated. It is possible that medical therapy was insufficient to correct the hypoalbuminemia in this patient and that the slow rate of resolution of albumin levels over the following 12 months prevented complete resorption of the fluid in the choroidal and retinal spaces. In cases of serous retinal detachment secondary to PLE from other causes (e.g., idiopathic and SLE related), resolution occurred following 1–3 months of medical therapy.2Mafi M Khodabandeh A Riazi-Esfahani H Mirghorbani M. Bilateral central serous retinal detachment in protein-losing enteropathy.Korean J Ophthalmol. 2019; 33: 577-578Crossref PubMed Google Scholar, 3Venkatramani J Gottlieb JL Thomassen TS Multari A. Bilateral serous retinal detachment due to protein-losing enteropathy.Arch Ophthalmol. 2004; 122: 1067-1070Crossref PubMed Scopus (11) Google Scholar, 4Wong AHY Li STL Ho CK ou PF Bilateral serous macular elevation associated with lupus protein-losing enteropathy.J Ophthalmic Vis Res. 2017; 12: 245-246PubMed Google Scholar In this patient, the intestinal lymphangiectasia causing her PLE was difficult to treat medically, and the patient could not pursue surgical intervention. Thus her albumin levels remained low for 12 months. It is possible, therefore, that the chronicity of the retinal detachment prevented complete resolution and healing even after partial resolution of her hypoalbuminemic state, thus causing her poor visual outcome. The authors have no proprietary or commercial interest in any materials discussed in this correspondence. Support provided in part by the Eye Tumor Research Foundation, Philadelphia, Pa. The funders had no role in the design and conduct of the study; in the collection, analysis, and interpretation of the data; or in the preparation, review, or approval of the manuscript. Carol L. Shields, MD, has had full access to all the data in the study and takes responsibility for the integrity of the data.