Skip to main content

Advertisement

SpringerLink
Log in

A clinicopathologic case report on macular hole associated with von Hippel–Lindau disease: a novel ultrastructural finding of wormlike, wavy tangles of filaments

  • Short Communication
  • Published:
Graefe's Archive for Clinical and Experimental Ophthalmology Aims and scope Submit manuscript

    We’re sorry, something doesn't seem to be working properly.

    Please try refreshing the page. If that doesn't work, please contact support so we can address the problem.

Abstract

Background

We describe a novel ultrastructural finding observed in an epiretinal membrane excised from a patient with von Hippel–Lindau disease.

Methods

This interventional case report presents a 45-year-old woman who noted decreased vision in her right eye and was referred for treatment of a macular hole secondary to von Hippel–Lindau disease. Blindness had occurred previously in the left eye because of exudative retinal detachment. Funduscopic examination revealed a macular hole associated with a delicate epiretinal membrane, multiple retinal angiomas, and multiple old laser scars. Vitrectomy was performed in her right eye. The epiretinal membrane was peeled off and processed for light and electron microscopy.

Results

Postoperatively the macular hole was successfully closed, and vision recovered to 20/20 from 20/40. In the excised epiretinal membrane, flattened glial cells and extracellular matrix containing fibrous elements formed a multilayered pattern. Ultrastructurally, the fibrous elements appeared as wormlike, wavy tangles of filaments (WWTF) with an orientation parallel to the surface of the epiretinal membrane. Just above the WWTF, flattened glial cells frequently extended thin cytoplasmic processes with pinocytotic vesicles.

Conclusion

The ultrastructure suggested that retinal glial cells had induced a remodeling of the collagenous stroma in the posterior vitreous cortex, resulting in tangential macular traction.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1A–D
Fig. 2A–D
Fig. 3A–E
Fig. 4A, B

References

  1. Ghadially F (1997) Ultrastructural pathology of the cell and matrix. Butterworth-Heinemann, Boston

  2. Ishida, S, Yamazaki, K, Shinoda, K, et al (2000) Macular hole retinal detachment in highly myopic eyes: ultrastructure of surgically removed epiretinal membrane and clinicopathologic correlation. Retina 20:176–183

    CAS  PubMed  Google Scholar 

  3. Kinouchi R, Hirokawa H, Miyokawa N, et al (2001) Immunohistochemical study of idiopathic and secondary epiretinal membrane. Nippon Ganka Gakkai Zasshi 105:673–681

    PubMed  Google Scholar 

  4. Latha B, Ramakrishnan M, Jayaraman V, Babu M (1999) Physicochemical properties of extracellular matrix proteins in post-burn human granulation tissue. Comp Biochem Physiol B Biochem Mol Biol 124:241–249

    Article  PubMed  Google Scholar 

  5. Loewenstein J (1995) Bilateral macular holes in von Hippel–Lindau disease. Arch Ophthalmol 113:143–144

    PubMed  Google Scholar 

  6. Machemer R, Williams JM Sr (1988) Pathogenesis and therapy of traction detachment in various retinal vascular diseases. Am J Ophthalmol 105:170–181

    PubMed  Google Scholar 

  7. Marshall GE, Konstas AGP, Reid GG, et al (1994) Collagens in the aged human macula. Graefes Arch Clin Exp Ophthalmol 232:133–140

    PubMed  Google Scholar 

  8. McDonald HR, Schatz H, Johnson RN, et al (1996) Vitrectomy in eyes with peripheral retinal angioma associated with traction macular detachment. Ophthalmology 103:329–335

    PubMed  Google Scholar 

  9. Mottow-Lippa L, Tso MOM, Peyman GA, Chejfec G (1983) Von Hippel angiomatosis. A light, electron microscopic, and immunoperoxidase characterization. Ophthalmology 90:848–855

    PubMed  Google Scholar 

  10. Oda Y, Kawahara E, Minamoto T, et al (1988) Immunohistochemical studies on the tissue localization of collagen types I, III, IV, V and VI in schwannomas. Correlation with ultrastructural features of the extracellular matrix. Virchows Arch B Cell Pathol Incl Mol Pathol 56:153–163

    PubMed  Google Scholar 

  11. Ohh M, Yauch RL, Lonergan KM, et al (1998) The von Hippel–Lindau tumor suppressor protein is required for proper assembly of an extracellular fibronectin matrix. Mol Cell 1:959–968

    PubMed  Google Scholar 

  12. Okada M, Ogino N, Matsumura M, et al (1995) Histological and immunohistochemical study of idiopathic epiretinal membrane. Ophthalmic Surg 27:118–128

    CAS  Google Scholar 

  13. Schreck E (1962) Phakomatosen und Sehorgan. Bericht über die Tagung bayerischer und österreicher Augenärzte, Vienna

  14. Schreck E (1977) Differentiakdiagnose in der Ophthalmologie. Enke, Stuttgart

  15. Sebag J, Balazs EA (1989) Morphology and ultrastructure of human vitreous fibers. Invest Ophthalmol Vis Sci 30:1867–1871

    PubMed  Google Scholar 

  16. Shinoda K, Hirakata A, Hida T, et al (2000) Ultrastructural and immunohistochemical findings in five patients with vitreomacular traction syndrome. Retina 20:289–293

    PubMed  Google Scholar 

  17. Swinscoe JC, Carlson EC (1995) Type II collagen is a major component of bovine retinal microvessel extracellular matrix. Microcirculation 2:253–265

    PubMed  Google Scholar 

  18. Von Hippel E (1904) Über eine sehr seltene erkrankung der Netzhaut. Klinische Beobachtungen. Graefes Arch Ophthalmol 59:83

    Google Scholar 

  19. Wing GL, Weiter JJ, Kelly PJ, Albert DM, Gonder JR (1981) Von Hippel–Lindau disease: angiomatosis of the retina and central nervous system. Ophthalmology 88:1311–1314

    PubMed  Google Scholar 

  20. Wykoff CC, Pugh CW, Maxwell PH, et al (2000) Identification of novel hypoxia dependent and independent target genes of the von Hippel–Lindau (VHL) tumor suppressor by mRNA differential expression profiling. Oncogene 19:6297–6305

    CAS  PubMed  Google Scholar 

Download references

Acknowledgements

The authors would like to acknowledge the technical assistance of T. Nagai and K. Fujita with respect to electron microscopy. The authors received no financial support.

Author information

Authors and Affiliations

  1. Department of Ophthalmology, Keio University, 35 Shinanomachi, Shinjuku-ku, 160-8582, Tokyo, Japan

    Makoto Inoue, Kei Shinoda, Susumu Ishida, Hajime Shinoda, Kousuke Noda & Yoshihisa Oguchi

  2. Department of Pathology, Saiseikai Central Hospital, Tokyo, Japan

    Kazuto Yamazaki

Authors
  1. Makoto Inoue
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Kazuto Yamazaki
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Kei Shinoda
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Susumu Ishida
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Hajime Shinoda
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Kousuke Noda
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Yoshihisa Oguchi
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Makoto Inoue.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Inoue, M., Yamazaki, K., Shinoda, K. et al. A clinicopathologic case report on macular hole associated with von Hippel–Lindau disease: a novel ultrastructural finding of wormlike, wavy tangles of filaments. Graefe's Arch Clin Exp Ophthalmol 242, 881–886 (2004). https://doi.org/10.1007/s00417-004-0908-9

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00417-004-0908-9

Keywords

Search

Navigation