Von Hippel–Lindau disease with concomitant Hodgkin’s disease and congenital hypertrophy of the retinal pigment epithelium

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Abstract

The article presents a rare case of combination of von Hippel–Lindau disease and Hodgkin’s disease. The disease began with neurological symptoms with gradual progression over the next 3 years. The diagnosis of von Hippel–Lindau disease was made after MRI of brain and spinal cord, abdominal MICTs, and detection of brain stem and spinal cord tumors, multiple pancreatic cysts. We performed resection trepanation of the posterior cranial fossa and microsurgical total removal of hemangioblastoma of the medulla oblongata. After 1.5 years the patient is diagnosed with Hodgkin’s disease and several courses of chemotherapy are carried out, reaching full remission, confirmed by PET with CT. 14 months later, the patient consulted an ophthalmologist due to visual impairment and floating opacities in her left eye. The ophthalmologic examination for the first time revealed multiple bilateral retinal hemangiomas and vitreal hemorrhages from tractional retinal tears caused by posterior hyaloid detachment and unrelated to hemangiomas in the left eye. The barrier laser coagulation of the left eye retinal tears was performed, and the observation tactics was adopted.

About the authors

Aleksandr V. Rakitsky

Diagnostic Center Nо. 7 (Ophthalmological) for Adults and Children

Author for correspondence.
Email: avr72@list.ru

Head of the Laser Department

Russian Federation, Saint Petersburg

Aida A. Shakhnazarova

Diagnostic Center Nо. 7 (Ophthalmological) for Adults and Children

Email: aida66@bk.ru

Candidate of Medical Sciences, Head of the Department of Retinal Pathology

Russian Federation, Saint Petersburg

Marina A. Shcherba

Diagnostic Center Nо. 7 (Ophthalmological) for Adults and Children

Email: sherba.v@yandex.ru

Ophthalmic Surgeon, laser department

Russian Federation, Saint Petersburg

References

  1. D’hondt R, Thomas J, Van Oosterom AT, Dewolf-Peeters C. Hodgkin’s disease in a patient with von Hippel – Lindau disease. A case report. Acta Clin Belg. 2000;55(5):276-278. https://doi.org/10.1080/17843286.2000.11754310.
  2. Lou LH, Shen H, Lin J, et al. T-cell lymphoma with von Hippel–Lindau disease: a rare case report and review of literature. Int J Clin Exp Pathol. 2015;8(5):5837-5843.
  3. Лазерная хирургия сосудистой патологии глазного дна / Под ред. А.Г. Щуко. – М.: Офтальмология, 2014. – 250 с. [Lazernaya khirurgiya sosudistoy patologii glaznogo dna. Ed. by A.G. Shchuko. Moscow: Oftal’mologiya; 2014. 250 р. (In Russ.)]
  4. Wong WT, Liang KJ, Hammel K, et al. Intravitreal ranibizumab therapy for retinal capillary hemangioblastoma related to von Hippel – Lindau disease. Ophthalmology. 2008b;(115):1957-1964. https://doi.org/10.1016/j.ophtha.2008.04.033.

Supplementary files

Supplementary Files
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1. JATS XML
2. Fig. 1. RE. Angiomatosis nodules with dilated afferent and efferent blood vessels extending from the optic disc

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3. Fig. 2. RE. Glial proliferation on the surface of hemangiomas

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4. Fig. 3. LE. Peripheral retinal tear with an overlying operculum

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5. Fig. 4. LE. Congenital hypertrophy of the RPE

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6. Fig. 5. LE. Angiomatosis nodules with dilated afferent and efferent blood vessels extending from the optic disc

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Copyright (c) 2020 Rakitsky A.V., Shakhnazarova A.A., Shcherba M.A.

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This work is licensed under a Creative Commons Attribution 4.0 International License.
 


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