硝子体手術により網膜血管腫を切除したvon Hippel Lindau病の1例

A case of von Hippel Lindau disease with retinal hemangioma resected by vitrectomy

要約　目的：網膜剝離を伴うvon Hippel Lindau（VHL）病に対し，硝子体手術にて網膜血管腫切除を施行した症例の報告。

症例：22歳，男性。両眼に網膜血管腫，右眼に漿液性網膜剝離を認めた。

所見：治療開始時の矯正視力は右0.6，左1.5であった。左眼は周辺部血管腫に網膜光凝固術を施行した。右眼は3視神経乳頭径程度の大型血管腫であり，網膜光凝固術および網膜冷凍凝固術を施行したが，術後の滲出性変化により網膜剝離は難治であった。また，2回目の網膜冷凍凝固術および網膜下液排液時には医原性網膜裂孔を生じた。その後硝子体手術を施行したが，血管腫近傍にも医原性裂孔を生じたうえに，術後の滲出性変化も強く，裂孔閉鎖は得られなかった。再手術時に網膜血管腫を含めた網膜切除を施行し，その後に網膜復位を得た。

結論：滲出性変化が制御不能であるVHL病の網膜血管腫に対して，血管腫切除が有効であった。

Abstract　Purpose：To report a case of retinal capillary hemangioblastoma（RCH） managed with vitrectomy in a patient with von Hippel Lindau（VHL） disease and retinal detachment.

Case：A 22-year-old male presented with RCH in both eyes and serous retinal detachment in the right eye.

Findings：Corrected visual acuity was 0.6 in the right and 1.5 in the left eye at the start of treatment. Retinal photocoagulation of the left eye for peripheral hemangioma was performed. The right eye had a large RCH with an approximate disc diameter of 3. The patient underwent retinal photocoagulation and retinal cryotherapy；however, the retinal detachment was refractory due to postoperative exudative changes. During the second retinal cryotherapy and external drainage of the subretinal fluid, an iatrogenic retinal tear formed. Subsequent vitrectomy was performed；however, a further retinal tear formed beside the RCH, and postoperative exudative changes were severe, preventing the closure of the tear. The patient underwent a reoperation to resect the retina, including the RCH, and the retina was reattached.

Conclusion：Endoresection of RCH was effective for RCH in VHL disease with uncontrolled exudative changes.