History/Chief Complaint

  • Thirty five years ago, a 3-year old girl presented for the first time and was diagnosed with subluxated lenses OU and myopic fundi. A high arched palate, pectus excavatum, and a history of heart disease were noted which all supported a diagnosis of Marian syndrome. At least yearly exams over the next 3 decades (several times prompted by various types of eye and head trauma) lead to the sequential diagnosis of dislocated lenses into the inferior vitreous and several retinal detachments in both eyes. After being compliant with exam follow-ups for 3 decades, the patient was lost to follow-up for the last 5 years. She finally presented recently and complained of reduced vision in the right eye. She admitted that she stopped taking her glaucoma drops several years earlier. A review of the thick previous records revealed previous trauma to the eyes with elbows, various balls and even the possibility of a metallic foreign body from an air hammer.

Clinical Findings

  • BCVA 20/200 OD and 20/40 OS. Goldmann IOPs 18 OD and 26 OS.
  • Slit Lamp: Folds in Descemet’s membrane and marked corneal edema were observed OD which prevented an adequate view of the fundus via all forms of traditional ophthalmoscopy in the right eye.

Panoramic Ophthalmoscopy

  • Panoramic viewing revealed a hazy view OD but did reveal the lens In the inferior vitreal cavity. The left eye was observable with BIO and imaged with optos® (see page 5).

B-scan

  • Retinal detachment RE with intra-retinal macrocyst (confirming a long standing RD).
  • The crystalline lens (which was never removed surgically) was in the inferior vitreal cavity (because the lens is heavier than vitreous).
  • B-scan was also able to rule out an embedded metallic foreign body.

Diagnosis

  • Long standing retinal detachment behind a decompensated cornea OD.
  • Open angle glaucoma that has progressed OU.

Disposition and Comments

  • Control of the lOP in the left eye is crucial and the patient is now on maximum medical therapy.
  • Retinal detachment surgery was recommended and performed in the OD.
  • A penetrating keratoplasty will also be necessary in order for any improved vision In the right eye to result but the prognosis for VA improvement is very poor, primarily because of the long standing RD.