Monocular elevation deficit with Pellucid Marginal Degeneration: A case report

Authors: Dr Jayesh Suresh Patil Dr Mangesh Dhobekar Dr Prasanna Aradhye Dr Amrita Ajani

Token No. 0414

IntroductionMonocular elevation deficiency

inability to elevate eye in all fields of gaze

presents with hypotropia of affected eye which is equal in all gazes

pseudoptosis with or without true ptosis

Aetiology includes supranuclear defects or primary superior rectus paresis or primary inferior rectus restriction or combination

Pellucid marginal degeneration

Peripheral corneal ectatic disorder

Band of thinning 1–2 mm in width, typically in the inferior cornea,

high irregular astigmatismCorneal topography shows

characteristic and typical appearances.

Purpose:To report and discuss a case of association between

monocular elevation deficiency(MED) and pellucid marginal degeneration(PMD) and its management.

Association of monocular elevation deficit with pellucid marginal degeneration is not reported before

History and examinationA 20 year old male came to OPD with drooping of

right upper eyelid since birth with poor vision.Examination: Right sided severe congenital ptosis with Marcus-

Gunn jaw winking and poor Bell's phenomenon was noted. Right hypotropia was present which was equal in all horizontal gazes.

Anterior segment and FundusRight Eye Left Eye

BCVA 6/45 6/6

Refraction -1.00DS/-8.50DCX60 Plano

Lid Severe Ptosis Normal

Conjuctiva Clear Clear

Cornea Clear Clear

Anterior chamber

Quiet, well formed Quiet, well formed

Iris Pattern Normal Normal

Pupil Round regular, brisk reaction

Round regular, brisk reaction

Lens Clear Clear

IOP 16 mm Hg 16 mm HgFundus WNL WNL

Squint Examination and plan

APCT/EOM

FDT Positive for Inferior rectusFGT negative for Superior Rectus

Plan :Stage I Inferior

rectus recessionStage II Knapp's

procedure with Foster augmentation

Pre-op 9 gaze photos

Status post OD Knapp's procedure

Topography and pachymetry was performed pre-operative and after 6 weeks

07-09-2016

20-07-2016

17-06-2016

Further plan

For Ptosis with Marcus-Gunn Jaw winkingPlanned for OD LPS muscle resection + Frontalis sling

procedure

For Pellucid Marginal degeneration : To wait and watch for now. If corneal topography is

stable, Nil intervention

If Progression noted, may plan for Collagen cross-linking or surgical inetervention

Discussion & Conclusion

Monocular elevation deficiency is commonly associated with congenital ptosis, Marcus-Gunn jaw winking phenomenon. Other ocular associations of MED were not much reported. Here we are presented a case of association between monocular elevation deficit with pellucid marginal degeneration. Further evaluation and follow up will be required to confirm association and aetiological correlation

Thank you...Shri Ganapati Netralaya Presentation