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Congenital anophthalmia


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Congenital anophthalmia

  1. 1. Congenital Anophthalmia: Current concepts in management Current Opinion in Ophthalmology 2011,22:380384
  2. 2. Introduction  Congenital Anophthalmia – a rare congenital eye anomaly due to deficiency in development of primary optic vesicle. There is no detectable ocular tissue. Associated with microblepharon,short conjunctival sac, absence of extraocular muscles. Replaced completely by a cyst.
  3. 3. Purpose of review  The introduction of hydrogel socket & orbital expanders has modified approach towards rehabilitation of congenital anophthalmia.
  4. 4. Recent advances Hydrogel socket expander as an outpatient procedure.  Increased orbital volume confirmed by CT ,MRI.  Inflatable orbital tissue expander new design 
  5. 5. Clinical evaluation  Associate findings – Coloboma,dermoids,sclerocornea,glaucoma,le ns & optic nerve abnormalities. -Canalicular stenosis Systemic abnormalitiesAbnormalities of ears,palate,lower face Cardiac,renal & genital anomalies,brain abnormalities - Anophthalmia-plus syndrome. CT Ultrasonography
  6. 6. Treatment  Goals: -Simultaneous expansion of lids,soft tissues,orbital bones /replace lost volume -maintain structure of orbit -impart motility to prosthesis
  7. 7. 1.Positioning of progressively enlarging static acrylic conformers asap after birth - Orbital cyst –dynamic expander like conformer Drainage or Excision – Rapid growth - Uncomfortable to wear MRI to exclude connection to brain
  8. 8. Orbital implant Spherical implants:  Inert material:  glass,silicone,methylmethacrylate  Biointegrated: Hydroxyapatite, porous polyethylene
  9. 9. Inert spherical implants Advantages  Provide comfort and low rates of extrusion.  Cost-effective choice in patients. Disadvantages  decreased motility and implant migration. Buried motility implants  anterior surface projections push the overlying prosthesis with direct force and can improve prosthetic motility.  may pinch the conjunctiva between the implant and the prosthesis - painful
  10. 10. Hydroxyapatite and porous polyethylene implants allow for drilling and placement of a peg to integrate the prosthesis directly with the moving implant.  Pegging is usually carried out 6-12 months after enucleation. Pegged porous implants offer excellent motility, 
  11. 11.  Locations for implants -within the Tenon capsule /behind the posteri or Tenon capsule in the muscle cone.  Spheres may be covered with other materials such as  sclera (homologous or cadaveric) or autogenous fascia,  Secure closure of Tenon fasci a over the anterior surface of an anophthalmic implant is an important barrier to later extrusion.
  12. 12. Types Hard spherical implant  Inflatable soft tissue expander  Hydrogel osmotic expander 
  13. 13. Hard silicone spheres - Need of series of surgeries Multiple general anaesthesias Repeated trauma to soft tissues
  14. 14. Inflatable soft tissue expanders - Better orbital bone stimulation & socket enlargement. Difficult to control direction ,maintain expansion pressure. Chance of displacing conformer,extrusion
  15. 15. Hydrogel expander implant To stimulate growth of conjunctival sac & eyelids followed by serial implantation for Orbital volume with temporary tarsorraphy.  Methylmethacrylate & Nvinylpyrrolidone materials.  Small Soft tissue incision – quick surgery , recovery 
  16. 16. Injectable pellet expanders through trocar tru skin at inferior orbital rim to deep orbit.  Safe & minimally invasive technique  Easy to insert , biocompatible.  Migration & extrusion. 
  17. 17. Positioning with cyanoacrylate glue - No suture related complications - Avoids multiple general anaesthesias - Outpatient procedure with topical anaesthesia. 
  18. 18. Dermis –fat grafts Outcomes: -Good orbital volume ,adequate fornices -No excessive growth / need of surgery -Allows lid & socket expansion  Problems: -second surgical site,unpleasant scar -delay in healing,chronic discharge 
  19. 19.  A study on evaluation of an integrated orbital tissue expander in congenital anophthalmos . Am J Ophthalmol 2011 - An inflatable silicone globe sliding on titanium T- plate secured to lateral orbital rim with screws. Inflating with transconjunctival inj of normal saline ,30 G needle. -
  20. 20. Outcomes: - ease of insertion - Absence of displacement - Uniform pressure - Reduced trauma 
  21. 21. Guidelines for enucleation  A functionally and aesthetically acceptable anophthalmic socket must have following – an orbital implant of sufficient volume centered within the orbit  a socket lined with conjunctiva or mucous membrane with fornices deep enough to hold a prosthesis.   eyelids with normal appearance and adequate tone to support a prosthesis good transmission of motility from the implant to the overlying prosthesis  a comfortable ocular prosthesis that looks similar to the normal eye 
  22. 22. Steps of Enucleation with orbital implant
  23. 23. Removal of contents
  24. 24. Advantages of Evisceration in orbital implant Less disruption of orbital anatomy.  Good motility of prosthesis  Lower rate of migration,extrusion,reoperation. 
  25. 25. Anophthalmic Socket Complications and Treatment Deep superior sulcus  Contracture of fornices  Exposure & extrusion of implant  Contracted socket  Anophthalmic ectropion  Anophthalmic ptosis  Lash margin entropion  Cosmetic Optics 