|Year : 2015 | Volume
| Issue : 1 | Page : 117-120
Prosthetic management of congenital anophthalmia-microphthalmia patient
Himanshi Aggarwal, Pradeep Kumar, Raghuwar D Singh
Department of Prosthodontics, Faculty of Dental Sciences, KGMU, Lucknow, Uttar Pradesh, India
|Date of Web Publication||13-Apr-2015|
Dr. Himanshi Aggarwal
Department of Prosthodontics, KGMU, Lucknow - 226 003, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
Congenital anophthalmia and microphthalmia are rare developmental defects of the globe that cause deficient orbitofacial growth and impaired visual capability. Anophthalmia whether congenital or acquired is not just a question of cosmesis. It has many ramifications such as monocular status, loss of facial esthetics and psychological challenges for a growing child. The management of such a patient requires the coordinated involvement of a multidisciplinary team of health care professionals, including pediatrician, pediatric ophthalmologist, geneticist, genetic counselor, oculoplasty surgeon, and prosthetist. This article focuses on the rehabilitation of an adult female patient with congenital anophthalmia who was successfully treated with progressive expansion therapy with custom conformer followed by custom ocular prosthesis.
Keywords: Congenital anophthalmos, expansion therapy, micro-orbitism, microphthalmos, prosthesis
|How to cite this article:|
Aggarwal H, Kumar P, Singh RD. Prosthetic management of congenital anophthalmia-microphthalmia patient. Arch Med Health Sci 2015;3:117-20
| Introduction|| |
Congenital anophthalmia, a rare developmental defect of the globe, is the complete absence of the eye due to deficient formation of the primary optic vesicle during early phases of gestation. It is often seen in conjunction with microphthalmia. The prevalence of anophthalmia and microphthalmia has been estimated as 0.2-3.0/10,000 births.  Generally, severe congenital unilateral microphthalmia or anophthalmia produces bony orbital hypoplasia, microblepharon, and facial asymmetry due to hemifacial microsomia. In bilateral cases, the typical features are sunken orbits and midfacial hypoplasia.
The diagnosis of anophthalmia or microphthalmia is clinical, obtained by ophthalmological examination demonstrating the complete absence of the globe or decreased ocular size relative to the contralateral eye. Once a diagnosis has been established, both ocular and systemic imaging tests (ultrasound, computed tomography [CT], and magnetic resonance imaging) should be performed to rule out neurological, renal, cardiac, and other associations. 
Patients with severe microphthalmia or anophthalmia, without potential vision, undergo early orbital rehabilitation with "artificial" stimulation of orbitofacial growth. Several techniques and materials (endogenous and exogenous) are used to expand orbital volume: Serial acrylic conformers for conjunctival sac, conventional spherical orbital implants, mucous grafts, dermal fat, bone and muscle grafts, inflatable balloon devices, and hemispherical and spherical self-inflating hydrophilic (hydrogel) expanders. ,,,,,
Orbital rehabilitation in congenital anophthalmia is a frustrating anatomical challenge for both parents and physicians. Early intervention undoubtedly makes a huge difference to the overall development of the child and the emotional well-being of the family. However, unfortunately not all cases are able to seek treatment in early childhood, as reported in this case report.
| Case Report|| |
A 21-year-old female presented with the complaint of unesthetic facial appearance due to the absence of the right eye [Figure 1]a]. She gave a history that her right eye was congenitally missing. Patient had a very low self-confidence and her interviewing gave an indication of psychological distress, as a result of her appearance.
|Figure 1: (a) Preoperative view showing congenital anophthalmia of right eye. (b) Preoperative view showing no ocular remnants in right eye|
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Clinical examination revealed a typical acorn shaped socket with no any remnants of ocular tissues and the eyelids were significantly smaller both horizontally and vertically compared with the normal eye [Figure 1]b]. Furthermore, the distance between the lid lashes and the brow was proportionally reduced and palpebral fissure was phimoric and contracted. Thorough ocular evaluation was done and it was diagnosed clinically as a case of unilateral congenital anophthalmia of the right eye with associated low vision and nystagmus of the left eye.
The CT scan of the orbits (axial and coronal views without contrast) was conducted to confirm the diagnosis. The CT scan revealed the condition to be microphthalmia with colobomatous cyst of the right eye and posterior staphyloma of the left eye [Figure 2].
|Figure 2: (a) Computed tomography (CT) scan-coronal view showing microphthalmia with colobomatous cyst of right eye. (b) CT scan-axial view showing microphthalmia with colobomatous cyst of right eye|
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After obtaining ophthalmologic consultation, the treatment plan was formulated. Patient refused to undergo any invasive surgical procedures for correction of her contracted socket. Hence, it was decided to initially fabricate a custom conformer for the patient until adequate socket size is attained, later followed by custom ocular prosthesis. Initially, conformer was given to the patient, for 3 weeks [Figure 3] and when adequate socket size was attained; the patient was ready for her custom ocular prosthesis.
|Figure 3: (a) Cast showing intra-orbital topography of right eye. (b) Conformer|
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The procedure was initiated by fabricating a custom impression tray using autopolymerizing polymethylmethacrylate (PMMA) (Trevalon, Dentsply India Pvt., Ltd., Gurgaon, India) in its dough stage and molding it by adapting on the thumb pad to form an ocular shaped tray, similar to one described by Allen and Webster.  This tray was then checked in patient's eye socket and adjusted by trimming in areas of overextension and impingement. Petroleum jelly was applied to the eyebrows for the easy removal of the impression when it sets. A syringe without the needle portion was attached to the center of the impression tray to serve as a handle for the tray and through which the impression material can be easily injected into the defect area.
A thin mix of ophthalmic grade alginate (Opthalmicmoldite, Milton Roy Co., Sarasota Florida, USA) was loaded into the syringe and then, injected in the socket. The patient was instructed to keep her head upright and to move her normal eye in all directions, to allow the alginate to flow into all areas of the defect. Impression [Figure 4]a] was examined for accuracy and a two-piece dental stone (Kalabhai Karson Pvt., Ltd., Mumbai, India) cast [Figure 4]b] was poured from the impression. The wax pattern was formed on the cast by using carving wax mixed with sticky wax, and tried in the patient's eye and checked for accuracy in terms of retention, proper extension in all directions, scleral contour, and convexity. The iris was obtained from the stock eye that has exactly matching size and color of the iris of contralateral eye. This iris was placed on the wax pattern using the graph grid method. 
|Figure 4: (a) Impression of the ocular defect. (b) Cast obtained after double-pour technique. (c) Wax pattern with iris button. (d) Intaglio surface view of wax pattern|
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The final altered wax pattern with iris button was evaluated in the patient's socket [Figure 5]a]. After making necessary modifications, the wax pattern was invested, flasked [Figure 5]b] and de-waxing was done [Figure 5]c]. The next step was to reproduce scleral shade of the normal eye. For this, shade tabs were prepared by mixing and matching different shades and proportions of tooth-colored acrylic (SC 10, Pyrax, Roorkee, India) until the color of sclera of the other eye was replicated. To simulate the conjunctival vessel markings of the normal eye, veins were separated from the veined heat cured PMMA (Trevalon, Dentsply India Pvt., Ltd., Gurgaon, India) and they were incorporated in the dough of the determined acrylic shade, followed by routine curing, finishing, and polishing.
|Figure 5: (a) Final wax pattern try-in. (b) Invested wax pattern. (c) Dewaxed mold|
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The properly finished and polished prosthesis was inserted in the socket [Figure 6] after disinfection and lubrication with an ophthalmic lubricant (Ecotears, Intas Pharmaceuticals Ltd., Ahmedabad, India). Instructions were given to the patient regarding the proper handling, insertion, removal and maintenance of the prosthesis. Patient was recalled after 24 h, 1 week, 1 month and 3 months, later followed by 6 monthly recall appointments. On 6 months recall appointment, further clinical ophthalmic examination was performed and no complications with regard to health of underlying residual ocular tissues were found. The patient reported that she had not encountered any problems and she was happy with the results. The prosthesis was in good shape, and there was no need for any repairs. The patient was successfully rehabilitated with highly cosmetic custom made ocular prosthesis.
|Figure 6: Postoperative view with ocular prosthesis in lieu of missing right eye|
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| Discussion|| |
Congenital anophthalmos or microphthalmos presents the prosthodontist and ophthalmologist team with their greatest of prosthetic and reconstructive challenges Achieving appropriate cosmetic outcome is extremely difficult in late reported cases as the detrimental effects of loss of orbital volume and lack of orbital growth stimulation have already occurred. Various concepts of ocular prosthetic management for anophthalmia have been described with their own pros and con. For successful rehabilitation, patient specific treatment modality is the key to management. The concept of conservative prosthetic "expansion therapy" with conformers plays a significant role in rehabilitating such patients nonsurgically.
| Conclusion|| |
The management of congenital anophthalmia is a longterm commitment for the team. The conservative prosthetic "expansion therapy" with sequentially large size conformers is a simple and noninvasive approach for socket enlargement in congenital anophthalmia-mirophthalmia cases.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]