Clear lens extraction and intraocular lens implantation in a case of microspherophakia with secondary angle closure glaucoma

Phacoemulsi ﬁ cation with implantation of single-piece acrylic foldable intraocular lens (IOL) in a 19-year-old boy with microspherophakia, high myopia and angle closure glaucoma is described. The associated myopia and angle closure glaucoma was severely compromising the quality of life. Post-surgical visual recovery was 20/20 with sustained normal intraocular pressure. Management of such cases at times calls for innovations in current surgical technique. We report two cases of intraocular cysticercosis which showed a peculiar presentation of neovascular glaucoma which is hitherto unreported. Two young adults presented with symptoms of raised intraocular pressure due to neovascular glaucoma. On dilated fundus examination both were found to have dead intravitreal cysticercosis. The cysts were removed by a three-port vitrectomy and intracameral injection of bevacizumab was given to help in the regression of rubeosis. Trabeculectomy had to be combined in one case. The intraocular pressure returned to normal. No recurrence of rubeosis was seen even a ft er one year.

due to a small tectal hemorrhage secondary to a probable midbrain cavernoma. Few cases of isolated trochlear nerve palsy with midbrain hemorrhage or due to midbrain vascular malformation are reported in the literature. [1][2][3][4][5] Sürücü et al. very recently reported a patient of cavernoma with symptomatic fourth nerve palsy that was surgically treated. [6] Chen et al. reviewed the reported world literature of nine patients with midbrain tectal bleed and noted unique presentation of diplopia along with either contralateral tinnitus or sensory disturbance. The transient tinnitus observed in our patient was probably due to involvement of the inferior colliculus. [1] Axons of the trochlear nerve originate from the nucleus in the ventral periaqueductal grey at the level of the inferior colliculus. These axons indent the dorsal surface of the medial longitudinal fasiculus, curve dorsolaterally and caudally to reach the superior medullary velum to decussate with the nerve of the opposite side and exit from the brainstem [ Fig. 1 E]. The auditory aff erents from the cochlear nucleus in the medulla oblongata aft er traversing the superior olivary nucleus and nucleus of lateral lemniscus in the rostral pons, pass through the inferior colliculus and adjacent lateral lemniscus in the lower midbrain. The eff erents are bilateral but dominantly contralateral to the inferior colliculus. The auditory fi bers from the inferior colliculus traverse the medial geniculate body to reach the cortex in the temporal lobe. The combination of ocular and auditory symptoms emphasizes the neuroanatomical contiguity of the trochlear nerve pathway and the adjacent inferior colliculus [ Fig. 1 E].
In the present case, the associated audiological symptoms could be due to irritation of the auditory pathway traversing the ipsilateral inferior colliculus by the seeping blood products, altered calcium homeostasis and disinhibition of GABAergic transmission in the neurons and axons in the close vicinity. [7][8][9] 301_08 visual recovery was 20/20 with sustained normal intraocular pressure. Management of such cases at times calls for innovations in current surgical technique. Microspherophakia is an uncommon bilateral congenital abnormality of the crystalline lens. Defective development of the zonules results in their defi ciency, increased length, weakness and non-attachment of posterior zonules to the ciliary processes. This leads to formation of a small spherical lens which is devoid of any cortico-nuclear demarcation. The condition may be isolated, idiopathic and familial anomaly or it may be associated with systemic defects like Marfan's syndrome, Weil-Marchesani syndrome, hyperlysinemia and congenital rubella. Microspherophakia results in lenticular myopia and late development of lens dislocation, usually inferiorly. Phakic pupillary block glaucoma and high lenticular myopia are common complications. [1][2][3][4][5] Management of such cases at times calls for innovations in current surgical techniques as reported Vol. 58 No. 1 in this case report.

Case Report
A 19-year-old boy reported to our institution with complaints of poor vision in both eyes since early childhood. He had a history of recurrent att acks of blurring of vision, associated with headache, irritation and redness of eyes. On examination, the best-corrected visual acuity (BCVA) with contact lens was 20/60, N12 in right eye (RE) and 20/80, N18 in left eye (LE). Refractive error was -23.00 /-1.00 x 80˚ (RE) and -24.00/-1.00 x100˚(LE Considering compromised quality of life with reference to dependence on contact lenses or glasses, lack of confi dence in independent living, and good visual potential in eyes, clear lens extraction and IOL implantation was suggested in both eyes and

Surgical Procedure
The patient underwent diffi cult, uneventful phacoaspiration with IOL implantation under topical anesthesia (HB), through a 2.75-mm superior clear corneal tunnel and two corneal side  ports. Puncturing of the capsule and initiation of capsulorrhexis was diffi cult, as the anterior capsule was less elastic and relatively unsupported and cystitome pressure made an umbilicated depression in the anterior capsule with radiating folds. The lens was unstable and bimanual capsulorrhexis was done by supporting the capsulorhexis margin with an iris hook through the side port [ Fig. 5]. A capsulorhexis of size 4.5 mm (approximately) was made. Aft er hydroprocedure and phacoaspiration, a foldable IOL of + 15.50D (AcrySof ® SA60AT Alcon Lab. Fortworth. Tx, USA) was implanted directly into the bag. Tight wound security and deep AC were maintained critically during the procedure. The capsular bag was small, but somehow accommodated the intraocular lens. On the fi rst postoperative day, AC was found to be deep, quiet and with presence of iridodonesis. The IOL was well centered. The BCVA (Plano) at two weeks was 20/20.

Discussion
Faulty development of lens zonules during embryogenesis is believed to be the cause of microspherophakia which can cause pupillary block glaucoma. Miotics aggravate the condition by increasing the pupillary block and allowing further forward displacement of the crystalline lens. Cycloplegics are the treatment of choice. However, laser-iridotomy, trabeculectomy, [6] were found useful in relieving angle closure glaucoma in such patients. Management of associated high myopia is always a challenge. Clear lens extraction through anterior route, pars plana lensectomy with or without IOL implantation are the treatment options for myopia and glaucoma in microspherophakia. [7][8][9] Indications for lens extraction in such cases are cataract, corneolenticular touch, high myopia, intermitt ent pupillary block and secondary glaucoma. Literature search revealed only few case reports, where clear lens phacoemulsifi cation was done with appropriate IOL implantation, with excellent postoperative visual recovery. [10,11] In the present case, pupillary block glaucoma was successfully treated with Nd:YAG-Laser PI followed by refractive error correction through clear lens extraction with IOL implantation. During the surgery, the capsulorrhexis needed an innovation in the form of using with an iris hook [ Fig. 5] to stabilize the lens. The option of stabilizing the capsular bag with capsular tension ring (CTR) could not be explored by us, because of nonavailability of such a small CTR [7] and also with apprehension that it will further reduce the capsular bag volume. The acrylic foldable lens was well accommodated in the small capsular bag with the unfolded haptics being in touch with the edge of the optics. The IOL was well centered in spite of zonular instability, but clinically phacodonesis persisted in the postoperative period, without compromising the vision. A similar procedure has been described by Khokhar et al. [10] but in their case there was residual myopia and underestimated visual potential acuitymetry result.
In the present case there was no residual myopia, suggesting that the IOL power calculation by the optical biometer using SRK-II formula as appropriate.