Couching of cataractous lens in microphthalmic eyes with irido-fundal coloboma: revisiting the historical technique.

PURPOSE: Cataract surgery in microphthalmic eyes is challenging due to anatomical restraints, hard bulky nucleus. This series aims to evaluate the safety and efficacy of couching of intraocular lens in irido-fundal coloboma with microphthalmos. SETTING: Tertiary care centre in South India. DESIGN: Retrospective non-comparative study in eyes with irido-fundal coloboma, corneal diameter < 7 mm and brown cataract. Visual acuity less than 6/60 in other eye. METHODS: Anterior chamber entry made, zonules broken and lens dislocated into the vitreous cavity in a controlled manner. Baseline Clinico-demographic details, corrected distance visual acuity (CDVA), Intra-ocular pressure (IOP), corneal diameter, axial length, lens status and post-surgery CDVA, IOP and complications recorded and followed up for atleast 6 months. RESULTS: Fifteen eyes of 15 subjects were evaluated with a mean age 49.4 ± 10.9 years. At baseline, mean IOP 14.5 ± 3.8 mmHg, mean axial length 19.3 ± 0.5 mm, mean corneal diameter was 6.5 ± 0.34 mm and CDVA 2 logMAR which improved to 1.5 logMAR at 3 months (p value 0.002). Transient spike in IOP in 33.3% subjects was medically managed with no significant difference in IOP (p > 0.05) at baseline (14.5 ± 3.8 mmHg), 3 months post-surgery (16 ± 2.8 mmHg) and 6 months post-surgery (14.9 ± 2.5 mmHg). One patient underwent re-couching. No other major complications were noted. CONCLUSION: Couching of cataractous lens is an effective and safe method in microphthalmic eyes with irido-fundal coloboma as last resort procedure, where no other surgical procedure may work. It provides an ambulatory gain of visual acuity in previously non-ambulatory subjects. Corneal measurements help in determining the subset of patients where couching offers viable option.

Reconstruction of an Eyelid Defect Associated with Congenital Coloboma in a 7-Month-Old Male Infant Using an Acellular Dermal Allograft.

BACKGROUND Congenital eyelid coloboma in children often faces complications such as keratitis, symblepharon, and amblyopia. Repairing defects involving at least 50% of the eyelid margin can be challenging. Acellular dermal allograft (ADA) has achieved excellent results as a substitute in adult eye plastic surgery, with minimal morbidity. This report describes a case of reconstruction of an eyelid defect in a 7-month-old male infant using an ADA. CASE REPORT A 7-month-old male infant was referred due to congenital eyelid coloboma in the left eye, which affected nearly one-half of the upper and lower eyelids medially, with more than 9 mm of lagophthalmos and lacrimal duct malformation inducing dacryocystitis. Under general anesthesia, A U-shaped silicone drainage tube was inserted in the nasolacrimal duct to ensure an unobstructed lacrimal duct. The symblepharon release, pseudopterygium excision, and medial canthus reconstruction were performed sequentially. Then, the upper eyelid defect was repaired through the advancement of the lateral segment of the eyelid, following lateral cantholysis. A trimmed ADA was placed as a substitute for the tarsal plate in the lower eyelid defect area and sutured with the free edge of the retractor. Finally, the lower and lateral skin orbicular muscle flap was advanced to cover the acellular dermis composite graft. The postoperative eyelid morphology was satisfactory. At 6 months after surgery, lower eyelid retraction gradually appeared. CONCLUSIONS ADA is presented as an effective solution for reconstructing significant eyelid defects of infants. However, the potential of postoperative eyelid retraction still deserves future research and refinement in surgical techniques.

Implantable Collamer Lens Subluxation in a Patient with Lenticular Coloboma: A Case Report.

BACKGROUND The efficacy and safety of the implantable collamer lens (ICL) in correcting high astigmatism have been previously reported. They are commonly used as an alternative to laser refractive surgery due to advantages such as leaving the cornea untouched, inducing fewer higher-order aberrations, resulting in better optical and visual quality, and it is a reversible procedure. We aim to present the outcome of ICL in managing anisometropia without cataract in an eye with unilateral lenticular coloboma. CASE REPORT A 27-year-old man with a Marfanoid body habitus was seeking refractive surgery for the correction of high astigmatism in the right eye. On presentation, the best corrected visual acuity was 20/30 and 20/20 in the right eye and left eye, respectively. Slit lamp examination indicated inferior lens coloboma extending from the 5 o'clock to the 7: 30 o'clock position in the right eye, after dilation of pupil. Following a complete refractive work-up, a toric ICL implantation was the presumed suitable surgery. Three weeks postoperatively, central vaulting was low, his ICL subluxated inferiorly, and the previously implanted temporal footplates were resting over the lenticular defect inferiorly. A high-resolution ultrasound biomicroscopy confirmed the presence of a ciliary body (CB) cyst at 9 o'clock position. Urgent explantation of the unstable ICL was performed. CONCLUSIONS This case report emphasizes the challenges and limitations associated with ICL implantation in patients with lenticular colobomas and coexisting CB cyst. Selecting smaller lenticular colobomas and avoiding direct interaction between the weak zonules area and the ICL haptics are important steps to ensure the stability of implanted lens.

Bilateral corneal dermoids associated with bilateral choroido-scleral colobomas in a cat: retinographic and optical coherence tomography study with surgical outcome and follow-up.

PURPOSE: To report a case of feline bilateral corneal dermoids, associated with unilateral iris coloboma and bilateral choroido-scleral colobomas in the same dorsolateral position, to describe retinographic and optical coherence tomography (OCT) characteristics, surgical outcome, and follow-up. ANIMAL STUDIED: A 9-month-old domestic shorthaired cat in which a full ophthalmoscopic examination was performed for evaluation of dermoids resulting in a diagnosis of associated iris coloboma in one eye and posterior colobomas in both eyes. PROCEDURES: Retinographies and OCT were performed under anesthesia to characterize the lesions of both fundi and allow surgical excision of the corneal dermoids. RESULTS: Ophthalmoscopy and retinographies revealed oval lesions in the dorsolateral fundi of both eyes. The lesions precisely mirrored their respective dermoids' (10-11 h OD and 1-2 h OS) clock positions, lacked a tapetum lucidum and choroidal vessels, and featured thin retinal vessels plunging to a posterior plane of the fundus. OCT crossline scans demonstrated preservation of retinal thickness and morphological layering in the fundic colobomas leading to the conclusion that the colobomas were purely choroido-scleral. The outcome of the surgical excision of the dermoids was satisfactory without hair recurrence and with acceptable corneal clarity making it possible to visualize the unilateral associated iris coloboma. Follow-ups did not reveal any fundic evolution nor retinal detachment. CONCLUSIONS: Retinographies and OCT made possible the characterization of choroido-scleral colobomas associated with corneal dermoids in this first reported case in a cat. We hypothesize that the recently described superior ocular sulcus might be the embryological link between these anomalies.

Severe Eyelid Malformation With Facial Clefting and Amniotic Bands.

Amniotic bands can lead to eyelid colobomas contiguous with facial clefts, resulting in severe and highly variable eyelid malformations. There is no known underlying genetic cause of amniotic band sequence. Here, the authors review the case of an infant born with large, 4-eyelid colobomatous defects in the setting of facial clefts, amniotic bands and an underlying SMOC1 mutation, which has not previously been linked to amniotic band sequence or eyelid colobomas. Reconstructive technique and the postoperative course are described, and underlying etiologic theories of amniotic band sequence are reviewed and expanded upon. Although amblyopia prevention in this patient with poor visual potential was not a consideration, the goals of improving the patient's ocular surface and maintaining eye contact were achieved.

Conforming to the anatomy and not the standard: A technique of eccentric capsulorrhexis and intraocular lens haptic amputation for eyes with iris coloboma.

PURPOSE: To describe a novel technique for cataract surgery in patients with iris coloboma. METHODS: The technique involves 1) creation of an inferiorly displaced capsulorrhexis and 2) amputation of one intraocular lens (IOL) haptic, thus allowing for controlled IOL decentration in the direction of an inferior iris defect. RESULTS: We report favorable outcomes in two eyes (one patient) where eccentric capsulorrhexis and haptic amputation were employed during one-piece IOL repositioning in one eye and cataract surgery with three-piece IOL implantation in the contralateral eye. CONCLUSION: In coloboma patients who are asymptomatic from their iris defect and do not have a cosmetic desire for repair, eccentric capsulorrhexis and IOL haptic amputation is a viable surgical option that allows for the preservation of a clear visual axis without the need for iris repair.

OPTIC DISK COLOBOMA AND CONTRALATERAL OPTIC DISK PIT MACULOPATHY TREATED BY VITRECTOMY IN A PATIENT WITH NOONAN SYNDROME WITH MULTIPLE LENTIGINES.

PURPOSE: To report a case of Noonan syndrome with multiple lentigines with unusual ocular features. METHODS: The authors describe a case of a 7-year-old girl with Noonan syndrome with multiple lentigines and anomalous optic disks. RESULTS: A 7-year-old girl with genetically proven Noonan syndrome with multiple lentigines ( PTPN11 gene mutation) and anomalous optic disks was referred for treatment of persistent macular detachment after 1 year of conservative follow-up. The right eye demonstrated an optic disk coloboma with the best-corrected visual acuity of 20/32, the left eye demonstrated an optic disk pit with serous macular detachment (best-corrected visual acuity 20/50-20/80). Optical coherence tomography demonstrated a neurosensory detachment. Twenty-five gauge pars plana vitrectomy was performed with posterior hyaloid detachment, drainage over disk pit area, and SF6 20% gas tamponade. Surgery resulted in subretinal fluid reduction and improvement of the visual acuity to 20/32. CONCLUSION: A case of Noonan syndrome with multiple lentigines with optic disk coloboma in the right eye and optic disk pit with related maculopathy in the left eye. To the best of the authors' knowledge, this is the first reported case describing the association of Noonan syndrome with multiple lentigines and congenital optic disk anomalies. Optic disk pit maculopathy was managed surgically because of its longstanding nature with the deteriorating visual acuity.

Outcomes on the Utilization of Lipodermoid Skin for Anterior Lamella Reconstruction in Severe Lipodermoids With Eyelid Coloboma: A Case Series.

Two young children with upper lid colobomas and associated lipodermoids underwent eyelid reconstruction using lipodermoid skin as an autograft with good cosmesis. We describe their presentation, surgical management, and clinical progress following reconstruction, demonstrating the suitability of this skin as an adequate graft avoiding the need for further skin graft harvesting in such cases.

Lunar lenses - Acquired lens colobomas.

Acquired lens colobomas secondary to ocular surgeries are scarcely described in the literature. We describe two cases of acquired lens coloboma in two infants with glaucoma who underwent ocular surgery. The coloboma in the first case was likely because of direct trauma to the lens zonules during an optical iridectomy with a vitrectomy cutter, resulting in localized loss of zonules and consequently localized lens coloboma. The coloboma in the second case was noticed during examination under anesthesia after scleral buckling and cryopexy for retinal detachment. The cause for coloboma development in this case could be disruption of the lens zonules because of stretching of the globe after scleral buckle surgery or because of injury to zonules during scleral buckling and the cryopexy procedure.

Coloboma associated retinal detachment: Anatomical and functional results in the era of microincision vitrectomy surgery with an evaluation of risk factors for a recurrence.

Purpose: : To analyze the anatomical and visual outcomes of microincision vitrectomy surgery (MIVS) with silicone oil tamponade in eyes having coloboma-related retinal detachment (RD) and evaluate the risk factors for recurrence of RD. Methods: : This was a retrospective, multicentric analysis of eyes having coloboma RD undergoing MIVS with silicone oil tamponade between March 2010 and July 2018. Results: : We evaluated 148 eyes of 144 patients. The mean age of presentation was 17.4 ± 9.8 years (range: 2-65 years) and the mean follow-up duration was 13.1 ± 13.8 months (range: 3-84 months). The single operation success rate was 88.5% (131 eyes), with an overall successful outcome achieved in 90.5% (134 eyes). Recurrence of RD occurred in 17 eyes (11.5%) over a mean duration of 2.59 ± 3 months. The risk of recurrence was found to be higher in eyes where relaxing retinectomy (RR) was performed (odds ratio [OR]: 3.22; P = 0.05). A statistically significant improvement in vision was noted in the majority of cases from logMAR 1.85 ± 0.34 preoperatively to logMAR 1.33 ± 0.6 post-surgery (P = 0.002). Conclusion: : MIVS with silicone oil tamponade provided an anatomical success rate of 90.5% in eyes with coloboma RD with a significant improvement in visual acuity. Eyes in which RR was performed were susceptible to higher rates of re-detachment.

Auricular skin-cartilage sandwich graft technique for full-thickness eyelid reconstruction.

Full-thickness deficiency of eyelid tissues can result in coloboma or retraction or both. Here we report our initial experience on the use of auricular skin-cartilage sandwich graft technique for full-thickness eyelid deformities. Five patients (4-32 years) underwent the procedure. Patients with full-thickness eyelid deformity were included. Three patients were operated for large-sized coloboma and two for eyelid retraction. One patient had congenital, and four patients had acquired etiology. The following parameters were specifically assessed: correction of deformity, ocular surface problems, graft status, and epithelization of skin-cartilage graft. All the patients had a good correction of eyelid position, except one patient who had severe eyelid retraction (8 mm) at presentation. None of our patients had corneal erosion/defect, persistent ocular surface redness, or graft loss. The auricular skin-cartilage sandwich graft technique produces optimal results with no graft loss. Advancement of orbicularis muscle in between the auricular skin and cartilage grafts (sandwich technique) is an imperative step that leads to the survival of both grafts.

Cataract surgery in colobomatous eyes with advanced cataract.

BACKGROUND: Cataract surgeries are challenging in colobomatous eyes as they are associated with features such as microphthalmia, microcornea, zonular deficit, etc. These factors predispose to capsulorhexis extension, posterior capsular rent, zonular dialysis and result in unfavourable visual outcomes. The most critical step of cataract surgery in such cases is the capsulorhexis as the chances of extension are high in the colobomatous area. In presence of pre-existing zonular dehiscence, nucleus management becomes more challenging in advanced cataracts. PURPOSE: To illustrate efficient management of advanced cataract in colobomatous eyes. SYNOPSIS: A 39-year old lady presented with complaints of gradually progressive diminution of vision in both eyes. Examination revealed bilateral nuclear sclerosis grade III-IV with irido-lenticular retinochoroidal coloboma (ILRCC). The patient was scheduled for bilateral cataract surgery after a thorough evaluation. Initially, the left eye was planned for phacoemulsification, in the course of which capsulorhexis extension occurred, leading to nucleus tilt and vitreous disturbance during emulsification. Automated anterior vitrectomy was done and the surgical approach was shifted to manual small incision cataract surgery (MSICS) with 3-piece intraocular lens placement in sulcus. The right eye was hence planned for MSICS and a rhexis extension was noted in this eye as well. Comparing the outcome of both the surgeries it was noted that both had capsulorhexis extension, but the management of nucleus and intraocular lens implantation was efficient in case of MSICS than phacoemulsification. Posterior chamber intraocular lens were placed in both eyes and the patient attained good visual outcome. HIGHLIGHTS: MSICS is a better approach to cases of ILRCC with advanced cataract. The capsulo should be tailored according to the site of coloboma such that it should be smaller in the area involving the coloboma and larger away from it. VIDEO LINK: https://youtu.be/d9FC0eavhRs.

Scleral Contact Lens as Initial Management in a Neonate With a Large Upper Eyelid Coloboma.

A neonate presented with a large full-thickness upper eyelid coloboma with near-complete exposure of the cornea. After failing lubrication and a moisture chamber, he was fit with a customized scleral contact lens that protected the ocular surface. It was tolerated well, and ocular surface health was maintained for 13 months to permit the growth of eyelid tissue for future oculoplastic surgery. Delaying reconstruction by using scleral contact lenses as management for large eyelid colobomas has not been previously described. A temporizing measure such as this could be considered for large eyelid colobomas in neonates.

Outcomes of manual small incision cataract surgery and phacoemulsification in eyes with chorioretinal coloboma.

PURPOSE: To evaluate outcomes of manual small-incision cataract surgery (MSICS) and phacoemulsification in eyes with chorioretinal coloboma. SETTING: Tertiary eye center, South India. DESIGN: Retrospective study. METHODS: Electronic medical records of 71 patients with chorioretinal coloboma undergoing cataract surgery from January 2017 to December 2019 were evaluated. Demographics, corrected distance visual acuity (CDVA), slitlamp biomicroscopy findings, grade of cataract, type of coloboma and associated posterior segment pathology, outcomes of different surgical techniques and risk factors for poor visual outcomes, and intraoperative complications were analyzed. RESULTS: Among the 78 eyes studied, 53.9% eyes achieved visual outcome of 20/40 or greater and 20.5% eyes achieved CDVA less than 20/200. For the MSICS group, the median preoperative CDVA was 1.78 (interquartile range [IQR] 1.08 to 2.60) logMAR), which improved to 0.60 (IQR 0.30-1.08) logMAR postoperatively. In phacoemulsification group, the CDVA improved from 0.78 (IQR 0.60-1.00) logMAR to 0.18 (IQR 0.18-0.30) logMAR. Statistically significant visual improvement was noticed in both groups (P < .001 in both). However, eyes that underwent phacoemulsification showed better visual recovery (P < .001). The mean age at presentation was 49.7 ± 10.8 years. MSICS was the most commonly performed surgery (61.54%), and 62 eyes had uneventful cataract surgery. Twelve eyes in MSICS group and 4 eyes in phacoemulsification group had intraoperative complications. Poor visual outcome was associated with male sex, microcornea, hard cataracts, and macular involvement of coloboma. CONCLUSIONS: MSICS is an alternative to phacoemulsification in colobomatous eyes with advanced cataract. Hard cataract and microcornea were risk factors for intraoperative complications. Significant postoperative improvement in CDVA was noticed in both macula involved and macula not involved groups.

Bilateral severe microphthalmos with bilateral colobomatus orbitopalpebral cyst: accessibility of speciality eye-care and rehabilitation services in low and middle-income countries.

A 12-year-old girl presented with an unusually large mass under the right lower eyelid and a smaller mass under the left lower lid since the last 6 months. The parents had noticed the absence of the right eyeball and a very small left eyeball and no vision in both eyes since birth but did not approach the healthcare system. The patient was diagnosed as a case of bilateral severe microphthalmos with colobomatous cyst with late presentation and was treated surgically. The parents were counselled for education and training of the child in schools for visually impaired. Early treatment and rehabilitation help patients lead a normal life in these cases. In rural areas, patients face challenges in getting access to the specialty eye-care services due to several barriers, including lack of availability and affordability. This case highlights the disparities in essential health services in low and middle-income countries.