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A man in his early 20s was referred for refractive surgery evaluation. His best corrected distance visual acuity was 20/60 in both eyes. Preoperative manifest refraction was +13.00 sphere with a 0.75 cylinder in both eyes. Posterior segment examination revealed bilateral pseudopapilloedema with a papillomacular retinal fold and a chorioretinal fold. Numerous formulas for intraocular lens (IOL) power were calculated. Bilateral clear lens extraction was performed, resulting in residual spherical equivalents of +4.50 in the right eye and +3.375 in the left eye. There was no accurate IOL power formula for posterior microphthalmos with extremely high hyperopia; however, the Hoffer Q formula may yield the least error in IOL power., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: To investigate the postoperative clinical outcomes and axial length (AL) growth of infants with congenital cataracts and microphthalmos following first-stage cataract surgery., Design: Retrospective case-control study., Methods: Setting: Single centre. Infants with congenital cataract that met the inclusion criteria were classified into two groups: the microphthalmos and comparison groups. All infants underwent a thorough ophthalmologic examination before surgery, and one week, 1 month, 3 months, and every 3 months after surgery., Results: This study enrolled 21 infants (42 eyes) in the microphthalmos group and 29 infants (58 eyes) in the comparison group. More glaucoma-related adverse events were observed in the microphthalmos group (7 eyes, 16.7%) than in the comparison group (0 eyes, 0%) (p < 0.001). At each subsequent follow-up, the comparison group had a greater AL than the microphthalmos group (all p < 0.001), and AL growth was significantly higher in the comparison group than in the microphthalmos group (all p = 0.035). Visual acuity improvement in the microphthalmos group was similar to that of the comparison group., Conclusion: Early surgical intervention improves visual function in infants with congenital cataracts and microphthalmos although with a higher incidence of glaucoma-related adverse events. After cataract removal, the AL growth of microphthalmic eyes is slower than that of normally developed eyes., (© 2024. The Author(s).)

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., (© 2024. The Author(s), under exclusive licence to Springer Nature B.V.)

Background: Self-inflating hydrogel expanders have been used to treat anophthalmia and blind microphthalmia. This study aimed to investigate the long-term outcomes of treatment with self-inflating hydrogel expanders for congenital anophthalmia and blind microphthalmia., Methods: In this retrospective study, the medical records of 161 patients with anophthalmia and blind microphthalmia who underwent hydrogel expansion were reviewed. We measured the palpebral fissure height (PFH), palpebral fissure length (PFL), and distance between the inner canthal and mid-nasal line (ICMN) before and after surgery. Cox regression analysis was conducted to determine which variables were related to the implantation of spherical expanders following hemispherical expander implantation., Results: After treatment, the PFH and PFL increased significantly (p < 0.001). Complications including expander migration and extrusion occurred in 15 cases. Five patients needed enucleation or further dermis fat graft implantation because of insufficient expansion. The necessity for further spherical expansion was substantially related to a relative axial length (rAL) <0.5 (p = 0.007)., Conclusion: Self-inflating hydrogel expansion can significantly increase the lid fissure. The occurrence of complications is rare, and surgical intervention can effectively address them. Abnormal eyes with a rAL of less than 0.5 demonstrate a higher possibility of needing additional orbital expansion., Competing Interests: Declaration of Competing Interest The authors report there are no competing interests to declare., (Copyright © 2024 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)

Bosma arhinia microphthalmia syndrome (BAMS) is a rare syndrome consisting of several craniofacial abnormalities, including congenital arhinia. In this case report, the authors present the first case of a patient with BAMS and dacryocystocele who successfully underwent dacryocystectomy. Dacryocystectomy may serve as a viable surgical approach for dacryocystocele in patients with abnormal nasal anatomy. [ J Pediatr Ophthalmol Strabismus . 2024;61(3):e16-e18.] .

Background/aims: Microphthalmia and anophthalmia are rare conditions, which represent ocular maldevelopment; both may be associated with orbital cysts. Current literature recommends retention to stimulate orbital growth during socket rehabilitation but does not illustrate their potential to deform the periocular tissues. This study aims to illustrate the long-term outcomes when other elements, such as forniceal and lid development, are also considered when formulating bespoke treatment plans for patients., Methods: Retrospective case series of 78 patients attending a single prosthetics clinic between 1988 and 2020. Clinical and surgical notes, radiological imaging, clinical photographs and patient/doctor satisfaction questionnaires were used to report patient outcomes and natural history data., Results: 89 sockets of 78 patients (11 bilateral) were included; average age of presentation being 2.8 years (9 days to 29.5 years). Cysts were clinically detected (48%) or were incidental findings (52%). The mean follow-up time was 7.2 years (6 months to 28 years). Cysts in 46% of sockets underwent surgical excision while the remainder were retained. Satisfaction surveys were obtained for 75 patients, with cosmetic outcomes rated as 'excellent' or 'good' in 90% of cases by physicians and 97% of cases by patients or guardians., Conclusion: The favourable long-term outcomes in this study have resulted from bespoke plans which considered periocular tissue development, regional orbital growth and orbital volume replacement. The authors contemplate cyst excision if the prosthetic fitting or retention is impeded by the cyst as this often heralds the increased risk of long-term periocular distortion., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Rationale: Microphthalmia with limb anomalies is a rare, autosomal recessive, multiple congenital anomaly syndrome. Patients with this syndrome particularly present with monocular or bilateral anophthalmia/microphthalmia and distal limb anomalies. However, details regarding associated spinal deformities have not been fully elucidated., Patient Concerns: A 12-year-old girl initially presented with progressive scoliosis, who was previously diagnosed with microphthalmia with limb anomalies. However, 4 years after the initial visit, the scoliosis deformity gradually progressed. The patient and family requested the surgical treatment to preserve standing/sitting balance., Diagnoses: She was diagnosed with microphthalmia with limb anomalies and progressive scoliosis., Interventions: A posterior corrective fusion surgery (including a pelvic fusion) was performed to prevent future standing/sitting imbalance., Outcomes: Significant improvement of spinal deformity was observed, with no adverse events., Lessons: This report demonstrated a case of progressive scoliosis associated with microphthalmia with limb anomalies. A posterior corrective spinal fusion was effective to preserve standing/sitting balance. To the best of our knowledge, this is the first report of surgical treatment of progressive scoliosis associated with microphthalmia with limb anomalies., Competing Interests: YTo, HO, and KN received a Grant-in-Aid for Scientific Research (C), 21K09207, supported by the Japan Society for the Promotion of Science. HO received a grant from Japan Orthopaedics and Traumatology Research Foundation, No. 521. YTo is on the editorial board of Cancer Diagnosis and Prognosis. KN is on the editorial board of Journal of Orthopaedic Research and a board member of the International Society for the Study of Lumbar Spine. For the remaining authors, no potential conflicts of interest are declared., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Purpose: The aim of this study was to determine the prevalence of late-onset glaucoma after surgery for congenital cataract in a cohort with long-term follow-up and to evaluate visual development following the diagnosis of postoperative glaucoma in comparison with no glaucoma development., Methods: All children born between 1980 and 1997 in the western counties of Sweden who had undergone congenital cataract surgery were included (patients n = 77, eyes n = 122). Cataract was considered congenital if there was no proof of clear lens at birth. Medical records were reviewed with regard to onset of glaucoma, age at surgery, surgical technique, coexisting eye anomalies and changes in visual acuity. Glaucoma was considered late onset if occurring after 1 year following surgery., Results: Total glaucoma prevalence was 14.8%, including late (10.7%) and early onset (4.1%), with a mean follow-up of 23.2 ± 6.6 years. Microphthalmos was a significant risk factor for developing glaucoma (RR 7.75, p < 0.001). Bilaterally treated eyes had a mean visual acuity of 0.43 ± 0.33 (decimal value) at the last follow-up. With glaucoma, mean visual acuity was 0.19 ± 0.17 (decimal value). Treated eyes of patients with unilateral cataract surgery had a lower visual acuity., Conclusions: Life-long follow-up of all patients who have undergone surgery for congenital cataract in childhood is recommended. Annual check-ups of adults, including measurements of IOP and visual acuity, are recommended for patients with microphthalmos and/or who had surgery <3 months of age., (© 2022 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Objectives: To compare the long-term efficacy and safety of combined phacoemulsification, anterior vitrectomy, and sclerectomy (triple procedure surgery, TS); combined phacoemulsification and anterior vitrectomy (double procedure surgery, DS); and filtering surgery (FS) in nanophthalmos with angle-closure glaucoma (NACG)., Methods: Retrospective cohort study. Forty patients (44 eyes) diagnosed with NACG who underwent TS, DS, and FS were included. All eyes in the TS group and seven (47%) eyes in the DS group also underwent goniosynechialysis during the surgery. The main outcome measures (intraocular pressure [IOP], best-corrected visual acuity, complications, and second surgeries) were recorded at the early- (within 1 week) and late-stage (>3 months) follow-up., Results: The late-stage IOP was significantly lower in the TS (mean ± standard deviation: 13.29 ± 2.49 mm Hg) than in the DS (19.69 ± 6.97 mm Hg) and FS groups (27.57 ± 12.26 mm Hg, p < 0.001). More visual improvements were observed in the TS and DS groups than in the FS group at late-stage follow-up (p = 0.04). The complication rates in the TS, DS, and FS groups were 26%, 33%, and 70%, respectively (p = 0.046); the second surgery rates were 0%, 33%, and 60%, respectively (p < 0.001). In total, one, three, and six severe complications were observed in the TS, DS, and FS groups, respectively. The mean follow-up durations in the TS, DS, and FS groups were 18.89, 20.02, and 25.75 months, respectively., Conclusions: NACG management remains challenging. TS presented relatively good clinical efficacy and safety with better postoperative IOP outcomes, lower complications, and second surgery rates among the three groups in eyes with NACG., (© 2022. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

One of the greatest challenges for ocularists is prosthetic fitting in children, especially in children with congenital anomalies such as clinical anophthalmia or functionless (blind) microphthalmia. The most frequent reason for prosthetic fitting in children is a condition following enucleation for retinoblastoma, followed by trauma and congenital pathologies. The standard treatment after enucleation or evisceration begins intraoperatively with the selection of an suitable implant and the use of a conformer at the end of the operation to shape the prosthetic cavity. An initial prosthesis can be fitted 4 weeks postoperatively, with a final fitting taking place 3 months later. If iatrogenic scarring or scarring due to an infection of the prosthetic cavity occurs, the approach of the ocularist must be appropriately adapted with the use of modified prosthesis shapes and shorter treatment intervals. Surgical options include scar excision and oral mucosa or amniotic membrane transplantation. Congenital anomalies require the shortest treatment intervals and even more so for anophthalmia than for microphthalmia. The strategy is characterized by simultaneous stimulation of the soft tissue of the ocular adnexa as well as the bony orbit. As self-inflating hydrogel expanders are no longer available, conservative prosthetic treatment is the only option. Close cooperation between child/parent, ocularist and ophthalmic plastic surgeon is the best prerequisite for a good long-term treatment outcome., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Purpose: To report the outcomes of acrylic conformer-assisted socket expansion in congenital anophthalmia and microphthalmia., Methods: In this noncomparative, interventional case series, the medical records of 24 eyes of 18 consecutive patients with congenital anophthalmia (n = 3), clinical anophthalmia (n = 8), and microphthalmia (n = 13) were reviewed retrospectively. Twelve cases were unilateral; 6 were cases bilateral (3 clinical anophthalmia and 3 microphthalmia). Serial socket expansion with progressively larger acrylic conformers was managed in clinic. Horizontal palpebral fissure (HPF) width was graded as good (final HPF ≥20 mm, or interocular difference ≤2 mm for unilateral cases), fair (17-19 mm, or 3 mm interocular difference), or poor (<17 mm, or ≥4 mm difference)., Results: The mean initial lid lengths in anophthalmia, clinical anophthalmia, and microphthalmia were 11.0, 12.4, and 16.9, increasing to 21.0, 19.9, and 22.2, respectively, over a mean period of 51 months. Mean age at the initiation of treatment was 19 months (range, 1-78). Percentage increases in lid length were 90.9%, 61.2%, and 31.3% in anophthalmia, clinical anophthalmia, and microphthalmia, respectively, with an average 7 conformer exchanges. For unilateral cases, the mean final lid length of involved eyes was 22.3 mm, comparable to 23.5 mm in normal contralateral eyes. Good outcomes were achieved in 18 orbits (75%); fair outcomes, in 6 (25%) cases. None of the sockets had poor expansion at final follow-up. All cases had good cosmesis with acceptable prosthesis wear at last visit., Conclusions: In our patient cohort, good socket expansion was achieved with acrylic conformers alone in congenital anophthalmia and microphthalmia, with acceptable prosthesis wear., (Copyright © 2022 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

Purpose: Cataract surgery in patients with nanophthalmos is complicated for ophthalmologists to perform. Due to the unique ocular anatomy, there is a high incidence of complex complications such as angle-closure glaucoma, fluid misdirection syndrome, and uveal effusion syndrome (UES) in the perioperative period of cataract surgery. This article will discuss the management options for cataract surgery in nanophthalmic eyes and complications., Methods: This review is searched through PubMed, focusing on articles published in the past 20 years. Articles were reviewed on the anatomical structure of nanophthalmic cataracts, the pathogenesis of complications, the selection of intraocular lenses, and surgical methods., Conclusion: There is a strong correlation between abnormal ocular anatomy and complications in patients with nanophthalmos. Clinicians must not only select the appropriate intraocular lens formula based on the depth of the anterior chamber but also formulate personalized surgical methods based on its unique anatomical structure to avoid complications.

Purpose: To investigate the long-term outcomes of intraorbital self-inflating hydrogel expander implantation with optic nerve transection in children with congenital microphthalmia., Methods: The medical records of unilaterally blind microphthalmic pediatric patients undergoing intraconal hydrogel expander implantation with optic nerve transection were reviewed retrospectively. For each patient, the microphthalmic eye was preserved. The orbital volume and globe volume were measured and analyzed based on computed tomography scans taken preoperatively and 36 months postoperatively. The palpebral length was measured between the medial and lateral canthus at every follow-up. Surgical complications were also recorded., Results: Twelve patients were included (median age, 44.25 ± 17.5 months). At 36 months postoperatively, the microphthalmic and contralateral orbital volumes increased by 3.07 ± 0.77 ml and 2.03 ± 0.67 ml, respectively. The mean microphthalmic/contralateral ratio (MCR) of the orbital volume increased significantly from 76.60% ± 5.46% to 83.81% ± 5.41% (P < 0.001). The microphthalmic palpebral length increased by 6.17 ± 1.85 mm, whereas the contralateral palpebral length increased by 2.67 ± 1.44 mm. Significant changes were observed in the palpebral length MCR (68.00% ± 4.83% vs 85.07% ± 3.87%; P < 0.001). There was no significant change in the microphthalmic globe volume at 36 months postoperatively (P = 0.215). For the fellow eye, the globe volume increased significantly by 0.53 ± 0.34 ml (P < 0.001). During the follow-up period, 2 patients developed a sunken prosthesis. One patient had difficulty opening the eye after wearing the conformer. There were no cases of expander rejection or extrusion., Conclusions: In this small cohort of patients with congenital microphthalmia, intraorbital self-expanding hydrogel expander implantation with optic nerve transection led to excellent osseous and eyelid growth throughout the 36-month follow-up period., (Copyright © 2022 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

Competing Interests: None

Background/aims: This study aimed to describe a cohort of patients with cryptophthalmos (CO), characterize associated oculofacial abnormalities, and expand the classification to summarize surgical strategies for managing CO., Methods: A retrospective, interventional case series was conducted on 86 patients (124 eyes) with CO. The study proposed further classifying complete and incomplete CO into cyst, microphthalmia, anophthalmia, and normal eyeball based on globe structures and then modifying surgery accordingly. The demography, ophthalmic features, systemic anomalies, operation methods, and treatment outcomes were reviewed., Results: CO was complete in seven eyes (5.6%) and incomplete in eight eyes (6.5%). A total of 109 eyes (87.9%) of abortive CO were encountered. Among 15 eyes (13 patients) of complete and incomplete types, 9 (60.0%) eyeballs were identified as cysts, 3 (20.0%) as microphthalmia, 1 (6.7%) as anophthalmia, and 2 (13.3%) as normal eyeballs. Cyst reduction was performed in eight eyes and one patient underwent enucleation with hydroxyapatite implantation. The socket was fit with an ocular prosthesis or a conformer after fornix and eyelid reconstruction. Microphthalmia was enucleated, and hydroxyapatites were implanted; patients were fit with ocular prosthesis or conformer after fornix and eyelid reconstruction. A complete CO with normal eyeball was reported with the eyesight of hand movement after ocular surface reconstruction. The upper eyelid contour and adequate fornix were maintained after coloboma repair and fornix reconstruction in all patients with abortive CO., Conclusion: This study demonstrates the clinical manifestations of different types of CO and expands the manifestation spectrum, proposing a refined classification of CO and modifying surgical strategies accordingly., Competing Interests: Declaration of Competing Interest All the authors have indicated that they have no potential conflicts of interest to disclose., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

Purpose: To describe a series of patients treated for congenital microphthalmia associated with orbital cyst and recommend a management protocol., Methods: This retrospective case series comprised 6 patients (7 eyes) who attended an oculoplastic tertiary medical center from 2001 to 2018. Clinical, treatment, and outcome data were collected from the electronic files. Main outcome measures were preservation of vision and cosmetic appearance., Results: Four patients were diagnosed at birth. Six cysts were located inferiorly and one superiorly. Two patients had a visual potential of light perception or better in the affected eye. In 4 eyes, the cyst was initially retained and the eye was fitted with a custom-made conformer. In 1 eye, the fornices were too shallow for a conformer, warranting fornix reconstruction and cyst excision. Early surgery was required in 1 eye for an expanded cyst and large orbit volume, and in another eye the cyst had overgrown the orbit, causing bone erosion and remodeling. Cosmetic results were good in 3 of the eyes in which the cyst was retained in early childhood, stimulating orbital growth., Conclusions: Congenital microphthalmia with orbital cyst is rare. Management should focus on preserving visual potential, especially in unilateral cyst cases when the other eye is also microphthalmic. Otherwise cosmetic symmetry is the main concern; cyst retention combined with ocular conformers may stimulate socket expansion. The authors found that, in most cases, if treated early, enucleation was avoidable during cyst excision. Early assessment, meticulous follow-up, and individually tailored treatment are warranted. [ J Pediatr Ophthalmol Strabismus . 2022;59(3):192-199.] .

Objectives: To report visual and anatomical outcomes following two- or four-quadrant partial-thickness sclerectomy and sclerotomy surgery to treat nanophthalmos (NO)-related uveal effusion (UE)., Materials and Methods: Consecutive patients with NO-related UE were treated with four-quadrant or two-quadrant (for those with associated glaucoma) partial-thickness sclerectomy and sclerotomy surgery. Axial length, extent of UE, preoperative, postoperative, and final best corrected visual acuity (BCVA), time to retinal reattachment, and rates of retinal reattachment and recurrence were noted., Results: Fourteen eyes of 10 patients with NO-related UE were operated. Retinal detachment (RD) involved mainly the peripheral retina in 7 (50%) eyes, macula in 2 eyes (14.2%), both macula and peripheral retina in 4 eyes (28.6%), and the whole retina in 1 eye. Eleven eyes had four-quadrant surgery, and 3 eyes with associated glaucoma had two-quadrant surgery. External subretinal drainage was performed in one patient who had total RD. The mean preoperative logMAR BCVA of 1.50±0.53 increased significantly to 0.92±0.49 after surgery (p=0.002). Resolution of RD could be achieved with two-quadrant surgery in only 1 of 3 eyes. In the other 2 eyes, retinal reattachment was achieved after a secondary surgery for the remaining two quadrants to complete four-quadrant sclerectomy. Final outcome was total reattachment of the retina in 11 eyes (78.6%), partial reattachment in 1 eye (7.1%), and recurrence of macular detachment in 2 (14.3%) eyes., Conclusion: Quadrantic partial-thickness sclerectomy and sclerotomy surgery seems effective for treating UE in eyes with NO. Twoquadrant surgery may be tried for mild UE associated with glaucoma to preserve the superior quadrants for future possible glaucoma surgeries, but secondary surgery for the superior quadrants may be needed. External drainage of subretinal fluid may be an option in severe cases to achieve quicker resolution.

Relative anterior microphthalmos, nanophthalmos and high-grade hyperopia are small eyes with different characteristic morphological relationships between the anterior segment and axis length. This article discusses the intraoperative challenges and surgical approaches to solutions for cataract operations in patients with one of the three named morphological alterations. Additionally, the article addresses possible comorbidities including glaucoma and preoperative planning., (© 2021. The Author(s).)

Background: Management of secondary glaucoma in nanophthalmos has always been challenging, especially for patients with extremely short axial length and extensive angle synechia. This case report discusses a nanophthalmic patient with secondary glaucoma and extensive angle synechia., Case Presentation: A 60-year-old woman was referred to our hospital with uncontrolled intraocular pressure (IOP) in her left eye (LE). Slitlamp examination revealed small cornea and shallow anterior chamber (AC) in both eyes. Extensive angle synechia was seen on gonioscopy, and the IOP and axial length were 36 mmHg/15.79 mm in the left eye. She was diagnosed with nanophthalmos and secondary angle-closure glaucoma LE. Laser peripheral iridectomy (LPI) was performed, but the IOP LE remained elevated and uncontrolled on brinzolamide and brimonidine. The IOP LE was then normalized with the application of 2% pilocarpine. During the follow-up period, the AC of the LE showed progressive shallowing, and 5 months post LPI the IOP became uncontrollable. Goniosynechialysis combined with phacoemulsification, lamellar sclerectomy, sclerostomy, peripheral iridectomy, capsulotomy, and anterior vitrectomy were performed LE. Immediately after surgery, the AC deepened and the IOP stabilized. However, the IOP rose again post-surgically at 1 week and again at 2 months. A generally shallow AC and obstructed capsule hole were present. YAG laser capsulotomy was performed, and the IOP normalized and the AC deepened. Eighteen months after surgery, the IOP LE was 12 mmHg and the AC remained stable., Conclusion: Goniosynechialysis combined with multiple surgical procedures could be considered an alternative treatment regimen for nanophthalmos patients with secondary glaucoma and extensive angle synechia.

Nanophthalmos-4 is a rare autosomal dominant disorder caused by two known variations in TMEM98. An Austrian Caucasian pedigree was identified suffering from nanophthalmos and late onset angle-closure glaucoma and premature loss of visual acuity. Whole exome sequencing identified segregation of a c.602G > C transversion in TMEM98 (p.Arg201Pro) as potentially causative. A protein homology model generated showed a TMEM98 structure comprising α4, α5/6, α7 and α8 antiparallel helix bundles and two predicted transmembrane domains in α1 and α7 that have been confirmed in vitro. Both p.Arg201Pro and the two missense variations representing proline insertions identified previously to cause nanophthalmos-4 (p.Ala193Pro and p.His196Pro) are located in the charge polarized helix α8 (p.183-p210). Stability of the C-terminal alpha helical structure of TMEM98 is therefore essential to prevent the development of human nanophthalmos-4. Precise molecular diagnosis could lead to the development of tailored therapies for patients with orphan ocular disease., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Purpose: To study the varied demographic, visual and clinical presentation of patients with nanophthalmos., Methods: Retrospective chart review of 144 consecutive subjects with nanophthalmos from January 2010 to January 2018 was done. Demographic details, visual acuity, refractive status, clinical parameters and surgical data were collected., Results: Mean age at presentation was 48.76 ± 15.99 years (5-74 years) and 55.6% were females. Median BCVA was 0.78 (6/36 Snellen equivalent) and median spherical equivalent was +10.0 (5.7-12.5) . Amblyopia was seen in 30.0% patients. Legal blindness was present in 16.7% of the study population. The mean IOP was 18.38 ± 9.38 mmHg. Angle closure disease was present in 67.7% and 35.7% had angle closure glaucoma. Presence of peripheral anterior synechia had higher odds (OR = 3.66; 95% CI, 1.71-7.84) of associated glaucoma. The mean axial length, 17.64 ± 1.74 mm was inversely correlated to the mean Retinochoroidal Sclera(RCS) thickness of 1.99 ± 0.25 mm (r = -0.28, p value < 0.001).All patients who had undergone surgery for glaucoma (n = 11) had associated intra or postoperative complication. Cataract surgery by manual small incision had more complications than Phacoemulsification (p value = 0.001). Occurrence of uveal effusion was significantly lower in eyes which underwent sclerostomy (p = 0.04) CONCLUSION: Nanophthalmos is a rare disorder with varying degree of visual impairment & amblyopia. High incidence of angle closure glaucoma was observed. Surgical management for glaucoma is often challenging with frequent complications. Cataract surgery by phacoemulsification had significantly lower complications than SICS and performing a concomitant sclerostomy reduced the occurrence of uveal effusions.

Purpose: To evaluate health-related quality of life (HR-QoL), vision-related (VR-)QoL and perceptual visual dysfunction (PVD) among individuals with anophthalmia (A) and microphthalmia (M) treated with ocular prosthesis., Methods: The study comprised 15 individuals (mean age 6.6 years; range 1.7-14.1) with unilateral A or M. Three validated instruments measuring HR-QoL and VR-QoL were used: The Pediatric QoL Inventory (PedsQL), consisting of physical and psychosocial self-report and parent-proxy report (2-18 years); Children's Visual Function Questionnaire (CVFQ); and Effects of Youngsters' Eyesight on Quality of Life (EYE-Q). Perceptual visual dysfunctions (PVDs) were assessed by history taking according to a specific protocol., Results: A/M children and their parents showed low HR-QoL scores (PedsQL total score: 66.3; 69.6) compared with controls (83.0; 87.61) (p = 0.0035 and <0.0001, respectively, unpaired t-test). No differences were found between A/M children and parents, but parents tended to underestimate their children's emotional state. A/M children with subnormal visual acuity (VA) for age scored lower in physical health compared with A/M children with normal VA (p = 0.03, Mann-Whitney U-test). No significant VR-QoL differences between A/M children and references or between A/M children with subnormal or normal VA for age were found. More A/M children than controls exhibited PVDs in ≥1 area (7/11 versus 4/118; p < 0.0001, Fisher's exact test)., Conclusion: A/M individuals show poor HR-QoL and increased PVDs. No difference in QoL was found between children and parents, though the children tended to score lower in emotional well-being. A/M children with subnormal VA showed lower physical health score. These problems indicate the necessity of a thorough multidisciplinary assessment and follow-up of children with A/M., (© 2020 The Authors. Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.)

Competing Interests: Competing interests: None declared.

A 36-year-old woman presented with eye pain and blurring of vision in her right eye. On eye examination, it was noted that there were angle-closure glaucoma and anterior uveitis in both eyes. Ocular ultrasound showed short axial lengths as well as a choroidal thickening in both eyes, confirming the diagnosis of nanophthalmos. Nanophthalmos is a condition where the eye is abnormally short, resulting in axial hyperopia and predisposing it to angle-closure glaucoma. The patient was initially managed medically, but the glaucoma was intractable. The patient underwent repeated sessions of transscleral cyclophotocoagulation which eventually lowered the intraocular pressure. The management of nanophthalmic eyes can be quite challenging due to the risk of inciting uveal effusion syndrome with any form of intraocular surgery. Controlled and repeated sessions of transscleral cyclophotocoagulation may be considered as a viable management option in these cases., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Uveal effusion syndrome is a rare entity of idiopathic exudative detachments of uveal tissues and retina. Medical treatments with systemic steroids and antimetabolites have been tried but with variable results. Scleral windows or vortex decompressions are usually performed and surgeons usually perform partial sclerectomy in all the quadrants., Case Presentation: For the first time, we report 2 cases of nanophthalmic uveal effusion syndrome managed with our technique., Conclusion: Quadrantic vortex vein decompression with external drainage for nanophthalmic uveal effusion can provide immediate and stable gain in vision.

Trauma is the major reason for globe loss in adults; however, there are less data regarding the causes for globe loss in children. We reviewed the underlying diagnoses of children who underwent ocular prosthesis fitting over a 1-year period at a referral eye hospital in the Middle East and found retinoblastoma, trauma, and congenital microphthalmia or anophthalmia to be the most common diagnoses, respectively. Enucleation and evisceration were the most common procedures and were exclusively performed for retinoblastoma and trauma, respectively. Ocular morbidity from the most common diagnoses related to pediatric globe loss in the region could be decreased by improved family education, safety precautions, and genetic counseling., Competing Interests: There are no conflicts of interest.

Purpose: To report the customized approach of patients with anophthalmia or microphthalmia with bespoke ocular prosthesis., Methods: Retrospective analysis of case series., Results: The study included cases with anophthalmia with upper eyelid deformity (one patient), microphthalmia and contralateral corectopia (one patient), microphthalmia with contralateral corneal graft (one patient), and congenital clinical anophthalmia with contralateral sclerocornea (one patient). Using techniques of embedded autologous hair and coating of adhesive pigment emulsion in the ocular prosthesis, the physical appearance of, respectively, an upper eyelid, corectopia, corneal graft, and sclerocornea was reproduced., Conclusion: Tailoring the ocular prosthesis to the distinct condition of the anophthalmic socket and contralateral eye adds to the success of rehabilitative prosthetic treatment of the patient.

Purpose : The purpose of the study is to evaluate demographic data and outcomes of the management of congenital anophthalmia/microphthalmia. Methods : This retrospective, descriptive, cross-sectional study evaluated patients with congenital anophthalmia/microphthalmia managed from 2004 to 2014 at a tertiary hospital in Saudi Arabia. Data were collected on patient age, gender, cause, orbital status, laterality, systemic comorbidities, ocular evaluation, and management (type of surgery, type of orbital implant, and complications). The main outcome measure was the ability to hold the prosthesis. Results : The study sample was composed of 513 eyes/sockets of 365 patients. Two-hundred and seventeen (59.4%) patients were unilateral cases. Forty-one (8%) sockets were due to congenital anophthalmia and 471 (92%) were due to microphthalmia. There were 73.2% isolated cases and 28.5% with systemic involvement. Systemic involvement was more common in bilateral cases. The most commonly associated conditions were central nervous disorders. One-hundred and nineteen (46.7%) cases had parental consanguinity. Two hundred and eighteen eyes/sockets (163 patients) underwent surgery including conjunctival flap (38; 17.4%), evisceration (38; 17.4%), enucleation (16; 7.3%), or procedures to improve the anophthalmic socket volume (45; 20.6%). Volume enhancing procedures included polymethylmethacrylate orbital implants (26; 57.8%), expanders (11; 24.4%), integrated hydroxyapatite or polyethylene implants (2; 4.4%), and dermis-fat graft (6; 13.3%). In most cases, clinical or surgical management resulted in a successful outcome. Conclusion : Anophthalmia/Microphthalmia was detected in 36.5 patients/year. The majority had isolated microphthalmia. Good outcomes were achieved with clinical or surgical management in the majority of cases.

Purpose: To study the effects of an individualized treatment approach to children with congenital microphthalmos and anophthalmos., Methods: Patients with congenital microphthalmos or anopthalmos with orbital cysts who were referred to Beijing Tongren Hospital between July 2009 and July 2017 were included in this retrospective case series study. For patients ≤6 years of age, the cyst was retained to promote orbital development unless a prosthesis could not be fitted at all or disproportionate orbital growth was detected. Hydrogel orbit expanders were implanted initially if orbital volume was poor. For patients >6 years of age, the cyst was removed if it caused cosmetic problems or unsatisfactory prosthesis fitting. Eyelid procedures were performed after puberty to improve appearance., Results: The study included 26 orbits of 24 patients. Of the 14 patients ≤6 years, 3 underwent cyst excision, 8 were treated with conformers only, and 3 had hydrogel orbit expander implantation initially. Of the 10 patients >6 years, 1 had the cyst removed, 5 had eyelid surgeries without removing the cyst, 3 wore an artificial eye without any surgery, and 1 used no prosthesis. During the follow-up period (range, 6 months to 8 years), 23 patients had a good cosmetic outcome and were eventually able to retain an ocular prosthesis; 1 patient could not wear prostheses but refused further treatment. No procedure-related complications were noted., Conclusions: The individualized treatment of congenital microphthalmos and anophthalmos with orbital cysts depends on the patient's age at presentation, the growth pattern of the cyst, and the volume of the affected orbit., (Copyright © 2019. Published by Elsevier Inc.)

BACKGROUND The pulmonary hypoplasia/agenesis, diaphragmatic hernia/eventration, anophthalmia/microphthalmia, and cardiac defect (PDAC) syndrome is a rare medical condition presumably of autosomal recessive way of inheritance with only a few reported cases. Recessive mutations in the STRA6 and both recessive and dominant mutations in RARB gene have been identified as the cause of anophthalmia/microphthalmia and other abnormalities included in the PDAC spectrum. However, those mutations have not been found in all PDAC syndrome cases reviewed from the literature. CASE REPORT We report a case of a full-term living male infant with pulmonary hypoplasia, left diaphragmatic eventration, bilateral microphthalmia, congenital cardiac defects, and severe pulmonary hypertension. CONCLUSIONS The main feature in the reported cases was anophthalmia/microphthalmia. Therefore, screening for the other associated congenital anomalies is highly suggested. Mutations in STRA6 and RARB genes are commonly encountered in this spectrum. However, whole exome sequencing of suspected cases and their parents is recommended to detect possible de novo mutations. Further reports are needed to identify risk factors and prognosis of this rare syndrome.

Purpose: To determine the effectiveness of iridectomy, capsulotomy and anterior vitrectomy through the anterior chamber to treat misdirection syndrome in pseudophakic nanophthalmic eyes., Methods: This was a non-comparative study of seven nanophthalmic eyes from four consecutive patients. All eyes developed misdirection syndrome after successful cataract surgery. Treatment for misdirection syndrome involved capsulotomy and anterior vitrectomy through a peripheral iridectomy from the anterior chamber using a 25-gauge vitreous cutter. The best-corrected visual acuity, intraocular pressure and anterior and posterior segment findings were recorded before and after surgery., Results: Resolution of the aqueous misdirection was achieved in all but one eye. The single case of recurrence was observed after a mean follow-up of 45.6 ± 21.5 months and was caused by closure of the capsule hole by Elschnig's pearls. This eye was successfully treated by enlargement of the lens capsule hole with a vitreous cutter. The mean intraocular pressure before surgery was 28.7 ± 4.4 mmHg, and this was significantly reduced to 13.7 ± 1.3 mmHg at the final visit. All but one patient, who had uveal effusion, maintained their best-corrected visual acuity., Conclusion: In this study, we investigated an alternative option for the treatment of misdirection syndrome in nanophthalmic eyes. We undertook a lens capsulotomy and anterior vitrectomy through a peripheral iridectomy from the anterior chamber using a 25-gauge vitreous cutter, which was able to create a communication hole between the anterior and posterior chambers.

Purpose: To report the surgical outcomes of children with cataract associated with microphthalmia, microcornea, or persistent fetal vasculature (MMP) and children with isolated cataract., Methods: The study included 111 children (cataract associated with MMP: n = 25) who underwent cataract surgery at younger than 16 years. Exclusion criteria were duration of follow-up less than 5 years, intraindividual differences in age at surgery, eye disorders other than MMP, secondary cataract, and syndromal diseases. Main outcome measures were proportion of eyes with glaucoma and best corrected visual acuity (BCVA). Both groups were dichotomized by age at surgery (early intervention group: ≤ 48 days). Descriptive analysis was performed throughout., Results: Median age at surgery was 3.9 months for cataract associated with MMP and 23.3 months for isolated cataract. The median (interquartile range [IQR]) duration of follow-up was 95.9 months (range: 76.0 to 154.5 months). In children with bilateral cataract associated with MMP, the proportion of eyes with final BCVA worse than 0.3 logMAR was similar regardless of age at surgery (early intervention: 80%, later intervention: 78%). In bilateral isolated cataract, the proportions were 56% and 33%, respectively. Children with cataract associated with MMP had a high prevalence of glaucoma (28%). Glaucoma prevalence was lower in the later intervention group., Conclusions: In the presence of MMP, early cataract surgery is associated with a high risk of post-lensectomy glaucoma, but does not offer the benefit of better visual outcomes. [J Pediatr Ophthalmol Strabismus. 2019;56(1):43-49.]., (Copyright 2018, SLACK Incorporated.)

Background: The management of eyes with nanophthalmos is a dilemma for ophthalmologists due to various complications, especial the eye with malignant glaucoma. We report a case of effective treatment for malignant glaucoma in nanophthalmos., Case Presentation: An 82-year-old man was performed phacoemulsification in the right eye with normal ocular pressure and nanophthalmos. The surgery was uneventful: an intraocular lens (IOL) was placed and centered in the capsular bag. 2 months later, the patient presented with malignant glaucoma, and the intraocular pressure fluctuated between 18.6 mmHg and 30.8 mmHg with antiglaucoma medications. The patient did not respond to surgical peripheral iridotomy and goniosynechialysis. Then a single treatment with laser peripheral lens posterior capsulotomy and vitreous anterior membranectomy was performed. The intraocular pressure normalized, and the anterior chamber deepened within 24 h. The patient's condition remained stable for 9 months with no further treatment, and his Snellen corrected distance visual acuity was 20/50. The left eye of this patient was treated by combined surgery including phacoemulsification, IOL implantation, anterior vitrectomy, surgical peripheral iridotomy (PI), and goniosynechialysis. No intraoperative or postoperative complications were observed., Conclusions: This case suggests that it is essential to choose a suitable treatment for nanophthalmos patients to deal with malignant glaucoma and to reduce the incidence of malignant glaucoma.

A 55-year-old female with an aphakic nanophthalmic eye underwent a secondary intraocular lens implantation (IOL) with double Artisan aphakia iris claw IOLs (ICIOLs) and was evaluated in this research. The patient's preoperative best-corrected visual acuity (BCVA) of the right eye was 0.4 (0.4 logMAR) (with + 21.00 D), postoperative 1st and 3rd month, 1st year, and 3 years BCVAs were 0.4 (0.4 logMAR). The intraocular pressure was 15 mmHg preoperatively, and 14, 12, 12, and 15 mmHg postoperatively at 1st and 3rd month, 1st year, and 3 years, respectively. The preoperative endothelial cell density (ECD) was 2372 cells/mm2, and postoperative ECDs were 2352, 2391, 2246, and 2240 cells/mm2 at 1st and 3rd months, at 1st year, and 3 years respectively. In aphakic nanophthalmic eyes with inadequate capsular support, which require high IOL dioptry, the implantation of double ICIOLs (one in front of the iris and the other behind the iris) seems to be safe and provides good visual rehabilitation.

Purpose: To prospectively evaluate visual outcomes and complications during and after cataract surgery with or without prophylactic sclerostomy in nanophthalmic eyes with visually significant cataract., Study Design: Randomized controlled trial., Methods: Sixty nanophthalmic eyes of 60 patients with visually significant cataract were randomly assigned to cataract surgery alone (control group, n = 31) or cataract surgery with concomitant prophylactic sclerostomy (sclerostomy group, n = 29). Surgery was performed using phacoemulsification or manual small-incision cataract surgery (SICS) based on the LOCS III grading score. Group differences in intraoperative and postoperative complications were analyzed and risk factors assessed., Results: Fewer complications were noted in eyes receiving sclerostomy (5/29, 17.2%) as compared to control group eyes (12/31, 38.7%), though differences were marginally significant (P = .065). Four control group, but no sclerostomy group, eyes developed postoperative uveal effusions (P = .04). In multivariable models, sclerostomy decreased the odds of an intraoperative or postoperative complication by 80% (odds ratio [OR] = 0.2, 95% confidence interval [CI] = 0.04-0.92, P = .039); SICS was associated with a significantly higher risk of complications as compared to phacoemulsification (OR = 5.95, 95% CI = 1.49-23.73, P = .012), while high preoperative intraocular pressure (OR = 4.54, 95% CI = 0.99-20.9, P = .052) and greater lens thickness (OR = 3.38, 95% CI = 0.88-12.91, P = .075) demonstrated a marginally significant association., Conclusions: Cataract surgery in eyes with nanophthalmos is associated with a high risk for vision-threatening complications. Performing a simultaneous prophylactic sclerostomy with cataract surgery reduces complication rates, particularly uveal effusions. Cataract surgery at earlier stages by phacoemulsification may be more beneficial than undergoing manual SICS., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Purpose: To evaluate the best piggyback intraocular lens (IOL) implantation method and the outcomes in nanophthalmos eyes and to define the postoperative structural changes., Setting: Farabi Eye Hospital, Tehran, Iran., Design: Prospective case series., Methods: Candidates for refractive surgery who were nanophthalmic were recruited. Patients had refractive lens exchange followed by implantation of both IOLs in the bag or 1 IOL in the bag and 1 IOL in the sulcus. The baseline and follow-up visual acuity, refractive status, and structural Scheimpflug imaging were evaluated. Ultrasound biomicroscopy (UBM) was performed 6 months postoperatively., Results: The study comprised 9 nanophthalmic patients (18 eyes) with a mean preoperative uncorrected distance visual acuity (UDVA) of 1.53 logarithm of the minimum angle of resolution (logMAR) ± 0.3 (SD), mean corrected distance visual acuity (CDVA) of 0.34 ± 0.2 logMAR, and mean spherical equivalent (SE) of +13.55 ± 4.0 diopters (D). The mean postoperative UDVA improved from baseline. There was no significant difference in the mean UDVA and CDVA between the 2 IOL groups. Postoperatively, both groups had a significant improvement in SE, a significant rise in anterior chamber depth and angle, and similar UBM measurements., Conclusions: Piggyback IOL implantation was an effective refractive procedure in nanophthalmic eyes. Both implantation methods resulted in similar outcomes; however, the small number of patients in each group made it less likely that possible differences would be found. The increase in angle values might help prevent the development of closed-angle glaucoma., (Copyright © 2017 ASCRS and ESCRS. Published by Elsevier Inc. All rights reserved.)

The aim of this study was to illustrate the surgical techniques and utility of stereotactic or image-guided navigation in the management of lacrimal drainage obstruction in congenital arhinia-microphtalmia syndrome and review the relevant literature. Image-guided combined external and endoscopic dacryocystorhinostomy was performed in a female, aged 16 years with congenital partial arhinia and ipsilateral microphthalmus. The lacrimal sac was bypassed to the contra lateral nasal cavity through a septal window. The surgical procedure was performed using the intra-operative optical image-guided Nav 1 Pico TM ENT navigation system with real-time intra-operative instrument geometry. Different phases of the surgical technique, adjunctive endoscopic procedures, intra-operative anatomical guidance, and utility at crucial phases of surgery were noted. A review of the literature was performed pertinent to arhinia and navigation guided lacrimal surgeries. Lacrimal bypass into the contra lateral nasal cavity even through a malformed septum is possible in partial arhinia syndromes. Detailed preoperative evaluation including 3D imaging studies, navigation guided planning of risk structures with intra-operative distance control and construction of meticulous surgical roadmaps were found to be essential factors in successful outcomes. At six months follow up after surgery, there was a complete and contiguous healed mucosal anastomosis with lacrimal system patent on irrigation and resolution of epiphora. Combined external and endoscopic approach is useful in partial arhinia syndromes. Image guidance is a very useful adjunctive tool that facilitates safe and precise surgery in the management of such complex lacrimal surgeries.

A full-term baby girl with Fraser syndrome was born with right incomplete cryptophthalmos. On examination, the globe was completely covered with skin with partially formed eyelids laterally. At 3 years of age, she underwent an evisceration with orbital implant and reconstruction of the eyelids and fornices using the pre-existing scleral remnant. Custom ocular prosthetic fitting was performed 5 weeks postoperatively. At 4 years follow up, she continued to successfully retain an ocular prosthesis.

Purpose: To provide guidelines for safe implantation of glaucoma drainage devices (GDDs) in small and pediatric eyes to avoid contact between the optic nerve (ON) and the posterior edge of the GDD plate., Methods: We developed a formula for calculating limbus-to-ON distance to estimate the available "real estate" for GDD placement in small eyes. The formula was validated using eyes of pediatric decedents undergoing clinical autopsy, with axial lengths (AL) of 15-24 mm. For each autopsy eye, we measured AL, anterior chamber depth, corneal diameter, and limbus-to-ON distances for the four eye quadrants. The main outcome measure was the degree of agreement between measured and calculated limbus-to-ON distances., Results: A total of 15 autopsy eyes were divided into derivation (n = 10) and validation (n = 5) groups. A formula was derived to estimate superotemporal limbus-to-ON distance (D ST ) using AL and corneal diameter data. Linear regression showed excellent correlation between the measured D ST and AL (R 2  = 0.98). There was excellent agreement between measured and calculated limbus-to-ON values for all four eye quadrants (R 2 range, 0.92-0.98)., Conclusions: Our formula accurately predicts limbus-to-ON distances across a wide range of clinically relevant ALs. Based on this information, GDD surgery in small eyes can be adjusted by positioning the GDD closer to the limbus or by trimming the posterior edge of the GDD plate. To our knowledge, this is the first set of guidelines developed to promote safe implantation of GDDs in small eyes., (Copyright © 2017 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

The small eye suggests an apparently robust anatomy with a more resistant sclera, good trabecular function, good uvea trophicity, a healthy retina, with a full papilla. The volume of these eyes is small. Usually, the volume of the eye is related to the sagittal diameter of the eye. However, the volume of a sphere varies with the third power of the radius of the sphere. These small eyes have a volume smaller than their sagittal diameter suggests. In this volume, highly decreased develop certain anatomical components without having to keep proportions (lens, choroid), and some have a continuous growing volume (lens). On long term, there is a balance inside these eyes despite a disproportion between their components. This internal disproportion inside the small eye can erupt through pressure differences between its structures: pupillary block, angle closure or a disproportionate response in case of typically uncomplicated surgery, which alters the apparent internal balance of these eyes. Seemingly simple surgeries, such as phacoemulsification or filtering surgery can trigger storm (storms occur by differences in atmospheric pressure) with the following characteristics: - intraocular "precipitation" in the form of uveal effusions, massive choroidal hemorrhage exudative retinal detachment, CME - breaks in anatomical barriers, lens posterior capsular tear - deviations courses: aqueous misdirection Surgical operations on these eyes are like a dangerous storm surfing, with risks, incidents, with unpredictable but great experience and courage request.

Background: Orbital cysts are rare developmental anomalies that can occur in microphthalmic and anophthalmic patients. Such cysts can promote orbital growth and subsequently markedly increase the size of the orbit, which is commonly underdeveloped in these patients. Cyst removal is therefore generally dissuaded (at least) in the first 5 years., Clinical Case: A 6-year-old boy with a microphthalmos and a cyst developed protrusion of his prosthetic eye and a swelling of the lower eyelid. MRI showed a large cyst causing distortion of the right orbit. Due to the expansive orbital growth and subsequent misfitting of the prosthesis, cystectomy and orbital floor reconstruction was performed using 3D technology., Conclusion: 3D imaging and printing enables exact delineation of orbital cysts and the adjacent bony structures. Furthermore it offers the possibility to plan an individual surgical approach and to design and fabricate a custom fit orbital floor implant.

An 11-month-old baby girl presented with white reflex in her left eye. On examination, there was a 6.5×5 mm(2)haemangioma present over her face involving on her lower lip. Systemic examinations were within normal limits. The left eye was small, with an axial length of 16.08 mm and had a cataract. Ultrasonography of the left eye was suggestive of the presence of a vascular stalk, persistent hyperplasia of a primary vitreous, or persistent fetal vasculature with vitreous haemorrhage. On MRI, the left eye was small with vitreous haemorrhage. Left eye lens aspiration was performed and the bleeding vascular stalk behind the lens was cauterised with diathermy. The right eye was normal. The patient was diagnosed as having PHACE syndrome (Posterior fossa malformations, Hemangiomas, Arterial anomalies, Coarctation of the aorta and other cardiac defects, and Eye abnormalities syndrome). On follow-up, she was able to follow light with her left eye., (2016 BMJ Publishing Group Ltd.)

Purpose: To evaluate phacoemulsification surgery results and complications in patients with nanophthalmos., Methods: The records of 9 patients with nanophthalmos who had cataract surgery from January 2011 through January 2015 were retrospectively reviewed. Nanophthalmos was diagnosed by the presence of an eye with an axial length (AL) less than 20.5 mm. The records were reviewed for ocular diagnosis, keratometry, anterior chamber depth (ACD), AL, ocular surgeries, visual acuity, and complications., Results: A total of 14 eyes of 9 patients (8 women, 1 man) with a mean age of 72 years (range 48-86) were reviewed. Mean AL was 18.72 mm and mean ACD was 2.30 mm. Mean preoperative spherical equivalent (+8.55 ± 6.44 diopters (D)) was reduced to +0.30 ± 2.17 D at last follow-up visit. Mean best-corrected visual acuity (BCVA) was +0.68 ± 0.55 logarithm of the minimum angle of resolution (logMAR) preoperatively and improved to +0.55 ± 0.73 logMAR at last follow-up visit. Visual acuity remained stable or improved in 11 eyes. Five eyes did not achieve final BCVA ≥+0.3 logMAR. Complications occurred in 3 eyes (21.4%) and included 1 case of posterior capsule rupture, 1 case of severe iritis and rhegmatogenous retinal detachment 2 weeks postoperatively, and 1 other case of iritis. No postoperative uveal effusion was seen., Conclusions: Although phacoemulsification seems to be relatively safe in nanophthalmic eyes, surgeons need to be aware of the challenges of this procedure in these high-risk eyes. Nevertheless, with careful preoperative evaluation and planning, most cases were uncomplicated and resulted in improvement of BCVA.