The current alternative for ocular surface and anophthalmic socket reconstruction, cryopreserved umbilical amniotic membrane (cUAM).

PURPOSE: This report presents the results of using cryopreserved umbilical amniotic membrane (cUAM) as an alternative mucosal graft for ocular surface reconstruction in cases of anophthalmic socket contracture (ASC), cicatricial entropion (CE), and conjunctival-scleral defects. METHODS: The study included patients who underwent non-commercial implantation of cUAM grafts (prepared by corneal banking methods) for ASC, CE, conjunctival defect, and scleral melting. The main success criteria for this study were the comfortable fitting of the ocular prosthesis in ASC patients, the natural eyelid position in CE patients, and the degree of conjunctivalisation in melting patients. RESULTS: cUAM transplantation was performed in 2 patients who could not use a prosthetic eye due to conjunctival contracture, 2 patients with CE, and 1 patient with conjunctival defect and 1 patient with conjunctival-scleral melting. The primary outcome was achieved in 83.3% (5/6) of patients. In one patient with CE, partial healing was achieved due to the persistence of CE in the medial upper eyelid. CONCLUSIONS: cUAM is a viable alternative to mucosal grafting for reconstructing the bulbar and palpebral conjunctival surface, fornix, and orbit, with reduced donor morbidity and shorter surgical time. Its regenerative ability allows for tissue defect healing and improves cosmetic appearance through epithelialization within weeks.

Factors affecting anophthalmic socket reconstruction outcomes using autologous oral mucosal graft.

BACKGROUND: Limited studies have reported surgical outcomes that are defined by strict criteria following grade 2 or 3 socket reconstruction using an oral mucosal graft (OMG). We aimed to determine factors influencing surgical outcomes of anophthalmic socket reconstruction using OMG in patients with grade 2 or 3 socket contractures. METHODS: Thirty-seven patients who underwent socket reconstruction with autologous OMG between January 2007 and December 2017 were retrospectively analyzed. The successful outcome was defined as an eye prosthesis wearing without experiencing displacement and the absence of any re-operations or additional surgeries following socket reconstruction. Factors affecting surgical outcomes were identified using multivariate analysis. RESULTS: A total of 15 male and 22 female patients (mean age: 40.2 ± 17.2 years) were included. The median duration of socket contracture was 21.5 years. Grade 2 and 3 socket contractures, based on Tawfik's classification, were reported in 20 and 17 patients, respectively. Twenty-eight and eight patients underwent socket reconstruction using OMG alone and OMG combined with a hard palate graft, respectively. The success rates of grades 2 and 3 socket contracture reconstruction were 80.0% and 52.9%, respectively. Multivariate analysis demonstrated that only grade 3 contractures were predictive of worse outcomes. At the final visit (mean follow-up: 6.3 years), 34 patients (91.9%) could wear their eye prostheses. CONCLUSIONS: Socket reconstruction using autologous OMG can provide acceptable results in grade 2 and 3 contractures; however, satisfactory results were more significantly reported in grade 2 than in grade 3 contractures.

Stepwise self-inflating hydrogel expansion for congenital anophthalmia and blind microphthalmia: Over 15 years' experience in China.

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.

Long-Term Outcome of Staged Socket Surgery for Acquired Anophthalmos.

PURPOSE: To evaluate long-term outcomes of staged volume rehabilitation for acquired anophthalmos. METHODS: Case-note review of patients who had preceding i) eye removal without implant, ii) eye removal with an intraconal implant, but ball-related problems, or iii) removal of exposed implant. Secondary interventions were a) a first-time ball implant, b) dermis-fat graft, c) ball repositioning, d) ball replacement after prior removal, or e) orbital floor implantation. RESULTS: Secondary volume-augmenting surgery was performed in 175 sockets at a mean age of 42.7 years (range 2-91), with 62% secondary ball implants, 3% dermis-fat grafts, 6% ball repositioning, 19% ball replacement after prior removal for exposure, and 10% having orbital floor implantation. After this surgery, further volume enhancement was required in 21% of sockets, this being 40% for spheres ≤18 mm diameter, in contrast to 6% for those ≥20 mm ( p < 0.001). Exposure or malposition of the secondary implant occurred in 8% (12/151) and was unrelated to implant type, size, wrapping, or prior irradiation. Tertiary surgery addressed lining deficiency (18%) or eyelid malposition (25%). Overall, 92/175 (53%) had tertiary surgery to improve cosmesis and comfort, with 49% (36/92) being related to small implants. At a mean follow-up of 9.1 years, 82% of sockets had adequate volume, 79% had excellent lining, and 93% were comfortable. Prosthetic fit was satisfactory in 96% of cases, and 97% reported improved cosmesis. CONCLUSION: Over half of the sockets having planned 2-stage volume enhancement may need further procedures, especially after small-volume secondary implants, but, with meticulous surgery, reasonable long-term results can be achieved with few complications.

Anophthalmic socket in retinoblastoma: Exploring complications and risk factors in a tertiary centre in Malaysia.

PURPOSE: To evaluate the complications of anophthalmic socket in retinoblastoma patients at a tertiary centre in Malaysia. DESIGN: Retrospective study. METHODS: Patients who underwent enucleation for retinoblastoma were reviewed from 2004-2020. Details were recorded, including demographics, diagnosis, surgical techniques, implant types, additional therapies, and complications. RESULTS: Of 250 patients with retinoblastoma managed over the period, the anophthalmic sockets of 160 eyes who underwent enucleation were analysed. The mean age at enucleation was 2.03 years (26 days to 9.18 years). The follow-up periods after enucleation range from 5 days to 16.83 years. Porous polyethylene (Medpor) implants were used in 135 patients (84.4%), as were Bioceramic in 9, glass balls in 7, acrylic in 7, dermis fat grafts in 1, and silicone implants (Aurosphere) in 1. The overall complications in our study were 28.8%. Complications seen in the study included implant exposure (12.5%), shallow inferior fornix (10.6%), granuloma formation (3.1%), discharge (2.5%), implant migration (1.9%), ptosis (0.6%), and orbital dystopia (0.6%). Implant exposure is solely found in Medpor, more common in those with donor sclera caps, and exposure times range from 28 days to 11.42 years. The suturing of the Tenon and conjunctiva in separate layers significantly reduced the rate of implant exposure. Six out of seven radiation patients had shallow inferior fornixes. CONCLUSIONS: Long-term post-enucleation complications were not uncommon. Luckily, most had good outcomes, with a few needing surgical intervention. Meticulous suturing technique on the Tenon and conjunctival layer is essential to prevent implant exposure.

Improved Reverse Postauricular Island Flap for Repair of Lower Conjunctival Fornix Retraction.

ABSTRACT: Repair of lower conjunctival fornix retraction is a great challenge for plastic surgeons in the aspects of orbital prosthesis retaining and cosmetic appearance. Hereby, a 25-year-old woman, suffering from lower conjunctival fornix retraction after the removal of retinoblastoma and radiotherapy, was undertaking the treatment with an improved reverse postauricular island flap for expansion of the lower conjunctival fornix, and volume augmentation of the anophthalmic socket in one stage. The flap was based on the parietal branch of the superficial temporal artery and its vascular architecture to the postauricular vascular system, and successfully transferred by passing through a subcutaneous tunnel. The satisfactory result indicated that the mentioned technique may be a good option in surgical rehabilitation of the stable depth of the lower conjunctival fornix, not only from the reliable blood supply and the suitable thickness of the flap but also from the less donor morbidity without an exposed unsightly scar. LEVEL OF EVIDENCE: Level V.

Clinical Characterization of Congenital Anophthalmic and Microphthalmic Cavities in Inidviduals With Craniofacial Anomalies.

OBJECTIVE: Measure the frequency of anophthalmic and microphthalmic patients with craniofacial anomalies (FCAs). DESIGN: Descriptive, cross-sectional, retrospective study. SETTING: Hospital for Rehabilitation of Craniofacial Anomalies of the University of São Paulo (HRAC-USP). The medical records of patients treated at HRAC from 2000 to 2012 with a diagnosis of congenital anophthalmia or microphthalmia were examined. Patients were excluded for secondary anophthalmia, incomplete medical records, or information that could not be accessed. OUTCOME MEASURES: Frequency of anophthalmia and microphthalmia; the proportions and diagnoses of associated FCAs; impairment of ocular appendages; extracranial or facial anomalies; genetic alterations; and surgical approach. RESULTS: A total of 56 patients had anophthalmia (52.3%), 35 had microphthalmia (32.7%), and 16 patients had both (15%). Individuals with FCAs associated with microphthalmia, anophthalmia, or both totaled 74, corresponding to 69.2%. Anophthalmia was more likely than microphthalmia to be accompanied by FCAs, at 76.4% of patients ( P < 0.05). Cleft lip and palate were the main malformations associated with anophthalmia (23.64%), with microphthalmia (45%), and with both (44.44%). Reconstructive surgery was done in 63.6% of cases. The ocular attachments were compromised in 71% of cases. Extracraniofacial malformations were found in 9.3% of patients. Only 7 records contained karyotypes, and no changes directly related to anophthalmia or microphthalmia were found. CONCLUSION: Anophthalmia is more frequent than microphthalmia and is more often accompanied by FCA. Cleft lip and cleft palate are the most frequent concomitant malformations.

Management of external ocular prosthesis by ocularists: results of an online survey conducted in Brazil and Spain.

PURPOSE: To analyse the ocularist's perspective on the management of the anophthalmic socket and external ocular prosthesis (EOP). METHODS: Ocularists from two countries were invited to participate in an online questionnaire. Data were collected on demographics, anophthalmic socket and EOP management (manufacturing, use, cleaning), complications, follow-up visits and multidisciplinary care. The frequency and proportions of the responses were statistically analysed. RESULTS: The questionnaire was addressed to 20 Brazilian and 17 Spanish ocularists, obtaining a response rate of 65% and 64.7%, respectively. 62.5% of respondents were men. The most common cause of anophthalmia in Brazil (69.2%) and Spain (36.4%) is an eye disease (chi square: p = 0.188). Polymethylmethacrylate (PMMA) is the most commonly used material in EOP manufacture (chi square: p = 0.448), and 70.8% reported using customized EOPs (chi square: p = 0.069). Deposits are frequently observed in both countries (chi square: p = 0.157). Changing the prosthesis is recommended after 5 to 10 years by Brazilian ocularists, and after less than 5 years of use by Spanish ocularists (81.8%) (chi square: p = 0.041). Annual follow-up is recommended by Spanish ocularists (45.5%), while semestral (38.5%) and case-dependent (38.5%) follow-up is recommended by Brazilian ocularists (chi square: p = 0.267). Daily cleaning is advocated by 61.5% of Brazilian ocularists and once a month by 45.5% of Spanish ocularists (chi square: p = 0.098), with 75% of ocularists from both countries not recommending EOP removal at night (Fisher´s exact test: p = 0.166). Good communication between ocularists and ophthalmologists was reported by 87.5% of our responders (chi square: p = 0.642). CONCLUSION: Although there are no unified protocols on the management of EOPs, Brazilian and Spanish ocularists follow similar guidelines. Differences between countries were the patients´ referral and the prosthesis´ useful life.

Reconstruction of Anophthalmic Sockets With Pedicled Nasoseptal Flap.

PURPOSE: Patients who undergo eye removal often present with orbital soft-tissue insufficiency and contraction of the eye sockets. The most commonly used reconstruction strategy is grafting the orbit with free grafts, which is associated with the drawback of harvesting tissue from an unconnected site. This study describes the use of the vascularized nasoseptal flap in the reconstruction and enlargement of the contracted anophthalmic cavity in patients with severe or recurrent contracted eye sockets and evaluates its efficacy. METHODS: A sphenopalatine-pedicled flap from the nasal septum was harvested and mobilized into the anophthalmic orbit for the reconstruction, coverage, and enlargement of the socket in 17 patients with anophthalmic socket syndrome. Data regarding the demographics, preoperative status, postoperative findings, follow-up, outcomes, dates of mutilant and reconstructive surgery, and relevant clinical or imaging were collected. RESULTS: Krishna´s classification was used to assess the postoperative outcomes. The final rating improved in all patients at a median follow-up duration of 35 months. A greater impact was observed in patients who underwent reconstructive surgery before nasoseptal flap creation. Two minor complications occurred; however, major surgical intervention was not required. Implant extrusion was observed in 2 patients. CONCLUSIONS: The novel strategy of applying nasoseptal flaps in the reconstruction of anophthalmic sockets results in improved socket grading and a low rate of recurrence (socket contracture or implant extrusion), and complications. The vascular nature of the flap makes it suitable for use in complex cases.

[Ocular prosthetics in children-Possibilities and challenges]. / Augenprothetik beim Kind ­ Möglichkeiten und Herausforderungen.

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.

Motility of the Ocular Prosthesis in Anophthalmic Patients: Objective and Patient-perceived Findings.

AIM: To study the impact of the prosthesis motility on the Quality of Life (QoL) in anophthalmic patients. METHODS: Cross-sectional, observational study of 100 anophthalmic patients, of whom 64% had an acryl implant, 6% an Allen implant, 1% a hydroxyapatite implant, 4% a dermis fat graft, 16% no implant, and 9% an unknown implant. We quantitatively assessed the motility of the prosthesis with Kestenbaum glasses and the QoL with a validated questionnaire covering five domains: General functional abilities and care, wearing comfort, physical appearance, psychological and social functioning. Associations between measured prosthetic eye motility, patient-perceived motility, and satisfaction were made. RESULTS: Motility of the prosthesis was impaired with an average loss of 76%, and correlated with Cosmetic satisfaction (adduction P = .02, abduction P = .008, elevation P = .04) and Social satisfaction (adduction P = .03, abduction P = .003). The patient-perceived motility of the prosthesis correlated with General functioning abilities (horizontal P = .0004, vertical P = .0004), Comfort (horizontal P = .001, vertical P = .003), Cosmetic satisfaction (horizontal P = .0002, vertical P = .0002), Psychological satisfaction (horizontal P = .001, vertical P = .001), and Social satisfaction (horizontal P = .002, vertical P = .003). CONCLUSIONS: Ocular prosthetic motility has a significant impact on patient-perceived satisfaction and physical appearance, and predicts coping with the prosthetic condition on the psychosocial level. This highlights the need of introducing patient-reported outcome measures in the prosthetic rehabilitation of the anophthalmic patient.

Nasal turbinate mucosal graft for management of contracted anophthalmic socket.

A 67-year-old Caucasian male presented with severe contraction of socket lining 8 years after enucleation, dermis fat graft and successful ocular prosthesis fitting. Following two failed attempts at using amniotic membrane grafts to reform the socket lining, a total socket reconstruction was attempted using a novel nasal turbinate mucosal graft technique. This was performed in a staged fashion with lower fornix reconstruction followed by upper fornix reconstruction 3 months later. The patient was stable at 12 months review, with a satisfactory cosmetic outcome. Nasal turbinate mucosa was used as it was surgically accessible, provided natural socket lubrication due to its mucosal surface, and avoided oral mucosa and its associated morbidity. This case report suggests that nasal turbinate mucosa is a suitable autologous grafting material for total socket reconstruction in contracted anophthalmic sockets.

Congenital Orbital Glial Heterotopia Associated with Clinicopathological Anophthalmia: A Case Report.

Glial heterotopia (GH) is the presence of glial tissue outside the cranial cavity, without communication with the brain. The orbital location usually presents as eyelid swelling, strabismus, and proptosis. This is considered a congenital location that usually presents at birth. Its association with anophthalmia is uncommon. We report the case of a 2-day-old male neonate with left congenital intraorbital lesion presenting with massive proptosis. No eyeball could be seen. Preoperative magnetic resonance imaging disclosed a large and predominantly cystic mass occupying and protruding from the left orbit without intracranial extension. In the operating theatre, a large amount of fluid was aspirated prior to total resection of the mass. Chemical analysis of the fluid was compatible with cerebrospinal fluid. Histologically, the tumor was composed of mature neuroglial tissue and ependymal cells. Despite multiple sections, no choroid plexus or intraocular content was found. The diagnosis of GH with anophthalmia was made.

Treatment strategies and long-term outcomes in patients with congenital microphthalmia-anophthalmia with cyst.

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.

Modified Treatment Of Shallow Fornix For Better Retention Of Ocular Prosthesis.

Background: The purpose of this study is to share the experience of a modified surgical technique for treating shallow inferior fornix to enhance the retention of cosmetic ocular prosthesis in a tertiary care hospital. It was a Quasi experimental study, carried out at Khalid Eye Hospital, Karachi, Pakistan from January to December 2018. Methods: This study included twenty-five patients between the ages of 20-40 years belonging to either gender having an anophthalmic socket with shallow inferior fornices unable to retain cosmetic ocular prosthesis. Three pieces of Silicone tube were placed deep within the inferior fornix with the help of 2-0 Vicryl suture, taking bite of the inferior orbital rim periosteum. Main outcome measure was deepening of the inferior fornix with better retention of the ocular prosthesis. All the patients were followed up for a period of one year after the surgery. Study dynamics were briefed to all the patients and study approval obtained from the hospital ethical review committee. Results: This study included a total of twenty-five patients of both genders. Mean age was 29.6±6.53 years. By the end of the follow up period, twenty-four (96%) out of the twenty-five patients were satisfied with a proper retention of ocular prosthesis within the fornix along with complete lid closure. One (4%) patient required revision surgery because of extrusion of the silicone tube piece after one month. Mean follow up period was 358.5±16.76 days. Conclusion: This modified technique does not require expensive consumables and proved to be effective in terms of cosmetic and functional outcome.

Socket expansion with conformers in congenital anophthalmia and microphthalmia.

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.