An Individualized 3-Dimensional Designed and Printed Conformer After Dermis Fat Grafting for Complex Sockets.

PURPOSE: To introduce a novel technique to design individually customized conformers for postenucleation sockets with dermis fat implants. METHODS: We use a 3-dimensional scan of the frontal face/orbit and eyelid contour to design an individualized conformer. This polymethylmetacrylate printed conformer is adapted to patients' socket, palpebral fissures, horizontal eyelid aperture, curvature of the eyelids, and mean diameter of patients' contralateral eye. Sutures through holes in the inferior part of the conformer and in the extension can be placed to fixate the conformer and anchor fornix deepening sutures. RESULTS: A correct fitting conformer can be printed and attached to the socket and eyelids. The shape of this conformer can be used subsequently postsurgically to design the ocular prosthesis. CONCLUSION: Presurgical planning is important to anticipate for a functional socket to adequately fit an artificial eye. The presented technique using 3-dimensional imaging, designing, and printing promises to prevent conformer extrusion and forniceal shortening.

A Novel Method to Measure Artificial Eye Motility.

PURPOSE: To measure objectively, reproducibly, and noninvasively artificial eye motility, a fundamental aspect in the (cosmetic) outcome of enucleation. METHOD: A gaze- and pupil-tracking system, the "iView X," was implemented to measure horizontal eye motility. The system, with adjusted software, was tested with patients wearing 1 prosthetic eye after enucleation for retinoblastoma. Measurements were repeated 5 times in every patient and analyses were performed twice by 2 independent observers. Reproducibility was tested via linear mixed models. After the implementation of the method, more data were obtained, including more patients with a history of enucleation for the treatment of retinoblastoma for eye tracker measurement, and differences in motility percentages between implant types and sizes were analyzed via Mann-Whitney U tests. RESULTS: The intraclass correlation coefficient of the interobserver variable in the patient test group (n = 27, 6-53 years) was 0.98 and 0.96 for measurement of left gaze and right gaze, respectively. Intraobserver variation was <0.001. In the total of 58 included patients for comparative analysis, mean difference of prosthesis motility compared with the contralateral eye in abduction was 57.1% (range 3.2-91.5%); in adduction 65.8% (range 24.0-92.0%). No statistical differences were found between the different implant types and sizes. CONCLUSION: Motility measurements of the prosthetic eye in comparison to the contralateral eye using the iView X system are reproducible and reliable. This is, to the authors' knowledge, the first easy applicable, noninvasive, reproducible, and commercially available instrument to evaluate prosthesis motility. With the adjusted software program (freely available on request), a similar objective measurement can be performed worldwide. The implant size or type did not influence the outcome of the motility measurement; this finding deserves additional study.

Parallax-free exophthalmometry: a comprehensive review of the literature on clinical exophthalmometry and the introduction of the first parallax-free exophthalmometer.

PURPOSE: To present the first parallax-free exophthalmometer design. BACKGROUND: Exophthalmometry is an important clinical tool. We provide a historic overview of clinical exophthalmometer designs, and we review current problems encountered in exophthalmometry. METHODS: We present a new and parallax-free exophthalmometer design that we have evaluated in 49 patients visiting our orbital clinic. RESULTS: The mean age of the patients was 49.8 years and 72% were female. The Pearson interobserver variation was 0.97, and 94% of the Hertel values measured by the two observers were within the limits (1.6 mm) of agreement. CONCLUSION: This meter appears to be a reliable instrument for exophthalmometry. It is the first instrument that allows for a complete parallax-free measurement.

Classification for treatment urgency for the microphthalmia/anophthalmia spectrum using clinical and biometrical characteristics.

PURPOSE: Current clinical classifications do not distinguish between the severity of the MICrophthalmia/Anophthalmia (MICA) spectrum with regard to treatment urgency. We aim to provide parameters for distinguishing mild, moderate and severe MICA using clinical and biometrical characteristics. METHODS: We performed a single-centre, cross-sectional analysis of prospective cohort of 58 MICA children from September 2013 to February 2018 seen at the Amsterdam University Medical Center, The Netherlands. All patients with a visible underdeveloped globe were included. We performed full ophthalmic evaluation including horizontal palpebral fissure length, axial length by ultrasound and/or MRI measurements, paediatric and genetic evaluation. Cases were subdivided based on clinical characteristics. Biometrical data were used to calculate the relative axial length (rAL) and the relative horizontal palpebral fissure length (rHPF) compared with the healthy contralateral eye for unilateral cases. RESULTS: In previously untreated patients, a strong correlation exists between rAL and rHPF, distinguishing between severe, moderate and mild subjects using rAL of 0-45%, 45-75% and 75%-100%, respectively. Clinical subgroups were randomly dispersed throughout the scatterplot. CONCLUSION: Current classifications lack clinical implications for MICA patients. We suggest measuring eyelid length and axial length to classify the severity and determine treatment strategy. The 'severe' group has obvious asymmetry and abnormal socket configuration for which therapy should quickly be initiated; the 'moderately' affected group has normal socket anatomy with a microphthalmic eye with disturbing asymmetry for which treatment should be initiated within months of development; the 'mild' group has a slightly smaller axial length or less obvious eyelid asymmetry for which reconstructive correction is possible, but expansive conformer treatment is unnecessary.

Cosmetic results of enucleation and/or external beam radiation therapy in 195 retinoblastoma survivors.

PURPOSE: To report the cosmetic outcome and late effects of enucleation and/or irradiation for retinoblastoma and to evaluate the role of orbital implants. METHODS: Patients (age >4 years) enucleated and/or irradiated for retinoblastoma, visiting the hospital for routine follow-up (April 2013 to May 2015), were included in our cross-sectional study. Data were obtained via clinical records, questionnaires, physical measurements and standardized photographs. Two independent observers scored the cosmetic features: upper eyelid position, lower eyelid position, volume deficiency, and prosthesis motility and overall cosmetic appearance. RESULTS: A total of 195 patients participated. Ptosis was seen in 45 (28.3%) patients, lower lid sagging in 45 (28.3%). Both complications were associated with the orbital implant; ptosis being more common with increasing implant size (5.6% without implant, 16.7% with small implant, 37.1% with medium implant and 76.9% with large implant) and lower lid sagging being more common in patients without implant (20% with implant versus 37.5% without). Volume loss of the superior sulcus was seen in 107 sockets (66.9%), most frequent in additionally irradiated patients (χ2 (2) = 42.7, p < 0.001) and in patients without implant (χ2 (2) = 11.92, p = 0.003). Prosthesis motility was better in patients with orbital implant, regardless of the size. CONCLUSION: Minor late and potentially treatable effects were seen in patients with implant. Larger sized implants were associated with a higher incidence of ptosis; implant size did not affect subjective outcome with respect to volume or prosthetic motility. Treatment with EBRT had a less favourable outcome.

Persistent socket pain postenucleation and post evisceration: a systematic review.

PURPOSE: To investigate causes, diagnostics and treatment modalities for persistent socket pain (PSP) after enucleation and evisceration. METHODS: A systematic search was undertaken in accordance with the PRISMA Statement, in PubMed, Embase.com and Thomson Reuters/Web of Science. We searched for relevant papers until the 28th of July 2016. Inclusion criteria were (1) patients with a history of enucleation or evisceration, (2) PSP, (3) report of the cause and/or used diagnostics and/or treatment modality, (4) full text in English, Dutch or Spanish language. Excluded were (1) review articles, (2) comments, and publications concerning, (3) nonhumans, (4) exenterated patients, (5) acute postoperative pain, or (6) periorbital pain without pain in the socket. Given the lack of high quality evidence from randomized controlled trials, we examined all available evidence from primary observational studies and assessed quality within this lower level of evidence. RESULTS: A total of 32 studies were included. Causes of PSP found were prosthesis-related (n = 5), dry socket (n = 2), trochleitis (n = 3), compression of the trigeminal nerve (n = 2), implant-related (n = unknown), inflammation (n = 5), surgery-related (n = 4), neuromas (n = 8), malignant tumours (n = 3), psychiatric/psychosocial (n = 2), phantom pain (n = 149), rarer entities (n = 3) or unknown (n = 14). Nonsurgical treatments suffice for conditions as trochleitis, prosthesis-related pain, dry socket and for phantom pain. Other causes of pain may require more invasive treatments such as implant removal. CONCLUSION: Careful history and examination can give some direction in the diagnostic procedure; however, PSP is probably multifactorial and the specific origin(s) may remain uncertain. Implant replacement can be an effective treatment. Studies to identifiy less invasive procedures are required.

Case series: effect of dermis-fat implants in different late onset socket problems encountered in retinoblastoma patients.

BACKGROUND: Cosmetic dissatisfaction, pain, and chronic discharge may present months till years after enucleation in patients operated because of retinoblastoma. If noninvasive treatment modalities are insufficient, socket reconstruction can be considered. In this study, we discuss the results of dermis-fat exchange to treat these problems. METHOD: Four patients with late onset post enucleation socket problems with a request for treatment were included in this prospective study. Socket inspection was documented and pictures at baseline and at a follow-up of at least 6 months were taken. To quantify the problem 'pain', a VAS score at baseline and at follow up was used. For the problem 'cosmetic dissatisfaction' standardized questionnaires were used. RESULTS: Two patients were included because of cosmetic dissatisfaction; one was included with chronic pain and one with chronic discharge. Reconstruction of the socket using autologous dermis-fat insertion was done in all four. In one of them, severe shrinking of the fat developed. This patient was treated with additional injectable fillers. Both of them, ultimately, had satisfactory results. Autologous fat transplantation also solved the problem of chronic discharge and pain in the two other patients. CONCLUSION: Socket reconstruction by autologous dermis-fat exchange may solve different post enucleation socket problems. However, shrinking of the transplanted fat may occur and require additional procedures.

Discharge and infection in retinoblastoma post-enucleation sockets.

PURPOSE: To investigate the causes and treatment options for socket discharge and infection in patients enucleated for retinoblastoma (Rb). METHODS: A questionnaire was filled out by (parents of) ocular prosthesis-wearing patients with a history of enucleation as treatment for Rb. We collected data on patients' characteristics, cleaning habits of the prosthesis, frequency of socket irritation, discharge, and infection, and use of antibiotics. With ordinal logistic regression analysis, factors related to the outcome parameters (frequency of irritation, mucoid and purulent discharge) were identified. In a subset of young asymptomatic and symptomatic patients, a swab culture of the socket was performed to determine the presence of microorganisms. RESULTS: A total of 186 patients or their parents (mean age of the patients: 17.3 years, ranging from 0.8 to 88.3 years) filled out the questionnaire. Irritation, mucoid discharge, and purulent discharge were frequently (once a month or more often) experienced in 75 (39.5%), 127 (66.8%), and 15 (13.2%) sockets, respectively. Younger age was associated with a higher frequency of mucoid and purulent discharge. Radiation therapy, chemotherapy, gender, age at surgery, cleaning frequency, and nocturnal wear were not associated with the outcome parameters. In a subgroup of 26 patients, the sockets were swabbed and cultured. All symptomatic patients had a positive bacterial culture versus 15% (2/13) of the asymptomatic patients (P<0.001). Common cold was correlated with both symptoms and presence of bacteria. Haemophilus influenzae and Staphylococcus aureus were the species most frequently cultured. CONCLUSION: Ocular prosthesis-wearing patients often experienced mucoid discharge, and less often irritation and socket infection. These complaints were found to decrease with increasing age, but did not seem to be influenced by cleaning or wearing habits. Symptomatic sockets, with and without discharge, were correlated with the presence of pathogenic bacteria for which local antibiotic treatment seemed effective in most cases.

Orbital implants in retinoblastoma patients: 23 years of experience and a review of the literature.

PURPOSE: To evaluate complications of different types of orbital implants following enucleation for retinoblastoma. METHODS: We performed a retrospective chart study of all patients that underwent enucleation as treatment of retinoblastoma between April 1991 and June 2013. Events of implant exposure, extrusion (defined as a complete loss of the implant, or a major exposure that could not be closed) and socket abnormalities were analysed for association with implant type and influence of additional external beam radiation therapy (EBRT) and/or chemotherapy. RESULTS: A total of 224 enucleations in 216 patients (eight bilateral) were identified. Mean age at surgery was 1.9 (median 1.5) years. Of the 219 included enucleated eyes, 20 were not replaced by a primary implant and 18 were replaced by an Allen implant. Scleral wrapped hydroxyapatite (HA) and acrylic implants (polymethylmethacrylate) were inserted in, respectively, 79 and 102 cases. In the total population, 29 treatment or implant-specific events (13.2%) were registered. Main complications were implant exposure n = 10 (4.6%) and extrusion n = 6 (2.7%). The acrylic/sclera group had less exposures or extrusions (5 of 102, 4.9%) compared to the HA/sclera group (10 of 79, 12.7%), although this difference did not quite reach statistical significance (p = 0.06). Additional treatment (chemotherapy and/or EBRT for the fellow eye) was administered in 78 cases (35.8%). The overall complication rate in the entire study population was significantly higher (16.7% versus 5.7%) in the group exposed to additional therapy (OR 3.3; 95% CI 1.30-8.36 p = 0.008). This negative effect of additional therapy was also significant in the combined acrylic/HA group (OR 2.9; 95% CI 0.97-8.46 p = 0.048). CONCLUSION: Our results suggest a favourable outcome for acrylic implants compared to the HA implant. Additional treatment with chemotherapy and/or EBRT is associated with an increased risk of complications.

Worldwide enucleation techniques and materials for treatment of retinoblastoma: an international survey.

PURPOSE: To investigate the current practice of enucleation with or without orbital implant for retinoblastoma in countries across the world. METHODS: A digital survey identifying operation techniques and material used for orbital implants after enucleation in patients with retinoblastoma. RESULTS: We received a response of 58 surgeons in 32 different countries. A primary artificial implant is routinely inserted by 42 (72.4%) surgeons. Ten (17.2%) surgeons leave the socket empty, three (5.2%) decide per case. Other surgeons insert a dermis fat graft as a standard primary implant (n=1), or fill the socket in a standard secondary procedure (n=2; one uses dermis fat grafts and one artificial implants). The choice for porous implants was more frequent than for non-porous implants: 27 (58.7%) and 15 (32.6%), respectively. Both porous and non-porous implant types are used by 4 (8.7%) surgeons. Twenty-five surgeons (54.3%) insert bare implants, 11 (23.9%) use separate wrappings, eight (17.4%) use implants with prefab wrapping and two insert implants with and without wrapping depending on type of implant. Attachment of the muscles to the wrapping or implant (at various locations) is done by 31 (53.4%) surgeons. Eleven (19.0%) use a myoconjunctival technique, nine (15.5%) suture the muscles to each other and seven (12.1%) do not reattach the muscles. Measures to improve volume are implant exchange at an older age (n=4), the use of Restylane SQ (n=1) and osmotic expanders (n=1). Pegging is done by two surgeons. CONCLUSION: No (worldwide) consensus exists about the use of material and techniques for enucleation for the treatment of retinoblastoma. Considerations for the use of different techniques are discussed.