Skeletal changes after midface surgery in patients with craniofacial deformities: a three-dimensional quantification method.

To determine the skeletal changes after midface surgery in patients with syndromic craniosynostosis who underwent Le Fort III (LFIII), monobloc (MB), or facial bipartition (FB). This was a retrospective study including 75 patients: 33 treated by LFIII, 29 by MB, and 13 by FB. Twenty-five had a diagnosis of Apert, 39 Crouzon, and 11 craniofrontonasal syndrome. A three-dimensional mesh was created from the preoperative scan and registered to the postoperative scan to visualise the advancement. LFIII at age 7-12 years effectuated a higher mean advancement in the maxillary (15.5 mm) and zygomatic (7.6 mm) regions when compared to ≥13 years (10.2 mm and 5.5 mm). After MB, mean advancement of the fronto-orbital region was higher at <7 years (16.4 mm), and similarly lower at ages 7-12 (13.8 mm) and ≥13 (12.5 mm). The mean preoperative inter-dacryon distance (34.4 ± 4.4 mm) was reduced by 8.7 ± 4.2 mm after FB without distraction (n = 10). More advancement was seen when midface surgery was performed at a younger age, due to more severe cases and a desire for overcorrection. The highest mean advancement was observed in the fronto-orbital region. Antero-inferior rotational movement was seen after all three techniques.

Detection of visual field defects using Eye Movement Pediatric Perimetry in children with intracranial lesions: feasibility and applicability.

The study aimed at evaluating the feasibility of Eye Movement Pediatric Perimetry (EMPP) among children in detecting Visual Field Defects (VFDs) associated with Intracranial Lesions (IL). Healthy controls (n = 35) and patients diagnosed with IL (n = 19) underwent a comprehensive clinical evaluation followed by a Goldmann Visual Field (GVF) and a customised EMPP protocol. During EMPP, all the participants were encouraged to fixate on a central target and initiate Saccadic Eye Movement (SEM) responses towards randomly appearing peripheral stimuli. The SEM responses were recorded using an eye-tracking device and further inspected to calculate Performance Scores (PS), Saccadic Reaction Times (SRTs), and an EMPP Index (EMPI). The mean age (years) of the controls and cases were 7.3 (SD: 1.5) and 9.4 (SD: 2.4) respectively. Among the controls, the older children (≥7 years) showed statistically significantly faster SRTs (p = 0.008) compared to the younger group. The binocular EMPP measurements compared between the controls and the cases revealed no statistically significant differences in PS (p = 0.34) and SRT (p = 0.51). EMPP failed in 4 children because of data loss or unacceptably poor PS whereas GVF failed in 7 children due to unreliable subjective responses. Of the 16 reports, with regard to the central 30-degree VF, 63% of the outputs obtained from both methods were comparable. EMPP is a reliable method to estimate and characterise the central 30-degree VF in greater detail in children with IL. EMPP can supplement the conventional methods, especially in those children who fail to complete a long duration GVF test.

Ocular and adnexal anomalies in craniofacial microsomia: Type and prevalence in a multicentre cohort study.

The aim of this multicentre retrospective cohort study was to describe and categorize the types of ocular and adnexal anomalies seen in patients with craniofacial microsomia (CFM) and to determine their prevalence. In addition, the relationship between the OMENS-Plus and Pruzansky-Kaban classification for each patient and the presence of ocular anomalies was investigated. A total of 881 patients with CFM from four different craniofacial centres were included. Data on ocular anomalies were gathered from the patient charts. Ocular anomalies were present in 33.9% of patients. Four subgroups of ocular and adnexal anomalies were identified. Type I ocular anomalies were present in 22.2%, type II in 19.0%, type III in 18.4%, and type IV in 14.5%. Several potentially preventable and treatable ocular anomalies were identified. Higher OMENS-Plus classification orbit and soft tissue scores and Pruzansky-Kaban classification mandible scores were associated with an increased risk of ocular anomalies. Based on these results and the clinical implications ocular anomalies may have, we underline the importance of targeted ophthalmological screening in CFM. Healthcare professionals should be aware of the possibility of ocular anomalies in these patients, especially during the critical period for visual development.

Ocular and adnexal anomalies in craniofacial microsomia: a systematic review.

Ocular anomalies may occur in craniofacial microsomia (CFM). The aim of this systematic review was to review the literature on ocular anomalies and their incidence, in order to estimate the need for ophthalmological screening in CFM patients. Online databases were searched, and data on the number of patients, type and incidence of ocular anomalies, and visual acuity were extracted. Four subgroups of ocular and adnexal anomalies were identified, to provide an overview of the different anomalies. Twenty-five papers analysing 1419 patients in total were included. Ocular anomalies were documented in 6.7-100% of patients. The most reported type I ocular anomalies were eyelid coloboma, lipodermoids, and orbital dystopia. The most reported type II ocular anomalies were epibulbar dermoid, microphthalmia, and anophthalmia. Ptosis and strabismus were the most reported type III anomalies, and irregular astigmatism was the most reported type IV ocular anomaly. Visual impairment in general was reported in 8-71.4% of patients, with severe visual impairment in 11.1-71.4% and amblyopia in 16.3%. This study provides a detailed overview of ocular anomalies in CFM and their prevalence. Furthermore, we propose a new classification to organize ocular anomalies into four clinically relevant subtypes. Finally, the high prevalence of ocular anomalies and visual impairment in this study suggests that CFM patients should undergo ophthalmological screening at least once during the sensitive period.

Implementation of an Educational Cartoon ("the Patchbook") and Other Compliance-Enhancing Measures by Orthoptists in Occlusion Treatment of Amblyopia.

PURPOSE: This implementation study evaluated orthoptists' use of an educational cartoon ("the Patchbook") and other measures to improve compliance with occlusion therapy for amblyopia. METHODS: Participating orthoptists provided standard orthoptic care for one year, adding the Patchbook in the second year. They attended courses on compliance and intercultural communication by communication skills training. Many other compliance-enhancing measures were initiated. Orthoptists' awareness, attitude, and activities regarding noncompliance were assessed through interviews, questionnaires, and observations. Their use of the Patchbook was measured. The study was performed in low socio-economic status (SES) areas and in other areas in the Netherlands. It was attempted to integrate education on compliance into basic and continuing orthoptic training. RESULTS: The Patchbook was used by all 9 orthoptists who participated in low-SES areas and 17 of 23 orthoptists in other areas. Courses changed awareness and attitude about compliance, but this was not sustained. Although orthoptists estimated compliance during patching at 70%, three-quarters never suspected noncompliance during a full day of observation in any of their patients. Explanations to parents who spoke Dutch poorly were short. In the second year, explanations to children were longer. Implementation of all 7 additional compliance-enhancing measures failed. Education on compliance was not integrated into orthoptists' training. CONCLUSION: Almost all orthoptists used the Patchbook and, as another study demonstrated, it proved to be very effective, especially in low-SES areas. Duration of explanation was inversely proportional to parents' fluency in Dutch. Noncompliance was rarely suspected by orthoptists. Although 7 additional compliance-enhancing measures had been conceived and planned with the best intentions, they were not realized. These required extra, unpaid time from the orthoptists, which is especially scarce in hospitals in low-SES areas where the educational cartoon is most needed.

Randomised comparison of three tools for improving compliance with occlusion therapy: an educational cartoon story, a reward calendar, and an information leaflet for parents.

BACKGROUND: We previously demonstrated that compliance with occlusion therapy for amblyopia was improved by the use of an educational programme, especially in children of parents of foreign origin and who spoke Dutch poorly. The programme consisted of: (i) a cartoon story for amblyopic children that explained without words why they should patch, (ii) a calendar with reward stickers, and (iii) an information leaflet for parents. In the current study, we assessed the individual effect of each component on compliance. METHODS: We recruited 120 3- to 6-year-old children who lived in a low socio-economic status (SES) area in The Hague and were starting occlusion therapy for the first time. They were randomised to receive one of the components (three intervention groups), or a picture to colour (control group). The randomisation was blinded for treating orthoptist and researcher. Compliance was measured electronically using the Occlusion Dose Monitor (ODM). Primary outcome was percentage of compliance (actual/prescribed occlusion time). Secondary outcome was absolute occlusion hours per day. Parental fluency in Dutch was rated on a five-point scale. RESULTS: Compliance could be measured electronically in 88 of the 120 children; in 32 others, it failed for various reasons. Parental fluency in Dutch was moderate or worse in 36.4 % (p = 0.327). Average compliance was 55 % standard deviation (SD) 40 (n = 18) in the control group, 89 % SD 25 in the group receiving the educational cartoon (n = 25, P = 0.002 compared with control group), 67 % SD 33 (n = 24, P = 0.301) in the reward-calendar group and 73 % SD 40 (n = 21, P = 0.119) in the parent-information-leaflet group. On average, children in the control group occluded 1:46 SD1:19 hours/day, 2:33 SD 1:18 hours/day in the group receiving the educational cartoon, 1:59 SD 1:13 hours/day in the reward-calendar group and 2:18 SD 1:13 hours/day in the parent-information-leaflet group. No child who received the cartoon story occluded less than 1 hour per day, against seven in the reward-calendar group, five in the parent-information-leaflet group and five in the control group. CONCLUSIONS: Although all three components of the programme improved compliance with occlusion therapy in children in low-SES areas, the educational cartoon had the strongest effect, as it explained without words to a 4- to 5-year-old child why it should wear the eye patch.

How Dutch orthoptists deal with noncompliance with occlusion therapy for amblyopia.

BACKGROUND: We previously found that compliance with occlusion therapy for amblyopia is poor, especially among children of non-native parents who spoke Dutch poorly and who were low educated. We investigated conception, awareness, attitude, and actions to deal with noncompliance among Dutch orthoptists. METHODS: Orthoptists working in non-native, low socioeconomic status (SES) areas and a selection of orthoptists working elsewhere in the Netherlands were studied. They were observed in their practice, received a structured questionnaire, and underwent a semi-structured interview. Finally, a short survey was sent to all working orthoptists in the Netherlands. RESULTS: Nine orthoptists working in non-native, low-SES areas and 23 working elsewhere in the Netherlands participated. One hundred and fifty-one orthoptists returned the short survey. Major discrepancies existed in conception, awareness, and attitude. Opinions differed on what should be defined as noncompliance and on what causes noncompliance. Some orthoptists found noncompliance annoying, unpleasant, and hard to imagine, others were more understanding. Many pitied the noncompliant child. Almost all thought that the success of occlusion therapy lies both with the parents and the orthoptist, but one third thought that noncompliance was not solely their responsibility. Patients' compliance was estimated at 69.3% in non-native, low-SES areas (electronically, 52% had been measured), at 74.1% by the other 23 orthoptists, and at 73.8% in the short survey. Actions to improve compliance were diverse; some increased occlusion hours whereas others decreased them. In non-native, low-SES areas, 22% spoke Dutch moderately to none; the allotted time for a patient's first visit was 21'; the time spent on explaining to the parents was 2'30" and to the child 10". In practices of the other 23 orthoptists, 6% spoke Dutch moderately to none (P<0.0001), the time for a patient's first visit was 27'24" (P=0.47), and the periods spent explaining were 2'51" (P=0.59) and 26" (P=0.17), respectively. CONCLUSION: Conception, awareness, attitude, and actions to deal with noncompliance varied among orthoptists. In non-native, low-SES areas, time spent on explanation was shorter, despite a lower fluency in Dutch among the parents.

Psychological causes of non-compliance with electronically monitored occlusion therapy for amblyopia.

AIM: To analyse psychological causes for low compliance with occlusion therapy for amblyopia. METHOD: In a randomised trial, the effect of an educational programme on electronically measured compliance had been assessed. 149 families who participated in this trial completed a questionnaire based on the Protection Motivation Theory after 8 months of treatment. Families with compliance less than 20% of prescribed occlusion hours were interviewed to better understand their cause for non-compliance. RESULTS: Poor compliance was most strongly associated with a high degree of distress (p<0.001), followed by low perception of vulnerability (p = 0.014), increased stigma (p = 0.017) and logistical problems with treatment (p = 0.044). Of 44 families with electronically measured compliance less than 20%, 28 could be interviewed. The interviews confirmed that lack of knowledge, distress and logistical problems resulted in non-compliance. CONCLUSION: Poor parental knowledge, distress and difficulties implementing treatment seemed to be associated with non-compliance. For the same domains, the scores were more favourable for families who had received the educational programme than for those who had not.

[Evaluation of visual acuity in a historical cohort of 137 patients treated for amblyopia by occlusion 30-35 years ago]. / Visusevaluierung in einer historischen Kohorte von 137 okkludierten Patienten, 30-35 Jahre nach Ende der Okklusionsbehandlung.

BACKGROUND: Opinions differ on the course of the visual acuity in the amblyopic eye after cessation of occlusion therapy. This study evaluated visual acuity in a historical cohort treated for amblyopia with occlusion therapy 30-35 years ago. MATERIALS AND METHODS: Between 1968 and 1975, 1250 patients had been treated by the orthoptist in the Waterland Hospital in Purmerend, The Netherlands. Of these, 471 received occlusion treatment for amblyopia (prevalence 5.0%, after comparison with the local birth rate). We were able to contact 203 of these patients, 137 were orthoptically re-examined in 2003. We correlated the current visual acuity with the cause of amblyopia, the age at start and end of treatment, the visual acuity at start and end of treatment, fixation, binocular vision and refractive errors. RESULTS: Mean age at the start of treatment was 5.4 +/- 1.9 years, 7.4 +/- 1.7 years at the end and 37 +/- 2.7 years at follow-up. Current visual acuity in the amblyopic eye was correlated with a low visual acuity at the start (p < 0.0001) and end (p < 0.0001) of occlusion therapy, an eccentric fixation (p < 0.0001), and the cause of amblyopia (p = 0.005). At the end of the treatment, patients with a strabismic amblyopia (n = 98) had a visual acuity in the amblyopic eye of 0.29 logMAR +/- 0.3, and in 2003 0.27 +/- 0.3 logMAR. In patients with an anisometropic amblyopia (> 1 D, n = 16) visual acuity had decreased from 0.17 +/- 0.23 logMAR to 0.21 logMAR +/- 0.23. In patients with both strabismic and anisometropic amblyopia (n = 23), visual acuity had decreased from 0.52 logMAR +/- 0.54 to 0.65 logMAR +/- 0.54. Overall, acuity had decreased in 54 patients (39%) after cessation of treatment. Of these, 18 patients had an acuity decrease to less than 50% of their acuity at the end of treatment. In 15 of these 18 patients anisohypermetropia had increased. CONCLUSIONS: A decrease in visual acuity after cessation of occlusion therapy occurred in patients with a combined cause of amblyopia or with an increase in anisohypermetropia.

A preliminary report about the relation between visual acuity increase and compliance in patching therapy for amblyopia.

PURPOSE: The aim of this study was to establish a relation between visual acuity increase and compliance in children who have been prescribed patching therapy for their amblyopic eye. METHODS AND MATERIALS: In 14 new amblyopic children (mean age 4.3 +/- 1.9 years) compliance was measured electronically during one week, six months after starting patching therapy, with an Occlusion Dose Monitor (ODM), distributed through house visits. The children were diagnosed with anisometropia (5), strabismus (4) and anisometropia and strabismus (5). The degree of amblyopia was expressed as the ratio between the acuity of the amblyopic eye and the acuity of the good eye. Satisfactory increase in acuity was assessed by means of the following three criteria: acuity amblyopic eye / acuity good eye >75%, acuity exceeding 0.5 E-chart, three lines LogMAR acuity increase. RESULTS: Fourteen reliable recordings were obtained, which showed that children who did not patch, or were patched inconsistently, did not reach satisfactory acuity increase. CONCLUSION: There is indeed a statistically significant relation between acuity increase and measured compliance.