congenital anophthalmia

  • 文章类型: Journal Article
    OBJECTIVE: To evaluate axial length (AL), orbital width (OW) and height (OH) development in congenital microphthalmia and anophthalmia (MICA) using serial ultrasonography measurements.
    METHODS: A longitudinal prospective cohort (n = 74) of unilaterally and bilaterally affected MICA patients was followed from 2013 to 2022 at the university hospital in Amsterdam, the Netherlands. Clinical entity, age, severity category based on axial length, conformer treatment and intra-orbital cysts were registered. The main outcome measures were the absolute and relative growth of AL, OW and OH. Surgical and intra-orbital cyst cases were described separately.
    RESULTS: Absolute microphthalmic eye size increased in 27/49 (55%) unilateral MICA eyes, but growth arrest/decrease in the remaining could shift the case to a more severe category over time. A final affected/unaffected orbital symmetry ≥80% was seen in the large majority of unilateral cases (45/46 for OW, 43/46 for OH). Cases with AL < 10.5 mm had orbital symmetry <80% more often. Most orbital symmetry changes were seen in moderate and severe unilateral cases treated with 3D-printed conformer therapy starting at age <1 year, with 6/10 (60%) symmetry increase, 30% unchanged symmetry and 10% symmetry decrease. All cases older than 6.5 years (n = 6) did not show any change anymore, regardless of treatment. For bilateral and unilateral mild cases, orbital dimensions kept the same proportions during follow-up, with or without conformer treatment.
    CONCLUSIONS: Using severity categories in MICA based on relative AL may aid the decision to start conformer treatment, as most orbital symmetry changes were seen in moderate and severe unilateral cases receiving 3D-printed conformer therapy that started under age 1.
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  • 文章类型: Journal Article
    UNASSIGNED: To evaluate the outcomes of orbital hydrogel expanders in the management of congenital anophthalmia.
    UNASSIGNED: In this retrospective one-armed cohort study, a chart review was performed of eight children with congenital anophthalmia who underwent orbital expansion using orbital hydrogel tissue expander from January 2006 to July 2018. Computed tomography (CT) of orbital parameters was evaluated before and after surgery. Changes in the orbital parameters were correlated with clinical factors.
    UNASSIGNED: The study sample comprised 11 anophthalmic orbits of eight children (seven males, one female; median age = 12 months), with a median postoperative follow-up of 3.8 years. The anophthalmic orbital parameters after hydrogel expander implantation improved significantly compared to preoperative assessment as follows: mean orbital height improved from 21.7 mm to 25.4 mm (P < .001); width from 19.2 mm to 23.8 mm (P < .001); depth from 27.5 mm to 32.6 mm (P = .008); and volume from 3.7 cm3 to 5.3 cm3 (P = .001). Despite enlargement in all dimensions, the anophthalmic orbits with hydrogel expander had a significantly lower development than the normal orbits, mainly in height and volume. At the last postoperative visit, four (36.4%) cases had fornices deep enough to maintain the conformer. Migration and extrusion occurred in two (18.2%) cases.
    UNASSIGNED: Orbital hydrogel expander can improve the orbital development in congenital anophthalmia. However, the enlargement is not as extensive as that observed in the normal orbit. Orbital expanders associated with external conformers were not sufficient to induce normal growth of lids and fornix.
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  • 文章类型: Journal Article
    Anophthalmic socket syndrome determines functional deficits and facial deformities, and may lead to poor psychological outcomes. This review aims to comprehensively evaluate the features of the syndrome, based on literature review and authors\' clinical and surgical experience. An electronic database (PubMed,MEDLINE and Google Scholar) search of all articles written in English and non-English language with abstract translated to English on anophthalmic socket syndrome was performed. Data reviewed included demographics, presentations, investigations, management, complications and outcomes. Different types of orbital implants were evaluated; the management of implant exposure was examined; different orbital volume enhancement procedures such as secondary implantation, subperiosteal implants and the use of fillers in anophthalmic patients were described; the problems related to socket contraction were outlined; the treatment options for chronic anophthalmic socket pain and phantom eye syndrome were assessed; the most recent advances in the management of congenital anophthalmia were described. Current clinical evidence does not support a specific orbital implant; late exposure of porous implants may be due to pegging, which currently is seldom used; filler absorption in the orbit appears to be faster than in the dermis, and repeated treatments could be a potential source of inflammation; socket contraction results in significant functional and psychological disability, and management is challenging. Patients affected by anophthalmic socket pain and phantom eye syndrome need specific counseling. It is auspicable to use a standardized protocol to treat children affected by clinical congenital anophthalmia; dermis fat graft is a suitable option in these patients as it helps continued socket expansion. Dermis fat graft can also address the volume deficit in case of explantation of exposed implants and in contracted sockets in both children and adults. Appropriate clinical care is essential, as adequate prosthesis wearing improves the quality of life of anophthalmic patients.
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  • 文章类型: Journal Article
    OBJECTIVE: To report the results of a simple outpatient method for soft tissue socket expansion in young children with congenital anophthalmos.
    METHODS: Seventeen congenital anophthalmic sockets of 15 infants of a mean age of 4.2±4.4 months were fitted with specially designed serial solid acrylic shapes or hydrogel expanders using cyanoacrylate for eyelids closure when using the latter.
    RESULTS: At the age of 2 years, the mean horizontal eyelid length increased from a mean of 11.6±4.5 to 19.4±4.6 mm and the volume of the last expander from a mean of 0.6±0.2 to 2.0±0.3 cm(2). The specially designed acrylic shapes could be a substitute to the custom-made molds, which require general anesthesia.
    CONCLUSIONS: Successful increase in the horizontal eyelid length as well as the conjunctival socket volume could be achieved by a simple outpatient procedure without the need for repeated hospitalization and general anesthesia in these infants.
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