PMC6198575_01
Patient with a clinical anophthalmos with orbital mass in the left orbit. A: protruded cyst. B: transillumination with a pen light revealed the cystic character of the structure. C: B-scan ultrasonography examination.
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At the last visit in Kinshasa in spring 2016, a Congolese mother of a 14-month-old female requested medical attendance for her daughter, who had a large orbital cyst with clinical anophthalmos in the left orbita since birth (Figure 1A). First examination by using a small pen light demonstrated transillumination, which indicated an expectation that it was filled with liquid (Figure 1B). B-scan ultrasound examination demonstrated an orbital cyst with undefinable ocular tissue structures or underdeveloped optic rudiments (Figure 1C).
Intrasurgical photodocumentation and follow-up. A: drained cystic liquid; B: decompressed cyst; C: opened cyst; D: excised tissues; bar: 5 mm; E, F: transcutaneous fornix deepening sutures fixed over bolsters; G, H: 6 months' follow-up; I: 8 months' follow-up.
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In a first step the cyst was punctured and 1.5 ml of liquid was drained (Figure 2A). After drainage, the decompressed cyst appeared flabby shape (Figure 2B). In a second step the cyst was widely opened and the rims were fixed by a lid speculum (Figure 2C). Undefined tissue structures were excised (Figure 2D). The removed specimen material (Figure 2D) was fixed in formalin. Six months after surgery the child was doing well and the examination of the orbit showed no complications (Figures 2G and 2H). Two months later, an acrylic prefabricated artificial eye was placed in the socket (Figure 2I) but the mother finally preferred to keep the child without any prosthesis.
Histological examination of intracystal tissue. Magnification A, B: 20x; C, D: 100x. A, B and D: haematoxylin and eosin (H&E) staining; C: PAS staining.
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Histologic sections showed the wall of a pseudocyst, consisting of fibrous tissue (Figures 3A and 3B). The calcified nodule was reminiscent of a degenerated lens with a typical, PAS-positive capsule (Figure 3C). Adjacent to the nodule, pigmented epithelial structures could be found, which is reminiscent of the ciliary body, and finally merged with retinal-type epithelium (Figure 3D).