Flat Anterior Chamber after Trabeculectomy in Secondary Angle-Closure Glaucoma with BEST1 Gene Mutation: Case Series.

PURPOSE: Trabeculectomy has been regarded as a mainstay of initial treatment in eyes of angle closure glaucoma (ACG) with peripheral anterior synechia > 180° in the Chinese population while its efficacy in secondary ACG with BEST1 gene mutation remains unclear. We set out to investigate the treatment outcome of trabeculectomy for secondary ACG in a group of patients with autosomal recessive bestrophinopathy (ARB). METHODS: In this retrospective case series study, 8 secondary ACG patients with ARB and their 4 recruited family members underwent a thorough ophthalmic examination including best-corrected visual acuity, Goldmann applanation tonometry, gonioscopy, and fundus examinations. Ultrasound biomicroscopy, optical coherence tomography (OCT), ultrasound A-scan, B-scan, electro-oculography (EOG), Humphrey perimetry, fundus photography, fundus fluorescein angiography (FFA) and indocyanine green angiography (ICGA) were also performed. Blood samples were obtained in the patients and their available family members to analyze the variants of the BEST1 gene. Trabeculectomy was performed in the 8 patients (15 eyes). RESULTS: The age of onset varied from 13 to 38 years. The average axial length (AL) of the affected eyes was 21.82 ± 0.92 mm and the average anterior chamber depth (ACD) was 2.19 ± 0.29 mm. There was marked axial shallowing of the anterior chamber in all 15 eyes after trabeculectomy, and was not improved with potent mydriatics. The IOP was elevated in 3 eyes. Variable degree of yellowish subretinal deposits was observed in the posterior retina. The FFA showed punctuate or patched hyperfluorescence suggesting retinal pigment epithelium impairment. The ICGA demonstrated dilatation of choroidal vessels. The OCT revealed diffused neuroretinal detachment in the posterior and midperipheral retina, with intraretinal fluid collections, and hyperreflective subretinal accumulations. The average subfoveal choroidal thickness of the patients was 382.36 ± 80.09 µm. All the patients and enrolled family members carried mutation in BEST1 gene. CONCLUSIONS: ARB is a rare condition with fundus manifestations mimicking various diseases. Careful discrimination should be taken to exclude any secondary causes for ACG before treatment. Concerning the high incidence of postoperative shallow anterior chamber, selection of filtering surgery should be very careful in these patients.

[Pathogenesis of congenital clubfoot]. / O patogeneze vrozhdennoi kosolaposti.

Experiments, carried out by the author, have shown that the pathogenesis of congenital talipes is based on congenital muscular disbalance between flexors-supinators and extensors-pronators at the expense of strengthening of the former and insufficiency of the latter. An object of treatment of the considered developmental defect is restoration of myogenic balance. The existing methods of conservative treatment result in preservation of insufficiency of the anterolateral group of the crus muscles. Only the method of Vilensky V. Ya. allows to completely solve the posed problem. Operations on the posteromedial section of foot result in reduction of the myogenic disbalance as there is preserved insufficiency of extensors-pronators. Only operations, accompanied by shortening of anterolateral groups of the crus muscles allow to restore the myogenic balance straight on the operating table. The cause of recurrencies in case of congenital talipes is preservation of myogenic disbalance between flexors-spinators and their antagonist more frequent at the expense of insufficiency of the latter.