Spontaneous dislocation of a crystalline lens to the anterior chamber with pupillary block glaucoma in Noonan Syndrome: a case report

Mukhopadhyaya, Udayaditya; Chakraborti, Chandana; Mondal, Anindita; Pattyanayak, Ujjal; Agarwal, Rahul; Tripathi, Partha

doi:10.11604/pamj.2014.17.135.3049

Cited by 7 publications

(4 citation statements)

References 6 publications

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“…We have nine etiologies about retinal diseases related to ACG, they were RP, bestrophinopathy, PFV, congenital retinoschisis, ROP, FEVR, congenital retinal folds, Coat's disease, and NVG due to RVO, PDR, and RD, altogether 35 patients (11.7%). Other reported etiologies such as Turner syndrome [47], Noonan syndrome [48], Alagille syndrome [49], childhood cystinosis [50], and drug-related [51] were not found in our patients.…”

Section: Number Of Etiologiescontrasting

confidence: 57%

Etiologies and clinical characteristics of young patients with angle-closure glaucoma: a 15-year single-center retrospective study

Gao

Wang

Chen

et al. 2021

Graefes Arch Clin Exp Ophthalmol

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Purpose To investigate the etiologies and the clinical characteristics of angle-closure glaucoma (ACG) patients younger than 40 years old in Chinese. Methods Inpatients with diagnosis of ACG and diagnosed age younger than or equal to 40 years old, who were admitted in Eye, Ear, Nose, and Throat Hospital Fudan University from 2002 to 2017, were included in this retrospective non-comparative case series. The underlying causes and clinical features for all the patients were analyzed by comprehensive review of medical charts. Results A total of 298 patients (463 eyes) met the criteria, including 153 females (51.3%) and 145 males (48.7%); the mean age was 25.6 ± 13.0 years. Primary angle-closure glaucoma (PACG), uveitis, and anterior segment dysgenesis (ASD) were the top three etiologies in our patients, which accounted for 32.6%, 20.3%, and 15.1% of the total patients respectively. PACG mainly occurs after 30 years of age and ASD is the top reason of ACG in patients younger than 20 years old. Other known etiologies include iridocorneal endothelial syndrome, neovascular glaucoma, nanophthalmos, retinitis pigmentosa, spherophakia, bestrophinopathy, persistent fetal vasculature, iridociliary cysts, congenital retinoschisis, Marfan’s syndrome, retinopathy of prematurity, familial exudative vitreoretinopathy, congenital retinal folds, Coat’s disease, and neurofibromatosis. Conclusions We described the uncommon presentation of ACG in Chinese young patients. Although unusual, most of the etiologies could be identified. Therefore, more careful and comprehensive examinations are needed for early detection and timely treatment for young ACG patients.

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Section: Number Of Etiologiescontrasting

confidence: 57%

Etiologies and clinical characteristics of young patients with angle-closure glaucoma: a 15-year single-center retrospective study

Gao

Wang

Chen

et al. 2021

Graefes Arch Clin Exp Ophthalmol

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“…The surgical techniques described in the literature for management of anterior dislocation of the lens include intracapsular cataract extraction (ICCE), lensectomy, and in the bag bimanual lens aspiration [1,2,[4][5][6][7]. Lens aspiration within the capsular bag has been described previously by Vasavada et al, Sinha et al, and Khokhar et al for various indications [1,7,8] (Table 2).…”

Section: Discussionmentioning

confidence: 99%

“…Early surgical intervention is warranted in these cases to avoid long-term blinding complications like corneal decompensation and glaucoma [1]. The management of such cases requires a comprehensive approach as most of these cases are associated with some systemic abnormalities such as Marfan syndrome, Weill Marchesini syndrome, Noonan syndrome, Homocystinuria that further complicate the management strategy in these cases [1][2][3][4].…”

Section: Introductionmentioning

confidence: 99%

“…The surgical management involves removal of the lens for which various techniques have been described in the literature [1,2,[4][5][6][7]. Herein we describe a modified technique of microscope integrated optical coherence tomography (MiOCT) guided intra-lenticular lens aspiration (ILLA) for management of anteriorly dislocated lens in paediatric cases along with its surgical outcome in perhaps the most extensive series of such cases reported till date.…”

Section: Introductionmentioning

confidence: 99%

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Intra-lenticular lens aspiration in paediatric cases with anterior dislocation of lens

Sahay

Maharana

Shaikh

et al. 2019

Eye

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Purpose To assess the outcomes of intra-lenticular lens aspiration (ILLA) in paediatric cases with anterior dislocation of lens. Methods A retrospective review of medical records of cases with anterior dislocation of the lens in children (age < 16 years) that underwent ILLA between June 2017 and May 2018 was performed. Corrected distance visual acuity (CDVA), intraocular pressure (IOP), and anterior segment findings were noted at presentation and follow-up. Surgical notes were reviewed for all cases. Post-operative central corneal thickness (CCT) and central macular thickness (CMT) were recorded. Results Eleven eyes of eight patients with a median age of ten years underwent ILLA. There were four males and four females. The median duration of symptoms was 2 months, CDVA was 1.77 logMAR, and IOP was 16 mm of Hg. Ten eyes had corneo-lenticular touch with corneal oedema, and two had raised IOP at presentation. Homocystinuria (n = 2/8), Microspherophakia (n = 2/8), Marfan syndrome (n = 1/8), Buphthalmos (n = 1/8) and Ectopia lentis et pupillae (n = 1/8) were the identifiable causes for anterior dislocation. There were no intra-operative complications in any case. Immediate post-operative corneal oedema and raised IOP was observed in nine and three cases respectively and was treated with medical therapy. The median post-operative CDVA and IOP at 6-months was 1 logMAR and 15 mm of Hg respectively. The median CCT and CMT were 516 and 248 μm respectively. Five eyes developed a central corneal descemet scar. Conclusions ILLA is a safe and effective technique for surgical removal of an anteriorly dislocated lens in paediatric cases.

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Correction to: Etiologies and clinical characteristics of young patients with angle-closure glaucoma: a 15-year single-center retrospective study

Gao

Wang

Chen

et al. 2021

Graefes Arch Clin Exp Ophthalmol

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Spontaneous dislocation of a crystalline lens to the anterior chamber with pupillary block glaucoma in Noonan Syndrome: a case report

Cited by 7 publications

References 6 publications

Etiologies and clinical characteristics of young patients with angle-closure glaucoma: a 15-year single-center retrospective study

Etiologies and clinical characteristics of young patients with angle-closure glaucoma: a 15-year single-center retrospective study

Intra-lenticular lens aspiration in paediatric cases with anterior dislocation of lens

Correction to: Etiologies and clinical characteristics of young patients with angle-closure glaucoma: a 15-year single-center retrospective study

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