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The most frequent ophthalmic lesions associated to neurofibromatosis type 1 include iris Lisch nodules, optic nerve gliomas, and neurofibromas located on the eyelid, conjunctiva or orbit. Glaucoma is much less frequent, and it may be difficult to diagnose and treat. We present two patients with neurofibromatosis type 1 and associated congenital glaucoma. In case 1, the glaucoma was present at birth, and was the first symptom of the disease. It was surgically treated by means of an Ahmed glaucoma valve implantation, with good intraocular pressure (IOP) control after 13 months of follow-up. In case 2, the glaucoma appeared at 5 months old, and a trabeculectomy was initially performed; it failed 2 years later and an Ahmed glaucoma valve was then implanted, with adequate IOP control after 7 years of follow-up. Both children were males, and in both cases there were orbital neurofibromas and a dysplasia of the greater wing of the sphenoid of the same side. In case 2, orbital enlargement surgery was also performed at 4 years old. Congenital glaucoma management in the context of neurofibromatosis is very complex due to the frequent association of orbital and eyelid tumors and bone dysplasia, and its prognosis is usually poor. We present two cases in which good control of IOP has been achieved with Ahmed glaucoma valve implantation after 13 months and 7 years of follow-up.

Introduction: The failure rate of both filtration surgery and of aqueous shunt implantation is higher for iridocorneal endothelial syndrome than in other scenarios, due to the continuous proliferation of abnormal endothelial cells over the trabecular meshwork and the filtration area and also due to the more pronounced cicatrizing response shown by these young patients. We present the first case ever described in the literature of a pregnant patient with iridocorneal endothelial syndrome and uncontrolled ocular hypertension who was implanted an Ex-PRESS mini-shunt. Clinical case: A 35-year-old female presented with diminution of vision in the left eye for 2 months. She was 20 weeks pregnant. Her visual acuity was 20/20 in right eye and 20/25 in left eye, and intraocular pressure was 11 mmHg in right eye and 34 mmHg in left eye. Slit lamp biomicroscopic examination revealed no alterations in right eye, whereas left eye showed corectopia and uveal ectropion, stroma of iris’ sectoral atrophy and moderate corneal epithelial edema. Gonioscopy showed some anterior iris synechiae in left eye. Fundus evaluation was normal. Based on clinical features and examination, the diagnosis of left eye iridocorneal endothelial syndrome with decompensated intraocular pressure was made. She was prescribed topical timolol (0.5%) and dorzolamide. As a result of uncontrolled intraocular pressure and the impossibility to prescribe other hypotensive treatment available due to her being pregnant, it was decided to perform surgery in left eye using an Ex-PRESS mini-shunt and Ologen®; 6 months post surgery, intraocular pressure was 9 mmHg with no need for hypotensive treatment. The cornea was transparent, and the patient maintained her left eye visual acuity. Conclusions: Ex-PRESS mini-shunt can be considered a surgical option for iridocorneal endothelial syndrome. Its composition allows the ostium to remain open and the device triggers a milder postoperative inflammatory response. In our particular case, taking into account that the subject was a young, phakic, pregnant woman, whose intraocular pressure had to be closely controlled and we had to ensure that her postoperative care included as few drugs and as few reoperations as possible, we thought that using this device was the most appropriate option.

In the case presented, we hypothesize a perforation of the conjunctiva as the most probable cause of the patient's endophthalmitis. This case, together with previous case reports, highlights the importance of periodic patient follow-up after the procedure. However, less invasive and seemingly safer new generation implants for glaucoma surgery are not fully devoid of complications.

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