Double hit: Mantle Cell Lymphoma associated with squamous cell carcinoma or chalazion? A case report.

Abstract: A few cases of ocular adnexal neoformations related with MCL have been reported in the literature. We present a rare case of tumour duplicity: mantle cell lymphomas (MCL) associated with squamous cell carcinoma (SCC) localised at the level of the ocular adnexa, on left upper eye lid mass since two years of 18 mounth duration in a 57-year-old man who had previously been diagnosed with stage IV MCL for 14 months. The patient had been treated according to the R-DHAP scheme for 4 cycles, in anticipation of a possible autologous HSC transplant, which was not carried out due to a positive diagnosis at the end-of-cycle osteomedullary biopsy (BOM) check. Ophthalmological examination was performed, and afther surgical removal histological examination proved to be squamous cell carcinoma (SCC). The aim of this case report is to decode the signs, symptoms and factors associated with the formation, that appear to be a chalazion, at an early stage in order to prevent the overgrowth of the mass that could invade the surrounding tissues by infiltrating them, as well as negative aesthetic outcomes of the surgery due to the excessive size of the mass, which could compromise the patient's quality of life.

Papilledema in childhood for diagnosis of pseudotumor cerebri or late hydrocephalus: a case report.

Abstract: A case of hydrocephalus is presented in a 13-year-old female with transient loss of vision from 1 week and papilledema, previous ophtalmological history was negative. Visual field was performed, and neurogical examination proved to be hydrocephalus. In literature few cases of Papilledema in adolescent children with hydrocephalus has been reported. The aim of this case report is to decode the signs, symptoms and factors associated with papilledema in children with hydrocephalus at an early stage in order to prevent a poor visual-functional residual (permanent low vision).

Medico legal considerations on refractive surgery.

This study is a summary of legal issues that occurred over time in Italy, concerning the evaluation of the professional responsibility of the health damage caused by refractive surgery, also in the light of the diagnostic elements obtained from the new techniques relating to diagnostic tests for preoperative surgical correction of the main ametropia.

Impact of cardiovascular risk factors on incidence and severity of Retinal Vein Occlusion.

PURPOSE: Retinal Vein Occlusion (RVO) is a thrombotic process affecting retinal veins. The purpose of this research is to study demographic characteristics and prevalence of cardiovascular comorbidities among subjects affected by RVO. In addition, authors explore the role of each variable in determining occlusion type and severity. SUBJECTS, MATERIALS AND METHODS: This was a prospective observational study recruiting subjects affected by RVO and secondary macular edema. Exclusion criteria included pre-existing macular edema, recent ocular surgery (<6 months), pregnancy, diagnosis other than RVO, diabetes mellitus type I, any systemic pathology that significantly reduced life expectancy. Each participant was studied through a comprehensive medical history, cardiovascular assessment, blood testing, ocular exam, and macular OCT imaging. RESULTS: A total of 145 eyes, 145 participants, thereof 80 males (55%) and 65 females. (45%) Mean age: 62.5 ± 14.3 SD. 61 eyes (42%) were affected by CRVO and 84 eyes (58%) by BRVO. No statistically significant differences were noted between genders. Hypertension was very prevalent (63%). Dyslipidemia was more associated with BRVO (p = 0.044). Subjects with hypertension had a mean central macular thickness (CMT) of 643 µm against a mean of 489 µm of those without hypertension. (p < 0.05). No other variable was associated with macular edema severity. CONCLUSIONS: Older age and hypertension are strong risk factors for RVO. Dyslipidemia was strongly associated with BRVO. (p=0.044) Hypertension was not only associated with RVO incidence, but also with its severity. In fact, hypertensive subjects had significantly worse macular edema.

Meta-analysis on the utility of radiotherapy for the treatment of Ocular Melanoma

Introduction: Uveal melanoma is the most common intraocular tumor in the adult population. It can affect any part of the uveal tract: the iris, ciliary body, and choroid. Historically, enucleation has been the mainstay of treatment for primary melanoma. In the last decade, however, radiotherapy has acquired an increasingly important role and has now become our first-line modality. However, it is still widely debated what is the most effective radiotherapy technique for this tumor. Purpose to perform a literature review on the utility of radiotherapy for primary ocular melanoma and determine the most effective radiotherapy technique Materials and Methods: We included all systematic and narrative reviews on the topic, published between September 2007 and November 2017 on PubMed and SCOPUS. Two independent reviewers assessed the eligibility criteria for each article using the PRISMA checklist. The methodological quality of narrative and systematic reviews was evaluated with the INSA and AMSTAR checklists, respectively Results: Our study analyzed a total of 23 studies, including 18 narrative reviews and 5 systematic reviews. Radiotherapy with Brachytherapy, Proton Therapy, SRS/SRT with gamma knife and cyber knife, are the most common choices for the treatment of primary ocular melanoma. These techniques allow for excellent lesion spread control, eye, and vision conservation, and improve overall patients' quality of life. Among the narrative reviews, the highest INSA score was 5/7, the lowest 2/7, the mean was 3.83/7 and median was 4/7. Among the systematic reviews, the highest AMSTAR score was 9/12, the lowest 4/12, the mean 5.6/7 and median 4/7 Conclusion: The number of studies available on this topic is scarce. Among those published, the methodological quality is modest, as assessed with the INSA and AMSTAR checklists. As a result, we are not able to determine what the most effective radiotherapy technique is

Ocular hypertension management in long-term treatments with intravitreal dexamethasone implants: a 3-year experience.

PURPOSE: A commonly recognized complication of intravitreal steroids is ocular hypertension (OHT).The aim of this study was to investigate the effectiveness of pharmacotherapy in controlling this side effect, even in patients receiving sequential injections. METHODS: A total of 146 injections were performed on 78 patients over 3 years. 78 eyes were treated with 1 injection, 44 eyes were treated with 2 injections; 24 eyes were treated with 3 injections. The intravitreal corticosteroid used in this observational study is 0.7mg dexamethasone, commercially known as 0.7mg Ozurdex®. RESULTS: Following the first injection, mean intraocular pressure (IOP) increased by 1,90 mmHg. Following the second injection, mean IOP increased by 0.23 mmHg. Following the third injection, there was no statistically significant change. Patients with IOP >= 21mmHg (7% of all participants) were started on topical pressure-lowering medications. CONCLUSIONS: Intravitreal dexamethasone implants increased IOP of variable degrees in different patients. However, secondary OHT was effectively controlled with pharmacotherapy alone. This allowed for continuation of dexamethasone therapy.

Anisometropia and isoametropia associated with microstrabismus: effectiveness of late anti-amblyopia treatment.

PURPOSE: Outcomes Research Study on patients suffering from anisometropia or isoametropia associated with amblyopia and microstrabismus to show the impact of late occlusion treatment on therapeutic prognosis. MATERIALS AND METHODS: Enrollment of all the eligible patients presenting to recruiting centers at the Department of Pediatric Ophthalmology and Strabismus - Ophthalmological Clinic - Policlinico Umberto I - Rome. At baseline, all patients will undergo an opthalmologic evaluation. Recruited patients will be followed for a 2-year period. Our test group was composed of 31 patients, 11 with bilateral and 20 with monolateral amblyopia, totalling 42 amblyopic eyes, and it was assessed - always by the same examiner - with the help of the following orthoptic examinations: Wirt test, Irvine-Jampolsky test (4 dioptre fixation task), Visuscope, Cover Test, Bagolini striated glasses, and Worth lights test. We assessed pre-post treatment variations observed in the overall group of amblyopic eyes, regardless of the differences between RE and LE. OUTCOMES: Of the 31 children, 58.1% were males and 41.9% female, average age between 6 and 14 years. Of the 42 amblyopic eyes, after daily occlusion of 8 hours ± 2.30 SD, we recorded an improvement of 71.4%, and visual acuity increased from an average of 0.4 LogMAR to 0.1 LogMAR. CONCLUSION: Late anti-amblyopic occlusion treatment proved effective in the long term for children 6 to 12 years, specifically regarding visual acuity, fixation, stereopsis, and binocular collaboration in patients suffering from anisometropia or isoametropia and microstrabismus.

Infections in hospital departments. What is Hospital Responsibility?

Infections in hospitals still have a high incidence and many of them could be avoided through better welfare standards. To try to overcome them, a strategy based on prevention is needed, but cleaning, disinfection and sterilization procedures are also a key tool. It is important to provide for all healthcare professionals a constant update and the creation of protocols that take into account the technical, scientific and economic aspects, but also specific operational needs, so that the proposed solutions can be applied in daily routines. The authors outline the mandatory duties to the doctors and hospital and underline the need to document in the clinical record the treatments performed. In case of infections occurred in hospital environment, the patient must demonstrate the guilty nature of the hospital's conduct, the existence of a harm and the causal connection. The hospital must demonstrate that asepsis measures were adopted according to the actual scientific knowledge and they must cover not only the treatment but also the diagnosis, all the activities prior to surgery and the postoperative phase. The sentences examined show that hospitals can avoid being accused of negligence and imprudence only if they can prove that they have implemented all prophylaxis measures contained in the guidelines and protocols. They must demonstrate that the infection was caused by an unforeseeable event. While some initiatives to improve the quality of hospital care have already allowed a decrease in the incidence and cost of these infections, much remains to be done.

Fast glycorrhachia and cerebrospinal fluid protein as predictors of sensory block in anesthesia with subarachnoid Ropivacaine.

BACKGROUND: Identify if glycorrhachia and cerebrospinal fluid protein could influence the time of sensory block to T10, the duration and the metameric block's level, after a standard dose of Ropivacaine. METHODS: 80 patients, ASA I - III undergoing to transurethral prostate resection with spinal anesthesia in a prospected open study were recruited. A 0.2 ml liquor's sample was taken; glycorrhachia, by glycemic stix and CSF protein, by urinary stix, were got, before Ropivacaine 0.5% 15 mg injection (0.10 - 0.15 mlsec). After anti-trendelemburg, with 30 ° tilting for 15 min, the onset of sensory block to T10, the maximum metameric level to 15' and the time of sensory block were reported. The data collection were analyzed using the software language R. RESULTS: A significant correlation liquor specific weigh preoperative glycemia (0.749), liquoral specific weigh glycorrhachia (rho = 0.751; R2 = 0.564; P 0.05) and specific weigh CSF protein (rho = 0.684; R2 = 0.468; P 0.05) were reported. Inverse relation CSF weightsensory block level (rho -0.789, P 0.05, R2 0.621) was evidenced. Inverse relation onset time to T10 glycorrhachia (84%) and cephalic block glycorrhachia (76%) were found. Inverse correlation onset time to T 10 CSF protein and cephalic block proteinorrachia was respectively 84% and 67%. A rho of 0.712 with R2 of 51% BMI onset to T10 and rho of 0.681 with R2 of 51% BMI maximum cephalic block with P 0.05 were reported. CONCLUSIONS: The predictability of a iso-hypobaric local anesthetic could reduce the risk of procedure failure and adverse events by further cephalic spread.

Histological and ultra-structural effects of rapid muscle expansion through intramuscular administration of carbon dioxide: an intra-operative study in an animal model.

INTRODUCTION: Carbon dioxide (CO2) therapy refers to trans-cutaneous or sub-cutaneous administration of CO2 for therapeutic purposes, and recent studies have pointed out that it produces a vasodilation effect after it is locally injected, which helps amplify the reconstructive potentiality of an expanded-muscle flap. MATERIALS AND METHODS: Thirty male Wistar rats, weighting between 350 and 400 g, were randomly divided into three groups of 10. In the first group, single intra-operative rapid expansion was carried out under the right latissimus dorsi muscle. In the second group, for five days prior to surgery, a pre-treatment with intramuscular injections of CO2 was performed. The third group served as controls. For each group, the latissimus dorsi muscle was fixed as soon possible after mice died, and ultrathin sections of it examined with transmission electron microscope. RESULTS: In the treated group, the majority of expanded muscles showed a normal striation pattern, whereas a few fibers showed mild disorganization of the myo-filaments in the sarcomeres, which appeared overstretched (average 2.37 µm). CONCLUSIONS: This evidence could demonstrate a greater capacity of muscle recovery after treatment by CO2 expansion.

Efficacy and safety of the intravitreal treatment of diabetic macular edema with pegaptanib: a 12-month follow-up.

AIM: This observational study was performed to evaluate the efficacy and safety of intra-vitreal injections of pegaptanib during a 12-month follow-up period. PATIENTS AND METHODS: Forty eyes (20 patients) affected by diabetic macular edema were monitored. Twenty were subjected to treatment, and 20 were controls. The treatment involved a cycle of three intravitreal injections of pegaptanib (0.3 mg every 6 weeks), at the end of which treated patients were submitted to a monthly follow-up for a period of 12 months. The aim was to evaluate the clinical condition of the eye after therapy and gauge the efficacy of the long-term use of this drug. Specific criteria were used to measure the efficacy and safety of pegaptanib. Regarding efficacy, we considered the following: an average improvement in the power of vision, or visual acuity, of →10 letters (2 lines), equivalent to an average logMAR score of →0.2, and a reduction in the central macular thickness of →250 µm. Regarding safety, we considered the occurrence of undesired eye and systemic side effects correlated to either the drug itself or the injection procedure. RESULTS: The logMAR score for the measurement of visual acuity at T3 (third intra-vitreal injection at week 13) with respect to T0 decreased from 0.7 ± 0.277 to 0.445 ± 0.216, suggesting an improvement, while the mean Early Treatment Diabetic Retinopathy Study (ETDRS) score increased from 25.75 ± 13.046 to 34.300 ± 11.770 letters. The central macular thickness was reduced from the initial value of 746.95 ± 293.601 to 334.050 ± 93.997 µm. In seven controls, we registered a worsening both in terms of visual acuity and macular thickness in some eyes, justifying a continuation of therapy in eight eyes of the control group. CONCLUSIONS: Pegaptanib proved to be efficacious and safe for the treatment of diabetic macular edema throughout the 12-month followup. To evaluate its long-term efficacy, further studies are required with larger numbers of patients and longer observational follow-up periods.

Pneumotrabeculoplasty as treatment for primary open angle glaucoma: critical review of the literature.

PURPOSE: To evaluate with criticism the available evidence shown in the literature about the efficacy of pneumotrabeculoplasty (PNT) as a treatment for primary open angle glaucoma. MATERIALS AND METHODS: PNT is a recent non-surgical and noninvasive technique to safely reduce the intraocular pressure (IOP) in eyes suffering from ocular hypertension or primary open angle glaucoma. The technique consists in the application on the ocular surface of a disposable suction ring that induces a temporary trabecular meshwork stretching, increasing outflow of aqueous humor in the Schlemm's canal. In our study, we compared the results on the efficacy and safety of this treatment published to date in the literature. RESULTS: During follow-up, Authors have observed significant reductions in IOP in about 70% of treated eyes. The first treatment consists in 3 applications at day 0, 7 and 90, repeatable every 90 days. Immediately after suction ring application there is a significant elevation of IOP, however, of short duration and without demonstrable visual field worsening. Side effects observed are of little clinical significance and spontaneously resolving in a short time. CONCLUSIONS: The PNT is a safe and effective technique to reduce IOP in patients with ocular hypertension or open-angle glaucoma. It is repeatable, but can not replace drug treatment in patients already receiving topical therapy for glaucoma; however, it has proven effectiveness in maintaining an IOP target over time if accompanied with only a single topical drug, favoring a reduction of antiglaucoma drugs in polytherapy and increasing the compliance with treatment.

[Epidemiology of syphilis: new cases of neurosyphilis]. / Epidemiologia della sifilide: nuovi casi di neurolue.

The Authors report three cases of ophthalmic neurosyphilis in patients who complained a visual decrease monocular. The diagnosis was performed with physical and fundus examination, electrophysiological, hemato-chemical and microbiological tests, fluorangiografy and RMN. The physical examination provided standard results about eyes interested while the fundus examination, as well as the electrofunctional and fuorangiografy examinations, showed some alterations. The sockets and encephalon RMN were normal. Moreover; the patients were submitted to specific haematic and microbiological laboratory tests which showed positivity for syphilis. To sum up all patients received an incidental diagnosis of syphilis. This trend could be attributable to an extensive use of antibiotics that changing the natural course of disease, mask clinical evidences and make them harder to diagnose.

A pilot clinical study on the effectiveness of mesoglycan against diabetic retinopathy.

PURPOSE: A double-blind placebo-controlled study on 68 patients suffering by Diabetic Retinopathy was aimed in order to evaluate the effectiveness of Mesoglycan in this pathology. This drug is particularly interested in treatment of disorders of microcirculation. MATERIALS AND METHODS: The two treatments were randomly assigned to each patient, using a 100 mg/day dosage of Mesoglycan, and both treatments were prescribed for 6 months. The efficacy of both treatments was based on clinical and instrumental check. RESULTS: The clinical results that emerged in the group treated with Mesoglycan were excellent, although observations are on a limited number of patients appears a direct action of Mesoglycan on the endothelium retinal blood vessels and circulation. Indeed, in the observed patients, was detected a significant reduction of microhemorrhages, microaneurysms and exudates. The same cannot be said of the placebo group; none of patients of that group showed signs of clinical improvement at the end of the study. CONCLUSION: Data emerging from our study show a direct action of Mesoglycan on endothelium retinal blood vessels and circulation, as we observed in patients we found a significant reduction in the number of microhemorrhages, microaneurysms and exudates. This action can be explained by the characteristics of drug as antithrombotic profibrinolytic and anti-edema, already found in vitro and experimentally. We conclude that our preliminary study showed an encouraging clinical efficacy, together with excellent tolerability, and therefore our objective has been met, which was to verify the existence of the prerequisites for a larger clinical study.

Testing the effectiveness of intravitreal ranibizumab during 12 months of follow-up in venous occlusion treatment.

AIMS: To determine the effectiveness and safety of treatment of intravitreal Ranibizumab for Central Retinal Vein Occlusion. PATIENTS AND METHODS: This non-randomized observational clinical study was comprised of a round of therapy with three IVI. Twenty eyes affected by CRVO were recruited. The average age was 65.06 +/- 15 years and criterion for inclusion: age >18 years, best Corrected Visual Acuity (BCVA) from 5 to 40 letters and macular edema with thickness greater than 275 micrometer. The criteria used for reinjection were: CMT> 150 micrometer, ETDRS <10 letters and LogMAR <0.2. The statistical analysis for continuous variables (ETDRS, logMar and CMT) was conducted calculating median and range (min-max), since these variables, due to sample size, were not normally distributed.Time trends of these variables were plotted with boxplot and differences. Events between T0 and T12 were assessed using the analysis of variance (ANOVA) for repeated measurements and the F test (Pillai's trace). The statistical significance was set at p <=0.05. RESULTS: All of the patients showed improvement. In fact, the ETDRS went from a median of 20.00 to 28.50, LogMAR went from a median of 0.75 to 0.55 and the values for CMT went from a median of 556.00 micrometer to 390.00 micrometer. The drug reaches maximum effectiveness after two months of therapy, with T2 remaining constant from the third injection at T3 until the end of 12 months at T12. CONCLUSIONS: The results produced by our study indicate that Ranibizumab is a valid treatment for CRVO.

Effectiveness of vision rehabilitation treatment through MP-1 microperimeter in patients with visual loss due to macular disease.

PURPOSE: To evaluate the effectiveness of biofeedback treatment for low-vision rehabilitation in patients affected by macular disease. MATERIALS AND METHODS: 171 eyes of 99 patients (42 female and 57 male) between 50 to 75 years old (mean age: 64.6) were included in this study. All patients were suffering from age-related macular degeneration (AMD) (122 eyes) or macular myopic degeneration (MMD) (49 eyes). All patients underwent an assessment of examinations including visual acuity, reading speed test, slit lamp examination and tonometry, ophthalmoscopic fundus examination, microperimetry, fixation test, retinal sensitivity, fluorangiography (FAG), optical coherence tomography (OCT). The treatment was divided in 16 sessions, the patients underwent other examination assessment at 6 and 12 months, except for FAG and OCT. Statistical analysis was performed using Student's t-test, and p-value <=0.05 was considered statistically significant. RESULTS: After training 130 eyes of 171 in the study group (76.02%) had a statistically significant improvement of the distant visual acuity (p<0.01): 38 eyes suffering from MMD and 92 eyes suffering from AMD. After 12 months of follow-up a group of 25 eyes of 130 (19.23%) had a loss of benefits that were observed at the end of the treatment sessions: 16 eyes and 9 eyes were suffering from MMD and AMD respectively. Examination assessment during follow-up showed that 4 eyes and 2 eyes of the group that lost benefits had a worsening of MMD and AMD primary disease respectively. CONCLUSIONS: It is not yet understood how biofeedback produces amelioration of visual function. According to the 'Eccentric fixation' theory, with biofeedback rehabilitation patients are trained to use the non-damaged retina areas to develop a new preferred retinal locus. In our study group we found a significant improvement in both visual acuity and fixation.

Levobupivacaine vs. racemic bupivacaine in peribulbar anaesthesia: a randomized double blind study in ophthalmic surgery.

AIMS: The Authors examine the employement of a new anaesthetic agent, levobupivacaine 0.50% (S - enantiomer of racemic mixture of bupivacaine), for peribulbar anaesthesia in a randomized double blind study vs. racemic bupivacaine 0.50% alone or in association with hyaluronidase 10 IU x ml(-1). MATERIALS AND METHODS: 120 patients were divided into four groups of 30 each: group L (levobupivacaine 0.50%), group B (racemic bupivacaine 0.50%), group LH (levobupivacaine-hyaluronidase 10 IU x ml(-1)), group BH (racemic bupivacaine-hyaluronidase 10 IU x ml(-1)). RESULTS: The onset-time (14 +/- 3.2 min vs. 13 +/- 4.8 min) and the duration of anaesthesia (195 +/- 34.2 vs. 204 +/- 37.6) were similar. The ocular akinesia was evaluated with an 8 point system: it was considered sufficient for surgery with values of less than 5 points. The association with hyaluronidase increased the spread of local anaesthetics (76.6% of group LH, 73.3% of group BH) with local anaesthetics alone (60% of group L, 56.6% of group B). Moderate hypotension (<30% baseline) was reported in 3 patients (10%) of group L, 2 (6.6%) of group B, 1 (3.3%) of group LH and 2 (6.6%) of group BH. Statistical analysis (Student-Newman-Keuls test) was significant between group L vs. BH, B vs. BH and LH vs. BH as regards onset-time of anaesthesia; between group B vs. LH, B vs. BH and L vs. LH for the duration of anaesthesia. Chi square test for the general akinesia score showed significant results in group L vs. LH (p=0.043) and B vs. LH 8P =0.018); as regards the score 0, test reported significant values between groups B vs. LH (p=0.004) and B vs. BH (p=0.017). CONCLUSIONS: In conclusion levobupivacaine, a longlasting local anaesthetic with limited cardio and neurotoxicity, might be useful for vitreoretinal surgery in elderly patients, compared with general anaesthesia.

The combination latanoprost-timolol versus twice daily 0.50% timolol administration either associated or not with latanoprost: efficacy and tolerability in the primary open-angle glaucoma.

AIMS: To evaluate the ocular hypotensive effects and tolerability of the once daily fixed combination latanoprost-timolol versus twice daily 0.50% timolol associated or not with once daily latanoprost in patients suffering from Primary Open-Angle Glaucoma (POAG). METHODS: We compared the effects of such a combination with those of 0.50% timolol alone twice daily in a group of 24 patients and with the effects of timolol 0.50% twice daily associated with once daily latanoprost in a second group of 20 patients with a follow-up of 24 months. RESULTS: In the first group of patients after one month the Intraocular Pressure (IOP) was reduced from a mean of 19.93 to a 17.04 mmHg. This reduction remained stable with a mean value of 17.00 mmHg at the third month, of 16.49 mmHg at the sixth month, of 17.04 at the twelfth month, 16.00 at the eighteenth month, and of 15.86 mmHg in the twenty-fourth month. In the second group there was a statistically significant reduction from 19.4 to 16.84 mmHg after one month. This reduction remained constant with mean values of 16.47 at the sixth month, of 16.20 at the twelfth month and of 16.00 mmHg at the twentyfourth month of treatment. CONCLUSIONS: The once daily latanoprost-timolol combination was shown to furtherly reduce the Intraocular Pressure (IOP) (p=0.001) and to maintain under control the intraocular pressure for the observation period (24 months). Both topical and systemic side-effects were scarse and tolerability was good.

Stickler syndrome in Pierre-Robin sequence prenatal ultrasonographic diagnosis and postnatal therapy: two cases report.

The Pierre-Robin Syndrome (PRS) is a rare congenital abnormality, with an approximately 1/30,000 estimated rate, characterized by the presence of the combination of mandibular hypoplasia (micrognathia or small jaw), glossoptosis (retrusion of the tongue into the pharyngeal airway) and, often, a posterior cleft of the secondary palate. It may be an isolated occurrence or part of a more complex syndrome and it is associated with long-term respiratory, nutritional, and developmental difficulties. Stickler syndrome (SS) is a rare autosomal dominant connective tissue disorder estimated to affect approximately 1/7500 newborns. It is diagnosed clinically and, at present, there is no consensus on a minimal clinical diagnostic criterion. The most frequent diagnosis in patients with syndromic Pierre Robin sequence is Stickler syndrome, which may be complicated by congenital high myopia and substantial risk of retinal detachment. However, cases of Stickler syndrome with probable visual complications are rarely identified among this group of patients by members of the cleft team. The patient had an acute unilateral hydrops, with a monolateral keratoconus. The ocular abnormalities included: severe myopia, abnormalities of the vitreous, and high risk of retinal detachment (with subsequent blindness). We report two extremely rare cases of prenatal diagnosis of PRS and SS, prematurely identified by prenatal ultrasonography and successively managed by oculists ophthalmogists.