Huge peripapillary staphyloma with craniofacial clefts: A case report

Purpose: We reported the occurrence of a congenital unilateral huge peripapillary staphyloma in association with craniofacial clefts for the first time. Case report: A 1-year-old boy presented with a large defect on his left eyelid, a wide oblique columella nasi and an atypical wedge-shaped extension of the unilateral anterior hairline. Magnetic resonance imaging (MRI) examinations revealed there were cracks on his nasal septum, palate, and superior alveolar midline. Moreover, we surprisingly uncovered a gourd-shaped eyeball with the compressed optic nerve on the right side, while the right eye seemed normal from appearance. Under anaesthesia, fundus examination of the right eye showed a 15 mm-deep excavation surrounding the optic disc with defective choroid and dysplastic optic papilla. We reconstructed the left eyelid of the patient to protect his cornea and would make other solutions according to the results of follow-up. Conclusion: Peripapillary staphyloma and craniofacial clefts are two dissimilar rare congenital anomalies. In this patient, we firstly observed the co-existence of the two defects, which may provide the experience to the diagnosis and treatment of peripapillary staphyloma and craniofacial clefts. This case also gives us the pathogenic inspiration for further studies of peripapillary staphyloma and craniofacial clefts.

Surgical management of a case of contractile peripapillary staphyloma with retinal detachment and macular hole

A healthy 4-year-old male presented a fundus examination with a unilateral contractile peripapillary staphyloma surrounded by redundant retina and retinal pigment epithelium atrophy. Five years later, best-corrected visual acuity decreased to hand motion due to a retinal detachment with macular hole. One month after first vitrectomy, scleral buckle and intraocular gas, retina re-detached. Second surgery was performed with silicon oil tamponade and lensectomy without intraocular lens (IOL). Subretinal silicon oil was detected at the third month of follow-up when vitrectomy, inferior retinectomy, and laser photocoagulation of temporal border of staphyloma with silicon oil tamponade were performed. The retina remained attached and best-corrected visual acuity was 20/600 with intraocular silicon oil. A fourth surgery was performed for emulsified silicon oil extraction replaced with intraocular gas. At 6 months of follow-up, the retina re-detached again. This is a challenging vitreoretinal surgery in which re-detachments were due to retinal folds around the contractile staphyloma that raised macular hole. This is the first report of the combined presentation of contractile peripapillary staphyloma, retinal detachment and macular hole with a long-time follow-up period of years.

Bilateral peripapillary staphyloma: a case report

Background: The peripapillary staphyloma (PS) is a rare non-hereditary congenital optic disc dysplasia, which is generally unilateral and is likely to occur with poor vision. Fundus uncovered a large deeply excavated optic nerve head, retinal pigment epithelium changes at its edges and normal disc and retinal blood vessels in size and features. Case presentation: This literature present a bilateral case of an especially congential peripaillary staphyloma (CPS), which the patient with esotropia and nystagmus was exposed to abnormality when she was only 5-month-old. Several interesting features are present in this patient. Conclusions: Usually they have a large extent on visual loss. Thus, although extreme rare, an early detection, treatment and follow ups are necessary. Keywords: Staphyloma; optic nerve; congenital