Parry Romberg Syndrome with contralateral iridocorneal endothelial syndrome: a unique case

Parry-Romberg syndrome (PRS) is a rare disorder which causes progressive hemifacial atrophy, with ocular manifestations like hypotony, enophthalmos and corneal edema on the ipsilateral atrophic side. This is a report of a unique case of PRS with contalateral manifestations like ectropion uvea, correctopia and endothelial deposits, along with polymegathism and pleomorphism seen on specular microscopy suggestive of Iridocorneal Endothelial (ICE) Syndrome. ICE syndrome and PRS have not been reported together in any literature so far. This case highlights the importance of a thorough glaucoma workup and corneal examination on the atrophic facial side as well as on the apparently normal side in all cases of PRS.

Unilateral Retinal Vasculitis as the Presenting Manifestation of Parry-Romberg Syndrome (Progressive Hemifacial Atrophy)

Purpose: This work aims to present a case of retinal vasculitis associated with Parry-Romberg syndrome. Method: A case report is presented. Results: A 17-year-old man with new floaters was found to have 20/40 vision with 1+ vitreous cell and retinal vasculitis in the right eye only. Workup for infectious etiologies did not reveal an explanation for the retinal vasculitis. However, magnetic resonance imaging of the head showed areas of linear band-like atrophy and scarring of the scalp and soft tissues as well as areas of gliosis and encephalomalacia in the subcortical white matter, all of which were consistent with Parry-Romberg syndrome. The patient was prescribed oral steroids and methotrexate, and the retinal vasculitis improved. Conclusions: Parry-Romberg syndrome is a rarely reported cause of retinal vasculitis and should be kept in the differential for retinal vasculitis.

Co-occurrence of Progressive Hemifacial atrophy due to Morphea with homolateral segmental Vitiligo: a case report

A female presented with segmental vitiligo on right Ophthalmic(V1) nerve distribution followed by hemifacial atrophy on right mandibular(V3) nerve distribution which stabilized after treatment with chloroquine and betamethasone pulse. Both dermatoses have younger onset, rapid progression followed by stabilization and dermatomal distribution suggests a possible common aetiological link.

Parry-Romberg syndrome: Which side of your face looks better?

Parry-Romberg syndrome (PRS) is a rare facial deformity characterised by slowly progressive hemifacial atrophy. We present a new case of hemifacial atrophy in an 18-year-old lady. Rudimentary understanding of the pathogenesis poses a challenge in deciding optimal timing and surgical intervention. Current suggested management are reviewed and discussed.

Parry-Romberg syndrome: is it a “relapsing-remitting” disease?

Parry-Romberg syndrome, also known as progressive hemifacial atrophy, is a rare, slowly progressive disorder characterized by unilateral, painless atrophy of the skin and subcutaneous tissue of the face. Neurological manifestations such as epilepsy, migraine and trigeminal neuralgia are relatively common and accompany in 15-20% of cases. Various etiologies such as infection, trauma, embryonic developmental dysfunction, sympathetic dysfunction and autoimmune disorders have been suggested as possible causes. Here we describe a 37-year-old woman whose disease manifested with dynamic contrast enhanced white matter changes over a period of two years, suggesting a “relapsing-remitting” course. Besides the inflammatory activity, positive serum-autoantibodies, inflammatory findings in cerebrospinal fluid, and an overlapping systemic autoimmune disorder may further support the hypothesis of autoimmune-inflammatory mediated pathogenesis.

Unilateral Papillitis as the Initial Presentation of Hemifacial Atrophy: Case Report and Review of Literature

Papillitis is a vision threatening condition, characterized by inflammation of the optic disc which often mimicked the features of papilledema; hence these patients often referred to the Neurosurgeon to exclude any intracranial pathology. This entity is associated with a number of intracranial, as well as extracranial pathologies. Among them, Parry–Romberg syndrome, also known as progressive hemifacial atrophy (PHA) possesses multiple ophthalmologic and neurologic manifestations. Here we report the case of a 14-year-old girl, who presented with the feature of progressive dimness of vision involving the right eye. Thorough physical examination demonstrated features of PHA overlapping with papillitis on fundoscopic examination. The patient treated with steroids, following which there was visual improvement. After evaluation in our facility, she was referred to department of plastic and reconstructive surgery for aesthetic improvement. Bang. J Neurosurgery 2020; 10(1): 97-101

TREATMENT OF PROGRESSIVE HEMIFACIALATROPHY (PARRYROMBERG SYNDROME) WITH POLYMETHYL METHACRYLATE: A CASE REPORT.

Progressive hemifacial atrophy, also called Parry-Romberg Syndrome (PRS), is a craniofacial disorder that typically involves the subcutaneous layer of one side of the face.This article We reports a case of a patient treated with polymethylmethacrylate for tissue augmentation and facial volume recovering asymmetry. Polymethylmethacrylate is effective and safe for treating progressive hemifacial atrophy and can leads a good aesthetic result.

Progressive hemifacial atrophy with characteristic ocular manifestations in a Chinese patient with a CRB1 mutation

Progressive hemifacial atrophy (PHA) is mainly characterized by asymmetrical atrophy of craniofacial tissue; however, 10% to 30% of patients with PHA exhibit ocular manifestations. Here, we describe abnormal ocular findings in a Chinese patient with PHA. The patient was a 29-year-old Chinese man. Characteristic ocular findings in his affected eye included keratic precipitate, corneal endothelial degeneration, fundus tessellation, pupillary dilation, direct light reflex loss, and visual evoked potential alteration. Whole exosome sequencing revealed that the patient harbored a mutation in the CRB1 gene; this gene has been associated with various retinal dystrophies. During 10 years of follow-up, the patient’s ocular status remained stable. To the best of our knowledge, this is the first report of ocular manifestations of PHA in a Chinese patient, and the first report of a CRB1 mutation in a patient with PHA; these findings may inform future research regarding PHA.