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.

Headache in progressive facial hemiatrophy (Parry-Romberg syndrome): A paradigmatic case and systematic review of the literature

Background Parry-Romberg syndrome is a neuro-cutaneous disease characterized by progressive hemifacial atrophy. Although common, headache in this population is scarcely reported in the literature. Objective To evaluate the clinical features of headache in pediatric and adult patients with Parry-Romberg syndrome, and to discuss diagnostic and treatment approaches of headache in Parry-Romberg syndrome. Methods We conducted a systematic review in accordance with PRISMA guidelines. We searched the MEDLINE database to identify eligible studies and identified patients with Parry-Romberg syndrome and headache. We further reported a paradigmatic case with a complex headache disorder and described its management and outcome. Results We identified 74 articles, 41 of which were included in the analysis. A total of 52 patients (55.8% female) were included for data analysis. The main age at onset of headache was 20 years (SD 15.2; range 3–56). A diagnosis of migraine was made in 53.9%. Abnormal brain imaging was found in 82.2% of patients. Conclusion Long-term follow-up of patients is required, because headache may develop (and evolve) at any time over the course of the disease. Primary and secondary headaches often co-occur in patients with Parry-Romberg syndrome. Further research into the underlying etiopathogenesis and therapeutic targets would be recommended.

Fundus changes of Parry-Romberg syndrome: A report of three cases and literature review

Purpose: To report three cases of Parry-Romberg syndrome (PRS) with progressive hemifacial atrophy and similar fundus changes. Methods: Retrospective case series of three patients who presented to the ophthalmology department with blurred vision in one eye. All of them underwent three-dimensional reconstruction of head computed tomography (CT), related physical examinations, and also monthly ocular examinations of both eyes, including the slit-lamp microscope, fundus autofluorescence, optical coherence tomography (OCT), and fundus fluorescein angiography (FFA). We also reviewed the current literature about the fundus change in PRS from inception to March 2020 via PubMed. Results: Coats-like fundus and exudative retinal detachment were found in all three eyes of three patients during the follow-up. No systematic changes were found except for hemifacial atrophy. The final diagnoses of these three patients were PRS. Conclusion: Only a subset of PRS patients have concomitant ocular changes, but it still should be noticed that the diagnosis of PRS should be considered if adults presented with Coats-like retinopathy and hemifacial atrophy.

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.

Morphea en coup de sabre and hemifacial atrophy in an interdisciplinary approach

The increase in the degree of fluctuation asymmetry is accompanied, among others, by diseases such as morphea en coup de sabre (morphea ECDS) or Parry‑Romberg syndrome (PHA). Patients suffering from them struggle not only with dermatological defects, but also with neurological, rheumatological, orthopedic, ophthalmological and dental symptoms. Morphological and functional disorders and craniofacial deformities related to them often generate psychosocial problems. The complexity of the issues to be solved proves the necessity of undertaking interdisciplinary actions aimed at developing objective diagnostic and therapeutic protocols, restoring (especially in pediatric patients) as close as possible to the correct developmental patterns, inhibiting the active phase of the disease and undertaking effective and aesthetically satisfying measures.