parry romberg syndrome
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2021 ◽  
Vol 8 (12) ◽  
pp. 1873
Usha K. Raina ◽  
Shruti Bhattacharya ◽  
Prateeksha Sharma ◽  
Varun Saini

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.

Cephalalgia ◽  
2021 ◽  
pp. 033310242110434
Thomas Foiadelli ◽  
Alessandra Rossi ◽  
Chiara Trabatti ◽  
Eugenia Spreafico ◽  
Viola Santi ◽  

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.

Maha Jamoussi ◽  
Hela Jamoussi ◽  
Saloua Fray ◽  
Slim Echebbi ◽  
Nadia Ben Ali ◽  

Giacomo Evangelista ◽  
Fedele Dono ◽  
Claudia Carrarini ◽  
Mirella Russo ◽  
Laura Bonanni

Seizure ◽  
2021 ◽  
Hyunjin Jo ◽  
Dongyeop Kim ◽  
Jooyeon Song ◽  
Young-Min Shon ◽  
Dae-Won Seo

2021 ◽  
pp. 112067212110155
Jianan Duan ◽  
Yun Zhang ◽  
Ge Ge ◽  
Meixia Zhang

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.

2021 ◽  
pp. 247412642110155
Elizabeth A. Atchison ◽  
Aimee J. Szewka ◽  
Pauline T. Merrill

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.

Sheenu Sachdeva ◽  
Varunkumar Jayakumar ◽  
Shubhlaxmi Atmaram Jaiswal

<p class="abstract">Waardenburg syndrome is a rare genetic disorder of neural crest cell development with incidence of 1:42000 to 1:50,000. The syndrome is not completely expressed and hence adds to its hetergenecity with symptoms varying from one type of syndrome to another and from one patient to another. Unilateral heterochromia that manifests in some people is associated with Waardenburg syndrome and Parry-Romberg syndrome. This is a case series of four cases with features of Waardenburg syndrome with variable presentations and familial inheritance.</p>

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