Advanced parry rhomberg syndrome with hemifacial atrophy, dental malalignment and unilateral vision loss: a rare case report

Ashish   Rai; Ayushi  Krishna; Poonam Gupta; Ajeet Kumar Chaurasia

doi:10.37022/jcscr.v4i3.774

Ashish Rai Assistant Professor, MD Medicine, DM Neurology, Department of Medicine, MLN Medical College, Prayagraj, Uttar Pradesh, India.
Ayushi Krishna Junior Resident 3rd Year, Department of Medicine, MLN Medical College, Prayagraj, PG Hostel , MLN Medical College Campus, Prayagraj, Uttar Pradesh, India 211002
Poonam Gupta Professor and Head, Department of Medicine, MLN Medical college,Prayagraj, L12, MLN Medical College Campus, Prayagraj, Uttar Pradesh, India 211002
Ajeet Kumar Chaurasia Professor, Department of Medicine, MLN Medical College, Prayagraj, L7, MLN Medical College Campus, Prayagraj, Uttar Pradesh, India 211002

DOI https://doi.org/10.37022/jcscr.v4i3.774

Abstract

Parry Rhomberg syndrome, also known as Progressive Hemifacial Atrophy is a rare medical entity, characterized by progressive thinning of the skin tissues resulting in "sunken in" appearance of one half of the face with secondary neurological symptoms, most common being seizures. The disease usually presents in the second decade of life and follows a self limiting course. There are no definite diagnostic criteria for the disease; clinical features and imaging modalities remain the mainstay for the diagnosis. Treatment follows a multi-modality approach, consisting of management of complications and reconstructive therapies. We hereby describe a unique case of a 45-year-old female with left hemifacial atrophy and misaligned teeth complicated with enophthalmos and extensive keratopathy leading to vision loss in the left eye; presenting as generalized tonic clonic seizures raising high suspicion of Parry Rhomberg syndrome. Parry Romberg syndrome, though rare and diagnostically challenging, requires early recognition; timely medical and reconstructive management can improve long-term outcomes.

Keywords: Parry Romberg syndrome, Hemifacial atrophy, dental malalignment, Enophthalmos, Keratopathy, Unilateral vision loss, Seizures

Author Biographies

Ashish Rai, Assistant Professor, MD Medicine, DM Neurology, Department of Medicine, MLN Medical College, Prayagraj, Uttar Pradesh, India.

Assistant Professor, MD Medicine, DM Neurology, Department of Medicine, MLN Medical College, Prayagraj, Uttar Pradesh, India.

Ayushi Krishna, Junior Resident 3rd Year, Department of Medicine, MLN Medical College, Prayagraj, PG Hostel , MLN Medical College Campus, Prayagraj, Uttar Pradesh, India 211002

Junior Resident 3^rd Year, Department of Medicine, MLN Medical College, Prayagraj, PG Hostel , MLN Medical College Campus, Prayagraj, Uttar Pradesh, India 211002

Poonam Gupta, Professor and Head, Department of Medicine, MLN Medical college,Prayagraj, L12, MLN Medical College Campus, Prayagraj, Uttar Pradesh, India 211002

Professor and Head, Department of Medicine, MLN Medical college,Prayagraj, L12, MLN Medical College Campus, Prayagraj, Uttar Pradesh, India 211002

Ajeet Kumar Chaurasia, Professor, Department of Medicine, MLN Medical College, Prayagraj, L7, MLN Medical College Campus, Prayagraj, Uttar Pradesh, India 211002

Professor, Department of Medicine, MLN Medical College, Prayagraj, L7, MLN Medical College Campus, Prayagraj, Uttar Pradesh, India 211002

References

1. Wong M, Phillips CD, Hagiwara M, Shatzkes DR. Parry Romberg Syndrome: 7 Cases and Literature Review. AJNR American Journal of Neuroradiology. 2015 Jul;36(7):1355 1361. doi:10.3174/ajnr.A4297.
2. Madasamy, Ramasamy; Jayanandan, Muruganandhan2,; Adhavan, Uma Revathy; Gopalakrishnan, Sivakumar1; Mahendra, Lodd. Parry Romberg syndrome: A case report and discussion. Journal of Oral and Maxillofacial Pathology 16(3):p 406-410, Sep–Dec 2012. | DOI: 10.4103/0973-029X.102498
3. Vix, Justine MD; Mathis, Stéphane MD, PhD; Lacoste, Mathieu MD; Guillevin, Rémy MD, PhD; Neau, Jean-Philippe MD. Neurological Manifestations in Parry–Romberg Syndrome: 2 Case Reports. Medicine 94(28):p e1147, July 2015. | DOI: 10.1097/MD.0000000000001147
4. Raina UK, Bhattacharya S, Sharma P, Saini V.Parry Romberg Syndrome with contralateral iridocorneal endothelial syndrome: a unique case. Int J Adv Med 2021;8:1873-5
5. Hasan Aydın, Zeynel Yologlu, Husamettin Sargın and Melike Rusen MetinNeurosciences Journal October 2015, 20 (4) 368-371
6. Smith, J. A., & Brown, L. M. (2012). A review of Parry-Romberg syndrome: Clinical features and pathogenesis. Journal of the American Academy of Dermatology, 66(3), 435–443
7. Guerrerosantos, J., & González, A. (2007). Classification and treatment of facial tissue atrophy in Parry–Romberg disease. Aesthetic Plastic Surgery, 31(5), 424–434
8. Tollefson, M. M., & Witman, P. M. (2013). Linear scleroderma en coup de sabre and Parry Romberg syndrome: A review of 54 patients. Pediatric Dermatology, 30(3), 363–369.t
9. Bien, C. G., Granata, T., Antel, J., et al. (2005). Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: a European consensus statement. Brain, 128(3), 454–471.
10. Posnick, J. C. (1997). Hemifacial Microsomia: Current concepts and classification. Plastic and Reconstructive Surgery, 100(5), 1229–1240.
11. Swarup, I., & Prasad, S. (2018). Parry-Romberg syndrome: A comprehensive review with emphasis on neurological and reconstructive management. Neurology India, 66(4), 1146–1152.