Parry-Romberg Syndrome Associated with Hypothyroidism

Hee Lee Yeon; Eun Lee Seong; Yeul Kim Jung; Nam Kim Kyoung; Bok Lee Sung

doi:10.3341/jkos.2017.58.7.857

Journal List > J Korean Ophthalmol Soc > v.58(7) > 1010818

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Yeon, Seong, Jung, Kyoung, and Sung: Parry-Romberg Syndrome Associated with Hypothyroidism

Original Article

J Korean Ophthalmol Soc 2017;58(7):857-861.

Published online: 8 September 2017

DOI: https://doi.org/10.3341/jkos.2017.58.7.857

Parry-Romberg Syndrome Associated with Hypothyroidism

Hee Lee Yeon, M.D., PhD, Eun Lee Seong, M.D., Yeul Kim Jung, M.D., PhD, Nam Kim Kyoung, M.D., PhD, Bok Lee Sung, M.D., PhD

Department of Ophthalmology, Chungnam National University School of Medicine, Daejeon, Korea

Address reprint requests to Sung Bok Lee, MD, PhD Department of Ophthalmology, Chungnam National University Hospital, #282 Munhwa-ro, Jung-gu, Daejeon 35015, Korea Tel: 82-42-280-8433, Fax: 82-42-255-3745 E-mail: sblee@cnu.ac.kr

* Conflicts of Interest: The authors have no conflicts to disclose.

Received 30 March 20178 May 2017 Accepted 17 June 2017

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose

We report a case of Parry-Romberg syndrome associated with right enophthalmos, hypotropia, up-gaze limitation, en coup the sabre, morphea, and hypothyroidism.

Case summary

A 41-year-old woman presented with slowly progressive right enophthalmos that started 5 years previous. With a Hertel exophthalmometer, the right eye measured 10 mm, and the left eye 13 mm. Right enophthalmos and atrophy of sub-cutaneous tissues of right side orbit were observed. Axial scan of computerized tomography shows atrophy of right side orbital subcutaneous tissues without bony abnormality. At primary gaze, 4 prism diopter hypotropia was observed in the right eye. Up-gaze was limited in both eyes. Visual acuity was 1.0 in both eyes. No specific sign was found in her anterior segment. The patient had experienced right side alopecia 20 years prior and had undergone biopsy, which proved morphea. Linear en coup de sabre morphea was found on the right forehead and scalp. Hypothyroidism due to Hashimoto’s thyroiditis was diagnosed based on a blood test, which also showed positive antinuclear antibody. Thus, the patient was diagnosed with Parry-Romberg syndrome associated with hypothyroidism.

Conclusions

Clinicians need to consider the rare disease Parry-Romberg syndrome when a patient with ophthalmologic symp-toms such as enopthlamos or eye movement disorders is accompanied by dermatologic symptoms such as linear en coup de sa-bre morphea or alopecia.

Keywords: Parry-Romberg syndrome, Progressive facial hemiatrophy

References

1. Kim YJ, Hong YJ, Kim HB. Romberg’s syndrome. J Korean Ophthalmol Soc. 1979; 20:567–71.

2. Choi SI, Yang JH, Park YL. . Parry-Romberg syndrome with band-like linear alopecia. Korean J Dermatol. 2012; 50:456–9.

3. Kini TA, Prakash VS, Puthalath S, Bhandari PL. Progressive hemi-facial atrophy with ciliary body atrophy and ocular hypotony. Indian J Ophthalmol. 2015; 63:61–3.

4. Bucher F, Fricke J, Neugebauer A. . Ophthalmological mani-festations of Parry-Romberg syndrome. Surv Ophthalmol. 2016; 61:693–701.

5. Madasamy R, Jayanandan M, Adhavan UR. . Parry Romberg syndrome: a case report and discussion. J Oral Maxillofac Pathol. 2012; 16:406–10.

6. Hakin KN, Yokoyama C, Wright JE. Hemifacial atrophy: an un-usual cause of enophthalmos. Br J Ophthalmol. 1990; 74:496–7.

7. El-Kehdy J, Abbas O, Rubeiz N. A review of Parry-Romberg syndrome. J Am Acad Dermatol. 2012; 67:769–84.

8. Fea AM, Aragno V, Briamonte C. . Parry Romberg syndrome with a wide range of ocular manifestations: a case report. BMC Ophthalmol. 2015; 15:119.

9. Duymaz A, Karabekmez FE, Keskin M, Tosun Z. Parry-Romberg syndrome: facial atrophy and its relationship with other regions of the body. Ann Plast Surg. 2009; 63:457–61.

10. Mazzeo N, Fisher JG, Mayer MH, Mathieu GP. Progressive hemi-facial atrophy (Parry-Romberg syndrome). Case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1995; 79:30–5.

11. Won TH, Park SD, Seo PS. A Case of Parry-Romberg syndrome with shortening of ipsilateral lower extremity. Korean J Dermatol. 2008; 46:1216–20.

12. Jo HY, Hong SH, Oh CH. A case of Romberg syndrome. Korean J Dermatol. 1995; 33:349–52.

13. Longo D, Paonessa A, Specchio N. . Parry-Romberg syndrome and Rasmussen encephalitis: possible association. Clinical and neuroimaging features. J Neuroimaging. 2011; 21:188–93.

14. Asai S, Kamei Y, Nishibori K. . Reconstruction of Romberg disease defects by omental flap. Ann Plast Surg. 2006; 57:154–8.

15. Koh KS, Oh TS, Kim H. . Clinical application of human adi-pose tissue-derived mesenchymal stem cells in progressive hemi-facial atrophy (Parry-Romberg disease) with microfat grafting techniques using 3-dimensional computed tomography and 3-di-mensional camera. Ann Plast Surg. 2012; 69:331–7.

16. Avelar RL, Göelzer JG, Azambuja FG. . Use of autologous fat graft for correction of facial asymmetry stemming from Parry- Romberg Syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2010; 109:e20–5.

17. Tollefson MM, Witman PM. En coup de sabre morphea and Parry-Romberg syndrome: a retrospective review of 54 patients. J Am Acad Dermatol. 2007; 56:257–63.

18. de Crecchio G, Forte R, Strianese D. . Clinical evolution of neuroretinitis in Parry-Romberg syndrome. J Pediatr Ophthalmol Strabismus. 2008; 45:125–6.

19. Park DH, Kim IT. Patient with Parry-Romberg syndrome compli-cated by Coat’s syndrome. Jpn J Ophthalmol. 2008; 52:520–2.

Figure 1.

Clinical photograph and Computed tomography showing right enophthalmos. (A) Right enophthalmos was observed at clinical photograph. (B) Axial scan of computed tomography shows right enophthalmos. There was no specific bony abnormality.

Figure 2.

At primary gaze, 4 prism diopter hypotropia was observed at right eye. Up-gaze was limited in both eyes.

Figure 3.

Clinical photographs showing alopecia and linear scleroderma. (A) Linear hair loss was observed on patient’s right scalp (yellow arrows). (B) Clinical photograph shows linear scleroderma (en coup de sabre, red arrows) on patient’s forehead with mild hemifacial atrophy on right side.

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