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Year : 2021  |  Volume : 59  |  Issue : 1  |  Page : 107-108

A rare case of goldenhar syndrome with situs inversus

Department of Cornea, RIO GOH, Chennai, Tamil Nadu, India

Date of Submission16-Oct-2020
Date of Acceptance10-Nov-2020
Date of Web Publication27-Mar-2021

Correspondence Address:
Dr. D Sakthi Priya
F7, Halls Towers, Thamizh Salai, Egmore, Chennai - 600 008, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/tjosr.tjosr_156_20

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How to cite this article:
Priya D S, Vadivelu SD, Kumar G T, Meenakshi B, Gandhi N. A rare case of goldenhar syndrome with situs inversus. TNOA J Ophthalmic Sci Res 2021;59:107-8

How to cite this URL:
Priya D S, Vadivelu SD, Kumar G T, Meenakshi B, Gandhi N. A rare case of goldenhar syndrome with situs inversus. TNOA J Ophthalmic Sci Res [serial online] 2021 [cited 2021 Oct 20];59:107-8. Available from: https://www.tnoajosr.com/text.asp?2021/59/1/107/312297

A 12-year-old male child presented with complaints of a yellowish-white mass in the right eye since birth and defective vision in the right eye, the informant being his father. The visual acuity in the right eye was 3/60 and the left eye was 6/6. Ocular examination of the right eye revealed limbal dermoid [Figure 1]a from 6'O clock to 10'O clock position encroaching over the cornea with a temporal extent up to the lower lid and the left eye revealed upper lid coloboma [Figure 1]b; rest of the anterior segment was normal. General and systemic examination revealed right preauricular tag [Figure 1]c, scoliosis [Figure 1]d, and hemivertebra. In addition, he had situs inversus and dextrocardia [Figure 1]d. Hence, a diagnosis of Goldenhar syndrome with situs inversus was made. The patient was taken for right eye limbal dermoid excision with lamellar patch graft [Figure 2]a and [Figure 2]b.
Figure 1: (a) Right eye depicting limbal dermoid encroaching cornea. (b) Left eye upper lid coloboma. (c) Right preauricular tag. (d) Red arrow showing scoliosis, green arrow showing dextrocardia

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Figure 2: (a) Postoperative picture showing lamellar patch graft in the right eye. (b) Excised limbal dermoid

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  Discussion Top

Goldenhar syndrome or oculo-auriculo-vertebral dysplasia is characterized by a heterogeneous constellation of malformation, classically involving the face, eyes, ears, and vertebral column.[1] The incidence of Goldenhar syndrome has been reported to be 1:3500–1:5000, with a male-to-female ratio of 3:2. Most cases of Goldenhar disease occur sporadically in people with no family history. Goldenhar disease may affect the heart, lungs, kidneys, and central nervous system. It is reported that the right side is more severely affected than the left.[2] A dermoid cyst is a soft, yellowish, globular swelling of varying shape and size arising from the lid, outer angle of orbit, eye brow, conjunctiva, and limbus. The epibulbar dermoid and lipodermoid are choristomas[3] which are normal tissue in abnormal position. The most frequent site of involvement is believed to be lower temporal segment.[4] Although Goldenhar syndrome itself is not very rare, its association with situs inversus made this case a rare entity.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's parent has given his consent for his images and other clinical information to be reported in the journal. The patient's parent understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Das A, Majumdar S, Chatterjee SS. Atypical case of oculofacioauriculovertebral dysplasis (Goldenhar Gorlin syndrome. Indian J Ophthalmol 1999;47:131-3.  Back to cited text no. 1
  [Full text]  
Tiwari US, Kujur R, Paul RK, Singh N. Anterior segment optical coherence tomography (ASOCT) in Goldenhar syndrome. J Clin Ophthalmol Res 2014;2:159-60.  Back to cited text no. 2
  [Full text]  
Baum JL, Feingold M. Ocular aspects of Goldenhar's syndrome. Am J Ophthalmol 1973;75:250-7.  Back to cited text no. 3
Mohan M, Mukherjee G, Panda A. Clinical evaluation and surgical intervention of limbal dermoid. Indian J Ophthalmol 1981;29:69-73.  Back to cited text no. 4
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