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Ophthalmic features and management outcomes of 30 children having Goldenhar syndrome

  • Manpreet SinghEmail author
  • Manpreet Kaur
  • Aditi Mehta Grewal
  • Sonam Yangzes
  • Deepti Yadav
  • Zoramthara Zadeng
  • Pankaj Gupta
Original Paper
  • 20 Downloads

Abstract

Purpose

To study the ophthalmic clinical profile and the management outcomes of children having Goldenhar syndrome (GS).

Methods

Retrospective review of included children fulfilling the diagnostic criteria of GS was performed. The demography, ophthalmic features, systemic anomalies, and treatment outcomes were recorded manually from patient files. An ENT, pediatrics, cardiology, and orthopedics consultation was sought for all GS patients before taking up for any ophthalmic surgical procedure. The anatomical (ocular surface and eyelid) and functional {vision and extraocular movements (EOM)} factors were exclusively studied. We ensured a minimum postoperative follow-up of 12 months, and our data were compared with the major studies featuring ‘ophthalmic features’ of GS.

Results

Totally 30 children (females = 18, 60%) were included with a median presenting age of 48 months. Twenty-seven (90%) had unilateral ophthalmic involvement with major features being upper eyelid coloboma (n = 25, 75.76%), lipodermoid (n = 18, 54.55%), and limbal dermoid (n = 10, 30.3%). Diminution of visual acuity was recorded in 22 (73.3%), while five (16.7%) had limitation of EOM. Systemically, the prominent features included hemifacial hypotrophy (100%), auricular anomalies (80%), cardiac anomalies (10%), and scoliosis (6.67%). The eyelid colobomas were repaired with the direct closure ± cantholysis technique or using a Tenzel’s flap. All children had satisfactory anatomical and functional outcomes after ophthalmic surgical interventions without any significant complications.

Conclusion

The tailored ophthalmic surgical intervention(s) provides satisfactory restoration of anatomy and functionality of the eye. These children need specific multi-discipline consultations for the holistic management and complete care.

Keywords

Goldenhar syndrome Eyelid coloboma Limbal dermoid Lipodermoid Dermolipoma 

Notes

Funding

No funding was received for this research.

Compliance with ethical standards

Conflict of interest

All authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the Post Graduate Institute of Medical Education and Research, Chandigarh, India, and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

An informed consent was obtained from all individual participants included in the study.

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Copyright information

© Springer Nature B.V. 2019

Authors and Affiliations

  1. 1.Department of OphthalmologyAdvanced Eye Centre, Post Graduate Institute of Medical Education and ResearchChandigarhIndia

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