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Asyndromic isolated unilateral Tessier cleft 8 with euryblepharon
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  1. Sahil Agrawal1,
  2. Sujeeth Modaboyina1,
  3. Saloni Gupta2 and
  4. Deepsekhar Das1
  1. 1Ophthalmology, Dr Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, Delhi, India
  2. 2Ophthalmology, Northern Railway Central Hospital, New Delhi, India
  1. Correspondence to Dr Deepsekhar Das; doc.deep.das{at}gmail.com

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Description

Tessier developmental cleft anomalies are exceedingly rare conditions. The nature and extent of the anomaly vary according to the cleft number.1 Although the exact incidence is unclear, it is estimated between 1.4 and 4.9 per 100 000 live births.2

A 13-year-old female patient presented to an ophthalmology outpatient department with problems of a large palpebral aperture on the left side along with watering and foreign body sensation. Her parents stated that the ocular deformity had been there since birth. On general examination, the patient was alert, conscious and cooperative with stable vitals. There was obvious left-sided malar hypoplasia (figure 1A).

Figure 1

(A) Clinical picture of the patient showing left-sided malar hypoplasia. (B and C) Clinical picture of the patient showing left euryblepharon, eyebrow madarosis, upper eyelid coloboma with entropion, lateral canthal dystopia with an elevation(B), stretched lower eyelid margin, lower eyelid ectropion with lacus lacrimalis lateralis and temporal bulbar conjunctival keratinisation.

On ocular adnexal examination, there was a lateral coloboma of the left eyebrow along with the inward rotation of the lateral half of the upper eyelid with a coloboma. The lateral canthus was dystopic, with a bunch of eyelashes located on top of it (figure 1B). There was a left-sided euryblepharon as the horizontal aperture of the left side was 34 mm, whereas, on the right side, it was 29 mm. The lower eyelid appeared stretched with mild ectropion, and a prominent lacus lacrimalis lateralis was noted. The lateral bulbar conjunctiva also had evidence of keratinisation (figure 1C). The ocular adnexa on the right side and rest of the anterior and posterior segments of either eye was within normal limits with visual acuity of 6/6 on Snellen’s chart.

Based on the clinical features, a diagnosis of Tessier cleft number 8 with euryblepharon was made. The patient underwent lateral canthoplasty (the lateral canthal angle was opened and the lateral eyelid reanchored to the periosteum of the lateral orbital rim using 6–0 prolene sutures) along with upper lid coloboma repair and had a cosmetically favourable outcome.

Tessier classified congenital craniofacial clefts based on their anatomical position by giving them numbers from 0 to 14.1 3 They can be further subdivided into the midline, paramedian, orbital and lateral clefts.4 The lateral clefts are in a horizontal plane with respect to the face, and they include numbers from 6 to 8.1 The lateral clefts are characteristically associated with some syndromes, likely Treacher Collin and Goldenhar syndrome. In Treacher Collins syndrome, clefts number 6, 7 and 8 occur in various combinations; however, Goldenhar syndrome is associated with cleft number 8.5 Tessier cleft 8 involves both bone and soft tissue and extends from the lateral canthus to the temporal region and often, a bone cleft along the frontozygomatic suture is associated with it. The lateral canthus in cleft 8 characteristically has an elevation that breaks the continuity of the orbicularis muscle leading to malfunctioning of the orbicularis muscle and other structures attached to the lateral canthus.1

Our patient had an elevation located at the lateral canthus with a patch of eyelashes on top. She had no other syndromic features to diagnose as Goldenhar or Treacher collin syndrome. The surgical corrections described for cleft 8 involves the use of cleft skin and local flaps.6 Few authors have described the surgical resection of dermolipoma and suturing the abnormal fibrous tissue and muscles to the periosteum of the lateral orbital rim.4 In our case, we were able to obtain a satisfactory cosmetic outcome by employing lateral canthoplasty along with upper eyelid coloboma repair.

Patient’s perspective

We have been told that our child has a uncommon anomaly and are glad that has been taken care of.

Learning points

  • Asyndromic isolated unilateral Tessier cleft 8 with euryblepharon is an extremely rare entity, and this is the first known documented case from the Indian subcontinent.

  • A lateral canthoplasty with repair of coloboma provides satisfactory outcome in such cases.

Ethics statements

Patient consent for publication

References

Footnotes

  • Contributors SA is the primary point of contact and operating surgeon. SM did final review of the manuscript. SG helped in drafting the manuscript. DD acts as a guarantor and operating surgeon.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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