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Year : 2019  |  Volume : 6  |  Issue : 1  |  Page : 16-19

Role of scleral tectonic graft in saving the vision of a child with apert syndrome

1 University of Khartoum, Faculty of Medicine, Department of Ophthalmology; Consultant Ophthalmologist, Albasar International Foundation, Khartoum, Sudan
2 Chief Executive MD, Albasar International Foundation, Makkah Eye Hospital; Assistant Professor of Community Medicine, Ibn Sina University, Faculty of Medicine, Department of Community Medicine, Khartoum, Sudan

Date of Submission28-Mar-2019
Date of Acceptance04-May-2020
Date of Web Publication13-Jul-2020

Correspondence Address:
Dr. Dinah Saad Ahmad Ibrahim
Albasar International Foundation, Makkah Eye Hospital, Department of Ophthalmology, University of Khartoum, Khartoum
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/bijo.bijo_2_19

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Apert syndrome (AP) is a rare form of acrocehpalosyndactyly with autosomal-dominant transmission. The most common ocular features of patients with AP include proptosis, shallow orbits, and hypertelorism; they can have exotropia and antimongoloid slant of the palpebral fissures. The aim of this report was to study the role of scleral tectonic patch graft (STPG) in managing the corneal exposure due to AP. This was the clinical case report of a 9-year-old girl with AP, presented with right eye corneal perforation. She was treated with homologous STPG instead of tectonic corneal graft with good improvement of her visual acuity (VA). The outcome of the patient VA after 1 year was the improvement of the vision in the affected eye from the hand movement to 6/12, which was considered very satisfactory to the child and her family. This case report revealed that tectonic scleral patch graft is a sight-saving procedure in condition where the scleral tissue is readily available rather than the corneal tissue that needs the presence of cornel banks.

Keywords: Apert syndrome, craniocynostosis, scleral tectonic patch graft

How to cite this article:
Ibrahim DS, Ibrahim MK. Role of scleral tectonic graft in saving the vision of a child with apert syndrome. Albasar Int J Ophthalmol 2019;6:16-9

How to cite this URL:
Ibrahim DS, Ibrahim MK. Role of scleral tectonic graft in saving the vision of a child with apert syndrome. Albasar Int J Ophthalmol [serial online] 2019 [cited 2022 Aug 14];6:16-9. Available from: https://www.bijojournal.org/text.asp?2019/6/1/16/289604

  Introduction Top

Apert syndrome (AP) is a rare form of acrocehpalosyndactyly with autosomal-dominant transmission. It is characterized by craniosynostosis, midface hypoplasia, and syndactyly of the hands and feet.[1] It was first mentioned as early as 1842 by Baumgartner and in 1894 by Wdeaton.[2] In 1906, a French pediatrician, Eugene Apert first described nine people with similar disorders. Since he was the first to do so, his name is associated with this syndrome.[3] The prevalence of AP is estimated to be 1:65000 in newborns and account for about 4.5% of all cases of craniosynostosis.[4]

The familial pattern affects males and females in equal proportion. The presence of a family history is not significant since most of the cases are sporadic and are associated with an older paternal age.[5] More than 98% of cases are caused by two mutations in the fibroblast growth factor receptor2 gene, located on the 10th chromosome (10q25-26). The first mutation replaces the amino acid serine instead of tryptophan at position 252, and the second mutation replaces proline instead of arginine at position 253 (Pro253Arg).[6]

Clinically, new-born infants with AP present with early closure of their coronal sutures, resulting in cloverleaf-skull appearance. They have a flattened occiput, steep forehead, and a horizontal groove over the supraorbital ridge. They also have midfacial hypoplasia with a parrot-beak nose, low-set ears, and an arched palate or the cleft palate. Syndactyly of the hands and feet are one of the main characteristics of this syndrome, whereas congenital anomalies of the heart, lungs, and kidneys have been reported as well.

The most common ocular features of patients with AP include proptosis, shallow orbits, and hypertelorism. They can have exotropia and antimongoloid slant of the palpebral fissures. Morbidities from corneal ulcers are often severe in patients with AP. These patients are at increased risk of developing corneal ulcers because of the compromised corneal environment secondary to exophthalmos. According to Khong JJ by the age of 4 years, exposure keratopathy and corneal scarring occur in 13% of patients and optic atrophy in 8% of patients, and at least 14% of patients develop amblyopia and 60% have strabismus.[7]

  Case report Top

This was the case of a 9-year-old girl presented to the clinic with a 10-day history of pain and redness of her right eye. On examination, the girl was of short stature with mid-face hypoplasia and proptosis. She has syndactyly of the hands and feet. She had been diagnosed with AP 8 years back, and she had been followed by a pediatrician since that time [Figure 1].
Figure 1: Child's facial features

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On examination, her visual acuity (VA) of the right eye was hand movement (HM), and on the left eye was 6/6. Right eye examination showed conjunctival and cilliary congestion, corneal defect at 4 and 5 O'clock with iris prolapse, and shallow anterior chamber (AC). The size of the corneal defect was 3.5 by 4.0 mm, and Seidel test was positive. The lens was clear with no cataractous changes. Fundus examination of the right eye was not clear.

Examination of the left eye anterior and posterior segments was normal. B-scan ultrasound examination of both eyes was normal, with no retinal or choroidal detachment. The diagnosis of right eye exposure keratopathy with corneal melt and perforation was made. The management plan was homologous scleral patch grafting instead of using a tectonic corneal graft, which was not available at that time. The scleral graft would seal the defect and reform and maintain the right AC. Written consent from her parents was obtained before the surgical procedure. An ethical clearance was obtained from the Institutional Review Board at Makkah Eye Hospitals, and written consent was obtained from her parents to report the case.

Under general anesthesia and complete aseptic conditions, the corneal defect was measured as 3.5 by 4 mm; scleral tissue measuring 5 by 6 mm was prepared from the homologous tissue. Using 15° blade, a side port at 10 O'clock was made, and then, viscoelastic was injected for the formation of the AC. A preserved scleral homologous tissue was applied to the defect and then sutured using 10.0 nylon; a preserved amniotic membrane was sutured on top of the scleral tissue with 10.0 nylon. Viscoelastic was washed from the AC, and then, a bandage contact lens was applied, and subconjunctival steroid was injected.

Postoperative, the patient was given paracetamol syrup (salzone 120 mg/5 ml) every 8 h for 3 days, moxifloxacin (vigamox 0.3%) eye drops for 1 month; prednisolone eye drops (optipred 1%) was given and tapered over a period of 1 month. On the 1st postoperative day, the girl was happy; her right eye was quiet, AC well-formed and her vision improved from HM to 6/60. Three months later, sutures were removed, and right lateral tarsorrhaphy was performed [Figure 2]. Lateral tarsorrhaphy is recommended by many studies for patients with AP, since it protects the cornea from damage by exposure keratopathy and save the patient vision.[8] After 1 year follow-up, the scleral graft was nonvascularized [Figure 3], and her vision continued to improve up to 6/12 with glasses (subjective refraction + 0.50/+2.50 × 90). Currently, the patient is on lubricants and tears substitutes to avoid the recurrence of exposure keratopathy and advised for regular follow-up every 6 months.
Figure 2: Scleral graft 3 months after surgery

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Figure 3: Scleral graft after 1 year

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  Discussion Top

This case was treated and reported at a tertiary care level ophthalmic hospital in Sudan. The aim of this report was to document the role of scleral tectonic graft in saving sight where there is no available corneal tissue or there is a limitation in performing corneal graft in such an emergency condition. The outcome of the procedure performed in this case was very satisfying and considered as an advance improvement if we compare a case presented with perforated eye and VA of HM to a well-formed AC with a VA of 6/12 after the procedure.

Implantation of the sclera into the cornea was first studied in rabbits by Thomas and in human eyes by Larsson. Levartovsky et al. reported the use of homologous preserved sclera in a 3-year-old child to repair a corneal perforation with a 2.5 mm diameter defect, which occurred during pars plana vitrectomy for an acute corneal ulcer and endophthalmitis. They used scleral tissue instead of corneal tissue which was not available at that time.

The aim of the procedure in stabilizing the globe was achieved, and visual outcome at 3 years later was a useful vision of 6/18.[9]

The case of a lorry driver with a traumatic peripheral corneal perforation of 1.5 mm in diameter reported by Prydal was managed with a partial-thickness autologous scleral patch graft to seal the corneal defect and after 1 year UCVA was 6/5.[10] The outcome in this case is much better than in our case, and this can be related to the smaller size of corneal defect, which induces less astigmatism and hence a better visual outcome.

Sealing the corneal defect in such cases is the cornerstone in saving the vision and even the eyes of the patients. Although the standard procedure is to use corneal tissue rather than scleral tissue, it is considered that scleral use in cases where there is no corneal tissue available is a considerable alternative. In addition, the advantages of scleral tissue in this case are that the collagen fibrils within the sclera are more irregular in diameter and arrangement than those in the cornea, thus the scleral tissue is tougher and of greater tensile strength than the corneal tissue. The outcome is very good and affected only by some factors related not directly to the tissue itself or the procedure if performed in a proper way, but to the condition of the eye and the size of the defect.

  Conclusion Top

In the absence or limitation of corneal buttons for corneal graft, the scleral tissue can be used for closing the corneal defect. This is considered as sight saving in many countries where the scleral tissue is readily available rather than the corneal tissue that requires the presence of corneal bank which is not usually available in most of the cases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images, and other clinical information to be reported in the journal. The patient 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 S, Munshi A. Research advances in Apert syndrome. J Oral Biol Craniofac Res 2018;8:194-9.  Back to cited text no. 1
Fedakâr A, Sakc Z. Apert syndrome: Case presentation. SM J Pediatr Surg 2016;2:1034.  Back to cited text no. 2
Bhatia PV, Patel PS, Jani YV, Soni NC. Apert's syndrome: Report of a rare case. J Oral Maxillofac Pathol 2013;17:294-7.  Back to cited text no. 3
[PUBMED]  [Full text]  
Hilton C. An exploration of the cognitive, physical and psychosocial development of children with Apert syndrome. Int J Disabil Dev Educ 2017;2:198-210.  Back to cited text no. 4
Kanski J. Developmental Malformations and Anomalies. Clinical Ophthalmology: A Systematic Approach. 6th ed., Ch. 3. Edinburgh: Elsevier Butterworth-Heinemann; 2007. p. 64.  Back to cited text no. 5
Fanganiello R, Sertié A, Reis M, Yeh E, Oliveira N, Bueno D, et al. Apert p. Ser252Trp mutation in FGFR2 alters osteogenic potential and gene expression of cranial periosteal cells. Mol Med 2007;13:422-42.  Back to cited text no. 6
Khong JJ, Anderson PJ, Hammerton M, Roscioli T, Selva D, David DJ. Differential effects of FGFR2 mutation in ophthalmic findings in Apert syndrome. J Craniofac Surg 2007;18:39-42.  Back to cited text no. 7
Saleem M. Early bilateral tarsorrhaphy is the simple ophthalmic surgical procedure to save the vision in Craniosynostosis syndromes. Albasar Int J Ophthalmol 2013;1:29-37.  Back to cited text no. 8
Levartovsky S, Springer A, Leiba H, Marcovich AL, Pollack A. Homologous scleral graft for corneal perforation in a child. Cornea 2008;27:230-1.  Back to cited text no. 9
Prydal JI. Use of an autologous lamellar scleral graft to repair a corneal perforation. Br J Ophthalmol 2006;90:924.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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