The Ophthalmology Department,  Universiti Malaysia Sarawak (UNIMAS), Kuching, Sarawak.

The Ophthalmology Department, Sarawak General Hospital, Kuching, Sarawak, East Malaysia.

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Case 33a

by Dr. Ngo Chek Tung & Professor Chua Chung Nen

 

A 54 year-old woman with type I neurofibromatosis presented with a 3-year history of progressive left ptosis. She had no previous surgery or another history of note.

 

On examination, she had a left complete ptosis with poor levator function. The eyelid (Figure 1 and 2) contained a rubbery mass that appeared to involve most of the upper tarsal plate with extension into the levator. Her vision measured 6/12 in the right eye and 6/18 in the left eye. Both vision could be improved to 6/9 with pinholes. The anterior segments revealed some Lisch's nodules in the iris. The posterior segment in both eyes were unremarkable.


She was keen to undergo surgery to improve her appearance and to restore the vision.

 

Figure 1. Picture showing multiple nodular lesions of the face and eyelids.

 

Figure 2. Lateral view of the left upper eyelid. 

 

a. What is the diagnosis?

 

Plexiform neuromas of the upper eyelid. These are diffuse proliferation in the nerve sheaths that give rise to thickened nerves. The lesions may be deeply infiltrative and in rare cases may become malignant.

 

 

b. How would you excise the lesion and reconstruct the upper eyelid?

 

There are several approaches. One of the ways is to excise the whole upper eyelid containing the lesion and reconstruct the defect using the Cutler-Beard's method (see case 8). However, this method has 2 main disadvantages:

i. The need to occlude the eye for a certain period to allow vascularization and

ii. The absence of upper eyelid lashes is cosmetically unsatisfactory.

 

In our patient, we excise the central big lesion and reconstruct the upper lids using the medial and lateral stumps of the eyelids. This technique was possible because the upper eyelid has been expanded through stretching from the neuroma.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Figure 3. The upper lid was incised horizontally above the lesion to expose the tumour (a and b).The lesion was excised from the levator muscle. The lesion involves the whole central tarsal plate. A complete excision of the central lesion was performed (c) leaving behind the skin and medial and lateral stumps. The medial stump was stretched laterally to cover the medial half of the eye. The levator was attached to the upper tarsal plate using continuous suture (e and f). The lateral stump also contained some neuroma and therefore needed to be trimmed before suturing it to the medial stump (g, h, i and j).

Figure 4. Post-operative appearance at one week.

 

Reference:

 

1. DiFrancesco LM, Codner MA, McCord CD. Upper eyelid reconstruction. Plast Reconstr Surg. 2004 Dec;114(7):98e-107e.

 

2. Verity DH, Collin JR. Eyelid reconstruction: the state of the art. Curr Opin Otolaryngol Head Neck Surg. 2004 Aug;12(4):344-8. Review.