Solitary Eyelid Neurofibroma: Case Report

The Merriam-Webster’s Collegiate Dictionary defines “neurofibroma” as “a fibroma composed of nervous and connective tissue” [1]. As it usually renders, 1892 was the earliest year of its use. Since then, emphasis has been on generalized bodily distribution of these lesions. Elsewhere [2], the appearances in infancy and childhood among the Ibo ethnic group were presented personally [3]. Here, this paper compares its odd appearance in the eyelid with reference to comparative examples from India [4-8], Korea [9,10], Taiwan [11] and USA [12].

UB, a 9-year-old girl, was brought to the Children’s Ward at the National Orthopaedic Hospital, Enugu, Nigeria, under the care of Dr Achebe and Dr Ogbonnaya. The complaint of her mother was swelling of the child’s left upper eyelid from birth. It progressively increased in size. Therefore, incisional biopsy was carried out under the impression of this being due to “Orbitofacial plexifom neurofibromatosis (Type 1).” The specimen was received by the senior author (WIBO). It was a 5cm elongated wedge containing the skin, and an underlying tumorous mass. On microscopy, the tumor was benign. The nuclei tended to be wavy. Abortive nerves abounded. Myxomatous change was noted in parts. Accordingly, neurofibromatosis was confirmed.

French authors wrote that neurofibromatosis of the orbitopalpebral complex is a debilitating disease. Their series demonstrated that a tailored often radical palpebral remodelling in orbitopalpehral neurofibromatosis may provide functional and aesthetic benefit. Our case was handled with little difficulty. Age at presentation is interesting. On looking at the Indian series, the range included 19 years [5], 64 years [6] and 53 years [8]. Clearly, our local patient came in early in good time. Indeed, it was only bettered by the 12-year-old Korean patient [9]. From USA, the authors concluded that “Ultimately, orbital exenteration may be needed for rehabilitation and cosmesis” [12]. One Indian patient underwent surgery for the same lesion twice [8]. Our patient has fared better. It may be added that the eyelid neurofibroma may present as a tarsal cyst [6], mimick chalazion [8,11], or simulate optic nerve glioma [9]. In contrast, the local surgeons were able to readily diagnose “Orbital plexiform neurofibromatosis (Type 1).” In Korea, a 34-year-old man, who underwent excision of a neurofibroma on the right eyelid, returned following orbital dystopia which had to be corrected [10]. Our patient’s recovery was uneventful..

1. Merriam-Webster’s Collegiate Dictionary. Mass, (12th edn), Springfield, Merriam-Webster Inc., Massachusetts, USA, p. 833.
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3. Basden GT (1966) Among the Ibos of Nigeria, 1921. Reprinted Cass, London.
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