Good evening revered Curofians. Identify the lesion and give a brief description. Best regards. ( Credit : Internet )
It's a Plexiform Neurofibromas, Plexiform neurofibromas that infiltrate the orbit, temporal region, or eyelids are less common than optic nerve gliomas but are potentially vision threatening. Orbitotemporal neurofibromas can cause strabismus and proptosis and change globe length. In young children, amblyopia may result from obscuration of the visual axis secondary to infiltration and edema of the orbit and eyelids. In a series of 21 children with plexiform neurofibroma of the orbit and temporal areas, 6 patients had only lid involvement and 3 patients had bilateral neurofibromas. Amblyopia secondary to the orbitotemporal plexiform neurofibroma was present in 62%, primarily from ptosis and anisometropia, The following are characteristics of plexiform neurofibromas of the eyelid: *Thickening of upper lid *S-shaped deformity *"Bag of worms" sensation Congenital glaucoma ipsilateral to plexiform neurofibromas has been described as a variation of anterior segment developmental disorders. Diagnostic Evaluation - When assessing a patient with suspected ophthalmologic manifestation(s) of neurofibromatosis (NF) type 1, also consider iris nevi, glaucoma, juvenile glaucoma, tuberous sclerosis, and NF-2. Careful ophthalmologic examination is necessary with periodic follow-up care, and special attention should be directed to optic nerve function to detect previously undiagnosed gliomas of the optic nerve. No laboratory tests are pertinent in evaluating ophthalmologic manifestations of NF-1. However, tissue biopsy of skin lesions is occasionally necessary to confirm the diagnosis of NF-1. Histological Features - Histologic findings of neurofibromas include a typical appearance of markedly enlarged nerves surrounded by thickened perineural structures, which vary depending on their location. As noted previously, Lisch nodules of the iris are histopathologically identical to benign iris nevi.
This is plexiform Neurofibromatosis. This is benign tumor that originates from nerve sheath cells or subcutaneous peripheral nerves and can involve multiple fascicles. Massive PNF results in functional disability and disfigurement. They occur in as much as in 30% of the patients with NF type 1. Such tumours are generally present at birth and often progress during early childhood. The condition can cause disfigurement. Most cases require surgery. Complete excision is not possible due to infiltrating nature of the tumour.
Rare DD would be HEMANGIOMA. Man with no face. This is because of abnormalities in capillaries and Veins. The tumour has taken over his mouth, tongue, ballooning his lips, twisting his gums, breaking his teeth off. Treatment Surgical excision bit by bit is the only option. This may be the ghostly face on the earth
I am highly thankful to dear my Kaji who posts very interesting cases, I would request him kindly tag Endocrinology too while posting the cases.
plexiform neurofibromatosis arise from multiple nerve bundles . they are difficult to treat and can turn malignant.
Massive Facial plexiform neurofibromatosis.It is neurofibromatosis type 1.Reconstructive surgical treatment with subtotal excision of tumor and re draping of facial skin is the only treatment .But unfortunately due to infiltrating nature of this benign tumor in head and neck there is higher rate of recurrence which again requires multiple surgeries.Moreover due to large tumor size it involves multiple nerves thus increasing the risk of neurological dysfunction after performing tumor resection surgery.
Plexiform neurofibromatosis.
Von Riclinghausen disease neurofibromatosis type 1
SUGGESTIVE OF PLEXIFORM NEUROFIBROMATOSIS
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18years old boy, right side upper eye lid extremely difficult since to the birth. *Chief Complaints* Vision problems, no any other complain. *History* Extremely difficult right upper eye lid since birth. *Vitals* Puls 72 bpm SpO2 99% *Physical Examination* One side vision (left eye) normal. Physically fitness positive for walking, running, cycling, reading and riding. *Investigations* Please suggest essential investigation. *Diagnosis* Please suggest, what should be done? *Management* Please suggest management???
Dr. Pranab Bera1 Like18 Answers - Login to View the image
28 yr male, with left orbital dystopia, ptosis and pulsatile exopthalmos. Opthalmic exam. ...visual acuity 20/20 OD (without correction) , 20 /400 OS (18 diopter sphere ). Presence of lisch nodules in iris bilaterally. Diagnosis?
Dr. Mrs Husna Shahzad1 Like6 Answers - Login to View the image
Today one patient came for regular Eye check. Pt is k/c/o Neurofibromatosis. his left upper lid is grossly tylosed with complete ptosis. RE BCVA is 6/9 and LE VA is PL+ . SLE shows Phacodonesis with early spokes in lens. Suggest views.
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