Bilateral Plexiform Neurofibromatosis Involving Brachial Plexus and Lumbosacral Plexus

A 22-year-old young female, a known case of neurofibromatosis type 1, presented to us with 3-month history of severe neurogenic pain of progressive severity in both upper and lower limbs. She on examination had café-au-lait pigmented patches all over the body with multiple cutaneous neurofibromas over the trunk and extremities. There was no gross sensorimotor neurological deficit. Gadolinium-enhanced magnetic resonance imaging scan revealed bilateral plexiform neurofibromas (PNs) involving roots of brachial plexus and lumbosacral plexus. In addition, there was extensive involvement of both sciatic nerves by multiple neurofibromas in gluteal and thigh regions.

PN is a common entity associated with neurofibromatosis 1. It occurs in multiple numbers as twisted masses and grow along the axis of the nerve. Its growth pattern is infiltrating and separating normal nerve fascicles. Malignant transformation is the known associated complication. Surgery is only indicated when PNs start causing functional compromise, deformity, or intractable pain.[1] Only two cases have been reported so far in the literature, as observed in our patient ([Figs. 1] [2] [3]).[2] [3]

Fig. 1 Short tau inversion recovery coronal magnetic resonance imaging of brachial plexus showing multiple neurofibromas along bilateral roots of brachial plexu.

Fig. 2 Short tau inversion recovery coronal magnetic resonance imaging of lumbosacral plexus showing multiple plexiform neurofibromas along lumbosacral roots.

Fig. 3 Short tau inversion recovery coronal magnetic resonance imaging of thigh showing multiple plexiform neurofibromas along bilateral sciatic nerves.

 

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Conflict of Interest

None declared.

• References

• 1 Serletis D, Parkin P, Bouffet E, Shroff M, Drake JM, Rutka JT. Massive plexiform neurofibromas in childhood: natural history and management issues. J Neurosurg 2007; 106 (Suppl. 05) 363-367
• 2 Kumar A, Devi BI. Enigma in management of plexiform neurofibromas of bilateral brachial and lumbosacral plexuses: shall we let nature take its course?. Neurol India 2019; 67 (Suppl) S154-S156
• 3 Souza FH, Dabdoub CB, Bernardino SN, Silveira EN, Litvin S, Azevedo-Filho H. Bilateral plexiform neurofibromas of the brachial and lumbosacral plexuses. Arq Neuropsiquiatr 2013; 71 (02) 128-130

Publication History

Article published online:
26 October 2020