Fibrolipomatous hamartoma of the median nerve: A cause of acute bilateral carpal tunnel syndrome in a three-year-old child: A case report and comprehensive literature review

Abstract

A three-year-old boy was investigated for inexplicable incessant crying. On examination, his left wrist was mildly swollen (three to four months) and sensitive. Exploration and carpal tunnel decompression of the left wrist with incisional biopsy was performed for the presence of a fusiform swelling intimately associated with the median nerve. Histopathology revealed the presence of enlarged nerve bundles admixed with mature fat cells and diffuse fibroblastic proliferation. Three months later, he underwent urgent contralateral carpal tunnel decompression for a similar presentation. The final diagnosis was bilateral fibrolipomatous hamartoma (FLH) of the median nerves causing acute bilateral compression neuropathy. FLH of the median nerve is an extremely unusual cause of acute bilateral carpal tunnel syndrome in a young child presenting with 'incessant crying'. A comprehensive review of FLH including epidemiology, etiology, clinical presentation, differential diagnosis, imaging, pathology, treatment and prognosis is discussed.

Lipofibromatous hamartoma of the median nerve – A case report

“…Some have proposed that LFH is congenital or developmental in nature, whereas others suggest it is a consequence of reactive processes secondary to nerve irritation or trauma. 4 The diagnosis of LFH is strongly supported by imaging findings. Traditionally MRI has been used extensively to investigate LFH.…”

“…3 Involvement of the brachial plexus, ulnar, radial, sciatic, peroneal, plantar and digital nerves has also been described. 4 Patients typically present in childhood with macrodactyly or at adolescence with a volar forearm mass. 5 Almost all cases present prior to the 3rd decade, with a mean age of 22 years.…”

Carpal Tunnel Syndrome Secondary to Fibrolipomatous Hamartoma of the Median Nerve

“…This growth is derived from the fibroadipose tissue within the nerve sheath. This tissue infiltrates the epineurium and perineurium [ 3 ]. Over time, this can lead to fusiform enlargement of the nerve causing compressive neuropathies.…”

“…FLH most commonly occurs in the first three decades of life, and Caucasians are predisposed compared to other races. In addition, males develop FLH more frequently than females unless macrodactyly is present, in which case, females are more likely to develop FLH than males [ 2 - 3 ].…”

Fibrolipomatous Hamartoma of the Median Nerve of the Hand: A Case Report and Literature Review

“…Because the pathological results cannot guide the clinic well, very few hospitals will continue to perform immunohistochemistry of pathological sections. Jenna-Lynn Senger 4 once reported a case of bilateral acute carpal tunnel syndrome in a 3-year-old child, of which the pathological immunohistochemistry showed: CD34(+), S-100(+), vimentin(+), epithelial membrane antigen(-), desmin(-), glial brillary acidic protein antibodies(-). Jose A. Plaza 29 proposed S-100 (+), EMA (+ -), brous cells (-).…”