Bilateral fibrolipomatous hamartoma of the median nerve with macrocheiria and late-onset nerve entrapment syndrome

Meyer, Bernd‐Ulrich; Röricht, Simone; Schmitt, Rainer

doi:10.1002/(sici)1097-4598(199805)21:5<656::aid-mus16>3.0.co;2-t

Cited by 23 publications

(12 citation statements)

References 9 publications

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“…The median nerve is affected in >80% of cases; however, several other nerves have been reported with this pathology including the brachial plexus, cranial, ulnar, radial, sciatic, peroneal, plantar and digital nerves (4). These nerves may be individually affected (isolated median/ulnar nerve), or as multiple nerves (15) with or without a constellation of features such as reported: bilateral median nerve, right ulnar nerve with macrocheiria and late-onset nerve entrapment syndrome in a 46-year-old man (1).…”

Section: Discussionmentioning

confidence: 99%

“…The cause of these transformations, however, remains elusive. Some believe this neoplasm is congenital/developmental in origin, whereas others maintain it is an acquired/reactive process due to factors such as nerve irritation or trauma (1,4,5,10,24,40). Those who support the congenital theory, especially in relation to FLH of the median nerve in the carpal tunnel, propose that this patient population is born with an abnormally developed flexor retinaculum or an abnormally developed transverse carpal ligament (26,28,41).…”

Section: Etiologymentioning

confidence: 94%

“…Nerve territory-oriented macrodactyly is a common concurrent finding, with 20% to 66% of FLH being associated with such macrodactyly (1,30,31). No hereditary or chromosomal abnormality is reported in these cases.…”

Section: Etiologymentioning

confidence: 99%

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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

Senger¹,

Classen²,

Bruce³

et al. 2014

CAN J PLAST SURG

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A lthough carpal tunnel syndrome (CTS) is the most common entrapment neuropathy encountered in an adult surgical practice, it is relatively rare in children. In the majority of adults, CTS is believed to be idiopathic in origin. However, in children, CTS is usually secondary to an underlying pathology, such as lysosomal storage diseases, particularly mucopolysaccharidosis and mucolipidosis. Other causes of CTS in children include genetic bony dysplasia, malformations of the anatomical structures of the carpal tunnel, or enlargement of the median nerve by intraneural lipoma, ganglionic cysts, traumatic neuromas, vascular malformations or neurofibromas. An unusual cause of childhood CTS is fibrolipomatous hamartoma (FLH), a rare benign lesion characterized histologically by the presence of mature fat cells admixed with fibroblastic proliferation within the distorted, expanded nerve bundles.We report the first case in the published literature of acute bilateral CTS secondary to bilateral FLH of the median nerves without macrodactyly in a three-year-old boy who presented with incessant crying due to acute bilateral compression neuropathy. Case presentationA three-year-old boy was brought to the emergency room by his mother due to 'incessant crying' that began earlier that morning. His distress had been progressive since he awoke and, on arrival to the emergency room, he was inconsolable. The mother was unable to identify a precipitating cause. On further questioning, the mother recalled his complaining of discomfort in the right wrist for the past three months. The boy was otherwise healthy, took no medications and his immunizations were up to date.On physical examination, a 4 cm to 5 cm swelling of the flexor aspect of the right wrist and thenar atrophy of this hand were recognized with associated weakness in the hand ( Figures 1A and 1B). Tinel's test was positive. The boy's distress escalated with active and passive movement of the wrist, which impeded the assessment of sensation and range of motion. His physical examination was otherwise unremarkable except for an incomplete syndactyly of the second and third toes.Suspecting a neurofibroma secondary to neurofibromatosis, dermatology and ophthalmology were consulted. A complete workup for the same showed no stigmata of neurofibromatosis. Plastic surgery was subsequently consulted to perform an examination under anesthetic for exploration of the right wrist. Intraoperatively, a fusiform enlargement of the median nerve causing carpal tunnel compression was identified (Figures 2A to 2C). The surrounding tissue was otherwise normal. A biopsy of the median nerve was taken for diagnostic purposes, and a carpal tunnel release (CTR) was performed to alleviate compression symptoms. 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 swe...

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Section: Discussionmentioning

confidence: 99%

Section: Etiologymentioning

confidence: 94%

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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

Senger¹,

Classen²,

Bruce³

et al. 2014

CAN J PLAST SURG

View full text Add to dashboard Cite

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“…FLH is a rare benign neoplasm derived from abnormal growth of fibroadipose tissue of the nerve sheath leading to fusiform enlargement of the affected nerve (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13). The tumour originates from fatty infiltration of the nerve bundle infiltrating the epineurium and perineurium (12,14).…”

Section: Discussionmentioning

confidence: 99%

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

Senger¹,

Classen

Bruce³

et al. 2014

Plast Surg (Oakv)

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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.

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“…Un fibrolipome bilatéral a été rapporté par Meyer [9]. L'atteinte du nerf collatéral de l'index et du médius est la plus fréquente [2,4].…”

Section: Discussionunclassified

Fibrolipome du nerf médian : à propos d’une observation

Kossoko

Allah

Kadio

et al. 2008

Chirurgie de la Main

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Bilateral fibrolipomatous hamartoma of the median nerve with macrocheiria and late-onset nerve entrapment syndrome

Cited by 23 publications

References 9 publications

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

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

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

Fibrolipome du nerf médian : à propos d’une observation

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