Lipofibromatous hamartoma of the median nerve. Three case reports

Nardella, D; Sohawon, Schoeb; Carlier, Alain

doi:10.1016/j.bjps.2007.11.061

Cited by 34 publications

(28 citation statements)

References 15 publications

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“…2012) restricted to the English-language literature (10,17,22,28,30,(31)(32)(33)(34)(35)(36)(37)(38). Among these publications, no reports of bilateral median nerve involvement were identified.…”

Section: Figure 4) Postoperative Examination Of the Hands Following Bmentioning

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: Figure 4) Postoperative Examination Of the Hands Following Bmentioning

confidence: 99%

“…When diagnosing FLH, several differential diagnoses have been described including ganglionic cysts, hereditary hypertrophic neuritis of Dejerine-Sottas syndrome, vascular malformations, traumatic neuromas, schwannomas, neurofibromas and intraneural lipomas (4,6,21,34,37).…”

Section: Differential Diagnosismentioning

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,

Classen²,

Bruce³

et al. 2014

CAN J PLAST SURG

View full text Add to dashboard Cite

show abstract

“…LFH most commonly involves the median nerve though it is unclear as to why this predilection exists [10,17]. It has been suggested that chronic microtrauma to the median nerve from the carpal ligament or pressure from an abnormally developing flexor retinaculum can initiate a reactive process which culminates in the development of this tumour.…”

Section: Discussionmentioning

confidence: 99%

“…LFH is often considered to be congenital in origin as most cases manifest in children and generally follow an indolent course [3,17,18]. However, some authors have demonstrated that a minority of patients will not present until at least the third decade [4].…”

Section: Discussionmentioning

confidence: 99%

Lipofibromatous hamartoma of the median nerve

Al-Jabri

Garg²,

Mani

2010

J Orthop Surg Res

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Lipofibromatous hamartoma is a rare tumour of peripheral nerves which is characterised by an excessive infiltration of the epineurium and perineurium by fibroadipose tissue. To the best of our knowledge, only approximately 88 cases are reported in the literature. We report a rare case of lipofibromatous hamartoma of the median nerve causing secondary carpal tunnel syndrome in a 25 year old patient. This patient was treated conservatively with decompression and biopsy and experienced a complete resolution of symptoms post-operatively. Magnetic resonance imaging may be used to diagnose this lesion as it has very distinctive characteristics. Multiple conditions have been associated with this lesion and a greater understanding of these associations may clarify the pathogenesis. The architecture of the tumour makes excision very challenging and the surgical management remains controversial. A review of the literature regarding the etiology, pathogenesis and surgical management of lipofibromatous hamartoma is included.

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“…Because of abnormal enlargement of the involved nerve, compression neuropathy is a frequent finding (11). The median nerve is most commonly involved peripheral nerve, often with a predilection for the carpal tunnel (2). Separation of the individual nerve fascicles by fibro fatty infiltration of perineurium and epineurium produce remarkable changes in diagnostic imaging.…”

Section: Introductionmentioning

confidence: 99%

Ultrasonographic Diagnosis of Lipofibromatous Hamartoma of Median Nerve in a Twenty-Seven-Year-Old Woman

2014

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Lipofibromatous hamartoma (LFH) is an extremely rare benign tumor, which is characterized by an excessive infiltration of the epineurium and perineurium by fibroadipose tissues.A 27-year-old woman was diagnosed with left carpal tunnel syndrome (CTS) due to (LFH) of the median nerve. LFH was diagnosed by MRI and sonography; the characteristic ultrasonographic feature of LFH showed a good correlation with pathognomonic MRI findings. The median nerve was involved along its course in the forearm; however, the patient needed carpal tunnel release because of severe compression of the median nerve under the flexor retinaculum.Radiologic evaluation of patient with CTS to evaluate probable secondary CTS is recommended.

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Lipofibromatous hamartoma of the median nerve. Three case reports

Cited by 34 publications

References 15 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

Lipofibromatous hamartoma of the median nerve

Ultrasonographic Diagnosis of Lipofibromatous Hamartoma of Median Nerve in a Twenty-Seven-Year-Old Woman

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