Recurrent carpal tunnel syndrome in a child due to fibrolipomatous hamartoma of the median nerve successfully treated by limited excision and decompression

Bains, Rob D.; Kotwal, Ashtosh N.; Saeed, Wajieha

doi:10.1016/j.bjps.2006.01.016

Cited by 21 publications

(11 citation statements)

References 8 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…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%

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

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...

show abstract

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

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

“…FLH of the median nerve remains a rare tumour in the pediatric (<18 years of age) population. Table 1 lists 14 published cases found in a PubMed/MEDLINE search (1986 to Figure 4) Postoperative examination of the hands following bilateral carpel tunnel release demonstrating full recovery 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: 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)

View full text Add to dashboard Cite

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.

show abstract

“…Dadas las características histológicas del fibrolipoma nervioso, cuando la escisión microquirúrgica no es posible sin dañar al nervio, es preferible una actitud limitada a la descompresión 6 con o sin epineurotomía. Como en cualquier proceso tumoral, las revisiones periódicas con estudios mediante RM y EMG son de rigor para observar la evolución del cuadro.…”

Section: Discussionunclassified

Fibrolipoma del nervio cubital. A propósito de un caso

Villarroya

Marco

Díaz

et al. 2008

Rev Iberoam Cir Mano

View full text Add to dashboard Cite

Introducción: Los fibrolipomas de los nervios periféricos, o hamartomas lipofibromatosos, son tumores benignos que infiltran el espacio entre los fascículos neurales. Son más frecuentes en el territorio nervio mediano. La resonancia magnética ofrece un diagnóstico patognomónico. El tratamiento debe ser descompresivo cuando la escisión microquirúrgica no sea posible sin ocasionar secuelas funcionales. Caso clínico: Varón de 30 años, trabajador manual, sin alteraciones morfológicas en sus manos con clí-nica de hipoestesia en 4º y 5º dedos, pérdida de fuerza en mano, atrofia hipotenar e interósea y principio de garra cubital de 8 meses de evolución. La electromiografía realizada indicó compresión del nervio cubital a nivel del canal de Guyon. En el acto quirúrgico se comprobó la existencia de un fibrolipoma del nervio cubital que comprometía a sus ramas terminales sensitiva y motora. Al no ser posible la exéresis microquirúrgica sin dañar al nervio, se realizó biopsia y descompresión amplia del nervio, mejorando la sintomatología del paciente.Palabras clave: fibrolipoma neural, hamartoma lipofibromatoso, nervio cubital.

show abstract

Recurrent carpal tunnel syndrome in a child due to fibrolipomatous hamartoma of the median nerve successfully treated by limited excision and decompression

Cited by 21 publications

References 8 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

Fibrolipoma del nervio cubital. A propósito de un caso

Contact Info

Product

Resources

About