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.
HighlightsLGASC is an uncommon form of triple-negative metaplastic breast carcinoma.Imaging, FNAC, & CNBx are usually indeterminate with no pathognomonic features.Histopathology is the gold standard for definitive diagnosis.Relationship between LGASC and benign mimickers (SAN, CSL) continues to evolve.LGASC is likely multicentric, thus mastectomy is the recommended management.
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...
In the context of evidence-based literature, and because no significant pathological findings were detected in this particular cohort of 452 cases with 2178 slides, the authors believe it is time to re-evaluate whether routine histopathological examination of tissue from gynecomastia remains necessary. The current climate of health care budget fiscal restraints warrants reassessment of the current policies and practices of sending tissue samples of gynecomastia incurring negative productivity costs on routine histopathological examination.
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