• Journal of Korean Neurosurgical Society
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• 제39권6호
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• pp.459-463
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• 2006

This report describes a case of diffuse neurofibroma[DNF] that presented with spontaneous intratumoral hemorrhage. A 31-year-old man with cutaneous manifestations typical of neurofibromatosis type 1[NF1] was referred to hospital with a progressively expanding scalp swelling. Magnetic resonance imaging[MRI] showed an acute hematoma surrounded by a dense mass on the parietooccipital scalp. Plain skull radiographs and a computed tomography[CT] scan revealed bony defects in the occipital bone around the lambdoid suture and the skull base, including the sphenoid and petrous bones. The tumor extended to the upper cervical region and infiltrated through the fascia of the musculature. The tumor and adjacent soft tissue were highly vascular, which made surgical resection difficult. We describe the clinical, radiological and pathological features of this patient md discuss the surgical methods used to avoid a life-threatening hemorrhage during surgery.

• Clinical Pain
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• 제18권2호
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• pp.97-101
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• 2019

Tardy ulnar nerve palsy is ulnar neuropathy at or around elbow and commonly evaluated in the electromyography laboratory. However, ulnar neuropathy at the elbow due to neurofibroma is rare. Neurofibromas are tumors that arise within nerve fasciculi and anywhere along a nerve from dorsal root ganglion to the terminal nerve branch. We report one case of ulnar neuropathy at the elbow due to neurofibroma. Patient had paresthesia on the left 5th finger and there had been left hypothenar atrophy since 2 months ago. Tinel's sign was positive at left elbow. As a result of electromyography, there were suggestive of right ulnar neuropathy at or around elbow, referred to as tardy ulnar nerve palsy. Ultrasonography showed a diffuse tortuous thickening with multiple neurofibromas arising from individual fascicles of the ulnar nerve in cubital tunnel area. Surgery was then performed to release cubital tunnel of left elbow, then the patient's symptoms improved.

• Journal of Chest Surgery
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• 제21권4호
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• pp.766-771
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• 1988

Von Recklinghausen`s neurofibromatosis, tuberous sclerosis and encephalotrigeminal angiomatosis[Sturge-Kalischer-Weber syndrome] are frequently classified under the heading of organic neurocutaneous syndromes. Both neurofibromatosis and tuberous sclerosis are believed to represent instances of simple autosomal dominant heredity. Multiple neurofibroma and cafe*-au-lait spots are the hallmarks of the van-Recklinghausen`s disease. The characteristic features of the fully developed syndrome are [1] pigmentation of the skin, including cafe*-au-lait spots, pigmented freckles and males, and occasionally a generalized darkening of the skin; [2] subcutaneous nodules and deep neurofibromatous tumors and diffuse plexiform growths of neural tissue; [3] skeletal anomalies, especially scoliosis; and [4] predilection to malignancy. In recent years cystic lung disease, usually of the so-called honeycomb lung variety, has been reported on several occasions in patients with tuberous sclerosis. This association has been shown to our sporadically as well as in members of a single family. Little attention has been paid to the presence of cystic lung disease in association with neurofibromatosis. Currently, most think of thoracic involvement in neurofibromatosis in terms of posterior mediastinal neuroma, pheochromocytoma, meningocele or, less commonly, parenchymal pulmonary neurofibromatosis. Author have experienced a case of von Recklinghausen`s disease. This case developed a huge neurofibroma in the both side thorax and invaded to the Lt. 7th rib.

• Childhood Kidney Diseases
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• 제16권1호
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• pp.68-71
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• 2012

신경섬유종증은 드문 전신질환 중의 하나로 비뇨생식기계를 침범하는 경우는 극히 드물다고 알려져 있으나 비뇨생식기계를 침범하는 경우에는 그 기관으로 방광이 가장 흔하다. 비뇨생식기계를 침범하는 경우 증상이 나타나기 전까지 그 침범여부를 알기 어려운 경우가 많기 때문에 정기적인 추적관찰 중 비뇨생식기계 증상이 나타난다면 정밀검사를 시행하여 침범상태를 정확히 판단할 필요가 있겠다. 치료 방법으로는 보존적인 치료가 대부분이지만 종양의 크기가 클 경우 종양절개를 하는 경우도 있으나 매우 드물고, 보존적 치료와 동시에 종양의 크기 증가여부에 대하여 정기적인 추적관찰이 요구된다. 저자들은 소아에서 방광을 침범한 신경섬유종증이 증상을 나타낸 경우로 보존적 치료 후 경과관찰 중인 환아 1례를 경험하였기에 보고하는 바이다.