von Recklinghausen Neurofibromatosis-Pachydermatocele Causing Lower Limb Gigantism: A Case Report

Rekha, Arcot; Gopalan, T. R.

doi:10.1177/1534734606286469

Cited by 10 publications

(9 citation statements)

References 20 publications

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“…Plexiform neurofibromas are also called pachydermatocele because of the similarity with elephant's skin. Their occurrence on the lower limb is, however, uncommon [9].…”

Section: Discussionmentioning

confidence: 98%

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Neurofibromatosis with unilateral lower limb gigantism

Rani

Devescovi

2009

Musculoskelet Surg

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“…Plexiform neurofibromas are also called pachydermatocele because of the similarity with elephant's skin. Their occurrence on the lower limb is, however, uncommon [9].…”

Section: Discussionmentioning

confidence: 98%

“…Once this change has taken place, the mutant gene can be genetically transmitted [9]. Neurofibromin, the protein transcribed by the NF1 gene, contains a region with a Fig.…”

Section: Discussionmentioning

confidence: 99%

Neurofibromatosis with unilateral lower limb gigantism

Rani

Devescovi

2009

Musculoskelet Surg

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“…Diffuse one is also known as elephantiasis neurofibromatosa [10]. Plexiform neurofibromas involving the genitourinary tract or the lower limb are rare, with bladder, upper urinary tract and genital involvement reported in decreasing order of frequency [11] [12]. Studies have shown that in cases of genital NF1, vulva appears to be the most frequently affected with rare reports of vagina, cervix and ovary [13].…”

Section: Discussionmentioning

confidence: 99%

Pregnancy with Elephantiasis Neurofibromatosa—A Rare Presentation

Gupta¹,

Gupta²,

Sapre³

et al. 2014

OALib

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Neurofibromatosis (NF1) is an autosomal dominant disorder primarily affecting the development and growth of nerve cell tissues. We report a rare case of pregnancy with giant plexiform neurofibroma involving the genital tract with adverse perinatal outcome. A 26-year-old female presented at term in labour with NF1 and a huge plexiform neurofibroma arising from sacral region, gluteal region, pelvis and involving left labium majus. She delivered by cesarean section and the baby had congenital NF1. The baby expired on day 4 of life. To the best of our knowledge, this large size neurofibroma involving this site in pregnancy has never been reported in English literature. Both pregnancy and NF1 have reciprocal adverse effects on each other. Congenital neurofibromatosis can be fatal. Hence, all pregnancies associated with NF1 should be considered as high risk and monitored carefully.

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“…There are a number of case reports and series found in the literature discussing giant neurofibromas [7][8][9][10][11][12][13][14][15][16]. PNFs are difficult to manage surgically as they are extensively infiltrative, highly vascularized and tend to recur.…”

Section: Introductionmentioning

confidence: 99%

Life Threatening Complications following Excision of Giant Neurofibroma

Choi

Kyung

et al. 2017

J Wound Management Res

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Plexiform neurofibromas are benign tumors originating from subcutaneous or visceral peripheral nerves, which are usually associated with neurofibromatosis type 1. Giant neurofibromas are very difficult to manage surgically as they are extensively infiltrative and highly vascularized. These types of lesions require complex preoperative and postoperative management strategies. This case report describes a 22-year-old female with a giant plexiform neurofibroma of the lower back and buttock who underwent pre-operative embolization and intraoperative use of a linear cutting stapler system to assist with haemostasis during the surgical resection. Minimal blood transfusion was required and the patient made a good recovery. This case describes how a multidisciplinary management of these large and challenging lesions is technically feasible and appears to be beneficial in reducing perioperative blood loss and morbidity. Giant neurofibroma is a poorly defined term used to describe a neurofibroma that has grown to a significant but undefined size. Through a literature review, we propose that the term "giant neurofibroma" be used for referring to those neurofibromas weighing 20% or more of the patient's total corporal weight.

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von Recklinghausen Neurofibromatosis-Pachydermatocele Causing Lower Limb Gigantism: A Case Report

Abstract: Gigantism of the lower limb can occur because of plexiform neurofibromas. This condition is seen with café au lait patches and multiple neurofibromatosis in this case of von Recklinghausen neurofibromatosis. We report our patient and review literature of this uncommon condition.

Cited by 10 publications

References 20 publications

Neurofibromatosis with unilateral lower limb gigantism

Neurofibromatosis with unilateral lower limb gigantism

Pregnancy with Elephantiasis Neurofibromatosa—A Rare Presentation

Life Threatening Complications following Excision of Giant Neurofibroma

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