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Under analysis were results of the surgical treatment of 24 patients with lymphostasis of genitalia (27 operations). The method of choice for the 1st stage of edema (lymphedema) is considered to be lympho-venous shunting. In cases with fibredema (elephantiasis) the authors prefered radical excisions of edematous tissues of genitalia.
Adult lymphatic filariae (Wuchereria bancrofti, Brugia malayi) can cause blocking of lymphatics producing obliterating endolymphitis lesions. The subsequent extravasation of lymph (or chyle when the obstruction is canal) is at the origin of the formation of lymphedema or elephantiasis, in which the main histological finding is great hypertrophy of collagen elements. This theory involving filaria only is not the full picture, and bacterial infection, mainly by streptococci, is an important factor. The association of filaria with microbes is particularly dangerous because the presence of the latter, or its toxins, causes death of local microfilariae and even adult worms, which are known to be more harmful dead than alive. The progression of the disease, especially in cases with lymphedema, which mainly affects the limbs and the genital organs, depends on three factors: the species of filaria, the degree of transmission, and the receptivity of the patient to the parasite. Large differences are found according to the region involved, and in the same endemic zone, according to the individuals affected. However, they almost always occur progressively in areas where there have been recurrent attacks of acute lymphangitis.
Evidence for an association of endemic non-filarial elephantiasis of the lower legs with areas of red clay soils around volcanoes has been noted in several countries bordering the Rift Valley in East Africa. It was of interest to observe whether the association is also present in an area of endemic prevalence of the disease in West Africa. Such an area occurs in the Cameroon highlands where it has been suggested that onchocerciasis might be an etiological factor. The present study describes the results of a survey in this area. There was no statistical evidence of a relationship with onchocerciasis and the content of mineral microparticles in lymphatic tissues of the lower legs was again established. Climatic, geological and soil characteristics were similar to those in East Africa. It is considered that the disease in the two widely separated non-bancrofti areas is of similar etiology and it is proposed that it may be interpreted as silicosis of peripheral lymphatics of the lower legs.
Elephantiasis is the outcome of persistent lymphedema. It is refractory to different therapy modalities. The social approachability of patients with this disease is impaired by severe functional and cosmetic disturbances. The causal relationship between etiology and recurrent disease is demonstrated by two cases. Treatment options are discussed. Radical surgery is decisive for primary success since chronic inflammation and chronic edema support each other. Metabolic equilibrium, disinfecting skin areas and antibiotic prophylaxis are urgent steps in the successful treatment of Erysipelas disease.
Exclusively penial lymphatic affections can be considered a highly infrequent occurrence. In the absence of an scrotal oedema, an "elephantiac" involvement with tumoral appearance in the penis, is indeed exceptional. A clinical case is briefly commented here. It refers to a patient with a significant sociopathy who presented a lymphedema confined to the penis in its maximum expression (elephantiasis verrucosa nostras).
We report a case of filaria scrotum elephantiasis explored by MRI. MRI and pathological description of the three muscular layers (superficial, medium, deep) were well correlated.
A 67-year-old man visited our hospital with complaints of scrotal swelling associated with occasional febrile episodes. Physical examination disclosed a huge scrotal mass, approximately the size of a child's head, with numerous papillomatous lesions on its surface. His past medical history was significant in that he was diagnosed with penile carcinoma at the age of 35 years old and was treated with partial penectomy followed by radiation and chemotherapy at other hospital. During this admission tumor marker squamous cell carcinoma (SCC) and microbiological tests for mcroflariae were both negative. Ultrasound (US), computed tomographic (CT) scan and magnetic resonance imaging (MRI) revealed markedly thickened scrotal skin and small hydrocele with no evidence of local recurrence of the previous penile carcinoma. A percutaneous cystostomy was created because of chronic urinary retention and possible urine extravasation into the scrotum. Histopathological examination of the biopsy specimen from the scrotal mass demonstrated lymphangiectasia consistent with elephantiasis of the scrotum. Surgical excision of this huge scrotal mass was performed in August 1997. The resected tissue weighed 1,400 g. Convalescene was uneventful. He subsequently underwent perineal urethrostomy in place of the suprapubic cystostomy.
An example of elephantiasis neuromatosa, an unusual variant of neurofibromatosis, coexistent with lipomatosis is presented. This dual cell population produced a diffuse swelling of the gluteal sulcus of a young female. The neurogenous origin of the spindle cell component was substantiated by immunoreactivity for S-100 protein as well as by the fine structural observation of a regular basement membrane coating and a profusion of Luse bodies. As far as can be ascertained, this is the first reported immunohistochemical and ultrastructural study of this particular variant of peripheral nerve sheath lesion.
This disease was first described by von Recklinghausen and Festscher and has been known as the von Recklinghausen's disease or neurofibromatosis (NF). Numerous articles have been published on this subject where majority of the authors have stressed their difficulty in achieving favourable surgical results. This disease is pathologically accepted to be of neuroectodermal origin with a positive family history in approximately 50% of the cases, autosomal dominant trait and is known to involve the periorbital regions, orbit (preoperative CT scan: upper left and right photographs), temporal region to a variable extent, mid-facial region to the mandibular region. The indicated treatment for this disease is surgery including cranio-maxillofacial surgery even though re-evaluation of the conventional methods of surgery should be considered. This particular case is an extremely difficult and challenging case for any reconstructive plastic surgeon and required extensive preoperative planning. The systematic multiple stage surgical approach for an extremely severe von Recklinghausen's disease, elephantiasis neurofibromatosa is presented and discussed in detail.
Verrucous elephantiasis of the scrotum is a severe debilitating variant of scrotal lymphedema. A case is reported, and its pathophysiology and treatment are discussed.
We report the case of a patient with NF-1 who presented with gross elephantiasis neuromatosa of her right leg. Prior to plastic surgery, Magnetic Resonance Imaging and Angiography (MRI and MRA) were performed to provide a detailed assessment of the extension as well as the vascular and muscular involvement of the neurofibroma.
Neurofibromas are benign tumors arising from neuroectodermal tissues. They may occur as solitary lesions, or multiple, in which case they are referred to as neurofibromatosis, or von Recklinghausen's disease. We report about a 17-year-old patient with neurofibromatous elephantiasis located in the lateral aspect of the left hemithorax. CT showed massive chest wall infiltration and retroperitoneal tumor growth, but no abnormalities of the cranium. Our surgical treatment consisted of a two-stage, palliative tumor debulking and defect coverage with a splitthickness skin graft. The operative and postoperative course was uneventful.
Graves' dermopathy, affecting upper as well as lower extremities, is reported in a 50-year-old male patient addicted to multiple narcotic drugs. The narcotic drug administration routes include subcutaneous and intravenous injection in all limbs over a period of 29 years. The combination of drug injection trauma and Graves' dermopathy produced elephantiasis-like appearance of upper and lower limbs with impairment of their function; the left hand required amputation. Other features of Graves' disease, ie, hyperthyroidism and ophthalmopathy were severe and required multiple modes of therapy. Although the pathogenesis of Graves' dermopathy is unclear, it is surmised that trauma to skin might exacerbate it and preventive measures for trauma might be helpful in the management of dermopathy.
A case of an unusually symmetric and grotesque elephantiasis neuromatosa of the trunk is presented. Computed tomography demonstrated the extent of the disease, which is essential if surgery is planned.
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