Chirurgische Therapie des Skrotumödems bei Elephantiasis congenita hereditaria (Typ Meige)

Abstract

The underlying cause of primary lymphedema is a malformation of the lymph vessel system. Secondary lymphedemas can be due to infections, recurrent inflammation, hypoproteinemia, tumors, operations, or irradiation. As a reaction to persistent edema and interstitial macromolecules, fibrosis occurs. Recurrent inflammations of the indurated and edematous tissue are clinically impressive. The massive form of the scrotal lymphedema leads to painful tautness and sexual dysfunction. A concurrent penile edema can cause dysuria. The deformity of the affected extremities and organs not only leads to restriction of mobility but also to psychological stress due to the disfigurement, even as far as to social deprivation. We report on a surgical technique for treating pronounced scrotal edema by resection and neoscrotal reconstruction using ventral pedunculated scrotal skin flaps in cases of congenital hereditary elephantiasis of the Meige type.

L'éléphantiasis péno-scrotal. A propos d'un cas

“…Les causes acquises sont dues & une obstruction mecanique ou inflammatoire chronique pouvant eves consecutives a une tumeur abdominale ou pelvienne, une chirurgie carcinologique ou une radiotherapie pelviennes [3,7], une maladie de Kaposi, une infection genitale a streptocoque ou & chlamydia [17], une lymphogranulomatose venerienne [14], ou a une hypoprotidemie [24].…”

“…Le lymphoedeme scrotal congenital ou primitif est cause par une malformation du systeme lymphatique d'origine dysplasique non regressive s'integrant dans le cadre des lymphangiomes [20,24]. Dans notre cas la cause de I'elephantiasis est restee indeterminee.…”

Asymptomatic erythematous‐violaceous scrotal vesicles in a 78‐year‐old man

“…Another hereditary disorder, Nonne-Milroy-Meige syndrome is characterized by pronounced lymphedema and disorders such as obesity, hypogonadism, dwarfi sm, mental retardation as well as abnormalities of the vertebral column, the cardiovascular system, and other organs [ 6 ] . Secondary cutaneous lymphangiectasia typically occurs in the context of infections, trauma, keloids, Crohn's disease, pregnancy, scleroderma, neoplasms, radiation therapy, or in association with certain surgical procedures [ 1,7,8 ] .…”

Giant scrotal elephantiasis of inflammatory etiology: a case report

“…Scrotal elephantiasis is extremely rare outside endemic regions in Africa and India [1,2]. Occasionally it has been attributed to radiotherapy, neoplasm and lymphadenectomy [3,4]. …”

“…Patients develop edema at adolescence without restriction to the external genitalia. In hereditary elephantiasis of the Meige type, lymphedema of the external genitalia occurs due to malformation of lymphatic vessels [4]. In some cases hidradenitis suppurativa and lichen sclerosus et atrophicus have also been described [5].…”