ELEPHANTIASIS (elephantiasis; synonym elefantiaz) — the permanent diffusion increase in volume of this or that part of a body caused by disturbance of a lymph drainage and which is characterized at the beginning of development by chronic hypostasis, and then irreversible fibrous changes of skin, hypodermic cellulose and a fascia.
The term «elephantiasis» for the first time occurs at A. Tsels. It is impossible to recognize attempts to replace it with such terms as «lymphostasis» and «limfedem» reasonable since the processes designated by them are only initial links in a pathogeny and a wedge, a picture of elephantiasis.
S. of the lower extremities is most often observed, is more rare than external genitals, upper extremities, persons, etc.
Depending on an etiology distinguish primary and secondary S. K of primary S. so-called idiopathic (essential) elephantiasis belongs, edges develops, as a rule, at young age. According to nek-ry researchers, in primary idiopathic S.'s emergence the inborn limfangiopatiya matters. Also nek-ry types of inborn S. Tak belong to primary, in 1892 Mr. W. F. Milroy described hereditary S. (W. F. Milroy's disease), to-ruyu it tracked in six generations of one family, having found S. of the lower extremities in 20 people from 97, especially progressing during puberty. Primary inborn S. (without instructions on it in the family anamnesis) can be connected with malformations lymphatic system (see) in a look aplasia (see), hypoplasias (see) or hyperplasia (see) collector absorbent vessels (see) with insufficiency of the valve device. At adults primary inborn S. makes, according to various data, from 2 to 6,2% of total number of patients of S. Nek-rye researchers connect separate cases of emergence of primary S. with damage of the central nervous system, endocrine frustration, etc.
Secondary S. can be connected, e.g., with development of amniotic banners, to-rye, squeezing circus-lyarno soft tissues of an extremity of a fruit, interfere with a normal lymph drainage (see. Amniotic threads, banners, unions ). As a rule, secondary S. is a complication of many inflammatory and noninflammatory diseases. Most often S. develops after the postponed erysipelatous inflammation (see. Ugly face ), occasionally at tuberculosis of bones, joints, limf, nodes and skin (see. Tuberculosis extra pulmonary ), lymphadenitis (see), to a limfangiita (see), a furunculosis (see. Furuncle ), pyodermas (see), syphilitic granulomas (see. Syphilis ), chronic inflammation vaginas (see), rectum (see), etc.
The special place in emergence and S.'s development occupies an erysipelatous inflammation. The ugly face quite often arises against the background of already developed frustration of a lymphokinesis, however, undoubtedly that each its recurrence sharply aggravates disturbance of a lymphokinesis. Nek-ry researchers consider that S.'s emergence at an ugly face, as well as at other inflammatory diseases, in many cases is caused by the hidden inborn limfangiopatiya. In the tropical and subtropical countries in S.'s etiology of the lower extremities, external genitals the large role is played by helminths — filarias (see. Filariases ), to-rye, getting in limf, vessels of skin scrotums (see), penis (see), is more rare than big and small vulvar lips, cause the chronic, torpidno proceeding inflammatory process and, besides, cork with the weight limf, capillaries. Under certain conditions can lead a prelum of proximal departments of an extremity to secondary S. hems after mechanical injuries, deep burns (see), operational removal collector limf, vessels and nodes, radiation therapy (see). Pages of external genitals, besides, can cause uric infiltration of fabrics at a stricture of an urethra, inguinal lymphogranulomas (see. Lymphogranulomatosis pakhovy ), erizipeloid (see), etc.
S.'s Emergence is caused by disturbance of passability limf, vessels and capillaries. At inf. process these disturbances are a consequence of an inflammation and the subsequent sclerous changes, at tumors — prelums limf, vessels and capillaries a tumor. At a filariasis, in addition to mechanical influence of helminths (filarias) on limf, vessels, leading to their varicosity, an inflammation and obstruction, can be observed toksiko-allergic reactions with development of a consecutive bacterial infection.
Permanent disturbance of passability limf, vessels brings to to a lymphostasis (see), hron. I will swell (see), to a disproteinoz and other disturbances of fabric exchange, to-rye arise in hypodermic cellulose and. slowly progressing, extend to a fascia and skin. There is an imbi-bition of intersticial fabric (see. Connecting fabric ) high-molecular proteins and their metabolites that leads to development of a sclerosis, progressing of insufficiency limf, capillaries and vessels, to increase of hypostasis. Quite often process is complicated by an inflammation, a cut promotes S.'s development, aggravating patol. changes in the struck body. In the outcome of elephantiasis irreversible fibrosis of skin, hypodermic cellulose and a fascia develops.
Distinguish a so-called soft stage, or lim-fedy, edges corresponds to existence expressed, at first passing, and then persistent hypostasis, mixed, or intermediate, a stage when the fibredema is localized only on the periphery of an extremity, and in its proximal part the limfedema, and a firm stage, or fibredy still takes place, edges corresponds to development of total fibrosis of fabrics. In an initial stage of S. the struck body is increased in volume at the expense of hypostasis. Skin remains smooth, without trophic changes. Hypostasis and moderate fibrosis of hypodermic cellulose is histologically noted, in a cut vessels are found expanded limf. In a stage hron. limfedema the struck body (especially lower extremities) sharply increases in volume, is deformed, skin hangs folds, is thickened, there are cracks, ulcers, papillomatous growths are observed. In superficial limf, vessels, and it is frequent also in deep limf, collectors are found limfangiit and perilimfangiit, productive obliterating inflammatory processes — subacute and hron. en-dolimfangiit (fig. 1, a), a hypertrophy (fig. 1, b), a sclerosis and a hyalinosis of walls and valves limf, vessels, etc. In limf, vessels there can be changes and without signs of an inflammation, napr, an obliterirukltsy and dystrophic limfangiopatiya, lymphangiectasia (see). As a result of disturbance of a lymph drainage on the main collectors there are signs of secondary reorganization limf, capillaries and vessels of skin: growth and deformation limf, capillaries, deformation, varicosity and emergence atypical (spiral-shaped) limf. vessels, lymphangiectasia of skin. In different sites of epidermis are noted an atrophy (see. Atrophy of skin ), hyperkeratosis (see), acanthosis (see), parakeratosis (see) and vacuolation of an epithelium. The atrophy of epidermis is especially sharply expressed at formation of lymphangiectasias. At the same time expanded limf, capillaries form more or less wide cavities putting pressure upon epidermis in a papillary layer of a derma, causing its atrophy (fig. 2), places before full thinning and a gap with the expiration of a lymph outside (see the Lymphorrhea). The lymphorrhea can be long, especially in the presence reflux (see), i.e. the retrograde movement of a lymph in connection with insufficiency of valves limf, vessels.
In a stage of a fibredema in a derma and hypodermic cellulose capillaries, considerable decrease in level acid are found sharply expanded limf, mucopolysaccharides (see), hypostasis, total fibrosis and hyalinosis (see), focal (peri-and paravascular) infiltrates from lymphocytes, plasmatic and mast cells, a myopachynsis raising a hair, changes of elastic and argyrophil fibers (hypertrophic and hyperplastic character), and also secondary changes of walls of blood vessels. The fascia is considerably thickened, dim, with an opaque shade, however its inner surface remains smooth, brilliant.
Primary idiopathic S. of the lower extremities is more often observed at women at young age and begins imperceptibly. Sometimes hypostasis of the lower extremity is found for the first time at selection of footwear or after long stay standing. Beginning usually with foot and an ankle joint, it then extends in the proximal direction, taking all shin, sometimes and a hip (fig. 3, a). The long time the only symptom of S. is hypostasis, to-ry originally has passing character, increasing after orthostatic loadings, and also in hot weather and decreasing or disappearing at horizontal position of the patient. In this so-called soft stage (a stage of a limfedema) of the patient weight and increased fatigue of an extremity disturb only nek-paradise. The struck fabrics have a pasty consistence. Skin in this area is not changed, usual coloring, easily is displaced and gathers in folds. On it after pressing there are deepenings. In the subsequent, is more often after the postponed inflammatory diseases, hypostasis becomes constant and continues to accrue gradually. At hemilesion asymmetry of extremities early enough becomes noticeable, and this cosmetic shortcoming most of all disturbs the patient. Fabrics of distal departments of an extremity owing to fibrous changes get density, edges gradually accrues and extends in the proximal direction, causing at first a picture mixed (i.e. existence of soft and firm sites), and then dominance of a firm stage — a fibredema. The pain syndrome usually is absent, only the feeling of weight and a raspiraniye in the affected extremity is noted. Skin of usual color or pale, is thickened, pleated gathers hardly, is a little displaced in relation to the subject fabrics, during the pressing of poles on it does not remain. The surface of skin more often smooth, sometimes by outward reminds a lemon crust. On site the postponed erysipelatous inflammation the xanthopathy appears. The expressed trophic disturbances are not observed. Sites of skin in cross folds over joints a long time keep the normal structure, then prisoye-. the hyperkeratosis and a papillomatosis dinyatsya, growth of fabric in the form of dense, shapeless hillocks («pillows») which in places are fancifully divided by deep folds is possible (fig. 3, b). Sometimes after an injury of skin there is a lymphorrhea which is followed by reduction of volume and weight of an extremity (quite often patients intentionally pierce skin for simplification of the state). At nek-ry patients on the affected extremity the increased perspiration promoting a recurrence of an erysipelatous inflammation and a hypertrichosis is noted. The general state (except for oppression of mentality) long time is not broken.
For secondary S.'s development the same stages, as are characteristic of primary S., but its current depends from etiol to a large extent. factor. E.g., S. caused by an ugly face begins violently, with fervescence, a dermahemia and bystry subsequent emergence of persistent hypostasis, to-ry has elastic character in connection with early the coming fibrosis of soft tissues. Inflammatory process can not be resumed further or is shown in the form of the erased forms of dermatitis (see. Dermatitis ). However a recurrence of an acute erysipelatous inflammation is more characteristic, to-rye over the years becomes frequent, repeating at nek-ry patients of 10 and more times a year, and each subsequent recurrence leads to increase of clinical symptoms of S., causing, finally, permanent irreversible changes.
S.'s feature at inguinal lymphadenitis is initial emergence of hypostasis on a hip and quite often on both sides.
The page, caused by noninflammatory diseases, occurs equally often at men and women. Development patol. process is in direct dependence on a damage rate of ways of a lymph drainage. At insignificant damage collector limf, vessels and nodes, a cut can be as a result of an injury of soft tissues, after a venectomy, operation for hernia, etc., hypostasis and fibrosis of soft tissues slowly accrues. Process can gradually progress, for many years if the erysipelatous inflammation does not join. The page, developing after extensive disturbance of ways of a lymph drainage, passes all stages of development from the outcome in irreversible fibrosis much quicker. Such S. of upper extremities often develops after radical mastectomies (see) in a combination with radiation therapy. Page of the lower extremities — after expanded operations for oncological diseases with removal ileal and inguinal limf, nodes. In the latter case, as well as at inguinal lymphadenitis, hypostasis can begin with a hip.
Peculiar S. of external genitals proceeds. More often she is secondary, but also inborn and idiopathic S.'s cases of a scrotum are described. Irrespective of an origin process by the most part begins with skin of a scrotum and then passes to a penis, without extending to cavernous bodies, an urethra, testicles. The page of external genitals occurs at women extremely seldom; big and (or) small vulvar lips without involvement in process of a clitoris are surprised (see. Vulva ). Clinically elephantiasis of external genitals at men and women is shown by a thickening of skin and hypodermic cellulose and sharp increase in a scrotum, the big or small vulvar lips reaching sometimes the huge sizes. Skin often accepts dark-brown or brown coloring and can remain smooth (a smooth form of S.). Quite often at a long current of S. on a scrotum, a crotch and a prepuce the hyperkeratosis is noted, there are warty and papillomatous growths (a warty form C.). In the started cases emergence of ulcerations is possible, excoriations (see), dermatitis (see) eczematic character. Except local symptoms, the allergic phenomena, temperature increase, exudative rashes, a hydrocele are quite often observed (see Gidrotsele). At accession of a staphylococcal and streptococcal infection process gets is purulent - septic character (see. Sepsis ).
The village of external genitals develops slowly more often and extends for years. The periods of relative calm are replaced by the aggravations which are quite often caused by accession of consecutive infection. Each aggravation of process is followed by a swelling and a dermahemia of a scrotum (a penis, vulvar lips), the sizes to-rykh increase even more. The hobotkoobrazny increase in a prepuce complicating the act of an urination is sometimes noted. Elephantiasis of external genitals leads to disturbances of sexual function: at men the erection becomes painful, there comes impotence.
Complications at S. are observed usually in the started cases. Treat them development of dermatitis, formation of ulcers, a long lymphorrhea, accession is purulent - a septic infection. The malignancy of the papillomas which arose because of S. Opisana also cases of persistent mental disorders is in rare instances possible.
the Diagnosis in typical cases does not represent difficulties and the wedge, a picture, and also given to a straight line is based on carefully collected anamnesis, characteristic limfografiya (see). At inborn S. on limfogramma note or an aplasia (total absence) collector limf, vessels, or the expressed their expansion. At idiopathic S. along with an aplasia come to light also a hypoplasia (reduction of number and diameter collector limf, vessels throughout all extremity or only in peripheral or its proximal department) and a hyperplasia (increase in number and diameter collector limf, vessels up to their varicose expansion, sometimes with valve insufficiency of vessels); limf, nodes at the same time are, as a rule, not changed. At secondary S. more often than at primary, retrograde filling collector limf is observed, vessels, sometimes with an ekstravaza-tion of a contrast agent, explained with the local blockade of a lymph drainage which is usually found in these cases and the related insufficiency of the valve device collector limf, vessels.
Crucial importance in diagnosis of S. caused by a filariasis has detection in blood and urine of filarias (see. Filariases ). At the same time it must be kept in mind that filarias of one types can be found in blood only at night, and other types — only in the afternoon.
Differential diagnosis carry out with the diseases proceeding with a syndrome hron. hypostasis. Diseases of blood vessels — congenital anomalies of deep veins, arteriovenous fistulas, a posttromboflebigi-chesky syndrome, etc. concern to them, at to-rykh characteristic changes also flebografiya are found by means of arteriography (see) (see). Sometimes (especially at the beginning of development) differential diagnosis of S. and hypostases of a renal and cordial origin, nek-ry forms of obesity (see), inborn partial is complicated giantism (see), Pedzhet's syndrome — Schröter (see Ped-zhet — Schröter a syndrome), trophedemas (see). In these cases, in addition to X-ray contrast researches limf, and blood vessels, carry out skin thermometry (see), a reografiya (see), an oscillography (see), a kapillyaroskopiya (see).
S.'s Treatment at early stages conservative. It includes observance of the motive mode without orthostatic overloads, a long compression of an extremity by means of elastic bandage or a stocking in the afternoon and its sublime situation at night, the sucking-away wavy massage (see). Medicinal therapy consists in purpose of diuretics, corticosteroids (see), carrying out novocainic blockade (see), intramuscular injections of trypsin (see). From physiotherapeutic procedures appoint UF-radiation (see. Ultraviolet radiation ), an electrophoresis (see) with lidazy, ronidazy, hyaluronidase, trypsin. At the elephantiasis of external genitals caused by filarias anthelmintic therapy is carried out.
At inefficiency of conservative treatment (after formation of stable hypostasis) apply operational treatment. In stages of a limfedema it is directed to drainage of a lymph in a venous bed by creation of various options of an artificial limfovenozny anastomosis (see. Microsurgery ) or its assignments in subfascial space by means of a fasciotomy or movement of rags of hypodermic cellulose to a deep vascular bundle and a bone. In a stage of a fibredema completely excise patholologically the changed fabrics (hypodermic cellulose, a fascia, and in nek-ry cases and skin). Wound defect is closed in one step or step by step the displaced skin rags or free skin autografts taken from the struck or healthy area of a body (see. Skin plastics ).
At an operative measure concerning S. of external genitals completely delete affected areas of a scrotum or vulvar lips and remove the main reasons which caused S.'s development (recover passability of an urethra, liquidate uric flow, delete a tumor, etc.). Fabrics of a scrotum exsect with an obligatory resection of a parietal layer of own cover of a small egg and create an artificial scrotum at the expense of healthy fabrics. If process is limited only to a penis, then it is degloved (see. Scalping ), and the integument is recovered skin of the scrotum or a hip taken in a femoral triangle. In the presence of rough changes in covers of a small egg or epididymite (see) hemicastration is shown (especially at elderly people) (see. Castration ).
The forecast and Prevention
the Forecast at timely carried out treatment favorable. The conservative treatment begun in an early stage quite often prevents further progressing of Page. Radical operation yields the good immediate and long-term results; at elephantiasis of external genitals operation provides positive effect both concerning recovery of sexual function, and in the cosmetic relation.
Prevention consists in the prevention and timely treatment of the diseases causing emergence of Page. At the same time prevention of an erysipelatous inflammation has extremely important value.
Features of elephantiasis at children
Primary inborn S., both hereditary (Milroy's disease), and caused by malformations collector limf, vessels and limf, nodes, is observed at children relatively more often than at adults. P. M. Medvedev (1964) noted inborn S. at 12 of 73 children, sick S. that makes 16,4%. Much less often also the acquired S. meets secondary inborn (owing to amniotic banners, an inborn tumor like lymphangioma, etc.) (after acute inflammatory processes in limf, vessels and nodes, injuries, operative measures, etc.).> Milroy's diseases are especially inherent bilateral defeat, the expressed changes of skin and frequent local inflammatory processes, damage of kidneys and development of the renal failure (see) which is often the reason of a lethal outcome.
The inborn S. caused by a malformation limf of vessels, is more often noted at girls. It can be combined with other malformations, napr, not fusion arterial channel (see), so-called. alate neck (see), malformations of veins, etc. As a rule, patol. process unilateral; bilateral defeat is observed seldom.
S.'s signs at a number of children come to light already at the birth or in the first 2 — 3 years of life, but a thicket at the age of 7 — 12 years. As well as at adults, process develops slowly and there pass three stages — from a lymph Dyoma to a fibredema. In a late stage at children disturbances of blood circulation and trophic changes of skin — the increased perspiration, pigmentation, warty growths, formation of papillomas, bubbles and cracks are more expressed, to-rye are entrance gate for contagiums and the reason of a long lymphorrhea. In connection with the reduced resilience of fabrics of a children's organism to pathogenic microflora repeated inflammatory processes (the erysipelatous inflammation, limfangiit, etc.) proceed hard — with high temperature, a picture of a septicopyemia.
From the general frustration paramount value has disturbance of mentality. Oversizes of an extremity, difficulty of movements, a lymphorrhea, frequent inflammatory processes deprive of the child of an opportunity to develop normally; it avoids peers, becomes irritable, closed, lags behind in physical and mental development.
For S.'s diagnosis at children use the same methods, as at adults. Studying of a state lymphatic and blood vessels of the affected extremity (a limfografiya and a venografiya) is of especially great importance.
For S.'s treatment extremities caused by malformations limf, vessels (primary inborn and hereditary S.), the operative measure consisting in full removal of skin together with hypodermic cellulose and a fascia, replantations of the leather processed by a dermatome across Krasovitov on muscles of an extremity is preferable. In secondary inborn S.'s cases remove the causes causing disturbance of a lymphokinesis in an extremity. Sovr. the microsurgical equipment allows at nek-ry patients from the acquired S. at the corresponding indications to recover passability limf, collectors or to impose the limfovenozny anastomosis providing outflow of a lymph in venous vessels.
The forecast and prevention same, as at adults.
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K. G. Abalmasov; D. D. Zerbino (stalemate. An.), Yu. A. Tikhonov (it is put. hir.), V. I. Shapoval (Ur.).