ORMOND DISEASE

From Big Medical Encyclopedia

ORMOND DISEASE (J. To. Ormond, sovr, American urologist; synonym retroperitoneal fibrosis) — a peculiar type of a mezenkhimopatiya — the nonspecific fibrosklerotichesky process developing in kletchatochny connective tissue spaces it is preferential in retroperitoneal.

For the first time I. Albarran in 1905 reported about this disease. In more detail it was described by Ormond (1948), and since then it is called his name.

The etiology and a pathogeny

Emergence and development of fibrosis in retroperitoneal cellulose can be caused by inflammatory process, coming from uric and other bodies, adjacent to retroperitoneal cellulose; uric extravasates, obuslov-lennykhm piyelorenalny refluxes; the injury which led to a retroperitoneal hematoma; autoimmune processes; aneurism of a ventral aorta; influence of some medicines; infectious disease (Toxoplasmosis, brucellosis, etc.). Among etiol, factors an important place, by data A. Ya. Pytelya, the uric granuloma caused by intermittent piyelosinusny refluxes with the subsequent distribution of uric infiltration on cellulose of retroperitoneal space borrows.

Pathogenetic connection of a disease of Ormond with collateral influence of nek-ry pharmaceuticals at their long reception, in particular high doses of nek-ry antibiotics, ergotamine, and - methyl-dofa is established. The extravasates of urine arising in retroperitoneal cellulose containing derivatives of nek-ry pharmaceuticals would promote O.'s development.

Defeat of cellulose on the one hand is more often observed, i.e. the left or right retroperitoneal space, it is rare — at the same time both. Two options of a current patol, process are possible: tubate, or futlyarny, and plane. The first should be considered as a result of an ekstravazation of urine in the cellulose surrounding uric ways and large retroperitoneal vessels in the form of the coupling. The second — as influence of nek-ry pharmaceuticals at their prolonged use.

Pathological anatomy

Fig. 1. Microdrug of retroperitoneal cellulose at Ormond's disease: a fibrosclerosis of fatty retroperitoneal tissue around an ureter; limfoplazmotsitarny infiltrates (are specified by shooters).

At O. sclerous the changed cellulose squeezes and displaces an ureter, large main vessels, limf, ways, are more rare — a pelvis, a kidney. Cellulose — very dense, lobular, light brown or gray color, penetrated by zhelatinoobrazny layers. At far come process sclerous changes in cellulose lead to deformation of a pelvis and its cups, displace an ureter in the medial party, narrow its gleam. Microscopically cellulose consists of the homogeneous masses containing infiltrates (fig. 1) with separate collagenic fibers, fatty and connective tissue cells. In infiltrates — hyaline deposits. Inflammatory reaction is insignificant. Among the homogeneous masses scattered in fatty tissue macrophages and fibroblasts are located. Macrophages contain gemosiderinovy granules and drops of fat. The more collagenic fibers, the scarring leading to a stenosis of an ureter, cups and a pelvis is expressed more intensively.

A clinical picture

At O. slowly there comes the prelum of an ureter and shift it to a backbone that leads to hydronephrotic transformation. The prelum of the lower vena cava, a ventral aorta and its large branches, limf, vessels and limf, nodes causes frustration krovo-and lymphokineses. Until in cellulose of retroperitoneal space well functions limf, the drainage and venous collateral outflow, fibroplastic process can latentno proceed a long time, without breaking a condition of the patient. But as soon as limf, ways as a result of their prelum and an obliteration are in whole or in part blocked, the disease accepts the progressing current. At most of patients symptoms from uric ways and an intestinal path prevail: periodically renal colic, hamaturia, abdominal pain and waist, dysfunction of intestines, etc. At O. arterial hypertension is often observed, edges at a part of patients disappears after the corresponding treatment. Such complications as a heart attack of a small egg, to the gidrotsela, a priapism, chyle ascites, disturbances of blood circulation of the lower extremities, etc.

== the Diagnosis == are possible

Fig. 2. Excretory Ormond's sick with a disease Urogramum: shift of ureters (are specified by shooters) in the medial party to a backbone.

Excretory urography (see) allows to diagnose an urinary stasis in upper uric ways, the shift of an ureter in the medial party to a backbone (fig. 2). When owing to an extra ureteralny prelum of an ureter it is impossible to make a retrograde piyelografiya, and the excretory urography is contraindicated, resort to an antegrade liyeloureterografiya (see. Piyelografiya ). Can be diagnostic help limfografiya (see) and a venokavografiya (see. Kavografiya ), aortografiya (see). Define by tracer techniques funkts, a condition of kidneys and uric ways. Differential diagnosis carry out with tumorous processes of retroperitoneal space, obstructive defeats of an ureter of other etiology (stones, new growths, beam damages, a segmented nonspecific urethritis, etc.), usual inflammatory processes of retroperitoneal cellulose, effects of injuries of upper uric ways, etc.

Treatment

Fig. 3. The diagrammatic representation of stages of operation of an ureterolysis with an intraperito-nization of an ureter at Ormond's disease: and — a section of a back leaf of a peritoneum over an ureter (the line of a section is specified by an arrow); — the peritoneum (1) is cut and the ureter (2) from the sclerosed cellulose is allocated; in — the ureter (1) is moved to an abdominal cavity and under it the opening (2) in a back leaf of a peritoneum is sewn up.

In initial stages of a disease treatment by corticosteroid drugs — Prednisolonum in a daily dose of 40 mg within a month, and then in gradually reduced doses within 4 — 5 months is shown; use of fabric therapy across Filatov (an aloe, a suspension of a placenta); a roentgenotherapy in small antiinflammatory doses. Therapy by corticosteroids can yield satisfactory result. Operational treatment is shown to most of patients: a) an ureterolysis with intraperitonizatsny an ureter (fig. 3); b) an ureterolysis with an enveloping of an ureter a big epiploon, (this type of intervention is at the most part of patients with operation of the choice, at a cut a recurrence is celebrated at 10% operated); c) a resection of the squeezed site of an ureter with imposing of an anastomosis the end in the end or substitution of the narrowed segment of an ureter a piece of an ileal gut on a mesentery; d) autotransplantation of a kidney. The postoperative lethality reaches 10%.

The forecast

threatens the Patient the slow, but progressing prelum of ureters, disturbance of blood supply of intestines as the root of a mesentery can be involved in process. As a result in the absence of effect of operation patients perish at the phenomena of uraemia or the joined infection in uric ways. Over-all mortality of 15 — 20%.



Bibliography: Vectors And., Patrashkov T. and Tsolov Ts. Diagnosis and treatment of retroperitoneal fibrosis, Urol, and nefrol., Jvft 1, page 55, 1971; Peschansky V. S. and A. A. K Gres of morphology of idiopathic retroperitoneal fibrosis (Ormond's disease), Arkh. patol., t. 35, No. 2, page 76, 1973; Pytel A. Ya. Pe-ripelvikalnaya an uric granuloma and its value in development of retroperitoneal fibrosis, Urol, and nefrol., No. 5, page 16, 1972; V. I. and Nechiporenko A. Hares 3. Ormond's disease (The fibrous stenosing periureteritis), in the same place, No. 6, page 18, 1967; Hardy J. Page of La fibrose retroperitoneale, Acta urol. belg., t. 42, p. 113, 1974, bibliogr.; Lepor H. Walsh P. G. Idiopathic retroperitoneal fibrosis, J. Urol. (Baltimore), v. 122, p. 1, 1979, bibliogr.; Mas ini G. C. e. a. II trattamento chirurgico delle stenosi ureterali in corso di fibrosi retroperitoneale, Urologia (Treviso), v. 44, p. 314, 1977, bibliogr.; Ormond J. K. Bilateral ureteralobstruction due to envelopment and compression by an inflammatory retroperitoneal process, J. Urol. (Baltimore), v. 59, p. 1072, 1948; W a g e n k-n e with h t L. V. Retroperitoneale Fibro-sen, Stuttgart, 1978.


A. Ya. Pytel.

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