Acta Medica Scandinavica. Vol. 167, fasc. 2,1960
From the Bispebjerg Hospital, Surgical Department F (Director: E. Thomsen, M. D.) and Medical Department C (Director: N. B. Krarup, M. D.), Copenhagen, Denmark
Periureteritis Fibrosa (Gerotas Fascitis)
FLEMMING LUND and J0RGEN PEDERSEN
In 1948 Ormond reported the first two cases of acute anuria due to bilateral ureteral compression caused by retroperi- toneal formation of fibrous connective tissue. The fibrotic tissue was situated in Gerotas fascia (vide infra) and it en- veloped not only the ureters, but also the aorta and inferior vena cava. The substantiated cases of this presumably new syndrome now number 26 (Hutch et al. 1959), 10 of which have been reported within the past two years.
After briefly describing the syndrome, the authors will report the history of a patient in whom the disease was diag- nosed at so early a stage that, unlike previous cases, the lesion had not yet encroached upon the ureters, a factor of decisive prognostic significance.
Pathology, aetiology, and pathogenesis,
In all reported cases the pathological lesion has affected an area delimited by the perirenal fascia which bears the name of Gerota. This well-defined structure Submitted for publication December 10, 1959.
envelops both kidneys and ureters as well as the aorta and inferior vena cava in a kind of hood. Inferiorly, the cavity communicates with the extraperitoneal connective tissue in the true pelvis, while superiorly and laterally it is closed, the lateral limits being formed by the convex border of the kidney and by the ureters.
From the pathological point of view the lesion is a rapidly progressing, invasive fibrosis with a varying degree of inflam- matory reaction, histologically showing a predominance of lymphocytes and plasma cells, with only occasional poly- morphonuclear leukocytes. Thus, the chronic proliferative changes resemble those of non-specific inflammation. How- ever, a primary focus of infection has never been demonstrated. Moreover, the disease is generally not accompanied by fever, and enlargement of regional lymph nodes has not been reported. Nor have bacterial foci been encountered in biopsy specimens.
The shrinking, fibrotic processes tend to pull the ureters, especially their middle third, towards the midline, compressing
106 FLEMMING LUND AND J0RGEN PEDERSEN
Fig. I . Schematic transverse section at the level of the upper part of the second lumbar 1-ertebra to show the relationship of the renai fascia.
and eventually obstructing them, making the terminal phase one of anuria and uraemia.
A striking feature of the syndrome is that retrograde pyelography or ureteral catheterization do not always reveal the obstruction,* even after anuria has oc- curred. I t is probable, therefore, that the anuria is caused by a greatly in-
Fig. 2. Typical localization and spread of the fibrous process in periureteritis fibrosa (after Hutch et al. 1959).
hibited ureteral peristalsis, as known also from cases with widespread mali, man t lesions of the retroperitoneal connective- tissue spaces.
Mulvaney (1 958) has suggested the possibility of urinary-tract infection as an aetiological factor, emphasizing that ex- perimentally certain common urinary- tract bacteria (B. lactis aerogenes) may induce pathological changes of a nature similar to those seen in periureteritis fibrosa and that a dye instilled into the bladder will be demonstrated in the lym- phatics of the periureteral connective tissue. Oppenheimer et al. (1952) and MacLean (1954) have reported an in- teresting case each of unilateral ureteral obstruction in which the ureter Ivas firmly adherent to the common iliac artery at the bifurcation and in which the under- lying cause was possibly arteritis. Other noxae have been suggested as possible aetiological factors, int. al. antibiotics, antipyretics, etc., but the aetiology is still unknown. For the time being, therefore, the disease must be classified with the group of collagen diseases.
Considering the well-defined patho- logical appearances, it is surprising that
PERIURETERITIS FIBROSA 107
the disease has not been described until the past decade, and this might indicate, as already mentioned, that the syndrome is a new one.
Symptoms and Signs Men about the age of 40 are most
commonly affected. In the early stages, the chief complaint is pain, localized over the sacrum, in the flank, or around the umbilicus, possibly in the testes if the spermatic vessels are obstructed. How- ever, the initial symptoms are varied and not characteristic. As a rule, the patient does not seek medical advice until the urinary obstruction has become fairly advanced, involving pain in the renal region and signs of uraernia. However, impotence may be a comparatively early sign, if the sacral plexus is involved. The terminal phase, in the form of anuria, generally occurs from 1-5 months after the first symptom has appeared. Actual urinary-tract complaints, such as urinary infection, are slight, and the condition has never been interpreted as acute ab- domen. The symptoms and signs tend more to indicate a subchronic to chronic disease. It must be mentioned that al- though the fibrous lesions always envelop the inferior vena cava, the abdominal aorta, and the iliac arteries, no case has presented congestion or signs of arterial insufficiency in the lower limbs.
The most common sign is infra-um- bilical tenderness, more rarely swelling. Digital examination of the rectum may reveal a firm swelling or resistance high up at the promontory.
Intravenous urography is generally the procedure which arouses a suspicion of the true condition, as very often the ureters in the involved area, usually the middle third, are pulled medially over
the vertebral bodies, there being a more or less marked hydroureter proximally. Retrograde pyelographic appearances generally indicate irregular stricture. In advanced cases, presacral insufflation of air may be of diagnostic significance, since the fibrous lesion prevents the air from entering the space delimited by the perirenal fascia.
Merentid diagnosis The differential diagnostic possibilities
suggested hitherto are various forms of ureteral obstruction (retroperitoneal tu- mour, cancer of the pancreas, aneurysm of the aorta, etc.) as the patients generally do not enter hospital until the ureters become involved. The most important sign is the above-mentioned course of the ureters on the urogram. In cases investigated prior to this stage, the dif- ferential diagnosis causes great difficulties because of the uncharacteristic symptoms and signs. In this connection, it is well to bear in mind that the patients are generally men about 40 years of age (cf. also case history).
Treatment So far, the treatment has been surgical,
directed, in the acute and fulminant stage, a t the predominant anuria and uraemia. The methods have been ureteral catheterization or nephrostomy. Both exert a good, but only temporary effect. Where the course has been prolonged, efforts have been centred on liberating the incarcerated ureter by ureterolysis, with or without resection of a segment of the ureter. In such cases, it seems reasonable to intraperitonealize the mobi- lized segment in order to reduce the risk of recurrence. If operation is carried out
108 FLEMMING LUND AND J0RGEN PEDERSEN
at a n early stage, there is a risk that the process is not yet burnt-out and that it will continue to spread, while operation at a late stage may reveal irreparable and irreversible changes of both ureters and kidneys. It is of the utmost im- portance, therefore, to make the diag- nosis a t an early date, i. e. before urinary- tract symptoms have become manifest. In that case, there may be a chance of arresting the process by medication, avoiding surgery.
Case history The patient was a man, aged 44, with a
history of right-sided pulmonary tuberculosis. Treated in 1953 with pneumothorax and specific drugs. Negative sputum and no pul- monary symptoms since 1954. No other serious illnesses.
Admitted on 22nd Oct. 1958 to the Bispe- bjerg Hospital, Department F, as a case of acute abdomen. For the past 3 months he had been complaining of constipation with periodical rectal discharge of blood and mucus, recurrent oppression and pressure pain in the lower left quadrant of the abdomen, and a weight loss of 10-15 kg.
Physical examination showed a man in good general condition. No acute abnormality of the abdomen, but lateral to the belly of the rectus on the left there was a non-tender, ill-defined swelling with transmitted pulsation. Other findings: Height 168 cm, weight 55 kg increasing to 58 kg. Hb. 85-80 0,;. E. S . R. 49-39 mm/hour. Faeces negative for blood on 5 occasions. Proctoscopy normal. Roent- gen: General view of the abdomen showed slight meteorism as well as 4-5 pea-sized, circular shadows on the right of the lumbar column (?gallstones). Barium enema revealed mild colitis. No abnormality of the stomach. Chest radiography showed fibrotic inveterated and calcified changes in the left apex.
No treatment apart from analgesics. After consultation with Medical Department C it was believed that the patient might be suffer- ing from a relapse of the old tuberculous lesion in addition to the chronic constipation. He was transferred for further investigation to
Department C on Nov. 7, 1958, where his condition fluctuated. Often, he would have abdominal pain all day, localized by himself to the above-mentioned swelling. The tem- perature and pulse were normal throughout. Other findings: Hb. 90-86 ;A, E. S. R. 45- 43 mm/hour. Thymol reaction 0.09. Icterus index 7. Takata 1.48. Alkaline serum phos- phatase 12.3-13.4 units. Urinary diastase normal. Serum creatinine 1.0-0.6 mg per 100 ml. Antistreptolysin titre (AST; 0. .\nti- streptococcal hyaluronidase titre (ASH) 0, streptococcal agglutination titre (SIT) 0. Urine negative for protein, sugar, and T.b., microscopic examination normal. B. 31. R. + 6 :A. Sputum: negative for T.b. in smears and upon culture. Faeces still negative for blood. Paper electrophoresis showed slightly elevated gamma globulin, indicating perhaps a chronic, active lesion.
Roentgen: Tomography of the pulmonary apices showed no cavitation, only fibrous strands and calcifications. Cholecystography : Delayed excretion in the gallbladder, possibly with a few small translucencies. Intestinal passage: No abnormality. Urography : Nor- mal findings apart from a slightly plump right pelvis. In particular, the localization of the ureters was not definitely abnormal.
Since the patient was believed to be suffer- ing from gallstones and possibly cancer of the tail of the pancreas or aneurysm of the ab- dominal aorta, he was transferred back to Department F for exploratory laparotomy. Operation on Dec. 11, 1958 showed no ascites, and thorough inspection of the ab- dominal organs failed to disclose an>- ab- normalities. Also no gallstones. The posterior abdominal wall was then explored : Pancreas of normal size and consistency. The connective tissue around the abdominal aorta. on the other hand, was the site of morbid changes in its caudal 5 cm. It was hard as bone, fibrous, with a shiny surface, and adhering to an intestinal coil. The inferior vena cava was not involved, and the changes terminated laterally at the ureters. No thrill over the aorta. 4 biopsy specimen of the fibrous tissue and one pre-aortic lymph node were removed for histological examination (Dr. J. Ringsted) which showed : A fibrous, partially hyalinized connective-tissue capsule surrounding amor- phous, granular masses with spindle-shaped translucencies and widespread amorphous,
PERIURETERITIS FIBROSA 109
Fig. 3 A.
Fig. 3 B.
Fig. 3. A. Histological appearance of the fibrous, preaortic lesion: Hyalinized connective tissue with clusters of lymphocytes and plasma cells. x 180. B. The same lesion x 445.
calcareous granules as well as clusters of lymphocytes. In the lymph node also hya- linized connective tissue with diffuse infiltra- tion of lymphocytes and plasma cells as well as moderate hyperaemia. No tumour cells or T.b. in either preparation.
The postoperative course was uneventful. After discharge, however, the patient went on having abdominal pain. As his condition re- mained practically unchanged he was re- admitted to Department C on March 23, 1959. After a study of the literature, a diag- nosis of periureteritis was now made on the
basis of the operative findings which had shown the characteristic anatomical delimi- tation of the: fibrous tissue. However, as the patient had no signs of ureteral involvement, a number of further supplementary investi- gations were performed in order to rule out other diseases, int. al. Hodgkins disease. These investigations showed: B. P. 110/50 mm Hg. Weight 54.6-53.3 kg. Hb. 89-46 %. E. S. R. 45-60 mm/hour. Complete blood count normal. MCV 87 cubic microns. MCHC 30 g per 100. Haematocrit 39 yo. Spinal fluid normal. Antistreptolysin titre, antistrepto-
110 FLEMMING LUND AND J0RGEN PEDERSEN
coccal hyaluronidase titre, and streptococcal agglutination titre normal.
Biopsy from an inguinal lymph node showed reticulosis and fibrosis, but no specific changes. Biopsy from skin and striated muscle from the thigh normal. Neurological examination also failed to disclose abnormalities. Roentgen of the lumbar column and sacro-iliac joint: Dextro-convex scoliosis, but no other abnor- malities. Retroperitoneal pmumgraphy by the method of Rivas on Apr. 23, 1959 revealed normal outlines of the spleen, kidneys, and adrenals on the left with a distinct shadow of the psoas muscle and air anterior to the 4th and 5th lumbar vertebrae and sacral bone. On the other hand, there was no passage of air from the left to the right retroperitoneal space, also after changing from the right to the left lateral position.
Treatment On Apr. 27, 1959 the patient was started
on butazolidin medication, 100 mg three times daily, gradually reduced to 100 mg daily. As early as May 1, he stated spontaneously that he was feeling better, having less pain. When seen in the Out-patient Clinic on June 1, he had gained 4.5 kg and had no pain.
The long observation period and the numerous diagnostic procedures prior to the institution of butazolidin treatment illustrate the great difficulties which this case caused us. Since the patient did not present urinary symptoms, we did not treat him with butazolidin until we had tried to rule out diseases other than that indicated by the operative and histo- logical findings.
We are dealing with a fairly well- defined and as a rule rapidly progressive disease whose pathological manifestations are restricted to the area within the peri- renal fascia of Gerota and which ends in fatal uraemia. At such an advanced stage the signs - especially the roentgen
signs - are so characteristic as to make...