0022-5347 /79/1221-0103$02. 00/0 Vol. 122, July Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright © 1979 by The Williams & Wilkins Co.
IDIOPATHIC RETROPERITONEAL FIBROSIS IN CHILDREN S. L. CHAN, H. W. JOHNSON
AND
M. G. McLOUGHLIN
From the University of British Columbia, Urology Clinic, Vancouver General Hospital, Vancouver, British Columbia
ABSTRACT
A case of idiopathic ret:roperitoneal fibrosis in an 11-year-old boy is presented. A review of 7 previous case reports revealed that there was no characteristic presentation in this disease process and that the diagnosis is suspected on an n=.nw,t-A-,.u u:rog-.cam. The treatment is primarily surgical but corticosteroids may be helpful in selective cases. ~u,A~ • . ,
Since Ormond first rPr,nrtPli on the clinical of idiopathic :retroperitoneal in 1948 numerous cases occurring in adults have been recorded. 1 The ~-""""''vu,u of this disease in children is still rare. 2-3 Herein we report a case of idiopathic ~~,,~n~ff~· fibrosis in an 11-year-old and review the literature on this CASE REPORT
An 11-year-old boy was hospitalized with a 10-day history of
and the antinuclear factor was Routine urinalysis was normal but amino acid r,vn,,•c,nnu revealed increased excretion of and hvclri,v,rn,·o indicative of active collagen metabolism. An c,v,.r,,rn,ru urogram (IVP) revealed extrinsic compression of the left ureter at the level of the brim causing moderate ureterocaliectasis (part A of figure). A left retrograde ureterogram :revealed an isolated segment of obstruction to be about 3 cm. in length (part B of figure). At transperitoneal laparotomy a plaque of fibrous
intermittent left loin pain. He had no urinary symptoms. Medical history included tonsillectomy and appendectomy. On physical examination the patient had a temperature of 38C. No mass was palpable in the abdomen but there was tenderness with guarding in the left costovertebral angle and left flank. Laboratory investigations revealed a white blood count of 8,400 with a normal differential. Renal function was normal. Uric acid was elevated at 5.7 mg. per cent and repeat determinations were 7.8 mg. per cent and 4.8 mg. per cent. Serum protein immunophoresis showed increased IgM at 183 mg. per cent. The sedimentation rate was 50 mm. per hour Accepted for publication September l, 1978. 103
tissue localized to the left side of the sacral promontory and encasing a segment of the left ureter just above the left common iliac artery was found. The right ureter was free of any induration. After ureterolysis the left ureter was placed in an omental sleeve brought extraperitoneally. Multiple biopsies of the fibrous plaque and para-aortic nodes revealed non-specific inflammation, active fibroplastic activity and collagenization. There also were scattered areas of fatty infiltration. Convalescence was uneventful and the patient was started on 60 mg. prednisone daily. A repeat IVP 1 month later showed complete resolution of the left ureteral obstruction C of figure). At that time the sedimentation rate was 2 mg. per cent. The patient is and free of any abdorr1inal
104
CHAN, JOHNSON AND McLOUGHLIN Reference
Age-Sex
Presentation
Treatment
Duffy'
7-mo. fetus-M
Hydramnios
Induction of labor, stillborn
Farrer and Peterson2
8 yrs. -F
U reterolysis
8 yrs.-? 15 yrs.-M
Lt. flank pain, inability to extend left hip, minimal urinary frequency ? Excessive thirst
15 yrs.-M
Backache and pain in lt. leg
12 yrs.-M
Abdominal pain, malaise
Laparotomy, anticoagulants Laparotomy, prednisone Laparotomy, resection of mass
Ormond' Packham and Yates-Bell' Husband and Knudsen' Peterson and asso-
8 yrs. -F
Lt. gluteal pain
ciates5
DISCUSSION
There have been 12 cases of idiopathic retroperitoneal fibrosis in children reported in the literature, with an age range from months in utero to 15 years (see table). The first reported case in 1962 presented with symptoms of abdominal, flank and hip pain. 2 A review of the presenting symptoms from the previous case reports shows that the urinary symptoms were varied in their presentation and often there were non-specific signs of inflammation, such as fever, leukocytosis and an increased erythrocyte sedimentation rate. The 7-month fetus reported on by Duffy was stillborn. 3 The mother had presented initially with hydramnios. Autopsy revealed the fetus to have an atrophic left kidney secondary to renal artery occlusion. The right kidney had a pathological picture compatible with proliferative glomerulonephritis. The pathogenesis of this lesion may have been related to renal vein and inferior vena cava obstruction. The fibrotic process also may be so extensive as to involve all midline structures with extension cephalad to include structures in the neck. 3 Most cases reported in the literature thus far have been treated with ureterolysis as the definitive form of therapy. In the adult there has been a high incidence of discovery of occult malignancy, such as lymphoma, Hodgkin's disease and metastatic neoplasms tliat were later found and implicated as causative agents in this retroperitoneal process. The association with occult malignancy has not been found in the 12 children reported on previously with the diagnosis of idiopathic retroperitoneal fibrosis. The diagnosis is often suspected on IVP and retrograde pyelogram. 6 An inferior venacavogram, lymphangiogram and ultrasonography aid in confirming the diagnosis. 7-" The management of this disease includes operation with ureterolysis and some form of mobilization of the ureter to prevent reencasement by fibrosis. This may take the form of an omental sleeve, intraperitonealization or lateral transposition of the ureters. The use of adjunctive therapy, such as corticosteroids, is still controversial but it generally is believed that if the
Associate Disease Atrophic lt. kidney, proliferative glomerulonephritis
?
U reterolysis
Inferior vena cava obstruction Lt. neck mass
fibrosis is still in a cellular stage, then the administration of steroids may be beneficial because of their inflammatory action and their ability to inhibit fibrous tissue maturation.8· 10 Measurement of the erythrocyte sedimentation rate and urinary determinations of proline and hydroxyproline can be used as monitors of fibrotic activity and, therefore, aid in monitoring the dose and schedule of corticosteroid administration. REFERENCES
1. Ormond, J. K.: Idiopathic retroperitoneal fibrosis. J.A.M.A., 174: 1561, 1960. 2. Farrer, J. and Peterson, C. G., Jr.: Idiopathic retroperitoneal fibrosis -report of first case observed in a child. Pediatrics, 30: 225, 1962. 3. Duffy, J. L.: Fetal retroperitoneal fibrosis associated with hydramnios. Case report with comments upon factors controlling amniotic fluid volume. J.A.M.A., 198: 993, 1966. 4. Husband, P. and Knudsen, A.: Idiopathic cervical and retroperitoneal fibrosis: report of a case treated with steroids. Postgrad. Med. J., 52: 788, 1976. 5. Peterson, A. S., Besecker, J. A. and Hutchison, W. A.: Retroperitoneal fibrosis and gluteal pain in a child. J. Ped., 85: 228, 1974. 6. Packham, D. A. and Yates-Bell, J. G.: The symptomology and diagnosis of retroperitoneal fibrosis. A review and presentation of case reports. Brit. J. Urol., 40: 207, 1968. 7. Kearney, G. P., Mahoney, E. M., Sciammas, F. D., Colpoys, F. L., Norton, A. T., Swinney, J. and Harrison, J. H.: Venacavography, corticosteroids and surgery in the management of idiopathic retroperitoneal fibrosis. J. Urol., 115: 32, 1976. 8. Sanders, R. C., Duffy, T., McLaughlin, M. G. and Walsh, P. C.: Sonography in the diagnosis of retroperitoneal fibrosis. J. Urol., 118: 944, 1977. 9. Bookstein, J. J., Schroeder, K. F. and Batsakis, J. G.: Lymphangiography in the diagnosis of retroperitoneal fibrosis: case report. J. Urol., 95: 99, 1966. 10. Mitchinson, M. J., Withycombe, J. F. and Jones, R. A.: The response of idiopathic retroperitoneal fibrosis to corticosteroids. Brit. J. Urol., 43: 44, 1971.
THE JOURNAL OF UROLOGY
Copyright © 1979 by The Williams & Wilkins Co.
IDIOPATHIC RETROPERITONEAL FIBROSIS IN CHILDREN S. L. CHAN, H. W. JOHNSON
AND
M. G. McLOUGHLIN
From the University of British Columbia, Urology Clinic, Vancouver General Hospital, Vancouver, British Columbia
ABSTRACT
A case of idiopathic ret:roperitoneal fibrosis in an 11-year-old boy is presented. A review of 7 previous case reports revealed that there was no characteristic presentation in this disease process and that the diagnosis is suspected on an n=.nw,t-A-,.u u:rog-.cam. The treatment is primarily surgical but corticosteroids may be helpful in selective cases. ~u,A~ • . ,
Since Ormond first rPr,nrtPli on the clinical of idiopathic :retroperitoneal in 1948 numerous cases occurring in adults have been recorded. 1 The ~-""""''vu,u of this disease in children is still rare. 2-3 Herein we report a case of idiopathic ~~,,~n~ff~· fibrosis in an 11-year-old and review the literature on this CASE REPORT
An 11-year-old boy was hospitalized with a 10-day history of
and the antinuclear factor was Routine urinalysis was normal but amino acid r,vn,,•c,nnu revealed increased excretion of and hvclri,v,rn,·o indicative of active collagen metabolism. An c,v,.r,,rn,ru urogram (IVP) revealed extrinsic compression of the left ureter at the level of the brim causing moderate ureterocaliectasis (part A of figure). A left retrograde ureterogram :revealed an isolated segment of obstruction to be about 3 cm. in length (part B of figure). At transperitoneal laparotomy a plaque of fibrous
intermittent left loin pain. He had no urinary symptoms. Medical history included tonsillectomy and appendectomy. On physical examination the patient had a temperature of 38C. No mass was palpable in the abdomen but there was tenderness with guarding in the left costovertebral angle and left flank. Laboratory investigations revealed a white blood count of 8,400 with a normal differential. Renal function was normal. Uric acid was elevated at 5.7 mg. per cent and repeat determinations were 7.8 mg. per cent and 4.8 mg. per cent. Serum protein immunophoresis showed increased IgM at 183 mg. per cent. The sedimentation rate was 50 mm. per hour Accepted for publication September l, 1978. 103
tissue localized to the left side of the sacral promontory and encasing a segment of the left ureter just above the left common iliac artery was found. The right ureter was free of any induration. After ureterolysis the left ureter was placed in an omental sleeve brought extraperitoneally. Multiple biopsies of the fibrous plaque and para-aortic nodes revealed non-specific inflammation, active fibroplastic activity and collagenization. There also were scattered areas of fatty infiltration. Convalescence was uneventful and the patient was started on 60 mg. prednisone daily. A repeat IVP 1 month later showed complete resolution of the left ureteral obstruction C of figure). At that time the sedimentation rate was 2 mg. per cent. The patient is and free of any abdorr1inal
104
CHAN, JOHNSON AND McLOUGHLIN Reference
Age-Sex
Presentation
Treatment
Duffy'
7-mo. fetus-M
Hydramnios
Induction of labor, stillborn
Farrer and Peterson2
8 yrs. -F
U reterolysis
8 yrs.-? 15 yrs.-M
Lt. flank pain, inability to extend left hip, minimal urinary frequency ? Excessive thirst
15 yrs.-M
Backache and pain in lt. leg
12 yrs.-M
Abdominal pain, malaise
Laparotomy, anticoagulants Laparotomy, prednisone Laparotomy, resection of mass
Ormond' Packham and Yates-Bell' Husband and Knudsen' Peterson and asso-
8 yrs. -F
Lt. gluteal pain
ciates5
DISCUSSION
There have been 12 cases of idiopathic retroperitoneal fibrosis in children reported in the literature, with an age range from months in utero to 15 years (see table). The first reported case in 1962 presented with symptoms of abdominal, flank and hip pain. 2 A review of the presenting symptoms from the previous case reports shows that the urinary symptoms were varied in their presentation and often there were non-specific signs of inflammation, such as fever, leukocytosis and an increased erythrocyte sedimentation rate. The 7-month fetus reported on by Duffy was stillborn. 3 The mother had presented initially with hydramnios. Autopsy revealed the fetus to have an atrophic left kidney secondary to renal artery occlusion. The right kidney had a pathological picture compatible with proliferative glomerulonephritis. The pathogenesis of this lesion may have been related to renal vein and inferior vena cava obstruction. The fibrotic process also may be so extensive as to involve all midline structures with extension cephalad to include structures in the neck. 3 Most cases reported in the literature thus far have been treated with ureterolysis as the definitive form of therapy. In the adult there has been a high incidence of discovery of occult malignancy, such as lymphoma, Hodgkin's disease and metastatic neoplasms tliat were later found and implicated as causative agents in this retroperitoneal process. The association with occult malignancy has not been found in the 12 children reported on previously with the diagnosis of idiopathic retroperitoneal fibrosis. The diagnosis is often suspected on IVP and retrograde pyelogram. 6 An inferior venacavogram, lymphangiogram and ultrasonography aid in confirming the diagnosis. 7-" The management of this disease includes operation with ureterolysis and some form of mobilization of the ureter to prevent reencasement by fibrosis. This may take the form of an omental sleeve, intraperitonealization or lateral transposition of the ureters. The use of adjunctive therapy, such as corticosteroids, is still controversial but it generally is believed that if the
Associate Disease Atrophic lt. kidney, proliferative glomerulonephritis
?
U reterolysis
Inferior vena cava obstruction Lt. neck mass
fibrosis is still in a cellular stage, then the administration of steroids may be beneficial because of their inflammatory action and their ability to inhibit fibrous tissue maturation.8· 10 Measurement of the erythrocyte sedimentation rate and urinary determinations of proline and hydroxyproline can be used as monitors of fibrotic activity and, therefore, aid in monitoring the dose and schedule of corticosteroid administration. REFERENCES
1. Ormond, J. K.: Idiopathic retroperitoneal fibrosis. J.A.M.A., 174: 1561, 1960. 2. Farrer, J. and Peterson, C. G., Jr.: Idiopathic retroperitoneal fibrosis -report of first case observed in a child. Pediatrics, 30: 225, 1962. 3. Duffy, J. L.: Fetal retroperitoneal fibrosis associated with hydramnios. Case report with comments upon factors controlling amniotic fluid volume. J.A.M.A., 198: 993, 1966. 4. Husband, P. and Knudsen, A.: Idiopathic cervical and retroperitoneal fibrosis: report of a case treated with steroids. Postgrad. Med. J., 52: 788, 1976. 5. Peterson, A. S., Besecker, J. A. and Hutchison, W. A.: Retroperitoneal fibrosis and gluteal pain in a child. J. Ped., 85: 228, 1974. 6. Packham, D. A. and Yates-Bell, J. G.: The symptomology and diagnosis of retroperitoneal fibrosis. A review and presentation of case reports. Brit. J. Urol., 40: 207, 1968. 7. Kearney, G. P., Mahoney, E. M., Sciammas, F. D., Colpoys, F. L., Norton, A. T., Swinney, J. and Harrison, J. H.: Venacavography, corticosteroids and surgery in the management of idiopathic retroperitoneal fibrosis. J. Urol., 115: 32, 1976. 8. Sanders, R. C., Duffy, T., McLaughlin, M. G. and Walsh, P. C.: Sonography in the diagnosis of retroperitoneal fibrosis. J. Urol., 118: 944, 1977. 9. Bookstein, J. J., Schroeder, K. F. and Batsakis, J. G.: Lymphangiography in the diagnosis of retroperitoneal fibrosis: case report. J. Urol., 95: 99, 1966. 10. Mitchinson, M. J., Withycombe, J. F. and Jones, R. A.: The response of idiopathic retroperitoneal fibrosis to corticosteroids. Brit. J. Urol., 43: 44, 1971.
