Ormand's disease is:
Ans. a (Idiopathic retroperitoneal fibrosis) (Ref. Bailey and Love 24th ed., 64.4)IDIOPATHIC RETROPERITONEAL FIBROSIS (Ormand's disease)# a rare condition in which one or both ureters become bound up in a progressive fibrosis of the retroperitoneal tissues.# The cause is unknown although some cases may be drug related.# A similar clinical picture occurs in patients with leaking aortic aneurysm and infiltrating retroperitoneal malignancy.# The patient complains of backache which is unremitting for several months, n ESR is markedly raised.# The onset of anuria and renal failure prompts investigation of the renal tract which reveals hydronephrosis.# The excretion urogram typically shows displacement of the obstructed ureters towards the midline.# The appearances on CT are diagnostic.# Treatment- It may be possible to insert ureteric stents as a temporary measure while renal function recovers.- If not, percutaneous nephrostomies will allow the obstructed kidneys to drain.- Some patients need renal replacement by dialysis.- Some advocate that these patients should be treated conservatively with high-dose steroids.- Surgical treatment involves careful dissection of the ureters from their entrapment (ureterolysis).- Wrapping omentum around the freed ureters make recurrent obstruction less likely.Educational Points:RUPTURE OF THE URETER# Injury during pelvic surgery is far more common.# Occurs most often during vaginal or abdominal hysterectomy.# Preliminary catheterisation of the ureters prevents such accidents.# Repair of the injured ureter- If there is no loss of length and the cut ends of the ureter can be brought together without tension, they should be joined by a spatulated anastomosis over a double pigtail catheter.- If the division is very low down, the bladder wall may be hitched up so that the ureter can be reimplanted into it. Extra length may be obtained by mobilising the kidney.- Boari's operation. A flap of bladder wall is fashioned into a tube to replace the lower ureter.- The ureter may be implanted end to side into the contralateral ureter. The disadvantage of a transureteroureterostomy is that it risks converting a unilateral injury into a bilateral one.- Occasionally, when conservation of all renal tissue is vital, replacement of the damaged ureter by a segment of ileum is necessary.- Nephrectomy may be the best course when the patient's outlook is poor and the other kidney is normal.
Idiopathic retroperitoneal fibrosis
Idiopathic retractile mesentritis
Idiopathic mediastinal fibrosis
Idiopathic thyroiditis
Peritoneum