A 34-year-old female presented with recurrent right flank pain with severe dysmenorrhoea. She was diagnosed with a right parametrial mass encasing the right ureter, leading to severe hydroureteronephrosis and non-functioning kidney. Initially she was managed with danazol and showed a positive response but there was a relapse following cessation of danazol. Finally, we performed a right nephroureterectomy with total abdominal hysterectomy and a bilateral salpingo-oophorectomy. The histopathological examination showed right ureteric wall involvement of endometrial tissue, adenomyosis, and chocolate ovarian cysts. The patient is doing well in the last 12 months of follow-up.
Dheeraj Kumar Gupta, Vishwajeet Singh, Rahul Janak Sinha, Pushp Lata Sankhwar
Submitted January 18, 2012 - Accepted for Publication February 17, 2012
KEYWORDS: Ureteric endometriosis, renal loss, chronic pelvic pain
CITATION: UroToday Int J. 2012 August;5(4):art 39.http://dx.doi.org/10.3834/uij.1944-5784.2012.08.12
Endometriosis is a reproductive disease in older women, with a prevalence of 3 to 10% [1,2]. The clinical presentations vary greatly depending on the number, size, and extent of the lesions. The diagnosis is strongly suspected from the patient’s clinical history and examinations [3,4]. Most patients complain of constant pelvic pain or a low sacral backache that occurs premenstrually and then subsides after menstruation. Lesions involving the urinary tract result in bloody urine in the perimenstrual periods with obstructive symptoms [5,6]. Medical treatment should be the initial treatment, but if it fails, and complications develop, surgical intervention is required [6-8].
A 34-year-old female presented with severe dysmenorrhoea over a period of 10 years and recurrent right flank pain for 10 months. Her general physical examination was unremarkable. Her vaginal and bimanual examinations revealed a bulky uterus and a normal cervix with right adnexal tenderness. Her abdominal ultrasonography showed right severe hydroureteronephrosis with an enlarged uterus and anechoic collection in its wall on the right side. Her renal function test was normal. The intravenous urogram showed a poorly functioning right kidney with severe hydroureteronephrosis. The contrast enhanced computed tomography (CT) scan of the abdomen showed a right-sided parametrial mass causing right lower ureteric compression. A double-J stent was inserted on the right side to relieve the obstruction. After a gynecological consultation, danazol (200 mg in a divided dose) was given for 6 months and her symptoms were relieved. The double-J stent was removed when hydroureteronephrosis improved. There was a relapse of symptoms following the cessation of danazol and stent removal. Her diethylenetriaminepentaacetic acid (DTPA) scan showed a non-functioning right kidney. The severe hydroureteronephrosis was complicated by an infection, which was initially managed by percutaneous nephrostomy. She was subjected to a right nephroureterectomy and total abdominal hysterectomy with bilateral salpingo-oophorectomy (Figure 1). Intraoperatively, there were dense adhesions and chocolate cysts, more on the right side. The incised uterine specimen showed a cavity in the right wall of the uterus (Figure 2). The postoperative period was uneventful. The histopathological examination showed the endometrial tissue in the right ovary, in the wall of uterus, and in the wall of the right lower ureter (Figure 3]. After endocrine and gynecological consultations, she was put on hormone replacement therapy. She is doing well in 12 months of follow-up.
Endometriosis is the presence of functional endometrial tissue at ectopic sites such as the ovaries, the uterosacral ligaments, and in the cul-de-sac. Genitourinary tract involvement is rare, with an incidence of 1 to 5%. Bladder involvement accounts for 70 to 80% of genitourinary tract involvement and the ureter in 15 to 20%, respectively [1,2]. Ureter involvement could be either intrinsic or extrinsic. The Intrinsic endometriosis is characterized by endometrial glands and stroma within the lamina propria, tunica muscularis, or ureteral lumen; extrinsic endometriosis is localized within periureteral tissue. Eighty percent of ureteral endometriosis is extrinsic and primarily involves the distal ureter. The left side is more often affected, and bilateral disease has been reported in up to 23% [1-3].
Classic symptoms and signs of urinary tract endometriosis include cyclical flank pain, dysuria, urgency, urinary tract infection, and hematuria. The patients with intrinsic endometriosis experience more symptoms than those with extrinsic disease. Ureteral endometriosis is generally underestimated due to its slow and silent nature, usually manifesting late as obstructive uropathy. Finally, there is the silent loss of renal function and nephrectomy in 30 to 40% of cases [1-3]. Urinary tract imaging is recommended for every woman presenting with pelvic endometriosis. Initial imaging may be done with ultrasonography. An intravenous urogram is needed if there is suspicion of ureteral involvement. Findings suggestive of intrinsic disease in an intravenous urogram are ureteral filling defects, whereas smooth-stricture ureteral segments are found in extrinsic disease. Retrograde ureteropyelography, a CT scan, and an MRI may be required to ascertain the exact location and volume of the disease [1-4]. Treatment should be individualized according to age, reproductive desire, symptom severity, extent of lesions, and the involvement of other organs. If renal function is normal and there is minimal to mild hydronephrosis with no functional obstruction, hormone therapy should be prescribed [5-7]. Hormonal therapy has a poor response in patients with intrinsic ureteral disease or extensive endometriosis [6,7].
Surgical intervention is the treatment of choice for most patients with significant hydroureteronephrosis and periureteral disease. If the patient does not desire future pregnancy, treatment is total abdominal hysterectomy and bilateral salpingo-oophorectomy . If pregnancy is desired, lesser extirpative procedures should be performed, such as unilateral oophorectomy [5,8]. Ureterolysis may correct ureteral obstruction in those with extrinsic disease. When intrinsic disease is present or when ureterolysis fails or is unlikely to work, distal ureterectomy with re-implantation has excellent long-term results [7,8].
In the present case, right hydroureteronephrosis progressed following the cessation of danazol, leading to severe hydroureteronephrosis and non-functioning kidneys. The patient had completed her family and did not desire a future pregnancy, thus right nephroureterectomy along with total abdominal hysterectomy and bilateral salpingo-oophorectomy was performed.
Urinary tract imaging is recommended for every woman presenting with pelvic endometriosis. Judicious and timely intervention may prevent renal loss.
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