Pyelonephritis is a frequent urinary tract infection, with global estimates ranging from 10.5 million to 25.9 million cases annually. Each year, pyelonephritis causes 4000 deaths in the United States and its treatment costs USD 2 billion [1].

However, despite being common in the population and well known by the medical community, urinary tract infections, especially pyelonephritis, can be challenging in some cases with an underlying anomalous anatomy because these rare anomalies can make the diagnosis and treatment substantially more difficult [2].

Among these rare anomalies, we highlight ureterocele, a congenital condition formed by the dilatation of the distal segment of the ureter. It occurs in 1 in every 5000 to 12,000 births and is frequently diagnosed in childhood. Thus, in adults, this condition usually presents as an incidental radiological finding and an orthotopic insertion. When uncommonly symptomatic, ureterocele is related to lithiasis or its own prolapse [3],[4].

However, we describe a rare case of an adult woman diagnosed with complicated pyelonephritis who was found to have several rare anatomical abnormalities, which were challenging to treat.

A 23-year-old married woman was admitted to the hospital for a 5-day progressive, severe colicky pain in the suprapubic region, irradiating to the left labia majora. This pain was associated with dysuria, fever, and hyporexia. Two days earlier, the pain began to irradiate to the left flank with a progressive increase in intensity and concentrated diuresis with a fetid odor.

The patient’s general condition was stable, with intense pain on deep palpation of the left hemiabdomen and Giordano's sign present on the left. She was tachycardic (HR = 110 bpm), eupneic, dehydrated (+1/+4), pale (+1/+4), febrile (T = 37.9°C), and hypotensive (BP = 90/50 mmHg). Laboratory tests confirmed an acute infectious process with marked neutrophilia (32,000 leukocytes with 29% neutrophils) and a positive urine culture for multi-sensitive Escherichia coli.

Ultrasonography of the kidneys (Figure 1), urinary tract, and pelvis showed a duplicated left kidney collecting system, hydronephrosis of the upper collecting system with probable pyogenic content, and a left ureterocele with thickened content. To better evaluate the case, the patient underwent contrast-enhanced computed tomography (CT) of the whole abdomen and pelvis, which demonstrated bilateral pyelocalyceal and ureteral duplicity, left pyonephrosis, and anomalous implantation of the upper left ureter into the vagina, forming a ureterocele with a probable pyogenic content (Figure 2). Tapering of the renal parenchyma of the left kidney's upper unit was also associated with hydronephrosis, without contrast uptake. The magnetic resonance and radioisotope renography weren’t available in the moment. Speculum examination confirmed the presence of a ureteral meatus inserted into the vagina (Figure 3). Thus, we could schematically represent the main anatomical alterations of the patient (Figure 4).

She was started on ceftriaxone 1 g every 12 h, and ureterocele drainage was performed through an intravaginal scalpel incision in the wall facing the ureterocele, with drainage of approximately 100 mL of purulent content. After 12 days of hospitalization, the clinical picture had significantly improved and an open upper left polar nephrectomy with closure of the ureteral stump was performed. The patient evolved satisfactorily and was discharged on the third postoperative day.

The most common pyelonephritis symptoms are systemic inflammation (fever, chills, headache, sweating, and malaise), bladder inflammation (dysuria, urgency, altered urination, and polyuria), gastrointestinal symptoms (anorexia, hyporexia, nausea, vomiting, and abdominal pain), and renal pain symptoms (pain in the lumbar region and flank ipsilateral to the affected kidney) [1],[2]. In this case, we immediately suspected pyelonephritis based on clinical symptoms and laboratory tests [1],[5].

However, this case is peculiar due to the infrequent findings on imaging exams. Bilateral duplication of the collecting system occurs in 0.07–0.11% of the population [6] and ureterocele occurs in 1 among 5000 to 12,000 autopsies [7]. However, the rarest finding is the insertion of the left ureter into the vagina forming a ureterocele, which is described in very few case reports. This rare insertion may be diagnosed due to urinary incontinence. Still, as the patient did not report any history of incontinence, but she answered about similar attacks of pain and fever, previous finding in occasional gynecology vaginal examination or recurrent urinary tract infection (UTI) during childhood, even when directly questioned. It is believed that the ureteral meatus in the vagina was not adequately formed, preventing urine outflow [8].

The clinical management and the drainage of the infectious content of the ureterocele were fundamental for the patient's recovery. Even with the scarce literature on the approach of vaginal ectopic ureterocele with infected content in adult patients, drainage was necessary due to the purulent collection in the ectopic ureterocele. Drainage of the ureterocele with bladder insertion ureter is usually done by the transurethral endoscopic approach [9], but vaginal drainage was chosen for the only possibility of access.

A partial open nephrectomy was indicated for pyonephrosis with destruction of the left kidney's upper unit. Complete duplicity of the ureter may become a challenge for urologists in pyelonephritis management due to anatomical variations. If the renal function in one segment is severely impaired, heminephrectomy becomes necessary [10]. The ureteric stump was closed with suture and kept in the patient's body.

Rare urinary tract malformations such as ureterocele with ectopic vaginal insertion greatly complicate the management of pyelonephritis. In this rare situation, intravaginal drainage is a possible intervention. In complete duplications of the pyeloureteral system, we must consider ureteral implantation in the vagina, even in the absence of urinary incontinence.