Ureteric Stump Syndrome After Nephrectomy in a Patient With Rare Incidentally Detected Congenital Anomalies: A Case Report

INTRODUCTION

Ureteral stump syndrome is a rare condition, with an incidence of 0.8 to 1%, and an under-recognised postoperative complication occurring after nephrectomy or ureterectomy, particularly when the distal ureter is retained. It may present with nonspecific symptoms, including recurrent urinary tract infections (UTIs), pelvic pain, or persistent discharge, and may remain undiagnosed for extended periods, especially in patients with complex congenital anomalies or atypical ureteral terminations.^[1,2] Ectopic ureters, commonly associated with congenital anomalies such as renal dysplasia or agenesis, often manifest with urinary incontinence in females due to ureteral opening below the external sphincter, frequently into the vagina.^[3,4] In such cases, nephrectomy alone may not resolve the clinical symptoms if the ureteral remnant remains as a site of infection or abscess formation.^[5]

The constellation of genitourinary and musculoskeletal anomalies observed in some patients, including vertebral anomalies, rib hypoplasia, and cardiac abnormalities, may suggest an underlying syndromic or developmental pathology.^[6] However, the absence of maternal obstetric history and lack of genetic analysis can obscure the definitive diagnosis and categorisation of such anomalies.

This case presents a unique clinical scenario involving ureteral stump syndrome developing seven years post-nephrectomy in a female with ectopic ureteral termination and multiple congenital anomalies, emphasising the need for complete surgical excision and long-term follow-up in such patients. It also highlights the psychosocial and financial challenges faced by individuals with congenital disorders, particularly in the absence of healthcare policies acknowledging internal or non-visible disabilities.

CASE REPORT

A 30-year-old female was evaluated for surgical management of ureteral stump syndrome, which developed seven years following a laparoscopic left total nephrectomy. The patient had a lifelong history of continuous urinary dribbling, which was diagnosed at the age of 18 as an ectopic left ureter with vaginal termination. Imaging and clinical assessment at that time confirmed ectopic insertion of the left ureter into the lower third of the vagina. Due to chronic pyelonephritis involving the left kidney, a laparoscopic total nephrectomy was performed; however, the distal ureteral stump was left in situ.

She now presented with complaints of chronic vaginal discharge, which initially appeared serous, later becoming copious and serosanguinous. Over the subsequent 3-4 weeks, the discharge became profuse, yellowish, and blood-tinged. She also reported recurrent high-grade fever, lower abdominal pain, and back pain persisting for approximately 1 year, which had not been medically evaluated. The current episode of symptoms had been ongoing for 6 weeks and was refractory to intravenous antibiotic therapy. On clinical examination, a small opening was noted in the left lower vaginal wall. A swab culture grew Staphylococcus aureus, sensitive to third-generation cephalosporins.

Surgical excision of the retained ureteral stump was planned. Intraoperatively, minute tissue deposits were observed over both ovaries; histopathological examination confirmed the presence of endometriosis.

Pelvic ultrasonography and transvaginal sonography revealed a 14 cm × 3 cm serpiginous tubular structure with echogenic internal content, extending cranially to the left lumbar region and caudally to the vaginal vault. Extensive perilesional fat stranding was also visualised, suggesting abscess formation within the retained ectopic ureter, complicated by secondary infection [Figure 1]. Laparoscopic hernioplasty with excision of the radical ureter and an excision of an incidentally found endometrial cyst was performed under general anesthesia.

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Figure 1:

Magnetic resonance image of the right and left ureter (arrows).

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A previous MRI of the abdomen and pelvis had demonstrated several congenital anomalies: agenesis of the sacral segments S4, S5, and the coccyx; lumbarisation of the S1 vertebra as illustrated in Figure 2; and a blunted conus medullaris terminating at the L1 level. These were highly suggestive of the caudal regression syndrome. Additionally, a Spigelian hernia measuring 9 mm × 9 mm was observed in the left iliac fossa at the site of the prior laparoscopic port, with herniation of omental fat into the subcutaneous tissue but without involvement of bowel loops was noted. A well-marginated lesion measuring 18 mm × 13 mm, T1 hyperintense and T2 hypointense, was identified in the right mesorectum adjacent to the rectum, consistent with a cystic lesion containing thick material.

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Figure 2:

The magnetic resonance image images of the sagittal section of the lumbosacral spine (arrow).

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Past medical history revealed the development of persistent proteinuria ∼5 years post-nephrectomy. She also experienced recurrent episodes of lower urinary tract infections, which were managed conservatively with oral antibiotics. Approximately 1 year post-nephrectomy, she was diagnosed with Grade 1 hypertension. The associated symptoms were headache, giddiness, and chest pain on exertion, on walking uphill. Antihypertensive therapy with Amlodipine 5 mg once daily, along with dietary and lifestyle modifications, led to normalisation of her blood pressure within 8 months. She remained normotensive thereafter without pharmacological therapy, and her blood pressure has been monitored regularly.

A routine chest radiograph, performed as part of a pre-employment medical evaluation, incidentally revealed hypoplasia of the left fifth rib as illustrated in Figure 3, with its lateral margin situated approximately 2.5 cm medially compared to the contralateral ribs. Further cardiovascular assessment via 2D echocardiography revealed a minute intra-atrial septal aneurysm with no major abnormalities [Figure 4]. As the patient remained asymptomatic, no active intervention was recommended, and annual follow-up imaging was advised. Another associated anomaly was limb length discrepancy. The right leg of the patient was 3.4 cm shorter than the left, with observable limping while walking.

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Figure 3:

Illustrating the hypoplasia of the left 5^th rib (arrow).

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Figure 4:

2D echo image with no major abnormality. 2D: Two-dimensional.

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Due to the unavailability of maternal obstetric records, the etiology of this cluster of anomalies remains uncertain, and it is unclear whether the findings are attributable to a congenital developmental syndrome or represent class D adverse effects of an unidentified exposure during gestation.

The patient reported significant emotional and psychological distress stemming from the chronic incontinence experienced since childhood. She has undergone multiple psychiatric and psychological interventions to manage the resultant trauma. The incidental discovery of additional anatomical anomalies during routine investigations has been a persistent source of anxiety and stress. She also expressed concern about the lack of health insurance provisions for individuals with congenital anomalies unless classified as physically handicapped.

DISCUSSION

This case underscores several important aspects of congenital urological anomalies and their long-term implications. The patient had an ectopic ureter terminating in the lower third of the vagina, a classic presentation in females, often leading to continuous urinary dribbling since childhood.^[3]

Although she underwent a left total nephrectomy due to chronic pyelonephritis at the age of 23, the failure to remove the distal ureter resulted in a remnant becoming a nidus for chronic infection, culminating in ureteral stump syndrome. The symptomatic evolution, beginning as serous discharge and progressing to purulent, serosanguinous fluid with systemic symptoms like fever and back pain, is characteristic of a chronically infected stump.^[1] Imaging studies confirmed a serpiginous, abscess-filled ureter extending from the lumbar region to the vaginal cavity, an unusual but illustrative example of how retained ureteral stumps may complicate the clinical picture years after primary surgery.

Ectopic ureter, particularly in females, often goes undiagnosed until adolescence or early adulthood due to its misleading presentation as urinary incontinence.^[4] When diagnosed late, it may already be associated with renal damage or recurrent infections. Ideally, surgical intervention in such cases should aim for complete excision of the ureter to prevent future complications.^[7] The same was performed for our patient. Also, the herniorrhaphy was performed for the incisional hernia. Omission of the removal of most of the ureter during nephrectomy in this patient highlights the potential oversight in surgical planning when congenital anomalies are involved.

Performing transperitoneal laparoscopy to excise the maximum ureter during the nephrectomy would avoid such complications,^[8] whereas Bullock et al. reported that endoscopic sub-ureteric injection of dextranomer/hyaluronic acid as treatment for refluxing ureteric stumps.^[9] Some authors also treated patients with endoscopic electrofulguration followed by ureteric filler material injection.^[1] So, the correction of ureteric stump syndrome depends on the individual’s signs, symptoms, and the duration of primary surgery.

Moreover, the presence of multisystem anomalies, including sacral and coccygeal agenesis, lumbarisation, rib hypoplasia, intra-atrial septal aneurysm, and spigelian hernia, raises suspicion of a syndromic or developmental etiology.^[10-12] Although these anomalies may not fit a clearly defined syndrome, they suggest an embryological insult or genetic predisposition affecting multiple organ systems. Unfortunately, due to financial constraints and a lack of insurance coverage for non-visible congenital disorders, further genetic or developmental assessments were not pursued by the patient.^[13]

The psychological impact of this prolonged illness cannot be overstated. The continuous dribbling of urine from childhood, subsequent infections, and multiple incidental findings contributed significantly to the patient’s mental health burden. Patients with internal congenital anomalies often experience neglect from health systems, which focus more on visible disabilities.

This case advocates for comprehensive surgical excision in congenital urinary tract anomalies, long-term follow-up, multidisciplinary support, including psychological counselling and broader inclusion of such conditions in healthcare and insurance frameworks. It also emphasises the importance of genetic counselling and evaluation, which can be instrumental in guiding future care and assessing recurrence risks in familial contexts.

CONCLUSION

Ureteral stump syndrome is a rare condition that results from incomplete ureterectomy. The present case has a cluster of genitourinary anomalies with cardiac and bone anomalies, which is a rare combination. This case also reveals the hidden burden of internal congenital anomalies and the need for inclusive healthcare policies. Comprehensive and empathetic care, including genetic assessment, psychological support, and policy reforms, is required for the management of such patients.