A rare case of advanced CKD with extensive cystic renal disease causing bowel obstruction

Abstract

Introduction:

Acquired cystic kidney disease (ACKD) occurs in patients with kidney failure, leading to renal cyst formation. Although typically asymptomatic, large cysts can cause unusual complications like gastrointestinal obstruction.

Case presentation:

An 18-year-old female with stage V chronic kidney disease developed abdominal distension and bowel obstruction caused by a large renal cyst. Imaging confirmed the diagnosis, and conservative management with fluid control, dialysis, and infection management was initiated.

Discussion:

The presentation of a renal cyst causing bowel obstruction is extremely rare, and the optimal management approach remains unclear. Diagnosis relies on imaging modalities such as ultrasound and CT, which help identify cyst characteristics and associated complications. In such cases, conservative treatment is generally preferred, focusing on symptom management through dietary modifications, while surgical intervention is considered only if symptoms persist despite medical therapy.

Conclusion:

This case highlights the rare presentation of bowel obstruction due to ACKD and emphasizes the importance of early imaging and a multidisciplinary approach in managing such complications.

Introduction

Renal cysts (RCs) are a group of disorders, either acquired or inherited, defined by the presence of benign fluid-filled sacs in the kidneys, typically of unknown etiology^[1]. Acquired cystic kidney disease (ACKD) arises as a consequence of structural alterations associated with kidney failure. It is marked by the presence of numerous small cysts in the kidneys, usually without any past history of congenital or inherited cystic diseases^[2] . ACKD can be distinguished from Autosomal dominant polycystic kidney disease (ADPKD) by genetic testing, as ADPKD involves mutations in genes like PKD1 or PKD2, which are absent in ACKD^[3].

Renal cysts are common, with prevalence rates ranging from 20% to 50% in the general population^[4]. It is estimated that by the age of 40, one in five adults will have a simple renal cyst, and this incidence increases to one in three by the age of 60^[5]. ACKD affects 7–22% of pre-dialysis patients, with male gender and underlying renal disease linked to its development^[6]. Most RCs are often discovered incidentally, located peripherally, and are asymptomatic. However, in some cases, they may grow large enough to cause symptoms such as pain, hematuria, hypertension, and obstruction of the pelvicalyceal system^[7]. Abdominal pain is considered an atypical presentation, described in various forms, such as dull, uncomfortable fullness, sharp stabbing sensations, and cramping^[8]. Gastrointestinal obstruction due to renal cysts is extremely rare and underreported, with large cysts only occasionally identified as a cause of bowel obstruction^[9].

HIGHLIGHTS

• Bowel obstruction from renal cysts is rare.

• CT and ultrasound help diagnose renal cysts.

• Treatment is mainly conservative; surgery if needed.

CT imaging plays a crucial role in evaluating renal cysts as it provides detailed visualization of the cyst wall and surrounding structures, helping to identify any complexities or changes^[10]. We present a rare case of an 18-year-old female with Stage V chronic kidney disease who developed bowel obstruction secondary to a large renal cyst, with the objective of highlighting this uncommon gastrointestinal manifestation of ACKD, outlining the associated diagnostic challenges, and discussing the clinical course and multidisciplinary management in accordance with the CARE guidelines^[11].

Case presentation

An 18-year-old female with advanced chronic kidney disease (Stage V) presented with abdominal distension that had progressively worsened over 10 days and mild pain initially localized to the lower abdomen, especially prominent in the lower quadrants. The pain was diffuse and unrelieved by analgesic medications. She also experienced nausea with vomiting 2–3 times daily, without any blood in the vomitus, which improved over the last 3 days. She reported a low-grade fever with a maximum temperature of 101°F, which responded to paracetamol. Additionally, she had sudden onset shortness of breath 2 days prior to presentation, likely related to fluid overload or uremic complications secondary to advanced chronic kidney disease. She denied dysuria, changes in urinary frequency, or foul-smelling urine. Her past medical history and family history were unavailable due to lack of reliable information from both the patient and her family.

On physical examination, the patient was mildly hypotensive with a blood pressure of 96/70 mmHg and a heart rate of 90 beats/min. Respiratory rate was 20/min and spO2 of 98% at room air. She had pallor but no edema. An abdominal examination revealed a non-tender, distended lump from the right upper quadrant to the left upper quadrant, with positive bowel sounds. A respiratory examination showed bilateral findings without specific abnormalities.

Laboratory findings revealed marked biochemical abnormalities, including elevated lipase, renal dysfunction, and secondary hyperparathyroidism as shown in Table 1.

Table 1.

Laboratory findings at the time of admission

Lab parameter	Result	Normal range
Lipase	1955 IU/L	23–300 IU/L
LDH	402 IU/L	140–280 IU/L
Hemoglobin (Hb)	8.6 g/dL	12–16 g/dL
Urea	73.4 mg/dL	15–40 mg/dL
Creatinine	1317 µmol/L	44–133 µmol/L
Sodium (Na)	124 mEq/L	135–145 mEq/L
Potassium (K)	3.1 mEq/L	3.5–5.0 mEq/L
Total calcium	1.6 mmol/L	2.1–2.6 mmol/L
Phosphorus	5.0 mg/dl	2.5–4.8 mg/dl
Vitamin D	24 ng/ml	30–50 ng/ml
Iron	40.3 µg/dl	60–130 µg/dl
Total iron binding capacity (TIBC)	142 µg/dl	200–400 µg/dl
Intact parathyroid hormone (iPTH)	1430.7 pg/ml	15-68.3 pg/ml

On the night of admission (Day 1), the patient’s ECG revealed significant abnormalities, including left atrial enlargement and lateral ST-T changes consistent with myocardial ischemia, reflecting her progressively worsening cardiovascular status secondary to advanced chronic kidney disease. The cardiac consultant immediately transferred her to the ICU for close monitoring and initiated necessary cardiac evaluations. On the first day, she was administered intravenous paracetamol 500 mg, intravenous ondansetron 4 mg, intravenous ranitidine (Aciloc) 50 mg, and injection dexamethasone (Xone) 1 mg.

On the second day, after discontinuation of noradrenaline infusion at 0.2 mcg/kg/min, hemodialysis was initiated following insertion of a jugular catheter. Cefazolin was administered intravenously post-catheter insertion. For persistent vomiting, intravenous ondansetron 4 mg was given as a stat dose. Additionally, intravenous calcium gluconate (3 amps) was infused over 30 minutes. Following hemodialysis, the patient received intravenous dextrose 50% (2 amps, 40 mL) as a stat dose to manage hypoglycemia.

Given her increased susceptibility to infections related to end-stage renal disease, she was started on broad-spectrum intravenous antibiotics, including cefazolin 1 g every 8 hours, metronidazole 500 mg every 8 hours, and ceftriaxone 1 g every 24 hours. In view of uremic gastropathy and fluid overload, she underwent hemodialysis with close monitoring of fluid balance and ultrafiltration. Anticoagulation was carefully managed by adjusting heparin doses (1000–2000 units) to minimize bleeding risk during dialysis.

The management on days 2 and 3 was similar, focusing on controlling vomiting and fever. The patient received intravenous ondansetron 4 mg, injection Perinorm 1 amp IV stat for nausea, and intravenous dextrose 50% (20 mL) as needed.

A CT scan on day 3 revealed a large, multiloculated cystic lesion (34 × 19.9 × 14.1 cm) in the right abdomen and pelvis, displacing adjacent bowel loops and vasculature, with a small calcific focus in the posterior wall (Fig. 1). The right kidney was not visualized, suggesting gross hydronephrosis, while the left kidney was small, dysplastic (5.11 × 2.23 cm), and exhibited cystic changes with thinned parenchyma. Splenomegaly was also noted, with the spleen measuring 14.7 cm in its longest axis. These findings were consistent with right hydroureteronephrosis, likely due to pelviureteric junction stenosis and venolymphatic malformation. The right kidney demonstrated gross enlargement and significant dilatation of the pelvicalyceal system with a thinned cortex. The left kidney showed increased echogenicity and mild pelvicalyceal dilatation. A DMSA scan confirmed bilateral gross hydronephrosis with cortical thinning. As shown in Fig. 2, ultrasonography revealed a large, incompletely septate cystic lesion with echogenic content occupying the abdomen and compressing bowel loops, supporting the diagnosis of gastrointestinal obstruction secondary to a renal cyst. Genetic testing was not performed due to the unavailability of diagnostic services in the hospital and the lack of accessible genetic analysis facilities in the country.

Figure 1.

Axial contrast-enhanced CT scan of the abdomen showing a large, multiloculated cystic lesion occupying the right retroperitoneal region. The cyst exerts significant extrinsic compression on adjacent bowel loops, leading to mechanical bowel obstruction. Mild displacement of surrounding vasculature is also evident, consistent with mass effect.

Figure 2.

Ultrasound image of the abdomen demonstrating a large renal cyst with internal septations and echogenic content. The cyst causes visible compression of adjacent bowel loops, suggestive of extrinsic obstruction. These findings support the diagnosis of gastrointestinal obstruction secondary to a renal cyst.

Post-dialysis management on the 4th day was complex, as she repeatedly experienced hypotension, hypoglycemia, and episodes of epigastric pain, which were managed with additional fluid support, dextrose infusions (D5W, 5% dextrose) at 75-100 mL/hour, and pain control using intravenous aciloc (omeprazole) 40 mg once daily.

Family members were extensively counseled on her condition, given the progressive nature of her renal disease and its systemic complications. Discussions included the need for continued dialysis, dietary adjustments, and vigilant monitoring of fluid and electrolyte balance. A renal diet of nil per os was advised due to her advanced CKD and anemia. She was given a low-protein diet to reduce kidney workload, potassium and phosphate restrictions to manage electrolyte imbalances, and iron supplementation to address her anemia.

The patient initially showed symptomatic improvement following hemodialysis, broad-spectrum antibiotics, fluid resuscitation, and pain management. She was discharged in stable condition with a 4-week course of Cefixime 200 mg twice daily, Calcium Acetate 667 mg with meals, Calcitriol 0.25 mcg once daily, Paracetamol 500 mg as needed for pain management and Amlodipine 5 mg once daily for blood pressure control . At her 2-week follow-up, she reported partial relief of symptoms, and renal function remained at baseline. However, this improvement was short-lived. Over the next few visits, she developed persistent electrolyte imbalances, worsening anemia, and fluctuating renal function. Imaging continued to reveal extensive cystic renal disease with bilateral hydronephrosis and splenomegaly. With minimal functional renal parenchyma remaining, management shifted to a multidisciplinary, supportive approach focused on symptom control and maintaining quality of life.

Discussion

Adult renal cystic diseases encompass a broad spectrum of conditions that can be classified into hereditary and non-hereditary categories. The common causes in patients with multiple renal cysts remain ADPKD, ACKD, and simple renal cysts^[10]. ACKD, as in the case presented, is characterized by the formation of multiple bilateral renal cysts (three or more per kidney) in patients with end-stage renal disease, occurring without a history of hereditary cystic kidney conditions^[12]. The prevalence of ACKD is about 7% in pre-dialysis patients and 22% in patients undergoing dialysis^[13].

Giant renal cysts larger than 15 cm are exceptionally rare pathological occurrence^[14]. In our case, the patient presented with a large, multiloculated cystic lesion in the right abdomen and pelvis, which was likely a manifestation of ACKD. Her imaging studies, particularly the CT scan, highlighted a large cystic mass in the right retroperitoneum, which displaced adjacent bowel loops and vessels. In ACKD, the kidneys are typically smaller than normal with multiple bilateral cysts, although some cases with enlarged kidneys have also been reported^[15]. The loss of nephrons and the consequent impairment of renal function appear sufficient to initiate cyst formation^[16]. This aligns with the known pathophysiology of ACKD, where cyst development occurs as a consequence of chronic renal injury.

Imaging techniques such as ultrasonography, CT scans, and magnetic resonance imaging (MRI) are essential for diagnosing, staging, and managing renal conditions. While ultrasound is the most cost-effective option, CT and MRI offer superior resolution and higher sensitivity and specificity, especially for detecting renal cysts smaller than 1 cm in size^[10].The ultrasonography and CT scan in our patient provided valuable diagnostic information, allowing for the identification of complex cystic lesions and contributing to accurate staging and management of her condition. In our case, MRI was not performed due to the high cost, which was beyond the patient’s financial means. Additionally, genetic testing was unavailable at our hospital, limiting our ability to further characterize the underlying pathology. Both MRI and genetic testing would have provided valuable information to differentiate the diagnosis and guide more precise management strategies.

The presentation of a simple renal cyst leading to bowel obstruction as observed in our case is extremely rare, and the ideal approach to management in such scenarios remains unclear. In cases where bowel compression arises from renal cysts, conservative treatment is generally advised^[9]. If symptoms persist despite medical therapy, surgical intervention may be considered^[17]. Given the patient’s condition and the complexity of her presentation, a conservative approach was prioritized, focusing on symptom management with a diet of frequent small meals and high-calorie, nutrient-rich liquids to alleviate the obstruction and maintain nutritional status.

Patients with ACKD require close monitoring both during dialysis and after transplantation, as immunosuppressive therapy can further elevate the risk of cancer development in the native kidneys^[18]. In this case, the patient with ACKD required meticulous monitoring during dialysis due to multiple complications, including recurrent infections, electrolyte imbalances, and systemic issues associated with advanced CKD. Although she had not undergone a kidney transplant, her condition underscores the importance of careful follow-up in managing ACKD, as the progression of cystic changes and potential for malignancy remain significant concerns in such patients.

Conclusion

Unusual presentations of bowel obstruction, such as those caused by large renal cysts, should raise suspicion in patients with underlying chronic kidney disease. Early diagnosis through advanced imaging and prompt conservative management can significantly improve patient outcomes. A multidisciplinary approach involving nephrology, gastroenterology, and surgical teams ensures comprehensive care and optimal management of potential complications, improving both short- and long-term outcomes in such complex cases.

Ethical approval

Case reports are exempt from ethical approval in our institution, Tribhuvan University Institute of Medicine, Maharajgunj.

Consent

Written informed consent was obtained from the patient for publication of this manuscript and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Sources of funding

This study received no funding.

Author contributions

A.S., K.A., S.G., B.T., R.B., and V.L.: conceptualization; B.T., R.B., and V.L.: patient management; V.L., A.S., and K.A.: writing – original draft; K.A., S.G., and B.T.: writing – reviewing & editing; S.G., B.T., R.B., and V.L.: visualization and supervision.

Conflicts of interest disclosure

There are no conflicts of interest.

Research registration unique identifying number (UIN)

This is not applied in our case report.

Guarantor

Aron Shrestha.

Provenance and peer review

Not commissioned, externally peer-reviewed.

Data availability statement

The datasets used during this study will be available from the corresponding author upon reasonable request.