Case Report: Recurrent Complicated Urinary Tract Infection in Prune Belly Syndrome With Solitary Functioning Kidney

ABSTRACT

Recurrent UTIs in prune belly Syndrome with a markedly reduced renal function pose significant renal injury risk. Normal creatinine can mask underlying damage. Long‐term, multidisciplinary follow‐up is crucial, especially in low‐resource settings, to preserve renal function and improve patient outcomes.

1. Introduction

Prune belly syndrome (PBS), also known as Eagle‐Barrett syndrome, is a rare congenital condition that affects 3.8 out of every 100,000 live male births [1]. It is defined by a triad of absent or deficient abdominal wall musculature, cryptorchidism, and urinary system abnormalities [2]. It was first identified in 1901 by William Osler [3]. It nearly affects only boys and is hence very rare in girls [1]. Although the exact etiology of PBS is unknown, variable characteristics of the condition, such as familial case reports and a higher occurrence in men, point to a sex‐linked genetic component, but some have proposed an autosomal recessive mode of transmission [4, 5]. Various degrees of kidney dysplasia; hydronephrosis, dilated and convoluted ureters, larger bladders, occasional diverticula near the vesicoureteric junction, and urethral blockage are examples of urinary system malformations [6, 7]. The PBS is frequently associated with a variety of extrarenal abnormalities. These include cardiac, gastrointestinal, respiratory, and skeletal abnormalities [8, 9]. In this report, we present a case of Prune belly syndrome with a recurrent complicated urinary tract infection and a severely atrophic right kidney with a presumed markedly reduced renal function.

2. Case Presentation

An 8‐year‐old male, with a known diagnosis of prune belly Syndrome, presented to our facility on April 14, 2026, with a 3‐day history of hematuria. The patient's mother reported the urine initially appeared brown, progressively darkening to a dark red hue, present throughout the urinary stream. This was accompanied by urinary urgency, frothy urine, and concurrent left flank and suprapubic pain. There was no reported new‐onset urinary incontinence or nocturia.

Three days prior to presentation, the patient developed a high‐grade fever that lasted for 1 day, without associated rigors, sweating, or convulsions. He received empirical antimalarial therapy, after which the fever subsided. A subsequent peripheral blood film for malaria was reported as negative. The patient also reported recent weight loss and poor appetite. A review of other systems was otherwise unremarkable.

The patient had a history of a similar episode 6 months prior, characterized by hematuria and dysuria, for which he was admitted for 4 days and treated with intravenous antibiotics (Figure 1: course of care). He was referred to a urologist at that time; however, he did not attend the referral and was subsequently lost to follow‐up. He had no history of previous surgical interventions or blood transfusions. In addition to prune belly syndrome, he had been previously diagnosed with severe right renal atrophy.

FIGURE 1.

Clinical timeline: Episode of care for prune belly syndrome case.

He was not on any long‐term medications and had no known drug allergies. There was no family history of similar conditions, chronic illnesses, or congenital disorders.

The patient was born at term via home vaginal delivery conducted by a trained midwife. He cried immediately after birth and passed urine and meconium within the first 24 h of life. An abdominal wall abnormality was noted at birth but was initially attributed to lax abdominal muscles and was not further investigated. At 12 days of age, he was evaluated by a physician, and an abdominal ultrasound was requested; however, he was subsequently lost to follow‐up. There was no history of antenatal care, and the mother did not receive supplements during pregnancy. She denied any history of febrile illness, rash, or exposure to radiation or teratogens during pregnancy. Additionally, the mother had no known history of diabetes. The postnatal period was otherwise unremarkable, with no history of neonatal jaundice, cyanosis, or convulsions.

2.1. Clinical Findings

On examination, the patient appeared ill and mildly pale but was not jaundiced or cyanosed. He was not in respiratory distress. Vital signs revealed a pulse rate of 102 beats per minute, a respiratory rate of 34 breaths per minute, an axillary temperature of 37.5°C, and oxygen saturation of 98% on room air. His weight was 17.3 kg.

Abdominal examination showed an abnormal contour with right flank fullness (Figure 2). The abdomen was soft, with tenderness over the left flank and suprapubic region. There was no organomegaly. Respiratory examination revealed clear breath sounds bilaterally. Cardiovascular examination showed normal first and second heart sounds with no murmurs or added sounds.

FIGURE 2.

Clinical presentation of prune belly syndrome demonstrating characteristic abdominal wall deficiency. Photograph of an 8‐year‐old male showing marked abdominal wall laxity with prominent wrinkling and redundancy of the skin, creating the distinctive prune‐like appearance.

2.2. Differential Diagnosis

The diagnosis was presumed recurrent complicated UTI (pyelitis) in a patient with PBS and a severely atrophic right kidney with presumed markedly reduced renal function. The significant hematuria and proteinuria, in conjunction with the frothy urine, raised suspicion for potential glomerular pathology in addition to suspected pyelitis/UTI but unconfirmed due to lack of biopsy and advanced investigations.

2.3. Diagnostic Assessment

2.3.1. Laboratory Tests

Laboratory investigations revealed a hemoglobin level of 7.6 g/dL (reference range: 11.5–15.5 g/dL) and a total white blood cell count of 7.6 × 10⁹/L (reference range: 4.5–13.5 × 10⁹/L). Peripheral blood film for malaria was negative. Urinalysis showed red‐colored urine with (++++) proteinuria (which may be false positive due to the concomitant hematuria), numerous red blood cells (uncountable), and 10–20 pus cells per high‐power field. Calcium oxalate crystals were also noted. Renal function tests showed a urea level of 47 mg/dL (reference range: 7–20 mg/dL) and serum creatinine of 0.6 mg/dL (reference range: 0.3–0.7 mg/dL). Quantitative proteinuria assessment and repeat urine analysis after resolution would be needed before attributing this to hyperfiltration injury or chronic renal damage.

2.3.2. Ultrasound Findings

An ultrasound demonstrated a normal‐positioned left kidney with compensatory hypertrophy and increased echogenicity, with preserved corticomedullary differentiation. Mild dilatation of the pelvicalyceal system with internal echoes suggestive of debris was observed, consistent with acute pyelitis and renal parenchymal involvement (Figure 3).

FIGURE 3.

Abdominal ultrasound demonstrating bilateral renal Involvement in prune belly syndrome. Upper panels show the compensated left kidney (11.0 cm) with increased echogenicity and mild pelvicalyceal dilatation consistent with chronic parenchymal disease. Lower left panel demonstrates the severely atrophied right kidney (10.0 cm) with cortical thinning and loss of corticomedullary differentiation. Lower right panel shows the empty urinary bladder with indwelling Foley catheter. The imaging findings are consistent with prune belly syndrome.

The right kidney was severely atrophied, showing marked cortical thinning and loss of corticomedullary differentiation, consistent with presumed markedly reduced function and chronic obstructive uropathy (Figure 3). The urinary bladder was empty with an indwelling catheter in situ. Additional findings included features consistent with prune belly syndrome, including anterior abdominal wall muscle deficiency and bilateral undescended testes or possible agenesis. No abnormalities were detected in the liver, spleen, pancreas, or biliary system, and no ascites or lymphadenopathy was noted. The diagnosis of a markedly reduced renal function was inferred from these ultrasound findings, as advanced functional imaging (e.g., DMSA scan) was not available.

2.4. Initial Management

Upon admission, the patient was managed with intravenous ceftriaxone (50 mg/kg once daily) and antipyretics as needed. He showed clinical improvement within 48 h, with resolution of fever and reduction in hematuria. The patient's hemoglobin concentration was 7.6 g/dL. Although he was mildly pale, he was hemodynamically stable and had no documented signs of active bleeding, tissue hypoxia, heart failure, respiratory compromise, or other symptoms attributable to acute anemia. Therefore, immediate blood transfusion was not performed. The decision was based on the patient's clinical status, in the absence of an urgent transfusion indication. Instead, iron supplementation and nutritional support were initiated. Nephrology consultation was sought, and a plan for long‐term management was discussed, emphasizing infection control and renal preservation. However, due to the limited resources, specific nephrology interventions such as renal biopsy for definitive glomerular pathology diagnosis or advanced imaging for detailed renal function assessment were not feasible. The patient was discharged after 5 days with oral antibiotic (cefixime 8 mg/kg/day PO once daily for 6 days with the final decision regarding the prophylactic antibiotic choice and duration to be made by the nephrologist) and a planned referral to a urologist and a nephrologist for comprehensive evaluation and long‐term management to preserve the renal function. Unfortunately, despite counseling, the patient was again lost to follow‐up.

3. Discussion

This case presented a patient of PBS with severely atrophic right kidney with presumed markedly reduced renal function, significant proteinuria despite normal creatinine and longitudinal description in a constrained diagnostic setting.

Our case highlights a severe spectrum of urological involvement. The right kidney was markedly shrunken and atrophic, with loss of corticomedullary differentiation, indicative of chronic damage. This significantly elevates the patient's risk of developing chronic kidney disease compared to individuals with two normally functioning kidneys, making the preservation of the remaining renal function paramount. Despite a normal serum creatinine level (0.6 mg/dL), evidence of ongoing renal injury was present, notably heavy proteinuria on urinalysis. This finding suggests potential hyperfiltration injury but quantitative proteinuria assessment and repeat urine analysis after resolution would be needed. The significant hematuria and proteinuria also raised concerns for a possible underlying glomerular pathology, which could not be definitively investigated due to resource limitations. Additionally renal function reserve and CKD stage could not be determined from serum creatinine alone.

A case series from Sudan reported ureterohydronephrosis in approximately 80% of PBS patients [6]. These abnormalities foster urinary stasis, vesicoureteral reflux, and incomplete bladder emptying, significantly increasing the risk of recurrent UTIs, which can ultimately lead to renal scarring and progressive renal insufficiency.

The presence of recurrent urinary tract infections in this patient further increases the risk of renal deterioration. These infections are likely related to underlying structural and functional abnormalities of the urinary tract. This highlights the importance of long‐term strategies aimed at reducing infection risk, which may include close urological follow‐up and consideration of preventive measures based on individual assessment. In addition, surgical planning in such cases should carefully consider the presence of a severely impaired renal function, with emphasis on preservation of renal function whenever possible. Management of PBS remains complex and lacks a standardized approach. A multidisciplinary strategy is considered the most appropriate, with the urologist playing a central role in coordinating care. Patients often require regular follow‐up with multiple specialties, including nephrology, pulmonology, gastroenterology, and orthopedics, to optimize overall outcomes, monitor growth, and prevent complications. In low‐resource settings, loss to follow‐up represents a major challenge in the management of PBS. This is evident in our case, where the patient was previously referred but did not attend follow‐up. Similar findings have been reported in a study from Uganda, where up to 46% of patients were lost to follow‐up, largely due to financial constraints and limited access to healthcare services [10], similar challenges were reported in Somalia [11]. This underscores the importance of effective counseling of caregivers regarding the chronic nature of the disease, the need for continuous monitoring, and the potential for progression to renal failure requiring dialysis or transplantation.

This case report provides a comprehensive description of a rare presentation of PBS with longitudinal follow‐up from birth to 8 years of age, emphasizing the challenges in a low‐resource context. However, several limitations must be acknowledged. First, the absence of a voiding cystourethrogram (VCUG) precluded a definitive assessment of vesicoureteral reflux, which is reported in up to 75% of PBS cases [12], thereby limiting the evaluation of the need for surgical intervention. Second, a urine culture was not obtained prior to the initiation of empiric antibiotic therapy, preventing the identification of the causative organism and hindering targeted treatment. Third, genetic testing, which could offer insights into associated abnormalities, was not performed due to unavailability. Finally, the persistent challenge of loss to follow‐up significantly limits the ability to ensure long‐term monitoring and comprehensive outcome assessment, a common barrier in our setting.

4. Conclusion

The presentation of recurrent complicated UTIs in a patient with prune belly Syndrome and a severely atrophic with presumed markedly reduced renal function underscores the severe vulnerability of the renal system to progressive damage. This case highlights that normal serum creatinine levels in pediatric patients can be misleading, masking significant underlying renal injury. Effective management necessitates timely urological intervention, rigorous infection control, and, crucially, strategies to mitigate loss to follow‐up, which remains a substantial barrier to care in resource‐limited environments. Comprehensive counseling of caregivers on the chronic nature of PBS and the absolute necessity of consistent, multidisciplinary long‐term follow‐up is paramount to preserving renal function and improving patient outcomes in such challenging settings.

Author Contributions

Mohammed Elhadi Badawi Mahjoub: writing – review and editing, writing – original draft, conceptualization, project administration, data curation, methodology, visualization. Shafee S. Almahi: writing – review and editing, writing – original draft, conceptualization, methodology, project administration. Mohamed Jadain: writing – original draft, writing – review and editing, conceptualization, data curation, project administration.

Funding

The authors have nothing to report.

Ethics Statement

The study was approved by the Ethical Committee of Kosti Teaching Hospital. Written consent for publication and the image was taken from the patient's guardian.

Conflicts of Interest

The authors declare no conflicts of interest.

Data Availability Statement

The data that support the findings of this study are available from the corresponding author upon reasonable request.