Pyonephrosis and urosepsis in a 41-year old patient with spina bifida: Case report of a preventable death

Abstract

Background: Urological complications are the major cause of ill health in patients with spina bifida. Urinary sepsis accounted for the majority of admissions in patients with spina bifida. As the patient grows older, changes occur in the adult bladder, leading to increases in storage pressure and consequent risk of deterioration of renal function, which may occur insidiously.

Case presentation: A 34-year-old male spinal bifida patient had been managing neuropathic bladder by penile sheath. Intravenous urography revealed normal kidneys. This patient was advised intermittent catheterisations. But self-catheterisation was not possible because of long, overhanging prepuce and marked spinal curvature. This patient developed repeated urine infections. Five years later, ultrasound examination of urinary tract revealed hydronephrotic right kidney with echogenic debris within the collecting system. There was no evidence of dilatation of the ureter near the vesicoureteric junction. The left kidney appeared normal. There was no evidence of calculus disease seen in either kidney. Indwelling urethral catheter drainage was established.Two years later, MAG-3 renogram revealed normal uptake and excretion by left kidney. The right kidney showed little functioning tissue. Following a routine change of urethral catheter this patient became unwell. Ultrasound examination revealed hydronephrotic right kidney containing thick hyper-echoic internal septations and debris in the right renal pelvis suspicious of pyonephrosis. Under both ultrasound and fluoroscopic guidance, an 8 French pig tail catheter was inserted into the right renal collecting system. 150 ml of turbid urine was aspirated immediately. This patient developed large left pleural effusion, collapse/consolidation of the left lower lobe, a large fluid collection in the abdomen extending into the pelvis and expired twenty days later because of sepsis and respiratory failure.

Conclusion: Although penile sheath drainage may be convenient for a spina bifida patient and the carers, hydronephrosis can occur insidiously. With recurrent urine infections, hydronephrotic kidney can become pyonephrosis, which is life-threatening. Therefore, every effort should be made to carry out intermittent catheterisations along with antimuscarinic drug therapy.

Figure 1

Intravenous urography performed when the patient was 31 years old: no radio opaque calculi, normal kidneys and ureters; trabeculated, small capacity urinary bladder.

Figure 2

Ultrasound examination of urinary tract, performed when the patient was 39 years old: the right kidney was hydronephrotic with dilated renal pelvis. There was echogenic debris within the right collecting system.

Figure 3

Ultrasound scan of urinary tract repeated ten days later after the patient had received antibiotic: there was less debris within the hydronephrotic right kidney than at the previous scan. There was however still significant hydronephrosis with antero-posterior diameter of right renal pelvis being 5.7 cm.

Figure 4

Ultrasound examination of urinary tract performed when the patient was 41 years old: moderate hydronephrosis of right kidney was seen. Right renal pelvis was grossly dilated with thick internal echogenic septations and echogenic debris suggestive of pyonephrosis.

Figure 5

Computed Tomography of chest, abdomen and pelvis, performed after percutaneous drainage of right pyonephrosis: a large fluid collection was seen in the abdomen extending into the pelvis with enhancing walls. Fluid was also noted around the spleen, in the paracolic gutters and in the root of the mesentery.