Case Quiz (January 2021)

A 2-year-6-month-old girl was referred because of severe bilateral hydronephrosis, acute obstructive nephropathy, sepsis, and recurrent urinary tract infection (UTI). There was no family history of renal diseases.

Three days after her admission, she developed generalized edema and abdominal pain with anuria. Her body weight increased from 10.3 to 11.8 kg; blood pressure abruptly increased to 135/95 mmHg. Laboratory data were as follows: BUN 74 mg/dl; serum creatinine, 5.8 mg/dl; and urine culture revealed Enterococcus species.

To control uremia with fluid retention, intermittent HD was initiated. Abdominal U/S and MRI demonstrated severe bilateral hydroureteronephrosis and no stricture of distal urethra. The spine and spinal cord were normal as seen on MRI. Ampicillin and ceftriaxone were administered for 2 weeks; BUN and creatinine decreased to 19 and 1.2 mg/dl, respectively, on the 7th hospital day, and HD was withdrawn. VCUG revealed chronic non-anatomical bladder outlet obstruction and severe bilateral reflux (right, grade 4–5; left, grade 5) with hydroureteronephrosis. Clean intermittent catheterization (CIC) four times a day was performed; however, on the 20th hospital day, she developed repeated UTI and was transferred to our hospital.

On admission to our hospital, she had a high fever and post-obstructive diuresis. Her height was 82.8 cm, body weight was 9.8 kg, and blood pressure was 114/72 mmHg. No neurological abnormality was detected by physical examination. Laboratory data showed: Hb 7.4 g/dl, TLC 18,200/mm3, Na 137 meq/L, K 4.1 meq/L, BUN 70 mg/dl and creatinine 1.7 mg/dl. Urine culture revealed Klebsiella pneumoniae. Meropenem was administered intravenously; an oral antimuscarinic drug and CIC seven times a day were started at the same time.

Diagnostic test was done

Case Answer (January 2021)

Hinman syndrome (detrusor-sphincter dyssenergia) is typically seen in older children with dysfunctional voiding and characterized by urodynamic evidence of an involuntary functional bladder outlet obstruction at the level of the external sphincter with no demonstrable neurological diseases.

It may result in irreversible renal damage and may progress to chronic renal insufficiency. Taking account of the possibility of this condition in any child who present UTI appears to be necessary, since early recognition and proper management of this syndrome may prevent serious complications.

The main lines of management include CIC, bladder toilet training with biofeedback mechanism, and psychosocial therapies for the parents. If renal function deteriorates further, surgical therapy, namely augmentation cystoplasty, may be effective in preventing or alleviating progression to end-stage renal disease.