Attention to detail: not simple bed wetting—Hinman–Allen syndrome

Excerpt

A 15-year-old autistic female with primary nocturnal enuresis and secondary daytime incontinence without urgency, presented with UTI. Chemistries revealed: BUN 203 mg/dL, and creatinine 15.7 mg/dL. Hemodialysis (HD) was initiated. Urine specific gravity (SG) ranged 1.005–1.012. Sonography (RBUS) revealed severe bilateral hydroureteronephrosis and a large trabeculated bladder. Voiding cystourethrography (VCUG) revealed grade I vesicoureteral reflux (VUR) on the right and a trabeculated bladder with diverticula. MRI spine was normal. Foley bladder decompression improved creatinine (5.7 mg/dL), BUN (58 mg/dL) and bilateral hydroureteronephrosis (Fig. 1a). Video urodynamic study (VUDS) demonstrated a compliant bladder without uninhibited contractions but decreased filling sensation. Voiding was associated with prominent valsalva activity in the abdominal pressure lead and substantial electromyography (EMG) activity when patient emptied a minimal volume (Fig. 1b). Fluoroscopy captured an open bladder neck with beaking of the urethra at a closed external urinary sphincter (Fig. 1b) consistent with detrusor-external sphincter dyssynergia (DSD), a hallmark of Hinman–Allen syndrome (HS). Patient is currently on chronic HD and underwent appendicovesicostomy and reduction cystoplasty awaiting a kidney transplant.

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