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Effects Of Spinal Cord Detethering On Children With Currarino Syndrome
Nora G. Lee, Joseph G. Borer, Carlos R. Estrada, Shahram Khoshbin, Stuart B. Bauer
Children's Hospital Boston, Boston, MA
Background
Currarino Syndrome (CS) is an inherited disorder involving a triad: anorectal anomalies, sacro-coccygeal defect and presacral mass. Only a few case reports and series discussing this rare condition exist; none report its effects on the genitourinary tract. We present the urologic issues in CS patients and determine how spinal cord untethering affects urinary tract function.
Methods
We retrospectively reviewed 13 patients diagnosed with CS. We evaluated patients' urinary signs/symptoms and urodynamic (UDS) findings before and after cord untethering.
Results
All 13 with CS having a sacral defect and presacral mass were diagnosed between birth and 6 years (Table1). 92% had a tethered spinal cord that was surgically detethered between 8 months to 6 years (average 3 years).
Four had recurrent urinary tract infections, 2 of whom had bilateral vesicoureteral reflux and both resolved spontaneously. Two had mild unilateral hydronephrosis without reflux. Three others with radiologic imaging were normal.
Eleven underwent comprehensive UDS. Three of four with pre- and post-surgery UDS showed improvement (Table1). Six had only post-surgery UDS; 5 being abnormal with small capacity, poor compliance, detrusor overactivity (DO), sphincter dyssynergy or high voiding pressures. One infant had no spinal surgery and normal UDS.
Conclusions
CS is a rare disorder with few published reports regarding long-term implications. Although UDS parameters improved after surgery, all toilet-trained patients continued to have ongoing voiding issues.
Table 1. | | | Characteristics | N=13 | | Female to male ratio | 9:4 | | Familial to sporadic ratio | 8:5 | Presacral mass
Myelomeningocele
Teratoma
Lipoma | 13
5
5
3 | Full triad (including anorectal abnormalities)
Anal stenosis
Imperforate anus
Anal duplication | 7
4
2
1 | | Tethered spinal cord | 12 | Surgical history
Presacral mass resection
Cord detethering | -
12
12 | Voiding complaints in toilet trained patients
Frequency
Urgency
Urinary incontinence
Noctural bedwetting
Recurrent urinary tract infection
Non-toilet trained patients | 10
3
5
5
3
4
3 | UDS evaluation
Pre- > post-surgery UDS
*Normal > normal
*Dyssynergy > synergy more than dyssynergy
*Small capacity, DO, dyssynergy > Small capacity, multiple DO with dyssynergy; later normal capacity, no DO with synergy
*DO > no DO
Post-surgery UDS
No surgery UDS | 11
4
-
-
-
-
-
6
1 |
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