Long-term functional outcomes in children with Currarino syndrome.
BACKGROUND
The aim of the study was to review the degree to which the long-term outcome and ongoing morbidity in Currarino syndrome (CS) has been established.


METHODS
Analysis of previously published reports that have included long-term outcome data in CS and review of five additional patients with CS.


RESULTS
Overall, long-term outcomes of children born with CS are not well described. Malignancy has been reported in six children of approximately 300 CS patients: four children with malignancy had a recurrence after primary excision. Malignancy has also occurred in four adults. Ongoing morbidity related to constipation, faecal incontinence, neurogenic bladder, urinary incontinence and presacral abscess, and more rarely meningitis, brain metastases, developmental delay and unusual gait. Almost certainly, previous reports have under-estimated the true incidence of these problems, given the methodology and focus of these series.


CONCLUSIONS
There is paucity of information on the long-term outcomes in CS. Few authors have focused on ongoing symptoms, such that we speculate the true incidence of long-term urinary and bowel dysfunction may have been under-estimated in CS. Greater emphasis on the functional assessment of these systems during childhood may help predict the long-term outcome in CS. The most severe cases are diagnosed during infancy and childhood, and these are also the ones who are more likely to have ongoing long-term morbidity.
Yoshida
Atsushi
A
Department of Paediatric Surgery, Christchurch School of Medicine and Health Sciences, Christchurch Hospital, Private Bag 4710, Christchurch, New Zealand.
Maoate
Kiki
K
Blakelock
Russell
R
Robertson
Stephen
S
Beasley
Spencer
S
D000015:Abnormalities, Multiple / Q000235:genetics / Q000601:surgery*; D001006:Anus, Imperforate / Q000235:genetics / Q000601:surgery*; D002648:Child; D003248:Constipation / Q000209:etiology; D003251:Constriction, Pathologic / Q000209:etiology / Q000601:surgery; D003884:Dermoid Cyst / Q000473:pathology / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007231:Infant, Newborn; D007415:Intestinal Obstruction / Q000209:etiology; D008297:Male; D011379:Prognosis; D012002:Rectal Diseases / Q000235:genetics / Q000601:surgery*; D012189:Retrospective Studies; D012447:Sacrum / Q000002:abnormalities*; D013577:Syndrome; D013724:Teratoma / Q000473:pathology / Q000601:surgery
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