“This bicycle gives me a headache”, a congenital anomaly

Hendt P Versteegh,Erik J Van Lindert,Ivo De Blaauw,Wout Fj Feitz,Carlo Marcelis

doi:10.1186/1756-0500-6-412

Abstract

BackroundThe combination of a presacral mass, a sacral bone deformity, and an anorectal malformation are also known as the Currarino triad or Currarino syndrome. The syndrome is associated with a very high rate of severe and intractable constipation and urinary incontinence. However, it can also result in less common complaints and symptoms. Although the syndrome is known since 1981 and the involved genes are clarified to a great extent, the diagnosis may be delayed or missed if unrecognized.Case presentationA 24-year old female presented with periodical headaches. She was born with an imperforate anus, absent rectum and colon, double bladder, and sacral defect. Soon after birth she underwent several surgical procedures for anorectal and bladder reconstructions. The patient now came to her pediatric urologist for urinary incontinence and mentioned severe headaches on the side, particularly when riding a bike. Finally, she solved her headache problem by stopping to ride her bicycle.On physical examination no abnormalities were found except the ileostomy that was present ever since soon after birth and her urinary incontinence. Blood tests showed no abnormalities. Additional MRI showed a large and previously not known anterior meningocele at the level of the sacrum. Surgical treatment consisted of closure of the dura by posterior approach.ConclusionIn this case report we describe the late discovery with an atypical presentation of an anterior meningocele in a young adult with urinary incontinence, a sacral defect, an anorectal malformation and headaches during bicycle riding. After surgical treatment of our patient the meningocele regressed. Three months after successful surgery she had no complaints and was able to ride a bike again.

Highlights

The Currarino syndrome is a congenital syndrome with a classical triad consisting of an anorectal malformation, a sacral defect and a presacral mass or tumor [1]
Surgical treatment consisted of closure of the dura by posterior approach. In this case report we describe the late discovery with an atypical presentation of an anterior meningocele in a young adult with urinary incontinence, a sacral defect, an anorectal malformation and headaches during bicycle riding
The variable expression makes recognition often difficult, in adolescents and adults with little or atypical symptoms [3,4]. With this case report we aim to demonstrate such an atypical presentation and discuss the etiology, management and outcome of patients with the Currarino syndrome

Summary

Conclusion

This case report illustrates the presentation of an anterior meningocele as part of the Currarino syndrome. The Currarino syndrome can present itself partially and often remains unknown for several years. Consent Written informed consent was obtained from the patient for publication of this case report and any accompanying images. Data acquisition and analysis was conducted by HPV and IdB. HPV, WFJF, EjvL, CM and IdB were responsible for data interpretation. Author details 1Department of Pediatric Surgery, Erasmus MC Sophia Children’s Hospital, Rotterdam, the Netherlands.

Background

Findings