Please use this identifier to cite or link to this item: https://scholarhub.balamand.edu.lb/handle/uob/5693
Title: Atypical presentation of currarino syndrome: A case report
Authors: Hage, Paul
Kseib, Cedric
Adem, Carmen
Chouairy, Camil J
Matta, Reva
Affiliations: Faculty of Medicine 
Keywords: Case report
Currarino syndrome
Dysraphism
Malignancy
Neurosurgery
Pediatric surgery
Issue Date: 2019
Publisher: National Library of Medicine
Part of: International Journal of Surgery Case Reports
Volume: 57
Start page: 102
End page: 105
Abstract: 
Introduction
Currarino syndrome is a rare congenital disorder characterized by a triad of anorectal malformation, a sacral bone defect, and a presacral mass. It results of an abnormal separation of the ectoderm from the endoderm caused by HLXB9 mutation in chromosome 7q36 in 50% of cases. The disorder is mostly hereditary as it can also be sporadic with a variable expression spectrum.

Presentation of Case
The case of a previously healthy 3-month-old girl with abdominal distension, post-prandial vomiting, obstipation, and anuria of 5 days’ history is presented in this article. Abdomino-pelvic magnetic resonance imaging (MRI) showed a large cystic multilobulated mass in the sacrococcygeal region with a dural communication evident of an anterior sacral meningocele. 1 year later, the child came back with constipation and was found to a have a malignant mixed germ cell tumor in the presacral area, a very rare presentation in Currarino syndrome.

Discussion
In a child presenting with at least one of the features of Currarino syndrome’s triad, a diagnosis should be suspected. After reviewing the literature, the syndrome is usually missed and hence is under diagnosed. MRI is the best imaging modality for diagnostics and follow-up for any mass, benign or malignant, can bring life saving measures. Most masses are benign but can undergo malignant transformation even after resection. De novo malignancy is very rare and is described in our case.

Conclusion
Physicians treating patients with spinal dysraphism should suspect a diagnosis of Currarino syndrome by follow up imaging for any new benign or malignant growth.
URI: https://scholarhub.balamand.edu.lb/handle/uob/5693
ISSN: 2210-2612
DOI: 10.1016/j.ijscr.2019.02.047
Open URL: Link to full text
Type: Journal Article
Appears in Collections:Faculty of Medicine

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