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Abstract

Gastroenterology Medicine & Research

Currarino Syndrome: Autosomal Dominant Sacral Agenesis Application of Sacral Nerve Stimulation in Currarino Syndrome

Submission: February 20, 2020; Published: March 10, 2020

DOI: 10.31031/GMR.2020.04.000592

ISSN 2637-7632
Volume4 Issue4

Abstract

Introduction: Currarino Triad is a complete form of Currarino Syndrome which consists of sacral anomaly, anorectal malformations, and a presacral mass. Individual case reports and small case series regarding the diagnosis and treatment have been described in the literature. Here we present a case of a young woman with Currarino Syndrome, managed with Sacral Nerve Stimulation (SNS).

Case report: A 32-year-old female presented with Currarino triad including anal atresia, dysplasia of the sacrum, and presacral teratoma with tethered cord. The patient is of Polish decent and for many years received treatment for chronic constipation in Poland. She underwent multiple surgeries to correct the anal atresia, first when she was thirteen years old with unsatisfactory results further requiring diamond flap anoplasty after her arrival to the United States at the age of twenty-nine. She has also had chronic, refractory constipation despite aggressive medical treatment. A sacral nerve stimulator was implanted to treat her refractory constipation with promising results.

Discussion: Our experience with SNS treatment for this patient with Currarino Syndrome provides encouraging results for the use of SNS in the treatment of constipation in patients with previously distorted anatomy. Given the success of SNS in adults with other congenital anal malformations, and the success with this patient, SNS may have a promising future for improving bowel dysfunction and quality of life in Currarino Syndrome patients.

Conclusion: Sacral nerve stimulation can be a successful surgical approach in managing chronic, refractory constipation, demonstrating possible future applicability for Currarino Syndrome patients struggling with chronic constipation. Further large cohort studies are necessary to evaluate the success rate of sacral nerve stimulation in patients with chronic constipation.

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