Advances in Pediatric Surgery

"Sacral agenesis"

Case Report

[English]

Inguinal Ectopic Scrotum, Anorectal Malformation with Sacral Agenesis and Limb Defects: An Unusual Presentation: Monika Bawa, Saurabh Garge, Virender Sekhon, KLN Rao; J Korean Assoc Pediatr Surg 2015;21(2):32-34. Published online December 22, 2015; DOI: https://doi.org/10.13029/jkaps.2015.21.2.32

Abstract
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A case of congenital ectopic scrotum in neonatal period is described. The ectopic scrotum was located in the right inguinal area and the left hemiscrotum was found in normal location and each hemi-scrotum contained their testis. The neonate also had imperforate anus as low anorectal malformation with spinal abnormalities (hemi-sacrum and hemi-pelvis), right knee flexion contracture and right club foot. The embryological explanation in the literature of ectopic scrotum and its associated anomalies is discussed.

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Original Article

[English]

Clinical Characteristics and Treatment of Currarino Syndrome: A Single Institutional Experience: Kim, Hyun Jeong , Ho, In Geol , Ihn, Kyong , Han, Seok Joo , Oh, Jung-Tak; Adv Pediatr Surg 2020;26(2):46-53.; DOI: https://doi.org/10.13029/aps.2020.26.2.46

Abstract
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Purpose
Currarino syndrome is a rare disease, and understanding its clinical characteristics is important because it involves complex anomalies and it requires a multidisciplinary approach for treatment. However, the accumulation of treatment experiences is challenging, and studies on this disease remain insufficient. Our study aimed to review the clinical characteristics and treatment of Currarino syndrome at our institution.
Methods
The medical records of patients diagnosed as Currarino triad or Currarino syndrome at the authors institution from 1997 to 2018 were retrospectively reviewed. Thirteen patients were included in this study.
Results
No significant difference in disease prevalence was observed in terms of sex (male:female, 7:6), and the median age at diagnosis was 7 months (1 day to 35 years). Currarino syndrome was diagnosed during the neonatal period in only one patient, whereas its diagnosis was delayed in the other patients. The most common initial symptom or diagnosis was anorectal malformation (ARM) followed by constipation and sacrococcygeal mass. All patients underwent simple abdominal X-ray and magnetic resonance imaging for the diagnosis of this disease. The most common sacral anomaly was partial sacral agenesis (type III, 62%) followed by hemisacrum (type IV, 38%). The most common presacral mass was mature teratoma. No pathologic malignant features were observed. In ARM, nine patients had anal stenosis, and posterior sagittal anorectoplasty was the most common operative method for ARM. Twelve patients were followed up beyond the age of three, and the median follow-up age was 8.1 years (range: 3.1–30.0 years). Among the 12 patients, 4 patients did not show symptoms of functional abnormality, whereas 8 patients showed constipation, fecal incontinence, urinary dysfunction, or gait disturbance.
Conclusion
The diagnosis of Currarino syndrome could be delayed when sacral bony anomaly and anorectal stenosis are not given attention. A careful follow up is essential because poor long-term functional outcomes are common.