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Caudal duplication syndrome: 10-year experiences with a comprehensive literature review

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Abstract

Purpose

Caudal duplication syndrome (CDS) has rarely been reported. The purpose was to describe the characteristics and discuss possible pathogenesis of CDS by reviewing our experience along with a comprehensive literature review.

Methods

A total of 51 patients including 3 from our team and 48 from literature were selected in this study. General condition, clinical manifestations, type of anomalies, treatment and prognosis was analyzed and summarized.

Results

Among the 51 patients were 30 females and 21 males, and age at first clinical visit was from birth to 39 years old. Except 12 patients, most of the patients had no troubling clinical manifestation. Physical examination showed that 30 patients had 1 perineum, 21 patients had 2 completely independent perineums. Degree of duplication varied; colon-rectum tubular, bladders and urethras, vaginas in females and penis shafts and glans in males were found to be the most common type of alimentary system and urogenital system duplication in this study with 24/51, 41/51, 10/30 and 16/21 patients, respectively. Anorectal malformation was calculated: 18 had 2 ARMs, 14 had 1 normal anus and 1 ARM on the other side, 12 had a normal anus, 5 had 2 normal anus, the remaining 2 patients had only 1 ARM. Spinal cord anomalies were showed as meningomyeloceles and lipomas in 13 and 3 patients. Vertebral anomalies of bifid, dysplasias, scoliosis, and hemivertebra were noticed in 28 patients and accessory dysplasia lower limbs were found in 10 patients. Prognosis showed 39 of the 51 patients had normal function in urination and defecation.

Conclusions

CDS is an extremely rare disease with uncertain pathogenesis. Colon-rectum tubular duplication with two ARMs, duplicated bladders and urethras, double vaginas in females and penis shafts and glans in males are the most common type. Long-term prognosis is good with multidisciplinary, individualized and staged surgical procedures.

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Acknowledgements

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. The authors thank the entire staff of the Department of General Surgery, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health.

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Authors and Affiliations

  1. Department of General Surgery, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, No.56 Nanlishi St, Xicheng District, Beijing, 100045, China

    Kai Wang, Chunhui Peng, Wenbo Pang, Zengmeng Wang, Dan Zhang, Dongyang Wu & Yajun Chen

  2. Department of General Surgery, Zhujiang Hospital of Southern Medical University, Guangdong, 510282, China

    Liucheng Yang

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  1. Kai Wang
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  3. Chunhui Peng
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  6. Dan Zhang
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  7. Dongyang Wu
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Contributions

KW and YC: designed the study, drafted the initial manuscript, and reviewed and revised the manuscript. LY, CP, WP, ZW, DZ, DW: coordinated and supervised data collection, and critically reviewed the manuscript for important content. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

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Correspondence to Yajun Chen.

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Research involving human participants and/or animals

All procedures performed in studies involving human participants were in accordance with the ethical standards and approved by the Medical Ethics Committee of Beijing Children’s Hospital. This article does not contain any studies with animals performed by any of the authors.

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Wang, K., Yang, L., Peng, C. et al. Caudal duplication syndrome: 10-year experiences with a comprehensive literature review. Pediatr Surg Int 38, 1283–1289 (2022). https://doi.org/10.1007/s00383-022-05159-2

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