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Current characteristics and management of congenital esophageal stenosis: 40 consecutive cases from a multicenter study in the Kyushu area of Japan

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Abstract

Purpose

Congenital esophageal stenosis (CES) is rare, and the available clinical data are limited. We explored the current diagnosis, treatment and outcomes of CES.

Methods

A questionnaire survey was performed using medical records at pediatric surgical centers in the Kyushu area.

Results

Over 10 years, 40 patients (24 males) had CES. The incidence of associated anomalies was 52.5% (21/40), and that of esophageal atresia was 20.0% (8/40). The mean age at the diagnosis was 12.0 months (range, 1 day–8.8 years). Seven (17.5%) patients were diagnosed in the neonatal period. Ten (25.0%) developed CES due to tracheobronchial remnants, 27 (67.5%) due to fibromuscular stenosis (FMS) and 1 (2.5%) due to membranous stenosis + FMS. Thirty-six (90.0%) were treated by balloon dilatation (mean, 3 times; range, 1-20). Perforation at dilatation occurred in 7 (17.5%) patients, and all were diagnosed with FMS. Eighteen (45.0%) patients underwent radical operation (3 primary, 15 secondary to dilatation).

Conclusions

Our study clarified the characteristics and outcomes of CES, including neonatal diagnoses. CES occurred in 1 in every 33,000 births in the Kyushu area. Careful attention should be paid, even in cases of dilatation for FMS. CES requires long-term follow-up for symptom persistence after adequate and repeated treatment.

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Acknowledgements

The authors thank Brian Quinn for editing the manuscript. The authors wish to thank all of the doctors in the 21 pediatric surgery departments at major universities and hospitals throughout the Kyushu area of Japan for replying to the questionnaires (Appendix).

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Correspondence to Satoshi Ieiri.

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Appendix

Appendix

Fukuoka Children’s Hospital

Fukuoka

Fukuoka University

Fukuoka

Japan Community Health care Organization Kyushu Hospital

Kitakyushu

Japanese Red Cross Kumamoto Hospital

Kumamoto

Kagoshima City Hospital

Kagoshima

Kagoshima University Graduate School of Medical and Dental Sciences

Kagoshima

Kitakyushu Municipal Medical Center

Kitakyushu

Kumamoto City Hospital

Kumamoto

Kumamoto University Hospital

Kumamoto

Kurume University School of Medicine

Kurume

Kyushu Medical Center

Fukuoka

Kyushu University Hospital

Fukuoka

Miyazaki University Hospital

Miyazaki

Nagasaki University Hospital

Nagasaki

Oita Children’s Hospital

Oita

Oita Prefectural Hospital

Oita

Oita University Hospital

Oita

Okinawa Prefectural Nanbu Medical Center and Children’s Hospital

Naha

Shimonoseki City Central Hospital

Shimonoseki

University of Occupational and Environmental Health

Kitakyushu

University of the Ryukyus

Naha

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Suzuhigashi, M., Kaji, T., Noguchi, H. et al. Current characteristics and management of congenital esophageal stenosis: 40 consecutive cases from a multicenter study in the Kyushu area of Japan. Pediatr Surg Int 33, 1035–1040 (2017). https://doi.org/10.1007/s00383-017-4133-0

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  • DOI: https://doi.org/10.1007/s00383-017-4133-0

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