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Postnatal Management of Antenatally Diagnosed Patent Urachus with Bladder Prolapse

  • Choi, Hyun-Shin (Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Kim, Hae-Eun (Department of Pediatric Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Kim, Eun-Sun (Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Oh, Soo-Young (Department of Obstetrics and Gynecology, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Chang, Yun-Sil (Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Seo, Jeong-Meen (Department of Pediatric Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Park, Won-Soon (Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine)
  • Published : 2010.11.30

Abstract

A case of bladder prolapse through a patent urachus is reported in a female infant born with a large, red, tubular mass inferior to the umbilical cord. A cystic mass communicating with fetal bladder was detected by prenatal ultrasound performed at $20^{+2}$ weeks of gestation. A fetal MRI was also performed to confirm the diagnosis and to exclude associated fetal anomalies. At $40^{+4}$ weeks, the cystic mass was no longer present and a new small solid mass was noted at the fetal abdominal wall. After birth, a protruded mucosal mass inferior to the umbilical cord was noted, and catheterization confirmed communication between the protruded mass and the urinary bladder. On the second day of life, reduction of the bladder and partial resection of the urachus was performed. A voiding cystourethrogram showed good bladder capacity and no vesicoureteral reflux. The patient voided well and was discharged after 10 days. Here, we present a case of urinary bladder prolapse through a patent urachus, diagnosed by fetal sonography and this is the first case reported that was treated by simple excision without complication.

요막관은 총 배설관에서 유래된 구조물로서, 태생기에 방광의 첨부와 제대 사이를 연결해주는 관이며, 임신 8-9개월에 완전히 막히게 되어 출생시에는 정중제인대로 남게된다. 드물게 요막관이 출생 후에도 요막관 게실, 요막관 낭종, 개방성 요막관 등으로 남아 있는 경우가 있는데, 그중 가장 심한 형태가 개방성 요막관으로 이를 통해 방광이 탈출되는 경우는 매우 드문 것으로 알려져 있다. 환아는 재태연령 41주, 출생체중 3,110 g, 아프가점수 1분 1점, 5분 7점, 정상 질식 분만되었으며, 산모는 35세로 임신 기간 중 약물 복용이나 다른 병력은 없었다. 재태연령 20주 2일에 시행한 산전 초음파상 낭성의 요막관 잔여물이 발견되었으며, 태아 자기공명영상에서 방광과 연결되는 낭종이 관찰되었다. 재태연령 40주 4일에는 고형종물 양상으로 관찰되어 초기의 낭종 형태에서 변화된 양상을 보였다. 출생 후 진찰 소견상, 제대 하방에 점막으로 둘러싸인 $3{\times}4cm$의 종물이 있었으며 요도로 삽입한 도관이 종물의 개구부를 통해 나오는 것이 확인되어 종물이 요도와 연결된 천공된 상태의 방광임을 추정할 수 있었다. 생후 1일에 시행한 복부 초음파상 수신증이나 다른 요로기형은 발견되지 않았으며, 생후 2일째 방광 복원술 및 요막관제거술을 시행하였다. 생후 9일째 시행한 배설성 방광 요로 조영술에서 방광 요로 역류는 없었고, 방광 기능은 좋은 것으로 확인되었다. 생후 10일째, 정상적인 배뇨를 보여 퇴원하였고 이후 특별한 문제없이 외래 추적 관찰 중이다. 저자들은 재태연령 20주 2일에 산전 초음파에서 요막관 잔여물로 추정되었던 낭종이 출생 후 요막관을 통한 방광 탈줄증으로 확진된 후 합병증 없이 수술적으로 완치되어 국내 최초로 보고하는 바이다.

Keywords

References

  1. Lee SB, Jung CH, Kim KS, Ryu MH, Lee DJ. A clinical observation of children with urachal anomalies. J Korean Soc Pediatr Nephrol 2005;9:213-21.
  2. Cilento BG Jr, Bauer SB, Retik AB, Peters CA, Atala A. Urachal anomalies: defining the best diagnostic modality. Urology 1998; 52:120-2. https://doi.org/10.1016/S0090-4295(98)00161-7
  3. Kang EY, Lee CK, Park KH, Seo JM, Lee SK. Urachal anomalies in children. J Korean Assoc Pediatr Surg 2005;11:150-6.
  4. Cuda SP, Vanasupa BP, Sutherland RS. Nonoperative management of a patent urachus. Urology 2005;66:1320.
  5. Yeats M, Pinch L. Patent urachus with bladder eversion. J Pediatr Surg 2003;38:E12-3.
  6. Choi YJ, Kim JM, Ahn SY, Oh JT, Han SW, Lee JS. Urachal anomalies in children: a single center experience. Yonsei Med J 2006; 47:782-6. https://doi.org/10.3349/ymj.2006.47.6.782
  7. Park MS, Lee SM, Hong SW. Clinical analysis of urachal anomalies. J Korean Surg Soc 2006;70:214-7.
  8. Matsui F, Matsumoto F, Shimada K. Prenatally diagnosed patent urachus with bladder prolapse. J Pediatr Surg 2007;42:e7-10.
  9. Lugo B, McNulty J, Emil S. Bladder prolapse through a patent urachus: fetal and neonatal features. J Pediatr Surg 2006;41:e5-7.
  10. McCollum MO, Macneily AE, Blair GK. Surgical implications of urachal remnants: Presentation and management. J Pediatr Surg 2003;38:798-803. https://doi.org/10.1016/jpsu.2003.50170
  11. Ashley RA, Inman BA, Routh JC, Rohlinger AL, Husmann DA, Kramer SA. Urachal anomalies: a longitudinal study of urachal remnants in children and adults. J Urol 2007;178:1615-8. https://doi.org/10.1016/j.juro.2007.03.194
  12. Rich RH, Hardy BE, Filler RM. Surgery for anomalies of the urachus. J Pediatr Surg 1983;18:370-2. https://doi.org/10.1016/S0022-3468(83)80183-3
  13. Osawa K, Ito M, Sugiyama M, Kanamori Y, Hashizume K, Marumo G. A case of fetal vesicoallantoic cyst in the umbilical cord. Fetal Diagn Ther 2003;18:87-90. https://doi.org/10.1159/000068067
  14. Frydman M, Cohen HA, Ashkenazi A, Varsano I. Familial segregation of cervical ribs, Sprengel anomaly, preaxial polydactyly, anal atresia, and urethral obstruction: a new syndrome? Am J Med Genet 1993;45:717-20. https://doi.org/10.1002/ajmg.1320450611
  15. Grinda A, Valla JS, Dupont D, Velin P, Fast M. Umbilical evagination of the bladder in a newborn infant. Chir Pediatr 1987;28:112-4.