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Right esophageal lung in a preterm child with VACTERL association and Mayer-Rokitansky-Küster-Hauser syndrome

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Abstract

We report on a preterm girl (birth weight 1,200 g) with a right esophageal lung in esophageal atresia type VIag (according to the extended classification of Kluth). Additionally, the child suffered from an atrioseptal defect, a dextrocardia with a left descending aorta, a duodenal atresia, a high type of anal atresia (VACTERL association), agenesis of the left kidney, and agenesis of the vagina, uterus, and ovarian tubes (Mayer-Rokitansky-Küster-Hauser syndrome or incomplete MURCS association). The child was treated with an emergency gastrostomy because of increasing abdominal dilatation. Thereafter, the parents refused further surgical treatment, and the child was maintained on basic therapy. After an uneventful period of 4 weeks, the child died of an acute massive aspiration. This case shows that sufficient spontaneous ventilation is possible in esophageal lung as long as a gastrostomy is kept on suction to prevent overinflation of the affected lung and the stomach. Ethical aspects have to be considered when treatment is planned in cases of prematurity and associated malformations when a chance of good survival is rather limited. The stepwise approach as proposed in the present case appears to be the only possible therapeutic regimen that can be offered in this complicated condition.

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References

  1. Bleicher M, Melmed A, Bogaerts X (1983) Vater association and unrecognized bronchopulmonary foregut malformation complicating anesthesia. Mt Sinai J Med 50:435–438

    CAS  PubMed  Google Scholar 

  2. Braun-Quentin C, Billes C, Bowing B, Kotzot D (1996) MURCS association: case report and review. J Med Genet 33:618–620

    CAS  PubMed  Google Scholar 

  3. Chiba T, Ohi R, Hayashi Y, Uchida T (1989) Bronchopulmonary foregut malformation in 3 infants—with special references to cases in childhood. Z Kinderchir 44:105–108

    CAS  PubMed  Google Scholar 

  4. David A, Mercier J, Verloes A (1996) Child with manifestations of Nager acrofacial dysostosis, and the MURCS, V ACTERL, and pulmonary agenesis associations: complex defect of blastogenesis? Am J Med Genet 62:1–5

    Google Scholar 

  5. Davies R, Kozlowski K, Wood B (1989) Right-upper-lobe esophageal bronchus (with VATER anomalies). Am J Dis Child 143:251–252

    CAS  PubMed  Google Scholar 

  6. Duncan P, Shapiro L, Stangel J, Klein R, Addonizio J (1979) The MURCS association: Mullerian duct aplasia, renal aplasia, and cervicothoracic somite dysplasia. J Pediatr 95:399–402

    CAS  PubMed  Google Scholar 

  7. Harrison M, Hanson B, Mahour G, Takahashi M, Weitzman J (1977) The significance of right aortic arch in repair of esophageal atresia and tracheoesophageal fistula. J Pediatr Surg 12:861–868

    Article  CAS  PubMed  Google Scholar 

  8. Keeley J, Schairer A (1960) The anomalous origin of the right main bronchus from the esophagus. Ann Surg 152:871–874

    CAS  PubMed  Google Scholar 

  9. Kluth D (1976) Atlas of esophageal atresia. J Pediatr Surg 11:901–919

    CAS  PubMed  Google Scholar 

  10. Leithiser R, Capitanio M, Macpherson R,Wood B (1986) “Communicating” bronchopulmonary foregut malformation. Am J Roentgenol 146:227–231

    Google Scholar 

  11. Matsumoto Y, Ohi R, Hayashi Y (1995) Right pneumonectomy syndrome. Surg Today 25:278–280

    Article  CAS  PubMed  Google Scholar 

  12. Michel J, Revillon Y, Salakos C, Blic JD, Jan D, Beringer A, Scheinmann P (1997) Successful bronchotracheal reconstruction in esophageal bronchus: two case reports. J Pediatr Surg 32:739–742

    Article  CAS  PubMed  Google Scholar 

  13. Orstavik K, Steen-Johnsen J, Foerster A, Fjeld J, Skullerud K, Lie S (1992) V ACTERL or MURCS association in a girl with neurenteric cyst and identical thoracic malformations in the father: a case of gonosomal mosaicism? Am J Med Genet 43:1035–1038

    CAS  PubMed  Google Scholar 

  14. Saydam T, Mychaliska G, Harrison M (1999) Esophageal lung with multiple congenital anomalies: conundrums in diagnosis and management. J Pediatr Surg 34:615–618

    Article  CAS  PubMed  Google Scholar 

  15. Schwartz D, So H, Anban T, Creedon J (1983) Total lung sequestration in association with oesophageal atresia and multiple tracheo-oesophageal fistulae. Z Kinderchir 38:410–411

    CAS  PubMed  Google Scholar 

  16. Scorpio R, Beals D, Powell R (1998) Vaginal agenesis discovered during repair of high imperforate anus: operative management. Am Surg 64:1066–1069

    CAS  PubMed  Google Scholar 

  17. Takahashi A, Matsuyama S, Suzuki N (1989) Esophageal atresia associated with pulmonary agenesis: report of two cases and review of the literature. J Jpn Pediatr Soc 25:282–289

    Google Scholar 

  18. Teich S, Barton D, Ginn-Pease M, King D (1997) Prognostic classification for esophageal atresia and tracheoesophageal fistula: Waterston versus Montreal. J Pediatr Surg 32:1075–1080

    Article  CAS  PubMed  Google Scholar 

  19. Toyama W (1972) Esophageal atresia and tracheoesophageal fistula in association with bronchial and pulmonary anomalies. J Pediatr Surg 7:302–307

    Article  CAS  PubMed  Google Scholar 

  20. Ward C, Myers N (1999) Babies born with major disabilities: the medical-legal interface. Pediatr Surg Int 15:310–319

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Pediatric Surgery, Johannes Gutenberg University, Langenbeckstrasse 1, 55131 , Mainz, Germany

    F. Linke & S. Berger

  2. Neonatal Intensive Care Unit, Department of Pediatrics, Johannes Gutenberg University, Mainz, Germany

    W. Kraemer

  3. Department of Pediatric Pathology, Institute of Pathology, Johannes Gutenberg University, Mainz, Germany

    M. Ansorge

  4. Pediatric Radiology Unit, Johannes Gutenberg University, Mainz, Germany

    R. Brzezinska

Authors
  1. F. Linke
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  2. W. Kraemer
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  3. M. Ansorge
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  4. R. Brzezinska
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  5. S. Berger
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Correspondence to F. Linke.

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Linke, F., Kraemer, W., Ansorge, M. et al. Right esophageal lung in a preterm child with VACTERL association and Mayer-Rokitansky-Küster-Hauser syndrome. Ped Surgery Int 21, 285–288 (2005). https://doi.org/10.1007/s00383-004-1304-6

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  • DOI: https://doi.org/10.1007/s00383-004-1304-6

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