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A case of severe Pierre Robin sequence with failure to thrive and tachycardia resolved after redo-fundoplication and hiatoplasty

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We report an infant suffering from Pierre Robin sequence complicated by gastro-oesophageal reflux and failure to thrive, which were resistant to conservative therapy and a hemifundoplication. Gastro-oesophageal reflux was accompanied by supraventricular tachycardia, treated with propafenone. Tachycardia may be present in Pierre Robin sequence as a consequence of cardiac parasympathetic imbalance. The patient recovered completely from the gastro-oesophageal reflux and tachycardia after redo-fundoplication (Nissen) and a hiatoplasty were performed. Conclusion:This case shows that a thorough search for gastro-oesophageal reflux is indicated in each case of Pierre Robin sequence with failure to thrive.

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failure to thrive


gastro-oesophageal reflux


gastro-oesophageal reflux disease


Pierre Robin sequence


  1. 1.

    Abadie V, Morisseau-Durand M-P, Beyler C, Manach Y, Couly G (2002) Brainstem dysfunction: a possible neuroembryological pathogenesis of isolated Pierre Robin sequence. Eur J Pediatr 161: 275–280

  2. 2.

    Axelrod FB, Maayan C, Hazzi C, Bougaru BS, Shannon DC (1987) Bradycardia associated with hiatal hernia and gastroesophageal reflux relieved by surgery. Am J Gastroenterol 82: 159–161

  3. 3.

    Baudon J-J, Renault F, Goutet JM, Flores-Guevara R, Soupre V, Gold F, Vazquez MP (2002) Motor dysfunction of the upper digestive tract in Pierre Robin sequence as assessed by sucking-swallowing electromyography and esophageal manometry. J Pediatr 140: 719–723

  4. 4.

    Chiaretti M, Fracassi RM, Ferrante P, Scalercio F, Principessa L, Caropreso E, Celli M, Finocchiaro R (1998) Jejunal enteral feeding in a severe case of reflux esophagitis in an infant with Pierre-Robin Syndrome. Minerva Pediatr 50: 175–178

  5. 5.

    Dudkiewicz Z, Sekula E, Jalosinska AN (2000) Gastroesophageal reflux in Pierre-Robin Sequence—early surgical treatment. Cleft Palate Craniofac J 37: 205–208

  6. 6.

    Inge TH, Carmeci C, Ohara LJ, Berquist WB, Cahill JC (1998) Outcome of Nissen fundoplication using intraoperative manometry in children. J Pediatr Surg 33: 1614–1617

  7. 7.

    Laparoscopic antireflux surgery for gastroesophageal reflux (GERD) (1997) Results of a Consensus Development Conference. Surg Endosc 11: 413–426

  8. 8.

    Marcus CL, Hamer A (1997) Significance of isolated bradycardia detected by home monitoring. J Pediatr 135: 321–326

  9. 9.

    Pandya AN, Boorman JG (2001) Failure to thrive in babies with cleft lip and palate. Br J Plast Surg 54: 471–475

  10. 10.

    Patterson EJ, Herron DM, Hansen PD (2000) Effect of an esophageal bougie on the incidence of dysphagia following Nissen fundoplication: a prospective, blinded, randomized controlled trial. Arch Surg 135: 1055–1062

  11. 11.

    Samuk I, Afriat R, Horne T, Bistritzer T, Barr J, Vinograd I (1996) Dumping syndrome following Nissen fundoplication, diagnosis and treatment. J Pediatr Gastroenterol Nutr 23: 235–240

  12. 12.

    Stoll C, Kiney JR, Dott B, Alembik Y, Finck S (1992) Ventricular extrasystoles with syncopal episodes, perodactyly, and Robin in sequence in three generations: a new inherited MCA syndrome? Am J Med Genet 42: 480–486

  13. 13.

    Vandenplas Y, Sacré-Smiths L (1987) Continuous 24-hour esophageal pH monitoring in 285 asymptomatic infants 0–15 months old. J Pediatr Gastroenterol Nutr 6: 220–224

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Correspondence to Klaus-Peter Zimmer.

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Reinshagen, K., Schellscheidt, J. & Zimmer, K. A case of severe Pierre Robin sequence with failure to thrive and tachycardia resolved after redo-fundoplication and hiatoplasty. Eur J Pediatr 164, 573–576 (2005). https://doi.org/10.1007/s00431-005-1682-3

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  • Gastro-oesophageal reflux
  • Pierre Robin sequence
  • Tachycardia