Abstract
Purpose
Congenital intrathoracic stomach (CIS) is an uncommon pediatric surgical diagnosis where > 2/3rd of the stomach lies within the chest through a hiatus defect. We reviewed our recent experience with this condition.
Methods
A retrospective single-center review of children with a diagnosis of CIS (2007–2018) was performed. Patient demographics, presentation, imaging and management were assessed. Results are expressed as median (range).
Results
Eleven patients (6 girls) were identified with onset of symptoms at 2 (0–26) months of age. Presenting symptoms were vomiting (8/11), respiratory symptoms (4/11) and failure to thrive (2/11). Two patients had Marfan’s syndrome. An upper gastrointestinal contrast study demonstrated gastric herniation in all. All were corrected laparoscopically with hiatus repair and fundoplication [age at surgery 10.5 (1.5–34.5) months]. A concurrent gastrostomy was done in children ≤ 6 months (n = 5). Enteral feeds were commenced on post-operative day one in 9 and second post-operative day in 2. At 7 (0–95) months follow-up, all were on full enteral feeds. One patient had a recurrence 6 months post-operatively, which was re-operated laparoscopically without any further recurrence.
Conclusion
This is the largest reported series of children with CIS. All could be managed laparoscopically with no conversions and a low recurrence.
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References
Woodward M, Crabbe DC, Kiely EM, Spitz L (1996) Congenital intrathoracic stomach. Pediatr Surg Int 11:116–118. https://doi.org/10.1007/BF00183740
Katkhouda N, Mavor E, Achanta K, Friedlander MH, Grant SW, Essani R, Mason RJ, Foster M, Mouiel J (2000) Laparoscopic repair of chronic intrathoracic gastric volvulus. Surgery 128:784–790. https://doi.org/10.1067/msy.2000.108658
Yagi M, Nose K, Yamauchi K, Nogami T, Yoshida H, Okuyama H, Kawahara H, Ohyanagi H (2003) Laparoscopic intervention for intrathoracic stomach in infants. Surg Endosc 17:1636–1639. https://doi.org/10.1007/s00464-002-8783-0
Bradley T, Stephenson J, Drugas G, Avansino JR (2010) Laparoscopic management of neonatal paraesophageal hernia with intrathoracic gastric volvulus. J Pediatr Surg 45:E21–23. https://doi.org/10.1016/j.jpedsurg.2010.05.033
Leung AW, Lam HS, Chu WC, Lee KH, Tam YH, Ng PC (2008) Congenital intrathoracic stomach: short esophagus or hiatal hernia? Neonatology 93:178–181. https://doi.org/10.1159/000108927
Deprez FC, Debauche C, Clapuyt P, de Ville de Goyet J (2009) Multiorgan developmental anomalies presenting as a variation of the serpentine-like syndrome: cervical fusion and brachioesophagus with intrathoracic stomach and malposition of duodenopancreas and spleen. J Pediatr Surg 44:E25–28. https://doi.org/10.1016/j.jpedsurg.2009.05.024
Beleza-Meireles A, Steenhaut P, Hocq C, Clapuyt P, Bernard P, Debauche C, Sznajer Y (2017) "Serpentine-like syndrome"—a very rare multiple malformation syndrome characterised by brachioesophagus and vertebral anomalies. Eur J Med Genet 60:100–104. https://doi.org/10.1016/j.ejmg.2016.11.002
Bokka S, Mohanty MK (2016) Neonatal intrathoracic stomach without gastric volvulus. Indian J Surg 78:420–421. https://doi.org/10.1007/s12262-016-1536-4
Serradilla J, Bueno A, Torre C, Gamarra EA, Romo MM, Hurtado Nava, de Saracho FB, Barrial MÁ, Cervantes MG, Santamaria ML (2018) Neonatal intrathoracic gastric volvulus in Marfan's syndrome. Eur J Pediatr Surg Rep 6:e48–e51. https://doi.org/10.1055/s-0038-1666795
Skandalakis JE, Gray SW, Ricketts R (1994) The oesophagus. In: Skandalakis JE, Gray SW (eds) Embryology for surgeons: the embryological basis for the treatment of congenital abnormalities. Williams and Wilkins, Baltimore, pp 65–112
Herman TE, Siegel MJ, Mathur A, Vachharajani A (2013) Neonatal marfan syndrome with hiatus hernia and intrathoracic stomach. J Perinatol 33:652–653. https://doi.org/10.1038/jp.2013.15
Arena F, Impellizzeri P, Antonuccio P, Montalto S, Racchiusa S, Romeo C (2009) Neonatal intrathoracic gastric volvulus in Marfan's syndrome. Minerva Pediatr 61:565–567
Robinson PN, Booms P (2001) The molecular pathogenesis of the Marfan syndrome. Cell Mol Life Sci 58:1698–1707
Peterson CM, Anderson JS, Hara AK, Carenza JW, Menias CO (2009) Volvulus of the gastrointestinal tract: appearances at multimodality imaging. Radiographics 29:1281–1293. https://doi.org/10.1148/rg.295095011
Yazici M, Karaca I, Etensel B, Temir G, Günşar C, Güçlü C, Mutaf O (2003) Paraesophageal hiatal hernias in children. Dis Esophagus 16:210–213. https://doi.org/10.1046/j.1442-2050.2003.00330.x
Daneman A, Kozlowski K (1977) Large hiatus hernias in infancy and childhood. Australas Radiol 21:133–139
van der Zee DC, Bax NM, Kramer WL, Mokhaberi B, Ure BM (2001) Laparoscopic management of a paraesophageal hernia with intrathoracic stomach in infants. Eur J Pediatr Surg 11:52–54. https://doi.org/10.1055/s-2001-12193
Petersons A, Liepina M, Spitz L (2003) Neonatal intrathoracic stomach in Marfan's syndrome: report of two cases. J Pediatr Surg 38:1663–1664. https://doi.org/10.1016/s0022-3468(03)00588-8
Krähenbühl L, Schäfer M, Farhadi J, Renzulli P, Seiler CA, Büchler MW (1998) Laparoscopic treatment of large paraesophageal hernia with totally intrathoracic stomach. J Am Coll Surg 187:231–237
Acknowledgements
Dr Kieran McHugh (Consultant, Department of radiology, Great Ormond Street Hospital, London, UK) for image review. Prof. Paolo De Coppi is supported by National Institute for Health Research (NIHR-RP-2014-04-046). All research at Great Ormond Street Hospital NHS Foundation Trust and UCL Great Ormond Street Institute of Child Health is made possible by the NIHR Great Ormond Street Hospital Biomedical Research Centre. The views expressed are those of the author (s) and not necessarily those of the NHS, the NIHR or the Department of Health.
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Gupta, A., Zia, B., Mullassery, D. et al. Congenital intrathoracic stomach can be safely managed laparoscopically. Pediatr Surg Int 36, 165–169 (2020). https://doi.org/10.1007/s00383-019-04588-w
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DOI: https://doi.org/10.1007/s00383-019-04588-w