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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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Pediatric Surgery International (2020) 36:165–169
https://doi.org/10.1007/s00383-019-04588-w
ORIGINAL ARTICLE
Congenital intrathoracic stomach can be safely managed
laparoscopically
AlishaGupta1· BushraZia1· DhanyaMullassery1· PaoloDeCoppi1,2· StefanoGiuliani1· JosephI.Curry1·
KateM.Cross1
Accepted: 8 October 2019 / Published online: 23 October 2019
© Springer-Verlag GmbH Germany, part of Springer Nature 2019
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 conver-
sions and a low recurrence.
Keywords Hiatus hernia· Children· Intrathoracic stomach· Laparoscopy
Introduction
True ‘Congenital intrathoracic stomach’ (CIS) has been
defined as an extreme form of hiatal herniation and the entity
has been distinguished from intrathoracic stomach second-
ary to a congenitally short esophagus (CSE) or acquired
shortening of the esophagus resulting from scarring as a
result of gastro-esophageal reflux (GER) [1].
Owing to a tendency of CIS to volve and the subsequent
high mortality associated with any gastric volvulus, timely
surgical intervention is warranted [2]. The surgical strategy
for management of CIS has evolved from employing a tran-
sthoracic approach for reduction of stomach into the abdo-
men, to a laparotomy with addition of fundoplication and
gastropexy to prevent secondary GER and recurrence [1].
With the advent of minimally invasive expertise in pediatric
surgery, laparoscopic repair of CIS has been described with
success [3, 4].
We had previously reported 6 patients with CIS managed
using open surgery [1]. No other large series of true CIS
* Kate M. Cross
kate.cross@gosh.nhs.uk
Alisha Gupta
doc.alishagupta@gmail.com
Bushra Zia
neelpori.zia784@gmail.com
Dhanya Mullassery
dhanya.mullassery@gosh.nhs.uk
Paolo De Coppi
paolo.decoppi@gosh.nhs.uk
Stefano Giuliani
stefano.giuliani@gosh.nhs.uk
Joseph I. Curry
joe.curry@gosh.nhs.uk
1 Department ofSpecialist Neonatal andPediatric Surgery,
Great Ormond Street Hospital forChildren, Great Ormond
Street, LondonWC1N3JH, UK
2 Stem Cells andRegenerative Medicine Section,
Institute ofChild Health, University College London,
LondonWC1N1EH, UK
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