1. “All disease begins in the gut” Hippocrates
R. Proost, P. Nafteux, H. Van Veer, I. Hoffman

2. “A yellow infant” 8 months of age No prior medical history Fatigue
Deteriorating intake
Poor weight gain
P10 to P3
Guggling sound while drinking

3. Pale-yellow, white sclerae Moderate tachycardia Hemoglobin 2,7 g/dL
Alert, comfortable
Pale-yellow, white sclerae
Moderate tachycardia
Hemoglobin 2,7 g/dL
MCV 66,7 fL
Iron 13 μg/dL, ferritin 3 μg/L, transferrin saturation 3%
Peripheral blood smear
Deep microcytic hypochromic anemia
Stool occult blood 1+
BP 94/37 mmHg, pulse 162/min
Weight 7,34 kg (P3)

4. Right paracardial mass

6. Haddad C, Youssef BA, Sammak M
Haddad C, Youssef BA, Sammak M. Right intrathoracic stomach secondary to congenital hiatal hernia and organoaxial torsion. AJR:167,July 1996:66-68.

7. Treatment Paraesophageal hiatal hernia type III
2 packed cell transfusions (15 ml/kg)
PPI + histamine H2-receptor antagonists
Hemoglobin level stabilized at 11,1 g/dL
Surgery
Laparoscopic approach
Giant hiatal hernia
No other diaphragmatic defects
No short esophagus
Paraesophageal hiatal hernia type III

8. Hiatal hernia classification
Type I – sliding hernia
Type II – true paraesophageal hernia
Type III – mixed type
GEJ & fundus herniating through the hiatus
Type IV – organs other than stomach
95%
> 90% 5%

9. Fischer's Mastery of Surgery, 6e, Chapter 62. 

10. Treatment Reduction of the stomach Resection of the sac
Nissen fundoplication
Gastrostomy
Thoracic drain

11. Treatment 1 night ICU After 4 days Enteral feeding restarted - orally
Upper GI-tract series
Patent esophagus
Stable position of the stomach
Enteral feeding restarted - orally
Better than preoperatively
Gastrostomy removed - 9 days
Hemoglobin stable and gaining weight

12. Literature Unknown incidence in children Congenital - acquired
Unknown etiology – hypothesis
(A)symptomatic
GERD, vomiting, acute life-threatening events, recurrent respiratory infections, feeding problems, failure to thrive, melena, anemia, …
Complications! Eg. perforation, volvulus

13. Based on 52 articles

14. Take home messages Think of the GI tract in iron deficiency anemia
Hiatal and paraesophageal hernia
Uncommon in children
Can present insidiously
Surgery is required
To treat symptoms (eg. anemia)
To prevent complications
Preferred approach
Laparoscopic with Nissen fundoplication

15. References
Kohn GP, Price R, Demeester SR, et al. Guidelines for the Management of Hiatal Hernia. Sages 2013;(February):1-42. doi: /s
Imamoglu M, Cay A, Kosucu P, et al. Congenital paraesophageal hiatal hernia: Pitfalls in the diagnosis and treatment. J. Pediatr. Surg. 2005;40(7): doi: /j.jpedsurg
Al-Salem AH. Congenital paraesophageal hernia with intrathoracic gastric volvolus in two sisters. ISRN Surg. 2011;2011: doi: /2011/
Yousef Y, Lemoine C, St-Vil D, Emil S. Congenital paraesophageal hernia: The Montreal experience. J. Pediatr. Surg. 2015;50(9): doi: /j.jpedsurg
Karpelowsky JS, Wieselthaler N, Rode H. Primary paraesophageal hernia in children. J. Pediatr. Surg. 2006;41(9): doi: /j.jpedsurg
Carrott PW, Markar SR, Hong J, Kuppusamy MK, Koehler RP, Low DE. Iron-Deficiency Anemia Is a Common Presenting Issue with Giant Paraesophageal Hernia and Resolves Following Repair. J. Gastrointest. Surg. 2013;17(5): doi: /s
Namgoong J-M. Hiatal hernia in pediatric patients: laparoscopic versus open approaches.pdf