Pediatr Gastroenterol Hepatol Nutr.  2016 Mar;19(1):71-75. 10.5223/pghn.2016.19.1.71.

Esophageal Stricture Secondary to Candidiasis in a Child with Glycogen Storage Disease 1b

• Lee KJ
• Choi SJ
• Kim WS
• Park SS
• Moon JS
• Ko JS

• KMID: 2160478
• DOI: http://doi.org/10.5223/pghn.2016.19.1.71

Abstract

Esophageal candidiasis is commonly seen in immunocompromised patients; however, candida esophagitis induced stricture is a very rare complication. We report the first case of esophageal stricture secondary to candidiasis in a glycogen storage disease (GSD) 1b child. The patient was diagnosed with GSD type 1b by liver biopsy. No mutation was found in the G6PC gene, but SLC37A4 gene sequencing revealed a compound heterozygous mutation (p.R28H and p.W107X, which was a novel mutation). The patient's absolute neutrophil count was continuously under 1,000/µL when he was over 6 years of age. He was admitted frequently for recurrent fever and infection, and frequently received intravenous antibiotics, antifungal agents. He complained of persistent dysphagia beginning at age 7 years. Esophageal stricture and multiple whitish patches were observed by endoscopy and endoscopic biopsy revealed numerous fungal hyphae consistent with candida esophagitis. He received esophageal balloon dilatation four times, and his symptoms improved.

Keyword

Glycogen storage disease; Esophageal stenosis; Candidiasis; Esophagitis; Child

MeSH Terms

Antifungal Agents
Biopsy
Candida
Candidiasis*
Child*
Constriction, Pathologic
Deglutition Disorders
Dilatation
Endoscopy
Esophageal Stenosis*
Esophagitis
Fever
Glycogen Storage Disease*
Glycogen*
Humans
Hyphae
Immunocompromised Host
Liver
Neutrophils
Antifungal Agents
Glycogen

Figure

• 

  Fig. 1 Initial esophageal stricture and candidiasis. (A) There were diffuse strictures and multiple small diverticulum in the mid-esophagus (thoracic vertebrae 3 levels) on esophagography when he was 8 years old. (B) There were multiple whitish, dense, confluent plaques throughout the esophagus on esophagogastroduodenoscopy when he was 8 years old.

• 

  Fig. 2 Mediastinal abscess on chest computed tomography (CT) and fistulous tract on esophagogram. (A) Paraesophageal abscess (arrow) and diffuse wall thickening of the upper esophagus (arrowhead) are noted on chest CT. (B) Esophagography showed a suspected esophageal fistulous track (arrowhead).

• 

  Fig. 3 Esophagogram showing diffuse stricture. Esophagography showed a diffuse narrowing of the mid-esophagus (arrowheads) and multiple concentric strictures in the upper esophagus (arrows).

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