A Case of Esophageal Obstruction Complicated in a Patient with Chronic Granulomatous Disease: Esophageal obstruction in Chronic Granulomatous Disease

Ji Soo Kim; Jinsol Hwang; Young Hun Choi; Woo Sun Kim; Joong Gon Kim

doi:10.14776/kjpid.2014.21.1.53

Journal List > Korean J Pediatr Infect Dis > v.21(1) > 1096031

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Kim, Hwang, Choi, Kim, and Kim: A Case of Esophageal Obstruction Complicated in a Patient with Chronic Granulomatous Disease: Esophageal obstruction in Chronic Granulomatous Disease

Case Report

Korean Journal of Pediatric Infectious Diseases 2014; 21(1): 53-58.

Published online: 23 April 2014

DOI: https://doi.org/10.14776/kjpid.2014.21.1.53

A Case of Esophageal Obstruction Complicated in a Patient with Chronic Granulomatous Disease: Esophageal obstruction in Chronic Granulomatous Disease

Ji Soo Kim, M.D., Jinsol Hwang, M.D., Young Hun Choi, M.D.^*, Woo Sun Kim, M.D.^*, Joong Gon Kim, M.D.

Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea.

^*Department of Radiology, Seoul National University College of Medicine, Seoul, Korea.

Corresponding author (jgkim@snu.ac.kr)

Received 24 May 2013 Revised 13 July 2013 Accepted 9 September 2013

Abstract

Chronic granulomatous disease (CGD) is an inherited immunodeficient disease characterized by recurrent infections and granuloma formation. Granulomatous obstruction of esophagus is one of the rare complications of CGD. The use of steroids and antimicrobials for esophageal obstruction by granuloma in CGD patients has been controversial due to the possibility of concomitant inapparent infection. We report a case of esophageal obstruction in an 8-year-old CGD patient showing the poor response to antibiotics therapy. However, dramatic improvement of symptoms and radiologic findings of esophageal obstruction were achieved after steroid therapy. One month after discontinuation of steroid, esophageal obstruction recurred and the patient was re-treated with steroid. After that time, he experienced one more recurrence of esophageal obstruction. This symptom subsided after antibiotics therapy without steroid and he has been followed up to the present without further relapse.

Keywords: Chronic granulomatous disease, Esophageal stenosis, Steroid

Figures and Tables

Fig. 1

(A) Initial esophagogram reveals segmental stenosis at the mid-esophagus with dilatation of the proximal esophagus (arrows). (B) On esophagogram following treatment, mid-esophageal stenosis has markedly improved.

Fig. 2

Magnified view of the CT scan obtained at the level of carina shows circumferential wall thickening of the mid-esophagus (arrow).

Table 1

Response of Treatments for Esophageal Obstruction in Chronic Granulomatous Disease

Abbreviations: M, male; F, female; yr, years; m, months; wk, weeks; d, days; Pd, prednisolone; N, no recurrence; R, recurrence

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